title: X-linked Agammaglobulinemia.
abstract: 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.
authors.affiliation: Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India. surideepti@gmail.com.
authors.forename: Deepti
authors.initials: D
authors.lastname: Suri
authors.affiliation: Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
authors.forename: Amit
authors.initials: A
authors.lastname: Rawat
authors.affiliation: Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
authors.forename: Surjit
authors.initials: S
authors.lastname: Singh
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000175:diagnosis* / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis* / Q000188:drug therapy; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D007116:Immunization, Passive / Q000379:methods*; D008297:Male
keywords: Antibody deficiency; Brutons disease; Hypogammaglobulinemia; Immunoglobulins