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題 名 | Persistent Hypertransaminasemia as the Presenting Findings of Muscular Dystrophy in Childhood=以持續高血清胺基轉移酶為肌肉失養症之臨床表現 |
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作 者 | 林裕誠; 李旺祚; 黃秀芬; 楊千立; 王本榮; 沈友仁; | 書刊名 | 臺灣兒科醫學會雜誌 |
卷 期 | 40:6 民88.11-12 |
頁 次 | 頁424-429+466 |
分類號 | 415.9413 |
關鍵詞 | 高血清胺基轉移酶; 肌肉失養症; 肌肉切片; Hypertransaminasemia; Muscular dystrophy; Muscle biopsy; Distrophin; |
語 文 | 英文(English) |
中文摘要 | 血清中胺基丙酸胺基轉移醃 (Alanine aminotransferase)和天門冬胺酸胺基轉移 醃 (Aspartate aminotransferase) 的持續升高經常是由肝臟疾病所造成。然而,這些醜存 在於許多肝臟以外的組織中,其中也包括了骨骼肌。 五個孩童 (全為男性 ) 由於血清中胺 基轉移懶的持續升高,轉診至台大醫院小兒部。這些孩童的平均年齡介於四月大與五歲半之 間。其神經學檢查並無明顯變化。最初的胺基丙酸胺基轉移瞞和天門冬胺酸胺基轉移棚值分 別為 114 至 581 U/L 與 183 至 700 U/L。在追蹤了 0 至 30 個月不等,血清中肌胺酸酥 活化醣 (Creatine kinase) 才被檢查站發現有明顯上升 (範圍, 10557 至 62508 U/ L)。 這五名孩童的肌肉切片全都顯示出壞死及再生的肌肉纖維與組織問的纖維化。在第三,四與 第五病童,完全沒有 Dystrophin 的免疫反應活性。基因檢查顯示 DMD 基因的缺失 [ 外基 因 (Exons)45 至 48,第二病童; 外基因 49 至 50,第四病童 ]。 這個經驗指出在不明原 因的長期血清中胺基轉移醣待續升高,潛在的肌肉疾病必須列人考慮。且須及早檢測血清中 肌胺酸酣涵化倆與肌肉切片檢查以得到正確的診斷。 |
英文摘要 | Prolonged elevation of the serum alanine aminotransf erase (ALT) and aspartate ami notransfe rase (AST) is often attributed to hepatic diseases. However, these enzymes are also present in a variety of extrahepatic tissues, including skeletal muscle. Five children (all boys) were referred to the pediatric department of the National Taiwan University Hospital because of persistent elevation of serum aminotransferase activities. The ages of these children were between 4 months and 5.5 years. The neurological findings were all not remarkable. The initial ALT and AST values were 1 14-581 U/L and 183-700 U/L, respectively. Serum creatine kinase was checked first after 0 to 30 months follow-up and found to be markedly elevated (range, 10557 U/L to 62508 U/L). Muscle biopsies in the five cases all showed degenerating and regenerating myofibers with interstitial fibrosis. In Cases 3. 4 and 5, complete absence of dystrophin immunoreactivity was found. Genetic studies showed deletions in the DMD gene (exons 45-48 in case 2 and 49-50 in case 4). This experience indicates that occult muscle diseases should be taken into account in patients with unexplained long-lasting hypertransaminasemia and therefore measurement of serum creatine kinase activity and muscle biopsy should be done early for the correct diagnosis of muscular dystrophy. |
本系統中英文摘要資訊取自各篇刊載內容。