Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).
Impairment of bile flow due to injury to the HEPATOCYTES; BILE CANALICULI; or the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC).
Passages within the liver for the conveyance of bile. Includes right and left hepatic ducts even though these may join outside the liver to form the common hepatic duct.
Impairment of bile flow in the large BILE DUCTS by mechanical obstruction or stricture due to benign or malignant processes.
A type of surgical portasystemic shunt to reduce portal hypertension with associated complications of esophageal varices and ascites. It is performed percutaneously through the jugular vein and involves the creation of an intrahepatic shunt between the hepatic vein and portal vein. The channel is maintained by a metallic stent. The procedure can be performed in patients who have failed sclerotherapy and is an additional option to the surgical techniques of portocaval, mesocaval, and splenorenal shunts. It takes one to three hours to perform. (JAMA 1995;273(23):1824-30)
A malignant tumor arising from the epithelium of the BILE DUCTS.
Gastrointestinal agents that stimulate the flow of bile into the duodenum (cholagogues) or stimulate the production of bile by the liver (choleretic).
Tumors or cancer of the BILE DUCTS.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
The channels that collect and transport the bile secretion from the BILE CANALICULI, the smallest branch of the BILIARY TRACT in the LIVER, through the bile ductules, the bile ducts out the liver, and to the GALLBLADDER for storage.
An epimer of chenodeoxycholic acid. It is a mammalian bile acid found first in the bear and is apparently either a precursor or a product of chenodeoxycholate. Its administration changes the composition of bile and may dissolve gallstones. It is used as a cholagogue and choleretic.
A tool for the study of liver damage which causes bile stasis and hyperbilirubinemia acutely and bile duct hyperplasia and biliary cirrhosis chronically, with changes in hepatocyte function. It may cause skin and kidney damage.
Steroid acids and salts. The primary bile acids are derived from cholesterol in the liver and usually conjugated with glycine or taurine. The secondary bile acids are further modified by bacteria in the intestine. They play an important role in the digestion and absorption of fat. They have also been used pharmacologically, especially in the treatment of gallstones.
Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE.
An emulsifying agent produced in the LIVER and secreted into the DUODENUM. Its composition includes BILE ACIDS AND SALTS; CHOLESTEROL; and ELECTROLYTES. It aids DIGESTION of fats in the duodenum.
Minute intercellular channels that occur between liver cells and carry bile towards interlobar bile ducts. Also called bile capillaries.
A bile pigment that is a degradation product of HEME.
Pathological processes of the LIVER.
The BILE DUCTS and the GALLBLADDER.
An intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.
FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cirrhosis involves the destruction of small intra-hepatic bile ducts and bile secretion. Secondary biliary cirrhosis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.
A subfamily of transmembrane proteins from the superfamily of ATP-BINDING CASSETTE TRANSPORTERS that are closely related in sequence to P-GLYCOPROTEIN. When overexpressed, they function as ATP-dependent efflux pumps able to extrude lipophilic drugs, especially ANTINEOPLASTIC AGENTS, from cells causing multidrug resistance (DRUG RESISTANCE, MULTIPLE). Although P-Glycoproteins share functional similarities to MULTIDRUG RESISTANCE-ASSOCIATED PROTEINS they are two distinct subclasses of ATP-BINDING CASSETTE TRANSPORTERS, and have little sequence homology.
Blood tests that are used to evaluate how well a patient's liver is working and also to help diagnose liver conditions.
Diseases in any part of the ductal system of the BILIARY TRACT from the smallest BILE CANALICULI to the largest COMMON BILE DUCT.
Abnormal increase of resistance to blood flow within the hepatic PORTAL SYSTEM, frequently seen in LIVER CIRRHOSIS and conditions with obstruction of the PORTAL VEIN.
Yellow discoloration of the SKIN; MUCOUS MEMBRANE; and SCLERA in the NEWBORN. It is a sign of NEONATAL HYPERBILIRUBINEMIA. Most cases are transient self-limiting (PHYSIOLOGICAL NEONATAL JAUNDICE) occurring in the first week of life, but some can be a sign of pathological disorders, particularly LIVER DISEASES.
Tumors or cancer of the LIVER.
A short thick vein formed by union of the superior mesenteric vein and the splenic vein.
A clinical manifestation of HYPERBILIRUBINEMIA, characterized by the yellowish staining of the SKIN; MUCOUS MEMBRANE; and SCLERA. Clinical jaundice usually is a sign of LIVER dysfunction.
Application of a ligature to tie a vessel or strangulate a part.
The main structural component of the LIVER. They are specialized EPITHELIAL CELLS that are organized into interconnected plates called lobules.
Inflammation of the biliary ductal system (BILE DUCTS); intrahepatic, extrahepatic, or both.
Jaundice, the condition with yellowish staining of the skin and mucous membranes, that is due to impaired BILE flow in the BILIARY TRACT, such as INTRAHEPATIC CHOLESTASIS, or EXTRAHEPATIC CHOLESTASIS.
Passages external to the liver for the conveyance of bile. These include the COMMON BILE DUCT and the common hepatic duct (HEPATIC DUCT, COMMON).
A bile acid formed from chenodeoxycholate by bacterial action, usually conjugated with glycine or taurine. It acts as a detergent to solubilize fats for absorption and is itself absorbed. It is used as cholagogue and choleretic.
Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.
Conditions or pathological processes associated with pregnancy. They can occur during or after pregnancy, and range from minor discomforts to serious diseases that require medical interventions. They include diseases in pregnant females, and pregnancies in females with diseases.
A group of diseases related to a deficiency of the enzyme ARGININOSUCCINATE SYNTHASE which causes an elevation of serum levels of CITRULLINE. In neonates, clinical manifestations include lethargy, hypotonia, and SEIZURES. Milder forms also occur. Childhood and adult forms may present with recurrent episodes of intermittent weakness, lethargy, ATAXIA, behavioral changes, and DYSARTHRIA. (From Menkes, Textbook of Child Neurology, 5th ed, p49)
A bile salt formed in the liver by conjugation of chenodeoxycholate with taurine, usually as the sodium salt. It acts as detergent to solubilize fats in the small intestine and is itself absorbed. It is used as a cholagogue and choleretic.
A branch of the celiac artery that distributes to the stomach, pancreas, duodenum, liver, gallbladder, and greater omentum.
An imaging test of the BILIARY TRACT in which a contrast dye (RADIOPAQUE MEDIA) is injected into the BILE DUCT and x-ray pictures are taken.
The largest bile duct. It is formed by the junction of the CYSTIC DUCT and the COMMON HEPATIC DUCT.
INFLAMMATION of the LIVER.
A major primary bile acid produced in the liver and usually conjugated with glycine or taurine. It facilitates fat absorption and cholesterol excretion.
A family of MEMBRANE TRANSPORT PROTEINS that require ATP hydrolysis for the transport of substrates across membranes. The protein family derives its name from the ATP-binding domain found on the protein.
A multisystem disorder that is characterized by aplasia of intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC), and malformations in the cardiovascular system, the eyes, the vertebral column, and the facies. Major clinical features include JAUNDICE, and congenital heart disease with peripheral PULMONARY STENOSIS. Alagille syndrome may result from heterogeneous gene mutations, including mutations in JAG1 on CHROMOSOME 20 (Type 1) and NOTCH2 on CHROMOSOME 1 (Type 2).
The transference of a part of or an entire liver from one human or animal to another.
Veins which drain the liver.
Surgical venous shunt between the portal and systemic circulation to effect decompression of the portal circulation. It is performed primarily in the treatment of bleeding esophageal varices resulting from portal hypertension. Types of shunt include portacaval, splenorenal, mesocaval, splenocaval, left gastric-caval (coronary-caval), portarenal, umbilicorenal, and umbilicocaval.
Diseases in any part of the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.
Excision of all or part of the liver. (Dorland, 28th ed)
A semisynthetic alkylated ESTRADIOL with a 17-alpha-ethinyl substitution. It has high estrogenic potency when administered orally, and is often used as the estrogenic component in ORAL CONTRACEPTIVES.
The circulation of BLOOD through the LIVER.
A synthetic hormone with anabolic and androgenic properties and moderate progestational activity.
A primary malignant neoplasm of epithelial liver cells. It ranges from a well-differentiated tumor with EPITHELIAL CELLS indistinguishable from normal HEPATOCYTES to a poorly differentiated neoplasm. The cells may be uniform or markedly pleomorphic, or form GIANT CELLS. Several classification schemes have been suggested.
A system of vessels in which blood, after passing through one capillary bed, is conveyed through a second set of capillaries before it returns to the systemic circulation. It pertains especially to the hepatic portal system.
A plant genus of the family Lamiaceae. The species of Coleus should be distinguished from PLECTRANTHUS BARBATUS - which is also known as Coleus forskohlii.
A condition in which the hepatic venous outflow is obstructed anywhere from the small HEPATIC VEINS to the junction of the INFERIOR VENA CAVA and the RIGHT ATRIUM. Usually the blockage is extrahepatic and caused by blood clots (THROMBUS) or fibrous webs. Parenchymal FIBROSIS is uncommon.
Proteins involved in the transport of organic anions. They play an important role in the elimination of a variety of endogenous substances, xenobiotics and their metabolites from the body.
A condition characterized by an abnormal increase of BILIRUBIN in the blood, which may result in JAUNDICE. Bilirubin, a breakdown product of HEME, is normally excreted in the BILE or further catabolized before excretion in the urine.
The product of conjugation of cholic acid with taurine. Its sodium salt is the chief ingredient of the bile of carnivorous animals. It acts as a detergent to solubilize fats for absorption and is itself absorbed. It is used as a cholagogue and cholerectic.
Fiberoptic endoscopy designed for duodenal observation and cannulation of VATER'S AMPULLA, in order to visualize the pancreatic and biliary duct system by retrograde injection of contrast media. Endoscopic (Vater) papillotomy (SPHINCTEROTOMY, ENDOSCOPIC) may be performed during this procedure.
Severe inability of the LIVER to perform its normal metabolic functions, as evidenced by severe JAUNDICE and abnormal serum levels of AMMONIA; BILIRUBIN; ALKALINE PHOSPHATASE; ASPARTATE AMINOTRANSFERASE; LACTATE DEHYDROGENASES; and albumin/globulin ratio. (Blakiston's Gould Medical Dictionary, 4th ed)
Imino acids are organic compounds containing a nitrogen atom in their structure, classified as derivatives of amino acids, where the carbon atom adjacent to the carboxyl group is bonded to a nitrogen atom instead of a hydrogen atom, forming a characteristic imino functional group.
A condition characterized by the formation of CALCULI and concretions in the hollow organs or ducts of the body. They occur most often in the gallbladder, kidney, and lower urinary tract.
An enzyme, sometimes called GGT, with a key role in the synthesis and degradation of GLUTATHIONE; (GSH, a tripeptide that protects cells from many toxins). It catalyzes the transfer of the gamma-glutamyl moiety to an acceptor amino acid.
A metabolite of 17-ALPHA-HYDROXYPROGESTERONE, normally produced in small quantities by the GONADS and the ADRENAL GLANDS, found in URINE. An elevated urinary pregnanetriol is associated with CONGENITAL ADRENAL HYPERPLASIA with a deficiency of STEROID 21-HYDROXYLASE.
A spectrum of clinical liver diseases ranging from mild biochemical abnormalities to ACUTE LIVER FAILURE, caused by drugs, drug metabolites, and chemicals from the environment.
Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
An infant during the first month after birth.
Enlargement of the liver.
Presence or formation of GALLSTONES in the BILIARY TRACT, usually in the gallbladder (CHOLECYSTOLITHIASIS) or the common bile duct (CHOLEDOCHOLITHIASIS).
An enzyme that catalyzes the conversion of L-alanine and 2-oxoglutarate to pyruvate and L-glutamate. (From Enzyme Nomenclature, 1992) EC 2.6.1.2.
Dilated blood vessels in the ESOPHAGUS or GASTRIC FUNDUS that shunt blood from the portal circulation (PORTAL SYSTEM) to the systemic venous circulation. Often they are observed in individuals with portal hypertension (HYPERTENSION, PORTAL).
Tumors or cancer in the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.
A subclass of ORGANIC ANION TRANSPORTERS whose transport of organic anions is driven either directly or indirectly by a gradient of sodium ions.
Adenocarcinoma of the common hepatic duct bifurcation. These tumors are generally small, sharply localized, and seldom metastasizing. G. Klatskin's original review of 13 cases was published in 1965. Once thought to be relatively uncommon, tumors of the bifurcation of the bile duct now appear to comprise more than one-half of all bile duct cancers. (From Holland et al., Cancer Medicine, 3d ed, p1457)
Examination of the portal circulation by the use of X-ray films after injection of radiopaque material.
An abnormal lipoprotein present in large amounts in patients with obstructive liver diseases such as INTRAHEPATIC CHOLESTASIS. LP-X derives from the reflux of BILE lipoproteins into the bloodstream. LP-X is a low-density lipoprotein rich in free CHOLESTEROL and PHOSPHOLIPIDS but poor in TRIGLYCERIDES; CHOLESTEROL ESTERS; and protein.
A benign tumor of the intrahepatic bile ducts.
Persistent flexure or contracture of a joint.
Abnormal passage in any organ of the biliary tract or between biliary organs and other organs.
Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
The venous pressure measured in the PORTAL VEIN.
Predominantly extrahepatic bile duct which is formed by the junction of the right and left hepatic ducts, which are predominantly intrahepatic, and, in turn, joins the cystic duct to form the common bile duct.
A tricyclic antidepressant with some tranquilizing action.
The administering of nutrients for assimilation and utilization by a patient who cannot maintain adequate nutrition by enteral feeding alone. Nutrients are administered by a route other than the alimentary canal (e.g., intravenously, subcutaneously).
A phenolphthalein that is used as a diagnostic aid in hepatic function determination.
Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.
Any fluid-filled closed cavity or sac that is lined by an EPITHELIUM. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.
The 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholanic acid family of bile acids in man, usually conjugated with glycine or taurine. They act as detergents to solubilize fats for intestinal absorption, are reabsorbed by the small intestine, and are used as cholagogues and choleretics.
Inbred C57BL mice are a strain of laboratory mice that have been produced by many generations of brother-sister matings, resulting in a high degree of genetic uniformity and homozygosity, making them widely used for biomedical research, including studies on genetics, immunology, cancer, and neuroscience.
Accumulation or retention of free fluid within the peritoneal cavity.
A bile salt formed in the liver from lithocholic acid conjugation with taurine, usually as the sodium salt. It solubilizes fats for absorption and is itself absorbed. It is a cholagogue and choleretic.
A bile salt formed in the liver from chenodeoxycholate and glycine, usually as the sodium salt. It acts as a detergent to solubilize fats for absorption and is itself absorbed. It is a cholagogue and choleretic.
A radiopharmaceutical used extensively in cholescintigraphy for the evaluation of hepatobiliary diseases. (From Int Jrnl Rad Appl Inst 1992;43(9):1061-4)
Solid crystalline precipitates in the BILIARY TRACT, usually formed in the GALLBLADDER, resulting in the condition of CHOLELITHIASIS. Gallstones, derived from the BILE, consist mainly of calcium, cholesterol, or bilirubin.
The delivery of nutrients for assimilation and utilization by a patient whose sole source of nutrients is via solutions administered intravenously, subcutaneously, or by some other non-alimentary route. The basic components of TPN solutions are protein hydrolysates or free amino acid mixtures, monosaccharides, and electrolytes. Components are selected for their ability to reverse catabolism, promote anabolism, and build structural proteins.
Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.
Enzymes of the transferase class that catalyze the conversion of L-aspartate and 2-ketoglutarate to oxaloacetate and L-glutamate. EC 2.6.1.1.
Agents capable of exerting a harmful effect on the body.
Experimentally induced chronic injuries to the parenchymal cells in the liver to achieve a model for LIVER CIRRHOSIS.
Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.
FIBROSIS of the hepatic parenchyma due to chronic excess ALCOHOL DRINKING.
Intracellular receptors that can be found in the cytoplasm or in the nucleus. They bind to extracellular signaling molecules that migrate through or are transported across the CELL MEMBRANE. Many members of this class of receptors occur in the cytoplasm and are transported to the CELL NUCLEUS upon ligand-binding where they signal via DNA-binding and transcription regulation. Also included in this category are receptors found on INTRACELLULAR MEMBRANES that act via mechanisms similar to CELL SURFACE RECEPTORS.
A bile acid, usually conjugated with either glycine or taurine. It acts as a detergent to solubilize fats for intestinal absorption and is reabsorbed by the small intestine. It is used as cholagogue, a choleretic laxative, and to prevent or dissolve gallstones.
The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.
Diseases of newborn infants present at birth (congenital) or developing within the first month of birth. It does not include hereditary diseases not manifesting at birth or within the first 30 days of life nor does it include inborn errors of metabolism. Both HEREDITARY DISEASES and METABOLISM, INBORN ERRORS are available as general concepts.
Bleeding in any segment of the GASTROINTESTINAL TRACT from ESOPHAGUS to RECTUM.
A storage reservoir for BILE secretion. Gallbladder allows the delivery of bile acids at a high concentration and in a controlled manner, via the CYSTIC DUCT to the DUODENUM, for degradation of dietary lipid.
A chlorinated epoxy compound used as an industrial solvent. It is a strong skin irritant and carcinogen.
A plant genus of the family RUBIACEAE. Members contain antimalarial (ANTIMALARIALS) and analgesic (ANALGESICS) indole alkaloids.
A syndrome characterized by central nervous system dysfunction in association with LIVER FAILURE, including portal-systemic shunts. Clinical features include lethargy and CONFUSION (frequently progressing to COMA); ASTERIXIS; NYSTAGMUS, PATHOLOGIC; brisk oculovestibular reflexes; decorticate and decerebrate posturing; MUSCLE SPASTICITY; and bilateral extensor plantar reflexes (see REFLEX, BABINSKI). ELECTROENCEPHALOGRAPHY may demonstrate triphasic waves. (From Adams et al., Principles of Neurology, 6th ed, pp1117-20; Plum & Posner, Diagnosis of Stupor and Coma, 3rd ed, p222-5)
A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.
An inactive metabolite of PROGESTERONE by reduction at C5, C3, and C20 position. Pregnanediol has two hydroxyl groups, at 3-alpha and 20-alpha. It is detectable in URINE after OVULATION and is found in great quantities in the pregnancy urine.
Lipid infiltration of the hepatic parenchymal cells resulting in a yellow-colored liver. The abnormal lipid accumulation is usually in the form of TRIGLYCERIDES, either as a single large droplet or multiple small droplets. Fatty liver is caused by an imbalance in the metabolism of FATTY ACIDS.
Emulsions of fats or lipids used primarily in parenteral feeding.
The glycine conjugate of CHOLIC ACID. It acts as a detergent to solubilize fats for absorption and is itself absorbed.
Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.
Any surgical procedure performed on the biliary tract.
Intradermal injection of a heated (pasteurized) saline suspension of sarcoid tissue obtained from a sarcoid spleen or lymph node. In patients with active sarcoidosis a dusky red nodule develops slowly over the next few weeks at the injection site. Histologic examination, an essential part of the complete test, reveals sarcoid tissue.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
An enzyme that catalyzes the conversion of an orthophosphoric monoester and water to an alcohol and orthophosphate. EC 3.1.3.1.
A sequence-related subfamily of ATP-BINDING CASSETTE TRANSPORTERS that actively transport organic substrates. Although considered organic anion transporters, a subset of proteins in this family have also been shown to convey drug resistance to neutral organic drugs. Their cellular function may have clinical significance for CHEMOTHERAPY in that they transport a variety of ANTINEOPLASTIC AGENTS. Overexpression of proteins in this class by NEOPLASMS is considered a possible mechanism in the development of multidrug resistance (DRUG RESISTANCE, MULTIPLE). Although similar in function to P-GLYCOPROTEINS, the proteins in this class share little sequence homology to the p-glycoprotein family of proteins.
INFLAMMATION of the LIVER in non-human animals.
A liver microsomal cytochrome P450 enzyme that catalyzes the 12-alpha-hydroxylation of a broad spectrum of sterols in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP8B1gene, converts 7-alpha-hydroxy-4-cholesten-3-one to 7-alpha-12-alpha-dihydroxy-4-cholesten-3-one and is required in the synthesis of BILE ACIDS from cholesterol.
INFLAMMATION of the LIVER due to ALCOHOL ABUSE. It is characterized by NECROSIS of HEPATOCYTES, infiltration by NEUTROPHILS, and deposit of MALLORY BODIES. Depending on its severity, the inflammatory lesion may be reversible or progress to LIVER CIRRHOSIS.
Elements of limited time intervals, contributing to particular results or situations.
Diseases of the GALLBLADDER. They generally involve the impairment of BILE flow, GALLSTONES in the BILIARY TRACT, infections, neoplasms, or other diseases.
A characteristic symptom complex.
Surgical portasystemic shunt between the portal vein and inferior vena cava.
Membrane proteins whose primary function is to facilitate the transport of molecules across a biological membrane. Included in this broad category are proteins involved in active transport (BIOLOGICAL TRANSPORT, ACTIVE), facilitated transport and ION CHANNELS.
Agents, usually topical, that relieve itching (pruritus).
A benign neoplasm derived from glandular epithelium, in which cystic accumulations of retained secretions are formed. In some instances, considerable portions of the neoplasm, or even the entire mass, may be cystic. (Stedman, 25th ed)
INFLAMMATION of the LIVER in humans due to infection by VIRUSES. There are several significant types of human viral hepatitis with infection caused by enteric-transmission (HEPATITIS A; HEPATITIS E) or blood transfusion (HEPATITIS B; HEPATITIS C; and HEPATITIS D).
Infection of the biliary passages with CLONORCHIS SINENSIS, also called Opisthorchis sinensis. It may lead to inflammation of the biliary tract, proliferation of biliary epithelium, progressive portal fibrosis, and sometimes bile duct carcinoma. Extension to the liver may lead to fatty changes and cirrhosis. (From Dorland, 27th ed)
Operation for biliary atresia by anastomosis of the bile ducts into the jejunum or duodenum.
INFLAMMATION of the LIVER in animals due to viral infection.
A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.
A subclass of enzymes of the transferase class that catalyze the transfer of an amino group from a donor (generally an amino acid) to an acceptor (generally a 2-keto acid). Most of these enzymes are pyridoxyl phosphate proteins. (Dorland, 28th ed) EC 2.6.1.
A 21-carbon steroid that is converted from PREGNENOLONE by STEROID 17-ALPHA-HYDROXYLASE. It is an intermediate in the delta-5 pathway of biosynthesis of GONADAL STEROID HORMONES and the adrenal CORTICOSTEROIDS.
Specialized phagocytic cells of the MONONUCLEAR PHAGOCYTE SYSTEM found on the luminal surface of the hepatic sinusoids. They filter bacteria and small foreign proteins out of the blood, and dispose of worn out red blood cells.
One of the CEPHALOSPORINS that has a broad spectrum of activity against both gram-positive and gram-negative microorganisms.
Tomography using x-ray transmission and a computer algorithm to reconstruct the image.
The return of a sign, symptom, or disease after a remission.
Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care. (Dictionary of Health Services Management, 2d ed)
Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery.
The transference of pancreatic islets within an individual, between individuals of the same species, or between individuals of different species.
A congenital anatomic malformation of a bile duct, including cystic dilatation of the extrahepatic bile duct or the large intrahepatic bile duct. Classification is based on the site and type of dilatation. Type I is most common.

