Congenital deficiency of 11β-hydroxysteroid dehydrogenase (apparent mineralocorticoid excess syndrome): Diagnostic value of urinary free cortisol and cortisone

2001 ◽  
Vol 24 (1) ◽  
pp. 17-23 ◽  
Author(s):  
Mario Palermo ◽  
G. Delitala ◽  
F. Mantero ◽  
P. M. Stewart ◽  
C. H. L. Shackleton
Keyword(s):  
Hydroxysteroid Dehydrogenase ◽  
Urinary Free Cortisol ◽  
Diagnostic Value ◽  
Congenital Deficiency ◽  
Free Cortisol ◽  
Apparent Mineralocorticoid Excess ◽  
Apparent Mineralocorticoid Excess Syndrome

scholarly journals Modulation of 11β-Hydroxysteroid Dehydrogenase (11βHSD) Activity Biomarkers and Pharmacokinetics of PF-00915275, a Selective 11βHSD1 Inhibitor

2008 ◽  
Vol 93 (2) ◽  
pp. 550-556 ◽  
Author(s):  
Rachel Courtney ◽  
Paul M. Stewart ◽  
Melvin Toh ◽  
Marie-Noella Ndongo ◽  
Roberto A. Calle ◽  
...  
Keyword(s):  
Enzyme Activity ◽  
Phase 1 ◽  
Oral Prednisone ◽  
Hydroxysteroid Dehydrogenase ◽  
Urinary Free Cortisol ◽  
Research Unit ◽  
Double Blind ◽  
Free Cortisol ◽  
Oral Doses

Abstract Context: 11β-Hydroxysteroid dehydrogenase type 1 (11βHSD1) is a promising target for the treatment of type 2 diabetes mellitus. 11βHSD1 catalyzes the intracrine conversion of inactive cortisone to the active glucocorticoid cortisol. Objective: Demonstrating inhibition of 11βHSD1 is challenging because there is no accessible way to directly assess the enzyme activity in vivo. Thus, it was proposed to assess the enzyme activity, in an indirect fashion, using two biomarker methods: the prednisolone generation study (conversion of oral prednisone to prednisolone in plasma) and the ratio of cortisol and cortisone metabolites in urine. Design: This was a phase 1, double-blind, placebo-controlled, randomized, multiple-dose study. Setting: The study was conducted in a clinical research unit. Participants: Sixty healthy adult volunteers participated in the study. Intervention: Oral doses of PF-00915275 (0.3–15 mg) and prednisone (10 mg) were administered during the study. Main Outcome Measures: Safety, tolerability, pharmacokinetics, and pharmacodynamics of PF-00915275, a selective 11βHSD1 inhibitor, were measured. Results: Overall, multiple oral doses of PF-00915275 were safe and well tolerated. After oral administration, PF-00915275 was rapidly absorbed, slowly eliminated, and generally displayed dose-proportional increases in exposure. At the 15-mg dose, mean exposure to prednisolone was reduced by 37%, and there was a dose-dependent fall in the 5α-tetrahydrocortisol + 5β-tetrahydrocortisol to tetrahydrocortisone ratio with maximum inhibition of 26% after 14 d. The urinary free cortisol to urinary free cortisone ratio, an indicator of 11βHSD2 inhibition, did not change. Conclusion: PF-00915275 was safe at all doses tested. The results of the prednisolone generation test and the urinary metabolite ratios confirm that PF-00915275 is a selective 11βHSD1 inhibitor.

Urinary free cortisol versus 17-hydroxycorticosteroids a comparative study of their diagnostic value in Cushing's syndrome

1992 ◽  
Vol 70 (7) ◽  
Author(s):  
T. Mengden ◽  
P. Hubmann ◽  
J. M�ller ◽  
P. Greminger ◽  
W. Vetter
Keyword(s):  
Comparative Study ◽  
Cushing’S Syndrome ◽  
Urinary Free Cortisol ◽  
Diagnostic Value ◽  
Cushing's Syndrome ◽  
Free Cortisol

How useful is 24 hour Urinary Free Cortisol as a screening tool for Cushing's syndrome?

