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    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 May 18. Epub 2017 May 18.
    A Gessl, Department of Medicine III, Clinical Division of Endocrinology & Metabolism, 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 May 15. 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

    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 Apr 21. Epub 2017 Apr 21.
    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

    Steroid metabolome analysis reveals prevalent glucocorticoid excess in primary aldosteronism.
    JCI Insight 2017 Apr 20;2(8). Epub 2017 Apr 20.
    Background: Adrenal aldosterone excess is the most common cause of secondary hypertension and is associated with increased cardiovascular morbidity. However, adverse metabolic risk in primary aldosteronism extends beyond hypertension, with increased rates of insulin resistance, type 2 diabetes, and osteoporosis, which cannot be easily explained by aldosterone excess.

    Methods: We performed mass spectrometry-based analysis of a 24-hour urine steroid metabolome in 174 newly diagnosed patients with primary aldosteronism (103 unilateral adenomas, 71 bilateral adrenal hyperplasias) in comparison to 162 healthy controls, 56 patients with endocrine inactive adrenal adenoma, 104 patients with mild subclinical, and 47 with clinically overt adrenal cortisol excess. Read More

    A Case Report of Cushing's Disease Presenting as Hair Loss.
    Case Rep Dermatol 2017 Jan-Apr;9(1):45-50. Epub 2017 Feb 21.
    Department of Dermatology, Roger Williams Medical Center, Providence, RI, USA.
    Cushing's syndrome is a rare endocrine disorder that comprises a large group of signs and symptoms resulting from chronic exposure to excess corticosteroids. Most cases of Cushing's syndrome are due to increased adrenocorticotropic hormone production from a pituitary adenoma, which is referred to as Cushing's disease. Most of the signs and symptoms are nonspecific and common in the general population, making a diagnosis often challenging. Read More

    Health-related quality of life of patients with hypothalamic-pituitary-adrenal axis dysregulations. A cohort study.
    Eur J Endocrinol 2017 Apr 12. Epub 2017 Apr 12.
    J Coste, Biostatistics and Epidemiology, Hôtel Dieu, Paris, France
    Objective: Health-related quality of life (HrQoL) is increasingly considered to be an important outcome of care for hypothalamic-pituitary-adrenal (HPA) axis dysregulation. The objective of this study was to assess the influence of type of HPA axis dysregulation and cortisol status on HrQOL and its evolution with time and treatment.

    Design: Prospective cohort study. Read More

    N-myc downstream-regulated gene 2 (NDRG2) promoter methylation and expression in pituitary adenoma.
    Diagn Pathol 2017 Apr 8;12(1):33. Epub 2017 Apr 8.
    Neuroscience Institute, Lithuanian University of Health Sciences, Eiveniu str.4, LT-50009, Kaunas, Lithuania.
    Background: Pituitary adenoma (PA) is a benign primary tumor that arises from the pituitary gland and is associated with ophthalmological, neurological and endocrinological abnormalities. However, causes that increase tumor progressing recurrence and invasiveness are still undetermined. Several studies have shown N-myc downstream regulated gene 2 (NDRG2) as a tumor suppressor gene, but the role of NDRG2 gene in pituitary adenoma pathogenesis has not been elucidated. Read More

    Current best practice in the management of patients after pituitary surgery.
    Ther Adv Endocrinol Metab 2017 Mar 1;8(3):33-48. Epub 2017 Mar 1.
    Department of Medicine, Division of Endocrinology, Diabetes and Metabolism, Johns Hopkins University, 1830 East Monument Street #333, Baltimore, MD 21287, USA.
    Sellar and parasellar masses are a common finding, and most of them are treated surgically via transsphenoidal approach. This type of surgery has revolutionized the approach to several hypothalamic-pituitary diseases and is usually effective, and well-tolerated by the patient. However, given the complex anatomy and high density of glandular, neurological and vascular structures in a confined space, transsphenoidal surgery harbors a substantial risk of complications. Read More

    Diagnostic tests for Cushing's syndrome differ from published guidelines: data from ERCUSYN.
    Eur J Endocrinol 2017 05;176(5):613-624
    Department of EndocrinologyChristie Hospital, Manchester, UK.
    Objective: To evaluate which tests are performed to diagnose hypercortisolism in patients included in the European Registry on Cushing's syndrome (ERCUSYN), and to examine if their use differs from the current guidelines.

