BACKGROUND People affected with sickle cell disease are at high risk of infection from Haemophilus influenzae type b. Before the implementation of Haemophilus influenzae type b conjugate vaccination in high-income countries, this was responsible for a high mortality rate in children under five years of age. In African countries, where coverage of this vaccination is still extremely low, Haemoph...

The aim of the study was to determine the factors associated with resting and exerciseinduced hemoglobin oxygen desaturation. The well-established 6-minute-walk test was conducted in 107 sickle cell children (50 with sickle hemoglobin C disease and 57 with sickle cell anemia) at steady state. Hemoglobin oxygen saturation was measured before and immediately after the 6-minute-walk test. Blood sa...

A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease. This recently reported form of sickle cell disease was not previously known to cause retinopathy and surprisingly was observed in a non-Asian individual. The ...

Rheumatoid arthritis (RA) is a chronic, systemic, autoimmune disease that primarily affects the synovial joints. Sickle cell anemia chronic inherited with vaso-occlusive attacks associated hemoglobin structure defect. The coexistence of rheumatoid and sickle rarely reported in literature. In this case report, diagnosis treatment plan 26-year-old female patient who was diagnosed applied to our o...

background: hemoglobin s arises is the result of a point mutation (a-t) in the sixth codon on the -globin gene on chromosome 11 causing sickle cell anemia. the presence of fetal hemoglobin in infancy plays a relatively protective role for vaso-occlusive symptoms that are the major contributor for the morbidity and mortality among patients with sickle cell anemia. hydroxyurea, an s-phase-specif...

BACKGROUND AND PURPOSE Age-related changes in brain morphology are crucial to understanding the neurobiology of sickle cell disease. We hypothesized that the growth trajectories for total GM volume, total WM volume, and regional GM volumes are altered in children with sickle cell disease compared with controls. MATERIALS AND METHODS We analyzed T1-weighted images of the brains of 28 children ...

Sickle cell disease is one of the most common hemoglobinopathy worldwide. It an autosomal recessive genetic disorder caused by replacement adenine to thymine nucleotide in beta chain hemoglobin results valine for glutamic acid at 6th position. This substitution formation sickle (HbS) which turn leads a reduced lifespan red blood cells (RBC). In hypoxic conditions, HbS has tendency aggregate and...

BACKGROUND Little is known about the effects of blood rheology on the occurrence of acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. DESIGN AND METHODS To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed ...

By JAMES V. NEEL, PH.D., M.D., HARVEY A. ITANO, PH.D., M.D., AND JOHN S. LAWRENCE, M.D. I N RECENT YEARS our knowledge of the etiology of sickle cell disease has increased considerably. As a rule, both parents of a child with this disease show the sickle cell trait. This has led to the hypothesis that usually sickle cell disease is due to homozygosity for a gene which, when heterozygous, result...