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    Screening for Cushing Syndrome at the Primary Care Level: What Every General Practitioner Must Know.
    Int J Endocrinol 2017 27;2017:1547358. Epub 2017 Jul 27.
    Directorate of Medicine, Endocrine and Diabetes Unit, Komfo Anokye Teaching Hospital, Kumasi, Ghana.
    Cushing's syndrome is a rare entity, and a high index of suspicion is needed for screening in a primary care setting. The clinical awareness of the primary care physician (PCP) to the highly indicative signs and symptoms such as facial plethora, proximal myopathy, reddish purple striae, and easy bruisability should alert him to look for biochemical evidence of Cushing's syndrome through any of the first-line screening tests, namely, 24-hour urinary free cortisol, overnight dexamethasone suppression test, or late-night salivary cortisol. Commonly used random cortisol measurements are unreliable; hence, general practitioners are encouraged to understand the use of these more reliable tests with increased sensitivity and specificity for screening Cushing's syndrome. Read More

    Adrenocortical carcinoma and succinate dehydrogenase gene mutations.
    Eur J Endocrinol 2017 Aug 17. Epub 2017 Aug 17.
    A Vaiyda, Endocrinology, Diabetes, and Hypertension, Brigham and Women's Hospital, Harvard Medical School, Boston, 02115, United States.
    Objective: Germline loss-of-function mutations in succinate dehydrogenase (SDHx) genes results in rare tumor syndromes that include pheochromocytoma, paraganglioma, and others. Here we report a case series of patients with adrenocortical carcinoma (ACC) that harbor SDHx germline mutations.

    Patients And Results: We report four unrelated patients with ACC and SDHx mutations. Read More

    Adrenal Cushing's syndrome during pregnancy.
    Eur J Endocrinol 2017 Aug 17. Epub 2017 Aug 17.
    R Feelders, Internal Medicine, Erasmus MC, Rotterdam, Netherlands.
    Cushing syndrome (CS) during pregnancy is a rare condition with only a few cases reported in the literature. Misdiagnosis of CS is common because of overlapping features like fatigue, weight gain, striae and emotional changes that can occur during normal pregnancy. Changes in maternal hormones and their binding proteins complicate assessment of glucocorticoid hormone levels during gestation. Read More

    High-Resolution, Accurate-Mass (HRAM) Mass Spectrometry Urine Steroid Profiling in the Diagnosis of Adrenal Disorders.
    Clin Chem 2017 Aug 16. Epub 2017 Aug 16.
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
    Background: Steroid profiling is a promising diagnostic tool for those with adrenal tumors, Cushing syndrome (CS), and disorders of steroidogenesis. Our objective was to develop a multiple-steroid assay using liquid-chromatography, high-resolution, accurate-mass mass spectrometry (HRAM LC-MS) and to validate the assay in patients with various adrenal disorders.

    Methods: We collected 24-h urine samples from 114 controls and 71 patients with adrenal diseases. Read More

    Case Report: An incidentaloma that catches your eye - adrenal myelolipoma.
    F1000Res 2017 18;6:1140. Epub 2017 Jul 18.
    WONCA Polaris - USA, Bangkok, 10500, Thailand.
    Background: Adrenal incidentaloma refers to the incidental finding of a tumor in the adrenal gland, where nonfunctional forms are the most common variant. Myelolipoma is a rare (0.08-0. Read More

    Cervicothoracic cutaneomeningospinal angiomatosis in adults (Cobb's syndrome): A case report of acute quadriparesis.
    Surg Neurol Int 2017 18;8:147. Epub 2017 Jul 18.
    Department of Neurosurgery, Hospital Córdoba, Córdoba, Argentina.
    Background: Cutaneomeningospinal angiomatosis or Cobb syndrome is a rare, not well understood phacomatosis that features metameric cutaneous and spinal arteriovenous malformations (AVMs). The first case was described in Boston in 1915, and since then, few more cases have been reported in the English literature. No case was found to be from Argentina. Read More

