scholarly journals Bilateral Adrenal Hyperplasia as a Possible Mechanism for Hyperandrogenism in Women With Polycystic Ovary Syndrome

2016 ◽  
Vol 101 (9) ◽  
pp. 3353-3360 ◽  
Author(s):  
Evgenia Gourgari ◽  
Maya Lodish ◽  
Meg Keil ◽  
Ninet Sinaii ◽  
Evrim Turkbey ◽  
...  
Keyword(s):  
Polycystic Ovary ◽  
Hormone Secretion ◽  
Urinary Free Cortisol ◽  
Adrenocortical Function ◽  
High Dose ◽  
High Dose Dexamethasone ◽  
Free Cortisol ◽  
Before And After ◽  
Pcos Group ◽  
Adrenal Volume

Abstract Context: Androgen excess may be adrenal and/or ovarian in origin; we hypothesized that a subgroup of patients with polycystic ovarian syndrome (PCOS) may have some degree of abnormal adrenocortical function. Objective: The objective of the study was to evaluate the pituitary adrenal axis with an oral low- and high-dose dexamethasone-suppression test (Liddle's test) in women with PCOS. Design: This was a case-control study. Setting: The study was conducted at the National Institutes of Health Clinical Center. Participants: A total of 38 women with PCOS and 20 healthy volunteers (HV) aged 16–29 years participated in the study. Main Outcome Measures: Urinary free cortisol (UFC) and 17-hydroxysteroids (17OHS) before and after low- and high-dose dexamethasone and assessment of adrenal volume by computed tomography scan were measured. Results: Twenty-four-hour urinary 17OHS and UFC were measured during day 1 to day 6 of the Liddle's test. Baseline UFC levels were not different between PCOS and HVs; on the day after the completion of high-dose dexamethasone administration (d 6), UFC was higher in the PCOS group (2.0 ± 0.7 μg/m2·d) than the HV group (1.5 ± 0.5) (P = .038). On day 5, 17OHS and UFC were negatively correlated with adrenal volumes (left side, rp = −0.47, P = .009, and rp = −0.61, P < .001, respectively). PCOS patients above the 75th percentile for UFC and/or 17OHS after high-dose dexamethasone (n = 15) had a significantly smaller total adrenal volume (6.9 ± 1.9 cm3 vs 9.2 ± 1.8 cm3, P = .003) when compared with the remaining PCOS patients (n = 22), but they did not have worse insulin resistance or hyperandrogenism. Conclusions: In a subset of young women with PCOS, we detected a pattern of glucocorticoid secretion that mimicked that of patients with micronodular adrenocortical hyperplasia: they had smaller adrenal volumes and higher steroid hormone secretion after dexamethasone compared with the group of PCOS with appropriate response to dexamethasone.

scholarly journals MON-483 Familial Dysalbuminemic Hyperthyroxinemia with False Hypercortisolemia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Koki Chiba ◽  
Hiraku Kameda ◽  
Hiroshi Nomoto ◽  
Kyu Yong Cho ◽  
Akinobu Nakamura ◽  
...  
Keyword(s):  
Clinical Endocrinology ◽  
Urinary Free Cortisol ◽  
High Dose ◽  
Acth Level ◽  
Factors Affecting ◽  
High Dose Dexamethasone ◽  
First Case ◽  
Free Cortisol ◽  
Control Serum ◽  
Dominant Disease

