By Lucy Piper, medwireNews reporter
medwireNews: Adolescents with Alagille syndrome (ALGS) who have relatively low total bilirubin levels and high platelet counts at the age of 12 years are more likely than those who do not to still have their own liver in adulthood, suggests research.
The investigators, who presented the findings in a poster at the EASL Congress 2026 in Barcelona, Spain, also report that “[r]eaching adulthood with a native liver [was] less common in patients who underwent surgical intervention in childhood.”
Dominika Wojdyla (Erasmus Medical Center, Rotterdam, the Netherlands) and colleagues say that “ALGS typically presents in infancy but can have incomplete penetrance or milder phenotype leading to a later diagnosis.”
To further characterize patients with this later diagnosis, the team evaluated native liver survival using data from the Global ALagille Alliance study on 1535 patients from 79 centers in 34 countries. Of these children, 58% survived to 12 years old with their native livers.
Analyzing 248 of these children (55% boys), the investigators found that 72% still had a functioning native liver at the age of 21 years.
The participants had a median total bilirubin level of 1.2 mg/dL at the age of 12 years and the risk for liver transplant or death was significantly reduced among those with lower levels at this age, with a hazard ratio (HR) for liver transplant or death of 1.61 with every 1 mg/dL increase in total bilirubin.
The likelihood of liver transplant or death was also significantly decreased among children who had a higher platelet count. At baseline, this was a median of 239 x 109/L and the HR was 0.93 with every 10 x 109/L increase in platelet count.
Wojdyla et al say that “[p]latelets and bilirubin appear to be useful indicators of disease progression,” at “meaningful” cutoff points at 12 years old of less than 150 x 109/L and more than 2 mg/dL, respectively.
They also note a “striking difference in survival at 18 years” between the 21 children who underwent a surgical intervention, such as biliary diversion, a Kasai procedure, or therapeutic endoscopy for esophageal or gastric varices, before the age of 12 years and the 227 children who did not. The respective rates of native liver survival by this age were 87% versus 56%, and the HR was a significant 4.91.
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EASL Congress 2026; Barcelona, Spain: 27–30 May
