Cholestasis: Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).Cholestasis, Intrahepatic: Impairment of bile flow due to injury to the HEPATOCYTES; BILE CANALICULI; or the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC).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.Cholestasis, Extrahepatic: Impairment of bile flow in the large BILE DUCTS by mechanical obstruction or stricture due to benign or malignant processes.Liver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.Liver Diseases: Pathological processes of the LIVER.Portasystemic Shunt, Transjugular Intrahepatic: 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)Liver Neoplasms: Tumors or cancer of the LIVER.Liver Transplantation: The transference of a part of or an entire liver from one human or animal to another.Cholangiocarcinoma: A malignant tumor arising from the epithelium of the BILE DUCTS.Liver Cirrhosis: 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.Cholagogues and Choleretics: Gastrointestinal agents that stimulate the flow of bile into the duodenum (cholagogues) or stimulate the production of bile by the liver (choleretic).Bile Ducts: 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.Bile Duct Neoplasms: Tumors or cancer of the BILE DUCTS.1-Naphthylisothiocyanate: 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.Ursodeoxycholic Acid: 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.Liver Function Tests: Blood tests that are used to evaluate how well a patient's liver is working and also to help diagnose liver conditions.Bile Acids and Salts: 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.Bile: 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.Fatty Liver: 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.Biliary Atresia: 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.Bilirubin: A bile pigment that is a degradation product of HEME.Liver Regeneration: Repair or renewal of hepatic tissue.Bile Canaliculi: Minute intercellular channels that occur between liver cells and carry bile towards interlobar bile ducts. Also called bile capillaries.Hepatocytes: The main structural component of the LIVER. They are specialized EPITHELIAL CELLS that are organized into interconnected plates called lobules.Biliary Tract: The BILE DUCTS and the GALLBLADDER.Drug-Induced Liver Injury: 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.Microsomes, Liver: Closed vesicles of fragmented endoplasmic reticulum created when liver cells or tissue are disrupted by homogenization. They may be smooth or rough.Liver Circulation: The circulation of BLOOD through the LIVER.Portal Vein: A short thick vein formed by union of the superior mesenteric vein and the splenic vein.Pruritus: An intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.Bile Duct Diseases: Diseases in any part of the ductal system of the BILIARY TRACT from the smallest BILE CANALICULI to the largest COMMON BILE DUCT.Liver Cirrhosis, Biliary: 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.P-Glycoproteins: 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.Hepatectomy: Excision of all or part of the liver. (Dorland, 28th ed)Hypertension, Portal: 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.Jaundice, Neonatal: 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.Hepatitis: INFLAMMATION of the LIVER.Hepatic Artery: A branch of the celiac artery that distributes to the stomach, pancreas, duodenum, liver, gallbladder, and greater omentum.Jaundice: 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.Ligation: Application of a ligature to tie a vessel or strangulate a part.Mitochondria, Liver: Mitochondria in hepatocytes. As in all mitochondria, there are an outer membrane and an inner membrane, together creating two separate mitochondrial compartments: the internal matrix space and a much narrower intermembrane space. In the liver mitochondrion, an estimated 67% of the total mitochondrial proteins is located in the matrix. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p343-4)Cholangitis: Inflammation of the biliary ductal system (BILE DUCTS); intrahepatic, extrahepatic, or both.Carcinoma, Hepatocellular: 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.Jaundice, Obstructive: 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.Hepatic Veins: Veins which drain the liver.Bile Ducts, Extrahepatic: Passages external to the liver for the conveyance of bile. These include the COMMON BILE DUCT and the common hepatic duct (HEPATIC DUCT, COMMON).Lithocholic Acid: 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.Alanine Transaminase: 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.Pregnancy Complications: 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.Cholangiography: 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.Citrullinemia: 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)Common Bile Duct: The largest bile duct. It is formed by the junction of the CYSTIC DUCT and the COMMON HEPATIC DUCT.Liver Failure, Acute: A form of rapid-onset LIVER FAILURE, also known as fulminant hepatic failure, caused by severe liver injury or massive loss of HEPATOCYTES. It is characterized by sudden development of liver dysfunction and JAUNDICE. Acute liver failure may progress to exhibit cerebral dysfunction even HEPATIC COMA depending on the etiology that includes hepatic ISCHEMIA, drug toxicity, malignant infiltration, and viral hepatitis such as post-transfusion HEPATITIS B and HEPATITIS C.Cholic Acid: A major primary bile acid produced in the liver and usually conjugated with glycine or taurine. It facilitates fat absorption and cholesterol excretion.Biliary Tract Diseases: Diseases in any part of the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.Taurochenodeoxycholic Acid: 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.ATP-Binding Cassette Transporters: 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.Liver Cirrhosis, Experimental: Experimentally induced chronic injuries to the parenchymal cells in the liver to achieve a model for LIVER CIRRHOSIS.Liver Neoplasms, Experimental: Experimentally induced tumors of the LIVER.Liver Abscess: Solitary or multiple collections of PUS within the liver as a result of infection by bacteria, protozoa, or other agents.Alagille Syndrome: 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).Liver Cirrhosis, Alcoholic: FIBROSIS of the hepatic parenchyma due to chronic excess ALCOHOL DRINKING.Ethinyl Estradiol: 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.Portasystemic Shunt, Surgical: 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.Portal System: 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.gamma-Glutamyltransferase: 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.Liver Extracts: Extracts of liver tissue containing uncharacterized specific factors with specific activities; a soluble thermostable fraction of mammalian liver is used in the treatment of pernicious anemia.Hepatomegaly: Enlargement of the liver.Hyperbilirubinemia: 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.Norethandrolone: A synthetic hormone with anabolic and androgenic properties and moderate