[Medullo-adrenal hyperfunction in a case of Cushing syndrome with adenoma of the adrenal cortex].
Explore the source record for details and available documents.
SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
Adrenocorticotropic hormone (ACTH)-independent hypercortisolism accounts for 15%-20% of cases of Cushing syndrome and always arises from primary adrenal disease. Computed tomographic (CT) and magnetic resonance (MR) imaging findings in 37 patients with primary adrenal Cushing syndrome were analyzed and correlated with pathologic findings. Hyperfunctioning adenomas (n = 24), together with functioning carcinomas (n = 10), accounted for 92% of cases. Adenomas had a significantly smaller mean size (3.5 vs 14.5 cm) and lower mean unenhanced CT attenuation value (11 vs 28 HU) than did carcinomas. The presence of necrosis, hemorrhage, and calcification favored a diagnosis of carcinoma. Six of 10 carcinoma patients had metastases at presentation. Two adenomas were seen within a myelolipoma, which was recognized at both CT and MR imaging due to its fat content, and two adenomas were of uncertain malignant potential. Bilateral disease--primary pigmented nodular adrenal dysplasia (PPNAD) (n = 2) and ACTH-independent macronodular adrenal hyperplasia (AIMAH) (n = 1)--had characteristic imaging features. In PPNAD, multiple tiny (2-5-mm) nodules were visible bilaterally, with no overall glandular enlargement and normal intervening adrenal tissue. In AIMAH, both glands were grossly enlarged and contained nodules up to 3 cm in diameter. Familiarity with the range of imaging appearances of the adrenal glands in primary adrenal Cushing syndrome may help establish the underlying diagnosis.
Severe generalized alopecia and marked vulvar enlargement were observed in a 5-year-old spayed ferret with high serum estradiol concentrations. A neoplastic left adrenal gland was removed. Staining of the neoplastic cells for estradiol was demonstrated by use of immunohistochemistry. Clinical findings in this ferret were typical of adrenal-associated endocrinopathy, a syndrome characterized by increased secretion of adrenocortical hormones by hyperplastic or neoplastic adrenal glands.
Explore the source record for details and available documents.
The amplitude and frequency of growth hormone (GH) secretory pulses are influenced by a variety of hormonal signals, among which glucocorticoids play an important role. The aim of this study was to investigate the pulsatile secretion pattern of GH in dogs in which the endogenous secretion of glucocorticoids is persistently elevated, i.e. in dogs with pituitary-dependent hyperadrenocorticism (PDH). Blood samples for the determination of the pulsatile secretion pattern of GH were collected at 10-min interval between 08:00 and 14:00 h in 16 dogs with PDH and in 6 healthy control dogs of comparable age. The pulsatile secretion patterns of GH were analyzed using the Pulsar program. GH was secreted in a pulsatile fashion in both dogs with PDH and control dogs. There was no statistical difference between the mean (+/-S.E.M.) basal GH level in dogs with PDH (0.7+/-0.1 microg/l) and the control dogs (0.6+/-0.1 microg/l). The mean area under the curve (AUC) for GH above the zero-level in dogs with PDH (4.6+/-0.6 microg/l per 6 h) was significantly lower than that in the control dogs (7.3+/-1.0 microg/l per 6 h). Likewise, the mean AUC for GH above the base-level in dogs with PDH (0.6+/-0.1 microg/l per 6 h) was significantly lower than that in the control dogs (3.7+/-1.0 microg/l per 6 h). The median GH pulse frequency in the dogs with PDH (2 pulses/6 h, range 0-7 pulses/6 h) was significantly lower (P = 0.04) than that (5 pulses/6 h, range 3-9 pulses/6 h) in the control group. The results of this study demonstrate that PDH in dogs is associated with less GH secreted in pulses than in control dogs, whereas the basal plasma GH concentrations were similarly low in both groups. It is discussed that the impaired pulsatile GH secretion in dogs with PDH is the result of alterations in function of pituitary somatotrophs and changes in supra-pituitary regulation.
Explore the source record for details and available documents.
