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PubMedDate not supplied

Urinary, plasma and adrenal tissue steroids in a male patient with Cushing's syndrome due to adrenal carcinoma.

The dynamics of urinary and plasma steroids (CPB and RIA) were simultaneously studied in a 36-yr-man with Cushing's syndrome due to adrenal carcinoma. The markedly increased basal excretion of 5-pregnenetriol, PG, PGT, DHA, THS and corticosteroids with nonsignificant changes during the endocrine tests decreased to normal levels after tumour removal. The basal low level of plasma testosterone, with nonsignificant changes during the endocrine tests, rose progressively after surgery. The steroid content of adrenal carcinoma tissue determined by CPB and RIA after LH-20-Sephadex CC separation revealed important proportion of unconjugated 17-OH-P.

Adrenal Gland Neoplasms↗
PubMed1993

Hypothalamo-pituitary-adrenal axis in uraemia: evidence for primary adrenal dysfunction?

To study hypothalamo-pituitary-adrenal axis disturbances in chronic renal failure, we administered corticotrophin-releasing hormone to patients undergoing haemodialysis and CAPD and to normal controls. Cortisol, ACTH, and ACTH precursors were measured before and after corticotrophin-releasing hormone using sensitive and specific two-site assays. Baseline ACTH and cortisol were similar in all groups. Peak values occurred at 30 min (ACTH) and between 30 and 60 min (cortisol). The cortisol (but not the ACTH) responses were significantly reduced in patients with renal failure (P < 0.05). ACTH precursors did not change from baseline following corticotrophin-releasing hormone but were significantly elevated in patients with renal failure compared to controls (P < 0.01). The reduced cortisol response to corticotrophin-releasing hormone may reflect a primary defect of adrenal function in renal failure.

Adolescent↗
PubMed2004

Congenital adrenal hyperplasia and multiple sclerosis: is there an increased risk of multiple sclerosis in individuals with congenital adrenal hyperplasia?

BACKGROUND: Congenital adrenal hyperplasia (CAH) is an inherited recessive disorder of adrenal steroidogenesis, generally caused by a total or partial deficiency in 21-hydroxylase, due to a deletion of or mutations in the CYP21 gene (the gene that codes for 21-hydroxylase). Impaired cortisol biosynthesis results in corticotropin hypersecretion, which leads to overproduction of intermediate metabolites and androgens. OBJECTIVE: To describe for the first time, to our knowledge, a patient with CAH and multiple sclerosis (MS). DESIGN: Case report. PATIENT: A 22-year-old woman, diagnosed at birth as having a salt-losing 21-hydroxylase deficiency, had sudden visual loss in the right eye and pyramidal, sensory, and cerebellar signs. Repeated brain magnetic resonance images showed focal white matter lesions in periventricular areas, the corpus callosum, the cerebellum, and the brainstem. A cerebrospinal fluid examination revealed several oligoclonal bands. Thereafter, she had 2 relapses, characterized by ataxia and diplopia, and recovered after corticosteroid treatment. RESULTS: The reported case fulfills the diagnostic criteria for CAH and MS. CONCLUSIONS: Some clues suggest that the association between CAH and MS could be nonincidental: a possible MS susceptibility locus is on chromosome 6p21, on which the CYP21 gene is located; the CYP21 gene and the CYP21P pseudogene alternate in tandem with the C4 genes (the genes that code for the homonym complement protein) (C4AQ0 is particularly frequent in patients with relapsing-remitting MS); and, in previous studies, brain magnetic resonance imaging showed T2-hyperintense focal areas in the white matter of CAH patients. Our observation should alert neurologists to the presence of signs and symptoms suggestive of late-onset CAH in MS patients and, in turn, endocrinologists to the appearance of neurological signs and symptoms in CAH patients.

Adrenal Hyperplasia, Congenital↗
PubMed2005

Osteosarcoma invading adrenal gland: rare cause of adrenal mass.

Adrenal gland metastasis from osteogenic sarcoma is extremely rare and is an atypical location for metastasis. Vascular hepatic exclusion techniques and the use of venovenous bypass with cardiac surgical techniques may be required to resect large adrenal masses that have invaded the inferior vena cava to achieve curative resection. The use of newer chemotherapeutic agents and aggressive surgical resection have prolonged the survival of patients with osteosarcoma.

