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Postgrad Med J. 1985 July; 61(717): 641–642.
PMCID: PMC2418332

Congenital hepatic fibrosis and adult-type autosomal dominant polycystic kidney disease in a child.


This reports a family with congenital hepatic fibrosis and adult polycystic kidney disease. Adult polycystic kidney disease was present in three generations. In addition, congenital hepatic fibrosis occurred in two members of the third generation. These conditions are generally held to have different modes of inheritance and the significance of their occurrence together is not clear.

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Selected References

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  • Blyth H, Ockenden BG. Polycystic disease of kidney and liver presenting in childhood. J Med Genet. 1971 Sep;8(3):257–284. [PMC free article] [PubMed]
  • Dupond JL, Miguet JP, Carbillet JP, Saint-Hiller Y, Perol C, LeConte-des-Floris R. Kidney polycystic disease in adult congenital hepatic fibrosis. Ann Intern Med. 1978 Apr;88(4):514–515. [PubMed]
  • Gaisford W, Bloor K. Congenital polycystic disease of kidneys and liver. Portal hypertension--portacaval anastomosis. Proc R Soc Med. 1968 Mar;61(3):304–304. [PubMed]
  • KERR DN, HARRISON CV, SHERLOCK S, WALKER RM. Congenital hepatic fibrosis. Q J Med. 1961 Jan;30:91–117. [PubMed]
  • Lieberman E, Salinas-Madrigal L, Gwinn JL, Brennan LP, Fine RN, Landing BH. Infantile polycystic disease of the kidneys and liver: clinical, pathological and radiological correlations and comparison with congenital hepatic fibrosis. Medicine (Baltimore) 1971 Jul;50(4):277–318. [PubMed]
  • Murray-Lyon IM, Ockenden BG, Williams R. Congenital hepatic fibrosis--is it a single clinical entity? Gastroenterology. 1973 Apr;64(4):653–656. [PubMed]
  • Sommerschild HC, Langmark F, Maurseth K. Congenital hepatic fibrosis: report of two new cases and review of the literature. Surgery. 1973 Jan;73(1):53–58. [PubMed]
  • Tazelaar HD, Payne JA, Patel NS. Congenital hepatic fibrosis and asymptomatic familial adult-type polycystic kidney disease in a 19-year-old woman. Gastroenterology. 1984 Apr;86(4):757–760. [PubMed]

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