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Paeds Vivasgastroenterology-hepatology-and-nutrition

Paeds Vivas · gastroenterology-hepatology-and-nutrition

Liver transplantation in children: Viva

Branching clinical structured oral on paediatric liver transplantation: the indications and graft types, the PELD score for allocation, tacrolimus-anchored maintenance immunosuppression, the diagnosis and treatment of acute cellular rejection, the recognition of hepatic artery thrombosis, and the long-term risks of post-transplant lymphoproliferative disorder and adolescent non-adherence.

branching clinical structured oral4 min readVerification in progress

Target exams

RACP DWERACP DCEMRCPCH Clinical
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Target exams

RACP DWERACP DCEMRCPCH Clinical
Prompt
An eight-month-old infant with biliary atresia is being assessed for liver transplantation. The examiner asks about the indications and graft types, how she would be prioritised on the waiting list, and the immunosuppression she will need, and then pivots to a post-transplant scenario of a rising transaminase.

Branch 1: Indications, graft types, and waiting-list priority

The candidate should establish that biliary atresia is the single most common indication for paediatric liver transplantation, accounting for up to half of all cases, and that this infant with a failing Kasai is a classic candidate. A strong candidate lists the accepted indications: synthetic failure, intractable pruritus, growth failure despite nutritional support, recurrent cholangitis, and portal hypertension that is difficult to control. The candidate should also name the other major indication categories: metabolic and genetic disease, acute liver failure, chronic cholestasis and cirrhosis, and liver tumours. [1]

If the examiner asks about graft types, the candidate should explain why children rarely receive a whole-organ deceased-donor graft: the small size of the recipient and the scarcity of size-matched paediatric deceased donors mean that most children receive a segmental graft, whether reduced-size, split, or living-donor. The candidate should explain that a left lateral segment from a living donor, typically a parent, fits an infant and shortens waiting time, and that living-donor and split grafts were developed specifically because the smallest infants have the highest waiting-list mortality. The trade-off is donor morbidity and a higher vascular and biliary complication rate. [1]

The candidate must then describe the Paediatric End-stage Liver Disease score. It is calculated from five variables: serum bilirubin, the international normalised ratio, serum albumin, growth failure, and age at listing, with additional weight for age under one year. A strong candidate explains the paediatric insight that growth failure is deliberately weighted because malnutrition reflects disease severity in children in a way that no adult variable captures, and that the score prioritises children on the deceased-donor waiting list. The candidate should mention that standardised exception scores exist for conditions whose severity the raw score underestimates, such as metabolic disease and unresectable tumours. [3]

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References5ShowHide
  1. [1]Smith SK, Miloh T Pediatric Liver Transplantation. Clin Liver Dis, 2022.PMID 35868688
  2. [3]McDiarmid SV, Merion RM, Dykstra DM, et al Use of a pediatric end-stage liver disease score for deceased donor allocation: the United States experience. Indian J Pediatr, 2007.PMID 17476086
  3. [6]Reding R Tacrolimus in pediatric liver transplantation. Pediatr Transplant, 2002.PMID 12453195
  4. [8]Antala S, DiNorcia J, Bucuvalas J Balancing immunosuppression in pediatric liver transplantation: Playing the long game. Pediatr Transplant, 2023.PMID 37439035
  5. [11]Okamoto T, Okajima H, Uebayashi EY, et al Management of Epstein-Barr Virus Infection and Post-Transplant Lymphoproliferative Disorder in Pediatric Liver Transplantation. J Clin Med, 2022.PMID 35456259
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