Adrenal Gland Neoplasms↗
PubMed1969

Sustained effects of brief daily stress (fighting) upon brain and adrenal catecholamines and adrenal spleen, and heart weights of mice.

Male white Swiss mice that had previously been made aggressive by several weeks of individual housing were allowed to fight for 5 to 10 minutes each day for 5, 10, or 14 consecutive days; fighting caused a marked enlargement of their adrenals, spleens, and hearts, and a large increase in adrenal catecholamines; brain catecholamines were slightly increased. Long-term group caging, under conditions where the mice did not fight, caused changes that were directionally the same but of smaller magnitude. Similar sociophysiological influences may be important in natural populations. Fighting mice, used under well-defined and closely controlled conditions, may be useful for studying normal mechanisms of neuroendocrine adaptation and control, and, possibly, for studying some forms of hypertension and cardiovascular-renal disease.

Adrenal Glands↗
PubMedDate not supplied

Laparoscopic adrenalectomy for adrenal tumours causing hypertension and for 'incidentalomas' of the adrenal on computerized tomography scanning.

1. In a 19 month period from June 1993 to December 1994, 60 patients (mean age 54.8 +/- 1.5 years s.e.m.; 32 males, 28 females) underwent unilateral laparoscopic adrenalectomy by one of us (JCR) for the treatment of hypertension due to primary aldosteronism (n = 48), phaeochromocytoma (n = 3) and cortisol-producing adenoma (n = 1) or to remove adrenal massess incidentally discovered on abdominal computerized tomography scanning ('incidentaloma') performed for other reasons (seven adenomas without biochemical evidence of excessive steroid hormone or catecholamine secretion and one carcinoma autonomously producing cortisol). 2. Compared with conventional open procedures, laparoscopic adrenalectomy was associated with reduced recovery time and a low complication rate (one pulmonary embolus and one port site incisional hernia). 3. Operation time with experience approximates that of open procedures (60 min), but is significantly longer in obese than in non-obese patients, and in males than in females. 4. Patients with adrenal causes of hypertension were cured or significantly improved by laparoscopic unilateral adrenalectomy. 5. Because of our concern regarding malignant potential of incidentalomas and high patient acceptance of laparoscopic techniques, we have reduced our size criteria for removal of incidentalomas.

Adenoma↗
PubMed1997

Adrenal and extra-adrenal retroperitoneal ganglioneuroma: imaging findings in 13 adults.

PURPOSE: To determine the radiologic appearance of ganglioneuromas of the adrenal gland and extra-adrenal retroperitoneum. MATERIALS AND METHODS: A retrospective review was performed of imaging findings in nine patients with ganglioneuroma, two with ganglioneuroma-pheochromocytoma, and two with malignant peripheral nerve sheath tumor (PNST) that arose in a ganglioneuroma. Patients underwent computed tomography (CT) (n = 13), magnetic resonance (MR) imaging (n = 5), and ultrasonography (US) (n = 5). RESULTS: In nine patients with ganglioneuroma, the mass ranged in size from 2 x 4 x 4 cm to 21 x 21 x 22 cm. Imaging findings included attenuation entirely or predominantly less than that of muscle on contrast material-enhanced CT images (n = 9); heterogeneous, predominantly high signal intensity on T2-weighted MR images (n = 5); partial or complete surrounding of one or more major blood vessels with little or no luminal narrowing (n = 4) and calcifications (n = 4) on CT scans; and homogeneous, moderate echogenicity (n = 3) on US scans. CT findings in two patients with ganglioneuroma-pheochromocytoma included central necrosis (n = 1) and calcifications (n = 1). In the two patients with malignant PNST that arose in a ganglioneuroma, contrast-enhanced CT showed a large, markedly heterogeneous mass. CONCLUSION: Diagnosis of ganglioneuroma should be considered when typical CT or MR imaging findings are present. Findings in cases of ganglioneuroma-pheochromocytoma and malignant PNST arising in a ganglioneuroma appear to be nonspecific.

