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This study was conducted to investigate the characteristics of the AlCrCuFeNi high-entropy alloy (HEA) synthesized through mechanical alloying (MA). In addition, effects of Process Control Agent (PCA) amount and milling time were investigated using X-ray diffraction analysis (XRD), scanning electron microscopy (SEM), and energy dispersive X-ray spectroscopy (EDS). The results indicated that the synthesized AlCrCuFeNi alloy is a dual phase (FCC + BCC) HEA and the formation of the phases is strongly affected by the PCA amount. A high amount of PCA postponed the alloying process and prevented solid solution formation. Furthermore, with an increase in the PCA amount, lattice strain decreased, crystallite size increased, and the morphology of the mechanically alloyed particles changed from spherical to a plate-like shape. Additionally, investigation of thermal properties and annealing behavior at different temperatures revealed no phase transformation up to 400 °C; however, the amount of the phases changed. By increasing the temperature to 600 °C, a sigma phase (σ) and a B2-ordered solid solution formed; moreover, at 800 °C, the FCC phase decomposed into two different FCC phases.

Maple Syrup Urine Disease (MSUD) disease is a defect in the function of the Branched-chain 2-ketoacid dehydrogenase complex (BCKDH). It is caused by pathogenic biallelic variants in BCKDHA, BCKA decarboxylase, or dihydrolipoamide dehydrogenase. The brain is the major organ involved in MSUD. MSUD happens in about 1 in 86,800 to 185,000 live births. According to some diversity in the management of Iranian patients with MSUD, the development of a national guideline is essential. This guideline is provided through a literature search on articles in PubMed, Scopus, Web of Sciences, Cochrane, and Embase databases from 2001 to 2022 accompanied by a consensus of physicians of different centers in Iran who are experts in the diagnosis and management of this disease. This article considers pathogenesis, epidemiology, clinical manifestations, diagnosis, treatment, and monitoring of MSUD patients with limited recourse.

Background The prevalence of celiac disease (CD) and hypothyroidism exhibit significant variation in different studies among patients with congenital heart disease (CHD). This study evaluated the frequency of laboratory test abnormalities in children and adolescents with CHD in Shiraz, Iran. Methods This prospective case-control study was conducted on 223 children and adolescents with CHD and healthy individuals referred to the heart clinic affiliated with Shiraz University of Medical Sciences between February 2019 and December 2021. They were classified into case and control groups. Blood tests were performed for total IgA antibody, anti-tissue transglutaminase IgA antibody (anti-TTG Ab), T4, and thyroid stimulating hormone (TSH) and anti-thyroid peroxidase antibodies in serum, along with transthoracic echocardiography. Likewise, demographic characteristics of patients, including age, sex, weight, height, and body mass index (BMI), were recorded. Also, anti-TTG Ab levels were compared among CHD patients according to cyanosis status, gender, age (above and below five years), and BMI (under and over 18.5). Results Ninety-eight CHD patients and 100 healthy individuals with an average age of 5.32 ± 4.05 years (1–18 years) were examined. In children with CHD, atrial septal defect (27%), ventricular septal defect (20%), and tetralogy of Fallot (13%) were the most prevalent disorders. Only one CHD patient had an anti-TTG Ab level of 16.6 unit/mL, considered borderline for seropositive CD diagnosis. There was no difference in anti-TTG Ab levels between age (above and below five years), BMI (under and over 18.5), cyanosis status, and gender groups. Seven CHD patients had high TSH levels, three had cyanotic CHD, and one had Down syndrome. The TSH levels and non-autoimmune hypothyroidism were significantly higher in CHD patients than in normal subjects (p < 0.05). Conclusions According to the results of this study, the serum level of TSH and prevalence of non-autoimmune hypothyroidism were higher in patients with CHD than in normal subjects, but the serum level of anti-TTG Ab was not different between the two groups.

Background Newborn screening is essential for the early detection of congenital genetic and metabolic disorders, enabling timely intervention to prevent morbidity, mortality, and disabilities associated with inherited metabolic disorders (IMDs). The Iranian Neonatal Screening Program piloted in Fars Province, screening nearly 100% of neonates for 20 disorders. This study aimed to assess the epidemiology of these metabolic diseases. From March 2019 to September 2021, 138,689 neonates were screened using tandem mass spectrometry (MS/MS) on dried blood spots. Those with abnormal results were referred to pediatric endocrinology and metabolism specialists for confirmatory testing per American College of Medical Genetics guidelines. Results Among the screened neonates, 139 patients of IMDs were identified, yielding an estimated birth prevalence of 1:1000. The positive cases included 55 aminoacidopathies, 47 organic acidemias, 31 fatty acid oxidation disorders, and 6 urea cycle defects were detected. The most prevalent IMDs were phenylalanine metabolism disorders, short-chain acyl-CoA dehydrogenase deficiency, 3-methylcrotonyl-CoA carboxylase deficiency, and methylmalonic acidemia. Notably, the prevalence of IMDs in Fars Province is significantly higher than average global statistics. Additionally, we observed that certain disorders previously deemed very rare exhibit a relatively high prevalence in this region. Conclusions Our data highlight the efficiency and robustness of neonatal screening for IMD in Iran. It demonstrates the need for expanded screening efforts across the entire country. One limitation of this study is that the screening was conducted in only one state, which may not reflect the broader population of Iran. Future research should involve nationwide implementation of screening programs to validate our findings and assess the prevalence of IMDs in diverse regions. Furthermore, exploring the applicability of our screening methods in other Middle Eastern countries could help promote early and life-changing diagnoses across the region.

Background:Diabetic ketoacidosis (DKA) can cause several complications. The myocardial damage in children with DKA was not evaluated clearly. This study aimed to investigate the troponin level as a biochemical marker of myocardial damage in children with DKA during the disease and after recovery.Methods:This cross-sectional study was conducted in the pediatric endocrinology department of Namazi Hospital, Shiraz, Iran, from May to December 2019. Fifty diabetic children aged 1 to 18 years old with DKA by census sampling method. All patients were examed, and electrocardiography (ECG) was carried out. Troponin level was measured at admission and 48 hours after recovery. Finally, data were analysed using SPSS software 20.Results:At the time of admission, the mean troponin level was 11.56±5.35 ng/mL and it became 2.15±1.21 ng/mL after recovery. Although the initial troponin level during DKA in three patients was higher than the expected normal reference range, their echocardiography and ECG were normal. Moreover, there was no statistical relationship between troponin level with age, diabetes duration, and DKA severity.Conclusion: The troponin level was remarkably different during DKA and after recovery. However, in merely 6% of DKA patients, the troponin level was higher than the expected value. We recommended that these patients be followed for future cardiovascular complications.

Although selenium is one of the nutrients that has an important role in the metabolism of thyroid hormones, it has been investigated in rare studies. This study aimed to evaluate role of selenium deficiency in children and adolescents with acquired hypothyroidism. This case and control study was conducted on 60 acquired hypothyroidism and 60 healthy children who had been referred to the pediatrics endocrine clinic in , from November 2018 to April 2019. Some information such as age, gender, weight, height, duration of disease, and level of plasma selenium were recorded. Plasma selenium level was measured by atomic absorption spectrophotometer. Data were analyzed using SPSS software 21.0. The mean of selenium level in the case and control groups were 93.77 ± 24.90 μg/dl and 85.96 ± 25.20 μg/dl, respectively. There was no significant difference between the two groups in the mean levels of selenium. Independent -test showed no significant difference in the mean levels of selenium in male and female samples in the case group, but this difference was significant in the control group. Selenium deficiency may not have significant role as a cause of acquired hypothyroidism in pediatric and adolescent age group, in south of . Thus, it does not seem necessary to routinely check the level of selenium for patients with thyroid dysfunction.

BACKGROUND: Morbid obesity is rising around the world. Surgery is a selective treatment intervention for it. Since most of the surgeons use weight loss for evaluating of the morbid obesity surgical outcomes and paying attention to the the quality of life (QOL) and body image evaluation are rare, this study aimed to assess the QOL and body image in pre- and postsurgery with other interventions. MATERIALS AND METHODS: This case-control study was done on 200 morbidly obese patients (n = 100 in each group) who referred to the obesity clinic in Shiraz. The data were collected by Body Image Concern Inventory and 12-item Short Form Survey. The data were analyzed by t-test, Pearson correlation coefficient test, analysis of variance, and regression analysis. P = 0.05 was considered as significant. RESULTS: The paired t-test showed a significant difference in mean scores of body image (P < 0.001) and all dimensions of the QOL (P < 0.001) in the case and control groups. In the case group, there was a significant difference between the mean scores of the QOL and the different categories of body mass index (BMI) in postsurgery (P < 0.05) and the different mean scores of body image and the different categories of BMI in pre- and postsurgery wasn't significant (P > 0.05). Age was a predictor variable for the QOL of morbidly obese persons after surgery (P < 0.001). After surgery, men showed better QOL than women (P < 0.001). CONCLUSION: Final results indicated that body image and QOL defects caused by obesity could be more improvement after surgery. This study can promote health-care team's knowledge about holistic supporting of all dimensions' QOL of obese individuals. It is suggested that supporting interventions should be done as effective methods of maintenance for effects of weight loss methods such as surgery.

