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秋水仙碱治疗家族性地中海热的AA型淀粉样变性。影响预后因素的分析。

Colchicine treatment of AA amyloidosis of familial Mediterranean fever. An analysis of factors affecting outcome.

作者信息

Livneh A, Zemer D, Langevitz P, Laor A, Sohar E, Pras M

机构信息

Sheba Medical Center, Tel-Hashomer, Israel.

出版信息

Arthritis Rheum. 1994 Dec;37(12):1804-11. doi: 10.1002/art.1780371215.

Abstract

OBJECTIVE

To elucidate factors possibly influencing the outcome of colchicine therapy in patients with amyloidosis of familial Mediterranean fever (FMF).

METHODS

Retrospective analysis of data abstracted from the charts of all 68 FMF patients with amyloidosis who presented during the study period (1974-1992) with proteinuria (> or = 0.5 gm/24 hours) and creatinine values < or = 2.5 mg/dl, received colchicine, and were followed up for > or = 5 years.

RESULTS

At the end of the study period, kidney disease had worsened in 31 patients and remained stable in 22. Proteinuria had regressed in 15 patients. Deterioration was related to initial serum creatinine values > or = 1.5 mg/dl (P < 0.01) and to mean colchicine dosage < or = 1.5 mg/day (P < 0.001). The 3 groups were comparable in terms of initial urinary protein levels, duration of proteinuria, presence of hypertension, occurrence of febrile attacks, sex distribution, and proportion of non-compliant patients.

CONCLUSION

The therapeutic dosage of colchicine for amyloidosis of FMF is > 1.5 mg/day. This dosage is effective only in patients with initial serum creatinine levels < 1.5 mg/dl.

摘要

目的

阐明可能影响家族性地中海热(FMF)淀粉样变性患者秋水仙碱治疗效果的因素。

方法

对研究期间(1974 - 1992年)出现蛋白尿(≥0.5克/24小时)且肌酐值≤2.5毫克/分升、接受秋水仙碱治疗并随访≥5年的所有68例FMF淀粉样变性患者的病历数据进行回顾性分析。

结果

在研究期末,31例患者的肾脏疾病恶化,22例保持稳定。15例患者的蛋白尿减轻。病情恶化与初始血清肌酐值≥1.5毫克/分升(P < 0.01)以及秋水仙碱平均剂量≤1.5毫克/天(P < 0.001)有关。三组在初始尿蛋白水平、蛋白尿持续时间、高血压的存在、发热发作的发生、性别分布以及不依从患者的比例方面具有可比性。

结论

FMF淀粉样变性的秋水仙碱治疗剂量>1.5毫克/天。该剂量仅对初始血清肌酐水平<1.5毫克/分升的患者有效。

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