Pulmonary interstitial glycogenosis - A new variant of neonatal interstitial lung disease
Pulmonary interstitial glycogenosis - A new variant of neonatal interstitial lung disease
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DOI：
10.1164/rccm.2105139
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发表时间：
2002-06-01
影响因子：
24.7
通讯作者：
O'Brodovich, H
中科院分区：
文献类型：
--
作者：
Canakis, AM;Cutz, E;O'Brodovich, H
我们报告7例非典型新生儿肺部疾病的临床、影像学和病理学表现。所有7名婴儿在出生后第一个月的胸片上均表现为呼吸急促、低氧血症和弥漫性间质浸润伴肺过度充气。所有病例的肺活组织检查显示出相似的病理学，梭形细胞扩张的结节含有与糖原一致的过碘酸-希夫阳性的过碘酸酶不稳定物质。免疫组化染色显示这些细胞呈波形蛋白阳性，但白细胞共同抗原、溶菌酶和其他巨噬细胞标志物呈阴性。电子显微镜显示原始间质间充质细胞，细胞器少，单颗粒糖原丰富。在肺泡衬里细胞中观察到极少量糖原或无糖原。5例患者接受皮质类固醇激素冲击治疗，1例患者加用羟氯喹。7名婴儿中有6名显示出良好的临床结果。一名婴儿死于极端早产和支气管肺发育不良的并发症。3例随访至少6年的病例显示临床消退和影像学改善。我们建议将新生儿的肺间质性糖原累积症与其他形式的间质性肺病区分开来。由于肺间质细胞中通常不存在丰富的糖原，我们推测肺间质细胞分化异常。
We present the clinical, radiologic, and pathologic findings in lung biopsies from seven infants with atypical neonatal lung disease. All seven infants presented with tachypnea, hypoxemia, and diffuse interstitial infiltrates with overinflated lungs on chest radiographs in the first month of life. Lung biopsies from all cases showed similar pathology, with expansion of the interstitium by spindle-shaped cells containing periodic acid-Schiff positive diastase labile material consistent with glycogen. Immunohistochemical staining showed these cells to be vimentin positive but negative for leucocyte common antigen, lysozyme, and other macrophage markers. Electron microscopy revealed primitive interstitial mesenchymal cells with few cytoplasmic organelles and abundant monoparticulate glycogen. Minimal or no glycogen was seen in the alveolar lining cells. Five cases were treated with pulse corticosteroids; hydroxychloroquine was added in one case. Six of seven infants have shown a favorable clinical outcome. One infant died from complications of extreme prematurity and bronchopulmonary dysplasia. Three cases that have been followed for at least 6 years have shown clinical resolution and radiographic improvement. We propose the term "pulmonary interstitial glycogenosis" of the neonate for this new entity to be differentiated from other forms of interstitial lung disease. Because abundant glycogen is not normally found in pulmonary interstitial cells, we postulate an abnormality in lung cytodifferentiation involving interstitial mesenchymal cells.