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https://www.readbyqxmd.com/read/29774538/oral-propranolol-in-the-treatment-of-proliferating-infantile-haemangiomas-the-british-society-for-paediatric-dermatology-consensus-guidelines
#1
L Solman, M Glover, P E Beattie, H Buckley, S Clark, J E Gach, A Giardini, I Helbling, R J Hewitt, B Laguda, S M Langan, A E Martinez, R Murphy, L Proudfoot, J Ravenscroft, H Shahidullah, L Shaw, S B Syed, L Wells, C Flohr
BACKGROUND: Infantile haemangiomas (IH) are the most common vascular tumours of infancy. Despite their frequency and potential complications, there are currently no unified UK guidelines for the treatment of IH with propranolol. There are still uncertainties and diverse opinions regarding indications, pre-treatment investigations, its use in PHACES syndrome, and cessation of treatment. METHODS: This study used a modified Delphi technique, which involved an international treatment survey, a systematic evidence review of the literature, a face-to-face multidisciplinary panel meeting and anonymous voting...
May 17, 2018: British Journal of Dermatology
https://www.readbyqxmd.com/read/29772682/the-association-of-dietary-fiber-intake-with-cardiometabolic-risk-in-four-countries-across-the-epidemiologic-transition
#2
Louise Lie, Laquita Brown, Terrence E Forrester, Jacob Plange-Rhule, Pascal Bovet, Estelle V Lambert, Brian T Layden, Amy Luke, Lara R Dugas
The greatest burden of cardiovascular disease is now carried by developing countries with cardiometabolic conditions such as metabolic syndrome, obesity and inflammation believed to be the driving force behind this epidemic. Dietary fiber is known to have protective effects against obesity, type 2 diabetes, cardiovascular disease and the metabolic syndrome. Considering the emerging prevalence of these cardiometabolic disease states across the epidemiologic transition, the objective of this study is to explore these associations of dietary fiber with cardiometabolic risk factors in four countries across the epidemiologic transition...
May 16, 2018: Nutrients
https://www.readbyqxmd.com/read/29772418/differences-between-manual-and-automatic-analysis-in-determining-the-severity-of-obstructive-sleep-apnea-using-home-sleep-apnea-testing
#3
Gonzalo Labarca, Jorge Dreyse, Constanza Salas, Andrea Contreras, Gonzalo Nazar, Maria I Gaete, Jorge Jorquera
INTRODUCTION: Home sleep apnea testing (HSAT) is a diagnostic measure for obstructive sleep apnea hypopnea syndrome (OSAHS) in moderate/high risk patients. Some HSAT companies contain automatic analysis (AA). However, guidelines recommend manual analysis (MA) despite the weak evidence for this recommendation. OBJECTIVE: Evaluate the concordance between AA and MA of HSAT to make either a diagnosis and severity classification. METHODS: We evaluated AA and MA of HSAT between 2015 and 2016...
April 7, 2018: Sleep Medicine
https://www.readbyqxmd.com/read/29771871/trends-in-antiretroviral-therapy-eligibility-and-coverage-among-children-aged-15-years-with-hiv-infection-20-pepfar-supported-sub-saharan-african-countries-2012-2016
#4
Amanda Burrage, Monita Patel, Kelsey Mirkovic, Eric Dziuban, Wondimu Teferi, Laura Broyles, Emilia Rivadeneira
Rapid disease progression and associated opportunistic infections contribute to high mortality rates among children aged <15 years with human immunodeficiency virus (HIV) infection (1). Antiretroviral therapy (ART) has decreased childhood HIV-associated morbidity and mortality rates over the past decade (2). As accumulating evidence revealed lower HIV-associated mortality with early ART initiation, the World Health Organization (WHO) guidelines broadened ART eligibility for children with HIV infection (2)...
May 18, 2018: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/29771759/finding-treatable-genetic-obesity-strategies-for-success
#5
Kristin Dayton, Jennifer Miller
PURPOSE OF REVIEW: Genetic obesity is responsible for up to 7% of severe childhood obesity. Although current Pediatric Endocrine Society guidelines recommend assessment of children with early-onset morbid obesity and hyperphagia for underlying genetic disorders, a vast majority of patients are not being appropriately screened for genetic obesity syndromes. RECENT FINDINGS: With advances in genetic testing, more genetic causes of obesity are being identified. Treatments are likely to be individualized, depending on the cause of the obesity, and must be targeted at addressing the underlying cause...
