Syndromic paucity of interlobular bile ducts or Alagille syndrome: a case report.

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How to Cite

Despontin K., Bottu J., Decroix J., Otte J.B., Lachapelle J.M. 2018. Syndromic paucity of interlobular bile ducts or Alagille syndrome: a case report. Eur. J. Pediat. Dermatol. 1 (1):35-39.

Authors

Despontin K. Bottu J. Decroix J. Otte J.B. Lachapelle J.M.
pp. 35-39

Abstract

A case of syndromic paucity of interlobular bile ducts (Alagille syndrome) is report­ed in an 8-year-old child. Chronic intrahepatic cholestasis is described in association with vertebral, cardio­vascular, ophthalmogical and renal abnormalities. Various skin manifestations includ­ing xanthomas, jaundice and pruritus are present secondary to biliary and liver failure.

Keywords

Chronic cholestasis, Alagille syndrome, Xanthomatosis, Fat soluble vitamin deficien­cy, Atrophia maculosa varioliformis cutis

Issue

Vol. 1 No. 1 (1991)