Congenital hepatic fibrosis
in autosomal-dominant polycystic kidney disease. Congenital hepatic fibrosis
was found in four families with autosomal-dominant polycystic kidney disease.
Congenital hepatic fibrosis is commonly throught to be characteristic for
autosomal-recessive polycystic kidney disease, but the reported families
show that it can also complicate autosomal-dominant polycystic kidney disease.
In three families close linkage between the mutation causing the disease
and DNA markers on chromosome 16 was demonstrated. The clinical course
of the congenital hepatic fibrosis differed considerably: in one family
the children with congenital hepatic fibrosis died soon after birth. In
the three other families an approximately 20 years follow-up showed no
detectable progression of the liver disease.
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