Causes of death in 57 patients with Alagille syndrome and chronic liver disease
| Patients with neonatal cholestatic jaundice | Patients with no clinically patent neonatal jaundice | ||||||
|---|---|---|---|---|---|---|---|
| Age | Age | ||||||
| n | Median | Range | n | Median | Range | ||
| Liver failure | 4 | 3 y | 1–17 y | 2 | 11 y 9/12 | 10–13 y 6/12 | |
| Portal hypertension | 3 | 8 y | 13 mos–23 y | 0 | |||
| Liver transplantation | 10 | 5 y 8/12 | 4 y 7/12–12 y | 0 | |||
| Haemorrhage | 6 | 3 y 6/12 | 3–5 y | 1 | 23 y | ||
| Cardiac abnormalities | 5 | 2 y 9/12 | 3 mos–7 y | 1 | 4 mos | ||
| Infection | 5 | 1 y | 2 mos–26 y | 3 | 10 y | 6 mos–25 y 6/12 | |
| Post surgery | 3 | 2 y 6/12 | 1 y 6/12–5 y | 0 | |||
| Renal failure | 3 | 2 y | 9 mos–8 y | 0 | |||
| Lung aspiration syndrome | 2 | 4 mos | 3 mos–5 mos | 0 | |||
| Sudden death | 1 | 1 y | 0 | ||||
| Heart-lung transplantation | 0 | 1 | 4 y | ||||
| During general anaesthesia | 1 | 6 y | 0 | ||||
| Accidental death | 0 | 1 | 17 mos | ||||
| Unknown | 5 | 4 y | 2 y 6/12–11 y | 0 | |||
| Total | 48 | 9 | |||||