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    1 OF 254

    Quality of Life in Patients with Adrenal Disease: A Systematic Review.
    Clin Endocrinol (Oxf) 2018 Apr 19. Epub 2018 Apr 19.
    Centre for Endocrinology, Barts and the London School of Medicine & Dentistry, William Harvey Research Institute, Queen Mary University of London.
    Background: Evaluating the patient with adrenal disease is challenging due to the lack of precise clinical and biochemical parameters for disease control. Quality of Life (QOL) evaluation aims to measure the patient's subjective experience.

    Objective: to describe how QOL is defined and measured in adrenal disease, critically appraise the use of QOL tools in published literature, discuss the implications of these findings and provide direction for further research in this field. Read More

    Activating PRKACB somatic mutation in cortisol-producing adenomas.
    JCI Insight 2018 Apr 19;3(8). Epub 2018 Apr 19.
    Cochin Institute, Paris Descartes University, CNRS (UMR 8104)/Inserm (U1016), Paris, France.
    Mutations in the gene encoding the protein kinase A (PKA) catalytic subunit α have been found to be responsible for cortisol-producing adenomas (CPAs). In this study, we identified by whole-exome sequencing the somatic mutation p.S54L in the PRKACB gene, encoding the catalytic subunit β (Cβ) of PKA, in a CPA from a patient with severe Cushing syndrome. Read More

    Non-adenomatous pituitary tumours mimicking functioning pituitary adenomas.
    Br J Neurosurg 2018 Apr 18:1-5. Epub 2018 Apr 18.
    b Key Laboratory of Pituitary Adenoma in Guangdong Province, Department of Neurosurgery , First Affiliated Hospital, SunYat-sen University , Guangzhou , China.
    Objective: Pituicytomas and granular cell tumours (GCTs) of the neurohypophysis are considered non-adenomatous neoplasms in the sellar region. The association between hormone hypersecretion and the tumours is seldom discussed and unclear. Therefore, we attempt to investigate this association based on our experience and a review of the literature. Read More

    Do the diagnostic criteria for subclinical hypercortisolism exist?
    Ann Endocrinol (Paris) 2018 Apr 13. Epub 2018 Apr 13.
    Department of endocrinology, diabetes and nutrition, CHU de Bordeaux, hopital Haut-Lévêque, 33604 Pessac, France. Electronic address:
    "Subclinical hypercortisolism" (SH) refers to a condition associated with a mild chronic increase in cortisol secretion. By definition, patients with SH do not exhibit specific symptoms of overt Cushing's syndrome (such as purple striae, easy bruising, proximal muscle weakness), SH has been preferred to "subclinical Cushing's syndrome", a semantic ambiguity since Cushing's syndrome is, by definition, a set of symptoms; and to the term "preclinical Cushing syndrome" because the progression toward overt clinical hypercortisolism is very rare. However, SH still is misnomer as a number of studies suggest that this condition may induce long-term non-specific adverse conditions related to the mild cortisol excess (i. Read More

    Ectopic Cushing syndrome: Report of 9 cases.
    Endocrinol Diabetes Nutr 2018 Apr 11. Epub 2018 Apr 11.
    Servicio de Endocrinología y Nutrición, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, España.
    Introduction: Ectopic Cushing's syndrome (ECS) is a rare condition caused by ACTH secretion by extrapituitary tumors. Its low frequency makes it difficult to acquire experience in its management. The aim of this study was to describe patients with ECS seen at the endocrinology department of a tertiary hospital over 15 years. Read More

    Harvey Cushing's management of neurogenic thoracic outlet syndrome.
    J Neurosurg 2018 Apr 13:1-4. Epub 2018 Apr 13.
    Department of Neurosurgery, Mayo Clinic, Jacksonville, Florida.
    Harvey Cushing is widely regarded as one of the forefathers of neurosurgery, and is primarily associated with his work on intracranial pathology. However, he had a clinical and academic interest in peripheral nerve surgery. Through the courtesy of the Alan Mason Chesney Medical Archives, the surgical records of the Johns Hopkins Hospital from 1896 to 1912 were reviewed. Read More

