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嗜酸性粒细胞增多性肌痛综合征

Eosinophilia-myalgia syndrome.

作者信息

Kazura J W

机构信息

Department of Medicine, Case Western Reserve University, Cleveland, Ohio.

出版信息

Cleve Clin J Med. 1991 May-Jun;58(3):267-70. doi: 10.3949/ccjm.58.3.267.

Abstract

The eosinophilia-myalgia syndrome is a newly described disease associated with ingestion of a contaminant or byproduct of the amino acid L-tryptophan. Patients typically present with intense myalgias, especially of the extremities, and commonly suffer from skin and subcutaneous manifestations (edema and induration of the skin, morphea-like lesions, pruritus). Less frequent findings are cardiorespiratory involvement (cough, dyspnea, pulmonary infiltrates) and neurologic disease (ascending polyneuropathy). Laboratory findings include blood eosinophilia (greater than 10(9) cells per liter), normal to slightly elevated serum aldolase levels, and negative studies for connective tissue diseases (normal erythrocyte sedimentation rate, negative antinuclear antibodies). Tissue damage in eosinophilia-myalgia syndrome is likely related to infiltration by eosinophils with subsequent release of toxic molecules such as major basic protein. Management in severely ill patients includes administration of corticosteroids.

摘要

嗜酸性粒细胞增多性肌痛综合征是一种新发现的疾病,与摄入氨基酸L-色氨酸的污染物或副产物有关。患者通常表现为剧烈肌痛,尤其是四肢,且常伴有皮肤和皮下表现(皮肤水肿和硬结、硬斑病样病变、瘙痒)。较少见的表现有心肺受累(咳嗽、呼吸困难、肺部浸润)和神经系统疾病(上行性多神经病)。实验室检查结果包括血液嗜酸性粒细胞增多(每升大于10⁹个细胞)、血清醛缩酶水平正常或略升高,以及结缔组织病相关检查阴性(红细胞沉降率正常、抗核抗体阴性)。嗜酸性粒细胞增多性肌痛综合征中的组织损伤可能与嗜酸性粒细胞浸润及随后释放主要碱性蛋白等毒性分子有关。重症患者的治疗包括使用糖皮质激素。

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