尼曼-匹克病患儿的麻醉管理
Anesthetic Management in a Child With Niemann-Pick Disease.
作者信息
Espahbodi Ebrahim, Yaghooti Amir Abbas, Ostadalipour Abbas, Marashi Shaqayeq
机构信息
Anesthesiology and Critical Care, Tehran University of Medical Sciences, Tehran, IR Iran.
出版信息
Iran J Pediatr. 2016 Jul 31;26(5):e5479. doi: 10.5812/ijp.5479. eCollection 2016 Oct.
Niemann-Pick is a lipid storage disease that results from a lysosomal enzyme deficiency (sphingomyelinase). It has different presentations, and it may affect various organs such as the central nervous system, kidney, liver, and spleen. Due to the complexity of the disease, careful perianesthetic management is necessary in order to reduce the risks and sequels. As there is little evidence available in the literature regarding the anesthetic implications of such patients, in this case report we describe the anesthetic management of a two-year-old female with Niemann-Pick disease.
尼曼-匹克病是一种由于溶酶体酶（鞘磷脂酶）缺乏导致的脂质贮积病。它有不同的表现形式，可能会影响多个器官，如中枢神经系统、肾脏、肝脏和脾脏。由于该疾病的复杂性，需要进行仔细的围麻醉期管理以降低风险和并发症。鉴于文献中关于此类患者麻醉影响的证据很少，在本病例报告中，我们描述了一名患有尼曼-匹克病的两岁女性的麻醉管理情况。
Zotero 插件