A 12‐year‐old boy with the Bardet‐Biedl syndrome (retinal dystrophy, polydactyly, mental retardation, and mild obesity) died of renal failure and was found to have hepatic fibrosis. Nephropathy and hepatic fibrosis in the Bardet‐Biedl syndrome suggest pathogenetic similarity of autosomal recessive disorders in which retinal dystrophy, hepatic fibrosis, and a nephronophthisis‐like disorder are associated.
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Pagon et al. (1982) studied this question.
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