12,904 results match your criteria Cushing Syndrome


Hyperglycemia and Hypokalemia in a 16-Year-Old Overweight Female Patient Misdiagnosed with Cushing Syndrome at First and Ultimately Diagnosed with Carney Complex Proven by PRKAR1A Gene Test: a Case Report and Literature Review.

Clin Lab 2019 Mar;65(3)

Background: Here we report on a 16-year-old female patient with typical Cushingoid features who was admitted because of purple striae, menostasis, and microsomia for 1 year, and laboratory tests showed hyperglycemia and hypokalemia.

Methods: For diagnosis, we employed a hormone test, abdominal and pituitary computed tomography scan, ultrasonography to detect endocrine and cardiocutaneous lesions. DNA sequencing to detect PRKAR1A gene mutation to make differential diagnosis for Cushing Syndrome. Read More

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http://dx.doi.org/10.7754/Clin.Lab.2018.180805DOI Listing
March 2019
1 Read

The Utrecht Score: A Novel Histopathological Scoring System to Assess the Prognosis of Dogs with Cortisol-Secreting Adrenocortical Tumours.

Vet Comp Oncol 2019 Mar 12. Epub 2019 Mar 12.

Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Utrecht, the Netherlands.

A cortisol-secreting adrenocortical tumour (ACT) is the cause of naturally occurring canine hypercortisolism in approximately 15-20% of cases. The differentiation between an adrenocortical adenoma and carcinoma is usually based on histopathology. However, histopathological parameters have never been linked to the dogs' survival. Read More

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http://dx.doi.org/10.1111/vco.12474DOI Listing

Cyclic Subclinical Hypercortisolism: A Previously Unidentified Hypersecretory Form of Adrenal Incidentalomas.

J Endocr Soc 2019 Mar 11;3(3):678-686. Epub 2019 Feb 11.

Division of Endocrinology, Department of Medicine, Universidade Federal de São Paulo (UNIFESP) Medical School, São Paulo, SP, Brazil.

Purpose: Most adrenal incidentalomas (AIs) are nonfunctioning adenomas (NFAs), but up to 30% may secrete cortisol autonomously without clinical evidence of Cushing syndrome (CS), which nevertheless may increase cardiovascular mortality. This subclinical hypercortisolism (SCH) is confirmed by cortisol resistance to a dexamethasone suppression test (DST). Cyclic cortisol secretion occurs in classic CS but was not reported in SCH. Read More

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http://dx.doi.org/10.1210/js.2018-00385DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399707PMC

Minimally Invasive Surgery for Primary and Metastatic Adrenal Malignancy.

Surg Oncol Clin N Am 2019 04 11;28(2):309-326. Epub 2019 Jan 11.

Department of Surgical Oncology, MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.

Since the first description of laparoscopic adrenalectomy (LA) for pheochromocytoma and Cushing syndrome in 1992, the utilization of and indications for a minimally invasive approach to the adrenal gland have vastly expanded. Although minimally invasive adrenalectomy has been established as the preferred approach for patients with benign tumors of the adrenal gland, minimally invasive adrenalectomy for cancer remains controversial. In this article, the authors review the indications for minimally invasive adrenalectomy for adrenal nodules suspicious for, or established to represent, a primary malignancy or a site of metastatic cancer. Read More

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http://dx.doi.org/10.1016/j.soc.2018.11.011DOI Listing
April 2019
2 Reads

Autonomous cortisol secretion in adrenal incidentalomas.

Endocrine 2019 Mar 7. Epub 2019 Mar 7.

Department of Endocrinology, Hospital Universitario La Princesa, Instituto de Investigación Princesa, Madrid, Spain.

Adrenal incidentalomas (AI) are one of the most frequent reasons for consultation in Endocrinology, as they are present in 3-10% of the general population. Up to 20% of them may have autonomous cortisol secretion (ACS), a term that refers to AI carriers with biochemical evidence of excess cortisol, but without the "specific" clinical signs of Cushing's syndrome. As ACS is associated with an increased risk of diabetes, obesity, high blood pressure (HBP), osteoporosis, cardiovascular events, and global mortality; its correct identification is of great importance. Read More

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http://dx.doi.org/10.1007/s12020-019-01888-yDOI Listing
March 2019
1 Read

A comprehensive diagnostic approach to detect underlying causes of obesity in adults.

Obes Rev 2019 Mar 1. Epub 2019 Mar 1.

Obesity Center CGG, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.

Obesity is a worldwide growing problem. When confronted with obesity, many health care providers focus on direct treatment of the consequences of adiposity. We plead for adequate diagnostics first, followed by an individualized treatment. Read More

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http://dx.doi.org/10.1111/obr.12836DOI Listing
March 2019
2 Reads

[Differences of brain functional alterations between subtypes of Cushing's syndrome patients].

