Pediatric gastroenterology, hepatology and nutrition Digestive System
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: 1. Biliary atresia diagnosis 2. Kasai portoenterostomy procedure performed at King’s College Hospital 3. Followed up at King's College Hospital for two years or until liver transplant or death if this occurred before two years
Exclusion criteria
Exclusion criteria: Serious comorbidities/ diseases (e.g. cardiac illness, chromosomal abnormalities, congenital malformations)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The following outcomes including medium-chain triglyceride (MCT) supplementation, growth and nutritional status and clinical outcomes are measured using patient medical records: MCT supplementation MCT intake (g/kg/day) prescription (% MCT) and duration of supplementation (months) determined based on caregiver-reported nutritional intake obtained at baseline, 6 weeks, 3 months, 6 months, 12 months and 24 months post kasai portoenterostomy Growth and nutritional status 1. Growth measurements (weight, length, head circumference, mid-upper arms circumference) obtained at baseline, 6 weeks, 3 months, 6 months, 12 months and 24 months post kasai portoenterostomy and converted to age- and sex-adjusted z-scores. 2. Fat-soluble vitamin status (serum concentrations of vitamin A, vitamin D and vitamin E) at baseline, 6 weeks, 3 months, 6 months, 12 months and 24 months post kasai portoenterostomy Clinical outcomes 1. Two-year outcome is classed as good (survival with native liver) or poor (transplant/listed for transplant/death) at two years post kasai portoenterostomy 2. Post-transplant outcome for those who are transplanted is determined at six months post-transplant and is classed as good (survival) or poor (re-transplant/listed for re-transplant/death) 3. Morbidity at the time of the analysis 4. Paediatric End-Stage Liver Disease score measured at baseline, 6 weeks, 3 months, 6 months, 12 months and 24 months post kasai portoenterostomy 5. The presence of ascites confirmed on abdominal ultrasound at baseline, 6 weeks, 3 months, 6 months, 12 months and 24 months post kasai portoenterostomy 6. Number of episodes of gastrointestinal bleeding and cholangitis recorded in the two years following kasai portoenterostomy 7. Liver function (serum albumin, alkaline phosphatase, aspartate aminotransferase, alanine aminotransferase, total bilirubin, gamma-glutamyl transferase and INR) obtained at baseline, 6 weeks, 3 months, 6 months, 12 months and 24 months post kasai portoenter | — |
Secondary
| Measure | Time frame |
|---|---|
| There are no secondary outcome measures | — |
Countries
England, United Kingdom