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青少年慢性关节炎中的淀粉样变性:一项发病率和死亡率研究。

Amyloidosis in juvenile chronic arthritis: a morbidity and mortality study.

作者信息

David J, Vouyiouka O, Ansell B M, Hall A, Woo P

机构信息

MRC, Clinical Research Centre, Northwick Park Hospital, Harrow, UK.

出版信息

Clin Exp Rheumatol. 1993 Jan-Feb;11(1):85-90.

PMID:8453805
Abstract

A retrospective study of 79 juvenile arthritic patients with reactive amyloidosis for a mean of 10 years (3 months-24.25 years) from the onset of amyloidosis was performed. Eighty percent of those treated with chlorambucil (n = 57) were alive compared with 23.5% of patients not treated with chlorambucil (n = 19) 10 years after diagnosis. Renal failure was the cause of death in 82.3% and infection in 11.7%. Side effects included one chlorambucil-treated patient who developed acute leukaemia, and seven patients who developed severe leucopenia and four thrombocytopenia. Fifteen patients are no longer on cytotoxic therapy and are in remission. Analysis of their fertility status showed that there were 5 normal births in 3 women and 2 terminations of pregnancy in 23 chlorambucil-treated women of child bearing age. Six women had ovarian failure. None of the male patients fathered a child.

摘要

对79例患有反应性淀粉样变性的青少年关节炎患者进行了回顾性研究,从淀粉样变性发病起平均随访10年(3个月至24.25年)。诊断10年后,接受苯丁酸氮芥治疗的患者(n = 57)中有80%存活,而未接受苯丁酸氮芥治疗的患者(n = 19)中这一比例为23.5%。82.3%的患者死于肾衰竭,11.7%死于感染。副作用包括1例接受苯丁酸氮芥治疗的患者发生急性白血病,7例患者出现严重白细胞减少,4例出现血小板减少。15例患者不再接受细胞毒性治疗且病情缓解。对其生育状况的分析显示,3名女性中有5例正常分娩,23名接受苯丁酸氮芥治疗的育龄女性中有2例终止妊娠。6名女性出现卵巢功能衰竭。男性患者均未生育子女。

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