학술논문

Management of children with glucose‐6‐phosphate dehydrogenase deficiency presenting with acute haemolytic crisis during the SARs‐COV‐2 pandemic.
Document Type
Article
Source
Vox Sanguinis. Jan2022, Vol. 117 Issue 1, p80-86. 7p. 2 Charts, 3 Graphs.
Subject
*GLUCOSE-6-phosphate dehydrogenase deficiency
*COVID-19 pandemic
*GLUCOSE-6-phosphate dehydrogenase
*RED blood cell transfusion
*COVID-19 testing
Language
ISSN
0042-9007
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. [ABSTRACT FROM AUTHOR]