Nakamura Y, Funatsu Y, Yamamoto I, Yamana K, Nishimura T, Hosokawa Y, Fukuda S, Nakashima H, Tsunosue M, Hashimoto T
Arch Pathol Lab Med. 1985 May;109(5):441-4.
We report on four autopsy cases of Potter's syndrome associated with characteristic Potter's face, pulmonary hypoplasia, and renal agenesis or dysplasia. There were no pathologic differences in typical cases with bilateral renal agenesis and variant cases with renal dysplasia instead of renal agenesis. All cases showed bilateral pulmonary hypoplasias and immature lung microscopically. Hyaline membrane disease was found in three cases in spite of late gestation. In one case with renal dysplasia, disaturated phosphatidylcholine, a pulmonary surfactant phospholipid, was measured and found to be decreased in comparison with that in age-matched controls. This suggests that functionally immature lung may be one feature of Potter's syndrome that is similar to hyaline membrane disease.
我们报告了4例波特综合征的尸检病例，这些病例伴有典型的波特面容、肺发育不全以及肾缺如或发育异常。双侧肾缺如的典型病例与肾发育异常而非肾缺如的变异病例之间没有病理差异。所有病例显微镜下均显示双侧肺发育不全且肺不成熟。尽管孕周较晚，但仍有3例发现有透明膜病。在1例肾发育异常的病例中，对肺表面活性物质磷脂二饱和磷脂酰胆碱进行了测量，发现与年龄匹配的对照组相比有所降低。这表明功能不成熟的肺可能是波特综合征与透明膜病相似的一个特征。
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