Background and Objective: G6PD (GLUCOSE -6- PHOSPHATE dehydrogenase) is the first enzyme of pentose PHOSPHATE pathway, which plays an important role in NADPH production. NADPH is necessary for preventing of cell oxidation. Deficiency of this enzyme led to hemolysis in some situations. This study has been done to determine the prevalence of G6PD deficiency and its relation with sex, blood group, Rh and familial history of new born in Rafsanjan.Materials and methods: In this cross sectional study blood sample was collected on a special filter paper and the activity of G6PD was measured by fluorescent spot test. The data were analyzed by Chi-square and Fisher exact test. Significance was defined as p<0.05.Results: There were 523 boys and 495 girls out of 1018 alive newborns. The G6PD deficiency was 5.7% for boys, 4.2% for girls and 5% for all the alive newborns. No significant relation, between prevalence of G6PD deficiency and blood group, sex, familial history and Rh were found.Conclusion: The G6PD deficiency prevalence in Rafsanjan is less than other parts of Iran and the worlds (10%-14.9%). Given the screening test is cheep and easy it is suggested to perform this test at birth to differentiate the relatively or completely deficient sample to prevent of health problems.

GLUCOSE-6-PHOSPHATE dehydrogenase (G6PD) Deficiency is the most prevalent enzymopathy in mankind. It has sex-linked inheritance. This enzyme exists in all cells. G6PD deficiency increases the sensitivity of red blood cells to oxidative damage. G6PD deficiency was discovered in 1950 when some people suffered hemolytic anemia as a result of taking antimalarial drugs (primaquin). Most people with G6PD deficiency do not have any symptoms, till they are exposed to certain medications, Fava beans and infections; and then their red blood cells are hemolyzed. The degree of hemolysis changes according to the degree of enzyme deficiency and the oxidant agent exposure. G6PD deficiency has many different variants and Mediterranean variant is the most common mutation in the world. G6PD deficiency is considered a health problem worldwide, especially in Asia, Middle East and Mediterranean countries. In this article, we have reviewed the importance and function of G6PD enzyme, incidence rate of G6PD deficiency in the world and Iran, genetic and variants of this enzyme, clinical manifestation, diagnosis and treatment of the enzyme deficiency.

Background: Jaundice is affecting over 60-80 percent of neonates in the first week of life. GLUCOSE-6-PHOSPHATE dehydrogenase (G6PD) deficiency, which is an important cause of pathologic hyperbilirubinemia, can lead to hemolytic anemia, jaundice and kernicterus. The present study was performed to determine the prevalence of G6PD deficiency among icteric neonates in Shirvan, Iran.Methods: This descriptive, analytical study was performed by evaluating the medical records of neonates with jaundice, admitted to the neonatal ward of Imam Khomeini Hospital of Shirvan in 2012-2013. All neonates, who were evaluated in terms of G6PD, were included in this study. Data including the clinical signs and symptoms, laboratory test results and maternal history during pregnancy were recorded in the questionnaires. The patients were divided into two groups: with and without G6PD deficiency. The recorded data were compared between the two groups, using t-test and Chi-square test. P-value less than 0.05 was considered statistically significant.Results: Among 452 admitted neonates, 16 (3.5%) presented with G6PD deficiency. There was no significant difference between the two groups in terms of birth weight, weight on admission, Coombs’ test results, hematocrit level, length of hospital stay and total bilirubin level. However, there was a significant difference between the two groups regarding reticulocyte count.Conclusion: Based on the findings, establishment of an early G6PD screening program, which can prevent further complications in neonates, seems essential, particularly in countries such as Iran where G6PD deficiency is highly prevalent.

Background: GLUCOSE 6-PHOSPHATE dehydrogenase (G6PD), the first enzyme in initiating the pentose PHOSPHATE shunt, is an important component in generation of NADPH. Although innumerable studies have been performed on human erythrocyte G6PD, however, the effect of trace elements on the enzyme activity requires further investigations. Objective: To study the effect of aluminum on human erythrocyte G6PD. Methods: In this experimental research, following the purification of G6PD using chromatographic methods, the effect of different concentrations of Al3+(up to 100 micro-molar) on G6PD activity was studied. The enzyme activity was measured at different concentrations of GLUCOSE 6-PHOSPHATE and NADP+ to determine the type of inhibitory action. Findings: Aluminum at the concentration of 100 µM showed a considerable inhibitory effect on G6PD activity (60%). The type of inhibitory action, depending on the use of GLUCOSE-6-PHOSPHATE or NADP+, was competitive and noncompetitive, respectively. Conclusion: Aluminum exerts an inhibitory action on human erythrocyte G6PD activity.

