TABLE 1.
Patient | Sex | Age (yrs) | BMI (kg/m2; | [C3] (mg/L) | [C4] (mg/L) | [C4]-9 mon. (mg/L) | Total C4 CNV | C4A CNV | C4B CNV | RCCX H1 | RCCX H2 | DRB1 H1 | DRB1 H2 |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | M | 9 | 15.7 | 1532 | 158 | 188 | 3 | 0 | 3 | LL | S | 17(3)* | 13.1 |
2 | F | 11 | 16.3 | 1137 | 187 | 138 | 4 | 3 | 1 | LL | LL | 17(3) | 4 |
3 | M | 10 | 15.6 | 1592 | 456 | 406 | 4 | 2 | 2 | LS | LS | 1 | 4 |
4 | M | 12 | 23.0 | 1414 | 254 | 236 | 4 | 3 | 1 | LL | LL | 4 | 13 |
6 | M | 9 | 23.9 | 2681 | 504 | 556 | 5 | 2 | 3 | LL | LLS | 7 | 9 |
7 | M | 13 | 17.2 | 1157 | 242 | 434 | 3 | 1 | 2 | LS | S | 17(3)* | 4 |
8 | M | 11 | 17.3 | 1288 | 179 | 362 | 4 | 4 | 0 | LL | LL | 1 | 4 |
9 | M | 4 | 14.4 | 2244 | 202 | 266 | 3 | 1 | 2 | LL | S | 17(3)* | 4 |
10 | M | 9 | 15.5 | 1222 | 209 | 271 | 3 | 1 | 2 | LL | S | 17(3)* | 11 |
11 | M | 10 | 17.5 | 1855 | 341 | n.d. | 3 | 1 | 2 | LL | S | 17(3)* | 4 |
12 | M | 14 | 15.4 | 849 | 181 | 128 | 4 | 3 | 1 | LL | LS | 1 | 4 |
13 | M | 14 | 17.0 | 1355 | 241 | 195 | 4 | 2 | 2 | LS | LS | 7 | 8 |
14 | M | 13 | 12.5 | 849 | 273 | n.d. | 3 | 1 | 2 | LL | S | 17(3)* | 4 |
15 | M | 10 | 21.8 | 1493 | 371 | 484 | 5 | 3 | 2 | LS | LSS | 12 | 13.1 |
16 | F | 12 | 17.0 | 1031 | 163 | 102 | 2 | 1 | 1 | L | S | 1 | 17(3) |
17 | M | 14 | 15.5 | 1493 | 469 | 222 | 5 | 2 | 3 | LS | LSS | 1 | 4 |
18 | M | 9 | 17.3 | 1306 | 278 | 261 | 4 | 3 | 1 | LL | LL | 4 | 4 |
19 | M | 12 | 16.3 | 1183 | 289 | 276 | 4 | 2 | 2 | LS | LS | 7 | 8 |
20 | M | 16 | 29.2 | 732 | 245 | 279 | 4 | 3 | 1 | LL | LS | 4 | 7 |
21 | M | 8 | 23.9 | 1984 | 298 | 399 | 4 | 2 | 2 | LS | LS | 17(3) | 17(3) |
22 | M | 4 | 19.4 | 1006 | 297 | 397 | 6 | 3 | 3 | LSS | LLS | 7 | 13.1 |
23 | M | 10 | 25.4 | 1306 | 335 | n.d. | 3 | 1 | 2 | LL | S | 4 | 4 |
24 | M | 16 | 17.6 | 1006 | 197 | 237 | 4 | 3 | 1 | LL | LL | 4 | 12 |
25 | F | 11 | 21.3 | 1123 | 236 | 196 | 3 | 1 | 2 | LS | S | 4 | 15 |
26 | F | 8 | 17.1 | 2441 | 404 | 321 | 4 | 2 | 2 | LS | LS | 4 | 4 |
27 | M | 11 | 17.5 | 485 | 158 | 246 | 3 | 2 | 1 | LS | L | 13.1 | 1 |
28 | M | 6 | 19.7 | 1006 | 153 | 323 | 3 | 2 | 1 | LL | L | 4 | 7 |
30 | M | 12 | 15.4 | 1386 | 341 | 340 | 5 | 3 | 2 | LSS | LL | 1 | 7 |
31 | M | 7 | 15.2 | 912 | 198 | n.d. | 2 | 0 | 2 | S | S | 17(3)* | 17(3)* |
32 | M | 13 | 19.8 | 1386 | 150 | 175 | 2 | 1 | 1 | L | S | 17(3)* | 17(3) |
33 | M | 17 | 20.7 | 1761 | 244 | n.d. | 4 | 3 | 1 | LL | LS | 13.1 | 12 |
34 | F | 3 | 14.9 | 727 | <30 | n.d. | 3 | 2 | 1 | LS | L | 13.1 | 4 |
35 | M | 16 | 17.0 | 735 | 135 | n.d. | 3 | 1 | 2 | LL | S | 17(3)* | 14.1 |
an asterisk signifies an extended haplotype with HLA-DR3 and C4A deficiency; [C4], C4 protein concentration; [C3], C3 protein concentration; H1, haplotype 1; H2, haplotype 2; n.d., not determined.