Plasma concentration of fucosyltransferase 7 is not associated with the number of clinically overt vaso-occlusive events in sickle cell disease.

Objective: To determine if the number of vaso-occlusive events in SCD relates to plasma concentration of fucosyltransferase 7 (FUT7), which catalyses the synthesis of selectin ligands.

Design: A prospective, analytical study.

Setting: Haematology and Chemical Pathology Departments of tertiary healthcare centres.

Impact of Binet Staging versus Tumour Bulk on Treatment Outcome in Chronic Lymphocytic Leukaemia.

Background: Most of the predictive tools put up to prognosticate treatment outcomes in patients with chronic lymphocytic leukaemia (CLL) are not easily available and affordable in our resource-constrained environment.

Aim: The aim of this study was to evaluate the impact of staging and some tumour bulk on treatment outcomes of persons with CLL, Enugu, Nigeria.

Patients And Methods: This is a 10-year review of the CLL data from the haemato-oncology unit of a Nigerian tertiary hospital to evaluate the impact of staging and tumour bulk indicators.

Cerebral artery conditional blood velocity in sickle cell disease: a multicentre study and evidence for active treatment.

Objective: To obtain multicentre data on the prevalence of normal, high or conditional (intermediate) blood velocity in the cerebral arteries among children with sickle cell disease (SCD) in Nigeria.

Design: A prospective observational study in five tertiary healthcare institutions. By transcranial Doppler (TCD) ultrasonography, cerebral artery peak systolic blood velocity (PSV) was determined in 193 children with SCD and time averaged mean of the maximum blood velocity (TAMMV) in a different cohort of 115 children.

Effect of Sickle Cell Trait on Human Immunodeficiency Virus Type 1 Infection.

Introduction: Whereas several studies show that homozygous (HbSS) sickle cell disease protects against human immunodeficiency virus infection, it is not clear if human immunodeficiency virus infection is affected by the heterozygous state of the sickle globin gene (HbAS or sickle cell trait).

Objective: To evaluate the effects of sickle cell trait on the prevalence and severity of human immunodeficiency virus type 1 infection in a large patient population.

Methods: Hemoglobin genotype was determined by high performance liquid chromatography (HPLC) in 1,226 HIV-1 patients in Nigeria.

Prevalence of venous thromboembolism and its associations in a large racially homogenous population of sickle cell disease patients.

Unlabelled: To determine the prevalence of venous thromboembolism (VTE) among adult sickle cell disease (SCD) patients in Nigeria.

Methods: This was a multicentre retrospective study in which the medical records of adult SCD patients were reviewed. Information on demographics, steady-state haemogram, clinical phenotypes, duration of follow-up, history of VTE including risk factors and management was collected.

Sickle Cell Disease Clinical Trials and Phenotypes.

Sickle cell disease, one of the world's most common genetic disorders is prevalent in sub-Saharan Africa. The trans-Atlantic slave trade accounted for the gene movement from Africa to the Caribbean and United States of America and lately, migration has resulted in the introduction of the gene to the United Kingdom and other parts of Europe. Different haplotypes exist, however the differences in these haplotypes are not sufficient to explain the different clinical variations within the same region or different settings.

Associations of α-thalassemia and BCL11A with stroke in Nigerian, United States, and United Kingdom sickle cell anemia cohorts.

Alpha-thalassemia and the rs1427407 T allele are commonly observed in sickle cell anemia (SCA) patients and are associated with reduced hemolysis and higher hemoglobin F levels, respectively. We investigated whether a high-risk genetic profile, defined as SCA patients who did not inherit either α-thalassemia or the rs1427407 T allele, had stronger associations with clinical and laboratory variables than the individual genetic components in the University of Ibadan cohort (n=249). We then replicated our findings in SCA cohorts from the University of Illinois at Chicago (UIC)(n=260) and Walk-Treatment of Pulmonary Hypertension and Sickle cell disease with Sildenafil Therapy (Walk-PHaSST)(n=387).