progestational activity.Organic Anion Transporters: 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.Imino AcidsColeus: A plant genus of the family Lamiaceae. The species of Coleus should be distinguished from PLECTRANTHUS BARBATUS - which is also known as Coleus forskohlii.Disease Models, Animal: 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.Cholangitis, Sclerosing: 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.Budd-Chiari Syndrome: 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.Retrospective Studies: 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.Rats, Wistar: A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.Taurocholic Acid: 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.Cholangiopancreatography, Endoscopic Retrograde: 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.Liver Diseases, Alcoholic: Liver diseases associated with ALCOHOLISM. It usually refers to the coexistence of two or more subentities, i.e., ALCOHOLIC FATTY LIVER; ALCOHOLIC HEPATITIS; and ALCOHOLIC CIRRHOSIS.Liver Failure: 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)Treatment Outcome: 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.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Lithiasis: 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.Infant, Newborn: An infant during the first month after birth.Esophageal and Gastric Varices: 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).Kupffer Cells: 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.Cholelithiasis: Presence or formation of GALLSTONES in the BILIARY TRACT, usually in the gallbladder (CHOLECYSTOLITHIASIS) or the common bile duct (CHOLEDOCHOLITHIASIS).Pregnanetriol: 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.RNA, Messenger: RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.Biopsy: Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.Rats, Inbred Strains: Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations or by parent x offspring matings carried out with certain restrictions. This also includes animals with a long history of closed colony breeding.Rats, Sprague-Dawley: 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.Portography: Examination of the portal circulation by the use of X-ray films after injection of radiopaque material.Organic Anion Transporters, Sodium-Dependent: A subclass of ORGANIC ANION TRANSPORTERS whose transport of organic anions is driven either directly or indirectly by a gradient of sodium ions.Fatal Outcome: 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.Biliary Tract Neoplasms: Tumors or cancer in the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.Cysts: Any fluid-filled closed cavity or sac that is lined by an EPITHELIUM. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Sulfobromophthalein: A phenolphthalein that is used as a diagnostic aid in hepatic function determination.Portal Pressure: The venous pressure measured in the PORTAL VEIN.Ascites: Accumulation or retention of free fluid within the peritoneal cavity.Hepatitis, Animal: INFLAMMATION of the LIVER in non-human animals.Klatskin's Tumor: 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)Receptors, Cytoplasmic and Nuclear: 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.Biliary Fistula: Abnormal passage in any organ of the biliary tract or between biliary organs and other organs.Hepatic Encephalopathy: 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)Lipoprotein-X: 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.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.Adenoma, Bile Duct: A benign tumor of the intrahepatic bile ducts.Hepatitis, Alcoholic: 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.Liver Glycogen: Glycogen stored in the liver. (Dorland, 28th ed)Pregnancy: The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.Cholic Acids: 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.Arthrogryposis: Persistent flexure or contracture of a joint.End Stage Liver Disease: Final stage of a liver disease when the liver failure is irreversible and LIVER TRANSPLANTATION is needed.Parenteral Nutrition: 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).Alkaline Phosphatase: An enzyme that catalyzes the conversion of an orthophosphoric monoester and water to an alcohol and orthophosphate. EC 3.1.3.1.Hepatic Duct, Common: 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.Mice, Knockout: Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.Dothiepin: A tricyclic antidepressant with some tranquilizing action.Glycochenodeoxycholic Acid: 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.Technetium Tc 99m Disofenin: A radiopharmaceutical used extensively in cholescintigraphy for the evaluation of hepatobiliary diseases. (From Int Jrnl Rad Appl Inst 1992;43(9):1061-4)Parenteral Nutrition, Total: 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.Gallstones: 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.Transaminases: 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.Taurolithocholic Acid: 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.Chenodeoxycholic Acid: 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.Gallbladder: 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.Caroli Disease: 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.Mice, Inbred C57BLHepatitis, Viral, Human: 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).Carbon Tetrachloride: A solvent for oils, fats, lacquers, varnishes, rubber waxes, and resins, and a starting material in the manufacturing of organic compounds. Poisoning by inhalation, ingestion or skin absorption is possible and may be fatal. (Merck Index, 11th ed)Liver, Artificial: Devices for simulating the activities of the liver. They often consist of a hybrid between both biological and artificial materials.Perfusion: Treatment process involving the injection of fluid into an organ or tissue.Gastrointestinal Hemorrhage: Bleeding in any segment of the GASTROINTESTINAL TRACT from ESOPHAGUS to RECTUM.Postoperative Complications: 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.Aspartate Aminotransferases: 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.Noxae: Agents capable of exerting a harmful effect on the body.Tomography, X-Ray Computed: Tomography using x-ray transmission and a computer algorithm to reconstruct the image.Necrosis: The pathological process occurring in cells that are dying from irreparable injuries. It is caused by the progressive, uncontrolled action of degradative ENZYMES, leading to MITOCHONDRIAL SWELLING, nuclear flocculation, and cell lysis. It is distinct it from APOPTOSIS, which is a normal, regulated cellular process.alpha-Fetoproteins: The first alpha-globulins to appear in mammalian sera during FETAL DEVELOPMENT and the dominant serum proteins in early embryonic life.Biliary Tract Surgical Procedures: Any surgical procedure performed on the biliary tract.Portacaval Shunt, Surgical: Surgical portasystemic shunt between the portal vein and inferior vena cava.Living Donors: Non-cadaveric providers of organs for transplant to related or non-related recipients.Infant, Newborn, Diseases: 