Severe combined XXXX with cartilage-hair hypoplasa: in vitro response to @entity10136 and attempted reconstitution.

@entity1 @entity308 @entity859 @entity55 @entity2905 @entity693 @entity509 @entity281 @entity465 @entity70771 @entity358 @entity1576 @entity10136
The present report describes an @entity1 with severe combined @entity465 and cartilage-hair @entity509 whose lymphocytes responded to @entity10136 in vitro. Immunologic evaluation was undertaken at 4 1/2 months of age following a history of recurrent severe @entity281 . Family history included three cousins who died in early infancy, one from @entity693 and @entity55 , one from generalized varicella, and another from @entity70771 . Quantitative immunoglobulins were markedly @entity308 : IgG 141, IgA 0, and IgM 24 mg/100 ml. There was an @entity2905 , multiple skin tests were negative, and in vitro lymphocyte responses to mitogens and antigens were @entity308 . Spontaneous E rosette determinations were 21% compared with control values of 65.7%. Erythrocyte @entity859 deaminase (ADA) activity was normal. The @entity1 's E rosette formation increased in the presence of @entity10136 , fraction 5, reaching a maximum of 56% with a concentration of 500 mug @entity10136 . Blastogenic responses to phytohemagglutinin also increased in the presence of @entity10136 . Transplantation of 24-week fetal thymus in Millipore diffusion chambers and subsequently transplantation of 18-week fetal thymus by intraperitoneal injection was accomplished. E rosettes increased to 35-40% and blastogenic responses to mitogens increased. Eight days after the second transplant the @entity1 underwent a mild graft vs. host reaction which subsided after 1 week and mitogen blastogenic responses again increased to 5-8 times previous values, but still well below control ranges. Repeated episodes of @entity358 ensued, cor pulmonale resulted, and the clinical course was relentlessly downhill with the @entity1 expiring from @entity1576 5 months after transplantation.