Adrenal Gland Neoplasms↗
PubMed1980

Plasma progesterone, 17-hydroxyprogesterone, androstenedione and testosterone in prepubertal, pubertal and adult subjects with congenital virilizing adrenal hyperplasia as indicators of adrenal suppression.

To determine whether a single morning plasma level of 17-hydroxyprogesterone (17OH-P), androstenedione, testosterone and progesterone reflected the degree of control of 21-hydroxylase congenital virilizing adrenal hyperplasia (CVAH) as indicated by 24-hour urinary 17-ketosteroid and pregnanetriol excretion, 142 simultaneous 24-hour urine and morning blood collections were made from 65 patients with CVAH. Patients were grouped into five categories on the basis of age, skeletal age, and sex. Paired blood and urinary data were analyzed. The results suggest that androstenedione is the most reliable indicator for all patient categories. Testosterone is an excellent indicator for children of both sexes and for adolescent and adult females. Levels of 17OH-P are difficult to interpret, as they can be several fold higher than the normal values when adrenal suppression appears adequate on the basis of urinary data. In general, progesterone is a poor indicator.

17-Ketosteroids↗
PubMed1986

ACTH regulation of adrenal mRNA coding for total and specific adrenal proteins.

The effects in vivo ACTH administration on the synthesis of mRNA coding for total adrenal proteins and for protein E a specific marker of ACTH action, have been studied. After 4 h of in vivo ACTH treatment, protein E is one of the major translational products. Its electrophoretic characteristics in a 2D gel acrylamide system are defined (molecular mass = 36,000 daltons, pHi = 7). We have investigated the effects of ACTH on both poly(A)-RNA coding for total adrenal proteins, and non-poly(A)-RNA. The time course of these effects is different: the effect on mRNA is maximal at 48 h whereas the effect on non-poly(A)-RNA continues to increase until the end of the experiment (5 days). In vitro translocational assays of mRNA indicate that the highest efficiency (protein synthesis/microgram of mRNA) is observed after 4 h of ACTH treatment in vivo. After 5 days this efficiency is similar to that of mRNA extracted from non ACTH-treated rats.

Adrenal Glands↗
PubMed1987

Biphasic 19-nor-deoxycorticosterone excretion after adrenal enucleation is associated with altered adrenal mitochondrial cytochrome P450 11 beta-, 18-, and 19-hydroxylase activities.

After adrenal enucleation (AE) rats avidly retain sodium (early phase), but after 7-10 days they lose this sodium avidity (late phase). Although increased production of a mineralocorticoid, 19-nor-deoxycorticosterone (19-Nor-DOC), has been implicated, 19-Nor-DOC levels during the early and late phases of AE have not been systematically measured. Furthermore it is not known why 19-Nor-DOC production should increase during a time when production of 11 beta- and 18-hydroxylated corticosteroids are decreased in AE. The purpose of this study was to examine the 11 beta, 18-, and 19-hydroxylase pathways in the early and late phases of AE. The results demonstrate increased urinary 19-Nor-DOC and decreased 18-OH-DOC and corticosterone excretion in the early phase of AE at a time when adrenal mitochondrial 11 beta- and 18-hydroxylase activities were decreased but 19-hydroxylase activity was unchanged. During the late phase of AE, urinary 19-Nor-DOC had decreased and 18-OH-DOC and corticosterone had increased to levels indistinguishable from those in sham controls. This reduction in 19-Nor-DOC was associated with a decrease in 19-hydroxylase activity in AE. Since the 11 beta, 18-, and 19-hydroxylases have a common substrate (DOC), it is possible that differential flux of DOC through these pathways could account for the changes in steroid production in AE. These data suggest that the increased 19-Nor-DOC excretion in AE may be due to alterations in enzyme activity leading to a shunting of DOC into the 19-Nor-DOC pathway. In addition, the synchronicity of 19-Nor-DOC with sodium excretion suggests that it has an important role in the pathogenesis of the sodium retention in AE.

Adrenal Glands↗
PubMed1985

Opioid peptides in human adrenal: partial characterization and presence of adrenal peptide E.

