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Pulmonary Hypertension in Sickle Cell Anemia

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21. Pulmonary Endarterectomy as Treatment for Chronic Thromboembolic Pulmonary Hypertension in Sickle Cell Disease Full Text available with Trip Pro

Pulmonary Endarterectomy as Treatment for Chronic Thromboembolic Pulmonary Hypertension in Sickle Cell Disease 26370739 2016 06 02 2018 11 13 1096-8652 90 12 2015 Dec American journal of hematology Am. J. Hematol. Pulmonary endarterectomy as treatment for chronic thromboembolic pulmonary hypertension in sickle cell disease. E223-4 10.1002/ajh.24192 Freeman Ashley T AT Division of Hematology/Oncology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina. Ataga Kenneth I KI (...) Division of Hematology/Oncology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina. eng R01 HL111659 HL NHLBI NIH HHS United States U01 HL117659 HL NHLBI NIH HHS United States R01HL111659 HL NHLBI NIH HHS United States U01HL117659 HL NHLBI NIH HHS United States Case Reports Letter Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't 2015 11 17 United States Am J Hematol 7610369 0361-8609 IM Adult Anemia, Sickle Cell complications surgery Endarterectomy methods

2015 American journal of hematology

22. Guidelines for the use of hydroxycarbamide in children and adults with sickle cell disease Full Text available with Trip Pro

Link Use the link below to share a full-text version of this article with your friends and colleagues. Copy URL Share a link Literature review details (Appendix ) A literature search was conducted on 15 June 2016. Databases searched include MEDLINE (OVID) and Embase (OVID) from 1995 to July, week 1, 2016. Key words were: Hydroxycarbamide; Hydroxyurea, Sickle cell disease; Sickle cell anaemia; Mode of Action: HbF; Pain; Chest crisis; stroke; silent infarct; cerebral flow velocities; primary (...) anaemia (SS), sickle cell/haemoglobin C (SC) sickle cell/βthalassemia (S/β thal) and other compound heterozygous conditions. SCD is characterised by the presence of the mutated β‐globin gene, HBB s (also termed β s ‐globin). On de‐oxygenation, this forms a polymeric structure resulting in deformed, rigid red blood cells, and is associated with a chronic haemolytic anaemia due to shortened red cell life span and vaso‐occlusion causing frequent episodes of severe bony pain (vaso‐occlusive crises

2018 British Committee for Standards in Haematology

23. Macitentan in Pulmonary Hypertension of Sickle Cell Disease

there is no plan to share IPD. Layout table for additional information Studies a U.S. FDA-regulated Drug Product: Yes Studies a U.S. FDA-regulated Device Product: No Keywords provided by Boston University: Pulmonary Hypertension Sickle Cell Disease Pulmonary Arterial Hypertension Macitentan Additional relevant MeSH terms: Layout table for MeSH terms Hypertension Hypertension, Pulmonary Anemia, Sickle Cell Vascular Diseases Cardiovascular Diseases Lung Diseases Respiratory Tract Diseases Anemia, Hemolytic (...) Macitentan in Pulmonary Hypertension of Sickle Cell Disease Macitentan in Pulmonary Hypertension of Sickle Cell Disease - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Macitentan in Pulmonary Hypertension

2016 Clinical Trials

24. Kidney Function in Sickle Cell Anemia

characteristics for the worsening of kidney function and for a rapid decline in kidney function. "Funding Source - FDA OOPD" Condition or disease Sickle Cell Disease Kidney Failure, Chronic Detailed Description: Sickle cell disease is a severe monogenic disorder which affects approximately 80,000 patients in the US. It is characterized by a vasculopathy with involvement of multiple organs and resulting in complications such as ischemic stroke, pulmonary hypertension, autosplenectomy, priapism, as well (...) Kidney Function in Sickle Cell Anemia Kidney Function in Sickle Cell Anemia - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Kidney Function in Sickle Cell Anemia The safety and scientific validity

