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Pulmonary Hypertension in Sickle Cell Anemia

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21. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia (PubMed)

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

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2014 Annals Of Thoracic Medicine

22. 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

23. The Hypoxic Response Contributes to Altered Gene Expression and Pre-Capillary Pulmonary Hypertension in Patients with Sickle Cell Disease. (PubMed)

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

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2014 Circulation

24. Reply: Practice Guideline for Pulmonary Hypertension in Sickle Cell: Direct Evidence Needed before Universal Adoption (PubMed)

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

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2014 American Journal of Respiratory and Critical Care Medicine

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

: Plerixafor Phase: Phase 1/2 Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Busulfan Plerixafor octahydrochloride Alkylating Agents Molecular Mechanisms of Pharmacological Action Immunosuppressive Agents Immunologic Factors Physiological Effects of Drugs Antineoplastic Agents, Alkylating Antineoplastic Agents Myeloablative Agonists Anti-HIV Agents Anti (...) A Study to Assess the Safety, Tolerability, and Efficacy of BIVV003 for Autologous Hematopoietic Stem Cell Transplantation in Patients With Severe Sickle Cell Disease A Study to Assess the Safety, Tolerability, and Efficacy of BIVV003 for Autologous Hematopoietic Stem Cell Transplantation in Patients With Severe 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

2018 Clinical Trials

26. 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 (...) 2018 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 City of Hope Medical Center: plerixafor sickle cell disease mobilization hematopoietic stem cell gene therapy Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Plerixafor octahydrochloride

2018 Clinical Trials

27. Mortality in Adults With Sickle Cell Disease and Pulmonary Hypertension: Sickle Cell Disease and Hypertension (PubMed)

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

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2012 JAMA : the journal of the American Medical Association

28. Mesenchymal Stromal Cells for Haplo Hematopoietic Cell Transplantation for Sickle Cell Disease

Update Posted: November 7, 2018 Last Verified: November 2018 Individual Participant Data (IPD) Sharing Statement: Plan to Share IPD: No 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 Elizabeth Stenger, Emory University: Hematopoietic cell transplantation (HCT) Haploidentical Mesenchymal stromal cells Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia (...) Mesenchymal Stromal Cells for Haplo Hematopoietic Cell Transplantation for Sickle Cell Disease Mesenchymal Stromal Cells for Haplo Hematopoietic Cell Transplantation for 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

2017 Clinical Trials

29. Management of acute chest syndrome in sickle cell disease

, including chronic anaemia, so hypoxia may need to be confirmed by arterial blood gas (ABG) measurement. A number of patients are chronically hypoxic for reasons such as pulmonary hypertension and chronic sickle lung disease and any further deterioration should be considered significant. Hypoxia may precede clinical signs and chest X‐ray abnormalities. Fever. Tachypnoea. Tachycardia. Wheeze. Chest signs including dullness to percussion, reduced air entry, crepitations, bronchial breath sounds, rhonchi (...) Management of acute chest syndrome in sickle cell disease Guideline on the management of acute chest syndrome in sickle cell disease - Howard - 2015 - British Journal of Haematology - Wiley Online Library By continuing to browse this site, you agree to its use of cookies as described in our . Search within Search term Search term The full text of this article hosted at iucr.org is unavailable due to technical difficulties. Guideline Free Access Guideline on the management of acute chest

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2015 British Committee for Standards in Haematology

30. Nonmyeloablative Haploidentical Peripheral Blood Mobilized Hematopoietic Precursor Cell Transplantation for Sickle Cell Disease

, and toxicity at a number of transplant centers. Our ongoing protocol for patients with severe congenital anemias, particularly sickle cell disease (SCD), and an HLA-matched sibling donor has had excellent preliminary results. None of the patients who engrafted had sickle-related events or any evidence of graft versus host disease (GVHD). There was no significant toxicity associated with the conditioning regimen. An additional protocol is ongoing for patients with high risk of graft rejection which employs (...) . Tricuspid regurgitant jet velocity (TRV) of greater than or equal to 2.5 m/s at baseline (without vaso-occlusive crisis) and/or pulmonary hypertension; OR Sickle hepatopathy defined as either ferritin >1000 mcg/L and platelet count < 250,000/uL (without vaso-occlusive crisis) OR direct bilirubin > 0.4 mg/dL and platelet count <250,000/uL (without vaso-occlusive crisis) C. Sickle hepatopathy defined as EITHER ferritin >1000mcg/L OR direct bilirubin >0.4 mg/dL AND platelet count <250,000/uL at baseline