    Contralateral adrenal width predicts the duration of prolonged post-surgical steroid replacement for subclinical Cushing syndrome.
    Int J Urol 2018 Apr 12. Epub 2018 Apr 12.
    Department of Urology, Chiba University Graduate School of Medicine, Chiba, Japan.
    Objectives: To identify pre-treatment factors affecting the duration of post-surgical steroid replacement in patients undergoing adrenalectomy for subclinical Cushing syndrome.

    Methods: The present retrospective analysis included 64 patients who underwent unilateral laparoscopic adrenalectomy for subclinical Cushing syndrome. Adrenal tumor and contralateral adrenal sizes together with various clinical factors were studied in association with the duration of post-surgical steroid replacement. Read More

    An update on Cushing syndrome in pediatrics.
    Ann Endocrinol (Paris) 2018 Apr 9. Epub 2018 Apr 9.
    Section on Endocrinology and Genetics (SEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), 10, Center Drive, CRC, Rm 1E-3216, 20892-1862 Bethesda, MD, USA. Electronic address:
    Cushing syndrome (CS) in childhood results mostly from the exogenous administration of glucocorticoids; endogenous CS is a rare disease. The latter is the main reason pediatric patients with CS escape diagnosis for too long. Other barriers to optimal care of a pediatric patient with CS include improper following of the proper sequence of testing for diagnosing CS, which stems from lack of understanding of pathophysiology of the hypothalamic-pituitary-adrenal axis; lack of access to proper (i. Read More

    Rebound thymic hyperplasia after adrenalectomy in a patient with Cushing syndrome caused by adrenocortical adenoma: A case report.
    Medicine (Baltimore) 2018 Apr;97(15):e0367
    Department of Pediatrics, Chonbuk National University Medical School.
    Rationale: The development of rebound thymic hyperplasia (RTH) has been reported in patients who have recovered from stressful conditions such as surgery and steroid therapy. We report a case of RTH following the resolution of hypercortisolism after adrenalectomy for the treatment of adrenocortical adenoma in a patient with Cushing syndrome.

    Patient Concerns: A 5-month-old female infant with a history of overeating, hirsutism, and excessive weight gain for the previous 2 months was referred to the hospital. Read More

    Kinetics and inhibition studies of the L205R mutant of cAMP-dependent protein kinase involved in Cushing's syndrome.
    FEBS Open Bio 2018 Apr 11;8(4):606-613. Epub 2018 Mar 11.
    Department of Medicinal ChemistrySchool of PharmacyVirginia Commonwealth UniversityRichmondVAUSA.
    Overproduction of cortisol by the hypothalamus-pituitary-adrenal hormone system results in the clinical disorder known as Cushing's syndrome. Genomics studies have identified a key mutation (L205R) in the α-isoform of the catalytic subunit of cAMP-dependent protein kinase (PKACα) in adrenal adenomas of patients with adrenocorticotropic hormone-independent Cushing's syndrome. Here, we conducted kinetics and inhibition studies on the L205R-PKACα mutant. Read More

    Quality of Life in Cushing's disease: A long term issue?
    Ann Endocrinol (Paris) 2018 Apr 3. Epub 2018 Apr 3.
    Endocrinology/Medicine Department, Research Center for Pituitary Diseases, Hospital Sant Pau, IIB-Sant Pau, Universitat Autònoma de Barcelona (UAB), Pare Claret 167, 08025 Barcelona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER, Unidad 747), ISCIII, Spain. Electronic address:
    The purpose of this review is to describe how quality of life (QoL) is impaired in patients with hypercortisolism due to Cushing's syndrome of any aetiology, including pituitary-dependent Cushing's disease. It is worse in active disease, but improvement after successful therapy is often incomplete, due to persistent physical and psychological co-morbidities, even years after endocrine "cure". Physical symptoms like extreme fatigability, central obesity with limb atrophy, hypertension, fractures, and different skin abnormalities severely impair the affected patients' everyday life. Read More