2017 Clinical Trials

25. Pediatric Pulmonary Hypertension: ATS/AHA Clinical Practice Guidelines

.) Key Words: AHA Scientific Statements ? bronchopulmonary dysplasia ? congenital diaphragmatic hernia ? congenital heart disease ? genetics ? persistent pulmonary hypertension of the newborn ? sickle cell disease © 2015 by the American Heart Association, Inc., and the American Thoracic Society. Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000329 †Deceased. The American Heart Association and the American Thoracic Society make every effort to avoid any actual (...) but includes subjects with elevated TPG (mPAP-left atrial pressure or PAWP >6 mm Hg) or high PVRI as observed in patients with cavopulmonary anastomoses without high mPAP HPAH indicates heritable pulmonary artery hypertension; IPAH, idiopathic pulmonary artery hypertension; mPAP, mean pulmonary artery pressure; PAH, pulmonary artery hypertension; PAWP, pulmonary artery wedge pressure; PH, pulmonary hypertension; PHVD pulmonary hypertensive vascular disease; PVRI, pulmonary vascular resistance index

2015 American Thoracic Society

26. Pediatric Pulmonary Hypertension Full Text available with Trip Pro

? bronchopulmonary dysplasia ? congenital diaphragmatic hernia ? congenital heart disease ? genetics ? persistent pulmonary hypertension of the newborn ? sickle cell disease © 2015 by the American Heart Association, Inc., and the American Thoracic Society. Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000329 †Deceased. The American Heart Association and the American Thoracic Society make every effort to avoid any actual or potential conflicts of interest that may arise (...) known to be associated with PAH Referred to as HPAH with positive family or genetic evaluation PHVD Broad category that includes forms of PAH but includes subjects with elevated TPG (mPAP-left atrial pressure or PAWP >6 mm Hg) or high PVRI as observed in patients with cavopulmonary anastomoses without high mPAP HPAH indicates heritable pulmonary artery hypertension; IPAH, idiopathic pulmonary artery hypertension; mPAP, mean pulmonary artery pressure; PAH, pulmonary artery hypertension; PAWP

2015 American Heart Association

27. CRACKCAST E121 – Anemia, Polycythemia, and White Blood Cell Disorders

) Iron deficiency, Vitamin B12/folate deficiency, Aplastic anemia, Myeloid metaplasia with myelofibrosis, Hypoendocrine state, Renal disease [4] List 5 causes of MAHA (review question) DIC, TTP, HUS, Malignant hypertension, Preeclampsia [5] What organ damage is seen in sickle cell disease? Skin – stasis ulcer CNS – CVA Eye – retinal hemorrhage, retinopathy Cardiac – CHF Pulmonary – PE, infarct, infection Vascular – occlusive phenomenon ANYWHERE Liver – infarct, transfusion-related hepatitis, hepatic (...) CRACKCAST E121 – Anemia, Polycythemia, and White Blood Cell Disorders CRACKCAST E121 - Anemia, Polycythemia, and White Blood Cell Disorders - CanadiEM CRACKCAST E121 – Anemia, Polycythemia, and White Blood Cell Disorders In , by Nathan Stefani October 26, 2017 This 121st episode of CRACKCast covers Rosen’s 9th edition, Chapter 112 and 113, Anemia, Polycythemia, and White Blood Cell Disorders. These blood disorders are numerous and this episode attempts to break their classification and approach

2017 CandiEM

28. Pulmonary Hypertension in Sickle Cell Anemia

Pulmonary Hypertension in Sickle Cell Anemia Pulmonary Hypertension in Sickle Cell Anemia Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer (...) Administration 4 Pulmonary Hypertension in Sickle Cell Anemia Pulmonary Hypertension in Sickle Cell Anemia Aka: Pulmonary Hypertension in Sickle Cell Anemia , Sickle Cell Anemia Related Pulmonary Hypertension From Related Chapters II. Epidemiology : Up to 30% of adults III. Symptoms IV. Signs Increased V. Imaging Right ventricular enlargement Tricuspid regiurgitation VI. Prognosis Increases mortality risk 10 fold VII. References Images: Related links to external sites (from Bing) These images are a random

2015 FP Notebook

29. Mortality in Adults With Sickle Cell Disease and Pulmonary Hypertension: Sickle Cell Disease and Hypertension Full Text available with Trip Pro

