TABLE 1.
De novo autoimmune hepatitis (n = 29) |
|
---|---|
| |
Gender (m/f) | 11/18 |
| |
Ethnicity | |
Black | 8 |
White | 15 |
Hispanic | 2 |
Asian | 4 |
| |
Age at transplant median (range) years | 1.5 (0.2–12.3) |
| |
Graft type (n): | |
DD | 24 |
LD | 4 |
UNK | 1 |
| |
Induction therapy at time of transplant (n): | |
OKT3 | 0 |
ATG | 0 |
sIL-2R antagonist | 1 |
anti-CD52 | 0 |
| |
Pre transplant diagnosis (n): | |
Biliary atresia | 17 |
Acute liver failure | 5 |
Metabolic | 2 |
Other | 5 |
| |
Duration from transplant at DAIH diagnosis median (range) years: | 5.3 (1.2 – 14.9) |
| |
Liver enzymes at diagnosis median (range): | |
ALT (5 – 45 IU/L) | 108 (17 – 918) |
GGT (5 – 32 IU/L) | 72 (16 – 695) |
Total bilirubin (<1 mg/dl) | 0.7 (0.2 – 12.2) |
| |
Immunoglobulin G at diagnosis median (25–75% quartile): IgG (mg/dl) | 1670 (1620 – 1910) |
| |
Calcineurin inhibitor at diagnosis (n): | |
Cyclosporine | |
Tacrolimus | 13 16 |
| |
Number of patients on steroids at diagnosis | 5 |
| |
Treatment once diagnosis made: | |
Steroid (mg/kg) (mean ± SD) | 0.66 ± 0.68 |
Azathioprine (n) | 14 |
Mycophenolate (n) | 1 |
Sirolimus (n) | 1 |
| |
Number of exacerbations mean ± SD (n=17) | 1.2 ± 1.4 |
| |
Complications pre DAIH diagnosis (n): | |
Biliary | 6 |
Arterial | 0 |
Portal Vein | 2 |
Hepatic Vein | 1 |
CMV Infection | 2 |
CMV Disease | 1 |
PTLD | 1 |
Episodes of Acute Cellular Rejection pre | 14 |
DAIH diagnosis (n): | |
0 | 15 |
1 | 8 |
2 | 1 |
>2 | 4 |
| |
Complications post DAIH diagnosis (n): | |
Re-listed | 3 |
Re-transplanted | 2 |
Portal hypertension | 4 |
GFR < 60 ml/min/1.73m2 | 4 |
PTLD | 0 |
CMV Infection | 1 |
Death | 1 |
| |
Duration of follow up median (range) years | 7.1 (1.6 – 15) |
Reference values from Nelson Textbook of Pediatrics, 20th Ed, chapter 727, 3464–3473.