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    DIAGNOSIS OF ENDOCRINE DISEASE: The role of the desmopressin test in the diagnosis and follow-up of Cushing's syndrome.
    Eur J Endocrinol 2018 Feb 22. Epub 2018 Feb 22.
    S Tsagarakis, Department of Endocrinology, Diabetes and Metabolism, Evangelismos Hospital, Athens, Greece
    Desmopressin is a vasopressin analogue selective for type 2 vasopressin receptors that mediate renal water retention. In contrast to the native hormone arginine-vasopressin, a well-known ACTH secretagogue, desmopressin exerts minimal or no activity on ACTH excretion. However, in a substantial proportion of patients with ACTH-dependent Cushing's syndrome (CS), desmopressin elicits an ACTH and cortisol response, which contrasts with the minimal responses obtained in healthy subjects. Read More

    Incidence of Autoimmune and Related Disorders After Resolution of Endogenous Cushing Syndrome in Children.
    Horm Metab Res 2018 Feb 19. Epub 2018 Feb 19.
    Section on Endocrinology and Genetics, Eunice Kennedy Shriver Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland, USA.
    Glucocorticoids are widely used for immunosuppression in autoimmune diseases. After the resolution of hypercortisolemia, the immune system recovers allowing for autoimmune diseases to manifest. Here we investigated the presence of autoimmune and related diseases that developed after cure of endogenous Cushing syndrome (CS) in children. Read More

    The Utility of Preoperative ACTH/Cortisol Ratio for the Diagnosis and Prognosis of Cushing's Disease.
    J Neurosci Rural Pract 2018 Jan-Mar;9(1):106-111
    Department of Neurosurgery, Faculty of Medicine, Kocaeli University, Umuttepe Kocaeli, Turkey.
    Purpose: Cushing's syndrome (CS) is a rare disease having diagnostic difficulties. Many diagnostic tests have been defined but none of these are diagnostic alone. Determination of the cause is another problem which sometimes requires more sophisticated and invasive procedures. Read More

    Hypokalemia associated with pseudo-Cushing's syndrome and magnesium deficiency induced by chronic alcohol abuse.
    CEN Case Rep 2018 Feb 15. Epub 2018 Feb 15.
    Division of Diabetes, Endocrinology and Metabolism, Department of Internal Medicine, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo, 663-8501, Japan.
    Hypokalemia and hypomagnesemia are frequently observed in patients with chronic alcoholism. However, the involvement of deranged cortisol regulation in patients with those conditions has not been reported. A 63-year-old Japanese male with chronic alcoholism was referred to the Department of Diabetes, Endocrinology and Metabolism for examination and treatment of hypokalemic periodic paralysis. Read More

    Iatrogenic Cushing's syndrome and adrenal insufficiency in infants on intranasal dexamethasone drops for nasal obstruction - Case series and literature review.
    Int J Pediatr Otorhinolaryngol 2018 Feb 11;105:123-126. Epub 2017 Nov 11.
    Weill Cornell Medical College, 428 East 72nd Street, Oxford Building, Suite 100, New York, NY 10021, USA. Electronic address:
    The use of intranasal steroid drops for nasal obstruction in infants is common practice and can prevent more invasive surgical procedures; however, it is not without complication. We describe 2 cases of iatrogenic Cushing's secondary to nasal steroids in infants with nasal obstruction, discuss the etiology of this unusual complication, and review previous literature reports. While reporting in the literature is sparse, these cases highlight the risk of development of adrenal insufficiency with usage of nasal steroid drops in infants as well as the need for close monitoring of administration and tapering of the drops. Read More

    Cushing's disease: major difficulties in diagnosis and management during pregnancy.
    Minerva Endocrinol 2018 Feb 13. Epub 2018 Feb 13.
    Endocrinology/Medicine Department, Hospital Sant Pau, Barcelona, Spain -
    Pregnancy in women with a diagnosis of Cushing' syndrome (CS) is an extremely rare event and its diagnosis and treatment are a real medical challenge. During pregnancy, the hypothalamus-pituitary-adrenal axis undergoes major changes leading to a significant increase in plasma cortisol levels throughout gestation. The difficulties in diagnosis are related to the resemblance of symptoms of CS and those of pregnancy, and to the complex interpretation of the screening tests. Read More

    Differentiation of pathologic/neoplastic hypercortisolism (Cushing syndrome) from physiologic/non-neoplastic hypercortisolism (formerly known as Pseudo-Cushing syndrome): response to Letter to the Editor.
    Eur J Endocrinol 2018 Mar;178(3):L3
    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.

