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Pulmonary Hypertension in Sickle Cell Anemia

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61. Cardiac Structural and Functional Changes Evaluated by Transthoracic and Tissue Doppler Echocardiography in Adult Patients with Sickle Cell Disease Full Text available with Trip Pro

Cardiac Structural and Functional Changes Evaluated by Transthoracic and Tissue Doppler Echocardiography in Adult Patients with Sickle Cell Disease One of the most common genetic blood disorders, resulting from inherited red blood cell disorders, is sickle cell disease (SCD) which is responsible for high death in adult patients with left ventricular diastolic dysfunction and pulmonary hypertension. Tissue Doppler Echocardiography (TDE) and transthoracic echocardiography (TTE) are two useful (...) LV diastolic velocity between two groups and other indicators showed no significant differences.The evaluation of LV systolic and diastolic function via TDE did not indicate significant differences between SCD patients compared to healthy subjects. High E and E' waves in these patients, could indicate progression towards cardiac disorders and pulmonary hypertension in future.

2017 Acta Informatica Medica

62. Cardiorespiratory pathogenesis of sickle cell disease in a mouse model Full Text available with Trip Pro

to weaning and the second in adulthood. At postnatal day 3, 43% of SAD mice showed marked apneas, anemia, and pulmonary vascular congestion typical of acute chest syndrome; none of these mice survived to maturity. The remaining SAD mice had mild lung histological changes in room air with an altered respiratory pattern, seizures, and a high rate of death in response to hypoxia. Approximately half the SAD mice that survived to adulthood had an identifiable respiratory phenotype including baseline tachypnea (...) at 7-8 months of age, restrictive lung disease, pulmonary hypertension, cardiac enlargement, lower total lung capacity, and pulmonary vascular congestion. All adult SAD mice demonstrated impairments in exercise capacity and response to hypoxia, with a more severe phenotype in the tachypneic mice. The model revealed distinguishable subgroups of SAD mice with cardiorespiratory pathophysiology mimicking the complications of human sickle cell disease.

2017 Scientific reports

63. Pathophysiology of Acute Pain in Patients With Sickle Cell Disease

written consent EXCLUSION CRITERIA FOR HEALTHY CONTROL SUBJECTS: Pregnancy Diagnosis of with any of the following chronic disease or conditions: Sickle cell disease or sickle cell trait Uncontrolled high blood pressure (systolic blood pressure must not be greater than 160 mmHg or diastolic pressure greater than 90 mmHg Uncontrolled diabetes (must not have both a documented history of diabetes and random blood glucose of greater than 200 mg/dL) History of coronary artery disease History of congestive (...) : National Heart, Lung, and Blood Institute (NHLBI) Information provided by (Responsible Party): National Institutes of Health Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) ) Study Details Study Description Go to Brief Summary: Background: Sickle Cell Disease (SCD) is a blood disorder that occurs mainly in people of African descent. Researchers want to learn more about the painful attacks and complications associated with SCD. They want to look for a relationship between SCD

2017 Clinical Trials

64. 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

65. Hydroxyurea in the Emergency Room to Lessen Pain in Sickle Cell Crisis

or disease Intervention/treatment Phase Anemia, Sickle Cell Anemia; Sickle-Cell, With Crisis Drug: Hydroxyurea Phase 2 Detailed Description: Sickle cell anemia (SCA) is a hereditary hemoglobinopathy; complications of the disease include, spleen enlargement, acute chest syndrome, pulmonary hypertension, stroke and cumulative damage to multiple organs, and painful vaso-occlusive crises (VOC). In Brazil, about 3,500 children are born each year with DF, and the number of individuals with sickle cell disease (...) Assignment Masking: None (Open Label) Primary Purpose: Treatment Official Title: Protocol for the Administration of Hydroxyurea During Painful Vaso-occlusive Crisis in Sickle Cell Anemia Study Start Date : November 2016 Estimated Primary Completion Date : April 2017 Estimated Study Completion Date : July 2017 Resource links provided by the National Library of Medicine related topics: related topics: available for: resources: Arms and Interventions Go to Arm Intervention/treatment No Intervention: Control

