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    Psychosis as a sequelae of paraneoplastic syndrome in Small- Cell Lung Carcinoma: A psycho-neuroendocrine interface.
    Clin Schizophr Relat Psychoses 2017 Nov 22. Epub 2017 Nov 22.
    Assistant Professor, Department of Psychiatry and Behavioral Neurobiology, University of Alabama, Birmingham.
    In 2013 more than 150,000 Americans died from all types of lung cancer. Small cell lung cancer (SCLC) represents about 13% of all lung cancers and is notoriously associated with paraneoplastic syndromes (PNS). Here we present an interesting case of psychosis associated with one such PNS-- ectopic Cushing syndrome of SCLC. Read More

    Failure to Thrive in the Context of Carney Complex.
    Horm Res Paediatr 2017 Nov 21. Epub 2017 Nov 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/aims: Carney complex (CNC) is a rare syndrome associated with multiple tumors and several other unique manifestations. We describe the clinical, genetic, and laboratory findings in a cohort of patients with CNC and failure to thrive (FTT).

    Methods: A retrospective case series of pediatric patients with CNC presenting with FTT. Read More

    Ann Endocrinol (Paris) 2017 Oct;78 Suppl 1:S11-S20
    Service d'endocrinologie, hôpital Haut-Lévèque, avenue Magellan, CHU de Bordeaux, 33600 Pessac, France.
    The 2017 Endocrine Society annual meeting included several communications and debates on the conservative adrenal surgery in bilateral hereditary pheochromocytomas (BHP), bilateral adrenal macronodular hyperplasia (BAMH) and primary hyperaldosteronism (PHA). The general principle is to preserve a part of the adrenal cortex to prevent the occurrence of a definitive adrenal insufficiency. In BHP, cortical sparing surgery allows more than 50% of patients to maintain normal corticotropic function at 10 years with a low recurrence rate (~ 10%). Read More

    Translational evidence of prothrombotic and inflammatory endothelial damage in Cushing Syndrome after remission.
    Clin Endocrinol (Oxf) 2017 Nov 20. Epub 2017 Nov 20.
    Group of Endocrine Disorders, IDIBAPS, Barcelona, Spain.
    Objective: Sustained evidence from observational studies indicates that after remission of Cushing Syndrome (CS) a cardiovascular risk phenotype persists. Here we performed a translational study in active CS and CS in remission to evaluate the subclinical cardiometabolic burden and to explore the direct proinflammatory and prothrombotic potential of their sera on the endothelium in an in vitro translational atherothrombotic cell model.

    Patients: Cross-sectional study. Read More

    Acromegaly and Cushing's syndrome caused by a neuroendocrine tumor arising within a sacrococcygeal teratoma.
    Clin Case Rep 2017 Nov 14;5(11):1768-1771. Epub 2017 Sep 14.
    Departments of Endocrinology and HistopathologyRoyal Devon and Exeter HospitalBarrack RoadExeterEX2 5DWUK.
    A 60-year-old man with a pre-existing stable sacrococcygeal teratoma developed acromegaly, ectopic Cushing's syndrome, and 5HIAA secretion. To our knowledge, this represents the first reported case of ACTH and serotonin secretion, and likely GHRH or GH cosecretion, from a sacrococcygeal teratoma in an adult. Read More

    Rare Presentation of Adrenocortical Carcinoma in a 4-Month-Old Boy.
    World J Oncol 2017 Jun 9;8(3):81-85. Epub 2017 Jun 9.
    Department of Pediatric Endocrinology, Infant and Children's Hospital of Brooklyn, Maimonides Medical Center, Brooklyn, NY, USA.
    Adrenocortical carcinoma (ACC) is a rare malignancy and even rarer in infancy. Most of these tumors in pediatric age group are hormonally active and predominantly present with virilization. Cortisol hypersecretion presenting as Cushing syndrome is extremely rare and seen in older age groups. Read More

    Endocr Pract 2017 Nov 16. Epub 2017 Nov 16.
    From: 1Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh.
    Objective: To evaluate the diagnostic efficacy of various screening tests for the diagnosis of Cushing's syndrome (CS).