    Emberger syndrome: A rare association with hearing loss.
    Int J Pediatr Otorhinolaryngol 2018 May 7;108:82-84. Epub 2018 Feb 7.
    Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Toronto, ON Canada; Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON Canada; Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, ON Canada. Electronic address:
    Emberger Syndrome (ES) is a rare genetic disorder characterized by lymphedema and myelodysplasia. It is also associated with hearing loss. The genetic mutations associated with ES are not part of the comprehensive 80 gene next generation sequencing (NGS) panel. Read More

    The Many Faces of Primary Aldosteronism and Cushing Syndrome: A Reflection of Adrenocortical Tumor Heterogeneity.
    Front Med (Lausanne) 2018 12;5:54. Epub 2018 Mar 12.
    Department of Pathology, University Health Network, Toronto, ON, Canada.
    Adrenal cortical tumors constitute a heterogeneous group of neoplasms with distinct clinical, morphological, and molecular features. Recent discoveries of specific genotype-phenotype correlations in adrenal cortical adenomas have transformed our understanding of their respective endocrine syndromes. Indeed, a proportion of patients with primary aldosteronism are now known to harbor adrenal cortical adenomas with heterogeneous molecular alterations (, and ) involving the calcium/calmodulin kinase signaling pathway. Read More

    Bone Health in Adrenal Disorders.
    Endocrinol Metab (Seoul) 2018 Mar;33(1):1-8
    Division of Endocrinology and Metabolism, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
    Secondary osteoporosis resulting from specific clinical disorders may be potentially reversible, and thus continuous efforts to find and adequately treat the secondary causes of skeletal fragility are critical to ameliorate fracture risk and to avoid unnecessary treatment with anti-osteoporotic drugs. Among the hyperfunctional adrenal masses, Cushing's syndrome, pheochromocytoma, and primary aldosteronism are receiving particularly great attention due to their high morbidity and mortality mainly by increasing cardiovascular risk. Interestingly, there is accumulating experimental and clinical evidence that adrenal hormones may have direct detrimental effects on bone metabolism as well. Read More

    Worse Health-Related Quality of Life at long-term follow-up in patients with Cushing's disease than patients with cortisol producing adenoma. Data from the ERCUSYN.
    Clin Endocrinol (Oxf) 2018 Mar 24. Epub 2018 Mar 24.
    Institute of Medicine at Sahlgrenska Academy, University of Gothenburg and the Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden.
    Objective: Hypercortisolism in Cushing's syndrome (CS) is associated with impaired health-related quality of life (HRQoL), which may persist despite remission. We used the data entered into the European Registry on Cushing's syndrome (ERCUSYN) to evaluate if patients with CS of pituitary origin (PIT-CS) have worse HRQoL, both before and after treatment than patients with adrenal causes (ADR-CS).

    Methods: Data from 595 patients (492 women; 83%) who completed the CushingQoL and/or EQ-5D questionnaires at baseline and/or following treatment were analysed. Read More

    Exogenous Cushing syndrome from an unexpected source of systemic steroids.
    Pediatr Dermatol 2018 Mar 25. Epub 2018 Mar 25.
    School of Medicine, Georgetown University, Washington, DC, USA.
    A 12-year-old Hispanic boy with chronic atopic dermatitis and cushingoid features presented to our institution. He was being treated with an unknown quantity of oral prednisolone 15 mg/5 mL, equivalent to 70 mg/m /d of oral prednisone, purchased over the counter in El Salvador. Systemic corticosteroids are not recommended for chronic therapy of atopic dermatitis because of their significant adverse effects. Read More