2017 Clinical Trials

66. Heart Arteries and Sickle Cell Disease / Coeur Artères DREpanocytose

table for additional information Studies a U.S. FDA-regulated Drug Product: No Studies a U.S. FDA-regulated Device Product: No Keywords provided by Xavier Jouven, Cardiologie et Développement: Sub-Saharan Africa Sickle Cell Disease Vasculopathy Nephropathy Pulmonary hypertension cardiac disease hemolysis pulse wave velocity blood viscosity Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases (...) . In addition to repeated acute ischemic insults due to the red blood cells sickling in the microcirculation, a chronic vasculopathy leads to organ injuries, such as kidney disease, stroke, pulmonary hypertension, retinopathy, bone infarcts, and leg ulcers. CADRE is a multinational prospective observational study undertaken in five countries in sub-Saharan Africa. Patients with SCD will be recruited through outpatients' clinics in public, university and private hospitals and research centers in five

2017 Clinical Trials

67. Clinical 'pearls' of maternal critical care Part 2: sickle-cell disease in pregnancy. (Abstract)

and chest crisis are also outlined. The role of the multidisciplinary team cannot be overstated.Massive obstetric hemorrhage in this population is difficult, and unique considerations such as cell-saver technology and tranexamic acid usage are discussed. Secondary complications such as pulmonary hypertension and stroke are also considered, with a summary of the latest treatment guidelines.This is a challenging cohort of pregnant patients who have a significantly increased morbidity and mortality (...) Clinical 'pearls' of maternal critical care Part 2: sickle-cell disease in pregnancy. The current review outlines the challenges in managing pregnant women with sickle-cell anemia, who are at risk of becoming critically ill during pregnancy.Sickle obstetric patients pose unique challenges to the anesthetist and intensivist. We discuss the role of prophylactic transfusions for specific indications like acute anemia and twin pregnancies. The management and prevention of vaso-occlusive crises

2017 Current Opinion in Anaesthesiology

68. Pulmonary hypertension associated with chronic hemolytic anemia and other blood disorders. Full Text available with Trip Pro

Pulmonary hypertension associated with chronic hemolytic anemia and other blood disorders. Pulmonary hypertension (PH) has emerged as a major complication of several hematologic disorders, including hemoglobinopathies, red cell membrane disorders, chronic myeloproliferative disorders, and splenectomy. With the exception of sickle cell disease, there are a limited number of studies systematically evaluating the prevalence of PH using the gold standard right heart catheterization (...) in these disorders. The cause of the PH in patients with hematologic disorders is multifactorial, and a thorough diagnostic evaluation is essential. More importantly, there are virtually no high-quality data on the safety and efficacy of PH-targeted therapy in this patient population. Copyright © 2013 Elsevier Inc. All rights reserved.

2013 Clinics in Chest Medicine

69. Management of Pulmonary Arterial Hypertension

hypoventilation disorders 3.6. Chronic exposure to high altitude 3.7. Developmental abnormalities 4. Chronic thromboembolic pulmonary hypertension (CTEPH) 5. Pulmonary hypertension with unclear multifactorial mechanisms 5.1. Haematologic disorders: myeloproliferative disorders, splenectomy 5.2. Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis 5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders (...) (IV) 2, 9, 10 and poor exercise capacity ( 20 mmHg) and CI ( 40 mmHg) Portal hypertension in patients considered for liver transplantation 5% prevalence of PAH (mPAP >25 mmHg and PVR >3.0 U) Congenital heart disease with systemic to pulmonary shunts Likely approximately 100% in high flow, non-restrictive L-R shunts Use of flenfluramine appetite suppressants (>3 months) Prevalence of 136/million users based on odds ratio of 23 times background HIV infection Prevalence 0.5/100 Sickle cell disease

2011 Ministry of Health, Malaysia

70. Cardiovascular complications and risk of death in sickle-cell disease. (Abstract)

Cardiovascular complications and risk of death in sickle-cell disease. In sickle-cell disease, a point mutation in the β-globin chain causes haemoglobin to polymerise within erythrocytes during deoxygenation, altering red blood cell rheology and causing haemolysis. Improvements in health infrastructure, preventive care, and clinical treatments have reduced the morbidity and mortality of sickle-cell disease in developed countries. However, as these patients live longer, the chronic effects (...) of sustained haemolytic anaemia and episodic vaso-occlusive events drive the development of end-organ complications. Cardiopulmonary organ dysfunction and chronic kidney injury have a large effect on morbidity and premature mortality, and typically accelerate in the second decade of life. These processes culminate in the development of pulmonary hypertension, left ventricular diastolic heart disease, dysrhythmia, and sudden death. In this Series paper, we review the mechanisms, clinical features

