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Selective adrenal venography in a 10 month old infant with adrenal cortical carcinoma and virilizing symptoms demonstrated grossly dilated, irregular tumor veins. This was an unexpected finding as at aortography no adrenal arteries which would allow selective catheterization, and only very small neoplastic vessels were seen.
The adrenal cortex has a local stimulatory action on synthesis and secretion of adrenaline by the adrenal medulla. This interaction may be based on nervous transmission and/or on glucocorticoids secreted trom the cortex either exerting paracrine effects or reaching the medulla through the local vascular system. The existence of a dual capillary network, a portal system, now seems unlikely. However, a new concept is postulated: the high demand for glucocorticoids in the medulla may be met by local transfer to blood vessels within the adrenal gland, particularly the medullary arteries.
Neoplasms of all the adrenal parenchymatous elements [i.e., a compound adrenal medullary tumor (MT) consisting of pheochromocytoma (Pheo) and ganglioneuroma (GN) and a cortical adenoma] were found in the right adrenal gland of a 53-year-old man. A mature GN element was predominant in the MT, and nodules of small polygonal Pheo cells were scattered in GN. No neuroblastomatous element or malignant Pheo was found. The cortical adenoma consisted of compact cells and clear cells; it showed 3 beta hydroxysteroid dehydrogenase, glucose-6-phosphate dehydrogenase, and succinate dehydrogenase activity. The nonneoplastic cortex was slightly atrophic and showed weaker activity of the enzymes, suggesting that the adenoma was cortisol-producing. The cortex surrounding the MT was invaded and replaced by either GN or Pheo. In some places, however, hypertrophic compact cells constituted the cortex and were in contact with ACTH-immunoreactive chromaffin cells. A few of the latter were also positive for other proopiomelanocortin (POMC)-derived peptides. Pheo cells in the other parts were negative for POMC-derived peptides.
A patient who was thought to have testicular feminization syndrome and primary aldosteronism had an adrenal scan that suggested an adrenal adenoma. After later diagnosis of 17-alpha-hydroxylase deficiency, she was treated with glucocorticoids rather than surgery. Her clinical course and a repeat adrenal scan confirmed she did not have a tumor.
We experienced 41 cases of Cushing's syndrome (12 males and 29 females, 15 years old - 65 years old) during the last 20 years. These included 20 patients with unilateral adrenal adenoma (Cushing's syndrome), 19 patients with bilateral adrenal hyperplasia (Cushing's disease), one patient with adrenal carcinoma and one patient with primary adrenocortical nodular dysplasia (PAND). Moreover, these cases included some special ones, i.e. 5 cases with destructive thyroiditis after treatment, 2 cases with aggravation of arthritis after treatment, a case of Carney's complex with PAND, one case with paradoxical response to dexamethasone, and one case combined with empty sella syndrome. The most specific clinical signs were moon face (95% occurrence), hypertension (95%) and subcutaneous bruising (80%). Other significant signs were eye edema (66%), buffalo hump (68%), subcutaneous purpura (63%) and osteoporosis (49%). Skin striae was not a common sign in our cases (41%). Renal stone was observed in only 20% of our patients but was a significant sign in this syndrome. There was no difference in the occurrence of each clinical sign between Cushing's syndrome and Cushing's disease. The elevation of white blood cell count (WBC) and serum sodium, a decrease of serum potassium, and a decrease of reabsorption of phosphate (%TRP) were observed. Thyroid-stimulating hormone (TSH) and human growth hormone (HGH) were suppressed in patients with Cushing's syndrome and patients with Cushing's disease. These results were consistent with those of previous reports. However, luteinizing hormone (LH), follicle-stimulating hormone (FSH) and prolactin (PRL) were high in those patients with Cushing's syndrome and those with Cushing's disease. Oral glucose tolerance test was carried out in 34 patients before and after treatment. Thirty-one percent of those had diabetes mellitus and 26% had impaired glucose tolerance (IGT). The response of IRI in this test was high in patients with Cushing's syndrome and patients with Cushing's disease, and decreased 4 weeks after treatment in those with Cushing's syndrome but remained high in those with Cushing's disease. Plasma ACTH level and urinary 17-OHCS excretion were significantly higher in Cushing's disease than in Cushing's syndrome. During an 8mg-high-dose dexamethasone suppression test, urinary 17-OHCS excretion in 13 of 14 patients with Cushing's disease (93%) was suppressed by more than 50% of baseline on the second day of testing. However, all of 18 patients with Cushing's syndrome, who had an 8mg-dexamethasone suppression test, failed to suppress urinary 17-OHCS by 50% of baseline.(ABSTRACT TRUNCATED AT 400 WORDS)
Experiments were conducted on 84 male albino rats. A study was made of the state of the bile forming process 7, 14 and 30 days after bilateral adrenalectomy and adrenal gland autotransplantation. Investigations carried out showed inhibition of the bile formation with the corresponding changes in the chemical composition of bile to occur both in adrenalectomized animals and the rats with adrenal autotransplantation; in the second case, however, these changes were less pronounced. A gradual suppression of the intensity of bile production, cholate formation, bilirubin- and cholesterol secretion occurred in the adrenalectomized rats, whereas in the animals a significant shift in the direction of restoration took place as soon as in 7 days after autotransplantation. The first to become restored is the cholesterin secretory function in the liver, then the intensity of the cholate formation, particularly the glycocholic and desoxycholic acids, and bilirubin secretion. The intensity of bile secretion, as well as the sum total content of cholates in the bile, chiefly on account of taurocholic acid, failed to reach the initial level even by the 30th day after the adrenal gland autotransplantation.
