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目的 了解儿童伴有新月体形成的原发性IgA肾病的临床与病理特点。方法 对 2 9例伴新月体形成的原发性IgA肾病患儿的临床及病理资料进行分析 ,并依受新月体累及的肾小球比例分组比较 ,≥ 5 0 % (A组 ) ,9例 ;<5 0 % (B组 ) ,2 0例。结果 (1)临床方面 :2 9例均有血尿 +蛋白尿 ,尿蛋白≥ 1g/ 2 4h者 2 2例 (76 % )和肉眼血尿 86 % ,水肿、高血压、肾功能异常者均不及半数。A组以肾病综合征和急进性肾炎为主 ,持续性肉眼血尿、大量蛋白尿、高血压、肾功能衰竭均较B组明显 (P<0 0 5 )。B组无症状性血尿 +蛋白尿者 6 5 %。 (2 )病理方面 :新月体形成累及肾小球 5 %～ 85 % ,A组为 5 2 %～ 85 % (其中新月体型IgA肾病 10 % ) ,B组 5 %～ 4 0 % ,以细胞性为主。均有系膜增生和小管 间质病变 ,球囊粘连易见。两组比较 :A组系膜增生严重、小球硬化和小管灶状萎缩明显 (P <0 0 5 ) ,B组球囊粘连多见 (P <0 0 5 )。 (3)免疫荧光 :均有IgA +IgM +C3沉积 ,合并IgG沉积者 18例(6 2 % ) ,其中 5例 (17% )为“满堂亮”(A组占 4例 )。未见一例单纯IgA沉积。结论 伴有新月体形成的原发性IgA肾病临床均有血尿合并蛋白尿 ,以持续性肉眼血尿和大量蛋白尿为主 ;以弥漫性系膜增生为主要病理改变 ,易见球囊粘连和小管 间?
Objective To understand the clinical and pathological features of primary IgA nephropathy in children with crescent formation. Methods The clinical and pathological data of 29 patients with primary IgA nephropathy formed by crescentic crescent were analyzed. According to the group of glomerulus involved in crescentic corpus, ≥50% (group A) 9 cases; <50% (group B), 20 cases. Results (1) In clinical stage, 29 cases had hematuria + proteinuria, 22 cases (76%) had urinary protein ≥ 1g / 24 hours and 86% of gross hematuria. Edema, hypertension and renal dysfunction were less than half . A group with nephrotic syndrome and radical nephritis, persistent gross hematuria, massive proteinuria, hypertension, renal failure were significantly more than the B group (P <0 05). Group B asymptomatic hematuria + proteinuria 65%. (2) Pathology: The crescents involved 5% ~ 85% of glomeruli, 52% ~ 85% of A (10% of crescentic IgA nephropathy) and 5% ~ 40% of B Cell-based. Mesangial proliferation and tubulointerstitial lesions, balloon adhesion is easy to see. There was significant mesangial hyperplasia in group A, significant glomerular sclerosis and small tubular atrophy (P <0.05), and balloon occlusion in group B was more common (P <0.05). (3) Immunofluorescence: IgA + IgM + C3 deposits were found in 18 cases (62%) with IgG deposition, of which 5 cases (17%) were “full bright” (4 cases in group A). No single case of IgA deposition. Conclusions The clinical manifestations of primary IgA nephropathy associated with crescent formation are both hematuria and proteinuria, with persistent gross hematuria and massive proteinuria. Diffuse mesangial proliferation is the main pathological change. It is easy to see that the balloon adhesion and Small tube?