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Adrenal Mass

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3641. Irregular and frequent cortisol secretory episodes with preserved diurnal rhythmicity in primary adrenal Cushing's syndrome. (PubMed)

Irregular and frequent cortisol secretory episodes with preserved diurnal rhythmicity in primary adrenal Cushing's syndrome. To evaluate the pathophysiology of altered cortisol secretion in patients with primary adrenal hypercortisolism, cortisol secretion was investigated in 12 patients, seven with a unilateral adenoma and five with ACTH-independent macronodular adrenal hyperplasia compared with age- and gender-matched controls and with patients with pituitary-dependent hypercortisolism (...) . Pulsatile secretion was increased 2-fold (P = 0.04), attributable to increased event frequency (P = 0.002). All patients showed a significant diurnal rhythm with a delay in phase shift of 3 h (P = 0.01). Approximate entropy ratio, a feedback-sensitive measure, was increased compared with controls (P = 0.00003) but similar to that of pituitary-dependent hypercortisolism (P = 0.77), denoting loss of autoregulation. Cortisol burst-mass tended to be smaller in patients with ACTH-independent macronodular

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2005 Journal of Clinical Endocrinology and Metabolism

3642. Clinical utility of noncontrast computed tomography attenuation value (hounsfield units) to differentiate adrenal adenomas/hyperplasias from nonadenomas: Cleveland Clinic experience. (PubMed)

Hounsfield units (HU) in characterizing whether an adrenal mass is adenoma or nonadenoma. The mean (+/- SD) HU value for the adrenocortical adenoma/hyperplasia group was 16.2 +/- 13.6 and significantly lower (P < 0.0001) than primary adrenocortical cancers (36.9 +/- 4.1), metastases (39.2 +/- 15.2), and pheochromocytomas (38.6 +/- 8.2). The sensitivity and specificity for 10- and 20-HU cutoff values to differentiate adenomas/hyperplasias from nonadenomas were 40.5 and 100% and 58.2 and 96.9 (...) %, respectively. The size of the adrenal tumor had less value with only 40.7 and 81.3% sensitivity and 94.7 and 61.4% specificity for 2- and 4-cm cutoff values. A combination of less than or equal to 4-cm adrenal mass size and noncontrast computed tomography HU less than or equal to 20 had 42.1% sensitivity and 100% specificity. Our study, the largest with surgical histopathology as the gold standard for diagnosis, supports a noncontrast computed tomography attenuation value of 10 HU as a safe cutoff value

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2005 Journal of Clinical Endocrinology and Metabolism

3643. Relationship between the growth hormone/insulin-like growth factor-I axis, insulin sensitivity, and adrenal androgens in normal prepubertal and pubertal girls. (PubMed)

Relationship between the growth hormone/insulin-like growth factor-I axis, insulin sensitivity, and adrenal androgens in normal prepubertal and pubertal girls. The aim of this study was to analyze the possible implication of changes in the GH/IGF-I axis and in insulin sensitivity for the regulation of adrenal androgen secretion of normal prepubertal and adolescent girls. A total of 61 normal girls were evaluated in prepuberty [Group (Gr)1, n = 33; early (Gr1A, n = 16) and late (Gr1B, n = 17 (...) in Gr1B were also significantly higher than in Gr3A (P < 0.02). However, body mass index (BMI) in Gr1A, Gr1B, and Gr3A was not significantly different, although a significant increment was observed between late prepuberty (Gr1B) and late puberty (Gr3B; P < 0.0001). On the other hand, serum IGF-I levels in Gr1A and Gr3A were significantly lower than those in Gr1B (P < 0.01) and Gr3B (P < 0.02), respectively. The mean serum DHEAS level in Gr1A and Gr3A was significantly lower than in Gr1B (P < 0.01

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2003 Journal of Clinical Endocrinology and Metabolism

3644. Altered 24-hour blood pressure profiles in children and adolescents with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. (PubMed)

Altered 24-hour blood pressure profiles in children and adolescents with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Children and adolescents with classical congenital adrenal hyperplasia have been shown to be at risk for obesity associated with higher insulin and leptin levels. Because these factors are also known to cause hypertension, the aim of this study was to analyze 24-h blood pressure profiles and their relation to different clinical and laboratory (...) parameters.Fifty-five subjects, aged between 5.3 and 19.0 yr, were enrolled in a prospective, cross-sectional study. All patients had genetically proven 21-hydroxylase deficiency and underwent ambulatory 24-h blood pressure monitoring during a period off school/work. RESULTS (MEDIAN, RANGE): The median body mass index of the cohort was significantly elevated [1.09 sd score (SDS), -2.45 to 3.77]. Daytime and nighttime systolic blood pressures were also significantly elevated (0.67 SDS, -1.5-4.1; 0.63 SDS, -0.91

