Diseases & Conditions


A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

Hepatic Fibrosis, Congenital


Synonyms of Hepatic Fibrosis, Congenital
  • CHF

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: info@arpkdchf.org
      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: helpline@kideyfund.org
      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: info@liverfoundation.org
      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: getinfo@madisonsfoundation.org
      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: nkudic@info.niddk.nih.gov
      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: info@kidney.org
      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