BACKGROUND Individuals living with sickle cell disease (SCD) have significantly increased emergency department (ED) use compared to the general population. In Saudi Arabia, health care is free for all individuals and therefore has no bearing on increased ED visits. However, little is known about the relationship between quality of life (QoL) and frequency of acute care utilization in this patie...

Selective cytopheretic inhibitory device (SCD) therapy is an immunomodulatory treatment provided by a synthetic biomimetic membrane in an extracorporeal circuit, which has shown promise in preclinical large animal models of severe sepsis as well as in clinical trials treating patients with acute kidney injury and multiple organ failure. During SCD therapy, citrate is administered to lower ioniz...

OBJECTIVES To explore the relationship between executive function (EF) and social skills in youth with sickle cell disease (SCD).  METHODS  20 youth with SCD completed objective tests of EF (Tasks of Executive Control; Animal Sorting subtest from the Developmental Neuropsychological Assessment-Second Edition), an IQ screener, and paper-and-pencil measures of social skills (Social Skills Impro...

OBJECTIVE To evaluate lung functional capacity (FC) for physical exercise in children and adolescents with sickle cell disease (SCD) through the six-minute walk test (6MWT). METHOD A cross-sectional prospective study was performed to evaluate the FC of 46 patients with SCD through the 6MWT. The following parameters were assessed: heart rate (HR), respiratory rate (RR), peripheral pulse oxygen...

Background Current heart failure guidelines recommend insertion of implantable cardiac defibrillators (ICD) for primary prevention of sudden cardiac death (SCD) in patients whose left ventricular ejection fraction is (LVEF) ≤ 35% on maximally tolerated medical therapy. However, the majority of sudden cardiac death (SCD) in patients with heart failure occurs in those with mild-moderate systolic ...

AIMS To assess the effects of cardiac resynchronization therapy (CRT) in > or =80-year-old patients vs. patients <80 years, in terms of clinical, functional, and echocardiographic parameters after 12 month of CRT, survival, and incidence of arrhythmic events. METHODS AND RESULTS The study population consisted of 1181 CRT patients (85 were > or =80 years old). They were enrolled in a national ...

BACKGROUND Observational data on sudden cardiac death (SCD) in the young are scarce, but the SCD incidence of this study is to examine regional differences in SCD incidence within a population among young individuals (<40 years) and to assess whether regional incidences are associated with socio-economic status (SES ). METHODS SCD cases aged <40 years were identified in 12 provinces of the Ne...

OBJECTIVE To verify genetic determinants associated with stroke in children with sickle cell disease (SCD). METHODS Prospective cohort with 110 children submitted to neonatal screening by the Neonatal Screening Program, between 1998 and 2007, with SCD diagnosis, followed at a regional reference public service for hemoglobinopathies. The analyzed variables were type of hemoglobinopathy, gender...

Patients who have experienced a myocardial infarction (MI) are at increased risk of sudden cardiac death (SCD). With the advent of implantable cardioverter-defibrillators (ICDs), accurate risk stratification has become very relevant. Numerous investigations have proven that a reduced left ventricular ejection fraction (LVEF) significantly increases the SCD risk. Furthermore, ICD implantation in...

Preclinical and clinical studies demonstrate the feasibility of treating β-thalassemia and Sickle Cell Disease (SCD) by lentiviral-mediated transfer of the human β-globin gene. However, previous studies have not addressed whether the ability of lentiviral vectors to increase hemoglobin synthesis might vary in different patients.We generated lentiviral vectors carrying the human β-globin gene wi...