Congenital hepatic fibrosis: the long-term prognosis.

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Congenital hepatic fibrosis: the long-term prognosis.

The long-term prognosis in congenital hepatic fibrosis has been assessed in 30 patients, 13 treated at Newcastle and 17 from other British centres. Twenty-four patients had been followed-up for more than five years from diagnosis. Shunt operations, performed in 18 patients, were successful in controlling haemorrhage with a low mortality (1/18), low incidence of recurrent haemorrhage (3/18) and ...

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Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure

Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys.  Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an...

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Congenital hepatic fibrosis.

Congenital Hepatic fibrosis is an uncommon disease, which is autosomal recessive. Two forms of the disease are distinguished: a rare one becoming manifest in the neonatal period with signs of progressive renal failure secondary to polycystic kidneys, in such cases the liver fibrosis is usually asymptomatic, and the diagnosis is therefore often first established post mortem. In the other more us...

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ژورنال

عنوان ژورنال: Gut

سال: 1978

ISSN: 0017-5749

DOI: 10.1136/gut.19.6.514