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.Hepatitis C, Chronic: INFLAMMATION of the LIVER in humans that is caused by HEPATITIS C VIRUS lasting six months or more. Chronic hepatitis C can lead to LIVER CIRRHOSIS.Metabolism, Inborn Errors: Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.Epichlorohydrin: A chlorinated epoxy compound used as an industrial solvent. It is a strong skin irritant and carcinogen.Multidrug Resistance-Associated Proteins: 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.Fat Emulsions, Intravenous: Emulsions of fats or lipids used primarily in parenteral feeding.Pregnanediol: 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.Hepatitis, Viral, Animal: INFLAMMATION of the LIVER in animals due to viral infection.Recurrence: The return of a sign, symptom, or disease after a remission.Mitragyna: A plant genus of the family RUBIACEAE. Members contain antimalarial (ANTIMALARIALS) and analgesic (ANALGESICS) indole alkaloids.Steroid 12-alpha-Hydroxylase: 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.Kinetics: The rate dynamics in chemical or physical systems.Prognosis: A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.Lipid Metabolism: Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS.Glycocholic Acid: The glycine conjugate of CHOLIC ACID. It acts as a detergent to solubilize fats for absorption and is itself absorbed.Syndrome: A characteristic symptom complex.Membrane Transport Proteins: 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.Kidney: Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.Kveim Test: 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.Gallbladder Diseases: Diseases of the GALLBLADDER. They generally involve the impairment of BILE flow, GALLSTONES in the BILIARY TRACT, infections, neoplasms, or other diseases.Organ Size: The measurement of an organ in volume, mass, or heaviness.Hepatitis B: INFLAMMATION of the LIVER in humans caused by a member of the ORTHOHEPADNAVIRUS genus, HEPATITIS B VIRUS. It is primarily transmitted by parenteral exposure, such as transfusion of contaminated blood or blood products, but can also be transmitted via sexual or intimate personal contact.Cystadenoma: 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)Tissue Distribution: Accumulation of a drug or chemical substance in various organs (including those not relevant to its pharmacologic or therapeutic action). This distribution depends on the blood flow or perfusion rate of the organ, the ability of the drug to penetrate organ membranes, tissue specificity, protein binding. The distribution is usually expressed as tissue to plasma ratios.Clonorchiasis: 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)Gene Expression Regulation: Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.Cells, Cultured: Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.Mutation: Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.Cholesterol: The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils.Antipruritics: Agents, usually topical, that relieve itching (pruritus).Fetus: The unborn young of a viviparous mammal, in the postembryonic period, after the major structures have been outlined. In humans, the unborn young from the end of the eighth week after CONCEPTION until BIRTH, as distinguished from the earlier EMBRYO, MAMMALIAN.Follow-Up Studies: Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.Reverse Transcriptase Polymerase Chain Reaction: A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.Cytochrome P-450 Enzyme System: A superfamily of hundreds of closely related HEMEPROTEINS found throughout the phylogenetic spectrum, from animals, plants, fungi, to bacteria. They include numerous complex monooxygenases (MIXED FUNCTION OXYGENASES). In animals, these P-450 enzymes serve two major functions: (1) biosynthesis of steroids, fatty acids, and bile acids; (2) metabolism of endogenous and a wide variety of exogenous substrates, such as toxins and drugs (BIOTRANSFORMATION). They are classified, according to their sequence similarities rather than functions, into CYP gene families (>40% homology) and subfamilies (>59% homology). For example, enzymes from the CYP1, CYP2, and CYP3 gene families are responsible for most drug metabolism.Phospholipids: Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides see GLYCEROPHOSPHOLIPIDS) or sphingosine (SPHINGOLIPIDS). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system.Biological Markers: Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Hep G2 Cells: A human liver tumor cell line used to study a variety of liver-specific metabolic functions.Hepacivirus: A genus of FLAVIVIRIDAE causing parenterally-transmitted HEPATITIS C which is associated with transfusions and drug abuse. Hepatitis C virus is the type species.Acute Disease: Disease having a short and relatively severe course.Infusions, Intra-Arterial: Regional infusion of drugs via an arterial catheter. Often a pump is used to impel the drug through the catheter. Used in therapy of cancer, upper gastrointestinal hemorrhage, infection, and peripheral vascular disease.Portoenterostomy, Hepatic: Operation for biliary atresia by anastomosis of the bile ducts into the jejunum or duodenum.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Survival Rate: The proportion of survivors in a group, e.g., of patients, studied and followed over a period, or the proportion of persons in a specified group alive at the beginning of a time interval who survive to the end of the interval. It is often studied using life table methods.Islets of Langerhans Transplantation: The transference of pancreatic islets within an individual, between individuals of the same species, or between individuals of different species.Taurine: A conditionally essential nutrient, important during mammalian development. It is present in milk but is isolated mostly from ox bile and strongly conjugates bile acids.17-alpha-Hydroxypregnenolone: 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.Liver Diseases, Parasitic: Liver diseases caused by infections with PARASITES, such as tapeworms (CESTODA) and flukes (TREMATODA).Blotting, Western: Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.Disease Progression: The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.Choledochal Cyst: 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.Cholesterol 7-alpha-Hydroxylase: A membrane-bound cytochrome P450 enzyme that catalyzes the 7-alpha-hydroxylation of CHOLESTEROL in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP7, converts cholesterol to 7-alpha-hydroxycholesterol which is the first and rate-limiting step in the synthesis of BILE ACIDS.Gene Expression: The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.Cefotiam: One of the CEPHALOSPORINS that has a broad spectrum of activity against both gram-positive and gram-negative microorganisms.Hepatitis, Chronic: INFLAMMATION of the LIVER with ongoing hepatocellular injury for 6 months or more, characterized by NECROSIS of HEPATOCYTES and inflammatory cell (LEUKOCYTES) infiltration. Chronic hepatitis can be caused by viruses, medications, autoimmune diseases, and other unknown factors.
