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Wilson disease guidelines

Andrew Duncan, Calum Yacoubian, Robert Beetham, Anthony Catchpole, David Bullock
BACKGROUND: US and European guidelines suggest the use of calculated non-caeruloplasmin bound copper (free copper index) for the diagnosis and management of Wilson's Disease. However, there is concern that the required analytical measurements of caeruloplasmin and copper may not be sufficiently robust at the concentrations usually found. METHODS: Aliquots of six plasma specimens were sent to laboratories participating in the UK National External Quality Assessment Scheme for copper and caeruloplasmin...
October 13, 2016: Annals of Clinical Biochemistry
Eli Muchtar, Daniele Derudas, Michelle Mauermann, Teerin Liewluck, Angela Dispenzieri, Shaji K Kumar, David Dingli, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, Nelson Leung, Rajshekhar Chakraborty, Wilson Gonsalves, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, Robert A Kyle, S Vincent Rajkumar, Morie A Gertz
OBJECTIVE: To characterize the natural history of immunoglobulin light chain amyloidosis-associated myopathy and to provide guidelines for recognition. PATIENTS AND METHODS: Fifty-one patients with systemic immunoglobulin light chain amyloidosis and biopsy-confirmed muscle amyloid deposition diagnosed between January 1, 1995, and December 31, 2015, were included in this study. RESULTS: Common presenting symptoms were muscle weakness in 49 patients (96%), dysphagia in 23 (45%), myalgia in 17 (33%), macroglossia in 17 (33%), jaw claudication in 13 (25%), and hoarseness in 9 (18%)...
October 2016: Mayo Clinic Proceedings
Steven H Abman, D Dunbar Ivy, Stephen L Archer, Kevin Wilson
Although pulmonary hypertension (PH) contributes significantly to poor outcomes in diverse pediatric diseases, approaches toward the care of children with PH have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a committee of experienced clinicians was formed to systematically identify, synthesize, and appraise relevant evidence and then to formulate evidence-based recommendations regarding the diagnosis and management of pediatric PH...
October 1, 2016: American Journal of Respiratory and Critical Care Medicine
R Douglas Wilson, Isabelle De Bie, Christine M Armour, Richard N Brown, Carla Campagnolo, June C Carroll, Nan Okun, Tanya Nelson, Rhonda Zwingerman, Francois Audibert, Jo-Ann Brock, Richard N Brown, Carla Campagnolo, June C Carroll, Isabelle De Bie, Jo-Ann Johnson, Nan Okun, Melanie Pastruck, Karine Vallée-Pouliot, R Douglas Wilson, Rhonda Zwingerman, Christine Armour, David Chitayat, Isabelle De Bie, Sara Fernandez, Raymond Kim, Josee Lavoie, Norma Leonard, Tanya Nelson, Sherry Taylor, Margot Van Allen, Clara Van Karnebeek
OBJECTIVE: This guideline was written to update Canadian maternity care and reproductive healthcare providers on pre- and postconceptional reproductive carrier screening for women or couples who may be at risk of being carriers for autosomal recessive (AR), autosomal dominant (AD), or X-linked (XL) conditions, with risk of transmission to the fetus. Four previous SOGC- Canadian College of Medical Geneticists (CCMG) guidelines are updated and merged into the current document. INTENDED USERS: All maternity care (most responsible health provider [MRHP]) and paediatric providers; maternity nursing; nurse practitioner; provincial maternity care administrator; medical student; and postgraduate resident year 1-7...
August 2016: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
Francis X McCormack, Nishant Gupta, Geraldine R Finlay, Lisa R Young, Angelo M Taveira-DaSilva, Connie G Glasgow, Wendy K Steagall, Simon R Johnson, Steven A Sahn, Jay H Ryu, Charlie Strange, Kuniaki Seyama, Eugene J Sullivan, Robert M Kotloff, Gregory P Downey, Jeffrey T Chapman, MeiLan K Han, Jeanine M D'Armiento, Yoshikazu Inoue, Elizabeth P Henske, John J Bissler, Thomas V Colby, Brent W Kinder, Kathryn A Wikenheiser-Brokamp, Kevin K Brown, Jean F Cordier, Cristopher Meyer, Vincent Cottin, Jan L Brozek, Karen Smith, Kevin C Wilson, Joel Moss
BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of LAM. METHODS: Systematic reviews were performed to summarize evidence pertinent to our questions. The evidence was summarized and discussed by a multidisciplinary panel. Evidence-based recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation approach...
