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Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia
Stine Skipper Madsen1, Nina Kvist, Jørgen Thorup
1Department of Paediatric Surgery 4272, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark. j-thorup@rh.dk.
Insights
Elevated conjugated bilirubin levels above 20 μmol/l warrant further evaluation for biliary atresia in infants. The percentage of total bilirubin is not a necessary diagnostic criterion and can cause confusion.
Area of Science:
- Pediatric Gastroenterology
- Neonatal Hepatology
- Surgical Pediatrics
Background:
- Biliary atresia is a leading cause of pediatric liver transplantation, affecting approximately 1:15,000 live births in Denmark.
- Early diagnosis and surgical intervention are critical to prevent irreversible biliary cirrhosis and liver failure.
- Current Danish Health and Medicines Authority (DHMA) guidelines require evaluation for biliary atresia if conjugated bilirubin exceeds 20 μmol/l and/or is more than 20% of total bilirubin, a threshold causing diagnostic challenges.
Purpose of the Study:
- To investigate the diagnostic utility of the conjugated bilirubin percentage threshold in diagnosing biliary atresia.
- To evaluate whether the total amount of conjugated bilirubin alone is sufficient for diagnostic evaluation.
Main Methods:
- Retrospective analysis of medical records for children who underwent portoenterostomy for biliary atresia between 1993 and 2012.
- Exclusion of patients older than 84 days at the time of surgery, resulting in a final cohort of 54 patients.
Main Results:
- Patients diagnosed with biliary atresia demonstrated significantly elevated conjugated bilirubin levels, averaging 129.7 μmol/l (range: 42-334 μmol/l).
- The percentage of conjugated bilirubin among these patients averaged 73% (range: 28-97%), consistently exceeding the DHMA's 20% threshold.
- All analyzed patients exceeded the 20 μmol/l threshold for conjugated bilirubin.
Conclusions:
- A total conjugated bilirubin level exceeding 20 μmol/l is a sufficient indicator for further diagnostic evaluation of biliary atresia.
- The requirement to assess the percentage of conjugated bilirubin is unnecessary and contributes to diagnostic ambiguity.
Introduction:
Biliary atresia is the leading cause of liver transplantation in children. It affects 1:15,000 in Denmark. With a national birth rate of 60,000, four children are born every year with biliary atresia. Early correction of biliary obstruction is essential to prevent fatal biliary cirrhosis. The Danish Health and Medicines Authority (DHMA) demands diagnostic evaluation of children with elevated level of serum bilirubin after two weeks of age. Biliary atresia has to be excluded if conjugated bilirubin level is above than 20 μmol/l, and/or more than 20% of total bilirubin. This percentage value has caused diagnostic trouble over the years. The objective of the present study was to investigate the possibility of changing the recommendations.
Methods:
This was a retrospective analysis of the medical records of children operated for biliary atresia in the 1993-2012 period.
Results:
During the period, 73 patients where operated with a portoenterostomy ad modum Kasai. Patients older than 84 days at the time of operation were excluded, 54 patients were available for analysis. Conjugated bilirubin in μmol/l and the percentage value were significantly above the DHMA threshold limit: mean 129.7 μmol/l (42-334 μmol/l) and 73% (28-97%), respectively.
Conclusion:
The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion.
Funding:
none.
Trial Registration:
not relevant.
