BACKGROUND Sickle Cell Disease (SCD) causes profound suffering and decrements in daily functioning. Demand is growing for valid and reliable measures to systematically document these effects, particularly in adults. The Adult Sickle Cell Quality of Life Measurement System, ASCQ-Me℠, was developed for this purpose. ASCQ-Me℠ is one of four measurement systems housed within the Person-Centered Ass...

Recent studies have demonstrated greater risks of cardiovascular events and mortality among persons who have lower 25-hydroxyvitamin D (25-OHD) and higher parathyroid hormone (PTH) levels. We sought to evaluate the association between markers of mineral metabolism and sudden cardiac death (SCD) among the 2312 participants from the Cardiovascular Health Study who were free of clinical cardiovasc...

BACKGROUND Acute chest syndrome (ACS) is a leading cause of hospitalization and death of children with sickle cell disease (SCD). An evidence-based ACS/SCD guideline was established to standardize care throughout the institution in February 2008. However, by the summer of 2009 use of the guideline was inconsistent, and did not seem to have an impact on length of stay. As a result, an implementa...

Sickle cell disease (SCD) is the most common life-limiting genetic disease among African Americans, affecting more than 100,000 people in the United States. Respiratory disorders in patients with sickle cell disease have been associated with increased morbidity and mortality. Associations between asthma and pain, acute chest syndrome (ACS), and even death have long been reported. More recently ...

BACKGROUND Sudden cardiac death (SCD) is a major cause of mortality secondary to coronary artery disease (CAD) in the industrialized societies. Although South Asians have a high prevalence of CAD, the frequency and underlying pathology of SCD among this population are unknown. METHODS Medical records of consecutive patients presenting with unexplained sudden death (USD) in a tertiary care cen...

The literature was reviewed to investigate the existence of unique gastrointestinal (GI) pathological lesions in sickle-cell disease (SCD). Chole- and choledocholithiasis have long been recognized, but bilirubin gallstones can occur in any chronic hemolytic anemia. Acute pancreatitis has been reported as a possible ischemic consequence of sickling. It is unclear if the hepatic lesions of SCD di...

OBJECTIVES To assess sickle cell disease (SCD) patient and carer perspectives on the primary care services related to SCD that they receive from their general practitioner (GP). DESIGN A focus group discussion was used to elicit the views of patients about the quality of care they receive from their primary health-care providers and what they thought was the role of primary care in SCD manage...

Background. Abnormal lipid homeostasis in sickle cell disease (SCD) is characterized by defects in plasma and erythrocyte lipids and may increase the risk of cardiovascular disease. This study assessed the lipid profile and non-HDL cholesterol level of SCD patients. Methods. A hospital-based cross-sectional study was conducted in 50 SCD patients, in the steady state, aged 8-28 years, attending ...

BACKGROUND Recent studies suggest that variants in two calcium handling genes (RyR2 and CASQ2) associated with sudden cardiac death (SCD) and non-sudden cardiac death (NSCD) in subjects with heart failure and coronary artery disease, respectively. The purpose of this study was to identify other calcium handling genes associated with SCD in the long-term of chronic heart failure (CHF) in Chinese...

BACKGROUND Treatment with hydroxyurea may alleviate the symptoms of sickle cell disease (SCD). Because treatment with hydroxyurea may be responsible for several cutaneous side effects and is often lifelong in patients with SCD, we conducted this study to evaluate the risk of cutaneous adverse reactions in SCD patients treated with hydroxyurea. OBSERVATIONS Seventeen adult patients with SCD tr...