Hepatic Fibrosis, Congenital
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Hepatic Fibrosis, Congenital is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys. The patient is born with this disorder (congenital), and it is inherited as an autosomal recessive trait. The typical liver abnormalities are an enlarged liver (hepatomegaly), increased pressure in the venous system that carries blood from different organs to the liver (portal hypertension), and fiber-like connective tissue that spreads over and through the liver (hepatic fibrosis), often referred to as hepatic lesions. Gastrointestinal (stomach and intestines) bleeding is frequently an early sign of this condition. Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an autosomal recessive polycystic kidney disease (ARPKD). Impaired renal function associated with CHF in adults is caused by an autosomal dominant polycystic kidney disease (ADPKD).
The relationship of ARPKD to CHF is the subject of substantial controversy. Some clinicians suggest that the two conditions represent one disorder with a range of clinical/pathological presentations.
American Kidney Fund, Inc.
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American Liver Foundation
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National Kidney Foundation
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It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
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Last Updated: 5/18/2008
Copyright 1992, 2003 National Organization for Rare Disorders, Inc.