Zhonghua Yi Xue Za Zhi 2019 Feb;99(8):593-598

Department of Endocrinology, Chinese PLA General Hospital, Key Laboratory of Endocrinology and Metabolism of PLA, Beijing 100853, China.

To compare the differences of brain functional damage of subtypes of patients with Cushing's syndrome (CS). A total of 11 adrenocorticotropic hormone (ACTH)-dependent CS patients and 29 ACTH-independent CS patients were recruited from Chinese PLA General Hospital between September 2015 and March 2017 with confirmed CS. The psychiatric scales and brain task functional magnetic resonance imaging (fMRI) were evaluated. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2019.08.006DOI Listing
February 2019
1 Read

THE EVALUATION OF INCIDENTALLY DISCOVERED ADRENAL MASSES.

Endocr Pract 2019 Feb;25(2):178-192

Objective: The objective of this Disease State Clinical Review is to provide clinicians with a practical approach to the evaluation of incidentally discovered adrenal masses.

Methods: A case-based clinical approach to the evaluation of adrenal masses is presented. Recommendations were developed using available prospective and randomized studies, cohort studies, cross-sectional studies, anecdotal observations, and expert opinions. Read More

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http://dx.doi.org/10.4158/DSCR-2018-0565DOI Listing
February 2019
1 Read

A unique case of ectopic Cushing's syndrome from a thymic neuroendocrine carcinoma.

Endocrinol Diabetes Metab Case Rep 2019 Feb 22;2019. Epub 2019 Feb 22.

Departments of Endocrinology, Diabetes & Metabolism, Cleveland Clinic, Cleveland, Ohio, USA.

Ectopic adrenocorticotropic hormone (ACTH) production leading to ectopic ACTH syndrome accounts for a small proportion of all Cushing's syndrome (CS) cases. Thymic neuroendocrine tumors are rare neoplasms that may secrete ACTH leading to rapid development of hypercortisolism causing electrolyte and metabolic abnormalities, uncontrolled hypertension and an increased risk for opportunistic infections. We present a unique case of a patient who presented with a mediastinal mass, revealed to be an ACTH-secreting thymic neuroendocrine tumor (NET) causing ectopic CS. Read More

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http://dx.doi.org/10.1530/EDM-19-0002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6391898PMC
February 2019

Severe Hypercortisolism with Hypokalemic Alkalosis Mimicking Ectopic Cushing Syndrome in a Patient with Cushing Disease Due to Pituitary Microadenoma.

Indian J Endocrinol Metab 2018 Nov-Dec;22(6):860-863

Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, India.

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http://dx.doi.org/10.4103/ijem.IJEM_581_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330873PMC
February 2019
1 Read

Bone Health in Patients with Cushing's Syndrome.

Indian J Endocrinol Metab 2018 Nov-Dec;22(6):766-769

Department of Endocrinology, AIIMS, New Delhi, India.

Introduction: Osteoporosis is a well-recognized complication of Cushing's syndrome (CS). Data on bone health in patients with CS from south Asian countries, which are vitamin D deficient, are scarce.

Aims: We assessed bone mineral density (BMD) in patients with CS in comparison to controls. Read More

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http://dx.doi.org/10.4103/ijem.IJEM_160_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330848PMC
February 2019
4 Reads

Hypercoagulability and Risk of Venous Thromboembolic Events in Endogenous Cushing's Syndrome: A Systematic Meta-Analysis.

Front Endocrinol (Lausanne) 2018 28;9:805. Epub 2019 Jan 28.

Northwest Pituitary Center, Oregon Health & Science University, Portland, OR, United States.

Hypercortisolism has been implicated in the development of venous thromboembolic events (VTE). We aimed to characterize VTE risk in endogenous Cushing's syndrome (CS) patients, compare that risk to other pathologies, and determine if there are any associated coagulation factor changes. Medline and Scopus search for "hypercortisolism" and "thromboembolic disease" from January 1980 to April 2017 to include studies that reported VTE rates and/or coagulation profile of CS patients. Read More

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http://dx.doi.org/10.3389/fendo.2018.00805DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360168PMC
January 2019
1 Read

Exacerbation of Cushing's syndrome during pregnancy: stimulation of a cortisol-secreting adrenocortical adenoma by ACTH originating from the foeto-placental unit.

Endocrinol Diabetes Metab Case Rep 2019 Feb 7;2019. Epub 2019 Feb 7.

A 29-year-old G4A3 woman presented at 25 weeks of pregnancy with progressive signs of Cushing's syndrome (CS), gestational diabetes requiring insulin and hypertension. A 3.4 × 3. Read More

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http://dx.doi.org/10.1530/EDM-18-0115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6373782PMC
February 2019
3 Reads

Adrenocorticotropic Hormone-Dependent Cushing Syndrome Caused by an Olfactory Neuroblastoma.

