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与木村病相关的类固醇抵抗型肾病综合征。

Steroid-resistant nephrotic syndrome associated with Kimura's disease.

作者信息

Chartapisak Wattana, Opastirakul Sauwalak

机构信息

Renal Unit, Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Thailand.

出版信息

Am J Nephrol. 2002 Jul-Aug;22(4):381-4. doi: 10.1159/000065232.

Abstract

Kimura's disease is a chronic inflammatory disease characterized by tumor-like lesions in the soft tissue and lymph nodes of head and neck area or parotid gland. It has a high frequency of an association with nephrotic syndrome. Reported cases of nephrotic syndrome and Kimura's disease were mostly from adult patients with only 5 children mentioned. This study reports the case of a 15-year-old-boy who manifested with steroid-resistant nephrotic syndrome for 4 years. Pathological examination of the kidney revealed mild mesangial proliferation. Subsequently, he developed a soft-tissue mass in the parotid gland area. Histopathological investigation of the mass revealed eosinophilic infiltration together with plasma cells and mast cells which is a main characteristic of Kimura's disease. The patient, however, continued to have nephrotic-range proteinuria after removing the subcutaneous mass.

摘要

木村病是一种慢性炎症性疾病,其特征为头颈部软组织、淋巴结或腮腺出现肿瘤样病变。它与肾病综合征的关联频率较高。报道的肾病综合征合并木村病的病例大多来自成年患者,仅提及5例儿童患者。本研究报告了一名15岁男孩的病例,该男孩患有激素抵抗性肾病综合征4年。肾脏病理检查显示轻度系膜增生。随后,他在腮腺区域出现了一个软组织肿块。肿块的组织病理学检查显示有嗜酸性粒细胞浸润以及浆细胞和肥大细胞,这是木村病的主要特征。然而,切除皮下肿块后,该患者仍持续存在肾病范围的蛋白尿。

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