Congenital hepatic fibrosis with extra-hepatic porto-systemic shunt and hepatopulmonary syndrome successfully managed with living related liver transplantation
نویسندگان
چکیده
منابع مشابه
Two-Stage Liver Transplantation with Temporary Porto-Middle Hepatic Vein Shunt
Two-stage liver transplantation (LT) has been reported for cases of fulminant liver failure that can lead to toxic hepatic syndrome, or massive hemorrhages resulting in uncontrollable bleeding. Technically, the first stage of the procedure consists of a total hepatectomy with preservation of the recipient's inferior vena cava (IVC), followed by the creation of a temporary end-to-side porto-cava...
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 کاملUnexplained cyanosis revealing hepatopulmonary syndrome in a child with asymptomatic congenital hepatic fibrosis: a case report
INTRODUCTION Hepatopulmonary syndrome is a rare disease that affects patients of any age with acute or chronic liver disease. Liver transplantation is the only therapeutic option of proved benefit, and can result in substantial improvement or total improvement in postoperative gas exchange abnormalities. CASE PRESENTATION We report the case of a cyanotic 13-year-old Pakistani boy whose chest ...
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 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...
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ژورنال
عنوان ژورنال: Tropical Gastroenterology
سال: 2014
ISSN: 0250-636X
DOI: 10.7869/tg.192