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相關文獻
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題 名 | Transplantation for Adrenoleukodystrophy with HLA-A and B Nonidentical Paternal Marrow: Report of One Case=以人體白血球抗原不一致之父親骨髓移植於腎上腺腦白質退化不良症病人:一病例報告 |
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作 者 | 林宏琪; 林凱信; 王本榮; | 書刊名 | 中華民國小兒科醫學會雜誌 |
卷 期 | 39:4 民87.07-08 |
頁 次 | 頁260-263+280 |
分類號 | 417.653 |
關鍵詞 | 腎上腺腦白質退化不良症; 人體白血球抗原; 骨髓移植; Adrenoleukodystrophy; Human leukocyte antigen; Bone marrow transplantation; |
語 文 | 英文(English) |
中文摘要 | 一名十四歲罹患腎上腺腦白質退化不良症男孩,於本院接受未經減去 T 型淋巴球 處理之骨髓移植,以嘗試治療其嚴重神經症狀;骨髓來源為人體白血球抗原不一致,混合淋 巴球培養不反應之其父。於移植後追蹤期間,我們發現骨髓移植雖然未能遏阻其神經症狀繼 續惡化,但血漿中超長鏈脂肪酸過量情形確有改善。我們相信,在骨髓移植於臨床應用愈加 精進的今日,若腎上腺腦白質病變病人能於受侵害之早期接受骨髓移植,其預後應可大為改 善。此為臺灣第一例以骨髓移植治療腎上腺腦白質退化不良症之報告。 |
英文摘要 | We report the result of allogeneic bone marrow transplantation (BMT) in a 14-year-old boy who was neurologically severely involved with the childhood form of adrenoleukodystrophy (ALD) and received marrow from his HLA-A and B nonidentical, MLC-nonreactive paternal donor without T-cell depletion processing. Bone marrow transplantation corrected the excess content of very long chain fatty acid in plasma but did not arrest the deterioration of the neurological status during 3.5-year post-transplant follow-up period. Since partially matched or unrelated donors have been applied to clinical BMT successfully with current new techniques, ALD patients will have a better prognosis when they are transplanted in status of mild and early involvement. Our first experience may be helpful in more trials of BMT for genetic leukodystrophy in Taiwan. |
本系統中英文摘要資訊取自各篇刊載內容。