Biliary atresia, Alagille linked to oral problems in children, young adults
Study: Groups more likely to have severe cavities, inflamed gums, enamel defects
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Children and young adults with biliary atresia or Alagille syndrome more frequently experience oral problems and poor oral hygiene than their healthy peers, according to a study in France.
People with either disease were more likely to have inflamed gums, tooth shape abnormalities, and defects in enamel, the outer layer of the teeth. Severe early childhood cavities were also more common in these patients.
Overall, the study suggests that “early involvement of pediatric dentists is essential to address the specific oral health needs of these children and to manage infectious risks,” researchers wrote.
The study, “Pediatric oral and dental manifestations of biliary atresia and Alagille syndrome: a comparative retrospective study,” was published in Archives de Pédiatrie.
Studies have reported oral problems in people with these conditions
Both biliary atresia and Alagille, two rare diseases that manifest early in life, are marked by cholestasis, or slowed or halted flow of the digestive fluid bile from the liver to the intestines. This can cause bile to accumulate to toxic levels in the liver, damaging the organ.
In biliary atresia, the cause of which remains largely unclear, cholestasis is due to blocked bile ducts, the tubes that carry bile. In Alagille, cholestasis is caused by a lower-than-normal number of bile ducts due to genetic defects that lead to problems in embryonic development.
Several studies have reported oral and dental problems in people with these conditions.
“Some of these symptoms are probably due to cholestasis, but others may be [manifestations] of AGS [ Alagille Syndrome] or BA [biliary atresia],” the researchers wrote.
In this study, a team of researchers conducted a retrospective study to describe the oral characteristics of 26 people (ages 1 to 23 years) with biliary atresia or Alagille (cholestasis group) who were followed at the Timone University Hospital in France between 2021 and 2023.
The cholestasis group included 16 people with biliary atresia and 10 with Alagille, and most patients were female (61.5%). For comparison, the team also analyzed data from 26 age-, sex-, and dentition-matched healthy individuals.
Several oral problems were much more common in cholestasis group
Most biliary atresia patients (81.3%) and nearly one-third (30%) of Alagille patients underwent liver transplant, at ages ranging from 9 months to 10 years.
Six biliary atresia patients underwent Kasai portoenterostomy — the first-line treatment of biliary atresia that surgically creates a new route for bile to flow — and four of them later required a liver transplant.
Several oral problems were significantly more common in the cholestasis group than in the control group, including discoloration of teeth ranging from yellowish to greenish (70% vs. 0%), tooth shape anomalies (53.8% vs. 23.1%), and tooth number anomalies (38.5% vs. 7.7%).
Other problems found at numerically higher rates in the cholestasis group included gingivitis, or inflamed gums (54% vs. 42%); tooth structural anomalies, including enamel problems (46.2% vs. 26.9%); and poor oral hygiene (73.1% vs. 50%)
Gingivitis was also more common in patients who underwent liver transplant than in those who didn’t (75% vs. 20%).
[This study describes] the broad spectrum of oral manifestations of … cholestasis and provides a more detailed description of these features.
While tooth decay was less common in the cholestasis group than in the control group (53.8% vs. 76.9%, severe early childhood tooth decay was more frequent in cholestasis patients (15.4% vs. 7.7%).
In addition, no significant association was detected between teeth discoloration and tooth decay or enamel structure anomalies in the cholestasis group.
Rates of misaligned teeth (malocclusions) and gum disease were generally comparable between the two groups.
Similar trends were reported when comparing individuals with biliary atresia or Alagille with healthy controls. When comparing patients with either disease, the team found that the only significantly different dental/oral problem was malocclusions, which were more frequent in Alagille patients (100% vs. 62.5%).
This study describes “the broad spectrum of oral manifestations of … cholestasis and provides a more detailed description of these features,” the researchers wrote. They noted, however, that further studies are needed to confirm these findings in larger patient populations.