Abstract Background: Familial dysalbuminemic hyperthyroxinemia (FDH) is an autosomal dominant disease characterized by hyperthyroxinemia without symptoms of thyrotoxicosis, due to a high affinity of mutant albumin for thyroxine. No report has described cortisol-albumin binding in FDH patient, and here we present a case of FDH presenting with false hypercortisolemia. Clinical Case: A 46-year man, previously diagnosed with FDH by genetic test (1), was pointed out hypercortisolemia with normal ACTH level (ACTH 64.7 pg/mL, Cortisol 34.7 μg/dL) during the investigation for Parkinson’s syndrome and referred to our department for further examination. Cortisol was 7.1 μg/dL at midnight, 14.9 μg/dL after low dose dexamethasone overnight test, and DDAVP test was positive. ACTH and cortisol responded to CRH load, although basal and peak cortisol levels were high (35.4 and 53.9 μg/dL, respectively). High dose dexamethasone overnight test showed suppressed ACTH and cortisol, and MRI showed no obvious pituitary adenoma. In spite of significant high cortisol level, no Cushing sign’s or metabolic abnormalities were observed and urinary free cortisol was within the normal range (30.7 μg/day), suggesting the presence of factors affecting the laboratory testing. We removed albumin from serum with immunoprecipitation using anti-albumin antibody and measured cortisol with LC-MS/MS. The decrease of cortisol was 4% in control serum but 38% in the patient serum after removing albumin, suggesting the binding rate of cortisol to mutant albumin in the patient was increased, leading to false hypercortisolemia. Conclusion: This is the first case demonstrating the false hypercortisolemia in a FDH patient. Clinicians should consider the possibility of the abnormal cortisol binding to albumin in differential diagnosis of hypercortisolemia with normal ACTH level. Reference: (1) Norio Wada, et al: A Novel Missense Mutation in Codon 218 of the Albumin Gene in a Distinct Phenotype of Familial Dysalbuminemic Hyperthyroxinemia in a Japanese Kindred. Journal of Clinical Endocrinology and Metabolism 1997;82;3246–3250

scholarly journals Co Existence of a Corticotroph Adenoma and a Multifunctional Pituitary Cyst

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A578-A578
Author(s):  
David John McDonnell ◽  
Carmel Kennedy ◽  
Yazan Qaoud ◽  
Karen Heverin ◽  
Diarmuid Smith ◽  
...  
Keyword(s):  
Urinary Free Cortisol ◽  
Needle Aspiration ◽  
Solid Component ◽  
High Dose ◽  
Pituitary Hormone ◽  
Cystic Fluid ◽  
Corticotroph Adenoma ◽  
High Dose Dexamethasone ◽  
Free Cortisol ◽  
Pituitary Cyst

Abstract The co-existence of a corticotroph adenoma and a pituitary cyst is very unusual. We present the case of a 50-year-old female who presented with a Cushingoid phenotype, severe hypokalaemia, hyperglycaemia and hypertension. Urinary free cortisol was markedly elevated at 50 fold. ACTH levels were elevated at 121.4 pg/ml. She failed both the low and high dose dexamethasone suppression tests. The CRF test did not show a satisfactory rise in ACTH levels but the Inferior petrosal sinus sampling revealed a central to peripheral ACTH gradient highly suggestive of Pituitary dependant Cushing’s. MRI pituitary revealed a large cystic lesion with a small solid component. Computed Tomography (CT) of thorax abdomen and pelvis was normal. A trans-sphenoidal hypophysectomy was performed, during which a fine needle aspiration of the intra-cystic fluid was obtained. This showed markedly elevated pituitary hormone levels of ACTH (1399pg/ml), prolactin (353,084mIU/L), TSH (217IU/L) FSH (205mIU/ml) and GH (519 ng/ml) consistent with a multifunctional pituitary cyst. Neuropathology of the solid component confirmed a corticotroph adenoma. Post-operative am cortisol levels were persistently suppressed to less than 50 nmol/l with marked improvement in clinical features. This case highlights the challenges in the work up of Cushing’s syndrome and the limitations of diagnostic tests. The coexistence of a corticotroph adenoma and a multifunctional pituitary cyst is very unusual and to our knowledge has not been reported before.

