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.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)Cholangiocarcinoma: A malignant tumor arising from the epithelium of the BILE DUCTS.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 Duct Neoplasms: Tumors or cancer of the BILE DUCTS.Liver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.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.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.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.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.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.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.Bile Canaliculi: Minute intercellular channels that occur between liver cells and carry bile towards interlobar bile ducts. Also called bile capillaries.Bilirubin: A bile pigment that is a degradation product of HEME.Liver Diseases: Pathological processes of the LIVER.Biliary Tract: The BILE DUCTS and the GALLBLADDER.Pruritus: An intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.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.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 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.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.Liver Neoplasms: Tumors or cancer of the LIVER.Portal Vein: A short thick vein formed by union of the superior mesenteric vein and the splenic vein.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.Hepatocytes: The main structural component of the LIVER. They are specialized EPITHELIAL CELLS that are organized into interconnected plates called lobules.Cholangitis: Inflammation of the biliary ductal system (BILE DUCTS); intrahepatic, extrahepatic, or both.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.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.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.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.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)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.Hepatic Artery: A branch of the celiac artery that distributes to the stomach, pancreas, duodenum, liver, gallbladder, and greater omentum.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.Common Bile Duct: The largest bile duct. It is formed by the junction of the CYSTIC DUCT and the COMMON HEPATIC DUCT.Hepatitis: INFLAMMATION of the LIVER.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.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.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 Transplantation: The transference of a part of or an entire liver from one human or animal to another.Hepatic Veins: Veins which drain the liver.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.Biliary Tract Diseases: Diseases in any part of the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.Hepatectomy: Excision of all or part of the liver. (Dorland, 28th ed)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.Liver Circulation: The circulation of BLOOD through the LIVER.Norethandrolone: A synthetic hormone with anabolic and androgenic properties and moderate progestational activity.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.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.Coleus: A plant genus of the family Lamiaceae. The species of Coleus should be distinguished from PLECTRANTHUS BARBATUS - which is also known as Coleus forskohlii.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.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.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.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 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)Imino AcidsLithiasis: 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.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.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.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.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.Biopsy: Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.Infant, Newborn: An infant during the first month after birth.Hepatomegaly: Enlargement of the liver.Cholelithiasis: Presence or formation of GALLSTONES in the BILIARY TRACT, usually in the gallbladder (CHOLECYSTOLITHIASIS) or the common bile duct (CHOLEDOCHOLITHIASIS).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.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).Biliary Tract Neoplasms: Tumors or cancer in the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.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.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)Portography: Examination of the portal circulation by the use of X-ray films after injection of radiopaque material.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.Adenoma, Bile Duct: A benign tumor of the intrahepatic bile ducts.Arthrogryposis: Persistent flexure or contracture of a joint.Biliary Fistula: Abnormal passage in any organ of the biliary tract or between biliary organs and other organs.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.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.Portal Pressure: The venous pressure measured in the PORTAL VEIN.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.Dothiepin: A tricyclic antidepressant with some tranquilizing action.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).Sulfobromophthalein: A phenolphthalein that is used as a diagnostic aid in hepatic function determination.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.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.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.Mice, Inbred C57BLAscites: Accumulation or retention of free fluid within the peritoneal cavity.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.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)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.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.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.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.Liver Cirrhosis, Experimental: Experimentally induced chronic injuries to the parenchymal cells in the liver to achieve a model for LIVER CIRRHOSIS.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.Liver Cirrhosis, Alcoholic: FIBROSIS of the hepatic parenchyma due to chronic excess ALCOHOL DRINKING.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.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.Pregnancy: The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.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.Gastrointestinal Hemorrhage: Bleeding in any segment of the GASTROINTESTINAL TRACT from ESOPHAGUS to RECTUM.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.Epichlorohydrin: A chlorinated epoxy compound used as an industrial solvent. It is a strong skin irritant and carcinogen.Mitragyna: A plant genus of the family RUBIACEAE. Members contain antimalarial (ANTIMALARIALS) and analgesic (ANALGESICS) indole alkaloids.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)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.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.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.Fat Emulsions, Intravenous: Emulsions of fats or lipids used primarily in parenteral feeding.Glycocholic Acid: The glycine conjugate of CHOLIC ACID. It acts as a detergent to solubilize fats for absorption and is itself absorbed.Metabolism, Inborn Errors: Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.Biliary Tract Surgical Procedures: Any surgical procedure performed on the biliary tract.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.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.Alkaline Phosphatase: An enzyme that catalyzes the conversion of an orthophosphoric monoester and water to an alcohol and orthophosphate. EC 3.1.3.1.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.Hepatitis, Animal: INFLAMMATION of the LIVER in non-human animals.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.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.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Gallbladder Diseases: Diseases of the GALLBLADDER. They generally involve the impairment of BILE flow, GALLSTONES in the BILIARY TRACT, infections, neoplasms, or other diseases.Syndrome: A characteristic symptom complex.Portacaval Shunt, Surgical: Surgical portasystemic shunt between the portal vein and inferior vena cava.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.Antipruritics: Agents, usually topical, that relieve itching (pruritus).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)Hepatitis, 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).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)Portoenterostomy, Hepatic: Operation for biliary atresia by anastomosis of the bile ducts into the jejunum or duodenum.Hepatitis, Viral, Animal: INFLAMMATION of the LIVER in animals due to viral infection.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.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 2.6.1.17-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.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.Cefotiam: One of the CEPHALOSPORINS that has a broad spectrum of activity against both gram-positive and gram-negative microorganisms.Tomography, X-Ray Computed: Tomography using x-ray transmission and a computer algorithm to reconstruct the image.Recurrence: The return of a sign, symptom, or disease after a remission.Chronic Disease: 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)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.Islets of Langerhans Transplantation: The transference of pancreatic islets within an individual, between individuals of the same species, or between individuals of different species.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.

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)

Progressive Familial Intrahepatic Cholestasis (PFIC) Market. Currently, the market of PFIC does not hold any approved interventions but changes in the diet, medicines, and surgical treatments can reduce the effects and complications of the condition. The treatment, which is generally symptomatic involves observation of the expert physicians depending on the features and severity of the condition and its effects.. The Progressive Familial Intrahepatic Cholestasis (PFIC) market outlook section of the report helps to build the detailed comprehension of the historic, current and forecasted Progressive Familial Intrahepatic Cholestasis (PFIC) market trends by analyzing the impact of current therapies on the market, unmet needs, drivers and barriers and demand for better technology. The report gives a thorough detail of Progressive Familial Intrahepatic Cholestasis (PFIC) market trend of each marketed drug and late-stage pipeline therapy by evaluating their impact based on the annual cost of therapy, ...
Progressive Familial Intrahepatic Cholestasis (PFIC) Market. Currently, the market of PFIC does not hold any approved interventions but changes in the diet, medicines, and surgical treatments can reduce the effects and complications of the condition. The treatment, which is generally symptomatic involves observation of the expert physicians depending on the features and severity of the condition and its effects.. The Progressive Familial Intrahepatic Cholestasis (PFIC) market outlook section of the report helps to build the detailed comprehension of the historic, current and forecasted Progressive Familial Intrahepatic Cholestasis (PFIC) market trends by analyzing the impact of current therapies on the market, unmet needs, drivers and barriers and demand for better technology. The report gives a thorough detail of Progressive Familial Intrahepatic Cholestasis (PFIC) market trend of each marketed drug and late-stage pipeline therapy by evaluating their impact based on the annual cost of therapy, ...
Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC2) is a rare congenital cholestatic liver disease that progresses to end stage liver disease. It is associated with fat soluble vitamin D deficiency rickets and severe dyslipidemia; however, treatment of these secondary effects remains a challenge. One year old twin males born to a mother with intrahepatic cholestasis during pregnancy presented with jaundice, pruritus and failure to thrive. Lab evaluation revealed significant transaminitis, direct hyperbilirubinemia and normal gamma glutamyl transferase (GGT). Genetic studies confirmed PFIC2. Further evaluation for fat soluble vitamin deficiencies revealed severe vitamin D deficiency rickets. High dose vitamin D replacement therapy using Ergocalciferol (Vitamin D2) 50,000 IU three times a week over 10 weeks led to the improvement of Vitamin D, 25-Hydroxy (25-OH) serum levels and resolution of rickets. Dyslipidemia with very low high density lipoprotein-cholesterol (HDL-C) and high triglycerides
1. Jacquemin E. Progres-sive familial intrahepatic cholestasis. Clin Res Hepatol Gastroenterol 2012; 36 (Suppl 1): S26- S35. doi: 10.1016/ S2210-7401(12)70018-9. 2. Strautnieks SS, Bull LN, Knisely AS et al. A gene encod-ing a liver-specific ABC transporter is mutated in progres-sive familial intrahepatic cholestasis. Nat Genet 1998; 20(3): 233- 238. 3. Jansen PL, Mül-ler M. The molecular genetics of familial intrahepatic cholestasis. Gut 2000; 47(1): 1- 5. 4. Gerloff T, Stieger B, Hagenbuch B et al. The sister of P-glycoprotein represents thecanalicular bile salt export pump of mam-malian liver. J Biol Chem 1998; 273(16): 10046- 10050. 5. Davis RA, Miyake JH, Hui TY et al. Regulation of cholesterol-7alpha-hydroxylase: BAREly mis-s-ing SHP. J Lipid Res 2002; 43(4): 533- 543. 6. Thompson R, Strautnieks SS. BSEP: function and role in progres-sive familial intrahepatic cholestasis. Semin Liver Dis 2001; 21(4): 545- 550. 7. Kaliciński PJ, Ismail H, Jankowska I et al. Surgical treatment of ...
