Seizure following acute hemolysis caused by Glucose-6-phosphate dehydrogenase Deficiency

Abstract

Background: Storage of platelet concentrates (PCs) at room temperature (20-24°C) limits its storage time to 5 Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common inherited enzyme deficiency of the human red blood cells . Most of G6PD deficient individuals are asymptomatic, but acute hemolytic anemia may be presented with nausea, vomiting, abdominal pain, headache, jaundice, pallor, discoloration of the urine, chills, and fever. Seizure is reported as a rare symptom, as well. The present study aimed to investigate seizure following acute hemolysis caused by Glucose-6-phosphate dehydrogenase deficiency. Material and Methods: This analytic cross-sectional study was conducted on all consecutive patients aged 1-12 years with G6PD deficiency hospitalized for hemolysis in 17 Shahrivar children hospital, Rasht, Iran, in 2016. Demographic characteristics and other variables such as place of inhabitants, type of drinking water, history of seizure in the patients and family, cause of hemolysis, hemoglobin level and hemoglobinuria on admission, and infection history prior to hemolysis were recorded. Data were analyzed by Mann-Whitney U test and Fischer Exact Test. P-value < 0.05 indicated statistical significance and data were assessed by SPSS (version 20). Results: The youngest patient was one year old and the oldest was 11 years old. Most of them were males (68.9%). Out of 244 patients, 8 ones (3.3%) experienced seizure. There was a significant correlation between seizure occurrence and family history of seizure (p=0.03) as well as fava bean consumption (p=0.019) as the causes of hemolysis; but not with infection as the cause of hemolysis, hemoglobin or hemoglobinuria level on admission, types of drinking water, place of living, and gender. Methemoglobinemia was considered as the main cause of the seizure. Conclusion: Although the rate of seizure was not so high (3.3%), it seems that seizure can be a critical and potentially life-threatening complication in these patients. Environmental factors may also play a role in the pathogenesis of the seizure in these patients.

The potential role of vitamin E in patients with glucose-6-phosphate dehydrogenase deficiency: A systematic review and meta-analysis

“…Of them, 6 studies were included in the meta-analysis. [25,[33][34][35][36][37] The references of the included studies were manually searched, and no further articles were included. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses flow diagram of the study selection process is shown in Figure 1.…”

“…[38] In our review, most of the included studies in the analysis investigated the efficacy of VitE on patients with chronic hemolysis [25,33,34,36,37] and only 1 study investigated the efficacy of acute hemolysis. [35]…”

“…This work expands the literature by providing evidence on the effects of VitE supplementation in patients with G6PD deficiency. The significance of this paper is not only to summarize the evidence of the use of Darbandi 2017 [35] The Hafez 1986 [36] Improved Johnson 1983 [37] High-dose vitamin E does not decrease the rate of chronic hemolysis in glucose- VitE in G6PD deficiency patients but also to evaluate the theory and open the door in front of future research about the use of any antioxidants in patients with G6PD deficiency.…”

“…[38] In our review, most of the included studies in the analysis investigated the efficacy of VitE on patients with chronic hemolysis [25,33,34,36,37] and only 1 study investigated the efficacy of acute hemolysis. [35] Table 2 Baseline characteristics of the included studies. The formation of NADPH from the NADP + in the pentose phosphate pathway protects the cells from oxidative stress, as it helps in the regeneration of the reduced GSH.…”