X-linked Agammaglobulinemia

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• 作者：Deepti Suri ; Amit Rawat ; Surjit Singh
• 关键词：Brutons disease ; Immunoglobulins ; Antibody deficiency ; Hypogammaglobulinemia
• 刊名：The Indian Journal of Pediatrics
• 出版年：2016
• 出版时间：April 2016
• 年：2016
• 卷：83
• 期：4
• 页码：331-337
• 全文大小：628 KB
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• 作者单位：Deepti Suri (1)
  Amit Rawat (1)
  Surjit Singh (1)
  
  1. Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
  
• 刊物主题：Pediatrics; Gynecology;
• 出版者：Springer India
• ISSN：0973-7693

文摘

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.