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Assessment of taurine deficiency in cystic fibrosis
1Department of Chemical Pathology, Adelaide Children's Hospital, South Australia.
Insights
Cystic fibrosis (CF) children often have bile acid taurine deficiency, impacting fat absorption. However, taurine levels in plasma, urine, and cells did not correlate with this deficiency in CF patients.
Area of Science:
- Biochemistry
- Pediatric Gastroenterology
- Metabolic Disorders
Background:
- Bile acid taurine deficiency is prevalent in cystic fibrosis (CF).
- This deficiency is hypothesized to contribute to impaired fat absorption in CF patients.
- The glycine: taurine bile acid conjugation ratio (G/T ratio) is a key indicator of bile acid conjugation.
Purpose of the Study:
- To investigate the relationship between the bile acid G/T ratio and taurine concentrations in plasma, urine, and leukocytes in children with CF.
- To determine if peripheral taurine levels reflect bile acid taurine status in CF.
Main Methods:
- Studied 27 children with CF (aged 4-15 years).
- Measured serum and duodenal juice bile acid G/T ratio.
- Assessed taurine concentrations in plasma, urine, and leukocytes.
Main Results:
- An elevated bile acid G/T ratio was observed in serum (14/27) and duodenal juice (5/6) of CF children.
- Despite elevated bile acid G/T ratios in some CF children, plasma, urine, and leukocyte taurine concentrations were similar across subgroups and controls.
- No correlation was found between bile acid conjugation status and peripheral taurine levels.
Conclusions:
- Peripheral taurine measurements (plasma, urine, leukocytes) may not accurately reflect overall taurine status or bile acid taurine deficiency in CF.
- Taurine deficiency in CF might be specifically localized to bile acids, not systemic.
- Further research is needed to understand the implications of bile acid taurine deficiency in CF pathophysiology.
Abstract:
Bile acid taurine deficiency is common in cystic fibrosis (CF) and is thought to be associated with impaired fat absorption. The relationship between the glycine: taurine bile acid conjugation ratio (bile acid G/T ratio) and taurine concentrations in plasma, urine and leucocytes was examined in 27 CF children aged 4-15 yr. The bile acid G/T ratio was elevated in serum in 14 of the 27 and in duodenal juice in 5 of 6 children. Subgroups of CF children with elevated and normal bile acid G/T ratio and controls (n = 8) all had similar plasma, urine and leucocyte taurine concentrations. The results suggest that either taurine measurement in plasma, urine and leucocytes does not accurately reflect stores elsewhere in CF, or that taurine deficiency is confined to bile acids.