    Methods: 35 patients of Cushing's syndrome and 16 patients of pseudo-Cushing's syndrome were enrolled. Assessment of 24h urinary free cortisol (UFC), late night salivary cortisol (LNSC), over night dexamethasone suppression test (ONDST), late night plasma cortisol (LNPC) and ACTH on outpatient basis, and during sleep as well as in awake state after 48 hours of hospital admission. Read More

    Adrenalectomy was recommended for patients with subclinical Cushing's syndrome due to adrenal incidentaloma.
    Cancer Biomark 2017 Oct 31. Epub 2017 Oct 31.
    Objective: To assess whether adrenalectomy may improve biochemical and metabolic impairment for patients with subclinical Cushing syndrome (SCS) due to adrenal incidentaloma (AI) compared with conservative management.

    Methods: A total of 87 patients with SCS due to AI in Peking Union Medical College Hospital between September 2011 and January 2016 have been treated. Forty-eight patients underwent laparoscopic adrenalectomy (operative group), whereas 39 were managed conservatively (control group). Read More

    Acute development of Cushing syndrome in an HIV-infected child on atazanavir/ritonavir based antiretroviral therapy.
    Endocrinol Diabetes Metab Case Rep 2017 28;2017. Epub 2017 Oct 28.
    Divisions of Infectious Diseases.
    An 11-year-old male with perinatally acquired human immune deficiency virus (HIV) infection on antiretroviral regimen, which included abacavir plus lamivudine (Epzicom), didanosine, ritonavir and atazanavir presented with bilateral axillary striae, increased appetite, fatigue, facial swelling and acute weight gain. Two months prior to presentation, the patient had received a diagnostic and therapeutic intra-articular triamcinolone injection in the knee for pain relief and subsequently became progressively swollen in the face, developed striae bilaterally at the axillae, experienced increased appetite, fatigue and an 8 pound weight gain. During the endocrine workup, suspicion for adrenal insufficiency prompted 24-h urine collection for free cortisol, which was found to be undetectable (below LLQ of 1. Read More

    Impact of hypercortisolism on skeletal muscle mass and adipose tissue mass in patients with adrenal adenomas.
    Clin Endocrinol (Oxf) 2017 Nov 8. Epub 2017 Nov 8.
    Department of Endocrinology, Metabolism University Hospital of Patras, Greece.
    Context: Abdominal visceral adiposity and central sarcopenia are markers of increased cardiovascular risk and mortality.

    Objective: To assess whether central sarcopenia and adiposity can serve as a marker of disease severity in patients with adrenal adenomas and glucocorticoid secretory autonomy.

    Design: Retrospective cohort study. Read More

    Iatrogenic Cushing Syndrome in a 47-Year-Old HIV-Positive Woman on Ritonavir and Inhaled Budesonide.
    J Int Assoc Provid AIDS Care 2017 Jan 1:2325957417736612. Epub 2017 Jan 1.
    1 Department of Internal Medicine, Eastern Virginia Medical School, Norfolk, VA, USA.
    Iatrogenic Cushing syndrome (CS) is a well-known complication of treating patients with systemic steroids. More rarely, it has been described in HIV-positive patients on ritonavir (RTV) while using the inhaled corticosteroid fluticasone, which is metabolized through the cytochrome P450 3A4 (CYP3A4) enzyme system. In the presence of RTV, a known CYP3A4 enzyme inhibitor, the interaction can result in impaired metabolism and systemic accumulation of inhaled fluticasone resulting in iatrogenic CS. Read More

    Functional pancreatic neuroendocrine tumour causing Cushing's syndrome: the effect of chemotherapy on clinical symptoms.
    Ecancermedicalscience 2017 13;11:773. Epub 2017 Oct 13.
    AC Camargo Cancer Center, Sao Paulo 01509-900, Brazil.
    Background: Neuroendocrine tumours (NETs) are a heterogeneous group of diseases that can originate from any part of the gastrointestinal tract, bronchi, thyroid and pancreas. These tumours may be functioning or not depending on their ability to produce active substances, such as adrenocorticotrophic hormone (ACTH). ACTH-producing pancreatic neuroendocrine tumours are rare, with limited data about effective antitumor therapies. Read More

    Secondary Hypertension: Discovering the Underlying Cause.
    Am Fam Physician 2017 Oct;96(7):453-461
    University of Alberta, Edmonton, Alberta, Canada.
    Most patients with hypertension have no clear etiology and are classified as having primary hypertension. However, 5% to 10% of these patients may have secondary hypertension, which indicates an underlying and potentially reversible cause. The prevalence and potential etiologies of secondary hypertension vary by age. Read More

    Efficacy of dexamethasone suppression test during the diagnosis of primary pigmented nodular adrenocortical disease in Chinese adrenocorticotropic hormone-independent Cushing syndrome.
    Endocrine 2017 Nov 1. Epub 2017 Nov 1.
    Department of Endocrinology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Key Laboratory of Endocrinology of National Health and Family Planning Commission, Beijing, China.
    Objective: To evaluate the cut-off value of the ratio of 24 h urinary free cortisol (24 h UFC) levels post-dexamethasone to prior-dexamethasone in dexamethasone suppression test (DST) during the diagnosis of primary pigmented nodular adrenocortical disease in Chinese adrenocorticotropic hormone-independent Cushing syndrome.

    Design: Retrospective study.

    Participants: The patients diagnosed with primary pigmented nodular adrenocortical disease (PPNAD, n = 25), bilateral macronodular adrenal hyperplasia (BMAH, n = 27), and adrenocortical adenoma (ADA, n = 84) were admitted to the Peking Union Medical College Hospital from 2001 to 2016. Read More

    Tumour Lateralization in Cushing's disease by Inferior Petrosal Sinus Sampling with desmopressin.
    Clin Endocrinol (Oxf) 2017 Oct 28. Epub 2017 Oct 28.
    Department of Neurosurgery, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No.1 Shuaifuyuan, Dongcheng District, Beijing, China.
    Background: Bilateral inferior petrosal sinus sampling (IPSS) with corticotropin-releasing hormone (CRH) is currently the gold standard in the diagnosis of Cushing's disease (CD) and has also been used in tumour lateralization. Our objective was to determine the diagnostic value and lateralization accuracy of IPSS with desmopressin.

    Methods: We retrospectively analyzed 91 patients with Cushing's syndrome who had either negative findings on pituitary dynamic enhanced magnetic resonance imaging (MRI) or non-suppressed high dose dexamethasone suppression tests (HDDST). Read More

    Tacrolimus Improves Symptoms of Children With Myasthenia Gravis Refractory to Prednisone.
    Pediatr Neurol 2017 Sep 5. Epub 2017 Sep 5.
    Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. Electronic address:
    Background: Myasthenia gravis tends to affect children in China. Oral pyridostigmine and prednisone could effectively improve the symptoms, but multiple side effects become a major concern after long-term oral prednisone. To avoid the long-term complications of prednisone therapy and to obtain more satisfactory improvement, we tested the efficacy and safety of tacrolimus in children with myasthenia gravis. Read More

    How useful is urinary-free cortisol in the clinic?
    Biomark Med 2017 Nov 17;11(11):1009-1016. Epub 2017 Oct 17.
    Hormones and Cancer Group, Garvan Institute of Medical Research, Sydney, Australia.
    Measurement of 24-h urine-free cortisol is frequently employed as a first-line screening and disease-monitoring test in Cushing's syndrome (CS). The quest for 'cortisol specificity' has seen the emergence of mass spectrometry (MS) based assays, particularly liquid chromatography/tandem mass spectrometry. In contrast to traditional immunoassays, liquid chromatography/tandem mass spectrometry 'free cortisol' measurement is less susceptible to 'interference' from cortisol precursors and metabolites. Read More