    Testosterone- and Cortisol-secreting Oncocytic Adrenocortical Adenoma in the Pediatric Age-group.
    Pediatr Dev Pathol 2018 Jan 1:1093526617753045. Epub 2018 Jan 1.
    3 College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.
    Oncocytic tumors are epithelial neoplasms that occur in various organs, including adrenal glands. Oncocytic adrenocortical adenomas and carcinomas are uncommon but well-known pathological entities in adults. However, generally oncocytic tumors, particularly in the adrenal glands, are very rare in the pediatric age-group. Read More

    Neonatal Cushing Syndrome: A Rare but Potentially Devastating Disease.
    Clin Perinatol 2018 Mar 12;45(1):103-118. Epub 2017 Dec 12.
    Section on Endocrinology and Genetics, Developmental Endocrine Oncology and Genetics Group, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), NIH-Clinical Research Center, 10 Center Drive, Building 10, Room 1-3330, MSC1103, Bethesda, MD 20892, USA; Pediatric Endocrinology Inter-Institute Training Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), NIH-Clinical Research Center, 10 Center Drive, Building 10, Room 1-3330, MSC1103, Bethesda, MD 20892, USA. Electronic address:
    Neonatal Cushing syndrome (CS) is most commonly caused by exogenous administration of glucocorticoids and rarely by endogenous hypercortisolemia. CS owing to adrenal lesions is the most common cause of endogenous CS in neonates and infants, and adrenocortical tumors (ACTs) represent most cases. Many ACTs develop in the context of a TP53 gene mutation, which causes Li-Fraumeni syndrome. Read More

    Carney complex with PRKAR1A gene mutation: A case report and literature review.
    Medicine (Baltimore) 2017 Dec;96(50):e8999
    Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing.
    Rationale: Carney complex (CNC) is a multiple neoplasia syndrome with autosomal dominant inheritance. CNC is characterized by the presence of myxomas, spotty skin pigmentation, and endocrine overactivity. No direct correlation has been established between disease-causing mutations and phenotype. Read More

    [Endocrine paraneoplastic syndromes].
    Internist (Berl) 2018 Feb;59(2):125-133
    Medizinische Klinik IV, Klinikum der Universität München, Ziemssenstr. 1, 80336, München, Deutschland.
    Endocrine paraneoplastic syndromes result from the production of bioactive substances from neoplastic cells, of endocrine or neuroendocrine origin. Typically these are located in the lungs, the gastrointestinal tract, pancreas, thyroid gland, adrenal medulla, skin, prostate or breast. In endocrine paraneoplastic syndromes the secretion of peptides, amines or other bioactive substances is always ectopic and not related to the anatomical source. Read More

    Case report of a bilateral adrenal myelolipoma associated with Cushing disease.
    Medicine (Baltimore) 2017 Dec;96(52):e9455
    Division of Endocrinology and Metabolism, Departments of Internal Medicine.
    Rationale: Adrenal myelolipomas are rare benign tumors, composed of a variable mixture of mature adipose tissue and hematopoietic tissue. These tumors are frequently detected incidentally and are usually asymptomatic, and hormonally inactive.

    Patient Concerns: During a routine health checkup, a 52-year-old man was found to have a tumor on the bilateral adrenal glands. Read More

    Brain Atrophy and Hypomyelination Associated with Iatrogenic Cushing Syndrome in an Infant.
    Iran J Child Neurol 2018 ;12(1):101-104
    Department of Pediatric Radiology, Trakya University, Faculty of Medicine, Edirne, Turkey.
    Prolonged use of topical corticosteroids, particularly in infants, albeit rare, may lead to Cushing syndrome. Central nervous system abnormalities including brain atrophy and delayed myelination on cranial magnetic resonance imaging has been reported in patients with corticosteroid treatment. We herein report a 5-month-old female infant referred to Department of Pediatric Endocrinology, Edirne, Turkey with brain atrophy and myelination delay that might be due to iatrogenic Cushing syndrome caused by topical corticosteroid use. Read More

