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Young biliary atresia patients don’t conform to existing transplant priority criteria

Radha Chitale
02 Jun 2016

Young patients over 11 years old with biliary atresia don’t conform to existing models that select and prioritize adults for liver transplants, according to a small UK-based study presented at the annual meeting of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN), held recently in Athens, Greece.

Delayed transplants can have long-term educational and employment impacts for these patients, as the study, a single-centre retrospective case notes analysis, also revealed that those who received transplants later were less likely to be in school or employed. [ESPGHAN 2016, abstract H-O-004]  

“The current UK listing mathematical models seem to have limited use in our young patients with biliary atresia,” said lead researcher Dr. Vandana Jain of King’s College Hospital in London, UK.

Biliary atresia is a result of damage to the bile ducts and presents with jaundice in infancy. About half of patients require liver transplant by age 2, Jain said, due to cirrhosis and/or cholestasis. Twenty-year native liver survival ranges between 22-44 percent, 4.3 percent go on to die, 7.6 percent receive liver transplants, and overall 60.5 percent suffer complications such as cholangitis and portal hypertension. [REFERENCE]

“It’s these young people with biliary atresia and their native livers that provide a unique cohort for adult gastro-hepatology colleagues and it’s crucial to assess and transplant them at the right time,” Jain said.

From a cohort of 514 biliary atresia patients who had a bile-drainage procedure between 1977-2000, 36 (7 percent) had a liver transplant at age >11 years. None were syndromic for biliary atresia.

The median age at which patients were waitlisted for a liver transplant was 14.7 years, median time spent on the waitlist was 6.5 months, and the median age at liver transplant was 16.6 years, with a median age of 25.69 years at last follow-up.

“It’s important to note that there were sig longer wait times in the adult versus the paediatric transplant group,” Jain said, noting one patient waited over 6 years having been temporarily delisted while pregnant and that a quarter of the patients were over age 18 when they received their liver transplant.

The most common primary indications for liver transplant in young people was cholangitis (31 percent), synthetic failure (19 percent), and acute variceal bleeding (15 percent).

Comparing the median laboratory values at listing and liver transplant, the researchers found that bilirubin was the only parameter that changed significantly while patients were waitlisted – 58 umol to 78 umol, respectively (p=0.008).

The current UK mathematical models for listing – Model for End-Stage Liver Disease (MELD) and United Kingdom Model for End-Stage Liver Disease (UKELD) – also significantly deteriorated (p=0.0087 and p=0.01, respectively) while patients were waitlisted, but Jain noted that only 73 percent and 91 percent of patients fulfilled the MELD and UKELD adult criteria, respectively, at listing. At liver transplant, 81 percent and 94 percent of patients fulfilled the MELD and UKELD adult criteria, respectively.

The researchers had education and data for 22 patients. Median age at liver transplant was 16.7 years and the researchers compared these young patients to a group of 14 patients who were transplanted at a median age of 1.2 years. The groups were both followed up into their early 20s.

Among those who received a liver transplant when they were over age 11 years, 36 percent were in education, 41 percent were in employment, and 23 percent were in neither. Among those who received a transplant younger than 11 years, half were in education and half were employed.

“Although very small numbers, this does suggest, in agreement with previous data, that being transplanted younger is associated with better education and employment outcomes,” Jain said. She suggested further longitudinal studies in larger cohorts of biliary atresia patients to find more appropriate prognostic models.  

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