Abstract
Introduction: Glucose-6-phosphate dehydrogenase deficiency (G6PD) or fauvism is the most common enzyme deficiency in human, so that 400 million people are living with this disease worldwide. This study aimed to investigate the role of some neonatal factors among newborns suffering from G6PD deficiency and neonatal outcomes associated with this disease. Materials and methods: In this study, two methods including case-control and retrospective cohort regarding some neonatal factors associated with G6PD deficiency were used. These methods were performed on 142 children with this kind of deficiency and 142 healthy infants in the city of Marvdasht during 2013- 2014. The analysis of data was based on chi-square tests, t-test, logistic regression, descriptive statistics and estimation of odds ratios or relative risks via SPSS16 software. Results: Totally 284 newborns including 132 (46.6%)/ 152 (53.4%) boys/girls and mean weight on birth of 3163 ± 471 (gr) were analyzed. Comparison of case and control samples did not show any significant differences between sex and involving with G6PD deficiency but the chance of having a baby with this defect in pregnancy intervals between 6 to 8 years was increased (95% CI: 1- 4.4, OR: 2). Relative risk of jaundice in infected and healthy infants was estimated as 3.73, which demonstrated a statistically significant association (95% CI: 1.33- 10.4). The frequency of low birth weight, birth order and type of delivery was associated with the disease, but their differences were insignificant compared to the healthy group. Conclusion: The results of this study showed that the number of hospitalization is increased due to jaundice in infants with G6PD. There is also an insignificant relation between low birth weight, rank of birth and type of delivery. Thus, it is recommended that other consequences of this deficiency need to be revealed by screening other populations with more samples.
Highlights
Glucose-6-phosphate dehydrogenase deficiency (G6PD) or fauvism is the most common enzyme deficiency in human, so that 400 million people are living with this disease worldwide
This study aimed to investigate some neonatal factors associated with G6PD deficiency and neonatal outcomes among involved individuals
The results showed that the annual incidence of disease among girls was more than boys, but gender difference was not statistically significant (P=0.6, OR: 0.88). 16.8 percent of infants in the case group and 8.5 percent of infants in the control group showed a pregnancy distance of 6-8 years with significant differences, so that chances of having a baby with G6PD in pregnancy with distance of 6 to 8 years was 2 times more than healthy babies (P=0.08, OR: 2)
Summary
Glucose-6-phosphate dehydrogenase deficiency (G6PD) or fauvism is the most common enzyme deficiency in human, so that 400 million people are living with this disease worldwide. G6PD enzyme-linked recessive gene has an inheritance pattern which is located on the X chromosome and often males are ill and females are carriers of the defect [2] This defect increases the susceptibility of red blood cells to oxidant agents such as oxidants present in raw beans, some medications and oxidative stress caused by infections [3] and in the case of facing with oxidative agents, clinical realizations of G6PD including acute hemolytic anemia, chronic hemolytic anemia and hyperbilirubinemia in newborns will appear and in severe cases it can lead to kernicterus.
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