Thymus Transplantation in DiGeorge Syndrome #668

Inclusion Criteria: * The subject's parent(s) signed the ICF. * For a diagnosis of DiGeorge Syndrome (DGS), the subject had one of the following: * Heart defect * Hypoparathyroidism * 22q11 hemizygosity * 10p13 hemizygosity * Coloboma, heart defect, choanal atresia, growth and development retardation, genital hypoplasia, ear anomalies/ deafness CHARGE association mutation (CHD7 deletion); * PHA proliferative responses less than 20-fold above background. * Subjects with typical Complete DiGeorge Anomaly (cDGA) had to have one of the following on 2 separate occasions: * Circulating CD3+ T cells by flow cytometry \< 50/mm3 or PHA \< 20-fold over background * If CD3+ were \> 50/mm3, then CD45RA+ (cluster of differentiation 45RA) CD62L+ had to be \< 50/mm3 * Or T cell receptor rearrangement excision circles (TRECs) by PCR had to be \< 100 per 100,000 CD3+ cells. * Subjects with atypical cDGA had to have both of the following with 2 studies each: * Circulating CD3+ T cells by flow cytometry \> 500/mm3 and CD45RA+ CD62L+ CD3+ T cells \< 50/mm3 and TRECs less than 100 per 100,000 CD3+ cells. * T cell proliferative response to PHA more than 20-fold over background. Circulating CD3+ T cells by flow cytometry \> 500/mm3 and CD45RA+ CD62L+ CD3+ T cells \< 50/mm3 and TRECs less than 100 per 100,000 CD3+ cells. * T cell proliferative response to PHA more than 20-fold over background. While T cell response to PHA might have been seen, eligible subjects were to have no T cell proliferative response to antigens (less than 20-fold response) and were to have serious clinical problems related to immunodeficiency, such as opportunistic infection or failure to thrive. Exclusion Criteria: * Subjects on ventilators, with tracheostomies, with cytomegalovirus (CMV) infections, or requiring ongoing steroids could still be enrolled, but their data were to be analyzed separately * Subjects who had heart surgery \< 4 weeks prior to transplant * Heart surgery anticipated within 3 months of the proposed time of transplantation * Ongoing parenteral steroid therapy between enrollment and transplantation * Present or past lymphadenopathy * Rash associated with T cell infiltration of the dermis and epidermis * Rejection by the surgeon or anesthesiologist as surgical candidates * Lack of sufficient muscle tissue to accept a transplant of 4 g/m2 body surface area (BSA) of the recipient * Prior attempts at immune reconstitution, such as bone marrow transplantation or previous thymus transplantation * Human immunodeficiency virus (HIV) infection