Abstract
Summary: Previous reports concerning children with Cockayne syndrome had described decreased T cell proliferative responses and renal anomalies which could be associated with immunologic disturbances. Herein, the thymic function was evaluated by measuring the serum level of thymic hormone. This serum level was found to be undetectable or decreased in seven cases of Cockayne syndrome. Active serum concentrations varied between 0 and 1/8, whereas normal children of the same age show activity in the range between 1/16 and 1/64. In contrast, T cell function, explored by phytohemagglutinin and Concavalin A responses, and mixed lymphocyte cultures was normal. Whether or not this premature sign of immunological aging is primary or secondary to other manifestations of the syndrome is still difficult to assess.
Speculation: The discrepancy between low levels of thymic hormone found in seven cases of Cockayne syndrome which represents a biologic sign of aging, and normal T cell function, is still unclear: we can suppose that the number of long-lived T cells is sufficient to provide normal T cell responses after decrease of thymic production.
The role of decreased thymic hormone level in the etiopathogenesis of the disease is difficult to evaluate: this decrease could be either responsible for clinical manifestations of the disease or only secondary to a more fundamental disorder.
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Bensman, A., Dardenne, M., Bach, JF. et al. Decrease of Thymic Hormone Serum Level in Cockayne Syndrome. Pediatr Res 16, 92–94 (1982). https://doi.org/10.1203/00006450-198202000-00002
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DOI: https://doi.org/10.1203/00006450-198202000-00002