Objectives Glucose-6-phosphate dehydrogenase deficiency (G6PDD) has remained ignored despite being a common red blood cell enzyme defect. The warnings concerning drugs such as hydroxychloroquine in this population are often criticised, and progress in pretreatment screening for this deficiency is lacking, even for drugs widely acknowledged as harmful. Further negligence was observed during the Covid 19 pandemic. This systematic review addressed the screening practices for this deficiency by examining the epidemiology of hydroxychloroquine and other related medications usage before and during the COVID-19 pandemic in G6PDD individuals. Methods EMBASE, MEDLINE, SCOPUS, Global Health, Web of Science, Journal@OVID and Imperial@OVID, and citation searching were used to obtain relevant evidence. The search terms used were developed around 'COVID-19', 'G6PD deficiency', 'hemoglobinopathies', 'hydroxychloroquine' and 'screening'. The search was open-ended till the end of July 2022. Reference management and sorting were conducted using Mendeley. Quality appraisal was done using Joanna Briggs Institute critical appraisal tools. Results 20 (6.6%) of 304 studies retrieved were eligible for narrative synthesis, of which 15 were case reports, three cross-sectional studies, one case-control study, and one case series. 18 (90%) of the 20 papers were appraised as good, and the rest as fair. 14 (93%) case reports involved male patients with ethnicities reported as Afro-Carribean/Asian. One case report also mentioned a first-time diagnosis of another coexisting hemoglobinopathy. 9(60%) case reports noted screening for G6PDd after initiating HCQ or related drugs and 4(27%) reported pretreatment screening. Two (50%) of the four case reports with pretreatment screening mentioned treatment initiation with HCQ and related drugs before receiving the screening results. One (25%) administered it after receiving a false negative result. Two (22%) out of nine case reports with post- medication screening included patients who were earlier treated with HCQ and related drugs on an outpatient basis before the documented presentation. All nine (100%) case reports that considered post-treatment screening involved haemolysis, with four (44%) also reporting methemoglobinemia. 10 (67%) case reports mentioned the final treatment outcome, including two mortality cases, each being patients who underwent G6PDd screening post-initiation of treatment with HCQ and related drugs. The case series, cross-sectional and case-control studies did not detail the pre and post-screening for G6PDD when administering HCQ and associated medications. Urban dwellers were more likely to screen for G6PDD. Conclusion Screening practices for G6PDD during COVID (and in general) were poor and often considered after the development of post-treatment complications. Studies arguing against screening had significant limitations, e.g. restricting screening of G6PDD to at-risk populations. However, given technological advances and globalisation, G6PDD screening and awareness are essential for adequate and safe treatment to be administered to G6PDD individuals who may present to A&E/admission wards.
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Ikpeme et al. (2024) studied this question.
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