    Transient pituitary ACTH-dependent Cushing syndrome caused by an immune checkpoint inhibitor combination.
    Melanoma Res 2017 Dec;27(6):649-652
    aAP-HP, Department of Dermatology bINSERM_U976, Department of Dermatology cAP-HP, CIC Department dAP-HP, Department of Radiology, Saint-Louis Hospital eAP-HP, Department of Diabetes and Endocrinology, DHU FIRE, Lariboisiere Hospital fParis-Diderot, Sorbonne Paris Cité University gAP-HP, Department of Endocrinology, Bicetre Hospital, University Paris-Sud, Paris, France.
    Immune checkpoint inhibitors have improved survival in numerous advanced malignancies, but are associated with a number of immune-related adverse events, including endocrinopathies. Endogenous Cushing's syndrome (CS) is a rare disorder resulting from exposure to high levels of circulating cortisol. CS can be caused either by adrenal cortex tumors or hyperplasia or by pituitary or extra-pituitary tumors over-secreting ACTH (known as ACTH-dependent CS). Read More

    A Complicated Case of Resistant Hypertension.
    Acta Med Iran 2017 Aug;55(8):525-529
    Department of Cardio Thoracic Vascular, San Raffaele Scientific Institute, Milan, Italy.
    A 47-year-old woman presented with a history of resistant arterial hypertension, associated with disabling headache. She was subjected to an enormous number of tests in order to identify an underlying cause of secondary hypertension, such as pheochromocytoma or Cushing syndrome, but all the most common causes of secondary hypertension were investigated and gradually excluded. Factitious use of amphetamine or cocaine was excluded, and therapy compliance was verified by witnessed ingestion of drug therapy, in order to rule out Munchausen syndrome. Read More

    Iatrogenic Cushing syndrome in a child with congenital adrenal hyperplasia: Erroneous compounding of hydrocortisone.
    J Clin Endocrinol Metab 2017 Sep 28. Epub 2017 Sep 28.
    Steven and Alexandra Cohen Children's Medical Center of New York, New Hyde Park, NY 11040.
    Context: Patients with 21-hydroxylase deficiency congenital adrenal hyperplasia (CAH) require lifelong treatment with glucocorticoids. In growing children the drug of choice is hydrocortisone. Commercially available hydrocortisone tablets do not conform to very low doses prescribed to infants and toddlers, and compounded hydrocortisone is often dispensed to meet therapeutic needs. Read More

    Clinical utility of late-night and post-overnight dexamethasone suppression salivary cortisone for the investigation of Cushing's syndrome.
    Hong Kong Med J 2017 Oct 13. Epub 2017 Oct 13.
    Department of Medicine, Queen Elizabeth Hospital, Jordan, Hong Kong.
    Introduction: There is a pressing need to identify diagnostic testing for Cushing's syndrome that can be achieved with ease and at low cost. This study aimed to explore the usefulness of late-night and post-overnight 1-mg dexamethasone suppression salivary cortisone, as measured by liquid chromatography-tandem mass spectrometry, for investigation of hypercortisolism.

    Methods: Salivary cortisone data of subjects were investigated according to a pre-specified protocol. Read More

    Effects of Ketoconazole on the Pharmacokinetics of Mifepristone, a Competitive Glucocorticoid Receptor Antagonist, in Healthy Men.
    Adv Ther 2017 Oct 11;34(10):2371-2385. Epub 2017 Oct 11.
    MedVal Scientific Information Services, LLC, Princeton, NJ, USA.
    Introduction: Mifepristone, a competitive glucocorticoid receptor antagonist approved for Cushing syndrome, and ketoconazole, an antifungal and steroidogenesis inhibitor, are both inhibitors of and substrates for cytochrome P450 (CYP3A4). This study evaluated the pharmacokinetic effects of concomitant ketoconazole, a strong CYP3A4 inhibitor, on mifepristone.

