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Pulmonary Hypertension in Sickle Cell Anemia

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261. AIUM Practice Parameter for the Performance of a Transcranial Doppler Ultrasound Examination for Adults and Children

) ? ?ultrasound ? ?is ? ?a ? ?noninvasive ? ?technique ? ?that ? ?assesses ? ?blood ? ?flow within ? ?the ? ?circle ? ?of ? ?Willis ? ?and ? ?the ? ?vertebrobasilar ? ?system. II. Indications A. Indications ? ?for ? ?a ? ?TCD ? ?ultrasound ? ?examination ? ?of ? ?children ? ?and ? ?adults ? ?include ? ?but are ? ?not ? ?limited ? ?to: 1. Evaluation ? ?of ? ?sickle ? ?cell ? ?disease ? ?to ? ?determine ? ?stroke ? ?risk. ? 1–3 2. Detection ? ?and ? ?follow-up ? ?of ? ?stenosis ? ?or ? ?occlusion (...) ? ?and ? ?management ? ?in sickle ? ?cell ? ?disease. ? ??Blood ? ??2016; ? ?127:829–838. 2. Brousse ? ?V, ? ?Kossorotoff ? ?M, ? ?de ? ?Montalembert ? ?M. ? ?How ? ?I ? ?manage ? ?cerebral ? ?vasculopathy ? ?in children ? ?with ? ?sickle ? ?cell ? ?disease. ? ??Br ? ?J ? ?Haematol ? ??2015; ? ?170:615–625. 3. Verlhac ? ?S. ? ?Transcranial ? ?Doppler ? ?in ? ?children. ? ??Pediatr ? ?Radiol. ? ??2011; ? ?41(suppl ? ?1):S153–S165. © ? ?2017 ? ?American ? ?Institute ? ?of ? ?Ultrasound ? ?in ? ?Medicine 14750

2017 American Institute of Ultrasound in Medicine

262. Cardiovascular Health in African Americans: A Scientific Statement From the American Heart Association Full Text available with Trip Pro

remained ≈10% to 12% higher than rates among non-Hispanic whites and Mexican Americans since 1999 to 2000 (the year that the National Health and Nutrition Examination Survey [NHANES] became semiannual). Percent of African admixture in African Americans and other racial/ethnic groups is positively associated with blood pressure (BP) levels and the prevalence of hypertension. , The origins of adult differences in hypertension begin in youth. African American boys and girls have higher BP levels (...) difference in SBP is associated with an 8% (95% CI, 0–16) increase in the stroke risk in whites but a 24% (95% CI, 14–35) increase in African Americans. Within strata of SBP, stroke risk goes up with an increasing number of classes of antihypertensive medications used to treat high BP (RR, 1.42 for 1 class up to 2.48 for ≥3 classes). Even when treatment recommendations are followed for African Americans, stroke risks remain elevated, suggesting primordial prevention as the best strategy to eliminate

2017 American Heart Association

263. Renal Mass and Localized Renal Cancer

, complete staging is typically finalized with chest radiography (x-ray) or chest CT. Chest CT scan should be obtained selectively, primarily for patients with pulmonary symptoms or abnormal chest x-ray, or for patients with high-risk disease. 47 , 48 Bone scans should be reserved primarily for patients with bone pain or elevated alkaline phosphatase and brain imaging for those with neurologic symptoms. 49 - 51 Importantly, positron emission tomography (PET) scan has no role in the routine evaluation (...) panel, complete blood count, and urinalysis. Metastatic evaluation should include chest imaging to evaluate for possible thoracic metastases. (Clinical Principle) For patients with a solid or complex cystic renal mass, physicians should assign CKD stage based on GFR and degree of proteinuria. (Expert Opinion) Counseling In patients with a solid or Bosniak 3/4 complex cystic renal mass, a urologist should lead the counseling process and should consider all management strategies. A multidisciplinary

2017 American Urological Association

264. Carrier Screening for Genetic Conditions

substitution in the β-globin gene; this alteration causes a substitution of valine for glutamic acid in the number six position of the β-globin polypeptide. Asymptomatic individuals with heterozygous hemoglobin S genotypes (carriers) are said to have sickle cell trait. The most severe form of the disease, hemoglobin SS (homozygous hemoglobin S), is called sickle cell anemia. Sickle cell disorders are found not only in patients who have the hemoglobin genotype SS, but also in those who have hemoglobin S (...) oxygen tension, in which the red blood cells become distorted into various shapes, some of which resemble sickles. The distorted red cells lead to increased viscosity, hemolysis, and anemia and a further decrease in oxygenation. When sickling occurs within small blood vessels, it can interrupt blood supply to vital organs (vasoocclusive crisis). Repeated vasoocclusive crises result in widespread microvascular obstruction with interruption of normal perfusion and function of several organs, including

