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    Paediatric sickle cell disease at a tertiary hospital in Malawi: a retrospective cross-sectional study

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    Author
    Chimbatata, Chikondi Sharon
    Chisale, Master Ro
    Kayira, Alfred Bornwell
    Sinyiza, Frank Watson
    Mbakaya, Balwani Chingatichifwe
    Kaseka, Paul Uchizi
    Kamudumuli, Pocha
    Wu, Tsung-Shu Joseph
    Date
    2021-09-08
    Journal
    BMJ Paediatrics Open
    Publisher
    BMJ Publishing Group
    Type
    Article
    
    Metadata
    Show full item record
    See at
    https://doi.org/10.1136/bmjpo-2021-001097
    Abstract
    Introduction: Sickle cell disease (SCD) remains a major cause of childhood mortality and morbidity in Malawi. However, literature to comprehensively describe the disease in the paediatric population is lacking. Methods: A retrospective review of clinical files of children with SCD was conducted. Descriptive statistics were performed to summarise the data. χ2 or Fisher’s exact test was used to look for significant associations between predictor variables and outcome variables (case fatality and length of hospital stay). Predictor variables that were significantly associated with outcome variables (p≤0.05) in a χ2 or Fisher’s exact test were carried forward for analysis in a binary logistic regression. A multivariable binary logistic regression was used to identify covariates that independently predicted length of hospital stay. Results: There were 16 333 paediatric hospitalisations during the study period. Of these, 512 were patients with SCD representing 3.1% (95% CI: 2.9%- 3.4%). Sixty-eight of the 512 children (13.3%; 95% CI: 10.5% - 16.5%) were newly diagnosed cases. Of these, only 13.2% (95% CI: 6.2% - 23.6%) were diagnosed in infancy. Anaemia (94.1%), sepsis (79.5%) and painful crisis (54.3%) were the most recorded clinical features. The mean values of haematological parameters were as follows: haemoglobin (g/dL) 6.4 (SD=1.9), platelets (×109/L) 358.8 (SD=200.9) while median value for white cell count (×109/L) was 23.5 (IQR: 18.0–31.2). Case fatality was 1.4% (95% CI: 0.6% - 2.8%)and 15.2% (95% CI: 12.2% -18.6%) of the children had a prolonged hospital stay (>5 days). Patients with painful crisis were 1.7 (95% CI: 1.02 - 2.86) times more likely to have prolonged hospital stay than those without the complication. Conclusion: Anaemia, sepsis and painful crisis were the most common clinical features paediatric patients with SCD presented with. Patients with painful crisis were more likely to have prolonged hospital stay. Delayed diagnosis of SCD is a problem that needs immediate attention in this setting. Although somewhat encouraging, the relatively low in-hospital mortality among SCD children may under-report the true mortality from the disease considering community deaths and deaths occurring before SCD diagnosis is made.
    Rights/Terms
    © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.
    Keyword
    health services research
    nursing
    palliative care
    Identifier to cite or link to this item
    http://hdl.handle.net/10713/16742
    ae974a485f413a2113503eed53cd6c53
    10.1136/bmjpo-2021-001097
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