Husain A N, Hessel R G
Department of Pathology, Loyola University Medical Center, Maywood, Illinois 60153.
Pediatr Pathol. 1993 Jul-Aug;13(4):475-84. doi: 10.3109/15513819309048237.
Pulmonary hypoplasia (PH) is defined as defective or incomplete development of lungs that are immature for gestational age. A prospective study was done to establish practical criteria for the diagnosis of pulmonary hypoplasia and to determine the relative frequency of pulmonary hypoplasia and its associated congenital malformations. Postmortem, formalin-inflated, routinely processed lungs were examined for fresh lung weight, fixed lung volume, radial alveolar count, and tissue maturity. Of these, lung volume was found to be the least useful. However, no single parameter was adequate for diagnosis in every case. Using the remaining three in conjunction, reliable diagnostic criteria were established. In this series of 113 cases, 22% had pulmonary hypoplasia as the primary cause of death, of which one-third had no associated congenital malformations to account for the development of pulmonary hypoplasia. It was found that premature rupture of membranes (PROM) for as short as 5 days could lead to fatal PH. It is concluded that PH is a common problem in neonatal autopsies and can be diagnosed in the average laboratory. Recognizing PH is important because once it has developed, intrauterine intervention in cases of oligohydramnios and postnatal treatment with surfactant inhalation cannot influence the outcome. It remains to be seen whether early fluid replacement in PROM will prevent development of PH.
肺发育不全（PH）被定义为肺的发育存在缺陷或不完全，且相对于胎龄而言肺不成熟。进行了一项前瞻性研究，以建立肺发育不全的实用诊断标准，并确定肺发育不全及其相关先天性畸形的相对发生率。对尸检时用福尔马林充气并常规处理的肺进行新鲜肺重量、固定肺体积、肺泡计数和组织成熟度检查。其中，发现肺体积最无用。然而，没有一个单一参数能在每种情况下都足以用于诊断。综合使用其余三个参数，建立了可靠的诊断标准。在这113例病例系列中，22%以肺发育不全为主要死因，其中三分之一没有相关先天性畸形来解释肺发育不全的发生。发现胎膜早破（PROM）仅5天就可导致致命的肺发育不全。得出的结论是，肺发育不全是新生儿尸检中的常见问题，且在普通实验室即可诊断。认识到肺发育不全很重要，因为一旦其发生，羊水过少病例的宫内干预和出生后吸入表面活性剂治疗都无法影响结局。胎膜早破时早期补液是否能预防肺发育不全的发生仍有待观察。
