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Increased urinary 11-deoxycortisol level

MedGen UID:
1698442
•Concept ID:
C5139271
•
Finding
HPO: HP:0032329

Definition

An abnormally elevated concentration of 11-deoxycortisol in the urine. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIncreased urinary 11-deoxycortisol level

Professional guidelines

PubMed

Steroid profile in urine: a useful tool in the diagnosis and follow up of adrenocortical carcinoma.
Gröndal S, Eriksson B, Hagenäs L, Werner S, Curstedt T
Acta Endocrinol (Copenh) 1990 May;122(5):656-63. doi: 10.1530/acta.0.1220656. PMID: 2141212
11 Beta-hydroxylase deficiency congenital adrenal hyperplasia: update of prenatal diagnosis.
Rösler A, Weshler N, Leiberman E, Hochberg Z, Weidenfeld J, Sack J, Chemke J
J Clin Endocrinol Metab 1988 Apr;66(4):830-8. doi: 10.1210/jcem-66-4-830. PMID: 3346360
Use of ketoconazole in the treatment of Cushing's syndrome.
Loli P, Berselli ME, Tagliaferri M
J Clin Endocrinol Metab 1986 Dec;63(6):1365-71. doi: 10.1210/jcem-63-6-1365. PMID: 3023421

Recent clinical studies

Etiology

Reduced activity of 11β-hydroxylase accounts for elevated 17α-hydroxyprogesterone in preterms.
Kamrath C, Hartmann MF, Boettcher C, Wudy SA
J Pediatr 2014 Aug;165(2):280-4. Epub 2014 May 23 doi: 10.1016/j.jpeds.2014.04.011. PMID: 24862381
Comprehensive study of urinary cortisol metabolites in hyperthyroid and hypothyroid patients.
Hoshiro M, Ohno Y, Masaki H, Iwase H, Aoki N
Clin Endocrinol (Oxf) 2006 Jan;64(1):37-45. doi: 10.1111/j.1365-2265.2005.02412.x. PMID: 16402926
Extra-adrenal effects of metyrapone include inhibition of the 11-oxoreductase activity of 11 beta-hydroxysteroid dehydrogenase: a model for 11-HSD I deficiency.
Raven PW, Checkley SA, Taylor NF
Clin Endocrinol (Oxf) 1995 Nov;43(5):637-44. doi: 10.1111/j.1365-2265.1995.tb02930.x. PMID: 8548950
11 Beta-hydroxylase deficiency congenital adrenal hyperplasia: update of prenatal diagnosis.
Rösler A, Weshler N, Leiberman E, Hochberg Z, Weidenfeld J, Sack J, Chemke J
J Clin Endocrinol Metab 1988 Apr;66(4):830-8. doi: 10.1210/jcem-66-4-830. PMID: 3346360
Adult manifestation of congenital adrenal hyperplasia due to incomplete 21-hydroxylase deficiency mimicking polycystic ovarian disease.
Lobo RA, Goebelsmann U
Am J Obstet Gynecol 1980 Nov 15;138(6):720-6. doi: 10.1016/0002-9378(80)90095-2. PMID: 6254362

Diagnosis

Reduced activity of 11β-hydroxylase accounts for elevated 17α-hydroxyprogesterone in preterms.
Kamrath C, Hartmann MF, Boettcher C, Wudy SA
J Pediatr 2014 Aug;165(2):280-4. Epub 2014 May 23 doi: 10.1016/j.jpeds.2014.04.011. PMID: 24862381
Licorice - or more?
Leitolf H, Dixit KC, Higham CE, Brabant G
Exp Clin Endocrinol Diabetes 2010 Apr;118(4):250-3. Epub 2010 Mar 8 doi: 10.1055/s-0029-1224123. PMID: 20213599
Urinary steroid profile in adrenocortical tumors.
Kikuchi E, Yanaihara H, Nakashima J, Homma K, Ohigashi T, Asakura H, Tachibana M, Shibata H, Saruta T, Murai M
Biomed Pharmacother 2000 Jun;54 Suppl 1:194s-197s. doi: 10.1016/s0753-3322(00)80043-8. PMID: 10915023
11 Beta-hydroxylase deficiency congenital adrenal hyperplasia: update of prenatal diagnosis.
Rösler A, Weshler N, Leiberman E, Hochberg Z, Weidenfeld J, Sack J, Chemke J
J Clin Endocrinol Metab 1988 Apr;66(4):830-8. doi: 10.1210/jcem-66-4-830. PMID: 3346360
Plasma 11-deoxycortisol, androstenedione, testosterone and ACTH in comparison with the urinary excretion of tetrahydro-11-deoxycortisol as indices of the pituitary-adrenal response to oral metyrapone.
de Lange WE, Sluiter WJ, Pratt JJ, Doorenbos H
Acta Endocrinol (Copenh) 1980 Apr;93(4):488-94. doi: 10.1530/acta.0.0930488. PMID: 6247869

