Table 2.
Infants followed at Mount Sinai Medical Center.
| Pt # | TREC avg | Ig levels (mg/dL) | Vaccine response | Mitogen response | Molecular studies | Clinical manifestations | Antibiotic prophylaxis | Current status; age |
|---|---|---|---|---|---|---|---|---|
| 17 | 107 | At 4 months: IgG 308, IgA 7, IgM 54 | At 9 months: Protective titers for Hemophilus, Diph + Tet; MMR given with no adverse problems | Low lymphocyte response to PHA at 2 months | None | Early watery diarrhea post rotavirus vaccine (rotavirus antigen negative); somewhat obese, currently no infections of note | TMP/SMX | Low muscle tone and sensory integration disorder; 5 years 6 months old |
| 18 | 75 | At 2 months: IgG 331, IgA 5, IgM 31 | At 1 year: Protective titers | Low lymphocyte response to ConA and PHA; at 3 months | IL7R, ADA = normal; chromosomal translocation of chromosome 3 and 14; same as her mother | None | TMP/SMX | Healthy; 5 years, 4 months |
| 19 | 77 | At 16 months: IgG 745, IgA 49, IgM 58 | Initial non responder for Hemophilus. Protective titers for Diphtheria, Tetanus and Pneumococcus; MMR given with no adverse reaction | Low lymphocyte response to ConA and PHA. Normal response to PWM | Chromosomal breakage study. Telomere length short. Metabolic panel | Failure to thrive; milk protein intolerance; macrocytic anemia | None | Mitochondrial dysfunction suspected; age 4 years |
| 20 | 18 | N/A | At 10 months: Protective titers for Tetanus. At 21 months, protective titers for Hemophilus; live viral vaccines withheld. | N/A | None | None | Prophylactic antibiotics were used for 6 months (CD3 < 500 mm3) | Healthy; 4 years 6 months |
| 21 | 79 | N/A | All vaccines, including MMR, up to date. No adverse effects. | N/A | None | At 6 months: Escherichia coli UTI treated with ceftriaxone (×2) and then cephalexin. At 8 months: acute otitis media treated with Amoxicillin. At 11 months: pneumonia treated with Azithromycin | None | Speech and cognitive delay; 5 years, 1 month |
| 22 | 103 | N/A | At 15 months: Protective titers for Hemophilus, Tetanus, Diphtheria and Pneumococcus | N/A | None | Coxsackie infection (self-resolved), also had a few viral respiratory infections | None | Healthy; 5 years |
| 23 | 22 | N/A | All vaccines, including MMR, up to date. Titers not done. | Normal ratio of CD 45 RO/RA cells | Amino acid panel | Possible autoimmune neutropenia of infancy | None | Healthy; Given live vaccines; 4 years 6 months |
| 24 | 0 | N/A | At 9 months: Protective titers for Diph and Tet: Non Protective for HiB. At 11 months: Protective titers for Pneumococcus | Normal mitogen proliferative response | FISH for DiGeorge | After the last Prevnar vaccination, developed a fever. URI without fevers and right hydronephrosis | None | Healthy; Given live vaccines with no problem; 18 months |
| 25 | 47 | At 8 months: IgG 334, IgA 5, IgG 18 | All vaccines, including MMR, up to date. | Normal mitogen response to PHA and Pokeweed. Low for ConA | Metabolic panel. Amino acid panel. CGH Array for DiGeorge negative | Failure to thrive. Poor feeding and high urine lactate. Macrocytoisis Pulmonic stenosis | None | Mitochondrial dysfunction is suspected; 17 months |
| 26 | 49 | N/A | At 3 months: Protective titers for Hep B and Tetanus | Normal mitogen proliferative response | ADA and PNP activity | Stooling 1–2 times per week; mild neutropenia | TMP/SMX | Normalized: lymphopenia due to utero 6-MP exposure; 2 years 6 months |