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Objective: To compare the cardiovascular features of patients with sickle cell anaemia (SCA) in steady-state with those in vaso-occlusive crisis (VOC) at the Wesley Guild Hospital (WGH)., Design: A descriptive cross-sectional, matched, case-control study among children with SCA at the WGH, a tertiary health facility in southwest Nigeria., Setting: The participants were recruited from the children's emergency unit and paediatric haematology clinic of the WGH., Participants: Consisted of 93 children with VOC (cases) and 93 age and sex-matched in steady state (controls), aged 5 - 15 years., Main Outcome Measures: Cardiovascular parameters, including pulse rate, blood pressure, and electrocardiographic profile, were assessed and compared using the appropriate statistical tests., Results: The mean (SD) age of the cases and controls were 8.8 (3.2) years and 9.0 (3.1) years, respectively (p= 0.106). There was no significant difference in the mean height of the groups. The mean pulse rate, diastolic, systolic, and mean arterial pressures were significantly higher in the cases than in the controls. A significantly higher proportion of the cases than the controls also had a higher frequency of heart blocks, prolonged QTc interval, ST elevation or depression, and T wave abnormality (p = 0.018, 0.039, 0.041, 0.009, respectively). The prevalence of chamber enlargements was not significantly different between the two groups., Conclusion: Cardiovascular dysfunction is worse during VOC when compared with steady state. Physicians should look for these dysfunctions in SCA children with VOC to reduce mortality from the disease., Funding: None declared., Competing Interests: Conflict of interest: None declared., (Copyright © The Author(s).)

Background: Early sickle cell disease (SCD) diagnosis has shown promise in combating SCD in many countries. The aim of this study was to assess the practice and perception of early SCD diagnosis among a group of parents and physicians in Nigeria. Patients and Methods . This was a cross-sectional descriptive study conducted to assess the opinions and practice of early diagnosis of SCD among 135 physicians caring for SCD patients and 164 mothers of children with SCD in a southwestern state of Nigeria., Results: Most physicians 132 (97.8%) were aware of prenatal SCD diagnosis, but only 51 (37.8%) would recommend it. Most physicians 129 (95.6%) routinely recommend premarital SCD genetic counseling and testing, and 89 (65.1%) were aware of the national government newborn screening program but lesser proportion 75 (55.6%) were willing to recommend it. Amongst the mothers, 154 (94%) and 158 (96%) had encountered genetic counseling for SCD and were willing to offer newborn screening to their children, respectively. On the contrary, fewer mothers 42 (25%) were aware of prenatal SCD diagnosis, 28 (17%) were willing to partake in it, and 44 (26%) were undecided. There were discrepancies in the willingness by physicians to practice early SCD diagnosis and its uptake by mothers ( p < 0.0001). The commonest reason given by both the physicians and mothers for not practicing SCD prenatal diagnosis was the high cost of the procedure., Conclusion: The perceptions and practice of early SCD diagnosis was suboptimal in the study locality. Scaling up awareness and universal coverage are required., Competing Interests: The authors declare no conflicts of interest with respect to this study., (Copyright © 2020 Oladele Simeon Olatunya et al.)

Microparticles are sub-micron vesicles possessing protein and other materials derived from the plasma membrane of their parent cells, and literature suggests that they may have a role in the pathophysiology and downstream manifestations of sickle cell disease (SCD). The contributions of red blood cells microparticles (RMP) to the pathogenic mechanisms and clinical phenotypes of SCD are largely unknown. There is a controversy as to whether the proportions of intravascular hemolysis (approximately ≤ 30% of total hemolysis) would be enough to explain some complications seen in patients with SCD. We investigated RMP among 138 SCD patients and 39 HbAA individuals. Plasma RMPs were quantified by flow cytometry, plasma hemoglobin and heme by colorimetric assays, and haptoglobin and hemopexin by ELISA. The patients had higher RMP, plasma hemoglobin, and heme compared to the controls. On the contrary, haptoglobin and hemopexin were depleted in the patients. The RMP correlated positively with heme, lactate dehydrogenase, plasma hemoglobin, serum bilirubin, reticulocyte counts, and tricuspid regurgitant jet velocity of the patients. Contrarily, it correlated negatively with HbF, hemopexin, red blood cells counts, hemoglobin concentration, and haptoglobin. Although patients treated with hydroxyurea had lower RMP, this did not attain statistical significance. Patients with sickle leg ulcer and elevated tricuspid regurgitant jet velocity had higher levels of RMP. In conclusion, these data suggest that RMPs are associated with hemolysis and may have important roles in the pathophysiology and downstream complications of SCD.