    Adrenocorticotropic Hormone-Secreting Neuroendocrine Tumor of the Rectum Demonstrated on 68Ga-DOTATATE and 18F-FDG PET Imaging.
    Clin Nucl Med 2017 Jul 29. Epub 2017 Jul 29.
    From the *Department of Nuclear Medicine, Concord Hospital, Concord; and †Sydney Medical School, University Sydney, Sydney, New South Wales, Australia.
    An 81-year-old man with Cushing syndrome was referred for a Ga-DOTATATE PET/CT study to investigate for an ectopic source of adrenocorticotropic hormone. The scan demonstrated mildly increased octreopeptide uptake at a rectal mass and focal uptake at multiple regions throughout the bone marrow of the axial skeleton, consistent with metastases. A subsequent F-FDG PET/CT study was performed for further evaluation and demonstrated markedly increased metabolism at the previously identified rectal mass, in addition to the liver and multiple regions throughout the skeleton. Read More

    Risk of obstructive sleep apnea among patients with Cushing's syndrome: a nationwide longitudinal study.
    Sleep Med 2017 Aug 27;36:44-47. Epub 2017 May 27.
    Department of Psychiatry, Taipei Veterans General Hospital, Taipei, Taiwan; Department of Psychiatry, College of Medicine, National Yang-Ming University, Taipei, Taiwan. Electronic address:
    Objective: Previous studies have demonstrated the association between Cushing's syndrome (CS), obstructive sleep apnea (OSA), and the risk factors for OSA, but rarely provided the evidence within a large population. Using the Taiwan National Health Insurance Research Database, we attempted to investigate the association between CS and OSA, and to provide persuading evidences.

    Methods: In our study, 1612 patients with CS and 1612 age-, sex-, and comorbidities-matched controls were included, and followed up to the end of 2011. Read More

    ANNALS EXPRESS: 20α- and 20β-dihydrocortisone may interfere in LC-MS/MS determination of cortisol in saliva and urine.
    Ann Clin Biochem 2017 Jan 1:4563217724178. Epub 2017 Jan 1.
    Norrlands universitetssjukhus.
    Background: LC-MS/MS methods offer high selectivity in cortisol determinations. However, endogenous steroid metabolites may still interfere and compromise the results, for example in the diagnosis of Cushing´s syndrome. Erroneously elevated cortisol may, in particular, be misleading at the low concentrations found in salivary samples obtained at late night and after dexamethasone suppression. Read More

    Angiogenesis and Lymphangiogenesis in the Adrenocortical Tumors.
    Pathol Oncol Res 2017 Jul 10. Epub 2017 Jul 10.
    Instituto de Investigação e Inovação em Saúde (I3S), Universidade do Porto, Porto, Portugal.
    Adrenocortical tumors (ACT) are common adrenal tumors. The majority of ACTs are non-functioning and benign, while adrenocortical carcinomas (ACC) are rare, usually very aggressive and often metastasized when first diagnosed. Our aim was to assess whether blood and lymph vessel density within ACTs correlate with the malignancy character or tumor functionality. Read More

    Frequency of familial pituitary adenoma syndromes among patients with functioning pituitary adenomas in a reference outpatient clinic.
    J Endocrinol Invest 2017 Jul 8. Epub 2017 Jul 8.
    Neuroendocrinology Research Center / Endocrinology Section, Medical School and Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rua Prof. Rodolpho Paulo Rocco, 255, 9th floor, Ilha do Fundão, Rio de Janeiro, 21941-913, Brazil.
    Introduction: Pituitary adenomas (PA) occur mainly as sporadic disease, but familial syndromes are found in approximately 5% of cases. Identification of these syndromes is important in order to diagnose individuals at risk at an earlier stage.

