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Specific GABA binding in the adrenals and blood corticosteroid levels in stress in intact rats and rats with changes in the functional activity of the hypothalamo-pituitary-adrenal system.

Thirty minutes of swimming led to decreases in the specific binding of GABA to adrenal plasma membranes in intact rats, but not in rats repeatedly given hydrocortisone to inhibit the baseline activity of the hypothalamic-pituitary-adrenal cortex system, where the level of reception of this amino acid was significantly greater than in controls. Pre-stress administration of baclofen and gamma-L-aminobutyryltaurine had no effect on specific GABA binding, even though these agents affected blood corticosterone concentrations.

Adrenal Cortex Hormones↗

Adrenal insufficiency possibly due to lipoid adrenal hyperplasia: a case report.

An infant who presented with adrenal insufficiency possibly due to lipoid adrenal hyperplasia at the age of 66 days is described. This is the first time that this very rare disorder is reported from Saudi Arabia. Adrenocorticotrophic hormone (ACTH) stimulation test is useful in the ante-mortem diagnosis of this condition.

Adrenal Hyperplasia, Congenital↗

Effects of angiotensin-II on inositol phosphate accumulation and calcium influx in bovine adrenal and Y-1 tumor adrenal cells.

The present study compared the action of angiotensin II (A-II) in bovine adrenal fasciculata cells (BA) and Y-1 adrenal tumor cells which are sensitive and resistant respectively to its steroidogenic effect. In both models, A-II induced a time and dose-dependent inositol phosphate (Ins-Ps) accumulation and calcium influx. However, in Y-1 cells the Ins-Ps production was low and only Ins-P1 and Ins-P2 were accumulated. The calcium influx in BA cells was observed after 15 seconds and remained linear as long as the hormone was present, whereas in Y-1 cells calcium influx started prior to 15 seconds and reverted to basal values after 45 seconds. The effects of A-II on both cell types were specific since they were blocked by A-II antagonists. Taken together these results demonstrate the presence of functional A-II receptors in both cell types which are coupled to the two main intracellular messenger systems. Thus, the A-II steroidogenic refractoriness of Y-1 cells is probably related to some alteration(s) located beyond the calcium and/or protein kinase C A-II-messenger system.

Adrenal Gland Neoplasms↗

[Relationship between food protein quality and adrenal cortex function. 7. Relationship between activity of selected liver and small intestine hydrolases and adrenal cortex function].

Male Wistar rats received 10% true digestible crude protein of varying quality (maize gluten (MK) = low protein quality; maize gluten + lysine, tryptophan, threonine, isoleucine (MK + AS) = high protein quality) for a period of three weeks. The trial was carried out to investigate the activity of the two enzymes leucine-arylamidase (LNA) and aminotripeptidase (TP) in the liver and the mucosa of the small intestine and that of alpha and gamma amylases and of total amylases in the mucosa of the small intestine in animals exhibiting differences in the functional state of their adrenal cortices (adrenalectomized animals (AE), normal rats (N) and animals receiving additional treatment with cortisol (KS). It was found that mucosal LNA and gamma amylase did not respond to functional changes in the adrenal cortex. Mucosal LNA had a higher activity if the dietary protein was of low quality and a lower activity if the protein quality was high. The level of liver LNA activity was significantly lower in N and KS animals than in AE animals. The highest activity of mucosal and liver TP was found in KS animals; it was lowest in N animals. Statistically significant differences brought about by changes in the quality of dietary proteins were only found to occur in the KS group. The activity of alpha amylase and total amylases was found to be lowest in N animals; it was significantly higher in animals of the AE and KS groups. Animals of the N and KS groups receiving MK + AS exhibited a significantly higher level in their alpha and total amylase activities than animals in the same group receiving MK. Here, the "permissive action" of glucocorticosteroids on metabolically active hydrolases is clearly evident. The pattern of mucosal LNA activity in the small intestine and that of gamma amylase may be regarded as further characteristic evidence for the digestive action of these enzymes. Liver LNA and liver TP as well as mucosal TP and alpha amylase acted like metabolic enzymes.

Adrenal Cortex↗

Circadian hydrocortisone infusions in patients with adrenal insufficiency and congenital adrenal hyperplasia.

