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Query: UMLS:C0338671 (Steroids)
 9,479 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

As a four hour morning test, plasma cortisol levels were radioimmunoassayed before and at two and four hours after dexamethasone (0, 0.5 mg, 1.0 mg or 2.0 mg) was administered at 8-9 a.m. in 20 normal subjects. The 1.0 mg four hour test was most effective in suppression of cortisol and it showed the same suppressibility as the widely used single-dose overnight test. With the 1.0 mg four hour test, 2 patients with Cushing's syndrome due to adrenal hyperplasia could be differentiated from normal and obese subjects. The four hour morning test would be more useful than the widely used overnight test from the reasons; i) it shows the same suppressibility as the overnight test, ii) it obviates the need for bothersome midnight administration of dexamethasone, iii) because it takes only one morning to perform, it can save a day, iv) and it might be applicable for the differential diagnosis of Cushing's syndrome because 4.0 mg morning test resulted in complete suppression of plasma cortisol in a tested Cushing's syndrome, whereas with even 8.0 mg, plasma cortisol was not suppressed in the overnight test in 2 such patients examined.
Steroids 1976 Sep
PMID:Single-dose four hour dexamethasone suppression test in normal men and its application for the diagnosis of Cushing's syndrome. 98 98

Urinary steroidal profiles were studied in five women with Cushing's syndrome and in two normal women that were chosen as controls, by means of gas chromatography and gas chromatography-mass spectrometry. Four patients presented ACTH-dependent adrenal hyperplasia and the last patient had an adrenocortical carcinoma. Steroids were analyzed in urinary extracts, as their respective trimethylsilyloximes and/or trimethylsilylderivatives. Qualitative and quantitative data about 36 urinary steroids were obtained. Three pituitary patients showed a well defined picture of "5-ene pathway" in adrenal function. The fourth patient depicted some primary deficiencies of corticosteroid biosynthesis that overshadowed most biochemical expressions of Cushing's disease. The patient with adrenal carcinoma developed a steroidal pattern resembling autonomous functioning of adrenal cortex inner zones, showing both "5-ene pathway" and "4-ene pathway" increase. This patient also had an unexpected excretion of a major metabolite of 18-hydroxycorticosterone, that did not correlate with parameters of aldosterone production. Tetrahydro-6-hydroxy-cortisol was determined in urine of two patients and original data about urinary cortoic acids in Cushing's syndrome are given. Peripheral reductive metabolism played the most important role in almost all patients. In turn, some oxidative metabolic pathways for cortisol were not specifically favoured in the cases of Cushing's disease here reported.
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PMID:Analysis of urinary steroid profiles of women with Cushing's syndrome by computerised gas chromatography-mass spectrometry. 667 7

A method was developed for the estimation of levels of cortisol-21-sulfate (F KS), cortisone-21-sulfate (ES), and 20(alpha + beta)-reduced cortisol-21-sulfates in blood plasma. Levels of these conjugates were determined in peripheral vein plasma of 42 normal subjects, 21 men, and 21 women (age range 20-64 years) and in adrenal vein plasma of patients with various adrenocortical disorders, six patients with primary hyperaldosteronism, five patients with Cushing's syndrome, and in two obese patients, suspected to have Cushing's syndrome, but with inconclusive laboratory findings. Adrenal vein blood was obtained by percutaneous, trans-femoral adrenal vein catheterization. Levels of non-conjugated (free) cortisol were determined in all plasma samples along with those of the sulfated steroids. F kappa S was found in all plasma samples, both in men and women. The variation in F kappa S levels paralleled that in the free cortisol levels, thus the ratio of F kappa/F kappa S was the same in the blood samples drawn at 8 AM as in those drawn at 4 PM or 5 PM (ranges: 17.5-36.3 in men, 23.6-45.8 in women). The levels of F kappa S were relatively lower in women than in men (women 610-880 ng/100 mL at AM, 300-510 ng/100 mL at PM; men: 760-1,220 ng/100 mL at AM, 380-760 ng/100 mL at PM). Plasma levels of total sulfate-conjugated delta 4-3-keto-C-21 steroids (F kappa S + E kappa S + 20(alpha+beta)-dihydrocortisol-21-sulfates) were 30-40% higher than those of the levels of cortisol-21-sulfate alone (separated by thin-layer chromatography). In the adrenal vein plasma, levels of delta 4-3-keto-C-21-steroid-21-yl sulfates were 20 to 40 times higher than levels of these steroids in the peripheral blood. The bulk of the steroid sulfate measured in the adrenal vein plasma consisted of cortisol-21-sulfate. The ratio of F kappa/F kappa S in the adrenal vein plasma was markedly smaller than in the peripheral vein plasma; it was 6.9-12.3 in males and 4.9-6.7 in females, whereas in the peripheral vein of the same subjects it was 19.2-43.7 in males and 21.4-48.3 in females. Cortisol-21-sulfate isolated from adrenal vein plasma was identified by mass spectrometry. The data presented provide evidence for the secretion of this conjugate by the adrenal cortex. Its secretion appears to be markedly elevated in patients with Cushing's syndrome, both due to hyperplasia and due to adrenal adenoma, as compared with normal subjects and patients with primary aldosteronism, both males and females. However, the F kappa/F kappa S ratio was markedly lower in Cushing's patients due to adrenal adenoma than due to adrenal hyperplasia, this suggesting that ACTH is stimulating intra-adrenal hydrolysis of cortisol sulfate.
Steroids 1995 Dec
PMID:Corticosteroids in human blood: IX. Evidence for adrenal secretion of sulfate-conjugated cortisol, 11 beta,17 alpha-dihydroxy-4-pregnene-3,20-dione-21-yl-sulfate. 865 Jul 5

Congenital lipoid adrenal hyperplasia (CLAH) is a rare autosomal recessive disorder caused by defective synthesis of all steroids. This disorder is characterized by 46,XY sex reversal, skin hyperpigmentation, early-onset adrenal crisis and enlarged adrenal with fatty accumulation. CLAH is caused by mutations in the STAR gene. The clinical features and STAR gene mutation spectrum of a large cohort of Chinese patients with CLAH were not reported previously. We performed clinical retrospective review and genetic analysis of the STAR gene in ten unrelated Chinese phenotypic female patients who were clinically diagnosed with CLAH and followed up in our hospital from 2006 to 2015. All ten patients, including two 46,XY females and eight 46,XX females, presented skin hyperpigmentation and early salt-wasting episode, and showed normal growth and development after steroid replacement treatment. Totally 20 mutant alleles containing 11 different STAR gene mutations were identified in these ten patients, including five novel variants (two missense and three null variants), all predicted to be pathogenic in bioinformatics analysis, and six mutations described in previous literature. Among these 11 mutations, a reported mutation c.772C>T and a novel variant c.707_708delinsCTT were most frequent, accounting for 35% and 15% of the total mutant alleles, respectively. This is the first report of a large Chinese cohort with CLAH, presenting the mutation spectrum of the STAR gene and two possible founder mutations in the Chinese population, which may contribute to better genetic counseling and prenatal diagnosis.
Steroids 2016 Apr
PMID:Identification of five novel STAR variants in ten Chinese patients with congenital lipoid adrenal hyperplasia. 2682 27