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2006 Journal of Clinical Endocrinology and Metabolism

3645. Metabolic profile and body composition in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. (PubMed)

Metabolic profile and body composition in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. The chronic, often supraphysiological glucocorticoid doses used in congenital adrenal hyperplasia (CAH) might increase morbidity in cardiovascular disease and diabetes.Our aim was to assess risk factors for cardiovascular disease and diabetes in CAH women. SUBJECTS, METHODS, AND DESIGN: We compared 61 women, 18-63 yr, with CAH due to 21-hydroxylase deficiency with 61 age (...) - and sex- matched controls. Twenty-seven were younger than 30 yr, and 34 were 30 yr or older. Anthropometry, fat and lean mass measured by dual-energy x-ray absorptiometry, serum lipids, insulin, and adrenocortical steroids were studied.Body composition and cardiovascular risk factors were the main outcome measures.Younger patients and controls had similar waist to hip ratio, lean and fat mass, and insulin. Older patients had higher waist to hip ratio, lean mass, and insulin than controls. Fat mass

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2007 Journal of Clinical Endocrinology and Metabolism

3646. Lack of Association of the 11beta-hydroxysteroid dehydrogenase type 1 gene 83,557insA and hexose-6-phosphate dehydrogenase gene R453Q polymorphisms with body composition, adrenal androgen production, blood pressure, glucose metabolism, and dementia. (PubMed)

reductase deficiency (CRD) when at least three alleles are affected.The aim was to study the separate and combined effects of these polymorphisms on body composition, adrenal androgen production, blood pressure, glucose metabolism, and the incidence of dementia in the healthy elderly population.The Rotterdam study (n = 6105) and the Frail Old Men study (n = 347) are population-based cohort studies in the elderly.Genotype distributions and influences of (combined) genotypes on body mass index, adrenal (...) Lack of Association of the 11beta-hydroxysteroid dehydrogenase type 1 gene 83,557insA and hexose-6-phosphate dehydrogenase gene R453Q polymorphisms with body composition, adrenal androgen production, blood pressure, glucose metabolism, and dementia. Recently, it was proposed that a combination of the 83,557insA polymorphism in the 11beta-hydroxysteroid dehydrogenase type 1 (HSD11B1) gene and the R453Q polymorphism in the hexose-6-phosphate dehydrogenase (H6PD) gene interacts to cause cortisone

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2007 Journal of Clinical Endocrinology and Metabolism

3647. Hypothalamic-pituitary-adrenal axis dysregulation and memory impairments in type 2 diabetes. (PubMed)

Hypothalamic-pituitary-adrenal axis dysregulation and memory impairments in type 2 diabetes. There is evidence of both hypothalamic-pituitary-adrenocortical (HPA) axis and cognitive dysfunction in type 2 diabetes mellitus (T2DM). However, the exact nature and the associations between these abnormalities remain unclear.The aim of the study was to characterize the nature of the HPA dysregulation in T2DM and ascertain whether impaired cognition in T2DM could be attributed to these abnormalities.A (...) , and 30 age-, gender-, and education-matched controls.Basal cortisol levels, cortisol levels during the DEX/CRH test, and performance on neuropsychological tests were measured.Individuals with T2DM had elevated basal plasma cortisol levels, higher levels after DEX suppression, and a larger response to CRH (all P mass index

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2007 Journal of Clinical Endocrinology and Metabolism

3648. Cardiovascular risk factors and ultrasound evaluation of intima-media thickness at common carotids, carotid bulbs, and femoral and abdominal aorta arteries in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. (PubMed)

Cardiovascular risk factors and ultrasound evaluation of intima-media thickness at common carotids, carotid bulbs, and femoral and abdominal aorta arteries in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. In congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, a tendency for obesity, high insulin, and high 24-h blood pressure levels has been reported in children and adolescents. Increased intima-media thickness (IMT) is considered (...) a measure of subclinical atherosclerosis and a predictor of myocardial infarction and stroke.The objective of the study was to evaluate glucose metabolism, lipid profile, IMT of the abdominal aorta, right and left common carotids, carotid bulbs, and common femoral arteries in adult CAH patients.Nineteen (10 females, nine males; 28 +/- 3.5 yr) patients (12 salt wasting and seven simple virilizing) and 19 (10 females, nine males) healthy subjects matched for anthropometric parameters (age, sex, body mass

