peripheral vein followed by an observation period of 5-10 minutes. 
The initial infusion will not exceed 6x10^ cells/kg body weight. Total volume of infused cells, 
will not exceed 10 ml/kg of body weight per day and the infusion should usually be completed 
within 120 minutes. The cell suspension will be mixed gently approximately every 5 minutes 
during the infusion while the child is being monitored for acute and subacute side effects. 
Antipyretics, antihistamines, narcotics and/or anti-inflammatory agents will be administered 
based on the experience gained in the N2/T1L protocol 86-C-183 as needed to control symptoms 
associated with the infusion. Substantial or persistent symptoms or signs of toxicity may 
require discontinuation of the infusion or consideration of intraperitoneal infusion of the cells 
{see section 7.1). Once the optimal conditions for infusion have been identified, all subsequent 
infusions will be based upon this experience. 
6.4 Pharmaceutical Information 
PEG-ADA. Each patient on this protocol will be concurrently treated with PEG-ADA unless 
there is clear evidence that they have failed treatment with this agent. PEG-ADA (Adagen) is 
commercially available and is supplied as a sterile liquid for injection manufactured by ENZON 
Inc., South Plainfield, N.J. It is administered as an intramuscular injection of 15-30 units/kg 
once or twice weekly. Each patient will have been treated with this drug for a minimum of 9 
months before enrollment on this protocol and will thereafter be maintained on the previously 
determined optimum dosing schedule. The drug is supplied in a form ready for injection. There 
have been no reported toxicities or side-effects associated with its use at the recommended 
dosages. 
7.0 Dose of Therapeutic Modifications 
7.1 Part 1 : Infusion of hADA Gene Transduced, Non-selected Lymphocytes. 
In part 1 of this protocol we plan to administer the corrected T cells as soon as possible after 
gene introduction in culture. This is designed to minimize the potential for clonal overgrowth 
in culture and therefore the development of a disproportionate mix of immune specificities that 
might be significantly different from those present in the fresh blood sample. Experiments in 
immunodeficient mice transplanted with human cells (including gene treated ADA(-)SCID 
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