2016 Lancet

71. Haemoglobinuria is associated with chronic kidney disease and its progression in patients with sickle cell anaemia. Full Text available with Trip Pro

Haemoglobinuria is associated with chronic kidney disease and its progression in patients with sickle cell anaemia. To evaluate the association between haemoglobinuria and chronic kidney disease (CKD) in sickle cell anaemia (SCA), we analysed 356 adult haemoglobin SS or Sβ(o) thalassaemia patients from the University of Illinois at Chicago (UIC) and 439 from the multi-centre Walk-Treatment of Pulmonary Hypertension and Sickle Cell Disease with Sildenafil Therapy (Walk-PHaSST) cohort. CKD (...) was classified according to National Kidney Foundation Kidney Disease Outcomes Quality Initiatives guidelines. Haemoglobinuria, defined as positive haem on urine dipstick with absent red blood cells on microscopy, was confirmed by enzyme-linked immunosorbent assay in a subset of patients. The prevalence of CKD was 58% in the UIC cohort and 54% in the Walk-PHaSST cohort, and haemoglobinuria was observed in 36% and 20% of the patients, respectively. Pathway analysis in both cohorts indicated an independent

2014 British journal of haematology

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

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

2014 American journal of hematology

73. Haemolysis and abnormal haemorheology in sickle cell anaemia. Full Text available with Trip Pro

Haemolysis and abnormal haemorheology in sickle cell anaemia. Although pulmonary hypertension, leg ulcers, priapism, stroke and glomerulopathy in sickle cell anaemia (SCA) result from the adverse effects of chronic haemolysis on vascular function (haemolytic phenotype), osteonecrosis, acute chest syndrome and painful vaso-occlusive crises are caused by abnormal vascular cell adhesion and increased blood viscosity (viscosity-vaso-occlusion phenotype). However, this model with two sub-phenotypes (...) does not take into account the haemorheological dimension. We tested the relationships between the biological parameters reflecting the haemolytic rate (haemolytic component) and red blood cell (RBC) rheological characteristics in 97 adults with SCA. No significant difference in the proportion of patients with low or high haemolytic component in the low and high blood viscosity groups was observed. The RBC elongation index (i.e. deformability) was negatively correlated with the haemolytic component

2014 British journal of haematology

74. 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 (...) . 29 ? Blood pressure and urinalysis should be performed to identify women with hypertension and/or proteinuria. Renal and liver function tests should be performed annually to identify sickle nephropathy and/or deranged hepatic function. 29 ? Retinal screening. Proliferative retinopathy is common in patients with SCD, especially patients with HbSC, and can lead to loss of vision. 32 There is no randomised evidence on whether routine screening should be performed or if patients should be screened

2011 Royal College of Obstetricians and Gynaecologists

75. Sickle cell disease

and spleen; and cause intense pain (known as sickle cell crisis), anaemia, infections, chest problems, and other complications of sickle cell disease. Sickle cell diseases can be life-threatening, particularly for young children. People with sickle cell disease have inherited the gene for sickle haemoglobin (Hb S) from one parent and a gene for an abnormal haemoglobin variant from the other parent. If the second abnormal gene is also for Hb S, the person has homozygous sickle cell disease (Hb SS (...) it to polymerize when deoxygenated, which distorts the erythrocyte into a sickle shape. The deformed erythrocytes form clusters which block blood vessels; damage large and small blood vessels; are sequestered in the liver and spleen; and cause anaemia (of varying degrees), intense pain (known as sickle cell crisis), infections, and other complications of sickle cell disease. People with sickle cell disease have inherited the gene for sickle haemoglobin (Hb S) from one parent and a gene for an abnormal

2016 NICE Clinical Knowledge Summaries

76. Sickle Cell Anemia (Overview)

) to identify these conditions in young patients. [ ] In the study, 17 patients (43.6%) had ambulatory hypertension, whereas 4 (10.3%) had hypertension on the basis of their clinic blood pressure. Twenty-three patients (59%) had impaired systolic blood pressure dipping, 7 (18%) had impaired diastolic blood pressure dipping, and 5 (13%) had reversed dipping. [ ] Imaging studies Imaging studies that aid in the diagnosis of sickle cell anemia in patients in whom the disease is suggested clinically include (...) P, Godeau B, et al. Acute kidney injury in sickle patients with painful crisis or acute chest syndrome and its relation to pulmonary hypertension. Nephrol Dial Transplant . 2010 Aug. 25(8):2524-9. . Scheinman JI. In: Holliday M, Barratt TM, Avner ED (Eds.). Sickle cell nephropathy . Baltimore: Williams and Wilkins; 1994. Pediatric Nephrology: 908. Nissenson AR, Port FK. Outcome of end-stage renal disease in patients with rare causes of renal failure. I. Inherited and metabolic disorders. Q J Med

