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 ...

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 ...

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 ...

BACKGROUNDAIMS: 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 ...

Xist deletion causes female-specific myeloproliferative neoplasm/myelodysplastic syndrome.

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 ...

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 ...

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 ...

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 ...

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, ...

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 ...

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 ...

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 ...

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 ...

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 ...

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 ...

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. ...

Cushing's syndrome is due to excess cortisol secretion and is associated to increased mortality and severe morbidity that are not fully reversible despite biochemical control. The syndrome consists of a set of systemic manifestations similar to those found in aging. Chronic stress, which also causes hyperstimulation of the hypothalamic-pituitary-adrenal axis, ...

Although, there are several tests available, not one of them fulfils the criteria of being an ideal screening test. Continuing the search for an ideal screening test, we explored the use of urine spot cortisol-creatinine ratio as a novel method of evaluating patients with Cushing's syndrome. A total of 35 ...

Biochemical markers are applied in gastroenteropancreatic neuroendocrine tumours (GEP-NETs) for diagnostic, prognostic or predictive purposes. Chromogranin A is the most important general marker and it is recommended to be measured in every patient with a suspected NET, whereas Neuron Specific Enolase is elevated mainly in poorly differentiated NETs. Pancreatic Polypeptide ...

Allgrove's syndrome is a rare autosomal-recessive disorder with only about 70 cases reported thus far and is characterized by alacrima, achalasia, and ACTH insensitivity among other clinical features. However, it has a widely variable clinical presentation, which may result in such cases remaining undiagnosed. To report a patient with impending ...

Hypoparathyroidism is most commonly isolated and idiopathic. Hypoparathyroidism in autoimmune polyendocrine syndrome type-2 (APS-2) is extremely rare with few isolated reports and usually presents late. We present perhaps for the first time, hypoparathyroidism being the initial manifestation of APS-2 which was diagnosed in a 32 year lady with tingling of ...

A 60-year-old male with POEMS syndrome received lenalidomide and high-dose dexamethasone combination therapy as an initial treatment, with no severe adverse events occurring during the treatment. Two cycles of the therapy led to significantly decreased serum VEGF level, and IgA-λ type M-protein was not detected by immunofixation electrophoresis. We next ...

Adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome is caused by an ACTH-producing tumor, as is the case with Cushing's disease and ectopic ACTH syndrome (EAS). Diagnosis and differential diagnosis of Cushing's disease from EAS in ACTH-dependent Cushing's syndrome are thus challenging problems in clinical endocrinology. The diagnostic criteria for Cushing's disease in ...

Context:Hyperkalemia and weight loss are critical clinical problems for hemodialysis patients. There have been no documented reports of adrenal Cushing's syndrome with central obesity and hypokalemia in a hemodialysis patient.Objective:The aim of the study was to report a patient with Cushing's syndrome after chronic hemodialysis, review the published literature, and ...

Background: Cushing's syndrome due to ectopic adrenocorticotrophic hormone (ACTH) and/or ectopic corticotropic releasing hormone (CRH) secretion accounts for less than 10% of all Cushing's syndrome. Neuroendocrine carcinomas rarely cause Cushings's syndrome. These carcinomas have been found to secrete either ACTH or rarely CRH. Herein we report a case of neuroendocrine ...

Congenital adrenal hyperplasia (CAH) is characterized by decreased adrenal hormone production due to enzymatic defects and subsequent rise of adrenocorticotrophic hormone that stimulates the adrenal cortex to become hyperplastic, and sometimes tumorous. As the pathophysiology is basically a defect in the biosynthesis of cortisol, one may not consider CAH in ...

Described in this article is the massive enlargement of both adrenal glands in 3 newborns-2 girls and 1 boy. Two had hemihypertrophy and other congenital abnormalities but no identified genetic mutation; the third had genetically proven Beckwith-Wiedemann syndrome. Two had severe Cushing syndrome, the third had hypercortisolemia but no clinical ...

Ectopic ACTH syndrome is a rare disease often associated with severe hypercortisolism. When feasible, optimal management is surgical excision of the tumor. A 33-year-old male patient was admitted to the hospital in 1993 with clinical manifestations suggestive of Cushing's syndrome. He presented high plasma ACTH and markedly elevated urinary free ...

Cushing syndrome is characterized by truncal obesity, growth deceleration, skin changes, muscle weakness, and hypertension. Cushing syndrome in childhood usually results from the exogenous administration of glucocorticoids. This article presents the causes and discusses the treatment of endogenous Cushing syndrome. It also discusses the clinical and molecular genetics of inherited ...

Objective: Outline of the potential role for adrenal vein sampling in the diagnosis and management of ACTH-independent Cushing's syndrome with a case description and review of the literature.Methods: A 59-year-old English woman presented with an 8-year history of weight gain, centripetal obesity, a round plethoric face, skin thinning, easy bruising, ...

We describe a patient presenting with vertical one-and-a-half syndrome and concomitant contralesional horizontal gaze paresis as the result of a solitary neurocysticercosis (NCC) lesion in the right midbrain extending into the thalamomesencephalic junction. The patient received an albendazole-dexamethasone course which resulted in resolution of his symptoms. The neuro-ophthalmological complications of ...

Prader-Willi syndrome (PW) is a rare genetic disorder with multi-organ system involvement. These patients present many perioperative challenges including sleep-related breathing disorders, morbid obesity, thick salivary secretions, mental retardation, and difficult intravenous access. PW has been suggested to be associated with central adrenal insufficiency. We report a novel case of ...