Use of In-111 pentetreotide scintigraphy in the diagnosis of a midgut carcinoid causing Cushing's syndrome

1997 ◽  
pp. 79-83 ◽  
Author(s):  
VB Segu ◽  
DM Mahvi ◽  
MA Wilson ◽  
SJ Hale ◽  
TF Warner ◽  
...  
Keyword(s):  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Single Photon ◽  
Photon Emission ◽  
Urinary Free Cortisol ◽  
Cushing's Syndrome ◽  
High Dose ◽  
High Dose Dexamethasone ◽  
Malignant Carcinoid ◽  
Free Cortisol

A 57-year-old man presented with clinical features of hypercortisolism and was diagnosed with ACTH-dependent Cushing's syndrome. Biochemical testing showed partial suppression of urinary free cortisol with high dose dexamethasone. Initial computed tomography (CT) of the chest and abdomen, and magnetic resonance imaging of the pituitary were negative. In-111 pentetreotide scintigraphy with single photon emission computerized tomography revealed two 'hot' lesions in the abdomen which were then confirmed by subsequent directed thin-slice abdominal CT and small bowel barium study. At surgery, two segments of ileum, adjoining mesentery and lymph nodes were resected. Histopathology was consistent with a malignant carcinoid tumor of the ileum which stained intensely for ACTH. Plasma ACTH, and serum and urinary cortisol normalized postoperatively. To our knowledge, this is the first reported case of ileal carcinoid tumor causing Cushing's syndrome with premortem diagnosis. Another unique feature of this case is that In-111 pentetreotide scan provided the decisive clue to localization of the tumor.

scholarly journals The pituitary–adrenal axis in adult thalassaemic patients

2010 ◽  
Vol 162 (1) ◽  
pp. 43-48 ◽  
Author(s):  
Massimo Scacchi ◽  
Leila Danesi ◽  
Agnese Cattaneo ◽  
Elena Valassi ◽  
Francesca Pecori Giraldi ◽  
...  
Keyword(s):  
Serum Cortisol ◽  
Urinary Free Cortisol ◽  
Stressful Events ◽  
Adrenocortical Function ◽  
High Dose ◽  
Plasma Acth ◽  
Mean Values ◽  
Free Cortisol ◽  
Basal Plasma ◽  
The Mean

ObjectiveWe previously described in young thalassaemic patients an altered cortisol and ACTH responsiveness suggesting an impaired adrenocortical reserve. Owing to iron overload, a worsening of adrenal function should be expected in adult patients.DesignIn 124 adults with β-thalassaemia, urinary free cortisol (UFC) and plasma ACTH levels were determined and compared with those measured in 150 controls. In 45 patients, cortisol was measured in response to: i) tetracosactide 1 μg as an i.v. bolus (low-dose test, LDT) and ii) tetracosactide 250 μg infused i.v. over 8 h (high-dose test, HDT).ResultsUFC and serum cortisol were within the reference range in all patients. Conversely, basal plasma ACTH values were above the upper limit of the normal range in 19 patients. There were no statistically significant differences in the mean values of UFC, basal serum cortisol and plasma ACTH between patients and controls. A subnormal cortisol response to the LDT was registered in 18 out of 56 patients. Three of these patients also displayed a subnormal response to the HDT, together with elevated baseline plasma ACTH levels. In the LDT, a positive correlation was found between basal and peak cortisol values (P<0.0001). The latter were negatively correlated with basal ACTH values in both LDT (P<0.0001) and HDT (P<0.0001).ConclusionsAdult thalassaemic patients often present a subtle impairment of adrenocortical function. This may become clinically relevant in case of major stressful events. Thus, we recommend an assessment of adrenocortical function in all adult thalassaemic patients.

High-dose dexamethasone shifts the balance of stimulatory and inhibitory Fcγ receptors on monocytes in patients with primary immune thrombocytopenia

Blood ◽  
2011 ◽  
Vol 117 (6) ◽  
pp. 2061-2069 ◽  
Author(s):  
Xin-guang Liu ◽  
Shi-hui Ma ◽  
Jian-zhi Sun ◽  
Juan Ren ◽  
Yan Shi ◽  
...  
Keyword(s):  
Immune Thrombocytopenia ◽  
High Dose ◽  
Fcγ Receptor ◽  
Fcγ Receptors ◽  
Phagocytic Capacity ◽  
High Dose Dexamethasone ◽  
Significant Difference ◽  
Primary Immune Thrombocytopenia ◽  
Before And After