Due to an increase in prevalence of cervical lesions, it is important to use appropriate restorative materials to reduce the incidence of secondary lesions. Owing to having antibacterial properties, cervical composite restorations containing different ratios of Zinc Oxide nanoparticles (ZnO NPs) have been analyzed using the Finite Element method to determine the optimal incorporation ratio from mechanical and thermal perspectives. A numerical simulation is conducted for a mandibular first premolar with a cervical lesion (1.5 × 2 × 3 mm3) restored with composites containing 0 to 5% wt. ZnO NPs. Subsequently, the samples are exposed to different thermo-mechanical boundary conditions, and stress distributions at different margins are examined. The accumulated stress in the restoration part increases for the 1% wt. sample, whereas the higher percentage of ZnO NPs leads to the reduction of stress values. In terms of different loading conditions, the least and most stress values in the restoration part are observed in central loading and lingually oblique force, respectively. The change in the surface temperature is inversely correlated with the ratio of ZnO NPs. In conclusion, the composite containing 5% wt. ZnO NPs showed the most proper thermo-mechanical behavior among all samples.

Background: Peripherally inserted central catheters (PICCs) are an effective method for medication and nutrition infusion in preterm neonates. The present study aimed to identify the incidence of the most common complications of PICC implantation and evaluate the risk factors of each complication. Methods: This historical cohort study was conducted on 2500 neonates with birth weights (BWs)≥500 g and gestational age (GA)>24 weeks who had a history of PICC inserted in three NICUs between August 2015 and August 2018. Data were collected by reviewing medical records. Demographic data and indices of catheter placement, duration of catheter placement, and common complications were recorded. Data analysis was done using SPSS-21. Results: The median cubital vein had the most PICC placement (43%). The most common complication was tip malposition (48.2%). The incidence rates (95% CI) of the main complications such as malposition, edema/occlusion, and PICC migration were 0.0356 (0.0337-0.0377), 0.0134 (0.0122-0.0147), and 0.0088 (0.0079-0.0099), respectively. PICC insertion position was the strongest predictor of malposition for the cephalic vein. Besides, the incidence of malformation in the cephalic vein was about six times higher than in the median cubital vein. Independent risk factors for non-technical complications included BW (OR=0.59, 95% CI 0.44-0.79), administration of hyperosmolar medications (OR=3.43, 95% CI 2.62-4.51), position (OR=2.43, 95% CI 1.92- 3.08), and duration of catheter presence (OR=1.02, 95% CI 1.01-1.03) (P<0.001). Conclusion: The most common complication was malposition related to catheter placement in an emergency. Moreover, BW, administration of hyperosmolar medications, and duration of catheter presence were the most critical risk factors for non-technical complications. Therefore, it is recommended to educate the PICC insertion team to reduce tip malposition and replace long-term catheters.

Objective This study aimed to find the common inborn errors of metabolism in Iranian patients with autism spectrum disorder. Methods In this cross-sectional multicenter study, 105 children and adolescents with autism spectrum disorder from six centers in different cities of Iran were enrolled between August, 2019 and October, 2020. Metabolic screening, including measuring plasma levels of amino acids, acylcarnitines, creatine, and guanidinoacetate, and urinary levels of organic acids, purines, and pyrimidines was performed. Other data, including age, parental consanguinity, history of seizure, developmental mile-stones, and physical examination, were also recorded. Results An inborn error of metabolism was found in 13 (12.4%) patients. Five patients (4.8%) had cerebral creatine deficiency syndrome, 4 (3.8%) had arginine succinate aciduria, 2-methylbutyryl glycinuria, short-chain acyl-CoA dehydrogenase deficiency, and combined methylmalonic aciduria/malonic aciduria. There was a strong association between positive metabolic evaluation and parental consanguinity, history of seizures, microcephaly, and delayed development. Conclusions Our results suggest that metabolic screening should be performed in the cases of autism associated with parental consanguinity, developmental delay, and a history of seizures. The assays to be considered as a screening panel include plasma or blood amino acids, acylcarnitines, creatine and guanidinoacetate, and urinary levels of organic acids.