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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 ...
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 ...
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 ...
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 ...
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 ...
Oh Jee-Young JY Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, Korea. - - 2013
The existence of an association between thyrotropin (thyroid stimulating hormone, TSH) levels and metabolic derangement in euthyroid subjects is controversial. We examined the association between high normal TSH levels and metabolic syndrome in healthy young women. The study recruited 2,760 young female volunteers (age, 18 to 39 years) with TSH ...
Wallace Kedra - - 2013
INTRODUCTION: Administration of dexamethasone to HELLP syndrome patients (10mg i.v. every 12hours) shortens disease course and reduces maternal morbidity, in patients treated at the University of Mississippi Medical Center (UMMC), associated with this severe form of preeclampsia. However, the pathophysiologic mechanisms involved with this intervention remain unclear. OBJECTIVE: We sought ...
Harisankar Chidambaram Natrajan Balasubramanian - - 2013
ABSTRACT: Marine-Lenhart syndrome has been described as a variant of Graves disease with the following criteria: (1) the thyroid scan shows an enlarged gland and 1 or 2 poorly functioning nodules; (2) the nodule is TSH dependent and the paranodular tissue is TSH independent; (3) after endogenous or exogenous TSH ...
Namburu Rajendra Prasad - - 2013
A high potency, long acting and/or the extended use of oral corticosteroids, particularly in children, may cause suppression of the hypothalamo-pituitary-adrenal axis. However, the iatrogenic Cushing's syndrome in the infantile age group is rare and only few patients have been reported to date in the literature. Here, we are reporting ...
De Leersnyder H - - 2013
Smith-Magenis syndrome (SMS) is a mental retardation syndrome with distinctive behavioral characteristics, dysmorphic features, and congenital anomalies ascribed to an interstitial deletion of chromosome 17p11.2. Severe sleep disturbances and maladaptive daytime behavior have been linked to an abnormal circadian rhythm of melatonin with a diurnal instead of nocturnal secretion of ...
Charmandari Evangelia - - 2013
Familial or sporadic primary generalized glucocorticoid resistance or Chrousos syndrome is a rare genetic condition characterized by generalized, partial, target-tissue insensitivity to glucocorticoids and a consequent hyperactivation of the hypothalamic-pituitary-adrenal (HPA) axis. Primary generalized glucocorticoid hypersensitivity (PGGH) represents the mirror image of the former, and is characterized by generalized, partial, ...
Zhang Da-Ming - - 2013
THE causes of Cushing's syndrome are mainly divided into adrenocorticotropic hormone (ACTH) dependent and independent. ACTH dependent hypercortisolism represents excess ACTH se-creting by the pituitary or tumor outside the pituitary; and the latter one is also called as ectopic ACTH syndrome. Thorax is the most common location of causative lesions ...
Kannan Subramanian - - 2013
Adrenal incidentalomas are commonly encountered in this era of ubiquitous imaging. The attenuation of the incidentaloma measured in Hounsfield units (HU) is an important step in the work up. Attenuation less than 10 HU indicates a benign lesion in more than 98% of cases, whereas attenuation greater than 30 HU is highly ...
Sofka Sarah - - 2013
Cushing's Syndrome is an endocrine condition with complex diagnostic pathways. Cortisol suppression from high dose dexamethasone usually points to the pituitary as the cause. We present the case of a patient with dexamethasone suppressible Cushing's Syndrome from a bronchopulmonary carcinoid tumor. The tumor was only able to be localized with ...
Hossain Muhammad Rajib - - 2013
Adrenocortical tumors (ACTs) causing Cushing's syndrome are extremely rare in children and adolescents. Bilateral macronodular adrenocortical disease which is a component of the McCune-Albright syndrome is the most common cause of endogenous Cushing's syndrome. We report the case of a boy with Cushing's syndrome who presented with obesity and growth ...
Giugni Aldo Schenone - - 2013
Exophthalmos is typically associated with Graves' ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing's syndrome. We present a case of a 38-year-old female who presented with severe bilateral proptosis and was subsequently diagnosed with Cushings disease. We discuss the possible mechanisms causing exophthalmos in ...
Espiard Stéphanie - - 2013
Carney complex is a rare, dominantly inherited multiple endocrine neoplasia syndrome, affecting endocrine glands as the adrenal cortex (causing Cushing's syndrome), the pituitary and the thyroid. It is associated with many other nonendocrine tumors, including cardiac myxomas, testicular tumors, melanotic schwannoma, breast myxomatosis, and abnormal pigmentation (lentiginosis) or myxomas of ...
Scherer Thomas - - 2013
Marine-Lenhart syndrome is defined as the co-occurrence of Graves' disease and functional nodules. The vast majority of autonomous adenomas are benign, whereas functional thyroid carcinomas are considered to be rare. Here, we describe a case of simultaneous occurrence of Marine-Lenhart syndrome and a papillary microcarcinoma embedded in a functional nodule. ...
von Stempel Conrad - - 2013
A 79-year-old woman, a smoker, presented to the emergency department with a 2-week history of progressive dyspnoea and peripheral oedema, preceded by flu-like symptoms that had failed to improve despite antibiotics. Examination identified severe hypertension and signs consistent with severe fluid overload. Baseline chest X ray showed a right-sided abnormality. ...
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