    Patients And Methods: We analyzed data on the diagnostic tests performed in 1341 patients with Cushing's syndrome (CS) who have been entered into the ERCUSYN database between January 1, 2000 and January 31, 2016 from 57 centers in 26 European countries. Sixty-seven percent had pituitary-dependent CS (PIT-CS), 24% had adrenal-dependent CS (ADR-CS), 6% had CS from an ectopic source (ECT-CS) and 3% were classified as having CS from other causes (OTH-CS). Read More

    Ectopic ACTH-producing large cell neuroendocrine Pancoast tumour presenting as Horner syndrome.
    BMJ Case Rep 2017 Mar 24;2017. Epub 2017 Mar 24.
    Department of Family Medicine, Apple Valley Medical Clinic, Apple Valley, Minnesota, USA.
    We present an interesting case where a patient is presented with a droopy left eyelid (as part of Horner syndrome) and Cushingoid features which were a result of a Pancoast tumour (apical lung tumour in superior pulmonary sulcus) involving the left lung. This tumour was secreting ectopic adrenocorticotropic hormone (ACTH), a paraneoplastic endocrine phenomenon, which resulted in Cushing syndrome symptomatology. Though most ectopic ACTH-producing lung cancers are either small cell or carcinoid tumours, this was in fact a large cell neuroendocrine cancer (LCNEC). Read More

    LONG-TERM OUTCOME OF THE DIFFERENT TREATMENT ALTERNATIVES FOR RECURRENT AND PERSISTENT CUSHING'S DISEASE.
    Endocr Pract 2017 Mar 23. Epub 2017 Mar 23.
    From: Endocrinology Service/Experimental Endocrinology Unit1, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, IMSS, Mexico City.
    Background: Treatment alternatives for persistent and recurrent Cushing's disease (CD) include pituitary surgical reintervention, radiotherapy, pharmacotherapy and bilateral adrenalectomy (BA). The decision of which of these alternatives is better suited for the individual patient rests on clinical judgment and the availability of resources.

    Objective: To evaluate the long-term efficacy of the different secondary interventions for persistent and recurrent Cushing's disease. Read More

    White matter involvement on DTI-MRI in Cushing's syndrome relates to mood disturbances and processing speed: a case-control study.
    Pituitary 2017 Jun;20(3):340-348
    Neuroradiology Unit, Hospital Sant Pau, IIB-Sant Pau, and UAB, C/ San Antoni Maria Claret 167, 08025, Barcelona, Spain.
    Purpose: Cushing's syndrome (CS) is an endocrine disorder due to prolonged exposure to cortisol. Recently, microstructural white matter (WM) alterations detected by diffusion tensor imaging (DTI) have been reported in CS patients, and related to depression, but other functional significances. remain otherwise unclear. Read More

    Acute Hypercortisolemia Exerts Depot-Specific Effects on Abdominal and Femoral Adipose Tissue Function.
    J Clin Endocrinol Metab 2017 Apr;102(4):1091-1101
    Institute of Metabolism and Systems Research, University of Birmingham B15 2TT, United Kingdom.
    Context: Glucocorticoids have pleiotropic metabolic functions, and acute glucocorticoid excess affects fatty acid metabolism, increasing systemic lipolysis. Whether glucocorticoids exert adipose tissue depot-specific effects remains unclear.

    Objective: To provide an in vivo assessment of femoral and abdominal adipose tissue responses to acute glucocorticoid administration. Read More

    Anatomic Variations in Pituitary Endocrinopathies: Implications for the Surgical Corridor.
    J Neurol Surg B Skull Base 2017 Apr 10;78(2):105-111. Epub 2016 Aug 10.
    Department of Head and Neck Surgery, David Geffen School of Medicine at the University of California, Los Angeles (UCLA) Medical Center, Los Angeles, California, United States.
    Objectives/Hypotheses Functioning pituitary adenomas may produce endocrinopathies such as acromegaly and Cushing syndrome. Both conditions lead to characteristic anatomic variations as a result of hormonally induced abnormal soft tissue deposition. We evaluate the anatomic differences between acromegalics and Cushing disease patients and compare these dimensions to controls. Read More

    Simultaneous assay of cortisol and dexamethasone improved diagnostic accuracy of the dexamethasone suppression test.
    Eur J Endocrinol 2017 Jun 15;176(6):705-713. Epub 2017 Mar 15.
    Department of Clinical ScienceUniversity of Bergen, Bergen, Norway.
    Objectives: The overnight dexamethasone (DXM) suppression test (DST) has high sensitivity, but moderate specificity, for diagnosing hypercortisolism. We have evaluated if simultaneous measurement of S-DXM may correct for variable DXM bioavailability and increase the diagnostic performance of DST, and if saliva (sa) is a feasible adjunct or alternative to serum.

    Design And Methods: Prospective study of DST was carried out in patients with suspected Cushing's syndrome (CS) (n = 49), incidentaloma (n = 152) and healthy controls (n = 101). Read More

    Scalp hair cortisol for diagnosis of Cushing's syndrome.
    Eur J Endocrinol 2017 Jun 13;176(6):695-703. Epub 2017 Mar 13.
    Division of EndocrinologyDepartment of Internal Medicine
    Objective: Current first-line screening tests for Cushing's syndrome (CS) only measure time-point or short-term cortisol. Hair cortisol content (HCC) offers a non-invasive way to measure long-term cortisol exposure over several months of time. We aimed to evaluate HCC as a screening tool for CS. Read More

    A successful case of pregnancy in a woman with ACTH-independent Cushing's syndrome treated with ketoconazole and metyrapone.
    Gynecol Endocrinol 2017 May 26;33(5):349-352. Epub 2017 Feb 26.
    b Department of Clinical Endocrinology and Metabolic Disease , Polish Mother's Memorial Hospital Research Institute , Lodz , Poland.
    Cushing's syndrome (CS) is a rare disease caused by a chronic excess of cortisol. Hypercortisolaemia may affect reproductive system leading to infertility in women. However, some of the patients remain fertile, although pregnancy is uncommon. Read More

    Jean Camus and Gustave Roussy: pioneering French researchers on the endocrine functions of the hypothalamus.
    Pituitary 2017 Mar 6. Epub 2017 Mar 6.
    Department of Neurosurgery, La Princesa University Hospital, Madrid, Spain.
    At the beginning of the twentieth century, the hypothalamus was known merely as an anatomical region of the brain lying beneath the thalamus. An increasing number of clinicopathological reports had shown the association of diabetes insipidus and adiposogenital dystrophy (Babinski-Fröhlich's syndrome), with pituitary tumors involving the infundibulum and tuber cinereum, two structures of the basal hypothalamus. The French physicians Jean Camus (1872-1924) and Gustave Roussy (1874-1948) were the first authors to undertake systematic, controlled observations of the effects of localized injuries to the basal hypothalamus in dogs and cats by pricking the infundibulo-tuberal region (ITR) with a heated needle. Read More

    Bilateral primary pigmented nodular adrenal disease as a component of Carney syndrome - case report.
    Endokrynol Pol 2017 ;68(1):70-72
    Department of Rheumatology and Rehabilitation, Poznan University of Medical Sciences, Poland.
    We report a case of a 20-year-old patient with Cushing's syndrome as a component of Carney syndrome. Carney syndrome is an autosomal dominant disease with co-existing bilateral pigmented nodular adrenal disease, heart and skin myxoma, skin pigmentation, breast fibroadenoma, testicular and ovarian tumours, thyroid tumours, and pituitary adenomas. (Endokrynol Pol 2017; 68 (1): 70-72). Read More

    Interference in ACTH immunoassay negatively impacts the management of subclinical hypercortisolism.
    Endocrine 2017 May 28;56(2):308-316. Epub 2017 Feb 28.
    Department of Endocrinology, Dokuz Eylul University Faculty of Medicine, Narlidere, 35340, Izmir, Turkey.
    Purpose: Low plasma corticotropin is considered a useful parameter for the diagnosis of subclinical hypercortisolism in patients with an adrenal incidentaloma. However, immunoassays are vulnerable to interference from endogenous antibodies. In this study, subjects who underwent Hypothalamus-pituitary-adrenal axis evaluation for the assessment of subclinical hypercortisolism were evaluated. Read More

    Forkhead box O3 plays a role in skeletal muscle atrophy through expression of E3 ubiquitin ligases Murf-1 and Atrogin-1 in Cushing's syndrome.
    Am J Physiol Endocrinol Metab 2017 Feb 28:ajpendo.00389.2016. Epub 2017 Feb 28.
    Kyungpook National University
    Cushing's syndrome is caused by overproduction of the adrenocorticotropic hormone (ACTH), which stimulates the adrenal grand to make cortisol. Skeletal muscle wasting occurs in pathophysiological response to Cushing's syndrome. The forkhead box (FOX) protein family has been implicated as a key regulator of muscle loss under conditions such as diabetes and sepsis. Read More

