The Alagille's syndrome and its anaesthetic considerations

Dinesh K. Choudhry, Mohammed A. Rehman, Roy E. Schwartz, David A. Piccoli

Research output: Contribution to journalArticle

Abstract

Alagille's syndrome is an inherited disorder affecting multiple organ systems. Most characteristic is the paucity of the interlobular bile ducts with cholestasis. The anaesthetic plan for these patients should be based on careful preoperative preparation and attention to the issues involving hepatobiliary, cardiac, neurodevelopmental, nutritional, haematological, ocular and facial abnormalities. We report the case of a five-and-a-half- year old patient with Alagille's syndrome who sustained a pathological fracture of the femur and was scheduled for closed reduction and application of a hip spica cast. Relevant anaesthetic issues are discussed.

Original languageEnglish (US)
Pages (from-to)79-82
Number of pages4
JournalPaediatric anaesthesia
Volume8
Issue number1
DOIs
StatePublished - Feb 19 1998
Externally publishedYes

Keywords

  • Alagille's syndrome: cholestasis
  • Cardiac defects
  • Growth failure

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Anesthesiology and Pain Medicine

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