    Niche point-of-care endocrine testing - Reviews of intraoperative parathyroid hormone and cortisol monitoring.
    Crit Rev Clin Lab Sci 2018 Mar 22;55(2):115-128. Epub 2018 Jan 22.
    b Laboratory Medicine and Pathology , Mayo Clinic , Rochester , MN , USA.
    Point-of-care (POC) testing, which provides quick test results in near-patient settings with easy-to-use devices, has grown continually in recent decades. Among near-patient and on-site tests, rapid intraoperative and intra-procedural assays are used to quickly deliver critical information and thereby improve patient outcomes. Rapid intraoperative parathyroid hormone (ioPTH) monitoring measures postoperative reduction of parathyroid hormone (PTH) to predict surgical outcome in patients with primary hyperparathyroidism, and therefore contributes to the change of parathyroidectomy to a minimally invasive procedure. Read More

    Effects of Mifepristone on Nonalcoholic Fatty Liver Disease in a Patient with a Cortisol-Secreting Adrenal Adenoma.
    Case Rep Endocrinol 2017 19;2017:6161348. Epub 2017 Nov 19.
    Corcept Therapeutics, 149 Commonwealth Drive, Menlo Park, CA 94025, USA.
    Cushing syndrome (CS), a complex, multisystemic condition resulting from prolonged exposure to cortisol, is frequently associated with nonalcoholic fatty liver disease (NAFLD). In patients with adrenal adenoma(s) and NAFLD, it is essential to rule out coexisting endocrine disorders like CS, so that the underlying condition can be properly addressed. We report a case of a 49-year-old woman with a history of hypertension, prediabetes, dyslipidemia, biopsy-confirmed steatohepatitis, and benign adrenal adenoma, who was referred for endocrine work-up for persistent weight gain. Read More

    Adrenal Oncocytoma - A Rare Functional Tumor Presenting as Cushing Syndrome.
    J Assoc Physicians India 2017 Dec;65(12):100-101
    Clinical Associate, Medicine, Saifee Hospital, Mumbai, Maharashtra.
    Adrenal oncocytoma is very rare pathological variant of adrenal neoplasm. These are usually large and non-functional; however, rarely functional adrenal oncocytomas are also presented as Cushing's syndrome and pheochromocytoma. We report a case of adrenal oncocytoma in 38 year old female presented with symptoms of Cushing Syndrome. Read More

    Single center experience with laparoscopic adrenalectomy on a large clinical series.
    BMC Surg 2018 Jan 11;18(1). Epub 2018 Jan 11.
    Endocrine Surgery Unit, University of Perugia, Piazza dell'Università, 06123, Perugia, Italy.
    Background: Laparoscopic adrenalectomy is considered the gold standard technique for the treatment of benign small and medium size adrenal masses (<6 cm), due to low morbidity rate, short hospitalization and patient rapid recovery. The aim of our study is to analyse the feasibility and efficiency of this surgical approach in a broad spectrum of adrenal gland pathologies.

    Methods: Pre-operative, intra-operative and post-operative data from 126 patients undergone laparoscopic adrenalectomy between January 2003 and December 2015 were retrospectively collected and reviewed. Read More

    Exogenous Cushing syndrome due to misuse of potent topical steroid.
    Pediatr Dermatol 2018 Jan 9. Epub 2018 Jan 9.
    Division of Pediatric Endocrinology, School of Medicine, Trakya University, Edirne, Turkey.
    We report an infant with exogenous Cushing syndrome after being treated for 2 months with a potent topical corticosteroid via the mother's application of topical clobetasol for diaper rash without a prescription. We emphasize that potent topical steroids should be used with great caution, especially when used under occlusion (e.g. Read More

    Pharmacophore Modeling andScreening for Human Cytochrome P450 11B1 and Cytochrome P450 11B2 Inhibitors.
    Front Chem 2017 19;5:104. Epub 2017 Dec 19.
    Institute of Pharmacy - Pharmaceutical Chemistry and Center for Molecular Biosciences Innsbruck (CMBI), University of Innsbruck, Innsbruck, Austria.
    Cortisol synthase (CYP11B1) is the main enzyme for the endogenous synthesis of cortisol and its inhibition is a potential way for the treatment of diseases associated with increased cortisol levels, such as Cushing's syndrome, metabolic diseases, and delayed wound healing. Aldosterone synthase (CYP11B2) is the key enzyme for aldosterone biosynthesis and its inhibition is a promising approach for the treatment of congestive heart failure, cardiac fibrosis, and certain forms of hypertension. Both CYP11B1 and CYP11B2 are structurally very similar and expressed in the adrenal cortex. Read More