September 15, 2016: American Journal of Respiratory and Critical Care Medicine
Rebecca K Simmons, Knut Borch-Johnsen, Torsten Lauritzen, Guy Ehm Rutten, Annelli Sandbæk, Maureen van den Donk, James A Black, Libo Tao, Edward Cf Wilson, Melanie J Davies, Kamlesh Khunti, Stephen J Sharp, Nicholas J Wareham, Simon J Griffin
BACKGROUND: Intensive treatment (IT) of cardiovascular risk factors can halve mortality among people with established type 2 diabetes but the effects of treatment earlier in the disease trajectory are uncertain. OBJECTIVE: To quantify the cost-effectiveness of intensive multifactorial treatment of screen-detected diabetes. DESIGN: Pragmatic, multicentre, cluster-randomised, parallel-group trial. SETTING: Three hundred and forty-three general practices in Denmark, the Netherlands, and Cambridge and Leicester, UK...
August 2016: Health Technology Assessment: HTA
Wilson Sui, Ifeanyi C Onyeji, Justin T Matulay, Marissa C Theofanides, Maxwell B James, G Joel DeCastro, Sven Wenske
INTRODUCTION: To assess 30-day morbidity and mortality following partial nephrectomy (PN) and radical nephrectomy (RN) with relation to the administration of perioperative blood transfusions PBT). MATERIALS AND METHODS: The National Surgical Quality Improvement Program was queried for patients with malignant renal tumors (International Classification of Diseases Ninth Revision codes 189-189.2) who underwent RN (Current Procedure Terminology codes 50220, 50225, 50230, 50234, 50236, 50545, 50546, 50548) or PN (50240, 50543) between 2005-2013...
August 2016: Canadian Journal of Urology
Robert Wilson, Timothy Aksamit, Stefano Aliberti, Anthony De Soyza, J Stuart Elborn, Pieter Goeminne, Adam T Hill, Rosario Menendez, Eva Polverino
BACKGROUND: An Expert Forum was held at the 2014 European Respiratory Society International Congress to address issues involved in the management of Pseudomonas aeruginosa infection in patients with non-cystic fibrosis bronchiectasis (NCFB). Multiple studies have found that chronic P. aeruginosa infection is associated with more severe disease and higher morbidity and mortality. OVERVIEW: Participants discussed appropriate management of P. aeruginosa infection at three stages: 1) first isolation, including eradication protocols; 2) during exacerbations; and 3) during chronic infection, including long-term antibiotic therapy to reduce the severity of symptoms and frequency of exacerbations...
August 2016: Respiratory Medicine
Wilson Petrushnko, Justin S Gundara, Philip R De Reuver, Greg O'Grady, Jaswinder S Samra, Anubhav Mittal
BACKGROUND: Pancreatic ductal adenocarcinoma (PDAC) continues to be associated with a poor prognosis. This systematic review aimed to summarize the literature regarding potential prognostic biomarkers to facilitate validation studies and clinical application. METHODS: A systematic review was performed (2004-2014) according to PRISMA guidelines. Studies were ranked using REMARK criteria and the following outcomes were examined: overall/disease free survival, nodal involvement, tumour characteristics, metastasis, recurrence and resectability...
August 2016: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
Lindsey Robert Baden, Sankar Swaminathan, Michael Angarone, Gayle Blouin, Bernard C Camins, Corey Casper, Brenda Cooper, Erik R Dubberke, Ashley Morris Engemann, Alison G Freifeld, John N Greene, James I Ito, Daniel R Kaul, Mark E Lustberg, Jose G Montoya, Ken Rolston, Gowri Satyanarayana, Brahm Segal, Susan K Seo, Shmuel Shoham, Randy Taplitz, Jeffrey Topal, John W Wilson, Karin G Hoffmann, Courtney Smith
Infectious diseases are important causes of morbidity and mortality in patients with cancer. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Prevention and Treatment of Cancer-Related Infections characterize the major pathogens to which patients with cancer are susceptible, with a focus on the prevention, diagnosis, and treatment of major common and opportunistic infections. This portion of the guidelines highlights the sections on antifungal and antiviral prophylaxis. Antifungal and antiviral prophylaxis recommendations have expanded over the past few years...
