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A Case of X-Linked Agammaglobulinemia Presenting with Recurrent Pneumonia and Bronchiectasis |
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| รหัสดีโอไอ | |
| Creator | Sirasuda Sommanus |
| Title | A Case of X-Linked Agammaglobulinemia Presenting with Recurrent Pneumonia and Bronchiectasis |
| Publisher | Text and Journal Publication |
| Publication Year | 2563 |
| Journal Title | Vajira Medical Journal |
| Journal Vol. | 64 |
| Journal No. | 5 |
| Page no. | 365-370 |
| Keyword | Recurrent pneumonia, Bronchiectasis, X-linked agammaglobulinemia |
| URL Website | https://tci-thaijo.org/index.php/VMED |
| Website title | Vajira Medical Journal (วชิรเวชสาร) |
| ISSN | 0125-1252 |
| 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. |
