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(13)C-phenylalanine breath test correlates with liver fibrosis in postoperative biliary atresia
Masaki Wada1, Yuko Wada, Makoto Uchiyama
1Division of Pediatrics, Department of Homeostatic Regulation and Development, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan. nicuwada@tymc.twmu.ac.jp
Insights
The (13)C-phenylalanine breath test (PBT) effectively assesses liver fibrosis in children post-biliary atresia surgery. This non-invasive PBT method provides reliable liver function monitoring for pediatric patients with liver fibrosis.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Biochemical Testing
Background:
- The (13)C-phenylalanine breath test (PBT) is a validated index for assessing liver fibrosis and predicting liver disease in adults.
- Biliary atresia (BA) is a serious condition in children requiring surgical intervention and subsequent monitoring for liver complications.
- Evaluating liver function and fibrosis in pediatric patients post-BA surgery is crucial for long-term health outcomes.
Purpose of the Study:
- To evaluate phenylalanine metabolism in the liver of children who underwent surgery for biliary atresia (BA).
- To compare (13)C-phenylalanine breath test (PBT) findings with biochemical data, particularly liver fibrosis indices, in postoperative BA children.
- To determine the utility of PBT as a non-invasive tool for monitoring liver fibrosis in this pediatric population.
Main Methods:
- Hepatofunctional evaluations and (13)C-phenylalanine breath tests (PBT) were performed on 10 postoperative BA children (moderate and severe liver dysfunction) and 13 healthy children.
- Participants received a single oral dose of (13)C-phenylalanine (3.5 mg/kg).
- Exhaled gas was collected for 120 minutes post-administration, with (13)CO(2) levels monitored using gas chromatography-mass spectrometry to assess phenylalanine metabolism.
Main Results:
- Significant correlations were observed between PBT findings and established liver fibrosis markers, including total bile acid, hyaluronic acid, type IV collagen 7S, total bilirubin, and albumin.
- PBT results were significantly lower in children with severe liver dysfunction (Group A) compared to those with moderate dysfunction (Group B).
- These findings indicate markedly attenuated phenylalanine metabolism in children with more severe liver dysfunction.
Conclusions:
- The (13)C-phenylalanine breath test (PBT) values demonstrated a strong correlation with liver fibrosis in children following biliary atresia surgery.
- PBT serves as a valuable, non-invasive method for assessing liver fibrosis.
- The study supports the use of PBT as a reliable index for post-surgical monitoring of liver fibrosis in pediatric patients.
Background:
Values derived from the (13)C-phenylalanine breath test (PBT) may serve as an index for liver fibrosis and clinically predictive readings for liver diseases in adults. In the present study the PBT was conducted in postoperative biliary atresia (BA) children to evaluate phenylalanine metabolism in the liver, and the results based on biochemical data, especially the index on liver fibrosis, were compared with PBT findings.
Methods:
Hepatofunctional evaluations were conducted in 10 postoperative BA children with moderate (group B; n = 4) and severe (group A; n = 6) liver dysfunction, and the PBT results were compared with those of 13 normal healthy children (group C). Subjects were orally given single-bolus (13)C-phenylalanine at 3.5 mg/kg (maximum dosing: 100 mg) in the morning. Time-related exhaled gas was periodically collected until 120 min after dosing. The (13)CO(2) levels were monitored with gas chromatography-mass spectrometry before and after administration, and the (13)C excretion rate, (13)C cumulative excretion and time of maximum (13)C excretion rate were monitored accordingly.
Results:
Total bile acid, hyaluronic acid, type IV collagen 7S, total bilirubin or albumin and the PBT findings were significantly correlated. The PBT findings in group A were significantly lower those of group B, indicating that phenylalanine metabolism was markedly attenuated in the former.
Conclusion:
The PBT values correlated well with liver fibrosis in postoperative BA children. Because PBT is a non-invasive approach, results from this method may serve as a useful and reliable index for post-surgical monitoring of children operated on for liver fibrosis.
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