Pulmonary interstitial glycogenosis in identical twins
Pulmonary interstitial glycogenosis in identical twins
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DOI：
10.1002/ppul.20255
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发表时间：
2005-10-01
影响因子：
3.1
通讯作者：
Geukers, VGM
中科院分区：
文献类型：
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作者：
Onland, W;Molenaar, JJ;Geukers, VGM
我们报告了6岁和8周呼吸窘迫的单卵早产男性双胞胎的临床、放射学和病理结果。放射学检查在胸片上显示网状结节样间质，高分辨率计算机断层扫描（HRCT）显示两个婴儿的毛玻璃混浊和间隔增厚。肺活检显示相似组织学，肺泡间隔间质细胞弥漫性增厚，未见2型肺细胞明显增生，无空腔渗出物。切片间质细胞胞浆内周期性酸希夫（PAS）阳性，提示存在糖原。诊断为肺间质性糖原症。两名婴儿均接受糖皮质激素治疗，结果良好。我们推测肺间质性糖原症可能是婴儿慢性肺病（CLD）的一种组织病理学形式。
We present the clinical, radiological, and pathological findings of open lung biopsies from monozygotic prematurely born male twins with respiratory distress at ages 6 and 8 weeks postnatally Radiological examination showed a reticular nodular interstititial pattern on chest radiography High-resolution computed tomography (HRCT) revealed ground-glass opacification and thickened interstitial septae in both infants. Lung biopsies showed a similar histology There was diffuse interstitial thickening of the alveolar septa by mesenchymal cells, without prominent hyperplasia of type 2 pneumocytes, and without airspace exudates. Sections were periodic acid-Schiff (PAS)-positive within the cytoplasm of interstitial cells, indicating the presence of glycogen. Thus the diagnosis of pulmonary interstitial glycogenosis was made. Both infants were treated with glucocorticoids and had a favorable outcome. We speculate that pulmonary interstitial glycogenosis could be a histopathological form of chronic lung disease (CLD) of infancy.