    LC-MSMS assays of urinary cortisol, a comparison between four in-house assays.
    Clin Chem Lab Med 2018 Jan 8. Epub 2018 Jan 8.
    Department of Biochemistry, INSERM U1235 University Hospital of Nantes, Nantes, France.
    Background: Twenty-four hour urinary free cortisol (UFC) determination can be used for screening and follow-up of Cushing syndrome (CS). As immunoassay methods lack specificity for UFC measurement, the use of high-performance liquid chromatography coupled to mass spectrometer (LC-MSMS) is recommended. The aim of our study was to compare UFC results using four LC-MSMS methods performed in four independent laboratories in order to evaluate interlaboratory agreement. Read More

    Histone deacetylase inhibition attenuates hepatic steatosis in rats with experimental Cushing's syndrome.
    Korean J Physiol Pharmacol 2018 Jan 22;22(1):23-33. Epub 2017 Dec 22.
    Department of Pharmacology, Kyungpook National University School of Medicine, Daegu 41944, Korea.
    Cushing's syndrome (CS) is a collection of symptoms caused by prolonged exposure to excess cortisol. Chronically elevated glucocorticoid (GC) levels contribute to hepatic steatosis. We hypothesized that histone deacetylase inhibitors (HDACi) could attenuate hepatic steatosis through glucocorticoid receptor (GR) acetylation in experimental CS. Read More

    Reduced-Intensity Allogeneic Transplant for Acute Myeloid Leukemia and Myelodysplastic Syndrome Using Combined CD34-Selected Haploidentical Graft and a Single Umbilical Cord Unit Compared with Matched Unrelated Donor Stem Cells in Older Adults.
    Biol Blood Marrow Transplant 2017 Dec 27. Epub 2017 Dec 27.
    Section of Hematology/Oncology, Department of Medicine, University of Chicago, Chicago, Illinois. Electronic address:
    Haplo/cord transplantation combines an umbilical cord blood (UCB) graft with CD34-selected haploidentical cells and results in rapid hematopoietic recovery followed by durable UCB engraftment. We compared outcomes of transplants in older patients with acute myeloid leukemia (AML) or high-risk myelodysplastic syndromes (MDS) who received either HLA-matched unrelated donor (MUD) cells or haplo/cord grafts. Between 2007 and 2013, 109 adults ages 50 and older underwent similar reduced-intensity conditioning with fludarabine and melphalan and antibody-mediated T cell depletion for AML (n = 83) or high-risk MDS (n = 26) followed by either a MUD (n = 68) or haplo/cord (n = 41) graft. Read More

    Comparison of hydrocortisone and prednisone in the glucocorticoid replacement therapy post-adrenalectomy of Cushing's Syndrome.
    Oncotarget 2017 Dec 31;8(62):106113-106120. Epub 2017 Aug 31.
    University of the District of Columbia, Washington, DC, USA.
    Cushing's syndrome requires glucocorticoid replacement following adrenalectomy. Based on a simplified glucocorticoid therapy scheme and the peri-operative observation, we investigated its efficacy and safety up to 6 months post-adrenalectomy in this cohort study. We found the adrenocorticotropic hormone (ACTH) levels were normal post-adrenalectomy, and sufficient to stimulate the recovery of the dystrophic adrenal cortex, thus exogenous supplemental ACTH might not be necessary. Read More

    The adrenal gland microenvironment in health, disease and during regeneration.
    Hormones (Athens) 2017 Jul;16(3):251-265
    Department of Internal Medicine III, Technische Universität Dresden, Dresden, Germany, Department of Endocrinology and Diabetes, King's College London, London, UK.
    The adrenal gland is a key component of the stress system in the human body. Multiple direct and paracrine interactions between different cell types and their progenitors take place within the adrenal gland microenvironment. These unique interactions are supported by high vascularization and the adrenal cortex extracellular matrix. Read More

    CHOROIDAL THICKENING AND PACHYCHOROID IN CUSHING SYNDROME: Correlation With Endogenous Cortisol Level.
    Retina 2017 Dec 20. Epub 2017 Dec 20.
    Departments of Ophthalmology, and.
    Purpose: To investigate subfoveal choroidal thickness and pachychoroid and their correlation with hormone level in patients with endogenous Cushing syndrome (CS).

