Children's Hospital & Medical Center, Creighton University Medical School, Omaha, NE 68114, USA.
Pediatr Radiol. 2010 Mar;40(3):361-5. doi: 10.1007/s00247-009-1455-7. Epub 2009 Nov 10.
Although bronchopulmonary dysplasia (BPD) is a common cause of interstitial lung disease in chronically intubated premature neonates, other interstitial lung disease in nonintubated infants is rare. We present a case of pulmonary interstitial glycogenosis that developed in a nonintubated, 31-week gestation infant in whom infectious etiologies had been excluded. The infant was well initially and then developed respiratory distress at 18 days of life. Radiographs at first day of life were normal, but CT and radiographic findings at 18 days of life showed severe interstitial lung disease, mimicking BPD. Lung biopsy showed pulmonary interstitial glycogenosis. This entity is not well described in the pediatric radiology literature and is important to consider, as the condition is responsive to a course of corticosteroids.
虽然支气管肺发育不良（BPD）是慢性插管早产儿间质性肺疾病的常见原因，但非插管婴儿的其他间质性肺疾病很少见。我们报告了一例在未插管、31 周胎龄婴儿中发生的肺间质糖原沉积症，该婴儿已排除感染性病因。婴儿最初情况良好，但在 18 天时出现呼吸窘迫。生后第 1 天的 X 线片正常，但 18 天时的 CT 和 X 线片表现为严重的间质性肺疾病，类似于 BPD。肺活检显示肺间质糖原沉积症。儿科放射学文献中对此疾病描述不多，因此需要考虑，因为该疾病对皮质类固醇治疗有反应。