We report a 10 years old boy, admitted with a history of asthenia, anorexia and weight loss of 4 kg. Initial laboratory work up showed metabolic acidosis and hyponatremia. The patient had no circadian rhythm of serum cortisol and an adrenal stimulation test confirmed the presence of adrenal insufficiency. Anti-adrenal antibodies were positive. Treatment with cortisol and fluorocortisone resulted in a complete remission of symptoms.
Two case reports are presented of adrenal insufficiency due to bilateral adrenal haemorrhage following surgery. This unusual complication with its non-specific manifestations may result in unexpected clinical deterioration of the postoperative patient. Corticosteroid replacement and repletion of sodium and water deficits should be given promptly when adrenal haemorrhage is suspected.
We report two cases of adrenal hemorrhage and review 17 others that were confirmed by hormone measurements and computed tomography. Precipitating factors included heparin therapy, surgery, hypotension, and sepsis. Heparin-associated thrombocytopenia may be an underappreciated cause of adrenal hemorrhage. Despite the availability of computed tomography, adrenal hemorrhage mimics many other diseases, and an early diagnosis remains difficult.
Eight patients with beta-thalassemia who were given long-term treatment with combined multiple transfusions and chelation therapy underwent adrenal testing. The six male and two female patients ranged in age from 7 to 19 years. Six of eight patients had delayed bone ages and height greater than 2.5 SDs below the mean. Of the six patients more than 13 years of age, two had clinical evidence of isolated adrenarche and only one had evidence of true puberty. Cortisol levels were similar in patients and controls at zero time (10.6 +/- 1.8 micrograms/dL [292 +/- 50 nmol/L] vs 10.8 +/- 1.4 micrograms/dL [298 +/- 39 nmol/L]) and at 60 minutes (26.6 +/- 2.5 micrograms/dL [734 +/- 69 nmol/L] vs 24.9 +/- 1.9 micrograms/dL [687 +/- 52 nmol/L]) after insulin hypoglycemia (all values are the mean +/- SE). During an eight-hour infusion of ACTH, cortisol responses in the patients with thalassemia were not significantly different from those of controls. Baseline levels of the adrenal androgens dehydroepiandrosterone (DHEA) and dehydroepiandrosterone sulfate (DHEA-S) were significantly lower in the subjects with thalassemia compared with controls of similar bone age and pubertal status. The prolonged ACTH infusion caused a significant increase in the DHEA level (79.2 +/- 14.7 ng/dL [2.74 +/- 0.51 nmol/L] vs 538.6 +/- 38.1 ng/dL [18.67 +/- 4.79 nmol/L]) and the DHEA-S level (37.5 +/- 10.8 micrograms/dL [1.02 +/- 0.29 mumol/L] vs 70.5 +/- 18.3 micrograms/dL [1.19 +/- 0.50 mumol/L]) in the patients. The patients' peak stimulated levels of DHEA-S were significantly lower than those of the controls, whereas peak levels of DHEA were similar in the patients and the controls. These results indicate that combined multiple transfusions and chelation therapy preserve the integrity of the ACTH-cortisol axis in patients with thalassemia. The reduced levels of adrenal androgens, short stature, and delayed puberty noted in our patients suggest, however, that alternative approaches to the therapy of thalassemia are needed.
