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Pulmonary Hypertension in Sickle Cell Anemia

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181. Male Sexual Dysfunction

and impotence in homozygous sickle cell disease. Arch Intern Med, 1980. 140: 1434. 10. Lionnet, F., et al. Hemoglobin sickle cell disease complications: a clinical study of 179 cases. Haematologica, 2012. 97: 1136. 11. Olujohungbe, A.B., et al. A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial--the priapism in sickle cell study. J Androl, 2011. 32: 375. 12. Wespes, E., et al., EAU Guidelines Panel (...) in the general population. Urology, 2001. 57: 970. 6. Kulmala, R.V., et al. Priapism, its incidence and seasonal distribution in Finland. Scand J Urol Nephrol, 1995. 29: 93. 7. Furtado, P.S., et al. The prevalence of priapism in children and adolescents with sickle cell disease in Brazil. Int J Hematol, 2012. 95: 648. 8. Adeyoju, A.B., et al. Priapism in sickle-cell disease; incidence, risk factors and complications - an international multicentre study. BJU Int, 2002. 90: 898. 9. Emond, A.M., et al. Priapism

2019 European Association of Urology

182. Home Oxygen Therapy for Children

important areas for future research. Keywords : ; ; ; ... Show All Contents Section: Summary of Recommendations Cystic Fibrosis Bronchopulmonary Dysplasia Sleep-disordered Breathing Sickle Cell Disease Pulmonary Hypertension without Congenital Heart Disease Pulmonary Hypertension with Congenital Heart Disease Interstitial Lung Disease Implementation Introduction Methods Definition of Hypoxemia Children Younger Than 1 Year Old Children Aged 1 Year Old and Older Consequences of Untreated Hypoxemia (...) Cell Disease • For patients with sickle cell disease complicated by severe chronic hypoxemia, we suggest that home oxygen therapy be prescribed (conditional recommendation, very low-quality evidence) . Pulmonary Hypertension without Congenital Heart Disease • For patients with pulmonary hypertension without congenital heart disease complicated by chronic hypoxemia, we recommend that home oxygen therapy be prescribed (strong recommendation, very low-quality evidence) . Pulmonary Hypertension

2019 American Thoracic Society

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

British Society of Paediatric Radiology College of Occupational Therapists London Ambulance Service NHS Trust Paediatric Intensive Care Society Royal College of Emergency Medicine Royal College of General Practitioners Royal College of Nursing Royal College of Physicians Royal College of Speech & Language Therapists Sickle Cell & Young Stroke Survivors UK Forum on Haemoglobin Disorders The principles of this document are supported by The Royal College of Radiologists [June 2017] ii Foreword Stroke (...) University Hospitals NHS Trust, Nottingham British Paediatric Neurosurgical Group representative Dr Mary Mathias Consultant Paediatric Haematologist Great Ormond Street Hospital for Children NHS Foundation Trust, London British Society of Haematology representative Mrs Nancy McStravick Parent representative Ms Carol Ossai-Nwosu Chief Executive Officer Sickle Cell & Young Stroke Survivors, London Sickle cell representative Dr John Pappachan Consultant in Paediatric Cardiac Intensive Care Medicine

2017 Royal College of Paediatrics and Child Health

184. Outcome Measures Framework: Information Model Report

heart valves: the mitral, aortic, tricuspid or pulmonary. A defective valve can either fail to close completely and leak (regurgitation), or cannot open completely so that blood pumps through a smaller opening (stenosis). The severity of VHD varies. In mild cases there may be no symptoms, while in advanced cases, VHD may lead to congestive heart failure (CHF) and other complications. The decision to proceed with medical or surgical treatment is currently typically based on clinical symptoms (...) ClinicalTrials.gov was evaluated. This analysis was conducted with the intention of examining the reproducibility of an outcome measure using the data from within the entry itself. Within each entry, the level of detail available was defined by the existence of three components: an outcome definition (e.g., hypertension), a measurement used to meet the definition (e.g., Systolic Blood Pressure), and a threshold by which this measurement could be classified (e.g., >140mm Hg). The existence of one

