Background Sickle cell disease (SCD) is a neglected burden of growing importance. >312,000 births are affected annually by sickle cell anaemia (SCA). Early interventions such as newborn screening, penicillin prophylaxis and hydroxyurea can substantially reduce the mortality and morbidity associated with SCD. Nevertheless, their implementation in African countries has been mostly limited to pilot projects. Recent development of low-cost point-of-care testing (POCT) devices for sickle haemoglobin (HbS) could greatly facilitate the diagnosis of those affected. Methods We conducted the first multi-centre, real-world assessment of a low-cost POCT device, HemoTypeSC, in a low-income country. Between September and November 2017, we screened 1121 babies using both HemoTypeSC and HPLC and confirmed discordant samples by molecular diagnosis. Findings We found that, in optimal field conditions, the sensitivity and specificity of the test for SCA were 93.4% and 99.9%, respectively. All 14 carriers of haemoglobin C were successfully identified. Our study reveals an overall accuracy of 99.1%, but also highlights the importance of rigorous data collection, staff training and accurate confirmatory testing. It suggests that HPLC results might not be as reliable in a resource-poor setting as usually considered. Interpretation The use of such a POCT device can be scaled up and routinely used across multiple healthcare centres in sub-Saharan Africa, which would offer great potential for the identification and management of vast numbers of individuals affected by SCD who are currently undiagnosed. Funding US Imperial College London's Wellcome Trust Centre for Global Health Research (grant #WMNP P43370).
Sickle cell disease affects about 150,000 births annually in Nigeria. Early diagnosis is hampered by factors such as centralized and urban localization of laboratories, high cost of diagnostic equipment and inadequate skilled manpower to operate them. The need for a low-cost, portable, easy-to-use diagnostic test for sickle cell disease is critical, especially in resource-poor countries. In this study, we evaluated the performance characteristics of a novel point-of-care testing device (SickleSCAN™), and its acceptability and feasibility, as a possible screening tool for sickle cell disease. In the first phase, we assessed the performance characteristics of SickleSCAN™ by evaluating 57 subjects comprising both children and adults attending a primary health center, for Hb SS (β/β; HBB: c.20A>T), Hb SC (β/β; HBB: c.19G>A) and Hb AS (β/β) using SickleSCAN™, cellulose acetate electrophoresis (CAE) and high performance liquid chromatography (HPLC). Performance characteristics such as diagnostic sensitivity and specificity were compared to HPLC as a standard method. We subsequently undertook a second phase wherein the acceptability and feasibility of the device for sickle cell disease screening, was evaluated using semi-structured and structured questionnaires among 197 healthcare personnel and 221 subjects, respectively. Sickle cell disease was carried by 3.4% of the subjects. The diagnostic sensitivity, specificity and test efficiency of SickleSCAN™ for sickle cell disease (Hb SS and Hb SC), were 100.0, 98.2 and 98.2%, respectively. Findings from this study showed SickleSCAN™ to be a viable screening tool that can easily be applied in community-based screening for early diagnosis of sickle cell disease with little expertise and low cost.
BackgroundInvolvement of the kidneys in patients with sickle cell anaemia is a well recognised chronic complication. This study seeks to determine the prevalence of chronic kidney disease in patients with homozygous sickle cell disease (HbSS) and to identify risk factors associated with its development.MethodologyThe subjects consisted of adolescents and adults with HbSS recruited sequentially from the adult haematology outpatient clinic and Daycare ward of the unit. Clinical variables including age at diagnosis of SCA, the frequency of vaso-occlusive crisis and transfusion therapy, as well as laboratory data including haematological profile and renal function tests were obtained. The glomerular filtration rate was estimated (eGFR) using the ‘modification of diet in renal disease’ (MDRD) formula.ResultsTwo hundred and eighty-four HbSS patients were recruited. The prevalence of CKD amongst them was 38.9%. Further stratification of the patients based on eGFR showed that sixty-nine (26.8%) had hyperfiltration; 35 (13.6%) stage 1 CKD; 53 (20.6%) stage 2 CKD; 33 (12.8%) stage 3a CKD; 28 (10.9%) stage 3b CKD; 30 (11.7%) stage 4 CKD and 9 (3.5%) had end stage renal disease. There was significant association between eGFR and clinical parameters such as age (r −0.353, p=0.000), SBP (r −0.148, p= 0.021), DBP (r −0.213, p=0.001) and total number of blood received (r −0.276, p=0.000); and laboratory parameters such as PCV (r 0.371, p=0.000); urea ( r 0.527, p=000); creatinine (r 0.625, p=0.000) and uric acid ( r −0.419, p=0.000).ConclusionsThe present study has revealed a high prevalence of CKD amongst patients with SCA in our region. Clinical and laboratory predictors of CKD using eGFR were identified to include age, SBP, number of units of blood transfusion, PCV, urea, creatinine and uric acid levels.
