Original Article

Familial Congenital Hepatic Fibrosis: Report of a Family with Three Affected Children

Abstract

Congenital hepatic fibrosis (CHF) is a developmental disorder of the biliary system, characterized by defective remodeling of the ductal plate. Herein a family of three children, from consanguineous parents, with minor thalassemia is presented who suffered from congenital hepatic fibrosis (CHF). Prompt diagnosis and appropriate treatment are necessary to avoid further complications in the affected patients.

Gunay-Aygun M, Gahl WA, Heller T. Congenital Hepatic Fibrosis Overview. In: Pagon RA, Bird TD, Dolan CR, Stephens K, Adam MP, editors. GeneReviews. Seattle (WA);1993.

Robert M. Kliegman MD REBM, Hal B. Jenson MD, Bonita M.D. Stanton MD. Nelson textbook of pediatrics. 18th edition ed; 2004.

Hisham Nazer M, BCh, FRCP, DCh, DTM&H. congenital hepatic fibrosis. In; 2012.

Poala SB, Bisogno G, Colombatti R. Thrombocytopenia and splenomegaly: an unusual presentation of congenital hepatic fibrosis. Orphanet J Rare Dis 2010;5:4.

Yonem O, Ozkayar N, Balkanci F, Harmanci O, Sokmensuer C, Ersoy O, Bayraktar Y. Is congenital hepatic fibrosis a pure liver disease? Am J Gastroenterol 2006;101(6):1253-9.

Zeitoun D, Brancatelli G, Colombat M, Federle MP, Valla D, Wu T, Degott C, Vilgrain V. Congenital hepatic fibrosis: CT findings in 18 adults. Radiology 2004;231(1):109-16.

Caine Y, Deckelbaum RJ, Weizman Z, Lijovetsky G, Schiller M. Congenital hepatic fibrosis--unusualpresentations. Arch Dis Child 1984;59(11):1094-6.

Shorbagi A, Bayraktar Y. Experience of a single center with congenital hepatic fibrosis: a review of the literature. World J Gastroenterol 2010;16(6):683-90.

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IssueVol 51, No 9 (2013) QRcode
SectionOriginal Article(s)
Keywords
Autoimmune hepatitis Congenital hepatic fibrosis Ductal plate malformation Transjugular intrahepatic portosystemic shunt
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How to Cite
1.
Farahmand F, Soleimani K, Hashemi M, Shafieyoun A, Rezaei N, Yousefi A. Familial Congenital Hepatic Fibrosis: Report of a Family with Three Affected Children. Acta Med Iran. 1;51(9):655-656.