Saleem M A, Milford D V, Raafat F, White R H
Department of Nephrology, Children's Hospital, Birmingham, UK.
Pediatr Nephrol. 1993 Jun;7(3):256-8. doi: 10.1007/BF00853212.
Malakoplakia is an inflammatory condition associated with persisting bacterial antigen in macrophages and characterized histologically by the Michaelis-Gutmann body, containing bacterial fragments. We review the pathogenesis of malakoplakia and report a novel form of treatment successfully used in an 8-week-old infant with bilateral renal malakoplakia. The patient presented with an acute Escherichia coli urinary tract infection and enlarged kidneys. Antibiotic regimes were ineffective, but once the diagnosis was made treatment was changed to an immunosuppressive regime of prednisolone and azathioprine, to which she responded promptly. Renal malakoplakia should be considered in any patient with a urinary tract infection unresponsive to antibiotics and enlarged kidneys. Although a large proportion of patients with malakoplakia have an underlying systemic disorder, which may account for their abnormal macrophage function, the rest demonstrate either an isolated macrophage defect or no detectable anomaly at all. It is in this latter group we suggest that an immunomodulating regime can be curative.
软斑病是一种与巨噬细胞中持续存在的细菌抗原相关的炎症性疾病,组织学特征为存在包含细菌碎片的米氏小体。我们回顾了软斑病的发病机制,并报告了一种成功用于一名8周大双侧肾软斑病婴儿的新型治疗方法。该患者表现为急性大肠杆菌尿路感染和肾脏肿大。抗生素治疗无效,但一旦确诊,治疗改为泼尼松龙和硫唑嘌呤的免疫抑制方案,患者对此迅速做出反应。对于任何患有对抗生素无反应的尿路感染和肾脏肿大的患者,都应考虑肾软斑病。虽然大部分软斑病患者存在潜在的全身性疾病,这可能解释了他们巨噬细胞功能异常,但其余患者要么表现为孤立的巨噬细胞缺陷,要么根本没有可检测到的异常。我们认为正是在后一组患者中,免疫调节方案可能具有治愈作用。