May 15, 2018: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/29770929/two-cases-of-sarcoidosis-presenting-as-longitudinally-extensive-transverse-myelitis
#6
REVIEW
Amanda Mary Scott, Janeth Yinh, Timothy McAlindon, Robert Kalish
Neurosarcoidosis is uncommon with an incidence of approximately 5 to 15%. Central nervous system involvement can be divided into brain and spinal cord neurosarcoidosis. Spinal cord sarcoidosis is extremely rare, occurring in less than 1% of all sarcoidosis cases. Its manifestations may include cauda equina syndrome, radiculopathy, syringomyelia, cord atrophy, arachnoiditis, and myelopathy or transverse myelitis. We highlight two cases of spinal cord sarcoidosis, each presenting with longitudinally extensive transverse myelitis, that demonstrate the dilemmas that physicians face with regard to diagnosis and treatment...
May 17, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29770854/german-contribution-to-development-and-innovations-in-the-management-of-acute-myocardial-infarction-and-cardiogenic-shock
#7
REVIEW
Hans-Josef Feistritzer, Steffen Desch, Suzanne de Waha, Alexander Jobs, Uwe Zeymer, Holger Thiele
Treatment of acute coronary syndromes has evolved over time leading to a significantly reduced mortality. Multiple major trials have been performed in Germany leading to new treatment strategies in acute coronary syndromes including cardiogenic shock. This review article will summarize major trials and their impact on guideline recommendations in acute myocardial infarction highlighting reperfusion strategies, antiplatelet regimens, prognosis assessment and also mechanical circulatory support in stable infarction patients and in cardiogenic shock...
May 16, 2018: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/29766482/-fragile-x-syndrome-new-therapeutic-strategies
#8
S Zeidler, B Dierckx, K Lubbers, A M van Eeghen, C R Lincke, J A Kievit, R Willemsen, A Rietman
Fragile X syndrome (fxs) is the most common hereditary cause of intellectual disability and autism spectrum disorders. Targeted treatment is currently lacking. In the past decades an enormous amount of knowledge has been obtained concerning the involved molecular pathways, introducing potential targets for disease modifying therapy.<br/> AIM: To present an overview of the development of targeted treatment for fxs.<br/> METHOD: Several important publications were collected and indexed.<br/> RESULTS: While preclinical animal model studies with targeted interventions are promising, the translation to the clinic has been disappointing...
2018: Tijdschrift Voor Psychiatrie
https://www.readbyqxmd.com/read/29761101/fibromyalgia-syndrome-a-case-report-on-controlled-remission-of-symptoms-by-a-dietary-strategy
#9
Silvia Maria Lattanzio, Francesca Imbesi
A 34-year-old woman suffered from significant chronic pain, depression, non-restorative sleep, chronic fatigue, severe morning stiffness, leg cramps, irritable bowel syndrome, hypersensitivity to cold, concentration difficulties, and forgetfulness. Blood tests were negative for rheumatic disorders. The patient was diagnosed with Fibromyalgia syndrome (FMS). Due to the lack of effectiveness of pharmacological therapies in FMS, she approached a novel metabolic proposal for the symptomatic remission. Its core idea is supporting serotonin synthesis by allowing a proper absorption of tryptophan assumed with food, while avoiding, or at least minimizing the presence of interfering non-absorbed molecules, such as fructose and sorbitol...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29759272/pharmacologic-and-nonpharmacologic-treatment-of-restless-legs-syndrome
#10
REVIEW
Galia V Anguelova, Monique H M Vlak, Arthur G Y Kurvers, Roselyne M Rijsman
This article provides an updated practical guide for the treatment of primary restless legs syndrome (RLS). Articles that appeared after the American Academy of Neurology guideline search were reviewed according to the same evidence rating schedule. We found limited evidence for nonpharmacologic treatment options. In moderate to severe primary RLS, pharmacologic options may be considered, including iron suppletion, an α2δ ligand, a dopamine agonist, a combination of an α2δ ligand and a dopamine agonist, or oxycodone/naloxone...
June 2018: Sleep Medicine Clinics
https://www.readbyqxmd.com/read/29759108/a-western-dietary-pattern-is-prospectively-associated-with-cardio-metabolic-traits-and-incidence-of-the-metabolic-syndrome
#11
Isabel Drake, Emily Sonestedt, Ulrika Ericson, Peter Wallström, Marju Orho-Melander
The aim of this study was to derive dietary patterns associated with cardio-metabolic traits and to examine whether these predict prospective changes in these traits and incidence of the metabolic syndrome (iMetS). Subjects from the Malmö Diet and Cancer Study cardiovascular cohort without cardio-metabolic disease and related drug treatments at baseline (n 4071; aged 45-67 years, 40 % men) were included. We applied reduced rank regression on thirty-eight foods to derive patterns that explain variation in response variables measured at baseline (waist circumference, TAG, HDL- and LDL-cholesterol, systolic and diastolic blood pressure, fasting glucose and insulin)...