NHLBI NIH HHS United States ZIA HL006011 HL NHLBI NIH HHS United States R01 HL096973 HL NHLBI NIH HHS United States R01HL096973 HL NHLBI NIH HHS United States Journal Article Research Support, N.I.H., Extramural Research Support, N.I.H., Intramural Research Support, Non-U.S. Gov't United States JAMA 7501160 0098-7484 AIM IM Adult Anemia, Sickle Cell complications mortality Cardiac Catheterization Cohort Studies Echocardiography Humans Hypertension, Pulmonary complications diagnosis mortality Middle (...) Mortality in Adults With Sickle Cell Disease and Pulmonary Hypertension: Sickle Cell Disease and Hypertension 22453563 2012 03 29 2018 11 13 1538-3598 307 12 2012 Mar 28 JAMA JAMA Mortality in adults with sickle cell disease and pulmonary hypertension. 1254-6 10.1001/jama.2012.358 Mehari Alem A College of Medicine, Howard University, Washington, DC, USA. Gladwin Mark T MT Tian Xin X Machado Roberto F RF Kato Gregory J GJ eng ZIA HL006012 HL NHLBI NIH HHS United States ZIA HL006012-02 NULL

2012 JAMA : the journal of the American Medical Association

30. Red cell transfusion in sickle cell disease Part l

some chronic complications (DeBaun, et al 2014) and to prevent perioperative acute complications, such as acute chest syndrome (Howard, et al 2013). We have reviewed the evidence and developed two linked guidelines on transfusion in SCD; Part I relates to general principles and laboratory aspects, whereas Part II addresses indications for transfusion in sickle cell disease. Here the term sickle cell disease refers to all genotypes of the disease and sickle cell anaemia to the homozygous state (SS (...) disease, sickle cell crisis), 2) transfusion (including transfusion, blood transfusion, red cell transfusion), 3) transfusion indications [including aplastic crisis, parvovirus, sequestration (splenic, liver, hepatic), acute chest syndrome, stroke, silent cerebral infarcts, multi-organ failure, girdle syndrome, intrahepatic cholestasis, surgery, pregnancy] and 4) transfusion complications (including alloimmunisation, haemolytic transfusion reactions, iron overload, viral infections). Opinions were

2016 British Committee for Standards in Haematology

31. Red cell transfusion in sickle cell disease Part II

) Sickle cell acute chest syndrome associated with parvovirus B19 infection: case series and review. Am J Hematol, 51, 207-213. Lusher, J.M., Haghighat, H. & Khalifa, A.S. (1976) A prophylactic transfusion program for children with sickle cell anemia complicated by CNS infarction. Am J Hematol, 1, 265-273. Machado, R.F. & Gladwin, M.T. (2005) Chronic sickle cell lung disease: new insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension. Br J Haematol, 129, 449-464. Malinowski (...) . & Bonds, D.R. (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med, 332, 1317-1322. Cho, G. & Hambleton, I.R. (2011) Regular long-term red blood cell transfusions for managing chronic chest complications in sickle cell disease. Cochrane Database Syst Rev, CD008360. Cohen, A.R., Martin, M.B., Silber, J.H., Kim, H.C., Ohene-Frempong, K. & Schwartz, E. (1992) A modified transfusion

2016 British Committee for Standards in Haematology

32. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia Full Text available with Trip Pro

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia Hereditary hemoglobin disorders affecting the globin chain synthesis namely thalassemia syndromes and sickle cell disease (SCD) are the most common genetic disorders in human. Around 7% of the world population carries genes for these disorders, mainly the Mediterranean Basin, Middle and Far East, and Sub-Saharan Africa. An estimated 30 million people worldwide (...) are living with sickle cell disease, while 60-80 million carry beta thalassemia trait. About 400,000 children are born with severe hemoglobinopathies each year. Cardiovascular complications of hemoglobinopathies include left and right ventricular (RV) dysfunction, arrhythmias, pericarditis, myocarditis, valvular heart disease, myocardial ischemia, and notably pulmonary hypertension (PH). Because of a unique pathophysiology, pulmonary hypertension associated with hemolytic disorders was moved from WHO

2014 Annals Of Thoracic Medicine

33. Pregnancy outcome in patients with sickle cell disease in the UK--a national cohort study comparing sickle cell anaemia (HbSS) with HbSC disease. (Abstract)