Progressive familial intrahepatic cholestasis (PFIC) comprises a group of rare cholestatic liver disorders of childhood that could lead to liver cirrhosis. Nowadays, the partial biliary diversion procedure is still a therapeutic option in non-cirrhotic children with PFIC1 or PFIC2 after an ineffective ursodeoxycholic acid (UDCA) therapy. However, the relevant disadvantage of the partial external biliary diversion (PEBD) is that adolescent patients could not accept a permanent stoma. In some of them, despite of good clinical and biochemical results of this procedure, the ileal exclusion (IE) procedure had to be performed many years after PEBD. 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. We examined retrospectively 8 liver biopsies from 4 PFIC2 patients comparing the results from the first biopsies done at ...
Progressive familial intrahepatic cholestasis (PFIC) is a rare genetic liver disease that affects infants and children. In many cases, patients diagnosed with PFIC experience end-stage liver disease by 10 years old.1 A serious consequence of PFIC is severe itching that can lead to sleepless nights for the whole family. If left unprotected, babies may even scratch through their skin causing bleeding, scabs, and wounds.
... is a rare genetic disease. Learn more about causes, symptoms and treatment for this condition.
Swiss drugmakers Roche and Novartis have provided financial backing French gene therapy start-up Vivet Therapeutics, with the latter raising EUR37.5 million (US$41 million) in an initial financing round.. The funds will be used by Vivet to advance a diversified pipeline of gene therapy programs targeting rare, inherited metabolic diseases, including Wilson Disease, progressive familial intrahepatic cholestasis type 2 (PFIC2), progressive familial intrahepatic cholestasis type 3 (PFIC3) and citrullinemia type I.. Vivet, created last year in Paris with a wholly owned subsidiary in Spain, is focused on developing novel gene therapies for rare, inherited metabolic diseases.. Its lead program VTX801, which is expected to enter clinical testing by the end of 2018, targets a condition called Wilson Disease.. This rare genetic disorder is caused by a defective gene in liver cells encoding the ATP7B protein, which reduces the livers ability to regulate copper levels in the liver and other tissues ...
MalaCards based summary : Cholestasis, Progressive Familial Intrahepatic 4, also known as pfic4, is related to bile acid synthesis defect, congenital, 1 and congenital bile acid synthesis defect, and has symptoms including hepatic failure, portal hypertension and intrahepatic cholestasis. An important gene associated with Cholestasis, Progressive Familial Intrahepatic 4 is TJP2 (Tight Junction Protein 2). The drugs Anticholesteremic Agents and Antimetabolites have been mentioned in the context of this disorder. Affiliated tissues include liver ...
Progressive familial intrahepatic cholestasis 2 is a rare condition and is one of many forms of cholestasis. Cholestasis is a rare disease where a persons liver can not move the bile it makes to the small intestine. The liver, an organ, is responsible for producing bile. Bile is a compound that helps people digest fats. Once the bile has been made, it is supposed to go to the small intestine, another organ, to digest the fats there. However, in people with cholestasis, the bile can not move to the small intestine because there is either a physical block or because the bile is stuck in the liver cells. Symptoms of cholestasis are itchiness, jaundice (yellowing of the skin), pale stool, and dark urine. People with progressive familial intraheptic cholestasis 2 are not able to move the bile from the cells in the liver that produce it to the small intestine to digest fats. Talk with your doctor to find the best treatment for you if you have been diagnosed with progressive familial intraheptic ...
ATP-binding cassette, sub-family B member 11 also known as ABCB11 is a protein which in humans is encoded by the ABCB11 gene. The product of the ABCB11 gene is an ABC transporter named BSEP (Bile Salt Export Pump), or sPgp (sister of P-glycoprotein). This membrane-associated protein is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Some members of the MDR/TAP subfamily are involved in multidrug resistance. This particular protein is responsible for the transport of taurocholate and other cholate conjugates from hepatocytes (liver cells) to the bile. In humans, the activity of this transporter is the major determinant of bile formation and bile flow. ABCB11 is a gene associated with progressive familial intrahepatic cholestasis type 2 ...
BACKGROUND: Though possession of androgenic anabolic steroids (AAS) is illegal, non-prescription use of AAS persists. METHODS: We describe two Caucasian males (aged 25 and 45 years) with cholestatic hepatitis following ingestion of the dietary supplement Mass-Drol (Celtic Dragon) containing the AAS 2α-17α-dimethyl-etiocholan-3-one,17β-ol.. RESULTS: Despite substantial hyperbilirubinaemia peak gamma-glutamyl transferase (GGT) remained normal. Besides bland intralobular cholestasis, liver biopsy in both found deficiency of canalicular expression of ectoenzymes as seen in ATP8B1 disease. In the older patient, bile salt export pump marking (encoded by ABCB11) was focally diminished. We hypothesized that AAS had either induced inhibition of normal ATP8B1/ABCB11 expression or triggered initial episodes of benign recurrent intrahepatic cholestasis (BRIC) type 1/or 2. On sequencing, ATP8B1 was normal in both patients although the younger was heterozygous for the c.2093G,A mutation in ABCB11, a ...
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BACKGROUND: Partial internal biliary diversion (PIBD) is an alternative approach for the treatment of devastating pruritus in patients with progressive familial intrahepatic cholestasis (PFIC). In these patients quality of life can be improved and progression of liver disease can be delayed while waiting for liver transplantation. The aim of our study was to evaluate six patients with PFIC who have undergone PIBD in long-term follow-up. METHODS: Retrospective review of the records of six patients who underwent PIBD for PFIC between 2008 and 2010 was conducted to evaluate age, growth, clinical and laboratory studies for long-term outcome ...
β-Catenin, the downstream effector of the Wnt signaling, plays important roles in hepatic development, regeneration and tumorigenesis. However, its role at hepatocyte adherens junctions (AJ) is relatively poorly understood, chiefly due to spontaneous compensation by γ-catenin. Here, we simultaneously ablate β- and γ-catenin expression in mouse liver by interbreeding β-catenin-γ-catenin double-floxed mice and albumin-cre transgenic mice. Double knockout mice (DKO) show failure to thrive, impaired hepatocyte differentiation, cholemia, ductular reaction, progressive cholestasis, inflammation, fibrosis and tumorigenesis, which was associated with deregulation of tight junctions (TJ) and bile acid transporters, leading to early morbidity and mortality, a phenotype reminiscent of Progressive Familial Intrahepatic Cholestasis (PFIC ...
Mutations in the ATP8B1 gene cause two autosomal recessive disorders affecting liver: cholestasis, benign recurrent intrahepatic, 1 (BRIC1), cholestasis, progressive familial intrahepatic, 1 (PFIC1) and one autosomal dominant disorder: cholestasis, intrahepatic, of pregnancy, 1 (ICP1). BRIC2 is caused by mutations in the ABCB11 gene. PFIC can be caused by mutations in three other genes: ABCB11 (PFIC2), ABCB4 (PFIC3) and TJP2 (PFIC4). Mutations in the ABCB4 gene have been reported in ICP3. BRIC is characterized by intermittent episodes of cholestasis without extrahepatic bile duct obstruction. PFIC is characterized by early onset of cholestasis that progresses to hepatic fibrosis, cirrhosis, and end-stage liver disease before adulthood. ICP typically occurs in the third trimester and it recurs in 45 to 70% of subsequent pregnancies. Findings include pruritus, jaundice, increased serum bile salts, and abnormal liver enzymes. This condition is reversible, but it can result in fetal complications ...
definition of SCIH, what does SCIH mean?, meaning of SCIH, Sickle Cell Intrahepatic Cholestasis, SCIH stands for Sickle Cell Intrahepatic Cholestasis
A 33-year-old Japanese man who had suffered from liver cirrhosis due to hepatitis C virus (HCV) underwent living related liver transplantation (LRLT). The allograft was given by his brother, who was healthy with no history of hepatitis or hepatic virus infection. After LRLT, the patients hepatitis C recurred. Liver biopsy revealed chronic viral hepatitis and no allograft rejection such as shown by portal lymphocytic infiltration or mild bridging fibrosis. Interferon and ribavirin were administered, and sustained viral response (SVR) was obtained. Although serum hepatitis B virus (HBV)-DNA/HCV-RNA polymerase chain reaction found no presence of hepatic virus, the serum examination demonstrated liver dysfunction seven months after SVR. Liver biopsies histopathologically showed portal fibrosis invading to the sinusoids, cholestasis, mild hyperplasia of the cholangioles, and no features of allograft rejection. Fibrosing cholestatic hepatitis (FCH) was diagnosed. The FCH was resistant to treatment ...
Background: Intrahepatic cholestasis of pregnancy (ICP) is characterised by troublesome maternal pruritus, raised serum bile acid levels and increased fetal risk. Mutations of the ABCB4 gene encoding the hepatobiliary phospholipid transporter have been identified in a small proportion of patients with cholestasis of pregnancy. In a recent prospective study on 693 patients with cholestasis of pregnancy, a cut-off level for serum bile acid (⩾40 μmol/l) was determined for increased risk of fetal complications.. Objectives: To investigate whether common combinations of polymorphic alleles (haplotypes) of the genes encoding the hepatobiliary ATP-binding cassette (ABC) transporters for phospholipids (ABCB4) and bile acids (ABCB11) were associated with this severe form of cholestasis of pregnancy.. Methods: For genetic analysis, 52 women with bile acid levels ⩾40 μmol/l (called cases) and 52 unaffected women (called controls) matched for age, parity and geographical residence were studied. Gene ...
A 57-yr-old man presented with clinical and laboratory signs of acute cholestatic hepatitis. Symptoms had appeared 7 wk after he was started on pravastatin 20 mg/day for hypercholesterolemia. A full evaluation including ultrasound, computed tomography, endoscopic cholangiography, and liver biopsy confirmed the diagnosis of intrahepatic nonobstructive jaundice. The liver function abnormalities normalized 7 wk after cessation of therapy. Pravastatin should be considered as a potential cause of cholestatic hepatitis with favorable clinical outcome after drug withdrawal ...
