Yao Yong, Liu Jing-cheng, Xiao Hui-jie, Huang Jian-ping, Yang Ji-yun
Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.
Zhonghua Er Ke Za Zhi. 2004 Jun;42(6):412-6.
To understand the clinical and pathological characteristics of IgA nephropathy (IgAN) with crescentic formation in children.
Clinicopathological data of 29 children with IgAN accompanied by crescents were analyzed. These patients were divided into two groups according to the percentage of glomeruli affected by crescents more or less than 50%, and their data were compared.
(1) CLINICAL FEATURES: all the patients had hematuria and proteinuria, and macrohematuria (86%) and proteinuria were also common, protein excreted in urine was more than 1 g per day in 76% of the patients. The patients with edema, hypertension, and renal insufficiency were less than fifty percent. Nine patients in Group A (glomeruli affected by crescents > or = 50%) were crescentic IgAN. Significantly more cases in Group A had persistent macrohematuria, hypertension and renal failure than in Group B (glomeruli affected by crescents < 50%) (P < 0.05), with especially severe proteinuria (P < 0.01). It was easy to find nephritic syndrome in Group A, and asymptomatic hematuria combined with proteinuria in Group B. (2) Renal pathology: the glomeruli were affected by crescents from 5% to 85%. There were 52% to 85% in Group A, and 5% to 40% in Group B. Most crescents were cellular. All the cases had a diffuse mesangial proliferation and tubular-interstitial injury to different degree. Three cases had crescentic IgAN. Glomerulosclerosis was significantly more often seen in Group A (P < 0.05) and tuft adhesion was more frequently seen in Group B (P < 0.05). (3) Immunofluorescence: All the patients presented deposition of IgA, IgM and C3. There were 45% specimens combined with the deposition of IgG. Five cases showed 'full house' (17%), four of them were in Group A. None had IgA deposition alone.
The main clinical feature of IgAN with crescentic formation were hematuria combined with proteinuria, especially persistent gross hematuria and severe proteinuria. All of them showed diffuse mesangial proliferation and tubular-interstitial injury in morphology of kidney. Most of them had tuft adhesion. The main type of immunofluorescence were IgA + IgM and IgA + IgM + IgG deposition. Some showed 'full house' phenomenon. The clinical manifestation and renal lesions of IgAN with diffuse crescentic formation were worse than IgAN with glomeruli affected by crescents < 50%.
了解儿童IgA肾病（IgAN）伴新月体形成的临床及病理特征。
分析29例伴有新月体的儿童IgAN患者的临床病理资料。根据新月体累及肾小球的比例是否超过50%将这些患者分为两组，并比较两组数据。
（1）临床特征：所有患者均有血尿和蛋白尿，肉眼血尿（86%）和蛋白尿也较为常见，76%的患者每日尿蛋白排泄量超过1g。水肿、高血压和肾功能不全的患者不到50%。A组（新月体累及肾小球≥50%）9例为新月体性IgAN。A组持续性肉眼血尿、高血压和肾衰竭的病例明显多于B组（新月体累及肾小球<50%）（P<0.05），蛋白尿尤其严重（P<0.01）。A组易出现肾病综合征，B组易出现无症状血尿合并蛋白尿。（2）肾脏病理：肾小球新月体累及率为5%～85%。A组为52%～85%，B组为5%～40%。大多数新月体为细胞性。所有病例均有不同程度的弥漫性系膜增生和肾小管间质损伤。3例为新月体性IgAN。A组肾小球硬化明显多见（P<0.05），B组毛细血管袢粘连多见（P<0.05）。（3）免疫荧光：所有患者均有IgA、IgM和C3沉积。45%的标本合并有IgG沉积。5例表现为“满堂亮”（17%），其中4例在A组。无单独IgA沉积病例。
IgAN伴新月体形成的主要临床特征为血尿合并蛋白尿，尤其是持续性肉眼血尿和严重蛋白尿。肾脏形态学上均表现为弥漫性系膜增生和肾小管间质损伤。多数有毛细血管袢粘连。免疫荧光主要类型为IgA+IgM和IgA+IgM+IgG沉积。部分表现为“满堂亮”现象。弥漫性新月体形成的IgAN的临床表现和肾脏病变比新月体累及肾小球<50%的IgAN更严重。
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