Early development of immunity in diGeorge syndrome.
Med Sci Monit
; 11(4): CR182-7, 2005 Apr.
Article
in En
| MEDLINE
| ID: mdl-15795698
ABSTRACT
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
T-Lymphocytes
/
DiGeorge Syndrome
Type of study:
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Risk_factors_studies
Limits:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
/
Newborn
Language:
En
Journal:
Med Sci Monit
Journal subject:
MEDICINA
Year:
2005
Document type:
Article
Affiliation country: