摘要目的:探讨儿童原发性IgA肾病(IgAN)临床表现和病理改变与免疫荧光之间的相关性。方法:收集近10年江西省儿童医院肾脏科确诊为原发性IgA肾病222例患儿资料，分析其临床和病理特点及相关性。结果:1．免疫荧光显示IgA仅伴系膜区沉积的患儿最多[115例(51.8%)]，其余为同时伴毛细血管袢沉积[107例(48.2%)]；IgA免疫荧光强度＋＋的患儿最多[122例(55.0%)]；IgA伴IgM沉积患儿最多[168例(75.7%)]，其次为伴C 3沉积[160例(72.1%)]；伴C 4沉积最少[7例(3.2%)]。2.高血压与免疫荧光伴IgM、C 3沉积、IgA沉积强度及IgA伴血管袢沉积均呈正相关(均 P<0.05)；高尿酸血症与伴IgM、IgG、C 3沉积及IgA伴血管袢沉积均呈正相关(均 P<0.05)；低蛋白血症与伴IgM、C 3沉积、IgA沉积强度及IgA伴血管袢沉积均呈负相关(均 P<0.05)；高胆固醇血症与伴C 3沉积及IgA伴血管袢沉积均呈正相关(均 P<0.05)；尿蛋白定量与伴IgM、IgG及IgA伴血管袢沉积均呈正相关(均 P<0.05)；估算肾小球滤过率(eGFR)与IgA伴血管袢沉积呈负相关( P<0.05)。3.Lee氏分级与免疫荧光伴IgM及C 3沉积、IgA沉积强度及IgA伴毛细血管袢沉积呈正相关(均 P<0.05)。4.牛津分型中系膜细胞增生(M1)与免疫荧光伴C 3沉积、IgA沉积强度均呈正相关(均 P<0.001);毛细血管内皮增生(E1)病变与IgA沉积强度及IgA伴毛细血管袢沉积均呈正相关(均 P<0.05)；节段肾小球硬化或黏连(S1)病变与免疫荧光病理无相关性；肾小管萎缩/间质纤维化(T1)与伴IgG及C 3沉积均呈正相关(均 P<0.05)。5.球性硬化与免疫荧光病理无相关性；新月体与免疫荧光伴IgM、IgG、C 3沉积、IgA沉积强度及IgA毛细血管袢沉积均呈正相关(均 P<0.05)；肾内动脉增厚与伴IgG沉积及IgA毛细血管袢沉积均呈正相关性(均 P<0.05)。 结论:儿童原发性IgAN免疫荧光IgA强度以＋＋为主，伴IgM沉积最多见；IgA伴毛细血管袢沉积或伴C 3沉积的临床表现及光镜病理更重。

abstractsObjective:To investigate the correlation among clinical manifestations, pathological changes and immunofluorescence in children with primary IgA nephropathy (IgAN).Methods:The data of a total of 222 cases diagnosed with IgAN by the Department of Nephrology, Jiangxi Children′s Hospital in recent 10 years were collected for the analysis of clinical and pathological features and their correlation.Results:(1)Immunofluorescence showed that 115 cases (51.8%) IgA patients had only mesangial deposition, and 107 cases (48.2%) IgA patients had both mesangial deposition and capillary loop deposition.Most IgA patients (122 cases, 55.0%) had immunofluorescence intensity+ + .In IgA patients, IgM deposition was the most common [168 cases (75.7%)], followed by C 3 deposition [160 cases (72.1%)]. Patients with C 4 deposition were the least[7 cases (3.2%)]. (2) Hypertension were positively correlated with IgM, C 3 deposition, IgA deposition intensity and IgA with vascular loop deposition(all P<0.05). Hyperuricemia was positively correlated with IgM deposition, IgG deposition, C 3 deposition and IgA with vascular loop deposition(all P<0.05); hypoalbuminemia was negatively correlated with IgM deposition, C 3 deposition, IgA deposition intensity and IgA with vascular loop deposition(all P<0.05). Hypercholesterolemia were positively correlated with C 3 deposition and IgA with vascular loop deposition(all P<0.05). Urine protein quantification were positively related to IgM, IgG and IgA with vascular loop deposition (all P<0.05). The estimated glomerular filtration rate(eGFR)was negatively related to IgA with vascular loop deposition( P<0.05). (3) Lee′s grade were positively correlated with IgM and C 3 deposition, IgA deposition intensity and IgA with capillary loop deposition(all P<0.05). (4) Oxford type mesangial hypercellularity(M1)were positively correlated with C 3 deposition and IgA deposition intensity(all P<0.001). Endocapillary hypercellularity(E1)lesions were positively correlated with IgA deposition intensity and IgA with capilla-ry loop deposition(all P<0.05). Segmental glomerulosclerosis(S1)lesions had no correlation with immunofluorescence pathology.Tubular atrophy and interstitial fibrosis(T1)was positively correlated with IgG and C 3 deposition(all P<0.05). (5) There was no correlation between glomerulosclerosis and immunofluorescence pathology.Crescent was positively correlated with IgM deposition, IgG deposition, C 3 deposition, IgA deposition intensity and IgA capillary loop deposition(all P<0.05). Renal artery thickening was positively correlated with IgG deposition and IgA capillary loop deposition(all P<0.05). Conclusions:IgA intensity of + + and IgM deposition are most commonly found in immunofluorescence of children with primary IgAN.IgA patients with capillary loops or C 3 deposition have more severe clinical manifestations and light microscopy results.