2018 Effective Health Care Program (AHRQ)

185. CRACKCast E144 – High Altitude Medicine

hypoxia stress. Up to 20% of patients with hemoglobin sickle cell and sickle cell–thalassemia disease may experience a vaso-occlusive crisis, even under pressurized aircraft conditions. Oxygen is therefore advised for air travelers who have sickle cell disease. Pregnancy an increased incidence of complications in maternal, fetal, and neonatal life. Lower birth weight, increased premature birth Increased risk of gestational hypertension, preeclampsia Travel above 13000 ft is not advised Children Most (...) cerebral autoregulation Systemic hypertension (from strenuous exercise) Vasogenic edema from multiple mediators and damage to the blood brain barrier Failure of CSF buffering HAPE: HAPE results from overly exuberant increases in pulmonary arterial pressures (hypoxia-induced acute pulmonary hypertension) that lead to stress failures of the delicate pulmonary capillary beds → progressing to alveolar and interstitial edema. It’s this hypoxic pulmonary vasoconstriction response (HPVR) that leads to blood

2018 CandiEM

186. CRACKCast E174 – Genitourinary and Renal Tract Disorders

is the engorgement of the dorsal corpora cavernosa, resulting in dorsal penile erection lasting more than 4 hours. Three main types: Low flow (ischemic, painful!!!, “limb” threatening!) Sickle cell disease (>50% of pts with SSD have at least one episode) Malignancy (lymphoma; leukemia) Medications Cocaine / drugs of abuse High flow (non-ischemic, not painful, scary!) Trauma, AVM, congenital disease Neurogenic (penis not threatened – harbinger of spinal cord injury) MANAGEMENT: LOW FLOW ***draw an ABG off (...) : The peripheral blood smear shows microangiopathic changes such as schistocytes, teardrop cells, helmet cells, microspherocytes, and burr cells. [18] Describe the management of Hemolytic Uremic Syndrome (HUS) and Henoch Schonlein Purpura (HSP) in kids. HUS HSP a microangiopathic hemolytic anemia, is one of the most common causes of acute kidney injury in children. HUS is rare after 5 years of age, Hypersensitivity small vessel vasculitis affecting the joints, GI system and skin. Usually post URTI or group

2018 CandiEM

187. 2018 guidelines for the early management of patients with acute ischemic stroke

alteplase in patients presenting with AIS who have a history of diabetic hemorrhagic retinopathy or other hemorrhagic ophthalmic conditions is reasonable to recommend, but the potential increased risk of visual loss should be weighed against the anticipated benefits of reduced stroke-related neurological deficits. ( Class IIa; LOE B-NR ) Sickle cell disease IV alteplase for adults presenting with an AIS with known sickle cell disease can be beneficial. ( Class IIa; LOE B-NR ) Illicit drug use Treating (...) 1994, American Heart Association, Inc. Table 5. Options to Treat Arterial Hypertension in Patients With AIS Who Are Candidates for Acute Reperfusion Therapy* Class IIb, LOE C-EO Patient otherwise eligible for acute reperfusion therapy except that BP is >185/110 mm Hg: Labetalol 10–20 mg IV over 1–2 min, may repeat 1 time; or Nicardipine 5 mg/h IV, titrate up by 2.5 mg/h every 5–15 min, maximum 15 mg/h; when desired BP reached, adjust to maintain proper BP limits; or Clevidipine 1–2 mg/h IV, titrate

2018 American Academy of Neurology

188. 2018 Guidelines for the Early Management of Patients With Acute Ischemic Stroke: A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association

period (<14 d after delivery) have not been well established. ( Class IIb; LOE C-LD ) Ophthalmological conditions Use of IV alteplase in patients presenting with AIS who have a history of diabetic hemorrhagic retinopathy or other hemorrhagic ophthalmic conditions is reasonable to recommend, but the potential increased risk of visual loss should be weighed against the anticipated benefits of reduced stroke-related neurological deficits. ( Class IIa; LOE B-NR ) Sickle cell disease IV alteplase (...) for adults presenting with an AIS with known sickle cell disease can be beneficial. ( Class IIa; LOE B-NR ) Illicit drug use Treating clinicians should be aware that illicit drug use may be a contributing factor to incident stroke. IV alteplase is reasonable in instances of illicit drug use–associated AIS in patients with no other exclusions. ( Class IIa; LOE C-LD ) Stroke mimics The risk of symptomatic intracranial hemorrhage in the stroke mimic population is quite low; thus, starting IV alteplase

