Immunological phenotype and additional epidemiological and clinical features
| . | PI (3-mo-old) . | PII (2-mo-old) . | PIII (5-mo-old) . | Age-matched normal values (0–6 mo) . |
|---|---|---|---|---|
| IgG (g/L) | <1.08 | 1.76 | 1.84 | 2–12 |
| IgA (g/L) | 0.51 | 0.21 | 0.39 | 0.8–0.9 |
| IgM (g/L) | 0.07 | 0.11 | 0.28 | 0.1–0.9 |
| CD3+ (/μl) | 151 | 1,318 | 1,757 | 2,500–6,000 |
| TCRγδ/CD3+ | NP | 10 | 1.8 | |
| CD4+ (/μl) | 110 | 301 | 1,384 | 1,300–4,000 |
| CD8+ (/μl) | 24 | 994 | 340 | 400–1,900 |
| CD31+CD45RA+/CD4+: naive (%) | 2 | 0 | 52 | 90–95 |
| CCR7+CD45RA+/CD8+: naive (%) | 1 | 1 | 85 | 78–97 |
| CCR7+CD45RA−/CD8+: central memory (%) | 3 | 1 | 4.5 | 3–15 |
| CCR7−CD45RA−/CD8+: effector memory (%) | 63 | 68 | 4.9 | 0–3 |
| CCR7−CD45RA+/CD8+: TEMRA (%) | 33 | 30 | 5.1 | 0.1–7 |
| CD19+ (/μl) | 24 | 58 | 269 | 9,600-3,700 |
| CD27+IgD+/CD19+: switched-memory B cells (%) | NP | 11.5 | 6.1 | 0.1–1 |
| CD16+CD56+ (/μl) | 20 | 936 | 505 | 150–950 |
| TCR Vα7.2/CD3+ (%) | 0.5 | 0.5 | 2 | 1.3–7.3 |
| Vβ repertoire/CD8+ | NP | Mildly biased | Normal | |
| Type-1 interferon signature | NP | NP | Normal | |
| PHA-induced T cell proliferation | NP | Normal | Normal | |
| . | PI (3-mo-old) . | PII (2-mo-old) . | PIII (5-mo-old) . | Age-matched normal values (0–6 mo) . |
|---|---|---|---|---|
| IgG (g/L) | <1.08 | 1.76 | 1.84 | 2–12 |
| IgA (g/L) | 0.51 | 0.21 | 0.39 | 0.8–0.9 |
| IgM (g/L) | 0.07 | 0.11 | 0.28 | 0.1–0.9 |
| CD3+ (/μl) | 151 | 1,318 | 1,757 | 2,500–6,000 |
| TCRγδ/CD3+ | NP | 10 | 1.8 | |
| CD4+ (/μl) | 110 | 301 | 1,384 | 1,300–4,000 |
| CD8+ (/μl) | 24 | 994 | 340 | 400–1,900 |
| CD31+CD45RA+/CD4+: naive (%) | 2 | 0 | 52 | 90–95 |
| CCR7+CD45RA+/CD8+: naive (%) | 1 | 1 | 85 | 78–97 |
| CCR7+CD45RA−/CD8+: central memory (%) | 3 | 1 | 4.5 | 3–15 |
| CCR7−CD45RA−/CD8+: effector memory (%) | 63 | 68 | 4.9 | 0–3 |
| CCR7−CD45RA+/CD8+: TEMRA (%) | 33 | 30 | 5.1 | 0.1–7 |
| CD19+ (/μl) | 24 | 58 | 269 | 9,600-3,700 |
| CD27+IgD+/CD19+: switched-memory B cells (%) | NP | 11.5 | 6.1 | 0.1–1 |
| CD16+CD56+ (/μl) | 20 | 936 | 505 | 150–950 |
| TCR Vα7.2/CD3+ (%) | 0.5 | 0.5 | 2 | 1.3–7.3 |
| Vβ repertoire/CD8+ | NP | Mildly biased | Normal | |
| Type-1 interferon signature | NP | NP | Normal | |
| PHA-induced T cell proliferation | NP | Normal | Normal | |
NP: not performed.