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    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 Mar 22. Epub 2017 Mar 22.
    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

    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 Feb 26:1-4. 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 Feb 22. 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 Feb 15. Epub 2017 Feb 15.
    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 Jan 28. 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

    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

    Long-Term Control of Hypercortisolism by Vandetanib in a Case of Medullary Thyroid Carcinoma with a Somatic RET Mutation.
    Thyroid 2017 Apr 13;27(4):587-590. Epub 2017 Mar 13.
    1 Department of Endocrinology, Cochin Hospital , Assistance Publique Hôpitaux de Paris, Paris, France .
    Background: Medullary thyroid carcinomas (MTCs) complicated by ectopic Cushing's syndrome (CS) have a poor prognosis, partially due to the difficulty in controlling hypercortisolism by adrenal blocking drugs. Recent reports (including the initial follow-up of this patient) have suggested that tyrosine kinase inhibitors (TKIs) may be a therapeutic option due to an anti-secretory action on ACTH. However, there is a lack of long-term follow-up studies. Read More

    Ritonavir and Topical Ocular Corticosteroid Induced Cushing's Syndrome in an Adolescent With HIV-1 Infection.
    Pediatr Infect Dis J 2017 May;36(5):502-503
    From the *University Hospital Southampton, Southampton, Hampshire, United Kingdom; †NHS Foundation Trust, United Kingdom; ‡Imperial College London, Kensington, London, United Kingdom; §Portsmouth Hospitals, Portsmouth, United Kingdom; and ¶Poole Hospitals, Poole, United Kingdom.
    Cushing's syndrome after topical ocular corticosteroid use is extremely rare. We describe a case of symptomatic Cushing's syndrome in an adolescent male with sight-threatening vernal keratoconjunctivitis on antiretroviral therapy for HIV-1 infection that included ritonavir, a potent cytochrome p450 CYP3A4 inhibitor. CYP3A4 inhibition reduces the metabolism of exogenous corticosteroids leading to suppression of endogenous steroid production and Cushing's syndrome. Read More

    Avascular Necrosis of the Hips With Increased Activity on 68Ga-DOTATATE PET/CT.
    Clin Nucl Med 2017 Mar;42(3):214-215
    From the *Radiology and Imaging Sciences, Warren Grant Magnuson Clinical Center (CC), Bethesda; †PET Department, Clinical Center, National Institutes of Health (NIH), Bethesda, MD; ‡Department of Radiology, Medical School, University of Crete, Crete, Greece; and §Center for Research in Computer Vision (CRCV), Electrical and Computer Science Department, University of Central Florida (UCF), Orlando, FL.
    Prolonged exposure to cortisol is one of the major causes of avascular bone necrosis (AVN). We report on a case of a woman with Cushing syndrome attributed to ectopic adrenocorticotropic hormone-secreting tumor who was evaluated with whole-body PET/CT study using Ga-DOTATATE. The scan showed increased activity by both femoral heads, corresponding to the margins of bilateral AVN seen on MRI. Read More

    Adrenocorticotropic hormone levels before treatment predict recurrence of Cushing's disease.
    J Formos Med Assoc 2016 Oct 28. Epub 2016 Oct 28.
    Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan; National Taiwan University College of Medicine, Taipei, Taiwan; Center of Anti-aging and Health Consultation, National Taiwan University Hospital, Taipei, Taiwan. Electronic address:
    Background/purpose: Cushing's disease (CD) is the most common cause of endogenous Cushing's syndrome. Transsphenoidal surgery (TSS) is the first choice of treatment. Predicting prognosis after treatment can benefit further strategies of management, but currently there is no convenient predictor. Read More

    The Recovery of Hypothalamic-Pituitary-Adrenal Axis Is Rapid in Subclinical Cushing Syndrome.
    Endocrinol Metab (Seoul) 2016 Dec;31(4):592-597
    Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.
    Background: In subclinical Cushing syndrome (SC), it is assumed that glucocorticoid production is insufficient to cause a clinically recognizable syndrome. Differences in hormonal levels or recovery time of the hypothalamic-pituitary-adrenocortical (HPA) axis after adrenalectomy between patients with overt Cushing syndrome (OC) and SC remain unknown.