OBJECTIVE: Conventional hydrocortisone therapy in adrenal insufficiency cannot provide physiological replacement. We have explored the potential of circadian delivery of hydrocortisone as proof of concept for such therapy delivered in modified-release tablet formulation. METHODS: We investigated whether the circadian intravenous infusion of hydrocortisone could improve control of ACTH and androgen levels. Two healthy subjects, two patients with Addison's disease and two patients with congenital adrenal hyperplasia (CAH) were studied. RESULTS: In patients on thrice daily oral hydrocortisone, peak serum cortisol levels were higher than in normal subjects and overnight levels were very low. Patients had very high plasma ACTH levels before their morning dose of hydrocortisone, both at the beginning and at the end of their conventional oral therapy: mean +/- SEM 311.8 +/- 123.2 and 311.2 +/- 85.4 ng/l, respectively. In the patients with CAH, serum 17-hydroxyprogesterone levels were also elevated: 550 and 642 nmol/l at the beginning and 550 and 777 nmol/l at the end of conventional treatment, respectively. The overall 24-h mean cortisol levels were similar for conventional oral hydrocortisone and the circadian infusion. At 0700 h, ACTH levels were much higher on conventional treatment than after circadian infusion: mean +/- SEM 311.2 +/- 85.4 vs. 70.5 +/- 45.0 ng/l, respectively (P < 0.05). The same pattern was observed in 17-hydroxyprogesterone levels, which were 550 and 777 nmol/l after conventional treatment and 3 and 64 nmol/l after circadian infusion. CONCLUSIONS: In patients with poor biochemical control of Addison's disease and CAH, a 24-h circadian infusion of hydrocortisone can decrease morning ACTH and 17-hydroxyprogesterone levels to near normal.

17-alpha-Hydroxyprogesterone↗

Failure to suppress adrenal function in congenital adrenal hyperplasia (21-hydroxylase deficiency). Three case reports.

Three patients, aged 14, 16, and 32 years respectively, with congenital adrenal hyperplasia (21-hydroxylase deficiency) are described. Excessive adrenal activity and ACTH secretion could not be suppressed with doses of corticosteroids sufficient to cause iatrogenic Cushing's syndrome, even though part of the steroid dosage was administered in the late evening. The resistance to feed-back suppression was of the same order as that seen in Cushing's syndrome. Adrenalectomy was performed in the 16-year-old girl, and was followed by a menarche. Adrenalectomy was considered inadvisable in the other two patients.

Adolescent↗

Autologous adrenal medullary, fetal mesencephalic, and fetal adrenal brain transplantation in Parkinson's disease: a long-term postoperative follow-up.

We report on the clinical status of 5 patients with Parkinson's disease (PD) 3 years after autologous adrenal medullary (AM)-to-caudate nucleus (CN) implantation, and of 2 PD patients, 2 years after fetal ventral mesencephalon (VM)- and fetal adrenal (A)-to-CN homotransplantation. Current clinical evaluation of 4 of the AM grafted patients revealed sustained bilateral amelioration of their PD signs, most notably of rigidity, postural imbalance and gait disturbances, resulting in a substantial improvement in their quality of life. The disease-related dystonia of one of them disappeared only 2 years after surgery. The levodopa requirements of 2 of these patients and the anticholinergic therapy of another have been reduced. In agreement with the satisfactory clinical evaluation of these 4 patients, their neuropsychological and electrophysiological improvements, initially registered 3 months after surgery, have been maintained for 3 years. After 1 year of significant recovery, the 5th patient of this group has almost returned to her preoperative state. The 2 homotransplanted patients also showed sustained bilateral improvement of their PD signs. Two years after surgery, the most improved signs of the fetal VM case were rigidity, bradykinesia, postural imbalance, gait disturbances and facial expression. The fetal A case has only shown amelioration of rigidity and bradykinesia. Neither of them has shown significant neuropsychological changes. Their current levodopa requirements are less than before surgery. The improvements shown here by PD patients after brain tissue grafts go beyond those obtained using any other therapeutic approach, when levodopa fails. Although more studies and the development of these procedures are obviously required, these initial human trials appear to be resisting the test of time.

Adrenal Glands↗

Auditory detection and perception in normal man and in patients with adrenal cortical insufficiency: effect of adrenal cortical steroids.