2017 Clinical Trials

31. Nonmyeloablative Stem Cell Transplant in Children With Sickle Cell Disease and a Major ABO-Incompatible Matched Sibling Donor

of priapism in males history of osteonecrosis pulmonary hypertension as documented by tricuspid regurgitation jet velocity (TRV) > 2.5 m/s on echocardiogram red cell allo-immunization (≥ 2 antibodies) during long term transfusion therapy Sickle complications should be present despite the use of hydroxyurea, but this is not an absolute requirement, if the treating team considers the patient to be at high risk for further crisis episodes. Exclusion Criteria: Patients who are unable to comply with or follow (...) Drug Product: No Studies a U.S. FDA-regulated Device Product: No Product Manufactured in and Exported from the U.S.: No Keywords provided by Tony H. Truong, University of Calgary: sickle cell disease stem cell transplant red blood cell engraftment nonmyeloablative pure red cell aplasia Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Red-Cell Aplasia, Pure Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases

2017 Clinical Trials

32. Study of HLA-Haploidentical Stem Cell Transplantation to Treat Clinically Aggressive Sickle Cell Disease

Acute chest syndrome with recurrent hospitalizations, defined as ≥ 2 lifetime events 1.5 Red-cell alloimmunization (≥ 2 antibodies) during long-term transfusion therapy 1.6 Bilateral proliferative retinopathy with major visual impairment in at least one eye 1.7 Osteonecrosis of 2 or more joints 1.8 Sickle cell nephropathy, defined by a GFR < 90mL/min/1.73m2 or the presence of macroalbuminuria (urine albumin > 300 mg/g creatinine) 1.9 Pulmonary hypertension, defined by a mean pulmonary arterypressure (...) , Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Cyclophosphamide Fludarabine phosphate Sirolimus Everolimus Thymoglobulin Fludarabine Mycophenolic Acid Immunosuppressive Agents Immunologic Factors Physiological Effects of Drugs Antirheumatic Agents Antineoplastic Agents, Alkylating Alkylating Agents Molecular Mechanisms of Pharmacological Action Antineoplastic Agents Myeloablative Agonists Antimetabolites

2017 Clinical Trials

33. Simple chronic transfusion therapy, a crucial therapeutic option for sickle cell disease, improves but does not normalize blood rheology: What should be our goals for transfusion therapy? (PubMed)

Simple chronic transfusion therapy, a crucial therapeutic option for sickle cell disease, improves but does not normalize blood rheology: What should be our goals for transfusion therapy? Sickle cell anemia is characterized by a mutation resulting in the formation of an abnormal beta-hemoglobin called hemoglobin S. Hemoglobin S polymerizes upon deoxygenation, causing impaired red blood cell deformability and increased blood viscosity at equivalent hematocrits. Thus, sickle cell disease (...) is a hemorheologic disease that results in various pathologic processes involving multiple organ systems including the lungs, heart, kidneys and brain. Red blood cell mechanics and the perturbations on blood flow-endothelial interaction underlie much of the pathology found in sickle cell disease. Transfusion therapy is one of the few therapeutic options available to patients, acting as both primary and secondary prevention of stroke. Transfusion therapy, both simple and exchange, is also used for unremitting

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2018 Clinical hemorheology and microcirculation

34. Efficacy of a Decision Aid for Hydroxyurea in Sickle Cell Disease

Studies a U.S. FDA-regulated Drug Product: No Studies a U.S. FDA-regulated Device Product: No Keywords provided by Lakshmanan Krishnamurti, Emory University: Blood Disorders Hydroxyurea (HU) Decision Aid Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Hydroxyurea Antineoplastic Agents Antisickling Agents Enzyme Inhibitors Molecular Mechanisms (...) Efficacy of a Decision Aid for Hydroxyurea in Sickle Cell Disease Efficacy of a Decision Aid for Hydroxyurea in 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. Efficacy of a Decision Aid

2018 Clinical Trials

35. Pediatric Pulmonary Hypertension

? 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 (...) per- formed with PubMed and Ovid Medline and made available through a common task force Web site. Standard search terms such as pulmonary hypertension and pediatric pulmonary hypertension were used. Other search terms addressed dis- eases associated with PH (eg, bronchopulmonary dysplasia [BPD], sickle cell disease [SCD], and congenital heart dis- ease [CHD]), PAH-specific drugs, and other related subjects (Supplemental Figure). Additional searches to supplement the primary data review were