Reyes H, Simon FR (August 1993). "Intrahepatic cholestasis of pregnancy: an estrogen-related disease". Semin Liver Dis. 13 (3 ... It has been used in the symptomatic treatment of itching due to intrahepatic cholestasis of pregnancy. Gonzalez MC, Iglesias J ... Reyes H (December 1992). "The spectrum of liver and gastrointestinal disease seen in cholestasis of pregnancy". Gastroenterol ... September 1992). "Epomediol ameliorates pruritus in patients with intrahepatic cholestasis of pregnancy". J Hepatol. 16 (1-2): ...
... function and role in progressive familial intrahepatic cholestasis". Seminars in Liver Disease. 21 (4): 545-50. doi:10.1055/s- ... "A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis". Nature Genetics ... ABCB11 is a gene associated with progressive familial intrahepatic cholestasis type 2 (PFIC2). PFIC2 caused by mutations in the ... "Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11". Gastroenterology. 127 (2): 379-84. doi: ...
"Genetic determinants of drug-induced cholestasis and intrahepatic cholestasis of pregnancy". Semin. Liver Dis. 30 (2): 147-59. ... 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. PMID ... Pusl T, Beuers U (2007). "Intrahepatic cholestasis of pregnancy". Orphanet J Rare Dis. 2: 26. doi:10.1186/1750-1172-2-26. PMC ...
Cholestasis means "the slowing or stopping of bile flow" which can be caused by any number of diseases of the liver (which ... Intrahepatic cholestasis of pregnancy List of cutaneous conditions Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. ( ... the liver conjugates the bile to make it water-soluble and because the bile has already been processed by the liver, when it ... This suggests that products made by the liver must have some role in pruritus although it is not known exactly which product is ...
Septal fibrosis and pseudolobules, inflammatory infiltrates, signs of cholestasis, and reduced numbers of intrahepatic bile ... Successful liver and kidney transplantation have been done in some cases. Doherty, D.; Parisi, M. A.; Finn, L. S.; Gunay-Aygun ... "Successful combined liver and kidney transplant for COACH syndrome and 5-yr follow-up". Clinical Transplantation. 19 (6): 717- ... a Joubert Syndrome related disorder with liver involvement". Human Mutation. 30 (2): E432-442. doi:10.1002/humu.20924. ISSN ...
Intrahepatic cholestasis of pregnancy[edit]. UDCA has been used for intrahepatic cholestasis of pregnancy. UDCA lessens itching ... UDCA use is associated with improved serum liver tests that do not always correlate with improved liver disease status.[11] WHO ... UDCA has also been used in non-alcoholic fatty liver disease, in liver bile duct-paucity syndromes such as biliary atresia, ... "Ursodeoxycholic acid versus placebo in women with intrahepatic cholestasis of pregnancy (PITCHES): a randomised controlled ...
Mutations in this gene may result in progressive familial intrahepatic cholestasis type 1 and in benign recurrent intrahepatic ... Harris MJ, Arias IM (2003). "FIC1, a P-type ATPase linked to cholestatic liver disease, has homologues (ATP8B2 and ATP8B3) ... 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. ...
Obstruction of the bile ducts by gallstones (choledocholithiasis), primary sclerosing cholangitis, liver damage (intrahepatic ... cholestasis), hypolipidemic drugs, or changes following gallbladder removal (cholecystectomy). Conditions producing intestinal ...
Type II citrullinemia may also develop in people who had a liver disorder called neonatal cholestasis during infancy. This ... Researchers have found many infants with neonatal intrahepatic cholestasis have the same mutations in the SLC25A13 gene as ... In some cases, liver transplant has been successful. Hyperammonemia Citrullinemia type I Freedberg, et al. (2003). ... The urea cycle is a sequence of chemical reactions taking place in the liver. These reactions process excess nitrogen, ...