2018 American Heart Association

189. Male Sexual Dysfunction

and impotence in homozygous sickle cell disease. Arch Intern Med, 1980. 140: 1434. 10. Lionnet, F., et al. Hemoglobin sickle cell disease complications: a clinical study of 179 cases. Haematologica, 2012. 97: 1136. 11. Olujohungbe, A.B., et al. A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial--the priapism in sickle cell study. J Androl, 2011. 32: 375. 12. Wespes, E., et al., EAU Guidelines Panel (...) in the general population. Urology, 2001. 57: 970. 6. Kulmala, R.V., et al. Priapism, its incidence and seasonal distribution in Finland. Scand J Urol Nephrol, 1995. 29: 93. 7. Furtado, P.S., et al. The prevalence of priapism in children and adolescents with sickle cell disease in Brazil. Int J Hematol, 2012. 95: 648. 8. Adeyoju, A.B., et al. Priapism in sickle-cell disease; incidence, risk factors and complications - an international multicentre study. BJU Int, 2002. 90: 898. 9. Emond, A.M., et al. Priapism

2018 European Association of Urology

190. CRACKCast E116 – Arthritis

consumption have been shown to decrease risk. [10] List the microbiology of bacterial septic arthritis related to patient groups. Table 106.5: Microbiology of Bacterial Septic Arthritis Related to Patient PATIENTS ORGANISMS Neonates and infants Staphylococcus aureus, group B streptococcus, GNR bacteria Children Haemophilus influenzae, S. aureus Adolescents and young adults Neisseria gonorrhoeae, Chlamydia trachomatis Older adults S. aureus, Streptococcus, GNR bacteria Sickle cell anemia Salmonella (...) Sarcopenia, obesity Rheumatoid nodules** ocular/oral dryness (sjogren’s syndrome)* Episcleritis, scleritis, uveitis Interstitial fibrosis*, pneumonias, pulmonary hemorrhage Pericarditis, myocarditis, CAD Mesenteric vasculitis Glomerulonephritis Carpal tunnel syndrome; mononeuritis multiplex Anemia* [3] What are two presentations of gonococcal arthritis? Mono-oligoarticular arthritis True disseminated gonococcal infection (sometimes termed arthritis-dermatitis syndrome: bacteremia, diffuse migratory

2017 CandiEM

191. CRACKCast E090 – Liver and Biliary Tract

stones form when there is an imbalance between cholesterol in the bile and other solubilizing constituents (bile acids, phospholipids, lecithin, etc.) Risk factors for cholesterol stones : Age Female Massive obesity Rapid weight loss Cystic fibrosis Parity Drugs (clofibrate, OCPs) Familial tendency Risk factors for pigmented stones : Black stones: Intravascular hemolytic diseases (sickle cell, spherocytosis) Brown stones: Infections from Ascaris lumbricoides Clonorchis sinensis 15) Describe (...) ) Empiric ceftriaxone ? 6) List 6 stigmata of chronic liver disease and list 3 complications. This stuff is all due to loss of hepatic metabolic or synthetic function → decreased portal vein blood flow → portal hypertension. Stigmata Scleral / cutaneous icterus +/- pruritus Hepatomegaly Spider angiomata Caput medusa Patchy ecchymosis with thin skin Splenomegaly Gray / acholic stools Gynecomastia Muscle wasting Palmar erythema Dupuytren’s contractures Testicular atrophy Complications: Hepatocellular

2017 CandiEM

192. CRACKCast E101 – Stroke

in young patients Big categories Hypercoagulable states Vasospasm Post-infectious Traumatic Connective tissue disorders Specific situations Pregnancy Use of oral contraceptives Antiphospholipid antibodies Protein C and S deficiencies Sickle cell anemia Polycythemia Migraine syndromes Recreational drugs **Cocaine Amphetamines Recent infection from varicella or fungal meningitis Carotid / vertebral trauma leading to dissection Spinal manipulation / cough / yoga / vomiting 2) Differentiate between (...) dissection (systolic goal 100-120 mmHg) Hypertensive encephalopathy (10-15% reduction) Severe left ventricular heart failure. (amelioration of heart failure and improvement in pulmonary edema, which can often be achieved with a 10 to 15 percent reduction in blood pressure) 12) What are the inclusion criteria for fibrinolytic therapy in stroke? Endovascular therapy: Usually performed after tPA for strokes caused by proximal large vessel occlusions within 3 hours of onset. 13) What are the AHA exclusion

2017 CandiEM

193. CRACKCast E097 – Renal Failure

and other toxin exposures, rash, dark urine, fever and arthritis. Refer to Fig 87.1 for a structured approach to Azotemia 2) List clinical features of AKI Box 87.1 Cardiovascular Pulmonary edema Arrhythmia Hypertension Pericarditis Pericardial effusion Myocardial effusion Pulmonary embolism Metabolic Hyponatremia Hyperkalemia Hypocalcemia Hyperphosphatemia Hypermagnesemia Hyperuricemia Neurologic Asterixis Neuromuscular irritability Mental status changes Somnolence Coma Seizures Gastrointestinal Nausea (...) granular casts, renal epi cells or casts Normal +/- hyaline casts or granular casts 5) List the intrinsic renal diseases that cause AKI Box 87.4 Vascular Diseases Large-Vessel Diseases Renal artery thrombosis or stenosis Renal vein thrombosis Atheroembolic disease Small- and Medium-Sized Vessel Diseases Scleroderma Malignant hypertension Hemolytic uremic syndrome Thrombotic thrombocytopenic purpura HIV-associated microangiopathy Glomerular (Systemic) Diseases Systemic lupus erythematosus Infective

2017 CandiEM

194. CRACKCast E081 – Heart Failure

in the vascular endothelin response → hypertension 4) List the 5 most common disease processes resulting in HF and briefly describe the contribution of each 1) Coronary artery disease #1 cause Myocardial necrosis, fibrosis and scarring → dyskinesis Aneurysmal dilation of infarcted areas Cardiogenic shock — occurs when 40% of the LV muscle is infarcted Chronic coronary insufficiency: Diffuse ischemic cardiomyopathy -> Worsened in sickle cell disease, diabetes mellitus Ventricular remodelling – dilation (...) to sodium retention and volume expansion **** NSAIDS are BAD for people with CHF!!! Impair renal homeostasis, interfere with ACE & diuretics Metabolic / systemic Missed dialysis Anemia Thyroid Sepsis Acute stress states/emotional upset 8) List 6 historical predictors of acute HF and 6 clinical features of acute HF Hx Past history of HF PND SOBOE Orthopnea Nocturia Hx of any type of heart disease Px Hypertension Diaphoretic Pulmonary crackles Pulmonary wheezes (cardiac asthma – due to peribronchial edema

2017 CandiEM

195. 2016 AHA/ACC Guideline on the Management of Patients With Lower Extremity Peripheral Artery Disease

(microangiopathy) Toes, foot, leg Diabetic microangiopathy End-stage renal disease Thromboangiitis obliterans (Buerger’s) Sickle cell anemia Vasculitis (eg, Churg-Strauss, Henoch-Schonlein purpura, leukocytoclastic vasculitis, microscopic polyangiitis, polyarteritis nodosa) Scleroderma Cryoagglutination Embolic (eg, cholesterol emboli, thromboemboli, endocarditis) Thrombotic (eg, antiphospholipid antibody syndrome, Sneddon’s syndrome, warfarin skin necrosis, disseminated intravascular coagulation, livedoid (...) Angiography and Interventions Representative. ¶ACC/AHA Task Force on Clinical Practice Guidelines Liaison. #Inter-Society Consensus for the Management of Peripheral Arterial Disease Representative. **Society for Vascular Medicine Representative. ††Society of Interventional Radiology Representative. ‡‡Society for Clinical Vascular Surgery Representative. §§Society for Vascular Surgery Representative. ? ?American Association of Cardiovascular and Pulmonary Rehabilitation Representative. ¶¶Society

2017 American Heart Association

197. Heart Disease and Stroke Statistics 2017 Update: A Report From the American Heart Association

Cardiac Arrest . . . . . . . . . . . . . . e468 19. Subclinical Atherosclerosis . . . . . . . . . . . . e487 20. Coronary Heart Disease, Acute Coronary Syndrome, and Angina Pectoris. . . . . . . . . . e505 21. Cardiomyopathy and Heart Failure . . . . . . . . e523 22. Valvular Diseases . . . . . . . . . . . . . . . . e539 23. Venous Thromboembolism (Deep Vein Thrombosis and Pulmonary Embolism), Chronic Venous Insufficiency, Pulmonary Hypertension. . . e548 24. Peripheral Artery Disease and Aortic (...) and monitor healthy diets, a new chapter on venous disease and pulmonary hypertension (PH), new information on stroke in young adults, an enhanced focus on underserved and minority populations, a substantively expanded focus and chapter on the global burden of CVD, and further evidence-based approaches to changing behaviors, implementation strat- egies, and implications of the AHA’s 2020 Impact Goals. Below are a few highlights from this year’s Update. Current State of Cardiovascular Health in the United

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2017 American Heart Association

198. Cardiovascular Health in African Americans: A Scientific Statement From the American Heart Association (PubMed)

cardiac death). Both sudden cardiac arrest and sudden cardiac death are higher in African Americans compared with whites, primarily because of a higher burden of traditional and nontraditional (eg, sickle cell trait) CVD risk factors in African Americans. In the Oregon Sudden Unexpected Death Study, a community-based epidemiological study initiated in 2002 to collect information about out-of-hospital cardiac arrest, African Americans were twice as likely to experience sudden cardiac death. From 2002 (...) are particularly troublesome given the long lifetime of exposure to higher glucose levels among African Americans that contribute to mortality and vascular complications of diabetes mellitus. The diagnosis of diabetes mellitus is made by a combination of fasting glucose, postchallenge glucose, and hemoglobin A 1c . However, relying solely on hemoglobin A 1c could underestimate the prevalence of diabetes mellitus in African Americans because of disorders such as sickle cell trait that occur among individuals

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2017 American Heart Association

199. Renal Mass and Localized Renal Cancer

, collecting duct and unclassified RCC. 37 A number of uncommon or rare subtypes exist including but not limited to acquired cystic disease-associated RCC, clear cell (tubulo) papillary, and renal medullary carcinoma, which is an aggressive variant typically seen in patients with sickle cell trait. The most common benign tumors of the kidney include oncocytoma and angiomyolipoma (AML). An abbreviated version of the 2016 World Health Organization classification of renal neoplasms is detailed in Table 2. 38 (...) ' Network (PMN) Patient Safety and Quality of Care Accreditations and Reporting Patient Education Renal Mass and Localized Renal Cancer: AUA Guideline Published 2017 Renal Mass and Localized Renal Cancer: AUA Guideline focuses on the evaluation and management of clinically localized renal masses suspicious for renal cell carcinoma (RCC). Dignosis, patient counseling, and renal biopsy are covered in addition to various management strategies, including partial and radical nephrectomy, thermal ablation

2017 American Urological Association

200. Pulmonary complications of hemoglobinopathies. (PubMed)

Pulmonary complications of hemoglobinopathies. Hemoglobinopathies are diseases caused by genetic mutations that result in abnormal, dysfunctional hemoglobin molecules or lower levels of normal hemoglobin molecules. The most common hemoglobinopathies are sickle cell disease (SCD) and the thalassemias. In SCD, an abnormal hemoglobin alters the erythrocyte, causing a chronic hemolytic anemia, which can lead to pulmonary parenchymal damage and impaired vascular function. Pulmonary complications (...) of SCD include the acute chest syndrome (ACS), reactive airways disease, pulmonary hypertension (PH), and pulmonary fibrosis. Episodes of ACS and the development of PH both increase the risk of death in patients with SCD. Both α and β thalassemia are characterized by impaired production of hemoglobin subunits, and severity of disease varies widely. Although screening studies suggest that PH is a common complication for patients with thalassemia, its impact on survival is unknown. Understanding

2010 Chest

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