Acute-on-chronic liver failure in BA linked to transplant complications

Children with biliary atresia who have a serious condition called acute-on-chronic liver failure (ACLF) tend to require a longer stay in the hospital and are more likely to experience complications during and after liver transplant surgery, a new study found.

These children are not at an increased risk of death after liver transplant, however.

“Despite having a longer length of stay, morbidity, and challenging intraoperative [during surgery] and immediate post-LT [liver transplant] course with higher [rates of repeat operations to address surgical complications] and readmission rates, the survival outcomes in the ACLF group were comparable to the no-ACLF group,” the researchers wrote.

The team added that, with advances in surgery and medical care, “successful long-term outcomes are noted.”

Their study, “Postoperative outcomes of acute-on-chronic liver failure in infants and children with biliary atresia,” was published in the journal Pediatric Transplantation.

Investigating the impact of acute-on-chronic liver failure in transplant outcomes

Biliary atresia is a rare disease of infancy in which the bile ducts, a set of tubes that normally carry a digestive liquid called bile from the liver to the intestines, are blocked or absent.

As a result, bile builds up in the liver and causes damage, which can lead to liver failure, where the liver is unable to function properly. In such cases, a liver transplant becomes necessary.

ACLF is a life-threatening condition that develops in patients with acute, or sudden, deterioration of chronic liver disease, and that often results in damage to other organs.

Data from a few studies in children with severe liver disease suggest that acute-on-chronic liver failure can develop in 12%-75% of cases, and is associated with “high early mortality ranging from approximately 20%-60%,” the researchers wrote.

However, according to the researchers, “there are limited data on the impact of ACLF on [post-liver transplant] outcomes in children with biliary atresia.”

To learn more, a team led by researchers at Baylor College of Medicine, in Texas, reviewed data from 107 children with biliary atresia who had undergone a liver transplant at their center. Among them, 13 (12%) had ACLF prior to the liver transplant.

The researchers compared outcomes during the time of surgery, after surgery, and over the long term between children with ACLF versus those without the condition.

The ACLF group was significantly younger when they were listed for transplant (median age 5 months vs. 9 months) and when the transplant was performed (median age 8 months vs. 16 months). However, there was no significant group difference in terms of time spent on the transplant waitlist, with a median of three versus five months.

No increased risk of death post-transplant found for children with ACLF

The liver transplant surgery itself took longer in children with ACLF, by more than an hour on average, and these patients were significantly more likely to lose more blood and to require a blood transfusion during surgery.

Among children without ACLF, more than half were managed at home following surgery, with about 1 in 5 managed in an intensive care unit (ICU). All 13 of the children with ACLF, however, were managed in the ICU.

Children with acute-on-chronic liver failure also had a significantly longer hospital stay. For those without ACLF, the total length of time spent hospitalized, including time before and after surgery, was nearly three weeks on average. For the ACLF group, it was close to six months.

Additionally, children with ACLF were more likely than those without to need medications to regulate blood pressure following liver transplant.

More than 1 in 3 children with ACLF (39%) needed additional surgery to address complications such as bleeding. This rate was notably lower among those without ACLF (17%), though the difference between the groups was not statistically significant.

Altogether, these findings indicate that biliary atresia patients with acute-on-chronic liver failure “have a critical operative and immediate post-transplant course with high risk of deterioration,” the researchers wrote.

Despite the initial [complications], long-term outcomes [for children with acute-on-chronic liver failure] were comparable to those without ACLF.

But despite the long hospitalizations and frequent complications, none of the children with ACLF died in the month immediately following their liver transplant. In the non-ACLF group, eight children died. Statistical tests showed the risk of death was not significantly increased by ACLF.

“Despite the initial morbidity, long-term outcomes [for children with acute-on-chronic liver failure] were comparable to those without ACLF,” the researchers wrote.

One potential explanation for this finding, according to the team, could be that children with ACLF are often viewed by healthcare providers as being “sicker” than young patients without the condition. As such, they may receive more rigorous monitoring that reduces the risk of long-term problems from complications.

Limitations of the study, as cited by researchers, included its single-center nature and the relatively small number of patients in the ACLF group. That highlights a need for further investigations to better understand how ACLF affects outcomes from liver transplants in children with biliary atresia, the team noted.

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