Pathogenesis of lipoid nephrosis: a disorder of T-cell function.
Pathogenesis of lipoid nephrosis: a disorder of T-cell function.
复制标题
DOI：
10.1016/s0140-6736(74)91880-7
复制
发表时间：
1974-09
期刊：
影响因子：
168.9
通讯作者：
R. Shalhoub
中科院分区：
文献类型：
--
作者：
R. Shalhoub
临床观察表明，类脂性肾病是由T细胞功能的全身异常引起的，导致分泌对免疫学上无辜的肾小球基底膜有毒的循环化学介质。缺乏体液抗体反应的证据，麻疹引起的缓解改变了细胞介导的免疫，类固醇和环磷酰胺的治疗益处也减弱了细胞介导的反应，以及霍奇金病中这种综合征的发生支持了这一假设。未经治疗的患者对肺炎球菌感染的易感性可能具有原发性或继发性发病的重要性。综上所述，这些数据表明，该综合征是一种自限性原发性免疫缺陷病的临床表现。
Clinical observations suggest that lipoid nephrosis is produced by a systemic abnormality of T-cell function resulting in the secretion of a circulating chemical mediator toxic to an immunologically innocent glomerular basement membrane. The lack of evidence of a humoral antibody response, remission induced by measles which modifies cell-mediated immunity, the therapeutic benefits of steroids and cyclophosphamide which also abate cell-mediated responses, and the occurrence of this syndrome in Hodgkin's disease support this hypothesis The susceptibility of untreated patients to pneumococcal infections may be of primary or secondary pathogenetic importance. Taken together, the data sugges that this syndrome is a clinical expression of a self limited primary immune-deficiency disease.