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Louiset E E INSERM, U982, Laboratory of Differentiation & Neuronal and Neuroendocrine Communication, IRIB, University of Rouen, Mont-Saint-Aignan, - - 2014
Illegitimate G-protein coupled receptors are known to control cortisol secretion in adrenal adenomas and bilateral macronodular adrenal hyperplasias (BMAHs) causing Cushing's syndrome. In the present study, we have evaluated the role of glucagon in the regulation of cortisol secretion in 13 patients with BMAH or adrenocortical adenoma causing subclinical or ...
Mukherjee Anirban A From the Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, - - 2014
A 35-year-old man with clinical suspicion of adrenal pheochromocytoma was evaluated using Ga DOTANOC PET/CT. PET/CT demonstrated Ga DOTANOC-avid right adrenal mass and cerebellar lesion, raising the suspicion of adrenal pheochromocytoma with cerebellar hemangioblastoma suggesting von Hippel-Lindau (VHL) syndrome. Cerebellar lesion on further evaluation with MRI was suggestive of cerebellar ...
Krysiak Robert R Department of Internal Medicine and Clinical Pharmacology, Medical University of Silesia, Katowice, - - 2014
Excessive prolactin release and/or receptor action may be implicated in the pathogenesis of many autoimmune disorders. We report here a case of a woman who developed Graves' disease and subclinical autoimmune adrenal failure, and was diagnosed as having autoimmune polyglandular syndrome type 2. Because of coexisting microprolactinoma she was treated ...
Starker Lee F LF Department of Surgery, Yale University School of Medicine, 333 Cedar Street, New Haven, CT, - - 2014
Owing to its diagnostic challenges, subclinical Cushing syndrome (SCS) is likely to be highly underdiagnosed and undertreated, and the overall incidence may be as high as 5% to 20% in patients with adrenal incidentalomas. The diagnosis can be established by a systematic and thorough biochemical evaluation. SCS has been associated ...
Borzouei Shiva S Department of Internal Medicine, Hamedan University of Medical Sciences, Hamedan, - - 2014
Multiple Endocrine Neoplasia type IIa (MEN IIa) is an autosomal dominant syndrome characterized by pheochromocytoma, medullary thyroid carcinoma and hyperparathyroidism. Pheochromocytoma occurs in approximately 50% of patients with MEN IIa. This tumor has the capacity to produce ACTH ectopically and manifests as the Cushing syndrome, although it is very rare. ...
Sato Yusuke Y Department of Pathology and Tumor Biology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. Department of Urology, Graduate School of Medicine, The University of Tokyo, Tokyo, - - 2014
Cushing's syndrome is caused by excess cortisol production from the adrenocortical gland. In corticotropin-independent Cushing's syndrome, the excess cortisol production is primarily attributed to an adrenocortical adenoma, in which the underlying molecular pathogenesis has been poorly understood. We report a hotspot mutation (L206R) in PRKACA, which encodes the catalytic subunit ...
Will Britta B Department of Cell Biology, Albert Einstein College of Medicine/Montefiore Medical Center, New York, NY 10461, USA; Ruth L. and David S. Gottesman Institute for Stem Cell and Regenerative Medicine Research, Albert Einstein College of Medicine/Montefiore Medical Center, New York, NY 10461, - - 2014
Heterozygous deletions of chromosome 7 are frequent in myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). In this issue of Cancer Cell, Chen and colleagues identify MLL3 as a novel haplo-insufficient tumor suppressor on 7q that, in combination with NF1 suppression and TP53 deficiency, mediates MDS and AML phenotypes in ...
Nakagishi Yasuo Y Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children's Hospital , Kobe, Hyogo , - - 2014
Macrophage activation syndrome (MAS) is a severe and potential life-threatening complication of childhood systemic inflammatory disorders. Corticosteroids are commonly used as the first-line therapy for MAS. We report four patients with MAS who were successfully treated with dexamethasone palmitate (DexP), a liposome-incorporated dexamethasone, much more efficient than free corticosteroids. DexP ...