Auditory detection thresholds for sinusoidal tones and various tests of auditory perception were determined in 12 patients with adrenal cortical insufficiency (seven with Addison's disease and five with panhypopituitarism) and compared to those in normal volunteers. In adrenal cortical insufficiency auditory detection sensitivity was significantly more acute than normal, and judgments of loudness and of the contralateral threshold shift were made at levels more than 20 db below those of normal subjects. Thus both the lower and the upper limits of the dynamic auditory range are significantly decreased in these patients. Speech discrimination ability of the patients was significantly impaired as was their difference limens, their alternate binaural loudness balances, and their ability to localize tones in space. Treatment of the patients with deoxycorticosterone acetate decreased serum potassium concentration, raised serum sodium concentration, and produced gains in body weight but did not alter auditory detection or perception. Treatment with prednisolone or with maintenance doses of carbohydrate-active steroids returned auditory detection and perception to normal in every patient tested. The inability of the untreated patients to perform the various auditory perception tasks indicates that they have a defect in their ability to integrate incoming sensory stimuli. This defect may be related to the alteration in the timing of transmission of neural impulses along axons and across synapses which occurs in these patients when their carbohydrate-active steroid is removed. This decreased integrative capacity occurs at the same time that they are able to detect many types of sensory stimuli significantly better than normal subjects. This interrelationship between increased detection sensitivity and decreased perceptual ability is dependent upon the absence of carbohydrate-active steroids, for when these steroids are replaced both detection sensitivity and perceptual ability revert to normal.

Addison Disease↗

Testicular adrenal rest tumors in adult males with congenital adrenal hyperplasia: evaluation of pituitary-gonadal function before and after successful testis-sparing surgery in eight patients.

CONTEXT: In male patients with congenital adrenal hyperplasia (CAH), testicular adrenal rest tumors (TART) are frequently present. These tumors can interfere with testicular function. Intensifying glucocorticoid therapy does not always lead to tumor regression and improvement of testicular function. Recently, testis-sparing surgery was introduced for treatment of TART. OBJECTIVE: The aim of this study was to evaluate tumor volume, symptoms, and pituitary-gonadal function in male patients with CAH caused by 21-hydroxylase deficiency and bilateral TART before and after testis-sparing surgery. SETTING: This study was conducted at Radboud University Nijmegen Medical Centre in The Netherlands. PATIENTS: Eight adult male CAH patients with bilateral TART and infertility were included. INTERVENTIONS: Evaluation of testicular magnetic resonance imaging, symptoms, fasting serum concentrations of ACTH, LH, FSH, inhibin B, 17-OH progesterone, androstenedione, testosterone, and estrone, and semen analysis (six of eight patients) was performed before and 6 and 22 months after testis-sparing surgery. MAIN OUTCOME MEASURES: The main outcome measures were absence of residual tumor and improvement of symptoms and pituitary-gonadal function. RESULTS: Residual tumors were not found on any of the patients' magnetic resonance imaging after surgery. Two patients reported testicular pain and discomfort that disappeared after surgery. Parameters of pituitary-gonadal function did not improve after surgery: semen analysis showed azoospermia (five patients) or oligospermia (one patient) without improvement, and all patients had persistently low inhibin B concentrations. CONCLUSION: Testis-sparing surgery did not improve pituitary-gonadal function despite successful removal of the tumors. Further studies are needed to investigate whether surgery at an earlier stage in the natural history of TART can prevent permanent testicular damage.

17-alpha-Hydroxyprogesterone↗

Angiotensin and adrenal steroidogenesis: study of 21-hydroxylase-deficient congenital adrenal hyperplasia.

The effects of angiotensin have been studied in four adult patients with the simple virilizing form of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. They were treated with hydrocortisone (25 mg/day) throughout this investigation. Plasma ACTH was normal in three cases, and androstenedione was normal in all cases. However, urinary pregnanetriol, plasma 17-hydroxyprogesterone (17 OHP), aldosterone, and renin activity was increased. The patients were then submitted to three protocols: sodium depletion (10 meq Na/day) for 5 days, sodium repletion (200 meq Na/day) for 5 days, and angiotensin infusion (sufficient to maintain a pressor response) for 60 min. Urinary pregnanetriol, plasma 17 OHP, and androstenedione levels increased in all patients after sodium depletion and decreased after sodium repletion. Plasma ACTH levels were not modified by changes in the sodium balance. Furthermore, angiotensin infusion increased aldosterone and 17 OHP plasma concentrations without any change in the plasma ACTH level. This study shows the direct action of angiotensin on adrenal steroidogenesis, at least in 21-hydroxylase deficiency. It confirms that even in the simple virilizing form, combined treatment with glucocorticoids and mineralocorticoids helps to normalize plasma 17 OHP levels.

Adrenal Glands↗

Ovarian adrenal rest tissue in congenital adrenal hyperplasia--a patient report.