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2007 Journal of Clinical Endocrinology and Metabolism

3649. Adrenal sensitivity to adrenocorticotropin 1-24 is reduced in patients with autoimmune polyglandular syndrome. (PubMed)

Adrenal sensitivity to adrenocorticotropin 1-24 is reduced in patients with autoimmune polyglandular syndrome. Autoimmune polyglandular syndromes are fairly common diseases that are classified into four constellations based on the clinical clustering of the various component diseases. In types 1, 2, and 4, primary adrenal insufficiency due to an autoimmune process is usually present, but its diagnosis is often delayed because it is difficult to detect in a subclinical phase. It is widely (...) accepted that the classical dose of 250 microg ACTH(1-24) is supramaximal, whereas 0.06 microg has been shown to be one of the lowest ACTH doses that is able to stimulate adrenal secretion in normal young subjects. The aim of this study was to clarify the sensitivity and maximal secretory response of the adrenal gland to ACTH in a group of patients with at least two autoimmune diseases, without clinical signs and symptoms of overt or subclinical hypocortisolism. Cortisol (F), aldosterone

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2004 Journal of Clinical Endocrinology and Metabolism

3650. Altered cortisol metabolism in polycystic ovary syndrome: insulin enhances 5alpha-reduction but not the elevated adrenal steroid production rates. (PubMed)

Altered cortisol metabolism in polycystic ovary syndrome: insulin enhances 5alpha-reduction but not the elevated adrenal steroid production rates. Androgen excess in women with polycystic ovary syndrome (PCOS) may be ovarian and/or adrenal in origin, and one proposed contributing mechanism is altered cortisol metabolism. Increased peripheral metabolism of cortisol may occur by enhanced inactivation of cortisol by 5alpha-reductase (5alpha-R) or impaired reactivation of cortisol from cortisone (...) with 19 lean controls who were closely matched for body mass index (BMI). Second, the impact of obesity was studied in a cross-section of 42 PCOS women of a broad range of BMI. We measured 24-h urinary excretion of steroid metabolites by gas chromatography/mass spectrometry and fasting metabolic and hormone profiles. Urinary excretion of androgens [androsterone (P = 0.003), etiocholanolone (P = 0.02), and C19 steroid sulfates (P = 0.009)], cortisone metabolites [tetrahydrocortisone (THE) (P = 0.02

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2003 Journal of Clinical Endocrinology and Metabolism

3651. Hypothalamic-pituitary-adrenal axis function and the cellular immune response in former preterm children. (PubMed)

Hypothalamic-pituitary-adrenal axis function and the cellular immune response in former preterm children. Animal data suggest that adverse early experiences may affect endocrine and immune functioning in later life.Our objective was to assess the impact of preterm delivery on hypothalamus-pituitary-adrenal axis functioning, heart rate responses, and immune function.Former preterm children [aged 8-14 yr (n = 18)], sex and age-matched full-term born control children (n = 18), data on birth weight (...) , gestational age, birth weight for gestational age (in sd units), actual body weight, height, and body mass index were assessed.Subjects were exposed to a standardized laboratory stressor ("Trier Social Stress Test for Children"). Cortisol in saliva was determined in 10-min intervals before and after the stress test; heart rates were obtained continuously during the stress test. Additional assessment of saliva cortisol was performed: 1) on 3 consecutive days after awakening and at +10, +20, and +30 min

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2007 Journal of Clinical Endocrinology and Metabolism

3652. Patients with classic congenital adrenal hyperplasia have decreased epinephrine reserve and defective glycemic control during prolonged moderate-intensity exercise. (PubMed)

Patients with classic congenital adrenal hyperplasia have decreased epinephrine reserve and defective glycemic control during prolonged moderate-intensity exercise. Patients with classic congenital adrenal hyperplasia (CAH) have adrenomedullary dysplasia and hypofunction, and their lack of adrenomedullary reserve has been associated with a defective glucose response to brief high-intensity exercise.Our objective was to assess hormonal, metabolic, and cardiovascular response to prolonged (...) moderate-intensity exercise comparable to brisk walking in adolescents with classic CAH.We compared six adolescents with classic CAH (16-20 yr old) with seven age-, sex-, and body mass index group-matched controls (16-23 yr old) using a 90-min standardized ergometer test. Metabolic, hormonal, and cardiovascular parameters were studied during exercise and recovery.Glucose did not change throughout exercise and recovery for controls, whereas CAH patients showed a steady decline in glucose during exercise