Clin Med Insights Endocrinol Diabetes 2019 31;12:1179551419825832. Epub 2019 Jan 31.

Department of Endocrinology, Hospital Clinico San Carlos, Madrid, Spain.

Olfactory neuroblastoma (ONB) is an unusual malignant neoplasm originating from the olfactory neuroepithelium. Secretion of adrenocorticotropic hormone (ACTH) from this tumor has been exceptionally reported. We describe a young man with resistant hypertension and a cushingoid phenotype. Read More

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http://dx.doi.org/10.1177/1179551419825832DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357293PMC
January 2019
1 Read

Minimally invasive approach to the adrenal gland in obese patients with Cushing's syndrome.

Minim Invasive Ther Allied Technol 2019 Jan 19:1-7. Epub 2019 Jan 19.

a Clinica Chirurgica , Universita Politecnica delle Marche , Ancona , Italy.

Introduction: The combination of Cushing's syndrome and obesity, one of the most common manifestations of the syndrome itself, may be associated with the worsening of post-operative outcomes in case of laparoscopic adrenalectomy. This study focused on the laparoscopic treatment of Cushing's syndrome with the purpose to identify any relevant difference between morbidly obese, mildly obese and non-obese patients.

Material And Methods: Patients were retrospectively divided into three groups according to their Body Mass Index (BMI). Read More

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http://dx.doi.org/10.1080/13645706.2018.1536669DOI Listing
January 2019
3 Reads

Is VTE Prophylaxis Necessary on Discharge for Patients Undergoing Adrenalectomy for Cushing Syndrome?

J Endocr Soc 2019 Feb 12;3(2):304-313. Epub 2018 Dec 12.

Endocrine Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.

Background: Patients with Cushing syndrome (CS) have an increased risk for venous thromboembolism (VTE). However, it is unclear whether patients undergoing adrenalectomy for CS are at increased risk for postoperative VTE. The aim of this study was to determine the rate of postoperative VTE in patients undergoing adrenalectomy for CS. Read More

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https://academic.oup.com/jes/article/3/2/304/5239843
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http://dx.doi.org/10.1210/js.2018-00278DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330172PMC
February 2019
6 Reads

Adipocyte GR Inhibits Healthy Adipose Expansion Through Multiple Mechanisms in Cushing Syndrome.

Endocrinology 2019 Mar 1;160(3):504-521. Epub 2019 Mar 1.

Department of Metabolic Medicine, Osaka University Graduate School of Medicine, Osaka, Japan.

In Cushing syndrome, excessive glucocorticoids lead to metabolic disturbances, such as insulin resistance, adipocyte hypertrophy, and liver steatosis. In vitro experiments have highlighted the importance of adipocyte glucocorticoid receptor (GR), but its metabolic roles in vivo have not been fully elucidated in Cushing syndrome. In this study, using clinical samples from patients with Cushing syndrome and adipocyte-specific GR knockout (AGRKO) mice, we investigated the roles of adipocyte GR and its clinical relevance in Cushing syndrome. Read More

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http://dx.doi.org/10.1210/en.2018-01029DOI Listing
March 2019
1 Read

Local hyperthermia cleared multifarious viral warts in a patient with Cushing's syndrome.

Dermatol Ther 2019 Jan 11:e12815. Epub 2019 Jan 11.

Department of dermatology, NHC/Ministry of Education/Liaoning Province Key Laboratory of Immunodermatology (China Medical University) The First Hospital of China Medical University, No. 155 Nanjing Bei Street, Heping District, Shenyang, Liaoning, P.R. China.

A female Cushing's syndrome patient had been suffering from extensive viral warts for months. She was diagnosed with flat warts, common warts and plantar warts. The plantar warts on her right foot were initially treated using local hyperthermia at 44°C for 30 min according to a defined protocol, followed by treatment targeting a common wart on her left thumb. Read More

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http://dx.doi.org/10.1111/dth.12815DOI Listing
January 2019
6 Reads

Three Novel MEN1 Variants in AIP-Negative Familial Isolated Pituitary Adenoma Patients.

Pathobiology 2019 Jan 10:1-7. Epub 2019 Jan 10.

Department of Genetics, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey.

Objectives: Pituitary adenomas (PAs) may rarely occur in well-defined hereditary conditions, like multiple endocrine neoplasia type 1 (MEN1) syndrome and familial isolated pituitary adenoma (FIPA) associated with germline mutations in MEN1 and AIP, respectively. This study aimed to assess MEN1 genetic abnormalities in AIP mutation-negative FIPA patients, not associated with MEN1 components.

Methods: Among 20 patients evaluated in 13 FIPA families, 12 were previously reported as AIP mutation-negative. Read More

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http://dx.doi.org/10.1159/000495252DOI Listing
January 2019
3 Reads

Hepatic Lesions Associated with McCune Albright Syndrome.

