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https://www.readbyqxmd.com/read/28648663/effect-of-enzyme-replacement-therapy-with-alglucosidase-alfa-myozyme%C3%A2-in-12-patients-with-advanced-late-onset-pompe-disease
#1
Constantinos Papadopoulos, David Orlikowski, Hélène Prigent, Arnaud Lacour, Céline Tard, Alain Furby, Julien Praline, Guilhem Solé, Jean-Yves Hogrel, Marie De Antonio, Claudio Semplicini, Joelle Deibener-Kaminsky, Pierre Kaminsky, Bruno Eymard, Nadjib Taouagh, Barbara Perniconi, Dalil Hamroun, Pascal Laforêt
BACKGROUND: The efficacy of enzyme replacement therapy (ERT) in patients at an advanced stage of Pompe disease has only been addressed in a few studies. Our objective was to assess the long term effects of ERT in a cohort of patients with severe Pompe disease. METHODS: We identified patients from the French Pompe Registry with severe respiratory failure and permanent wheelchair use (assisted walk for a few meters was allowed) when starting ERT. Patients' medical records were collected and reviewed and respiratory and motor functions, before ERT initiation and upon last evaluation were compared...
June 20, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28647978/-severe-cases-with-hand-foot-and-mouth-disease-data-based-on-national-pilot-hand-foot-and-mouth-disease-surveillance-system
#2
Y M Zheng, Z R Chang, L L Jiang, H Ji, G P Chen, P Luo, J J Pan, X L Tian, L L Wei, D Huo, Z P Miao, X N Zou, J H Chen, Q H Liao
Objective: To investigate the clinical severity, etiological classification and risk factors of severe cases with hand, foot and mouth disease (HFMD). Methods: A total of 1 489 records on severe and fatal HFMD cases reported to the national pilot surveillance system of HFMD were used to analyze the demographic, medical treatment, etiological classification of the cases. Treatment outcome related risk factors were also studied with multi-variable stepwise logistic regression method. Results: Seven out of the 1 489 severe HFMD cases died of this disease...
June 10, 2017: Zhonghua Liu Xing Bing Xue za Zhi, Zhonghua Liuxingbingxue Zazhi
https://www.readbyqxmd.com/read/28647781/computer-navigation-assisted-surgery-for-musculoskeletal-tumors-a-closer-look-into-the-learning-curve
#3
Kevin Staats, Joannis Panotopoulos, Thomas M Tiefenboeck, Reinhard Windhager, Philipp T Funovics
BACKGROUND: Computer navigation-assisted surgery for musculoskeletal tumors has shown to reduce the risk of intra-lesional margins in resection. Experiences with this method are still limited to smaller case series. METHOD: We reviewed our first experiences in 24 patients in whom computer navigation-assisted surgery had been performed. In 7 of these patients (6 male and 1 female), this has influenced the surgical treatment plan and navigation was used for both tumor resection and reconstruction...
June 24, 2017: European Journal of Orthopaedic Surgery & Traumatology: Orthopédie Traumatologie
https://www.readbyqxmd.com/read/28646964/global-advocacy-needed-for-sepsis-in-children
#4
Sagar Dugani, Niranjan Kissoon
Sepsis in children is a complex syndrome that develops from various infections and results in 15-30% mortality in high-income countries and up to 50% or higher in low-income countries. Worldwide, this represents an annual burden of 30 million cases resulting in 8 million deaths. Not surprisingly, there is a significantly higher burden in low-income countries with children there being 18 times more likely to die before the age of 5 years compared to high-income countries. Factors such as commercial air travel, climate change, and unchecked population growth have contributed to the growing burden of infectious diseases and ensuing sepsis...
June 2017: Journal of Infection
https://www.readbyqxmd.com/read/28642353/when-sanctity-of-life-and-self-determination-clash-briggs-versus-briggs-2016-ewcop-53-implications-for-policy-and-practice
#5
Jenny Kitzinger, Celia Kitzinger, Jakki Cowley
In a landmark judgment in the English Court of Protection, the judge (Charles J) found it to be in the best interests of a minimally conscious patient for clinically assisted nutrition and hydration (CANH) to be withdrawn, with the inevitable consequence that the patient would die. In making this judgment, it was accepted that the patient's level of consciousness - if CANH were continued and rehabilitation provided - might improve, and that he might become capable of expressing emotions and making simple choices...
