Findings of High-Resolution Chest Computed Tomography in a Young Child with Type C Niemann-Pick Disease

罹患C型尼門匹克症幼兒之高解析胸部電腦斷層影像所見

彭信逢(Steven Shinn-Forng Peng) ； 李瑤華(Yiu-Wah Li) ； 胡務亮(Wuh-Liang Hwu)

《慈濟醫學雜誌》 10卷4期 (1998/12) Pp. 327-331

https://doi.org/10.6440/TZUCMJ.199812.0327

摘要

尼門匹克症是一罕見之脂肪儲存疾病，患病幼兒的胸部高解析度電腦斷層影像表現，文獻上罕有報告。本例為一歲兩個月大患C型尼門匹克症的兒童，其胸部高解析度電腦斷層影像以背側肋膜之不平滑增厚與廣泛性肺小葉間、肺小葉中央以及支氣管血管周圍等處間質增厚主要表現。本例顯示受C型尼門匹克症侵犯之肋膜與肺間質增厚在胸部高解析度電腦斷層影像較胸部普通片能更清楚顯現；而具廣泛性肺部X光影像間質變化卻少有肺部症狀的幼童，代謝性肺部疾病為可能原因之一。

關鍵字

尼門匹克症 ； 肺與肋膜 ； 電腦斷層影像

並列摘要

Niemann-Pick disease is a rare storage disease involving with deposition of lipids. Manifestations of pulmonary involvement in patients with this disease found using high-resolution computed tomography (HRCT) have been rarely reported in literature. We report findings of high-resolution computed tomography in a 14-month-old child suffering from type C Niemann-Pick desease. The major findings on HRCT of the chest were unevenly thickened dorsal pleura and diffusely thickened interlobular, centrilobular as well as peribronchovascular interstitium. These findings became unremarkable on the follow-up HRCT 6 months after the patient received bone marrow transplantation. Involvement of pulmonary interstitium and pleura in patients with type C Niemann-Pick disease is better demonstrated using HRCT that using chest roentgenogram. Diffuse interstitial involvement in a young child without pulmonary symptoms is suggestive of the possibility of metabolic storage disease.

並列關鍵字

Niemann-Pick disease ； pulmonary and pleura ； computed tomography