Intrahepatic cholestasis of pregnancy (ICP), also known as obstetric cholestasis, cholestasis of pregnancy, jaundice of pregnancy, and prurigo gravidarum, is a medical condition in which cholestasis occurs during pregnancy. It typically presents with troublesome itching and can lead to complications for both mother and fetus. Pruritus (itching) has long been considered to be a common symptom of pregnancy. The vast majority of times, itching is a minor annoyance caused by changes to the skin, especially that of the abdomen. However, there are instances when itching is a symptom of ICP. This is usually most intense on the palms of the hands, and the soles of the feet, but can be widespread. ICP occurs most commonly in the third trimester, but can begin at any time during the pregnancy. Most women with this condition present in third trimester with itching without a rash. Typically, the itching is localized to the palms of the hands and soles of the feet but can be anywhere on the body. Hallmarks ...
ICP (intrahepatic cholestasis of pregnancy) is characterized by pruritus and biochemical cholestasis, including raised SBAs (serum bile acids) and, usually, elevated aminotransferases levels. However, AHP (asymptomatic hypercholanaemia of pregnancy) is defined as the presence of total SBA levels above the cut-off value (11 μM) in healthy pregnant women, thus elevation of total SBAs do not necessarily reflect an ICP condition. The aim of the present study was to describe clinical, obstetric, perinatal and biochemical findings, as well as the SBA profile, in pregnant women studied in the third trimester of pregnancy in order to define characteristic patterns of individual bile acids that enable women with ICP to be distinguished from AHP and healthy pregnancies. Free and conjugated ursodeoxycholic (UDCA), cholic (CA), lithocholic (LCA), deoxycholic (DCA) and chenodeoxycholic (CDCA) acids were evaluated by CE (capillary electrophoresis) in 41 patients (15 of them simultaneously by HPLC), in 30 ...
Participation of cholestatic factor in the pathogenesis of intrahepatic cholestasis in acute viral hepatitis. - Y Mizoguchi, Y Sakagami, H Tsutsui, T Monna, S Yamamoto, S Morisawa
Objective : To determine the risk of adverse pregnancy outcomes resulting from intrahepatic cholestasis. Methods : We analyzed 91 women with singleton pregnancies complicated by cholestasis who gave birth at Kuopio University Hospital from January 1990 to December 1996. Logistic regression analysis was used to compare pregnancy outcomes of this...
Find out about itching during pregnancy, including causes, ways to ease itching, and when you need to seek medical attention fast for possible intrahepatic cholestasis of pregnancy (ICP), also called obstetric cholestasis.
A prospective study was undertaken to evaluate fat malabsorption during intrahepatic cholestasis of pregnancy (ICP), a disease characterized by a mild cholestasis of short duration appearing in otherwise healthy young women. An abnormal fecal fat exc
Abu-Hayyeh, S., Papacleovoulou, G., Lövgren-Sandblom, A., Tahir, M., Oduwole, O., Jamaludin, N. A., Ravat, S., Nikolova, V., Chambers, J., Selden, C., Rees, M., Marschall, H.-U., Parker, M. G. and Williamson, C. (2013), Intrahepatic cholestasis of pregnancy levels of sulfated progesterone metabolites inhibit farnesoid X receptor resulting in a cholestatic phenotype. Hepatology, 57: 716-726. doi: 10.1002/hep.26055 ...
The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is the major canalicular bile salt export pump in man. Mutations in this gene cause a form of progressive familial intrahepatic cholestases which are a group of inherited disorders with severe cholestatic liver disease from early infancy. [provided by RefSeq, Jul 2008 ...
Intrahepatic cholestasis of pregnancy (ICP) is the most prevalent pregnancy-specific liver disease. It occurs mainly in the second or third trimester of pregnancy. It typically resolves after delivery spontaneously but it is associated with an increased risk of adverse fetal outcomes.. The cause of ICP is heterogeneous, pathophysiology is poorly understood and therapies have been empiric. Genetic predispositions, environmental influences, dietary factors and hormonal influences have been studied and cited in the literature.. Comparing with placebo, ursodeoxycholic acid (UDCA) has been shown improvement in treatment of pruritus in previous studies. S-adenosylmethionine, guar gum, activated charcoal, dexamethasone, cholestyramine, etc. are not effective in the treatment of symptoms.. CD4+ T cells are an essential regulators of immune responses and inflammatory diseases. They are also called chief of orchestra cells of immune system. The balance between T helper-(Th)1, Th-2 and Th-17 cells and ...
Background Intrahepatic cholestasis of pregnancy (ICP) is an uncommon obstetric condition characterised by intense maternal pruritis and biochemical abnormality. There is a degree of contention regarding the diagnosis and management of ICP, and currently, there are no nationally accepted guidelines. Aims To conduct a survey of Fellows and Members of the Royal Australian and New Zealand College of Obstetricians and Gynaecologists (RANZCOG) regarding their diagnosis and management ICP. Methods An online survey of currently practising RANZCOG Fellows and Members, utilising Survey Monkey. Results Thirty percent of those sent the survey responded, comprising approximately 40% of practising obstetricians. Fasting bile acid and serum transaminase elevation in association with the characteristic itch define the disease process for the majority of respondents and also inform management decisions. There was no critical level of bile acid elevation that mandated treatment for the majority of respondents. ...
A retrospective case-control study of 21,008 women in Finland has found that those with intrahepatic cholestasis of pregnancy (ICP), an itchy skin condition when bile gets backed up in the liver, are significantly more likely to suffer other liver diseases later in life.
Cholestasis, benign recurrent intrahepatic, 2 (BRIC2) [MIM:605479]: A disorder characterized by intermittent episodes of cholestasis without progression to liver failure. There is initial elevation of serum bile acids, followed by cholestatic jaundice which generally spontaneously resolves after periods of weeks to months. The cholestatic attacks vary in severity and duration. Patients are asymptomatic between episodes, both clinically and biochemically. {ECO:0000269,PubMed:15300568, ECO:0000269,PubMed:16039748}. Note=The disease is caused by mutations affecting the gene represented in this entry ...
Intrahepatic cholestasis of pregnancy (obstetric cholestasis) is characterised by pruritus, otherwise unexplained deranged liver enzyme levels, and elevated levels of serum bile acid.1 The itching typically subsides almost immediately after delivery and the serum bile acid and liver enzyme levels normalise within a few weeks.2 Intrahepatic cholestasis of pregnancy usually presents in the late second and third trimester3 although it has been reported as early as 6-10 weeks gestation.4. Intrahepatic cholestasis of pregnancy affects about 0.7% of pregnancies in the United Kingdom, varying by ethnic group,5 and usually runs a relatively benign course. The condition is associated with increased rates of spontaneous preterm labour, antepartum passage of meconium, and asphyxial events, but its relation to perinatal mortality is uncertain; early studies reported an increased risk of stillbirth, but some recent studies have cast doubt on the magnitude of the increased risk.1 Interpretation has been ...
Looking for cholestatic hepatitis? Find out information about cholestatic hepatitis. inflammation of the liver. There are many types of hepatitis. Causes include viruses, toxic chemicals, alcohol consumption, parasites and bacteria, and... Explanation of cholestatic hepatitis
The diagnoses of liver and biliary disease were traced from the Finnish Hospital Discharge Register with an almost 100% coverage. The researchers found that patients with intrahepatic cholestasis of pregnancy had a higher incidence of liver and biliary disease than in controls. The rate ratio for Hepatitis C was 4, for nonalcoholic liver cirrhosis 8.. The team noted that the rate ratio for gallstones and cholecystitis was 4, and 3 for nonalcoholic pancreatitis. Dr Ropponens team concluded, There is an association of intrahepatic cholestasis of pregnancy with several liver and biliary diseases. Some patients with Intrahepatic cholestasis of pregnancy are at risk of the subsequent development of cirrhosis and other severe chronic diseases. Contrary to what has been previously thought, follow-up may need to be considered for these patients. ...
The prominent finding of this large retrospective study is the presence of marked intraparenchymal cholestasis on liver biopsy as an independent predictor of survival, along with age and the Maddreys score. Among other histological lesions commonly observed in ASH[20] bilirubinostasis was also the sole predictor of outcome. Interpretation of intrahepatic cholestasis is especially challenging in patients with decompensated cirrhosis at risk of developing biliary tract disease, infection or sepsis. In the latter situation, intrahepatic cholestasis is a prominent finding[13]. Having reasonably excluded the role of bile duct lesions or concomitant sepsis with the complete work-up performed at admission, intraparenchymal cholestasis can be considered as a lesion associated with ASH, as previously suggested[21]. Our results are in line with the study by Nissenbaum et al.[9] who reported lobular cholestasis in 38% of patients that correlated to malnutrition and a poor clinical outcome. In a recent ...
Viral hepatitis characterized by prolonged cholestasis has not been associated with a specific serologic marker. We report the cases of six patients presenting with a clinical syndrome typical of cholestatic hepatitis who were subsequently found to have acute hepatitis A. Usual features include pruritus, fever, diarrhea, and weight loss with serum bilirubin levels greater than 10 mg/dL, and a clinical course lasting at least 12 weeks. All patients recovered completely without sequelae. Knowledge of this unusual manifestation of hepatitis A may help avoid potentially invasive procedures involved in the evaluation of suspected obstructive jaundice and facilitate appropriate immunoprophylactic measures. ...
