X-linked Agammaglobulinemia

Indian Journal of Pediatrics
Deepti SuriSurjit Singh

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.

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Citations

Mar 1, 2016·Indian Journal of Pediatrics·Surjit Singh, Sudhir Gupta
Feb 15, 2020·Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology·Saba FekrvandAsghar Aghamohammadi
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Sep 10, 2021·Neurology. Neuroimmunology and Neuroinflammation·Martin S WeberMichael R Yeaman
Dec 17, 2021·Frontiers in Cell and Developmental Biology·Feng LiangLin Li

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