    Methods: In an open-label, two-period, single-center study, healthy adult men received mifepristone 600 mg orally daily for 12 days (period 1) followed by mifepristone 600 mg daily plus ketoconazole 200 mg orally twice daily for 5 days (period 2). Read More

    [Adrenal disease diagnosis: management of adrenal incidentaloma].
    Rev Med Brux 2017 ;38(4):325-333
    Service d'Endocrinologie, Hôpital Erasme, ULB.
    Adrenal glands are specialized in biosynthesis of several hormones correlated to different clinical phenotypes in case of excess or lack of production. In addition to secretion disorders, tumors, secreting or not, can take place in adrenal glands. Incidentalomas are the most common adrenal diseases in clinical practice. Read More

    Genetic causes of functional adrenocortical adenomas.
    Endocr Rev 2017 Aug 2. Epub 2017 Aug 2.
    INSERM, UMRS_970, Paris Cardiovascular Research Center, Paris, France.
    Aldosterone and cortisol, the main mineralocorticoid and glucocorticoid hormones in humans, are produced in the adrenal cortex, which is composed of three concentric zone with specific functional characteristics. Adrenocortical adenomas (ACA) may lead to the autonomous secretion of aldosterone responsible for primary aldosteronism, the most frequent form of secondary arterial hypertension. In the case of cortisol production, ACA lead to overt or subclinical Cushing syndrome. Read More

    Assessment of Safety, Tolerability, Pharmacokinetics, and Pharmacological Effect of Orally Administered CORT125134: An Adaptive, Double-Blind, Randomized, Placebo-Controlled Phase 1 Clinical Study.
    Clin Pharmacol Drug Dev 2017 Oct 2. Epub 2017 Oct 2.
    Corcept Therapeutics, Menlo Park, CA, USA.
    CORT125134 is an orally active, high-affinity, selective antagonist of the glucocorticoid receptor that is being developed for indications that may benefit from the modulation of cortisol activity. This first-in-human study was conducted to evaluate the dose-related safety, tolerability, pharmacokinetics and pharmacological effects of CORT125134 and its active metabolite CORT125201. Eighty-one healthy male or female subjects received a single dose of 5 to 500 mg CORT125134 or matching placebo across 9 cohorts; 1 cohort received 150 mg CORT125134 after a high-fat breakfast; and 46 subjects received 50 to 500 mg CORT125134 or matching placebo once daily for up to 14 days across 4 cohorts. Read More

    Adulteration of proprietary Chinese medicines and health products with undeclared drugs: experience of a tertiary toxicology laboratory in Hong Kong.
    Br J Clin Pharmacol 2017 Oct 1. Epub 2017 Oct 1.
    Hospital Authority Toxicology Reference Laboratory, Princess Margaret Hospital, Hong Kong.
    Aims: Proprietary Chinese medicines (pCMs) and health products, generally believed to be natural and safe, are gaining popularity worldwide. However, the safety of pCMs and health products has been severely compromised by the practice of adulteration. The current study aimed to examine the problem of adulteration of pCMs and health products in Hong Kong. Read More

    The Eosinophil Count Tends to Be Negatively Associated with Levels of Serum Glucose in Patients with Adrenal Cushing Syndrome.
    Endocrinol Metab (Seoul) 2017 Sep;32(3):353-359
    Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea.
    Background: Cushing syndrome is characterized by glucose intolerance, cardiovascular disease, and an enhanced systemic inflammatory response caused by chronic exposure to excess cortisol. Eosinopenia is frequently observed in patients with adrenal Cushing syndrome, but the relationship between the eosinophil count in peripheral blood and indicators of glucose level in patients with adrenal Cushing syndrome has not been determined.

    Methods: A retrospective study was undertaken of the clinical and laboratory findings of 40 patients diagnosed with adrenal Cushing syndrome at Chungnam National University Hospital from January 2006 to December 2016. Read More

    A Long-Term Study of the Treatment of Nelson's Syndrome With Gamma Knife Radiosurgery.
    Neurosurgery 2017 Aug 5. Epub 2017 Aug 5.
    Department of Neurological Surgery, University of Virginia Health System, Charlottesville, Virginia.
    Background: Nelson's syndrome may be a devastating complication for patients with Cushing's disease who underwent a bilateral adrenalectomy. Previous studies have demonstrated that stereotactic radiosurgery (SRS) can be used to treat patients with Nelson's syndrome.

