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https://www.readbyqxmd.com/read/28721348/systematic-genetic-screening-in-a-prospective-group-of-danish-patients-with-pheochromocytoma
#1
Morten Steen Svarer Hansen, Niels Jacobsen, Anja Lisbeth Frederiksen, Lars Lund, Marianne Skovsager Andersen, Dorte Glintborg
Recent guidelines recommend consideration of genetic screening in all newly diagnosed patients with pheochromocytoma. Patients diagnosed with pheochromocytoma in the Region of Southern Denmark during 2006-2013 without previously recognized monogenetic etiology were offered genetic screening for mutations in the VHL, RET, SDHB, SDHC, and SDHD genes. A total of 41 patients were included, and genetic data were available in 35. In four of the 35 patients, a pathogenic variant was identified prior to the diagnosis of pheochromocytoma (von Hippel-Lindau disease, n=2; neurofibromatosis type 1, n=2)...
2017: Research and Reports in Urology
https://www.readbyqxmd.com/read/28697215/update-on-the-systemic-risks-of-superpotent-topical-steroids
#2
Mio Nakamura, Michael Abrouk, Henry Zhu, Benjamin Farahnik, John Koo, Tina Bhutani
<p>INTRODUCTION: The potential for systemic effects due to percutaneous absorption of superpotent topical steroids has been a longstanding concern. The Food and Drug Administration currently recommends limiting the use of superpotent topical steroids to 50g per week for 2 or 4 consecutive weeks depending on the formulation, which is mostly based on the exact duration with which phase 3 clinical trials were allowed to be conducted per the FDA. This article reviews all published clinical incidence of adrenal adverse effects in the medical literature, specifically Cushing's syndrome (CS) and pathologic adrenal suppression (PAAS), to try to ascertain a more realistic limit for the safe use of superpotent topical steroids as it pertains to its potential systemic effects...
July 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28689203/global-application-of-the-assessment-of-communication-skills-of-paediatric-endocrinology-fellows-in-the-management-of-differences-in-sex-development-using-the-espe-e-learning-org-portal
#3
Laura J C Kranenburg, Sam T H Reerds, Martine Cools, Julie Alderson, Miriam Muscarella, Ellie Magrite, Martijn Kuiper, Shereen Abdelgaffar, Antonio Balsamo, Raja Brauner, Jean Pierre Chanoine, Asma Deeb, Patricia Fechner, Alina German, Paul Martin Holterhus, Anders Juul, Berenice B Mendonca, Kristen Neville, Anna Nordenstrom, Wilma Oostdijk, Rodolfo A Rey, Meilan M Rutter, Nalini Shah, Xiaoping Luo, Kalinka Grijpink, Stenvert L S Drop
BACKGROUND: Information sharing in chronic conditions such as disorders of/differences in sex development (DSD) is essential for a comprehensive understanding by parents and patients. We report on a qualitative analysis of communication skills of fellows undergoing training in paediatric endocrinology. Guidelines are created for the assessment of communication between health professionals and individuals with DSD and their parents. METHODS: Paediatric endocrinology fellows worldwide were invited to study two interactive online cases (www...
July 7, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28685511/clinical-guidelines-for-the-management-of-adrenal-incidentaloma
#4
REVIEW
Jung Min Lee, Mee Kyoung Kim, Seung Hyun Ko, Jung Min Koh, Bo Yeon Kim, Sang Wan Kim, Soo Kyung Kim, Hae Jin Kim, Ohk Hyun Ryu, Juri Park, Jung Soo Lim, Seong Yeon Kim, Young Kee Shong, Soon Jib Yoo
An adrenal incidentaloma is an adrenal mass found in an imaging study performed for other reasons unrelated to adrenal disease and often accompanied by obesity, diabetes, or hypertension. The prevalence and incidence of adrenal incidentaloma increase with age and are also expected to rise due to the rapid development of imaging technology and frequent imaging studies. The Korean Endocrine Society is promoting an appropriate practice guideline to meet the rising incidence of adrenal incidentaloma, in cooperation with the Korean Adrenal Gland and Endocrine Hypertension Study Group...
