Table 2 Characteristics of the survivors and nonsurvivors among the 1226 NF1 patients whose vital status was known
Clinical features* | Survivors n = 1159 No (%)† | Nonsurvivors n = 67 No (%)† | OR (95%CI)‡ | P value* |
|---|---|---|---|---|
Age, years, median [range] | 25.5 [2-74] | 31.7 [0-80] | - | 0.008 |
Male gender | 548 (47) | 31 (46) | 1.0 (0.6-1.7) | 0.98 |
Familial case (n = 897) | 498 (58) | 17 (53) | 0.7 (0.3-1.4) | 0.62 |
At least 6 café-au-lait spots (1220) | 998 (86) | 48 (74) | 0.6 (0.3-1.1) | 0.07 |
Freckles (n = 1217) | 985 (86) | 58 (88) | 1.1 (0.5-2.4) | 0.74 |
Neurofibromas (n = 1215) | 863 (75) | 60 (90) | 3.1 (1.2-8.4 | 0.02 |
Plexiform neurofibromas (n = 1209) | 333 (29) | 28 (42) | 1.8 (1.1-3.0) | 0.02 |
Lisch nodules (n = 954) | 366 (40) | 16 (40) | 0.9 (0.5-1.9) | 0.89 |
Renal artery stenosis (n = 601) | 5 (1) | 0 (0) | - | 0.60 |
Scoliosis (n = 934) | 222 (25) | 9 (18) | 0.6 (0.3-1.3) | 0.23 |
Nonunion (n = 1 194) | 43 (4) | 2 (3) | 0.7 (0.2-3.2) | 0.70 |
Hydrocephalus (n = 1 190) | 31 (3) | 1 (2) | 0.6 (0.1-4.4) | 0.60 |
OBNI (n = 526) | 202 (40) | 8 (30) | 0.6 (0.3-1.5) | 0.30 |
Optic pathway glioma (n = 1 172) | 151 (13) | 11 (17) | 1.2 (0.6-2.6) | 0.58 |
MPNST (n = 1198) | 44 (4) | 36 (55) | 31.2 (17.4-55.9) | <10-4 |
Pheochromocytoma (n = 1193) | 10 (1) | 3 (5) | 4.3 (1.1-16.3) | 0.03 |