Microvascular occlusion in sickle cell disease can be initiated by adhesion of sickle red blood cells (RBCs) to the endothelium. Our objective in this study was to verify the relevance in vivo of our discovery that sickle RBCs adhere abnormally to endothelial P-selectin in vitro. We used computer-assisted intravital microscopy to characterize RBC flow velocity (V(RBC)) in mice. We found faster ...

Sickle cell disease can lead to hepatic complications ranging from acute hepatic crises to chronic liver disease including intrahepatic cholestasis, and iron overload. Although uncommon, intrahepatic cholestasis may be severe and medical treatment of this complication is often ineffective. We report a case of a 37 year-old male patient with sickle cell anemia, who developed liver failure and un...

OBJECTIVE The present study prospectively investigated the role of self-efficacy in predicting disease symptomatology and health services utilization for adult patients with sickle cell disease. METHODS These data are derived from a 12-month prospective cohort study of African American adults with sickle cell disease. Disease-specific perceptions of coping self-efficacy and indices of disease...

abdominal trauma and findings did not suggest perforation of bowel or biliary tree. Infections are a major cause of morbidity and mortality in sickle cell disease.1 Patients present with pulmonary, meningeal, bone, or bloodstream infections usually caused by bacteria, although there is increasing awareness of the role of mycoplasmas and viruses in sickle lung disease. Fungal infections in sickl...

Translocation renal cell carcinoma (RCC) is a family of rare tumors recently identified in the pediatric and young adult population. We report the first case of a young woman from French West Indies with sickle cell anemia who developed a translocation RCC t(6;11)(p21;q12). Usually people with the sickle cell condition are known to develop renal medullary carcinoma (RMC). To our knowledge, this...

Sickle cell haemoglobin D disease (D-Punjab) is a rare variant of sickle (SCD) reported from central India. Exact incidence pulmonary thrombosis in patients with HbSD unknown. Pulmonary known complication SCD but case pattern. We 34-year-old male patient acute chest syndrome (ACS). CT angiogram revealed near complete right middle lobe segmental and subsegmental branches infarct. Our responded t...

BACKGROUND Sickle cell disease is the most common monogenic hereditary disease in Brazil. Although strokes are one of the main causes of morbidity and mortality in these patients, the use of transcranial Doppler to identify children at risk is not universally used. OBJECTIVE To develop Brazilian guidelines for the use of transcranial Doppler in sickle cell disease children and adolescents, so...

AIMS Patients with sickle cell disease have significant morbidity and mortality. Pulmonary hypertension is suggested to be an important contributor but its nature and severity in these patients and how best to non-invasively assess it are controversial. We hypothesised that a high-output state rather than primary pulmonary vascular pathology may be the major abnormality in sickle cell disease. ...

BACKGROUND Patients with sickle cell disease in the USA have been noted to have lower levels of vitamin D - measured as 25-hydroxyvitamin D (25(OH)D) - compared to controls. Average serum 25(OH)D levels are also substantially lower in African Americans than whites, while population distributions of 25(OH)D among Jamaicans of African descent and West Africans are the same as among USA whites. Th...