July 2016: Journal of the National Comprehensive Cancer Network: JNCCN
Pedro M Azevedo, Tony R Merriman, Ruth K Topless, Nigel J Wilson, Sue Crengle, Diana R Lennon
INTRODUCTION: Rheumatic fever (RF) incidence among New Zealand (NZ) individuals of Polynesian (Māori and Pacific) ancestry remains among the highest in the world. Polymorphisms in the IL-6, IL1RN, and CTLA4 genes have been associated with RF, and their products are modulated by new medications. Confirmation of these previous associations could help guide clinical approaches. We aimed to test IL-6, IL-1RA (IL1RN), and CTLA4 functional SNPs in 204 rheumatic heart disease (RHD) patients and 116 controls of Māori and Pacific ancestry...
September 2016: Cytokine
Wilson Sui, Ifeanyi C Onyeji, Justin T Matulay, Maxwell B James, Marissa C Theofanides, Sven Wenske, Guarionex J DeCastro
OBJECTIVES: To determine whether perioperative blood transfusion is associated with worse 30-day postoperative outcomes in radical cystectomy patients. METHODS: Utilizing the National Surgical Quality Improvement Program database, we identified 2934 patients diagnosed with bladder cancer (International Classification of Diseases Ninth Revision codes 188-188.9) who underwent radical cystectomy (Current Procedure Terminology codes 51570, 51575, 51580, 51585, 51590, 51595, 51596) between 2005 and 2013...
September 2016: International Journal of Urology: Official Journal of the Japanese Urological Association
Jeffrey C Hageman, Carmen Hazim, Katie Wilson, Paul Malpiedi, Neil Gupta, Sarah Bennett, Amy Kolwaite, Abbigail Tumpey, Kristin Brinsley-Rainisch, Bryan Christensen, Carolyn Gould, Angela Fisher, Michael Jhung, Douglas Hamilton, Kerri Moran, Lisa Delaney, Chad Dowell, Michael Bell, Arjun Srinivasan, Melissa Schaefer, Ryan Fagan, Nedghie Adrien, Nora Chea, Benjamin J Park
The 2014-2016 Ebola virus disease (Ebola) epidemic in West Africa underscores the need for health care infection prevention and control (IPC) practices to be implemented properly and consistently to interrupt transmission of pathogens in health care settings to patients and health care workers. Training and assessing IPC practices in general health care facilities not designated as Ebola treatment units or centers became a priority for CDC as the number of Ebola virus transmissions among health care workers in West Africa began to affect the West African health care system and increasingly more persons became infected...
2016: MMWR Supplements
Brian A Lynch, Amenah Agunwamba, Patrick M Wilson, Seema Kumar, Robert M Jacobson, Sean Phelan, Valeria Cristiani, Chun Fan, Lila J Finney Rutten
While exposure to adverse family experiences (AFEs), subset of adverse childhood experiences (ACEs), has been associated with childhood obesity, less is known about the impact of exposures to each type of AFE. Using 2011-2012 National Survey of Children's Health data, we evaluated associations between exposure to individual AFEs and overweight/obesity status in children 10years or older, adjusting for socio-demographic factors. Caregivers reported their child's height, weight, and exposure to nine AFEs; body mass index (BMI) was classified by Center for Disease Control and Prevention's (CDC) guidelines...
September 2016: Preventive Medicine
Martin C S Wong, David K L Chan, Harry H X Wang, Wilson W S Tam, Clement S K Cheung, Bryan P Yan, Andrew J S Coats
BACKGROUND: Major international guidelines do not offer explicit recommendations on any specific angiotensin-converting enzyme inhibitor (ACEI) agent over another within the same drug group. This study compared the effectiveness of lisinopril vs. perindopril in reducing the incidence of hospital admission due to all-cause, cardiovascular disease and respiratory disease. METHODS: Adult patients who received new prescriptions of lisinopril or perindopril from 2001 to 2005 in all public hospitals and clinics in Hong Kong were included, and followed up for ≥2years...