    Coagulation Profile in Patients with Different Etiologies for Cushing Syndrome: A Prospective Observational Study.
    Horm Metab Res 2017 May 22;49(5):365-371. Epub 2017 Feb 22.
    Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
    Previous studies reported a higher prevalence of venous-thromboembolic events among patients with Cushing disease (CD) compared to those with ACTH-independent Cushing syndrome (CS) from adrenal sources. The objective of the current study was to evaluate the coagulation profile of patients with CS from different etiologies. A prospective observational study was conducted at a clinical research center. Read More

    Ambulatory Arterial Stiffness Indexes in Cushing's Syndrome.
    Horm Metab Res 2017 Mar 22;49(3):214-220. Epub 2017 Feb 22.
    Department of Medicine-DIMED, University of Padova, Padova, Italy.
    Long-standing exposure to endogenous cortisol excess is associated with high cardiovascular risk. The aim of our study was to investigate arterial stiffness, which has been recognized as an independent predictor of adverse cardiovascular outcome, in a group of patients with Cushing's syndrome. Twenty-four patients with Cushing's syndrome (3 males, mean age 49±13 years; 20 pituitary-dependent Cushing's disease and 4 adrenal adenoma) underwent 24-h ambulatory blood pressure monitoring (ABPM) and evaluation of cardiovascular risk factors. Read More

    MELANOCORTIN-4 RECEPTOR GENE MUTATIONS IN A GROUP OF TURKISH OBESE CHILDREN AND ADOLESCENTS.
    J Clin Res Pediatr Endocrinol 2017 Feb 20. Epub 2017 Feb 20.
    Objective: Melanocortin-4 receptor (MC4R) mutations are the most common known cause of monogenic obesity but there is limited data regarding MC4R mutations in Turkish subjects. To determine the prevalence of MC4R mutations in a group of Turkish morbid obese children and adolescents.

    Methods: MC4R was sequenced in 47 consecutive morbidly obese children and adolescents (28 girls and 19 boys, aged 1-18 years) who presented during a 1-year period. Read More

    Study of awareness of adrenal disorders among interns and postgraduate students of Hamidia Hospital, Bhopal.
    Indian J Endocrinol Metab 2017 Jan-Feb;21(1):251-254
    Department of Medicine, Gandhi Medical College, Bhopal, Madhya Pradesh, India.
    Introduction: Adrenal disorders could be a life-threatening emergency, hence requires immediate therapeutic management. For this awareness regarding its diagnosis, management, and treatment is prime important.

    Aims And Objective: To study the awareness of adrenal disorders among interns and postgraduates students of Hamidia Hospital, Bhopal. Read More

    Mycophenolate Mofetil Combined With Prednisone Versus Full-Dose Prednisone in IgA Nephropathy With Active Proliferative Lesions: A Randomized Controlled Trial.
    Am J Kidney Dis 2017 Jun 16;69(6):788-795. Epub 2017 Feb 16.
    National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China. Electronic address:
    Background: Observational studies suggest that patients with immunoglobulin A nephropathy (IgAN) with active proliferative lesions show a good response to immunosuppressive treatment.

    Study Design: Multicenter, prospective, randomized, controlled trial.

    Setting & Participants: 176 patients with IgAN with active proliferative lesions (cellular and fibrocellular crescents, endocapillary hypercellularity, or necrosis), proteinuria with protein excretion ≥ 1. Read More

    Hair cortisol in the evaluation of Cushing syndrome.
    Endocrine 2017 Apr 13;56(1):164-174. Epub 2017 Feb 13.
    Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, 20892, USA.
    Purpose: Hair cortisol evaluation has been used to help detect patients with suspected Cushing syndrome. Our goal was to correlate segmental hair cortisol with biochemical testing in patients with Cushing syndrome and controls. This study was a prospective analysis of hair cortisol in confirmed Cushing syndrome cases over 16 months. Read More

    Prognostic factors in ectopic Cushing's syndrome due to neuroendocrine tumors: a multicenter study.
    Eur J Endocrinol 2017 Apr;176(4):451-459
    Section of EndocrinologyDepartment of Medicine, University of Verona, Verona, Italy.
    Objective: Evidence is limited regarding outcome of patients with ectopic Cushing's syndrome (ECS) due to neuroendocrine tumors (NETs).

