[Thymic and parathyroid agenesia (DiGeorge's syndrome). Apropos of a case with abnormal vena caval return].
A child with a complete absence of thymus and parathyroids (Di George's syndrome) associated with a cardiac malformation (truncus arteriosus and abnormal caval venous return) is described ; the child died at the age of 1 month. An exchange-transfusion, performed at 4 days of age induced a mild graft versus host reaction which could only be recognized histologically and distorted the results of immunological tests. On this occasion, the main clinical, anatomical and immunological features of the Di George's syndrome are reviewed. They suggest a possible relationship between the severity of the cardiac malformation and the more or less complete character of the dysgenesia of the thymus and the parathyroids.
D000015:Abnormalities, Multiple; D002675:Child, Preschool; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D008297:Male; D010280:Parathyroid Glands / Q000002:abnormalities*; D013577:Syndrome; D013950:Thymus Gland / Q000002:abnormalities*; D014683:Vena Cava, Superior / Q000002:abnormalities*
L'agenesie thymique et parathyroidienne (syndrome de Di George). A propos d'un cas comportant un retour veineux cave anormal