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頁籤選單縮合
題名 | Serial MR Images and MRS Following Treatment in a Newborn with Maple Syrup Urine Disease=腦部磁振造影及氫離子磁振頻譜用於追蹤楓糖尿症新生兒治療成效之案例 |
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作者 | 林彥夫; 劉孟宜; 趙垂勳; 趙美琴; 邱燕玉; 黎俊蔚; 蔣宜璋; 林威辰; 郭禹廷; Lin, Yen-fu; Liu, Meng-yi; Jaw, Twei-shiun; Chao, Mei-chyn; Chou, Yen-yu; Li, Chun-wei; Chang, chih-ching; Lin, Wei-chen; Kuo, Yu-ting; |
期刊 | Journal of Radiological Science |
出版日期 | 20120900 |
卷期 | 37:3 2012.09[民101.09] |
頁次 | 頁133-138+148 |
分類號 | 417.517 |
語文 | eng |
關鍵詞 | 腦部磁振造影; 氫離子磁振頻譜; 新生兒; 楓糖尿症; |
英文摘要 | Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic disorder affecting branched-chain amino acids. We report a newborn with MSUD established by neonatal metabolic screening at 2 days old which showed elevation of plasma level of leucine/isoleucine and valine. The magnetic resonance (MR) imaging at 16 days old revealed typical finding of high signal intensity lesions within the myelinated white matter areas on T2-weighted and diffusion-weighted images(DWI). Proton MR Spectroscopy (MRS) showed elevation of lactate (1.3 ppm) and BCAA/BCKA (0.9 ppm) peaks. The MRS findings in our case were well-corresponded to the change of serum levels of leucine, isoleucine, and valine by serial MR images and laboratory data following treatment. MR images and MRS provided specific diagnosis of MSUD and effective monitoring of the response following treatment and dietary restriction. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。