J Pediatr Gastroenterol Nutr 2019 Jan 4. Epub 2019 Jan 4.

Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA.

McCune Albright Syndrome (MAS) results from a GNAS gene mutation. It is associated with café-au-lait macules, fibrous dysplasia, and several endocrinopathies to include gonadotropin-independent precocious puberty, growth hormone excess, Cushing syndrome, thyroid disease, and renal phosphate wasting. It is recognised to be a rare cause of neonatal cholestasis. Read More

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http://dx.doi.org/10.1097/MPG.0000000000002266DOI Listing
January 2019
2 Reads

Alterations in Protein Kinase A Substrate Specificity as a Potential Cause of Cushing Syndrome.

Endocrinology 2019 Feb;160(2):447-459

Institute of Pharmacology and Toxicology and Bio-Imaging Center, University of Würzburg, Würzburg, Germany.

Cushing syndrome is a severe endocrine disorder of cortisol excess associated with major metabolic and cardiovascular sequelae. We recently identified somatic mutations in PRKACA, the gene encoding the catalytic (C) α subunit of protein kinase A (PKA), as being responsible for cortisol-producing adrenocortical adenomas (CPAs), which are a major cause of Cushing syndrome. In spite of previous studies on the two initially identified mutations (L206R, 199_200insW), the mechanisms of action of the clinically highly relevant PRKACA mutations remain poorly understood. Read More

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http://dx.doi.org/10.1210/en.2018-00775DOI Listing
February 2019
16 Reads

Optical Imaging Technology: A Useful Tool to Identify Remission in Cushing Disease After Surgery.

Horm Metab Res 2019 Feb 2;51(2):120-126. Epub 2019 Jan 2.

Section on Endocrinology and Genetics Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA.

We recently reported the use of optical imaging technology to quantify facial plethora in endogenous Cushing syndrome (CS). In the present study, we studied a larger cohort of patients with Cushing disease (CD) and examined water content fraction as well as blood volume fraction as bio-optic markers for determining the efficacy of this methodology as a predictor of lasting remission after surgery for CS. We imaged 49 patients before and after transsphenoidal surgery (TSS) for Cushing disease (CD); 22 patients were also seen at 3-6 months, and 13 patients 12 months post-operatively. Read More

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http://dx.doi.org/10.1055/a-0801-8917DOI Listing
February 2019
11 Reads

Unilateral Adrenalectomy Could Be a Valid Option for Primary Nodular Adrenal Disease: Evidence From Twins.

J Endocr Soc 2019 Jan 24;3(1):129-134. Epub 2018 Oct 24.

Division of Endocrinology, Hôpital Erasme, Brussels, Belgium.

Primary pigmented nodular adrenal disease (PPNAD) accounts for <1% of ACTH-independent Cushing syndrome. We describe the case of twin female patients with PPNAD who both had sustainable disease control after unilateral adrenalectomy, which corroborates current evidence in favor of unilateral adrenalectomy for a subset of patients with PPNAD. Patient A presented with a 10-kg weight gain over the past year and facial plethora. Read More

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http://dx.doi.org/10.1210/js.2018-00261DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302904PMC
January 2019
1 Read

Avascular necrosis of the femoral head: An exceptional complication of cushing's disease.

Ann Afr Med 2018 Oct-Dec;17(4):225-227

Department of Endocrinology and Diabetology, University Hospital of Fez, Fez, Morocco.

Avascular necrosis (AVN) of the femoral head is a well-recognized complication of steroid treatment. Exogenous glucocorticoids' administration and alcoholism are the most common nontraumatic etiologies. AVN secondary to endogenous hypercortisolism (HC) is rare; very few case reports are available on this complication. Read More

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http://dx.doi.org/10.4103/aam.aam_75_17DOI Listing
December 2018
10 Reads

Development of Iatrogenic Cushing Syndrome After Peri-articular Glucocorticoid Injection in an Human Immunodeficiency Virus Patient on Protease Inhibitor Therapy.

J Clin Rheumatol 2018 Dec 5. Epub 2018 Dec 5.

Rheumatology Department Ramón y Cajal University, Hospital Madrid, Spain Rheumatology Department Ramón y Cajal University Hospital Madrid, Spain. Infectious Diseases Department Ramón y Cajal University Hospital Madrid, Spain.

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http://dx.doi.org/10.1097/RHU.0000000000000940DOI Listing
December 2018
1 Read

Peripheral primitive neuroectodermal tumor associated with paraneoplastic Cushing's syndrome: The rare case.

Ann Med Surg (Lond) 2019 Jan 29;37:21-24. Epub 2018 Nov 29.

Iran University of Medical Science, Tehran, Iran.

Introduction: Primitive neuroectodermal tumors (PNET) form a group of tumors defined by their appearance that are thought to develop from primitive (undifferentiated) nerve cells in the brain. They are rare tumors and their incidence is not well defined.

