Agammaglobulinemia: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management.
Endocr Metab Immune Disord Drug Targets
; 20(9): 1434-1447, 2020.
Article
em En
| MEDLINE
| ID: mdl-32384040
ABSTRACT
Agammaglobulinemia is a type of primary antibody deficiencies, characterized by severe reduction in serum level of all types of immunoglobulins level and absence of B cells in the peripheral blood. X-linked and various autosomal recessive/dominant mutations have been identified underlying the pathogenesis of this disorder. Affected patients present a broad range of clinical manifestations, including respiratory infections, gastrointestinal complications, Enterovirus infections, autoimmunity, and malignancies. This disease can be controlled by different therapeutic strategies. In this review, we describe different aspects of agammaglobulinemia such as epidemiology, pathogenesis, clinical phenotype, diagnosis, management, and prognosis of congenital agammaglobulinemia.
Palavras-chave
Texto completo:
1
Temas:
ECOS
/
Gestao
Bases de dados:
MEDLINE
Assunto principal:
Fenótipo
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Gerenciamento Clínico
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Agamaglobulinemia
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Prognostic_studies
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Screening_studies
Limite:
Animals
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Humans
Idioma:
En
Revista:
Endocr Metab Immune Disord Drug Targets
Assunto da revista:
ALERGIA E IMUNOLOGIA
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ENDOCRINOLOGIA
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METABOLISMO
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TERAPIA POR MEDICAMENTOS
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Suécia