X-linked agammaglobulinemia in northern Thailand.

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dc.contributor.authorTrakultivakorn, Muthitaen_US
dc.contributor.authorOchs, Hans Den_US
dc.date.accessioned2009-05-27T17:19:26Z
dc.date.available2009-05-27T17:19:26Z
dc.date.issued2006-03-18en_US
dc.descriptionPublished by the Allergy and Immunology Society of Thailand.en_US
dc.description.abstractX-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by a failure to generate immunoglobulins of all isotypes due to the absence of mature B cells and plasma cells, secondary to mutations in the Bruton's tyrosine kinase (Btk) gene. We report six patients with XLA, confirmed by mutation analysis, from northern Thailand. The mean age of onset was 2.5 years and the mean age at diagnosis was 7.3 years. All patients had a history of otitis media, pneumonia and arthritis at the time of diagnosis, five patients had developed bronchiectasis and 3 patients septicemia. Other infections reported included sinusitis (5/6), pericarditis (1/6), meningitis (1/6) and pyoderma (1/6). Haemophilus influenzae, Streptococcus pneumoniae, Pseudomonas aeruginosa and Staphylococcus aureus were isolated on multiple occasions. One patient died of sepsis at the age of 16 years. These observations demonstrate that early diagnosis and treatment can improve prognosis and quality of life.en_US
dc.description.affiliationDepartment of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. mtrakult@mail.med.cmu.ac.then_US
dc.identifier.citationTrakultivakorn M, Ochs HD. X-linked agammaglobulinemia in northern Thailand. Asian Pacific Journal of Allergy and Immunology. 2006 Mar; 24(1): 57-63en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/36941
dc.language.isoengen_US
dc.subject.meshAgammaglobulinemia --complicationsen_US
dc.subject.meshAge of Onseten_US
dc.subject.meshArthritis --complicationsen_US
dc.subject.meshBacterial Infections --complicationsen_US
dc.subject.meshChilden_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshDNA Mutational Analysisen_US
dc.subject.meshGenetic Diseases, X-Linked --complicationsen_US
dc.subject.meshHumansen_US
dc.subject.meshImmunoglobulins, Intravenous --therapeutic useen_US
dc.subject.meshInfanten_US
dc.subject.meshMaleen_US
dc.subject.meshOtitis Media --complicationsen_US
dc.subject.meshPedigreeen_US
dc.subject.meshPneumonia --complicationsen_US
dc.subject.meshThailanden_US
dc.titleX-linked agammaglobulinemia in northern Thailand.en_US
dc.typeJournal Articleen_US
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