2018 ◽  
Author(s):  
Ahmed Hanafy ◽  
Chinnadorai Rajeswaran ◽  
Saad Saddiq ◽  
Warren Gillibrand ◽  
John Stephenson
Keyword(s):  
Cushing’S Syndrome ◽  
Screening Tool ◽  
Urinary Free Cortisol ◽  
Cushing's Syndrome ◽  
Free Cortisol

The R337C mutation generates a high Km 11 beta-hydroxysteroid dehydrogenase type II enzyme in a family with apparent mineralocorticoid excess.

1995 ◽  
Vol 80 (11) ◽  
pp. 3381-3383 ◽  
Author(s):  
V R Obeyesekere ◽  
P Ferrari ◽  
R K Andrews ◽  
R C Wilson ◽  
M I New ◽  
...  
Keyword(s):  
Hydroxysteroid Dehydrogenase ◽  
Type Ii ◽  
Apparent Mineralocorticoid Excess

A Comparative Study of Urinary 17-Hydroxycorticosteroids, Urinary Free Cortisol, and the Integrated Concentration of Plasma Cortisol*

1980 ◽  
Vol 51 (5) ◽  
pp. 1099-1101 ◽  
Author(s):  
ZVI ZADIK ◽  
LUIZ DE LACERDA ◽  
LUIZ A. H. DE CARMARGO ◽  
BRUCE P. HAMILTON ◽  
CLAUDE J. MIGEON ◽  
...  
Keyword(s):  
Comparative Study ◽  
Plasma Cortisol ◽  
Urinary Free Cortisol ◽  
Free Cortisol

THE EFFECTS OF DEXAMETHASONE AND ANDROGENS ON SEXUAL RECEPTIVITY OF FEMALE RHESUS MONKEYS

1971 ◽  
Vol 51 (3) ◽  
pp. 575-588 ◽  
Author(s):  
B. J. EVERITT ◽  
J. HERBERT
Keyword(s):  
Rhesus Monkeys ◽  
Testosterone Propionate ◽  
Secretory Activity ◽  
Urinary Free Cortisol ◽  
Sexual Receptivity ◽  
Adrenal Androgens ◽  
Electrolyte Metabolism ◽  
Free Cortisol ◽  
Female Rhesus ◽  
The Central Nervous System

SUMMARY The effect of dexamethasone, given either alone or together with testosterone propionate or androstenedione, was studied in nine female rhesus monkeys (paired with three males) by making quantitative observations on behaviour in the laboratory. Dexamethasone (0·5 mg/kg/day) given to oestrogen-treated ovariectomized female monkeys made them sexually unreceptive, and there was an associated decline in the level of the male's mounting activity. Testosterone propionate (100 or 200 μg/day) reversed completely the effects of dexamethasone on sexual behaviour. Androstenedione (100, 200 or 400 μg/day) had similar, but less marked, effects whereas cortisol (10 mg/day) or progesterone (100, 200 or 500 μg/day) were ineffective. Treating a female with testosterone prevented dexamethasone from reducing sexual receptivity. Parallel determinations of urinary free cortisol showed that the dexamethasone had suppressed the secretory activity of the adrenal cortex. There were no consistent changes, under any treatment, in the females' vaginal epithelia, sexual skins or clitorides, or in their water or electrolyte metabolism. These findings indicate that adrenal androgens regulate sexual receptivity in these female primates, probably by an action on the central nervous system.

scholarly journals Relationships Between 24-Hour Urinary Free Cortisol Concentrations and Metabolic Syndrome in Obese Children

2014 ◽  
Vol 99 (7) ◽  
pp. 2391-2399 ◽  
Author(s):  
Thomas Reinehr ◽  
Alexandra Kulle ◽  
Barbara Wolters ◽  
Caroline Knop ◽  
Nina Lass ◽  
...  
Keyword(s):  
Metabolic Syndrome ◽  
Urinary Free Cortisol ◽  
Obese Children ◽  
Free Cortisol

Paediatric reference range for overnight urinary cortisol corrected for creatinine

