Neonatal management of trisomy 18: Clinical details of 24 patients receiving intensive treatment
Neonatal management of trisomy 18: Clinical details of 24 patients receiving intensive treatment
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DOI：
10.1002/ajmg.a.31175
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发表时间：
2006-05-01
影响因子：
2
通讯作者：
Fukushima, YL
中科院分区：
文献类型：
--
作者：
Kosho, T;Nakamura, T;Fukushima, YL
18三体新生儿的治疗是有争议的，可能是因为预后和缺乏关于治疗效果的准确临床信息。为了描述18三体在强化治疗下的自然病史，我们回顾了1994~2003年间长野儿童医院新生儿重症监护室收治的24例18三体完全性三体患者的临床资料。剖宫产16例(67%)，插管复苏15例(63%)，外科手术10例(42%)。21例(88%)需要机械通气，其中6例(29%)拔管。1周、1个月和1年存活率分别为88%、83%和25%。中位生存期为152.5天。2例左侧横隔肌膨出和发育不良合并King发育不良的患者呼吸不稳定，即使在最大通气量的情况下也是如此。与死亡相关的常见潜在因素是先天性心脏病和心力衰竭(96%)，其次是肺动脉高压(78%)。常见的最终死亡模式是心脏骤停或心肺骤停(26%)和可能的进行性肺动脉高压相关事件(26%)。这些通过新生儿强化治疗提高存活率的数据有助于临床医生向18三体患者的家属提供关于治疗选择的最佳信息。(C)2006年Wiley-Liss，Inc.
Management of neonates with trisomy 18 is controversial, supposedly due to the prognosis and the lack of precise clinical information concerning efficacy of treatment. To delineate the natural history of trisomy 18 managed under intensive treatment, we reviewecl detailed clinical data of 24 patients with full trisomy 18 admitted to the neonatal intensive care Unit of Nagano Children's Hospital, providing intensive treatment to those with trisomy 18, from 1994 to 2003. Cesarean, resuscitation by intubation, and surgical Operations were performed on 16 (67%), 15 (63%), and 10 (42%) of the patients, respectively. Mechanical ventilation was required by 21 (88%), and 6 (29%) of them were extubated. survival rate at age 1 week, 1 month, and 1 year was 88%, 83%, and 25%, respectively. Median survival time was 152.5 days. Respiration was not stabilized in two patients with left diaphragmatic eventration ancl hypoplasia accompanied by king hypoplasia, even with maximal ventilation. The common underlying factors associated with death were congenital heart defects ancl heart failure (96%), followed by pulmonary hypertension (78%). The common final modes of death were sudden cardiac or cardiopulmonary arrest (26%) ancl possible progressive pulmonary hypertension-related events (26%). These data of improved survival, through neonatal intensive treatment, are helpful for clinicians to offer the best information on treatment options to families of patients with trisomy 18. (c) 2006 Wiley-Liss, Inc.