[Congenital hepatic fibrosis].
نویسندگان
چکیده
منابع مشابه
Congenital 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 کاملمعرفی یک مورد فیبروز مادرزادی کبد
Congenital hepatic fibrosis (CHF) is a rare disease that primarily involves hepatobiliary and renal systems. It is characterized by hepatic fibrosis, portal hypertension and renal cystic disease. We present a 22 years old man with fever, abdominal pain, icterus and hematemesis. On complete work up of the patient and liver with kidney biopsy, the diagnosis was congenital hepatic fibrosis.
متن کاملClinical and Paraclinical Findings in Children With Congenital Hepatic Fibrosis: A Single Center 10-Year Study
Background: Congenital hepatic fibrosis (CHF) is an autosomal hereditary disorder affecting the porto-biliary system. It is a rare hereditary disorder often presenting in childhood or adolescence with hepatomegaly, splenomegaly, and gastrointestinal bleeding. A timely diagnosis of organomegalies by sonography can prevent esophageal varices. Liver transplantation is now the only...
متن کاملCongenital Hepatic Fibrosis and Need for Liver Transplantation
Herein, we describe two patients who underwent liver transplantation with the clinical diagnosis of hepatic failure and cryptogenic cirrhosis; histopathology of the explanted hepatectomy specimen revealed congenital hepatic fibrosis. To the best of our knowledge, coexistence of hepatic failure and cirrhosis in congenital hepatic fibrosis, have not yet been reported in the English literature.
متن کاملIsolated congenital hepatic fibrosis associated with TMEM67 mutations: report of a new genotype–phenotype relationship
We report an otherwise healthy 32-year-old man with portal hypertension, variceal bleeding, and congenital hepatic fibrosis with ductal plate malformation. Genetic screening identified two TMEM67 mutations. Biallelic TMEM67 mutations are known to cause Joubert/Meckel syndrome or nephronopthisis with hepatic fibrosis, but have never been found in isolated hepatic fibrosis.
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ورودعنوان ژورنال:
- Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke
دوره 92 15 شماره
صفحات -
تاریخ انتشار 1970