Background: (TA) n repeat sequence (rs8175347) of UGT1A1 gene promoter polymorphism is associated with serum bilirubin levels and gallstones among different sickle cell anaemia (SCA) populations. There are no data on UGT1A1 polymorphisms and their impact on Nigerian SCA patients. In this study, we determined the distribution of the UGT1A1 (TA) n genotypes among a group of young Nigerian SCA patients and healthy controls. In addition, the influence of UGT1A1 (TA) n genotypes on the laboratory and clinical events among the patients was determined., Methods: The distribution of the UGT1A1 (TA) n genotypes among 101 young Nigerian SCA patients and 64 normal appropriate controls were determined and studied. The UGT1A1 (TA) n genotypes were further classified into subgroups and used to differentiate the clinical events and laboratory parameters of the patients., Results: Four (TA) n alleles:(TA)5, 6, 7, and 8 were found. These were associated with 10 genotypes: TA5/5, 5/6, 5/7, 5/8, 6/6, 6/7, 6/8, 7/7, 7/8, 8/8. The normal (wild-type)-(TA) 6/6), low- (TA) 7/7, 7/8, 8/8), intermediate- (TA) 5/7, 5/8, 6/7, 6/8), and high-activity (TA) 5/5, 5/6,) genotypes were found in 24.8, 24.8, 41.5, and 8.9% patients and 20.3, 15.6, 61, and 3.1% controls respectively. The general genotype distribution of the patients and control group were not significantly different. There were significant differences in serum bilirubin and lactate dehydrogenase (LDH) of the patients when differentiated by the UGT1A1 (TA) n genotypes (p<0.05). Asymptomatic gallstones were found in 5.9% of patients and were significantly of the low-activity genotypes sub-group 5 (20%) vs 1(1.3%) p = 0.0033. Although, bilirubin and fetal hemoglobin (HbF) of patients with gallstones were significantly different from those without gallstone, only the serum bilirubin was associated with UGT1A1 (TA) n genotypes on multivariate analysis (p < 0.0001)., Conclusion: This study highlights the contribution of UGT1A1 polymorphisms, a non-globin genetic factor, to the laboratory and clinical manifestations of young Nigerian SCA patients for the first time. It also shows that children with co-inheritance of low UGT1A1 (TA) n affinity genotypes may be at risk of gallstone, hence the need to follow them up.

Background: There is paucity of data on the influence of alpha thalassemia on the clinical and laboratory parameters among Nigerian sickle cell anemia (SCA) patients. This study aimed to determine the prevalence of alpha thalassemia and the influence of alpha thalassemia on laboratory parameters and clinical manifestations in a group of young Nigerian SCA patients., Methods: This was a cross-sectional retrospective study conducted on 100 patients with SCA and 63 controls. The diagnosis of SCA was confirmed by DNA studies. Alpha thalassemia genotyping was performed by multiplex gap-PCR method. Laboratory parameters including complete blood count, hemoglobin quantitation, serum lactate dehydrogenase (LDH), and bilirubin were determined with standard techniques., Results: Alpha thalassemia was found in 41 (41.0%) patients compared to 24 (38.1%) controls (P = 0.744), and all were due to the 3.7 κb α-globin gene deletions. Alpha thalassemia was associated with more frequent bone pain crisis, higher hemoglobin concentration, red blood cell count, and HbA 2 level among the patients. On the contrary, patients with alpha thalassemia had lower mean corpuscular volume, mean corpuscular hemoglobin, and white blood cell count (WBC) (P ˂ 0.05). There were 6 (6.0%) patients with leg ulcers, and none of them had alpha thalassemia, P = 0.04., Conclusion: This study confirms that coexistence of alpha thalassemia with SCA significantly influences both the clinical and laboratory manifestations of young Nigerian SCA patients. The coexistence of this genetic modifier is associated with increased bone pain crisis and protects against sickle leg ulcers among the patients., (© 2018 Wiley Periodicals, Inc.)