Abstract The human Fcγ receptor (FcγR) system is composed of 2 opposing families, the activating FcγRs (FcγRI, FcγRIIa, and FcγRIII) and the inhibitory FcγR (FcγRIIb). The disturbed balance of the activating and inhibitory FcγRs has been implicated in the pathogenesis of many autoimmune diseases. In this study, the expression of FcγRs on monocytes was determined in 23 patients with primary immune thrombocytopenia (ITP) before and after high-dose dexamethasone (HD-DXM) treatment. The FcγRI expression was significantly higher in ITP patients and decreased after HD-DXM treatment. The ratio of FcγRIIa/IIb mRNA expression on monocytes was significantly higher in untreated patients than in healthy controls. After HD-DXM therapy, the ratio decreased and the increased expression of FcγRIIb mRNA and protein coincided with a remarkable decrease in the expression of FcγRIIa, FcγRI, and monocyte phagocytic capacity. There was no significant difference in FcγRIII expression on monocytes between patients and controls. In vitro cell-culture experiments showed that DXM could induce FcγRIIa and FcγRIIb expression in monocytes from ITP patients, with FcγRIIb at higher amplitudes. These findings suggested that the disturbed FcγR balance might play a role in the pathogenesis of ITP, and that HD-DXM therapy could shift monocyte FcγR balance toward the inhibitory FcγRIIb in patients with ITP.

scholarly journals Partial restoration of GH responsiveness to ghrelin in Cushing's disease after 6 months of ketoconazole treatment: comparison with GHRP-6 and GHRH

2009 ◽  
Vol 161 (5) ◽  
pp. 681-686 ◽  
Author(s):  
Silvia R Correa-Silva ◽  
Sérgio O Nascif ◽  
Patrícia Molica ◽  
Larissa B P C Sá ◽  
José G Vieira ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Urinary Free Cortisol ◽  
Cushing's Disease ◽  
Partial Restoration ◽  
Gh Secretion ◽  
Treatment Comparison ◽  
Time Period ◽  
Free Cortisol ◽  
Before And After ◽  
Serum Gh

ObjectiveIn Cushing's disease (CD), GH responsiveness to several stimuli, including ghrelin, GHRP-6, and GHRH, is blunted. Recovery of GH secretion after remission of hypercortisolism after transsphenoidal surgery, radiotherapy, or adrenalectomy is controversial. There are no studies evaluating the effect of primary clinical treatment with ketoconazole on GH secretion in CD. The aim of this study is to compare ghrelin-, GHRP-6-, and GHRH-induced GH release before and after ketoconazole in CD.DesignGH responses to ghrelin, GHRP-6, and GHRH of eight untreated patients with CD (mean age: 33.8±3.1 years; body mass index: 28.5±0.8 kg/m2) were evaluated before and after 3 and 6 months of ketoconazole treatment, and compared with 11 controls (32.1±2.5; 25.0±0.8).MethodsSerum GH was measured by an immunofluorometric assay and urinary free cortisol (UFC) by liquid chromatography and tandem mass spectrometry.ResultsAfter ketoconazole use, mean UFC decreased significantly (before: 222.4±35.0 μg/24 h; third month: 61.6±10.1; sixth month: 39.1±10.9). Ghrelin-induced GH secretion increased significantly after 6 months (peak before: 6.8±2.3 μg/l; sixth month: 16.0±3.6), but remained lower than that of controls (54.1±11.2). GH release after GHRP-6 increased, although not significantly, while GH responsiveness to GHRH was unchanged.ConclusionsGhrelin-induced GH release increases significantly after 6 months of ketoconazole treatment in CD. This could suggest that a decrease in cortisol levels during this time period can partially restore glucocorticoid-induced GH suppression in CD. GH-releasing mechanisms stimulated by ghrelin/GHS could be more sensitive, as no changes in GHRH-induced GH release were observed.

