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目的 :探讨儿童Kimura病合并系膜增生性肾小球肾炎(mesangial proliferative glomerulonephritis,Ms PGN)的临床表现、诊断与治疗方法。方法:分析南京医科大学附属南京儿童医院肾脏科2010年收治的1例儿童Kimura病合并Ms PGN的临床和病理资料,并进行文献复习。结果:患儿表现为肾病综合征、颈部及耳后淋巴结肿大、左侧腮腺轻度肿大、嗜酸性粒细胞增多症、高Ig E血症。淋巴窦内见大量嗜酸性粒细胞浸润、嗜酸性微脓肿形成。肾脏病理为Ms PGN。血清Ig E在病初及肾病复发时显著增高,在肾病缓解期显著下降。参考相关文献发现9例儿童Kimura病伴发Ms PGN,均为男性,表现为肾病综合征。治疗方案包括单用糖皮质激素5例及联合其他免疫抑制剂治疗,这些免疫抑制剂包括环磷酰胺、他克莫司、苯丁酸氮芥。激素治疗敏感的7例,其中3例治疗过程中停药后复发。结论:儿童Kimura病合并Ms PGN,大部分激素治疗敏感,但停用免疫抑制剂后易复发,如激素耐药或肾病反复需联合其他免疫抑制剂治疗。血清Ig E的动态改变可能作为判断Kimura病肾内活动的早期指标。
Objective: To investigate the clinical manifestations, diagnosis and treatment of children with Kimura’s disease complicated with mesangial proliferative glomerulonephritis (Ms PGN). Methods: The clinical and pathological data of 1 case of Kimura disease combined with Ms PGN admitted to Department of Nephrology, Nanjing Children’s Hospital Affiliated to Nanjing Medical University in 2010 were reviewed and reviewed. Results: Children showed nephrotic syndrome, neck and ear lymph nodes, left parotid mild enlargement, hypereosinophilia, hyper IgA hyperlipidemia. Lymph sinus see a large number of eosinophil infiltration, eosinophilic abscess formation. Kidney pathology is Ms PGN. Serum Ig E was significantly higher in the early stage of disease and nephropathy relapse, and significantly decreased in the remission stage of nephropathy. Reference to the relevant literature found that 9 cases of children with Kimura disease associated with PGNP, are male, manifested as nephrotic syndrome. Treatment regimens include 5 cases of glucocorticoid alone and in combination with other immunosuppressive agents, including cyclophosphamide, tacrolimus, chlorambucil. Hormone therapy in 7 cases, of which 3 cases relapsed after treatment. Conclusion: Child Kimura disease complicated with Ms PGN is sensitive to most hormone therapy. However, it is easy to relapse after stopping the use of immunosuppressive drugs. For example, hormone resistance or nephropathy should be treated repeatedly with other immunosuppressive agents. The dynamic changes in serum IgE may be used as early indicators of renal disease in Kimura disease.