G6PD deficiency is the most common enzyme deficiency in human beings-It can lead to severe hyperbilirubinemia which my progress to bilirubin encephalopathy, mental retardation and death in newborn infant.This cross sectional analysis was carried out in newborn services of Amirkola children hospital. In this study 165 icteric neonates (cases) and 123 nonicteric neonates were assessed. Enzyme activity were assesed by fluorescent spot test and the results were analysed by chi-square and fisher exact tests and a p value<0.05 was considered significant.G6PD deficiency was seen in 23.6% of the icteric and 11.6% of the nonicteric newborns (P <0.0001).36% of G6PD deficient and 18.2% of non-deficient neonates required exchange transfusion (P<0.0010).In view of the higher frequency of G6PD deficiency and the greater need for exchange transfusion in these patients, we suggest G6PD screening on cord blood at birth for all neonates, and G6PD level determination in all pathological jaundice of newborn.

Background: GLUCOSE-6-PHOSPHATE dehydrogenase (G6PD) deficiency can cause hemolytic anemia and neonatal jaundice. Screening of newborns by examining the cord blood for enzyme activity has been proposed for prevention of its complications. The aim of the present study was to determine the incidence of G6PD deficiency in newborns in Vali-e-Asr hospital, Tehran. The relation between G6PD deficiency and the variables of sex, gestational age, jaundice, hemolysis, and anemia was also examined.Methods: 450 newborns during a 9-month period were examined for G6PD activity by Fluorescent Spot Test of specimens of cord blood and for other variables.Findings: 9 neonates (8 males and 1 female) were G6PS deficient. The incidence of G6PD deficiency was 2% (3.3% for boys and 0.5% for girls). Six (66.7%) cases of G6PD deficiency developed jaundice. None of the G6PD-deficient neonates developed anemia. Only one case had a high cord blood reticulocyte count. G6PD deficiency was correlated with sex and neonatal jaundice. There was no correlation between G6PD deficiency and gestational age, hematocrit and reticulocyte count of the cord blood. Conclusion: The incidence of G6PD deficiency in newborns in Vali-e-Asr hospital in Tehran was 2% Screening of the male newborns for enzyme deficiency by examining the cord blood is suggested.

Some kinetic properties of NAD+- and NADP+- dependent GLUCOSE 6-PHOSPHATE dehydrogenase (G6PD) purified from streptomyces aureofaciens were studied. Both NADH and NADPH inhibited the enzyme competitively and noncompetitively, with respect to the corresponding coenzymes and GLUCOSE 6-PHOSPHATE, respectively. ATP inhibited the NAD+ - linked reaction but not that of the NADP+- linked activity. The inhibition was competitive with respect to NAD+ and noncompetitive with respect to GLUCOSE 6-PHOSPHATE. Km values were 0.14 mM for NAD+ and 0.075 mM for NADP+. Similar Km values (0.75-0.79 mM) were obtaind for GLUCOSE 6-PHOSPHATE using either NAD+ or NADP+ as a coenzyme. The optimum pH was 6.6 for NAD+- and 7.4 for NADP+- dependent activity. Maximum protein fluorescence was increased by NAD+ (49%) and NADP+ (8%). Among bivalent cations studied, Cu2+ decreased NAD+- linked activity (40%), but increased the NADP+- linked reaction (10%). Ni2+ did not affect NAD+- linked, but stimulated NADP+- linked activity. Other cations such as Zn2+ and Mn2+ also differently affected the two reactions. The data suggested that binding of NAD+ and NADP+ produces a different conformational change in S. aureofaciens G6PD or an isomerisation process regulates coenzyme utilization.

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.

Background: Studies have shown that G6PD deficiency results in indirect hyperbilirubinemia in newborns. Objective: Determining the relationship between G6PD deficiency and neonatal hyperbilirubinemia. Methods: Through a case-control study, 200 neonates with indirect hyperbilirubinemia were equally divided into two case and control groups and examined for G6PD deficiency using a commercial G6PD kit and a fluorometric analysis. The data were further analyzed statistically. Findings: Results showed that out of 200 neonates, 24 had G6PD deficiency (10 in case group and 14 in control group). There was no statistically significant difference between two groups. Conclusion: Since the prevalence of G6PD deficiency among nonicteric group (control group) was higher than the icteric group (case group), it seems that the performance of a screening test to measure the G6PD activity in all neonates to be useful.