ANIT-induced disruption of biliary function in rat hepatocyte couplets. (1/294)

alpha-Naphthylisothiocyanate (ANIT) induces intrahepatic cholestasis in rats, involving damage to biliary epithelial cells; our study aims to investigate whether disruption of biliary function in hepatocytes can contribute to early stages of ANIT-induced intrahepatic cholestasis. Isolated rat hepatocyte couplets were used to investigate biliary function in vitro by canalicular vacuolar accumulation (cVA) of a fluorescent bile acid analogue, cholyl-lysyl-fluorescein (CLF), within the canalicular vacuole between the two cells. After a 2-h exposure to ANIT, there was a concentration-dependent inhibition of cVA (cVA-IC50; 25 microM), but no cytotoxicity (LDH leakage or [ATP] decline) within this ANIT concentration range. There was no loss of cellular [GSH] at low ANIT concentrations, but, at 50 microM ANIT, a small but significant loss of [GSH] had occurred. Diethylmaleate (DEM) partially depleted cellular [GSH], but addition of 10 microM ANIT had no further effect on GSH depletion. Reduction in cVA was seen in DEM-treated cells; addition of ANIT to these cells reduced cVA further, but the magnitude of this further reduction was no greater than that caused by ANIT alone, indicating that glutathione depletion does not enhance the effect of ANIT. F-actin distribution (by phalloidin-FITC staining) showed an increased frequency of morphological change in the canalicular vacuoles but only a small, non-significant (0.05 < p < 0.1) increase in proportion of the F-actin in the region of the pericanalicular web. The results are in accord with a disruption of hepatocyte canalicular secretion within two h in vitro, at low, non-cytotoxic concentrations of ANIT, and the possible involvement of a thiocabamoyl-GSH conjugate of ANIT (GS-ANIT) in this effect.  (+info)

Bile acid patterns in meconium are influenced by cholestasis of pregnancy and not altered by ursodeoxycholic acid treatment. (2/294)

BACKGROUND: Data on meconium bile acid composition in newborn babies of patients with intrahepatic cholestasis of pregnancy (ICP) are relatively scant, and changes that occur on ursodeoxycholic acid (UDCA) administration have not been evaluated. AIMS: To investigate bile acid profiles in meconium of neonates from untreated and UDCA treated patients with ICP. Maternal serum bile acid composition was also analysed both at diagnosis and delivery to determine whether this influences the concentration and proportion of bile acids in the meconium. PATIENTS/METHODS: The population included eight healthy pregnant women and 16 patients with ICP, nine of which received UDCA (12.5-15.0 mg/kg body weight/day) for 15+/-4 days until parturition. Bile acids were assessed in the meconium by gas chromatography-mass spectrometry and in maternal serum by high performance liquid chromatography. RESULTS: Total bile acid and cholic acid concentrations in the meconium were increased (p<0.01) in newborns from patients with ICP (13.5 (5.1) and 8.4 (4.1) micromol/g respectively; mean (SEM)) as compared with controls (2.0 (0.5) and 0.8 (0.3) micromol/g respectively), reflecting the total bile acid and cholic acid levels in the maternal serum (r = 0.85 and r = 0.84, p<0.01). After UDCA administration, total bile acid concentrations decreased in the mother ( approximately 3-fold, p<0. 05) but not in the meconium. UDCA concentration in the meconium showed only a 2-fold increase after treatment, despite the much greater increase in the maternal serum (p<0.01). Lithocholic acid concentration in the meconium was not increased by UDCA treatment. CONCLUSIONS: UDCA administration does not influence the concentration and proportion of bile acids in the meconium, which in turn are altered by ICP. Moreover, this beneficial treatment for the mother does not increase meconium levels of potentially toxic metabolites of UDCA such as lithocholic acid.  (+info)

Sensorineural hearing loss associated with Byler disease. (3/294)

Progressive familial intrahepatic cholestasis, sometimes described as Byler disease, is a lethal liver disease and its inheritance is autosomal recessive. There is a previous report on the occasional association between this disease and sensorineural hearing loss without any audiological findings. We report here two siblings, an 18-year-old female and a 16-year-old male, suffering from Byler disease and hearing loss. Pure tone, Bekesy and speech audiometries and auditory brain stem response examination were performed. Audiometric data showed hearing characteristics of cochlear origin, high-frequency loss and progressiveness. This sensorineural hearing loss possibly results from a genetic mutation. The mechanism of cochlear disorder in patients with Byler disease is unknown, however, a novel gene responsible for deafness might be found to be related to Byler disease.  (+info)

Review article: mechanisms of action and therapeutic applications of ursodeoxycholic acid in chronic liver diseases. (4/294)

Ursodeoxycholic acid (ursodiol) is a non-toxic, hydrophilic bile acid used to treat predominantly cholestatic liver disorders. Better understanding of the cellular and molecular mechanisms of action of ursodeoxycholic acid has helped to elucidate its cytoprotective, anti-apoptotic, immunomodulatory and choleretic effects. Ursodeoxycholic acid prolongs survival in primary biliary cirrhosis and it improves biochemical parameters of cholestasis in various other cholestatic disorders including primary sclerosing cholangitis, intrahepatic cholestasis of pregnancy, cystic fibrosis and total parenteral nutrition-induced cholestasis. However, a positive effect on survival remains to be established in these diseases. Ursodeoxycholic acid is of unproven efficacy in non-cholestatic disorders such as acute rejection after liver transplantation, non-alcoholic steatohepatitis, alcoholic liver disease and chronic viral hepatitis. This review outlines the present knowledge of the modes of action of ursodeoxycholic acid, and presents data from clinical trials on its use in chronic liver diseases.  (+info)

Manganese-bilirubin effect on cholesterol accumulation in rat bile canalicular membranes. (5/294)

Manganese-bilirubin (Mn-BR)-induced cholestasis in rats is associated with altered lipid composition of various hepatic subcellular fractions. Increased bile canalicular (BCM) cholesterol content in Mn-BR cholestasis and the intracellular source of the accumulating cholesterol were investigated. To label the total hepatic cholesterol pool, male Sprague-Dawley rats were given ip 3H-cholesterol, followed 18 h later by 2-14C-mevalonic acid (a precursor of cholesterol synthesis). To induce cholestasis, manganese (Mn, 4.5 mg/kg) and bilirubin (BR, 25 mg/kg) were injected iv; animals were killed 30 min after BR injection; canalicular and sinusoidal membranes, microsomes, mitochondria, and cytosol were isolated. Total cholesterol content of each fraction was determined by spectrophotometric techniques as well as radiolabeled techniques. In Mn-BR cholestasis, the total cholesterol concentrations of BCM and cytosol were significantly increased. Also, the contribution of 14C-labeled cholesterol (newly synthesized cholesterol) was enhanced in all isolated cellular fractions. The results are consistent with the hypothesis that accumulation of newly synthesized cholesterol in BCM is involved in Mn-BR cholestasis. An enhanced rate of synthesis of cholesterol, however, does not appear to be the causal event, as the activity of HMG-CoA reductase (rate-limiting enzyme in cholesterol synthesis), assessed in vitro, was decreased following Mn-BR treatment. Treatment with the Mn-BR combination may affect other aspects of intracellular cholesterol dynamics.  (+info)

Fibrosing cholestatic hepatitis: a report of three cases. (6/294)

Fibrosing cholestatic hepatitis is an aggressive and usually fatal form of viral hepatitis in immunosuppressed patients. We report three cases of fibrosing cholestatic hepatitis in various clinical situations. Case 1 was a 50-year-old man who underwent a liver transplant for hepatitis B virus (HBV)-associated liver cirrhosis. Two and a half years after the transplant, he complained of fever and jaundice, and liver enzymes were slightly elevated. Serum HBsAg was positive. Case 2 was a 30-year-old man in an immunosuppressed state after chemotherapy for acute lymphoblastic leukemia. He was a HBV carrier. Liver enzymes and total bilirubin were markedly elevated. Case 3 was a 50-year-old man who underwent renal transplantation as a known HBV carrier. One year after the transplant, jaundice developed abruptly, but liver enzymes were not significantly elevated. Microscopically lobules were markedly disarrayed, showing ballooning degeneration of hepatocytes, prominent pericellular fibrosis, and marked canalicular or intracytoplasmic cholestasis. Portal inflammation was mild, but interphase activity was definite and cholangiolar proliferation was prominent. Hepatocytes were diffusely positive for HBsAg and HBcAg in various patterns. Patients died of liver failure within 1 to 3 months after liver biopsy in spite of anti-viral treatment.  (+info)

Plasma antioxidant levels in chronic cholestatic liver diseases. (7/294)

BACKGROUND: [corrected] A predictable consequence of cholestasis is malabsorption of fat-soluble factors, (vitamins A, D, E, K) and other free radical scavengers, such as carotenoids. It has been suggested that oxygen-derived free radicals may be involved in the pathogenesis of chronic liver damage. AIMS: (i) To evaluate retinol, alpha-tocopherol and carotenoid plasma levels in two groups of patients with chronic cholestatic liver disease (primary biliary cirrhosis and primary sclerosing cholangitis); (ii) to compare the respective plasma levels with those of the general population; (iii) to correlate the plasma levels with disease severity. METHODS: A total of 105 patients with chronic cholestasis were included in the study: 86 with primary biliary cirrhosis (81 female, five male, mean age 55.5 +/- 11 years), 19 with primary sclerosing cholangitis (seven female, 12 male, mean age 35 +/- 11 years; six patients had associated inflammatory bowel disease); 105 sex- and age-matched subjects from the general population in the same geographical area (88 female, 17 male, mean age 51.3.5 +/- 10 years) served as controls. Carotenoids (lutein zeaxanthin, lycopene, beta-carotene, alpha-carotene, beta-cryptoxanthin), retinol and alpha-tocopherol were assayed by high-pressure liquid chromatography. A food frequency questionnaire was administered to each subject to evaluate the quality and the quantity of dietary compounds. Data were processed by analysis of variance and linear regression analysis, as appropriate. RESULTS: Both primary biliary cirrhosis and primary sclerosing cholangitis patients had significantly lower levels of retinol, alpha-tocopherol, total carotenoids, lutein, zeaxanthin, lycopene, alpha- and beta-carotene than controls (P < 0.0001). Among the cholestatic patients, no significant difference in the concentration of antioxidants was observed between primary biliary cirrhosis and primary sclerosing cholangitis subjects. Anti-oxidant plasma levels were not affected by the severity of the histological stage in primary biliary cirrhosis, but a negative correlation was found between total carotenoids and both alkaline phosphatase (ALP) and gammaglutamyl transpeptidase (GGT) (P < 0.013 and P < 0.018, respectively). Within the primary sclerosing cholangitis group, no correlation was found between total carotenoids and cholestatic enzymes. Nutritional intake in cholestatic patients was comparable to controls, including fruit and vegetable intake. CONCLUSIONS: Although no clinical sign of deficiency is evident, plasma levels of antioxidants are low in cholestatic patients even in early stages of the disease. This is probably due to malabsorption of fat-soluble vitamins, as well as other mechanisms of hepatic release, suggesting the need for dietary supplementation.  (+info)

Heterozygous MDR3 missense mutation associated with intrahepatic cholestasis of pregnancy: evidence for a defect in protein trafficking. (8/294)

Intrahepatic cholestasis of pregnancy (ICP) is a liver disease of pregnancy with serious consequences for the mother and fetus. Two pedigrees have been reported with ICP in the mothers of children with a subtype of autosomal recessive progressive familial intrahepatic cholestasis (PFIC) with raised serum gamma-glutamyl transpeptidase (gamma-GT). Affected children have homozygous mutations in the MDR3 gene (also called ABCB4 ), and heterozygous mothers have ICP. More frequently, however, ICP occurs in women with no known family history of PFIC and the genetic basis of this disorder is unknown. We investigated eight women with ICP and raised serum gamma-GT, but with no known family history of PFIC. DNA sequence analysis revealed a C to A transversion in codon 546 in exon 14 of MDR3 in one patient, which results in the missense substitution of the wild-type alanine with an aspartic acid. We performed functional studies of this mutation introduced into MDR1, a closely related homologue of MDR3. Fluorescence activated cell sorting (FACS) and western analysis indicated that this missense mutation causes disruption of protein trafficking with a subsequent lack of functional protein at the cell surface. The demonstration of a heterozygous missense mutation in the MDR3 gene in a patient with ICP with no known family history of PFIC, analysed by functional studies, is a novel finding. This shows that MDR3 mutations are responsible for the additional phenotype of ICP in a subgroup of women with raised gamma-GT.  (+info)

Cholestasis is a medical condition characterized by the interruption or reduction of bile flow from the liver to the small intestine. Bile is a digestive fluid produced by the liver that helps in the breakdown and absorption of fats. When the flow of bile is blocked or reduced, it can lead to an accumulation of bile components, such as bilirubin, in the blood, which can cause jaundice, itching, and other symptoms.

Cholestasis can be caused by various factors, including liver diseases (such as hepatitis, cirrhosis, or cancer), gallstones, alcohol abuse, certain medications, pregnancy, and genetic disorders. Depending on the underlying cause, cholestasis may be acute or chronic, and it can range from mild to severe in its symptoms and consequences. Treatment for cholestasis typically involves addressing the underlying cause and managing the symptoms with supportive care.

Intrahepatic cholestasis is a medical condition characterized by the interruption or reduction of bile flow within the liver. Bile is a digestive fluid produced by the liver that helps in the absorption of fats and fat-soluble vitamins. Intrahepatic cholestasis occurs when there is a problem with the transport of bile components inside the liver cells (hepatocytes). This can lead to an accumulation of bile acids, bilirubin, and other substances in the liver, which can cause damage to liver cells and result in symptoms such as jaundice, itching, and dark urine.

Intrahepatic cholestasis can be caused by various factors, including medications, alcohol abuse, hepatitis viruses, autoimmune disorders, genetic defects, and cancer. Depending on the underlying cause, intrahepatic cholestasis can be acute or chronic, and it can range from mild to severe. Treatment typically involves addressing the underlying cause of the condition, as well as providing supportive care to manage symptoms and prevent complications.

Intrahepatic bile ducts are the small tubular structures inside the liver that collect bile from the liver cells (hepatocytes). Bile is a digestive fluid produced by the liver that helps in the absorption of fats and fat-soluble vitamins from food. The intrahepatic bile ducts merge to form larger ducts, which eventually exit the liver and join with the cystic duct from the gallbladder to form the common bile duct. The common bile duct then empties into the duodenum, the first part of the small intestine, where bile aids in digestion. Intrahepatic bile ducts can become obstructed or damaged due to various conditions such as gallstones, tumors, or inflammation, leading to complications like jaundice, liver damage, and infection.

Extrahepatic cholestasis is a medical condition characterized by the impaired flow of bile outside of the liver. Bile is a digestive fluid produced by the liver that helps in the absorption and digestion of fats. When the flow of bile is obstructed or blocked, it can lead to an accumulation of bile components, such as bilirubin, in the bloodstream, resulting in jaundice, dark urine, light-colored stools, and itching.

Extrahepatic cholestasis can be caused by various factors, including gallstones, tumors, strictures, or inflammation of the bile ducts. It is essential to diagnose and treat extrahepatic cholestasis promptly to prevent further complications, such as liver damage or infection. Treatment options may include medications, endoscopic procedures, or surgery, depending on the underlying cause of the condition.

A Transjugular Intrahepatic Portosystemic Shunt (TIPS) is a medical procedure that creates an alternative pathway for blood flow from the portal vein to the hepatic vein within the liver. This shunt is composed of a stent, which is a small metal tube that is inserted into the liver using a long needle that is passed through a vein in the neck (jugular vein).

TIPS is typically used to treat complications of portal hypertension, such as variceal bleeding, ascites, and hepatic hydrothorax. By creating a shunt that bypasses the liver, TIPS reduces the pressure in the portal vein, which can help to alleviate these symptoms. However, because the shunt allows blood to bypass the liver, it can also impair liver function and lead to other complications, such as hepatic encephalopathy.

It is important to note that TIPS is a complex procedure that should only be performed by experienced interventional radiologists in a hospital setting with appropriate medical backup and monitoring capabilities.

Cholangiocarcinoma is a type of cancer that arises from the cells that line the bile ducts, which are small tubes that carry digestive enzymes from the liver to the small intestine. It can occur in different parts of the bile duct system, including the bile ducts inside the liver (intrahepatic), the bile ducts outside the liver (extrahepatic), and the area where the bile ducts join the pancreas and small intestine (ampulla of Vater).

Cholangiocarcinoma is a relatively rare cancer, but its incidence has been increasing in recent years. It can be difficult to diagnose because its symptoms are often nonspecific and similar to those of other conditions, such as gallstones or pancreatitis. Treatment options depend on the location and stage of the cancer, and may include surgery, radiation therapy, chemotherapy, or a combination of these approaches.

Cholagogues and choleretics are terms used to describe medications or substances that affect bile secretion and flow in the body. Here is a medical definition for each:

1. Cholagogue: A substance that promotes the discharge of bile from the gallbladder into the duodenum, often by stimulating the contraction of the gallbladder muscle. This helps in the digestion and absorption of fats. Examples include chenodeoxycholic acid, ursodeoxycholic acid, and some herbal remedies like dandelion root and milk thistle.
2. Choleretic: A substance that increases the production of bile by the liver or its flow through the biliary system. This can help with the digestion of fats and the elimination of waste products from the body. Examples include certain medications like ursodeoxycholic acid, as well as natural substances such as lemon juice, artichoke extract, and turmeric.

It is important to note that while cholagogues and choleretics can aid in digestion, they should be used under the guidance of a healthcare professional, as improper use or overuse may lead to complications like diarrhea or gallstone formation.

Bile duct neoplasms, also known as cholangiocarcinomas, refer to a group of malignancies that arise from the bile ducts. These are the tubes that carry bile from the liver to the gallbladder and small intestine. Bile duct neoplasms can be further classified based on their location as intrahepatic (within the liver), perihilar (at the junction of the left and right hepatic ducts), or distal (in the common bile duct).

These tumors are relatively rare, but their incidence has been increasing in recent years. They can cause a variety of symptoms, including jaundice, abdominal pain, weight loss, and fever. The diagnosis of bile duct neoplasms typically involves imaging studies such as CT or MRI scans, as well as blood tests to assess liver function. In some cases, a biopsy may be necessary to confirm the diagnosis.

Treatment options for bile duct neoplasms depend on several factors, including the location and stage of the tumor, as well as the patient's overall health. Surgical resection is the preferred treatment for early-stage tumors, while chemotherapy and radiation therapy may be used in more advanced cases. For patients who are not candidates for surgery, palliative treatments such as stenting or bypass procedures may be recommended to relieve symptoms and improve quality of life.

The liver is a large, solid organ located in the upper right portion of the abdomen, beneath the diaphragm and above the stomach. It plays a vital role in several bodily functions, including:

1. Metabolism: The liver helps to metabolize carbohydrates, fats, and proteins from the food we eat into energy and nutrients that our bodies can use.
2. Detoxification: The liver detoxifies harmful substances in the body by breaking them down into less toxic forms or excreting them through bile.
3. Synthesis: The liver synthesizes important proteins, such as albumin and clotting factors, that are necessary for proper bodily function.
4. Storage: The liver stores glucose, vitamins, and minerals that can be released when the body needs them.
5. Bile production: The liver produces bile, a digestive juice that helps to break down fats in the small intestine.
6. Immune function: The liver plays a role in the immune system by filtering out bacteria and other harmful substances from the blood.

Overall, the liver is an essential organ that plays a critical role in maintaining overall health and well-being.

Bile ducts are tubular structures that carry bile from the liver to the gallbladder for storage or directly to the small intestine to aid in digestion. There are two types of bile ducts: intrahepatic and extrahepatic. Intrahepatic bile ducts are located within the liver and drain bile from liver cells, while extrahepatic bile ducts are outside the liver and include the common hepatic duct, cystic duct, and common bile duct. These ducts can become obstructed or inflamed, leading to various medical conditions such as cholestasis, cholecystitis, and gallstones.

Ursodeoxycholic acid (UDCA) is a naturally occurring bile acid that is used medically as a therapeutic agent. It is commonly used to treat gallstones, particularly cholesterol gallstones, and other conditions associated with abnormal liver function, such as primary biliary cholangitis (PBC). UDCA works by decreasing the amount of cholesterol in bile and protecting liver cells from damage. It is also known as ursodiol or Ursotan.

1-Naphthylisothiocyanate (also known as 1-NIT or ANS) is a chemical compound that is used in research and scientific studies. It is an isothiocyanate derivative of 1-naphthol, which means it has a molecular structure containing a naphthalene ring with an isothiocyanate functional group attached to it.

In medical and biological research, 1-Naphthylisothiocyanate has been used as a tool for studying various cellular processes, including the regulation of calcium signaling and the activation of certain enzymes. It can also act as an irritant and may cause respiratory and skin irritation in humans.

It is important to note that 1-Naphthylisothiocyanate is not a drug or medication, and it should only be used under controlled laboratory conditions by trained professionals.

Bile acids and salts are naturally occurring steroidal compounds that play a crucial role in the digestion and absorption of lipids (fats) in the body. They are produced in the liver from cholesterol and then conjugated with glycine or taurine to form bile acids, which are subsequently converted into bile salts by the addition of a sodium or potassium ion.

Bile acids and salts are stored in the gallbladder and released into the small intestine during digestion, where they help emulsify fats, allowing them to be broken down into smaller molecules that can be absorbed by the body. They also aid in the elimination of waste products from the liver and help regulate cholesterol metabolism.

Abnormalities in bile acid synthesis or transport can lead to various medical conditions, such as cholestatic liver diseases, gallstones, and diarrhea. Therefore, understanding the role of bile acids and salts in the body is essential for diagnosing and treating these disorders.

Biliary atresia is a rare, progressive liver disease in infants and children, characterized by the inflammation, fibrosis, and obstruction of the bile ducts. This results in the impaired flow of bile from the liver to the intestine, leading to cholestasis (accumulation of bile in the liver), jaundice (yellowing of the skin and eyes), and eventually liver cirrhosis and failure if left untreated.

The exact cause of biliary atresia is not known, but it is believed to be a combination of genetic and environmental factors. It can occur as an isolated condition or in association with other congenital anomalies. The diagnosis of biliary atresia is typically made through imaging studies, such as ultrasound and cholangiography, and confirmed by liver biopsy.

The standard treatment for biliary atresia is a surgical procedure called the Kasai portoenterostomy, which aims to restore bile flow from the liver to the intestine. In this procedure, the damaged bile ducts are removed and replaced with a loop of intestine that is connected directly to the liver. The success of the Kasai procedure depends on several factors, including the age at diagnosis and surgery, the extent of liver damage, and the skill and experience of the surgeon.

Despite successful Kasai surgery, many children with biliary atresia will eventually develop cirrhosis and require liver transplantation. The prognosis for children with biliary atresia has improved significantly over the past few decades due to earlier diagnosis, advances in surgical techniques, and better postoperative care. However, it remains a challenging condition that requires close monitoring and multidisciplinary management by pediatric hepatologists, surgeons, and other healthcare professionals.

Bile is a digestive fluid that is produced by the liver and stored in the gallbladder. It plays an essential role in the digestion and absorption of fats and fat-soluble vitamins in the small intestine. Bile consists of bile salts, bilirubin, cholesterol, phospholipids, electrolytes, and water.

Bile salts are amphipathic molecules that help to emulsify fats into smaller droplets, increasing their surface area and allowing for more efficient digestion by enzymes such as lipase. Bilirubin is a breakdown product of hemoglobin from red blood cells and gives bile its characteristic greenish-brown color.

Bile is released into the small intestine in response to food, particularly fats, entering the digestive tract. It helps to break down large fat molecules into smaller ones that can be absorbed through the walls of the intestines and transported to other parts of the body for energy or storage.

Bile canaliculi are the smallest bile-transporting structures in the liver. They are formed by the close apposition of hepatocyte (liver cell) plasma membranes, and they are responsible for the majority of bile production. The bile canaliculi merge to form bile ductules, which then merge to form larger bile ducts that transport bile to the gallbladder and small intestine. Bile is a fluid that contains water, electrolytes, bile salts, cholesterol, phospholipids, and bilirubin, which are produced by the liver and play important roles in digestion and elimination of waste products.

Bilirubin is a yellowish pigment that is produced by the liver when it breaks down old red blood cells. It is a normal byproduct of hemoglobin metabolism and is usually conjugated (made water-soluble) in the liver before being excreted through the bile into the digestive system. Elevated levels of bilirubin can cause jaundice, a yellowing of the skin and eyes. Increased bilirubin levels may indicate liver disease or other medical conditions such as gallstones or hemolysis. It is also measured to assess liver function and to help diagnose various liver disorders.

Liver diseases refer to a wide range of conditions that affect the normal functioning of the liver. The liver is a vital organ responsible for various critical functions such as detoxification, protein synthesis, and production of biochemicals necessary for digestion.

Liver diseases can be categorized into acute and chronic forms. Acute liver disease comes on rapidly and can be caused by factors like viral infections (hepatitis A, B, C, D, E), drug-induced liver injury, or exposure to toxic substances. Chronic liver disease develops slowly over time, often due to long-term exposure to harmful agents or inherent disorders of the liver.

Common examples of liver diseases include hepatitis, cirrhosis (scarring of the liver tissue), fatty liver disease, alcoholic liver disease, autoimmune liver diseases, genetic/hereditary liver disorders (like Wilson's disease and hemochromatosis), and liver cancers. Symptoms may vary widely depending on the type and stage of the disease but could include jaundice, abdominal pain, fatigue, loss of appetite, nausea, and weight loss.

Early diagnosis and treatment are essential to prevent progression and potential complications associated with liver diseases.

The biliary tract is a system of ducts that transport bile from the liver to the gallbladder and then to the small intestine. Bile is a digestive fluid produced by the liver that helps in the breakdown and absorption of fats in the small intestine. The main components of the biliary tract are:

1. Intrahepatic bile ducts: These are the smaller branches of bile ducts located within the liver that collect bile from the liver cells or hepatocytes.
2. Gallbladder: A small pear-shaped organ located beneath the liver, which stores and concentrates bile received from the intrahepatic bile ducts. The gallbladder releases bile into the small intestine when food is ingested, particularly fats, to aid digestion.
3. Common hepatic duct: This is a duct that forms by the union of the right and left hepatic ducts, which carry bile from the right and left lobes of the liver, respectively.
4. Cystic duct: A short duct that connects the gallbladder to the common hepatic duct, forming the beginning of the common bile duct.
5. Common bile duct: This is a larger duct formed by the union of the common hepatic duct and the cystic duct. It carries bile from the liver and gallbladder into the small intestine.
6. Pancreatic duct: A separate duct that originates from the pancreas, a gland located near the liver and stomach. The pancreatic duct joins the common bile duct just before they both enter the duodenum, the first part of the small intestine.
7. Ampulla of Vater: This is the dilated portion where the common bile duct and the pancreatic duct join together and empty their contents into the duodenum through a shared opening called the papilla of Vater.

Disorders related to the biliary tract include gallstones, cholecystitis (inflammation of the gallbladder), bile duct stones, bile duct strictures or obstructions, and primary sclerosing cholangitis, among others.

Pruritus is a medical term derived from Latin, in which "prurire" means "to itch." It refers to an unpleasant sensation on the skin that provokes the desire or reflex to scratch. This can be caused by various factors, such as skin conditions (e.g., dryness, eczema, psoriasis), systemic diseases (e.g., liver disease, kidney failure), nerve disorders, psychological conditions, or reactions to certain medications.

Pruritus can significantly affect a person's quality of life, leading to sleep disturbances, anxiety, and depression. Proper identification and management of the underlying cause are essential for effective treatment.

Biliary cirrhosis is a specific type of liver cirrhosis that results from chronic inflammation and scarring of the bile ducts, leading to impaired bile flow, liver damage, and fibrosis. It can be further classified into primary biliary cholangitis (PBC) and secondary biliary cirrhosis. PBC is an autoimmune disease, while secondary biliary cirrhosis is often associated with chronic gallstones, biliary tract obstruction, or recurrent pyogenic cholangitis. Symptoms may include fatigue, itching, jaundice, and abdominal discomfort. Diagnosis typically involves blood tests, imaging studies, and sometimes liver biopsy. Treatment focuses on managing symptoms, slowing disease progression, and preventing complications.

P-glycoproteins (P-gp), also known as multidrug resistance proteins (MDR), are a type of transmembrane protein that functions as an efflux pump, actively transporting various substrates out of cells. They play a crucial role in the protection of cells against xenobiotics, including drugs, toxins, and carcinogens. P-gp is expressed in many tissues, such as the intestine, liver, kidney, and blood-brain barrier, where it helps limit the absorption and distribution of drugs and other toxic substances.

In the context of medicine and pharmacology, P-glycoproteins are particularly relevant due to their ability to confer multidrug resistance in cancer cells. Overexpression of P-gp in tumor cells can lead to reduced intracellular drug concentrations, making these cells less sensitive to chemotherapeutic agents and contributing to treatment failure. Understanding the function and regulation of P-glycoproteins is essential for developing strategies to overcome multidrug resistance in cancer therapy.

