Hydroxyurea reduces cerebral metabolic stress in patients with sickle cell anemia

Chronic transfusion therapy (CTT) prevents stroke in selected patients with sickle cell anemia (SCA). We have shown that CTT mitigates signatures of cerebral metabolic stress, reflected by elevated oxygen extraction fraction (OEF), which likely drives stroke risk reduction. The region of highest OEF falls within the border zone, where cerebral blood flow (CBF) nadirs; OEF in this region was reduced after CTT. The neuroprotective efficacy of hydroxyurea (HU) remains unclear. To test our hypothesis that patients receiving HU therapy have lower cerebral metabolic stress compared with patients not receiving disease-modifying therapy, we prospectively obtained brain magnetic resonance imaging scans with voxel-wise measurements of CBF and OEF in 84 participants with SCA who were grouped by therapy: no disease-modifying therapy, HU, or CTT. There was no difference in whole-brain CBF among the 3 cohorts (P = .148). However, whole-brain OEF was significantly different (P
Source: Blood - Category: Hematology Authors: Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Source Type: research

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Source: NYT Health - Category: Consumer Health News Authors: Tags: Drugs (Pharmaceuticals) Health Insurance and Managed Care Sickle Cell Anemia Prices (Fares, Fees and Rates) Stroke Clinical Trials Hemoglobin Food and Drug Administration Novartis AG Global Blood Therapeutics Source Type: news
Conclusion: Our findings suggest that hydroxyurea is safe and may prevent silent stroke and stroke in sickle cell disease. More high-quality studies including RCTs are needed.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Systematic Review and Meta-Analysis Source Type: research
This study was carried out to compare the fetomaternal outcome in patients with SCD with those without SCD. This was a retrospective cohort study done in the Eastern Province of Saudi Arabia in a tertiary care, teaching hospital, by retrieving the data through the code ICD-9 for SCD, the control group was also selected with comparable characteristics. A total of 302 SCD pregnant patients were included for comparison with 600 pregnant women without SCD as control, during the period of Jan 1, 2008 to December 31, 2018. After the data retrieval, percentages of complications were calculated between the study and control groups...
Source: African Journal of Reproductive Health - Category: African Health Tags: Afr J Reprod Health Source Type: research
To evaluate adherence to annual transcranial Doppler ultrasound (TCD) screening to prevent stroke among patients with sickle cell anemia (SCA) seen in the emergency department (ED).
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Original Articles Source Type: research
DISCUSSION: In the 1980s and 1990s we used the Haemonetics Corporation's V-50 machine to perform intermittent whole blood (WB) exchange to treat severe complications of sickle cell disease (SCD.) The results were excellent, as previously described [1]. PMID: 31882437 [PubMed - in process]
Source: Annals of Clinical and Laboratory Science - Category: Laboratory Medicine Authors: Tags: Ann Clin Lab Sci 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
Inherited hemoglobin disorders, including beta-thalassemia (BT) and sickle-cell disease (SCD) are the most common monogenic diseases worldwide, with a global carrier frequency of over 5%. With migration they are becoming more common worldwide, making their management and care an increasing concern for health care systems.BT is characterized by an imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and compensatory haemopoietic expansion. Globally, there are over 25,000 births each year with transfusion-dependent thalassemia (TDT). The current available tr...
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research
Children with sickle cell anemia (SCA) are highly susceptible to stroke and other manifestations of pediatric cerebral vasculopathy. Detailed evaluations in sub-Saharan Africa are limited.
Source: BMC Pediatrics - Category: Pediatrics Authors: Tags: Research article Source Type: research
Abstract *_. PMID: 31649130 [PubMed - as supplied by publisher]
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
AbstractVascular complications of sickle cell anemia (SCA) are influenced by many factors. Elevated plasma homocysteine (Hcy) is supposed to be an independent risk factor and is either genetic or nutritional origin. The present study evaluated the plasma Hcy level, MTHFR C677T gene polymorphism, effect of folic acid (FA) supplementation ‚ and hemato-biochemical parameters in SCA and their effect on the vaso-occlusive crisis (VOC) in SCA patients of an Asian-Indian haplotype population. One hundred twenty cases of SCA (HbSS) and 50 controls with normal hemoglobin(HbAA) were studied. It was found that the plasma Hcy le...
Source: Annals of Hematology - Category: Hematology Source Type: research
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