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先天性膈疝规范化管理的疗效:100例病例回顾

Efficacy of protocolized management for congenital diaphragmatic hernia. a review of 100 cases.

作者信息

Okawada Manabu, Okazaki Tadaharu, Yamataka Atsuyuki, Yanai Toshihiro, Kato Yoshifumi, Kobayashi Hiroyuki, Lane Geoffrey J, Miyano Takeshi

机构信息

Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.

出版信息

Pediatr Surg Int. 2006 Nov;22(11):925-30. doi: 10.1007/s00383-006-1759-8.

Abstract

A review of 100 consecutive cases of congenital diaphragmatic hernia (CDH) treated at our institute focusing on the efficacy of protocolized management (PM) was conducted. Of the 100 cases, 14 who became symptomatic more than 24 h after birth, and seven with fatal anomalies (four cardiac and three chromosomal) were excluded, leaving 79 subjects for this study. Of these, 41 were diagnosed prenatally (PD). Subjects were divided into four groups. Group I: No PD, no PM (n = 34), Group II: No PD, PM (n = 4), Group III: PD, no PM (n = 21), and Group IV: PD, PM (n = 20). PM includes criteria for planned delivery, use of high frequency oxygenation, nitric oxide, echocardiography (EC), and a medication schedule. Overall survival rates for Groups I, II, III, and IV were 73.5% (25/34), 75% (3/4), 38.1% (8/21), and 70.0% (14/20), respectively. Survival rates were higher when PM was used: 70.8% (Groups II, IV) versus 60.0% (Groups I, III). Survival rates were significantly lower if diagnosed prenatally (PD+): 53.7% (Groups III, IV) versus 73.7% (Groups I, II) (P < 0.01). However, in PD+ groups, survival was significantly higher if PM was used (P < 0.05). PM significantly reduced length of hospital stay (35.5 vs. 52.0 days: P < 0.05). EC was found to be a predictor for survival while post-ductal AaDO(2) was not. In 17 cases with cardiac anomalies, PM did not affect survival. Our study suggests that use of PM for prenatally diagnosed CDH cases is associated with improved outcome, although the components of PM need to be tested in prospective trials to determine their true value.

摘要

我们对本研究所治疗的100例连续性先天性膈疝(CDH)病例进行了回顾,重点关注规范化管理(PM)的疗效。在这100例病例中,排除了14例出生后24小时以上出现症状的病例,以及7例伴有致命畸形(4例心脏畸形和3例染色体畸形)的病例,本研究共纳入79例受试者。其中,41例为产前诊断(PD)。受试者被分为四组。第一组:无产前诊断,无规范化管理(n = 34),第二组:无产前诊断,规范化管理(n = 4),第三组:产前诊断,无规范化管理(n = 21),第四组:产前诊断,规范化管理(n = 20)。规范化管理包括计划分娩标准、高频氧疗的使用、一氧化氮、超声心动图(EC)以及用药方案。第一组、第二组、第三组和第四组的总体生存率分别为73.5%(25/34)、75%(3/4)、38.1%(8/21)和70.0%(14/20)。使用规范化管理时生存率更高:70.8%(第二组、第四组)对比60.0%(第一组、第三组)。如果是产前诊断(PD+),生存率显著更低:53.7%(第三组、第四组)对比73.7%(第一组、第二组)(P < 0.01)。然而,在PD+组中,如果使用规范化管理,生存率显著更高(P < 0.05)。规范化管理显著缩短了住院时间(35.5天对比52.0天:P < 0.05)。发现超声心动图是生存的预测指标,而导管后AaDO(2)不是。在17例有心脏畸形的病例中,规范化管理不影响生存。我们的研究表明,对于产前诊断的CDH病例使用规范化管理与改善预后相关,尽管规范化管理的组成部分需要在前瞻性试验中进行测试以确定其真正价值。

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