Raff Hershel H Endocrine Research Laboratory, Aurora St. Luke's Medical Center, Aurora Research Institute and Departments of Medicine, Surgery, and Physiology, Medical College of Wisconsin, Milwaukee, - - 2014
The hypothalamic-pituitary-adrenal (HPA) axis is a classic neuroendocrine system. One of the best ways to understand the HPA axis is to appreciate its dynamics in the variety of diseases and syndromes that affect it. Excess glucocorticoid activity can be due to endogenous cortisol overproduction (spontaneous Cushing's syndrome) or exogenous glucocorticoid ...
Walls Gerard V GV Nuffield Department of Surgical Sciences, University of Oxford, Headington, Oxford, UK; Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Headington, Oxford, UK. Electronic address: - - 2014
Multiple endocrine neoplasia (MEN) syndromes are characterised by the combined occurrence of two or more endocrine tumours in a patient. These autosomal dominant conditions occur in four types: MEN1 due to inactivating MEN1 mutations; MEN2A and MEN2B (MEN3) due to activating mutations of RET and MEN4 due to inactivating cyclin-dependent ...
Elias Pcl P Division of Endocrinology-Department of Medicine - - 2014
Context: The comparison of variability, reproducibility, and diagnostic performance of late-night salivary cortisol (LNSF) and urinary free cortisol (UFC) using concurrent and consecutive samples in Cushing's syndrome (CS) is lacking. Objectives, Patients and Methods: In a prospective study, we evaluate three simultaneous and consecutive samples of LNSF by RIA and ...
Nella Aa A Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, - - 2014
Context: Ectopic Cushing syndrome due to ACTH secretion from metastatic medullary thyroid cancer (MTC) is associated with significant morbidity and mortality. Objective: To describe the first case of Cushing syndrome associated with MTC in a pediatric patient and the successful reversal of Cushing syndrome with vandetanib therapy. Patient and Methods: ...
Liu Min - - 2014
Abstract Aim: To investigate the etiology and clinical characteristics of hypothalamic syndrome in Chinese children. Methods: Thirty-three cases of hypothalamic syndrome were analyzed for etiology, initial symptoms, and clinical characteristics. Results: All of the 33 patients manifested symptoms of hypothalamic dysfunction and disorders of the hypothalamus-hypophysis-target gland axis. Fourteen patients ...
Choi Hong Seok HS Department of Internal Medicine, Korea Cancer Center Hospital, Seoul, - - 2014
Ectopic adrenocorticotropic hormone (ACTH) syndrome is caused most frequently by a bronchial carcinoid tumor or by small cell lung cancer. Medullary thyroid carcinoma (MTC) is a rare etiology of ectopic ACTH syndrome. We describe a case of Cushing syndrome due to ectopic ACTH production from MTC in a 48-year-old male. ...
Efstathiadou Zoe A ZA Department of Endocrinology, 'Hippokration' General Hospital of Thessaloniki, Thessaloniki, - - 2014
An increased prevalence of thyroid autoimmunity has been observed after successful treatment of Cushing's syndrome. On the other hand, De Quervain's thyroiditis (DQT), in which autoimmunity is not a pathogenetic contributor, has not been reported during recovery from Cushing's syndrome. We describe 2 female patients with DQT coinciding with the ...
Barroso-Sousa Romualdo R Instituto do Cancer do Estado de Sao Paulo, Medical Oncology, Sao Paulo, Brazil ; - - 2014
Background: The treatment of advanced medullary thyroid carcinoma (MTC) has significantly evolved over the past decade. The discovery of genetic abnormalities in MTC led to the development of targeted therapies such as vandetanib and cabozantinib. Other kinase inhibitors (KI), such as Sorafenib, have been investigated in this setting and are ...
Di Dalmazi Guido G Endocrinology Unit, Department of Medical and Surgical Sciences, University Alma Mater Studiorum of Bologna, S Orsola-Malpighi Hospital, Bologna, - - 2014
Incidental discovery of adrenal masses has increased over the past few years. Mild alterations in cortisol secretion without clinical signs of overt hypercortisolism (subclinical Cushing's syndrome) are a common finding in patients with these tumours. Although metabolic alterations and increased cardiovascular risk have been noted in patients with subclinical Cushing's ...
Reich Jerome M JM Earl A Chiles Research Institute, 5251 NE Glisan, Bldg A, Portland, OR 97213-2967, United States. Electronic address: - - 2014
Volitional cough suppression, identified exclusively in females, is an unusual causal mechanism for instances of lobar atalectasis and bronchiectasis. It is a postulated mechanism for the genesis of Lady Windermere Syndrome.