Case Presentation: An 18-month-old male presenting with typical Cushingoid appearance (moon face, central obesity, hirsutism and growth arrest) was admitted for evaluation of endocrine problems. Read More

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http://dx.doi.org/10.1016/j.amsu.2018.11.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287080PMC
January 2019
1 Read

Drug design strategies for Cushing's syndrome.

Expert Opin Drug Discov 2019 Feb 20;14(2):143-151. Epub 2018 Dec 20.

a Institute of Bioorganic Chemistry of the National Academy of Science of Belarus , Minsk , Republic of Belarus.

Introduction: Cushing's syndrome (CS) is a metabolic disorder caused by chronic hypercortisolism. CS is associated with cardiovascular, metabolic, skeletal and psychological dysfunctions and can be fatal if left untreated. The first-line treatment for all forms of CS is a surgery. Read More

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http://dx.doi.org/10.1080/17460441.2019.1559146DOI Listing
February 2019
6 Reads

[Cushing's syndrome during pregnancy : diagnostic and therapeutic difficulties].

Rev Med Liege 2018 Dec;73(12):603-609

Service d`Endocrinologie, CHU de Liège, Liège, Belgique.

Cushing's syndrome (CS), which is often associated with infertility, exceptionally occurs in pregnancy, and markedly increases maternal and fetal morbidity and mortality. Gestational CS may be challenging. Indeed, symptoms of hypercorticism may overlap with physiological hyperactivity of the hypothalamus-pituitary-adrenal axis in normal pregnancy. Read More

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December 2018
3 Reads

A challenging case of laparoscopic synchronous bilateral adrenalectomy for Cushing's disease. Case report.

Ann Med Surg (Lond) 2018 Dec 8;36:261-263. Epub 2018 Sep 8.

University-Hospital of Parma, Department of Urology, Via Gramsci 14, Parma, 43126, Italy.

Introduction: Laparoscopic adrenalectomy is a treatment option in patients with Cushing's syndrome. Preoperative comorbities as well as surgical and anesthesiological difficulties can make the procedure challenging.

Presentation Of The Case: We present the case of a 53-year-old obese man diagnosed with Cushing's syndrome, also suffering from other endocrine pathologies, neurofibromatosis type 2, cardiomiopathy with severe hypertrophy and diastolic dysfunction, deep vein thrombosis (DVT) and obstructive sleep apnea syndrome (OSAS). Read More

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http://dx.doi.org/10.1016/j.amsu.2018.07.015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287572PMC
December 2018
1 Read

Continuous Etomidate Infusion for the Management of Severe Cushing Syndrome: Validation of a Standard Protocol.

J Endocr Soc 2019 Jan 26;3(1):1-12. Epub 2018 Oct 26.

Endocrine Center and Clinics, Froedtert Hospital and the Medical College of Wisconsin, Menomonee Falls, Wisconsin.

Objective: Demonstrate the safety and efficacy of a standardized intravenous etomidate infusion protocol in normalizing cortisol levels in patients with severe and life-threatening hypercortisolism.

Methods: A retrospective case series of seven patients representing nine episodes of severe hypercortisolism at two large academic medical centers was conducted. Patients were included in this series if they received an etomidate infusion for the treatment of severe and life-threatening hypercortisolism. Read More

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http://dx.doi.org/10.1210/js.2018-00269DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6291660PMC
January 2019
1 Read

An ectopic adrenocortical adenoma in renal hilum presenting with Cushing's syndrome: A case report and literature review.

Medicine (Baltimore) 2018 Dec;97(50):e13322

Department of Endocrinology, Peking University First Hospital, China.

Rationale: Ectopic adrenal tissue is the adrenal rests along the path from gonads to adrenal glands during embryogenesis. Ectopic adrenocortical adenoma is a rare disease represented with over-production of cortisol by the ectopic adrenocortical tissue.

Patient Concerns: An 18-year-old Chinese female patient was presented with weight-gain for 6 months. Read More

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http://dx.doi.org/10.1097/MD.0000000000013322DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319990PMC
December 2018
3 Reads

An update on the genetics of benign pituitary adenomas in children and adolescents.

Curr Opin Endocr Metab Res 2018 Aug 24;1:19-24. Epub 2018 Apr 24.

Section on Endocrinology & Genetics (SEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD, 20892, USA.

Pituitary adenomas in children and adolescents are rare tumors that often result from a tumor predisposition syndrome. Several inherited causes for pituitary adenomas have been identified in the last few years, including multiple endocrine neoplasia type 1 and 4, Carney's complex, Tuberous sclerosis, DICER1 syndrome, neurofibromatosis type 1, McCune Albright syndrome, familial isolated pituitary adenoma, and pituitary adenoma association due to defects in succinate dehydrogenase genes. Recently, our group discovered X-linked acrogigantism (X-LAG), a new pediatric disorder that is caused by an Xq26. Read More

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http://dx.doi.org/10.1016/j.coemr.2018.04.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289528PMC
August 2018
3 Reads

Concurrent pituitary and adrenocortical lesions on computed tomography imaging in dogs with spontaneous hypercortisolism.

