Table 2b.
Differences between standard-risk (SR) and high-risk (HR) partial DiGeorge syndrome (pDGS) patients.
| Patients with pDGS | SR | HR§¶ |
|---|---|---|
| Number of patients | 9 | 11 |
| Range of CD4+T cells (cells/µl) | 1060–2825 (NR 1400–4300)† | 940–1340§ (NR 1400–4300)† |
| 871–2833 (NR 700–3400)† | 500–1340¶ (NR 1400–4300)† | |
| 354–1900¶ (NR 700–3400)† | ||
| Number of patients with CD4+T cells < P10 | 0 | 5 |
| Range of CD45RA+CD4+ T cells (cells/µl) | 820–2492 (NR 1100–3700)† | 635–1031§ (NR 1100–3700)† |
| 652–2381 (NR 430–2900)† | 390–1031¶ (NR 1100–3700)† | |
| 180–420¶ (NR 430–2900)† | ||
| Number of patients with CD45RA+CD4+ T cells < P10 | 0 | 11 |
| Range of CD8+ T cells (CTLs) (cells/µl) | 447–1442 (NR 500–1700)† | 331–484§ (NR 500–1700)† |
| 624–1198 (NR 490–2000)† | 280–1450¶ (NR 500–1700)† | |
| 305–488¶ (NR 490–2000)† | ||
| Number of patients with CD3+CD8+ T cells < P10 | 0 | 11 |
| Number of patients with CD45RA+CD4+/CD45RO+CD4+ ratios < 1 | 0 | 6 |
| Number of patients with MAPCAs | 1 | 6 |
Range of the highest single value of each individual patient of both risk-groups compared with the normal age-related normal range (NR) in period A†and period B‡ (according to data from Shearer et al.[14]);
§
HR patients 6 and 10 (both died in period A at the age of 4 and 12 months respectively).
¶
hr patients 2, 4, 7, 8, 9, 13, 14, 15 and 20 (patients 2 and 14 died in period B). MAPCAs, major aortopulmonary collateral arteries; bold, abnormal.