Bakkaloğlu A, Pascual M, Schifferli J A, Ozen S, Beşbaş N, Saatçi U
Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.
Turk J Pediatr. 1995 Apr-Jun;37(2):147-51.
Systemic lupus erythematosus (SLE) is a rare disease in childhood. Here two cases with SLE are presented, both with C4 null alleles yielding a functional C4 deficiency. The first case, a 14-year-old girl with a C4A null allele only, had a mild disease course, whereas the second child, a seven-year-old boy with both C4A0 and C4B0, had a more relentless course leading to death in five years. We conclude that complement activation by the classical pathway might be an essential mechanism that protects against the emergence of autoimmune or immune-complex diseases, and that the deficient state in our patients predisposed them to the early development of SLE.
系统性红斑狼疮（SLE）在儿童期是一种罕见疾病。本文报告两例SLE病例，均具有C4无效等位基因，导致功能性C4缺乏。第一例，一名仅具有C4A无效等位基因的14岁女孩，疾病病程较轻；而第二例患儿，一名同时具有C4A0和C4B0的7岁男孩，病程更为严重，5年后死亡。我们得出结论，经典途径的补体激活可能是预防自身免疫性或免疫复合物疾病发生的重要机制，并且我们患者的缺陷状态使他们易患SLE的早期发展。
