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. Author manuscript; available in PMC: 2015 Mar 31.
Published in final edited form as: J Clin Immunol. 2011 Oct 20;32(1):36–38. doi: 10.1007/s10875-011-9598-3

Table I.

Current practices for specific settings

Issue Jennifer Puck Jack Routes Alexandra Filipovich
Low TRECs in a premature infant If a replicate is low, the baby is referred for flow cytometry. TRECs are repeated until >37 weeks corrected gestational age; if low at that point, referral is made to an immunologist. Reassessment when the baby reaches term.
The ideal time interval between a positive TREC result and a formal immunologic assessment Under 2 weeks, less if TRECs are undetectable; a baby with low TRECs should not receive live vaccines. Within 2 weeks. (The mean time from any abnormal TREC assay to flow cytometric evaluations in Wisconsin is about 7 days; if the TREC is zero; the mean time is 2 days.) If the TREC is zero, the family is cautioned to avoid sick contacts and isolate the baby. 2 weeks. The family should be cautioned to isolate and protect the baby
The ideal time between the definitive diagnosis of SCID and transplantation 2 weeks to 4 months. The workup is overseen by a designated Immunology Referral Center, which coordinates referral to a transplant center. Once the diagnosis is established, a referral is made to a transplant center. The timing is dictated by donor availability. 3–6 months
If the gene defect is not known, is the transplant protocol modified to accommodate potential DNA repair defects? Radiation sensitivity testing is available for all California SCID infants. DNA damaging conditioning regimens are particularly dangerous in patients with radiation-sensitive SCID. Reduced intensity conditioning is preferred. Infants without a molecular diagnosis are tested for radiation sensitivity, although if clinically indicated, transplantation is not delayed based on the pending results. Reduced intensity conditioning is preferred.