Congenital Hepatic Fibrosis

National Organization for Rare Disorders, Inc.

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It is possible that the main title of the report Congenital Hepatic Fibrosis is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Congenital hepatic fibrosis (CHF) is a rare disease that is present at birth (congenital) and affects the liver. CHF rarely occurs as an isolated problem, and is usually associated with ciliopathies that affect the kidneys, called hepatorenal fibrocystic diseases (FCD). These include polycystic kidney disease (PKD), nephronophthisis (NPHP) chronic tubulointerstitial disease, and others. Typical liver abnormalities include 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). Gastrointestinal (stomach and intestine) bleeding, splenomegaly (enlarged spleen) and hypersplenism (decreased platelet and other blood counts due to enlarged spleen) may be early signs of this condition.

Supporting Organizations

ARPKD/CHF Alliance

PO Box 70
Kirkwood, PA 17536
Tel: (717)529-5555
Fax: (800)807-9110
Tel: (800)708-8892

American Kidney Fund, Inc.

11921 Rockville Pike
Suite 300
Rockville, MD 20852
Tel: (800)638-8299

American Liver Foundation

39 Broadway, Suite 2700
New York, NY 10006
Fax: (212)483-8179
Tel: (800)465-4837

Children's Liver Disease Foundation

36 Great Charles Street
Birmingham, B3 3JY
United Kingdom
Tel: 1212123839
Fax: 1212124300

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311

NIH/National Kidney and Urologic Diseases Information Clearinghouse

3 Information Way
Bethesda, MD 20892-3580
Fax: (703)738-4929
Tel: (800)891-5390

National Kidney Foundation

30 East 33rd Street
New York, NY 10016
Tel: (212)889-2210
Fax: (212)689-9261
Tel: (800)622-9010

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

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.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

Last Updated:  8/31/2015
Copyright  2015 National Organization for Rare Disorders, Inc.