2017 American College of Obstetricians and Gynecologists

265. Imaging Program Guidelines: Pediatric Imaging

Imaging Program Guidelines: Pediatric Imaging Clinical Appropriateness Guidelines: Advanced Imaging Imaging Program Guidelines: Pediatric Imaging Effective Date: November 20, 2017 Proprietary Guideline Last Revised Last Reviewed Administrative 07-26-2016 07-26-2016 Head and Neck 11-01-2016 11-01-2016 Chest 08-27-2015 07-26-2016 Abdomen and Pelvis 11-01-2016 11-01-2016 Spine 08-27-2015 07-26-2016 Extremity 08-27-2015 07-26-2016 Copyright © 2017. AIM Specialty Health. All Rights Reserved 8600 W (...) ) Brain – Pediatrics 24 PET Brain Imaging – Pediatrics 25 CT Orbit, Sella Turcica, Posterior Fossa, Temporal Bone, including Mastoids – Pediatrics 26 MRI Orbit, Face & Neck (Soft Tissues) – Pediatrics 29 CT Paranasal Sinus & Maxillofacial Area – Pediatrics 33 MRI Temporomandibular Joint (TMJ) – Pediatrics 36 CT Neck for Soft Tissue Evaluation – Pediatrics 37 CTA/MRA of the Neck – Pediatrics 40 Chest Imaging 42 CT Chest – Pediatrics 42 CTA of the Chest (Non-Coronary) – Pediatrics 47 MRI Chest

2017 AIM Specialty Health

266. Radiologic Management of Central Venous Access

Be Appropriate Single lumen PICC Usually Not Appropriate Double lumen PICC May Be Appropriate Tunneled small bore central venous catheter single lumen May Be Appropriate Tunneled small bore central venous catheter double lumen Usually Appropriate Variant 2: Device selection. Adult or child =13 years of age. Sickle cell anemia requires intravenous access for the treatment of recurrent sickle cell crisis. Treatment/Procedure Appropriateness Category Chest port Usually Appropriate Arm port May Be Appropriate (...) access for long-term total parenteral nutrition and intermittent intravenous antibiotics. Variant 2: Device selection. Adult or child =13 years of age. Sickle cell anemia requires intravenous access for the treatment of recurrent sickle cell crisis. Variant 3: Device selection. Adult or child =13 years of age. Stage 3 chronic kidney disease requires central venous access for 6 weeks of antibiotic treatment. Variant 4: Device selection. Adult or child =13 years of age. Intensive care unit (ICU

2017 American College of Radiology

267. CRACKCast E090 – Liver and Biliary Tract

≥1.1 g/dL (≥11 g/L) predicts that the patient has portal hypertension with 97 percent accuracy. A gradient <1.1 g/dL (<11 g/L) indicates that the patient does not have portal hypertension. The SAAG will be elevated with any disorder leading to portal hypertension and is not specific to ascites due to cirrhosis. Other testing may be needed to differentiate cirrhotic from noncirrhotic portal hypertension. Additional testing will depend upon the clinical setting and may include an evaluation for heart (...) Children, MSM, IVDUs. Food handling / daycares IVDU, homosexual men Blood transfusions / organ transplant before ‘92 IVDU, > 20 lifetime sex partners Carrier state NONE 90% of neonates, and 10% of adults will become asymptomatic chronic carriers = HBsAg + Yes. Acute infection (N/V/D symptoms, jaundice, +ALT) High ALT Anti-HAV IgM High ALT HBsAg HBcAb IgM** HBeAg = HIGH infectivity (symptomatic in 70% of cases) Previous infection Anti-HAV IgG (may also have anti-HAV IgM) HBsAb → HBsAb IgG Anti-HBcAg