June 2017: Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28685301/steroid-use-for-refractory-hypotension-in-congenital-diaphragmatic-hernia
#5
Jason O Robertson, Cory N Criss, Lily B Hsieh, Niki Matsuko, Josh S Gish, Rodrigo A Mon, Kevin N Johnson, Samir K Gadepalli
PURPOSE: Guidelines for diagnosis and treatment of adrenal insufficiency (AI) in newborns with congenital diaphragmatic hernia (CDH) are poorly defined. METHODS: From 2002 to 2016, 155 infants were treated for CDH at our institution. Patients with shock refractory to vasopressors (clinically diagnosed AI) were treated with hydrocortisone (HC). When available, random cortisol levels <10 μg/dL were considered low. Outcomes were compared between groups. RESULTS: Hydrocortisone was used to treat AI in 34% (53/155) of patients...
July 6, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28672763/a-step-by-step-approach-in-differential-diagnosing-of-adrenal-incidentaloma-epinephroma-with-comments-on-the-new-clinical-practice-guidelines-of-the-european-society-of-endocrinology
#6
Frederick-Anthony Farrugia, Evangelos Misiakos, Georgios Martikos, Panagiotis Tzanetis, Anestis Charalampopoulos, Nicolaos Zavras, Dimitrios Sotiropoulos, Nikolaos Koliakos
OBJECTIVES: To present a step by step approach for the diagnosis of adrenal incidentaloma (AI). METHOD: An extensive review of the literature was conducted, searching the Pub-Med and Google Scholar using the Mesh terms; Adrenal; Incidentaloma; Adrenal tumours; Radiology; Diagnosis. We also did a cross-referencing search of the literature. Comments on the new European guidelines are presented. RESULTS: The majority of the tumours are non-functioning benign adenomas...
June 23, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/28661901/long-term-and-late-treatment-consequences-endocrine-and-metabolic-effects
#7
Thang S Han, Helena K Gleeson
PURPOSE OF REVIEW: Cancer therapies often result in the 'late effect of cancer treatment' whereby secondary health complications emerge years after radiotherapy and chemotherapy. This review focuses on endocrine and metabolic consequences in adult cancer survivors as late treatment effects. RECENT FINDINGS: Endocrine and metabolic disorders are among the most common late effects. Endocrine disorders include hypopituitarism, which leads to growth hormone deficiency, hypogonadism, adrenal insufficiency and hypothyroidism and related clinical manifestations...
June 28, 2017: Current Opinion in Supportive and Palliative Care
https://www.readbyqxmd.com/read/28658197/hydrocortisone-therapy-in-catecholamine-resistant-pediatric-septic-shock-a-pragmatic-analysis-of-clinician-practice-and-association-with-outcomes
#8
Blake Nichols, Sherri Kubis, Jennifer Hewlett, Nadir Yehya, Vijay Srinivasan
OBJECTIVES: The 2012 Surviving Sepsis Campaign pediatric guidelines recommend stress dose hydrocortisone in children experiencing catecholamine-dependent septic shock with suspected or proven absolute adrenal insufficiency. We evaluated whether stress dose hydrocortisone therapy in children with catecholamine dependent septic shock correlated with random serum total cortisol levels and was associated with improved outcomes. DESIGN: Retrospective cohort study. SETTING: Non-cardiac PICU...
June 27, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28647413/omega-6-fatty-acids-opposing-associations-with-risk-the-ludwigshafen-risk-and-cardiovascular-health-study
#9
Graciela E Delgado, Winfried März, Stefan Lorkowski, Clemens von Schacky, Marcus E Kleber
BACKGROUND: Omega-6 polyunsaturated fatty acids (omega-6 PUFA) are recommended in European cardiovascular prevention guidelines. However, individual fatty acids have distinct biological functions, and there have been conflicting reports about the association of omega-6 PUFA with cardiovascular risk. OBJECTIVE: The aim of our study was to investigate the association of individual omega-6 fatty acids with mortality in a cohort of patients referred for coronary angiography...
June 1, 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28643981/-adrenal-surgery-multidisciplinarity-is-mandatory
#10
Marie-Laure Matthey Gié, Marie Nicod Lalonde, Elena Gonzalez Rodriguez, Nicolas Demartines, Maurice Matter
Adrenal tumours are rare and their management is challenging. Every patient presenting with adrenal mass or symptoms of hormones hypersecretion should be investigated. The two important questions to be answered are : 1. Is the tumour secreting ? Is the tumour malignant or not ? A complete endocrine work-up and a nativ CT-scan may usually answer these two questions but have to be interpreted by specialists in a multidisciplinary team setting. The decisions about managements of adrenal pathologies do follow international guidelines which are regularly updated...