Bilirubin levels greater than 10x normal could indicate neoplastic or intrahepatic cholestasis. Levels lower than this tend to ... The typical liver panel will include blood levels of enzymes found primarily from the liver, such as the aminotransferases (ALT ... Also, a group of parasites known as "liver flukes" can live in the common bile duct, causing obstructive jaundice. Other causes ... which will only be elevated in liver-specific conditions. The second step is distinguishing from biliary (cholestatic) or liver ...
Davit-Spraul, A; Gonzales, E; Baussan, C; Jacquemin, E (Jan 8, 2009). "Progressive familial intrahepatic cholestasis". Orphanet ... Liver Cancer at Johns Hopkins University Liver cancer at Mayo Clinic Liver cancer information from Cancer Research UK. ... known as liver metastasis, is more common than that which starts in the liver. Symptoms of liver cancer may include a lump or ... but It may also form a liver mass in rare occasions. Many cancers found in the liver are not true liver cancers, but are ...
... spectrum disorder Thyroid disease in pregnancy Pruritic urticarial papules and plaques of pregnancy Intrahepatic cholestasis of ... trophoblastic disease Gestational diabetes Hyperemesis gravidarum Pelvic girdle pain HELLP syndrome Acute fatty liver of ... syndrome Gestational trophoblastic disease Antiphospholipid antibody syndrome Hyperemesis gravidarum Acute fatty liver of ...
... cholestasis, intrahepatic MeSH C06.130.120.135.250.125 --- alagille syndrome MeSH C06.130.120.135.250.250 --- liver cirrhosis, ... liver abscess, amebic MeSH C06.552.597.758 --- liver abscess, pyogenic MeSH C06.552.630.380 --- liver cirrhosis, alcoholic MeSH ... liver cirrhosis, biliary MeSH C06.552.241.390 --- fatty liver, alcoholic MeSH C06.552.241.649 --- reye syndrome MeSH C06.552. ... fatty liver, alcoholic MeSH C06.552.645.490 --- hepatitis, alcoholic MeSH C06.552.645.590 --- liver cirrhosis, alcoholic MeSH ...
... progressive familial intrahepatic cholestasis, Langerhans cell histiocytosis and hepatic hemangioma a benign tumour the most ... Living donor liver transplantation is a technique in which a portion of a living person's liver is removed (hepatectomy) and ... Liver is often made into spreads. Well-known examples include liver pâté, foie gras, chopped liver, and leverpastej. Liver ... Within the liver, these ducts are termed intrahepatic bile ducts, and once they exit the liver they are considered extrahepatic ...
... hepatocanalicular transport defects such as Dubin-Johnson Syndrome and progressive familial intrahepatic cholestasis (PFIC), ... Hep G2 is a human liver cancer cell line. Hep G2 is a perpetual cell line which was derived from the liver tissue of a 15-year- ... Mersch-Sundermann, V.; Knasmüller, S.; Wu, X. J.; Darroudi, F.; Kassie, F. (2004). "Use of a human-derived liver cell line for ... This can be important for the study of human liver diseases that are caused by an incorrect subcellular distribution of cell ...
Progressive familial intrahepatic cholestasis, bile duct paucity, Alagille syndrome, alpha 1-antitrypsin deficiency, and other ... In the newborn liver, activity of glucuronyl transferase is only at 0.1-1% of adult levels, so conjugation of bilirubin is ... Family history of jaundice and anemia, family history of neonatal or early infant death due to liver disease, maternal illness ... liver disease, infection, hypothyroidism, or metabolic disorders (pathologic). A bilirubin level more than 34 μmol/l (2 mg/dL) ...
Intrahepatic cholestasis of pregnancy (acute or in history), vaginal bleeding of unknown origin, and severe diseases of the ... The distribution and elimination half-lives of medrogestone are 4 hours and 35-36 hours, respectively. The drug is largely ... liver such as tumors are absolute contraindications for medrogestone, as are thrombotic events such as thrombophlebitis or ...
Nuchal cord Placental abruption Premature closure of the fetal ductus arteriosus Uterine rupture Intrahepatic cholestasis of ... pregnancy, a liver disorder during pregnancy Instead of referring to "fetal distress", current recommendations hold to look for ...
... progressive familial intrahepatic cholestasis), Caroli disease, choledochal cyst, cholestasis, congenital cytomegalovirus ... Further testing may include radioactive scans of the liver and a liver biopsy. The differential diagnoses are extensive and ... Liver transplantation is an option for those children whose liver function and symptoms fail to respond to a Kasai operation.[ ... a condition in which bile is unable to leave the liver and builds up inside of it. When the liver is unable to excrete ...
Renal dysfunction or hepatorenal syndrome Progressive intrahepatic cholestasis Fulminant Wilson disease Acute liver dysfunction ... Liver support systems are therapeutic devices to assist in performing the functions of the liver in persons with liver damage. ... 2005). "Outcome from molecular adsorbent recycling system (MARS) liver dialysis following drug-induced liver failure". Liver ... "Artificial liver support system in acute liver failure patients waiting liver transplantation". Hepato-gastroenterology. 56 (90 ...