    Aims: To evaluate the frequency of familial PA in a reference outpatient clinic devoted to PA treatment and to identify family members suspected to have pituitary disease. Read More

    Late night salivary cortisol is unaltered in patients with polycystic ovarian syndrome (PCOS) irrespective of disease phenotype and in obese women irrespective of the presence of PCOS.
    Endocr Pract 2017 Jul 6. Epub 2017 Jul 6.
    From: 1Department of Endocrinology and Metabolism, Cerrahpasa Medical School, University of Istanbul, Istanbul, Turkey.
    Objective: To determine cut-off values of late-night salivary cortisol (LNSC) using an electrochemiluminescent immunoassay (ECLIA) and to investigate whether the diagnostic performance of the assay was influenced by the presence of obesity or polycystic ovary syndrome (PCOS).

    Methods: A total of 124 subjects comprising 25 patients with Cushing's syndrome (CS), 44 with PCOS (22 nonobese and 22 obese), 21 with constitutional obesity (CO), and 34 healthy subjects (HS) were included in the study. Two consecutive LNSC samples were collected from all participants. Read More

    The role of ARMC5 in human cell cultures from nodules of primary macronodular adrenocortical hyperplasia (PMAH).
    Mol Cell Endocrinol 2017 Jul 1. Epub 2017 Jul 1.
    Laboratory of Hormone and Molecular Genetic LIM/42, University of Sao Paulo, SP, Brazil; Adrenal Unit, Discipline of Endocrinology & Metabolism, University of Sao Paulo, SP, Brazil.
    The participation of aberrant receptors and intra-adrenal ACTH in hyperplastic tissue are considered mechanisms that regulate hypercortisolism in PMAH. Additionally, germline ARMC5 mutations have been described as the most frequent genetic abnormality found in patients diagnosed with PMAH. Previous functional studies analyzed ARMC5 role using H295R cells. Read More

    A polymorphism in the CYP17A1 gene influences the therapeutic response to steroidogenesis inhibitors in Cushing's syndrome.
    Clin Endocrinol (Oxf) 2017 Jun 30. Epub 2017 Jun 30.
    Endocrinology/Medicine Department, Hospital Sant Pau and IIB, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII and Universitat Autònoma de Barcelona (UAB), Barcelona, Spain.
    Context: Steroidogenesis inhibitors, such as ketoconazole (KTZ) and metyrapone (MTP), are used to lower hypercortisolism in patients with Cushing's syndrome (CS). Cortisol normalization is not reached in all patients taking these medications.

    Objective: To test the hypothesis that variants in genes affecting steroidogenesis contribute to different responses to KTZ and/or MTP in patients with CS. Read More

    A Case of Pulmonary Carcinoid Tumor with a Superimposed Aspergilloma Presenting As a Covert Ectopic Adrenocorticotropic Hormone Syndrome.
    Front Endocrinol (Lausanne) 2017 8;8:123. Epub 2017 Jun 8.
    Division of Endocrinology, Department of Internal Medicine, Korea University College of Medicine, Seoul, South Korea.
    Ectopic adrenocorticotropic hormone (ACTH) syndrome is a challenging diagnosis only responsible for approximately 10% of Cushing syndrome cases. It has been associated with a variety of benign and malignant tumors including a carcinoid tumor accompanied by aspergilloma in our case that was significantly difficult to be detected. We report a patient over 70 years old with uncontrolled hypertension and hypokalemia presenting with generalized edema. Read More

    Acquired Resistance to Corticotropin Therapy in Nephrotic Syndrome: Role of De Novo Neutralizing Antibody.
    Pediatrics 2017 Jul;140(1)
    Blood Purification Center, Institute of Nephrology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China;
    There is increasing evidence supporting the use of corticotropin as an alternative treatment of refractory proteinuric glomerulopathies. The efficacy of short-acting corticotropin, however, remains unknown and was tested here in an adolescent with steroid-dependent nephrotic syndrome caused by minimal change disease. After developing Cushing syndrome and recently being afflicted with severe cellulitis, the patient was weaned off all immunosuppressants, including corticosteroids. Read More

