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Iranian Journal of Pediatric Hematology and Oncology، جلد ۱۵، شماره ۲، صفحات ۴۴۰-۴۵۵

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عنوان انگلیسی Prevalence, Disease Manifestations and Outcomes of Paediatric Sickle Cell Anaemia in Calabar, Nigeria: A Single Center 6-Year Review
چکیده انگلیسی مقاله
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.
کلیدواژه‌های انگلیسی مقاله Morbidity, Mortality, Paediatric, Prevalence, Sickle cell

نویسندگان مقاله | Anthony Nlemadim
Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria


| Jacintha Okoi-obuli
Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria


| Elizabeth Nkanga
Calabar Children&#039;s Eye Centre, Department of Ophthalmology, University of Calabar, Calabar, Nigeria


| Chimaeze Torty
Neurology unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria


| Kingsley Akaba
Department of Haematology, University of Calabar, Calabar, Nigeria


| Juliet Venn
Children emergency room, Department of Paediatrics, UCTH, Calabar, Nigeria


| Kelechi Uhegbu
Gastroenterology unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria


| Sunday Okonkwo
Department of Ophthalmology, University of Calabar, Calabar, Nigeria


| Grace Nwankwo
Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria


| Ofonime Essien
Department of Haematology, University of Calabar, Calabar, Nigeria


| Glory Bassey
Respiratory unit, Department of Paediatrics, University of Calabar, Calabar, Nigeria


| Friday Odey
Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria


| Martin Meremikwu
Haematology-Oncology unit, Department of Paediatrics, University of Calabar Teaching Hospital (UCTH), Calabar, Nigeria



نشانی اینترنتی http://ijpho.ssu.ac.ir/browse.php?a_code=A-10-1167-1&slc_lang=en&sid=1
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