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Factor IX Deficiency

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181. Bruising

disorders are caused by a reduction or inhibition of circulating clotting factors, and may be inherited or acquired. Be aware that a negative family history does not exclude a genetically inherited disorder. Haemophilia A (factor VIII deficiency) and haemophilia B (factor IX deficiency) There are X-linked conditions, affecting 1 in 5000 males and 1 in 30,000 males respectively. The most severe forms occur almost exclusively in males. One third of cases arise secondary to new genetic mutations, where (...) . Liver disease can cause impaired synthesis of clotting factors. Vitamin K deficiency This can result in functional deficiencies of factors II, VII, IX, and X, and proteins C and S, and can cause bleeding in an infant in the first weeks of life, when it is known as Haemorrhagic Disease of the Newborn (HDN). HDN is more prevalent in exclusively breastfed babies, and those with an inadequate or omitted dose of prophylactic vitamin K after birth. Bleeding can vary from bruising or petechiae in the first

2017 Prodigy

182. Antiocoagulation - oral

ischaemia. How do anticoagulants work? Warfarin is a coumarin derivative that acts by inhibiting vitamin K dependent clotting factors (II, VII, IX, X) as well as the anticoagulant proteins C and S [ ]. Warfarin had been used for decades to treat and prevent venous thromboembolism. Apixaban, dabigatran, edoxaban and rivaroxaban are newer anticoagulants with a novel mode of action: Apixaban, edoxaban and rivaroxaban are direct and reversible inhibitors of factor Xa. Inhibition of factor Xa prevents (...) No NICE quality standards were found during the review of this topic. Background information Licensed indications What are the indications for apixaban? Apixaban is licensed for the [ ] : Prevention of stroke and systemic embolism in adults with non-valvular atrial fibrillation (NVAF), with one or more of the following risk factors: Previous stroke or transient ischaemic attack. Age 75 years or more. Hypertension. Diabetes mellitus. Symptomatic heart failure, New York Heart Association (NYHA) Class 2

2017 Prodigy

183. Palliative Care for the Patient with Incurable Cancer or Advanced Disease: Part 3: Grief and Bereavement

grieving, but it is helpful to provide a listening ear, to be supportive, and to provide information. • Distinguish grief from depression and treat grief-related major depression once you are confident it is pathological. • In the case of Prolonged Grief Disorder (complicated grief), assess and take note of any risk factors or concerns. • Primary care providers play a key role in the continued monitoring of patients’ grief responses and may refer the patient to grief counselling and treatment options (...) for hastened death. If present, assess for suicide risk. • Focus on personal strengths and coping mechanisms: what has worked in the past? • Protective factors / resiliency for a patient or caregiver: o Has an internalized belief in his / her own ability to cope effectively. o Perceives the need for AND is willing to access social support. o Is predisposed to a high level of optimism / positive state of mind. o Has spiritual / religious beliefs that assist in coping with the death. All of us grieve

2017 Clinical Practice Guidelines and Protocols in British Columbia

184. Stroke in childhood - clinical guideline for diagnosis, management and rehabilitation

. The most important factor in stroke care in adults is being managed on a stroke unit by staff with specific expertise and interest in the disease. Stroke units have never been tested for childhood stroke and as a result are not even mentioned in these guidelines. Clot busting treatment for ischemic stroke, or clot removal, are becoming mainstays of care in adults, yet there is very little evidence for these treatments in children because research has not been done. These guidelines are the first stage (...) those who have been involved in producing such an important clinical guideline. Professor Anne Greenough Professor of Clinical Respiratory Physiology, King’s College London RCPCH Vice President for Science & Research v Contents Endorsement i Foreword ii Preface iv Acknowledgments vii Funding vii Guideline development group (GDG) vii RCPCH team ix Guideline preparation ix Parent and young people representation x Delphi panellists* x Stakeholders xi Intellectual property xi Acronyms and abbreviations

