X-linked agammaglobulinemia in northern Thailand.
Asian Pac J Allergy Immunol
;
2006 Mar; 24(1): 57-63
Artículo
en Inglés
| IMSEAR
| ID: sea-36941
ABSTRACT
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.
Texto completo:
Disponible
Índice:
IMSEAR (Asia Sudoriental)
Asunto principal:
Otitis Media
/
Linaje
/
Neumonía
/
Artritis
/
Tailandia
/
Infecciones Bacterianas
/
Humanos
/
Masculino
/
Análisis Mutacional de ADN
/
Niño
Tipo de estudio:
Estudio pronóstico
/
Estudio de tamizaje
País/Región como asunto:
Asia
Idioma:
Inglés
Revista:
Asian Pac J Allergy Immunol
Año:
2006
Tipo del documento:
Artículo
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