Albafouille V, Sayegh N, De Coudenhove S, Scheinmann P, De Blic J, Mamou-Mani T, Hassine A, Jaubert F, Brunelle F
Department of Paediatric Radiology, Hôpital Necker-Enfants Malades, Paris, France.
Pediatr Radiol. 1999 Mar;29(3):147-52. doi: 10.1007/s002470050560.
To study computed tomographic (CT) findings in children with pulmonary alveolar proteinosis (PAP) more extensively.
To describe the CT features at the time of diagnosis and after therapeutic broncho-alveolar lavage (BAL).
We retrospectively reviewed the CT scans of five children (aged 3 months to 4 years) examined because of incidental bronchitis (n = 1), disease in a sibling (n = 1) and relapsing fever, cough and dyspnoea (n = 3). Each patient had an initial CT scan. Two asymptomatic cases were not treated but were followed up by plain chest films. The other three had BAL and follow-up CT.
Initial CT in all cases showed a diffuse reticulomicronodular pattern associated in three cases with posterior bilateral alveolar infiltrates. CT in the two asymptomatic patients remained unchanged or slightly improved without BAL. After BAL, a variable decrease of lung infiltrates was observed.
Correlation between the extent of alveolar consolidation and severity of disease was found. Anatomical and pathological considerations allow us to consider that the classical reticulomicronodular pattern is not due to an interstitial infiltration but to alveoli filled with the abnormal material characteristic of PAP.
更广泛地研究儿童肺泡蛋白沉积症（PAP）的计算机断层扫描（CT）表现。
描述诊断时及治疗性支气管肺泡灌洗（BAL）后的CT特征。
我们回顾性分析了5例儿童（年龄3个月至4岁）的CT扫描结果，这些儿童因偶然的支气管炎（n = 1）、同胞患病（n = 1）以及复发性发热、咳嗽和呼吸困难（n = 3）而接受检查。每位患者均进行了首次CT扫描。2例无症状病例未接受治疗，但通过胸部平片进行随访。另外3例接受了BAL及随访CT检查。
所有病例的首次CT均显示弥漫性网状微结节影，3例伴有双侧后部肺泡浸润。2例无症状患者未经BAL治疗，CT表现无变化或略有改善。BAL后，观察到肺部浸润有不同程度的减轻。
发现肺泡实变程度与疾病严重程度之间存在相关性。从解剖学和病理学角度考虑，我们认为典型的网状微结节影并非由于间质浸润，而是由于肺泡内充满了PAP特有的异常物质。
