Increased circulating copeptin levels are associated with vaso-occlusive crisis and right ventricular dysfunction in sickle cell anemia

Conclusion The study showed that copeptin and hs-CRP levels were increased in patients with VOC, and a significant relationship was fo und between RV dysfunction in VOC patients. As a conclusion copeptin can be used as a potential biomarker in predicting VOC crisis in SCA patients and in early detection of patients with SCA who have the potential to develop RV dysfunction.
Source: Medical Principles and Practice - Category: Internal Medicine Source Type: research

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The objective of this narrative review is to discuss the prevalence, pathophysiology mechanisms, diagnostic techniques, treatment options, and prognostic indicators in the setting of sickle cell disease with pulmonary hypertension. Additionally, the review also highlights other advancements that are being investigated. Considering the significant morbidity, mortality, and prevalence of pulmonary hypertension in patients with sickle cell disease, it is important to account for the aforementioned domains in the future guidelines to provide optimal and individualized care to the high-risk individuals as well as reduce the pro...
Source: Pulse - Category: Cardiology Source Type: research
CONCLUSION: The prevalence of SLU, AVN, priapism, nephropathy and the other complications of SCD show some variations from other studies. This variation in the clinical parameters across different clinical phenotypes indicates an interplay between age, genetic and environmental factors.PMID:34493173 | DOI:10.1080/16078454.2021.1972242
Source: Hematology - Category: Hematology Authors: Source Type: research
Purpose of review Sickle cell disease (SCD), one of the most common genetic diseases in the world, is characterized by repeated episodes of hemolysis and vaso-occlusion. Hemolytic anemia is a risk factor for the development of pulmonary hypertension, and currently SCD-related pulmonary hypertension is classified as World Health Organization group 5 pulmonary hypertension. Patients with SCD-related pulmonary hypertension have unique hemodynamics, multiple comorbidities, and distinct phenotypes that may contribute to the development of pulmonary hypertension. Recent findings SCD-related pulmonary hypertension is d...
Source: Current Opinion in Pulmonary Medicine - Category: Respiratory Medicine Tags: DISORDERS OF THE PULMONARY CIRCULATION: Edited by Steven D. Nathan, Christopher King and Oksana A. Shlobin Source Type: research
Hemolysis is a pathological feature of several diseases of diverse etiology such as hereditary anemias, malaria, and sepsis. A major complication of hemolysis involves the release of large quantities of hemoglobin into the blood circulation and the subsequent generation of harmful metabolites like labile heme. Protective mechanisms like haptoglobin-hemoglobin and hemopexin-heme binding, and heme oxygenase-1 enzymatic degradation of heme limit the toxicity of the hemolysis-related molecules. The capacity of these protective systems is exceeded in hemolytic diseases, resulting in high residual levels of hemolysis products in...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Conclusions: In sum, our results suggest that total cholesterol, HDL-C, and LDL-C levels are associated with hemolysis and anemia markers and, most importantly, with clinical complications related to vasculopathy in SCA. PMID: 32884585 [PubMed - in process]
Source: Disease Markers - Category: Laboratory Medicine Tags: Dis Markers Source Type: research
CONCLUSIONS: 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 to generate wild type and sickle cell mice on a HK-deficient background. We found that short-term HK deficiency attenuated thrombin generation and inflammation in sickle mice at steady state, which was independent of bradykinin signaling. Moreover, long-term HK deficiency attenuates kidney injury, reduces chronic inflammation, and ultimately improves of sickle mice. PMID: 32573897 [PubMed - as supplied by publisher]
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: J Thromb Haemost Source Type: research
Sickle cell disease (SCD) is a hemoglobin disorder leading to chronic hemolysis and multi-organ morbidity. Increasing evidence suggests that activation of coagulation contributes to its complications such as stroke, pulmonary hypertension, venous thromboembolism and avascular necrosis [1]. The effects are most profound in homozygous state Hemoglobin SS (HbSS) and Hemoglobin S-Beta zero thalassemia referred together as sickle cell anemia (SCA). With improved survival of patients with SCA in the current era, early recognition of long-term complications is a priority, but there is a paucity of biomarkers to identify SCA hypercoagulability.
Source: Transfusion and Apheresis Science - Category: Hematology Authors: Source Type: research
Authors: Aubry A, Paternot A, Vieillard-Baron A Abstract Cor pulmonale is a disease of the heart characterised by dilatation of the right ventricle and paradoxical movement of the interventricular septum. The diagnosis depends on echocardiography even if pulmonary artery catheterisation suggests it. It is secondary to pulmonary disease or a disorder of the pulmonary circulation. These two mechanisms, which are often connected, involve pulmonary hypertension as the origin of a systolic and diastolic overload of the right ventricle, which then leads to the alterations of its structure and performance. Acute cor pulmo...
Source: Revue des Maladies Respiratoires - Category: Respiratory Medicine Tags: Rev Mal Respir Source Type: research
Publication date: Available online 2 November 2019Source: Nitric OxideAuthor(s): Luis E.F. Almeida, Sayuri Kamimura, Celia M. de Souza Batista, Nicholas Spornick, Margaret Y. Nettleton, Elizabeth Walek, Meghann L. Smith, Julia Finkel, Deepika Darbari, Paul Wakim, Zenaide M.N. QuezadoAbstractThe hypothesis of decreased nitric oxide (NO) bioavailability in sickle cell disease (SCD) proposes that multiple factors leading to decreased NO production and increased consumption contributes to vaso-occlusion, pulmonary hypertension, and pain. The anion nitrite is central to NO physiology as it is an end product of NO metabolism and...
Source: Nitric Oxide - Category: Chemistry Source Type: research
Abstract Sickle cell disease (SCD) is among the most common genetic diseases in the United States, affecting approximately 100,000 people. In the United States, SCD is characterized by a shortened life expectancy of only about 50 years in severe subtypes, significant quality-of-life impairments, and increased healthcare utilization and spending. SCD is characterized by chronic hemolytic anemia, vaso-occlusion, and progressive vascular injury affecting multiple organ systems. The pathophysiology is directly related to polymerization of deoxygenated hemoglobin, leading to a cascade of pathologic events including ery...
Source: The American Journal of Managed Care - Category: Health Management Authors: Tags: Am J Manag Care Source Type: research
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