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Ole D. Wolthers ◽  
Mark Lomax ◽  
Anne Vibeke Schmedes
Keyword(s):  
Mass Spectrometry ◽  
Reference Range ◽  
Inhaled Corticosteroids ◽  
Prediction Interval ◽  
Geometric Mean ◽  
Urinary Free Cortisol ◽  
Urinary Cortisol ◽  
Cross Sectional ◽  
Systemic Activity ◽  
Free Cortisol

Abstract Objectives Systemic activity of inhaled corticosteroids (ICS) may be assessed via urinary cortisol measurement. Overnight urinary free cortisol corrected for creatinine (OUFCC) has been extensively reported in adult studies. However, a paediatric mass spectrometric (MS) reference range for OUFCC is not established. MS methods for OUFCC avoid cross-reactivity with other steroid hormones and are thus preferable to immunoassays. The aim of the present study was to define an MS OUFCC normative range in children. Methods This was a cross-sectional study of healthy pre-pubertal children from 5 to 11 years. Children collected urine from 10 pm or bedtime, whichever was earlier, until 8 am. Urinary free cortisol was measured via a liquid chromatography tandem mass spectrometry (LC-MS/MS) assay (Acquity UPLC with Xevo TQ-S Mass Spectrometer [Waters]) with in-house reagents. Urinary creatinine was measured using a commercial assay (Roche). Results Complete urine collections were obtained from 72 males and 70 females, mean age (SD) 8.6 (1.9) (range 5.0–11.8) years. The OUFCC 95% prediction interval was 1.7–19.8 nmol/mmol. Geometric mean OUFCC was 5.7; range 1.1–24.8 nmol/mmol. Conclusions The obtained normative LC-MS/MS OUFCC reference data facilitate the use of mass spectrometry OUFCC assays in assessment of systemic activity of endogenous and exogenous corticosteroids in children.

scholarly journals Spontaneous remission of hypercortisolism presumed due to asymptomatic tumor apoplexy in ACTH-producing pituitary macroadenoma

2013 ◽  
Vol 57 (6) ◽  
pp. 486-489 ◽  
Author(s):  
Marcio Carlos Machado ◽  
Patricia Sampaio Gadelha ◽  
Marcello Delano Bronstein ◽  
Maria Candida Barisson Vilares Fragoso
Keyword(s):  
Salivary Cortisol ◽  
Signs And Symptoms ◽  
Spontaneous Remission ◽  
Pituitary Surgery ◽  
Serum Cortisol ◽  
Urinary Free Cortisol ◽  
High Plasma ◽  
Imaging Data ◽  
Cystic Component ◽  
Free Cortisol

Cushing's disease (CD) is usually caused by secretion of ACTH by a pituitary corticotroph microadenoma. Nevertheless, 7%-20% of patients present with ACTH-secreting macroadenomas. Our aim is to report a 36-year-old female patient with CD due to solid-cystic ACTH-macroadenoma followed up during 34 months. The patient presented spontaneous remission due to presumed asymptomatic tumor apoplexy. She showed typical signs and symptoms of Cushing's syndrome (CS). Initial tests were consistent with ACTH-dependent CS: elevated urinary free cortisol, abnormal serum cortisol after low dose dexamethasone suppression test, and elevated midnight salivary cortisol, associated with high plasma ACTH levels. Pituitary magnetic resonance imaging (MRI) showed a sellar mass of 1.2 x 0.8 x 0.8 cm of diameter with supra-sellar extension leading to slight chiasmatic impingement, and showing hyperintensity on T2-weighted imaging, suggesting a cystic component. She had no visual impairment. After two months, while waiting for pituitary surgery, she presented spontaneous resolution of CS. Tests were consistent with remission of hypercortisolism: normal 24-h total urinary cortisol and normal midnight salivary cortisol. Pituitary MRI showed shrinkage of the tumor with disappearance of the chiasmatic compression. She has been free from the disease for 28 months (without hypercortisolism or hypopituitarism). The hormonal and imaging data suggested that silent apoplexy of pituitary tumor led to spontaneous remission of CS. However, recurrence of CS was described in cases following pituitary apoplexy. Therefore, careful long-term follow-up is required.

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