2017 Royal College of Paediatrics and Child Health

185. Effects of Dietary Sodium and Potassium Intake on Chronic Disease Outcomes and Risks

No. 18-EHC009-EF June 2018ii Key Messages Purpose of Review To synthesize the evidence regarding the effects of dietary sodium reduction and increased potassium intake on blood pressure and risk for cardiovascular diseases (CVD) and renal disease outcomes and related risk factors. Key Messages • Decreasing dietary sodium intake most likely reduces blood pressure in normotensive adults and more so in those with hypertension. • Higher sodium intake may be associated with greater risk for developing (...) Heart and Vascular Center Lebanon, NH Stephen R. Daniels, M.D., Ph.D. Chairman, Department of Pediatrics University of Colorado School of Medicine Pediatrician-in-Chief L. Joseph Butterfield Chair in Pediatrics Children's Hospital Colorado Aurora, CO Bonita Faulkner, M.D., FAHA, FASH Professor Emeritus of Medicine and Pediatrics Thomas Jefferson University Philadelphia, PA Joachim Ix, M.D., M.A.S., FASN Chief, Division of Nephrology Professor of Medicine, UC San Diego La Jolla, CA Jens Marc Titze

2018 Effective Health Care Program (AHRQ)

186. Emicizumab for Hemophilia A with Inhibitors: Effectiveness and Value

WTP Willingness to pay ©Institute for Clinical and Economic Review, 2018 Page ES1 Evidence Report: Emicizumab for Hemophilia A with Inhibitors Return to Table of Contents Executive Summary Background Hemophilia A Hemophilia A is a condition of increased tendency to bleed due to an inherited deficiency of factor VIII, which disrupts the clotting cascade (Figure ES1). Hemophilia A has X-linked recessive inheritance, and so predominantly affects males. It is the most common form of hemophilia (...) to as “emicizumab” in this report) is a monoclonal antibody with dual targets (“bispecific”) that allow it to bridge activated factor IX and factor X, the role normally played by activated factor VIII in the clotting cascade (see Figure 1.1 in main report). 13 Emicizumab was approved by the United States (US) Food and Drug Administration (FDA) on November 16, 2017 as a prophylactic treatment for hemophilia A in patients who have inhibitors to factor VIII. 16 Emicizumab is administered subcutaneously

2018 California Technology Assessment Forum

187. Physiologic Predictors of Severe Injury: Systematic Review

Compromise 49 Respiratory Rate 50 Oxygen Saturation 53 Airway/Ventilatory Support 56 Other Respiratory Measures 57 Key Question 3: Combination Measures 59 Combinations of Physiologic Measures and Glasgow Coma Scale 64 Combinations of Physiologic Measures Without Glasgow Coma Scale 77 Key Questions 1a, 2a, and 3a: Differences Across Age Groups 80 Pediatrics 80 Older Individuals 84 Discussion 90 ix Key Findings 90 Findings in Relationship to What Is Already Known 92 Applicability 92 Limitations (...) support. 5 If steps 1 and 2 do not specify the patient as requiring transport to a major trauma center, steps 3 and 4 consider the mechanism of injury and additional factors such as age and comorbidities. The purpose of this systematic review is to identify and summarize the research evidence evaluating measures of circulatory and respiratory compromise, focusing on measures that can be used in the field to triage trauma patients. This review is designed to help inform decisions about what measures

2018 Effective Health Care Program (AHRQ)

188. Regional Anesthesia in the Patient Receiving Antithrombotic or Thrombolytic Therapy

problem and a significant source of morbidity and mortality. Nearly all hospitalized patients have at least 1 risk factor for thromboembolism; approximately 40% have 3 or more risk factors ( ). Consequently, the majority of hospitalized patients are candidates for thromboprophylaxis. The agent, dosing regimen, and duration of thromboprophylaxis are based on identification of risk factors, both individual (eg, age, sex, previous history of thromboembolism) and group-specific (eg, primary reason (...) for hospitalization, surgery, medical illness) ( and ). Depending on the risks of thromboembolism and bleeding, thromboprophylaxis may be achieved with compression stockings, intermittent compression devices, with medications (chemoprophylaxis), or a combination of both. Because an individualized approach to thromboprophylaxis is complex, most recommendations are group specific, with modifications based on the presence/absence of additional risk factors. Guidelines for antithrombotic therapy including appropriate

2018 American Society of Regional Anesthesia and Pain Medicine

189. Cancer Treatment Pathways

and drug schedules (i.e. the interval between doses) in selection of a Pathway regimen. Selecting a Pathway depends upon a number of factors – the type of cancer, the stage of disease, and the biomarkers or specific genetic profile of the cancer. Within each cancer type, separate Pathways are usually available for early stage and advanced cancer, sub-types of cancer (e.g. HER2 positive) and different lines of therapy. Pathways are not available for every medical condition, but are intended (...) to be applicable for individuals with the most common cancer types. Selecting the best cancer treatment depends upon a number of factors – the type of cancer, the stage, the biomarkers or specific genetic profile of the cancer, and unique aspects of each individual’s medical condition. Given the complexity of cancer and all of the unique individual circumstances, it would not be possible to have a Pathway option available for every specific situation. The treating oncologist will determine if, in his/her

2018 AIM Specialty Health

190. Breastfeeding Programs and Policies, Breastfeeding Uptake, and Maternal Health Outcomes in Developed Countries

(with no additional breastfeeding support services) may not be effective for increasing breastfeeding initiation rates. • For women enrolled in the WIC Program, peer-support interventions offered by WIC agencies may improve rates of breastfeeding initiation and duration. • Breastfeeding is associated with reduced maternal risk of breast and ovarian cancer, hypertension, and type 2 diabetes. • Workplace, school-based, and community-based interventions and underlying socioeconomic factors need further research.iii (...) , Switzerland Laurie Nommsen-Rivers, Ph.D., R.D., IBCLC University of Cincinnati Cincinnati, OH Leanne Redman, Ph.D. Pennington Biomedical Research Center Baton Rouge, LA ix Breastfeeding Programs and Policies, Breastfeeding Uptake, and Maternal Health Outcomes in Developed Countries Structured Abstract Objectives. To summarize the effectiveness of community, workplace, and health care system– based programs and policies aimed at supporting and promoting breastfeeding and determine the association between

2018 Effective Health Care Program (AHRQ)

191. Interventional Spine and Pain Procedures in Patients on Antiplatelet and Anticoagulant Medications

Procite Reference Manager Save my selection doi: 10.1097/AAP.0000000000000700 CHRONIC AND INTERVENTIONAL PAIN: SPECIAL ARTICLE Free The American Society of Regional Anesthesia and Pain Medicine (ASRA) 2012 survey of meeting attendees showed that existing ASRA anticoagulation guidelines for regional anesthesia were insufficient for their needs. Those surveyed agreed that procedure-specific and patient-specific factors required separate guidelines for pain and spine procedures. In response, a guidelines (...) outcomes. As with any clinical guideline recommendation, these are subject to revision as knowledge of specific complications advances. | DISCUSSION Pain-specific procedural guidelines are important because the technical and anatomical considerations for pain interventions are significantly different than for peripheral regional anesthesia technique. These factors can be divided into procedure-specific factors and patient-specific factors. The spectrum of interventional spine and pain procedures is far

2018 American Society of Regional Anesthesia and Pain Medicine

192. Sodium and Potassium Intake: Effects on Chronic Disease Outcomes and Risks

-EHC009-EF June 2018ii Key Messages Purpose of Review To synthesize the evidence regarding the effects of dietary sodium reduction and increased potassium intake on blood pressure and risk for cardiovascular diseases (CVD) and renal disease outcomes and related risk factors. Key Messages • Decreasing dietary sodium intake most likely reduces blood pressure in normotensive adults and more so in those with hypertension. • Higher sodium intake may be associated with greater risk for developing (...) Heart and Vascular Center Lebanon, NH Stephen R. Daniels, M.D., Ph.D. Chairman, Department of Pediatrics University of Colorado School of Medicine Pediatrician-in-Chief L. Joseph Butterfield Chair in Pediatrics Children's Hospital Colorado Aurora, CO Bonita Faulkner, M.D., FAHA, FASH Professor Emeritus of Medicine and Pediatrics Thomas Jefferson University Philadelphia, PA Joachim Ix, M.D., M.A.S., FASN Chief, Division of Nephrology Professor of Medicine, UC San Diego La Jolla, CA Jens Marc Titze

2018 Effective Health Care Program (AHRQ)

193. A Guide to Utilization of the Microbiology Laboratory for Diagnosis of Infectious Diseases: 2018 Update by the Infectious Diseases Society of America and the American Society for Microbiology

. Ocular Infections IV. Soft Tissue Infections of the Head and Neck V. Upper Respiratory Tract Bacterial and Fungal Infections VI. Lower Respiratory Tract Infections VII. Infections of the Gastrointestinal Tract VIII. Intra-abdominal Infections IX. Bone and Joint Infections X. Urinary Tract Infections XI. Genital Infections XII. Skin and Soft Tissue Infections XIII. Arthropod-Borne Infections XIV. Viral Syndromes XV. Blood and Tissue Parasite Infections Contents Introduction and Executive Summary I (...) . Bloodstream Infections and Infections of the Cardiovascular System II. Central Nervous System Infections III. Ocular Infections IV. Soft Tissue Infections of the Head and Neck V. Upper Respiratory Tract Bacterial and Fungal Infections VI. Lower Respiratory Tract Infections VII. Infections of the Gastrointestinal Tract VIII. Intra-abdominal Infections IX. Bone and Joint Infections X. Urinary Tract Infections XI. Genital Infections XII. Skin and Soft Tissue Infections XIII. Arthropod-Borne Infections XIV

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2018 Infectious Diseases Society of America

194. Guideline for the management of knee and hip osteoarthritis

tomography DALYs disability-adjusted life-years DHA docosahexaenoic acid DMOADs disease-modifying osteoarthritis drugs DMSO dimethylsulfoxide EMA European Medicines Agency EPA eicosapentaenoic acid ESR erythrocyte sedimentation rate FGF fibroblast growth factor GP general practitioner GRADE Grading of Recommendations Assessment, Development and Evaluation HANDI Handbook of non-drug interventions HOOS Hip Disability and Osteoarthritis Outcome Score ICTRP International Clinical Trials Registry Platform (...) IgG2 immunoglobulin G2 IL-1 interleukin-1 IPDAS International Patient Decision Aids Standards KOOS Knee Injury and Osteoarthritis Outcome Score MBS Medicare Benefits Schedule MD mean difference MRI magnetic resonance imaging MSC mesenchymal stem cell MSM methylsulfonylmethane NATSIHS National Aboriginal and Torres Strait Islander Health Survey NGF nerve growth factor NHMRC National Health and Medical Research Council NICE National Institute for Health and Care Excellence NPRS Numeric Pain Rating

2018 Clinical Practice Guidelines Portal

195. European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas

at presentation to identify secretory tumours that may indicate specific therapies or endocrine deficiencies, which if left untreated would contribute to patient morbidity. Assessment of pituitary endocrine function should be performed, at appropriate intervals (3–6 months on an individualised basis), both to characterise potential biomarkers of disease progression to monitor in parallel with imaging studies, and to manage endocrine deficiencies. R 2.1.4 In patients with aggressive pituitary tumours (...) -67 proliferative index evaluation. The p53 immunodetection and the mitotic count should be evaluated at least, when the Ki-67 index is ≥3% (+000). Reasoning Based on immunohistochemistry (IHC), pituitary tumours are classified into somatotroph (GH, Pit 1 positive), lactotroph (PRL, Pit1 and ER positive), corticotroph (ACTH, Tpit positive), thyrotroph (TSH, Pit1 positive), gonadotroph (FSH/LH, SF1 positive), null cell (negative for hormones and transcription factors) tumours and plurihormonal

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2018 European Society of Endocrinology

196. HTA of extending the HPV vaccination to boys

in the discount rate from 5% to 4%, it is likely that gender neutral 9-valent vaccination would be considered cost-effective at €45,000 per quality-adjusted life year (QALY). ? HPV vaccination of boys provides direct protection against HPV-related disease to boys. It also provides indirect herd protection to girls who have not been vaccinated. Other important factors to consider include the additional protection provided by a gender neutral programme to vulnerable groups (for example, men who have sex (...) , there are other important factors to consider for including boys in the national HPV immunisation schedule. HPV vaccination of boys provides direct protection against HPV- related disease to those individuals. It also provides indirect herd protection to girls, and ensures vulnerable groups who do not benefit from the herd Health Technology Assessment (HTA) of HPV vaccination of boys Health Information and Quality Authority Page 21 of 450 effects of girls only being vaccinated (for example, men who have sex

