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对13例系统性淀粉样变性患者进行二甲亚砜延长治疗。

Prolonged dimethylsulphoxide treatment in 13 patients with systemic amyloidosis.

作者信息

Ravid M, Shapira J, Lang R, Kedar I

出版信息

Ann Rheum Dis. 1982 Dec;41(6):587-92. doi: 10.1136/ard.41.6.587.

Abstract

Continuous oral dimethylsulphoxide (DMSO) treatment (7-15 g/day) was given to 3 patients with amyloidosis of familial Mediterranean fever (FMF), 3 patients with idiopathic amyloidosis, and 7 patients with secondary amyloidosis. The nephrotic syndrome and various degrees of renal insufficiency were the major clinical manifestation in all case. Renal function was used as the main parameter for evaluation of therapy. DMSO treatment for 7-16 months produced no effect in the FMF patients and in the patient with idiopathic amyloidosis; they all ran the predictable clinical course of their disease and either died of cardiac failure or have been maintained on chronic haemodialysis. In the 7 patients with secondary amyloidosis an unequivocal improvement of renal function was observed following 3-6 months of DMSO treatment. It was shown by a 30-100% rise of creatinine clearance and a decline in proteinuria. This new equilibrium has been maintained as long as DMSO was administered. No serious side effects of DMSO wee encountered. Mild nausea and an unpleasant breath odour were the patients' main concern. We conclude that a therapeutic trial with oral DMSO is warranted in all patients with secondary amyloidosis. This treatment is unpleasant but bears no exceptional risks. It may significantly prolong life, though its effect on amyloid deposits themselves is doubtful.

摘要

对3例家族性地中海热(FMF)淀粉样变性患者、3例特发性淀粉样变性患者和7例继发性淀粉样变性患者进行连续口服二甲基亚砜(DMSO)治疗(7 - 15克/天)。肾病综合征和不同程度的肾功能不全是所有病例的主要临床表现。肾功能被用作评估治疗效果的主要参数。对FMF患者和特发性淀粉样变性患者进行7 - 16个月的DMSO治疗均无效果;他们都经历了疾病可预测的临床病程,要么死于心力衰竭,要么一直依赖慢性血液透析维持生命。在7例继发性淀粉样变性患者中,经过3 - 6个月的DMSO治疗后,观察到肾功能有明确改善。表现为肌酐清除率提高30% - 100%,蛋白尿减少。只要持续给予DMSO,这种新的平衡状态就能维持。未发现DMSO有严重的副作用。患者主要担心的是轻微恶心和口气难闻。我们得出结论,对所有继发性淀粉样变性患者进行口服DMSO治疗试验是有必要的。这种治疗虽令人不适,但并无特殊风险。它可能显著延长患者生命,尽管其对淀粉样沉积物本身的作用尚不确定。

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本文引用的文献

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Dimethylsulphoxide-induced toxicity.二甲基亚砜诱导的毒性。
Lancet. 1980 Nov 8;2(8202):1004-6. doi: 10.1016/s0140-6736(80)92158-3.
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Amyloidosis: review of 236 cases.淀粉样变性:236例病例回顾
Medicine (Baltimore). 1975 Jul;54(4):271-99. doi: 10.1097/00005792-197507000-00001.
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