    Objective: To report a retrospective study of patients with Nelson's syndrome treated with Gamma Knife radiosurgery to evaluate the effect of SRS on endocrine remission and tumor control. Read More

    Preventing Cushing: Iatrogenic Cushing Syndrome due to Ritonavir-Fluticasone Interaction.
    Cureus 2017 Jul 17;9(7):e1484. Epub 2017 Jul 17.
    Department of Internal Medicine, Howard University Hospital.
    Ritonavir is commonly used in low doses to boost plasma levels of protease inhibitors in patients with human immunodeficiency virus (HIV) infections. It is also a potent inhibitor of cytochrome P450. We present a 50-year-old African American male with past medical history of HIV on highly active antiretroviral therapy (HAART), which also included ritonavir and long standing asthma that has been treated with inhaled fluticasone, who presented with back pain. Read More

    Corticotroph Pituitary Carcinoma in a Patient With Lynch Syndrome (LS) and Pituitary Tumors in a Nationwide LS Cohort.
    J Clin Endocrinol Metab 2017 Nov;102(11):3928-3932
    Department of Endocrinology, Skåne University Hospital, Malmö, University of Lund, 20502 Malmö, Sweden.
    Context: Lynch syndrome (LS) is a cancer-predisposing syndrome caused by germline mutations in genes involved in DNA mismatch repair (MMR). Patients are at high risk for several types of cancer, but pituitary tumors have not previously been reported.

    Case: A 51-year-old man with LS (MSH2 mutation) and a history of colon carcinoma presented with severe Cushing disease and a locally aggressive pituitary tumor. Read More

    Histone Deacetylase Inhibition Ameliorates Hypertension and Hyperglycemia in a model of Cushing's Syndrome.
    Am J Physiol Endocrinol Metab 2017 Sep 19:ajpendo.00267.2017. Epub 2017 Sep 19.
    Kyungpook National University
    Cushing's syndrome (CS) caused by hypercortisolism is occasionally accompanied by metabolic disorders such as hypertension, diabetes mellitus (DM), dyslipidemia and central obesity. Thus, morbidity and mortality, observed in cardiovascular disease, are elevated in patients with CS. We hypothesized that HDAC inhibition (HDACi) decreased transcriptional activity of glucocorticoid receptor (GR), which ameliorates hypertension and hyperglycemia in patients with CS. Read More

    Near-Fatal Gastrointestinal Hemorrhage in a Child with Medulloblastoma on High Dose Dexamethasone.
    Cureus 2017 Jul 7;9(7):e1442. Epub 2017 Jul 7.
    Division of Pediatric Critical Care, Department of Pediatrics, Stanford University School of Medicine and Lucile Packard Children's Hospital.
    A four-year-old female was admitted to a university-based children's hospital with a newly-diagnosed posterior fossa tumor. She was started on famotidine and high-dose dexamethasone and underwent gross total resection of a medulloblastoma. She was continued on dexamethasone and famotidine. Read More

    Age-dependent effects of Armc5 haploinsufficiency on adrenocortical function.
    Hum Mol Genet 2017 Sep;26(18):3495-3507
    Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
    Inactivating mutations in the Armadillo repeat-containing 5 (ARMC5) gene have recently been discovered in primary macronodular adrenal hyperplasia (PMAH), a cause of Cushing syndrome. Biallelic ARMC5 inactivation in PMAH suggested that ARMC5 may have tumor suppressor functions in the adrenal cortex. We generated and characterized a new mouse model of Armc5 deficiency. Read More

    [The localization diagnosis of patients with adrenocorticotropic hormone-dependent Cushing's syndrome in adolescence].
    Zhonghua Yi Xue Za Zhi 2017 Sep;97(34):2674-2677
    Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academe of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
    Objective: To elucidate the clinical characteristics and localization diagnosis of patients with adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS) in adolescence. Methods: The clinical data, laboratory examination and localization diagnosis from 35 patients aged less than 18 years old with adolescent CS who were treated at Peking Union Medical College Hospital between January 1990 and March 2012 were analyzed. Results: There were 29 cases of Cushing's disease (CD) and 6 cases of ectopic ACTH syndrome (EAS). Read More

    Accessory ovarian steroid cell tumor producing testosterone and cortisol: A case report.
    Medicine (Baltimore) 2017 Sep;96(37):e7998
    aDepartment of Endocrinology bDepartment of Gynecology cDepartment of Pathology, Chinese PLA General Hospital, Beijing, China.
    Rationale: An accessory ovary is a rare structure containing normal ovarian tissue, which has a direct or ligamentous connection with a normal and eutopic ovary.