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2007 Journal of Clinical Endocrinology and Metabolism

3653. Adrenal hyperandrogenism is induced by fetal androgen excess in a rhesus monkey model of polycystic ovary syndrome. (PubMed)

of similar age, body weight, and body mass index were studied during d 2-6 of two menstrual cycles or anovulatory 30-d periods.Predexamethasone adrenal steroid levels were assessed in the first cycle (cycle 1). In a subsequent cycle (cycle 2), occurring one to three cycles after cycle 1, adrenal steroids were determined 14.5-16.0 h after an i.m. injection of 0.5 mg/kg dexamethasone (postdexamethasone levels) and after an i.v. injection of 50 microg ACTH-(1-39).Both before and after dexamethasone, serum (...) Adrenal hyperandrogenism is induced by fetal androgen excess in a rhesus monkey model of polycystic ovary syndrome. Adrenal androgen excess is found in approximately 25-60% of women with polycystic ovary syndrome (PCOS), but the mechanisms underlying PCOS-related adrenal androgen excess are unclear.The objective of this study was to determine whether adrenal androgen excess is manifest in a nonhuman primate model for PCOS.Six prenatally androgenized (PA) and six control female rhesus monkeys

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2005 Journal of Clinical Endocrinology and Metabolism

3654. Overrepresentation of the N363S variant of the glucocorticoid receptor gene in patients with bilateral adrenal incidentalomas. (PubMed)

Overrepresentation of the N363S variant of the glucocorticoid receptor gene in patients with bilateral adrenal incidentalomas. Some variants of the glucocorticoid receptor (GR) gene have been found to alter glucocorticoid sensitivity and have been associated with altered metabolic profiles.The objective of the study was to examine whether N363S and ER22/23K variants of the GR gene may be associated with the development of adrenal incidentalomas and whether these variants may contribute (...) to metabolic abnormalities frequently present in these patients.The study included 99 patients with unilateral and 44 patients with bilateral adrenal incidentalomas, 102 population-matched control subjects, and 100 patients with type 2 diabetes mellitus.Metabolic and hormonal parameters and GR gene variants were determined.When compared with control subjects, the carrier frequency for the N363S variant was markedly and significantly higher in patients with bilateral (7.8 vs. 20.5%, P < 0.05

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2006 Journal of Clinical Endocrinology and Metabolism

3655. Corticotroph adenoma of the pituitary in a patient with X-linked adrenal hypoplasia congenita due to a novel mutation of the DAX-1 gene. (PubMed)

. Three transsphenoidal operations and radiotherapy were necessary to remove the tumor mass and control ACTH secretion. Histologically, the adenoma was composed of chromophobic and basophilic neoplastic cells with positive immunostaining for ACTH. Moreover, a novel mutation was found both in the patient and his mother: a 4 bp insertion (AGCG) at nucleotide 259, in exon 1 resulting in a frame shift and premature termination.This case suggests that in adrenal hypoplasia congenita the development (...) Corticotroph adenoma of the pituitary in a patient with X-linked adrenal hypoplasia congenita due to a novel mutation of the DAX-1 gene. Mutations in the DAX-1 gene result in X-linked congenital adrenal hypoplasia. The classic clinical presentation is primary adrenal insufficiency in early life and hypogonadotropic hypogonadism at the time of expected puberty, but recent data have expanded the phenotypic spectrum of DAX-1 mutations. We report the occurrence of an ACTH-secreting adenoma

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2005 European Journal of Endocrinology

3656. Adrenal neoplasms: CT-guided radiofrequency ablation--preliminary results. (PubMed)

Adrenal neoplasms: CT-guided radiofrequency ablation--preliminary results. To evaluate initial experience with radiofrequency (RF) ablation of adrenal neoplasms.Thirteen adrenal masses in 12 patients (bilateral metastases in one patient) were treated with computed tomography (CT)-guided percutaneous RF ablation. Eleven adrenal lesions were metastases (five from lung cancer, four from renal cell carcinoma, and two from melanoma); one lesion was a pheochromocytoma and one was an aldosteronoma (...) . There were 10 men and two women (average age, 58 years; range, 40-77 years) in the study; average adrenal mass diameter was 3.9 cm (range, 1-8 cm). Average number of RF applications per adrenal mass was 2.7 (range, 1-5 applications); average time per application was 7.8 minutes (range, 4-13 minutes). An internally cooled single electrode was used in five sessions; an internally cooled cluster electrode was used in eight sessions.Average follow-up was 11.2 months (range, 1-46 months). Eleven of 13 lesions

