Paediatric liver transplants: excellent outcomes challenged by supply

Liver transplants have become the standard of care for children with end-stage liver disease but there are looming challenges including an exponential rise in demand for donor organs.

Speaking at the GESA AGW 2020, Dr Michael Storman presented a review of more than 1,000 paediatric liver transplants in Australian and New Zealand since the inception of the program in 1985.

The findings from the Australia and New Zealand Liver Transplant Registry, also published in the Journal of Paediatrics and Child Health, showed that outcomes for children, most of whom were under three years of age at the time of transplant were “pretty good”.

For example, the one-year paediatric survival rate in recent years was 97%. About 12% of children required a second transplant and of them, about 15% go on to receive a third transplant.

Overall, since 1985, the program’s mortality rate was 17% but had markedly decreased from 36% in the first ten years, to 16% from 1997-2007, and now just 6.5%.

Graft failures and sepsis were the most common causes of death.

Dr Stormon, from the Children’s Hospital at Westmead, said that as in the rest of the world, biliary atresia was the most common indication for liver transplantation (54%).

“There’s a Nobel Prize awaiting any young person out there who can figure out what is going on with biliary atresia,” he told the virtual meeting.

Another common and increasing reason for transplantation was metabolic disorders (14%) including alpha-1 antitrypsin deficiency, urea cycle disorders and Crigler-Najjar syndrome.

“Other” diagnoses, which collectively made up 13% of the indications for liver transplant, included mostly Alagille syndrome, progressive familial intrahepatic cholestasis and cystic fibrosis.

Fulminant hepatic failure (FHF) was the indication in 11% of transplants and malignancy (4%) such as hepatoblastoma was a “small but important indication”.

He noted that while historically children with FHF and malignancy had poorer outcomes than other children requiring transplant, those differences in outcomes based on indication had gradually disappeared.

Dr Stormon said split grafts were now the most common type of graft (47.2%) compared to whole grafts (23.7%), reduced size grafts (16.5%) and live donor grafts (12.5%).

Split grafts have been necessary to get children transplanted, he said.

Dr Stormon said the exponential increase in adults requiring liver transplant due to metabolic diseases and a more modest increase in demand in children, particularly those with metabolic disorders and inborn errors of metabolism, posed a significant challenge.

“Clearly we are in competition if you like for grafts, and that is going to be an ongoing discussion.”

He said organ donation was also going to be an increasing challenge in the future.

“The donor age is increasing. Donor obesity is increasing. If you’ve got an older donor who is obese with a fatty liver then these grafts are less likely to be split. And this may mean we need to embrace live donation more.”

He said that families of children with metabolic disorders were on chat groups and knew there was a possibility of benefit with transplant.

“Bear in mind that in some situations you may not completely cure them of their metabolic disorder but hopefully give them enough metabolic activity to prevent particularly the neurological deterioration of these children.”

Dr Stormon briefly mentioned a recent study in MJA which showed outcomes for children following liver retransplantation were similar to those having their first transplant.

“It in fact demonstrated superior outcomes to most other centres around the world,” he said.

He said 42% of paediatric retransplants in Australia and New Zealand had been split grafts – an approach which has not been as popular in other countries such as the US.

“They have a much superior donation rate than we do but for lots of logistical reasons, they don’t split grafts.”

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