Department of Surgery, Boston Children's Hospital, Boston, MA; Vascular Biology Program, Boston Children's Hospital, Boston, MA.
Division of Pulmonary Medicine, Boston Children's Hospital, Boston, MA; Channing Division of Network Medicine, Brigham and Women's Hospital, Boston, MA.
J Pediatr. 2020 Jan;216:158-164.e2. doi: 10.1016/j.jpeds.2019.09.072. Epub 2019 Nov 6.
To analyze longitudinal trends of pulmonary function testing in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic.
This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in 1 second (FEVpp), percent predicted forced vital capacity (FVCpp), and FEV/FVC over time.
Of 268 patients with CDH who survived to discharge, 119 had at least 1 pulmonary function test study. The FEVpp (P < .001), FVCpp (P = .017), and FEV/FVC (P = .001) decreased with age. Compared with defect size A/B, those with defect size C/D had lower FEVpp by an average of 11.5% (95% CI, 2.9%-20.1%; P = .010). A history of oxygen use at initial hospital discharge also correlated with decreased FEVpp by an average of 8.0% (95% CI, 1.2%-15.0%; P = .023).
In a select cohort of CDH survivors, average pulmonary function declines with age relative to expected population normative values. Those with severe CDH represent a population at risk for worsening pulmonary function test measurements who may benefit from recognition and monitoring for complications.
分析在我们多学科诊所中接受治疗的先天性膈疝（CDH）患者的肺功能检测的纵向趋势。
这是一项对 1991 年至 2013 年期间出生的 CDH 患者的回顾性队列研究。使用线性混合效应模型来估计 1 秒用力呼气量预测百分比（FEVpp）、用力肺活量预测百分比（FVCpp）和 FEV/FVC 的时间趋势。
在 268 例存活至出院的 CDH 患者中，有 119 例至少进行了 1 次肺功能检测研究。FEVpp（P<.001）、FVCpp（P=.017）和 FEV/FVC（P=.001）随年龄增长而降低。与缺陷大小 A/B 相比，缺陷大小 C/D 的患者的 FEVpp 平均低 11.5%（95%CI，2.9%-20.1%；P=.010）。初始出院时使用氧气的病史也与 FEVpp 平均降低 8.0%（95%CI，1.2%-15.0%；P=.023）相关。
在一组选择的 CDH 幸存者中，平均肺功能相对于预期人群的正常参考值随年龄下降。那些患有严重 CDH 的患者代表着一个肺功能检测结果恶化风险较高的人群，他们可能受益于识别和监测并发症。