    Design: We assessed the prognostic factors affecting the survival of patients with NETs and ECS.

    Methods: Retrospective analysis of clinicopathological features, severity of hormonal syndrome, treatments from a large cohort of patients with NETs and ECS collected from 17 Italian centers. Read More

    Appearance of a thymic mass after treatment of Cushing's syndrome.
    Asian Cardiovasc Thorac Ann 2017 Feb 19;25(2):150-153. Epub 2016 Dec 19.
    1 Internal Medicine Unit, University Hospital of Sassari, Sassari, Italy.
    A 23-year-old woman was referred to our center with hirsutism, acne, weight gain, weakness, and irregular menses. Laboratory tests revealed increased levels of cortisol and sex hormones, and reduced adrenocorticotropic hormone levels. The patient underwent a right adrenalectomy. Read More

    Cushing's syndrome in children and adolescents: a Danish nationwide population-based cohort study.
    Eur J Endocrinol 2017 May 8;176(5):567-574. Epub 2017 Feb 8.
    Departments of Internal Medicine and Endocrinology.
    Objective: Cushing's syndrome (CS) affects all age groups, but epidemiologic data in young patients are very limited. We therefore examined the incidence, prevalence and hospital morbidity of CS in children and adolescents.

    Design: In a nationwide cohort study, we included all Danish citizens aged 0-20 years from 1977 to 2012. Read More

    DIAGNOSIS OF ENDOCRINE DISEASE: Differentiation of pathologic/neoplastic hypercortisolism (Cushing's syndrome) from physiologic/non-neoplastic hypercortisolism (formerly known as pseudo-Cushing's syndrome).
    Eur J Endocrinol 2017 May 8;176(5):R205-R216. Epub 2017 Feb 8.
    Departments of MedicineSurgery, and Physiology, Medical College of Wisconsin and Endocrine Research Laboratory, Aurora St Luke's Medical Center, Aurora Research Institute, Milwaukee, Wisconsin, USA.
    Endogenous hypercortisolism (Cushing's syndrome) usually implies the presence of a pathologic condition caused by either an ACTH-secreting neoplasm or autonomous cortisol secretion from a benign or malignant adrenal neoplasm. However, sustained or intermittent hypercortisolism may also accompany many medical disorders that stimulate physiologic/non-neoplastic activation of the HPA axis (formerly known as pseudo-Cushing's syndrome); these two entities may share indistinguishable clinical and biochemical features. A thorough history and physical examination is often the best (and sometimes only) way to exclude pathologic/neoplastic hypercortisolism. Read More

    Romiplostim therapy as a second-line treatment before splenectomy for refractory immune thrombocytopenia in a cirrhotic patient with iatrogenic Cushing syndrome secondary to corticosteroids.
    Clin Case Rep 2017 Feb 23;5(2):159-163. Epub 2017 Jan 23.
    Gastroenterology Service Hepatitis and Liver Transplant Unit IDIBELL Hospital Universitari de Bellvitge Feixa Llarga s/n L'Hospitalet de Llobregat 08907 Barcelona Spain.
    Our case report discusses the usefulness of administering romiplostim as a second-line treatment before splenectomy in a cirrhotic patient with immune thrombocytopenia who developed corticosteroid-induced Cushing's syndrome. Corticosteroids were tapered and consequently withdrawn. The patient made a full recovery postsplenectomy. Read More

    Adrenocortical neoplasms in adulthood and childhood: distinct presentation. Review of the clinical, pathological and imaging characteristics.
    J Pediatr Endocrinol Metab 2017 Mar;30(3):253-276
    Adrenocortical tumors (ACT) in adulthood and childhood vary in clinical, histopathological, molecular, prognostic, and imaging aspects. ACT are relatively common in adults, as adenomas are often found incidentally on imaging. ACT are rare in children, though they have a significantly higher prevalence in the south and southeast regions of Brazil. Read More

    Metabolomic Biomarkers in Urine of Cushing's Syndrome Patients.
    Int J Mol Sci 2017 Jan 29;18(2). Epub 2017 Jan 29.
    Department of Food Sciences, Faculty of Pharmacy, Medical University of Gdańsk, Al. Gen. J. Hallera 107, 80-416 Gdańsk, Poland.
    Cushing's syndrome (CS) is a disease which results from excessive levels of cortisol in the human body. The disorder is associated with various signs and symptoms which are also common for the general population not suffering from compound hypersecretion. Thus, more sensitive and selective methods are required for the diagnosis of CS. Read More