Bakiner Okan O Department of Endocrinology and Metabolism Diseases, Faculty of Medicine, Baskent University, Dadaloglu Mah Serinevler, 01250 Adana, - - 2014
We aimed to observe the association between degree of obesity and metabolic syndrome and plasma thyrotropin levels in obese, euthyroid Turkish patients. 947 obese and overweight patients who admitted to our outpatient clinic were assessed retrospectively. 150 healthy euthyroid cases were also recruited as the control group. Cases with metabolic ...
Sakihara Satoru S Department of Endocrinology and Metabolism Hirosaki University Graduate School of Medicine 5 Zaifu-cho, Hirosaki, Aomori, 036-8562 - - 2014
ACTH-dependent Cushing's syndrome includes Cushing's disease and ectopic ACTH syndrome (EAS). The differential diagnosis of Cushing's disease from EAS in cases of ACTH-dependent Cushing's syndrome is a challenging problem. We report here a case of EAS with an unknown source of ACTH secretion. Extensive imaging procedures, involving computed tomography (neck ...
Ueda Shuji S Department of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, - - 2014
POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated with very-low-dose lenalidomide and dexamethasone. A 57-year-old Japanese ...
Paredes Sílvia S Faculty of Medicine, University of - - 2014
Objective This article reviews the state of the art regarding the association between glucocorticoid actions and both obesity and insulin resistance, two main features of the metabolic syndrome. Methods A methodological assessment of the literature on PubMed and SciELO databases was conducted by using the following terms: stress, metabolic syndrome, ...
Lacroix André - - 2013
Cushing's syndrome is a challenging disease in which excess cortisol is secondary to diverse tumors with complex molecular mechanisms. The syndrome has been categorized as corticotropin-dependent or corticotropin-independent. Approximately 20% of cases - mainly cortisol-secreting unilateral adenomas or carcinomas - are considered corticotropin-independent. Cushing's syndrome rarely (in <2% of cases) ...
Poiană C - - 2013
Cushing’s syndrome is a pathological condition where surgerymay be lifesaving. The proper diagnosis depends upon thehormonal pattern of the patient, various dynamic tests andimagistic investigations. We report a case of a patient withCushing’s syndrome, with bilateral adrenal tumors and apituitary microadenoma. She presented increased levels ofbasal cortisol, unsuppressed during a ...
Thynne T - - 2013
Factitious Cushing's syndrome is extremely rare. The diagnosis is challenging as cross-reactivity of synthetic corticosteroids or their metabolites in immunoassay measurements of plasma or urinary cortisol can make distinguishing between true and factitious Cushing's syndrome difficult. Adrenocorticotropin (ACTH) is usually suppressed in factitious Cushing's syndrome. A 54 year old woman ...
Goyal Ashutosh A Department of Endocrinology, Sir Ganga Ram Hospital, New Delhi, - - 2013
Adrenal incidentalomas (AIs) are a cluster of different pathologies, but AIs with dual functional aspects are very rare. We report a case of AI with the evidence of both pheochromocytoma and sub-clinical Cushing's syndrome. A 42-year-old female patient presented with the history of abdominal pain. Abdominal computed tomography revealed right ...
Sanyal Debmalya D Department of Endocrinology, KPC Medical College, Kolkata, West Bengal, - - 2013
Addison's disease or primary adrenal insufficiency (PAI) is a rare manifestation of antiphospholipid syndrome (APS). PAI is the most common among the endocrinologic manifestations and can also rarely be the presenting symptom of APS. Venous thrombosis and/or adrenal hemorrhage are the leading cause of PAI in APS. Autoimmune adrenal failure ...
Tiwari Ashish A Department of Paediatrics, Gandhi Medical College, Bhopal, Madhya Pradesh, - - 2013
Cushing syndrome, a systemic disorder, is the result of abnormally high blood level of cortisol or other glucocorticoids. The most common cause of Cushing syndrome is prolonged exogenous administration of glucocorticoid hormones. Prolonged use of topical corticosteroids, particularly in children, may cause Cushing syndrome and suppression of the hypothalamopituitory-adrenal axis, ...
Ray Animesh A Department of Pulmonary Critical Care and Sleep Medicine, Vardhman Mahavir Medical College and Safdarjang Hospital, New Delhi, - - 2013
Adrenal crisis occurs when there is decreased secretions of steroid hormones (mainly cortisol) from the adrenal glands due to varied reasons. It may arise due to a primary adrenal condition or due to decreased hormonal signals from the pituitary secondary to a hypofunctioning pituitary. Hypopituitarism may result due to direct ...
Gurazada K - - 2013
We report the first case of an Ectopic adrenocorticotrophin (ACTH)-secreting pituitary adenoma (EAPA) located within the posterior nasal septum associated with Nelson's syndrome, which eluded diagnosis for over a decade. In this report, we explore the reasons for such diagnostic difficulty and suggest ways in which an earlier diagnosis may ...
Zhang Qian - - 2013
The cortisol concentration in saliva is 10-fold lower than total serum cortisol and accurately reflects the serum concentration, both levels being lowest around midnight. The salivary cortisol assay measures free cortisol and is unaffected by confounding factors. This study analysed published data on the sensitivity and specificity of salivary cortisol ...
Giovansili Iama - - 2013
Objective: To report the case of a patient with Cushing disease revealed by bilateral Central Serous Chorioretinopathy (CSCR).Methods: We present the clinical history, physical findings, laboratory results, and imaging studies in a 53-year-old woman with a Cushing disease revealed by bilateral CSCR. The association with CSCR and the pertinent literature ...
Starkman Monica N - - 2013
This article reviews the neuropsychiatric presentations elicited by spontaneous hypercortisolism and exogenous supraphysiologic glucocorticoids. Patients with Cushing disease and syndrome develop a depressive syndrome: irritable and depressed mood, decreased libido, disrupted sleep and cognitive decrements. Exogenous short-term glucocorticoid administration may elicit a hypomanic syndrome with mood, sleep and cognitive disruptions. ...
Li Wei-Wei - - 2013
"Qi" and "blood" are two essential concepts in Chinese medicine (CM). As qi is intangible, the concept of qi is still controversial between CM and Western medicine. However, the endogenous hydrogen sulfide (H2S) and other gaseous signaling molecules provides a new approach for understanding the essence of qi in CM. ...
Goldberg Alyse S - - 2013
Abstract Ectopic ACTH syndrome is a rare but important cause of pediatric Cushing syndrome, for which management by a multidisciplinary team is required. Although diagnostic evaluation is similar to that in adults, the variation in epidemiology may sway investigations, leading to inappropriate and/or incomplete diagnostic interventions. We present a case ...
Sharma S T - - 2013
Inferior petrosal sinus sampling (IPSS) is considered the gold standard test to distinguish between Cushing's disease (CD) and ectopic ACTH syndrome (EAS). Anomalous venous drainage, abnormal venous anatomy and lack of expertise can lead to false-negative IPSS results and thereby misclassification of patients with ACTH-dependent Cushing's syndrome. Prolactin measurement during ...
Ortega Margarita - - 2013
A 72-year-old woman was diagnosed with 5q- myelodysplastic syndrome in the course of an indolent multiple myeloma (MM). Bone marrow (BM) cytogenetics disclosed two unrelated clones: 46,XX,del(5)(q13q33), and [47,X,-X,der(1;21)(q10;q10),-4,-4,+5,del(5)(q13q31),+7,der(7)t(1;7)(p34.2;p22),add(8)(p23),-13,+15,der(16) t(1;16)(q23;q12.2),+19,-21,+mar1,+mar2]. The last complex karyotype belonged to malignant plasma cells. FISH and SKY techniques demonstrated different 5q deletions. EGR1 gene (on ...
Raff Hershel - - 2013
Late-night salivary cortisol (LNSC) is now considered the best approach to screen patients suspected of having endogenous hypercortisolism (Cushing's syndrome). As the use of LNSC increases, new preanalytic and analytic issues have arisen. The routine immunoassay for salivary cortisol seems to have better diagnostic performance than liquid chromatograph/tandem mass spectrometry, ...
Aulinas Anna - - 2013
Cushing syndrome (CS), due to an ACTH-secreting pituitary adenoma, adrenal tumors, or ectopic ACTH secretion, causes hypercortisolism. CS is associated with major morbidity, especially metabolic and cardiovascular complications, osteoporosis, psychiatric changes, and cognitive impairment. Despite biochemical "cure" of hypercortisolism and clinical improvement after effective treatment, these complications are only partially ...
