Suri Deepti, Rawat Amit, Singh Surjit
Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Indian J Pediatr. 2016 Apr;83(4):331-7. doi: 10.1007/s12098-015-2024-8. Epub 2016 Feb 24.
X-linked agammaglobulinemia (XLA) is one of the commonest primary immune deficiencies encountered in pediatric clinical practice. In adults, common variable immunodeficiency (CVID) is the most common primary immunodeficiency disease (PID). It is an X-linked disorder characterized by increased susceptibility to encapsulated bacteria, severe hypergammaglobulinemia and absent circulating B cells in the peripheral blood. Replacement immunoglobulin therapy is the main cornerstone of treatment. Aggressive management of intercurrent infections and prophylactic antimicrobials are needed. This review attempts to highlight varied clinical manifestations and management of XLA, especially in the context of developing country.
X连锁无丙种球蛋白血症（XLA）是儿科临床实践中最常见的原发性免疫缺陷病之一。在成人中，常见变异型免疫缺陷（CVID）是最常见的原发性免疫缺陷病（PID）。它是一种X连锁疾病，其特征是对包膜细菌易感性增加、严重高球蛋白血症以及外周血中循环B细胞缺失。替代免疫球蛋白治疗是主要的治疗基石。需要积极处理并发感染并使用预防性抗菌药物。本综述试图强调XLA的各种临床表现及治疗，尤其是在发展中国家的背景下。
