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

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.

Study of ß-thalassemia biomarkers and their relationship to cognition among children.

The current study is to detect some biomarkers of beta-thalassemia (ferritin, serum transferrin receptors, and nitric oxide levels) and to examine the relation between these markers and cognition in children with beta-thalassemia. Thirty children with beta-thalassemia were selected from the Pediatric Department at Assiut University hospital. Another 40 healthy children of the same age, sex, years of schooling, body mass index (BMI), and social scale were chosen as the control group. Assessment of clinical, laboratory, cognitive functions, and event related potential was done for patients and control groups. Significantly higher levels of ferritin and serum transferrin receptors with decreased nitric oxide were detected among children with beta-thalassemia. There were significant correlations between serum transferrin receptors and nitric oxide levels with event related potential latencies and with some cognitive function tests and P300-N2 amplitude. Frequent blood transfusion was associated with increased serum transferrin receptors and decreased nitric oxide levels.