    Case-series of paraneoplastic Cushing syndrome in small-cell lung cancer.
    Endocrinol Diabetes Metab Case Rep 2018 8;2018. Epub 2018 Mar 8.
    Department of Endocrinology, Mount Lebanon Hospital, Beirut, Lebanon.
    The objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome. Read More

    Secreting ectopic adrenal adenoma: A rare condition to be aware of.
    Ann Endocrinol (Paris) 2018 Apr 7;79(2):75-81. Epub 2018 Mar 7.
    Department of Endocrinology, The First Affiliated Hospital of Xi'an Jiaotong, University School of Medicine, Xi'an, Shannxi 710061, PR China. Electronic address:
    Ectopic adrenal adenoma causing chronic Cushing's syndrome (CS) is a rare phenomenon. Diagnosis is usually made years after disease onset because of the insidious nature of the ectopic adrenal gland and because it overlaps with common symptoms, such as overweight and hypertension, in the general population (Kreitschmann-Andermahr et al., 2015). Read More

    [Homeostasis and Disorder of Musculoskeletal System.Influence of steroid therapy on muscuoskeletal system.]
    Clin Calcium 2018;28(3):402-409
    Division of Rheumatology, Center for Antibody and Vaccine Therapy/Department of Rheumatology & Allergy, IMSUT Hospital, Institute of Medical Science, University of Tokyo, Japan.
    Muscle atrophy occurs when glucocorticoid steroids are administered in pharmacological doses or in Cushing syndrome, and such pathological condition is termed as steroid myopathy. Its molecular mechanism is clarified from the study of the gene expression regulation mechanism mediated by steroid receptors in skeletal muscle, progressing to translational research and also addressing the mechanism by which steroids participate in the regulation of whole body energy metabolism via skeletal muscle. Read More

    Case Of Iatrogenic Cushing's Syndrome By Topical Triamcinolone.
    J Ayub Med Coll Abbottabad 2018 Jan-Mar;30(1):121-123
    Rush University, Chicago, Illinois-USA.
    Cushing's syndrome is a collection of signs and symptoms due to hypercortisolism. Prolong use of topical steroid may cause this syndrome and suppression of hypothalamic and pituitary function, however such events are more common with oral and parenteral route. There are very few cases of Cushing's syndrome with a topical application amongst which triamcinolone is the rarest drug. Read More

    Evaluation of individual low-dose dexamethasone suppression test patterns in naturally occurring hyperadrenocorticism in dogs.
    J Vet Intern Med 2018 Mar 2. Epub 2018 Mar 2.
    University College Dublin Veterinary Hospital, University College Dublin, Belfield, Dublin, Ireland.
    Background: Dogs with hyperadrenocorticism (HAC) may be more mildly affected at the time of diagnosis today, which could influence the prevalence of associated clinical and clinicopathological abnormalities and diagnostic test performance. Different low-dose dexamethasone suppression test (LDDST) result patterns have not been evaluated individually.

    Objectives: To assess the current features of HAC and evaluate if the diagnostic test performance of individual LDDST result patterns differ. Read More

    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 Apr 19;50(4):290-295. 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 May 15;7(1):148-152. 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

    Preoperative medical treatment in Cushing's syndrome: frequency of use and its impact on postoperative assessment: data from ERCUSYN.
    Eur J Endocrinol 2018 Apr 12;178(4):399-409. Epub 2018 Feb 12.
    Centre Hospitalier Universitaire de BordeauxBordeaux, France.
    Background: Surgery is the definitive treatment of Cushing's syndrome (CS) but medications may also be used as a first-line therapy. Whether preoperative medical treatment (PMT) affects postoperative outcome remains controversial.

    Objective: (1) Evaluate how frequently PMT is given to CS patients across Europe; (2) examine differences in preoperative characteristics of patients who receive PMT and those who undergo primary surgery and (3) determine if PMT influences postoperative outcome in pituitary-dependent CS (PIT-CS). 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 Mar 9;35(2):e121-e123. 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 and Screening 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

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