Document Type : Original Article(s)
Authors
1
Associate Professor, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran
2
Medical Student, Department of Pediatrics, Isfahan University of Medical Sciences, Isfahan, Iran
3
PhD, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran
4
Assistant Professor, Department of Pediatrics, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran
10.48305/jims.v43.i838.1455
Abstract
Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most common enzymopathies and an important cause of hemolysis and neonatal jaundice. In this regard, the study aimed to determine the risk factors and clinical manifestations of patients with G6PD deficiency hospitalized at Amin Hospital, Isfahan, in 2018 and 2019.
Methods: In this retrospective cross-sectional study, 73 children (1 to 15 years old) with a diagnosis of G6PD deficiency and evidence of acute hemolysis were examined. Demographic and clinical data (such as jaundice, pallor, dark urine, lethargy, history of fava bean consumption, stimulant drugs, and infection) and laboratory data (including hemoglobin levels, serum total bilirubin, reticulocyte count, and direct Coombs test results) were extracted from the patients' medical records. The need for blood exchange was also recorded. Statistical analysis was performed using Mann-Whitney and Wilcoxon tests.
Findings: Of the 73 patients studied, 59 (80.8%) were male and 14 (19.2%) were female. The mean (±SD) total bilirubin level was 11.14 ± 1.8 mg/dL and the mean hemoglobin was 10.14 ± 0.53 g/dL. In total, 21 patients (28.8%) required blood exchange. Comparison of hemolysis parameters between 2018 and 2019 indicated a statistically significant difference in hemolysis rate (based on reticulocyte count) (P < 0.0001).
Conclusion: Given the significant increase in the hemolysis rate and the high prevalence of this deficiency, early diagnosis and screening for G6PD deficiency, regardless of gender, and careful monitoring of affected children can play a major role in preventing severe complications such as acute hemolysis and reducing the need for costly interventions like blood exchange.
Highlights
Motahar Heidari‐Beni: Google Scholar
Mohammad Ali Pourmirzaiee: Google Scholar
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