2017 CandiEM

268. CRACKCast E081 – Heart Failure

– with – Dyspnea, exercise intolerance Chronic fatigue Huge morbidity and mortality Progressive disease, may begin long before symptoms are evident HF definition: Pathophysiologic state where the heart is incapable to pump adequate blood supply to meet the metabolic demands of the body OR requires elevated filling pressures to meet these demands We’ll discuss more of the pathophys. In question 3. Huge costs – “40 billion dollars per year in the USA” 50% mortality after 5 yrs from time of symptoms Death from (...) Afterload Preload Preload The amount of force stretching the myofibrils before the heart’s contraction Factors: Venous return to the chamber Compliance characteristics of the heart muscle (ischemia, hypertrophy) We want this to be optimal – as per the Frank-Starling relationship The exact value is patient and time specific – we want it to be in the Goldilocks zone If it gets too high though you can tip them into pulmonary edema at their max filling pressure Afterload “The pressure against which

2017 CandiEM

269. An update to the Greig Health Record: Preventive health care visits for children and adolescents aged 6 to 17 years ? Technical report

and screening questions should be tailored for each individual. A small area for family history has been included on the top left-hand corner of each template to help identify children at risk for conditions such as mood disorders, cardiovascular disease and diabetes. Other risk factors and allergies can also be recorded here. Note that for the physical examination section, consensus opinion supports the inclusion of height, weight, blood pressure and visual acuity screening. Other examinations are included (...) . The quality of nutrition may influence the timing of puberty by several months even in the absence of obesity. A review of observational studies shows a delay of puberty onset in young girls with higher intakes of vegetable protein and lower intakes of animal protein. Nutrition and anemia In Canada, an estimated 3% of primary school-aged children are anemic. More are iron deficient. Iron deficiency has been associated with impaired cognitive and physical development. The supplementary pages in the Greig

2016 Canadian Paediatric Society

270. Treatment of Hepatitis C

-containing and IFN-free as in non-PWIDs. They do not require specific methadone and Haemoglobinopathies The most frequent haemoglobinopathy associated with chronic hepatitis C is thalassemia major, which requires frequent blood transfusions and is prevalent in countries where blood supply screening may be, or has been, suboptimal. Chronic HCV infec- tion is also frequent in individuals with sickle cell anaemia or thalassemia, with a more rapid course of liver disease because of the concurrent iron (...) , with limitations [13,14]. Systematic one-time testing has been recommended in countries with high endemicity and/or a goal of complete eradication. However, the optimal regional or national screening approaches should be determined. Screening for HCV infection is based on the detection of anti-HCV antibodies. In addition to EIAs, rapid diagnostic tests (RDTs) can be used to screen for anti-HCV antibodies. RDTs use various matrices, including serum, plasma, but also ?ngerstick capillary whole blood or, for some

2016 European Association for the Study of the Liver

271. Addyi - Flibanserin

was 83/49 mmHg (baseline blood pressure was 120/75 mmHg). In addition, subjects receiving flibanserin and high dose ethanol also exhibited similar decreases in blood pressures. When standing from a sitting position, the magnitude of systolic blood pressure reduction in six male subjects (25%, 6/24) ranged from 22 to 48 mmHg and the reduction in diastolic blood pressure ranged from 0 to27 mmHg. One of the six subjects needed ammonia salt and leg elevation. Effects on CNS depression and blood pressure (...) were assessed only up to 4 hours post-dose, at which time full recovery from the CNS depression had not occurred. Therefore, the time course for full recovery from the ethanol/flibanserin interaction remains unknown. Study SPR-12-03 also assessed for orthostatic vital signs (defined by an increase of = 20 beats per minute, bpm, in pulse rate or a decrease of = 20 mmHg in blood pressure from sitting to standing position). Orthostatic hypotension was observed with flibanserin alone and when

2015 FDA - Drug Approval Package

272. Antenatal care - uncomplicated pregnancy

for anaemia, red cell alloantibodies (such as anti-D and less commonly anti-c and anti-Kell), and haemoglobinopathies (such as sickle cell disease and thalassaemias). Testing for red cell alloantibodies and haemoglobinopathies should be offered as early as possible in pregnancy (ideally by 10 weeks). The type of haemoglobinopathy screening depends on the local prevalence of the disorder. The advises that in high-prevalence areas (more than 1.5 infants born with sickle cell disease per 10,000 births): All (...) are sufficient for a parous woman (having borne at least one viable offspring — usually after 24 weeks). Two ultrasound scans are usually offered during the course of an uncomplicated pregnancy: A dating scan (10–13 weeks). A fetal anomaly scan (18–20 weeks). Women with an uncomplicated pregnancy are usually offered screening for: Gestational diabetes. Hypertension in pregnancy and pre-eclampsia. Anaemia. Down's syndrome. Haemoglobinopathies (sickle cell disease and thalassaemia). Infections (asymptomatic

2019 NICE Clinical Knowledge Summaries

273. Reducing the Risk - Thrombosis and Embolism during Pregnancy and the Puerperium

titre anticardiolipin antibodies and/or ß 2 -glycoprotein 1 antibodies Medical comorbidities e.g. cancer; heart failure; active SLE, inflammatory polyarthropathy or IBD; nephrotic syndrome; type I diabetes mellitus with nephropathy; sickle cell disease; 49 current intravenous drug user Age > 35 years Obesity (BMI = 30 kg/m 2 ) either prepregnancy or in early pregnancy Parity = 3 (a woman becomes para 3 after her third delivery) Smoking Gross varicose veins (symptomatic or above knee (...) dose of LMWH on the day prior to delivery and, on the day of delivery, any morning dose should be omitted and the operation performed that morning. C C B C C C C P P P P PRCOG Green-top Guideline No. 37a © Royal College of Obstetricians and Gynaecologists 6 of 40 The first thromboprophylactic dose of LMWH should be given as soon as possible after delivery provided there is no postpartum haemorrhage and regional analgesia has not been used. [ New 2015] Women at high risk of haemorrhage with risk

2015 Royal College of Obstetricians and Gynaecologists

274. Acute pain management: scientific evidence (3rd Edition)

to be a fundamental human right and integral to the ethical, patient-centred and cost-effective practice of modern medicine. This progress is the result of dedicated efforts by health care professionals worldwide, including many in Australia and New Zealand who have contributed to past and present editions of Acute Pain: Scientific Evidence. The consistently high standards of Acute Pain: Scientific Evidence have established it as the foremost English-language resource of its type worldwide. Changes between (...) is to be effective.’ Personalised medicine and individualised care — in part necessitated by genetic differences in drug metabolism and action, as discussed in the third edition — require such a balanced approach. Cochrane himself voiced disdain for ‘the considerable pressure…to provide physicians with a simple rule to tell them what it all meant’ [Cochrane AL: Effectiveness and Efficiency: Random Reflections on Health Services. Cambridge (UK): Cambridge University Press, 1989, p. 41]. The first edition of Acute

2015 National Health and Medical Research Council

275. Treatment of Hepatitis C

. A number of drugs are contra-indicated because elevated plasma exposure would lead to serious adverse events, including: alfuzosin, amiodarone, astemizole, terfenadine, cisapride, ergot deriva- tives, lovastatin, simvastatin, atorvastatin, oral midazolam, tri- azolam, quetiapine, quinidine, salmeterol, sildena?l when used for pulmonary arterial hypertension. Also contra-indicated are enzyme inducers that might compromise virological ef?cacy, e.g. carbamazepine, phenytoin, phenobarbital, rifampicin, St (...) biopsy to resolve uncertainty [20,21].Thesetestsareofparticularinterestinpatientswithcoa- gulation disorders, though transjugular liver biopsy may also be used safely in this situation with the bonus that portal pressure can also be assessed. In case of contradictory results with non- invasive markers, liver biopsy may be indicated. Also, histology may be required in cases of known or suspected mixed aetiolo- gies (e.g. HCV infection with HBV infection, metabolic syndrome, alcoholism or autoimmunity

2015 European Association for the Study of the Liver

276. Childhood Cancer Genomics (PDQ®): Health Professional Version

% of cases of B-ALL, but very rarely in cases of T-cell ALL.[ ] Hyperdiploidy can be evaluated by measuring the DNA content of cells (DNA index) or by karyotyping. In cases with a normal karyotype or in which standard cytogenetic analysis was unsuccessful, interphase fluorescence in situ hybridization (FISH) may detect hidden hyperdiploidy. High hyperdiploidy generally occurs in cases with clinically favorable prognostic factors (patients aged 1 to <10 years with a low white blood cell [WBC] count (...) chromosomes (n = 46). Low-hypodiploid: 33 to 39 chromosomes (n = 26). High-hypodiploid: 40 to 43 chromosomes (n = 13). Near-diploid: 44 chromosomes (n = 54). Most patients with hypodiploidy are in the near-haploid and low-hypodiploid groups, and both of these groups have an elevated risk of treatment failure compared with nonhypodiploid cases.[ , ] Patients with fewer than 44 chromosomes have a worse outcome than do patients with 44 or 45 chromosomes in their leukemic cells.[ ] A number of studies have

