de Mouzon-Cambon A, Bouissou F, Dutau G, Barthe P, Parra M T, Sevin A, Ohayon E
Tissue Antigens. 1981 May;17(5):518-24. doi: 10.1111/j.1399-0039.1981.tb00739.x.
Idiopathic nephrotic syndrome (INS) of childhood is likely to be underlain by an immunopathological mechanism; we investigated the presence of immunogenetic HLA markers in this disease. Fifty-four unrelated INS-affected children, among them 20 with an allergic status, were studied for 33 HLA-A,B and 6 HLA-DR antigens. The results were compared to those obtained in 49 children with glomerulonephritis, 28 children with atopy but without nephropathy, and 91 healthy blood donors. The HLA-A and B antigen frequencies were not significantly different from normal frequencies. The incidence of HLA-DR7 was significantly increased in INS-affected patients as compared to the other groups (66.7% in patients vs 31.1% in healthy controls; corrected P value less than 0.001; relative risk = 4.4), and more so in those with atopy than in those without atopy (90% vs 46%; P = 0.002). The frequency of this antigen is not increased in atopic non-nephrotic children. No relationship between HLA-DR7, clinical outcome and steroid-responsiveness was found. We suggest that the pathogenesis of INS could be influenced by an HLA-linked immune response gene, especially in its atopy associated form.
儿童特发性肾病综合征（INS）可能存在免疫病理机制；我们研究了该疾病中免疫遗传HLA标志物的存在情况。对54名无亲缘关系的INS患儿进行了研究，其中20名有过敏状态，检测了33种HLA - A、B抗原和6种HLA - DR抗原。将结果与49名肾小球肾炎患儿、28名有特应性但无肾病的患儿以及91名健康献血者的结果进行比较。HLA - A和B抗原频率与正常频率无显著差异。与其他组相比，INS患儿中HLA - DR7的发生率显著增加（患者中为66.7%，健康对照中为31.1%；校正P值小于0.001；相对风险 = 4.4），有特应性的患儿比无特应性的患儿增加得更多（90%对46%；P = 0.002）。该抗原频率在有特应性的非肾病患儿中未增加。未发现HLA - DR7与临床结局及激素反应性之间存在关联。我们认为，INS的发病机制可能受HLA相关免疫反应基因的影响，尤其是在其与特应性相关的形式中。