Liver function tests (LFTs) are a group of blood tests that are used to assess the functioning and health of the liver. These tests measure the levels of various enzymes, proteins, and waste products that are produced or metabolized by the liver. Some common LFTs include:

1. Alanine aminotransferase (ALT): An enzyme found primarily in the liver, ALT is released into the bloodstream in response to liver cell damage. Elevated levels of ALT may indicate liver injury or disease.
2. Aspartate aminotransferase (AST): Another enzyme found in various tissues, including the liver, heart, and muscles. Like ALT, AST is released into the bloodstream following tissue damage. High AST levels can be a sign of liver damage or other medical conditions.
3. Alkaline phosphatase (ALP): An enzyme found in several organs, including the liver, bile ducts, and bones. Elevated ALP levels may indicate a blockage in the bile ducts, liver disease, or bone disorders.
4. Gamma-glutamyl transferase (GGT): An enzyme found mainly in the liver, pancreas, and biliary system. Increased GGT levels can suggest liver disease, alcohol consumption, or the use of certain medications.
5. Bilirubin: A yellowish pigment produced when hemoglobin from red blood cells is broken down. Bilirubin is processed by the liver and excreted through bile. High bilirubin levels can indicate liver dysfunction, bile duct obstruction, or certain types of anemia.
6. Albumin: A protein produced by the liver that helps maintain fluid balance in the body and transports various substances in the blood. Low albumin levels may suggest liver damage, malnutrition, or kidney disease.
7. Total protein: A measure of all proteins present in the blood, including albumin and other types of proteins produced by the liver. Decreased total protein levels can indicate liver dysfunction or other medical conditions.

These tests are often ordered together as part of a routine health checkup or when evaluating symptoms related to liver function or disease. The results should be interpreted in conjunction with clinical findings, medical history, and other diagnostic tests.

Bile duct diseases refer to a group of medical conditions that affect the bile ducts, which are tiny tubes that carry bile from the liver to the gallbladder and small intestine. Bile is a digestive juice produced by the liver that helps break down fats in food.

There are several types of bile duct diseases, including:

1. Choledocholithiasis: This occurs when stones form in the common bile duct, causing blockage and leading to symptoms such as abdominal pain, jaundice, and fever.
2. Cholangitis: This is an infection of the bile ducts that can cause inflammation, pain, and fever. It can occur due to obstruction of the bile ducts or as a complication of other medical procedures.
3. Primary Biliary Cirrhosis (PBC): This is a chronic autoimmune disease that affects the bile ducts in the liver, causing inflammation and scarring that can lead to cirrhosis and liver failure.
4. Primary Sclerosing Cholangitis (PSC): This is another autoimmune disease that causes inflammation and scarring of the bile ducts, leading to liver damage and potential liver failure.
5. Bile Duct Cancer: Also known as cholangiocarcinoma, this is a rare form of cancer that affects the bile ducts and can cause jaundice, abdominal pain, and weight loss.
6. Benign Strictures: These are narrowing of the bile ducts that can occur due to injury, inflammation, or surgery, leading to blockage and potential infection.

Symptoms of bile duct diseases may include jaundice, abdominal pain, fever, itching, dark urine, and light-colored stools. Treatment depends on the specific condition and may involve medication, surgery, or other medical interventions.

Portal hypertension is a medical condition characterized by an increased pressure in the portal vein, which is the large blood vessel that carries blood from the intestines, spleen, and pancreas to the liver. Normal portal venous pressure is approximately 5-10 mmHg. Portal hypertension is defined as a portal venous pressure greater than 10 mmHg.

The most common cause of portal hypertension is cirrhosis of the liver, which leads to scarring and narrowing of the small blood vessels in the liver, resulting in increased resistance to blood flow. Other causes include blood clots in the portal vein, inflammation of the liver or bile ducts, and invasive tumors that block the flow of blood through the liver.

Portal hypertension can lead to a number of complications, including the development of abnormal blood vessels (varices) in the esophagus, stomach, and intestines, which are prone to bleeding. Ascites, or the accumulation of fluid in the abdominal cavity, is another common complication of portal hypertension. Other potential complications include encephalopathy, which is a condition characterized by confusion, disorientation, and other neurological symptoms, and an increased risk of bacterial infections.

Treatment of portal hypertension depends on the underlying cause and the severity of the condition. Medications to reduce pressure in the portal vein, such as beta blockers or nitrates, may be used. Endoscopic procedures to band or inject varices can help prevent bleeding. In severe cases, surgery or liver transplantation may be necessary.

Neonatal jaundice is a medical condition characterized by the yellowing of a newborn baby's skin and eyes due to an excess of bilirubin in the blood. Bilirubin is a yellowish substance produced by the normal breakdown of red blood cells, which are then processed by the liver and excreted through the bile. In neonatal jaundice, the liver is not yet fully developed and cannot process bilirubin quickly enough, leading to its accumulation in the body.

Neonatal jaundice typically appears within the first 2-4 days of life and can range from mild to severe. Mild cases may resolve on their own without treatment, while more severe cases may require medical intervention such as phototherapy or a blood transfusion. Risk factors for neonatal jaundice include prematurity, bruising during birth, blood type incompatibility between mother and baby, and certain genetic disorders.

It is important to monitor newborns closely for signs of jaundice and seek medical attention if concerned, as untreated neonatal jaundice can lead to serious complications such as brain damage or hearing loss.

Liver neoplasms refer to abnormal growths in the liver that can be benign or malignant. Benign liver neoplasms are non-cancerous tumors that do not spread to other parts of the body, while malignant liver neoplasms are cancerous tumors that can invade and destroy surrounding tissue and spread to other organs.

Liver neoplasms can be primary, meaning they originate in the liver, or secondary, meaning they have metastasized (spread) to the liver from another part of the body. Primary liver neoplasms can be further classified into different types based on their cell of origin and behavior, including hepatocellular carcinoma, cholangiocarcinoma, and hepatic hemangioma.

The diagnosis of liver neoplasms typically involves a combination of imaging studies, such as ultrasound, CT scan, or MRI, and biopsy to confirm the type and stage of the tumor. Treatment options depend on the type and extent of the neoplasm and may include surgery, radiation therapy, chemotherapy, or liver transplantation.

The portal vein is the large venous trunk that carries blood from the gastrointestinal tract, spleen, pancreas, and gallbladder to the liver. It is formed by the union of the superior mesenteric vein (draining the small intestine and a portion of the large intestine) and the splenic vein (draining the spleen and pancreas). The portal vein then divides into right and left branches within the liver, where the blood flows through the sinusoids and gets enriched with oxygen and nutrients before being drained by the hepatic veins into the inferior vena cava. This unique arrangement allows the liver to process and detoxify the absorbed nutrients, remove waste products, and regulate metabolic homeostasis.

Jaundice is a medical condition characterized by the yellowing of the skin, sclera (whites of the eyes), and mucous membranes due to an excess of bilirubin in the bloodstream. Bilirubin is a yellow-orange pigment produced when hemoglobin from red blood cells is broken down. Normally, bilirubin is processed by the liver and excreted through bile into the digestive system. However, if there's an issue with bilirubin metabolism or elimination, it can accumulate in the body, leading to jaundice.

Jaundice can be a symptom of various underlying conditions, such as liver diseases (hepatitis, cirrhosis), gallbladder issues (gallstones, tumors), or blood disorders (hemolysis). It is essential to consult a healthcare professional if jaundice is observed, as it may indicate a severe health problem requiring prompt medical attention.

Ligation, in the context of medical terminology, refers to the process of tying off a part of the body, usually blood vessels or tissue, with a surgical suture or another device. The goal is to stop the flow of fluids such as blood or other substances within the body. It is commonly used during surgeries to control bleeding or to block the passage of fluids, gases, or solids in various parts of the body.

Hepatocytes are the predominant type of cells in the liver, accounting for about 80% of its cytoplasmic mass. They play a key role in protein synthesis, protein storage, transformation of carbohydrates, synthesis of cholesterol, bile salts and phospholipids, detoxification, modification, and excretion of exogenous and endogenous substances, initiation of formation and secretion of bile, and enzyme production. Hepatocytes are essential for the maintenance of homeostasis in the body.

Cholangitis is a medical condition characterized by inflammation of the bile ducts, which are the tubes that carry bile from the liver to the small intestine. Bile is a digestive juice produced by the liver that helps break down fats in food.

There are two types of cholangitis: acute and chronic. Acute cholangitis is a sudden and severe infection that can cause symptoms such as abdominal pain, fever, jaundice (yellowing of the skin and eyes), and dark urine. It is usually caused by a bacterial infection that enters the bile ducts through a blockage or obstruction.

Chronic cholangitis, on the other hand, is a long-term inflammation of the bile ducts that can lead to scarring and narrowing of the ducts. This can cause symptoms such as abdominal pain, itching, and jaundice. Chronic cholangitis can be caused by various factors, including primary sclerosing cholangitis (an autoimmune disease), bile duct stones, or tumors in the bile ducts.

Treatment for cholangitis depends on the underlying cause of the condition. Antibiotics may be used to treat bacterial infections, and surgery may be necessary to remove blockages or obstructions in the bile ducts. In some cases, medications may be prescribed to manage symptoms and prevent further complications.

Obstructive Jaundice is a medical condition characterized by the yellowing of the skin, sclera (whites of the eyes), and mucous membranes due to the accumulation of bilirubin in the bloodstream. This occurs when there is an obstruction or blockage in the bile ducts that transport bile from the liver to the small intestine.

Bile, which contains bilirubin, aids in digestion and is usually released from the liver into the small intestine. When the flow of bile is obstructed, bilirubin builds up in the blood, causing jaundice. The obstruction can be caused by various factors, such as gallstones, tumors, or strictures in the bile ducts.

Obstructive jaundice may present with additional symptoms like dark urine, light-colored stools, itching, abdominal pain, and weight loss, depending on the cause and severity of the obstruction. It is essential to seek medical attention if jaundice is observed, as timely diagnosis and management can prevent potential complications, such as liver damage or infection.

Extrahepatic bile ducts refer to the portion of the biliary system that lies outside the liver. The biliary system is responsible for producing, storing, and transporting bile, a digestive fluid produced by the liver.

The extrahepatic bile ducts include:

1. The common hepatic duct: This duct is formed by the union of the right and left hepatic ducts, which drain bile from the corresponding lobes of the liver.
2. The cystic duct: This short duct connects the gallbladder to the common hepatic duct, allowing bile to flow into the gallbladder for storage and concentration.
3. The common bile duct: This is the result of the fusion of the common hepatic duct and the cystic duct. It transports bile from the liver and gallbladder to the duodenum, the first part of the small intestine, where it aids in fat digestion.
4. The ampulla of Vater (or hepatopancreatic ampulla): This is a dilated area where the common bile duct and the pancreatic duct join and empty their contents into the duodenum through a shared opening called the major duodenal papilla.

Extrahepatic bile ducts can be affected by various conditions, such as gallstones, inflammation (cholangitis), strictures, or tumors, which may require medical or surgical intervention.

Lithocholic acid (LCA) is a secondary bile acid that is produced in the liver by bacterial modification of primary bile acids, specifically chenodeoxycholic acid. It is a steroid acid that plays a role in various physiological processes such as cholesterol metabolism, drug absorption, and gut microbiota regulation. However, high levels of LCA can be toxic to the liver and have been linked to several diseases, including colon cancer and cholestatic liver diseases.

Liver cirrhosis is a chronic, progressive disease characterized by the replacement of normal liver tissue with scarred (fibrotic) tissue, leading to loss of function. The scarring is caused by long-term damage from various sources such as hepatitis, alcohol abuse, nonalcoholic fatty liver disease, and other causes. As the disease advances, it can lead to complications like portal hypertension, fluid accumulation in the abdomen (ascites), impaired brain function (hepatic encephalopathy), and increased risk of liver cancer. It is generally irreversible, but early detection and treatment of underlying causes may help slow down its progression.

Pregnancy complications refer to any health problems that arise during pregnancy which can put both the mother and the baby at risk. These complications may occur at any point during the pregnancy, from conception until childbirth. Some common pregnancy complications include:

1. Gestational diabetes: a type of diabetes that develops during pregnancy in women who did not have diabetes before becoming pregnant.
2. Preeclampsia: a pregnancy complication characterized by high blood pressure and damage to organs such as the liver or kidneys.
3. Placenta previa: a condition where the placenta covers the cervix, which can cause bleeding and may require delivery via cesarean section.
4. Preterm labor: when labor begins before 37 weeks of gestation, which can lead to premature birth and other complications.
5. Intrauterine growth restriction (IUGR): a condition where the fetus does not grow at a normal rate inside the womb.
6. Multiple pregnancies: carrying more than one baby, such as twins or triplets, which can increase the risk of premature labor and other complications.
7. Rh incompatibility: a condition where the mother's blood type is different from the baby's, which can cause anemia and jaundice in the newborn.
8. Pregnancy loss: including miscarriage, stillbirth, or ectopic pregnancy, which can be emotionally devastating for the parents.

It is important to monitor pregnancy closely and seek medical attention promptly if any concerning symptoms arise. With proper care and management, many pregnancy complications can be treated effectively, reducing the risk of harm to both the mother and the baby.

Citrullinemia is a rare inherited metabolic disorder characterized by the body's inability to properly process and eliminate certain toxic byproducts that are generated during the breakdown of proteins. This condition results from a deficiency of the enzyme argininosuccinate synthetase, which is required for the normal functioning of the urea cycle. The urea cycle is a series of biochemical reactions that occur in the liver and help to convert ammonia, a toxic substance, into urea, which can then be excreted by the kidneys.

There are two main types of citrullinemia: type I (also known as classic citrullinemia) and type II (also known as citrullinemia type II or adult-onset citrullinemia). Type I is typically more severe and can present in newborns with symptoms such as poor feeding, vomiting, seizures, and developmental delays. If left untreated, it can lead to serious complications, including intellectual disability, coma, and even death.

Type II citrullinemia, on the other hand, tends to present later in life, often in adulthood, and may cause symptoms such as confusion, seizures, and neurological problems. It is important to note that some individuals with type II citrullinemia may never develop any symptoms at all.

Treatment for citrullinemia typically involves a combination of dietary restrictions, supplements, and medications to help manage the buildup of toxic byproducts in the body. In severe cases, liver transplantation may be considered as a last resort.

Taurochenodeoxycholic acid (TCDCA) is a bile acid that is conjugated with the amino acid taurine. Bile acids are synthesized from cholesterol in the liver and released into the small intestine to aid in the digestion and absorption of fats and fat-soluble vitamins. TCDCA, along with other bile acids, is reabsorbed in the terminal ileum and transported back to the liver through the enterohepatic circulation. It plays a role in maintaining cholesterol homeostasis and has been studied for its potential therapeutic effects in various medical conditions, including gallstones, cholestatic liver diseases, and neurological disorders.

The hepatic artery is a branch of the celiac trunk or abdominal aorta that supplies oxygenated blood to the liver. It typically divides into two main branches, the right and left hepatic arteries, which further divide into smaller vessels to supply different regions of the liver. The hepatic artery also gives off branches to supply other organs such as the gallbladder, pancreas, and duodenum.

It's worth noting that there is significant variability in the anatomy of the hepatic artery, with some individuals having additional branches or variations in the origin of the vessel. This variability can have implications for surgical procedures involving the liver and surrounding organs.

Cholangiography is a medical procedure that involves taking X-ray images of the bile ducts (the tubes that carry bile from the liver to the small intestine). This is typically done by injecting a contrast dye into the bile ducts through an endoscope or a catheter that has been inserted into the body.

There are several types of cholangiography, including:

* Endoscopic retrograde cholangiopancreatography (ERCP): This procedure involves inserting an endoscope through the mouth and down the throat into the small intestine. A dye is then injected into the bile ducts through a small tube that is passed through the endoscope.
* Percutaneous transhepatic cholangiography (PTC): This procedure involves inserting a needle through the skin and into the liver to inject the contrast dye directly into the bile ducts.
* Operative cholangiography: This procedure is performed during surgery to examine the bile ducts for any abnormalities or blockages.

Cholangiography can help diagnose a variety of conditions that affect the bile ducts, such as gallstones, tumors, or inflammation. It can also be used to guide treatment decisions, such as whether surgery is necessary to remove a blockage.

The common bile duct is a duct that results from the union of the cystic duct (which drains bile from the gallbladder) and the common hepatic duct (which drains bile from the liver). The common bile duct transports bile, a digestive enzyme, from the liver and gallbladder to the duodenum, which is the first part of the small intestine.

The common bile duct runs through the head of the pancreas before emptying into the second part of the duodenum, either alone or in conjunction with the pancreatic duct, via a small opening called the ampulla of Vater. The common bile duct plays a crucial role in the digestion of fats by helping to break them down into smaller molecules that can be absorbed by the body.

Hepatitis is a medical condition characterized by inflammation of the liver, often resulting in damage to liver cells. It can be caused by various factors, including viral infections (such as Hepatitis A, B, C, D, and E), alcohol abuse, toxins, medications, and autoimmune disorders. Symptoms may include jaundice, fatigue, abdominal pain, loss of appetite, nausea, vomiting, and dark urine. The severity of the disease can range from mild illness to severe, life-threatening conditions, such as liver failure or cirrhosis.

Cholic acid is a primary bile acid, which is a type of organic compound that plays a crucial role in the digestion and absorption of fats and fat-soluble vitamins in the body. It is produced in the liver from cholesterol and is then conjugated with glycine or taurine to form conjugated bile acids, which are stored in the gallbladder and released into the small intestine during digestion.

Cholic acid helps to emulsify fats, allowing them to be broken down into smaller droplets that can be absorbed by the body. It also facilitates the absorption of fat-soluble vitamins such as vitamin A, D, E, and K. In addition to its role in digestion, cholic acid is also involved in the regulation of cholesterol metabolism and the excretion of bile acids from the body.

Abnormalities in cholic acid metabolism can lead to various medical conditions, such as cholestatic liver diseases, gallstones, and genetic disorders that affect bile acid synthesis.

ATP-binding cassette (ABC) transporters are a family of membrane proteins that utilize the energy from ATP hydrolysis to transport various substrates across extra- and intracellular membranes. These transporters play crucial roles in several biological processes, including detoxification, drug resistance, nutrient uptake, and regulation of cellular cholesterol homeostasis.

The structure of ABC transporters consists of two nucleotide-binding domains (NBDs) that bind and hydrolyze ATP, and two transmembrane domains (TMDs) that form the substrate-translocation pathway. The NBDs are typically located adjacent to each other in the cytoplasm, while the TMDs can be either integral membrane domains or separate structures associated with the membrane.

The human genome encodes 48 distinct ABC transporters, which are classified into seven subfamilies (ABCA-ABCG) based on their sequence similarity and domain organization. Some well-known examples of ABC transporters include P-glycoprotein (ABCB1), multidrug resistance protein 1 (ABCC1), and breast cancer resistance protein (ABCG2).

Dysregulation or mutations in ABC transporters have been implicated in various diseases, such as cystic fibrosis, neurological disorders, and cancer. In cancer, overexpression of certain ABC transporters can contribute to drug resistance by actively effluxing chemotherapeutic agents from cancer cells, making them less susceptible to treatment.

Alagille syndrome is a genetic disorder that affects the liver, heart, and other parts of the body. It is also known as Arteriohepatic dysplasia or Alagille-Watson syndrome. The main features of this condition include:

1. Liver disease: Most individuals with Alagille syndrome have a liver disorder called bile duct paucity, which means that the small tubes (bile ducts) inside the liver that carry bile to the intestine are narrowed or missing. This can lead to liver scarring and damage over time.
2. Heart defects: About 90% of people with Alagille syndrome have a congenital heart defect, such as pulmonary stenosis (narrowing of the pulmonary valve) or tetralogy of Fallot (a combination of four heart defects).
3. Skeletal abnormalities: Many individuals with Alagille syndrome have distinctive facial features and skeletal changes, such as a broad forehead, wide-set eyes, a pointed chin, and butterfly-shaped vertebrae in the spine.
4. Eye problems: Approximately 90% of people with Alagille syndrome have eye abnormalities, including posterior embryotoxon (a narrowing of the drainage angle of the eye) or retinal changes.
5. Kidney issues: Up to 40% of individuals with Alagille syndrome may experience kidney problems, such as renal dysplasia (abnormal kidney development) or vesicoureteral reflux (backflow of urine from the bladder into the ureters).
6. Other features: Some people with Alagille syndrome may have growth delays, cognitive impairment, or hearing loss.

Alagille syndrome is caused by mutations in one of two genes: JAG1 or NOTCH2. These genes play crucial roles in embryonic development and tissue growth. Inheritance of Alagille syndrome is autosomal dominant, meaning that a person has a 50% chance of inheriting the condition if one parent carries the mutated gene. However, about 30-40% of cases result from new (de novo) mutations and have no family history of the disorder.

Liver transplantation is a surgical procedure in which a diseased or failing liver is replaced with a healthy one from a deceased donor or, less commonly, a portion of a liver from a living donor. The goal of the procedure is to restore normal liver function and improve the patient's overall health and quality of life.

Liver transplantation may be recommended for individuals with end-stage liver disease, acute liver failure, certain genetic liver disorders, or liver cancers that cannot be treated effectively with other therapies. The procedure involves complex surgery to remove the diseased liver and implant the new one, followed by a period of recovery and close medical monitoring to ensure proper function and minimize the risk of complications.

The success of liver transplantation has improved significantly in recent years due to advances in surgical techniques, immunosuppressive medications, and post-transplant care. However, it remains a major operation with significant risks and challenges, including the need for lifelong immunosuppression to prevent rejection of the new liver, as well as potential complications such as infection, bleeding, and organ failure.

The hepatic veins are blood vessels that carry oxygen-depleted blood from the liver back to the heart. There are typically three major hepatic veins - right, middle, and left - that originate from the posterior aspect of the liver and drain into the inferior vena cava just below the diaphragm. These veins are responsible for returning the majority of the blood flow from the gastrointestinal tract and spleen to the heart. It's important to note that the hepatic veins do not have valves, which can make them susceptible to a condition called Budd-Chiari syndrome, where blood clots form in the veins and obstruct the flow of blood from the liver.

A portosystemic shunt is a surgical procedure that creates a connection between the portal vein (the blood vessel that carries blood from the digestive organs to the liver) and another systemic vein (a vein that carries blood away from the liver). This procedure is typically performed in animals, particularly dogs, to treat conditions such as portal hypertension or liver disease.

In a surgical portosystemic shunt, the surgeon creates a connection between the portal vein and a systemic vein, allowing blood from the digestive organs to bypass the liver. This can help to reduce the pressure in the portal vein and improve blood flow to the liver. The specific type of shunt created and the surgical approach used may vary depending on the individual patient's needs and the surgeon's preference.

It is important to note that while a surgical portosystemic shunt can be an effective treatment for certain conditions, it is not without risks and potential complications. As with any surgical procedure, there is always a risk of infection, bleeding, or other complications. Additionally, the creation of a portosystemic shunt can have long-term effects on the liver and overall health of the patient. It is important for pet owners to carefully consider the risks and benefits of this procedure and to discuss any questions or concerns they may have with their veterinarian.

Biliary tract diseases refer to a group of medical conditions that affect the biliary system, which includes the gallbladder, bile ducts, and liver. Bile is a digestive juice produced by the liver, stored in the gallbladder, and released into the small intestine through the bile ducts to help digest fats.

Biliary tract diseases can cause various symptoms such as abdominal pain, jaundice, fever, nausea, vomiting, and changes in stool color. Some of the common biliary tract diseases include:

1. Gallstones: Small, hard deposits that form in the gallbladder or bile ducts made up of cholesterol or bilirubin.
2. Cholecystitis: Inflammation of the gallbladder, often caused by gallstones.
3. Cholangitis: Infection or inflammation of the bile ducts.
4. Biliary dyskinesia: A motility disorder that affects the contraction and relaxation of the muscles in the biliary system.
5. Primary sclerosing cholangitis: A chronic autoimmune disease that causes scarring and narrowing of the bile ducts.
6. Biliary tract cancer: Rare cancers that affect the gallbladder, bile ducts, or liver.

Treatment for biliary tract diseases varies depending on the specific condition and severity but may include medications, surgery, or a combination of both.

Hepatectomy is a surgical procedure that involves the removal of part or all of the liver. This procedure can be performed for various reasons, such as removing cancerous or non-cancerous tumors, treating liver trauma, or donating a portion of the liver to another person in need of a transplant (live donor hepatectomy). The extent of the hepatectomy depends on the medical condition and overall health of the patient. It is a complex procedure that requires significant expertise and experience from the surgical team due to the liver's unique anatomy, blood supply, and regenerative capabilities.

Ethinyl estradiol is a synthetic form of the hormone estrogen that is often used in various forms of hormonal contraception, such as birth control pills. It works by preventing ovulation and thickening cervical mucus to make it more difficult for sperm to reach the egg. Ethinyl estradiol may also be used in combination with other hormones to treat menopausal symptoms or hormonal disorders.

It is important to note that while ethinyl estradiol can be an effective form of hormonal therapy, it can also carry risks and side effects, such as an increased risk of blood clots, stroke, and breast cancer. As with any medication, it should only be used under the guidance and supervision of a healthcare provider.

Liver circulation, also known as hepatic circulation, refers to the blood flow through the liver. The liver receives blood from two sources: the hepatic artery and the portal vein.

The hepatic artery delivers oxygenated blood from the heart to the liver, accounting for about 25% of the liver's blood supply. The remaining 75% comes from the portal vein, which carries nutrient-rich, deoxygenated blood from the gastrointestinal tract, spleen, pancreas, and gallbladder to the liver.

In the liver, these two sources of blood mix in the sinusoids, small vessels with large spaces between the endothelial cells that line them. This allows for efficient exchange of substances between the blood and the hepatocytes (liver cells). The blood then leaves the liver through the hepatic veins, which merge into the inferior vena cava and return the blood to the heart.

The unique dual blood supply and extensive sinusoidal network in the liver enable it to perform various critical functions, such as detoxification, metabolism, synthesis, storage, and secretion of numerous substances, maintaining body homeostasis.

Norethandrolone is a synthetic anabolic-androgenic steroid, which is a type of drug that is similar to the male hormone testosterone. It is used in the treatment of conditions such as breast cancer in women, delayed puberty in boys, and wasting syndrome in people with HIV/AIDS. Norethandrolone works by promoting the growth of muscle tissue, increasing appetite, and changing the way the body uses certain nutrients.

It is important to note that anabolic-androgenic steroids are controlled substances in many countries due to their potential for abuse and serious side effects, including liver damage, cardiovascular disease, and hormonal imbalances. They should only be used under the close supervision of a healthcare provider.

Hepatocellular carcinoma (HCC) is the most common type of primary liver cancer in adults. It originates from the hepatocytes, which are the main functional cells of the liver. This type of cancer is often associated with chronic liver diseases such as cirrhosis caused by hepatitis B or C virus infection, alcohol abuse, non-alcoholic fatty liver disease (NAFLD), and aflatoxin exposure.

