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先天性膈疝:文献回顾反映未解决的困境。

Congenital diaphragmatic hernia: review of the literature in reflection of unresolved dilemmas.

机构信息

Department of Obstetrics and Gynecology, Shaare Zedek Medical Center, Jerusalem, Israel.

出版信息

Acta Paediatr. 2009 Dec;98(12):1874-81. doi: 10.1111/j.1651-2227.2009.01436.x. Epub 2009 Jul 20.

Abstract

BACKGROUND

Congenital diaphragmatic hernia (CDH) is a rare but clinically and scientifically challenging condition. The introduction of ultrasound has enabled early prenatal detection and consequently, hope of early therapeutic intervention.

AIM

We undertook the task to review the recent developments in understanding the pathology of CDH as well as the history and current management strategies to aid perinatologists in consultations with parents of CDH-affected foetuses.

STUDY DESIGN

A Medline search was undertaken of all reports and reviews published between 1980 and 2008 using MeSH search terms 'diaphragmatic hernia', 'congenital' and 'newborn'.

RESULTS

The true incidence of CDH is still difficult to estimate because of the high incidence of hidden mortality of CDH. Complete case ascertainment also poses difficulties in assessment of the impact of new therapeutic modalities on overall survival. Recent improvements in prenatal detection are a milestone in affording time for re-assessments and parental counselling. The true benefit of antenatal therapy is circumscribed and should be offered only in selected cases of isolated severe CDH as defined by existing guidelines. Postnatal intensive respiratory supportive therapy and innovative surgical techniques within specialized tertiary centres has had a major impact on survival of babies with CDH.

CONCLUSION

The high survival of 'selected cases' that are live births and benefit from optimal care will be difficult to improve by antenatal interventions. The multidisciplinary approach to basic research and randomized clinical trials will further define the best approach to the foetus and neonate with CDH.

摘要

背景

先天性膈疝(CDH)是一种罕见但具有临床和科学挑战性的疾病。超声技术的引入使得能够在产前进行早期检测,并因此带来了早期治疗干预的希望。

目的

我们承担了这项任务,旨在回顾对 CDH 病理的最新认识,以及历史和当前的管理策略,以帮助围产医生与 CDH 胎儿的父母进行咨询。

研究设计

使用 MeSH 搜索词“膈疝”、“先天性”和“新生儿”,对 1980 年至 2008 年间发表的所有报告和综述进行了 Medline 搜索。

结果

由于 CDH 高隐藏死亡率,CDH 的真实发病率仍难以估计。完全病例确定也给评估新治疗模式对总体生存率的影响带来困难。最近产前检测的改进是为重新评估和父母咨询提供时间的一个里程碑。产前治疗的真正益处是有限的,仅应在现有指南定义的孤立性严重 CDH 的选定病例中提供。在专门的三级中心进行强化呼吸支持治疗和创新的外科技术,对 CDH 婴儿的存活率产生了重大影响。

结论

通过产前干预,很难提高“选定病例”(即活产并受益于最佳护理的病例)的高存活率。多学科方法进行基础研究和随机临床试验将进一步确定对 CDH 胎儿和新生儿的最佳方法。

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