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32例嗜酸性粒细胞增多性肌痛综合征患者的临床随访及免疫遗传学研究

Clinical follow-up and immunogenetic studies of 32 patients with eosinophilia-myalgia syndrome.

作者信息

Kaufman L D, Gruber B L, Gregersen P K

机构信息

Department of Medicine, State University of New York, Stony Brook 11794-8161.

出版信息

Lancet. 1991 May 4;337(8749):1071-4. doi: 10.1016/0140-6736(91)91717-9.

DOI:10.1016/0140-6736(91)91717-9
PMID:1673503
Abstract

31 patients with L-tryptophan-associated eosinophilia-myalgia syndrome (EMS) that developed during the United States outbreak in 1989 were followed up prospectively at a university hospital outpatient rheumatology clinic for 16 to 24 months from the onset of their illness. Another patient with EMS associated with L-tryptophan in 1988 was followed up for 30 months. 93% of the 28 survivors from the 1989 cohort continue to have symptoms affecting 1-4 organ systems (median 3) and 3 have died, so the disorder produces considerable morbidity and mortality. The chronic sequelae most often associated with long-term disability are sclerodermatous skin thickening (54%), sensorimotor polyneuropathy (61%), proximal myopathy (36%), and severe episodic myalgias (64%). Thrombocytopenia developed in 1 patient. HLA-class II typing revealed a non-significant trend towards an association with HLA-DR4. Early therapy with corticosteroids did not seem to prevent the development of chronic manifestations.

摘要

1989年美国爆发期间出现的31例与L-色氨酸相关的嗜酸性粒细胞增多性肌痛综合征(EMS)患者,在一所大学医院门诊风湿病诊所从发病起进行了16至24个月的前瞻性随访。另一名1988年患有与L-色氨酸相关的EMS的患者接受了30个月的随访。1989年队列中的28名幸存者中有93%仍有影响1至4个器官系统(中位数为3个)的症状,3人死亡,因此该疾病会导致相当高的发病率和死亡率。最常与长期残疾相关的慢性后遗症是硬皮病样皮肤增厚(54%)、感觉运动性多发性神经病(61%)、近端肌病(36%)和严重发作性肌痛(64%)。1例患者出现血小板减少症。HLA-II类分型显示与HLA-DR4存在无统计学意义的关联趋势。早期使用皮质类固醇治疗似乎无法预防慢性表现的发展。

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Clinical follow-up and immunogenetic studies of 32 patients with eosinophilia-myalgia syndrome.32例嗜酸性粒细胞增多性肌痛综合征患者的临床随访及免疫遗传学研究
Lancet. 1991 May 4;337(8749):1071-4. doi: 10.1016/0140-6736(91)91717-9.
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A case of L-tryptophan-induced eosinophilia-myalgia resulting in death.一例因L-色氨酸诱发嗜酸性粒细胞增多性肌痛导致死亡的病例。
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