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In recent years, our knowledge about the pathogenesis, pathophysiology and treatment of hepatobiliary diseases has increased considerably. The molecular basis of cholestatic disorders as well as of gallstone disease is increasingly recognized. This has resulted in improved diagnosis, for instance in hereditary forms of intrahepatic cholestasis, and advances in treatment, for example in primary biliary cirrhosis and other chronic cholestatic disorders. This book, the proceedings of a Falk Workshop held in Cluj-Napoca, Romania, on June 9-10, 2000, brings together contributions from scientists and clinicians to highlight the most recent advances in molecular biology, pathophysiology, diagnosis and therapy of diseases of the hepatobiliary system. World experts cover a broad spectrum of topics from genetic studies to endoscopy and from medical treatment to liver transplantation.Acalovschi, M. is the author of Hepatobiliary Diseases Cholestasis and Gallstone with ISBN 9780792387701 and ISBN ...
Christina DePino thought the severe itching she felt was a normal symptom of pregnancy. Luckily she had gone to a checkup before things turned for the worse as she found out she could have given birth to a premature or stillborn baby and then shares awareness of cholestasis of pregnancy causes.
27 week appt yesterday and on top of out Trisomy 21 diagnosis I now gave cholestasis which means she will be delivered at 37 weeks to avoid possible complications. My poor baby girl. Even more so because of the DS diagnosis I just wanted her to stay cozy as long as possible
UDCA (ursodeoxycholic acid): This is believed to be a "friendly" bile acid that displaces the more harmful bile acids from the blood. Many doctors believe that UDCA helps to protect the baby from the damaging effects of bile acid as well as helping to relieve symptoms. A recent pilot trial that looked at UDCA versus placebo showed that the incidence of meconium staining was reduced in those women who received UDCA and that the ALT/AST levels improved. However, it did not show that bile acid levels were improved for a significant number of women. A further larger-powered trial is planned. UDCA is typically administered starting at 500mg per day, and rising in 500mg increments to a maximum dose of 2000mg per day. Some clinicians still adhere to the recommended prescribing policy of basing the dosage on weight i.e. 12-15mg per kg of bodyweight but in the UK where the condition is the subject of extensive research most clinicians begin with 500mg twice a day. UDCA is still unlicensed for use during ...
Some providers choose to prescribe vitamin K to all Intrahepatic Cholestasis of Pregnancy patients. Others only do so when there is evidence of a deficiency such as abnormal clotting shown in PT (prothrombin time) and/or PTT (partial thromboplastin time) tests, abnormal bruising, or pale stools.. 2) Early Delivery in the management plan of ICP.. Since it is not possible to predict which pregnancies are at risk for stillbirth, it is recommended that all ICP pregnancies, both mild and severe, be delivered early, even if serum bile acids return to normal after treatment with UDCA. In most cases, delivery will occur at 36-37+0 weeks gestation as recommended by (American College of Obstetrics and Gynecology) recommends delivery at 36+0 to 37+0, by committee opinion published in February, 2019. In some cases, when Intrahepatic Cholestasis of Pregnancy is not well-controlled, providers may choose to deliver even earlier such as women with bile acids of 100 μmol/L. Exact timing of delivery can be ...
For maternal pruritus, antihistamines and topical therapy with emollients may provide some relief 2, 3, 6. Although cholestyramine may be effective, it may decrease the absorption of fat-soluble vitamins, leading to vitamin K deficiency and fetal coagulopathy.. Considering the previous beneficial experience in primary biliary cirrhosis with the use of Ursodeoxycholic acid (UDCA), an oral hydrophilic tertiary bile acid, our Chilean group was the first one to publish an open clinical trial showing the beneficial effects of UDCA in ICP 22. In this trial, nine patients with severe ICP received oral UDCA 15mg/kg/day (divided twice a day) obtaining relief of pruritus in most mothers and improvement of liver tests without any adverse effect. After discontinuing UDCA, pruritus and biochemical abnormalities reappeared, but they improved again after re-challenge with oral UDCA. Since then, other clinical series and then controlled studies have shown that UDCA administration provides a significant ...
Having a liver transplant is a major life event and research shows that such a substantial physical and emotional change can put you under considerable stress. It is therefore common to experience a whole range of emotions and some people will experience psychological problems following their transplant. This is particularly likely for people without good support from family and friends, those who have had problems like depression before transplant and those with substance use disorders.. However, there are studies that show that people who receive a liver transplant also receive psychological benefits. Many people who have problems before transplant, including memory impairment, slow reactions, anxiety and depression that are common in end-stage liver disease, find that the transplant helps these considerably.. It is important to be realistic about life with your new liver and be patient with yourself. Years of living with a serious liver problem, the uncertainty about receiving a transplant ...
This is my second pregnancy. I had ICP with my last and delivered at 36 weeks. At that time it took approximately 2 weeks to get a diagnosis. I...
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Fifteen patients with cholestatic disorders were treated for 1 to 5 months with phenobarbital. Primary biliary cirrhosis was diagnosed in seven, sclerosing cholangitis in two, intrahepatic biliary hypoplasia in three, and cholestatic hepatitis in three. Except for the patients with cholestatic hepatitis, in whom marked cholestasis was virtually the only abnormality in liver biopsy specimens, serum bilirubin and bile acid concentrations were diminished during therapy, the hepatic clearance of sulfobromophthalein and 131I-rose bengal was variably enhanced, and there was relief from pruritus. Serum cholesterol concentrations and other measures of hepatic function were not significantly changed during therapy except for serum alkaline phosphatase activity, which rose in twelve patients. Parallel changes occurred in 5′-nucleotidase, suggesting a hepatic origin for the alkaline phosphatase activity. These studies indicate that phenobarbital therapy is associated with improvement in organic anion ...
TY - JOUR. T1 - X-linked cholestasis in mouse due to mutations of the P4-ATPase ATP11C. AU - Siggs, Owen M.. AU - Schnabl, Bernd. AU - Webb, Bill. AU - Beutler, Bruce. PY - 2011/5/10. Y1 - 2011/5/10. N2 - Transporters at the hepatic canalicular membrane are essential for the formation of bile and the prevention of cholestatic liver disease. One such example is ATP8B1, a P4-type ATPase disrupted in three inherited forms of intrahepatic cholestasis. Mutation of the X-linked mouse gene Atp11c, which encodes a paralogous P4-type ATPase, precludes B-cell development in the adult bone marrow, but also causes hyperbilirubinemia. Here we explore this hyperbilirubinemia in two independent Atp11c mutant mouse lines, and find that it originates from an effect on nonhematopoietic cells. Liver function tests and histology revealed only minor pathology, although cholic acid was elevated in the serum of mutant mice, and became toxic to mutant mice when given as a dietary supplement. The majority of homozygous ...
Cholestatic liver disease refers to a condition that impairs the production or flow of bile. It can cause itchiness in pregnant women and jaundice for newborns.
The Autoimmune and Cholestatic Liver Disease clinic provides care for patients with liver issues related to complications of autoimmunity.
Recipients of extra-hepatic organ transplants with chronic hepatitis C (HCV) infection pre-transplant can develop progressive post-transplant liver disease, which is well reported to be a significant cause of morbidity and mortality. 7 to 24 percent of renal transplant (RT) patients are found to have serum liver biochemical abnormalities, and liver failure is determined to be the cause of death in 8 to 28 percent of post-renal transplant recipients.1,2 A major concern in these transplant recipients is the onset of an aggressive and rapid course of HCV-related infection and liver disease, facilitated by ongoing immunosuppression to prevent graft rejection.3,4 Transplant associated immunosuppression has been reported to cause increased hepatitis C replication in addition to a range of liver-related complications such as chronic active hepatitis, fibrosing cholestatic hepatitis, fulminant liver failure and hepatocellular carcinoma. Organ transplant recipients with hepatitis C have been shown to ...
The bile salt export pump (BSEP) is an ABC-transporter expressed at the canalicular membrane of hepatocytes. Its physiological role is to expel bile salts into the canaliculi from where they drain into the bile duct. Inhibition of this transporter may lead to intrahepatic cholestasis. Predictive computational models of BSEP inhibition may allow for fast identification of potentially harmful compounds in large databases. This article presents a predictive in silico model based on physicochemical descriptors that is able to flag compounds as potential BSEP inhibitors. This model was built using a training set of 670 compounds with available BSEP inhibition potencies. It successfully predicted BSEP inhibition for two independent test sets and was in a further step used for a virtual screening experiment. After in vitro testing of selected candidates, a marketed drug, bromocriptin, was identified for the first time as BSEP inhibitor. This demonstrates the usefulness of the model to identify new BSEP ...
Intrahepatic cholestasis of pregnancy (ICP) is a potentially serious liver disorder that can develop in pregnancy. Normally, bile acids flow from your liver to your gut to help you digest food. In ICP, the bile acids do not flow properly and build up in your body instead. Theres no cure for ICP, but it should go once youve had your baby. ICP seems to run in families, but it can occur even if there is no family history. It is more common in women of south Asian origin; affecting around 1 in 70 to 80 pregnancies. If you have had ICP in a previous pregnancy, you have a high chance of developing it again in a subsequent pregnancy. Some studies have found that babies whose mothers have ICP have a higher chance of being born prematurely or stillborn. The most recent research suggests the risk of stillbirth is between 1 and 2 in 100 for those women whose bile acid levels are greater than 40µmol/L. The risk of stillbirth rises to between 4 and 5 in 100 when the bile acids are 80µmol/L. Because of ...
My search led me to information about intrahepatic cholestasis of pregnancy. Symptoms of ICP include itching without a rash (most often on the soles of the feet and palms of the hands), upper right quadrant pain, dark urine and light stools, among other things (Mayo Clinic 2014). Google also told me that ICP has a high rate of fetal demise (Reid et al. 1976). I became nauseous and my head started spinning. "One to two out of 1000 pregnancies are diagnosed with ICP" (CHOP 2014). My husband saw these odds and said, "This is so rare. I doubt you have it." My gut said otherwise.. I called my midwives. I didnt want to sound like the hypochondriac they had come to know and love, so I simply said, "Im really itchy and I dont have a rash." They told me to come in immediately for blood work.. They drew my blood and sent it out to test my bile acid levels. With ICP, the pregnant womans liver secretes bile acids into her bloodstream, leading to a buildup of bile acids in the baby. These bile acids are ...
