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題名 | 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 |
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作者 | 彭信逢; 李瑤華; 胡務亮; Peng, Steven Shinn-forng; Li, Yiu-wah; Hwu, Wuh-liang; |
期刊 | 慈濟醫學 |
出版日期 | 19981200 |
卷期 | 10:4 1998.12[民87.12] |
頁次 | 頁327-331 |
分類號 | 417.59 |
語文 | eng |
關鍵詞 | 尼門匹克症; 肺與肋膜; 電腦斷層影像; Niemann-pick disease; Pulmonary and pleura; Computed tomography; |
英文摘要 | 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 disease. 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 than using chest roentgenogram. Diffuse interstitial involvement in a yound child without pulmonary symptoms is suggestive of the possibility of metabolic storage disease. (Tzu Chi Med J 1998; 10:327-331) |
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