Early development of immunity in diGeorge syndrome.

Autor:	Sedivá A; Institute of Immunology, Motol University Hospital, Prague, Czech Republic. anna.sediva@lfmotol.cuni.cz, Bartůnková J, Zachová R, Poloucková A, Hrusák O, Janda A, Kocárek E, Novotná D, Novotná K, Klein T
Jazyk:	angličtina
Zdroj:	Medical science monitor : international medical journal of experimental and clinical research [Med Sci Monit] 2005 Apr; Vol. 11 (4), pp. CR182-7. Date of Electronic Publication: 2005 Mar 24.
Abstrakt:	Background: diGeorge syndrome is a relatively common congenital disorder with developmental defects, including hypoplasia or pathologic migration of the thymus, associated with deletion of contiguous genes on chromosome 22. We prospectively followed a cohort of children with confirmed 22q11.2 deletion. Material/methods: One to six repeated examination were performed in 13 boys and 21 girls, age 4 days to 19 years. Due to the proposed role of the thymus in T lymphocyte selection, we studied T lymphocytes and their function, and also the presence of double positive CD4+CD8+ and gamma/delta T lymphocytes in peripheral blood. Results: A low number of T lymphocytes was detected in the majority of patients before the age of 2 years. Both spontaneous and PHA-induced proliferation were unexpectedly higher than in normal samples from children <2 years old. Both T cell numbers and function normalized thereafter in the majority of patients. Double positive T cells were detected in one boy, together with transient positivity of antinuclear antibodies. Gamma/delta T cells were greater than 5% in 21% of the children. In our 5-year prospective study we have not yet observed serious clinical signs of immunodeficiency or autoimmunity in these patients, except for repeated respiratory infections. Conclusions: All patients classified as partial diGeorge syndrome presented with delayed but gradual development of immune function against a background of impaired support by the thymus.
Databáze:	MEDLINE
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