OBJECTIVE: To compare adrenal gland stimulation achieved following administration of cosyntropin (5 microg/kg [2.3 microg/lb]) IM versus IV in healthy dogs and dogs with hyperadrenocorticism. DESIGN: Clinical trial. Animals-9 healthy dogs and 9 dogs with hyperadrenocorticism. PROCEDURES: In both groups, ACTH stimulation was performed twice. Healthy dogs were randomly assigned to receive cosyntropin IM or IV first, but all dogs with hyperadrenocorticism received cosyntropin IV first. In healthy dogs, serum cortisol concentration was measured before (baseline) and 30, 60, 90, and 120 minutes after cosyntropin administration. In dogs with hyperadrenocorticism, serum cortisol concentration was measured before and 60 minutes after cosyntropin administration. RESULTS: In the healthy dogs, serum cortisol concentration increased significantly after administration of cosyntropin, regardless of route of administration, and serum cortisol concentrations after IM administration were not significantly different from concentrations after IV administration. For both routes of administration, serum cortisol concentration peaked 60 or 90 minutes after cosyntropin administration. In dogs with hyperadrenocorticism, serum cortisol concentration was significantly increased 60 minutes after cosyntropin administration, compared with baseline concentration, and concentrations after IM administration were not significantly different from concentrations after IV administration. CONCLUSIONS AND CLINICAL RELEVANCE: Results suggest that in healthy dogs and dogs with hyperadrenocorticism, administration of cosyntropin at a dose of 5 microg/kg, IV or IM, resulted in equivalent adrenal gland stimulation.
The effect of different dietary intake of pantothenic acid (150;100;25 and 0% of the requirement) on the metabolism of rats was studied during 15 months. The ability of the adrenals for synthesis of corticosterone and the rate of acetylation of a sulfonamid (Sulfisomidin) were taken as parameters. The above mentioned parameters were influenced significantly already after two weeks and a pantothenic acid free diet containing the antagonist omega-methyl-pantothenic acid. With a 25% supply of pantothenic acid and without omega-methyl-pantothenic acid it lasted half a year until significant alterations of acetylation could be demonstrated. The synthetic ability of the adrenals for corticosteroids was significantly increased after a year in the 25% group. After the end of the study, this hyperfunctional state was followed by hypofunction, resulting in a significantly reduced ability of synthesis of the glands. Studies of this type were conducted to obrain basic informations for later experiments in human beings.
UNLABELLED: The goal of this study was to evaluate the clinical reliability of the (131)I-6 beta-iodomethyl-norcholesterol ((131)I-NP-59) uptake semiquantitative evaluation method we propose for the characterization of adrenocortical masses in a selected population of patients with disease clinically classified as subclinical (SC) and preclinical (PC) Cushing's syndrome (CS) according to Reincke's definition. METHODS: Forty-seven consecutive patients with incidentally discovered unilateral adrenal masses were examined by a triple-head SPECT system after intravenous injection of (131)I-NP-59. Abdominal SPECT was performed at 24, 48, 72, and, in selected cases, 96 h after tracer injection. Connected with adrenals and liver, a standard elliptic region of interest (ROI) was manually drawn, taking care to avoid the gallbladder region. The adrenal ROI integral count, obtained by summing the 24-, 48-, and 72-h counting values, was normalized by the hepatic integral count. Subsequently, the adrenal percentage of relative uptake (UPT%) was computed. RESULTS: Discriminant analysis was performed on the variables UPT%, adrenocorticotropic hormone (ACTH) serum concentration, and CT mass dimension (CTMD) to determine the variable, or combination thereof, best discriminating between the SC-CS and PC-CS groups. Compared with both ACTH and CTMD variables, univariate analysis confirmed the UPT% variable as the most significant to discriminate between these 2 clinical groups. In fact, UPT% alone correctly classified 8 of 9 patients in the SC-CS group and 20 of 22 patients in the PC-CS group with 95% positive and 80% negative predictive values and with overall accuracy, sensitivity, and specificity equal to 90%, 91%, and 89%, respectively. When all 3 variables were submitted to stepwise discriminant analysis, the derived classification matrix, after cross-validation, correctly classified 9 of 9 patients in the SC-CS group and 18 of 22 patients in the PC-CS group with 100% positive and 69% negative predictive values and with overall accuracy, sensitivity, and specificity equal to 87%, 82%, and 100%, respectively. CONCLUSION: According to these initial results, use of the proposed semiquantitative approach associated with both laboratory screening for cortisol production and CTMD measure seems to be able to increase the clinical diagnostic accuracy of PC-CS. This approach could be used in the follow-up of adrenal mass function every time hormonal or clinical features are suggestive of adrenocortical hyperfunction.