Acute adrenal hemorrhage (AAH) is an extremely rare disorder, especially in adults, and may be difficult to diagnose because of its nonspecific presentation. We report a case of AAH following a sepsis, resulting in persistant adrenal insufficiency contrasting with the radiologic normalisation of the adrenals on computed tomography (CT) scan imaging.
The purpose of this study was to investigate the prevalence of testicular adrenal rest tumours in patients with congenital adrenal hyperplasia (CAH), and to describe sonographic and MR features of these lesions. Seventeen postpubertal male CAH patients underwent scrotal sonography, with colour Doppler, and in 16 of them pre- and postcontrast enhanced T1- and T2-weighted MR images of the testes were obtained. Ultrasound revealed lesions in 16 of 17 patients (94%), bilateral in 10 patients and unilateral in 6 patients. The lesions were typically located adjacent to the mediastinum testis. The maximal diameter of the lesions varied from 2 to 40 mm. Margins were blurred in 11 of 31 lesions. Seventeen of the 20 lesions smaller than 2 cm in diameter were hypoechoic, whereas all 11 lesions larger than 2 cm showed hyperechoic reflections. On MR all lesions were isointense on T1- and hypointense on T2-weighted images and lesion margins were clearly defined. Enhancement of the lesions after intravenous contrast was seen in 13 of 15 patients. In our series the prevalence of testicular adrenal rest tumours in postpubertal CAH patients is much higher than in other reported studies. The lesions may develop from some small, hypoechoic, and multifocal nodules and coalesce to large hypoechoic lesions with hyperechoic reflections on ultrasound. As our results suggest that ultrasonography and MR show the lesions equally well, ultrasonography should be the method of first choice for detection and follow-up of these lesions, because it is the cheapest and quickest imaging technique. In case of a partial orchiectomy, MR is recommended because it shows lesion margins optimally.
With the widespread use of computed tomography (CT), it is not unusual to find calcification within the adrenal glands. There are a variety of adrenal lesions that may calcify, but usually the appearance of the calcification is not specific. However, when the pattern and morphology of the adrenal calcification are combined with the other imaging features and the appropriate clinical history, the correct diagnosis may be suggested.
Plasma ACTH concentrations in 2, 3, 7, 8-tetrachlorodibenzo-p-dioxin (TCDD)-treated rats (50 micrograms/kg; single, oral dose) were 2.1-, 2.1-, 2.9-, 1.7-, 1.5-, 2.0- and 3.0-fold greater than control values, respectively, at days 1, 3, 5, 7, 10, and 14. At days 1 and 5 plasma corticosterone concentrations were increased 5.1- and 8.0-fold, respectively; whereas, at days 10 and 14 they were depressed to values of 50% and 39% of controls, respectively. Adrenal glands were excised from rats treated with TCDD and corticosterone production was assessed. Basal corticosterone concentrations produced by treated adrenals were depressed to 81%, 72%, and 71% of control values at days 5, 7, and 14, respectively. Corticosterone secretion by ACTH stimulated adrenals was equivalent to controls. These findings suggest that TCDD exposure decreases the bioactivity of the ACTH secreted by the anterior pituitary.
We present ultrasonographic and magnetic resonance imaging findings of intratesticular adrenal rests in a 16-year-old patient with congenital adrenal hyperplasia. Scrotal ultrasonography showed bilateral well-delineated homogenous hypoechoic lesions located around the mediastinum testis, which were highly vascularized on power Doppler ultrasonography. Relative to normal testicular parenchyma the lesions were iso- or hyperintense on T1-weighted and hypointense on T2-weighted images. T2-weighted images also showed a target-like appearance caused by a more hypointense peripheral halo around the lesions. The lesions enhanced remarkably on post-contrast images. This case suggests that radiological evaluation of testes, even in the presence of normal physical examination findings, should be included in periodical follow-up of patients with congenital adrenal hyperplasia. Magnetic resonance (MR) imaging is useful in demonstrating the lesions, because the contrast resolution better than with ultrasonography.
