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Abstract. Objective: To evaluate the age at menarche and menstrual characteristics in patients with transfusion-dependent thalassemia (TDT) who started early chelation therapy (≤ 3 years) with a variety of chelating agents. Design: A retrospective multicenter study promoted by International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A). Setting: Eight of 13 International Thalassemia Centers (61.5%) in the ICET-A Network participated. Patients: Fifty-seven female TDT patients, aged 11 to 26 years, were enrolled in the study. Seven patients were excluded, 4 who were still prepubertal (age 12-14 years) and 3 with primary amenorrhea. The remaining 50 patients were from Iran (33 patients), 9 from Bulgaria, 8 from Greece, 4 from Oman, 2 from Cyprus, and 1 from Italy. Results: At start of chelation therapy, 22 patients received desferrioxamine mesylate (DFO), 26 deferasirox (DFX) and 2 deferiprone (DFP). All fifty TDT patients developed spontaneous menarche at a mean age of 14.2 ± 2.24 years (range 9 – 20). A significant positive correlation was observed between age at menarche and serum ferritin (SF) levels (r: 0. 41, P: 0.005). Thirty-two patients (64%) reported regular menstrual cycles, 7 (14 %) oligomenorrhea, 3 (6%) short/light menses (hypomenorrhea), and 8 (16%) secondary amenorrhea (SA) (16%). Conclusions: Early chelation does not necessary correlate with efficient chelation during pubertal age. Delayed menarche, related to high SF levels, was still frequent in most Centers and was a forerunner of irregular menstrual cycles, SA and associated complications. Neglecting the importance of adherence to iron chelation therapy (ICT), despite innovative and expensive therapies, may lead to complications and decreased quality of life. Key words: Transfusion-dependent thalassemia, menarche, menstrual cycles, iron chelation therapy (ICT), iron overload, adherence to treatment.

Background and Aims: Asthma is a chronic inflammatory pulmonary disease which affects 10%–20% of children and adolescents. Inhaled corticosteroids (ICS) is one of its most effective therapies. The effect of systemic corticosteroids on decreasing bone mineral density (BMD) was investigated and proved in children; however, the influence of ICSs on bone density has still remained unclear. This study evaluates the bone mineral density of children and adolescents with asthma in southern Iran and the associated factors, for example, amount of used inhaled steroid. Method: This case‐control study enrolled 41 children and adolescents (aged 8–18 years) with asthma and their age and gender‐matched controls in 2019–2020. Serum Calcium, phosphate, vitamin D, and bone mineral density were measured. Their physical activity, sun exposure, and fracture history were evaluated subjectively. Results: Lumbar BMD and BMD Z‐score in patients showed no significant difference with controls (p = 0.23, p = 0.73). Also, it showed that there was no significant difference in biochemical studies, growth, and bone densitometry parameters between patients who used ICSs for less than 3 months/year corticosteroid therapy compared to those with equal or more than 3 months/year usage. Prevalence of vitamin D deficiency was 28% and 8% in the controls and patients, respectively (p = 0.005). Conclusion: The present study showed that 9.46% of children and adolescents with asthma had low bone mass for chronological age, and it is not significantly higher than normal population. Dosage of inhaled steroid did not associate with osteoporosis in these patients. Prevalence of vitamin D deficiency in patients was lower than normal population, probably due to receiving vitamin D in their routine follow‐ups. [ABSTRACT FROM AUTHOR]

Background and Objectives: The cause of hyperbilirubinemia cannot be found in about 45% of cases of neonatal jaundice. Gilbert syndrome (GS) is the most common congenital disease associated with bilirubin metabolism in the liver. Since the screening value of genetic tests cannot be fully determined until accurate data on the prevalence and penetrance of the GS genotype are known, we sought to estimate whether the prevalence of GS is higher in the parents of neonates with severe unexplained indirect hyperbilirubinemia. Design and Setting: Case-control study of parents of neonates with severe unexplained indirect hyperbilirubinemia admitted to a neonatal ward. Methods: We used the rifampin test (checked bilirubin before and 4 hours after administration of 600 mg rifampin) for diagnosis of GS in parents of 115 neonates with severe unexplained indirect hyperbilirubinemia. We compared the prevalence of GS in these parents with that of a control group of 115 couples referred for premarital counseling. Results: The 115 neonates were aged 5.2 (1.6) days (mean, standard deviation), all were breast-fed, and males constituted 56.5%. Mean total serum bilirubin (TSB) level was 20.96 (5.48) mg/dL. 14.8% were glucose 6 phosphate dehydrogenase (G6PD) deficiency was present in 14.8%, and 10.4% had A, B or O blood group (ABO) incompatibilities with their mothers. There was no difference in the prevalence of GS between parents of the group with hyperbilirubinemia (22.2%) and the control group (19.13%) (P=.42). Mean TSB in neonates with parents who had GS was more (about 3 mg/dL) than in neonates with normal parents (P=.004). Fathers had GS twice as often as the mothers among the parents of neonates with hyperbilirubinemia (P=.003), among the control group (P=.009) and among neonates (P=.014). Conclusion: This study showed that GS cannot cause severe indirect hyperbilirubinemia by itself, but it may have a summative effect on rising bilirubin when combined with other factors, for example, G6PD. Our results showed that in GS, males are affected about twice as much as the females.