Pregnancy outcome in patients with sickle cell disease in the UK--a national cohort study comparing sickle cell anaemia (HbSS) with HbSC disease. We describe the findings from a national study of maternal and fetal outcomes of pregnancy in women with sickle cell disease (SCD). Data were collected via the United Kingdom Obstetric Surveillance System between 1 February 2010 and 31 January 2011 from 109 women, of whom 51 (46·8%) had HbSS and 44 (40·4%) had HbSC. Data included antenatal, maternal (...) and fetal outcomes. Comparisons were made between women with HbSS and HbSC. Incidence of complications were acute pain (57%), blood transfusion (26%), urinary tract infection (UTI; 12%) and critical care unit admission (23%) and these were all more common in women with HbSS than HbSC. There was no difference in the incidence of acute chest syndrome, hypertension and venous thromboembolism between HbSS and HbSC. Women with HbSS were more likely to deliver at <37 weeks gestation (P = 0·01

2015 British journal of haematology

34. High molecular weight kininogen contributes to early mortality and kidney dysfunction in a mouse model of sickle cell disease. (Abstract)

High molecular weight kininogen contributes to early mortality and kidney dysfunction in a mouse model of sickle cell disease. Sickle cell disease (SCD) is characterized by chronic hemolytic anemia, vaso-occlusive crises, chronic inflammation, and activation of coagulation. The clinical complications such as painful crisis, stroke, pulmonary hypertension, nephropathy and venous thromboembolism lead to cumulative organ damage and premature death. High molecular weight kininogen (HK) is a central (...) cofactor for the kallikrein-kinin and intrinsic coagulation pathways, which contributes to both coagulation and inflammation.We hypothesize that HK contributes to the hypercoagulable and pro-inflammatory state that causes end-organ damage and early mortality in sickle mice.We evaluated the role of HK in the Townes mouse model of SCD.We found elevated plasma levels of cleaved HK in sickle patients compared to healthy controls, suggesting ongoing HK activation in SCD. We used bone marrow transplantation

2020 Journal of Thrombosis and Haemostasis

35. The Hypoxic Response Contributes to Altered Gene Expression and Pre-Capillary Pulmonary Hypertension in Patients with Sickle Cell Disease. Full Text available with Trip Pro

The Hypoxic Response Contributes to Altered Gene Expression and Pre-Capillary Pulmonary Hypertension in Patients with Sickle Cell Disease. We postulated that the hypoxic response in sickle cell disease (SCD) contributes to altered gene expression and pulmonary hypertension, a complication associated with early mortality.To identify genes regulated by the hypoxic response and not other effects of chronic anemia, we compared expression variation in peripheral blood mononuclear cells from 13 (...) ). This association was confirmed in an independent Walk-Treatment of Pulmonary Hypertension and Sickle Cell Disease With Sildenafil Therapy cohort (allele frequency, 0.65; odds ratio, 11.3; n=519). The homozygous AA genotype of rs10857560 was associated with decreased MAPK8 expression and present in all 14 of the identified precapillary pulmonary hypertension cases among the combined 757 patients.Our study demonstrates a prominent hypoxic transcription component in SCD and a MAPK8 expression quantitative trait

2014 Circulation

36. Reply: Practice Guideline for Pulmonary Hypertension in Sickle Cell: Direct Evidence Needed before Universal Adoption Full Text available with Trip Pro

IM Am J Respir Crit Care Med. 2014 Mar 15;189(6):727-40 24628312 Am J Respir Crit Care Med. 2014 Jul 15;190(2):237-8 25025359 Anemia, Sickle Cell complications Humans Hypertension, Pulmonary diagnosis therapy 2014 7 16 6 0 2014 7 16 6 0 2014 9 10 6 0 ppublish 25025360 10.1164/rccm.201404-0733LE PMC4226058 Blood. 2012 Nov 22;120(22):4304-10; quiz 4448 22915643 Blood. 2004 Mar 15;103(6):2039-45 14630791 Am J Hematol. 2008 Jan;83(1):6-14 17724704 Am J Hematol. 2010 Jun;85(6):403-8 20513116 Am J (...) Reply: Practice Guideline for Pulmonary Hypertension in Sickle Cell: Direct Evidence Needed before Universal Adoption 25025360 2014 09 08 2018 12 02 1535-4970 190 2 2014 Jul 15 American journal of respiratory and critical care medicine Am. J. Respir. Crit. Care Med. Reply: Practice guideline for pulmonary hypertension in sickle cell: direct evidence needed before universal adoption. 238-40 10.1164/rccm.201404-0733LE Klings Elizabeth S ES 1 Boston University School of Medicine Boston