The symptoms of HCC can vary but may include unexplained weight loss, lack of appetite, abdominal pain or swelling, jaundice, and fatigue. The diagnosis of HCC typically involves imaging tests such as ultrasound, CT scan, or MRI, as well as blood tests to measure alpha-fetoprotein (AFP) levels. Treatment options for Hepatocellular carcinoma depend on the stage and extent of the cancer, as well as the patient's overall health and liver function. Treatment options may include surgery, radiation therapy, chemotherapy, targeted therapy, or liver transplantation.

A portal system in medicine refers to a venous system in which veins from various tissues or organs (known as tributaries) drain into a common large vessel (known as the portal vein), which then carries the blood to a specific organ for filtration and processing before it is returned to the systemic circulation. The most well-known example of a portal system is the hepatic portal system, where veins from the gastrointestinal tract, spleen, pancreas, and stomach merge into the portal vein and then transport blood to the liver for detoxification and nutrient processing. Other examples include the hypophyseal portal system, which connects the hypothalamus to the anterior pituitary gland, and the renal portal system found in some animals.

'Coleus' is a plant genus that belongs to the family Lamiaceae. It is native to tropical regions of Africa, Asia, and Australia. The plants in this genus are grown for their ornamental leaves, which come in various colors and patterns. While 'Coleus' species have been used in traditional medicine in some cultures, there is no widely accepted medical definition or specific medicinal use of the term 'Coleus' in modern Western medicine.

It is worth noting that one species of Coleus, Coleus forskohlii, has been studied for its potential medicinal properties. The root extract of this plant contains a compound called forskolin, which has been found to have various effects on the body, such as increasing cyclic AMP (a cellular messenger) levels and relaxing smooth muscles. However, more research is needed before any definitive medical claims can be made about its effectiveness or safety.

Budd-Chiari syndrome is a rare condition characterized by the obstruction of the hepatic veins, which are the blood vessels that carry blood from the liver to the heart. This obstruction can be caused by blood clots, tumors, or other abnormalities, and it can lead to a backflow of blood in the liver, resulting in various symptoms such as abdominal pain, swelling, and liver enlargement. In severe cases, Budd-Chiari syndrome can cause liver failure and other complications if left untreated. The diagnosis of this condition typically involves imaging tests such as ultrasound, CT scan, or MRI, and treatment may include anticoagulation therapy, thrombolytic therapy, or surgical intervention to remove the obstruction.

Organic anion transporters (OATs) are membrane transport proteins that are responsible for the cellular uptake and excretion of various organic anions, such as drugs, toxins, and endogenous metabolites. They are found in various tissues, including the kidney, liver, and brain, where they play important roles in the elimination and detoxification of xenobiotics and endogenous compounds.

In the kidney, OATs are located in the basolateral membrane of renal tubular epithelial cells and mediate the uptake of organic anions from the blood into the cells. From there, the anions can be further transported into the urine by other transporters located in the apical membrane. In the liver, OATs are expressed in the sinusoidal membrane of hepatocytes and facilitate the uptake of organic anions from the blood into the liver cells for metabolism and excretion.

There are several isoforms of OATs that have been identified, each with distinct substrate specificities and tissue distributions. Mutations in OAT genes can lead to various diseases, including renal tubular acidosis, hypercalciuria, and drug toxicity. Therefore, understanding the function and regulation of OATs is important for developing strategies to improve drug delivery and reduce adverse drug reactions.

Hyperbilirubinemia is a medical condition characterized by an excessively high level of bilirubin in the bloodstream. Bilirubin is a yellowish pigment produced by the liver when it breaks down old red blood cells. Normally, bilirubin is conjugated (made water-soluble) in the liver and then excreted through the bile into the digestive system. However, if there is a problem with the liver's ability to process or excrete bilirubin, it can build up in the blood, leading to hyperbilirubinemia.

Hyperbilirubinemia can be classified as either unconjugated or conjugated, depending on whether the bilirubin is in its direct (conjugated) or indirect (unconjugated) form. Unconjugated hyperbilirubinemia can occur due to increased production of bilirubin (such as in hemolytic anemia), decreased uptake of bilirubin by the liver, or impaired conjugation of bilirubin in the liver. Conjugated hyperbilirubinemia, on the other hand, is usually caused by a problem with the excretion of conjugated bilirubin into the bile, such as in cholestatic liver diseases like hepatitis or cirrhosis.

Symptoms of hyperbilirubinemia can include jaundice (yellowing of the skin and eyes), dark urine, light-colored stools, itching, and fatigue. Treatment depends on the underlying cause of the condition and may involve medications, dietary changes, or surgery.

Taurocholic acid is a bile salt, which is a type of organic compound that plays a crucial role in the digestion and absorption of fats and fat-soluble vitamins in the small intestine. It is formed in the liver by conjugation of cholic acid with taurine, an amino sulfonic acid.

Taurocholic acid has a detergent-like effect on the lipids in our food, helping to break them down into smaller molecules that can be absorbed through the intestinal wall and transported to other parts of the body for energy production or storage. It also helps to maintain the flow of bile from the liver to the gallbladder and small intestine, where it is stored until needed for digestion.

Abnormal levels of taurocholic acid in the body have been linked to various health conditions, including gallstones, liver disease, and gastrointestinal disorders. Therefore, it is important to maintain a healthy balance of bile salts, including taurocholic acid, for optimal digestive function.

Endoscopic retrograde cholangiopancreatography (ERCP) is a medical procedure that combines upper gastrointestinal (GI) endoscopy and fluoroscopy to diagnose and treat certain problems of the bile ducts and pancreas.

During ERCP, a flexible endoscope (a long, thin, lighted tube with a camera on the end) is passed through the patient's mouth and throat, then through the stomach and into the first part of the small intestine (duodenum). A narrow plastic tube (catheter) is then inserted through the endoscope and into the bile ducts and/or pancreatic duct. Contrast dye is injected through the catheter, and X-rays are taken to visualize the ducts.

ERCP can be used to diagnose a variety of conditions affecting the bile ducts and pancreas, including gallstones, tumors, strictures (narrowing of the ducts), and chronic pancreatitis. It can also be used to treat certain conditions, such as removing gallstones from the bile duct or placing stents to keep the ducts open in cases of stricture.

ERCP is an invasive procedure that carries a risk of complications, including pancreatitis, infection, bleeding, and perforation (a tear in the lining of the GI tract). It should only be performed by experienced medical professionals in a hospital setting.

Liver failure is a serious condition in which the liver is no longer able to perform its normal functions, such as removing toxins and waste products from the blood, producing bile to help digest food, and regulating blood clotting. This can lead to a buildup of toxins in the body, jaundice (yellowing of the skin and eyes), fluid accumulation in the abdomen, and an increased risk of bleeding. Liver failure can be acute (sudden) or chronic (developing over time). Acute liver failure is often caused by medication toxicity, viral hepatitis, or other sudden illnesses. Chronic liver failure is most commonly caused by long-term damage from conditions such as cirrhosis, hepatitis, alcohol abuse, and non-alcoholic fatty liver disease.

It's important to note that Liver Failure is a life threatening condition and need immediate medical attention.

Imino acids are organic compounds that contain a nitrogen atom as part of an amide-like structure. They are structurally similar to amino acids, which contain a carboxyl group and an amino group, but instead of the amino group, imino acids have a structural unit known as an imine or Schiff base, which is a carbon-nitrogen double bond with a hydrogen atom attached to the nitrogen atom.

One example of an imino acid is proline, which is a cyclic imino acid that plays important roles in protein structure and function. Proline is unique among the 20 standard amino acids because its side chain is linked to the nitrogen atom of the backbone, forming a ring-like structure. This structural feature gives proline unique properties, such as restricted rotation around the bond between the nitrogen and alpha carbon atoms, which can affect protein folding and stability.

Other imino acids may be formed through chemical reactions or enzymatic processes, and they can play important roles in various biological pathways, including the biosynthesis of amino acids, nucleotides, and other biomolecules. However, imino acids are not typically considered to be part of the standard set of 20 amino acids that make up proteins.

Lithiasis is a medical term that refers to the formation of stones or calculi in various organs of the body. These stones can develop in the kidneys (nephrolithiasis), gallbladder (cholelithiasis), urinary bladder (cystolithiasis), or salivary glands (sialolithiasis). The stones are usually composed of minerals and organic substances, and their formation can be influenced by various factors such as diet, dehydration, genetic predisposition, and chronic inflammation. Lithiasis can cause a range of symptoms depending on the location and size of the stone, including pain, obstruction, infection, and damage to surrounding tissues. Treatment may involve medication, shock wave lithotripsy, or surgical removal of the stones.

Gamma-glutamyltransferase (GGT), also known as gamma-glutamyl transpeptidase, is an enzyme found in many tissues, including the liver, bile ducts, and pancreas. GGT is involved in the metabolism of certain amino acids and plays a role in the detoxification of various substances in the body.

GGT is often measured as a part of a panel of tests used to evaluate liver function. Elevated levels of GGT in the blood may indicate liver disease or injury, bile duct obstruction, or alcohol consumption. However, it's important to note that several other factors can also affect GGT levels, so abnormal results should be interpreted in conjunction with other clinical findings and diagnostic tests.

Pregnanetriol is not a medication, but rather a metabolite of the hormone progesterone. It is a steroid compound that is produced in the body and can be detected in urine. Pregnanetriol is often used as a biomarker to help diagnose certain medical conditions related to steroid hormone metabolism, such as congenital adrenal hyperplasia (CAH). In these cases, abnormal levels of pregnanetriol in the urine can indicate an enzyme deficiency that affects the production or breakdown of steroid hormones.

Drug-Induced Liver Injury (DILI) is a medical term that refers to liver damage or injury caused by the use of medications or drugs. This condition can vary in severity, from mild abnormalities in liver function tests to severe liver failure, which may require a liver transplant.

The exact mechanism of DILI can differ depending on the drug involved, but it generally occurs when the liver metabolizes the drug into toxic compounds that damage liver cells. This can happen through various pathways, including direct toxicity to liver cells, immune-mediated reactions, or metabolic idiosyncrasies.

Symptoms of DILI may include jaundice (yellowing of the skin and eyes), fatigue, abdominal pain, nausea, vomiting, loss of appetite, and dark urine. In severe cases, it can lead to complications such as ascites, encephalopathy, and bleeding disorders.

The diagnosis of DILI is often challenging because it requires the exclusion of other potential causes of liver injury. Liver function tests, imaging studies, and sometimes liver biopsies may be necessary to confirm the diagnosis. Treatment typically involves discontinuing the offending drug and providing supportive care until the liver recovers. In some cases, medications that protect the liver or promote its healing may be used.

Sclerosing cholangitis is a chronic progressive disease characterized by inflammation and scarring (fibrosis) of the bile ducts, leading to their narrowing or obstruction. This results in impaired bile flow from the liver to the small intestine, which can cause damage to the liver cells and eventually result in cirrhosis and liver failure.

The condition often affects both the intrahepatic (within the liver) and extrahepatic (outside the liver) bile ducts. The exact cause of sclerosing cholangitis is not known, but it is believed to involve an autoimmune response, genetic predisposition, and environmental factors.

Symptoms of sclerosing cholangitis may include jaundice (yellowing of the skin and eyes), itching, abdominal pain, fatigue, weight loss, dark urine, and light-colored stools. The diagnosis is typically made through imaging tests such as magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP), which can visualize the bile ducts and detect any abnormalities.

Treatment for sclerosing cholangitis is aimed at managing symptoms, preventing complications, and slowing down the progression of the disease. This may include medications to relieve itching, antibiotics to treat infections, and drugs to reduce inflammation and improve bile flow. In severe cases, a liver transplant may be necessary.

A biopsy is a medical procedure in which a small sample of tissue is taken from the body to be examined under a microscope for the presence of disease. This can help doctors diagnose and monitor various medical conditions, such as cancer, infections, or autoimmune disorders. The type of biopsy performed will depend on the location and nature of the suspected condition. Some common types of biopsies include:

1. Incisional biopsy: In this procedure, a surgeon removes a piece of tissue from an abnormal area using a scalpel or other surgical instrument. This type of biopsy is often used when the lesion is too large to be removed entirely during the initial biopsy.

2. Excisional biopsy: An excisional biopsy involves removing the entire abnormal area, along with a margin of healthy tissue surrounding it. This technique is typically employed for smaller lesions or when cancer is suspected.

3. Needle biopsy: A needle biopsy uses a thin, hollow needle to extract cells or fluid from the body. There are two main types of needle biopsies: fine-needle aspiration (FNA) and core needle biopsy. FNA extracts loose cells, while a core needle biopsy removes a small piece of tissue.

4. Punch biopsy: In a punch biopsy, a round, sharp tool is used to remove a small cylindrical sample of skin tissue. This type of biopsy is often used for evaluating rashes or other skin abnormalities.

5. Shave biopsy: During a shave biopsy, a thin slice of tissue is removed from the surface of the skin using a sharp razor-like instrument. This technique is typically used for superficial lesions or growths on the skin.

After the biopsy sample has been collected, it is sent to a laboratory where a pathologist will examine the tissue under a microscope and provide a diagnosis based on their findings. The results of the biopsy can help guide further treatment decisions and determine the best course of action for managing the patient's condition.

A newborn infant is a baby who is within the first 28 days of life. This period is also referred to as the neonatal period. Newborns require specialized care and attention due to their immature bodily systems and increased vulnerability to various health issues. They are closely monitored for signs of well-being, growth, and development during this critical time.

Hepatomegaly is a medical term that refers to an enlargement of the liver beyond its normal size. The liver is usually located in the upper right quadrant of the abdomen and can be felt during a physical examination. A healthcare provider may detect hepatomegaly by palpating (examining through touch) the abdomen, noticing that the edge of the liver extends past the lower ribcage.

There are several possible causes for hepatomegaly, including:
- Fatty liver disease (both alcoholic and nonalcoholic)
- Hepatitis (viral or autoimmune)
- Liver cirrhosis
- Cancer (such as primary liver cancer, metastatic cancer, or lymphoma)
- Infections (e.g., bacterial, fungal, or parasitic)
- Heart failure and other cardiovascular conditions
- Genetic disorders (e.g., Gaucher's disease, Niemann-Pick disease, or Hunter syndrome)
- Metabolic disorders (e.g., glycogen storage diseases, hemochromatosis, or Wilson's disease)

Diagnosing the underlying cause of hepatomegaly typically involves a combination of medical history, physical examination, laboratory tests, and imaging studies like ultrasound, CT scan, or MRI. Treatment depends on the specific cause identified and may include medications, lifestyle changes, or, in some cases, surgical intervention.

Cholelithiasis is a medical term that refers to the presence of gallstones in the gallbladder. The gallbladder is a small pear-shaped organ located beneath the liver that stores bile, a digestive fluid produced by the liver. Gallstones are hardened deposits that can form in the gallbladder when substances in the bile, such as cholesterol or bilirubin, crystallize.

Gallstones can vary in size and may be as small as a grain of sand or as large as a golf ball. Some people with gallstones may not experience any symptoms, while others may have severe abdominal pain, nausea, vomiting, fever, and jaundice (yellowing of the skin and eyes) if the gallstones block the bile ducts.

Cholelithiasis is a common condition that affects millions of people worldwide, particularly women over the age of 40 and those with certain medical conditions such as obesity, diabetes, and rapid weight loss. If left untreated, gallstones can lead to serious complications such as inflammation of the gallbladder (cholecystitis), infection, or pancreatitis (inflammation of the pancreas). Treatment options for cholelithiasis include medication, shock wave lithotripsy (breaking up the gallstones with sound waves), and surgery to remove the gallbladder (cholecystectomy).

Alanine transaminase (ALT) is a type of enzyme found primarily in the cells of the liver and, to a lesser extent, in the cells of other tissues such as the heart, muscles, and kidneys. Its primary function is to catalyze the reversible transfer of an amino group from alanine to another alpha-keto acid, usually pyruvate, to form pyruvate and another amino acid, usually glutamate. This process is known as the transamination reaction.

When liver cells are damaged or destroyed due to various reasons such as hepatitis, alcohol abuse, nonalcoholic fatty liver disease, or drug-induced liver injury, ALT is released into the bloodstream. Therefore, measuring the level of ALT in the blood is a useful diagnostic tool for evaluating liver function and detecting liver damage. Normal ALT levels vary depending on the laboratory, but typically range from 7 to 56 units per liter (U/L) for men and 6 to 45 U/L for women. Elevated ALT levels may indicate liver injury or disease, although other factors such as muscle damage or heart disease can also cause elevations in ALT.

Esophageal varices and gastric varices are abnormal, enlarged veins in the lower part of the esophagus (the tube that connects the throat to the stomach) and in the stomach lining, respectively. They occur as a result of increased pressure in the portal vein, which is the large blood vessel that carries blood from the digestive organs to the liver. This condition is known as portal hypertension.

Esophageal varices are more common than gastric varices and tend to be more symptomatic. They can cause bleeding, which can be life-threatening if not treated promptly. Gastric varices may also bleed, but they are often asymptomatic until they rupture.

The most common causes of esophageal and gastric varices are cirrhosis (scarring of the liver) and portal hypertension due to other liver diseases such as schistosomiasis or Budd-Chiari syndrome. Treatment options for esophageal and gastric varices include medications to reduce bleeding, endoscopic therapies to treat active bleeding or prevent recurrent bleeding, and surgical procedures to relieve portal hypertension.

Biliary tract neoplasms refer to abnormal growths or tumors that develop in the biliary system, which includes the gallbladder, bile ducts inside and outside the liver, and the ducts that connect the liver to the small intestine. These neoplasms can be benign (non-cancerous) or malignant (cancerous).

Malignant biliary tract neoplasms are often referred to as cholangiocarcinoma if they originate in the bile ducts, or gallbladder cancer if they arise in the gallbladder. These cancers are relatively rare but can be aggressive and difficult to treat. They can cause symptoms such as jaundice (yellowing of the skin and eyes), abdominal pain, weight loss, and dark urine.

Risk factors for biliary tract neoplasms include chronic inflammation of the biliary system, primary sclerosing cholangitis, liver cirrhosis, hepatitis B or C infection, parasitic infections, and certain genetic conditions. Early detection and treatment can improve outcomes for patients with these neoplasms.

Organic anion transporters (OATs) are membrane transport proteins that facilitate the movement of organic anions across biological membranes. The term "sodium-dependent" refers to a specific type of OAT that requires sodium ions (Na+) as a co-transport substrate to move organic anions across the membrane. These transporters play crucial roles in the elimination and distribution of various endogenous and exogenous organic anions, including drugs, toxins, and metabolites. Sodium-dependent OATs are primarily located in the kidneys and liver, where they help maintain homeostasis by regulating the reabsorption and secretion of these substances.

A Klatskin's tumor, also known as a perihilar cholangiocarcinoma, is a rare and aggressive form of cancer that occurs at the junction where the right and left hepatic ducts come together to form the common hepatic duct, which then becomes the common bile duct. This type of tumor can obstruct the flow of bile from the liver into the small intestine, leading to jaundice, itching, abdominal pain, and other symptoms. Klatskin's tumors are often difficult to diagnose and treat due to their location and tendency to spread quickly. Surgical resection is the preferred treatment option when possible, although chemotherapy and radiation therapy may also be used in some cases.

Portography is a medical term that refers to an X-ray examination of the portal vein, which is the large blood vessel that carries blood from the digestive organs to the liver. In this procedure, a contrast dye is injected into the patient's veins, and then X-rays are taken to visualize the flow of the dye through the portal vein and its branches. This test can help diagnose various conditions that affect the liver and surrounding organs, such as cirrhosis, tumors, or blood clots in the portal vein. It is also known as a portovenogram or hepatic venography.

Lipoprotein-X (Lp-X) is a type of lipoprotein that is typically found in the blood under certain pathological conditions. Unlike other lipoproteins such as low-density lipoprotein (LDL) or high-density lipoprotein (HDL), Lp-X does not contain apolipoproteins and is not associated with cholesterol transport. Instead, Lp-X is rich in free cholesterol and phospholipids, and it can be formed when there is an increase in the concentration of these lipids in the blood due to the breakdown of cell membranes or other lipoproteins.

Lp-X is often found in the blood of patients with liver diseases such as cirrhosis or hepatitis, as well as in those with severe malnutrition or who have experienced massive trauma. It can also be present in the blood of pregnant women, particularly those with preeclampsia or HELLP syndrome.

Because Lp-X lacks apolipoproteins, it is not recognized by the liver and cannot be cleared from the blood efficiently. As a result, high levels of Lp-X can contribute to the development of fatty liver disease, inflammation, and other complications associated with liver dysfunction.

Adenoma of the bile duct is a benign (noncancerous) tumor that develops in the bile ducts, which are tiny tubes that carry bile from the liver to the gallbladder and small intestine. Bile is a digestive fluid produced by the liver.

Bile duct adenomas are rare and usually do not cause any symptoms. However, if they grow large enough, they may obstruct the flow of bile and cause jaundice (yellowing of the skin and whites of the eyes), abdominal pain, or itching. In some cases, bile duct adenomas may become cancerous and develop into bile duct carcinomas.

The exact cause of bile duct adenomas is not known, but they are more common in people with certain genetic disorders, such as Gardner's syndrome and von Hippel-Lindau disease. Treatment for bile duct adenomas typically involves surgical removal of the tumor.

Arthrogryposis is a medical term that describes a condition characterized by the presence of multiple joint contractures at birth. A contracture occurs when the range of motion in a joint is limited, making it difficult or impossible to move the joint through its full range of motion. In arthrogryposis, these contractures are present in two or more areas of the body.

The term "arthrogryposis" comes from two Greek words: "arthro," meaning joint, and "gyros," meaning curved or bent. Therefore, arthrogryposis literally means "curving of the joints."

There are many different types of arthrogryposis, each with its own specific set of symptoms and causes. However, in general, arthrogryposis is caused by decreased fetal movement during pregnancy, which can be due to a variety of factors such as genetic mutations, nervous system abnormalities, or environmental factors that restrict fetal movement.

Treatment for arthrogryposis typically involves a combination of physical therapy, bracing, and surgery to help improve joint mobility and function. The prognosis for individuals with arthrogryposis varies depending on the severity and type of contractures present, as well as the underlying cause of the condition.

A biliary fistula is an abnormal connection or passage between the biliary system (which includes the gallbladder, bile ducts, and liver) and another organ or structure, usually in the abdominal cavity. This connection allows bile, which is a digestive fluid produced by the liver, to leak out of its normal pathway and into other areas of the body.

Biliary fistulas can occur as a result of trauma, surgery, infection, or inflammation in the biliary system. Symptoms may include abdominal pain, fever, jaundice (yellowing of the skin and eyes), nausea, vomiting, and clay-colored stools. Treatment typically involves addressing the underlying cause of the fistula, such as draining an infection or repairing damaged tissue, and diverting bile flow away from the site of the leak. In some cases, surgery may be necessary to repair the fistula.

Retrospective studies, also known as retrospective research or looking back studies, are a type of observational study that examines data from the past to draw conclusions about possible causal relationships between risk factors and outcomes. In these studies, researchers analyze existing records, medical charts, or previously collected data to test a hypothesis or answer a specific research question.

Retrospective studies can be useful for generating hypotheses and identifying trends, but they have limitations compared to prospective studies, which follow participants forward in time from exposure to outcome. Retrospective studies are subject to biases such as recall bias, selection bias, and information bias, which can affect the validity of the results. Therefore, retrospective studies should be interpreted with caution and used primarily to generate hypotheses for further testing in prospective studies.

Animal disease models are specialized animals, typically rodents such as mice or rats, that have been genetically engineered or exposed to certain conditions to develop symptoms and physiological changes similar to those seen in human diseases. These models are used in medical research to study the pathophysiology of diseases, identify potential therapeutic targets, test drug efficacy and safety, and understand disease mechanisms.

The genetic modifications can include knockout or knock-in mutations, transgenic expression of specific genes, or RNA interference techniques. The animals may also be exposed to environmental factors such as chemicals, radiation, or infectious agents to induce the disease state.

Examples of animal disease models include:

1. Mouse models of cancer: Genetically engineered mice that develop various types of tumors, allowing researchers to study cancer initiation, progression, and metastasis.
2. Alzheimer's disease models: Transgenic mice expressing mutant human genes associated with Alzheimer's disease, which exhibit amyloid plaque formation and cognitive decline.
3. Diabetes models: Obese and diabetic mouse strains like the NOD (non-obese diabetic) or db/db mice, used to study the development of type 1 and type 2 diabetes, respectively.
4. Cardiovascular disease models: Atherosclerosis-prone mice, such as ApoE-deficient or LDLR-deficient mice, that develop plaque buildup in their arteries when fed a high-fat diet.
5. Inflammatory bowel disease models: Mice with genetic mutations affecting intestinal barrier function and immune response, such as IL-10 knockout or SAMP1/YitFc mice, which develop colitis.

Animal disease models are essential tools in preclinical research, but it is important to recognize their limitations. Differences between species can affect the translatability of results from animal studies to human patients. Therefore, researchers must carefully consider the choice of model and interpret findings cautiously when applying them to human diseases.

Portal pressure, also known as portal hypertension, refers to an increase in the pressure within the portal vein, which is the large blood vessel that carries blood from the gastrointestinal tract and spleen to the liver. Normal portal pressure is usually between 5-10 mmHg.

Portal hypertension can occur as a result of various conditions that cause obstruction or narrowing of the portal vein, or increased resistance to blood flow within the liver. This can lead to the development of collateral vessels, which are abnormal blood vessels that form to bypass the blocked or narrowed vessel, and can result in complications such as variceal bleeding, ascites, and encephalopathy.

The measurement of portal pressure is often used in the diagnosis and management of patients with liver disease and portal hypertension.

The common hepatic duct is a medical term that refers to the duct in the liver responsible for carrying bile from the liver. More specifically, it is the duct that results from the convergence of the right and left hepatic ducts, which themselves carry bile from the right and left lobes of the liver, respectively. The common hepatic duct then joins with the cystic duct from the gallbladder to form the common bile duct, which ultimately drains into the duodenum, a part of the small intestine.

The primary function of the common hepatic duct is to transport bile, a digestive juice produced by the liver, to the small intestine. Bile helps break down fats during the digestion process, making it possible for the body to absorb them properly. Any issues or abnormalities in the common hepatic duct can lead to problems with bile flow and potentially cause health complications such as jaundice, gallstones, or liver damage.

Dothiepin is a tricyclic antidepressant (TCA) that was commonly used in the past to treat depression and anxiety disorders. It works by increasing the levels of certain neurotransmitters, such as serotonin and noradrenaline, in the brain. However, due to its side effects and the availability of safer and more effective antidepressants, dothiepin is not commonly prescribed anymore.