Increased urinary free cortisol and decreased serum corticosteroid-binding globulin in polycystic ovary syndrome

1991 ◽  
Vol 125 (1) ◽  
pp. 28-32 ◽  
Author(s):  
Cecilia Invitti ◽  
Francesca Pecori Giraldi ◽  
Antonella Dubini ◽  
Martina De Martin ◽  
Francesco Cavagnini
Keyword(s):  
Polycystic Ovary Syndrome ◽  
Polycystic Ovary ◽  
Free Testosterone ◽  
Urinary Free Cortisol ◽  
Sex Hormone ◽  
Sex Hormone Binding Globulin ◽  
Hormone Binding ◽  
Ovary Syndrome ◽  
Free Cortisol ◽  
Corticosteroid Binding Globulin

Abstract. The adrenal participation in the pathogenesis of polycystic ovary syndrome is still under debate. In order to reappraise androgen and glucocorticoid secretion in this disease, we measured serum androstenedione, dehydroepiandrosterone-sulphate, total and free testosterone, sex hormone-binding globulin, LH, FSH, PRL, cortisol, corticosteroid-binding globulin, and urinary free cortisol in 45 women with polycystic ovary syndrome and 27 controls, subdivided in obese and normal-weight subjects. Androstenedione, total and free testosterone were significantly increased, whereas sex hormone-binding globulin tended to be reduced in patients with polycystic ovary syndrome compared with controls, reaching a significant difference between obese patients and matched controls. Free testosterone and sex hormone-binding globulin were significantly increased and reduced, respectively, in obese compared with normal-weight patients. Urinary free cortisol and serum corticosteroid-binding globulin were significantly increased (p<0.001) and decreased (p<0.005), respectively. Urinary free cortisol exceeded the upper limit of the normal range in 50% of our patients. No appreciable differences were found in PRL and cortisol levels. Besides confirming a hyperandrogenic state, our findings point to an overactivity of the hypothalamic-pituitary-adrenal axis with subsequent diminution of corticosteroid-binding globulin in polycystic ovary syndrome. They also indicate that urinary free cortisol is not a reliable index in differentiating polycystic ovary syndrome from Cushing's disease.

scholarly journals Ectopic Acth-Producing Pulmonary Carcinoid: A Rare Case of Simulating the Cushing’s disease

2020 ◽  
Vol 3 (4) ◽  
pp. 01-04
Author(s):  
Manouchehr Nakhjavan
Keyword(s):  
Cushing’S Syndrome ◽  
Mediastinal Lymph Node ◽  
Left Lung ◽  
Cushing's Syndrome ◽  
Whole Body ◽  
High Dose ◽  
Ectopic Acth ◽  
Pulmonary Carcinoid ◽  
High Dose Dexamethasone ◽  
Free Cortisol

Excess amounts of blood glucocorticosteroids, either exogenous or endogenous, results in Cushing’s syndrome (CS). the current study presents a 45-year-old man, ACTH dependent Cushing’s syndrome. The basal cortisol and urine free cortisol level were significantly suppressed following high dose dexamethasone suppression test and pituitary MRI showed microadenoma with a size of less than 6 mm. After bilateral inferior petrosal sinus sampling, we investigated the ectopic source of ACTH production. The computed tomography revealed a mediastinal lymph node, and the whole body octreotide scan revealed octreotide avid lesion in the medial aspect of the left lung. The patient underwent left lung lobectomy and the pathological study indicated the carcinoid tumor of the left lung.

scholarly journals Volumetric Modeling of Adrenal Gland Size in Primary Bilateral Macronodular Adrenocortical Hyperplasia

2020 ◽  
Vol 5 (1) ◽  
Author(s):  
Rachel Wurth ◽  
Amit Tirosh ◽  
Crystal D C Kamilaris ◽  
Jancarlos Camacho ◽  
Fabio R Faucz ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Tertiary Care ◽  
Secondary Analysis ◽  
Urinary Free Cortisol ◽  
Clinical Genetic ◽  
Cross Sectional ◽  
Gland Size ◽  
Free Cortisol ◽  
Adrenocortical Hyperplasia ◽  
Adrenal Volume

