Background: Glucose-6 phosphate dehydrogenase (G6PD) deficiency is the most common inherited enzyme deficiency of red blood cells involving the enzyme pathway of hexose monophosphate. This study was conducted to examine the effect of vitamin E and folic acid on the improvement of acute hemolysis caused by the G6PD deficiency in patients referred to 17 Shahrivar Hospital, Rasht.
Materials and Methods: This was a randomized clinical trial conducted on 120 patients with G6PD deficiency. The patients were divided in 4 groups, including vitamin E, folic acid, a combination of both supplements, and control groups. The hemoglobin level and the reticulocyte count of patients during hospitalization, at discharge and two weeks after discharge were evaluated. All patients received standard treatment for acute hemolysis.
Results: Mean age of the patients was 44.19± 16.43 months. There was no significant difference between 4 groups in terms of age, gender, and etiology of hemolysis (p>0.05). The consumption of fava bean was the main cause of hemolysis in 95% of patients. The level of hemoglobin and hematocrit, and reticulocyte count during hospitalization and discharge among groups showed no significant difference (p>0.05). However, a significant increase was observed in the level of hemoglobin and hematocrit and reduction of reticulocyte count in patients receiving supplements compared with control group (p=0.001).
Conclusion: Considering the low cost, availability, and safety profile of theses supplements, it seems that usage of folic acid and vitamin E can be highly recommended during favism.

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: Osteoporosis is one of the main causes of morbidity in patients with thalassemia major. Osteoprotegerin (OPG) is secreted by osteoblasts and osteogenic stromal stem cells and protects the skeleton from excessive bone reabsorption. In this study, the authors aimed to assess the relationship between OPG with osteoporosis and osteopenia in patients with thalassemia major.
Materials and Methods: In this analytic cross-sectional study, 37 patients aged 8-18 years, with thalassemia major were enrolled. Biochemical markers including hemoglobin, ferritin, calcium, phosphorus levels, and MRI T2* heart and liver were assessed. A bone mineral densitometry (BMD) was performed as well. Statistical analysis was performed by the independent T-test and Chi-Square test using the SPSS 20. The Multiple linear regression analysis was used to investigate the association between the BMD Z-score and OPG by the effect modification.
Results: The mean age of patients was 14.86±3.72 years. Normal bone density, osteopenia, and osteoporosis were noted in 2 (5.4%), 21 (56.8%), and 14 (37.08%) patients, respectively. The number of girls (P=0.042), mean age (P=0.045), and MRI T2* heart (P=0.033) in patients with osteopenia was significantly higher than patients with osteoporosis. The BMD Z-score was not significantly associated with OPG regarding the total number of participants, whereas in patients with osteoporosis, this association was significant (P=0.001). In all effect modified models, BMD remained statistically non-significant except for body mass index modification (P=0.046).
Conclusion: Based on the results, it seems that further complicated studies are needed to be performed on this issue.

Background: In pediatric care settings, intravenous cannulation (IVC) is usually needed for diverse purposes. Considering the painfulness and invasiveness of sampling by direct venipuncture (DVP), using a painless and less invasive method would be promising. Therefore, this study aimed to compare the effect of substitution of routine DVP with direct blood sampling through IVC on the accuracy of hematologic results.
Materials and Methods: This was a cross-sectional study conducted on 5-14-year-old children admitted to the emergency ward of 17th Shahrivar Pediatric Hospital in Rasht, north of Iran. After discarding only one ml of blood, paired-samples were taken from IVC and DVP and analyzed for 30 most frequently requested electrolytes, hematologic, and blood gas tests. The similarity of the obtained results by the two methods indicated the probability of substituting DVP with IVC and was defined by the absence of significant statistical difference (P>0.05).
Results: The comparison between the mean of hematologic factors by two methods showed significant similarity between groups regarding all parameters (P>0.05) except the mean of red blood cell count in the two groups (P<0.05). Assessing the level of electrolytes by two collection methods showed that there was a significant similarity between the mean of all parameters (P>0.05) except for phosphorus (P=.002). Furthermore, assessing the level of electrolytes showed a significant similarity between the potential of hydrogen, partial pressure of carbon dioxide, bicarbonate, and buffer base in the two groups (P>0.05). However, there was a significant difference between partial pressure of oxygen, base excess, and O2 saturation in the two collection methods (P<0.05).
Conclusion: Based on the promising results obtained in this study, it seems that these methods could be interchangeably used, and IVC can be an alternative method for DVP by discarding the minimum amount of blood and less invasiveness in children.

Background: This study aimed to assess cutoff of ferritin for evaluation of osteoporosis in patients with Thalassemia Major (TM).
Materials and Methods: This analytic cross-sectional study was conducted in17 Shahrivar children's hospital, Rasht, Iran, from November 2017 to November 2018. The inclusion criteria were indicated as the presence of  TM in patients aged 12-19 years old with records of their regular visits. The exclusion criteria were noted as the presence of any chronic bone diseases such as osteomalacia or osteogenesis imperfecta, delayed puberty, hypothyroidism, parathyroid dysfunction, renal failure, liver failure, and growth hormone deficiency. Ferritin level was assessed, and bone densitometry was performed for all patients with TM.
Results: In this study, 53 females (54.6%) and 44 males (43.4%) were enrolled. Results showed that 36 (37.1%), 49 (50.5%), and 12 (12.4%) patients had a normal bone density, osteopenia, and osteoporosis, respectively. Comparing these three groups showed that despite higher mean serum level of ferritin in TM patients with osteoporosis than patients with osteopenia and normal bone density, no significant statistical difference was noted in these three groups (P >0.05). Besides, the mean ferritin level in patients with abnormal bone densitometry (osteopenia and osteoporosis) was higher than in patients with normal ones. A significant difference was noted between abnormal and normal densitometries (p=0.03). The Area under the Curve for ferritin was 0.708, and the cutoff point was indicated for ferritin was 2006 ng/ml.
Conclusion:  Regarding the results, there was a high frequency of osteoporosis and osteopenia in teenagers with TM. As bone density abnormality formation is time-consuming, and prevention is the primary strategy for management, it is highly recommended to assess bone mineral density regularly starting from early childhood.

Background: Atherosclerosis is an important cardiovascular disorder in beta-thalassemia major patients. The present study aimed to predict factors of atherosclerosis in children with beta-thalassemia major.
Materials and Methods: This cross-sectional study was conducted on 36 patients with beta-thalassemia major and 36 healthy children as the control group. The carotid intima-media thickness (CAIMT) and osteoprotegerin (OPG) were compared between groups.
Results: The mean age of the patients in the case and control groups were 13.5 ± 3.7 and 13 ± 3.1 years old, respectively. Significant differences in CAIMT in the right (P = 0.001) and left (P = 0.001) arteries were recorded between the two groups. The mean serum OPG in the beta-thalassemia group was significantly higher than the control group (3.9 ng/ml and 1.4 ng/ml, respectively, P=0.001).
Conclusion: CAIMT is a non-invasive method for diagnosing atherosclerosis. According to the significant difference between groups, serum OPG could be helpful in the diagnosis of early atherosclerosis in beta-thalassemia major.