Dothiepin has a sedative effect, which can help people with depression who have trouble sleeping. It also has an analgesic effect, which can be helpful in treating chronic pain conditions. However, its use is associated with several side effects, including dry mouth, blurred vision, constipation, dizziness, and weight gain. In addition, dothiepin can cause orthostatic hypotension (a drop in blood pressure upon standing), which can increase the risk of falls and fractures in older adults.

Dothiepin is available in immediate-release and sustained-release formulations, and it is usually taken orally once or twice a day. The dosage depends on the individual's response to treatment and the severity of their symptoms. It is important to follow the doctor's instructions carefully when taking dothiepin and to report any bothersome side effects promptly.

Like other TCAs, dothiepin has a potential for overdose and can be fatal if taken in large quantities. Therefore, it should be stored in a safe place, away from children and pets. People who are taking dothiepin should inform their healthcare provider about any other medications they are taking, as well as any medical conditions they have, to avoid dangerous interactions and complications.

Parenteral nutrition (PN) is a medical term used to describe the delivery of nutrients directly into a patient's bloodstream through a vein, bypassing the gastrointestinal tract. It is a specialized medical treatment that is typically used when a patient cannot receive adequate nutrition through enteral feeding, which involves the ingestion and digestion of food through the mouth or a feeding tube.

PN can be used to provide essential nutrients such as carbohydrates, proteins, fats, vitamins, minerals, and electrolytes to patients who have conditions that prevent them from absorbing nutrients through their gut, such as severe gastrointestinal tract disorders, malabsorption syndromes, or short bowel syndrome.

PN is administered through a catheter that is inserted into a vein, typically in the chest or arm. The nutrient solution is prepared under sterile conditions and delivered through an infusion pump to ensure accurate and controlled delivery of the solution.

While PN can be a life-saving intervention for some patients, it also carries risks such as infection, inflammation, and organ damage. Therefore, it should only be prescribed and administered by healthcare professionals with specialized training in this area.

Sulfobromophthalein (BSP or bromsulphthalein) is not typically defined in a medical dictionary as it is a chemical compound. However, I can provide you with some information about its use in a medical context:

Sulfobromophthalein is a chemical compound primarily used for liver function tests. It is a dye that is injected into the patient's bloodstream, and then its clearance rate from the blood is measured to evaluate liver function. A healthy liver should quickly remove the dye from the blood and excrete it through the bile ducts into the digestive system. If the liver is not functioning properly, the clearance of sulfobromophthalein will be slower, leading to higher levels of the dye remaining in the bloodstream over time.

The test using sulfobromophthalein has largely been replaced by more modern and specific liver function tests; however, it was once widely used for assessing overall liver health and diagnosing conditions such as hepatitis, cirrhosis, and liver damage due to various causes.

Treatment outcome is a term used to describe the result or effect of medical treatment on a patient's health status. It can be measured in various ways, such as through symptoms improvement, disease remission, reduced disability, improved quality of life, or survival rates. The treatment outcome helps healthcare providers evaluate the effectiveness of a particular treatment plan and make informed decisions about future care. It is also used in clinical research to compare the efficacy of different treatments and improve patient care.

A cyst is a closed sac, having a distinct membrane and division between the sac and its surrounding tissue, that contains fluid, air, or semisolid material. Cysts can occur in various parts of the body, including the skin, internal organs, and bones. They can be caused by various factors, such as infection, genetic predisposition, or blockage of a duct or gland. Some cysts may cause symptoms, such as pain or discomfort, while others may not cause any symptoms at all. Treatment for cysts depends on the type and location of the cyst, as well as whether it is causing any problems. Some cysts may go away on their own, while others may need to be drained or removed through a surgical procedure.

Cholic acids are a type of bile acid, which are naturally occurring steroid acids that play a crucial role in the digestion and absorption of fats and fat-soluble vitamins in the body. Cholic acid is the primary bile acid synthesized in the liver from cholesterol. It is then conjugated with glycine or taurine to form conjugated cholic acids, which are stored in the gallbladder and released into the small intestine during digestion to aid in fat emulsification and absorption.

Cholic acid and its derivatives have also been studied for their potential therapeutic benefits in various medical conditions, including liver diseases, gallstones, and bacterial infections. However, more research is needed to fully understand the mechanisms of action and potential side effects of cholic acids and their derivatives before they can be widely used as therapeutic agents.

C57BL/6 (C57 Black 6) is an inbred strain of laboratory mouse that is widely used in biomedical research. The term "inbred" refers to a strain of animals where matings have been carried out between siblings or other closely related individuals for many generations, resulting in a population that is highly homozygous at most genetic loci.

The C57BL/6 strain was established in 1920 by crossing a female mouse from the dilute brown (DBA) strain with a male mouse from the black strain. The resulting offspring were then interbred for many generations to create the inbred C57BL/6 strain.

C57BL/6 mice are known for their robust health, longevity, and ease of handling, making them a popular choice for researchers. They have been used in a wide range of biomedical research areas, including studies of cancer, immunology, neuroscience, cardiovascular disease, and metabolism.

One of the most notable features of the C57BL/6 strain is its sensitivity to certain genetic modifications, such as the introduction of mutations that lead to obesity or impaired glucose tolerance. This has made it a valuable tool for studying the genetic basis of complex diseases and traits.

Overall, the C57BL/6 inbred mouse strain is an important model organism in biomedical research, providing a valuable resource for understanding the genetic and molecular mechanisms underlying human health and disease.

Ascites is an abnormal accumulation of fluid in the peritoneal cavity, which is the space between the lining of the abdominal wall and the organs within it. This buildup of fluid can cause the belly to swell and become distended. Ascites can be caused by various medical conditions, including liver cirrhosis, cancer, heart failure, and kidney disease. The accumulation of fluid in the peritoneal cavity can lead to complications such as infection, reduced mobility, and difficulty breathing. Treatment for ascites depends on the underlying cause and may include diuretics, paracentesis (a procedure to remove excess fluid from the abdomen), or treatment of the underlying medical condition.

Taurolithocholic acid (TLCA) is not a medical term per se, but rather a chemical compound that can be mentioned in the context of medical or biological research. TLCA is a bile acid, which is a type of organic compound that plays a crucial role in digestion and metabolism. Specifically, TLCA is a taurine conjugate of lithocholic acid, meaning it contains a taurine molecule attached to the lithocholic acid molecule.

Bile acids are synthesized from cholesterol in the liver and then released into the small intestine to aid in the digestion and absorption of fats and fat-soluble vitamins. TLCA is a secondary bile acid, which means it is formed in the gut by the bacterial metabolism of primary bile acids.

Abnormal levels of TLCA or other bile acids can be associated with various medical conditions, such as liver disease, cholestasis (a condition characterized by reduced bile flow), and intestinal disorders. Therefore, measuring the levels of TLCA and other bile acids in blood, urine, or stool samples can provide valuable diagnostic information for these conditions.

Glycochenodeoxycholic acid (GCDCA) is a type of bile acid that is produced in the liver and then conjugated with glycine. Bile acids are formed from cholesterol and play an important role in the digestion and absorption of fats and fat-soluble vitamins in the small intestine.

GCDCA is a secondary bile acid, which means that it is produced by bacterial metabolism of primary bile acids (such as cholic acid and chenodeoxycholic acid) in the colon. Once formed, GCDCA is then reabsorbed into the bloodstream and transported back to the liver, where it can be conjugated with glycine or taurine and excreted into bile again.

Abnormal levels of GCDCA and other bile acids have been associated with various health conditions, including cholestatic liver diseases, gallstones, and colon cancer. Therefore, measuring the levels of these acids in blood, urine, or feces can provide valuable diagnostic information for these conditions.

Technetium Tc 99m Disofenin is not a medical condition, but rather a radiopharmaceutical used in diagnostic imaging. It is a radioactive tracer used in nuclear medicine scans, specifically for liver and biliary system imaging. The compound consists of the radioisotope Technetium-99m (Tc-99m) bonded to the pharmaceutical Disofenin.

The Tc-99m is a gamma emitter with a half-life of 6 hours, making it ideal for diagnostic imaging. When administered to the patient, the compound is taken up by the liver and excreted into the bile ducts and gallbladder, allowing medical professionals to visualize these structures using a gamma camera. This can help detect various conditions such as tumors, gallstones, or obstructions in the biliary system.

It's important to note that Technetium Tc 99m Disofenin is used diagnostically and not for therapeutic purposes. The radiation exposure from this compound is generally low and considered safe for diagnostic use. However, as with any medical procedure involving radiation, the benefits and risks should be carefully weighed and discussed with a healthcare professional.

Gallstones are small, hard deposits that form in the gallbladder, a small organ located under the liver. They can range in size from as small as a grain of sand to as large as a golf ball. Gallstones can be made of cholesterol, bile pigments, or calcium salts, or a combination of these substances.

There are two main types of gallstones: cholesterol stones and pigment stones. Cholesterol stones are the most common type and are usually yellow-green in color. They form when there is too much cholesterol in the bile, which causes it to become saturated and form crystals that eventually grow into stones. Pigment stones are smaller and darker in color, ranging from brown to black. They form when there is an excess of bilirubin, a waste product produced by the breakdown of red blood cells, in the bile.

Gallstones can cause symptoms such as abdominal pain, nausea, vomiting, and bloating, especially after eating fatty foods. In some cases, gallstones can lead to serious complications, such as inflammation of the gallbladder (cholecystitis), infection, or blockage of the bile ducts, which can cause jaundice, a yellowing of the skin and eyes.

The exact cause of gallstones is not fully understood, but risk factors include being female, older age, obesity, a family history of gallstones, rapid weight loss, diabetes, and certain medical conditions such as cirrhosis or sickle cell anemia. Treatment for gallstones may involve medication to dissolve the stones, shock wave therapy to break them up, or surgery to remove the gallbladder.

Total Parenteral Nutrition (TPN) is a medical term used to describe a specialized nutritional support system that is delivered through a vein (intravenously). It provides all the necessary nutrients that a patient needs, such as carbohydrates, proteins, fats, vitamins, and minerals. TPN is typically used when a patient cannot eat or digest food through their gastrointestinal tract for various reasons, such as severe malabsorption, intestinal obstruction, or inflammatory bowel disease. The term "total" indicates that the nutritional support is complete and meets all of the patient's nutritional needs.

A fatal outcome is a term used in medical context to describe a situation where a disease, injury, or illness results in the death of an individual. It is the most severe and unfortunate possible outcome of any medical condition, and is often used as a measure of the severity and prognosis of various diseases and injuries. In clinical trials and research, fatal outcome may be used as an endpoint to evaluate the effectiveness and safety of different treatments or interventions.

Aspartate aminotransferases (ASTs) are a group of enzymes found in various tissues throughout the body, including the heart, liver, and muscles. They play a crucial role in the metabolic process of transferring amino groups between different molecules.

In medical terms, AST is often used as a blood test to measure the level of this enzyme in the serum. Elevated levels of AST can indicate damage or injury to tissues that contain this enzyme, such as the liver or heart. For example, liver disease, including hepatitis and cirrhosis, can cause elevated AST levels due to damage to liver cells. Similarly, heart attacks can also result in increased AST levels due to damage to heart muscle tissue.

It is important to note that an AST test alone cannot diagnose a specific medical condition, but it can provide valuable information when used in conjunction with other diagnostic tests and clinical evaluation.

"Noxae" is a term derived from Latin, which means "causes of damage or injury." In medical contexts, it is used to refer to harmful agents or factors that can cause harm, damage, or disease in an organism or a biological system. These harmful agents can include physical, chemical, or biological factors such as radiation, toxins, infectious microorganisms, and mechanical injuries.

Experimental liver cirrhosis refers to a controlled research setting where various factors and substances are intentionally introduced to induce liver cirrhosis in animals or cell cultures. The purpose is to study the mechanisms, progression, potential treatments, and prevention strategies for liver cirrhosis. This could involve administering chemicals, drugs, alcohol, viruses, or manipulating genes associated with liver damage and fibrosis. It's important to note that results from experimental models may not directly translate to human conditions, but they can provide valuable insights into disease pathophysiology and therapeutic development.

Caroli disease is a rare genetic disorder that affects the liver and bile ducts. It is characterized by abnormal dilations or sac-like structures in the intrahepatic bile ducts, which are the ducts that carry bile from the liver to the gallbladder and small intestine. These dilations can lead to recurrent cholangitis (inflammation of the bile ducts), stone formation, and liver damage.

Caroli disease is usually diagnosed in childhood or early adulthood, and it can be associated with other congenital anomalies such as polycystic kidney disease. The exact cause of Caroli disease is not fully understood, but it is believed to be inherited in an autosomal recessive manner, meaning that an individual must inherit two copies of the abnormal gene, one from each parent, to develop the condition.

Treatment for Caroli disease may include antibiotics to manage cholangitis, endoscopic procedures to remove stones or dilate strictures, and surgery to bypass or remove affected bile ducts. In severe cases, liver transplantation may be necessary. Regular monitoring of liver function and surveillance for complications are essential in the management of this condition.

Alcoholic Liver Cirrhosis is a medical condition characterized by irreversible scarring (fibrosis) and damage to the liver caused by excessive consumption of alcohol over an extended period. The liver's normal structure and function are progressively impaired as healthy liver tissue is replaced by scarred tissue, leading to the formation of nodules (regenerative noduli).

The condition typically develops after years of heavy drinking, with a higher risk for those who consume more than 60 grams of pure alcohol daily. The damage caused by alcoholic liver cirrhosis can be life-threatening and may result in complications such as:

1. Ascites (accumulation of fluid in the abdomen)
2. Encephalopathy (neurological dysfunction due to liver failure)
3. Esophageal varices (dilated veins in the esophagus that can rupture and bleed)
4. Hepatorenal syndrome (kidney failure caused by liver disease)
5. Increased susceptibility to infections
6. Liver cancer (hepatocellular carcinoma)
7. Portal hypertension (increased blood pressure in the portal vein that supplies blood to the liver)

Abstaining from alcohol and managing underlying medical conditions are crucial for slowing down or halting disease progression. Treatment may involve medications, dietary changes, and supportive care to address complications. In severe cases, a liver transplant might be necessary.

Cytoplasmic receptors and nuclear receptors are two types of intracellular receptors that play crucial roles in signal transduction pathways and regulation of gene expression. They are classified based on their location within the cell. Here are the medical definitions for each:

1. Cytoplasmic Receptors: These are a group of intracellular receptors primarily found in the cytoplasm of cells, which bind to specific hormones, growth factors, or other signaling molecules. Upon binding, these receptors undergo conformational changes that allow them to interact with various partners, such as adapter proteins and enzymes, leading to activation of downstream signaling cascades. These pathways ultimately result in modulation of cellular processes like proliferation, differentiation, and apoptosis. Examples of cytoplasmic receptors include receptor tyrosine kinases (RTKs), serine/threonine kinase receptors, and cytokine receptors.
2. Nuclear Receptors: These are a distinct class of intracellular receptors that reside primarily in the nucleus of cells. They bind to specific ligands, such as steroid hormones, thyroid hormones, vitamin D, retinoic acid, and various other lipophilic molecules. Upon binding, nuclear receptors undergo conformational changes that facilitate their interaction with co-regulatory proteins and the DNA. This interaction results in the modulation of gene transcription, ultimately leading to alterations in protein expression and cellular responses. Examples of nuclear receptors include estrogen receptor (ER), androgen receptor (AR), glucocorticoid receptor (GR), thyroid hormone receptor (TR), vitamin D receptor (VDR), and peroxisome proliferator-activated receptors (PPARs).

Both cytoplasmic and nuclear receptors are essential components of cellular communication networks, allowing cells to respond appropriately to extracellular signals and maintain homeostasis. Dysregulation of these receptors has been implicated in various diseases, including cancer, diabetes, and autoimmune disorders.

Chenodeoxycholic acid (CDCA) is a bile acid that is naturally produced in the human body. It is formed in the liver from cholesterol and is then conjugated with glycine or taurine to become a primary bile acid. CDCA is stored in the gallbladder and released into the small intestine during digestion, where it helps to emulsify fats and facilitate their absorption.

CDCA also has important regulatory functions in the body, including acting as a signaling molecule that binds to specific receptors in the liver, intestines, and other tissues. It plays a role in glucose and lipid metabolism, inflammation, and cell growth and differentiation.

In addition to its natural functions, CDCA is also used as a medication for the treatment of certain medical conditions. For example, it is used to dissolve gallstones that are composed of cholesterol, and it is also used to treat a rare genetic disorder called cerebrotendinous xanthomatosis (CTX), which is characterized by the accumulation of CDCA and other bile acids in various tissues.

It's important to note that while CDCA has therapeutic uses, it can also have adverse effects if taken in high doses or for extended periods of time. Therefore, it should only be used under the supervision of a healthcare professional.

Pregnancy is a physiological state or condition where a fertilized egg (zygote) successfully implants and grows in the uterus of a woman, leading to the development of an embryo and finally a fetus. This process typically spans approximately 40 weeks, divided into three trimesters, and culminates in childbirth. Throughout this period, numerous hormonal and physical changes occur to support the growing offspring, including uterine enlargement, breast development, and various maternal adaptations to ensure the fetus's optimal growth and well-being.

A "newborn infant" refers to a baby in the first 28 days of life outside of the womb. This period is crucial for growth and development, but also poses unique challenges as the infant's immune system is not fully developed, making them more susceptible to various diseases.

"Newborn diseases" are health conditions that specifically affect newborn infants. These can be categorized into three main types:

1. Congenital disorders: These are conditions that are present at birth and may be inherited or caused by factors such as infection, exposure to harmful substances during pregnancy, or chromosomal abnormalities. Examples include Down syndrome, congenital heart defects, and spina bifida.

2. Infectious diseases: Newborn infants are particularly vulnerable to infections due to their immature immune systems. Common infectious diseases in newborns include sepsis (bloodstream infection), pneumonia, and meningitis. These can be acquired from the mother during pregnancy or childbirth, or from the environment after birth.

3. Developmental disorders: These are conditions that affect the normal growth and development of the newborn infant. Examples include cerebral palsy, intellectual disabilities, and vision or hearing impairments.

It is important to note that many newborn diseases can be prevented or treated with appropriate medical care, including prenatal care, proper hygiene practices, and timely vaccinations. Regular check-ups and monitoring of the newborn's health by a healthcare provider are essential for early detection and management of any potential health issues.

Gastrointestinal (GI) hemorrhage is a term used to describe any bleeding that occurs in the gastrointestinal tract, which includes the esophagus, stomach, small intestine, large intestine, and rectum. The bleeding can range from mild to severe and can produce symptoms such as vomiting blood, passing black or tarry stools, or having low blood pressure.

GI hemorrhage can be classified as either upper or lower, depending on the location of the bleed. Upper GI hemorrhage refers to bleeding that occurs above the ligament of Treitz, which is a point in the small intestine where it becomes narrower and turns a corner. Common causes of upper GI hemorrhage include gastritis, ulcers, esophageal varices, and Mallory-Weiss tears.

Lower GI hemorrhage refers to bleeding that occurs below the ligament of Treitz. Common causes of lower GI hemorrhage include diverticulosis, colitis, inflammatory bowel disease, and vascular abnormalities such as angiodysplasia.

The diagnosis of GI hemorrhage is often made based on the patient's symptoms, medical history, physical examination, and diagnostic tests such as endoscopy, CT scan, or radionuclide scanning. Treatment depends on the severity and cause of the bleeding and may include medications, endoscopic procedures, surgery, or a combination of these approaches.

The gallbladder is a small, pear-shaped organ located just under the liver in the right upper quadrant of the abdomen. Its primary function is to store and concentrate bile, a digestive enzyme produced by the liver, which helps in the breakdown of fats during the digestion process. When food, particularly fatty foods, enter the stomach and small intestine, the gallbladder contracts and releases bile through the common bile duct into the duodenum, the first part of the small intestine, to aid in fat digestion.

The gallbladder is made up of three main parts: the fundus, body, and neck. It has a muscular wall that allows it to contract and release bile. Gallstones, an inflammation of the gallbladder (cholecystitis), or other gallbladder diseases can cause pain, discomfort, and potentially serious health complications if left untreated.

Epichlorohydrin is an industrial chemical with the formula C3H5ClO. It is a colorless liquid with an irritating odor, and it is used primarily as a building block in the production of other chemicals, including epoxy resins, synthetic gums, and plastics. Epichlorohydrin is produced by reacting chlorine with propylene in the presence of a catalyst. It is classified as a probable human carcinogen based on evidence from animal studies, and exposure to this chemical can cause irritation of the eyes, skin, and respiratory tract. Therefore, it is important to handle epichlorohydrin with care and to use appropriate safety measures when working with this chemical.

"Mitragyna" is a genus of plants in the coffee family (Rubiaceae). The most well-known species within this genus is "Mitragyna speciosa," also known as kratom. Kratom is a tropical evergreen tree native to Southeast Asia, including countries like Thailand, Malaysia, and Indonesia.

The leaves of the kratom tree contain various alkaloids, with mitragynine being the most abundant. Mitragynine has psychoactive properties and can have stimulant-like effects at low doses and opioid-like pain-relieving effects at higher doses. Kratom is often used as a traditional medicine in Southeast Asia to manage pain, fatigue, and opioid withdrawal symptoms. However, its legal status and safety profile are controversial in many other parts of the world.

Hepatic encephalopathy (HE) is a neuropsychiatric syndrome associated with liver dysfunction and/or portosystemic shunting. It results from the accumulation of toxic substances, such as ammonia and inflammatory mediators, which are normally metabolized by the liver. HE can present with a wide range of symptoms, including changes in sleep-wake cycle, altered mental status, confusion, disorientation, asterixis (flapping tremor), and in severe cases, coma. The diagnosis is based on clinical evaluation, neuropsychological testing, and exclusion of other causes of cognitive impairment. Treatment typically involves addressing the underlying liver dysfunction, reducing ammonia production through dietary modifications and medications, and preventing further episodes with lactulose or rifaximin therapy.

"Wistar rats" are a strain of albino rats that are widely used in laboratory research. They were developed at the Wistar Institute in Philadelphia, USA, and were first introduced in 1906. Wistar rats are outbred, which means that they are genetically diverse and do not have a fixed set of genetic characteristics like inbred strains.

Wistar rats are commonly used as animal models in biomedical research because of their size, ease of handling, and relatively low cost. They are used in a wide range of research areas, including toxicology, pharmacology, nutrition, cancer, cardiovascular disease, and behavioral studies. Wistar rats are also used in safety testing of drugs, medical devices, and other products.

Wistar rats are typically larger than many other rat strains, with males weighing between 500-700 grams and females weighing between 250-350 grams. They have a lifespan of approximately 2-3 years. Wistar rats are also known for their docile and friendly nature, making them easy to handle and work with in the laboratory setting.

Pregnanediol is a steroid hormone that is produced as a metabolite of progesterone. It is primarily used as a biomarker to measure the exposure to progesterone, particularly in cases where progesterone levels need to be monitored, such as during pregnancy or in certain medical conditions. Pregnanediol can be measured in urine, blood, or other bodily fluids and is often used in clinical and research settings to assess hormonal status. It is important to note that pregnanediol itself does not have any known physiological effects on the body, but rather serves as an indicator of progesterone levels.

Fatty liver, also known as hepatic steatosis, is a medical condition characterized by the abnormal accumulation of fat in the liver. The liver's primary function is to process nutrients, filter blood, and fight infections, among other tasks. When excess fat builds up in the liver cells, it can impair liver function and lead to inflammation, scarring, and even liver failure if left untreated.

Fatty liver can be caused by various factors, including alcohol consumption, obesity, nonalcoholic fatty liver disease (NAFLD), viral hepatitis, and certain medications or medical conditions. NAFLD is the most common cause of fatty liver in the United States and other developed countries, affecting up to 25% of the population.

Symptoms of fatty liver may include fatigue, weakness, weight loss, loss of appetite, nausea, abdominal pain or discomfort, and jaundice (yellowing of the skin and eyes). However, many people with fatty liver do not experience any symptoms, making it essential to diagnose and manage the condition through regular check-ups and blood tests.

Treatment for fatty liver depends on the underlying cause. Lifestyle changes such as weight loss, exercise, and dietary modifications are often recommended for people with NAFLD or alcohol-related fatty liver disease. Medications may also be prescribed to manage related conditions such as diabetes, high cholesterol, or metabolic syndrome. In severe cases of liver damage, a liver transplant may be necessary.

Fat emulsions for intravenous use are a type of parenteral nutrition solution that contain fat in the form of triglycerides, which are broken down and absorbed into the body to provide a source of energy and essential fatty acids. These emulsions are typically used in patients who are unable to consume food orally or enterally, such as those with gastrointestinal tract disorders, malabsorption syndromes, or severe injuries.

The fat emulsion is usually combined with other nutrients, such as carbohydrates and amino acids, to create a complete parenteral nutrition solution that meets the patient's nutritional needs. The emulsion is administered through a vein using a sterile technique to prevent infection.

Fat emulsions are typically made from soybean oil or a mixture of soybean and medium-chain triglyceride (MCT) oils. MCTs are more easily absorbed than long-chain triglycerides (LCTs), which are found in soybean oil, and may be used in patients with malabsorption syndromes or other conditions that affect fat absorption.

It is important to monitor patients receiving intravenous fat emulsions for signs of complications such as infection, hyperlipidemia (elevated levels of fats in the blood), and liver function abnormalities.

I'm sorry for any confusion, but "Glycocholic Acid" is not a widely recognized or established medical term. It seems there might be a misunderstanding or a typo in your request.

If you meant "Glycocholic," it's a term that refers to a substance conjugated with glycine, which is an amino acid. This process often occurs in the liver during the metabolism of certain substances, like bile acids.

"Glycocholic" could theoretically refer to a glycine conjugate of a bile acid such as cholic acid, which would make it a derivative called "Glycocholic Acid." However, I couldn't find any specific medical or scientific literature that directly refers to "Glycocholic Acid" as a known compound or concept.

If you could provide more context or clarify your question, I would be happy to help further!

Inborn errors of metabolism (IEM) refer to a group of genetic disorders caused by defects in enzymes or transporters that play a role in the body's metabolic processes. These disorders result in the accumulation or deficiency of specific chemicals within the body, which can lead to various clinical manifestations, such as developmental delay, intellectual disability, seizures, organ damage, and in some cases, death.

