Congenital hepatic fibrosis

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Overview

WikiDoc Resources for Congenital hepatic fibrosis
Articles
Most recent articles on Congenital hepatic fibrosis Most cited articles on Congenital hepatic fibrosis Review articles on Congenital hepatic fibrosis Articles on Congenital hepatic fibrosis in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Congenital hepatic fibrosis Images of Congenital hepatic fibrosis Photos of Congenital hepatic fibrosis Podcasts & MP3s on Congenital hepatic fibrosis Videos on Congenital hepatic fibrosis
Evidence Based Medicine
Cochrane Collaboration on Congenital hepatic fibrosis Bandolier on Congenital hepatic fibrosis TRIP on Congenital hepatic fibrosis
Clinical Trials
Ongoing Trials on Congenital hepatic fibrosis at Clinical Trials.gov Trial results on Congenital hepatic fibrosis Clinical Trials on Congenital hepatic fibrosis at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Congenital hepatic fibrosis NICE Guidance on Congenital hepatic fibrosis NHS PRODIGY Guidance FDA on Congenital hepatic fibrosis CDC on Congenital hepatic fibrosis
Books
Books on Congenital hepatic fibrosis
News
Congenital hepatic fibrosis in the news Be alerted to news on Congenital hepatic fibrosis News trends on Congenital hepatic fibrosis
Commentary
Blogs on Congenital hepatic fibrosis
Definitions
Definitions of Congenital hepatic fibrosis
Patient Resources / Community
Patient resources on Congenital hepatic fibrosis Discussion groups on Congenital hepatic fibrosis Patient Handouts on Congenital hepatic fibrosis Directions to Hospitals Treating Congenital hepatic fibrosis Risk calculators and risk factors for Congenital hepatic fibrosis
Healthcare Provider Resources
Symptoms of Congenital hepatic fibrosis Causes & Risk Factors for Congenital hepatic fibrosis Diagnostic studies for Congenital hepatic fibrosis Treatment of Congenital hepatic fibrosis
Continuing Medical Education (CME)
CME Programs on Congenital hepatic fibrosis
International
Congenital hepatic fibrosis en Espanol Congenital hepatic fibrosis en Francais
Businness
Congenital hepatic fibrosis in the Marketplace Patents on Congenital hepatic fibrosis
Experimental / Informatics
List of terms related to Congenital hepatic fibrosis

Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis that do not alter hepatic lobular architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension. The condition usually has an autosomal recessive pattern of transmission but sporadic cases have also been reported. It may be associated with other congenital defects; commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation^[1].

Embryogenically CHF is due to malformation of duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive hepatocytes, which differentiate into cholangiocytes^[1]. It usually present in adolescent or young adulthood, but onset of sign and symptom can range from early childhood to 5th or 6th decade of life. Clinical features may vary but commonly include Cholangitis, hepatomegaly and signs of portal hypertension.

See also

• Other fibrocystic liver diseases:
  • Caroli disease
  • Polycystic kidney disease
  • Von Meyenburg complex
  • Biliary hamartomas

References

v • d • e WikiDoc Research Resources for Congenital hepatic fibrosis (Click show to right to view)
Articles on Congenital hepatic fibrosis	Most recent articles on Congenital hepatic fibrosis • Most cited articles on Congenital hepatic fibrosis • Review articles on Congenital hepatic fibrosis • Articles on Congenital hepatic fibrosis in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Congenital hepatic fibrosis	Powerpoint slides on Congenital hepatic fibrosis • Images of Congenital hepatic fibrosis • Photos of Congenital hepatic fibrosis • Podcasts & MP3s on Congenital hepatic fibrosis • Videos on Congenital hepatic fibrosis
Evidence Based Medicine Regarding Congenital hepatic fibrosis	Cochrane Collaboration on Congenital hepatic fibrosis • Bandolier on Congenital hepatic fibrosis • TRIP on Congenital hepatic fibrosis
Cost Effectiveness of Congenital hepatic fibrosis	Cost Effectiveness of Congenital hepatic fibrosis
Clinical Trials Involving Congenital hepatic fibrosis	Ongoing Trials on Congenital hepatic fibrosis at Clinical Trials.gov • Trial results on Congenital hepatic fibrosis • Clinical Trials on Congenital hepatic fibrosis at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Congenital hepatic fibrosis	US National Guidelines Clearinghouse on Congenital hepatic fibrosis • NICE Guidance on Congenital hepatic fibrosis • NHS PRODIGY Guidance • FDA on Congenital hepatic fibrosis • CDC on Congenital hepatic fibrosis
Textbook Information on Congenital hepatic fibrosis	Books and Textbook Information on Congenital hepatic fibrosis
Pharmacology Resources on Congenital hepatic fibrosis	Dosing of Congenital hepatic fibrosis • Drug interactions with Congenital hepatic fibrosis • Side effects of Congenital hepatic fibrosis • Allergic reactions to Congenital hepatic fibrosis • Overdose information on Congenital hepatic fibrosis • Carcinogenicity information on Congenital hepatic fibrosis • Congenital hepatic fibrosis in pregnancy • Pharmacokinetics of Congenital hepatic fibrosis •
Genetics, Pharmacogenomics, and Proteinomics of Congenital hepatic fibrosis	Genetics of Congenital hepatic fibrosis • Pharmacogenomics of Congenital hepatic fibrosis • Proteomics of Congenital hepatic fibrosis
Newstories on Congenital hepatic fibrosis	Congenital hepatic fibrosis in the news • Be alerted to news on Congenital hepatic fibrosis • News trends on Congenital hepatic fibrosis
Commentary on Congenital hepatic fibrosis	Blogs on Congenital hepatic fibrosis
Patient Resources on Congenital hepatic fibrosis	Patient resources on Congenital hepatic fibrosis • Discussion groups on Congenital hepatic fibrosis • Patient Handouts on Congenital hepatic fibrosis • Directions to Hospitals Treating Congenital hepatic fibrosis • Risk calculators and risk factors for Congenital hepatic fibrosis
Healthcare Provider Resources on Congenital hepatic fibrosis	Symptoms of Congenital hepatic fibrosis • Causes & Risk Factors for Congenital hepatic fibrosis • Diagnostic studies for Congenital hepatic fibrosis • Treatment of Congenital hepatic fibrosis
Continuing Medical Education (CME) Programs on Congenital hepatic fibrosis	CME Programs on Congenital hepatic fibrosis
International Resources on Congenital hepatic fibrosis	Congenital hepatic fibrosis en Espanol • Congenital hepatic fibrosis en Francais
Business Resources on Congenital hepatic fibrosis	Congenital hepatic fibrosis in the Marketplace • Patents on Congenital hepatic fibrosis
Informatics Resources on Congenital hepatic fibrosis	List of terms related to Congenital hepatic fibrosis

v • d • e Health science - Medicine - Cystic diseases
Respiratory system	Langerhans cell histiocytosis  · Lymphangiomyomatosis · Cystic bronchiectasis
Skin	Adenoid cystic carcinoma
Musculoskeletal system	Cystic hygroma · Fibrocystic breast diseaese
Digestive system	Polycystic Liver Disease · Congenital hepatic fibrosis · Biliary hamartomas · Caroli disease · Choledochal cysts · Von Meyenburg complexes · Peliosis hepatis
Nervous system	Cystic leukoencephalopathy
Genitourinary system	Polycystic kidney disease · Medullary cystic kidney disease (Nephronophthisis)  · Congenital cystic dysplasia
Other conditions	Hydatid cyst · Von Hippel-Lindau syndrome · Tuberous sclerosis

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .