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. 2022 Jan;117(1):80-86.
doi: 10.1111/vox.13123. Epub 2021 Jun 8.

Management of children with glucose-6-phosphate dehydrogenase deficiency presenting with acute haemolytic crisis during the SARs-COV-2 pandemic

Mohsen Elalfy  1 , Amira Adly  1 , Khadiga Eltonbary  1 , Islam Elghamry  1 , Omar Elalfy  2 , Mohamed Maebid  3 , Khaled Elsayh  4 , Heba-T-Allah N Elsayed  5 , Magdy El Ekiaby  6
Affiliations

Management of children with glucose-6-phosphate dehydrogenase deficiency presenting with acute haemolytic crisis during the SARs-COV-2 pandemic

Mohsen Elalfy et al. Vox Sang. 2022 Jan.

Abstract

Background and objectives: Shortage of blood during the severe acute respiratory syndrome-COV-2 (SARs-COV-2) pandemic impacted transfusion practice. The primary aim of the study is to assess management of acute haemolytic crisis (AHC) in glucose-6-phosphate dehydrogenase(G6PD)- deficient children during SARs-COV-2 pandemic, and then to assess blood donation situation and the role of telemedicine in management.

Methods: Assessment of G6PD-deficient children attending the Emergency Department (ER) with AHC from 1 March 2020 for 5 months in comparison to same period in the previous 2 years, in three paediatric haematology centres. AHC cases presenting with infection were tested for SARs-COV-2 using RT-PCR. Children with Hb (50-65 g/L) and who were not transfused, were followed up using telemedicine with Hb re-checked in 24 h.

Results: A 45% drop in ER visits due to G6PD deficiency-related AHC during SARs-COV-2 pandemic in comparison to the previous 2 years was observed. 10% of patients presented with fever and all tested negative for COVID-19 by RT-PCR. 33% of patients had Hb < 50 g/L and were all transfused. 50% had Hb between 50 and 65 g/L, half of them (n = 49) did not receive transfusion and only two patients (4%) required transfusion upon follow up. A restrictive transfusion strategy was adopted and one of the reasons was a 39% drop in blood donation in participating centres.

Conclusion: Fewer G6PD-deficient children with AHC visited the ER during SARs-COV-2 and most tolerated lower Hb levels. Telemedicine was an efficient tool to support their families. A restrictive transfusion strategy was clear in this study.

Keywords: acute haemolytic crisis (AHC); glucose-6-phosphate dehydrogenase (G6PD); packed red blood cell transfusions (PRBCs); severe acute respiratory syndrome-COV-2 (SARs-COV-2).

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

FIGURE 1
FIGURE 1
G6PD‐deficient cases in AHC visits to ER in (a): Ain shams; (b) Assiut and (c) Beni Suef for 5 months period over 3 years, 2018–2020
FIGURE 2
FIGURE 2
Differences in transfusion rates between 2020 and previous 2 years
FIGURE 3
FIGURE 3
Number of donors in (a): Ain shams; (b) Assiut and (c) Beni Suef for 5 months period over 3 years, 2018–2020
See this image and copyright information in PMC

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