Examples of IEM include phenylketonuria (PKU), maple syrup urine disease (MSUD), galactosemia, and glycogen storage diseases, among many others. These disorders are typically inherited in an autosomal recessive manner, meaning that an affected individual has two copies of the mutated gene, one from each parent.

Early diagnosis and management of IEM are crucial to prevent or minimize complications and improve outcomes. Treatment options may include dietary modifications, supplementation with missing enzymes or cofactors, medication, and in some cases, stem cell transplantation or gene therapy.

Biliary tract surgical procedures refer to a range of operations that involve the biliary system, which includes the liver, gallbladder, and bile ducts. These procedures can be performed for various reasons, including the treatment of gallstones, bile duct injuries, tumors, or other conditions affecting the biliary tract. Here are some examples of biliary tract surgical procedures:

1. Cholecystectomy: This is the surgical removal of the gallbladder, which is often performed to treat symptomatic gallstones or chronic cholecystitis (inflammation of the gallbladder). It can be done as an open procedure or laparoscopically.
2. Bile duct exploration: This procedure involves opening the common bile duct to remove stones, strictures, or tumors. It is often performed during a cholecystectomy if there is suspicion of common bile duct involvement.
3. Hepaticojejunostomy: This operation connects the liver's bile ducts directly to a portion of the small intestine called the jejunum, bypassing a damaged or obstructed segment of the biliary tract. It is often performed for benign or malignant conditions affecting the bile ducts.
4. Roux-en-Y hepaticojejunostomy: This procedure involves creating a Y-shaped limb of jejunum and connecting it to the liver's bile ducts, bypassing the common bile duct and duodenum. It is often performed for complex biliary tract injuries or malignancies.
5. Whipple procedure (pancreaticoduodenectomy): This extensive operation involves removing the head of the pancreas, the duodenum, a portion of the jejunum, the gallbladder, and the common bile duct. It is performed for malignancies involving the pancreas, bile duct, or duodenum.
6. Liver resection: This procedure involves removing a portion of the liver to treat primary liver tumors (hepatocellular carcinoma or cholangiocarcinoma) or metastatic cancer from other organs.
7. Biliary stenting or bypass: These minimally invasive procedures involve placing a stent or creating a bypass to relieve bile duct obstructions caused by tumors, strictures, or stones. They can be performed endoscopically (ERCP) or percutaneously (PTC).
8. Cholecystectomy: This procedure involves removing the gallbladder, often for symptomatic cholelithiasis (gallstones) or cholecystitis (inflammation of the gallbladder). It can be performed laparoscopically or open.
9. Biliary drainage: This procedure involves placing a catheter to drain bile from the liver or bile ducts, often for acute or chronic obstructions caused by tumors, strictures, or stones. It can be performed endoscopically (ERCP) or percutaneously (PTC).
10. Bilioenteric anastomosis: This procedure involves connecting the biliary tract to a portion of the small intestine, often for benign or malignant conditions affecting the bile ducts or pancreas. It can be performed open or laparoscopically.

The Kveim test is a specific intradermal skin test that was used in the diagnosis of certain forms of vasculitis, such as sarcoidosis. The test involves the injection of a small amount of tissue from a patient with known sarcoidosis into the skin of the person being tested. If the individual has sarcoidosis, a positive reaction will occur within 2-3 months, characterized by the formation of a granuloma (a small nodular inflammatory lesion) at the injection site.

However, it's important to note that the Kveim test is no longer widely used due to several limitations, including the subjective nature of the results and the risk of transmitting infectious diseases from the donor tissue. Currently, sarcoidosis is diagnosed based on a combination of clinical symptoms, radiological findings, laboratory tests, and sometimes biopsy results.

Immunohistochemistry (IHC) is a technique used in pathology and laboratory medicine to identify specific proteins or antigens in tissue sections. It combines the principles of immunology and histology to detect the presence and location of these target molecules within cells and tissues. This technique utilizes antibodies that are specific to the protein or antigen of interest, which are then tagged with a detection system such as a chromogen or fluorophore. The stained tissue sections can be examined under a microscope, allowing for the visualization and analysis of the distribution and expression patterns of the target molecule in the context of the tissue architecture. Immunohistochemistry is widely used in diagnostic pathology to help identify various diseases, including cancer, infectious diseases, and immune-mediated disorders.

Alkaline phosphatase (ALP) is an enzyme found in various body tissues, including the liver, bile ducts, digestive system, bones, and kidneys. It plays a role in breaking down proteins and minerals, such as phosphate, in the body.

The medical definition of alkaline phosphatase refers to its function as a hydrolase enzyme that removes phosphate groups from molecules at an alkaline pH level. In clinical settings, ALP is often measured through blood tests as a biomarker for various health conditions.

Elevated levels of ALP in the blood may indicate liver or bone diseases, such as hepatitis, cirrhosis, bone fractures, or cancer. Therefore, physicians may order an alkaline phosphatase test to help diagnose and monitor these conditions. However, it is essential to interpret ALP results in conjunction with other diagnostic tests and clinical findings for accurate diagnosis and treatment.

Multidrug Resistance-Associated Proteins (MRPs) are a subfamily of ATP-binding cassette (ABC) transporter proteins that play a crucial role in the efflux of various substrates, including drugs and organic anions, out of cells. They are located in the plasma membrane of many cell types, including epithelial cells in the liver, intestine, kidney, and blood-brain barrier.

MRPs are known to transport a wide range of molecules, such as glutathione conjugates, bilirubin, bile acids, and various clinical drugs. One of the most well-known MRPs is MRP1 (ABCC1), which was initially identified in drug-resistant tumor cells. MRP1 can confer resistance to chemotherapeutic agents by actively pumping them out of cancer cells, thereby reducing their intracellular concentration and effectiveness.

The activity of MRPs can have significant implications for the pharmacokinetics and pharmacodynamics of drugs, as they can affect drug absorption, distribution, metabolism, and excretion (ADME). Understanding the function and regulation of MRPs is essential for developing strategies to overcome multidrug resistance in cancer therapy and optimizing drug dosing regimens in various clinical settings.

I'm sorry for any confusion, but "Animal Hepatitis" is not a medical term used to describe a specific disease. Hepatitis refers to inflammation of the liver, and it can be caused by various factors, including viruses, alcohol, drugs, and certain medical conditions.

However, there are several viral hepatitis types that can infect animals, such as Hepatitis A, B, and C, which primarily affect humans. But there are also other hepatitis viruses that are species-specific and primarily infect animals, such as:

1. Canine Hepatitis (Adenovirus Type 1): This is a viral infection that affects dogs and causes liver damage, respiratory signs, and occasionally death.
2. Feline Infectious Peritonitis (FIP) Virus: While not strictly a hepatitis virus, this feline coronavirus can cause severe inflammation of the liver and other organs in cats.
3. Equine Infectious Anemia Virus (EIAV): This retrovirus affects horses and causes cyclic fever, anemia, and occasionally liver disease.
4. Avian Hepatitis E Virus: A recently discovered virus that infects birds and can cause hepatitis and other systemic signs in chickens and other avian species.

If you're looking for information on a specific animal hepatitis virus or a different medical term, please provide more context so I can give you a more accurate answer.

Steroid 12-alpha-hydroxylase is an enzyme that is involved in the metabolism of steroids. It is specifically responsible for adding a hydroxyl group (-OH) to the 12th carbon atom of certain steroid molecules. This enzyme plays a crucial role in the biosynthesis of bile acids and corticosteroids, including cortisol and aldosterone, which are important hormones produced by the adrenal gland.

The gene that encodes this enzyme is called CYP12A1, and mutations in this gene can lead to various disorders related to steroid metabolism. For example, a deficiency in steroid 12-alpha-hydroxylase can result in the accumulation of bile acids that are not properly hydroxylated, which can cause liver damage and cholestatic pruritus (itching). Additionally, impaired cortisol and aldosterone production due to defects in this enzyme can lead to conditions such as congenital adrenal hyperplasia and salt-wasting crisis.

Alcoholic hepatitis is a medical condition characterized by inflammation and damage to the liver caused by excessive alcohol consumption. It is a type of hepatitis that specifically results from alcohol abuse, rather than from viral infections or other causes. The condition can vary in severity, and long-term heavy drinking increases the risk of developing alcoholic hepatitis.

The inflammation in alcoholic hepatitis can lead to symptoms such as jaundice (yellowing of the skin and eyes), abdominal pain, nausea, vomiting, loss of appetite, and fever. In severe cases, it can cause liver failure, which may be life-threatening. Treatment typically involves alcohol abstinence, supportive care, and medications to manage symptoms and prevent further liver damage. In some cases, hospitalization and more intensive treatments may be necessary.

In the field of medicine, "time factors" refer to the duration of symptoms or time elapsed since the onset of a medical condition, which can have significant implications for diagnosis and treatment. Understanding time factors is crucial in determining the progression of a disease, evaluating the effectiveness of treatments, and making critical decisions regarding patient care.

For example, in stroke management, "time is brain," meaning that rapid intervention within a specific time frame (usually within 4.5 hours) is essential to administering tissue plasminogen activator (tPA), a clot-busting drug that can minimize brain damage and improve patient outcomes. Similarly, in trauma care, the "golden hour" concept emphasizes the importance of providing definitive care within the first 60 minutes after injury to increase survival rates and reduce morbidity.

Time factors also play a role in monitoring the progression of chronic conditions like diabetes or heart disease, where regular follow-ups and assessments help determine appropriate treatment adjustments and prevent complications. In infectious diseases, time factors are crucial for initiating antibiotic therapy and identifying potential outbreaks to control their spread.

Overall, "time factors" encompass the significance of recognizing and acting promptly in various medical scenarios to optimize patient outcomes and provide effective care.

Gallbladder diseases refer to a range of conditions that affect the function and structure of the gallbladder, a small pear-shaped organ located beneath the liver. The primary role of the gallbladder is to store, concentrate, and release bile into the small intestine to aid in digesting fats. Gallbladder diseases can be chronic or acute and may cause various symptoms, discomfort, or complications if left untreated. Here are some common gallbladder diseases with brief definitions:

1. Cholelithiasis: The presence of gallstones within the gallbladder. Gallstones are small, hard deposits made of cholesterol, bilirubin, or a combination of both, which can vary in size from tiny grains to several centimeters.
2. Cholecystitis: Inflammation of the gallbladder, often caused by obstruction of the cystic duct (the tube connecting the gallbladder and the common bile duct) due to a gallstone. This condition can be acute or chronic and may cause abdominal pain, fever, and tenderness in the right upper quadrant of the abdomen.
3. Choledocholithiasis: The presence of gallstones within the common bile duct, which can lead to obstruction, jaundice, and potential infection of the biliary system (cholangitis).
4. Acalculous gallbladder disease: Gallbladder dysfunction or inflammation without the presence of gallstones. This condition is often seen in critically ill patients and can lead to similar symptoms as cholecystitis.
5. Gallbladder polyps: Small growths attached to the inner wall of the gallbladder. While most polyps are benign, some may have malignant potential, especially if they are larger than 1 cm in size or associated with certain risk factors.
6. Gallbladder cancer: A rare form of cancer that originates in the gallbladder tissue. It is often asymptomatic in its early stages and can be challenging to diagnose. Symptoms may include abdominal pain, jaundice, or a palpable mass in the right upper quadrant of the abdomen.

It is essential to consult with a healthcare professional if experiencing symptoms related to gallbladder disease for proper diagnosis and treatment.

A syndrome, in medical terms, is a set of symptoms that collectively indicate or characterize a disease, disorder, or underlying pathological process. It's essentially a collection of signs and/or symptoms that frequently occur together and can suggest a particular cause or condition, even though the exact physiological mechanisms might not be fully understood.

For example, Down syndrome is characterized by specific physical features, cognitive delays, and other developmental issues resulting from an extra copy of chromosome 21. Similarly, metabolic syndromes like diabetes mellitus type 2 involve a group of risk factors such as obesity, high blood pressure, high blood sugar, and abnormal cholesterol or triglyceride levels that collectively increase the risk of heart disease, stroke, and diabetes.

It's important to note that a syndrome is not a specific diagnosis; rather, it's a pattern of symptoms that can help guide further diagnostic evaluation and management.

A portacaval shunt is a surgical procedure that creates an alternate pathway for blood flow between the portal vein and the inferior vena cava. The portal vein carries blood from the gastrointestinal tract, liver, spleen, and pancreas to the liver. In certain medical conditions, such as severe liver disease or portal hypertension, the blood pressure in the portal vein becomes abnormally high, which can lead to serious complications like variceal bleeding.

In a surgical portacaval shunt procedure, a surgeon creates a connection between the portal vein and the inferior vena cava, allowing a portion of the blood from the portal vein to bypass the liver and flow directly into the systemic circulation. This helps reduce the pressure in the portal vein and prevent complications associated with portal hypertension.

There are different types of portacaval shunts, including:

1. Direct portacaval shunt: In this procedure, the surgeon directly connects the portal vein to the inferior vena cava.
2. Side-to-side portacaval shunt: Here, the surgeon creates an anastomosis (connection) between a side branch of the portal vein and the inferior vena cava.
3. H-type shunt: This involves creating two separate connections between the portal vein and the inferior vena cava, forming an "H" shape.

It is important to note that while portacaval shunts can be effective in managing complications of portal hypertension, they may also have potential risks and side effects, such as worsening liver function, encephalopathy, or heart failure. Therefore, the decision to perform a portacaval shunt should be made carefully, considering the individual patient's medical condition and overall health.

Membrane transport proteins are specialized biological molecules, specifically integral membrane proteins, that facilitate the movement of various substances across the lipid bilayer of cell membranes. They are responsible for the selective and regulated transport of ions, sugars, amino acids, nucleotides, and other molecules into and out of cells, as well as within different cellular compartments. These proteins can be categorized into two main types: channels and carriers (or pumps). Channels provide a passive transport mechanism, allowing ions or small molecules to move down their electrochemical gradient, while carriers actively transport substances against their concentration gradient, requiring energy usually in the form of ATP. Membrane transport proteins play a crucial role in maintaining cell homeostasis, signaling processes, and many other physiological functions.

Antipruritics are a class of medications or substances that are used to relieve or prevent itching (pruritus). They work by reducing the sensation of itchiness and can be applied topically to the skin, taken orally, or administered intravenously. Some common antipruritics include diphenhydramine, hydroxyzine, and corticosteroids.

Cystadenoma is a type of benign tumor (not cancerous), which arises from glandular epithelial cells and is covered by a thin layer of connective tissue. These tumors can develop in various locations within the body, including the ovaries, pancreas, and other organs that contain glands.

There are two main types of cystadenomas: serous and mucinous. Serous cystadenomas are filled with a clear or watery fluid, while mucinous cystadenomas contain a thick, gelatinous material. Although they are generally not harmful, these tumors can grow quite large and cause discomfort or other symptoms due to their size or location. In some cases, cystadenomas may undergo malignant transformation and develop into cancerous tumors, known as cystadenocarcinomas. Regular medical follow-up and monitoring are essential for individuals diagnosed with cystadenomas to ensure early detection and treatment of any potential complications.

Viral hepatitis in humans refers to inflammation of the liver caused by infection with viruses that primarily target the liver. There are five main types of human viral hepatitis, designated as Hepatitis A, B, C, D, and E virus (HAV, HBV, HCV, HDV, and HEV). These viruses can cause a range of illnesses, from acute self-limiting hepatitis to chronic hepatitis, which can lead to cirrhosis and liver cancer.

1. Hepatitis A virus (HAV) is typically spread through the fecal-oral route, often through contaminated food or water. It usually results in an acute self-limiting infection, but rarely can cause chronic hepatitis in individuals with weakened immune systems.
2. Hepatitis B virus (HBV) is primarily transmitted through contact with infected blood, semen, and other bodily fluids. It can lead to both acute and chronic hepatitis, which may result in cirrhosis and liver cancer if left untreated.
3. Hepatitis C virus (HCV) is predominantly spread through exposure to infected blood, such as through sharing needles or receiving contaminated blood transfusions. Chronic hepatitis C is common, and it can lead to serious liver complications like cirrhosis and liver cancer if not treated.
4. Hepatitis D virus (HDV) is an incomplete virus that requires the presence of HBV for its replication. HDV infection occurs only in individuals already infected with HBV, leading to more severe liver disease compared to HBV monoinfection.
5. Hepatitis E virus (HEV) is primarily transmitted through the fecal-oral route, often through contaminated food or water. It usually results in an acute self-limiting infection but can cause chronic hepatitis in pregnant women and individuals with weakened immune systems.

Prevention measures include vaccination for HAV and HBV, safe sex practices, avoiding sharing needles, and ensuring proper hygiene and sanitation to prevent fecal-oral transmission.

Clonorchiasis is a parasitic infection caused by the trematode worm Clonorchis sinensis, also known as the Chinese liver fluke. This flatworm infects the bile ducts and liver of humans and other animals, leading to inflammation, obstruction, and potential complications such as cholangitis, cirrhosis, and cholangiocarcinoma (bile duct cancer).

Humans become infected with Clonorchis sinensis by consuming raw or undercooked freshwater fish that contain metacercariae, the infective larval stage of the parasite. The larvae excyst in the small intestine and migrate to the bile ducts, where they mature into adult worms and reproduce. Eggs are released into the stool and can contaminate water sources if proper sanitation is not maintained.

Symptoms of clonorchiasis may include abdominal pain, diarrhea, nausea, vomiting, and liver enlargement. In severe cases, patients may experience jaundice, ascites (fluid accumulation in the abdomen), and weight loss. Diagnosis is typically made by detecting eggs in stool samples or identifying the parasite in biopsied tissue. Treatment involves administering anthelmintic drugs such as praziquantel to eliminate the infection. Preventive measures include avoiding consumption of raw or undercooked fish and maintaining good hygiene practices.

A hepatic portoenterostomy, also known as Kasai procedure, is a surgical operation performed on infants with extrahepatic biliary atresia. This condition is characterized by the absence or abnormal formation of the bile ducts that carry bile from the liver to the small intestine, leading to obstruction and damage to the liver.

During a hepatic portoenterostomy, the surgeon creates an anastomosis (connection) between the portal vein, which brings blood to the liver, and a loop of intestine. This connection allows bile to flow directly from the liver into the intestine, bypassing the blocked or absent bile ducts. The goal of the procedure is to restore bile flow and prevent further damage to the liver.

The success of the procedure varies, but it can help improve the child's quality of life and delay or prevent the need for a liver transplant in some cases. However, many children with biliary atresia will eventually require a liver transplant as the disease progresses.

I'm sorry for any confusion, but "Viral Hepatitis, Animal" is not a standard medical classification or definition. Hepatitis refers to inflammation of the liver, and viral hepatitis refers to inflammation caused by a virus. The term "animal" in this context doesn't provide a clear meaning.

However, it's worth noting that some animals can contract viral hepatitis, similar to humans. For instance, there are hepatitis A, B, and C-like viruses that have been identified in various animal species. These are typically not transmissible to humans.

If you're referring to a specific medical condition or context, could you please provide more details? I'd be happy to help further with more information.

Sprague-Dawley rats are a strain of albino laboratory rats that are widely used in scientific research. They were first developed by researchers H.H. Sprague and R.C. Dawley in the early 20th century, and have since become one of the most commonly used rat strains in biomedical research due to their relatively large size, ease of handling, and consistent genetic background.

Sprague-Dawley rats are outbred, which means that they are genetically diverse and do not suffer from the same limitations as inbred strains, which can have reduced fertility and increased susceptibility to certain diseases. They are also characterized by their docile nature and low levels of aggression, making them easier to handle and study than some other rat strains.

These rats are used in a wide variety of research areas, including toxicology, pharmacology, nutrition, cancer, and behavioral studies. Because they are genetically diverse, Sprague-Dawley rats can be used to model a range of human diseases and conditions, making them an important tool in the development of new drugs and therapies.

Transaminases, also known as aminotransferases, are a group of enzymes found in various tissues of the body, particularly in the liver, heart, muscle, and kidneys. They play a crucial role in the metabolism of amino acids, the building blocks of proteins.

There are two major types of transaminases: aspartate aminotransferase (AST) and alanine aminotransferase (ALT). Both enzymes are normally present in low concentrations in the bloodstream. However, when tissues that contain these enzymes are damaged or injured, such as during liver disease or muscle damage, the levels of AST and ALT in the blood may significantly increase.

Measurement of serum transaminase levels is a common laboratory test used to assess liver function and detect liver injury or damage. Increased levels of these enzymes in the blood can indicate conditions such as hepatitis, liver cirrhosis, drug-induced liver injury, heart attack, and muscle disorders. It's important to note that while elevated transaminase levels may suggest liver disease, they do not specify the type or cause of the condition, and further diagnostic tests are often required for accurate diagnosis and treatment.

17-alpha-Hydroxypregnenolone is a steroid hormone that is produced in the adrenal glands and, to a lesser extent, in the gonads (ovaries and testes). It is an intermediate in the biosynthesis of steroid hormones, including cortisol, aldosterone, and sex hormones such as testosterone and estrogen.

17-alpha-Hydroxypregnenolone is formed from pregnenolone through the action of the enzyme 17α-hydroxylase. It can then be converted to 17-hydroxyprogesterone, which is a precursor to both cortisol and androgens such as testosterone.

While 17-alpha-Hydroxypregnenolone itself does not have significant physiological activity, its role in the biosynthesis of other steroid hormones makes it an important intermediate in the endocrine system. Dysregulation of its production or metabolism can contribute to various medical conditions, such as congenital adrenal hyperplasia and certain forms of cancer.

Kupffer cells are specialized macrophages that reside in the liver, particularly in the sinusoids of the liver's blood circulation system. They play a crucial role in the immune system by engulfing and destroying bacteria, microorganisms, and other particles that enter the liver via the portal vein. Kupffer cells also contribute to the clearance of damaged red blood cells, iron metabolism, and the regulation of inflammation in the liver. They are named after the German pathologist Karl Wilhelm von Kupffer who first described them in 1876.

Cefotiam is a type of antibiotic known as a cephalosporin, which is used to treat various bacterial infections. It works by interfering with the bacteria's ability to form a cell wall, leading to bacterial cell death. Cefotiam has a broad spectrum of activity and is effective against many gram-positive and gram-negative bacteria.

Here is the medical definition of 'Cefotiam':

Cefotiam is a semisynthetic, broad-spectrum, beta-lactam antibiotic belonging to the cephalosporin class. It has activity against both gram-positive and gram-negative bacteria, including many strains that are resistant to other antibiotics. Cefotiam inhibits bacterial cell wall synthesis by binding to penicillin-binding proteins (PBPs), leading to bacterial cell death.

Cefotiam is available in various formulations, including intravenous (IV) and intramuscular (IM) injections, for the treatment of a wide range of infections, such as:

* Lower respiratory tract infections (e.g., pneumonia, bronchitis)
* Urinary tract infections (e.g., pyelonephritis, cystitis)
* Skin and soft tissue infections (e.g., cellulitis, wound infections)
* Bone and joint infections (e.g., osteomyelitis, septic arthritis)
* Intra-abdominal infections (e.g., peritonitis, appendicitis)
* Septicemia (bloodstream infections)

Cefotiam is generally well tolerated, but like other antibiotics, it can cause side effects, including gastrointestinal symptoms (e.g., nausea, vomiting, diarrhea), skin rashes, and allergic reactions. In rare cases, cefotiam may cause serious adverse effects, such as seizures, interstitial nephritis, or hemorrhagicystitis. It should be used with caution in patients with a history of allergy to beta-lactam antibiotics, impaired renal function, or a history of seizure disorders.

It is essential to complete the full course of treatment as prescribed by a healthcare professional, even if symptoms improve, to ensure that the infection is entirely eradicated and to reduce the risk of developing antibiotic resistance.

X-ray computed tomography (CT or CAT scan) is a medical imaging method that uses computer-processed combinations of many X-ray images taken from different angles to produce cross-sectional (tomographic) images (virtual "slices") of the body. These cross-sectional images can then be used to display detailed internal views of organs, bones, and soft tissues in the body.

The term "computed tomography" is used instead of "CT scan" or "CAT scan" because the machines take a series of X-ray measurements from different angles around the body and then use a computer to process these data to create detailed images of internal structures within the body.

CT scanning is a noninvasive, painless medical test that helps physicians diagnose and treat medical conditions. CT imaging provides detailed information about many types of tissue including lung, bone, soft tissue and blood vessels. CT examinations can be performed on every part of the body for a variety of reasons including diagnosis, surgical planning, and monitoring of therapeutic responses.

In computed tomography (CT), an X-ray source and detector rotate around the patient, measuring the X-ray attenuation at many different angles. A computer uses this data to construct a cross-sectional image by the process of reconstruction. This technique is called "tomography". The term "computed" refers to the use of a computer to reconstruct the images.

CT has become an important tool in medical imaging and diagnosis, allowing radiologists and other physicians to view detailed internal images of the body. It can help identify many different medical conditions including cancer, heart disease, lung nodules, liver tumors, and internal injuries from trauma. CT is also commonly used for guiding biopsies and other minimally invasive procedures.

In summary, X-ray computed tomography (CT or CAT scan) is a medical imaging technique that uses computer-processed combinations of many X-ray images taken from different angles to produce cross-sectional images of the body. It provides detailed internal views of organs, bones, and soft tissues in the body, allowing physicians to diagnose and treat medical conditions.

Recurrence, in a medical context, refers to the return of symptoms or signs of a disease after a period of improvement or remission. It indicates that the condition has not been fully eradicated and may require further treatment. Recurrence is often used to describe situations where a disease such as cancer comes back after initial treatment, but it can also apply to other medical conditions. The likelihood of recurrence varies depending on the type of disease and individual patient factors.

A chronic disease is a long-term medical condition that often progresses slowly over a period of years and requires ongoing management and care. These diseases are typically not fully curable, but symptoms can be managed to improve quality of life. Common chronic diseases include heart disease, stroke, cancer, diabetes, arthritis, and COPD (chronic obstructive pulmonary disease). They are often associated with advanced age, although they can also affect children and younger adults. Chronic diseases can have significant impacts on individuals' physical, emotional, and social well-being, as well as on healthcare systems and society at large.