Background: The role of phosphate hemostasis in development of thalassemia bone disease has not been extensively studied yet. Due to the lack of sufficient human studies about the changes of serum Fibroblast growth factor-23(FGF23) in patients with beta-thalassemia major as the first step of investigating the role of FGF23 in thalassemia bone disease, the present study aimed to investigate the serum level of FGF23 in patients with thalassemia major. Material and Method: In this case-control study, 25 patients with beta thalassemia major and their age- and sex-matched healthy volunteers were enrolled. Serum phosphorous, calcium, parathyroid hormone (PTH), 25(OH) D, erythropoietin (EPO), serum intact FGF23 (iFGF23) and 1,25 (OH)2 D were checked and analyzed. Result: Patients with beta-thalassemia major had lower 1,25 (OH)2D, (p = 0.025), higher phosphate (p = 0.002), and higher PTH (P <0.001) compared to the control group; however, all of them were in their normal blood range. They also had higher serum FGF23 (p = 0.007) and higher EPO (P<0.001). Serum FGF23 had an independent association with serum Iron (p=0.016), 1, 25(OH)2 Vitamin D (p<0.001), and hemoglobin (p=0.002). Conclusion: Serum FGF23 was associated with serum Iron, 1, 25(OH)2 Vitamin D, and hemoglobin in betathalassemia major patients. Hence, it seems that regular transfusions and chelating agents which can decrease the serum iron and increase hemoglobin level can be associated with lower iFGF23. [ABSTRACT FROM AUTHOR]

Background: The role of human papillomavirus (HPV), as a common infection, has been evaluated in many cancers such as the cervix and squamous cell carcinoma of the head and neck. To the best of our knowledge, for the first time, the association of HPV with papillary thyroid carcinoma (PTC) and its pathologic features are investigated. Methods: A retrospective cross-sectional study was conducted from May 2014 to January 2018 in several hospitals affiliated to Shiraz University of Medical Sciences, Shiraz, Iran. Thyroid tissue specimens of patients diagnosed with PTC (n=82) and benign thyroid nodules (n=77) were collected using the consecutive sampling method. The presence of HPV in PTC, adjacent normal tissue, and benign thyroid nodules was evaluated using the polymerase chain reaction (PCR) method. The frequency of HPV positivity in PTC tissues was compared with benign thyroid nodules and adjacent normal tissue. Association of pathologic features of PTC with HPV positivity was also investigated. Data were analyzed using SPSS version 21.0, and P values less than 0.05 were considered statistically significant. Results: HPV PCR positivity was observed in 3.8% of benign thyroid nodules and 13.4% of PTC samples but in none of the adjacent normal tissues. After adjustment for age and sex, the prevalence of HPV PCR positivity in the PTC tissues was significantly more than the benign thyroid nodules (P=0.015). The prevalence was also significantly higher than the adjacent normal tissues (P<0.001). Conclusion: There was a significant association between PTC and HPV positivity. Further studies are required to determine the cause and effect of the association between these two conditions. [ABSTRACT FROM AUTHOR]