2014 American Journal of Respiratory and Critical Care Medicine

37. A Study to Assess the Safety, Tolerability, and Efficacy of BIVV003 for Autologous Hematopoietic Stem Cell Transplantation in Patients With Severe Sickle Cell Disease

Frame: Baseline up to Week 104 ] Change from baseline in HbF up to Week 104 will be assessed. Change From Baseline in Peripheral Blood Percent (%)F cells [ Time Frame: Baseline up to Week 104 ] Change from baseline in %F cells up to Week 104 will be assessed. Change From Baseline in Peripheral Blood Sickle Hemoglobin (HbS) Levels [ Time Frame: Baseline up to Week 104 ] Change from baseline in peripheral blood HbS levels up to Week 104 will be assessed. Change From Baseline in Peripheral blood total (...) history of hematologic malignancy, or a family history of a cancer predisposition syndrome (without negative result of candidate) Current diagnosis of uncontrolled seizures History of significant bleeding disorder Clinically significant infection Any major organ dysfunction involving brain, kidney, liver, lung, or heart (e.g., congestive heart failure, pulmonary hypertension) Corrected QT interval of more than 500 millisecond (ms) based on screening electrocardiogram (ECG) Positive for human

2018 Clinical Trials

38. How I manage red cell transfusions in patients with sickle cell disease. Full Text available with Trip Pro

How I manage red cell transfusions in patients with sickle cell disease. Sickle cell disease is one of the commonest serious inherited diseases in the world, and red cell transfusion is still one of the few effective treatments for acute and chronic complications. Transfusion corrects anaemia and dilutes out the number of red cells able to cause vaso-occlusion and vascular damage. Urgent red cell transfusions are used to correct acute anaemia, treat acute chest syndrome and patients with acute (...) neurological symptoms. We use elective transfusions preoperatively for moderate risk surgery, and in some pregnant women. There is good evidence for the use of long-term regular transfusions in primary stroke prevention, with the aim of keeping the percentage of sickle haemoglobin below 30%. Long-term transfusions are also used in secondary stroke prevention, and the management of progressive organ damage, including renal impairment and pulmonary hypertension. Blood needs to be matched for ABO, RH and Kell

2018 British journal of haematology

39. Safety of Blood Stem Cell Mobilization With Plerixafor in Patients With Sickle Cell Disease

Investigator discretion). History of pulmonary hypertension, proven by cardiac catheterization; History of malignancy or immunodeficiency disorder, (i.e., subjects with prior malignancy must be disease-free for 5 years), except curatively-treated basal cell carcinoma or cutaneous squamous cell carcinoma; Participation in any study with an investigational agent or medical device within 90 days of screening; Major surgery in the past 30 days; Prior receipt of any gene transfer product; Bone marrow harvest (...) Safety of Blood Stem Cell Mobilization With Plerixafor in Patients With Sickle Cell Disease Safety of Blood Stem Cell Mobilization With Plerixafor in Patients With Sickle Cell Disease - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more

2018 Clinical Trials

40. Stem Cell Transplant in Patients With Severe Sickle Cell Disease

infusion at 0.1 mg/kg IV, and 5 thru 3 days prior to stem cell infusion at 0.3 mg/kg IV. Study Design Go to Layout table for study information Study Type : Interventional (Clinical Trial) Estimated Enrollment : 12 participants Intervention Model: Single Group Assignment Masking: None (Open Label) Primary Purpose: Treatment Official Title: A Pilot Study Evaluating the Efficacy of Non-Myeloablative Matched Related Donor Peripheral Blood Stem Cell Transplant in Patients With Severe Sickle Cell Disease (...) Actual Study Start Date : March 29, 2018 Estimated Primary Completion Date : February 15, 2022 Estimated Study Completion Date : February 15, 2023 Resource links provided by the National Library of Medicine related topics: available for: resources: Arms and Interventions Go to Arm Intervention/treatment Experimental: Non Myeloablative regimen (Alemtuzumab) Sickle cell patient receives sibling donor peripheral blood stem cell transplant with non-myeloablative pre-transplant conditioning. Drug

2018 Clinical Trials

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