Sickel cell disease is common in urban areas of Britain and it is estimated that in London alone there are nearly 2000 patients. One hundred and eighty four patients with sickle cell disease are known to the Central Middlesex Hospital, and 155 of those attend the sickle cell clinic regularly. The commonest cause for admission to hospital is acute painful or vaso-occlusive crisis, which accounts...

The renal complications of sickle cell anemia are well known. Gross hematuria is one of these, although it is associated more often with the sickle cell trait than with the disease. Nonetheless, in Togo, where the frequency of S gene is 16%, hematuria is rarely report in people with this gene. We describe 3 cases of gross hematuria in patients with sickle cell disease: primary hematuria associa...

The tendency for sickle cells to adhere to each other is increased in oxygenated sickle blood in parallel with cell density. The increased adherence of these cells occurred despite their reduced deformability and diminished ability to form rouleaux. Using a method developed in our laboratory, we measured the yield stress: a sensitive index of cell-cell adhesion of deoxygenated suspensions of si...

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...

OBJECTIVE Evaluate the Neonatal Screening Program of the Health Secretariat of the State of Santa Catarina for sickle-cell disease, from January 2003 to December 2012, regarding program coverage and disease frequency. METHODS Descriptive, observational, cross-sectional study with retrospective data collection. The variables analyzed were: number of live births in the State of Santa Catarina; ...

Background: Until now, trimodal distribution of HbS has been seen by six different studies in the world when associated with alpha-thalassemia with confirmation by corresponding alpha-genotyping studies. The RBC indices reduce as alpha-globin genes reduce in sickle cell trait (SCT) patients, which decreases the extent of intra-vascular sickling and thus betters the clinical cou...

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...

Recently, ithas been demonstrated thatthe offspring of one parent with the sickle cell trait and one w’ith thalassemia may develop a severe hemolytic anemia with some of the characteristics of both thalassemia and sickle cell disease, ineluding microcytosis and numerous sickled erythrocytes in the blood film. This syndrome has been called mi(’rodrepanocytic,9 or sickle cell-thalassemia, disease...