An Unusual Case of Congenital Hepatic Fibrosis with Retinitis Pigmentosa
نویسندگان
چکیده
منابع مشابه
A Family of Congenital Hepatic Fibrosis and Atypical Retinitis Pigmentosa
Congenital hepatic fibrosis is a rare cause of portal hypertension and esophageal varices in children. We report cases of siblings with biopsy proven congenital hepatic fibrosis and with atypical retinitis pigmentosa. They presented with repeated episodes of jaundice along with progressive decrease of vision in night. They had hepatosplenomegaly and portal hypertension with esophageal varices. ...
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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...
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ژورنال
عنوان ژورنال: Cureus
سال: 2019
ISSN: 2168-8184
DOI: 10.7759/cureus.5788