Whether the underlying SCID gene defect prevents the TCR recombination process itself, impairs cytokine mediated signaling essential for T cell maturation and activation, or allows the accumulation of toxic purine metabolites such as with adenosine deaminase deficiency (ADA) SCID, all genotypes are characterized by paucity of recent thymic emigrant T cells and TRECs. Here, ADA is linked to hyperinsulinemic hypoglycemia, familial, 4.