We describe the case of a 20-year-old patient with salt-wasting congenital adrenal hyperplasia (CAH) related to 21-hydroxylase deficiency. Bilateral craggy testicular tumours were found, requiring histological evaluation. Prior to the surgical procedure, the patient was treated with dexamethasone (he presented cortisol deficiency) and was stimulated with ACTH. High levels of 11beta-OH steroids measured in the gonadal vein, compared with peripheral blood samples suggested the presence of adrenal rests. Incubation of the tumours (which could not be differentiated histologically, from Leydig tissue), with radioactive steroid precursors was carried out. The results revealed the testicular tumours were of adrenal tissue origin, associated with 21-hydroxylase deficiency. The patient's non-compliance to glucocorticoid treatment was the main cause of his hypogonadotropic hypogonadism.
Opiates and opioids have complex effects on the hypothalamic-pituitary-adrenal axis, and they stimulate the sympathetic nervous system. This study was designed to clarify the role of brain beta-endorphin in the mechanism by which stress increases plasma concentrations of adrenocorticotropin (ACTH), epinephrine (E), and norepinephrine (NE). Intracerebroventricular (i.c.v.) administration of beta-endorphin to rats significantly increased plasma ACTH levels at doses of 0.09, 0.3, and 1.5 nmol, and plasma E and NE levels at doses of 0.3 and 1.5 nmol. The rise of plasma ACTH, E, and NE levels by 0.3 nmol beta-endorphin was inhibited by intravenous (i.v.) administration of 2 mg/kg b.wt. naloxone. I.v. administration of anti-rat corticotropin-releasing hormone (CRH) rabbit serum completely blocked the beta-endorphin-induced ACTH secretion without affecting the secretion of E and NE. I.c.v. administration of anti-beta-endorphin rabbit gamma-globulin attenuated a 30-min restraint stress-induced rise of plasma ACTH levels without significant influence on the rise of E and NE levels, whereas i.v. administration of naloxone attenuated the restraint stress-induced rise of plasma ACTH, E and NE levels. These results suggest that i.c.v. administration of beta-endorphin stimulates the secretion of ACTH, E, and NE through opiate receptor, and that brain CRH mediates the beta-endorphin-induced secretion of ACTH. The results also suggest that brain beta-endorphin is, at least in part, involved in the restraint stress-induced stimulation of the hypothalamic-pituitary-adrenal axis, and that some opioids other than beta-endorphin are involved in the stimulatory mechanism of the autonomic nervous system and the adrenal medulla in the rat.
OBJECTIVE: To test the hypothesis that adrenal steroidogenesis in nonclassic adrenal hyperplasia (NCAH) patients is, at least in part, independent of adrenocorticotropic hormone (ACTH) control. DESIGN: Prospective controlled clinical study. SETTING: Patients and healthy volunteers in an academic research environment. PATIENT(S): Four patients with 21-hydroxylase (21-OH) deficient NCAH and four healthy control women. INTERVENTION(S): Patients received the long-acting gonadotropin-releasing hormone analog (GnRH-a) leuprolide acetate (3.75 mg/month IM) on weeks 0 and 4; and dexamethasone (DEX) in weekly incremented doses (0.25 mg/day, 0.50 mg/day, 1.0 mg/day, and 1.5 mg/day), beginning on weeks 4, 5, 6, and 7, respectively. MAIN OUTCOME MEASURE(S): The levels of 17-hydroxyprogesterone (17-HP), progesterone (P4), androstenedione (A4), dehydroepiandrosterone sulfate (DHS), and cortisol (F) were measured at the beginning of weeks 0, 4, 5, 6, 7, and at the end of the study (beginning of week 8). RESULT(S): Patients and controls had a similar median age and body mass index (BMI). There were no significant decreases in the median levels of the studied hormones following 4 weeks of treatment with GnRH-a only, in either NCAH patients or controls. Analysis of individual hormonal values demonstrated that by the end of the study (after DEX of 1.5 mg/day during a week) only 2 of 4, 0 of 4, 3 of 4 and 3 of 4 NCAH patients had 17-HP, P4, A4, and DHS levels within the range of control values, respectively. CONCLUSION(S): Ovarian and incremental adrenal suppression did not fully suppress progestogen and androgen production in all of the study patients with 21-OH-deficient NCAH, suggesting that their production was partially independent of ACTH stimulation. Potentially in these patients subtle degrees of adrenocortical hyperplasia and/or abnormal enzymatic kinetics are responsible for the nonsupressibility.