What is the role of screening for pulmonary hypertension in adults and children with sickle cell disease?

What is the role of screening for pulmonary hypertension in adults and children with sickle cell disease? Hematology Am Soc Hematol Educ Program. 2017 Dec 08;2017(1):431-434 Authors: Willen SM, Gladwin MT Abstract Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography. The patient's case is described in detail in Lancet1 He had been treated with regular transfusions since childhood for stroke, had rare episodes of vaso-occlusive pain episodes, and did not take narcotic pain medications. He presented with progressive severe dyspnea on exertion and lower extremity edema. His laboratory tests were notable for a total hemoglobin level of 11.8 g/dL and hemoglobin S levels 140 mm Hg (4 times the TRV squared = 4V2). Additional images in Figure 1D show a dilated right ventricle and right atrium with a compressed left ventricle. The patient's right heart catheterization revealed a pulmonary artery systolic pressure of 147 mm Hg and diastolic pressure of 49 mm Hg; note that the normal values are ∼25/10 mm Hg. PMID: 29222289 [PubMed - in process]
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Related Links:

Publication date: Available online 15 December 2018Source: Process BiochemistryAuthor(s): Hümeyra İspirli, Osman Sağdıç, Mustafa Tahsin Yılmaz, Enes DertliAbstractLactobacillus reuteri strain (E81) is an alpha glucan producer from traditional sourdough and the physicochemical characteristics of this glucan was determined in this study. The HPLC analysis confirmed the presence of glucose as the only sugar monomer in the repeating structure of this exopolysaccharide (EPS). The functional groups within the glucan structure was detected by FTIR analysis. The scanning electron microscopy (SEM) analysis demonstra...
Source: Process Biochemistry - Category: Biochemistry Source Type: research
We present a new algorithm to compute the voxel-wise genetic contribution to brain fiber microstructure using diffusion tensor imaging (DTI) in a dataset of 25 pairs of monozygotic (MZ) twins and 25 pairs of dizygotic (DZ) twins. First, the structural and DT scans were linearly co-registered. The structural MR scans were nonlinear mapped via a 3D fluid transformation to a geometrically centered mean template, and the deformation fields were applied to the DTI volumes. After tensor re-orientation to realign them to the anatomy, we computed several scalar and multivariate DT-derived measures including the geodesic anisotropy...
Source: Proceedings - International Symposium on Biomedical Imaging - Category: Radiology Tags: Proc IEEE Int Symp Biomed Imaging Source Type: research
COMPARISON OF FRACTIONAL AND GEODESIC ANISOTROPY IN DIFFUSION TENSOR IMAGES OF 90 MONOZYGOTIC AND DIZYGOTIC TWINS. Proc IEEE Int Symp Biomed Imaging. 2008 May;2008:943-946 Authors: Lee AD, Leporé N, Barysheva M, Chou YY, Brun C, Madsen SK, McMahon KL, de Zubicaray GI, Meredith M, Wright MJ, Toga AW, Thompson PM Abstract We used diffusion tensor magnetic resonance imaging (DTI) to reveal the extent of genetic effects on brain fiber microstructure, based on tensor-derived measures, in 22 pairs of monozygotic (MZ) twins and 23 pairs of dizygotic (DZ) twins (90 scans). After Log-Euclidean denoising ...
Source: Proceedings - International Symposium on Biomedical Imaging - Category: Radiology Tags: Proc IEEE Int Symp Biomed Imaging Source Type: research
This study examined influenza vaccination in patients with SCD as a potential marker of quality of care delivery. The study population included black individuals aged 1 to =1 diagnosis of stroke, TIA or epilepsy/recurrent seizures); cardio pulmonary complications (>=1 diagnosis of chronic pulmonary heart disease, pulmonary hypertension, malaise and fatigue, edema, chest pain or hypoxemia); kidney disease (>=1 diagnosis of chronic kidney disease/renal failure, proteinuria), avascular necrosis or ulcer of lower limbs (adapted from Afenyi-Annan, 2008, Candrilli. 2011, Elmariah. 2014). A total of 1544 patients with SCD w...
Source: Blood - Category: Hematology Authors: Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster II Source Type: research
Conclusions: Data from Part A of the HOPE study demonstrate that treatment with voxelotor resulted in a dose-dependent increase in Hb with a large proportion of patients achieving Hb>1 g/dL improvement from baseline compared with placebo at 12 weeks. In addition, there was a dose-dependent decrease in hemolysis markers. Voxelotor was generally well tolerated at both doses. Hemolytic anemia of SCD has severe and life-threatening consequences and presents an unmet medical need. Voxelotor has potential to ameliorate complications of anemia associated with SCD.DisclosuresVichinsky: bluebird bio: Membership on an entity's Bo...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Novel or Improved Approaches To Treating Sickle Cell Disease Source Type: research
ConclusionsThis is the first report describing prevalence of SCD-related complications in the MU-SCD Cohort. We identified this population to have an increasing frequency of hemolytic complications and sickle cell nephropathy with advancing age. Onset of persistent proteinuria occurred in the second decade of life, followed by renal insufficiency or end stage renal disease in subsequent decades. As previously demonstrated in the Cooperative Study of Sickle Cell Disease and the Jamaican SCD Cohort study, renal insufficiency was a significant risk factor for early mortality. Further studies are required for identification of...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster I Source Type: research
Abstract 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...
Source: Clinical Hemorheology and Microcirculation - Category: Hematology Authors: Tags: Clin Hemorheol Microcirc Source Type: research
Abstract Sickle cell disease (SCD) is one of the most common single disease disorders world-wide. It is remarkable for its clinical heterogeneity, even among individuals with identical genotypes. Some individuals experience morbidity and mortality in early childhood, while others have a relatively mild course, and normal or near normal life expectancy. Many clinical complications are associated with SCD; most notably frequent pain episodes, stroke, acute chest syndrome, avascular necrosis, nephropathy, retinopathy and pulmonary hypertension. While the effects of higher fetal hemoglobin (HbF) levels, UGTA1A polymor...
Source: Clinical Hemorheology and Microcirculation - Category: Hematology Authors: Tags: Clin Hemorheol Microcirc Source Type: research
Sickle cell disease (SCD) is a hemoglobinopathy affecting 1 in every 500 African American newborns and an estimated 100,000 persons in the United States [1]. Vascular occlusion caused by the sickled RBCs leads to decreased perfusion in target organs and manifests clinically as vaso-occlusive pain crisis, stroke (cerebral infarction), pulmonary restrictive disease, and pulmonary hypertension [2]. Despite advances in supportive care, including the use of hydroxyurea (HU), RBC transfusion, and iron chelation therapy, patients with SCD continue to experience considerable morbidity and mortality in their lifetime.
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Source Type: research
Sickle cell disease (SCD) is a hemoglobinopathy affecting 1 in every 500 African American newborns and an estimated 100,000 persons in the United States [1]. Vascular occlusion caused by the sickled RBCs leads to decreased perfusion in target organs and manifests clinically as vaso-occlusive pain crisis, stroke (cerebral infarction), pulmonary restrictive disease, and pulmonary hypertension [2]. Despite advances in supportive care, including the use of hydroxyurea (HU), RBC transfusion, and iron chelation therapy, patients with SCD continue to experience considerable morbidity and mortality in their lifetime.
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Source Type: research
More News: Bilirubin | Cardiac Catheterization | Cardiology | Children | CT Scan | Education | Heart | Hematology | Hypertension | Laboratory Medicine | Pain | Perfusion | PET Scan | Pulmonary Artery Catheterization | Pulmonary Hypertension | Sickle Cell Anemia | Stroke | Study | Universities & Medical Training