[Clinical and immunological profile of 15 Moroccan patients with Hyper IgM syndrome]. / Le profil clinique et immunologique de 15 patients Marocains atteints de syndrome hyper IgM.
Pan Afr Med J
; 26: 212, 2017.
Article
em Fr
| MEDLINE
| ID: mdl-28690727
ABSTRACT
Hyper IgM syndrome is a well known genetic (primary) immunodeficiency disorder which was first described in 1961. It is caused by B lymphocyte deficiency characterized by normal or elevated serum IgM levels and low or zero levels of IgG, IgA, IgE resulting from isotype-switching deficiency. Clinical manifestations are dominated by recurrent infections, especially involving the digestive tube of the ENT sphere and the lungs. This syndrome is caused by B-cell immunoglobulin class switch deficiency and decreased capacity to induce proliferation of T lymphocytes. The net result of these deficiencies is reflected in increased susceptibility to Pneumocystis jiroveci, Cryptosporidium spp and other intracellular organisms as well as high rate of bacterial and viral infections. This study aimed to illustrate the importance of understanding the pathophysiological mechanisms associated with this increased susceptibility to infections in order to allow a better diagnosis and therapy in patients with Hyper IgM syndrome (HIM).
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Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Infecções Oportunistas
/
Linfócitos B
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Síndrome de Imunodeficiência com Hiper-IgM
Limite:
Child
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Child, preschool
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Female
/
Humans
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Infant
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Male
País como assunto:
Africa
Idioma:
Fr
Ano de publicação:
2017
Tipo de documento:
Article