Abstract
Thymic hypofunction and increased susceptibility to infection in the DiGeorge Syndrome have been associated with reduced humoral and cellular immunity. As a result, thymic transplantation has been utilized to modify immune responses, with improved lymphocyte responsiveness to mitogens and antigens being attributed to the effects of therapy.
Over the past year we have studied a male infant with the DiGeorge syndrome as documented by 1)characteristic facies, 2)neonatal hypocalcemic tetany, 3)absent thymic shadow, 4) quintallogy of Fallot, 5)antibody deficiency and 6)cellular immune dysfunction. During observation in reverse isolation, serial studies showed spontaneous acquisition of normal levels of serum immunoglobulins and cell mediated immunity as measured in vitro with phytohemagglutinin, concanavalin A, pokeweed and staphylococcal antigens. Macrophage inhibitory factor and interferon were produced. Sheep RBC - lymphocyte “T-rosettes” have remained decreased. At one year of age parathormone levels and growth are normal. Only recurrent otitis media and one episode of pneumonia have occurred.
Significantly, we have documented progressive spontaneous improvement in immuno-responsiveness without immunotherapy or transplantation. Thus, spontaneous recovery may occur in selected patients with DiGeorge syndrome, and careful observation of such infants may avoid unnecessary transplantation.
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Sieber, O., Durie, B., Hattler, B. et al. SPONTANEOUS EVOLUTION OF IMMUNE COMPETENCE IN DIGEORGE SYNDROME. Pediatr Res 8, 418 (1974). https://doi.org/10.1203/00006450-197404000-00471
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DOI: https://doi.org/10.1203/00006450-197404000-00471
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