2018 PDQ - NCI's Comprehensive Cancer Database

277. Late Effects of Treatment for Childhood Cancer (PDQ®): Health Professional Version

treated with chest radiation for childhood, adolescent, or young adult cancer. Ann Intern Med 152 (7): 444-55; W144-54, 2010. [ ] [ ] Jones DP, Spunt SL, Green D, et al.: Renal late effects in patients treated for cancer in childhood: a report from the Children's Oncology Group. Pediatr Blood Cancer 51 (6): 724-31, 2008. [ ] [ ] Liles A, Blatt J, Morris D, et al.: Monitoring pulmonary complications in long-term childhood cancer survivors: guidelines for the primary care physician. Cleve Clin J Med 75 (...) . Treatment-related factors Radiation therapy: Total dose, fraction size, organ or tissue volume, type of machine energy. Chemotherapy: Agent type, dose-intensity, cumulative dose, schedule. Surgery: Technique, site. Hematopoietic cell transplantation. Use of combined modality therapy. Blood product transfusion. Management of chronic graft-versus-host disease. Host-related factors Sex. Genetic predisposition. Premorbid health state. Developmental status. Age at diagnosis. Time from diagnosis/therapy

2018 PDQ - NCI's Comprehensive Cancer Database

278. Malaria prophylaxis

take days or occur within a few hours. Poor prognostic factors include high levels of parasitaemia, peripheral P. falciparum blood schizonts, pigment deposits in leucocytes, metabolic acidosis, older age, coma and renal impairment. Most cases of severe malaria are due to P. falciparum infection but other species (such as P. vivax and P. knowlesi ) can also cause serious illness. Infections with P. vivax can be associated with severe anaemia, splenic rupture, acute respiratory distress syndrome (...) by acute malaria and the risk of developing severe malaria is increased in HIV infection. People with a dysfunctional or absent spleen are at increased risk of severe malaria. Psychiatric conditions such as depression. A personal or family history of a severe mental health disorder may affect choice of chemoprophylaxis. Epilepsy and other seizure disorders. Certain anti-malarial drugs are contraindicated in people with seizure disorders. Inherited haemoglobinopathies such as sickle cell and thalassemia

2018 NICE Clinical Knowledge Summaries

279. Towards a better managed off-label use of drugs

3). Choice for a cheaper off-label alternative instead of a more expensive orphan drug At present, several generics of Viagra (sildenafil) indicated for "erectile dysfunction of miscellaneous origins" are available on the market. That same molecule sildenafil is also recognised as an orphan medicine (Revatio) and is reimbursed by the health insurance to treat patients suffering from abnormally high blood pressure in the pulmonary artery (pulmonary arterial hypertension - primary or associated (...) occur as well. KCE Report 252Cs ‘Off-label’ drugs 11 Choice for off-label instead of an orphan drug that is not available in Belgium Hydroxycarbamide is the active ingredient of two medicinal products that are registered for different indications. The first one, Siklos, has the status of orphan drug and is the only medicine with the registered indication of preventing vaso-occlusive crises in patients suffering from sickle cell anaemia. However, Siklos is not on the market in Belgium with the result

2015 Belgian Health Care Knowledge Centre

280. Canadian cardiovascular society guidelines for the diagnosis and management of stable ischemic heart disease

others were at intermediate risk (yellow). CAD, coronary artery disease; CASS, Coronary Artery Surgery Study. Data from Diamond and Forrester 22 and Weiner et al. 29 Table 3. Conditions that provoke or exacerbate ischemia Increased oxygen demand Decreased oxygen supply Noncardiac Hyper/hypothermia Anemia Hyperthyroidism Hypoxemia/high altitude Sympathomimetic toxicity (eg, cocaine use) Pneumonia Hypertension Asthma Anxiety Chronic obstructive pulmonary disease High cardiac output states (eg (...) , arteriovenous ?stulae) Pulmonary hypertension Interstitial pulmonary ?brosis Obstructive sleep apnea Sickle cell disease Sympathomimetic toxicity (eg, cocaine use, pheochromocytoma) Hyperviscosity (polycythemia, leukemia, thrombocytosis, hypergammaglobulinemia) Cardiac Left ventricular hypertrophy Aortic stenosis Aortic stenosis Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Obstructive coronary artery disease Dilated cardiomyopathy Microvascular disease Tachycardia (ventricular, supraventricular

2014 CPG Infobase

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