Background: Nigeria is the most populous black nation with the heaviest burden of Sickle Cell Anaemia divided into a cold and dusty harmattan season and a hot non-harmattan dry season. We studied the impact of seasonal variations of climatic factors on the frequencies of acute vaso-occlusive morbidities, including Vaso-Occlusive Crisis (VOC), Acute Chest Syndrome (ACS), priapism and stroke among patients with SCA in northern Nigeria.Methods: This is a six-year prospective study conducted from 2005 to 2010 in university teaching hospitals of Maiduguri and Kano cities of northern Nigeria. Consecutive patients with SCA who presented with VOC, ACS, priapism and stroke were diagnosed, recruited and enumerated. The monthly and seasonal frequencies of each morbidity (VOC, ACS, priapism or stroke) during the period of study were calculated and graphically evaluated. Results:The frequencies of VOC showed 3 peaks: during the harmattan dry season in January, during the non-harmattan dry season in April and during the rainy season in August. The frequencies of ACS showed a peak during the harmattan dry season in December. The frequencies of priapism showed a peak during the nonharmattan dry season in April. The frequencies of stroke showed a peak during the rainy season in July/August. Conclusion:The frequencies of acute vaso-occlusive morbidities in SCA patients could be adversely affected by seasonal variations in climatic factors. There is need for patients and care givers to be adequately educated on how to mitigate the adverse effects of weather on SCA. Moreover, governmental and non-governmental organizations should take seasonal variations of climatic factors into consideration when drafting health care plan for patients with SCA.(SCA). The climate in the north is characterized by a short rainy season and a long dry season that can be sub- Journal of Blood Disorders & TransfusionJ ou rnal of B lo o d Dis orde rs & Tr ans fusi on
Sickle cell anaemia (SCA) is a globally widespread genetic disorder affecting 5% of the world's over 6 billion people. Parvovirus infection and the resulting aplastic crisis is a recognised complication in individuals with SCA. Aplastic crisis increases the need for blood transfusion and its attendant risk of Transfusion Transmissible Infection (TTI). Hence there is a vicious cycle in which Parvovirus B19 causes aplastic crisis which in turn causes increased transfusion need; and transfusion increases risk of transfusion transmissible infection in which parvovirus B19 is included in certain parts of the world. Sickle cell anaemia is associated with foetal death and infection with parvovirus B19 increases the risk to early mortality. The objective of this study was to determine the seroprevalence of parvovirus B19 among SCA and compare with that of controls in the study area. Furthermore clinical and laboratory profile of subjects were analysed to identify possible correlation with parvovirus B19 seropositivity and explore the possibility of involvement of white cell and platelets. A total of 90 subjects comprising 45 consecutive SCA case subjects and 45 age-and sex-matched non SCA controls were studied in a cross sectional comparative study. Ten millilitres of blood was drawn from the antecubital fossa of each subject after obtaining informed consent. The 10mls of blood was divided into two aliquots, 4.5 mls was added into EDTA anticoagulated bottle and was used for basic complete blood count (CBC), while the remaining 5mls was added into a plain specimen container allowed to clot and serum obtained to test for anti-parvovirus B19 IgG and IgM using an immunochromatography based technique specifically BIOCARD TM Parvo B19 diagnostic test kit. There was male preponderance in the study. The SCA subjects comprised 26 males and 19 females (male to female ratio = 1.4:1), while the non-SCA controls comprised 25 males and 20 females (male to female ratio 1.3:1).. The analysis of anti-parvovirus B19 IgG antibody revealed a prevalence of 23.3% among SCA cases with 18.9% among controls. The haematological profile is not affected by IgG seropositivity. However pregnancy outcome revealed that the total number of stillbirths is 12 among IgG seropositive SCA cases which is higher than the 6 encountered in IgG seronegative SCA subjects; the difference is statistically significant (p=0.04).
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