Therapy

Clinical and biochemical consequences of CYP17A1 inhibition with abiraterone given with and without exogenous glucocorticoids in castrate men with advanced prostate cancer.
Attard G, Reid AH, Auchus RJ, Hughes BA, Cassidy AM, Thompson E, Oommen NB, Folkerd E, Dowsett M, Arlt W, de Bono JS
J Clin Endocrinol Metab 2012 Feb;97(2):507-16. Epub 2011 Dec 14 doi: 10.1210/jc.2011-2189. PMID: 22170708
Licorice - or more?
Leitolf H, Dixit KC, Higham CE, Brabant G
Exp Clin Endocrinol Diabetes 2010 Apr;118(4):250-3. Epub 2010 Mar 8 doi: 10.1055/s-0029-1224123. PMID: 20213599
Urinary steroid profile in adrenocortical tumors.
Kikuchi E, Yanaihara H, Nakashima J, Homma K, Ohigashi T, Asakura H, Tachibana M, Shibata H, Saruta T, Murai M
Biomed Pharmacother 2000 Jun;54 Suppl 1:194s-197s. doi: 10.1016/s0753-3322(00)80043-8. PMID: 10915023
Specificity of low dose fadrozole hydrochloride (CGS 16949A) as an aromatase inhibitor.
Santen RJ, Demers LM, Lynch J, Harvey H, Lipton A, Mulagha M, Hanagan J, Garber JE, Henderson IC, Navari RM
J Clin Endocrinol Metab 1991 Jul;73(1):99-106. doi: 10.1210/jcem-73-1-99. PMID: 1646219
Use of ketoconazole in the treatment of Cushing's syndrome.
Loli P, Berselli ME, Tagliaferri M
J Clin Endocrinol Metab 1986 Dec;63(6):1365-71. doi: 10.1210/jcem-63-6-1365. PMID: 3023421

Prognosis

Steroid profile in serum: increased levels of sulphated pregnenolone and pregn-5-ene-3 beta,20 alpha-diol in patients with adrenocortical carcinoma.
Gröndal S, Curstedt T
Acta Endocrinol (Copenh) 1991 Apr;124(4):381-5. doi: 10.1530/acta.0.1240381. PMID: 2031435
Steroid profile in urine: a useful tool in the diagnosis and follow up of adrenocortical carcinoma.
Gröndal S, Eriksson B, Hagenäs L, Werner S, Curstedt T
Acta Endocrinol (Copenh) 1990 May;122(5):656-63. doi: 10.1530/acta.0.1220656. PMID: 2141212
11 Beta-hydroxylase deficiency congenital adrenal hyperplasia: update of prenatal diagnosis.
Rösler A, Weshler N, Leiberman E, Hochberg Z, Weidenfeld J, Sack J, Chemke J
J Clin Endocrinol Metab 1988 Apr;66(4):830-8. doi: 10.1210/jcem-66-4-830. PMID: 3346360
Prenatal diagnosis of 11beta-hydroxylase deficiency congenital adrenal hyperplasia.
Rösler A, Leiberman E, Rosenmann A, Ben-Uzilio R, Weidenfeld J
J Clin Endocrinol Metab 1979 Oct;49(4):546-51. doi: 10.1210/jcem-49-4-546. PMID: 314453

Clinical prediction guides

Urinary steroid profile in adrenocortical tumors.
Kikuchi E, Yanaihara H, Nakashima J, Homma K, Ohigashi T, Asakura H, Tachibana M, Shibata H, Saruta T, Murai M
Biomed Pharmacother 2000 Jun;54 Suppl 1:194s-197s. doi: 10.1016/s0753-3322(00)80043-8. PMID: 10915023
Specificity of low dose fadrozole hydrochloride (CGS 16949A) as an aromatase inhibitor.
Santen RJ, Demers LM, Lynch J, Harvey H, Lipton A, Mulagha M, Hanagan J, Garber JE, Henderson IC, Navari RM
J Clin Endocrinol Metab 1991 Jul;73(1):99-106. doi: 10.1210/jcem-73-1-99. PMID: 1646219
11 Beta-hydroxylase deficiency congenital adrenal hyperplasia: update of prenatal diagnosis.
Rösler A, Weshler N, Leiberman E, Hochberg Z, Weidenfeld J, Sack J, Chemke J
J Clin Endocrinol Metab 1988 Apr;66(4):830-8. doi: 10.1210/jcem-66-4-830. PMID: 3346360
Plasma 11-deoxycortisol, androstenedione, testosterone and ACTH in comparison with the urinary excretion of tetrahydro-11-deoxycortisol as indices of the pituitary-adrenal response to oral metyrapone.
de Lange WE, Sluiter WJ, Pratt JJ, Doorenbos H
Acta Endocrinol (Copenh) 1980 Apr;93(4):488-94. doi: 10.1530/acta.0.0930488. PMID: 6247869
Licorice raises urinary cortisol in man.
Epstein MT, Espiner EA, Donald RA, Hughes H, Cowles RJ, Lun S
J Clin Endocrinol Metab 1978 Aug;47(2):397-400. doi: 10.1210/jcem-47-2-397. PMID: 233669

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