摘要
目的:分析不同类型IgA肾病(IgAN)的临床病理特点。方法:759例IgAN根据临床和病理特点分为:单纯性镜下血尿型(I-H,22例)、尿检异常型(U-ab,336例)、反复发作肉眼血尿型(R-GH,104例)、新月体型(Cres.,57例)、大量蛋白尿型(MP,86例)、高血压型(HT,149例)和终末期肾病型(ESRD,5例)等七种类型。回顾性分析不同类型IgA肾病的临床病理资料。结果:I-H型患者无高血压,无肾功能不全和贫血,肾间质无明显纤维化;U-ab型患者除有镜下血尿和蛋白尿外,约有5·8%的患者出现慢性肾功能不全,23%的患者有不同程度的肾间质纤维化;R-GH型患者扁桃体肿大发生率(15·4%)显著高于其他各型,血尿发作间期尿红细胞计数均值为(214±336)万/ml;Cres·型患者尿沉渣红细胞计数(405±485)万/ml在各型中最高,伴大量蛋白尿(占21·1%)、贫血(占31·5%)和血肌酐升高(占17·5%),病理上细胞型新月体和节段袢坏死的发生率均达50·9%,高于其它各型;MP型患者尿蛋白均值为(3·93±2·68)g/24h,伴低白蛋白(29·1±6·8)g/L、高胆固醇血症(6·99±2·67)mmol/L,尿液C3、NAG含量显著增高;HT型患者尿液小分子蛋白比例达10·8%,伴有明显的低渗尿,肾功能不全发生率为54·4%,肾小球球性硬化比例达(24·3±23·2)%;ESRD型则是各型发展后的终末状态,需行替代治疗。结论:IgAN是一组综合征,不同类型具有不同的病理生理机制。临床上对不同类型IgAN区别对待,将有助于提高治疗效果,加深对IgAN本质的认识。
Objective： To investigate the clinicopathological features in seven subtypes of IgA nephropathy （ IgAN ）. Methodology ： Based on the clinicopathological criteria, 759 cases of IgAN were divided into 7 subtypes. They were isolated microscopic hematuria （I-H ,22 ）, asymptomatic abnormal urinalysis （U-ab ,336 ）, recurrent gross hematuria （ R-GH, 104）, crescentic IgAN （ Cres. , 57 ）, mass proteinuria （ MP, 86）, hypertension （ HT, 149） and end-stage renal disease（ ESRD, 5 cases）. The retrospective analysis was made on the clinical and pathological data of different subtypes of IgAN. Results：In I-H, there was only microscopic hematuria [ （ 110 ± 100） ×10^4/ml], without hypertension, anemia and renal insufficiency. The incidence of chronic histological lesions was lower. In U-ab, there were both microscopic Hematuria [ （98 ± 195 ） ×10^4/ml] and proteinuria [ （0. 80 ± 0. 57）g/24h ]. 5.8% of patients were chronic renal insufficiency, and 23% were various degrees of renal interstitial fibrosis. In R-GH patients, the incidence of tonsillitis was 15.4%, which significantly higher than that in other patterns （ P 〈 0. 05 ）. The count of urine red blood cells during the interval stage of gross hematuria were （214 ±336） ×10^4/ml. In Cres. cases, urine red blood cells count [ （405 ±485） ×10^4/ml] ranked the highest, 21.1% of cases with mass proteinuria, 17. 5% with increasing serum creatinine, and 31.5% with anemia. The incidence of cellular crescents and segmental necrosis was 50.9% , which was more than that in any other patterns. In MP subtype, the mean of urinary protein excretion was （3.93 ± 2. 68 ）g/24h, associated with hypoalbuminaemia （29. 1 ± 6. 8 ）g/L and hyperlipidaemia （6.99 ± 2. 67 ）mmol/L. Furthermore, the urine C3 and NAG was also increased significantly. In HT patients, the incidence of low molecular weight proteinuria reached 10.8% , with hypotonic urine. The rate of renal insufficiency was high to 54. 4%, and the proportion of global sclerosis was （ 24. 3 ± 23.2 ） %. The ESRD subtype was a terminal condition of various subtypes of development, who needed replacement therapy. Conclusion. IgAN is a clinical syndrome with similar immunopathological features as well as different pathophysiological mechanisms. The different treatment in different subtypes of IgAN will improve the effect of treatment, and deepen the understanding of the nature of IgAN.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
2006年第5期409-415,共7页
Chinese Journal of Nephrology,Dialysis & Transplantation
关键词
IGA肾病
临床
病理
IgA nephropathy clinical pathological