    Methods: We enrolled a consecutive series of patients with CS and healthy controls. All participants had swept-source optical coherence tomography. Read More

    Falsely Elevated Steroid Hormones in a Postmenopausal Woman Due to Laboratory Interference.
    J Endocr Soc 2017 Aug 23;1(8):1062-1066. Epub 2017 Jun 23.
    Department of Obstetrics and Gynecology, Division of Reproductive Endocrinology & Infertility, University of Texas Southwestern Medical Center, Dallas, Texas 75390.
    Laboratory interference is a drawback in hormonal testing, and clinicians should have a high index of suspicion when faced with biochemical results discordant with the patient's clinical manifestations. A 62-year-old postmenopausal woman initially consulted her primary care physician for mood lability; laboratory workup showed markedly elevated levels of total serum estradiol, progesterone, testosterone, and cortisol as measured by immunoassay. Further investigation demonstrated no evidence of estrogen effect on uterus, no adrenal or adnexal mass, and no evidence of Cushing syndrome. Read More

    Corticotropinoma as a Component of Carney Complex.
    J Endocr Soc 2017 Jul 30;1(7):918-925. Epub 2017 May 30.
    Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892.
    Known germline gene abnormalities cause one-fifth of the pituitary adenomas in children and adolescents, but, in contrast with other pituitary tumor types, the genetic causes of corticotropinomas are largely unknown. In this study, we report a case of Cushing disease (CD) due to a loss-of-function mutation in, providing evidence for association of this gene with a corticotropinoma. A 15-year-old male presenting with hypercortisolemia was diagnosed with CD. Read More

    Evaluation of Evidence of Adrenal Insufficiency in Trials of Normocortisolemic Patients Treated With Mifepristone.
    J Endocr Soc 2017 Apr 21;1(4):237-246. Epub 2017 Feb 21.
    Corcept Therapeutics, Menlo Park, California 94025.
    Context: Adrenal insufficiency (AI) is an important medical concern for clinicians when normocortisolemia is achieved during treatment of endogenous Cushing syndrome (CS).

    Objective: To examine symptoms of potential AI in a large population of normocortisolemic patients without CS treated with mifepristone, a glucocorticoid receptor antagonist indicated for the treatment of patients with CS.

    Methods: We conducted a pooled safety analysis of five phase 3, placebo-controlled clinical trials of normocortisolemic adults without CS but diagnosed with psychotic depression (n = 1460). Read More

    Harvey Cushing Treated the First Known Patient With Carney Complex.
    J Endocr Soc 2017 Oct 28;1(10):1312-1321. Epub 2017 Sep 28.
    Section of Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, Maryland 20892.
    Context: Carney complex (CNC) is a syndrome characterized by hyperplasia of endocrine organs and may present with clinical features of Cushing syndrome and acromegaly due to functional adrenal and pituitary gland tumors. CNC has been linked to mutations in the regulatory subunit of protein kinase A type I-alpha () gene.

    Design: Tissue samples were taken from the hypothalamus or thalamus or tumors of patients with pituitary adenomas seen and operated on by neurosurgeon Harvey Cushing between 1913 and 1932. Read More

    Functional Implications of LH/hCG Receptors in Pregnancy-Induced Cushing Syndrome.
    J Endocr Soc 2017 Jan 12;1(1):57-71. Epub 2017 Jan 12.
    Department of Physiology, Institute of Biomedicine, 20520 Turku, Finland.
    Context: Elevated human choriogonadotropin (hCG) may stimulate aberrantly expressed luteinizing hormone (LH)/hCG receptor (LHCGR) in adrenal glands, resulting in pregnancy-induced bilateral macronodular adrenal hyperplasia and transient Cushing syndrome (CS).

    Objective: To determine the role of LHCGR in transient, pregnancy-induced CS.

    Design Setting Patient And Intervention: We investigated the functional implications of LHCGRs in a patient presenting, at a tertiary referral center, with repeated pregnancy-induced CS with bilateral adrenal hyperplasia, resolving after parturition. Read More

    Maffucci Syndrome Associated With Adrenocorticotropic Hormone-Independent Bilateral Macronodular Adrenal Hyperplasia.
    J Endocr Soc 2017 Jan 12;1(1):51-56. Epub 2017 Jan 12.
    Department of Clinical and Experimental Medicine, University of Pisa, 56126 Pisa, Italy.
    Context: Maffucci syndrome is a rare, nonhereditary, mesodermal dysplastic disease characterized by the presence of multiple hemangiomas and enchondromas. This pathological condition, which is often unrecognized, is associated with a high prevalence of benign and malignant endocrine tumors involving pituitary, adrenal, thyroid, and parathyroid glands.