Tribe, Rachel M., Dann, Anthony T., Kenyon, Anna P., Seed, Paul, Shennan, Andrew H. and Mallet, Anthony (2010) Longitudinal profiles of 15 serum bile acids in patients with intrahepatic cholestasis of pregnancy. The American Journal of Gastroenterology, 105. pp. 585-595. ISSN 0002-9270 (Print), 1572-0241 (Online) (doi:10.1038/ajg.2009.633) ...
III. MUCOSAL INTEGRITY Studies of gastrointestinal physiology reveal an intricate interaction between central and peripheral nerves, hormones, and peptides inside and outside the intestinal cell. This is exemplified by the different phases of vagal stimulation, made known to the rest of the world by Pavlovs classic studies in dogs in the 19th century. To use a slight understatement: Our knowledge has expanded since the work of Pavlov. We know now that physiological intestinal homeostasis is mediated through several mechanisms, whereby the way in which cellular mucosal defense is achieved is of utmost importance for maintaining mucosal integrity and thus also very relevant to drug absorption. 2. 3. If there is an impaired secretion of bile acids into the lumen (obstructive jaundice, intrahepatic cholestasis, primary biliary cirrhosis) If there is an extensive bile acid loss from the lumen, higher than the synthetic capacity in the liver If bile acids are deconjugated in the intestinal lumen ...
Difficulties arise in the interpretation of liver tests in the pregnant subject, since some values increase (alkaline phosphatase) whilst others remain unchanged (transaminases) or fall during pregnancy. The diagnosis and management of some causes of jaundice in pregnancy, such as viral hepatitis, gall stones, benign intrahepatic cholestasis and acute fatty liver of pregnancy are discussed. Little is known about the commonest symptoms of pregnancy (nausea, vomiting and constipation) other than that they might be due to hormonally induced alteration of sphincter tone. However, pre-existing bowel disease has a greater effect on pregnancy. Fertility is reduced in poor nutritional states (e.g. coeliac and Crohns diseases) and an increased occurrence of spontaneous abortion has been noted. For inflammatory bowel diseases, the time of onset is important in determining the outcome of pregnancy. Relapse in the disease is commonest in the first trimester and in the puerperium. Treatment of these ...
Relief for Itchy Stretch Marks During Pregnancy. Stretch marks during pregnancy might itch because of dry skin and irritation or because of an underlying medical condition such as pruritic urticarial papules and plaques of pregnancy (PUPPP) or intrahepatic cholestasis of pregnancy. Medical conditions may require medical intervention, while normal itching usually responds to nonprescription comfort measures.
Sigma-Aldrich offers abstracts and full-text articles by [Brandy Garzel, Hui Yang, Lei Zhang, Shiew-Mei Huang, James E Polli, Hongbing Wang].
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Publication Details (including relevant citation information): Prestrelski, Steven J., Byler, D. Michael, Liebman, Michael N. Biochemistry 1991
Hepatic retransplantation in cholestatic liver disease: Impact of the interval to retransplantation on survival and resource utilization (pages 395-400). W. Ray Kim, Russell H. Wiesner, John J. Poterucha, Terry M. Therneau, Michael Malinchoc, Joanne T. Benson, Jeffrey S. Crippin, Goran B. Klintmalm, Jorge Rakela, Thomas E. Starzl, Ruud A. Krom, Roger W. Evans and E. Rolland Dickson. Version of Record online: 30 DEC 2003 , DOI: 10.1002/hep.510300210. ...
포르말린으로 고정된 장 조직을 DNA 를 추출할려고 합니다. 포르말린 성분을 없앨수 있는 방법 좀 알고 싶습니다.빠른 답변 부...
폰슈로 확인해보니 트랜스퍼까지 잘된것 같고 PI3K 1차 안티바디(1:1000) 을 사용했는데 이전에는 이 희...
Additional file 1: of Risk factors associated with mortality in neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and clinical implications
Women with a change in the ABCB11 gene are at risk of developing a condition called intrahepatic cholestasis of pregnancy. Affected women typically develop impaired bile secretion (cholestasis) and pruritus during the third trimester of pregnancy, and these features disappear after the baby is born. A common variation (polymorphism) in the ABCB11 gene is found more often in women who develop this condition than women who do not. This variation leads to a change in a single protein building block (amino acid) in the BSEP protein. Specifically, the amino acid valine is replaced by the amino acid alanine at position 444 of the protein (written as V444A). This change leads to a reduction in the amount of BSEP protein in liver cells. In rare cases, an uncommon change (a mutation) in one copy of the ABCB11 gene is found in women with intrahepatic cholestasis of pregnancy. A single mutation in this gene increases the risk of developing intrahepatic cholestasis of pregnancy. These mutations likely ...
We believe that this is the first report of a patient developing FCH while being treated with lamivudine. The response to treatment of lamivudine resistant FCH with adefovir dipivoxil was rapid and complete and sustained at 16 months.. The risk of graft infection with HBV after liver transplantation is greater in those patients with detectable HBV replication at the time of transplantation. In the mid-1990s, many centres excluded patients with HBeAg or HBV DNA in serum from liver transplantation because of the poor associated prognosis following graft infection with HBV. The Eurohep consensus document in 1994 recommended that such patients should only undergo transplantation in the context of a clinical trial.18 For those patients without detectable HBV replication at the time of transplantation, the risk of graft infection and death from HBV related graft disease is reduced by HBIg.19 The introduction of lamivudine has revolutionised the management of patients undergoing liver transplantation ...
Cholestasis is characterized by impaired bile flow, reduction of bile acids in the intestine, and retention of bile acids in the liver. Rats taken in alpha naphthylisothiocyanate (ANIT) have been one of the most common experimental models of intrahepatic cholestasis and used extensively, which was permitted to describe not only cholestatic alterations but also compensatory mechanisms [30]. The liver in ANIT-treated rats showed cholangiolitic hepatitis characterized by intrahepatic cholestasis, necrosis of hepatocytes and biliary epithelial cells and bile obstruction [31].. In current clinical practice, initial assessment of hepatobiliary diseases is accomplished by measuring serum concentrations of bile acids and bilirubin as well as serum activities of liver-associated enzymes which reveal information about the state of liver. Our previous study showed that the indexes of liver damage and pathological changes start to rise at 24h after ANIT treatment, reach a maximum at 48h and trend to restore ...
Farnesoid X receptor (FXR) is a bile acid-activated transcription factor that is a member of the nuclear hormone receptor superfamily. Fxr-null mice exhibit a phenotype similar to Byler disease, an inherited cholestatic liver disorder. In the liver, activation of FXR induces transcription of transporter genes involved in promoting bile acid clearance and represses genes involved in bile acid biosynthesis. We investigated whether the synthetic FXR agonist GW4064 could protect against cholestatic liver damage in rat models of extrahepatic and intrahepatic cholestasis. In the bile duct-ligation and alpha-naphthylisothiocyanate models of cholestasis, GW4064 treatment resulted in significant reductions in serum alanine aminotransferase, aspartate aminotransferase, and lactate dehydrogenase, as well as other markers of liver damage. Rats that received GW4064 treatment also had decreased incidence and extent of necrosis, decreased inflammatory cell infiltration, and decreased bile duct proliferation. Analysis
Cholestasis comprises aetiologically heterogeneous conditions characterized by accumulation of bile acids in the liver that actively contribute to liver damage. Sirtuin 1 (SIRT1) regulates liver regeneration and bile acid metabolism via modulating the farnesoid X receptor (FXR); we here investigate its role in cholestatic liver disease. We determined SIRT1 expression in livers from patients with cholestatic disease, in two experimental models of cholestasis, as well as in human and murine liver cells in response to bile acid loading. SIRT1 overexpressing (SIRToe) and hepatocyte‐specific SIRT1‐KO mice (SIRThep‐/‐) were subjected to BDL and were fed with 0.1%DDC diet to determine the biological relevance of SIRT1 during cholestasis. The effect of NorUDCA was tested in BDL/SIRToe mice. We found that SIRT1 was highly expressed in livers from cholestatic patients, mice after BDL and Mdr2‐/‐ animals. The detrimental effects of SIRT1 during cholestasis were validated in vivo and in vitro. ...
Neonatal cholestasis eligibility inclusion criteria (from rare disease eligibility criteria v1.8.1) - Neonatal cholestasis in which a known genetic disease has been excluded and in which a monogenic cause is considered likely by a specialist Liver Unit. Neonatal cholestasis eligibility exclusion criteria - Infective causes after excluding known genetic disease. Prior genetic testing guidance - Results should have been reviewed for all genetic tests undertaken, including disease-relevant genes in exome sequencing data. The patient is not eligible if they have a molecular diagnosis for their condition. - Genetic testing should continue according to routine local practice for this phenotype regardless of recruitment to the project; results of these tests must be submitted via the Genetic investigations section of the data capture tool to allow comparison of WGS with current standard testing. PLEASE NOTE: The sensitivity of WGS compared to current diagnostic genetic testing has not yet been ...
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We report on a 71-year-old man who presented to the medical department of Our Lady of Maryknoll Hospital with progressive cholestatic hepatitis. Tests for hepatitis viral markers gave negative results and ultrasonography revealed no dilated bile ducts. Endoscopic retrograde cholangiopancreatography showed a normal biliary tree. The patient had completed a 5-month course of methimazole to treat thyrotoxicosis a few weeks before the onset of the jaundice. Methimazole was suspected to be the cause of the cholestatic hepatitis; this diagnosis was supported by the results from a liver biopsy. The presentation of the patient was unusual by virtue of the delayed onset and prolonged course of cholestasis ...