Abstract Context Radiological characterization of adrenal size in primary bilateral macronodular adrenocortical hyperplasia (PBMAH) has not been previously investigated. Objective We hypothesized that volumetric modeling of adrenal gland size may correlate with biochemical disease severity in patients with PBMAH. Secondary analysis of patients with concurrent primary aldosteronism (PA) was performed. Design A retrospective cross-sectional analysis of 44 patients with PBMAH was conducted from 2000 to 2019. Setting Tertiary care clinical research center. Patients Patients were diagnosed with PBMAH based upon clinical, genetic, radiographic and biochemical characteristics. Intervention Clinical, biochemical, and genetic data were obtained. Computed tomography scans were used to create volumetric models by manually contouring both adrenal glands in each slice using Vitrea Core Fx v6.3 software (Vital Images, Minnetonka, Minnesota). Main Outcome and Measures 17-hydroxycorticosteroids (17-OHS), ARMC5 genetics, and aldosterone-to-renin ratio (ARR) were retrospectively obtained. Pearson test was used for correlation analysis of biochemical data with adrenal volume. Results A cohort of 44 patients with PBMAH was evaluated, with a mean age (±SD) of 53 ± 11.53. Eight patients met the diagnostic criteria for PA, of whom 6 (75%) were Black. In the Black cohort, total adrenal volumes positively correlated with midnight cortisol (R = 0.76, P = 0.028), urinary free cortisol (R = 0.70, P = 0.035), and 17-OHS (R = 0.87, P = 0.0045), with a more pronounced correlation with left adrenal volume alone. 17-OHS concentration positively correlated with total, left, and right adrenal volume in patients harboring pathogenic variants in ARMC5 (R = 0.72, P = 0.018; R = 0.65, P = 0.042; and R = 0.73, P = 0.016, respectively). Conclusions Volumetric modeling of adrenal gland size may associate with biochemical severity in patients with PBMAH, with particular utility in Black patients.

scholarly journals A Properly Balanced Reduction Diet and/or Supplementation Solve the Problem with the Deficiency of These Vitamins Soluble in Water in Patients with PCOS

Nutrients ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 746
Author(s):  
Małgorzata Szczuko ◽  
Iwona Szydłowska ◽  
Jolanta Nawrocka-Rutkowska
Keyword(s):  
Liquid Chromatography ◽  
Vitamin C ◽  
Dietary Intervention ◽  
Polycystic Ovary ◽  
Glycaemic Index ◽  
Reproductive Age ◽  
Control Group ◽  
Before And After ◽  
Pcos Group ◽  
Biochemical Analyses

Polycystic ovary syndrome (PCOS) is an increasingly common problem for women in the reproductive age throughout the entire world. A reduction diet with a low glycaemic index (GI) has proved to support the treatment of PCOS. The aim of the study was to analyse the influence of the diet on the level of vitamins soluble in water. The study included 55 women, 40 of which suffered from PCOS (identified by means of the Rotterdam Criteria) and 15 healthy women of the Caucasian race. The level of vitamins before and after the dietary intervention was measured. The diet was a reduction diet with a reduced glycaemic index (GI). Biochemical analyses were made on the basis of liquid chromatography—Infinity 1260 Binary liquid chromatography (LC) Agilent Technology. The level of vitamins in the serum was analysed together with the consumption before and after the dietary intervention. A higher level of vitamin C in the plasma was observed before and after the dietary intervention in the PCOS group in comparison to the control group despite the lower intake of this vitamin in the PCOS group. The remaining vitamins were at a comparable or lower level (B1, B3, B5, B6 and B12). After the dietary intervention, only B1 and B9 were at a clearly lower level (a trend of p = 0.093 and p = 0.085). A properly balanced reduction diet with reduced GI improves the supply of vitamins in women with PCOS. An additional recommendation should be the additional supplementation of B1, niacinamide and the combination of folates with inositol. The level of vitamin C in the plasma may not be a good marker of its supply in the PCOS group.

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