    Case Description: We describe the case of a young patient presenting a history suggestive of secondary arterial hypertension and typical features of Maffucci syndrome (multiple hemangiomas and enchondromas), which were unrecognized over the previous 3 decades. Read More

    Reversal of a Blunted Follicle-Stimulating Hormone by Chemotherapy in an Inhibin B-Secreting Adrenocortical Carcinoma.
    J Endocr Soc 2017 Jan 12;1(1):46-50. Epub 2017 Jan 12.
    Departments of Endocrinology.
    Context: Adrenocortical carcinomas (ACCs) are revealed in 60% of cases by steroid hypersecretion. Alternatively, it is uncommon to observe a paraneoplastic syndrome due to a peptide oversecretion.

    Case Description: We describe a 60-year-old man with a right adrenal mass. Read More

    Alopecia and the metabolic syndrome.
    Clin Dermatol 2018 Jan - Feb;36(1):54-61. Epub 2017 Sep 8.
    National Skin Centre, Singapore. Electronic address:
    Hair loss is a common condition that affects most people at some point in their lives. It can exist as an isolated problem or with other diseases and conditions. Androgenetic alopecia (AGA) and its association with the metabolic syndrome (MetS) have received increasing interest since 1972, when the first link between cardiovascular risk factors and hair loss was raised. Read More

    Genetics of tumors of the adrenal cortex.
    Endocr Relat Cancer 2018 Mar 12;25(3):R131-R152. Epub 2017 Dec 12.
    Institut CochinINSERM U1016, CNRS UMR8104, Paris Descartes University, Paris, France
    This review describes the molecular alterations observed in the various types of tumors of the adrenal cortex, excluding Conn adenomas, especially the alterations identified by genomic approaches these last five years. Two main forms of bilateral adrenocortical tumors can be distinguished according to size and aspect of the nodules: primary pigmented nodular adrenal disease (PPNAD), which can be sporadic or part of Carney complex and primary bilateral macro nodular adrenal hyperplasia (PBMAH). The bilateral nature of the tumors suggests the existence of an underlying genetic predisposition. Read More

    Steroidogenic factor-1 inverse agonists as a treatment option for canine hypercortisolism: in vitro study.
    Domest Anim Endocrinol 2017 Nov 21;63:23-30. Epub 2017 Nov 21.
    Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Yalelaan 108, 3584 CM Utrecht, The Netherlands. Electronic address:
    Hypercortisolism is one of the most commonly diagnosed endocrinopathies in dogs, and new targeted medical treatment options are desirable. Steroidogenic factor-1 (SF-1), an orphan nuclear hormone receptor, is a key regulator of adrenal steroidogenesis, development, and growth. In pituitary-dependent hypercortisolism (PDH), high plasma ACTH concentrations increase the transcriptional activity of SF-1. Read More

    Circulatory Immune Cells in Cushing Syndrome: Bystanders or Active Contributors to Atherometabolic Injury? A Study of Adhesion and Activation of Cell Surface Markers.
    Int J Endocrinol 2017 20;2017:2912763. Epub 2017 Sep 20.
    Group of Endocrine Disorders, IDIBAPS, Barcelona, Spain.
    Glucocorticoids (GC) induce cardiometabolic risk while atherosclerosis is a chronic inflammation involving immunity. GC are immune suppressors, and the adrenocorticotrophic hormone (ACTH) has immune modulator activities. Both may act in atherothrombotic inflammation involving immune cells (IMNC). Read More

    Hypercortisolemia and Depressive-like Behaviors in a Rhesus Macaque () Involved in Visual Research.
    Comp Med 2017 Dec;67(6):529-536
    Animal Resources Department, The Salk Institute for Biological Studies, La Jolla, California;, Email:
    A 10-y-old cranially implanted rhesus macaque (Macaca mulatta) involved in visual research was presented for dull mentation and weight loss. Physical examination revealed alopecia and poor body conditioning, and bloodwork revealed marked hypercortisolemia (23 μg/dL). Differential diagnoses for hypercortisolemia, weight loss, and alopecia included Cushing and pseudo-Cushing syndromes. Read More