J Vet Intern Med 2019 Jan 11;33(1):72-78. Epub 2018 Dec 11.

Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Utrecht, The Netherlands.

Background: Spontaneous hypercortisolism or Cushing's syndrome in dogs is either pituitary or adrenal dependent, but concurrent pituitary and adrenal hypercortisolism also has been reported.

Objective: To determine how often concurrent pituitary and adrenal lesions are present in dogs with spontaneous hypercortisolism.

Animals: Two hundred one client-owned dogs with spontaneous hypercortisolism. Read More

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http://dx.doi.org/10.1111/jvim.15378DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335443PMC
January 2019
2 Reads

The coexistence of Cushing syndrome and gynecomastia as the manifestations of adrenocortical carcinoma.

Kaohsiung J Med Sci 2018 12 4;34(12):705-706. Epub 2018 Jul 4.

Division of Endocrinology and Metabolism, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand; Excellence Center in Diabetes, Hormones and Metabolism, King Chulalongkorn Memorial Hospital, Bangkok, Thailand. Electronic address:

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http://dx.doi.org/10.1016/j.kjms.2018.06.004DOI Listing
December 2018
2 Reads
0.805 Impact Factor

Carcinoid tumors of the thymus and Cushing's syndrome: Clinicopathologic features and current best evidence regarding the cell of origin of these unusual neoplasms.

Ann Diagn Pathol 2019 Feb 22;38:71-79. Epub 2018 Nov 22.

Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, United States of America.

It is uncertain whether thymic neuroendocrine tumors (NET) associated with Cushing's syndrome (CS) produce corticotropin-releasing hormone (CRH) and adrenocorticotropin hormone (ACTH) and whether the thymus contains ACTH and/or CRH cells that could originate NET. The clinicopathologic features of 5 typical (TC) and 6 atypical carcinoids (ATC), 10 additional non-neoplastic thymi, 6 adrenal glands with bilateral nodular hyperplasia and 8 adrenal cortical adenomas were reviewed. Representative slides were immunostained for ACTH and CRH. Read More

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http://dx.doi.org/10.1016/j.anndiagpath.2018.11.006DOI Listing
February 2019
4 Reads

Tisagenlecleucel in Adult Relapsed or Refractory Diffuse Large B-Cell Lymphoma.

N Engl J Med 2019 01 1;380(1):45-56. Epub 2018 Dec 1.

From the Lymphoma Program, Abramson Cancer Center, University of Pennsylvania, Philadelphia (S.J.S.); the Hematopoietic Cellular Therapy Program, University of Chicago Medicine, Chicago (M.R.B.); Peter MacCallum Cancer Centre, St. Vincent's Hospital and University of Melbourne, Melbourne, VIC (C.S.T.), and the Royal Prince Alfred Hospital and Department of Medicine, University of Sydney, Sydney (P.J.H.) - both in Australia; Winship Cancer Institute of Emory University, Bone Marrow and Stem Cell Transplant Center, Atlanta (E.K.W.); the Department of Hematology and Oncology, University Hospital of Cologne, Cologne (P.B.), and the Würzburg University Medical Center, Center for Allogeneic Stem Cell Transplantation, Würzburg (S.M.) - both in Germany; the Division of Hematologic Malignancies and Cellular Therapeutics, University of Kansas Cancer Center, Kansas City (J.P.M.); the Department of Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna (U.J.); James Cancer Hospital and Solove Research Institute, Ohio State University Comprehensive Cancer Center, Columbus (S.J.); the Department of Hematology and Blood and Marrow Transplant, University of California, San Francisco, Helen Diller Family Comprehensive Cancer Center, San Francisco (C.A.); the Department of Lymphoma and Myeloma, Division of Cancer Medicine, University of Texas M.D. Anderson Cancer Center, Houston (J.R.W.); Maisonneuve-Rosemont Hospital, University of Montreal, Montreal (I.F.), and the Juravinski Hospital and Cancer Centre, McMaster University, Hamilton, ON (S.R.F.) - both in Canada; the Division of Hematology, Oncology, and Transplantation, University of Minnesota, Minneapolis (V.B.); Karolinska Institutet and University Hospital, Department of Laboratory Medicine/Department of Cell Therapy and Allogeneic Stem Cell Transplantation, Stockholm (S.M.); University of Michigan Comprehensive Cancer Center, Ann Arbor (J.M.M.); the Department of Oncology, Oslo University Hospital, Oslo (H.H.); Novartis Pharma, Basel, Switzerland (S.P., O.A.); Novartis Pharmaceuticals (L.B.P., J.C.) and Novartis Institutes for BioMedical Research (R.A.), East Hanover, NJ; the Department of Hematology, Hospices Civils de Lyon, Université de Lyon, Lyon, France (G.S.); and the Center for Hematologic Malignancies, Oregon Health and Science University Knight Cancer Institute, Portland (R.T.M.).

