Nakayama D K, Motoyama E K, Mutich R L, Koumbourlis A C
Department of Surgery, Children's Hospital of Pittsburgh, PA 15213-3417.
Pediatr Pulmonol. 1991;11(1):49-55. doi: 10.1002/ppul.1950110109.
Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia that limits survival, but the nature and extent of pulmonary dysfunction in neonates with CDH have not been studied. We performed pulmonary function tests (PFTs) in eight intubated infants who survived neonatal repair of CDH (wt, 3.33 +/- 0.15 kg; age, 20.1 +/- 2.7 d; mean +/- S.E.M.). PFTs obtained from six full-term infants (wt, 3.56 +/- 0.15 kg; age, 25.0 +/- 3.3 d) with no respiratory illness served as controls. The deflation flow-volume curve technique produced maximum expiratory flow-volume (MEFV) curves, giving reproducible measurements of forced vital capacity (FVC) and maximal expiratory flow at 25% of FVC (MEF25). Respiratory system compliance (Crs) and resistance (Rrs) were obtained with a modified passive mechanics technique. In seven of eight infants PFTs were repeated after nebulized bronchodilator (0.1% isoetharine). In neonates surviving CDH repair, as compared to those with normal lung function, FVC was significantly reduced (20.78 +/- 3.32 vs. 39.83 +/- 3.30 mL.kg-1, P less than 0.05). MEF25 was also markedly reduced (20.78 +/- 3.32 vs. 39.83 +/- 3.30 mL.kg-1.s-1, P less than 0.05), indicating lower airway obstruction. After administration of nebulized bronchodilator, PFTs showed significant increases from control values in both FVC (15.9%) and MEF25 (200%) without changes in Crs and Rrs. These findings indicate that neonates with CDH have restrictive lung defects, reflecting hypoplasia. After surgical repair and mechanical ventilation airway reactivity develops, primarily in smaller airways, and this may complicate the postoperative course.
先天性膈疝（CDH）与限制生存的肺发育不全相关，但CDH新生儿肺功能障碍的性质和程度尚未得到研究。我们对8名在新生儿期接受CDH修复手术且存活的插管婴儿进行了肺功能测试（PFTs）（体重，3.33±0.15kg；年龄，20.1±2.7天；均值±标准误）。从6名无呼吸系统疾病的足月儿（体重，3.56±0.15kg；年龄，25.0±3.3天）获得的PFTs作为对照。放气流量-容积曲线技术产生最大呼气流量-容积（MEFV）曲线，可重复测量用力肺活量（FVC）和FVC的25%时的最大呼气流量（MEF25）。采用改良的被动力学技术获得呼吸系统顺应性（Crs）和阻力（Rrs）。在8名婴儿中的7名中，雾化支气管扩张剂（0.1%异他林）后重复进行PFTs。与肺功能正常的新生儿相比，存活的CDH修复术后新生儿的FVC显著降低（20.78±3.32 vs. 39.83±3.30mL.kg-1，P<0.05）。MEF25也显著降低（20.78±3.32 vs. 39.83±3.30mL.kg-1.s-1，P<0.05），表明存在下气道阻塞。雾化支气管扩张剂给药后，PFTs显示FVC（15.9%）和MEF25（200%）均较对照值显著增加，而Crs和Rrs无变化。这些发现表明，CDH新生儿存在限制性肺缺陷，反映了肺发育不全。手术修复和机械通气后气道反应性出现，主要在较小气道，这可能使术后病程复杂化。
