ABSTRACT
Aim: The serum trace elements statuses of sickle cell patients attending at General Hospital Owerri, Nigeria were investigated to determine whether or not the serum levels of these elements were normal. Materials and Methods: One hundred confirmed sickle cell patients (HbSS) age 5–30 years were selected. One hundred normal subjects (HbAA) age 5–30 years were used as control. Results: The levels of trace elements were significantly decreased in sickle cell anemia (p<0.05), except copper, when compared with the control. Conclusion: The result suggests, but not conclusively, that supplementation of sickle cell patients with food and drug containing trace elements might be helpful, particularly if diminished mineral levels predispose patients to crises.
ABSTRACT
Aims: The aim is to investigate the neck-shaft angle or Collo-Diaphyseal Angle (CDA) of femur and the effect of homozygous sickle cell (HbSS) on the angle. Study Design: A retrospective study. Place and Duration of Study: Radiology Departments of two Hospitals in Maiduguri, namely Umaru Shehu Modern Hospital and University of Maiduguri Teaching Hospital (UMTH), between January, 2009 - December, 2010. Methodology: Using plain radiographs of the femur, a total of 194 HbSS and 40 control (non-HbSS) children below 17 years of age were selected. The study samples whose clinical data had been excluded from any diseases that could modify the femur were studied. The Technical Error of Measurement (TEM) was performed by the evaluator. Ethical clearance for the study was obtained from the relevant body of these government hospitals. Results: The TEM values obtained were less than 1 and were considered as a good measurement method for the evaluator performance. The CDA (mean ± standard deviations) were higher in males than females. The CDA in the left femur was greater than the right both for males and females, respectively. Furthermore, the study documents, probably for the first time that in few cases there was a reduction in the CDA of HbSS when compared with the control groups. The study revealed that there was sexually significant variation (p<0.05). The demarking points and index of sexual dimorphism of CDA show sex differences and can be used for sex determination. Conclusion: The results from this study reveal that the mean CDA of femur of HbSS children of the study population were sexually dimorphic. The information from this study may aid forensic pathologists, orthopaedic surgeons and future research in evaluation of the femur.
ABSTRACT
Introduction: Role of transcranial Doppler in prevention of stroke in sickle cell children has been well appreciated. Studies are being done to develop the protocol in children. Since we don’t find stroke very commonly in this part of the world, this study was done in order to see the prevalence of abnormal flow velocity in sickle children attending sickle cell clinic. The aims of this study were to measure mean flow velocity in different vessels in homozygous sickle cell patients using transcranial Doppler study, to compare the mean velocity in sickle children with age and sex matched controls and to correlate mean velocity with headache or stroke if any and also to correlate mean velocity with number of transfusions. Materials and Methods: The study was done in Paediatric wards. It was a prospective crosssectional comparative study. Twenty six children below 14yrs of age with homozygous sickle cell disease attending the Sickle cell clinic were selected as the cases. Forty cases of similar age and sex were recruited as normal control group. Transcranial Doppler was done in six different vessels in both the groups and mean flow velocity was measured. Mean flow velocity was correlated with symptoms and number of transfusions. Velocity was classified as normal (<170cm/sec), conditional (170-199cm/sec) or abnormal (>200cm/sec). Statistical analysis was done using SPSS 10 software. Results: In normal age and sex matched controls mean blood flow velocity was 50cm/sec where as in the cases of sickle cell disease was 180cm/sec. Maximum mean velocity was observed in middle and posterior cerebral artery. In two Sickle cell cases (8%) blood flow velocity was abnormal, these children had headache though received 5-10 transfusions/year. In only 4% sickle cell children flow velocity was normal and rest had conditional velocity. Among these children 39% received less than 5 and rest received 5-9 transfusions /yr and had no symptoms of stroke. Conclusions: Flow velocity measured by Transcranial Doppler is highest in middle cerebral artery and Posterior cerebral artery which appear to be the best arteries for this test in this region. Flow velocity was significantly high in children with sickle cell disease as compared to normal children. Prevalence of abnormal flow velocity in our children was 8% and children with abnormal mean flow velocity presented with headache.
ABSTRACT
Purpose: One of the features of homozygous sickle cell disease (HbSS) is the impaired elasticity of the erythrocyte membrane that could impede microcirculatory blood flow and cause hypoxia and tissue damage. We investigated the effect of sildenafil, a phosphodiesterase 5 (PDE5) inhibitor that inhibits the breakdown of cyclic guanosine monophosphate (cGMP) resulting in vasodilatation, on the elasticity of HbSS erythrocyte. Materials and Methods: Blood samples from ten HbSS patients in steady state was exposed to different doses (5, 10, 20, and 40 μg/mL) of sildenafil and the elasticity of the erythrocytes measured at native hematocrit with the BioProfiler. An equal number of subjects with normal hemoglobin (HbAA) served as the control group. Results: There was a marginal increase in elasticity with 5 μg/mL of the drug and this became significant (P < 0.05) with the 10 μg/mL dose. Thereafter, gradual nonsignificant decreases were observed with the 20 and 40 μg/mL doses. A similar trend was observed for the control group. The elasticity values for the HbSS subjects at native hematocrit were significantly (P < 0.05) less when compared with the corresponding concentrations for the HbAA controls. This was reversed at a corrected hematocrit of 45%. Conclusion: The result of this study shows that sildenafil caused an initial increase in erythrocyte membrane elasticity in both HbSS and HbAA subjects, and this later decreased with increasing concentration of the drug possibly due to the dual effect of cyclic adenosine monophosphate (cAMP).