儿童木村病合并肾脏损害病例报告并文献复习

A Child with Kimura’s Disease Had Multiple Lymph Nodes Enlargement as the Initial Sign: A Case Report and Literature Review

摘要: 目的：研究分析青岛大学附属医院1例儿童木村病合并肾脏损害的临床特征，诊断，治疗及预后，旨在对儿童木村病的诊疗提供一定的经验。方法：回顾性分析我院一名5岁男孩临床表现为肘部和腋窝淋巴结肿大，实验室检查表现为嗜酸性粒细胞和免疫球蛋白IgE显著增加，根据淋巴结病理切片，诊断为木村病。治疗起初用醋酸泼尼松治疗2周后，肿大的淋巴结完全消退，在泼尼松减量过程中，该患儿出现木村病复发，改用环孢菌素A，在随后的1年随访期间，症状逐渐显著改善。结论：针对木村病的治疗包括手术切除皮下肿块和药物治疗。尽管糖皮质激素是药物治疗的首选药物，但在糖皮质激素不敏感或不耐受的儿童中，免疫抑制剂如环孢菌素A可能是首选的替代药物。

Abstract: Objective: To analyze the clinical features, diagnosis, treatment and prognosis of a child with Mucun disease and kidney damage in a hospital affiliated to Qingdao University, aiming to provide some experience in the diagnosis and treatment of children’s Mucun disease. Methods: Retrospective analysis of a 5-year-old boy in our hospital showed clinical manifestations of elbow and axillary lymphadenopathy. Laboratory tests showed a significant increase in eosinophils and immunoglobulin IgE. According to the pathological section of the lymph nodes, the diagnosis was Mucun disease. After treatment for 2 weeks with prednisone acetate, the swollen lymph nodes completely resolved. During the prednisone reduction, the patient developed a recurrence of Mucun disease and switched to cyclosporin A for follow-up in the following 1 year. During the period, the symptoms gradually improved significantly. Conclusion: Treatment for Kimura disease includes surgical removal of subcutaneous masses and medication. Although glucocorticoids are the drug of choice for drug therapy, immunosuppressive agents such as cyclosporin A may be the preferred alternative in children with glucocorticoid insensitivity or intolerance.