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相關文獻
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題名 | Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome: Report of One Case=巨膀胱-細小結腸-腸蠕動不良症候群:一病例報告 |
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作者 | 李妮鍾; 刁翠美; 宋文舉; 岑秋良; 黃碧桃; 魏拙夫; | 書刊名 | 臺灣兒科醫學會雜誌 |
卷期 | 44:4 民92.07-08 |
頁次 | 頁238-241+260 |
分類號 | 417.509 |
關鍵詞 | 巨膀胱細小結腸蠕動不良症候群; 氣管支氣管軟化症; 十二指腸瓣; 自體隱性遺傳; Megacystis-microcolon-intestinal hypoperistalsis syndrome; Duodenal web; Tracheobronchial malacia; |
語文 | 英文(English) |
中文摘要 | 巨膀胱細小結腸蠕動不良症候群是罕見的自體陷性遺傳的先天性異常,病人會有明顯的腹脹、餵食困難及排尿不良,有些會合併並其他先天性異常。影像檢查可以發現脹大的胃,腸氣很少,以及巨在的膀胱。致病原因尚未有定論,目前傾向與乙烯膽堿受器缺陷進而影響腸道及泌尿道神經功能不良有關。治療成效大多不佳,以治療腹脹,尿液儲留及餵食困難爲主。此病死亡率很高,大多數的病人在很小的時候便死亡。本文報告一位産前即發現腹部有囊腫,出生後診斷爲巨膀胱細小結腸蠕動不良症候群,合併有氣管支氣管軟化症及二指腸瓣的女嬰,並回顧文獻資料。 |
英文摘要 | Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a very rare congenital disease characterized by abdominal distension due to a dilated urinary bladder, microcolon and decreased or absent intestinal peristalsis. Most patients die at an early age. We report on a female baby with this syndrome which was associated with duodenal web and tracheobronchial malacia. The literature on this syndrome is also reviewed. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。