Citrin is the liver-type mitochondrial aspartate glutamate carrier. Its deficiency, also known as type II citrullinemia, is an autosomal recessive genetic disorder causing metabolic derangements in aerobic glycolysis and gluconeogenesis. Urea cycle mechanisms, uridine diphosphate-galactose epimerase activity, acylcarnitine metabolism, and fatty acid synthesis and utilization are also affected mainly due to a defective aspartate export from the mitochondria to the cytosol and impairment of the malate-aspartate shuttle. Patients with this defect may harbor different mutations on gene SLC25A13 located on chromosome 7q21.3. Mutations have a carrier rate of 1:65 in Japan and China, whereas they are much less frequent in the Western world, and are responsible for 2 phenotypes of the disease. The first is a usually self-limiting neonatal (intrahepatic) cholestatic and steatotic condition (neonatal intrahepatic cholestasis caused by citrin deficiency [NICCD], OMIM #605814). The second is an adult-onset ...
TY - JOUR. T1 - The genetics of complex cholestatic disorders. AU - Hirschfield, Gideon M.. AU - Chapman, Roger W.. AU - Karlsen, Tom H.. AU - Lammert, Frank. AU - Lazaridis, Konstantinos N.. AU - Mason, Andrew L.. PY - 2013/6. Y1 - 2013/6. N2 - Cholestatic liver diseases are caused by a range of hepatobiliary insults and involve complex interactions among environmental and genetic factors. Little is known about the pathogenic mechanisms of specific cholestatic diseases, which has limited our ability to manage patients with these disorders. However, recent genome-wide studies have provided insight into the pathogenesis of gallstones, primary biliary cirrhosis, and primary sclerosing cholangitis. A lithogenic variant in the gene that encodes the hepatobiliary transporter ABCG8 has been identified as a risk factor for gallstone disease; this variant has been associated with altered cholesterol excretion and metabolism. Other variants of genes encoding transporters that affect the composition of ...
PMID 12424587] Neonatal intrahepatic cholestasis caused by citrin deficiency: severe hepatic dysfunction in an infant requiring liver transplantation. ...
Cholestatic hepatitis C (CHC) is a severe form of hepatitis C virus (HCV) infection recurrence that leads to high graft loss rates early after liver transplantation (LT). To investigate the pathogenic mechanisms of CHC, we analysed HCV quasispecies in CHC patients compared to a control group (mild hepatitis C recurrence) by deep pyrosequencing. At the time of LT, NS5B quasispecies complexity was similar between the two groups but, after LT, it decreased more sharply in CHC patients than in the control group. Interestingly, the major variant before LT propagated efficiently and remained as the dominant sequence after LT in 62 % of CHC patients versus 11 % of controls (P=0.031). Sequence analysis of the complete non-structural region in a limited number of patients revealed a potential 12 aa signature specific to the CHC group. These data suggest that intrinsic molecular determinants in the circulating HCV quasispecies may provide a fitness advantage, contributing to the development of CHC.
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 ...
Cholestasis is a condition that results when excretion of bile acids from the liver is interrupted. Liver injury occurs in both humans and animals as a result of cholestasis, and recent studies have shown that inflammation is required for injury. The mechanism by which cholestasis increases production of proinflammatory mediators is not completely understood. One recent study showed that farnesoid X receptor (FXR), a bile acid nuclear receptor, upregulates proinflammatory mediators in response to bile acids in vitro. This suggests that FXR is important for inflammation during cholestasis. To test this hypothesis in vivo, wild-type and FXR knockout mice were subjected to bile duct ligation (BDL), a commonly used model of cholestasis. Three days later, levels of intercellular adhesion molecule-1 (ICAM-1) and macrophage inflammatory protein-2 (MIP-2), both important for the recruitment of neutrophils to the liver, were measured. ICAM-1 levels were increased to a similar extent in wild-type and FXR ...
Author(s): Herold, Jessica; Grimaldo, Felipe | Abstract: Infectious mononucleosis is primarily caused by Epstein-Barr virus (EBV) and is a common di-agnosis made in emergency departments worldwide. Subclinical and transient transaminase ele-vations are a well-established sequela of EBV. However, acute cholestatic hepatitis is a rare complication. EBV infection should be considered as part of the differential diagnosis in patients with an obstructive pattern on liver function tests without evidence of biliary obstruction demon-strated on advanced imaging.
Belgiums Federal Agency for Food Chain Safety (AFSCA) warns consumers to avoid the same curcumin-based supplement known to Italian authorities after the country also reports cases of acute cholestatic hepatitis.
The most common liver disease in pregnancy, intrahepatic cholestasis (ICP), reduces the release of digestive fluid bile from the liver causing bile acids to build up in the blood, impairing liver function. This causes severe itching in the mother and increases risks of stillbirth and preterm birth for the baby.. Previous studies suggest that children of women with ICP are more likely to develop childhood obesity.. For the findings, the research team investigated how gut microbiota are affected in the offspring of a mouse model of ICP.. The results reported that the offspring had a different gut microbiome composition and liver function, particularly when fed a high-fat diet, which could contribute to impaired metabolism and increase risk of obesity.. The results suggest that mice born to mothers with ICP, or other liver diseases, may benefit from maintaining a healthy diet and should avoid fatty foods.. These findings also suggest that targeting microbiome composition with treatment strategies ...
The liver diseases unique to pregnancy include hyperemesis gravidarum, acute fatty liver of pregnancy, intrahepatic cholestasis of pregnancy, and hemo..
Abdominal pain resulting from vasoocclusive crises is common and is typically abrupt in onset, and poorly localized. Tenderness and guarding may be present on examination. Rebound and rigidity are usually absent. If not typical of a pain crisis, non-SCA-related causes need to be considered (e.g., appendicitis), as well as SCA-related cholelithiasis/cholecystitis (can occur as early as 2 years of age), intrahepatic cholestasis (sudden right upper quadrant pain and tenderness, jaundice, anorexia, hepatomegaly, and sometimes fever), splenic sequestration, or hepatic sequestration (anemia and hepatomegaly). Constipation, urinary tract infection, and peptic ulcer disease are more common in SCA patients. Laboratories, ultrasound, and CT scan may be necessary to differentiate among these etiologies. ...
Introduction Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease common in postmenopausal women, characterised by the presence of serum antimitochondrial antibodies. Exposure to environmental xenobiotics and estrogenic drugs has been linked to an increased incidence of PBC and cholestasis respectively. We hypothesized that exposure to environmental xenoestrogens may be a risk factor for PBC. Methods A screening system by which compounds could be tested for transcriptional estrogenic activity was developed and validated. Compounds were screened for human estrogenic activity by dual luciferase reporter assay and by measurement of the induction of a known estrogen responsive gene, TFF1. The effects of exposure to xenoestrogens on mitochondrial function was assessed by MTS assay and by measurement of TMRM localization. In vivo effects of exposure to xenoestrogens was examined in a range of mouse models. Results A number of xenoestrogens were identified, notably the azo dyes sunset ...
Preface, vii. Acknowledgement, ix. I Antenatal Care, 2. Antenatal Health Assessment, 3. Anxiety and Depression, 6. Bio-physical Tests, 8. Bleeding in Pregnancy, 15. Fetal Growth and Development, 18. Gestational Diabetes Mellitus, 21. Infections in Pregnancy, 23. Intrahepatic Cholestasis of Pregnancy, 33. Minor Disorders of Pregnancy, 35. Pre-conceptual Health, 37. Pre-eclampsia, 39. Preparation for Parenthood, 42. References, 43. II Labour and Birth, 49. First Stage of Labour, 50. Promoting Normality, 58. Second Stage of Labour, 61. Third Stage of Labour, 67. Challenges, 70. Cord Prolapse, 70. Eclampsia, 71. Primary Postpartum Haemorrhage, 74. Shoulder Dystocia, 77. References, 78. III Postnatal Care, 85. Contraception and Sexual Health, 86. Facilitating Breastfeeding, 88. Postnatal Health Assessment, 91. Mental Illness After Childbirth, 94. References, 96. IV Hot Topics, 99. Breech Birth, 100. Domestic Abuse, 104. Obesity, 106. Recognising the Deteriorating Woman, 108. Sepsis, 110. References, ...
Rabbit Polyclonal to Tau (phospho-Thr534/217). the membranes, pregnancy induced hypertension and intrahepatic cholestasis of pregnancy variables (P?>?0.05). The most commonly identified comorbidity was sepsis (5.1%, n?=?13), followed by hypothermia (4.7%, n?=?12) and hypoglycemia (4%, n?=?10). Approximate 23% (n?=?60) of the neonates had atrial septal defects (ASD). Other frequently observed congenital heart defects included patent ductus arteriosus (PDA, 7.1%, n?=?18) and ventricular septal defects (VSD, 2.8%, n?=?7). These conditions were not found to differ significantly between the early-onset group and the late-onset group (P?>?0.05). Major complications occurring after NEC diagnosis included sepsis (27.7%, n?=?70), peritonitis (15.9%, n?=?40), respiratory failure (5.9%, n?=?15), shock CHIR-99021 (3.2%, n?=?8), kidney failure (2.8%, n?=?7), heart failing (0.8%, n?=?2), multiple body organ dysfunction symptoms (0.8%, n?=?2), pulmonary hemorrhage and disseminated intravascular coagulation ...
