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

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We describe the components of a CLSM system and assess how modern implementations of the approach have further expanded the use of the technique. Finally, we briefly outline some practical considerations to take into account when acquiring data using a CLSM system. © 2018 by John Wiley &Sons, Inc. PMID: 29927100 [PubMed - as supplied by publisher]
Source: Current Protocols in Cytometry - Category: Molecular Biology Tags: Curr Protoc Cytom Source Type: research
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Purpose: To investigate the relationship of the pre–anti-vascular endothelial growth factor (VEGF) retinal tissue area (RTA) and optical density (ODRT) of the retinal optical slice portion located in the central subfield, and their ratio (RTA/ODRT), in the presence of diabetic macular edema or of inraretinal cystic fluid in neovascular age-related macular degeneration, to central retinal thickness and best-corrected visual acuity after anti-VEGF treatment with ME resolution. Methods: The optical coherence tomography images and medical records of 33 patients (41 eyes) with neovascular age-related macular degenera...
Source: RETINA - Category: Opthalmology Tags: Original Study 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
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