May 2018: British Journal of Nutrition
https://www.readbyqxmd.com/read/29757053/applying-a-low-fodmap-dietary-intervention-to-a-female-ultra-endurance-runner-with-irritable-bowel-syndrome-during-a-multi-stage-ultra-marathon
#12
Stephanie K Gaskell, Ricardo J S Costa
Malabsorption of Fermentable Oligo- Di- and Mono-saccharides and Polyols (FODMAPs) in response to prolonged exercise may increase incidence of upper- and lower-gastrointestinal symptoms (GIS), which are known to impair exercise performance. The case-study aimed to explore the impact of a low-FODMAP diet on exercise-associated GIS in a female ultra-endurance runner diagnosed with irritable bowel syndrome (IBS), competing in a six-day 186.7 km mountainous multi-stage ultra-marathon (MSUM). IBS symptom severity score at diagnosis was 410 and following a low-FODMAP diet (3...
May 14, 2018: International Journal of Sport Nutrition and Exercise Metabolism
https://www.readbyqxmd.com/read/29754644/cushing-s-syndrome-in-pediatrics-an-update
#13
REVIEW
Maya B Lodish, Margaret F Keil, Constantine A Stratakis
Cushing syndrome (CS) is a multisystem disorder resulting from the prolonged exposure to excess glucocorticoids. In children, CS most commonly results from the exogenous administration of steroids and the typical presentation is height deceleration concomitant with weight gain. Endogenous and ectopic causes are rare. CS in children may be associated with distinct germline and somatic mutations. Clinical practice guidelines are available assist clinicians. Patients should be referred to multidisciplinary centers of excellence with experience in endocrinology and surgery...
June 2018: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/29753700/spectrum-and-prevalence-of-genetic-predisposition-in-medulloblastoma-a-retrospective-genetic-study-and-prospective-validation-in-a-clinical-trial-cohort
#14
Sebastian M Waszak, Paul A Northcott, Ivo Buchhalter, Giles W Robinson, Christian Sutter, Susanne Groebner, Kerstin B Grund, Laurence Brugières, David T W Jones, Kristian W Pajtler, A Sorana Morrissy, Marcel Kool, Dominik Sturm, Lukas Chavez, Aurelie Ernst, Sebastian Brabetz, Michael Hain, Thomas Zichner, Maia Segura-Wang, Joachim Weischenfeldt, Tobias Rausch, Balca R Mardin, Xin Zhou, Cristina Baciu, Christian Lawerenz, Jennifer A Chan, Pascale Varlet, Lea Guerrini-Rousseau, Daniel W Fults, Wiesława Grajkowska, Peter Hauser, Nada Jabado, Young-Shin Ra, Karel Zitterbart, Suyash S Shringarpure, Francisco M De La Vega, Carlos D Bustamante, Ho-Keung Ng, Arie Perry, Tobey J MacDonald, Pablo Hernáiz Driever, Anne E Bendel, Daniel C Bowers, Geoffrey McCowage, Murali M Chintagumpala, Richard Cohn, Timothy Hassall, Gudrun Fleischhack, Tone Eggen, Finn Wesenberg, Maria Feychting, Birgitta Lannering, Joachim Schüz, Christoffer Johansen, Tina V Andersen, Martin Röösli, Claudia E Kuehni, Michael Grotzer, Kristina Kjaerheim, Camelia M Monoranu, Tenley C Archer, Elizabeth Duke, Scott L Pomeroy, Redmond Shelagh, Stephan Frank, David Sumerauer, Wolfram Scheurlen, Marina V Ryzhova, Till Milde, Christian P Kratz, David Samuel, Jinghui Zhang, David A Solomon, Marco Marra, Roland Eils, Claus R Bartram, Katja von Hoff, Stefan Rutkowski, Vijay Ramaswamy, Richard J Gilbertson, Andrey Korshunov, Michael D Taylor, Peter Lichter, David Malkin, Amar Gajjar, Jan O Korbel, Stefan M Pfister
BACKGROUND: Medulloblastoma is associated with rare hereditary cancer predisposition syndromes; however, consensus medulloblastoma predisposition genes have not been defined and screening guidelines for genetic counselling and testing for paediatric patients are not available. We aimed to assess and define these genes to provide evidence for future screening guidelines. METHODS: In this international, multicentre study, we analysed patients with medulloblastoma from retrospective cohorts (International Cancer Genome Consortium [ICGC] PedBrain, Medulloblastoma Advanced Genomics International Consortium [MAGIC], and the CEFALO series) and from prospective cohorts from four clinical studies (SJMB03, SJMB12, SJYC07, and I-HIT-MED)...