    An Unlikely Cause of Hypokalemia.
    J Emerg Med 2017 May 28;52(5):e187-e191. Epub 2017 Jan 28.
    Temple University Hospital, Philadelphia, Pennsylvania.
    Background: Hypokalemia is a common clinical disorder caused by a variety of different mechanisms. Although the most common causes are diuretic use and gastrointestinal losses, elevated cortisol levels can also cause hypokalemia through its effects on the renin-angiotensin-aldosterone system. Cushing's syndrome refers to this general state of hypercortisolemia, which often manifests with symptoms of generalized weakness, high blood pressure, diabetes mellitus, menstrual disorders, and psychiatric changes. Read More

    Lead exposure is related to hypercortisolemic profiles and allostatic load in Brazilian older adults.
    Environ Res 2017 Apr 19;154:261-268. Epub 2017 Jan 19.
    Centre for Studies on Human Stress, Mental Health University Institute, Department of Psychiatry, University of Montreal, QC, Canada H1N 3V2.
    Lead levels (Pb) have been linked to both hyper- and hypo-reactivity of hypothalamic-pituitary-adrenal axis (HPA) axis to acute stress in animals and humans. Similarly, allostatic load (AL), the 'wear and tear' of chronic stress, is associated with inadequate HPA axis activity. We examined whether Pb levels would be associated with altered diurnal cortisol profile, as a primary mediator of AL, during aging. Read More

    Ectopic Cushing syndrome in small cell lung cancer: A case report and literature review.
    Thorac Cancer 2017 Mar 8;8(2):114-117. Epub 2016 Nov 8.
    Department of Thoracic Medical Oncology, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing, China.
    Small cell lung cancer (SCLC) is a neuroendocrine tumor with the potential to secrete various peptides or hormones that can lead to paraneoplastic syndromes, such as Ectopic Cushing syndrome (ECS). Because of the aggressive nature of the syndrome and its atypical features, ECS in small-cell lung cancer is difficult to diagnose and has a poor prognosis. We report a case of a 74-year-old male patient who presented with severe hypokalemia, proximal muscle weakness, peripheral edema, metabolic alkalosis, and worsening hyperglycemia. Read More

    Cushing's syndrome mutant PKA(L)(205R) exhibits altered substrate specificity.
    FEBS Lett 2017 Feb 3;591(3):459-467. Epub 2017 Feb 3.
    Department of Physiology and Neurobiology, University of Connecticut, Storrs, CT, USA.
    The PKA(L)(205R) hotspot mutation has been implicated in Cushing's syndrome through hyperactive gain-of-function PKA signaling; however, its influence on substrate specificity has not been investigated. Here, we employ the Proteomic Peptide Library (ProPeL) approach to create high-resolution models for PKA(WT) and PKA(L)(205R) substrate specificity. We reveal that the L205R mutation reduces canonical hydrophobic preference at the substrate P + 1 position, and increases acidic preference in downstream positions. Read More

    Source of Ectopic ACTH Secretion Easily Identified by 68 Ga DOTANOC PET/CT.
    Clin Nucl Med 2017 Apr;42(4):295-296
    From the Nuclear Medicine Division, Groote Schuur Hospital/University of Cape Town, Cape Town, South Africa.
    Malignant tumors account for most sources of ectopic ACTH Cushing syndrome (EA-CS). Early localization of the source and complete removal can be curative and also prevent metastasis. Diagnostic CT is known to perform better than PET/CT (low dose) in characterizing lung pathologies. Read More

    Characterizing and predicting the Nelson-Salassa syndrome.
    J Neurosurg 2017 Jan 13:1-11. Epub 2017 Jan 13.
    Department of Neurologic Surgery, and.
    OBJECTIVE Nelson-Salassa syndrome (NSS) is a rare consequence of bilateral adrenalectomy (ADX) for refractory hypercortisolism due to Cushing disease (CD). Although classically defined by rapid growth of a large, invasive, adrenocorticotropin hormone (ACTH)-secreting pituitary tumor after bilateral ADX that causes cutaneous hyperpigmentation, visual disturbance, and high levels of ACTH, clinical experience suggests more variability. METHODS The authors conducted a retrospective chart review of all patients 18 years and older with a history of bilateral ADX for CD, adequate pituitary MRI, and at least 2 years of clinical follow-up. Read More

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