Background: Sickle cell disease is a genetic disorder of hemoglobin causing myriad of pathology including anemia. The purpose of this study was to evaluate the baseline values of steady state hemoglobin and packed cell volume as a guide to managing the early recognition of hemolytic crises in sickle cell anemia. Methods: A cross-sectional study was conducted among the sickle cell patients atte...

Hepatic dysfunction is a frequent manifestation in patients with sickle cell anemia. It is usually a multifactorial process. A rare benign form of extreme hyperbilirubinemia, presumably due to intrahepatic sickling, may be the cause. We report a 9 year old girl with sickle-thalassemia hemoglo binopathy, presenting with profound jaundice. Sickle cell disease is often mild in the Iranian popu...

The ventilatory response to carbon dioxide was measured by a rebreathing technique in 21 Nigerians who had homozygous sickle-cell disease. The slope of the carbon dioxide response curve was obtained by plotting the ventilation at successive half-minute intervals against the corresponding mean end-tidal PCO2. Our results showed that sickle-cell patients are as sensitive to carbon dioxide as are ...

BACKGROUND On basis of clinical observation paediatricians in Kinshasa had the impression that children with homozygous sickle cell disease have a special face characterised by hypertelorism. OBJECTIVE The purpose of the study is to determine outer orbital, inner canthal and interpupillary distances as well as proptosis in children with sickle cell disease. METHODS These measurements were p...

In sickle cell retinopathy vascular involvement is most frequently recognised at the retinal periphery, but obstruction of perimacular arterioles and of major retinal vessels may also occur. This report describes a patient with homozygous sickle cell (SS) disease with recurrent occlusion of major retinal vessels associated with recurring transient impairment of visual function.

Sickle cell disease affects approximately 5000 people in the UK predominantly of African and Afro-Caribbean origin.1 The majority of those affected live in inner city areas; for example it is the most common inherited condition in the City and Hackney district of London. There is a high morbidity and mortality in children with up to 10% dying within the first 10 years of life.2 Sickle cell rela...

Sickle cell disease (SCD) is a major genetic disorder among the tribal population. Hence the objective of the present study was to determine the prevalence and frequency of the sickle cell gene in some selected tribal population of the Ghatanji and Kelapur taluka of Yavatmal District (Central India). A total of 1078 tribal individuals were screened for SCD from 17 tribal villages constituting 3...