Connelly Annette, Powell Harley R, Chan Yuen F, Fuller David, Taylor Russell G
Department of Nephrology, Royal Children's Hospital, Parkville 3052, Melbourne, Victoria, Australia.
Pediatr Nephrol. 2005 Apr;20(4):516-8. doi: 10.1007/s00467-004-1754-z. Epub 2005 Feb 3.
Kimura disease is a rare inflammatory condition of unknown aetiology. It typically presents in young Asian males with the triad of non-tender subcutaneous swellings in the head and neck region, peripheral eosinophilia and raised serum IgE. About 16% of cases have associated renal disease. We present the case of a 10-year-old boy with a past history of steroid responsive, frequently relapsing nephrotic syndrome who developed a right submandibular swelling and eosinophilia. Kimura disease was diagnosed on the basis of clinical and histological findings. The condition recurred during relapses of nephrotic syndrome. Because of poor adherence with oral medication, our patient was treated with intravenous vincristine with synchronous remissions of his nephrotic syndrome and Kimura swellings on each occasion.
木村病是一种病因不明的罕见炎症性疾病。它通常见于年轻亚洲男性,表现为头颈部无痛性皮下肿胀、外周血嗜酸性粒细胞增多和血清IgE升高三联征。约16%的病例伴有肾脏疾病。我们报告一例10岁男孩,既往有类固醇反应性、频繁复发的肾病综合征病史,出现右侧下颌下肿胀和嗜酸性粒细胞增多。根据临床和组织学检查结果诊断为木村病。该疾病在肾病综合征复发时复发。由于口服药物依从性差,我们的患者接受了静脉注射长春新碱治疗,每次肾病综合征和木村病肿胀均同步缓解。