    Cushing Syndrome: Diagnostic Workup and Imaging Features, With Clinical and Pathologic Correlation.
    AJR Am J Roentgenol 2017 Jul;209(1):19-32
    1 Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030.
    Objective: Cushing syndrome (CS) is a constellation of clinical signs and symptoms resulting from chronic exposure to excess cortisol, either exogenous or endogenous. Exogenous CS is most commonly caused by administration of glucocorticoids. Endogenous CS is subdivided into two types: adrenocorticotropic hormone (ACTH) dependent and ACTH independent. Read More

    Rectal Metyrapone for Treatment of Hypercortisolism in an Infant with McCune-Albright Syndrome.
    J Pediatr Pharmacol Ther 2017 May-Jun;22(3):233-236
    Infantile Cushing syndrome is an infrequent yet potentially fatal manifestation of McCune-Albright syndrome, for which there are few safe treatments available. Ketoconazole is limited by potential hepatotoxicity in this population. Metyrapone may be an effective treatment, but it may not be tolerated when given orally. Read More

    Cushing's syndrome caused by use of synthetic ocular steroid.
    J Clin Pharm Ther 2017 Jun 18. Epub 2017 Jun 18.
    Department of Pediatrics Istanbul, Haseki Training and Research Hospital, Istanbul, Turkey.
    What Is Known And Objective: Cases of Cushing's syndrome (CS) following ocular steroid use have been reported in recent years, albeit rarely.

    Case Description: We report a case of iatrogenic CS in a child induced by fluorometholone-containing eyedrops. Our patient was referred to our endocrinology clinic due to rapid weight gain. Read More

    Prolactin correction for adequacy of petrosal sinus cannulation may diminish diagnostic accuracy in Cushing's disease.
    Clin Endocrinol (Oxf) 2017 Jun 19. Epub 2017 Jun 19.
    Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, SA, Australia.
    Objective: Petrosal venous prolactin concentrations have been promoted to improve the diagnostic accuracy of inferior petrosal sinus sampling (IPSS), beyond that achieved with ACTH measurement alone, in diagnosing a pituitary ACTH source and determining corticotrophinoma side (L/R). Our objective was to assess the effect of using prolactin to confirm adequacy of petrosal cannulation in a cohort of patients with ACTH-dependent Cushing's syndrome.

    Design: Retrospective cohort study. Read More

    AN INDIVIDUALIZED APPROACH TO THE EVALUATION OF CUSHING SYNDROME.
    Endocr Pract 2017 Jun;23(6):726-737
    Cushing syndrome (CS) is caused by chronic exposure to excess glucocorticoids. Early recognition and treatment of hypercortisolemia can lead to decreased morbidity and mortality. The diagnosis of CS and thereafter, establishing the cause can often be difficult, especially in patients with mild and cyclic hypercortisolism. Read More

    ECTOPIC CUSHING SYNDROME: A 10-YEAR EXPERIENCE FROM A TERTIARY CARE CENTER IN SOUTHERN INDIA.
    Endocr Pract 2017 Aug 14;23(8):907-914. Epub 2017 Jun 14.
    Objective: Ectopic adrenocorticotropic hormone (ACTH) secretion is a less common cause of Cushing syndrome and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care center in Southern India.

    Methods: The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. Read More

    BIOCHEMICAL CONTROL DURING LONG-TERM FOLLOW-UP OF 230 ADULT PATIENTS WITH CUSHING DISEASE: A MULTICENTER RETROSPECTIVE STUDY.
    Endocr Pract 2017 Aug 14;23(8):962-970. Epub 2017 Jun 14.
    Objective: Cushing disease (CD) results from excessive exposure to glucocorticoids caused by an adrenocorticotropic hormone-secreting pituitary tumor. Inadequately treated CD is associated with significant morbidity and elevated mortality. Multicenter data on CD patients treated in routine clinical practice are needed to assess treatment outcomes in this rare disorder. Read More

    Paraneoplastic Cushing Syndrome Due To Wilm's Tumor.
    J Coll Physicians Surg Pak 2017 May;27(5):313-315
    Department of Pediatrics, Lahore General Hospital/PGMI, Lahore.
    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Read More

