Departments of, Department of, Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Department of, Pediatric Surgery, Graduate School of Medicine, Chiba University, Chiba, Japan.
Pediatr Int. 2021 Apr;63(4):371-390. doi: 10.1111/ped.14473. Epub 2021 Apr 13.
Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm in which abdominal organs herniate through the defect into the thoracic cavity. The main pathophysiology is respiratory distress and persistent pulmonary hypertension because of pulmonary hypoplasia caused by compression of the elevated organs. Recent progress in prenatal diagnosis and postnatal care has led to an increase in the survival rate of patients with CDH. However, some survivors experience mid- and long-term disabilities and complications requiring treatment and follow-up. In recent years, the establishment of clinical practice guidelines has been promoted in various medical fields to offer optimal medical care, with the goal of improvement of the disease' outcomes, thereby reducing medical costs, etc. Thus, to provide adequate medical care through standardization of treatment and elimination of disparities in clinical management, and to improve the survival rate and mid- and long-term prognosis of patients with CDH, we present here the clinical practice guidelines for postnatal management of CDH. These are based on the principles of evidence-based medicine using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. The recommendations are based on evidence and were determined after considering the balance among benefits and harm, patient and society preferences, and medical resources available for postnatal CDH treatment.
先天性膈疝(CDH)是一种膈肌先天性缺陷,其特点是腹部器官通过缺陷疝入胸腔。其主要的病理生理学改变是由于升高的器官压迫导致肺发育不全,引起呼吸窘迫和持续性肺动脉高压。产前诊断和新生儿期护理的最新进展使 CDH 患者的生存率提高。然而,一些幸存者会经历需要治疗和随访的中长期残疾和并发症。近年来,在各个医学领域都在推动临床实践指南的建立,旨在提供最佳的医疗服务,改善疾病结局,从而降低医疗费用等。因此,为了通过治疗标准化和消除临床管理中的差异来提供充分的医疗服务,并提高 CDH 患者的生存率和中长期预后,我们在此介绍 CDH 的新生儿期管理临床实践指南。这些指南基于使用推荐评估、制定与评价(GRADE)方法的循证医学原则制定。推荐意见基于证据,并在考虑了获益与危害、患者和社会偏好以及新生儿 CDH 治疗的可用医疗资源之间的平衡后确定。