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A case of congenital hepatic fibrosis (CHF) is described. CHF is characterized by hepatomegaly, portal hypertension, extensive portal fibrosis, ectatic bile ducts, and hypoplasia of terminal portal vein branches. In contrast to the severe portal hypertension liver function tests are largely normal. In our case the disease was first detected when the patient was 7 years old. During the following 9 1/2 years three sequential liver biopsies were performed. Each of them showed the same picture and no progression occurred. The characteristic histological picture of CHF includes mature bile ducts without epithelial proliferation, absence of significant intraportal or interlobular inflammatory infiltrates, and small or hypoplastic distal portal vein branches. On the basis of these features the disease can easily be separated from other forms of liver cirrhosis.
K Donhuijsen, and
L D Leder
July 1976,
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology,
K Donhuijsen, and
L D Leder
October 1981,
Casopis lekaru ceskych,
K Donhuijsen, and
L D Leder
November 1975,
Klinische Padiatrie,
K Donhuijsen, and
L D Leder
January 1974,
Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie. Albrecht von Graefe's archive for clinical and experimental ophthalmology,
K Donhuijsen, and
L D Leder
April 1982,
Gastroenterologie clinique et biologique,
K Donhuijsen, and
L D Leder
March 1974,
Klinische Monatsblatter fur Augenheilkunde,
K Donhuijsen, and
L D Leder
December 1977,
Nippon Ganka Gakkai zasshi,
K Donhuijsen, and
L D Leder
January 1975,
Langenbecks Archiv fur Chirurgie,
K Donhuijsen, and
L D Leder
August 1979,
Kyobu geka. The Japanese journal of thoracic surgery,
K Donhuijsen, and
L D Leder
May 1977,
Klinische Padiatrie,
