1. Absolute lymphopenia, (<1500/mm^) 
2. T-cell lymphopenia, (<1000/mm^) 
3. CMI skin test panel (7 antigens + 1 control), < 3 of 7 positive 
4. In vitro antigen stimulated lymphocyte proliferation, < 30% of control for 2 or more 
antigens 
5. In vitro proliferation to allogeneic cells, < 50% of control 
6. In vitro proliferation to mitogens, < 50% of control 
7. In vitro T helper function, < 50% of control 
8. In vitro T-cell cytotoxicity, < 50% of control against allogeneic targets or virus- 
modified autologous targets 
9. Deficient (< 40% of control) immunoglobulin production in vitro 
10. Deficiency of serum IgG, IgA, or IgM, (more than 2 S.D. below normal mean) 
11. Isohemagglutinins, titer of < 1:16 by age 3 
12. Deficient specific antibody responses to challenge with non-viable vaccine antigens 
based on norms for the response to each. Responses to the bacteriophage antigen 
0X174 have been well characterized by Ochs, et al. Our own laboratory has 
established norms for responses to pneumococcal polysaccharides types 1 , 2, and 3 as 
well as to KLH and Brucella abortus antigens. Norms are currently being established 
for responses to VEE and RVF vaccines. 
Patients admitted for study under this protocol will be registered with the PI at Building 10, 
Room 6B05, National Institutes of Health, Bethesda, MD. 20892. [(301) 496-5396]. The PI 
is also to be notified immediately whenever a protocol patient is taken off study. 
4.0 Clinical Evaluation Prior to Treatment 
Prior to enrollment into this protocol the child will be admitted to Patient Care Unit 3 B South 
of the Clinical Center for an evaluation to determine eligibility as per Metabolism Branch 
Protocol 66-C-77, which permits the immunologic evaluation outlined in section 4.2. 
4.1. Obtain and review previous records detailing clinical history, physical examination, 
general laboratory and specific immunologic evaluations. Complete history and physicial 
examination at admission. Radiographic evaluation as indicated, depending on availability of 
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