Demographic data, peak serum bilirubin, and results of bile bilirubin pigment analysis in nine children with prolonged and severe unconjugated hyperbilirubinaemia
| Case No | Sex | Ethnicity 1-150 | Onset of jaundice (days) | Peak SB (μmol/l) | Conjugated SB (μmol/l) | UC bilirubin (μmol/l) | Age of bile analysis (days) | PB | UC (%) (0–2) 1-151 | MC (%) (7–16) 1-151 | DC (%) (79–91) 1-151 | Diagnosis 1-152 | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | European | 1 | 431 | 0 | 431 | 19 | − | 100 | 0 | 0 | CNS 1 | |
| 2 | F | European | 4 | 396 | 2 | 394 | 38 | + | 19 | 81 | 0 | CNS 2 | |
| 3 | M | Asian | 3 | 650 | 10 | 640 | 25 | − | 100 | 0 | 0 | CNS 1 | |
| 8 m1-154 | + | 1 | 91 | 8 | |||||||||
| 4 | M | European | 2 | 300 | 0 | 300 | 25 | − | 2 | 34 | 64 | Gilbert | |
| 5 | F | Asian | 1 | 456 | 20 | 436 | 18 | − | 36 | 57 | 7 | CNS 2 | |
| 6 | F | European | 6 | 390 | 10 | 380 | 33 | + | 4 | 64 | 31 | ||
| 7 | M | European | 2 | 340 | 20 | 320 | 58 | + | 25 | 46 | 29 | ||
| 8 | M | European | 5 | >500 | 0 | >500 | 10 | − | 20 | 53 | 27 | ||
| 9 | F | Asian | 4 | 389 | 19 | 380 | 41 | + | 43 | 26 | 31 | ||
| 10 | M | European | 5 | – | – | – | 13 y | − | 1 | 18 | 82 | ||
| 11 | F | European | 2–3 y | – | – | – | 16 y | − | 4 | 18 | 78 | ||
| 12 | M | European | 5 | 403 | 20 | 383 | 13 y | + | 14 | 80 | 5 | ||
| 13 | M | European | 16 y | 63 | 21 | 52 | 16 y | − | 12 | 70 | 18 | ||
↵1-150 The three Asians infants were born to consanguineous parents.
↵1-151 Reference range for normal individuals (Clarke DJ, Burchell B, unpublished data).
↵1-152 Diagnoses based on analysis of bile bilirubin pigments.
↵1-154 Second analysis performed at 8 months of age while on phenobarbitone.
CNS 1, Crigler–Najjar type 1; CNS 2: Crigler–Najjar type 2; DC, bilirubin diglucuronides; m, months; MC, bilirubin monoglucuronides; PB, phenobarbitone treatment during bile bilirubin analysis (+, on phenobarbitone, −, not on phenobarbitone); SB, serum bilirubin; UC, unconjugated; y, years.