Journal article

Publication year: 2019

Journal: Steroids

Volume number: 150

ISSN: 0039-128X

eISSN: 1878-5867

DOI Link: https://doi.org/10.1016/j.steroids.2019.108426

Publisher: Elsevier

Abstract: 

Propose: Primary adrenal insufficiency (PAI) is a rare and life-threatening disease. A recent Endocrine Society guideline argued against hormonal monitoring of glucocorticoid replacement. However, about 50% of adolescents and young adults (AYAs) with chronic diseases are non-adherent to their treatment regimens. Therefore, suitable hormonal monitoring of glucocorticoid replacement would be highly desirable in AYAs with PAI. We investigated whether quantitative targeted gas chromatography-mass spectrometry urinary steroid metabolome analysis would be suitable for monitoring glucocorticoid replacement in AYAs with autoimmune PAI.

Method: Retrospective analysis of 21 urinary steroid profiles of four AYAs aged 15.6 +/- 2.0 years with autoimmune PAI on hydrocortisone and fludrocortisone treatment. 24-hr cortisol metabolite excretion rates (CMERs) were calculated using the sum of major seven urinary cortisol metabolites. CMERs were transformed into z-scores according to reference values of healthy age- and sex matched subjects.

Results: Three patients showed good treatment adherence (17 of 21 samples). Mean CMER of these samples was 7.4 +/- 1.8 mg/m(2)/d, corresponding to a z-score of 1.8 +/- 1.1. CMER reflected 59.7 +/- 14.5% of prescribed hydrocortisone dosages. A forth patient displayed clinical symptoms of PM during treatment. CMER was only 0.3 mg/m(2) ( -3.4 z), reflecting only 3.1% of prescribed hydrocortisone dosage, compatible with lack of treatment adherence. Thereafter, the parents supervised the intake of tablets and treatment adherence improved.

Conclusion: Quantitative targeted GCMS steroid metabolome analysis could support monitoring of glucocorticoid replacement treatment in patients with PAI.

Harvard Citation style: Kamrath, C., Hartmann, M. and Wudy, S. (2019) Quantitative targeted GC-MS-based urinary steroid metabolome analysis for treatment monitoring of adolescents and young adults with autoimmune primary adrenal insufficiency, Steroids, 150, Article 108426. https://doi.org/10.1016/j.steroids.2019.108426

APA Citation style: Kamrath, C., Hartmann, M., & Wudy, S. (2019). Quantitative targeted GC-MS-based urinary steroid metabolome analysis for treatment monitoring of adolescents and young adults with autoimmune primary adrenal insufficiency. Steroids. 150, Article 108426. https://doi.org/10.1016/j.steroids.2019.108426