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Microdeletion 22q11.2 syndrome: Does thymus incidental surgical resection affect its immunological profile?
Allergol. immunopatol ; 47(2): 141-151, mar.-abr. 2019. tab
Artigo em Inglês | IBECS | ID: ibc-180802
Biblioteca responsável: ES1.1
Localização: BNCS
ABSTRACT

BACKGROUND:

The del22q11 syndrome patients present immunological abnormalities associated to thymus alterations. Up to 75% of them present cardiopathies and thymus is frequently removed during surgery. The thymectomy per se has a deleterious effect concerning lymphocyte subpopulations, and T cell function. When compared to healthy controls, these patients have higher infections propensity of variable severity. The factors behind these variations are unknown. We compared immunological profiles of del22q11.2 Syndrome patients with and without thymectomy to establish its effect in the immune profile.

METHODS:

Forty-six del22q11.2 syndrome patients from 1 to 16 years old, 19 of them with partial or total thymectomy were included. Heart disease type, heart surgery, infections events and thymus resection were identified. Immunoglobulin levels, flow cytometry for lymphocytes subpopulations and TREC levels were determined, and statistical analyses were performed.

RESULTS:

The thymectomy group had a lower lymphocyte index, both regarding total cell count and when comparing age-adjusted Z scores. Also, CD3+, CD4+ and CD8+ lower levels were observed in this group, the lowest count in those patients who had undergone thymus resection during the first year of life. Their TREC level median was 23.6/μL vs 16.1 miL in the non-thymus group (p = 0.22). No differences were identified regarding immunoglobulin levels or infection events frequencies over the previous year.

CONCLUSION:

Patients with del22q11.2 syndrome subjected to thymus resection present lower lymphocyte and TREC indexes when compared to patients without thymectomy. This situation may be influenced by the age at the surgery and the time elapsed since the procedure
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Coleções: Bases de dados nacionais / Espanha Base de dados: IBECS Assunto principal: Timectomia / Timo / Linfócitos T / Subpopulações de Linfócitos T Aspecto clínico: Diagnóstico Limite: Adolescente / Criança / Criança, pré-escolar / Feminino / Humanos / Lactente / Masculino Idioma: Inglês Revista: Allergol. immunopatol Ano de publicação: 2019 Tipo de documento: Artigo Instituição/País de afiliação: 1Hospital Infantil de México Federico Gómez/Mexico / Hospital Infantil de México Federico Gómez/Mexico / Instituto Nacional de Pediatría/Mexico