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2015 American Heart Association

36. 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 (...) ) CHD indicates congenital heart disease; MELAS, mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes; PH, pulmonary hypertension; and SCD, sickle cell disease. by guest on March 15, 2016 http://circ.ahajournals.org/ Downloaded from Abman et al Pediatric Pulmonary Hypertension 21 paradoxically worsen pulmonary vascular tone, reactivity, and permeability edema 203 and may produce cerebral constriction, reduced cerebral blood flow, and worse neurodevelopmental outcomes

2015 American Thoracic Society

37. Exercise tolerance, lung function abnormalities, anemia, and cardiothoracic ratio in sickle cell patients. (PubMed)

Exercise tolerance, lung function abnormalities, anemia, and cardiothoracic ratio in sickle cell patients. Many patients with sickle cell disease (SCD) have a reduced exercise capacity and abnormal lung function. Cardiopulmonary exercise testing (CPET) can identify causes of exercise limitation. Forty-four consecutive SCD patients (27 HbSS, 11 HbSC, and 6 HbS-beta thalassemia) with a median age (interquartile range) of 26 (21-41) years underwent pulmonary function tests, CPET, chest x-ray (...) ), cardiovascular dysfunction (n = 2), musculoskeletal function (n = 10), pulmonary ventilatory abnormalities (n = 1), pulmonary vascular exercise limitation (n = 1), and poor effort (n = 3). In the present study we demonstrate that anemia is the most important determinant of reduced exercise tolerance observed in SCD patients without signs of pulmonary hypertension. We found a strong correlation between various parameters of lung volume and cardiothoracic ratio and we hypothesize that cardiomegaly and relative

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2014 American journal of hematology

38. Sickle cell disease

('stuttering') may lead to erectile dysfunction in the long term, or permanent deformity of the penis (due to scarring). Pulmonary hypertension Pulmonary hypertension is common and is associated with increased morbidity and mortality in sickle cell disease [ ]. A third of adults with sickle cell disease develop pulmonary hypertension, which may lead to sudden death. It is more common in people with a low steady-state haemoglobin level and may be related to haemolysis. The free haemoglobin released from (...) , infarction (due to fat emboli that have moved to the lungs from bone infarcts, causing pulmonary infarction), or a combination of both. Half of all people with sickle cell disease will experience at least one episode of acute chest syndrome in their lifetime. It is common in early childhood, when it may present similarly to an episode of pneumonia. In adolescence and adulthood, it may develop during a painful crisis. Onset may be abrupt and the person may deteriorate over a few hours. Acute infections

2016 NICE Clinical Knowledge Summaries

39. Management of Sickle Cell Disease in Pregnancy

, which leads to the formation of rigid and fragile sickle-shaped red cells. These cells are prone to increased breakdown, which causes the haemolytic anaemia, and to vaso-occlusion in the small blood vessels, which causes most of the other clinical features, including acute painful crises. Other complications of SCD include stroke, pulmonary hypertension, renal dysfunction, retinal disease, leg ulcers, cholelithiasis and avascular necrosis (which commonly affects the femoral head and may necessitate (...) Management of Sickle Cell Disease in Pregnancy Management of Sickle Cell Disease in Pregnancy Green–top Guideline No. 61 July 2011RCOG Green-top Guideline No. 61 2 of 20 © Royal College of Obstetricians and Gynaecologists Management of Sickle Cell Disease in Pregnancy This is the first edition of this guideline. 1. Purpose and scope The purpose of this guideline is to describe the management of pregnant women with sickle cell disease (SCD). It will include preconceptual screening and antenatal

2011 Royal College of Obstetricians and Gynaecologists

40. A Phase Ib Study of NVX-508 in Sickle Cell Disease

: July 2018 Individual Participant Data (IPD) Sharing Statement: Plan to Share IPD: No Layout table for additional information Studies a U.S. FDA-regulated Drug Product: Yes Studies a U.S. FDA-regulated Device Product: No Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn (...) A Phase Ib Study of NVX-508 in Sickle Cell Disease A Phase Ib Study of NVX-508 in 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. A Phase Ib Study of NVX-508 in Sickle Cell Disease

2017 Clinical Trials

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