May 9, 2018: Lancet Oncology
https://www.readbyqxmd.com/read/29752989/a-novel-pathogenic-variant-in-the-marveld2-gene-causes-autosomal-recessive-non-syndromic-hearing-loss-in-an-iranian-family
#15
Afsaneh Taghipour-Sheshdeh, Fatemeh Nemati-Zargaran, Narges Zarepour, Parisa Tahmasebi, Nader Saki, Mohammad Amin Tabatabaiefar, Javad Mohammadi-Asl, Morteza Hashemzadeh-Chaleshtori
BACKGROUND AND AIMS: Hearing loss (HL) is the most common sensorineural disorder and one of the most common human defects. HL can be classified according to main criteria, including: the site (conductive, sensorineural and mixed), onset (pre-lingual and post-lingual), accompanying signs and symptoms (syndromic and non-syndromic), severity (mild, moderate, severe and profound) and mode of inheritance (Autosomal recessive, autosomal dominant, X-linked and mitochondrial). Autosomal recessive non-syndromic HL (ARNSHL) forms constitute a major share of the HL cases...
May 9, 2018: Genomics
https://www.readbyqxmd.com/read/29750288/recognizing-and-managing-children-with-a-pediatric-cancer-predisposition-syndrome-a-guide-for-the-pediatrician
#16
Stephanie A Coury, Katherine A Schneider, Jaclyn Schienda, Wen-Hann Tan
It is estimated that at least 8% to 10% of children diagnosed with cancer have an inherited cancer predisposition syndrome. Pediatricians may be called upon to (1) identify children with symptoms suggestive of cancer that require further diagnostic testing, (2) identify children who should be referred to cancer genetics based on their personal and family histories, and (3) provide primary care to children who have an inherited cancer syndrome. This review article provides a list of clinical warning signs suggestive of childhood malignancy, discusses the personal and family history "red flags" suggestive of hereditary cancer, offers checklists to help identify patients who are candidates for cancer genetics evaluation, and describes features of the major pediatric cancer syndromes involving solid tumors and surveillance guidelines...
May 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29750227/facilitators-context-of-and-barriers-to-acute-coronary-syndrome-care-at-kenyatta-national-hospital-nairobi-kenya-a-qualitative-analysis
#17
Ehete Bahiru, Tecla Temu, Julia Mwanga, Kevin Ndede, Sophie Vusha, Bernard Gitura, Carey Farquhar, Frederick Bukachi, Mark D Huffman
BACKGROUND: The prevalence of ischaemic heart disease and its acute manifestation, acute coronary syndrome (ACS), is growing throughout sub-Saharan Africa, including Kenya. To address this increasing problem, we sought to understand the facilitators, context of and barriers to ACS care at Kenyatta National Hospital, with the aim of improving the quality of care of ACS. METHODS: We conducted in-depth interviews with healthcare providers involved in the management of ACS patients from January to February 2017 at Kenyatta National Hospital in Nairobi, Kenya...
April 20, 2018: Cardiovascular Journal of Africa
https://www.readbyqxmd.com/read/29750091/a-pragmatic-evidence-based-clinical-management-algorithm-for-burning-mouth-syndrome
#18
Yohanan Kim, Timothy Yoo, Peter Han, Yuan Liu, Jared C Inman
Background: Burning mouth syndrome is a poorly understood disease process with no current standard of treatment. The goal of this article is to provide an evidence-based, practical, clinical algorithm as a guideline for the treatment of burning mouth syndrome. Material and Methods: Using available evidence and clinical experience, a multi-step management algorithm was developed. A retrospective cohort study was then performed, following STROBE statement guidelines, comparing outcomes of patients who were managed using the algorithm and those who were managed without...
April 2018: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/29749581/preconception-counseling-for-patients-with-thoracic-aortic-aneurysms
#19
REVIEW
Prashant Rao, Eric M Isselbacher
PURPOSE OF THE REVIEW: Acute aortic dissection is a potentially catastrophic cardiovascular emergency that is associated with a high mortality rate. Pregnancy, with its attendant hormonal and physiological changes, increases the risk of dissection in women with known thoracic aortic aneurysms. In this review, we highlight the importance of preconception counseling to help women with known thoracic aortic aneurysms better understand their risk of dissection and the heritable nature of thoracic aortic disease and its associated syndromes...
May 10, 2018: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29747678/a-potential-link-between-polycystic-ovary-syndrome-and-non-alcoholic-fatty-liver-disease-an-update-meta-analysis
#20
Jia Wu, Xin-Yu Yao, Ru-Xia Shi, Su-Fen Liu, Xiao-Yong Wang
BACKGROUND: Epidemiological literature regarding the effect of polycystic ovary syndrome (PCOS) as a risk factor for non-alcoholic fatty liver disease (NAFLD) remains inconsistent. Furthermore, it remains debatable whether NAFLD is associated with PCOS as a consequence of shared risk factors or whether PCOS contributes to NAFLD in an independent fashion. Therefore, this meta-analysis was conducted. METHODS: This meta-analysis was conducted in accordance with the guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)...
May 10, 2018: Reproductive Health
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