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Prognostic roles of tetrahydroxy bile acids in infantile intrahepatic cholestasis

Chee-Seng Lee1,2, Akihiko Kimura3, Jia-Feng Wu1

  • 1Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.

Journal of Lipid Research
|January 12, 2017
PubMed

Insights

High levels of tetrahydroxy bile acids (THBAs) in infants with cholestasis indicate a good prognosis. Urinary THBA proportion greater than 7.23% predicts better outcomes and transplant-free survival in these patients.

Area of Science:

  • Hepatology
  • Biochemistry
  • Genetics

Background:

  • Tetrahydroxy bile acids (THBAs) are hydrophilic and typically undetectable in healthy adults.
  • Elevated THBA levels are observed in *abcb11*-knockout mice, but their role in human cholestatic diseases remains unclear.

Purpose of the Study:

  • To investigate the presence of THBAs in patients with infantile intrahepatic cholestasis.
  • To determine the correlation between THBA levels and patient outcomes.

Main Methods:

  • Urinary bile acids (BAs) were analyzed using gas chromatography-mass spectrometry (GC-MS).
  • Data were compared between good (n=21) and poor prognosis (n=19) groups.
  • Genetic mutations in *ABCB11*, *TJP2*, and *ATP8B1* were analyzed.

Main Results:

  • Good prognosis patients exhibited a significantly higher urinary THBA proportion (25.89%) compared to poor prognosis patients (1.93%).
  • A urinary THBA proportion >7.23% accurately predicted good prognosis (95.24% sensitivity, 84.21% specificity).
  • Higher THBA levels were independently associated with decreased transplant-free survival (HR=7.16, P=0.028).
  • Patients with *ABCB11* or *TJP2* mutations had minimal THBA, while *ATP8B1* mutations showed elevated THBA.

Conclusions:

  • High urinary THBA levels are associated with a good outcome in infantile intrahepatic cholestasis.
  • Urinary THBA proportion serves as a predictive biomarker for prognosis in these patients.
  • Disease entity and genetic mutations influence THBA levels and patient outcomes.

Keywords:
bile acid metabolismbile saltsclinical studiescytochrome P450diseasesliverprogressive familial intrahepatic cholestasis

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