Macrophage recruitment by fibrocystin-defective biliary epithelial cells promotes portal fibrosis in congenital hepatic fibrosis
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چکیده
منابع مشابه
Congenital hepatic fibrosis in an aborted calf
An aborted female Holstein foetus with marked generalized anasarca was referred to the Excellence Centre for Ruminant Abortion and Neonatal Mortality, Ferdowsi University of Mashhad. On postmortem examination, red-tinged ascites, pale and firm liver with extreme irregularity and numerous round to oval slightly raised foci on the capsular surface were seen. Histological examination revealed wide...
متن کاملCongenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure
Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys. Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an...
متن کاملcongenital hepatic fibrosis in an aborted calf
an aborted female holstein foetus with marked generalized anasarca was referred to the excellence centre for ruminant abortion and neonatal mortality, ferdowsi university of mashhad. on postmortem examination, red-tinged ascites, pale and firm liver with extreme irregularity and numerous round to oval slightly raised foci on the capsular surface were seen. histological examination revealed wide...
متن کاملCongenital hepatic fibrosis.
Congenital Hepatic fibrosis is an uncommon disease, which is autosomal recessive. Two forms of the disease are distinguished: a rare one becoming manifest in the neonatal period with signs of progressive renal failure secondary to polycystic kidneys, in such cases the liver fibrosis is usually asymptomatic, and the diagnosis is therefore often first established post mortem. In the other more us...
متن کاملCongenital Hepatic Fibrosis
An 8-year-old boy was admitted with slowly progressive distension of abdomen and fullness in upper abdomen of 9 months duration, and history of minor episodes of epistaxis for 7 months. There was no history of pain abdomen, jaundice, hematemesis, malena or any skin bleeds or hyperpigmentation. No umbilical catheterisation was done in the neonatal period. On tracing the pedigree no other family ...
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ژورنال
عنوان ژورنال: Hepatology
سال: 2016
ISSN: 0270-9139
DOI: 10.1002/hep.28382