Evaluation of hypotheses concerning the role of opioid peptides in the human adrenal is handicapped by the lack of information concerning the nature of these peptides. We studied the content of opioid peptides in whole adrenal tissue using several RIAs, including one which cross-reacts with all opioid peptides tested. Opioid peptides were localized to granules which behaved like chromaffin granules on crude sucrose density separation. beta-Endorphin immunoreactivity was a minor component, which was found principally in the form of beta-endorphin-(1-31). The majority of the remaining peptides probably were products of proenkephalin. The human postmortem tissue differed from bovine tissue in that the major accumulating products of this precursor were the size of the enkephalins and their small congeners, and not the intermediate-sized (mol wt, approximately 1500-4000) peptides that predominated in bovine tissue. We also found evidence of the presence of the 25-amino acid complex opioid, peptide E, in human tissue.

Adolescent↗
PubMed1997

Transcription of the human genes for cytochrome P450scc and P450c17 is regulated differently in human adrenal NCI-H295 cells than in mouse adrenal Y1 cells.

Human NCI-H295 cells, which express all of the genes for the steroidogenic enzymes in a hormonally regulated fashion, should be an ideal system in which to study the transcriptional regulation of these genes. Using deletional promoter/reporter constructions for the human P450scc and P450c17 genes, we identified the regions conferring basal and cAMP-induced transcription of these two genes in NCI-H295 human adrenal cells. In the P450scc gene, both basal and cAMP-induced transcriptional activation elements lie within the first 79 bp upstream (-79) from the transcriptional start site. In the P450c17 promoter, both basal and cAMP-responsive elements lie within the first upstream 63 bp, and a second basal element lies between -184 and -206 bp. The locations of these elements are substantially different from the locations of elements that appear to be functionally equivalent when these human gene promoters are transfected into mouse adrenal Y1, mouse testicular MA-10, or human choriocarcinoma JEG-3 cells. These data indicate that the transcriptional regulation of these genes in their native species and cell type differs substantially from their regulation in cells from other species and tissues, and suggests that the results from transfection experiments examining genes for steroidogenic enzymes in heterologous cells may not reflect events in vivo.

Adrenal Glands↗
PubMed2000

A survey on adrenal incidentaloma in Italy. Study Group on Adrenal Tumors of the Italian Society of Endocrinology.

The aim of this study was to perform a national survey on occasionally discovered adrenal masses [adrenal incidentalomas (AI)] under the auspices of the Italian Society of Endocrinology. This multicentric and retrospective evaluation of patients with AI includes 1096 cases collected in 26 centers between 1980 and 1995. Relevant information was obtained by means of a specifically tailored questionnaire. Of the 1096 forms received, 1004 were retained for final analysis. Patients were 420 males and 584 females, aged between 15-86 yr (median, 58 yr). Mass size (computed tomography measurement) ranged from 0.5-25 cm (median, 3.0 cm). Hormonal work-up demonstrated that 85% of the masses were nonhypersecretory, 9.2% were defined as subclinical Cushing's syndrome, 4.2% were pheochromocytomas, and 1.6% were aldosteronomas. Adrenalectomy was performed in 380 patients with removal of 198 cortical adenomas (52%), 47 cortical carcinomas (12%), 42 pheochromocytomas (11%), and other less frequent tumor types. Patients with carcinoma were significantly younger than patients with adenoma (median, 46; range, 17-84; vs. 57, 16-83 yr; P = 0.05). Adenomas were significantly smaller than carcinomas (3.5, 1-15 vs. 7.5, 2.6-25 cm; P < 0.001), and a cut-off at 4.0 cm had the highest sensitivity (93%) in differentiating between benign and malignant tumors. Hormonal work-up of patients with subclinical Cushing's syndrome showed low baseline ACTH in 79%, cortisol unsuppressibility after 1 mg dexamethasone in 73%, above normal urinary free cortisol in 75%, disturbed cortisol rhythm in 43%, and blunted ACTH response to CRH in 55%. Only 43% of patients with pheochromocytoma were hypertensive, and 86% showed elevated urinary catecholamines. All patients with aldosteronoma were hypertensive and had suppressed upright PRA. These results indicate that mass size is the most reliable variable in separating benign from malignant AI. Adrenalectomy should be recommended for AI greater than 4.0 cm because of the increased risk of malignancy, especially in young patients. Endocrine evaluation should be performed in all patients to identify silent states of hormone excess.