2004 Radiology

3657. Minor morphologic abnormalities of adrenal glands at CT: prognostic importance in patients with lung cancer. (PubMed)

a focal adrenal mass at baseline CT. Two readers independently classified the morphologic features of each adrenal gland as normal, smoothly enlarged, or nodular at initial CT examination. They separately recorded the presence or absence of metastases to the adrenal glands (ie, any new focal adrenal mass) at final CT examination; a third independent reader arbitrated when interpretations were discordant (n = 11). Multivariate Cox proportional hazard models were used to assess for associations between (...) Minor morphologic abnormalities of adrenal glands at CT: prognostic importance in patients with lung cancer. To determine the prognostic importance of minor morphologic abnormalities of the adrenal glands at computed tomography (CT) in patients with lung cancer.The study was approved by the committee on human research; written informed consent was not required. The authors retrospectively identified 197 patients with lung cancer who underwent serial chest or abdominal CT and did not have

2005 Radiology

3658. The role of adrenal venous sampling in the surgical management of primary aldosteronism. (PubMed)

at the Surgical Pathology Institute, University of Padua since 1993. All the patients underwent a CT scan. Adrenal venous sampling was performed in those patients with negative or equivocal findings on imaging studies.Computed tomography was non-contributory in 12 patients and frankly misleading in 2 patients, demonstrating a probable mass lesion in the contralateral but not in the ipsilateral adrenal. Eighteen patients had selective venous sampling that was successful in altering the management of 14 cases (...) . Eleven patients who biochemically had an adrenal adenoma, had normal/equivocal CT, while the remaining 3 had bilateral or contralateral adrenal masses. Venous sampling localized aldosterone secretion and an adenoma, less than 1 cm in diameter, was removed, curing their hypertension. Eleven patients were treated by open adrenalectomy and 35 by the lateral transperitoneal laparoscopic approach. Histological examination revealed 45 Conn's adenomas, of which 13 had a diameter of less than 1 cm (range 0.3

2006 World Journal of Surgery

3659. Bilateral adrenal neuroblastoma and nephroblastoma occurring synchronously in a child with Fanconi's anemia and VACTERL syndrome. (PubMed)

(horseshoe kidney, cerebella hypoplasia, microcephaly, sacral agenesis) and esophageal atresia, which was repaired in neonatal period. Such association led quickly to the diagnosis of FA. At age of 11 months, she developed simultaneously a renal tumor in a horseshoe kidney and bilateral adrenal tumors. The left adrenal mass was removed, and partial nephrectomy was performed. Histological analysis concluded to adrenal neuroblastoma and nephroblastoma. We also evaluated the c-kit expression in these tumors (...) Bilateral adrenal neuroblastoma and nephroblastoma occurring synchronously in a child with Fanconi's anemia and VACTERL syndrome. Fanconi's anemia (FA) is an autosomal recessive inherited syndrome with a predisposition to malignancy. The association between FA and solid pediatric tumors is extremely rare. The authors report a rare case of VACTERL syndrome associated with FA and multiple solid pediatric tumors occurring in a very young girl. This child had numerous congenital anomalies

2006 Journal of Pediatric Surgery

3660. Composite pheochromocytoma associated with adrenal neuroblastoma in an infant: a case report. (PubMed)

Composite pheochromocytoma associated with adrenal neuroblastoma in an infant: a case report. A 5-year-old boy presented with headache and hypertension. Screening abdominal ultrasonography showed a 6-cm-sized mass in the left adrenal gland. Laboratory data included urine vanillylmandelic acid/creatinine (208.3 microg/mg), urine homovanillic acid/creatinine (114.3 microg/mg), serum epinephrine (237 pg/mL), and serum norepinephrine (1842 pg/mL). Metaiodobenzyl guanidine I 123 scintigram (...) , abdominal computed tomography, and abdominal magnetic resonance imaging revealed only left adrenal tumor with no metastasis. Left adrenalectomy was performed transperitoneally, and histopathological diagnosis was an adrenal composite pheochromocytoma associated with neuroblastoma. Immunoreactivity for vimentin was positive in pheochromocytoma, which was negative in neuroblastoma. After operation, the symptom of hypertension disappeared. We should examine the identification of composite pheochromocytoma

2006 Journal of Pediatric Surgery

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