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目的 分析儿童丙基硫氧嘧啶 (PTU)致抗中性粒细胞胞浆抗体 (ANCA)阳性新月体肾炎的临床、病理特点及发病机制。方法 对 1例PTU致ANCA阳性新月体肾炎患儿的临床、实验室资料、病理进行分析 ,并复习相关文献。结果 本例ANCA阳性新月体肾炎患儿有PTU服用史 ;临床上有血尿、蛋白尿、肾功能损害 ,贫血 ,咳嗽、咯血痰、肺部湿音 ,ANCA阳性 ,P ANCA、抗单氧化酶阳性 ,血沉快 (70mm/h) ,血γ 球蛋白增高 ,抗肾小球基底膜抗体阴性 ;肾脏病理改变以细胞性新月体为主 ,免疫荧光IgG(++) ,IgA(+) ,IgM、C3 、纤维蛋白均阴性。经皮质激素治疗 ,肾功能恢复正常 ,临床表现改善。结论 PTU可致儿童ANCA阳性新月体肾炎 ,其临床表现、肾脏病理改变与成人相似 ,但有其自身特点。
Objective To analyze the clinical, pathological features and pathogenesis of propylthiouracil (PTU) -resistant neutrophil cytoplasmic antibody (ANCA) -positive crescentic nephritis in children. Methods A case of ANCA-positive crescentic glomerulonephritis caused by PTU was analyzed in clinical and laboratory data and pathology, and the related literatures were reviewed. Results The patients with ANCA positive crescentic glomerulonephritis had PTU taking history. The patients had clinically hematuria, proteinuria, renal dysfunction, anemia, cough, hemoptysis sputum, wet lung sounds, ANCA positive, P ANCA, (70mm / h), increased serum γ-globulin and anti-glomerular basement membrane antibody; the pathological changes of renal were mainly crescentus, immunofluorescence IgG (++), IgA , IgM, C3, fibrin were negative. Corticosteroid therapy, renal function returned to normal, clinical manifestations improved. Conclusions PTU can cause ANCA positive crescentic glomerulonephritis in children. The clinical manifestations and renal pathological changes are similar to those in adults, but have their own characteristics.