Table 2. Excretion of coproporphyrin isomers I and III in urine and serum bilirubin in patients with DJS (+/+) and GS (7/7) and in healthy carriers (+/-) or (6/7).
| F1.IV/13 | F1.IV/17 | F2.II/5 | F3.II/1 | F1.II/5 | F1.IV/16 | F2.II/1 | F3.I/1 | F4.I/1 | F4.I/2 | F4.II/1 | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Total porphyrins (nmol/l) | 212 | 348 | 258 | 78 | 587 | 230 | 205 | 91 | 93 | 58 | 133 |
| Uroporphyrin (nmol/l) | 88 | 80 | 78 | 13 | 118 | 74 | 84 | 10 | 6 | 6 | 19 |
| Coproporphyrin (nmol/l) | 124 | 268 | 180 | 63 | 469 | 156 | 121 | 78 | 75 | 52 | 110 |
| Isomer I (%) | 99 | 97 | 92 | 100 | 98 | 96 | 93 | 43 | 53 | 57 | 89 |
| Isomer III (%) | 1 | 3 | 8 | — | 2 | 4 | 7 | 57 | 47 | 43 | 11 |
| Conjugated bilirubin (μmol/l) | 17.7 | 31.0 | 16.9 | 21.4 | 55.2 | 9.5 | 11.4 | — | — | — | 28.0 |
| Total bilirubin (μmol/l) | 46.7 | 35.0 | 60.4 | 49.5 | 72.2 | 18.8 | 32.4 | — | — | — | 42.0 |
| DJS | +/+ | +/+ | +/+ | +/+ | +/+ | +/+ | +/+ | +/− | +/− | +/− | +/+ |
| GS | 7/7 | 7/7 | 7/7 | 7/7 | 6/7 | 6/7 | 6/7 | 6/7 | 6/7 | 6/7 | 6/6 |
Reference ranges: total porphyrins <300 nmol/l; uroporphyrin <40 nmol/l; coproporphyrin <150 nmol/l;
Conjugated bilirubin <7 μmol/l, total bilirubin <20 μmol/l.