Document Detail


X-linked agammaglobulinemia in northern Thailand.
MedLine Citation:
PMID:  16913189     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
X-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.
Authors:
Muthita Trakultivakorn; Hans D Ochs
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Asian Pacific journal of allergy and immunology / launched by the Allergy and Immunology Society of Thailand     Volume:  24     ISSN:  0125-877X     ISO Abbreviation:  Asian Pac. J. Allergy Immunol.     Publication Date:  2006 Mar 
Date Detail:
Created Date:  2006-08-17     Completed Date:  2006-12-01     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8402034     Medline TA:  Asian Pac J Allergy Immunol     Country:  Thailand    
Other Details:
Languages:  eng     Pagination:  57-63     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. mtrakult@mail.med.cmu.ac.th
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MeSH Terms
Descriptor/Qualifier:
Agammaglobulinemia / complications,  diagnosis*,  physiopathology*
Age of Onset
Arthritis / complications
Bacterial Infections / complications
Child
Child, Preschool
DNA Mutational Analysis
Genetic Diseases, X-Linked / complications,  diagnosis*,  physiopathology*
Humans
Immunoglobulins, Intravenous / therapeutic use
Infant
Male
Otitis Media / complications
Pedigree
Pneumonia / complications
Thailand
Chemical
Reg. No./Substance:
0/Immunoglobulins, Intravenous

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