September 15, 2016: International Journal of Cardiology
K L Ashwood, N Gillan, J Horder, H Hayward, E Woodhouse, F S McEwen, J Findon, H Eklund, D Spain, C E Wilson, T Cadman, S Young, V Stoencheva, C M Murphy, D Robertson, T Charman, P Bolton, K Glaser, P Asherson, E Simonoff, D G Murphy
BACKGROUND: Many adults with autism spectrum disorder (ASD) remain undiagnosed. Specialist assessment clinics enable the detection of these cases, but such services are often overstretched. It has been proposed that unnecessary referrals to these services could be reduced by prioritizing individuals who score highly on the Autism-Spectrum Quotient (AQ), a self-report questionnaire measure of autistic traits. However, the ability of the AQ to predict who will go on to receive a diagnosis of ASD in adults is unclear...
September 2016: Psychological Medicine
Anthony A Izuagie, Wilson M Gitari, Jabulani R Gumbo
The quest to reduce fluoride in groundwater to WHO acceptable limit of 1.5 mg/L to prevent diseases such as teeth mottling and skeletal fluorosis was the motivation for this study. Al/Fe oxide-modified diatomaceous earth was prepared and its defluoridation potential evaluated by batch method. The sorbent with pHpzc 6.0 ± 0.2 is very reactive. The maximum 82.3% fluoride removal attained in 50 min using a dosage of 0.3 g/100 mL in 10 mg/L fluoride was almost attained within 5 min contact time; 81.3% being the percent fluoride removal at 5 min contact time...
August 23, 2016: Journal of Environmental Science and Health. Part A, Toxic/hazardous Substances & Environmental Engineering
MeiLan K Han, Carlos H Martinez, David H Au, Jean Bourbeau, Cynthia M Boyd, Richard Branson, Gerard J Criner, Ravi Kalhan, Thomas J Kallstrom, Angela King, Jerry A Krishnan, Suzanne C Lareau, Todd A Lee, Kathleen Lindell, David M Mannino, Fernando J Martinez, Catherine Meldrum, Valerie G Press, Byron Thomashow, Laura Tycon, Jamie Lamson Sullivan, John Walsh, Kevin C Wilson, Jean Wright, Barbara Yawn, Patrick M Zueger, Surya P Bhatt, Mark T Dransfield
The burden of chronic obstructive pulmonary disease (COPD) in the USA continues to grow. Although progress has been made in the the development of diagnostics, therapeutics, and care guidelines, whether patients' quality of life is improved will ultimately depend on the actual implementation of care and an individual patient's access to that care. In this Commission, we summarise expert opinion from key stakeholders-patients, caregivers, and medical professionals, as well as representatives from health systems, insurance companies, and industry-to understand barriers to care delivery and propose potential solutions...
June 2016: Lancet Respiratory Medicine
Johann D Pitout, Wilson W Chan, Deirdre L Church
INTRODUCTION: Urinary tract infections (UTIs) are among the most common infectious diseases occurring in either the community or healthcare settings. A wide variety of bacteria are responsible for causing UTIs, however extra-intestinal pathogenic E. coli or ExPEC) remains the most common etiological agent. Since 2000, resistance to antibiotics emerged globally among ExPEC and is causing delays in appropriate therapy with subsequent increased morbidity and mortality. AREAS COVERED: The aim of this review article is to provide an overview on the definitions, etiology, treatment guidelines (including agents for infections due to antimicrobial resistant bacteria) of lower UTIs and to highlight recent aspects on antimicrobial resistance of ExPEC...
July 2016: Expert Review of Anti-infective Therapy
J Lane Wilson, Kellner L Pruett
Gastroesophageal reflux disease (GERD) is commonly managed in both primary and secondary care settings, as this condition occurs in patients of all ages and has a wide variety of clinical presentations. However, evidence suggests that GERD is commonly overdiagnosed and overtreated. Adherence to guidelines may help reduce the harms of overdiagnosis.
May 2016: North Carolina Medical Journal
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