    Methods: Thirty-six patients (10 with OC and 26 with SC) with adrenal Cushing syndrome who underwent adrenalectomy from 2004 to 2014 were reviewed retrospectively. Read More

    Choroidal and Retinal Abnormalities by Optical Coherence Tomography in Endogenous Cushing's Syndrome.
    Front Endocrinol (Lausanne) 2016 9;7:154. Epub 2016 Dec 9.
    Department of Endocrinology, University of São Paulo Medical School , São Paulo, São Paulo , Brazil.
    Context: Cortisol has been suggested as a risk factor for choroidal thickening, which may lead to retinal changes.

    Objective: To compare choroidal thickness measurements using optical coherence tomography (OCT) in patients with endogenous active Cushing's syndrome (CS) and to evaluate the occurrence of retinal abnormalities in the same group of patients.

    Design: Cross-sectional study. Read More

    Hypertension: The role of biochemistry in the diagnosis and management.
    Clin Chim Acta 2017 Feb 19;465:131-143. Epub 2016 Dec 19.
    Dept. of Clinical Biochemistry & Diagnostic Endocrinology, Mater Misericordiae University Hospital, Dublin, Ireland.
    Hypertension is defined as a persistently elevated blood pressure ≥140/90mmHg. It is an important treatable risk factor for cardiovascular disease, with a high prevalence in the general population. The most common cause, essential hypertension, is a widespread disease - however, secondary hypertension is under investigated and under diagnosed. Read More

    Endoscopic Endonasal Surgery for Remission of Cushing Disease Caused by Ectopic Intracavernous Macroadenoma: Case Report and Literature Review.
    World Neurosurg 2017 Feb 18;98:870.e5-870.e10. Epub 2016 Dec 18.
    Department of Otolaryngology, University of Louisville, Louisville, Kentucky, USA.
    Background: Complete surgical resection of an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is the gold standard of treatment of Cushing disease. Ectopic location of these adenomas is an extremely rare condition that may compromise the diagnosis and surgical success. We present the first case of an ectopic intracavernous ACTH-secreting macroadenoma totally resected with endoscopic endonasal surgery (EES). Read More

    Clinical characteristics of adrenal tumors in children: a retrospective review of a 15-year single-center experience.
    Int Urol Nephrol 2017 Mar 17;49(3):381-385. Epub 2016 Dec 17.
    Department of Pediatric Surgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.
    Objective: Adrenal tumors are rare in children. The aim of this study is to review and analyze clinical data on the diagnosis and management of adrenal tumors in children.

    Methods: Between 2001 and 2015, 48 pediatric patients (<14 years old) admitted to our institute with adrenal tumors were reviewed. Read More

    Prevention and management of glucocorticoid-induced side effects: A comprehensive review: Gastrointestinal and endocrinologic side effects.
    J Am Acad Dermatol 2017 Jan;76(1):11-16
    Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:
    Part 2 of this 4-part continuing medical education series continues with a discussion of the prevention and management of gastrointestinal side effects associated with corticosteroid use, including peptic ulcer disease, gastrointestinal bleeding, and pancreatitis, followed by a review of corticosteroid-related endocrinologic side effects, such as diabetes, adrenal suppression, and Cushing syndrome. Read More

    Diagnosis of Cushing's disease in a patient with consistently normal urinary free cortisol levels: a case report.
    Clin Case Rep 2016 Dec 9;4(12):1181-1183. Epub 2016 Nov 9.
    Seattle Pituitary Center Swedish Neuroscience Institute Seattle Washington USA.
    The urinary free cortisol (UFC) test is widely used for the screening of Cushing's syndrome. This case study illustrates the potential failure of the UFC test to correctly diagnose Cushing's disease (CD), indicating that the use of other complementary tests may be necessary to diagnose this disease in some cases. Read More

    Hypokalemia associated with a solitary pulmonary nodule: A case report.
    Medicine (Baltimore) 2016 Dec;95(50):e5046
    aDepartment of Internal Medicine bEndocrine and Metabolism Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
    Background: Differential diagnosis of hypokalemia and adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome often presents challenging in endocrinology and requires careful clinical, biochemical, radiological, and pathological investigations. Hypokalemia is a common abnormality and systematic approach is required to avoid delays in diagnosis of important underlying causes.

    Case Summary & Conclusion: A 49-year-old woman presented with moderate hypokalemia. Read More

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