A Case of X-Linked Agammaglobulinemia Presenting with Recurrent Pneumonia and Bronchiectasis

Authors

DOI:

https://doi.org/10.14456/vmj.2020.32

Keywords:

Recurrent pneumonia, Bronchiectasis, X-Linked Agammaglobulinemia

Abstract

Recurrent infection especially recurrent sinopulmonary tract infection is a typical clinical condition for referral to pediatrician. Patients with recurrent pneumonia should be evaluated for underlying diseases including asthma, congenital heart diseases, gastroesophageal reflux, foreign body aspiration, structure anomalies, cystic fibrosis and immunodeficiency. Our one patient with recurrent pneumonia had hypoglobulinemia, low number of circulating B cell with deficiency of CD19 B cell, absence of tonsils and recurrent pneumonia requiring hospitalization. PCR and sequencing revealed mutation of Bruton tyrosine kinase (Btk) gene on Xq22, suggested that he had X-linked agammaglobulinemia (XLA). The appropriate treatment of XLA was good hygiene care, treatment of infection and prophylactic therapy with intravenous immunoglobulin. This report should alert the pediatrician and general practitioners to consider the diagnosis of primary immunodeficiency diseases. The early diagnosis and proper management can prevent comorbidity diseases and improve quality of patient’s life

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Author Biography

Sirasuda Sommanus, Department of Pediatrics, Taksin Hospital, Bangkok, Thailand

Sommanus S, Kerddonfak S, Kamchaisatian W, Vilaiyuk S, Sasisakulporn C, Teawsomboonkit W, Benjaponpita S. Cow’s milk protein allergy: immunological response in children with cow’s milk protein tolerance. Asian Pac J Allergy Immunol. 2013 June;32(2):171-77.

Sommanus S, Direkwattanachai C, Lawpoolsri S, Sitcharungsi R. Accuracy of childhood asthma control test among Thai childhood asthma patients. Asian Pac J Allergy Immunol. 2018 Sep;36(3):152-58.

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Published

2020-10-30

How to Cite

Sommanus, S. (2020). A Case of X-Linked Agammaglobulinemia Presenting with Recurrent Pneumonia and Bronchiectasis. Vajira Medical Journal : Journal of Urban Medicine, 64(5), 365–370. https://doi.org/10.14456/vmj.2020.32

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Section

Case Report