ArticleEuropean journal of endocrinology2016
Adrenal-derived 11-oxygenated 19-carbon steroids are the dominant androgens in classic 21-hydroxylase deficiency.
Article in European journal of endocrinology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 101 papers, 1 of them a synthesis that pooled it.
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Who cites it
101 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline.The Journal of clinical endocrinology and metabolism · 2018Guideline
- 24-Hour Profiles of 11-Oxygenated CFrontiers in endocrinology · 2021Trial
- Approach to the patient: investigation of postmenopausal androgen excess.The Journal of clinical endocrinology and metabolism · 2026Review
- Steroid 5α-reductases in the brain: From neurosteroidogenesis to therapeutic implications.Frontiers in neuroendocrinology · 2026Review
- 21-Deoxycortisone: A Novel Sensitive and Specific Newborn Screening Marker for Congenital Adrenal Hyperplasia.International journal of neonatal screening · 2026Article
- Development of an Anti-Immunocomplex Antibody and Non-competitive Immunoassay for the Detection of Testosterone.Analytical chemistry · 2026Article
- Characteristics of androgen metabolic pathways in patients with 21-hydroxylase deficiency and their association with disease control status.Frontiers in endocrinology · 2026Article
- Current insights into monitoring of congenital adrenal hyperplasia.Frontiers in endocrinology · 2026Review
- Serum 11-ketotestosterone is associated with overall disease-control status but adds limited information beyond androstenedione in children with 21-hydroxylase deficiency.Frontiers in endocrinology · 2026Article
- FDA-Approved Fluorine-Containing Molecules in 2024: Significance, Synthesis, and Therapeutic Applications.Current topics in medicinal chemistry · 2026Review
- Salivary Testosterone, Androstenedione and 11-Oxygenated 19-Carbon Concentrations Differ by Age and Sex in Children.Clinical endocrinology · 2025Article
- Radioactive Tracing of Testosterone Reveals Minimal Formation of 5α-DHT in SGBS Cells and Human Primary Adipocytes.Journal of the Endocrine Society · 2025Article
- POLYCYSTIC OVARY SYNDROME: ORIGINS AND IMPLICATIONS: The significance of functional adrenal hyperandrogenism in polycystic ovary syndrome across the lifespan.Reproduction (Cambridge, England) · 2025Review
- Artifactual elevations of 11β-hydroxyandrostenedione and 11-ketoandrostenedione in mass spectrometry assays.The Journal of steroid biochemistry and molecular biology · 2025Article
- Impaired 11β-Hydroxysteroid Dehydrogenase Type 2 Activity in Kidney Disease Disrupts 11-Oxygenated Androgen Biosynthesis.The Journal of clinical endocrinology and metabolism · 2025Observational
- Human Gonads Do Not Contribute to the Circulating Pool of 11-Oxygenated Androgens.The Journal of clinical endocrinology and metabolism · 2025Article
- Genetics and Pathophysiology of Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.The Journal of clinical endocrinology and metabolism · 2025Review
- Exploring the Predictive Role of 11-Oxyandrogens in Diagnosing Polycystic Ovary Syndrome.Endocrinology, diabetes & metabolism · 2025Article
- The Role of 11-Oxygenated Androgens and Endocrine Disruptors in Androgen Excess Disorders in Women.International journal of molecular sciences · 2024Article
- Serum 21-Deoxycortisol for Diagnosis of Nonclassic Congenital Adrenal Hyperplasia in Women With Androgen Excess.The Journal of clinical endocrinology and metabolism · 2023Article
41 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
9 authors.
Funding
Abstract
objectiveTo comprehensively characterize androgens and androgen precursors in classic 21-hydroxylase deficiency (21OHD) and to gain insights into the mechanisms of their formation.
designSerum samples were obtained from 38 patients (19 men) with classic 21OHD, aged 3-59, and 38 sex- and age-matched controls; 3 patients with 11β-hydroxylase deficiency; 4 patients with adrenal insufficiency; and 16 patients (8 men) undergoing adrenal vein sampling. Paraffin-embedded normal (n = 5) and 21OHD adrenal tissues (n = 3) were used for immunohistochemical studies.
methodsWe measured 11 steroids in all sera by liquid chromatography-tandem mass spectrometry. Immunofluroescence localized 3β-hydroxysteroid dehydrogenase type 2 (HSD3B2) and cytochrome b5 (CYB5A) within the normal and 21OHD adrenals.
resultsFour 11-oxygenated 19-carbon (11oxC19) steroids were significantly higher in male and female 21OHD patients than in controls: 11β-hydroxyandrostenedione, 11-ketoandrostenedione 11β-hydroxytestosterone, and 11-ketotestosterone (3-4-fold, P < 0.0001). For 21OHD patients, testosterone and 11-ketotestosterone were positively correlated in females, but inversely correlated in males. All 11oxC19 steroids were higher in the adrenal vein than in the inferior vena cava samples from men and women and rose with cosyntropin stimulation. Only trace amounts of 11oxC19 steroids were found in the sera of patients with 11β-hydroxylase deficiency and adrenal insufficiency, confirming their adrenal origin. HSD3B2 and CYB5A immunoreactivities were sharply segregated in the normal adrenal glands, whereas areas of overlapping expression were identified in the 21OHD adrenals.
conclusionsAll four 11oxC19 steroids are elevated in both men and women with classic 21OHD. Our data suggest that 11oxC19 steroids are specific biomarkers of adrenal-derived androgen excess.
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