Introduction/objective: Sickle cell disease (SCD) is associated with episodic illnesses, multi-systemic affectations and end-organs damages. Otolaryngological related complications are not unexpected. Studies on the overall Otolaryngological pathologies in children with SCD relative to their non-SCD counterparts are scanty in Nigeria. We hypothesized that children with SCD are likely to have more otological burdens than their non-SCD counterparts. Thus, we embarked on this study to describe and compare the overall ear diseases burdens seen in children with sickle cell disease relative to their non-SCD counterparts., Methodology: A cross-sectional study of otologic diseases among children with SCD and their non-SCD counterparts attending the paediatrics and otolaryngological clinics of a Nigerian tertiary institution was conducted., Results: Overall, 80 (47.62%) of the 168 ears of SCD patients compared to 37 (22.02%) of the 168 ears of their non-SCD counterparts were affected by diseases (p < 0.0001). The diseases were Wax, Otitis Media with Effusion, Suppurative Otitis Media, Otosclerosis and Sensorineural Hearing Loss (SNHL). There was a significant difference in the prevalence of SNHL and solitary otosclerosis between the SCD patients and their non-SCD counterparts (P < 0.05) respectively. Both the Haemoglobin concentration and HbF did not discriminate between the SCD participants with or without SNHL (P > 0.05)., Conclusion: This study showed that otological burdens are more prevalent in children with SCD than the non-SCD population. The microbiological peculiarity of suppurative otitis media (SOM) among participants stresses the need for concerted efforts at preventing SOM in SCD children. There is need for special Otolaryngological care for SCD children., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: There is a paucity of information on factors that influence the school performance of children with sickle cell anaemia (SCA) in Nigeria, despite her huge burden of the disease., Methods: In total, 101 children with SCA were recruited at a paediatric clinic in Nigeria. Their socio-demographic-matched classmates were the controls. Academic performance and cognitive functioning were obtained from school reports and Ziler's Draw-a-Person Test, respectively. Factors influencing the academic performance were determined., Results: Children with SCA had higher rates of school absence and lower haemoglobin concentration (p < 0.05). There was no difference in overall school performance between children with SCA and controls (59.0 vs. 60.6%; p = 0.256). School absenteeism > 1 week independently predicts poor school performance among the study participants (odds ratio = 15.71; 95% confidence interval = 5.93-41.66; p =0.000). Most SCA children with poor performance were absent from school for > 1 week., Conclusion: There is need to address causes of school absenteeism among children with SCA., (© The Author [2017]. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com)

Background: Sickle cell disease (SCD) is a genetic haematological disorder that affects millions of people around the world especially people of African heritage. The treatment of the symptoms of SCD includes the use of analgesics, antibiotics, and anti-malarial drugs. Studying the pattern of drug prescription is a veritable tool for establishing the current practice and how it conforms to existing guidelines., Objectives: The main objective of this study was to assess the pattern of drug prescription in children with sickle cell disease (SCD) attending the paediatric outpatients' clinic of a tertiary care centre in Ado-Ekiti, South-West Nigeria., Methods: This was a cross-sectional retrospective study carried out using the medical records of all patients with SCD who attended the paediatric outpatient clinic of the teaching hospital between January 1 and December 31, 2014. The information retrieved from the case notes included the bio-demographic data, associated co-morbid conditions and the list of prescribed drugs., Results: A total of 202 SCD patients aged below 18 years were seen in the clinic during the study period with males accounting for 61.9% of them. The mean age of all patients was 6.9 ±3.8 yrs. A total of 1015 medications were prescribed during the study period giving a mean of 5.02 ± 1.9. Vitamins/micronutrients, anti-malarial drugs, antibiotics and analgesics accounted for 41.4%, 29.0%, 15.7% and 13.9% of all prescribed drugs respectively. Antibiotics from the penicillin group were the most commonly prescribed followed by macrolides and cephalosporins while Ibuprofen (60.3%) and Acetaminophen (32.6%) were the commonly prescribed analgesics., Conclusion: High rate of antibiotic prescription, low use of opioid analgesics and non-prescription of prophylactic penicillin/pneumococcal vaccination were the main findings in this study. There is need for the introduction of standard treatment protocols for this group of patients.

Background: Sub-Saharan Africa has the largest burden and worst outcome of sickle cell disease (SCD). This gloomy outlook has been attributed to the lack of use of simple and cost-effective measures for diagnosis and treatment of the disease. Although haematology analysers are the gold standard for accurate measurement of haemoglobin (Hb) concentration, they are often out of reach of most health facilities in resource-poor settings, thus creating a care gap. We conducted this study to examine the agreement between a point-of-care device and haematology analyser for determining the Hb concentration in children with SCD and its usefulness in resource-poor settings., Methods: Ethylenediaminetetraacetic acid blood samples collected from participants were processed to estimate their Hb concentration using two devices (Sysmex KX21N haematology analyser and portable mission Hb device). The agreement between the two sets of measurements was assessed by the Bland and Altman method., Results: The intraclass and concordance correlation coefficients were 0.854 and 0.936, respectively. Sensitivity and specificity were 84.2% and 98.6%, respectively. The positive and negative predictive values were 94.1% and 96.0%, respectively. The Bland and Altman's limit of agreement was -2.3 to 1.6 and the mean difference was -0.34 with non-significant variability between the two measurements (p  =  0.949)., Conclusion: Hb concentration determined by the portable testing system is comparable with that determined by the haematology analyser. We recommend its use as a point-of-care device for determining Hb concentration of SCD children in resource-poor settings where haematology analysers are not available., (© The Author [2016]. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)