Postoperative complications refer to any unfavorable condition or event that occurs during the recovery period after a surgical procedure. These complications can vary in severity and may include, but are not limited to:

1. Infection: This can occur at the site of the incision or inside the body, such as pneumonia or urinary tract infection.
2. Bleeding: Excessive bleeding (hemorrhage) can lead to a drop in blood pressure and may require further surgical intervention.
3. Blood clots: These can form in the deep veins of the legs (deep vein thrombosis) and can potentially travel to the lungs (pulmonary embolism).
4. Wound dehiscence: This is when the surgical wound opens up, which can lead to infection and further complications.
5. Pulmonary issues: These include atelectasis (collapsed lung), pneumonia, or respiratory failure.
6. Cardiovascular problems: These include abnormal heart rhythms (arrhythmias), heart attack, or stroke.
7. Renal failure: This can occur due to various reasons such as dehydration, blood loss, or the use of certain medications.
8. Pain management issues: Inadequate pain control can lead to increased stress, anxiety, and decreased mobility.
9. Nausea and vomiting: These can be caused by anesthesia, opioid pain medication, or other factors.
10. Delirium: This is a state of confusion and disorientation that can occur in the elderly or those with certain medical conditions.

Prompt identification and management of these complications are crucial to ensure the best possible outcome for the patient.

Islets of Langerhans transplantation is a surgical procedure that involves the transplantation of isolated islets from a deceased donor's pancreas into another person with type 1 diabetes. The islets of Langerhans are clusters of cells within the pancreas that produce hormones, including insulin, which regulates blood sugar levels.

In type 1 diabetes, the body's immune system mistakenly attacks and destroys these insulin-producing cells, leading to high blood sugar levels. Islet transplantation aims to replace the damaged islets with healthy ones from a donor, allowing the recipient's body to produce and regulate its own insulin again.

The procedure involves extracting the islets from the donor pancreas and infusing them into the recipient's liver through a small incision in the abdomen. Once inside the liver, the islets can sense glucose levels in the bloodstream and release insulin as needed to maintain normal blood sugar levels.

Islet transplantation has shown promising results in improving blood sugar control and reducing the risk of severe hypoglycemia (low blood sugar) in people with type 1 diabetes. However, it requires long-term immunosuppressive therapy to prevent rejection of the transplanted islets, which can have side effects and increase the risk of infections.

A Choledochal cyst is a congenital dilatation or abnormal enlargement of the bile ducts, which are the tubes that carry bile from the liver to the small intestine. Bile is a digestive juice produced by the liver that helps in the digestion of fats.

Choledochal cysts can be classified into several types based on their location and the anatomy of the biliary tree. The most common type, called Type I, involves dilatation of the common bile duct. Other types include dilatation of the intrahepatic bile ducts (Type II), dilatation of both the intrahepatic and extrahepatic bile ducts (Type III), and multiple cystic dilatations of the bile ducts (Type IV).

Choledochal cysts are more common in females than males, and they can present at any age. Symptoms may include abdominal pain, jaundice, vomiting, and fever. Complications of choledochal cysts can include bile duct stones, infection, and cancer. Treatment typically involves surgical removal of the cyst, followed by reconstruction of the biliary tree.