2018 Health Information and Quality Authority

197. Improving Quality of Life: Substance Use and Aging

physical or mental health, we are quick to assume it’s the result of “getting older” and might not consider the possibility of substance use as a contributing factor. To find a path forward that recognizes the needs of this growing population and responds effectively, we need to set our assumptions aside and look to the evidence. We need to build a more thorough understanding of what’s happening to people during this stage of life and why they might be more susceptible to experiencing harms associated (...) (SUD) or experiencing harmful interactions between prescription and non-prescription medications. This chapter presents evidence on the prevalence of and contributing factors behind some of the psychiatric disorders that commonly co-occur with SUDs, including schizophrenia, mood and anxiety disorders, post-traumatic stress disorder, and Alzheimer’s and Parkinson’s diseases. It also looks at how SUDs (and their lack of treatment) can worsen physical health issues such as coronary heart disease

2018 Canadian Centre on Substance Abuse

198. Hypercoagulable state

) antithrombin deficiency protein C deficiency protein S deficiency plasminogen deficiency elevated fibrinogen dysfibrinogenaemia factor V Leiden prothrombin gene mutation (G-20210-A) elevated factor VIII levels (>150 U/L) elevated levels of factor IX or XI hyperhomocysteinaemia sickle cell disease elevated levels of thrombin-activatable fibrinolysis inhibitor (TAFI) Diagnostic investigations FBC peripheral blood smear activated partial thromboplastin time fibrinogen prothrombin time D-dimer serum albumin (...) identified. Many factors contribute to the hypercoagulable state. Inherited thrombophilia affects up to 10% of the population; acquired risk factors, such as obesity and hormonal therapy, are frequent in the community. Most patients with heritable thrombophilia remain asymptomatic. Venous thromboembolism is the most common manifestation of the hypercoagulable state. It can be prevented in some patients, with identification of those at risk and appropriate thromboprophylaxis during periods of high risk

2016 BMJ Best Practice

199. Coagulation factors IX through XIII and the risk of future venous thrombosis: the Longitudinal Investigation of Thromboembolism Etiology. (PubMed)

Coagulation factors IX through XIII and the risk of future venous thrombosis: the Longitudinal Investigation of Thromboembolism Etiology. Higher levels of procoagulant factors and factor XII deficiency may be risk factors for first venous thromboembolism (VTE). We studied associations of coagulation factors IX through XIII with risk of future VTE in 2 general population samples. Using a nested case-control study combining the 21 860 participants of the Atherosclerosis Risk in Communities study (...) and the Cardiovascular Health Study, we determined antigenic levels of these coagulation factors in primarily pre-event blood samples from 462 participants who subsequently developed VTE and 1047 participants who remained free of VTE. Only elevated levels of factors IX and XI were associated with increased risk of VTE after adjustment for age, sex, race, and study. For factor IX, the odds ratio (OR) was 1.4 (95% confidence interval [CI], 1.0-2.0) comparing the top to bottom quintile. The OR for factor XI was higher

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2009 Blood

200. Preventive chemotherapy to control soil-transmitted helminth infections in at-risk population groups

warranty of any kind, either expressed or implied. The responsibility for the interpretation and use of the material lies with the reader. In no event shall WHO be liable for damages arising from its use. Photo credit (front cover): Frederic Noy Cover design and layout: Alberto March (Barcelona, Spain)VII Guideline: preventive chemotherapy to control soil-transmitted helminth infections in at-risk population groups CONTENTS PUBLICATION HISTORY IX ACKNOWLEDGEMENTS IX Financial support X GLOSSARY XI (...) of adverse effects in specific populations, such as individuals living with HIV (especially in children and those on antiretroviral therapy), breastfeeding mothers and their infants, pregnant mothers and their unborn babies, and infants (less than 12 months of age); and l factors that influence compliance with large-scale preventive chemotherapy programmes, including the values and preferences of children, adolescent girls and adult women, as well as the prevailing social attitudes about treatment

2017 World Health Organisation Guidelines

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