Background: Patients with diffuse large B-cell lymphoma that is refractory to primary and second-line therapies or that has relapsed after stem-cell transplantation have a poor prognosis. The chimeric antigen receptor (CAR) T-cell therapy tisagenlecleucel targets and eliminates CD19-expressing B cells and showed efficacy against B-cell lymphomas in a single-center, phase 2a study.

Methods: We conducted an international, phase 2, pivotal study of centrally manufactured tisagenlecleucel involving adult patients with relapsed or refractory diffuse large B-cell lymphoma who were ineligible for or had disease progression after autologous hematopoietic stem-cell transplantation. Read More

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http://www.nejm.org/doi/10.1056/NEJMoa1804980
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http://dx.doi.org/10.1056/NEJMoa1804980DOI Listing
January 2019
22 Reads

[Pediatric pancreoblastoma with Cushing syndrome].

Authors:
L Song G Y Wang H Pan

Zhonghua Er Ke Za Zhi 2018 Dec;56(12):952-953

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2018.12.013DOI Listing
December 2018
2 Reads

Neurosurgical treatment of Cushing disease in pediatric patients: case series and review of literature.

Childs Nerv Syst 2018 Nov 28. Epub 2018 Nov 28.

Pediatric Endocrinology and Adolescent Divisions, Department of Pediatrics, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey.

Aim: Pituitary adenomas are rare in childhood in contrast with adults. Adrenocorticotropic hormone (ACTH)-secreting adenomas account for Cushing's disease (CD) which is the most common form of ACTH-dependent Cushing's syndrome (CS). Treatment strategies are generally based on data of adult CD patients, although some difficulties and differences exist in pediatric patients. Read More

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http://dx.doi.org/10.1007/s00381-018-4013-5DOI Listing
November 2018
3 Reads

Hormonal aggressiveness according to the expression of cellular markers in corticotroph adenomas.

Endocrine 2018 Nov 24. Epub 2018 Nov 24.

Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea.

Purpose: The molecular mechanisms underlying tumor growth in Cushing's disease (CD) still remain a challenge. Moreover, clinical manifestations of CD may vary depending on hormonal activity; however, factors involved in the hormonal aggressiveness of adrenocorticotropic hormone (ACTH)-secreting pituitary tumors have not been fully clarified. We investigated the association between the expression of cellular markers regarding pituitary tumor progression and initial or postoperative hormone levels in patients with CD. Read More

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http://dx.doi.org/10.1007/s12020-018-1815-xDOI Listing
November 2018
2 Reads

An update on adrenal endocrinology: significant discoveries in the last 10 years and where the field is heading in the next decade.

Hormones (Athens) 2018 Dec 19;17(4):479-490. Epub 2018 Nov 19.

Section on Endocrinology and Genetics & Inter-Institute Endocrinology Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), NIH-Clinical Research Center, 10 Center Drive, Building 10, Room 1-3330, MSC1103, Bethesda, MD, 20892, USA.

The last 10 years have produced an amazing number of significant discoveries in the field of adrenal endocrinology. The development of the adrenal gland was linked to specific molecules. Cortisol-producing lesions were associated mostly with defects of the cyclic AMP (cAMP) signaling pathway, whereas aldosterone-producing lesions were found to be the result of defects in aldosterone biosynthesis or the potassium channel KCNJ5 and related molecules. Read More

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http://dx.doi.org/10.1007/s42000-018-0072-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6294814PMC
December 2018
3 Reads

Comparison of Constructive Interference in Steady-State and T1-Weighted MRI Sequence at Detecting Pituitary Adenomas in Cushing's Disease Patients.

J Neurol Surg B Skull Base 2018 Dec 10;79(6):593-598. Epub 2018 May 10.

Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, Ohio, United States.

 T1-weighted magnetic resonance imaging (MRI) remains the standard pituitary imaging sequence. However, up to 50% of pituitary adenomas are missed with standard imaging in Cushing's disease (CD) patients. Utility of other imaging sequences needs be explored. Read More

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http://dx.doi.org/10.1055/s-0038-1642032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6239881PMC
December 2018
18 Reads

Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature.

World J Surg Oncol 2018 Nov 19;16(1):227. Epub 2018 Nov 19.

Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan.

Background: Calcifying nested stromal epithelial tumor (CNSET) is a primary neoplasm of the liver, characterized by well-demarcated nests consisting of spindle and epithelioid cells with calcification and bone formation. An association of Cushing syndrome with CNSET has drawn attention, but the origin of CNSET has not been clarified.

