Hepatic Fibrosis, Congenital / Disease

Diseases & Conditions

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Hepatic Fibrosis, Congenital

Synonyms of Hepatic Fibrosis, Congenital

Disorder Subdivisions

General Discussion
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.
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Organizations related to Hepatic Fibrosis, Congenital

ARPKD/CHF Alliance
PO Box 70
Kirkwood PA 17536
Phone #: 717-529-5555
800 #: 800-708-8892
e-mail: [email protected]
Home page: http://www.arpkdchf.org
American Kidney Fund, Inc.
6110 Executive Boulevard
Rockville MD 20852
Phone #: 301-881-3052
800 #: 800-638-8299
e-mail: [email protected]
Home page: http://www.kidneyfund.org
American Liver Foundation
75 Maiden Lane
New York NY 10038
Phone #: 212-668-1000
800 #: 800-465-4837
e-mail: [email protected]
Home page: http://www.liverfoundation.org
International Patient Advocacy Association
800 Bellevue Way
Bellevue WA 98004
Phone #: 426-646-3768
800 #: --
e-mail: N/A
Home page: N/A
Madisons Foundation
PO Box 241956
Los Angeles CA 90024
Phone #: 310-264-0826
800 #: N/A
e-mail: [email protected]
Home page: http://www.madisonsfoundation.org
NIH/National Kidney and Urologic Diseases Information Clearinghouse
3 Information Way
Bethesda MD 20892-3580
Phone #: N/A
800 #: 800-891-5390
e-mail: [email protected]
Home page: http://kidney.niddk.nih.gov/
National Kidney Foundation
30 East 33rd Street
New York NY 10016
Phone #: 212-889-2210
800 #: 800-622-9010
e-mail: [email protected]
Home page: http://www.kidney.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html