Immunoregulation in an Isolated 12-year-old Boy with Congenital Severe Combined Immunodeficiency

Nine-year microflora study of an isolator-maintained immunodeficient child

“…This study incorporates a 9-year period from age 3 to age 12 in the life of a male child with congenital SCID. Up to age 12 the subject was retained in a series of isolated enviroments, and he has been the subject of a variety of other investigations (3,5,7,14,19,20,22,26,28,35,37). Because a deceased antecedent brother of the patient was shown to have SCID which contributed to his death by pneumocystic pneumonia in his first year (35) and because several members of the subject's maternal lineage may have had immune deficiencies (18), the subject was delivered by caesarean section and maintained in isolation until appropriate therapy could be applied to correct the deficiency (29).…”

David’s Story

“…T-cell function was absent as measured by the blastogenic response to PHA and there was no change in the results over several years (7,10) (Tables 25.2, 25.4). Also, in assessing his T-cell responses, the child failed to reject an allogeneic skin patch (from Dr South) placed on his arm (7).…”

“…Suggestive evidence is that the weak 2-to-3-fold response to alloantigen in mixed lymphocyte cultures prevented engraftment (10). Also, there must have been some degree of immune resistance in David since he lived 12 years in a "germfree" environment that contained small amounts of several bacteria.…”

Importance of technology for the future of allergy and immunology

“…These are juxtaposed with a picture of a patient with SCIDX1, David, the Houston ''Bubble Boy,'' when he was 4 years old and confined to the sterile plastic isolator system in which he spent the first 12 years of his life (Fig 1 and the cover of this issue). 1,2 As seen in the histogram, David lacked the immunogically critical CD3 1 T-cell population and consequently was at continuous risk for life-threatening infections. There is an increase in the proportion of B cells demonstrated on the histogram, a classic finding in SCIDX1 that should be a definitive clue to the allergistimmunologist evaluating an infant for congenital immunodeficiencies.…”