The kidney of patients with sickle cell disease (SCD) is affected by both haemodynamic changes of chronic anaemia and by the consequences of vaso-occlusion which are especially marked within the renal medulla. There are many abnormalities in renal structure and function as a result of these changes. Functional changes occur with increasing age in subjects with sickle cell disease. Proteinuria, ...

Sickle hemoglobin (HbS) is caused by a single nucleotide substitution (A → T) in the 6 th codon of -globin gene on chromosome 11 which results in replacement of Glutamic acid (GAG) by Valine (GTG) (Pauling et al., 1949; Ingram 1956). Under deoxygenated conditions, this substitution causes HbS polymerization (Bunn, 1997) and modifies the stability of the hemoglobin leading to the clinical disor...

Editorial left ventricular dysfunction in sickle cell disease: the value of an electrocardiographic marker of increased risk of arrhythmia Sickle cell disease is one of the most prevalent genetic diseases worldwide; affecting 1/400 individuals of African descent as well as people of Arab, Indian and Hispanic descents. Abnormalities of cardiovascular function have increasingly been documented in...

BACKGROUND Human induced pluripotent stem cells offer perspectives for cell therapy and research models for diseases. We applied this approach to the normal and pathological erythroid differentiation model by establishing induced pluripotent stem cells from normal and homozygous sickle cell disease donors. DESIGN AND METHODS We addressed the question as to whether these cells can reach comple...

Left ventricular performance was determined by echocardiography in 44 black children with homozygous sickle cell anaemia and a control group of 28 normal black children of comparable age. Statistically significant differences were observed between the children with sickle cell anaemia and the normal group in left ventricular ejection fraction (sickle cell anaemia group: 0-59 + 0.01 [mean + stan...

The aim of this study was to determine hematological profile of sickle cell disease (SCD) from Surat, South Gujarat, India. This prospective cross-sectional study was conducted in the Department of Pediatrics and Sickle Cell Anemia Laboratory, Faculty of Pathology, Government Medical College, Surat, India, between July 2009 and December 2010. Patients included in this study were in their steady...

BACKGROUND The active ingredients of marijuana may have beneficial properties in the treatment of chronic pain and inflammation and is being used by sufferers of chronic pain and arthritis in some settings. Anecdotally, marijuana is believed by some sickle cell disease (SCD) patients to improve their health. This study aimed to determine the prevalence of marijuana smoking in the Jamaica Sickle...

OBJECTIVE To investigate the cause and outcome of high fever in Jamaican children with homozygous sickle cell disease. DESIGN Retrospective review of febrile episodes in a three year period (1 September 1993 to 31 August 1996). SETTING Sickle cell clinic, an outpatient clinic in Kingston run by the Medical Research Council Laboratories (Jamaica). PATIENTS Patients with homozygous sickle c...

The disorders associated with the sickle cell trait have not, in the past, been seen very often in Britain because they are largely confined to the Negro race. However, increased immigration from the West Indies, where the trait can be found in about 10% of the Negro population (Tomlinson, 1945), has made the recognition of these diseases more important in this country (Diamond, 1959). The sick...