ALP levels in plasma rise with large bile duct obstruction, intrahepatic cholestasis, or infiltrative diseases of the liver. ... Liver Function Tests at Lab Tests Online Overview at Mayo Clinic Abnormal Liver Function Tests Overview of liver enzymes ... are useful biomarkers of liver injury in a patient with some degree of intact liver function. Most liver diseases cause only ... The liver is responsible for clearing the blood of unconjugated bilirubin, and about 30% of it is taken up by a normal liver on ...
... intrahepatic cholestasis of pregnancy, and autoimmune hepatitis. If a liver biopsy is needed for diagnosis of the condition, ... Fatty liver Ko H, Yoshida EM (2006). "Acute fatty liver of pregnancy". Can. J. Gastroenterol. 20 (1): 25-30. PMC 2538964 . PMID ... Many laboratory abnormalities are seen in acute fatty liver of pregnancy. Liver enzymes are elevated, with the AST and ALT ... The diagnosis of acute fatty liver of pregnancy is suggested by jaundice with a lesser elevation of liver enzymes, elevated ...
... steroid oxidoreductase is mutated in progressive intrahepatic cholestasis". J. Clin. Invest. 106 (9): 1175-84. doi:10.1172/ ... a form of progressive liver disease. 3-beta-HSD Wikvall K (April 1981). "Purification and properties of a 3 β-hydroxy-delta 5- ... Mutations in the HSD3B7 gene are associated with a congenital bile acid synthesis defect which leads to neonatal cholestasis, ... C27-steroid oxidoreductase from rabbit liver microsomes". J. Biol. Chem. 256 (7): 3376-80. PMID 6937465. Schwarz M, Wright AC, ...
Complications of PBC can be related to chronic cholestasis or cirrhosis of the liver. Chronic cholestasis leads to osteopenic ... intrahepatic ductules) being affected early in the disease. This progresses to the development of fibrosis, cholestasis and, in ... causing bile and other toxins to build up in the liver, a condition called cholestasis. Further slow damage to the liver tissue ... It helps reduce the cholestasis and improves liver function tests. It has a minimal effect on symptoms and whether it improves ...
Jaundice Liver function tests Lipoprotein-X - an abnormal low density lipoprotein found in cholestasis Intrahepatic cholestasis ... In a later stage of cholestasis AST, ALT and bilirubin may be elevated due to liver damage as a secondary effect of cholestasis ... Jaundice is an uncommon occurrence in intrahepatic (metabolic) cholestasis, but is common in obstructive cholestasis. Pale ... and statins can cause cholestasis and may result in damage to the liver. Bile is secreted by the liver to aid in the digestion ...
Acute fatty liver of pregnancy. *Hepatitis E. *Hyperemesis gravidarum. *Intrahepatic cholestasis of pregnancy ...
The results of liver transplantation in Slovak children * Progressive familial intrahepatic cholestasis type 2 - paediatric ... Progressive familial intrahepatic cholestasis type 2 is an autosomal recessive cholestatic liver disease caused by a deficiency ... A gene encod-ing a liver-specific ABC transporter is mutated in progres-sive familial intrahepatic cholestasis. Nat Genet 1998 ... progressive familial intrahepatic cholestasis type 2 - BSEP - ursodeoxycholic acid - pruritus - liver transplantation. The ...
... is a rare genetic disease. Learn more about causes, symptoms and treatment ... Liver Transplant. A liver transplant may be used if PEBD is not effective or if the patient has liver cirrhosis. It is the only ... Progressive Familial Intrahepatic Cholestasis (PFIC) Progressive familial intrahepatic cholestasis (PFIC) is a rare inherited ... In PFIC children are not able to drain bile from the liver even though the large bile ducts are open (cholestasis). This gets ...
... is a rare congenital cholestatic liver disease that progresses to end stage liver disease. It is associated with fat soluble ... One year old twin males born to a mother with intrahepatic cholestasis during pregnancy presented with jaundice, pruritus and ... Maternal intrahepatic cholestasis during pregnancy can be an early warning sign. ... Higher doses of Vitamin D therapy are needed for treatment of rickets secondary to cholestasis. Extremely low HDL-C levels are ...
As per NIH, (PFIC) is a disorder that causes progressive liver disease, which typically leads to liver failure in which liver ... 6. Progressive Familial Intrahepatic Cholestasis (PFIC) Patient Journey. 7. Progressive Familial Intrahepatic Cholestasis (PFIC ... Progressive Familial Intrahepatic Cholestasis (PFIC) Market Drivers. 19. Progressive Familial Intrahepatic Cholestasis (PFIC) ... Progressive Familial Intrahepatic Cholestasis (PFIC) Pipeline Insights, 2020. Progressive Familial Intrahepatic Cholestasis ( ...
Progressive familial intrahepatic cholestasis (PFIC) comprises a group of rare cholestatic liver disorders of childhood that ... Long-term follow-up in children with progressive familial intrahepatic cholestasis type 2 after partial external biliary ... Cholestasis was observed in each patient, but only in two of them, centrally located bile plugs were found. The majority of ... Based on Batts and Ludwig fibrosis scoring system, the liver fibrosis had resolved in two out of three patients. The formation ...
