Sickle cell disease (HbS) is a haemolytic anaemia characterized by the formation of abnormal haemoglobin. In patients with sickle cell disease, high rates of erythrocyte generation, degradation, and hyperbilirubinemia increase the risk for cholelithiasis. Previous studies have found that the incidence of cholelithiasis is 70% in adult patients. In sickle cell disease, decreased oxygen concentra...

Serum immunoreactive prolyl hydroxylase protein, galactosylhydroxylysyl glucosyltransferase activity and the aminoterminal propeptide of type III procollagen were measured in 20 patients with sickle cell disease and the values were compared with those in 20 apparently healthy Nigerians. The means for the two enzymes and serum aminoterminal propeptide of type III procollagen were significantly h...

year history of sickle cell disease was admitted to our hospital with shortness of breath and severe abdominal and joint pain consistent with a sickle cell crisis. Figure 1 shows the acute and chronic effects of this disease. Three days after admission, a space-occupying epidural hematoma developed, most likely caused by expansion of hematopoietic tissue and disruption of the bony margins with ...

Sickle cell disease (SCD) is a group of inherited disorders of the beta-hemoglobin chain. Normal hemoglobin has 3 different types of hemoglobin – hemoglobin A, A2, and F. Hemoglobin S in sickle cell disease contains an abnormal beta globin chain encoded by a substitution of valine for glutamic acid on chromosome 11. This is an autosomal recessive disorder. Sickle cell disease refers to a specif...

Although sickle cell disease was designated by a number of onomatopoetic native names in Africa for centuries ( 1 ), it was clearly described as a distinct hematological and clinical entity in the United States in 1910 (2). During the next three decades, a considerable body of descriptive, clinical, and genetic data was slowly accumulated; but progress accelerated after protein chemists such as...

SCD is a major health problem requiring lifelong multidisciplinary care to manage the wide range of medical and social consequences. A number of new approaches offer the potential to have an impact on the natural history of this disease.

BACKGROUND & OBJECTIVES Children with sickle cell disease require more frequent hospital care and younger children (<5 yr of age) are more vulnerable to mortality. There are limited data on the events leading to hospitalizations and death in younger children with sickle cell disease from India. This study was, therefore, undertaken to evaluate the morbidity pattern in hospitalized under five ch...