    Influence of age, gender and body mass index on late-night salivary cortisol in healthy adults.
    Clin Chem Lab Med 2017 May 5. Epub 2017 May 5.
    Graduate Program in Medical Sciences: Endocrinology, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS.
    Background: Late-night salivary cortisol (LNSC) is one of the most reliable tests to screen for endogenous Cushing syndrome. This test is simple, inexpensive and noninvasive and has high sensitivity and specificity. The aim of our study was to analyze the putative influence of age, gender and body mass index (BMI) on LNSC levels in a healthy population. Read More

    Diagnostic accuracy of increased urinary cortisol/cortisone ratio to differentiate ACTH-dependent Cushing's syndrome.
    Clin Endocrinol (Oxf) 2017 Jun 7. Epub 2017 Jun 7.
    Endocrinology Unit, Department of Medicine DIMED, University Hospital of Padova, Padova, Italy.
    Background And Aim: Differential diagnosis between Cushing's Disease (CD) and Ectopic ACTH Syndrome (EAS) may be a pitfall for endocrinologists. The increasing use in clinical practice of chromatography and mass spectrometry improves the measurement of urinary free cortisol (UFF) and cortisone (UFE). We have recently observed that cortisol to cortisone ratio (FEr) was higher in a small series of EAS; in this study we collected a larger number of ACTH-dependent Cushing's Syndrome (CS) to study the role of FEr to characterize the source of corticotropin secretion. Read More

    Diagnosing endocrine hypertension: a practical approach.
    Nephrology (Carlton) 2017 Sep;22(9):663-677
    Centre for Endocrinology and Metabolism, Hudson Institute of Medical Research, Melbourne, Victoria, Australia.
    Hypertension is a leading cardiovascular risk factor that remains difficult to manage in a large segment of the population. Secondary causes of hypertension, which are amenable to targeted treatment or even cure, may contribute to poor blood pressure control. The most common endocrine cause, primary aldosteronism, requires biochemical screening as there are often no symptoms or signs other than hypertension. Read More

    A Rare Presentation of Infantile Virilization Secondary to Malignant Etiology.
    Indian J Surg Oncol 2017 Jun 18;8(2):203-205. Epub 2016 Nov 18.
    GMR Varalakshmi Care Hospital, Dist. Srikakulam, Rajam, Andhra Pradesh India.
    Adrenocortical tumor is a rare malignancy (1-2/million) in children with a heterogenous presentation and generally poor prognosis. We report two cases of adrenocortical carcinoma who presented primarily with virilization along with cushingoid features and hypertension. Both children were managed with surgical resection of tumor, steroid replacement, and antihypertensives. Read More

    Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome.
    Horm Res Paediatr 2017 May 19. Epub 2017 May 19.
    Department of Pediatrics, Oita University Faculty of Medicine, Oita, Japan.
    Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Read More

    PRKAR1A mutation causing pituitary-dependent Cushing disease in a patient with Carney complex.
    Eur J Endocrinol 2017 Aug 18;177(2):K7-K12. Epub 2017 May 18.
    Clinical Division of Endocrinology and MetabolismDepartment of Medicine III, Medical University of Vienna, Vienna, Austria
    Context: Carney complex (CNC) is an autosomal dominant condition caused, in most cases, by an inactivating mutation of the PRKAR1A gene, which encodes for the type 1 alpha regulatory subunit of protein kinase A. CNC is characterized by the occurrence of endocrine overactivity, myxomas and typical skin manifestations. Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD) is the most frequent endocrine disease observed in CNC. Read More

    Immune plasma for the treatment of severe influenza: an open-label, multicentre, phase 2 randomised study.
    Lancet Respir Med 2017 Jun 15;5(6):500-511. Epub 2017 May 15.
    National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA.
    Background: Influenza causes substantial morbidity and mortality despite available treatments. Anecdotal reports suggest that plasma with high antibody titres to influenza might be of benefit in the treatment of severe influenza.