2014 eMedicine.com

77. Sickle Cell Anemia (Treatment)

regular blood transfusions (to reduce HbS to < 30%-50% total hemoglobin), hydroxyurea, or bypass surgery for advanced occlusive disease. [ ] Transfusion therapy, aimed at keeping the proportion of HbS below 30%, is now considered standard care for primary and secondary stroke prevention in children with SCD. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 (...) , and a history of priapism. Even modestly increased pulmonary artery pressures are associated with severe reduction in exercise capacity, as assessed by both the 6-minute walk and cardiopulmonary exercise testing, and herald a poor prognosis. Both pulmonary hypertension and cardiac sequelae, such as diastolic dysfunction, have been associated with accelerated mortality in the sickle cell disease population. For symptomatic patients, hydroxyurea and chronic transfusion have been used. Enothelin-1 receptor

2014 eMedicine.com

78. Sickle Cell Anemia, Skeletal

. Pathology . 2017 Apr. 49 (3):297-303. . da Silva Junior GB, Daher Ede F, da Rocha FA. Osteoarticular involvement in sickle cell disease. Rev Bras Hematol Hemoter . 2012. 34 (2):156-64. . Ganguly A, Boswell W, Aniq H. Musculoskeletal manifestations of sickle cell anaemia: a pictorial review. Anemia . 2011. 2011:794283. . Saito N, Nadgir RN, Flower EN, Sakai O. Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics . 2010 Jul-Aug. 30 (4):1021-34. . Dahnert W, ed (...) . 122(6):1332-42. . Linguraru MG, Orandi BJ, Van Uitert RL, Mukherjee N, Summers RM, Gladwin MT, et al. CT and image processing non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc . 2008. 2008:859-62. . Cerci SS, Suslu H, Cerci C, Yildiz M, Ozbek FM, Balci TA, et al. Different findings in Tc-99m MDP bone scintigraphy of patients with sickle cell disease: report of three cases. Ann Nucl Med . 2007 Jul. 21(5):311-4. . Solav SV, Bhandari R, Solav P

2014 eMedicine Radiology

79. Sickle Cell Anemia (Follow-up)

regular blood transfusions (to reduce HbS to < 30%-50% total hemoglobin), hydroxyurea, or bypass surgery for advanced occlusive disease. [ ] Transfusion therapy, aimed at keeping the proportion of HbS below 30%, is now considered standard care for primary and secondary stroke prevention in children with SCD. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 (...) , and a history of priapism. Even modestly increased pulmonary artery pressures are associated with severe reduction in exercise capacity, as assessed by both the 6-minute walk and cardiopulmonary exercise testing, and herald a poor prognosis. Both pulmonary hypertension and cardiac sequelae, such as diastolic dysfunction, have been associated with accelerated mortality in the sickle cell disease population. For symptomatic patients, hydroxyurea and chronic transfusion have been used. Enothelin-1 receptor

2014 eMedicine.com

80. Sickle Cell Anemia (Diagnosis)

) to identify these conditions in young patients. [ ] In the study, 17 patients (43.6%) had ambulatory hypertension, whereas 4 (10.3%) had hypertension on the basis of their clinic blood pressure. Twenty-three patients (59%) had impaired systolic blood pressure dipping, 7 (18%) had impaired diastolic blood pressure dipping, and 5 (13%) had reversed dipping. [ ] Imaging studies Imaging studies that aid in the diagnosis of sickle cell anemia in patients in whom the disease is suggested clinically include (...) P, Godeau B, et al. Acute kidney injury in sickle patients with painful crisis or acute chest syndrome and its relation to pulmonary hypertension. Nephrol Dial Transplant . 2010 Aug. 25(8):2524-9. . Scheinman JI. In: Holliday M, Barratt TM, Avner ED (Eds.). Sickle cell nephropathy . Baltimore: Williams and Wilkins; 1994. Pediatric Nephrology: 908. Nissenson AR, Port FK. Outcome of end-stage renal disease in patients with rare causes of renal failure. I. Inherited and metabolic disorders. Q J Med

2014 eMedicine.com

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