    Decreased lymphocytes and increased risk for infection are common in endogenous pediatric Cushing syndrome.
    Pediatr Res 2017 Dec 6. Epub 2017 Dec 6.
    Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland.
    BackgroundHypercortisolemia results in changes of the immune system and elevated infection risk, but data on the WBC changes in pediatric Cushing syndrome (CS) are not known. We describe the changes of the WBC lineages in pediatric endogenous hypercortisolemia, their associations with the markers of disease severity, and the presence of infections.MethodsWe identified 197 children with endogenous CS. Read More

    Plasma Steroid Metabolome for Diagnosis and Subtyping Patients with Cushing Syndrome.
    Clin Chem 2017 Dec 5. Epub 2017 Dec 5.
    Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximilians-Universität München, Munich, Germany.
    Background: Diagnosis of Cushing syndrome requires a multistep process that includes verification of hypercortisolism followed by identification of the cause of adrenocortical hyperfunction. This study assessed whether pituitary, ectopic, and adrenal subtypes of Cushing syndrome were characterized by distinct plasma steroid profiles that might assist diagnosis.

    Methods: In this retrospective cross-sectional study, mass spectrometric measurements of a panel of 15 plasma steroids were applied to 222 patients tested for Cushing syndrome. Read More

    Glucocorticoids Reprogram β-Cell Signaling to Preserve Insulin Secretion.
    Diabetes 2018 Feb 4;67(2):278-290. Epub 2017 Dec 4.
    Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston, U.K.
    Excessive glucocorticoid exposure has been shown to be deleterious for pancreatic β-cell function and insulin release. However, glucocorticoids at physiological levels are essential for many homeostatic processes, including glycemic control. We show that corticosterone and cortisol and their less active precursors 11-dehydrocorticosterone (11-DHC) and cortisone suppress voltage-dependent Cachannel function and Cafluxes in rodent as well as in human β-cells. Read More

    Case report: Ectopic Cushing's syndrome in a young male with hidden lung carcinoid tumor.
    Int J Surg Case Rep 2018 28;42:13-16. Epub 2017 Nov 28.
    Department of Surgery, Damascus University Hospital, Syria. Electronic address:
    Introduction: Ectopic Cushing syndrome is a form of Cushing's in which a tumor outside the pituitary gland produces adrenocorticotropic hormone (ACTH). Small cell carcinoma and carcinoid of the lung comprises half of its cases. The main purpose of this study is to present a case of ectopic Cushing syndrome caused by a hidden lung carcinoid and how to manage it. Read More

    A Case of Cushing's Syndrome in Pregnancy.
    Iran J Med Sci 2017 Nov;42(6):607-610
    Department of Obstetrics and Gynecology, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
    Cushing's syndrome (CS) occurs rarely during pregnancy. CS can be caused by prolonged abnormal exposure to excess glucocorticoids leading to special and significant signs and symptoms. It is often difficult to diagnose pathological hypercortisolism in pregnant women since some symptoms of the disease might be due to a complicated pregnancy, including preeclampsia or gestational diabetes. Read More

    Successful Treatment of Estrogen Excess in Primary Bilateral Macronodular Adrenocortical Hyperplasia with Leuprolide Acetate.
    Horm Metab Res 2018 Feb 28;50(2):124-132. Epub 2017 Nov 28.
    Section on Endocrinology & Genetics (SEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD, USA.
    Primary bilateral macronodular adrenocortical hyperplasia (PBMAH) is an uncommon cause of adrenal Cushing syndrome (CS) in which cortisol and occasionally other steroid hormones can be secreted under the influence of aberrantly expressed G-protein coupled receptors (GPCRs) in the adrenal cortex. We describe the unique case of a 64-year-old postmenopausal female with PBMAH whose adrenal lesions expressed luteinizing hormone receptors (LHr). She presented initially with CS and underwent right adrenalectomy; a few years later she presented with macromastia and mastodynia, possibly due to estrogen excess from her remaining left adrenocortical masses. Read More

    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 2018 21;89(1):38-46. 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) 2018 Mar 17;88(3):415-424. Epub 2017 Dec 17.
    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 (RCS) to evaluate the subclinical cardiometabolic burden and to explore the direct pro-inflammatory and prothrombotic potential of their sera on the endothelium in an in vitro translational atherothrombotic cell model.

    Patients: Cross sectional study. Read More

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