June 22, 2017: Journal of Medical Ethics
https://www.readbyqxmd.com/read/28628540/evaluating-disability-insurance-assistance-as-a-specific-intervention-by-physiatrists-at-a-cancer-center
#6
Jack B Fu, Melissa P Osborn, Julie K Silver, Benedict S Konzen, An Ngo-Huang, Rajesh Yadav, Eduardo Bruera
Because of their expertise, physiatrists provide disability insurance assistance for cancer survivors. In this brief report, we perform a descriptive retrospective analysis of all new (354) outpatient physiatry consultations from January 1, 2009, to December 31, 2013, at a National Cancer Institute Comprehensive Cancer Center. Disability and/or work accommodations were brought up at some point with the physiatrist during the duration of their care for 131 (37%) of 354 patients. More than 90% of the discussions took place during the first visit...
July 2017: American Journal of Physical Medicine & Rehabilitation
https://www.readbyqxmd.com/read/28627303/the-surprise-question-in-paediatric-palliative-care-a-prospective-cohort-study
#7
Kimberley Burke, Lucy Helen Coombes, Antoinette Menezes, Anna-Karenia Anderson
BACKGROUND: The question 'would you be surprised if this patient died in the next 12-months' is widely used for identifying adult patients in the last year of life. However, this has not yet been studied in children. AIM: To assess the prognostic accuracy of the surprise question when used by a multidisciplinary team to predict survival outcomes of children with life-limiting conditions over a 3 and 12 month period. DESIGN: A prospective cohort study...
June 1, 2017: Palliative Medicine
https://www.readbyqxmd.com/read/28626086/nursing-skill-mix-in-european-hospitals-cross-sectional-study-of-the-association-with-mortality-patient-ratings-and-quality-of-care
#8
Linda H Aiken, Douglas Sloane, Peter Griffiths, Anne Marie Rafferty, Luk Bruyneel, Matthew McHugh, Claudia B Maier, Teresa Moreno-Casbas, Jane E Ball, Dietmar Ausserhofer, Walter Sermeus
OBJECTIVES: To determine the association of hospital nursing skill mix with patient mortality, patient ratings of their care and indicators of quality of care. DESIGN: Cross-sectional patient discharge data, hospital characteristics and nurse and patient survey data were merged and analysed using generalised estimating equations (GEE) and logistic regression models. SETTING: Adult acute care hospitals in Belgium, England, Finland, Ireland, Spain and Switzerland...
July 2017: BMJ Quality & Safety
https://www.readbyqxmd.com/read/28624976/are-concerns-about-irremediableness-vulnerability-or-competence-sufficient-to-justify-excluding-all-psychiatric-patients-from-medical-aid-in-dying
#9
William Rooney, Udo Schuklenk, Suzanne van de Vathorst
Some jurisdictions that have decriminalized assisted dying (like Canada) exclude psychiatric patients on the grounds that their condition cannot be determined to be irremediable, that they are vulnerable and in need of protection, or that they cannot be determined to be competent. We review each of these claims and find that none have been sufficiently well-supported to justify the differential treatment psychiatric patients experience with respect to assisted dying. We find bans on psychiatric patients' access to this service amount to arbitrary discrimination...
June 17, 2017: Health Care Analysis: HCA: Journal of Health Philosophy and Policy
https://www.readbyqxmd.com/read/28622379/end-of-life-care-for-people-with-dementia-the-views-of-health-professionals-social-care-service-managers-and-frontline-staff-on-key-requirements-for-good-practice
#10
Richard Philip Lee, Claire Bamford, Marie Poole, Emma McLellan, Catherine Exley, Louise Robinson
BACKGROUND: Evidence consistently shows that people with advanced dementia experience suboptimal end of life care compared to those with cancer; with increased hospitalisation, inadequate pain control and fewer palliative care interventions. Understanding the views of those service managers and frontline staff who organise and provide care is crucial in order to develop better end of life care for people with dementia. METHODS AND FINDINGS: Qualitative interviews and focus groups were conducted from 2013 to 2015 with 33 service managers and 54 staff involved in frontline care, including doctors, nurses, nursing and care home managers, service development leads, senior managers/directors, care assistants and senior care assistants/team leads...
