Significant increases in lymphocyte adenosine deaminase activity, T cell numbers and immune function have been achieved in the two children with SCID thus far treated with autologous T cells genetically-corrected by retroviral-mediated insertion of a normal ADA gene. Although the data obtained to date demonstrate that the use of ADA gene corrected peripheral T cells appears to be an effective treatment for ADA(-)SCID, it is theoretically preferable to try to develop a treatment for these children that will result in stem cell gene correction. The genetic correction of T cell progenitors with long-term immune reconstituting ability would be more desirable because repeated infusions of genetically altered cells should not be necessary and the generation of a more complete repertoire of T cell specificities might also be possible. Furthermore, the present treatment protocol involves indefinite continuation of enzyme replacement treatment with PEG-ADA. The demonstration of ADA gene expression in the progeny of transduced stem cells may simplify the decision concerning cessation of this very costly enzyme treatment (approximately $250,000/yr./patient). Recent evidence suggests that a small fraction of bone marrow or peripheral blood mononuclear cells bearing the CD34 antigen contains hematopoietic stem cells with both lymphoid and myeloid reconstituting ability. We propose in this amendment to supplement the infusion of human ADA gene-transduced autologous T cells in children with ADA(-)SCID with autologous peripheral blood CD34+ cells transduced with a second, readily distinguishable ADA vector.(ABSTRACT TRUNCATED AT 250 WORDS)

Desmoid tumours are not common but have a distinct resemblance to fibrosarcomata. Their clinical appearance and progress should be recognised since failure to distinguish them from sarcomata could result in extensive and unnecessarily mutilating operations. Three cases of extra-abdominal desmoid tumours are reported. Two of the patients had tumours arising from multicentric foci in the same limb. The disappearance of the tumours in two patients after the menopause, and the variation in the tumours during the menstrual cycle in the third patient, add weight to the theories about endocrine control.

We report two cases of acute haematogenous osteomyelitis in the anterior superior iliac spine (ASIS) in adolescent goalkeepers following trauma of the iliac crest apophysis. Both patients complained of pain over their right ASIS and were pyrexial. They were given antibiotics and were discharged from follow up without complication 64 and 90 days after starting treatment.

The fibrinogen uptake test has been used to detect deep vein thrombosis after total hip replacement in 90 patients. The upper third of the thigh in each leg was ignored and peripheral leg counting carried out distal to this. The results were directly compared with the findings of venography in the 170 legs of the 90 patients. There was a high false positive rate (30 per cent) with the fibrinogen uptake test which was associated with the presence of the knee joint effusion or varicose veins, but not with the site of operation. There was a low false negative rate (less than 5 per cent), and the fibrinogen uptake test, used in this way, may fail to detect a percentage of small thrombi in the wound area that are probably produced by the local trauma of operation. It is considered that the fibrinogen uptake test has a clinical use as a screening test for deep vein thrombosis after hip replacement, but venography is required in the interests of accuracy.

partners of two general practices in East Anglia, that the increase in lifespan which has resulted from advances in medical science and public health should not be years of a "prison sentence" but should be enjoyed. Surely to advise elderly patients against going on holiday simply because they may fail to take some necessary medicine would be a complete travesty of this philosophy?