小儿高免疫球蛋白A血症及其相关疾病(附90例临床分析)

90例高IgA血症中,单纯高IgA血症56例,高IgA+IgG血症29例,高IgA+IgM血症5例。男:女为1.65:1,且以7岁以上儿童多见。其相关疾病为急/慢性肾小球肾炎(53.3％)、风湿热/风湿性心脏病(18.9％)、过敏性紫癜(6.7％)、其他自身免疫病(5.6％)、急/慢性感染性疾病(12.2％)和其它(3.3％)。63.3％的病例血清IgA在2～3g/L。IgA增高与血沉、ASO、ClC的增高和C_3的变化有关联,部分患者外周血淋巴细胞计数降低,淋转率降低,这均提示高IgA血症可能是一种非特异性的体液免疫反应过高的表现。其机理推测可能与机体潜在的调节性T细胞功能失常致多克隆B细胞活化有关。

HYPERIMMUNOGLOBULIN A SYNDROME AND ITS ASSOCIATED DISEASES IN CHILDREN

90 cases of hyperimmunoglobulin A Syndrome (hyper-IgA) were determined.Most of them were older than the age of 7 ; the ratio of male to famale was 1.65: 1 Among these children, sole hyper-IgA, was 56; accompanied with hyper-IgM, 29; and with hyper-IgM, 5 cases.Associated disorders of hyper-IgA included acute chronic glomerulonephritis(53.3%), rheumatic fever/rheumatic heart diseases (18.9%), Henoch-Schonleim purpura(6.7%), other autoimmunologic disorders (5.6%), acute/chronic infections (12.2%), and miscelaneous diseases (3.3%).63.3% of these cases had a serum level of IgA up to 2-3g/L.Hyper-IgA was associated with increase of ESR, ASO, CIC and abnormality of C_3.The count of PBL and the lymphocyte transformation were decreased in some patients.These findings could suggest that potential dysfunction of T.regulating cells result in proliferation and differentiation of polyclonal B cells initiated by non-specific stimulative factors.