Table 2

Causes of death in 57 patients with Alagille syndrome and chronic liver disease

Patients with neonatal cholestatic jaundicePatients with no clinically patent neonatal jaundice
AgeAge
nMedianRangenMedianRange
Liver failure43 y1–17 y211 y 9/1210–13 y 6/12
Portal hypertension38 y13 mos–23 y0
Liver transplantation105 y 8/124 y 7/12–12 y0
Haemorrhage63 y 6/123–5 y123 y
Cardiac abnormalities52 y 9/123 mos–7 y14 mos
Infection51 y2 mos–26 y310 y6 mos–25 y 6/12
Post surgery32 y 6/121 y 6/12–5 y0
Renal failure32 y9 mos–8 y0
Lung aspiration syndrome24 mos3 mos–5 mos0
Sudden death11 y0
Heart-lung transplantation014 y
During general anaesthesia16 y0
Accidental death0117 mos
Unknown54 y2 y 6/12–11 y0
Total489