    Patient Concerns: In the study, we reported a 46-year-old woman presented with secondary amenorrhea and virilization symptoms for 1 year.

    Diagnoses: Endocrine evaluation revealed slightly elevated serum cortisol, extremely elevated 24-hour urinary-free cortisol and serum testosterone. Read More

    Iatrogenic Adrenal Insufficiency Secondary to Combination Therapy with Elvitegravir/Cobicistat/Tenofovir Disoproxil Fumarate/Emtricitabine and Interlaminar Triamcinolone Injection in an AIDS Patient.
    J Int Assoc Provid AIDS Care 2017 Jan 1:2325957417729748. Epub 2017 Jan 1.
    1 Department of Pharmacy, Kingsbrook Jewish Medical Center, Brooklyn, NY, USA.
    We report the first identified case of suspected iatrogenic adrenal insufficiency after an interlaminar injection of triamcinolone acetonide while on concomitant Stribild (elvitegravir 150 mg/cobicistat 150 mg/tenofovir disoproxil fumarate 300 mg/emtricitabine 200 mg [EVG/c/TDF/FTC]). A 49-year-old female with HIV on EVG/c/TDF/FTC therapy presented to our endocrinology clinic to be evaluated for suspected Cushing syndrome. Prior to presentation, the patient had been given 2 interlaminar spinal injections of triamcinolone. Read More

    Paraneoplastic Cushing syndrome, case-series and review of the literature.
    Acta Clin Belg 2017 Sep 12:1-7. Epub 2017 Sep 12.
    a Departments of Respiratory Medicine and Endocrinology , AZ Sint-Jan Hospital , Bruges , Belgium.
    Objectives: Paraneoplastic Cushing syndrome is a rare condition, representing a small fraction of the adrenocorticotropic hormone (ACTH)-dependent cases of Cushing syndrome Methods: Four case descriptions and literature review, highlighting the diagnostic challenges and treatment options are presented.

    Results: Different tumor types can be associated with ectopic ACTH secretion. The most common types are bronchial carcinoids and small cell lung carcinoma (SCLC). Read More

    Endocrine Surgery: A Hopkins Legacy.
    Ann Surg 2017 Sep 6. Epub 2017 Sep 6.
    Director of the Endocrine Neoplasia Institute, Miami Cancer Institute, Miami, FL.
    : The field of Endocrine Surgery is linked to extraordinary contributions made by Hopkins leaders in surgery including William Stewart Halsted, Harvey Cushing, and John L Cameron. Halsted's contributions to the anatomic basis of thyroid and parathyroid surgery were based on his experimental and clinical work performed at Johns Hopkins Hospital. Halsted's disciple, Harvey Cushing, created the field of modern neurosurgery and recognized the disease and syndrome that are immortalized with his name. Read More

    A Novel PRKAR1A Mutation Identified in a Patient with Isolated Primary Pigmented Nodular Adrenocortical Disease.
    Case Rep Oncol 2017 May-Aug;10(2):769-776. Epub 2017 Aug 16.
    Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
    Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing syndrome, especially the isolated form without Carney complex, associated with germline mutations in PRKAR1A, the protein kinase A regulatory subunit type 1 alpha gene. We report a 31-year-old female who presented with secondary amenorrhea, cushingoid appearance, and hypertension without Carney complex. Biochemical laboratory examinations confirmed the ACTH-independent adrenal Cushing syndrome with negative Liddle test. Read More

    Optimal follow-up strategies for adrenal incidentalomas: reappraisal of the 2016 ESE-ENSAT guidelines in real clinical practice.
    Eur J Endocrinol 2017 Dec 4;177(6):475-483. Epub 2017 Sep 4.
    Department of Internal MedicineSeoul National University College of Medicine, Seoul, South Korea.
    Objective: Recently, the European Society of Endocrinology (ESE), in collaboration with the European Network for the Study of Adrenal Tumors (ENSAT), asserted that adrenal incidentalomas (AIs) <4 cm and ≤10 Hounsfield units (HU) do not require further follow-up imaging. To validate the clinical application of the follow-up strategies suggested by the 2016 ESE-ENSAT guidelines, we explored the clinical characteristics and natural course of AIs in a single center over 13 years.

