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題名 | Anesthesia for Pediatric Patients with Prader-Willi Syndrome: Report of Two Cases=Prader-Willi症候群之兒科患者的麻醉--二例報告 |
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作者 | 曾紀浩; 陳捷; 黃仲衡; 黃樹欽; 黃潔文; | 書刊名 | 長庚醫學 |
卷期 | 26:6 2003.06[民92.06] |
頁次 | 頁453-457 |
分類號 | 417.661 |
關鍵詞 | Prader-Willi症候群; 肥胖; 阻塞性睡眠窒息症候群; 多項睡眠生理檢查; 連續性正壓換氣; Prader-Willi syndrome; Obesity; Obstructive sleep apnea syndrome; Polysomnogram; Continuous positive airway pressure; |
語文 | 英文(English) |
中文摘要 | Prader-Willi症候群(PWS)是一種罕見之遺傳疾病,此症候群為染色體異常所引起,估計流行率約為一萬五千分之一。PWS主要影響中樞神經系統,尤其是下視丘部位。由於此症候群常導致病態性肥胖的關係,無論採用全身或區域麻醉都相當困難。除此之外,尚有肌肉無力,體溫調節障礙,心律不整,肺心症,糖尿病,異常行為問題,痙攣等。我們報告兩例患有PWS的兒科患者的麻醉,兩者均採用全身麻醉。第一例患者於手術後,分別在麻醉恢復室及兒科加護病房內,歷經數次相當危險嚴重血氧過低狀態,經會診小兒胸腔科醫師,建議採用經鼻管作正壓換氣後,病人狀況才逐漸改善。第二例患者的手術與麻醉過程相當平順,只有在麻醉慢慢消退時,發生短暫的氣管痙攣現象。我們希望透過檢討以上兩病例的經驗,將平在處理這一類病患時,能夠避免或減少術後呼吸併發症的發生。 |
英文摘要 | Prader-will syndrome (PWS) is a sporadic disorder of chromosome abnormalities with an estimated prevalence of 1 in 15,000. It mainly affects the central nervous system, and often involves the hypothalamus. Both general and regional anesthesia for these patients is difficult mainly due to morbid obesity. Other common problems include hypotonia, disturbance in thermoregulation, arrhythmia, corpulmonale, diabetes mellitus, behavior problems, and convulsions. We report on 2 pediatric patients with PWS receiving general anesthesia. The first patient experienced life-threatening episodes of severe hypoxemia in the postanestesia care unit (PACU) as well as in the pediatric intensive care unit (PICU). Nasal continuous positive airway pressure (CPAP) was suggested by the pediatric pulmonary medicine specialist, and thereafter the patient’s condition improved. The clinical course of second patient was uneventful except for transient intermittent episodes of bronchospasms during emergence. In addition, we discuss differences between these 2 cases and our strategy for the prevention of perioperative complications for PWS patients in the future. |
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