Peces R, Ablanedo P, Seco M
Servicio de Nefrología, Hospital Central de Asturias, Oviedo.
Nefrologia. 2002;22(5):486-91.
Chronic granulomatous disease is a group of syndromes which share a defect in a component of the phagocyte NADPH-oxidase complex. Without this enzyme activity, phagocytic cells cannot produce superoxide, peroxide, and other potent microbicidal radicals, and are less able to kill ingested pathogens. The clinical picture is characterised by recurrent life-threatening bacterial and fungal infections and abnormal tissue granuloma formation. On the other hand, amyloidosis is a systemic disease with renal involvement occurring in the majority of cases. Recurrent amyloidosis is a rare but well documented event in renal transplant recipients. However, graft loss secondary to amyloidosis has been noted infrequently. In addition, de novo amyloidosis has not been previously associated with graft loss. We report here a renal transplant recipient with chronic granulomatous disease and history of recurrent urinary tract infections, who developed nephrotic syndrome and progressive renal insufficiency secondary to de novo AA amyloidosis leading to graft loss 66 months after transplantation.
慢性肉芽肿病是一组综合征，其共同特征是吞噬细胞NADPH氧化酶复合物的一个成分存在缺陷。没有这种酶活性，吞噬细胞就无法产生超氧化物、过氧化物和其他强效杀菌自由基，杀灭摄入病原体的能力也会降低。临床表现为反复出现危及生命的细菌和真菌感染以及异常的组织肉芽肿形成。另一方面，淀粉样变性是一种大多数病例会累及肾脏的全身性疾病。复发性淀粉样变性在肾移植受者中虽罕见但有充分记录。然而，继发于淀粉样变性的移植肾丢失情况并不常见。此外，既往新发淀粉样变性与移植肾丢失并无关联。我们在此报告一名患有慢性肉芽肿病且有复发性尿路感染病史的肾移植受者，该患者在移植后66个月因新发AA型淀粉样变性继发肾病综合征和进行性肾功能不全，最终导致移植肾丢失。