Case Presentation: We report here the case of a 20-year-old male with Klinefelter syndrome who underwent liver resection for an increasing liver tumor that was pathologically diagnosed with CNSET. Read More

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http://dx.doi.org/10.1186/s12957-018-1528-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245822PMC
November 2018
3 Reads

Carney Syndrome Presented as a Pathological Spine Fracture in a 35-Year-Old Male.

Am J Case Rep 2018 Nov 16;19:1366-1369. Epub 2018 Nov 16.

Fifth Surgical Clinic, Department of Surgery, National and Kapodistrian University of Athens School of Medicine, Athens, Greece.

BACKGROUND Carney complex (CNC) is a genetic disorder that presents as an adrenocorticotropic hormone (ACTH)-independent variant of endogenous Cushing syndrome. It was first reported in 1985 and was described as a form of multiple endocrine hyperplasia associated with mutations of the c-AMP-dependent protein kinase (PRKAR1A) gene that causes bilateral adrenal hyperplasia. We report a case of an incidentally found CNC in a 35-year-old male, and this case report focuses on the diagnostic scheme as well as the surgical treatment of this rare challenging condition. Read More

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https://www.amjcaserep.com/abstract/index/idArt/911962
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http://dx.doi.org/10.12659/AJCR.911962DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6251001PMC
November 2018
9 Reads

Carney Complex.

Exp Clin Endocrinol Diabetes 2019 Feb 14;127(2-03):156-164. Epub 2018 Nov 14.

Section on Endocrinology and Genetics & Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD, USA.

Carney complex is a rare, autosomal dominant, multiple endocrine neoplasia and lentiginosis syndrome, caused in most patients by defects in the gene, which encodes the regulatory subunit type 1α of protein kinase A. Inactivating defects of lead to aberrant cyclic-AMP-protein kinase A signaling. Patients may develop multiple skin abnormalities and a variety of endocrine and non-endocrine tumors. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/a-0753-4943
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http://dx.doi.org/10.1055/a-0753-4943DOI Listing
February 2019
11 Reads

Corticotropin hormone assay interference: A case series.

Clin Biochem 2019 Jan 10;63:143-147. Epub 2018 Nov 10.

Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, MN, United States. Electronic address:

Measuring the plasma corticotropin (ACTH) concentration is an important step in determining the underlying cause in patients with hypercortisolemia. Interfering substances in immunoassays can lead to erroneous results impacting clinical management. We describe a case series of 12 patients, the majority of whom were being investigated for possible Cushing's syndrome and in whom inconsistencies between the clinical picture and biochemical testing raised concerns of assay interference. Read More

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http://dx.doi.org/10.1016/j.clinbiochem.2018.11.006DOI Listing
January 2019
2 Reads

Secondary Arterial Hypertension: From Routine Clinical Practice to Evidence in Patients with Adrenal Tumor.

High Blood Press Cardiovasc Prev 2018 Dec 10;25(4):345-354. Epub 2018 Nov 10.

Endocrinology Unit, Department of Medicine DIMED, University-Hospital of Padova, Via Ospedale Civile, 105, 35128, Padova, Italy.

Prevalence of arterial hypertension is up to 30-40% in epidemiological studies, it increases with aging and affects the cardiovascular risk. Essential form of hypertension is the most frequent; however, 5-10% of patients are affected by a specific and potentially reversible cause of increased blood pressure levels, called secondary hypertension. In general, all patients with young onset-age (< 40-50 years) or resistant hypertension should be screened for secondary forms. Read More

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http://link.springer.com/10.1007/s40292-018-0288-6
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http://dx.doi.org/10.1007/s40292-018-0288-6DOI Listing
December 2018
22 Reads

Thromboelastometry. Reproducibility of duplicate measurement performed by the RoTem® device.

Thromb Res 2018 12 31;172:139-141. Epub 2018 Oct 31.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy.

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http://dx.doi.org/10.1016/j.thromres.2018.10.030DOI Listing
December 2018
2 Reads

Accuracy of new recommendations for adrenal incidentalomas in the evaluation of excessive cortisol secretion and follow-up.

Eur J Clin Invest 2019 Feb 7;49(2):e13048. Epub 2018 Dec 7.

Department of Endocrinology and Nutrition, Joan XXIII University Hospital, Tarragona, Spain.

Objective: To evaluate whether the 2016 European Society of Endocrinology (ESE) recommendations for the management of adrenal incidentalomas accurately classifies those patients who do not require further follow-up.

Design And Methods: Single centre retrospective study. From 2010 to 2015, 130 patients with adrenal incidentaloma were evaluated and followed-up. Read More

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http://dx.doi.org/10.1111/eci.13048DOI Listing
February 2019
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