We report a young girl who died in an Addisonian crisis due to previously undiagnosed congenital adrenal hyperplasia (CAH), in whom ovarian adrenal rest tissue was detected at postmortem histopathological examination. This is a very rare complication in female patients with CAH with only two previously reported cases.

Addison Disease↗

Adrenal paragonimiasis simulating adrenal tumor--a case report.

We describe a case of adrenal paragonimiasis with its computed tomographic and ultrasonographic findings. Computed tomogram showed a well enhancing oval mass at right adrenal gland and ultrasonogram showed a dumbbell-shaped hyperechoic mass saddling on the top of the right kidney. Surgical specimen was multicystic mass filled with creamy material.

Adrenal Gland Diseases↗

Primary adrenal lymphoma presenting as adrenal insufficiency. A case report and review of literature.

Primary adrenal lymphoma (PAL) with adrenal insufficiency is a rare entity that has a unique presentation and prognosis when compared to other high-grade B-cell Non-Hodgkin's Lymphomas (NHL). Radiologic characteristics and image-guided biopsy are helpful in diagnosis. Current chemotherapy of choice is CHOP [Cyclophosphamide, Hydroxydaunomycin (doxorubicin), Oncovin (vincristine sulfate) and Prednisone]. More cases need to be documented to formulate an effective approach to PAL.

Adrenal Gland Neoplasms↗

Bilateral primary non-Hodgkin's lymphoma of the adrenal glands with adrenal insufficiency: a case report.

A 72-year-old woman with bilateral non-Hodgkin's lymphoma of the adrenal glands causing adrenal insufficiency is reported. The left-side lymphoma (diffuse large B-cell lymphoma) was removed surgically but the right-side lymphoma could not be removed. Complete response was obtained with subsequent combined chemotherapy and was maintained for 6 months with repeated chemotherapies. However, the patient died of liver recurrence 1 year after admission. In cases of malignant lymphoma forming a bulky mass, surgery with subsequent chemotherapy appears to reduce the cancer volume and improve the therapeutic outcome.

Adrenal Gland Neoplasms↗

[Adrenal tumors and surgical approaches to the adrenal glands].

In 1987 at the First Surgical Clinic in Bratislava four patients with adrenal tumours were operated. Hormonally inactive tumours are, however, diagnosed late on account of their latent growth. To the diagnosis of tumours sonography and computed tomography contributed in a major way. Adrenal tumours must be radically removed, sometimes even with surrounding organs (kidney, spleen, pancreas). Malignant tumours have a very adverse prognosis.

Adrenal Gland Neoplasms↗

[Status of the hypophyseo-adrenal system and functional morphology of the adrenal glands in residents of Northern European areas of the USSR].

Serum corticotropin and cortisol levels were measured in 20-40-year old healthy residents (144 males and 361 females) in the North. A functional Synacthen Depot test was performed in 5 volunteers. The adrenal glands from 56 males and 8 females of the same age who had died from accidents were pathohistologically and stereometrically examined. The signs of functional tension were established in the pituitary-adrenal cortex system. The Synacthen Depot test determined sufficient potentials of the organ to show responses to specific stimulation. Morphologically, a number of changes were found, which might be regarded as a prerequisite to the development of functional and pathological disturbances in the above-mentioned link of the endocrine system.

Adaptation, Physiological↗

[Glucocorticoid function of the adrenals and the state of the sympathetic-adrenal system in newborn infants with an hypoxic lesion of the central nervous system].

The convened studies demonstrated an activation of the hypothalamo-hypophysis-adrenal and sympathico-adrenal system in the newborn with slight and moderately expressed forms of CNS lesions. At the same time there was a relative insufficiency of such functions in children with severe forms of CNS lesions. The achieved data permit to substantiate the pathogenetical therapy.

11-Hydroxycorticosteroids↗

Neurofilaments in adrenal and extra-adrenal pheochromocytoma. Demonstration using immunofluorescence microscopy.

Three cases of pheochromocytomas (two adrenal and one retroperitoneal) were studied immunohistologically, using specific antibodies against different types of intermediate filaments. A strong positive reaction was seen in immunofluorescence microscopy with antineurofilament antibodies, while no staining of tumor cells was observed with antikeratin, antivimentin, or antidesmin antibodies. The results are in accordance with the neuroepithelial derivation of adrenal medulla and paraganglia and suggest that antineurofilament antibodies can be used as an adjunct aid in identifying pheochromocytomas.

Adolescent↗