Rabbit Polyclonal to Tau (phospho-Thr534/217). the membranes, pregnancy induced hypertension and intrahepatic cholestasis of pregnancy variables (P?>?0.05). The most commonly identified comorbidity was sepsis (5.1%, n?=?13), followed by hypothermia (4.7%, n?=?12) and hypoglycemia (4%, n?=?10). Approximate 23% (n?=?60) of the neonates had atrial septal defects (ASD). Other frequently observed congenital heart defects included patent ductus arteriosus (PDA, 7.1%, n?=?18) and ventricular septal defects (VSD, 2.8%, n?=?7). These conditions were not found to differ significantly between the early-onset group and the late-onset group (P?>?0.05). Major complications occurring after NEC diagnosis included sepsis (27.7%, n?=?70), peritonitis (15.9%, n?=?40), respiratory failure (5.9%, n?=?15), shock CHIR-99021 (3.2%, n?=?8), kidney failure (2.8%, n?=?7), heart failing (0.8%, n?=?2), multiple body organ dysfunction symptoms (0.8%, n?=?2), pulmonary hemorrhage and disseminated intravascular coagulation ...
A study published in the British Journal of Obstetrics and Gynecology in April (116(5):626-36) of this year examined the rising induction rates for labor and birth. Researchers (from the Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Michigan, Ann Arbor, MI) searched MEDLINE and the Cochrane Library between 1980 and April 2008 using several terms and combinations, including induction of labour, premature rupture of membranes, post-term pregnancy, preterm prelabour rupture of membranes (PROM), multiple gestation, suspected macrosomia, diabetes, gestational diabetes mellitus, cardiac disease, fetal anomalies, systemic lupus erythematosis, oligohydramnios, alloimmunization, rhesus disease, intrahepatic cholestasis of pregnancy (IHCP), and intrauterine growth restriction (IUGR). ...
ONeill SM; Liu J; ORourke MF; Khoo SK, 2013, The menopausal transition does not appear to accelerate age-related increases in arterial stiffness, Climacteric, vol. 16, no. 1, pp. 62 - 69,. Vancaillie T; Eggermont J; Armstrong G; Jarvis S; Liu J; Beg N, 2012, Response to Pudendal Nerve Block in Women with Pudendal Neuralgia, Pain Medicine, vol. 13, no. 4, pp. 596 - 603,. Liu J, Eden JA. The menopause experience of Greek women in Sydney. Menopause 2008; 15(3):476-481.. Liu J, Eden J. Experience and Attitudes toward Menopause in Chinese Women Living in Sydney - A Cross Sectional Survey.Maturitas2007;58(4):359-365.. Lu J, Liu J, Eden J. The experience of menopausal symptoms by Arabic women in Sydney. Climacteric 2007;10: 72-9.. Hafiz I, Liu J, Eden J. A quantitative analysis of the menopause experience of Indian women living in Sydney. The Australian and New Zealand Journal of Obstetrics and Gynaecology 2007;47(4):329-334.. Liu, J, Gao, WW. Intrahepatic Cholestasis in pregnancy. Ningxia ...
Cholestatic liver diseases in childhood frequently result in growth retardation. The pathophysiology is usually multifactoral including reduced calorie intake, abnormal protein metabolism, fat and fat soluble vitamin malabsorption, increased energy expenditure, pancreatic insufficiency, accompanying infections and genetic disposition. All children with cholestatic liver diseases should undergo an assessment for their nutritional status and dietary intake and receive dietary counseling from a dietitian with monitoring of intake to ensure adequate energy and nutrient intake. After liver transplantation growth improves in the majority of children with good liver function. However some children, especially with genetic diseases such as Alagille syndrome, PFIC or CF do not grow normally. In selected cases therapy with growth hormone should be considered ...
Professor Gideon Hirschfield discusses the three autoimmune liver diseases, reviewing the current treatment options available and the latest clinical trials. Professor Hirschfield also considers how to target treatment more effectively to encompass quantity and quality of life. This lecture was filmed at the RSM in London for the event the liver day: new perspectives in clinical hepatology 2017 on Wednesday 8th November 2017. Length: 00:44:05 ...
View details of top cholestasis hospitals in Mumbai. Get guidance from medical experts to select best cholestasis hospital in Mumbai
Cholestasis, Intrahepatic: Idiopathic hepatitis. In: Hay, Jr WW, Levin MJ, Deterding RR, Abzug MJ. Hay, Jr W.W., Levin M.J., Deterding R.R., Abzug M.J. Eds. William W. Hay, Jr, et al.eds. Quick Medical Diagnosis & Treatment Pediatrics New York, NY: McGraw-Hill; . http://accesspediatrics.mhmedical.com/content.aspx?bookid=2196§ionid=166956345. Accessed October 22, 2017 ...
Itching (pruritus) is a common symptom associated with numerous skin diseases, as well as a secondary symptom of numerous serious conditions such as renal failure and liver disease. Itching, unlike other skin sensations, is generally a result of CNS activities and typically goes untreated by standard medical therapies.. The endocannabinoid system is acknowledged to play an important role in maintaining skin health[1] and cannabinoids have been regarded as "promising" agents for the treatment of itch.[2]. A review of the scientific literature identifies at least three clinical trials investigating the use of cannabinoids in the treatment of pruritus. Writing in the August 2002 issue of the American Journal of Gastroentrology, investigators from the University of Miami Department of Medicine reported successful treatment of pruritus with 5 mg of THC in three patients with cholestatic liver disease.[3] Prior to cannabinoid therapy, subjects had failed to respond to standard medications and had lost ...
GD/Cholestasis: So being my third pregnancy i was surprised to get GD but it wasnt as bad as i had thought and since my last two pregnancies ended in emergency c-sections this one was going to be a scheduled c-section and i was scheduled for July 7 and original due date was July 16th... But last week i was having extreme itchiness on feet and arms so i got sent to get blood work. ...
Has anyone experienced intraheptic Cholestasis before ? I have an extreme itch that I cannot get to go away . No amount of itching or lotion or anything is helping . Ive been reading stories on the internet (I know , bad idea) about this and they all result in still birth . I have an appointment tomorrow but has anyone who has experienced this tell me differences between this and a regular itch ? I feel like crying because I cant stop itching
Semantic Scholar extracted view of Is fatigue associated with cholestasis mediated by altered central neurotransmission? by E. A. Jones et al.
The Childhood Liver Disease Research Network (ChiLDReN) is a collaborative team of doctors, scientists, nurses, research coordinators, medical facilities, patient support organizations and the National Institutes of Health. The ChiLDReN Network has clinical sites and research laboratories in the U.S., Canada, and the United Kingdom. These sites are working together to improve the lives of children and families dealing with rare cholestatic liver diseases. ...
Intrahepatic cholestasis of pregnancy[edit]. UDCA has been used for intrahepatic cholestasis of pregnancy. UDCA lessens itching ... "Ursodeoxycholic acid versus placebo in women with intrahepatic cholestasis of pregnancy (PITCHES): a randomised controlled ... Cholestasis[edit]. UDCA use is not licensed in children, as its safety and effectiveness have not been established. Evidence is ... accumulating that ursodeoxycholic acid is ineffective and unsafe in neonatal hepatitis and neonatal cholestasis.[15][16][17] ...
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. PMID ... 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 (Jan 8, 2009). "Progressive familial intrahepatic cholestasis". Orphanet ... Progressive familial intrahepatic cholestasis (associated with HCC) and Trisomy 18 (associated with hepatoblastoma). Many ... High levels of alpha-fetoprotein (AFP) in the blood can be found in many cases of HCC and intrahepatic cholangiocarcinoma. ... "SEER Stat Fact Sheets: Liver and Intrahepatic Bile Duct Cancer". NCI. Archived from the original on 2017-07-28. Retrieved 2016 ...
... is a gene associated with progressive familial intrahepatic cholestasis type 2 (PFIC2). PFIC2 caused by mutations in the ... Thompson R, Strautnieks S (Nov 2001). "BSEP: function and role in progressive familial intrahepatic cholestasis". Seminars in ... "Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11". Gastroenterology. 127 (2): 379-84. doi: ... "Impaired expression and function of the bile salt export pump due to three novel ABCB11 mutations in intrahepatic cholestasis ...
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). Lymphedema-distichiasis syndrome Lymphedema praecox Blueberry muffin ... Apr 2003). "Evidence for genetic heterogeneity in lymphedema-cholestasis syndrome". Journal of Pediatrics. 142 (4): 441-447. ... "Mapping of the locus for cholestasis-lymphedema syndrome (Aagenaes syndrome) to a 6.6-cM interval on chromosome 15q". Am J Hum ...
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 ...
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". ...
Bilirubin levels greater than 10x normal could indicate neoplastic or intrahepatic cholestasis. Levels lower than this tend to ... However, although pale stools and dark urine are a feature of biliary obstruction, they can occur in many intra-hepatic ... Low levels of albumin tend to indicate a chronic condition, while it is normal in hepatitis and cholestasis.[citation needed] ... In hepatic jaundice, there is invariably cholestasis. Defects in bilirubin metabolism also leads to jaundice, as in Gilbert's ...
... is associated with progressive familial intrahepatic cholestasis type 3. The membrane-associated protein encoded by this ... 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 ...
... may refer to: Progressive familial intrahepatic cholestasis, a disease. Passive foreign investment company, a ...
Progressive familial intrahepatic cholestasis synd/729 at Who Named It? Alagille D, Odièvre M, Gautier M, Dommergues JP ( ... Because notch signaling has been found to regulate formation of three-dimensional intrahepatic biliary architecture in murine ...
Intrahepatic cholestasis of pregnancy List of cutaneous conditions Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. ( ... 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. ...
Progressive intrahepatic cholestasis Treatment Schedule: 3 to 5 eight-hour treatment sessions in consecutives days Continuous ... Progressive intrahepatic cholestasis Treatment Schedule: 3 to 5 eight-hour treatment sessions in consecutives 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' ...
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. PMID ...
"Therapeutic interventions in progressive familial intrahepatic cholestasis: experience from a tertiary care centre in north ...
... is associated with type II citrullinemia and neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). The ...
