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Overview of Progressive Familial Intrahepatic Cholestasis.
Clinical Liver Disease, 2022Bile acid transport is a complex physiologic process, of which disruption at any step can lead to progressive intrahepatic cholestasis (PFIC). The first described PFIC disorders were originally named as such before identification of a genetic cause ...
S. Hassan, P. Hertel
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Best Practice & Research Clinical Gastroenterology, 2010
Progressive familial intrahepatic cholestasis (PFIC) type 1, 2 and 3 are due to mutations in ATP8B1, ABCB11 and ABCB4, respectively. Each of these genes encodes a hepatocanalicular transporter, which is essential for the proper formation of bile. Mutations in ABCB4 can result in progressive cholestatic disease, while mutations in ATP8B1 and ABCB11 can ...
van der Woerd, Wendy L. +5 more
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Progressive familial intrahepatic cholestasis (PFIC) type 1, 2 and 3 are due to mutations in ATP8B1, ABCB11 and ABCB4, respectively. Each of these genes encodes a hepatocanalicular transporter, which is essential for the proper formation of bile. Mutations in ABCB4 can result in progressive cholestatic disease, while mutations in ATP8B1 and ABCB11 can ...
van der Woerd, Wendy L. +5 more
openaire +3 more sources
Revista espanola de las enfermedades del aparato digestivo, 1976
Intrahepatic cholestasis has become a very important aspect of liver disease. The author, in the light of electron microscopy and the theory of filtration-reabsorption of bile secretion proposes that the disease may be explained by obstructions situated at various levels of the inter-cellular spaces of the liver. This obstruction is functional at first,
L, Yuguero del Moral +4 more
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Intrahepatic cholestasis has become a very important aspect of liver disease. The author, in the light of electron microscopy and the theory of filtration-reabsorption of bile secretion proposes that the disease may be explained by obstructions situated at various levels of the inter-cellular spaces of the liver. This obstruction is functional at first,
L, Yuguero del Moral +4 more
+6 more sources
BENIGN RECURRENT INTRAHEPATIC CHOLESTASIS
Clinics in Liver Disease, 1999Benign recurrent intrahepatic cholestasis is a rare autosomal recessive disorder characterized by repeated episodes of intense pruritus and jaundice. Each attack lasts from several weeks to months before resolving spontaneously. Patients are completely asymptomatic for months to years between symptomatic periods.
Velimir A, Luketic, Mitchell L, Shiffman
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Guideline No. 452: Diagnosis and Management of Intrahepatic Cholestasis of Pregnancy.
Journal of Obstetrics and Gynaecology CanadaOBJECTIVE To summarize the current evidence and to make recommendations for the diagnosis and management of intrahepatic cholestasis of pregnancy. TARGET POPULATION Pregnant people with intrahepatic cholestasis of pregnancy.
Sebastian R Hobson +6 more
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Hypertension in Pregnancy, 2020
Objective To investigate the association between intrahepatic cholestasis of pregnancy (ICP) and the risk of gestational diabetes mellitus (GDM) and preeclampsia via meta-analysis.
A. Arafa, Jiayi Dong
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Objective To investigate the association between intrahepatic cholestasis of pregnancy (ICP) and the risk of gestational diabetes mellitus (GDM) and preeclampsia via meta-analysis.
A. Arafa, Jiayi Dong
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PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS
Clinics in Liver Disease, 1999Abstract Progressive familial intrahepatic cholestasis (PFIC), also known as Byler disease, is an inherited disorder of childhood in which cholestasis of hepatocellular origin often presents in the neonatal period and leads to death from liver failure before adolescence. The pattern of appearance of affected children within families is consistent with
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La Clinica terapeutica, 1997
The intrahepatic cholestasis is not an common syndrome, in particular way in people between 50 and 60 years of age. It is often unknown or confused, because of itching, with allergic or dermatologic diseases. The most frequent causes of intrahepatic cholestasis are primary sclerosing cholestasis, primary biliary cirrhosis and hepatic cirrhosis.
F, Stazi, P, Farello, C, Stazi
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The intrahepatic cholestasis is not an common syndrome, in particular way in people between 50 and 60 years of age. It is often unknown or confused, because of itching, with allergic or dermatologic diseases. The most frequent causes of intrahepatic cholestasis are primary sclerosing cholestasis, primary biliary cirrhosis and hepatic cirrhosis.
F, Stazi, P, Farello, C, Stazi
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Intrahepatic Cholestasis of Pregnancy
Clinical Obstetrics & Gynecology, 2020Intrahepatic cholestasis of pregnancy is a common disorder of pregnancy manifested by pruritus and elevated bile acids. The etiology of cholestasis is poorly understood and management is difficult due to the paucity of data regarding its diagnosis, treatment, and related adverse outcomes.
Devin D, Smith, Kara M, Rood
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Liver international (Print), 2020
In about 20% of children with cholestasis and normal or low serum gamma‐glutamyltransferase (GGT) activity, no aetiology is identified. We sought new genes implicated in paediatric hepatobiliary disease.
Jing Zhang +11 more
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In about 20% of children with cholestasis and normal or low serum gamma‐glutamyltransferase (GGT) activity, no aetiology is identified. We sought new genes implicated in paediatric hepatobiliary disease.
Jing Zhang +11 more
semanticscholar +1 more source