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Overdiagnosis of adrenal insufficiency in children with biliary atresia

Suparat Ekawaravong1, Suporn Treepongkaruna1, Preamrudee Poomthavorn1

  • 1Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Clinical Pediatric Endocrinology : Case Reports and Clinical Investigations : Official Journal of the Japanese Society for Pediatric Endocrinology
|June 26, 2023
PubMed

Insights

Low cortisol-binding globulin (CBG) in children with biliary atresia (BA) can lead to overdiagnosis of adrenal insufficiency (AI). Using free cortisol index (FCI) and calculated free cortisol (cFC) may improve diagnostic accuracy.

Area of Science:

  • Pediatric Endocrinology
  • Hepatology
  • Endocrinology

Background:

  • Serum total cortisol (TC) levels are influenced by cortisol-binding globulin (CBG).
  • Children with biliary atresia (BA) may exhibit low serum CBG levels.
  • Low CBG can potentially lead to the overdiagnosis of adrenal insufficiency (AI) in BA patients.

Purpose of the Study:

  • To evaluate adrenal function in pediatric patients diagnosed with biliary atresia (BA).
  • To investigate the role of cortisol-binding globulin (CBG) in the interpretation of adrenal function tests in children with BA.
  • To assess the utility of free cortisol index (FCI) and calculated free cortisol (cFC) in reducing AI overdiagnosis.

Main Methods:

  • Adrenocorticotropic hormone (ACTH) stimulation tests were performed on 71 children with BA.
  • Measurements included plasma ACTH, serum total cortisol (TC), and serum CBG levels.
  • Free cortisol index (FCI) and calculated free cortisol (cFC) were derived to assess adrenal function.

Main Results:

  • Thirty-five percent of patients were diagnosed with AI based on peak TC levels.
  • Patients diagnosed with AI had significantly lower median serum CBG levels compared to non-AI patients.
  • Only 11% of patients met all three criteria for AI, suggesting overdiagnosis based on peak TC alone.

Conclusions:

  • Low serum CBG levels in children with BA contribute to reduced peak TC, potentially leading to AI overdiagnosis.
  • Peak FCI and cFC are valuable metrics that can help mitigate the overdiagnosis of adrenal insufficiency in this population.
  • Accurate assessment of adrenal function in BA requires consideration of CBG levels and utilization of free cortisol estimations.

Keywords:
adrenal insufficiencycirrhosiscortisol binding globulinfree cortisol

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