2017: PloS One
https://www.readbyqxmd.com/read/28621620/deep-brain-stimulation-for-the-early-treatment-of-the-minimally-conscious-state-and-vegetative-state-experience-in-14-patients
#11
Darko Chudy, Vedran Deletis, Fadi Almahariq, Petar Marčinković, Jasenka Škrlin, Veronika Paradžik
OBJECTIVE An effective treatment of patients in a minimally conscious state (MCS) or vegetative state (VS) caused by hypoxic encephalopathy or traumatic brain injury (TBI) is not yet available. Deep brain stimulation (DBS) of the thalamic reticular nuclei has been attempted as a therapeutic procedure mainly in patients with TBI. The purpose of this study was to investigate the therapeutic use of DBS for patients in VS or MCS. METHODS Fourteen of 49 patients in VS or MCS qualified for inclusion in this study and underwent DBS...
June 16, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28621461/the-use-of-eptifibatide-alone-or-in-combination-with-heparin-or-argatroban-for-suspected-thrombosis-in-patients-with-left-ventricular-assist-devices
#12
Abbas Bitar, Rajakrishnan Vijayakrishnan, Andrew Lenneman, Emma Birks, Todd Massey, Mark Slaughter, Dmitry Abramov
Pump thrombosis and hemolysis in patients with left ventricular assist devices (LVADs) are associated with significant morbidity and mortality. Intensification of anticoagulation has been suggested as potential therapy, with mixed results. The aim of this study is to assess the safety and efficacy of adding eptifibatide with or without an anticoagulation agent in managing patients with LVAD presenting with hemolysis and suspected pump thrombosis. This retrospective single center study included all patients who presented with their first episode of suspected pump thrombosis and were treated with eptifibatide with or without an anticoagulant between March 1, 2011 and July 30, 2015...
June 16, 2017: Artificial Organs
https://www.readbyqxmd.com/read/28616906/-diagnosis-and-surgical-treatment-of-lung-ground-glass-opacities-a-review-of-663-cases
#13
Shi-You Wei, Ke-Jia Zhao, Cheng-Lin Guo, Jian-Dong Mei, Qiang Pu, Lin Ma, Guo-Wei Che, Long-Qi Chen, Zhu Wu, Yun Wang, Ying-Li Kou, Yi-Dan Lin, Wei-Min Li, Lun-Xu Liu
OBJECTIVES: To retrospectively investigate the clinical characteristics, surgical treatments of the patients with lung ground-glass opacities (GGO). METHODS: All the patients, who underwent surgical resection of GGO in our department from Jan. 2013 to Dec. 2016 were retrospectively reviewed. The clinicpathological features were analyzed. RESULTS: A total of 663 patients were included in this study. The rate of malignancy was 92.6% (614/663)...
May 2017: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/28616601/minimally-invasive-surgery-for-gastric-cancer-in-brazil-current-status-and-perspectives-a-report-from-the-brazilian-laparoscopic-oncologic-gastrectomy-group-blogg
#14
REVIEW
Paulo Kassab, Wilson Luiz da Costa, Carlos Eduardo Jacob, Roberto de Moraes Cordts, Osvaldo Antônio Prado Castro, Leandro Cardoso Barchi, Ivan Cecconello, Amir Zeide Charruf, Felipe José Fernández Coimbra, Antônio Moris Cury, Alessandro Landskron Diniz, Igor Correia de Farias, Wilson Rodrigues de Freitas, André Luis de Godoy, Elias Jirjoss Ilias, Carlos Alberto Malheiros, Marcus Fernando Kodama Pertille Ramos, Heber Salvador de Castro Ribeiro, André Roncon Dias, Fábio Rodrigues Thuler, Osmar Kenji Yagi, Laércio Gomes Lourenço, Bruno Zilberstein
The minimally invasive surgery for gastric cancer in Brazil has begun about two years after the first laparoscopic gastrectomy (LG) performed by Kitano in Japan, in 1991. Although the report of first surgeries shows the year of 1993, there was no dissemination of the technique until the years 2010. At that time with the improvement of optical devices, laparoscopic instruments and with the publications coming from Asia, several Brazilian surgeons felt encouraged to go to Korea and Japan to learn the standardization of the LG...
2017: Translational Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28616283/the-use-of-thrombolytics-in-the-management-of-complex-pleural-fluid-collections
#15
Jessica Heimes, Hannah Copeland, Aditya Lulla, Marjulin Duldulao, Khaled Bahjri, Salman Zaheer, Jason M Wallen
BACKGROUND: To determine the efficacy of thrombolytics for the management of complex pleural fluid collections. METHODS: We reviewed patients that received alteplase for persistent loculated pleural fluid collections after simple tube drainage between July 01, 2007 and November 01, 2012. Our alteplase protocol is 6 mg of alteplase in 50 mL of normal saline injected into the pleural chest tube. The chest tube is clamped for four hours and then opened. Normally this is repeated daily for 2 to 3 days (d)...