    Methods: In this randomised, open-label, multicentre, phase 2 trial, 29 academic medical centres in the USA assessed the safety and efficacy of anti-influenza plasma with haemagglutination inhibition antibody titres of 1:80 or more to the infecting strain. Read More

    Cushing's Syndrome, Cortisol, and Cognitive Competency: A Case Report.
    Case Rep Oncol 2017 Jan-Apr;10(1):325-327. Epub 2017 Apr 6.
    bEpicentRx, Inc., San Diego, CA, USA.
    Glucocorticoids are associated with immunosuppression and neuropsychiatric complications. We describe the case of a carcinoid patient with Cushing's syndrome (CS) and neurocognitive impairment due to ectopic ACTH production who developed sepsis and died because of his family's decision to withdraw antibiotic treatment. This report is presented to illustrate the importance of advanced-care planning in patients with CS. Read More

    A Case of Paraneoplastic Cushing Syndrome Presenting as Hyperglycemic Hyperosmolar Nonketotic Syndrome.
    Case Rep Oncol 2017 Jan-Apr;10(1):321-324. Epub 2017 Apr 6.
    aWalter Reed National Military Medical Center, Bethesda, MD, USA.
    Carcinoid tumors are neuroendocrine tumors that mainly arise in the gastrointestinal tract, lungs, and bronchi. Bronchopulmonary carcinoids have been associated with Cushing syndrome, which results from ectopic adrenocorticotrophic hormone (ACTH) secretion. We report the case of a 65-year-old man, a colonel in the US Air Force, with metastatic bronchopulmonary carcinoid tumors treated on a clinical trial who was hospitalized for complaints of increasing thirst, polydipsia, polyuria, weakness, and visual changes. Read More

    Dyslipidemia, weight gain, and decreased growth velocity in a 14-year-old male.
    J Clin Lipidol 2017 Mar - Apr;11(2):562-566. Epub 2017 Feb 2.
    Fort Worth Pediatrics, Cook Children's Medical Center, Fort Worth, TX, USA.
    A 14-year-old male was referred for dyslipidemia. His findings were consistent with metabolic syndrome. Although he lacked the typical physical appearance, his accelerated weight gain combined with a decreased linear growth velocity suggested Cushing syndrome. Read More

    Growth hormone deficiency in treated acromegaly and active Cushing's syndrome.
    Best Pract Res Clin Endocrinol Metab 2017 Feb 9;31(1):79-90. Epub 2017 Mar 9.
    Endocrinology, Univeristy of Brescia, Italy. Electronic address:
    Growth hormone deficiency (GHD) in adults is characterized by reduced quality of life and physical fitness, skeletal fragility, increased weight and cardiovascular risk. It may be found in (over-) treated acromegaly as well as in active Cushing's syndrome. Hypopituitarism may develop in patients after definitive treatment of acromegaly, although the exact prevalence of GHD in this population is still uncertain because of limited awareness, and scarce and conflicting data so far available. Read More

    Fluticasone furoate induced iatrogenic Cushing syndrome in a pediatric patient receiving anti-retroviral therapy.
    Endocrinol Diabetes Metab Case Rep 2017 6;2017. Epub 2017 Apr 6.
    Departments of Pediatrics, Amphia Hospital, BredaThe Netherlands.
    Summary: We present a case of iatrogenic Cushing's syndrome, induced by treatment with fluticasone furoate (1-2 dd, 27.5 µg in each nostril) in a pediatric patient treated for congenital HIV. The pediatric patient described in this case report is a young girl of African descent, treated for congenital HIV with a combination therapy of Lopinavir/Ritonavir (1 dd 320/80 mg), Lamivudine (1 dd 160 mg) and Abacavir (1 dd 320 mg). Read More

