Hydroxyurea to lower TCD velocities and prevent primary stroke: the Uganda NOHARM sickle cell anemia cohort.

Hydroxyurea to lower TCD velocities and prevent primary stroke: the Uganda NOHARM sickle cell anemia cohort. Haematologica. 2019 Oct 24;: Authors: Opoka RO, Hume HA, Latham TS, Lane A, Williams O, Tymon J, Nakafeero M, Kasirye P, Ndugwa CM, John CC, Ware RE Abstract *_. PMID: 31649130 [PubMed - as supplied by publisher]
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research

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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
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
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
This study aimed to investigate the influence of genetic variants on the levels of fetal hemoglobin (Hb F) and biochemical parameters related with chronic hemolysis, as well as on ischemic stroke risk, in ninety-one unrelated SCA patients, children of sub-Saharan progenitors. Our results show that a higher Hb F level has an inverse relationship with the occurrence of stroke, since the group of patients who suffered stroke presents a significantly lower mean Hb F level (5.34  ± 4.57% versus 9.36 ± 6.48%;p = 0.024). Furthermore, the co-inheritance of alpha-thalassemia imp...
Source: Annals of Hematology - Category: Hematology Source Type: research
It’s only been seven years since scientists first learned how to precisely and reliably splice the human genome using a tool called CRISPR, making it possible to think about snipping out disease-causing mutations and actually cure, once and for all, genetic diseases ranging from sickle cell anemia to certain types of cancer and even blindness. Doctors are plunging ahead in search of ways to use the relatively new technology to start treating patients. In China last November, scientist Jiankui He stunned—and dismayed—the genetic community when he announced he had already used CRISPR, which many believe sti...
Source: TIME: Health - Category: Consumer Health News Authors: Tags: Uncategorized crispr Genetics Source Type: news
CONCLUSIONS: Patients exhibiting greater daily HbA decrement were more likely to have multiple emergency department visits or admissions for sickling crises. Modulating HbA decrement may merit study as an intermediate metric for interventions to improve outcomes in hemoglobin SS disease. PMID: 31161685 [PubMed - as supplied by publisher]
Source: Transfusion - Category: Hematology Authors: Tags: Transfusion Source Type: research
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 t...
Source: Blood - Category: Hematology Authors: Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Source Type: research
ConclusionIndividuals with SCD are at risk for cognitive deficits across domains, infarct status, and the lifespan. More research is necessary to determine unbiased effects for cognitive function in adults with SCD.
Source: Journal of Pediatric Psychology - Category: Pediatrics Source Type: research
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