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KMID : 0878620020060010026
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Korean Journal of Pediatric Anesthesia
2002 Volume.6 No. 1 p.26 ~ p.30
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The Anesthetic Management for the Patient with Alagille’s Syndrome -Case report-
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Lee Jun-Geol
Kim Kyoung-Ok
Song Jin-Seok
Oh Ah-Young
Kim Hee-Soo
Park Chong-Doo
Kim Chong-Sung
Kim Seong-Deok
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Abstract
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Alagille’s syndrome was first described by Alagille and Watson in 1973 and is characterized by cholestasis, occurring in association with facial, cardiac, ocular, musculoskeletal, and neurodevelopmental abnormalities. Hepatic dysfunction after
heart surgery and cardiopulmonary bypass (CPB) has been frequently reported in clinical situation, especially in the patients with preoperative hepatic dysfunction or chronic cardiac failure. We report the anesthetic management of 15 months-old boy with Alagille’s syndrome who was scheduled to have open heart surgery for pulmonary atresia. We will discuss the anesthetic considerations in Alagille’s syndrome from the case report.
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KEYWORD
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Alagille’s syndrome, open heart surgery
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