TABLE 1.
Basic characteristics of patients in the good prognosis and poor prognosis groups
| Good Prognosis (n = 21) | Poor Prognosis (n = 19) | P | |
| Gender (male:female) | 15:6 | 10:9 | 0.23 |
| Diagnosis | |||
| CMV infection | 8 | — | — |
| Urinary tract infection | 2 | — | — |
| Transient neonatal cholestasisa | 3 | — | — |
| Idiopathic NH | 8 | — | — |
| Phenotypic PFIC | — | 7 | — |
| FIC1 deficiency | — | 1 | — |
| BSEP deficiency | — | 2 | — |
| TJP2 deficiency | — | 2 | — |
| NICCD | — | 2 | — |
| IEBAM (CYP7B1 and AKR1D1 mutations) | — | 4 | — |
| PNAC | — | 1 | — |
| Disease onset (months) | 1.9 (0.6–8.9) | 2.2 (0.2–5.7) | 0.54 |
| Follow-up duration (years) | 1.6 (0.1–8.0) | 0.6 (0.2–11.7) | 0.08 |
| Initial biochemistry | |||
| Total bilirubin (mg/dl) | 8.55 (0.68–14.64) | 10.90 (2.65–23.6) | 0.13 |
| Direct bilirubin (mg/dl) | 5.32 (0.29–8.28) | 6.30 (1.72–15.40) | 0.17 |
| ALT (U/l) | 232 (18–814) | 200 (39–400) | 0.34 |
| GGT (U/l) | 67 (4–391) | 48 (14–117) | 0.03 |
| Serum BAsb | 166 (41–166) | 122 (36–400) | 0.27 |
The good prognosis group was jaundice-free before 1 year of age and the poor prognosis group had a persistence of jaundice after 1 year of age. CMV, cytomegalovirus; NICCD, neonatal cholestasis caused by citrin deficiency; PNAC, parenteral nutrition-associated cholestasis; .
a
Transient neonatal cholestasis, SLC25A13 or VPS33B heterozygous mutation.
b
Serum BA data for 27 patients (16 in the good prognosis group and 11 in the poor prognosis groups) were included.