    ARMC5 mutation in a Portuguese family with primary bilateral macronodular adrenal hyperplasia (PBMAH).
    Endocrinol Diabetes Metab Case Rep 2017 27;2017. Epub 2017 Mar 27.
    Endocrinology Department, Hospital Curry Cabral, Centro Hospitalar de Lisboa Central, LisbonPortugal.
    Summary: PBMAH is a rare etiology of Cushing syndrome (CS). Familial clustering suggested a genetic cause that was recently confirmed, after identification of inactivating germline mutations in armadillo repeat-containing 5 (ARMC5) gene. A 70-year-old female patient was admitted due to left femoral neck fracture in May 2014, in Orthopedics Department. Read More

    [Atypical Cushing's syndrome in a dog. A case report. Ein Fallbericht].
    Tierarztl Prax Ausg K Kleintiere Heimtiere 2017 Jun 27;45(3):186-192. Epub 2017 Apr 27.
    Prof. Dr. Reinhard Mischke, Klinik für Kleintiere, Stiftung Tierärztliche Hochschule Hannover, Bünteweg 9, 30559 Hannover, E-Mail:
    In a 12-year-old male Labrador Retriever, presented due to other disease symptoms, clinical signs of hyperadrenocorticism (polyuria, polydipsia, abdominal distention, muscle atrophy) were an incidental finding. Abnormal laboratory results and sonographic findings of the adrenal glands, but negative low-dose dexamethasone suppression tests with low basal cortisol concentrations, a negative andrenocorticotropic hormone (ACTH)-stimulation test and exclusion of iatrogenic hyperadrenocorticism, suggested an atypical hyperadrenocorticism (AHAC). Results of further examinations, particularly stimulation of progesterone production by ACTH (0 h value: 0. Read More

    Zinc alpha-2 glycoprotein is overproduced in Cushing's syndrome.
    Endocrinol Diabetes Nutr 2017 Jan 18;64(1):26-33. Epub 2017 Jan 18.
    Group of Endocrine Disorders, IDIBAPS, Barcelona, Spain; Department of Endocrinology and Nutrition, Hospital Clínic, Barcelona, Spain; University of Barcelona, Spain. Electronic address:
    Introduction: Cushing syndrome (CS), an endogenous hypercortisolemic condition with increased cardiometabolic morbidity, leads to development of abdominal obesity, insulin resistance, diabetes and proatherogenic dyslipidemia. Zinc alpha-2 glycoprotein (ZAG) is a recently characterized lipolytic adipokine implicated in regulation of adipose tissue metabolism and fat distribution. In vitro and animal studies suggest that glucocorticoids interact with ZAG secretion and action. Read More

    Cushing Disease in a patient with Multiple Endocrine Neoplasia type 2B.
    J Clin Transl Endocrinol Case Rep 2017 Jun;4:1-4
    National Institute of Child Health & Human Development, National Institutes of Health (NIH), Building 10-CRC, room 1-3330 10 Center Drive Bethesda MD 20892.
    Context: Multiple endocrine neoplasia type 2B (MEN2B) is a rare autosomal-dominant cancer syndrome characterized in part by metastatic medullary thyroid cancer (MTC) and pheochromocytoma. Cushing disease is a rare cause of endogenous hypercortisolism in children.

    Case Description: We describe a 21-year-old African-American male who was diagnosed at age 10 with an ACTH-secreting pituitary microadenoma. Read More

    Circadian Plasma Cortisol Measurements Reflect Severity of Hypercortisolemia in Children with Different Etiologies of Endogenous Cushing Syndrome.
    Horm Res Paediatr 2017 21;87(5):295-300. Epub 2017 Apr 21.
    Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA.
    Background: The utility of circadian cortisol variation in estimating the degree of hypercortisolemia in different forms of endogenous Cushing syndrome (CS) has not been evaluated in children yet.

    Methods: A retrospective cohort study, including children who underwent surgery due to CS (n = 115), was divided into children with a pituitary adenoma (Cushing disease) (n = 88), primary adrenal CS (n = 21), or ectopic adrenocorticotropin- or corticotropin-releasing hormone (ACTH-/CRH)-secreting tumors (n = 6). Circadian plasma cortisol measurements were obtained at 11: 30 p. Read More

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