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Elevated 18-hydroxycorticosterone to aldosterone ratio

MedGen UID:
1054077
•Concept ID:
CN377346
•
Finding
HPO: HP:6000515

Definition

The relative concentration in the blood circulation of 18-hydroxycorticosterone compared to that of aldosterone is above the upper limit of normal. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVElevated 18-hydroxycorticosterone to aldosterone ratio

Recent clinical studies

Etiology

Measurement of 18-hydroxycorticosterone during adrenal vein sampling for primary aldosteronism.
Auchus RJ, Chandler DW, Singeetham S, Chokshi N, Nwariaku FE, Dolmatch BL, Holt SA, Wians FH Jr, Josephs SC, Trimmer CK, Lopera J, Vongpatanasin W, Nesbitt SD, Leonard D, Victor RG
J Clin Endocrinol Metab 2007 Jul;92(7):2648-51. Epub 2007 May 1 doi: 10.1210/jc.2006-2631. PMID: 17473070
Steroids and hypertension.
Mantero F, Armanini D, Boscaro M, Carpenè G, Fallo F, Opocher G, Rocco S, Scaroni C, Sonino N
J Steroid Biochem Mol Biol 1991;40(1-3):35-44. doi: 10.1016/0960-0760(91)90165-2. PMID: 1958539
The constant plasma 18-hydroxycorticosterone to aldosterone ratio: an expression of the efficacy of corticosterone methyloxidase type II activity in disorders with variable aldosterone production.
Kater CE, Biglieri EG, Rost CR, Schambelan M, Hirai J, Chang BC, Brust N
J Clin Endocrinol Metab 1985 Feb;60(2):225-8. doi: 10.1210/jcem-60-2-225. PMID: 2981240
The natural history of salt-wasting disorders of adrenal and renal origin.
Rösler A
J Clin Endocrinol Metab 1984 Oct;59(4):689-700. doi: 10.1210/jcem-59-4-689. PMID: 6384251

Diagnosis

Measurement of 18-hydroxycorticosterone during adrenal vein sampling for primary aldosteronism.
Auchus RJ, Chandler DW, Singeetham S, Chokshi N, Nwariaku FE, Dolmatch BL, Holt SA, Wians FH Jr, Josephs SC, Trimmer CK, Lopera J, Vongpatanasin W, Nesbitt SD, Leonard D, Victor RG
J Clin Endocrinol Metab 2007 Jul;92(7):2648-51. Epub 2007 May 1 doi: 10.1210/jc.2006-2631. PMID: 17473070
Congenital hypoaldosteronism: the Visser-Cost syndrome revisited.
Peter M, Sippell WG
Pediatr Res 1996 Mar;39(3):554-60. doi: 10.1203/00006450-199603000-00027. PMID: 8929880
Multisteroid analysis in children with terminal aldosterone biosynthesis defects.
Peter M, Partsch CJ, Sippell WG
J Clin Endocrinol Metab 1995 May;80(5):1622-7. doi: 10.1210/jcem.80.5.7745009. PMID: 7745009
Steroids and hypertension.
Mantero F, Armanini D, Boscaro M, Carpenè G, Fallo F, Opocher G, Rocco S, Scaroni C, Sonino N
J Steroid Biochem Mol Biol 1991;40(1-3):35-44. doi: 10.1016/0960-0760(91)90165-2. PMID: 1958539
The nature of the defect in a salt-wasting disorder in Jews of Iran.
Rösler A, Rabinowitz D, Theodor R, Ramirez LC, Ulick S
J Clin Endocrinol Metab 1977 Feb;44(2):279-91. doi: 10.1210/jcem-44-2-279. PMID: 838841

Therapy

Severe hypoaldosteronism due to corticosterone methyl oxidase type II deficiency in two boys: metabolic and gas chromatography-mass spectrometry studies.
Hauffa BP, Sólyom J, Gláz E, Shackleton CH, Wambach G, Vecsei P, Stolecke H, Homoki J
Eur J Pediatr 1991 Jan;150(3):149-53. doi: 10.1007/BF01963554. PMID: 2044581
Biochemical diagnosis and management of corticosterone methyl oxidase type II deficiency.
Lee PD, Patterson BD, Hintz RL, Rosenfeld RG
J Clin Endocrinol Metab 1986 Jan;62(1):225-9. doi: 10.1210/jcem-62-1-225. PMID: 3510001
Studies of impaired aldosterone response to spironolactone-induced renin and potassium elevations in adenomatous but not hyperplastic primary aldosteronism.
Kater CE, Biglieri EG, Schambelan M, Arteaga E
Hypertension 1983 Nov-Dec;5(6 Pt 3):V115-21. doi: 10.1161/01.hyp.5.6_pt_3.v115. PMID: 6360878
The nature of the defect in a salt-wasting disorder in Jews of Iran.
Rösler A, Rabinowitz D, Theodor R, Ramirez LC, Ulick S
J Clin Endocrinol Metab 1977 Feb;44(2):279-91. doi: 10.1210/jcem-44-2-279. PMID: 838841

Clinical prediction guides

The effects of CGS 16949A, an aromatase inhibitor on adrenal mineralocorticoid biosynthesis.
Demers LM, Melby JC, Wilson TE, Lipton A, Harvey HA, Santen RJ
J Clin Endocrinol Metab 1990 Apr;70(4):1162-6. PMID: 2156889
Biochemical diagnosis and management of corticosterone methyl oxidase type II deficiency.
Lee PD, Patterson BD, Hintz RL, Rosenfeld RG
J Clin Endocrinol Metab 1986 Jan;62(1):225-9. doi: 10.1210/jcem-62-1-225. PMID: 3510001
The significance of elevated levels of plasma 18-hydroxycorticosterone in patients with primary aldosteronism.
Biglieri EG, Schambelan M
J Clin Endocrinol Metab 1979 Jul;49(1):87-91. doi: 10.1210/jcem-49-1-87. PMID: 447820

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