June 14, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28585593/incidentaloma-from-general-practice-to-specific-endocrine-frame
#11
Ancuta Augustina Gheorghisan-Galateanu, Mara Carsote, Ana Valea
Incidentaloma, a modern concept connected to technology progress, represents an accidentally discovered tumour, usually used for hypophysis and adrenals, and rarely for thyroid, parathyroids, and ovaries. This is a narrative review based on PubMed research, between 2012 and 2016 focusing on general and endocrine approach and current controversies. Main dilemma is the terminology itself: randomly imagery finding is enough or non-functioning profile and low-growth rate (not requiring surgery) should be mandatory? The controversies refers to best time framing of re-scanning pituitary and adrenal incidentaloma and setting of clear criteria for subclinical Cushing's syndrome...
June 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28584010/androstenedione-and-17-%C3%AE-hydroxyprogesterone-are-better-indicators-of-adrenal-vein-sampling-selectivity-than-cortisol
#12
Giulio Ceolotto, Giorgia Antonelli, Giuseppe Maiolino, Maurizio Cesari, Giacomo Rossitto, Valeria Bisogni, Mario Plebani, Gian Paolo Rossi
For identification of potentially surgically curable primary aldosteronism, guidelines recommend use of adrenal vein sampling (AVS) that requires selective catheterization of both adrenal veins as verified by using the cortisol-derived selectivity index. Unfortunately, bilaterally selective studies are not obtained under unstimulated conditions in a proportion of the cases ranging between 15% and 50% depending on the cutoff used. We therefore investigated whether 17-α-hydroxyprogesterone and androstenedione, which showed a higher step-up between adrenal vein and inferior vena cava blood than cortisol, can ascertain selectivity when cortisol failed to do so...
June 5, 2017: Hypertension
https://www.readbyqxmd.com/read/28576687/outcomes-after-adrenalectomy-for-unilateral-primary-aldosteronism-an-international-consensus-on-outcome-measures-and-analysis-of-remission-rates-in-an-international-cohort
#13
Tracy A Williams, Jacques W M Lenders, Paolo Mulatero, Jacopo Burrello, Marietta Rottenkolber, Christian Adolf, Fumitoshi Satoh, Laurence Amar, Marcus Quinkler, Jaap Deinum, Felix Beuschlein, Kanako K Kitamoto, Uyen Pham, Ryo Morimoto, Hironobu Umakoshi, Aleksander Prejbisz, Tomaz Kocjan, Mitsuhide Naruse, Michael Stowasser, Tetsuo Nishikawa, William F Young, Celso E Gomez-Sanchez, John W Funder, Martin Reincke
BACKGROUND: Although unilateral primary aldosteronism is the most common surgically correctable cause of hypertension, no standard criteria exist to classify surgical outcomes. We aimed to create consensus criteria for clinical and biochemical outcomes and follow-up of adrenalectomy for unilateral primary aldosteronism and apply these criteria to an international cohort to analyse the frequency of remission and identify preoperative determinants of successful outcome. METHODS: The Primary Aldosteronism Surgical Outcome (PASO) study was an international project to develop consensus criteria for outcomes and follow-up of adrenalectomy for unilateral primary aldosteronism...
May 30, 2017: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/28536363/update-on-treatment-guideline-in-fibromyalgia-syndrome-with-focus-on-pharmacology
#14
REVIEW
Sanam Kia, Ernet Choy
Fibromyalgia syndrome (FMS) is a chronic condition with unknown aetiology. The pathophysiology of the disease is incompletely understood; despite advances in our knowledge with regards to abnormal central and peripheral pain processing, and hypothalamo-pituitary-adrenal dysfunction, there is no clear specific pathophysiological therapeutic target. The management of this complex condition has thus perplexed the medical community for many years, and several national and international guidelines have aimed to address this complexity...
May 8, 2017: Biomedicines
https://www.readbyqxmd.com/read/28474235/patients-treated-with-immunosuppressive-steroids-are-less-aware-of-sick-day-rules-than-those-on-endocrine-replacement-therapy-and-may-be-at-greater-risk-of-adrenal-crisis
#15
M R Salehmohamed, M Griffin, T Branigan, M Cuesta, C J Thompson
BACKGROUND: Patients taking corticosteroids for immune suppression are vulnerable to adrenal crisis during acute illness or if steroids are stopped abruptly. Although patients treated for adrenal failure in endocrine units are routinely provided with sick day guidelines, we wished to ascertain whether patients on immunosuppressive steroids are appropriately advised. AIM: This study sets out to compare patient awareness of steroid sick day rules in endocrine and non-endocrine patients...
