Comparison of quantitative and qualitative tests for glucose-6-phosphate dehydrogenase deficiency in the neonatal period.

INTRODUCTION: Considering the high prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency among newborns, different screening methods have been established in various countries. In this study, we aimed to assess the prevalence of G6PD deficiency among newborns in Rasht, Iran, and compare G6PD activity in cord blood samples, using quantitative and qualitative tests. METHODS: This cross-sectional, prospective study was performed at five largest hospitals in Rasht, Guilan Province, Iran. The screening tests were performed for all the newborns, referred to these hospitals. Specimens were characterized in terms of G6PD activity under ultraviolet light, using the kinetic method and the qualitative fluorescent spot test (FST). We also determined the sensitivity, specificity, negative predictive value, and positive predictive value of the qualitative assay. RESULTS: Blood samples were collected from 1474 newborns. Overall, 757 (51.4%) subjects were male. As the findings revealed, 1376 (93.4%) newborns showed normal G6PD activity, while 98 (6.6%) had G6PD deficiency. There was a significant difference in the mean G6PD level between males and females (P = 0.0001). Also, a significant relationship was detected between FST results and the mean values obtained in the quantitative test (P < 0.0001). CONCLUSION: According to the present study, FST showed acceptable sensitivity and specificity for G6PD activity, although it appeared inefficient for diagnostic purposes in some cases.

Association between height and malignancy among children in the north of Iran.

BACKGROUND: This study aim to determine the association between height and cancer in the children aged 14 years at the time of diagnosis in Rasht, Iran. MATERIALS AND METHODS: In this cross-sectional study, height of patients with a malignancy (≤14) at the time of diagnosis measured in the standard charts of United States National Center for the Health Statistics (NCHS). Data were reported by descriptive statistics and analyzed by Regression tests in SPSS version 19. RESULTS: Overall, 78 male (38.6%) and 124 female (61.4%) patients with various kinds of malignancies were evaluated for their heights. Leukemia was the most common type of cancer. The median height of the patients was more than 20(th) percentile and under 50(th) percentile of the NCHS. No significant association was found between height and leukemia. CONCLUSION: Previously, the median height of Iranian girls and boys (≤15) reported under 20(th) percentile of the NCHS. In this study, the median height of the patients at the time of diagnosis was more than 20(th) percentile of the NCHS. There was a correlation between height and cancer among our patients, although, this correlation can be assessed by further cohort study.

Medical expenses of patients with Favism admitted to 17th Shahrivar Hospital compared to G6PD enzyme screening cost, in north of Iran.

BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) enzyme deficiency is one of the prevalent disorders in Guilan province, northern Iran, causing many patients to suffer from acute hemolysis. This disease has imposed tremendous costs both on patients and Health systems. The aim of this study was to compare the direct costs of favism treatment on patients and health system with G6PD enzyme screening test. MATERIALS AND METHODS: In this descriptive prospective study, the medical and hospital costs of acute hemolysis due to G6PD deficiency were calculated and compared with the expenses of screening newly born infants for this disorder in Rasht. Data was collected by a questionnaire.Student's t-Test and chi-squared test were recruited and data was analyzed using SPSS ver. 20. RESULTS: In this study, 101 hospitalized patients with favism (72 male and 29 female) admitted from October 2011 to the end of September 2012 were included. The average cost of treatment for these patients was approximately 726000000Rials (for each patient 7190000 Rials), which was about half of the cost of screening for all newborn infants in Rasht during this period. CONCLUSION: The cost of G6PD enzyme screening in Rasht is substantially lower than the cost of treating hospitalized patients with Favism.

Efficacy and safety of Iranian made Deferasirox (Osveral®) in Iranian major thalassemic patients with transfusional iron overload: A one year prospective multicentric open-label non-comparative study.

PURPOSE OF THE STUDY: to determine the efficacy, adverse effects and safety of a new Iranian generic product of deferasirox (Osveral®) in Iranian transfusion dependent major thalassemic (TD-MT) patients. METHODS: In 9 main thalassemia treatment centers, all of TD-MT patients (aged ≥2 yrs) with serum ferritin (SF) levels≥1000 ng/ml, or >100 ml/kg of RBC transfusion,who could not tolerate parental iron chelating were recruited regardless of their previous iron chelation therapy. Periodical clinical and laboratory evaluations were conducted for adverse effects (AEs). Primary efficacy end point was Mean of Relative Change of Serum Ferritin (MRC-SF) from the baseline level during one year. Analysis of variance (ANOVA), t test, chi-square or Fisher exact test were used for statistic analysis appropriately (P values <0.05 were considered as statistical significant). RESULTS: In 407 cases the male/female ratio was 0.98. Mean age was 11.5±7.4 (2-58) years. The mean of initiating dose of Osveral® and mean usage dose during the study was 23.5±4.9 mg/kg and 24.9±4.9 mg/kg respectively. MRC-SF was -11.44% ±38.92 and it showed significant decline in SF (P value<0.001) one hundred and forty eight patients out of 407 patients experienced at least one. AE, the most common of them were transient increase in serum creatinin (97;24.1%) and>5 time increase in transaminases (24;5.89%).The causes of discontinuation of treatment were non-satisfactory treatment ( 24; 5.8%), poor or non-compliance of patients (21;5.1%), and adverse effects (13; 3.1%) CONCLUSION: A detailed comparison with similar studies on deferasirox (Exjade®) shows a promising efficacy and safety for its Iranian generic product (Osveral ®).