Nam Kyung-Jee - - 2013
Subclinical Cushing syndrome (SCS) is a hypothalamic-pituitary-adrenal axis abnormality characterized by autonomous cortisol secretion in patients with no typical signs or symptoms of Cushing syndrome. SCS patients may have adverse metabolic and cardiovascular effects due to slight, but continuous glucocorticoid secretion. Glucocorticoids also affect behavior, mood, neural activity, and a ...
Odeniyi I A - - 2013
Cushing's syndrome results from chronic exposure to excessive circulating levels of glucocorticoids. To confirm the clinical suspicion, biochemical tests are needed. These biochemical tests include the measurement of excess total endogenous cortisol secretion assessed by 24-hour urinary free cortisol (UFC), loss of the normal feedback of the hypothalamo-pituitary-adrenal axis assessed ...
Song Yu - - 2013
Triamcinolone is a long-acting glucocorticoid medication that can be responsible for transient suppression of the hypothalamic-pituitary-adrenal (HPA) axis. This physiologic alteration may persist for weeks after repeated or even single localized injection of this agent. However, when this glucocorticoid agent is given to patients receiving the HIV protease inhibitor (PI) ...
Alcantara Valeria A - - 2013
Objective: We present a rare case of Cushing syndrome due to ectopic ACTH secretion (EAS). To our knowledge only two similar cases have been previously reported.Methods: This is a case report of EAS by a Metastatic Lingual Adenoid Cystic Carcinoma (ACC).Results: The patient was diagnosed of a Cushing Syndrome caused ...
Ku Eu Jeong EJ Department of Internal Medicine, Seoul National University College of Medicine, Seoul, - - 2013
A 48-year-old woman was incidentally found to have bilateral adrenal masses, 2.8 cm in diameter on the right, and 2.3 cm and 1.7 cm in diameter on the left, by abdominal computed tomography. The patient had a medical history of hypertension, which was not being controlled by carvedilol, at a ...
Katz Leila - - 2013
BACKGROUND: Hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome is one of the most severe forms of preeclampsia and aggravates both maternal and perinatal prognosis. The systematic review available in Cochrane Library compared corticosteroid (dexamethasone, betamethasone, or prednisolone) given during pregnancy, just after delivery or in the postnatal period, ...
Tamada Daisuke - - 2013
Context:Hyperthyroidism with the syndrome of inappropriate secretion of thyroid stimulating hormone (TSH) (SITSH) occurred by a decrease in hydrocortisone dose after surgery for Cushing's syndrome. This is a novel cause of SITSH.Objective:The aim of this study was to describe and discuss the two cases of SITSH patients who were found ...
Hashemzadeh Shahriar - - 2013
Adrenocorticotropic hormone (ACTH) overproduction within the pituitary gland or ectopically leads to hypercortisolism. In this study a case of Cushing's syndrome caused by an ectopic ACTH-secreting carcinoid tumor in lung is discussed, as are the available diagnostic procedures. The patient was a 28-year-old woman with clinical features starting about 6 ...
Kharb Sandeep - - 2013
Multiple endocrine neoplasia-1 (MEN1) is an autosomal dominant syndrome with classic triad of parathyroid hyperplasia, pancreatic neuroendocrine tumors, and pituitary adenomas. Other recognized manifestations include carcinoid, cutaneous or adrenocortical tumors. It is commonly presented with clinical features related to parathyroid, pancreas or pituitary lesions. Here, we have presented a case ...
Parvez Yusuf - - 2013
Apparent mineralocorticoid excess (AME) syndrome is a rare autosomal recessive disorder due to the deficiency of 11b hydroxysteroid dehydrogenase type 2 enzyme (11beta-HSD2). Mutations in this gene affect the enzymatic activity resulting to an excess of cortisol, which causes its inappropriate access to mineralocorticoid receptor leading to inherited hypertension.This is ...
Trainer Peter J - - 2013
A number of drugs have been advocated for the medical management of Cushing's syndrome but few have gained widespread acceptance. The most reliably effective agents are metyrapone and ketoconazole as monotherapy, or in combination. Cabergoline may be of value in a minority of patients but pasireotide is a more reliable ...
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