Explore the source record for details and available documents.
Metastases to the thyroid gland from renal carcinoma are observed in 10% of the uncommon metastatic thyroid tumours. They are the first sign of renal carcinoma in 25% of cases or appear after primary treatment of renal neoplasia, up to 27 years later. Metastases are more frequent in pathological glands, perhaps because intraparenchymal blood flow is slowed and tissue oxygen partial pressure is reduced. Thyroid metastases are frequently associated with lung ones, whether those associated with metastases to other organs (duodenum, pancreas, skin) are very unusual. Clinical manifestations are thyroid node without hormonal modifications or acute swelling of the gland with hormonal hyperfunction; other signs are dysphonia and/or dysphagia. Thyroid nodes are "cold" at 131I scanning and with mixed structure at ultrasonography. Fine needle aspiration is often diagnostic. Problems in differential diagnosis are among clear-cell renal carcinoma and some types of thyroid adenoma, adrenal carcinoma, parathyroid carcinoma, testicular seminoma and hypernephroid thyroid carcinoma. Treatment is surgical: subtotal or total lobectomy, with or without isthmectomy, if metastasis is single, total thyroidectomy in diffuse disease. Some Authors always choice total thyroidectomy to erase microscopic foci of disease in contralateral lobe. Reported survival after treatment is from a few months to 27 years. Survival is longer with a metastasis appearing after nephrectomy and with unique versus multiple ones.
Adrenocortical carcinoma is a rare tumor in children. This tumor is more likely to be hormonally active in children than in adults and tends to cause a variety of symptoms, which may mimic other benign endocrinopathies. These tumors are usually diagnosed at advanced stages and portend a dismal prognosis. We describe two cases of adrenocortical carcinoma. One child presented with Cushingoid symptoms secondary to hypercortisolism, including amenorrhea, hirsutism and weight gain. The other child presented with precocious puberty. Both children underwent resection of the tumors. We describe their presenting symptoms, postoperative course, adjuvant therapy and clinical course. Pertinent literature regarding the anatomy of the adrenal gland, pathology of adrenocortical carcinoma, factors influencing outcome, diagnostic modalities and treatment, are discussed.
Offspring of mothers with adrenal hyperactivity during pregnancy have been reported to have changes in brain monoamines and altered emotional, reactive, sexual and maternal behavior. Since the hypothalamic-pituitary-adrenal (HPA) axis is known to be involved in the expression of such behaviors and is itself under monoaminergic control, we examined the development of the HPA axis and brain monoamines in pups whose mothers had adrenal hyperactivity, reflecting administration of ACTH during the last third of their pregnancy. The adrenals of the experimental animals weighed less and had aberrant morphology. The abnormal histology was more pronounced in the adrenals of the experimental females than of the males, suggesting that females were more vulnerable to the prenatal treatment. In both experimental males and females, basal plasma corticosterone levels were higher compared to the controls, while after exposure to stress, experimental animals attained lower plasma corticosterone levels than the controls. In the brain of the experimental animals, dopaminergic activity appeared to be decreased, while serotonergic activity increased. Our results indicate that the prenatal treatment affected brain development in the offspring and as a consequence programmed the developing HPA axis in such a way as to hyperfunction under basal conditions, leading to its exhaustion and its inability to react properly to stress.
Radiographic features of 23 dogs with functioning adrenocortical tumors are described. In 56% of the dogs, radiographic detection of adrenomegaly, with or without calcification, was an aid in the diagnosis of adrenocortical neoplasia. Adrenal gland calcification did not distinguish adrenocortical adenoma from carcinoma.