Adolescent↗
PubMed1987

[Neonatal screening for congenital adrenal hyperplasia due to 21-hydroxylase deficiency. 2. Analysis of steroids with high-performance liquid chromatography for diagnosis of congenital adrenal hyperplasia].

A quantitative analysis of steroids in serum, dried blood samples on filter paper and amniotic fluid with high-performance liquid chromatography has been developed and applied to the diagnosis of risk infants on neonatal screening for congenital adrenal hyperplasia. The present method is simple, rapid and accurate. The detection limits of cortisol and 17 alpha-hydroxyprogesterone are 0.2 ng and 0.3 ng, respectively. The twenty-two steroids can be analysed within 60 minutes using the isogradient mobile phase. The method is highly correlated with radioimmunoassay and enzymeimmunoassay. In 4 patients with the salt-losing form, 17 alpha-hydroxyprogesterone was higher and cortisol lower than in 4 patients with the simple-virilizing form. This method seems suitable for the routine clinical elucidation of congenital adrenal hyperplasia.

17-alpha-Hydroxyprogesterone↗
PubMed1998

Hypothalamo-pituitary-adrenal axis and adrenal function before and after ovariectomy in premenopausal women.

The hypothalamo-pituitary-adrenal (HPA) axis is modulated by sex hormones. Few data exist on the relation between acute estrogen deficit and HPA axis response to corticotropin-releasing hormone (CRH). The effects of a sudden drop in estradiol levels on basal and CRH-stimulated levels of ACTH, cortisol, testosterone, androstenedione and 17-hydroxyprogesterone (17-OHP) were assessed in nine premenopausal women (44-48 years of age), before and after ovariectomy. The CRH test was performed before and 8 days after ovariectomy. A significant reduction in ACTH and adrenal steroids but not in cortisol response to CRH was observed after ovariectomy. The ratio of deltamax androstenedione/17-OHP after CRH stimulation was substantially the same before and after ovariectomy, whereas deltamax 17-OHP/cortisol was significantly lower in ovariectomized women showing increased 21- and 11beta-hydroxylase activity. The results show that the acute estrogen deficit induces changes in the HPA axis characterized by reduced stimulated secretion of ACTH and steroids but normal stimulated cortisol production.

17-alpha-Hydroxyprogesterone↗
PubMed2003

Differential production of adrenal steroids by purified cells of the human adrenal cortex is relative rather than absolute.

OBJECTIVES: The adrenal cortex produces aldosterone, cortisol and androgens in response to ACTH and angiotensin II. To define the differential response of morphologically distinct cells of the adrenal cortex, we examined the phenotypical and functional characteristics of human adrenocortical cells. RESULTS: Tumour growth factor-beta receptor-1 (TGFbeta-R1) and CYP-11 were found to be expressed predominantly in the zona fasciculata, whereas human leukocyte antigen (HLA-DR) and CYP-17 were localised to the zona reticularis. The angiotensin II receptor, AT-1, was found to be predominantly expressed in the zona glomerulosa. Adrenocortical cells, separated by density, yielded two distinct fractions which displayed differential growth patterns. Lipid-rich cells of fraction I expressed TGFbeta-R1 and produced significantly more cortisol relative to androstenedione than unseparated or fraction II cells, whereas lipid-poor cells of fraction II expressed HLA-DR and produced more androstenedione relative to cortisol in the presence of ACTH. Aldosterone production by fraction II was significantly greater than fraction I or unseparated cells. TGFbeta-R1-positive fasciculata-type cells separated into fraction I and HLA-DR-positive cells consistent with reticularis cells separated into fraction II. Aldosterone-producing cells indicative of glomerulosa cells separated into fraction II. CONCLUSIONS: Our findings are consistent with the concept that all adrenocortical cells are capable of producing a range of steroids, but the relative production of cortisol, androgen and aldosterone differs.

Adrenal Cortex↗