Background: Papillary thyroid carcinoma (PTC) is the most prevalent form of thyroid cancer. In some studies, parvovirus B19 (PVB19) infection was involved in the pathogenesis of thyroid diseases such as Graves' disease, Hashimoto thyroiditis, and thyroid cancer. PVB19 induces chronic inflammation in thyroid, which can lead to carcinogenesis through the effect of inflammatory mediators. The association of PVB19 with PTC tumorigenesis is still a matter of controversy. We evaluated the correlation of PVB19 with PTC and, for the first time, pathologic features. Method: This cross-sectional retrospective study focused on the thyroid specimens of 82 patients with PTC and 77 patients with benign thyroid nodules. We conducted the present study from March 2014 to November 2017 in the hospitals affiliated with Shiraz University of Medical Sciences, Shiraz, Iran. We evaluated the presence of PVB19 DNA by nested polymerase chain reaction method in PTC, adjacent nonmalignant tissues, and benign thyroid nodules. PVB19 positivity was also compared between PTC and two other groups. We further investigated the association of pathologic features and tumor staging with PVB19 positivity. Results: Of the patients, 81% were female. We detected PVB19 positivity in 9.8% of PTC specimens and 0.01 of adjacent non-malignant tissues (P=0.016). None of the benign thyroid nodule specimens had PVB19 DNA, and they were significantly different from PTC specimens (P=0.007). There was no significant correlation between PVB19 positivity and tumor stages (P=0.988) and histologic types (P=0.560). Conclusion: This research, similar to some other studies, showed a significant association between PTC and PVB19 positivity. For the first time, we showed that no significant relationship existed between PVB19 positivity and tumor stages and histologic types. Further investigations are needed to evaluate the relationship between this virus and PTC. [ABSTRACT FROM AUTHOR]

Objectives Previous studies in adults with epilepsy revealed a higher prevalence of metabolic syndrome, resulting in cerebrovascular and cardiovascular events. However, there is insufficient data about body composition and metabolic syndrome in children, especially in the Middle Eastern region. We aimed to investigate metabolic syndrome criteria and body composition in ambulatory children with Epilepsy in Southern Iran. Material & Methods Seventy seven epileptic children with an average age of 11.4 ± 3.2 years and their age-gender-matched controls were included in this study. Anthropometric data, lipid profile, blood glucose, and blood pressure were checked. Body composition was also evaluated by Hologic system dual-energy X-ray absorptiometry. Results The prevalence of metabolic syndrome as well as the fat mass index in patients were higher than the controls, and p values are 0.032 and 0.012, respectively. Moreover, the lean mass with Bone Mineral Content (BMC) index was detected lower than the controls (P= 0.017). Regarding drugs consumption, serum triglyceride and the blood pressure in patients who receiving carbamazepine was higher than the control individuals with P = 0.019, Beta = 0.379 and P = 0.016, Beta = -0.26, respectively. Fat mass index was also higher in patients using sodium valproate (P = 0.031, Beta = 0.238). Conclusion Our study revealed that children with epilepsy are more prone to metabolic syndrome and higher body fat mass. Therefore, early diagnosis and prevention of metabolic syndrome criteria in patients with epilepsy, With performing regular exercise and having a healthy diet should be encouraged in these children. [ABSTRACT FROM AUTHOR]

Objective(s): Type 1 diabetes (T1D) is an autoimmune disease resulting from inflammatory destruction of islets ß-cells. Nowadays, progress in cell therapy, especially mesenchymal stem cells (MSCs) proposes numerous potential remedies for T1D. We aimed to investigate the combination therapeutic effect of these cells with insulin and metformin on neuropeptide Y, melanocortin-4 receptor, and leptin receptor genes expression in TID. Materials and Methods: One hundreds male rats were randomly divided into seven groups: the control, diabetes, insulin (Ins.), insulin+metformin (Ins.Met.), Wharton's Jelly-derived MSCs (WJ-MSCs), insulin+metformin+WJ-MSCs (Ins.Met.MSCs), and insulin+WJ-MSCs (Ins.MSCs). Treatment was performed from the first day after diagnosis as diabetes. Groups of the recipient WJ-MSCs were intraportally injected with 2× 106 MSCs/kg at the 7th and 28th days of study. Fasting blood sugar was monitored and tissues and genes analysis were performed. Results: The blood glucose levels were slightly decreased in all treatment groups within 20th and 45th days compared to the diabetic group. The C-peptide level enhanced in these groups compared to the diabetic group, but this increment in Ins.MSCs group on the 45th days was higher than other groups. The expression level of melanocortin-4 receptor and leptin receptor genes meaningfully up-regulated in the treatment groups, while the expression of neuropeptide Y significantly down-regulated in the treatment group on both times of study. Conclusion: Our data exhibit that infusion of MSCs and its combination therapy with insulin might ameliorate diabetes signs by changing the amount of leptin and subsequent changes in the expression of neuropeptide Y and melanocortin-4 receptor. [ABSTRACT FROM AUTHOR]