We tested the efficacy of the Healthy Workplace programme for metabolic syndrome. A total of 226 workers with metabolic syndrome from 12 companies enrolled in the study and completed baseline and 6-month follow-up. The Healthy Workplace programme consisted of a 4-week structured education module, telephone counselling, mobile phone messages (SMS) ...

AIMS OF THE STUDY: Traditional Chinese medicine (TCM) has a long history and particular advantages in the diagnosis and treatment of type 2 diabetes mellitus (T2DM). Syndrome differentiation is the foundation and essence of TCM theories. The aims of the study are to discriminate T2DM corresponding to different syndromes (Qi-deficiency, ...

We describe the anaesthetic management of adrenalectomy in a patient with Cushing's syndrome due to adrenal mass with coexisting non-compaction cardiomyopathy. The problems due to hypersecretion of cortisol in Cushing's syndrome were compounded by the association of a rare form of genetic cardiomyopathy with very few guidelines regarding the perioperative ...

A 3-year-old boy presented with recurrent strokes and pallor. Hematological investigations revealed severe iron deficiency anemia without thrombocytosis. The magnetic resonance angiogragraphy findings were suggestive of moyamoya syndrome. The association of moyamoya syndrome with severe iron deficiency anemia has not been reported earlier. The likely pathophysiological mechanisms are discussed.

Polyglandular auto-immune syndromes are uncommon constellation of organ specific auto-immune diseases, characterised by the existence of two or more endocrinopathies. Polyglandular autoimmune type II syndrome also known as Schmidt's syndrome is more common, comprising Addison's disease, auto-immune thyroid disease, type 1 diabetes mellitus and/or hypogonadism, pernicious anaemia, coeliac disease, vitiligo, ...

Growing awareness that heart failure, renal impairment, and anaemia are frequent co-morbidities which can exacerbate one another in a vicious circle of clinical deterioration has led to the concept of the cardiorenal anaemia syndrome (CRAS). The role of iron deficiency within this complex interplay has been less well examined. Scrutiny ...

Abstract Background: The role of resistin in insulin sensitivity and metabolic syndrome has been controversial until now. Increased plasma/serum resistin levels are associated with metabolic syndrome and insulin resistance. The aim of this study was to investigate the relationship between serum resistin levels with markers of metabolic syndrome in males. ...

Abstract Background: The aim of the current study was to elucidate the clustering pattern of metabolic syndrome components along with apolipoproteins (Apo) A-I and B in diabetic and nondiabetic subjects. Methods: Factor analysis of conventional variables of metabolic syndrome [i.e., waist circumference, homeostasis model assessment of insulin resistance (HOMA-IR), triglycerides ...

Marfan syndrome is a multisystemic connective tissue disorder caused mainly by mutations in the fibrillin-1 gene. The entire cardiovascular system is affected in patients with Marfan syndrome. Aortic root dilatation, aortic valve regurgitation or - the most feared and life-threatening symptom - aortic root dissection are the most common manifestations. ...

BACKGROUND: Iron overload syndromes encompass a wide range of hereditary and acquired conditions. Major developments in the field of genetics and the discovery of hepcidin as a central regulator of iron homeostasis have greatly increased our understanding of the pathophysiology of iron overload syndromes. AIM: To review advances in iron ...

Metabolic syndrome (MetS) or "Syndrome X" which is a constellation of insulin resistance, hyperglycemia, hypertension, low high-density lipoprotein cholesterol (HDL-C), and increased very-low-density lipoprotein (VLDL) and triglyceride (TG) levels. It is one of the main threats for public health in the 21st century with its associated risk of cardiovascular disease. ...

Developing countries are undergoing an epidemiologic transition accompanied by increasing burden of cardiovascular disease (CVD) linked to urbanization and lifestyle modifications. Metabolic syndrome is a cluster of CVD risk factors whose extent in Kenya remains unknown. The aim of this study was to determine the prevalence of metabolic syndrome and ...

AIMS: Low serum 25-hydroxyvitamin D (25(OH)D) levels have been linked to metabolic syndrome. However, community-based data for healthy Korean individuals are lacking. We aimed to assess the vitamin D status and the association of 25(OH)D deficiency with metabolic syndrome in the South Korean population (latitude 33-38°N). METHODS: In this population-based ...

Acute lung injury and acute respiratory distress syndrome have been reported in a significant proportion of patients with critical neurologic illness. Our aim was to identify risk factors for acute lung injury/acute respiratory distress syndrome in this population. Prospective, observational study. A 22-bed, adult neurosciences critical care unit at a ...

Hypercortisolism is a common condition in dogs and can be defined as the physical and biochemical changes that result from prolonged exposure to inappropriately high plasma concentrations of (free) cortisol, whatever its' cause. This disorder is often called Cushing's syndrome, after Harvey Cushing, the neurosurgeon who first described the human ...

It has been suggested previously that rebound hypercoagulability may be responsible for morbidity and mortality following clopidogrel cessation in adults with acute coronary syndrome. We report a case of acute occlusion of a modified Blalock-Taussig shunt in an infant after clopidogrel discontinuation.

The pull-through technique is an interventional radiological procedure used when an occluded lesion cannot be traversed from one direction. To pass the lesion, a long guidewire is traversed from the opposite side and pulled through the ipsilateral sheath using a snare wire. The present report describes a case of severe ...