May 4, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/28471129/management-of-a-multiple-endocrine-neoplasia-type-1-during-pregnancy-a-case-report-and-review-of-the-literature
#16
REVIEW
Halil Korkut Daglar, Ayse Kirbas, Ebru Biberoglu, Bergen Laleli, Nuri Danisman
Multiple Endocrine Neoplasia Type 1 (MEN1) or Wermer's syndrome is a rare hereditary endocrine syndrome with high penetrance caused by mutations in MEN1 tumor suppressor gene. MEN1 is characterized by hyperplasia or tumoral enlargement in a number of endocrine organs (parathyroid glands, pancreas, pituitary gland, adrenal gland) and it could be hormonally active or inactive. MEN1 is a significant cause of morbidity due to hormone secretion and mass effect. Since it is a rare condition, there are no guidelines with respect to the follow-up of pregnant women with MEN1...
July 2016: Journal of Experimental Therapeutics & Oncology
https://www.readbyqxmd.com/read/28432798/therapeutic-drug-monitoring-of-mitotane-analytical-assay-and-patient-follow-up
#17
Catherine Feliu, Yoann Cazaubon, Helene Guillemin, Damien Vautier, Olivier Oget, Hervé Millart, Claire Gozalo, Zoubir Djerada
Adrenocortical carcinoma (ACC) is an aggressive malignancy of the adrenal gland. Mitotane (o,p'-DDD) is the most effective chemotherapy for ACC. According to the literature, mitotane plasma trough concentrations within 14-20 mg L(-1) are correlated with a higher response rate with acceptable toxicity. Therapeutic drug monitoring (TDM) of mitotane is therefore recommended. The aim of this study was to propose a robust and simple method for mitotane quantification in plasma. The validation procedures were based on international guidelines...
April 22, 2017: Biomedical Chromatography: BMC
https://www.readbyqxmd.com/read/28420172/subtype-diagnosis-of-primary-aldosteronism-is-adrenal-vein-sampling-always-necessary
#18
REVIEW
Fabrizio Buffolo, Silvia Monticone, Tracy A Williams, Denis Rossato, Jacopo Burrello, Martina Tetti, Franco Veglio, Paolo Mulatero
Aldosterone producing adenoma and bilateral adrenal hyperplasia are the two most common subtypes of primary aldosteronism (PA) that require targeted and distinct therapeutic approaches: unilateral adrenalectomy or lifelong medical therapy with mineralocorticoid receptor antagonists. According to the 2016 Endocrine Society Guideline, adrenal venous sampling (AVS) is the gold standard test to distinguish between unilateral and bilateral aldosterone overproduction and therefore, to safely refer patients with PA to surgery...
April 17, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28416079/management-of-childhood-congenital-adrenal-hyperplasia-an-integrative-review-of-the-literature
#19
Louise Fleming, Marcia Van Riper, Kathleen Knafl
Congenital adrenal hyperplasia (CAH) is an endocrine condition that requires parents to administer steroids up to three times daily, supplementing maintenance doses with oral or injectable doses during times of illness. The purpose of this integrative review was to explore the management, care, and associated health issues for children with CAH and the family response to the condition. Four literature indexes were searched, with 39 articles included. Four themes emerged: (a) Health- and Development-Related Issues, (a) Effects of Excess Androgens, (c) Life Experience of CAH, and (d) Managing and Averting Adrenal Crisis...
April 13, 2017: Journal of Pediatric Health Care
https://www.readbyqxmd.com/read/28400402/molecular-targeted-therapies-in-adrenal-pituitary-and-parathyroid-malignancies
#20
Anna Angelousi, Georgios K Dimitriadis, Gn Zografos, Svenja Nölting, Gregory A Kaltsas, Ashley B Grossman
Tumourigenesis is a relatively common event in endocrine tissues. Currently, specific guidelines have been developed for common malignant endocrine tumours which also incorporate advances in molecular targeted therapies (MTT), as in thyroid cancer and gastrointestinal neuroendocrine malignancies. However, there is little information regarding the role and efficacy of MTT in the relatively rare malignant endocrine tumours mainly involving the adrenal medulla, adrenal cortex, pituitary and parathyroid glands...
April 11, 2017: Endocrine-related Cancer
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