... progressive familial intrahepatic cholestasis type 2 (PFIC2), progressive familial intrahepatic cholestasis type 3 (PFIC3) and ... This rare genetic disorder is caused by a defective gene in liver cells encoding the ATP7B protein, which reduces the livers ... Vivet has developed a next-generation AAV gene therapy technology designed to increase gene expression levels in the liver, ... ability to regulate copper levels in the liver and other tissues causing severe hepatic and neurologic symptoms, leading to ...
It can lead to liver disease. Learn the causes. ... Intrahepatic cholestasis is a condition caused by slow or ... Pediatric Intrahepatic Cholestasis Liver Diseases. Intrahepatic cholestasis is a problem that affects the release of bile from ... What are the signs and symptoms of Pediatric Intrahepatic Cholestasis Liver Diseases?. Babies with intrahepatic cholestasis ... What are the causes of Pediatric Intrahepatic Cholestasis Liver Diseases?. Intrahepatic cholestasis is caused by genetic ...
if this happens, your doctor may suggest a liver transplant. ... by age 10 or older and may be more likely to get liver cancer. ... How can liver transplant help with treating low gamma-GT intrahepatic cholestasis?. ANSWER ... Childhood Liver Disease Research Network: "What is Progressive Familial Intrahepatic Cholestasis (PFIC)?" ... Childhood Liver Disease Research Network: "What is Progressive Familial Intrahepatic Cholestasis (PFIC)?" ...
... an itchy skin condition when bile gets backed up in the liver, are significantly more likely to suffer other liver diseases ... A retrospective case-control study of 21,008 women in Finland has found that those with intrahepatic cholestasis of pregnancy ( ... Intrahepatic cholestasis of pregnancy as an indicator of liver and biliary diseases. *Download PDF Copy ... Intrahepatic Cholestasis of Pregnancy, Liver, Obstetrics, Pancreatitis, Placenta, Pregnancy, Skin, Steroid ...
Intrahepatic Cholestasis of Pregnancy (ICP), (formerly known as Obstetric Cholestasis or OC), is a liver disorder that occurs ... The results of liver transplantation for people with Hepatitis B virus with acute or chronic liver failure and/or primary liver ... Liver function tests (LFTs). Your doctor can diagnose ICP from blood tests called liver function tests (LFTs) and a serum bile ... Liver function tests are performed to gain an idea of how your liver is functioning. A number of separate properties of your ...
A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis.. Strautnieks SS1 ... The progressive familial intrahepatic cholestases (PFIC) are a group of inherited disorders with severe cholestatic liver ... MalaCards for cholestasis, benign recurrent intrahepatic, 2 - The Weizmann Institute of Science GeneCards and MalaCards ... The PFIC2 gene has now been identified by mutations in a positional candidate, BSEP, which encodes a liver-specific ATP-binding ...
Open-label study of ademetionine for the treatment of intrahepatic cholestasis associated with alcoholic liver disease. ... Open-label study of ademetionine for the treatment of intrahepatic cholestasis associated with alcoholic liver disease. Minerva ... on clinical symptoms and biochemical markers of intrahepatic cholestasis (IHC) was investigated in subjects with alcoholic ... liver disease (ALD) and compensated liver function.. METHODS: Prospective, multicenter, open-label study consisting of a ...
11 patients with progressive familial intrahepatic cholestasis experience fatigue, insomnia, depressed mood, pain, and anxious ... Find the most comprehensive real-world symptom and treatment data on progressive familial intrahepatic cholestasis at ... Progressive familial intrahepatic cholestasis nonprofits on PatientsLikeMe. * American Liver Foundation, Great Lakes Division 9 ... What is progressive familial intrahepatic cholestasis?. Progressive Familial Intrahepatic Cholestasis (PFIC) is a rare genetic ...
Kreek MJ (February 1987). "Female sex steroids and cholestasis". Semin. Liver Dis. 7 (1): 8-23. doi:10.1055/s-2008-1040559. ... Intrahepatic cholestasis of pregnancy (ICP), also known as obstetric cholestasis, cholestasis of pregnancy, jaundice of ... The causes of intrahepatic cholestasis of pregnancy are still not fully understood. Hormones and genetic factors are likely to ... July 1989). "Intrahepatic cholestasis of pregnancy in twin pregnancies". J. Hepatol. 9 (1): 84-90. doi:10.1016/0168-8278(89) ...
... is characterized by episodes of liver dysfunction called cholestasis. Explore symptoms, inheritance, genetics of this condition ... Benign recurrent intrahepatic cholestasis (BRIC) is characterized by episodes of liver. dysfunction called cholestasis. During ... Differential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type ... intrahepatic), the condition is described as intrahepatic cholestasis. Episodes of cholestasis can last from weeks to months, ...
Liver Int. 2005 Jun;25(3):548-54. Research Support, Non-U.S. Govt ... Liver Int. 2005 Jun;25(3):548-54.. Ursodeoxycholic acid in the treatment of intrahepatic cholestasis of pregnancy. A 12-year ... Intrahepatic cholestasis of pregnancy: from bedside to bench to bedside. [Liver Int. 2005] ... To assess the efficacy of ursodeoxycholic acid (UDCA) in patients with intrahepatic cholestasis of pregnancy (ICP) and in the ...
