[Congenital hepatic fibrosis].

Abstract

In 1961 Kerr et al separated congenital hepatic fibrosis from other types of 'juvenile cirrhosis'. They emphasized the histological appearances of the liver, namely, normal lobules of parenchyma separated by broad bands of fibrous tissue, abnormal interlobular bile ducts which were multiple and dilated, and scarce portal veins, and also the association with renal abnormalities. More than 150 cases of this condition have now been reported (Sommerschild et al, 1973). The presentation between birth and early adult life, the rarity of the condition, and the good prognosis have often been emphasized. Indeed, in this country in 1972 only 20 cases were known to a team surveying the condition (Okonkwo and Choa, 1972), but it may not be as rare as this, especially since some cases are misdiagnosed as cirrhosis (Kerr et al, 1962; Campana et al, 1974). In this paper we describe four more patients, only one ofwhom was referred from outside the hospital's district, while three were more than 30 years old at the time of diagnosis. It is suggested that, due to its incorrect diagnosis, the condition may be more common than is usually thought.

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@article{Sommerschild1970CongenitalHF, title={[Congenital hepatic fibrosis].}, author={Henrich C. Sommerschild and Fr\oydis Langmark}, journal={La Presse médicale}, year={1970}, volume={78 17}, pages={794} }