GeneReview/NIH/UW entry on Low γ-GT Familial Intrahepatic Cholestasis OMIM entry on CHOLESTASIS, PROGRESSIVE FAMILIAL ... Progressive familial intrahepatic cholestasis (PFIC) is a group of familial cholestatic conditions caused by defects in biliary ... "eMedicine - Progressive Familial Intrahepatic Cholestasis : Article by Karan M Emerick, MD". Retrieved 2007-07-21. Bull LN, van ... "Orphanet: Progressive familial intrahepatic cholestasis". www.orpha.net. Retrieved 29 September 2019. Shneider BL (2004). " ...
... (ICP), also known as obstetric cholestasis, cholestasis of pregnancy, jaundice of ... The causes of intrahepatic cholestasis of pregnancy are still not fully understood, but are thought to be caused through a ... July 1989). "Intrahepatic cholestasis of pregnancy in twin pregnancies". Journal of Hepatology. 9 (1): 84-90. doi:10.1016/0168- ... "Intrahepatic cholestasis of pregnancy". www.marchofdimes.org. Retrieved 2022-04-27. Dixon, PH; Wadsworth, CA; Chambers, J; ...
... benign recurrent intrahepatic cholestasis, biliary atresia, and intrahepatic cholestasis of pregnancy. Chronic cholestasis ... Familial intrahepatic cholestasis (FIH) is a group of disorders that lead to intrahepatic cholestasis in children. Most often, ... density lipoprotein found in cholestasis Intrahepatic cholestasis of pregnancy Progressive familial intrahepatic cholestasis ... Intrahepatic cholestasis of pregnancy (ICP) is an acute cause of cholestasis that manifests most commonly in the third ...
Pauli-Magnus C, Meier PJ, Stieger B (2010). "Genetic determinants of drug-induced cholestasis and intrahepatic cholestasis of ... This is seen in intrahepatic cholestasis of pregnancy, which occurs in 0.4 to 15% of pregnancies (highly variable depending on ... Arrese M, Reyes H (2006). "Intrahepatic cholestasis of pregnancy: a past and present riddle". Ann Hepatol. 5 (3): 202-5. doi: ... Pusl T, Beuers U (2007). "Intrahepatic cholestasis of pregnancy". Orphanet J Rare Dis. 2: 26. doi:10.1186/1750-1172-2-26. PMC ...
... including types of cholestasis such as intrahepatic cholestasis of pregnancy, portosystemic shunt, and hepatic microvascular ... Pusl T, Beuers U (2007). "Intrahepatic cholestasis of pregnancy". Orphanet J Rare Dis. 2: 26. doi:10.1186/1750-1172-2-26. PMC ... Glantz A, Marschall HU, Lammert F, Mattsson LA (December 2005). "Intrahepatic cholestasis of pregnancy: a randomized controlled ... primary sclerosing cholangitis or intrahepatic cholestasis of pregnancy. Treatment with ursodeoxycholic acid has been used for ...
Davit-Spraul A, Gonzales E, Baussan C, Jacquemin E (January 2009). "Progressive familial intrahepatic cholestasis". Orphanet ... Intrahepatic cholangiocarcinoma (CCA) is an epithelial cancer of the intra-hepatic biliary tree branches. Intrahepatic CCA is ... Progressive familial intrahepatic cholestasis (associated with HCC) and Trisomy 18 (associated with hepatoblastoma). Many ... In terms of intrahepatic cholangiocarcinoma, we currently do not have sufficient epidemiological data because it is a rare ...
ABCB11 Cholestasis, benign recurrent intrahepatic; 243300; ATP8B1 Cholestasis, familial intrahepatic, of pregnancy; 147480; ... ABCB4 Cholestasis, progressive familial intrahepatic 1; 211600; ATP8B1 Cholestasis, progressive familial intrahepatic 2; 601847 ... ABCB11 Cholestasis, progressive familial intrahepatic 3; 602347; ABCB4 Cholestasis, progressive familial intrahepatic 4; 607765 ... and cholestasis 1; 208085; VPS33B Arthrogryposis, renal dysfunction, and cholestasis 2; 613404; VIPAR Arthropathy, progressive ...
It has been used in the symptomatic treatment of itching due to intrahepatic cholestasis of pregnancy. Gonzalez MC, Iglesias J ... Reyes H, Simon FR (August 1993). "Intrahepatic cholestasis of pregnancy: an estrogen-related disease". Semin Liver Dis. 13 (3 ... September 1992). "Epomediol ameliorates pruritus in patients with intrahepatic cholestasis of pregnancy". J Hepatol. 16 (1-2): ... Reyes H (December 1992). "The spectrum of liver and gastrointestinal disease seen in cholestasis of pregnancy". Gastroenterol ...
UDCA has been used for intrahepatic cholestasis of pregnancy. UDCA lessens itching in the mother and may reduce the number of ... September 2019). "Ursodeoxycholic acid versus placebo in women with intrahepatic cholestasis of pregnancy (PITCHES): a ... "Pharmacological interventions for treating intrahepatic cholestasis of pregnancy". The Cochrane Database of Systematic Reviews ... "Ursodeoxycholic acid use is associated with significant risk of morbidity and mortality in infants with cholestasis: A strobe ...
Mutations in this gene may result in progressive familial intrahepatic cholestasis type 1 and in benign recurrent intrahepatic ... This protein is associated with progressive familial intrahepatic cholestasis type 1 as well as benign recurrent intrahepatic ... Fatal familial intrahepatic cholestasis in an Amish kindred". Am. J. Dis. Child. 117 (1): 112-24. doi:10.1001/archpedi. ... 2004). "Progressive familial intrahepatic cholestasis, type 1, is associated with decreased farnesoid X receptor activity". ...
"Recurrent intrahepatic cholestasis of pregnancy - biochemical and clinical". Ginekologia Polska, 1974. "Free amino acids in the ... cholestasis in pregnancy, pathophysiology of blood coagulation in pregnancy, gestational diabetes, infections in pregnancy, ...
"Intrahepatic 'cholestasis facies': Is it specific for Alagille syndrome?". The Journal of Pediatrics. 103 (2): 205-8. doi: ... Cholestasis facies are a type of facies considered a symptom of Alagille syndrome. However it appears not to be specific but "a ... Specific or cholestasis facies?". American Journal of Medical Genetics. 112 (2): 163-70. doi:10.1002/ajmg.10579. PMID 12244550 ... general feature of congenital intrahepatic cholestatic liver disease". Sokol, Ronald J.; Heubi, James E.; Balistreri, William F ...
... is a gene associated with progressive familial intrahepatic cholestasis type 2 (PFIC2). PFIC2 caused by mutations in the ... November 1998). "A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis ... April 2009). "Contribution of variant alleles of ABCB11 to susceptibility to intrahepatic cholestasis of pregnancy". Gut. 58 (4 ... Kalaranjini KV, Glaxon JA, Vasudevan S, Arunkumar ML (June 2021). "Benign recurrent intrahepatic cholestasis - 2 (BRIC-2)/ ...
Sokol RJ, Heubi JE, Balistreri WF (August 1983). "Intrahepatic "cholestasis facies": is it specific for Alagille syndrome?". ... Progressive familial intrahepatic cholestasis synd/729 at Who Named It? Alagille D, Odièvre M, Gautier M, Dommergues JP ( ... but they are characteristic of patients with intrahepatic cholestatic liver disease. So while these facial characteristics are ...
... is associated with progressive familial intrahepatic cholestasis type 3 and intrahepatic cholestasis of pregnancy. The ... 1998). "Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis". Proceedings of the National Academy of ... 1999). "Heterozygous non-sense mutation of the MDR3 gene in familial intrahepatic cholestasis of pregnancy". Lancet. 353 (9148 ... 2000). "Heterozygous MDR3 missense mutation associated with intrahepatic cholestasis of pregnancy: evidence for a defect in ...
Hernández R, Nazar E (1982). "Effect of silymarin in intrahepatic cholestasis of pregnancy (preliminary communication)". ... including but not limited to effectively treating intrahepatic cholestasis of pregnancy. Silymarin is also devoid of ...
Ray K (September 2022). "Positive phase III results for odevixibat for progressive familial intrahepatic cholestasis". Nature ... is a medication for the treatment of progressive familial intrahepatic cholestasis. It is taken by mouth. Odevixibat is a ... in the European Union for the treatment of progressive familial intrahepatic cholestasis in people aged six months and older. ... and evaluate its effects on bile acid levels and symptoms in people with progressive familial intrahepatic cholestasis. A 24- ...
Foitl DR, Hyman G, Lefkowitch JH (February 1989). "Jaundice and intrahepatic cholestasis following high-dose megestrol acetate ... Case reports of deep vein thrombosis, pulmonary embolism, jaundice, intrahepatic cholestasis, and meningiomas in association ...
Progressive intrahepatic cholestasis Treatment Schedule: 3 to 5 eight-hour treatment sessions on consecutive days Continuous ... Progressive intrahepatic cholestasis Treatment Schedule: 3 to 5 eight-hour treatment sessions on consecutive days Continuous ... Saich, R; Collins, P; Ala, A; Standish, R; Hodgson, H (May 2005). "Benign recurrent intrahepatic cholestasis with secondary ... Benign intrahepatic cholestasis (BIC) Biliary Atresia Goals of MARS Therapy Attenuate pruritus symptoms and improve patients' ...
... genetic defect resulting in hypoplastic intrahepatic bile ducts) Progressive familial intrahepatic cholestasis Pyknocytosis ( ... Bilirubin levels greater than 10 times normal could indicate neoplastic or intrahepatic cholestasis. Levels lower than this ... Low levels of albumin tend to indicate a chronic condition, while the level is normal in hepatitis and cholestasis.[citation ... GGT levels greater than 10 times normal typically indicate cholestasis. Levels 5-10 times tend to indicate viral hepatitis. ...
"Mutations in the nuclear bile acid receptor FXR cause progressive familial intrahepatic cholestasis". Nature Communications. 7 ...
Aagenaes, O.; van der Hagen, C. B.; Refsum, S. (1968-12-01). "Hereditary recurrent intrahepatic cholestasis from birth". ... Heiberg A (May 2001). "Aagenaes syndrome: lymphedema and intrahepatic cholestasis". Tidsskr Nor Laegeforen. 121 (14): 1718-9. ... It is also called cholestasis-lymphedema syndrome (CLS). The first case of cholestasis usually improves spontaneously during ... Aagenaes, Øystein (January 1998). "Hereditary Cholestasis with Lymphoedema (Aagenaes Syndrome, Cholestasis-Lymphoedema Syndrome ...
used IBD sharing to identify the chromosomal location of a gene responsible for benign recurrent intrahepatic cholestasis in an ... Mapping a gene for benign recurrent intrahepatic cholestasis". Nature Genetics. 8 (4): 380-386. doi:10.1038/ng1294-380. hdl: ...
Intrahepatic cholestasis of pregnancy List of cutaneous conditions Rapini RP, Bolognia JL, Jorizzo JL (2007). Dermatology: 2- ... Cholestasis means "the slowing or stopping of bile flow" which can be caused by any number of diseases of the liver (which ... cholestasis (also see drug-induced pruritus), and chronic hepatitis C viral infection and other forms of viral hepatitis. ...
Intrahepatic cholestasis of pregnancy List of cutaneous conditions Matz H, Orion E, Wolf R (2006). "Pruritic urticarial papules ... Intrahepatic Cholestasis of Pregnancy, and Atopic Eruption of Pregnancy". Dermatology Research and Practice. 2015: 979635. doi: ...
"Therapeutic interventions in progressive familial intrahepatic cholestasis: experience from a tertiary care center in north ...
... is associated with type II citrullinemia and neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). ...
Other vitamin K deficient states include: biliary obstruction, intrahepatic cholestasis, intestinal malabsorption and chronic ...
... steroid oxidoreductase is mutated in progressive intrahepatic cholestasis". J. Clin. Invest. 106 (9): 1175-84. doi:10.1172/ ... Mutations in the HSD3B7 gene are associated with a congenital bile acid synthesis defect which leads to neonatal cholestasis, a ...
... intrahepatic cholestasis), hypolipidemic drugs, or changes following gallbladder removal (cholecystectomy). Conditions ...
GeneReview/NIH/UW entry on Low γ-GT Familial Intrahepatic Cholestasis OMIM entry on CHOLESTASIS, PROGRESSIVE FAMILIAL ... Progressive familial intrahepatic cholestasis (PFIC) is a group of familial cholestatic conditions caused by defects in biliary ... "eMedicine - Progressive Familial Intrahepatic Cholestasis : Article by Karan M Emerick, MD". Retrieved 2007-07-21. Bull LN, van ... "Orphanet: Progressive familial intrahepatic cholestasis". www.orpha.net. Retrieved 29 September 2019. Shneider BL (2004). " ...
... is characterized by episodes of liver dysfunction called cholestasis. Explore symptoms, inheritance, genetics of this condition ... Differential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type ... medlineplus.gov/genetics/condition/benign-recurrent-intrahepatic-cholestasis/ Benign recurrent intrahepatic cholestasis. ... Benign recurrent intrahepatic cholestasis (BRIC) is characterized by episodes of liver. dysfunction called cholestasis. During ...
... is a class of chronic cholestasis disorders that begin in infancy and usually progress to cirrhosis within the first decade of ... Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic ... encoded search term (Progressive Familial Intrahepatic Cholestasis) and Progressive Familial Intrahepatic Cholestasis What to ... Progressive familial intrahepatic cholestasis (PFIC) is a class of chronic cholestasis disorders that comprises a variety of ...
... level and fetal pulmonary surfactant in rats and study the effects of BA on fetal lung in rat model of intrahepatic cholestasis ... Effect of Bile Acid on Fetal Lung in Rat Model of Intrahepatic Cholestasis of Pregnancy. Ling Yu. ,1Yiling Ding. ,1Ting Huang. ... Intrahepatic cholestasis of pregnancy (ICP) is liver disease which could lead to premature birth, fetal distress and neonatal ... X. Tian and J. Sun, "Establishment of model of intrahepatic cholestasis in pregnant rats," Chinese Journal of Comparative ...
... is a class of chronic cholestasis disorders that begin in infancy and usually progress to cirrhosis within the first decade of ... Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic ... encoded search term (Progressive Familial Intrahepatic Cholestasis) and Progressive Familial Intrahepatic Cholestasis What to ... Progressive familial intrahepatic cholestasis (PFIC) is a class of chronic cholestasis disorders that comprises a variety of ...
17Q12 MICRODELETION: A RARE ETIOLOGY OF INTRAHEPATIC CHOLESTASIS. Tania Carvalho 1 Margarida Gomes Gonçalves Pedro Antunes ...
Intrahepatic cholestasis of pregnancy (ICP) is a condition specific to pregnancy, leading to increased fetal morbidity and ... Figure 1. Flow chart of intrahepatic cholestasis of pregnancy (ICP) cases included in the study. Flow chart showing the ICP ... Figure 1. Flow chart of intrahepatic cholestasis of pregnancy (ICP) cases included in the study. Flow chart showing the ICP ... Table 1. Clinical characteristics of women with intrahepatic cholestasis of pregnancy (ICP) and normal pregnancies.. Table 2. ...
Learn about diagnosis and specialist referrals for Benign recurrent intrahepatic cholestasis type 2. ... Benign recurrent intrahepatic cholestasis type 2. Other Names: BRIC type 2; BRIC2BRIC type 2; BRIC2. Read More ...
Primum non nocere: how active management became modus operandi for intrahepatic cholestasis of pregnancy.. Cassandra E ... The Royal College of Obstetrics and Gynecology does not endorse routine active management of intrahepatic cholestasis of ...
... is a reversible type of hormonally influenced cholestasis. It frequently develops in late pregnancy in individuals who are ... encoded search term (Intrahepatic Cholestasis of Pregnancy) and Intrahepatic Cholestasis of Pregnancy What to Read Next on ... Bile acid profiles in intrahepatic cholestasis of pregnancy: is this the solution to the enigma of intrahepatic cholestasis of ... Intrahepatic Cholestasis of Pregnancy Medication. Updated: Aug 30, 2017 * Author: Fidelma B Rigby, MD; Chief Editor: Ronald M ...
Intrahepatic" by people in this website by year, and whether "Cholestasis, Intrahepatic" was a major or minor topic of these ... "Cholestasis, Intrahepatic" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH (Medical ... The incidence of coagulopathy in pregnant patients with intrahepatic cholestasis: should we delay or avoid neuraxial analgesia ... Below are the most recent publications written about "Cholestasis, Intrahepatic" by people in Profiles. ...
Judds Legacy is a nonprofit raising awareness for a rare pregnancy condition called Intrahepatic Cholestasis Pregnancy that ... All proceeds from the event go to Judds Legacy, a non profit organization that raises awareness of Intrahepatic Cholestasis of ... Judds Legacy was founded to bring awareness to Intrahepatic Cholestasis of Pregnancy (ICP) in memory of Judd William Gardner, ...
... is a class of chronic cholestasis disorders that begin in infancy and usually progress to cirrhosis within the first decade of ... Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic ... encoded search term (Progressive Familial Intrahepatic Cholestasis) and Progressive Familial Intrahepatic Cholestasis What to ... Progressive familial intrahepatic cholestasis (PFIC) is a class of chronic cholestasis disorders that begin in infancy and ...
... the itching can be a symptom of intrahepatic cholestasis of pregnancy (ICP). Learn more about ICP and how it can affect your ... SMFM Recommendations: Intrahepatic Cholestasis of Pregnancy. *Risk of Stillbirth with Intrahepatic Cholestasis of Pregnancy: ... While some itching is common in pregnancy, if it becomes excessive, the itching can be a symptom of intrahepatic cholestasis of ...
Information on Benign recurrent intrahepatic cholestasis, which may include symptoms, causes, inheritance, treatments, orphan ... Dont fight Benign recurrent intrahepatic cholestasis alone.. Find your community on the free RareGuru App. Connect with other ... Benign recurrent intrahepatic cholestasis (BRIC) is a rare condition that affects the liver. People with this condition ... Connect with other users with Benign recurrent intrahepatic cholestasis on the RareGuru app Get the Free App!. ...
website Progressive Familial Intrahepatic Cholestasis Advocacy and Resource Network facebook Progressive Familial Intrahepatic ... Progressive familial intrahepatic cholestasis type 4. Synonyms: PFIC4 , TJP2 deficit. Related Disorders. Progressive familial ... Progressive familial intrahepatic cholestasis type 4. Get in touch with RARE Concierge.. Contact RARE Concierge ... Progressive familial intrahepatic cholestasis type 4?. Our RARE Concierge Services Guides are available to assist you by ...
Find featured drugs and generic for Intrahepatic Cholestasis at MIMS Singapore ... Intrahepatic cholestasis may be due to functional defects hepatocellularly or from obstructive lesions of the intrahepatic ... It can be classified into intrahepatic or extrahepatic cholestasis.. Extrahepatic cholestasis develops from mechanical blockage ... Cholestasis is bile formation and/or bile flow impairment that manifests as fatigue, pruritus and jaundice.. ...
Intrahepatic answers are found in the 5-Minute Clinical Consult powered by Unbound Medicine. Available for iPhone, iPad, ... Cholestasis of Pregnancy, Intrahepatic. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters ... "Cholestasis of Pregnancy, Intrahepatic." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im. ... Cholestasis of Pregnancy, Intrahepatic. (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5- ...
Intrahepatic cholestasis of pregnancy is characterized by pruritus, raised maternal liver enzymes and bile acids. It usually ... It is concluded that ursodeoxycholic acid in case of a severe early-onset intrahepatic cholestasis may be started in the early ... Ursodeoxycholic acid administration from the first trimester in case of a severe early-onset intrahepatic cholestasis of ... https://akjournals.com/search?q=%22ICP%2C+intrahepatic+cholestasis+of+pregnancy%22. The link was not copied. Your current ...
CURF Introduction: Modelling Progressive Familial Intrahepatic Cholestasis in vitro. Posted by Sriram Amirneni on September 17 ... My project is about the genetic disease progressive familial intrahepatic cholestasis (PFIC), which is estimated to affect ... These include cholestasis, jaundice, and pruritus. Pruritus, the intense urge to itch, is one of the most debilitating symptoms ...
... episodic cholestasis) to the severe end of the spectrum (persistent cholestasis). Sensorineural hearing loss (SNHL) is common ... Although mild-to-moderate ATP8B1 deficiency initially was thought to involve intermittent symptomatic cholestasis with a lack ... Severe ATP8B1 deficiency is characterized by infantile-onset cholestasis that progresses to cirrhosis, hepatic failure, and ... Familial intrahepatic cholestasis*Progressive familial intrahepatic cholestasis*Progressive familial intrahepatic cholestasis ...
1.1 Progressive Familial Intrahepatic Cholestasis (PFIC) 1.2 Alagille Syndrome (ALGS) 2 DOSAGE AND ADMINISTRATION 2.1 ... Progressive Familial Intrahepatic Cholestasis (PFIC) *the treatment of pruritus in patients 3 months of age and older with ... 1.1 Progressive Familial Intrahepatic Cholestasis (PFIC). BYLVAY is indicated for the treatment of pruritus in patients 3 ... 2.1 Recommended Dosage for Progressive Familial Intrahepatic Cholestasis (PFIC) in Patients Aged 3 Months and Older. * The ...
We have optimized all our processes to accept a wide range of samples, always adapting to each case ...
Do you qualify for these Progressive Familial Intrahepatic Cholestasis studies? Were researching treatments for 2023. ... Progressive Familial Intrahepatic Cholestasis clinical trials at University of California Health 2 in progress, 0 open to ... Maralixibat in Infant Participants With Cholestatic Liver Diseases Including Progressive Familial Intrahepatic Cholestasis ( ...
Intrahepatic Cholestasis of Pregnancy. Intrahepatic cholestasis of pregnancy (ICP) or obstetric cholestasis is a pregnancy- ... Tags: Intrahepatic Cholestasis of Pregnancy, Randox, Randox Reagents, Bile Acids, Reagents Educational Brochures, reagent, Bile ... Intrahepatic cholestasis of pregnancy is a condition that warrants attention, and accurate measurement of total bile acid ... Intrahepatic Cholestasis of Pregnancy. During pregnancy, the metabolic processes in the liver undergo significant adaptations ...
Intrahepatic cholestasis. 1 (12.5). Reduced fetal movements. 1 (12.5). Premature rupture of membranes. 1 (12.5). ...
Intrahepatic Cholestasis of Pregnancy. December 3, 2021. Sharon J. Parish, M.D.. Testosterone for Women: Everything You Need to ...
Intrahepatic cholestasis of pregnancy and associated causes of death : a cohort study with follow-up of 27-46 years. Hämäläinen ...
Maternal and fetal outcome in intrahepatic cholestasis of pregnancy at tertiary care institute of North India ... Mutlu MF, Aslan K, Guler I, Mutlu I, Erdem M, Bozkurt N, et al. Two cases of first onset intrahepatic cholestasis of pregnancy ... Raz Y, Lavie A, Vered Y, Goldiner I, Skornick-Rapaport A, Asher YL, et al. Severe intrahepatic cholestasis of pregnancy is a ... Floreani A, Gervasi MT. New insights on intrahepatic cholestasis of pregnancy. Clin Liver Dis. 2016;20:177-89. [CrossRef] [ ...
  • citation needed] Similar transport protein mutations are believed to pose a higher risk for intrahepatic cholestasis of pregnancy. (wikipedia.org)
  • citation needed] Alagille syndrome Intrahepatic cholestasis of pregnancy Liver transplantation RESERVED, INSERM US14-- ALL RIGHTS. (wikipedia.org)
  • To determine the correlation between maternal bile acid (BA) level and fetal pulmonary surfactant in rats and study the effects of BA on fetal lung in rat model of intrahepatic cholestasis of pregnancy. (hindawi.com)
  • Intrahepatic cholestasis of pregnancy (ICP) is liver disease which could lead to premature birth, fetal distress and neonatal asphyxia, and increasing risk of fetal morbidity and mortality [ 1 ]. (hindawi.com)
  • Intrahepatic cholestasis of pregnancy (ICP) is a condition specific to pregnancy, leading to increased fetal morbidity and mortality. (medscimonit.com)
  • Primum non nocere: how active management became modus operandi for intrahepatic cholestasis of pregnancy. (qxmd.com)
  • The Royal College of Obstetrics and Gynecology does not endorse routine active management of intrahepatic cholestasis of pregnancy (ICP)-affected pregnancies. (qxmd.com)
  • Many pharmacological agents have been used in the treatment of intrahepatic cholestasis of pregnancy (ICP). (medscape.com)
  • Intrahepatic cholestasis of pregnancy: an estrogen-related disease. (medscape.com)
  • Poupon R. Intrahepatic cholestasis of pregnancy: from bedside to bench to bedside. (medscape.com)
  • Heterozygous MDR3 missense mutation associated with intrahepatic cholestasis of pregnancy: evidence for a defect in protein trafficking. (medscape.com)
  • Schneider G, Paus TC, Kullak-Ublick GA, Meier PJ, Wienker TF, Lang T. Linkage between a new splicing site mutation in the MDR3 alias ABCB4 gene and intrahepatic cholestasis of pregnancy. (medscape.com)
  • Keitel V, Vogt C, Häussinger D, Kubitz R. Combined mutations of canalicular transporter proteins cause severe intrahepatic cholestasis of pregnancy. (medscape.com)
  • Intrahepatic cholestasis of pregnancy: three novel MDR3 gene mutations. (medscape.com)
  • Hardikar W, Kansal S, Oude Elferink RP, Angus P. Intrahepatic cholestasis of pregnancy: when should you look further? (medscape.com)
  • Judd's Legacy was founded to bring awareness to Intrahepatic Cholestasis of Pregnancy (ICP) in memory of Judd William Gardner, a baby that ICP robbed of the chance to draw breath. (juddslegacy.com)
  • All proceeds from the event go to Judd's Legacy, a non profit organization that raises awareness of Intrahepatic Cholestasis of Pregnancy. (juddslegacy.com)
  • While some itching is common in pregnancy, if it becomes excessive, the itching can be a symptom of intrahepatic cholestasis of pregnancy (ICP). (babiesafter35.com)
  • Intrahepatic cholestasis of pregnancy (ICP) is characterized by generalized pruritus without skin lesions in pregnancy, accompanied by elevated serum bile acid levels +/− elevated serum transaminase levels ( 1 , 2 ). (unboundmedicine.com)
  • Medicine Central , im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816694/all/Cholestasis_of_Pregnancy__Intrahepatic. (unboundmedicine.com)
  • Intrahepatic cholestasis of pregnancy is characterized by pruritus, raised maternal liver enzymes and bile acids. (akjournals.com)
  • We report here a case of an extremely rare, severe obstetric cholestasis with early onset treated by ursodeoxycholic acid from the 9th week of pregnancy, the earliest ever reported in the literature. (akjournals.com)
  • It is concluded that ursodeoxycholic acid in case of a severe early-onset intrahepatic cholestasis may be started in the early pregnancy to improve maternal condition and prevent fetal complications. (akjournals.com)
  • In this article, we will delve into the structural and functional aspects of bile acids and explore their significance in a condition called intrahepatic cholestasis of pregnancy (ICP). (randox.com)
  • One condition that can arise during pregnancy is intrahepatic cholestasis, commonly known as ICP. (randox.com)
  • Arora S, Huria A, Goel P, Kaur J, Dubey S. Maternal and fetal outcome in intrahepatic cholestasis of pregnancy at tertiary care institute of North India. (ijmsweb.com)
  • Intrahepatic cholestasis of pregnancy (IHCP) is the most common reversible form of hepatic disease in pregnancy. (ijmsweb.com)
  • There was no correlation found between cholestasis of pregnancy with preterm labor and meconium-stained liquor in the present study. (ijmsweb.com)
  • Itching over whole body was the predominant presenting complaints of cholestasis of pregnancy. (ijmsweb.com)
  • Pregnancy outcomes following antenatal screening for intrahepatic cholestasis of pregnancy (ICP). (nih.gov)
  • Antenatal thiopurine exposure in women with IBD is associated with intrahepatic cholestasis of pregnancy. (nih.gov)
  • MDR3 rs2109505 and rs1202283 polymorphisms are associated with susceptibility to intrahepatic cholestasis of pregnancy: A meta-analysis. (nih.gov)
  • Listen to Annalisa Post, MD, Assistant Professor of Obstetrics, Gynecology and Reproductive Sciences, Division of Maternal-Fetal Medicine, University of California, San Francisco School of Medicine , discuss ways to improve the management of intrahepatic cholestasis of pregnancy in this CME-accredited lecture. (audio-digest.org)
  • Explain the pathophysiology of intrahepatic cholestasis of pregnancy. (audio-digest.org)
  • Choose appropriate management strategies for intrahepatic cholestasis of pregnancy. (audio-digest.org)
  • Progressive familial intrahepatic cholestasis (PFIC) is a group of familial cholestatic conditions caused by defects in biliary epithelial transporters. (wikipedia.org)
  • However, episodes of liver dysfunction occasionally develop into a more severe, permanent form of liver disease known as progressive familial intrahepatic cholestasis (PFIC). (medlineplus.gov)
  • BRIC and PFIC are sometimes considered to be part of a spectrum of intrahepatic cholestasis disorders of varying severity. (medlineplus.gov)
  • Progressive familial intrahepatic cholestasis (PFIC) is a class of chronic cholestasis disorders that comprises a variety of genetic diseases. (medscape.com)
  • Progressive familial intrahepatic cholestasis (PFIC) is a class of chronic cholestasis disorders that begin in infancy and usually progress to cirrhosis within the first decade of life. (medscape.com)
  • Our mission is to improve the lives of patients and families worldwide affected by Progressive Familial Intrahepatic Cholestasis, PFIC. (globalgenes.org)
  • My project is about the genetic disease progressive familial intrahepatic cholestasis (PFIC), which is estimated to affect roughly 1 in 50,000 to 1 in 100,000 live births. (pitthonors.blog)
  • Progressive familial intrahepatic cholestasis (PFIC) is a disorder that causes progressive liver disease, which typically leads to liver failure. (nih.gov)
  • the treatment of pruritus in patients 3 months of age and older with progressive familial intrahepatic cholestasis (PFIC). (nih.gov)
  • Maralixibat in Infant Participants With Cholestatic Liver Diseases Including Progressive Familial Intrahepatic Cholestasis (PFIC) and Alagille Syndrome (ALGS). (ucbraid.org)
  • 12 months of age with Alagille Syndrome [ALGS] or Progressive Familial Intrahepatic Cholestasis [PFIC]. (ucbraid.org)
  • Odevixibat is used to treat pruritus (skin itching) in patients with progressive familial intrahepatic cholestasis (PFIC) and alagille syndrome (ALGS). (drugs.com)
  • Appropriate studies have not been performed on the relationship of age to the effects of odevixibat in children younger than 3 months of age with progressive familial intrahepatic cholestasis (PFIC) and in children younger than 12 months of age with alagille syndrome (ALGS) . (drugs.com)
  • When the results came back, the Bonczyk family finally had the answers they were looking for - Charles had Progressive Familial Intrahepatic Cholestasis (PFIC) - a rare genetic condition that causes increasingly severe damage to the liver and can lead to cirrhosis and complete liver failure. (liverfoundation.org)
  • Further to the recent announcement that the first drug treatment for PFIC has been approved by Medicines and Healthcare products Regulatory Agency (MHRA), CLDF are disappointed to hear that the National Institute for Health and Care Excellence (NICE) has decided to not recommend odevixibat for the treatment of progressive familial intrahepatic cholestasis (PFIC) in patients aged 6 months or older. (childliverdisease.org)
  • Jacquemin E, De Vree JM, Cresteil D, Sokal EM, Sturm E, Dumont M. The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood. (medscape.com)
  • Severe ATP8B1 deficiency is characterized by infantile-onset cholestasis that progresses to cirrhosis, hepatic failure, and early death. (nih.gov)
  • Reduced biliary phosphatidylcholine cannot counteract the detergent effects of bile salts, leading to cholestasis, cholangitis, cirrhosis and ultimately liver failure. (unav.edu)
  • The clinical presentation usually occurs first in childhood with progressive cholestasis. (wikipedia.org)
  • citation needed] Types of progressive familial intrahepatic cholestasis are as follows:[citation needed] Type 1 (OMIM #211600), also called Byler disease Type 2 (OMIM #601847), also called ABCB11 deficiency or BSEP deficiency Type 3 (OMIM #602347), also called ABCB4 deficiency or MDR3 deficiency Type 4 (OMIM #615878), from mutation in TJP2 The onset of the disease is usually before age 2, but patients have been diagnosed with PFIC even into adolescence. (wikipedia.org)
  • These terms now have been superseded by the term progressive familial intrahepatic cholestasis . (medscape.com)
  • Newly diagnosed with Progressive familial intrahepatic cholestasis type 4? (globalgenes.org)
  • Progressive familial intrahepatic cholestasis-2 (PFIC2) is an autosomal recessive disorder characterized by progressive liver disease with impairment of bile flow, but without hepatobiliary structural abnormality. (nih.gov)
  • 1998). For a phenotypic description and a discussion of genetic heterogeneity of progressive familial intrahepatic cholestasis, see PFIC1 (211600). (nih.gov)
  • Liver-directed gene therapy results in long-term correction of progressive familial intrahepatic cholestasis type 3 in mice. (genethon.com)
  • Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare monogenic disease caused by mutations in the ABCB4 gene, resulting in a reduction in biliary phosphatidylcholine. (unav.edu)
  • citation needed] Initial treatment is supportive, with the use of agents to treat cholestasis and pruritus, including the following:[citation needed] Ursodeoxycholic acid Cholestyramine Rifampin Naloxone, in refractory cases The partial external biliary diversion (PEBD) procedure is a surgical approach that diverts bile from the gallbladder externally into an ileostomy bag. (wikipedia.org)
  • Cholestasis is bile formation and/or bile flow impairment that manifests as fatigue, pruritus and jaundice. (mims.com)
  • These include cholestasis, jaundice, and pruritus. (pitthonors.blog)
  • Mutations in the ATP8B1 gene cause benign recurrent intrahepatic cholestasis type 1 (BRIC1), and mutations in the ABCB11 gene cause benign recurrent intrahepatic cholestasis type 2 (BRIC2). (medlineplus.gov)
  • Don't fight Benign recurrent intrahepatic cholestasis alone. (rareguru.com)
  • Connect with other caregivers and patients with Benign recurrent intrahepatic cholestasis and get the support you need. (rareguru.com)
  • Benign recurrent intrahepatic cholestasis (BRIC) is a rare condition that affects the liver. (rareguru.com)
  • BSEP is the major canalicular bile acid pump, and thus the loss of BSEP function results in severe hepatocellular cholestasis. (medscape.com)
  • Furthermore, in some persons with ATP8B1 deficiency the clinical findings can span the phenotypic spectrum, shifting over time from the mild end of the spectrum (episodic cholestasis) to the severe end of the spectrum (persistent cholestasis). (nih.gov)
  • We speculate that this compound ABCB4-ABCB11 genotype led to a severe intrahepatic cholestasis in the setting of HAV infection. (elsevier.es)
  • Retention of bile salts within hepatocytes, which are the only cell type to express BSEP, causes hepatocellular damage and cholestasis. (wikipedia.org)
  • citation needed] Liver biopsies typically show evidence of cholestasis (including bile plugs and bile infarcts), duct hypoplasia, hepatocellular injury, and Zone 3 fibrosis. (wikipedia.org)
  • The condition was inherited in an autosomal recessive manner and was characterized by hepatocellular cholestasis. (medscape.com)
  • Extrahepatic cholestasis develops from mechanical blockage in the duct system or hepatocellular defects. (mims.com)
  • Patients usually present in early childhood with cholestasis, jaundice, and failure to thrive. (wikipedia.org)
  • In this talk, Prof. Richard Thompson, King's College Hospital, discusses genetic causes of cholestasis. (bspghan.org.uk)
  • It can be classified into intrahepatic or extrahepatic cholestasis. (mims.com)
  • How ATP8B1 mutation leads to cholestasis is not yet well understood. (wikipedia.org)
  • Although mild-to-moderate ATP8B1 deficiency initially was thought to involve intermittent symptomatic cholestasis with a lack of hepatic fibrosis, it is now known that hepatic fibrosis may be present early in the disease course. (nih.gov)
  • DeLeon A, De Oliveira GS, Kalayil M, Narang S, McCarthy RJ, Wong CA. The incidence of coagulopathy in pregnant patients with intrahepatic cholestasis: should we delay or avoid neuraxial analgesia? (rush.edu)
  • Pseudohyponatremia needs to be considered in patients with low sodium and co-existing cholestasis from metastatic liver disease. (ama.ba)
  • Serum cholesterol levels are typically not elevated, as is seen usually in cholestasis, as the pathology is due to a transporter as opposed to an anatomical problem with biliary cells. (wikipedia.org)
  • The prevalence of IHCP was 4.08% in our population, however, women from urban area had higher incidence of cholestasis than rural population. (ijmsweb.com)
  • The first episode of cholestasis usually occurs in an affected person's teens or twenties. (medlineplus.gov)
  • Intrahepatic cholestasis may be due to functional defects hepatocellularly or from obstructive lesions of the intrahepatic biliary tract distal from the bile canaliculi. (mims.com)
  • It was previously identified as clinical entities known as Byler's disease and Greenland-Eskimo familial cholestasis. (wikipedia.org)
  • We describe a rare case of pseudohyponatremia in the setting of hypercholesterolemia caused by cholestasis due to metastatic liver disease and provide a review of the published cases in the literature. (ama.ba)
  • Episodes of cholestasis can last from weeks to months, and the time between episodes, during which there are usually no symptoms, can vary from weeks to years. (medlineplus.gov)
  • Because of a lack of fat absorption and loss of appetite, affected individuals often lose weight during episodes of cholestasis. (medlineplus.gov)
  • People with this condition experience episodes of cholestasis , during which the liver cells have a reduced ability to release bile (a digestive fluid). (rareguru.com)
  • In rare cases, however, it may relapse or cause prolonged cholestasis. (elsevier.es)
  • Because the problems with bile release occur within the liver (intrahepatic), the condition is described as intrahepatic cholestasis. (medlineplus.gov)
  • Pour plus d'information, et notamment pour consulter la liste des tiers intervenant sur notre site, consultez la Politique de cookies accessible en bas de page. (genethon.com)
  • This graph shows the total number of publications written about "Cholestasis, Intrahepatic" by people in this website by year, and whether "Cholestasis, Intrahepatic" was a major or minor topic of these publications. (rush.edu)
  • Below are the most recent publications written about "Cholestasis, Intrahepatic" by people in Profiles. (rush.edu)
  • Cholestasis, Intrahepatic" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (rush.edu)
  • Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare condition that affects the liver. (nih.gov)
  • When Do Symptoms of Progressive familial intrahepatic cholestasis type 3 Begin? (nih.gov)
  • Progressive familial intrahepatic cholestasis type 3 is a genetic disease. (nih.gov)
  • Progressive familial intrahepatic cholestasis (PFIC) is a group of familial cholestatic conditions caused by defects in biliary epithelial transporters. (wikipedia.org)
  • citation needed] Types of progressive familial intrahepatic cholestasis are as follows:[citation needed] Type 1 (OMIM #211600), also called Byler disease Type 2 (OMIM #601847), also called ABCB11 deficiency or BSEP deficiency Type 3 (OMIM #602347), also called ABCB4 deficiency or MDR3 deficiency Type 4 (OMIM #615878), from mutation in TJP2 The onset of the disease is usually before age 2, but patients have been diagnosed with PFIC even into adolescence. (wikipedia.org)
  • However, episodes of liver dysfunction occasionally develop into a more severe, permanent form of liver disease known as progressive familial intrahepatic cholestasis (PFIC). (medlineplus.gov)
  • Genetic mutations affecting hepatic bile salt transport molecules have also been found in patients with progressive familial intrahepatic cholestasis . (wikipedia.org)
  • Progressive familial intrahepatic cholestasis (PFIC) is a rare hereditary disease in which the liver cells struggle to produce and secrete bile. (liver.ca)
  • Progressive familial intrahepatic cholestasis (PFIC) refers to heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. (nih.gov)
  • Efficacy and Safety of Maralixibat in Patients with Progressive Familial Intrahepatic Cholestasis (MARCH): A Randomized Placebo-Controlled Phase 3 Study. (consultantlive.com)
  • Progressive familial intrahepatic cholestasis (PFIC) is a class of chronic cholestasis disorders that comprises a variety of genetic diseases. (medscape.com)
  • These terms now have been superseded by the term progressive familial intrahepatic cholestasis . (medscape.com)
  • Progressive Familial Intrahepatic Cholestasis (PFIC) is an inherited disorder that causes liver damage in the form of cirrhosis and related symptoms due to the accumulation of bile in the liver. (igenomix.net)
  • The Igenomix Progressive Familial Intrahepatic Cholestasis Precision Panel can be used to make a directed and accurate differential diagnosis of liver disease, ultimately leading to a better management and prognosis of the disease. (igenomix.net)
  • Systematic review of progressive familial intrahepatic cholestasis. (igenomix.net)
  • the treatment of pruritus in patients 3 months of age and older with progressive familial intrahepatic cholestasis (PFIC). (nih.gov)
  • More than 45 mutations in the ABCB4 gene have been found to cause a severe form of liver disease called progressive familial intrahepatic cholestasis type 3 (PFIC3) that usually leads to liver failure. (medlineplus.gov)
  • Progressive familial intrahepatic cholestasis or PFIC is a general term used to describe a group of genetic disorders involving the hepatocanalicular transporters. (gastrores.org)
  • Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of rare, genetic autosomal recessive disorders, resulting from defects in the mechanisms involved in bile formation with typical clinical, biochemical and histological features. (gastrores.org)
  • Bylvay is indicated for the treatment of progressive familial intrahepatic cholestasis (PFIC) in patients aged 6 months or older (see sections 4.4 and 5.1). (europa.eu)
  • 5. Combined features of low phospholipid-associated cholelithiasis and progressive familial intrahepatic cholestasis 3. (nih.gov)
  • 10. Liver-directed gene therapy results in long-term correction of progressive familial intrahepatic cholestasis type 3 in mice. (nih.gov)
  • 14. Clinical and genetic characterization of pediatric patients with progressive familial intrahepatic cholestasis type 3 (PFIC3): identification of 14 novel ABCB4 variants and review of the literatures. (nih.gov)
  • 16. A novel etiologic factor of highly elevated cholestanol levels: progressive familial intrahepatic cholestasis. (nih.gov)
  • 17. A missense mutation in ABCB4 gene involved in progressive familial intrahepatic cholestasis type 3 leads to a folding defect that can be rescued by low temperature. (nih.gov)
  • In this CME, James Squires MD, MS, discusses how to define the current understanding of progressive familial intrahepatic cholestasis (PFIC), delineate the gaps in current knowledge of PFIC disorders, and define opportunities for future collaborative research in PFIC. (upmcphysicianresources.com)
  • Define current understanding of progressive familial intrahepatic cholestasis (PFIC). (upmcphysicianresources.com)
  • Molecular overview of progressive familial intrahepatic cholestasis. (upmcphysicianresources.com)
  • Less common considerations include inborn errors of bile acid metabolism (urine for bile acids) and progressive familial intrahepatic cholestasis . (medscape.com)
  • It can also be called obstetric cholestasis. (pregnancy-baby-care.com)
  • There is a FB group called Obstetric Cholestasis Assist Australia and New Zealand, join if you like there's lots of help there! (babyhintsandtips.com)
  • I had obstetric cholestasis and was induced at 37+4 weeks. (babyhintsandtips.com)
  • TBA level determination in pregnant women is considered to be the most important biomarker for diagnosis and monitoring of ICP, also known as obstetric cholestasis (OC) [10-12]. (total-bile-acids.com)
  • The cause of obstetric cholestasis is not fully understood but it is believed to be multifactorial with genetic, environmental and hormonal factors being involved [13]. (total-bile-acids.com)
  • Mutations in the ATP8B1 gene cause benign recurrent intrahepatic cholestasis type 1 (BRIC1), and mutations in the ABCB11 gene cause benign recurrent intrahepatic cholestasis type 2 (BRIC2). (medlineplus.gov)
  • Piazzolla M, Castellaneta N, Novelli A, Agolini E, Cocciadiferro D, Resta L, Duda L, Barone M, Ierardi E, Di Leo A. Nonsense variant of ATP8B1 gene in heterozygosis and benign recurrent intrahepatic cholestasis: A case report and review of literature. (wjgnet.com)
  • The reversible type of hormonally influenced cholestasis leads to a restricted bile flow through the gallbladder and in turn, to an accumulation of bile acids in the liver and possibly in the bloodstream [7,14]. (total-bile-acids.com)
  • Jacquemin E, De Vree JM, Cresteil D, Sokal EM, Sturm E, Dumont M. The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood. (medscape.com)
  • Poddar U, Thapa BR, Das A, Bhattacharya A, Rao KN, Singh K. Neonatal cholestasis: differentiation of biliary atresia from neonatal hepatitis in a developing country. (ac.ir)
  • Clinical features differentiating biliary atresia from other causes of neonatal cholestasis. (ac.ir)
  • Bhatia V, Bavdekar A, Matthai J, Waikar Y, Sibal A. Management of neonatal cholestasis: consensus statement of the Pediatric Gastroenterology Chapter of Indian Academy of Pediatrics. (ac.ir)
  • It was previously identified as clinical entities known as Byler's disease and Greenland-Eskimo familial cholestasis. (wikipedia.org)
  • 3. First description of ABCB4 gene deletions in familial low phospholipid-associated cholelithiasis and oral contraceptives-induced cholestasis. (nih.gov)
  • As proof of principle, we applied DUCT to a mouse model for Alagille syndrome ( Jag1 Ndr/Ndr mice), characterized by intrahepatic bile duct paucity, that can spontaneously generate a biliary system in adulthood. (elifesciences.org)
  • Several abnormalities of liver function commonly noted in patients with Alagille syndrome reflect chronic cholestasis. (medscape.com)
  • Biliary atresia (BA) is an important etiology of liver disease in pediatric patients which manifests as extrahepatic cholestasis. (ac.ir)
  • Deficiency of ATP8B1 in the hepatocyte may result in loss of asymmetric distribution of the phospholipids in the canalicular membrane, thus reducing membrane stability and function of transmembrane transporters like ABCB11, impairing bile salt transport and decreasing hepatocyte resistance to bile salts, ultimately leading to reduced transport of bile salts in liver and gut resulting in cholestasis and watery diarrhea. (gastrores.org)
  • 18. Review article: liver disease in adults with variants in the cholestasis-related genes ABCB11, ABCB4 and ATP8B1. (nih.gov)
  • This health condition can be extrahepatic cholestasis that happens outside the liver, or intrahepatic cholestasis that happens inside the liver. (pregnancy-baby-care.com)
  • Diagnosis is based on clinical manifestations, liver ultrasonography, cholangiography and liver histology, as well as on specific tests for excluding other causes of childhood cholestasis. (nih.gov)
  • Estrogens, and particularly glucuronides such as estradiol-17β-D-glucuronide, have been shown to cause cholestasis in animal studies, by reducing bile acid uptake by hepatocytes . (wikipedia.org)
  • Infections can cause cholestasis. (msdmanuals.com)
  • in neonates receiving parental nutrition can also cause cholestasis. (msdmanuals.com)
  • citation needed] Initial treatment is supportive, with the use of agents to treat cholestasis and pruritus, including the following:[citation needed] Ursodeoxycholic acid Cholestyramine Rifampin Naloxone, in refractory cases The partial external biliary diversion (PEBD) procedure is a surgical approach that diverts bile from the gallbladder externally into an ileostomy bag. (wikipedia.org)
  • may result from extrahepatic or intrahepatic disorders, although some conditions overlap. (msdmanuals.com)
  • Serum cholesterol levels are typically not elevated, as is seen usually in cholestasis, as the pathology is due to a transporter as opposed to an anatomical problem with biliary cells. (wikipedia.org)
  • A higher incidence is seen in twin pregnancies, following in-vitro fertilization, in women older than 35 years, with history of cholestasis in previous pregnancies and in women with history of biliary disease [16-18]. (total-bile-acids.com)
  • In most cases, biliary atresia manifests several weeks after birth, probably after inflammation and scarring of the extrahepatic (and sometimes intrahepatic) bile ducts. (msdmanuals.com)
  • The clinical presentation usually occurs first in childhood with progressive cholestasis. (wikipedia.org)
  • The first episode of cholestasis usually occurs in an affected person's teens or twenties. (medlineplus.gov)
  • Cholestasis occurs in 1/2500 full-term infants. (msdmanuals.com)
  • Episodes of cholestasis can last from weeks to months, and the time between episodes, during which there are usually no symptoms, can vary from weeks to years. (medlineplus.gov)
  • Cholestasis is a major clinical sign in all three types [ 3 ]. (gastrores.org)
  • Liver biopsy specimens typically exhibit features suggestive of chronic cholestasis and paucity of interlobular bile ducts. (medscape.com)
  • When this happens, the common cause is generally cholestasis, a liver disease that can only affect pregnant women. (pregnancy-baby-care.com)
  • Pregnant women with cholestasis are monitored closely and if the baby's lungs have reached maturity, then the physician might induce labor. (pregnancy-baby-care.com)
  • I had cholestasis when pregnant with my son and was induced at 37 +2. (babyhintsandtips.com)
  • All infants less than 2 years old with cholestasis referred to the pediatric gastrointestinal ward were included in this study. (ac.ir)
  • 200 mg/dL) and hypertriglyceridemia (500-2000 mg/dL) are commonly present, reflective of underlying chronic cholestasis. (medscape.com)
  • Brzhozovskiy A, Kononikhin A, Bugrova AE, Kovalev GI, Schmit PO, Kruppa G, Nikolaev EN, Borchers CH . The Parallel Reaction Monitoring-Parallel Accumulation-Serial Fragmentation (prm-PASEF) Approach for Multiplexed Absolute Quantitation of Proteins in Human Plasma. (mcgill.ca)
  • Cholestasis can be diagnosed by doing a physical examination, a complete medical history, and blood tests to check the bilirubin, bile acids and liver function. (pregnancy-baby-care.com)
  • It is believed that one to two pregnancies out of every thousand suffer from cholestasis. (pregnancy-baby-care.com)