May 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28604999/percutaneous-cardiac-support-during-myocardial-infarction-drastically-reduces-mortality-perspectives-from-a-swine-model
#16
Maria Giovanna Trivella, Alessandra Piersigilli, Fabio Bernini, Gualtiero Pelosi, Silvia Burchielli, Stefano Puzzuoli, Claudia Kusmic, Antonio L'Abbate
BACKGROUND/AIMS: Acute myocardial infarction (AMI) with cardiogenic shock (CS) remains the leading cause of in-hospital death in acute coronary syndromes. In the AMI-CS pig model we tested the efficacy of temporary percutaneous cardiorespiratory assist device (PCRA) in rescuing the failing heart and reducing early mortality. METHODS: In open-chest pigs we induced AMI by proximal left anterior descending coronary artery (LAD) ligation. Eight animals without PCRA (C group) were compared with 12 animals otherwise treated with PCRA (T group), starting approximately at 60 minutes post-occlusion and lasting 120-180 minutes...
June 6, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28592080/-complete-revascularization-for-multi-vessel-coronary-diseases-through-lower-ministernotomy-on-beating-heart-off-pump-surgery
#17
Z B Xiao, Y Zhang, J Z Guo, Y Y Li, Y P Zhang
Objective: To discuss a novel method of complete revascularization for multi-vessel coronary diseases on beating heart off-pump surgery through lower ministernotomy. Methods: Clinical data of 79 patients underwent ministernotomy off-pump coronary artery bypass from January 2015 to May 2016 at Department of Cardiac Surgery, Heart Center, Beijing Friendship Hospital, Capital Medical University were analyzed retrospectively. There were 62 male and 17 female patients, with an average age of (65±9) years. All the patients were multi-vessel coronary diseases and planned to receive coronary artery bypass grafting...
June 1, 2017: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/28591192/perinatal-outcomes-and-congenital-heart-defect-prognosis-in-53313-non-selected-perinatal-infants
#18
Donghua Xie, Hua Wang, Zhiyu Liu, Junqun Fang, Tubao Yang, Shujin Zhou, Aihua Wang, Jiabi Qin, Lili Xiong
OBJECTIVE: To evaluate perinatal outcomes and congenital heart defect (CHD) prognosis in a non-selected population. METHODS: The population-based surveillance data used in this assessment of CHDs were based on birth defect surveillance data collected from 2010-2012 in Liuyang City, China. Infants living with CHDs were followed up for 5 years to determine their prognosis. Prevalence, prenatal diagnosis, perinatal outcomes, and total and type-specific prognosis data were assessed using SPSS 18...
2017: PloS One
https://www.readbyqxmd.com/read/28589020/countdown-to-2015-an-analysis-of-donor-funding-for-prenatal-and-neonatal-health-2003-2013
#19
Catherine Pitt, Christopher Grollman, Melisa Martínez-Álvarez, Leonardo Arregoces, Joy E Lawn, Josephine Borghi
BACKGROUND: In 2015, 5.3 million babies died in the third trimester of pregnancy and first month following birth. Progress in reducing neonatal mortality and stillbirth rates has lagged behind the substantial progress in reducing postneonatal and maternal mortality rates. The benefits to prenatal and neonatal health (PNH) from maternal and child health investments cannot be assumed. METHODS: We analysed donor funding for PNH over the period 2003-2013. We used an exhaustive key term search followed by manual review and classification to identify official development assistance and private grant (ODA+) disbursement records in the Countdown to 2015 ODA+ Database...
March 2017: BMJ Global Health
https://www.readbyqxmd.com/read/28588957/spiritual-issues-of-people-living-and-dying-with-advanced-heart-failure-in-kenya-a-qualitative-serial-interview-study
#20
Kellen N Kimani, Scott A Murray, Liz Grant
BACKGROUND: Exploring patients' spiritual issues is vital for informing holistic care. The spiritual concerns of patients living and dying with advanced heart failure in sub-Saharan Africa remains unknown. Our aim was to describe spiritual issues in the context of the lived experiences of patients with heart failure in Kenya. METHODS: We purposively recruited 18 patients admitted with advanced heart failure at a rural district hospital in Kenya. We conducted serial in-depth interviews with patients at 0, 3 and 6 months after recruitment...
November 2016: BMJ Global Health
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