Pediatric Radiology

, Volume 23, Issue 2, pp 131–133 | Cite as

Association of congenital hepatic fibrosis with autosomal dominant polycystic kidney disease

Report of a family with review of literature
  • B. Lipschitz
  • W. E. Berdon
  • A. R. Defelice
  • J. Levy


The association of autosomal recessive polycystic kidney disease (ARPKD) with congential hepatic fibrosis (CHF) is well known; a rare occurrence is that of congenital hepatic fibrosis with autosomal dominant polycystic kidney disease (ADPKD). We report a family with ADPKD in which congenital hepatic fibrosis with severe portal hypertension (PHT) presented in a 4-year-old girl; the kidneys were initially normal. Typical changes of autosomal dominant polycystic kidney disease developed in the next decade and were also found in the mother and sister (neither of whom had any evidence of portal hypertension). Severe variceal bleeding was treated by sclerotherapy and beta receptor blocade.


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Copyright information

© Springer-Verlag 1993

Authors and Affiliations

  • B. Lipschitz
    • 1
  • W. E. Berdon
    • 2
  • A. R. Defelice
    • 1
  • J. Levy
    • 3
  1. 1.Department of Pediatric Gastrointestinal Diseases, Babies HospitalColumbia Presbyterian Medical CenterNew YorkUSA
  2. 2.Department of Pediatric Radiology, Babies HospitalColumbia Presbyterian Medical CenterNew YorkUSA
  3. 3.Department of Gastrointestinal DiseasesNew York Hospital, Cornell UniversityNew YorkUSA

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