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先天性膈疝:利弊与挑战

Congenital diaphragmatic hernia: the good, the bad, and the tough.

作者信息

Aydin Emrah, Lim Foong-Yen, Kingma Paul, Haberman Beth, Rymeski Beth, Burns Patricia, Peiro Jose L

机构信息

Division of Pediatric General and Thoracic Surgery, The Center for Fetal Cellular and Molecular Therapy, Cincinnati Fetal Center, 3333 Burnet Avenue, MLC 11025, Cincinnati, OH, 45229-3039, USA.

Division of Neonatology and Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

出版信息

Pediatr Surg Int. 2019 Mar;35(3):303-313. doi: 10.1007/s00383-019-04442-z. Epub 2019 Jan 24.

DOI:10.1007/s00383-019-04442-z
PMID:30680439
Abstract

OBJECTIVES

We aim to determine factors that are associated with better outcomes of CDH patients.

METHODS

A retrospective review was performed on all CDH patients admitted to our institution between 2003 and 2016. This study was performed at a single institution which has a fetal care center. Patients admitted with CDH with at least 1-year follow-up during the analysis were included in the study.

RESULTS

Twenty-six (13.8%) patients had a hernia sac, 124 (59%) patients had liver herniation, and 56 (25.1%) patients had an accompanying syndrome. Overall survival to discharge was 73.1% while overall survival to date was 69.5%. The presence of a hernia sac, liver herniation, and accompanying syndromes showed as independent predictors influencing the survival, B 1.968, p = 0.04, OR 7.158, 95% CI 0.907-56.485, B - 1.178, p = 0.01, OR 3.932, 95% CI 1.798-8.602 and B - 1.032, p = 0.05, OR 2.795, 95% CI 0.976-7.764, respectively.

CONCLUSION

In our CDH cohort, the presence of a hernia sac was proven to be associated with better outcomes, while thoracic herniation of the liver was associated with worse outcomes. The accompanying syndromes although being more difficult to manage had a little effect on the outcome of the disease itself.

摘要

目的

我们旨在确定与先天性膈疝(CDH)患者更好预后相关的因素。

方法

对2003年至2016年期间入住本机构的所有CDH患者进行回顾性研究。本研究在一家设有胎儿护理中心的单一机构进行。分析期间接受CDH治疗且随访至少1年的患者纳入本研究。

结果

26例(13.8%)患者有疝囊,124例(59%)患者有肝脏疝入,56例(25.1%)患者伴有综合征。出院时的总体生存率为73.1%,而目前的总体生存率为69.5%。疝囊、肝脏疝入和伴发综合征的存在显示为影响生存的独立预测因素,其B值分别为1.968,p = 0.04,OR值为7.158,95%可信区间为0.907 - 56.485;B值为 - 1.178,p = 0.01,OR值为3.932,95%可信区间为1.798 - 8.602;B值为 - 1.032,p = 0.05,OR值为2.795,95%可信区间为0.976 - 7.764。

结论

在我们的CDH队列中,已证明疝囊的存在与更好的预后相关,而肝脏胸内疝与更差的预后相关。伴发综合征虽然更难处理,但对疾病本身的预后影响较小。

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本文引用的文献

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Hernia Sac Presence Portends Better Survivability of Isolated Congenital Diaphragmatic Hernia with "Liver-Up".疝囊的存在预示着“肝脏上移型”孤立性先天性膈疝具有更好的生存能力。
Am J Perinatol. 2017 Apr;34(5):515-519. doi: 10.1055/s-0036-1593765. Epub 2016 Oct 25.
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Controversies in the management of severe congenital diaphragmatic hernia.严重先天性膈疝治疗中的争议
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Advances in the surgical approach to congenital diaphragmatic hernia.
导致先天性膈疝和膈膨升新生儿死亡的因素。
Afr J Paediatr Surg. 2023 Apr-Jun;20(2):85-88. doi: 10.4103/ajps.ajps_165_21.
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Prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia: a systematic review and meta-analysis.先天性膈疝胎儿死亡的产前预测因素:系统评价和荟萃分析。
Pediatr Surg Int. 2022 Dec;38(12):1745-1757. doi: 10.1007/s00383-022-05232-w. Epub 2022 Sep 14.
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The Survivorship Bias in Congenital Diaphragmatic Hernia.先天性膈疝中的幸存者偏差
Children (Basel). 2022 Feb 6;9(2):218. doi: 10.3390/children9020218.
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Transuterine Fetal Tracheal Occlusion Model in Mice.子宫内胎儿气管阻塞模型的建立。
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Congenital diaphragmatic hernia-does the presence of a hernia sac improve outcome? A systematic review of published studies.先天性膈疝——疝囊的存在是否能改善预后?已发表研究的系统评价
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