We report a case of Cushing's syndrome due to ACTH-independent macro-nodular adrenal hyperplasia (AIMAH). The patient, a 51-year-old man, had been suffering from poorly controlled arterial hypertension for the previous 6 years and he complained of progressive weight gain, gynecomastia and impotence. Physical examination revealed classic cushingoid features. Endocrine basal assessment showed increased urinary free cortisol (264-600 micrograms/24 hr). Urinary steroid profile showed an increase of total 17-hydroxycorticosteroids (17-OHCS: 23 mg/24 hr), with a threefold increase of tetrahydrocortisol (THF:9.66 mg/24 hr) and of tetrahydrocorticosterone (THB: 1.35 mg/24 hr). Tetrahydrodesossicortisol was only slightly elevated (THS:0.67 mg/24 hr) and tetrahydrodesossicorticosterone was at the inferior limit of the normal range (THDOC: 0.03 mg/24 hr). Total 17-ketosteroids were decreased (17-KS: 3 mg/24 hr). Plasma cortisol level was elevated and without circadian rhythm (26-29 micrograms/dl in the morning, 26-28 micrograms/dl at 24:00 h). DHEAs and free testosterone levels were significantly reduced (106 ng/dl and 3.9 pg/ml respectively). ACTH was undetectable and unresponsive to CRH. Both dexamethasone and octreotide failed to suppress plasma cortisol levels. Abdomen computed tomography scan demonstrated bilaterally enlarged multinodular adrenal glands. Cerebral magnetic resonance revealed no alteration of the pituitary gland. The patient underwent bilateral adrenalectomy. On macroscopic examination, adrenal glands were occupied by multiple yellow nodules and their compressive weight was 190 g, with left adrenal heavier than the right one (120 g and 70 g respectively). Histologically, nodular lesions were predominantly composed of large clear cells, with small foci of "hybrid" cells and adipose tissue metaplasia. Reticularis zone was atrophic. In the immediate post-operatory course pulmonary embolism occurred, despite prophylaxis with low molecular weight heparin. After having recovered from this complication, the patient showed progressive regression of cushingoid status. The findings of increased THF/THS and THB/THDOC ratios were in agreement with a relative hyperfunction of 11-beta-hydroxylase "in vivo", which might have contributed to the hypercortisolism, in addition to the marked increase of secernent adrenal mass.
INTRODUCTION: Adrenal incidentalomas are unsuspected, clinically silent adrenal lesions discovered incidentally by imaging tests performed a priori for problems unrelated to the adrenal glands. The aim of this study was to present a series of incidentalomas and review the diagnostic process and treatment techniques. PATIENTS AND METHOD: Of a series of 63 patients studied and/or treated for adrenal disease, there were 34 patients with adrenal incidentalomas. The mean age of the patients with incidentaloma was 50.6 years. There were 23 women (67.6%) and 11 men (32.3%). All patients underwent hormonal investigations to rule out hyperfunction. Imaging techniques consisted of ultrasonography, computed tomography (CT) and/or magnetic resonance imaging (MRI) to determine the size and characteristics of the lesion. In patients with an indication for surgery, the lateral transabdominal approach was used in laparoscopic surgery and the anterior approach was used in open surgery. RESULTS: Of the 34 patients, 23 patients (67.6%) (18 women and 5 men) with a mean age of 50.9 years underwent surgery. The mean tumoral diameter was 10.18 cm. Incidentalomas were located in the right adrenal gland in 16 patients and in the left adrenal gland in 7 patients. Laparoscopic surgery was performed in 9 patients (39.1%) and open surgery was performed in the remaining 14 (60.8%). The mean length of hospital stay was 8.6 days in open surgery and 4 days in laparoscopic surgery. Complications consisted of pneumonia in 2 patients (8.6%). There was no intraoperative mortality in the series. Surgery was not performed in 11 patients (32.3%) (5 women and 6 men) with a mean age of 56.3 years. The mean size of the tumor in these patients, identified by CT, was 2.5 cm. These patients underwent subsequent monitoring with a mean follow-up of 32 months. CONCLUSIONS: Due to the high resolution of ultrasonography, CT and MRI, as well as the greater number of radiological investigations performed, identification of adrenal incidentalomas has increased. In our series these tumors represented 53.9% of adrenal disease.
In this chapter we have discussed the pathogenesis of canine PDH focusing on its relationship to aging, dopamine deficiency, and neurodegenerative disease. We have outlined the successful management of canine PDH patients with l-deprenyl, a selective MAO-B inhibitor. Treatment with l-deprenyl results in clinical and endocrinologic improvement (partial to complete) in approximately 83% of dogs, with improvement noted within the first 1 to 2 months of therapy. The safety profile of l-deprenyl is excellent, especially in light of the fact that the majority of patients are elderly. l-Deprenyl is a safe and effective first-line therapy for the medical management of uncomplicated cases of canine PDH.
Explore the source record for details and available documents.