Iranian journal of Pediatric Hematology and Oncology

en Prevalence, Disease Manifestations and Outcomes of Paediatric Sickle Cell Anaemia in Calabar, Nigeria: A Single Center 6-Year Review هماتولوژی Hematology پژوهشي Research <div style="border:double windowtext 1.5pt; padding:1.0pt 4.0pt 1.0pt 4.0pt"></div> <div style="border: 1.5pt double windowtext; padding: 1pt 4pt; text-align: justify;">Background: The burden of sickle cell anaemia (SCA) is worsening globally. Recognition of paediatric SCA burden is vital to comprehending the overall SCA burden. Aim was to determine the prevalence, disease manifestations and outcomes of paediatric SCA. Materials and Methods: It was a retrospective study of all children with SCA, 0.5 year (6months) - 17 years old, managed from 2018 through 2023. Data obtained were sex, age at first visit per year, diagnoses and date per visit, location (clinics or emergency-room) of visit, intervals between visits, and death. Disease manifestations per subject were summarized into diagnosis while default was defined as >3 months visit interval. Results: Of the 532 subjects, 55.6% were males with overall median (interquartile [IQR]) age on first visit of 9 (5 - 13) years. On average, paediatric SCA constituted 4% of first visits per year. There were 252 sick visits per year, 544 diagnoses per year and 146 hospitalisations per year. Commonest diagnosis in emergency-room and clinics were bone pain crises (46.2%) and steady-state (48.5%), respectively. The 11 - 17-year-olds were more likely to have bone pain crises than 0.5 - 4-year-olds (Odds Ratio [OR] 0.381; 95%CI: 0.487-0.787) and 5 - 9-year-olds (OR 0.298; 95%CI: 0.573-0.861). They were also more likely to have avascular necrosis than the 0.5 - 4-year-olds (OR 0.789; 95%CI: 0.047-0.938) and 5 - 9-year-olds (OR 0.777; 95%CI: 0.064-0.781). Overall median (IQR) default time was 6 (5 - 7) months with more defaults (85.1%) than compliants (14.9%) (p<0.001) while 0.56% died. Conclusion: The overall prevalence of Paediatric SCA in the region is 4% with approximately one hospitalisation per sick visit and more than one diagnoses per visit. There is a high default rate but a low mortality rate (0.56%). Sustained improvement in the management of SCA, from childhood through adulthood, may help alleviate the increasing burden of the condition.</div> <div style="text-align: justify;"></div> Morbidity, Mortality, Paediatric, Prevalence, Sickle cell 440 455 http://ijpho.ssu.ac.ir/browse.php?a_code=A-10-1167-1&slc_lang=en&sid=1 Anthony Nlemadim tonynlemas@yahoo.com 0000-0003-0095-8975 Yes Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Jacintha Okoi-obuli okoiobulijacintha@gmail.com No Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Elizabeth Nkanga bettienkanga@gmail.com 0000-0002-7178-1567 No Calabar Children's Eye Centre, Department of Ophthalmology, University of Calabar, Calabar, Nigeria Calabar Children's Eye Centre, Department of Ophthalmology, University of Calabar, Calabar, Nigeria Chimaeze Torty chimaezetorty@unical.edu.ng 0000-0002-3288-802X No Neurology unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria Neurology unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria Kingsley Akaba akaba_kingsley@yahoo.com 0000-0002-4573-1187 No Department of Haematology, University of Calabar, Calabar, Nigeria Department of Haematology, University of Calabar, Calabar, Nigeria Juliet Venn odyvenn04@yahoo.co.uk No Children emergency room, Department of Paediatrics, UCTH, Calabar, Nigeria Children emergency room, Department of Paediatrics, UCTH, Calabar, Nigeria Kelechi Uhegbu kelechi4u2001@gmail.com 0000-0002-1668-8386 No Gastroenterology unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria Gastroenterology unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria Sunday Okonkwo okosund@yahoo.com 0000-0003-1909-0600 No Department of Ophthalmology, University of Calabar, Calabar, Nigeria Department of Ophthalmology, University of Calabar, Calabar, Nigeria Grace Nwankwo drgracenwankwo@gmail.com No Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Ofonime Essien ofonimessien29@yahoo.com 0000-0002-3111-740X No Department of Haematology, University of Calabar, Calabar, Nigeria Department of Haematology, University of Calabar, Calabar, Nigeria Glory Bassey globass75@gmail.com No Respiratory unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria Respiratory unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria Friday Odey faodey@gmail.com 0000-0003-1066-5631 No Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Martin Meremikwu mmeremiku@gmail.com 0000-0002-6144-9413 No Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria