The Alagille's syndrome and its anaesthetic considerations |
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| Authors: | DINESH CHOUDHRY MD,FRCA,,MOHAMMED REHMAN MD,,ROY SCHWARTZ MD, & DAVID PICCOLI MD |
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| Affiliation: | Department of Anaesthesia and Critical Care, St. Christopher's Hospital for Children, Erie Avenue at Front Street, Philadelphia, PA 19134-1095, USA,;Department of Gastroenterology, Children's Hospital of Philadelphia, One Children's Center, 34th and Civic Center, Philadelphia, PA 19104, USA |
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| 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. |
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| Keywords: | Alagille's syndrome: cholestasis cardiac defects growth failure |
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