Robert J, Souillet G, Chapuis-Cellier C, Ghipponi J, Bienvenu J, Frobert Y, Carron R
Nouv Presse Med. 1979;8(33):2659-62.
Among 998 children with recurrent respiratory diseases 26 children with selective IgA deficiency were found. Three groups were considered according to IgA level in serum: group I with IgA under 0.05 g per litre; group II with IgA between 0.05 and 0.3 g per litre; group III with IgA above 0.3 and under 1 g per litre. Non specific immunity was studied in these patients including immunoglobulin levels, alpha-1-antitrypsin (A.A.T.) phenotypes, phagocytosis of staphylococcus aureus by PMN, lysozyme level, complement system. Cellular immunity was evaluated by IDR tests and rosette forming cells (RE). Only non specific immune systems were disturbed in some patients and appeared as aggravating factors in IgA deficient patients. We found: Abnormal phenotypes of ATT in 11 cases; deficiencies of engulfment in 6 cases, of bactericidal activities of PMN in 7 cases out of 16 studied; decrease of lysozyme level in 4 cases out of 17 studied; increase of IgE level in 9 cases with atopic symptoms in 7 patients. In our experience the chief aggravating factor in IgA deficient patients is abnormal phenotype of AAT.
在998名患有复发性呼吸道疾病的儿童中,发现了26名选择性IgA缺乏症患儿。根据血清中IgA水平将患儿分为三组:第一组IgA低于0.05克/升;第二组IgA在0.05至0.3克/升之间;第三组IgA高于0.3克/升且低于1克/升。对这些患者的非特异性免疫进行了研究,包括免疫球蛋白水平、α-1-抗胰蛋白酶(A.A.T.)表型、中性粒细胞对金黄色葡萄球菌的吞噬作用、溶菌酶水平、补体系统。通过迟发型超敏反应试验和玫瑰花结形成细胞(RE)评估细胞免疫。仅部分患者的非特异性免疫系统受到干扰,并在IgA缺乏症患者中表现为加重因素。我们发现:11例AAT表型异常;16例中有6例吞噬功能缺陷,7例中性粒细胞杀菌活性缺陷;17例中有4例溶菌酶水平降低;7例有特应性症状的患者中有9例IgE水平升高。根据我们的经验,IgA缺乏症患者的主要加重因素是AAT表型异常。