Ursodeoxycholic acid in the treatment of intrahepatic cholestasis of pregnancy. A 12-year experience. Liver Int2005;25:548-54. ... Intrahepatic cholestasis of pregnancy (obstetric cholestasis) is characterised by pruritus, otherwise unexplained deranged ... Intrahepatic cholestasis of pregnancy affects about 0.7% of pregnancies in the United Kingdom, varying by ethnic group,5 and ... Intrahepatic cholestasis of pregnancy: review of the literature and evaluation of current evidence. J Womens Health (Larchmt) ...
Intrahepatic cholestasis of pregnancy (ICP) is the most prevalent pregnancy-specific liver disease. It occurs mainly in the ... Genetics Home Reference related topics: intrahepatic cholestasis of pregnancy progressive familial intrahepatic cholestasis ... Cholestasis. Cholestasis, Intrahepatic. Pregnancy Complications. Bile Duct Diseases. Biliary Tract Diseases. Digestive System ... with intrahepatic cholestasis of pregnancy and to find out if Th-17 cells have a role in progress of intrahepatic cholestasis ...
Cholestasis. Cholestasis, Intrahepatic. Bile Duct Diseases. Biliary Tract Diseases. Digestive System Diseases. Liver Diseases. ... Genetics Home Reference related topics: Progressive familial intrahepatic cholestasis Genetic and Rare Diseases Information ... Efficacy and Safety of Maralixibat (SHP625) in the Treatment of Participants With Progressive Familial Intrahepatic Cholestasis ... in the Treatment of Subjects With Progressive Familial Intrahepatic Cholestasis (PFIC). Estimated Study Start Date :. October ...
Intrahepatic cholestasis of pregnancy (ICP) is a unique disease of the liver resulting in abnormal bile acid levels and liver ... Intrahepatic cholestasis of pregnancy (ICP) is a unique disease of the liver resulting in abnormal bile acid levels and liver ... Genetics Home Reference related topics: Intrahepatic cholestasis of pregnancy Progressive familial intrahepatic cholestasis ... Cholestasis. Cholestasis, Intrahepatic. Pregnancy Complications. Bile Duct Diseases. Biliary Tract Diseases. Digestive System ...
Certain liver diseases are uniquely associated with pregnancy, whereas others are unrelated. ... Liver disease that occurs during pregnancy can present a challenge for health care providers. ... Intrahepatic Cholestasis of Pregnancy. Intrahepatic cholestasis of pregnancy (ICP) occurs in approximately 1-2 per 1,000 ... acute fatty liver of pregnancy (AFLP), intrahepatic cholestasis of pregnancy (ICP), and hemolysis and elevated liver enzymes ...
Fowler on intrahepatic cholestasis of pregnancy abdominal pain: Cholestasis of pregnancy does not generally cause pain. The ... Doctors help you with trusted information about Abdominal Pain in Cholestasis Of Pregnancy: Dr. ... What is the result of liver function tests in intrahepatic cholestasis of pregnancy? ... Cholestasis (Definition) A condition where the flow of bile, a digestive fluid produced by the liver, slows or stops. As a ...
Intrahepatic Cholestasis of Pregnancy. My search led me to information about intrahepatic cholestasis of pregnancy. Symptoms of ... Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-related liver disorder in which there are abnormalities in the flow ... "Complications of Obstetric Cholestasis." Br Med J. 1 (6014): 870-72.. *Rigby, F, et al. 2014. "Intrahepatic Cholestasis of ... www.smfm.org/publications/96-understanding-intrahepatic-cholestasis-of-pregnancy.. Share this:. *Click to share on Facebook ( ...
It is sometimes referred to as extrahepatic cholestasis which occurs outside the liver, intrahepatic cholestasis which occurs ... Intrahepatic cholestasis of pregnancy. Some women experience a very severe itching (pruritus) in late pregnancy (often begins ... Acute fatty liver of pregnancy (AFLP). Acute fatty liver of pregnancy (AFLP) is a serious condition that occurs in the third ... It was developed by the Canadian Liver Foundation as a means to link Canadians like you who have a family member who has liver ...
Genetic determinants of drug-induced cholestasis and intrahepatic cholestasis of pregnancy. Semin Liver Dis. 2010;30:147-59. ... Intrahepatic cholestasis of pregnancy (ICP) is a special complication of pregnancy, which occurs in the middle and late stage ... 12 Yu L, Ding Y, Huang T, Huang X. Effect of bile acid on fetal lung in rat model of intrahepatic cholestasis of pregnancy. Int ... Intrahepatic cholestasis of pregnancy: maternal and fetal outcomes associated with elevated bile acid levels. Am J Obstet ...
  • A liver biopsy may be done to check the liver tissue. (cincinnatichildrens.org)
  • Our aims were to find the most characteristic early microscopic features of the disease as well as to compare changes in the liver biopsy specimens at the time of diagnosis and long- time (more than 10 years) after a surgical procedure . (bvs.br)
  • We examined retrospectively 8 liver biopsies from 4 PFIC2 patients comparing the results from the first biopsies done at the time of PFIC diagnosis and the second ones, done many years after PEBD. (bvs.br)
  • The formation of lobular rosettes with centrally located bile plugs and degenerative changes of hepatocytes seem to be the most characteristic microscopic features in early liver biopsies in PFIC2 patients . (bvs.br)
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