    Design And Methods: This retrospective cohort study included a total of 1149 patients diagnosed with AIs between 2000 and 2013 in a single tertiary center. Read More

    Genetics of Cushing's disease.
    Clin Endocrinol (Oxf) 2017 Aug 29. Epub 2017 Aug 29.
    Medizinische Klinik und Poliklinik IV, Klinikum der Universität, Ludwig-Maximilians-Universität München, Munich, Germany.
    Cushing's disease (CD) is a rare disabling condition caused by Adrenocorticotropic hormone (ACTH)-secreting adenomas of the pituitary. The majority of corticotropic adenomas are monoclonal and occur sporadically. Only rarely does CD arise in the context of genetic familial syndromes. Read More

    Baseline Demographic and Clinical Characteristics of Patients with Adrenal Incidentaloma from a Single Center in China: A Survey.
    Int J Endocrinol 2017 7;2017:3093290. Epub 2017 Aug 7.
    Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
    Aim: To investigate the clinical and endocrinological characteristics of patients with adrenal incidentaloma (AI).

    Materials And Methods: This retrospective study enrolled 1941 AI patients hospitalized at the Department of Endocrinology, Chinese PLA General Hospital, Beijing, China, between January 1997 and December 2016. The patient gender, age at visits, imaging features, functional status, and histological results were analyzed. Read More

    Otolaryngic manifestations of Cushing disease.
    Ear Nose Throat J 2017 Aug;96(8):E28-E30
    Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, 10833 Le Conte Ave., CHS 62-132, Los Angeles, CA 90095-1624, USA.
    Cushing disease is a relatively rare cause of Cushing syndrome secondary to a hyperfunctioning pituitary adenoma. In addition to signs and symptoms of hypercortisolism, Cushing disease may present with diverse otolaryngic manifestations, which may guide diagnosis and management. We performed a retrospective chart review of patients who were found to have Cushing disease and who underwent transnasal transsphenoidal surgery for pituitary adenomas between January 1, 2007, and July 1, 2014, at a tertiary academic medical center. Read More

    Lipofuscin Accumulation in Cortisol-Producing Adenomas With and Without PRKACA Mutations.
    Horm Metab Res 2017 Oct 23;49(10):786-792. Epub 2017 Aug 23.
    Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland 20892, USA.
    The adrenal cortex accumulates lipofuscin granules with age. Lipofuscin accumulation is also seen in adrenocortical tumors associated with Cushing syndrome (CS), particularly those with PRKAR1A mutations, such as in primary pigmented nodular adrenocortical disease (PPNAD). We investigated the presence of lipofuscin in cortisol-producing adenomas (CPAs) responsible for CS with and without the PRKACA (pLeu206Arg) somatic mutation. Read More

    Cushing's syndrome secondary to typical pulmonary carcinoid with mutation in BCOR gene: A case report.
    Medicine (Baltimore) 2017 Aug;96(34):e7870
    aDepartment of Thoracic Surgery, the Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou b3D Medicines Corporation, Shanghai, China.
    Rationale: Typical pulmonary carcinoid is a kind of low-grade malignancy neuroendocrine tumor. Cushing's syndrome is a very rare clinical feature of typical pulmonary carcinoid caused by hypercorticism. Complete tumor resection is the standard curative treatment for primary typical pulmonary carcinoid. Read More

    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: an observational case series.
    Eur J Endocrinol 2017 Nov 17;177(5):439-444. Epub 2017 Aug 17.
    Brigham and Women's Hospital/Dana-Farber Cancer InstituteHarvard Medical School, Boston, Massachusetts, USA
    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 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 Nov 17;177(5):K13-K20. Epub 2017 Aug 17.
    Department of Internal MedicineErasmus Medical Center, Rotterdam, The 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

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