... 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 of pregnancy List of cutaneous conditions Matz H, Orion E, Wolf R (Mar-Apr 2006). "Pruritic urticarial ...
ALP levels in plasma rise with large bile duct obstruction, intrahepatic cholestasis, or infiltrative diseases of the liver. ... Other tests commonly requested alongside LFTs include 5' Nucleotidase (5'NTD) is another test specific for cholestasis or ... AST/ALT elevations instead of ALP elevations favor liver cell necrosis as a mechanism over cholestasis. When AST and ALT are ... clay-colored stool is an indicator for a blockage in bilirubin processing and thus potential liver dysfunction or cholestasis. ...
Septal fibrosis and pseudolobules, inflammatory infiltrates, signs of cholestasis, and reduced numbers of intrahepatic bile ...
... may refer to: a gene encoding the progressive familial intrahepatic cholestasis Chyetverikov MDR-3, a long range Russian ...
... intrahepatic cholestasis), hypolipidemic drugs, or changes following gallbladder removal (cholecystectomy). Conditions ...
Researchers have found many infants with neonatal intrahepatic cholestasis have the same mutations in the SLC25A13 gene as ... Type II citrullinemia may also develop in people who had a liver disorder called neonatal cholestasis during infancy. This ...
Intrahepatic cholestasis of pregnancy. *Linea nigra. *Prurigo gestationis. *Pruritic folliculitis of pregnancy ...
... is a rare genetic disease. Learn more about causes, symptoms and treatment ... Progressive Familial Intrahepatic Cholestasis (PFIC) Progressive familial intrahepatic cholestasis (PFIC) is a rare inherited ... Medicines to Treat Symptoms of Progressive Familial Intrahepatic Cholestasis. In most cases of PFIC, the biggest issue is ... In PFIC children are not able to drain bile from the liver even though the large bile ducts are open (cholestasis). This gets ...
Progressive familial intrahepatic cholestasis type 2 - paediatric patients followed at the Paediatric Clinic of the 2nd Medical ... Progressive familial intrahepatic cholestasis type 2 is an autosomal recessive cholestatic liver disease caused by a deficiency ... Progressive familial intrahepatic cholestasis type 2 - paediatric patients followed at the Paediatric Clinic of the 2nd Medical ... Progres-sive familial intrahepatic cholestasis. Orphanet J Rare Dis 2009; 4: 1. doi: 10.1186/ 1750-1172-4-1. ...
Progressive familial intrahepatic cholestasis (PFIC) is a rare genetic liver disease that affects infants and children. In many ... Progressive familial intrahepatic cholestasis (PFIC) is a rare genetic liver disease that affects infants and children. In many ... Davit-Spraul, Gonzales E, Baussan C, Jacquemin E. Progressive familial intrahepatic cholestasis. Orphanet J Rare Dis. 2009;4:1. ... Liver transplantation and the management of progressive familial intrahepatic cholestasis in children. World J Transplant. 2016 ...
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 ( ... The Progressive Familial Intrahepatic Cholestasis (PFIC) market report covers Progressive Familial Intrahepatic Cholestasis ( ...
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 ... Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC2) is a rare congenital cholestatic liver disease that progresses to ...
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 ... In the follow-up biopsies, cholestasis completely disappeared in 3 patients and decreased significantly in 1 other patient. ...
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) ... Pusl T, Beuers U (2007). "Intrahepatic cholestasis of pregnancy". Orphanet J Rare Dis. 2: 26. doi:10.1186/1750-1172-2-26. PMC ...
How is low gamma-GT intrahepatic cholestasis diagnosed?. *What are the effects of severe low gamma-GT intrahepatic cholestasis? ... What is low gamma-GT intrahepatic cholestasis?. ANSWER Its a rare liver disease that usually shows up in children. It happens ... Childhood Liver Disease Research Network: "What is Progressive Familial Intrahepatic Cholestasis (PFIC)?" ... Childhood Liver Disease Research Network: "What is Progressive Familial Intrahepatic Cholestasis (PFIC)?" ...
... and when you need to seek medical attention fast for possible intrahepatic cholestasis of pregnancy (ICP), also called ... Intrahepatic cholestasis of pregnancy. Intrahepatic cholestasis of pregnancy (ICP) is a potentially serious liver disorder that ... Itching and intrahepatic cholestasis of pregnancy. Itching is common in pregnancy. Usually its thought to be caused by raised ... However, itching can be a symptom of a liver condition called intrahepatic cholestasis of pregnancy (ICP), also known as ...
Patient pamphlet GeneReview/NIH/UW entry on Low γ-GT Familial Intrahepatic Cholestasis OMIM entry on CHOLESTASIS, PROGRESSIVE ... Alagille syndrome Intrahepatic cholestasis of pregnancy Liver transplantation Shneider BL (2004). "Progressive intrahepatic ... 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 ...
... Ayse Sulu,1 Osman Baspinar,1 Selim Kervancıoglu,2 and Samil ... In addition, her liver biopsy confirmed the diagnosis of progressive familial intrahepatic cholestasis (PFIC) type 3. Although ... Its comorbidity with cholestasis has not previously been reported in the literature. An 11-month-old baby female, who was an ...
Intrahepatic cholestasis (ICP, also known as obstetric cholestasis) is a condition of pregnancy that commonly manifests as ... Other symptoms of Intrahepatic cholestasis of pregnancy (ICP). In addition to itching and jaundice, other symptoms include:. * ... Jaundice and Intrahepatic cholestasis of pregnancy (ICP). The increased build-up of bilirubin leads to jaundice, dark colored ... Long-term risks of Intrahepatic cholestasis of pregnancy (ICP). Risks of long term liver problems after birth of the baby is ...
ICP or obstetric cholestasis) may be mild and harmless but in severe cases may cause damage to the fetus. This is the reason ... Intrahepatic cholestasis of pregnancy (ICP or obstetric cholestasis) may be mild and harmless but in severe cases may cause ... Intrahepatic cholestasis of pregnancy (ICP) and alcohol consumption. Obstetric cholestasis is usually not worsened by alcohol ... Oral contraceptive pills and intrahepatic cholestasis of pregnancy (ICP). Women with obstetric cholestasis cannot take oral ...
Intrahepatic cholestasis is a condition caused by slow or blocked release of bile from the liver. It can lead to liver disease ... Pediatric Intrahepatic Cholestasis Liver Diseases. Intrahepatic cholestasis is a problem that affects the release of bile from ... Intrahepatic cholestasis is caused by genetic defects that may lead to:. *Progressive familial intrahepatic cholestasis - an ... What are the signs and symptoms of Pediatric Intrahepatic Cholestasis Liver Diseases?. Babies with intrahepatic cholestasis ...
... and some glycoproteic substances in patients during the course of extrahepatic cholestasis... ... The pathophysiological bilirubin pattern was similar in patients with intrahepatic cholestasis. At stage A, in a number of ... Five patients with liver cirrhosis and a picture of intrahepatic cholestasis following anesthesia were also investigated. Serum ... Simon FR, Arias IM (1973) Alteration of bile canalicular enzymes in cholestasis. J Clin Invest 52:765-775Google Scholar ...
What is progressive familial intrahepatic cholestasis?. Progressive familial intrahepatic cholestasis (PFIC) is disorder that ...
A successful transplant can greatly ease the symptoms and complications of low gamma-GT intrahepatic cholestasis. However, ... 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)?" ...
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 ... What is progressive familial intrahepatic cholestasis?. Progressive Familial Intrahepatic Cholestasis (PFIC) is a rare genetic ... 0 progressive familial intrahepatic cholestasis patients report mild anxious mood (0%). * 1 a progressive familial intrahepatic ...
... Progressive familial intrahepatic cholestasisClassification & external resources ... Progressive familial intrahepatic cholestasis. Classification & external resources OMIM 211600 601847 602347 eMedicine ped/2771 ... Progressive familial intrahepatic cholestasis (PFIC) refers to a group of familial cholestatic conditions caused by defects in ... It uses material from the Wikipedia article "Progressive_familial_intrahepatic_cholestasis". A list of authors is available in ...
... Maria Maldonado, Ali Alhousseini ... Maria Maldonado, Ali Alhousseini, Michael Awadalla, et al., "Intrahepatic Cholestasis of Pregnancy Leading to Severe Vitamin K ...
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 ... A retrospective case-control study of 21,008 women in Finland has found that those with intrahepatic cholestasis of pregnancy ( ... Intrahepatic Cholestasis of Pregnancy, Liver, Obstetrics, Pancreatitis, Placenta, Pregnancy, Skin, Steroid ...
Any gentle ways that I can put myselfInto labor?? No castor oil or anything I have to ingest. OB said sex but that if my body wasnt ready it wouldnt really do anything. I dont want to put a lot of stress on the baby, but Im hoping to put myself into labor so that I dont need and medication...
... a disease characterized by a mild cholestasis of short duration appearing in otherwise healthy young women. An abnormal fecal ... A prospective study was undertaken to evaluate fat malabsorption during intrahepatic cholestasis of pregnancy (ICP), ... Cholestasis, Intrahepatic / metabolism*. Fats / analysis. Feces / analysis. Female. Follow-Up Studies. Humans. Infant, Newborn ... A prospective study was undertaken to evaluate fat malabsorption during intrahepatic cholestasis of pregnancy (ICP), a disease ...
Intrahepatic Cholestasis of Pregnancy (ICP), (formerly known as Obstetric Cholestasis or OC), is a liver disorder that occurs ...
... is a rare disorder characterized by recurrent episodes of cholestasis without permanent liver damage. Familial and sporadic ... Benign recurrent intrahepatic cholestasis (BRIC) is a rare disorder characterized by recurrent episodes of cholestasis without ... Cholestasis, Intrahepatic / genetics*. Consanguinity. Female. Genes, Recessive*. Heterozygote Detection. Humans. Infant. Male. ...
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