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Background: According to previous reports, between 2%-5% of multiple sclerosis (MS) cases occur before the age of 16 years. Objectives: This study aims to investigate the clinical and epidemiological features of MS in Iranian children to provide diagnostic criteria for policymakers and physicians. Methods: This study objectives are achieved in two phases. The first phase was conducted and the second phase is underway. The first phase was performed in one year to design and finalize a pediatric MS registry software. For this reason, all variables and registration methods were determined. The second phase which has been started since 2016 consists of a routine data registry in the national phase. The project information, including all cases, epidemiological, clinical, and diagnostic factors is being reported based on different geographical areas of Iran. Results: We registered 932 cases with MS since 2017, of whom 74.4% were girls. There was no significant difference in mean age between girls and boys with MS (mean difference=0.73 years, P=0.133). Most of the children who were diagnosed were between the ages of 15 to 18 years (52.4%). Conclusions: The registry for MS in children in Iran can improve the knowledge of health policymakers on the incidence of MS in children throughout the country, the first and most common symptoms of MS in children, raising physicians’ awareness about early diagnosis and treatment that consequently reduce the economic, psychological, and psychological burden of the disease.

Abstract Background To investigate the genetics of early-onset progressive cerebellar ataxia in Iran, we conducted a study at the Children’s Medical Center (CMC), the primary referral center for pediatric disorders in the country, over a three-year period from 2019 to 2022. In this report, we provide the initial findings from the national registry. Methods We selected all early-onset patients with an autosomal recessive mode of inheritance to assess their phenotype, paraclinical tests, and genotypes. The clinical data encompassed clinical features, the Scale for the Assessment and Rating of Ataxia (SARA) scores, Magnetic Resonance Imaging (MRI) results, Electrodiagnostic exams (EDX), and biomarker features. Our genetic investigations included single-gene testing, Whole Exome Sequencing (WES), and Whole Genome Sequencing (WGS). Results Our study enrolled 162 patients from various geographic regions of our country. Among our subpopulations, we identified known and novel pathogenic variants in 42 genes in 97 families. The overall genetic diagnostic rate was 59.9%. Notably, we observed PLA2G6, ATM, SACS, and SCA variants in 19, 14, 12, and 10 families, respectively. Remarkably, more than 59% of the cases were attributed to pathogenic variants in these genes. Conclusions Iran, being at the crossroad of the Middle East, exhibits a highly diverse genetic etiology for autosomal recessive hereditary ataxia. In light of this heterogeneity, the development of preventive strategies and targeted molecular therapeutics becomes crucial. A national guideline for the diagnosis and management of patients with these conditions could significantly aid in advancing healthcare approaches and improving patient outcomes.

Discussions over paid peering and usage fees have become a global phenomenon, with ISPs insisting content providers pay for the substantial downstream traffic they generate, while content providers argue for settlement-free agreements, citing that consumers already pay ISPs for content delivery and content providers reduce costs for ISPs by delivering traffic close to customers. This contention is echoed in debates around net neutrality and related policies across the United States, Europe, and South Korea, prompting regulators to consider whether to regulate peering prices and/or impose usage fees. This question gains significance in the United States in light of the recent Federal Communications Commission (FCC) decision to consider the reinstatement of net neutrality rules. Our study examines whether the peering price set by the market aligns with the socially optimal peering price. We first analyze the cost-based peering price, which reflects the ISP’s incremental costs for direct content delivery versus via transit providers. We discover that this price can effectively be zero with sufficient content localization, yet remains above zero without it, influenced by the number of interconnection points. We then evaluate the profit-maximizing peering price using a two-sided market model, finding that increased content localization decreases this price. These prices establish a range if the peering price is unregulated, from the cost-based peering price (at the low end) to the profit-maximizing peering price (at the high end). Regulatory oversight of peering prices may be warranted when there is a substantial difference between cost-based and profit-maximizing prices. Finally, our study informs the debate on potential regulatory interventions about peering prices or usage fees. We compare unregulated market outcomes with those under regulatory measures aimed at maximizing consumer surplus or social welfare. Through our analysis, we identify optimal peering prices within our determined range, considering the influence of content localization and interconnection points on these prices.

Aims Forensic psychiatric services address the therapeutic needs of mentally disordered offenders in a secure setting. Clinical, ethical, and legal considerations underpinning treatment emphasize that the Quality of Life (QOL) of patients admitted to forensic hospitals should be optimised. This study aims to examine changes in the QOL in Ireland's National Forensic Mental Health Service (NFMHS) following its relocation from the historic 1850 site in Dundrum to a new campus in Portrane, Dublin. Methods This multisite prospective longitudinal study is part of the Dundrum Forensic Redevelopment Evaluation Study (D-FOREST). Repeated measures were taken for all inpatients in the service at regular 6 monthly intervals. The WHOQOL-BREF questionnaire was offered to all inpatients. An anonymised EssenCES questionnaire was used to measure atmosphere in wards. Data were obtained at 5 time points for each individual patient and ward. WHOQOL-BREF ratings were obtained across 5 time points with comparisons available for 4 time intervals, including immediately before and after relocation. For 101 subjects across 4 time intervals, 215 sets of data were obtained; 140 before and 65 after relocation with 10 community patients who did not move. Using Generalised Estimating Equations (GEE) to correct for multiple comparisons over time, the effect of relocation, with community patients as a control, was analysed by ward cluster and whether patients moved between wards. Observations were categorised according to security level – high dependency, medium secure, rehabilitation, or community – and trichotomised based on positive moves to less secure wards, negative moves to more secure wards, or no moves. Results Relocation of the NFMHS was associated with a significant increase in environmental QOL (Wald X2 = 15.9, df = 1, p < 0.001), even when controlling for cluster location, positive and negative moves. When controlling for ward atmosphere, environmental QOL remained significantly increased after the move (Wald X2 = 10.0, df = 1, p = 0.002). EssenCES scores were obtained within the hospital for 3 time points before relocation and 2 time points afterwards. No significant differences were found on the three subscales before and after the move. All three EssenCES subscales progressively improved with decreasing security level (Patient Cohesion: Wald X2 = 958.3, df = 1, p < 0.001; Experiencing Safety: Wald X2 = 152.9, df = 5, p < 0.001; Therapeutic Hold: Wald X2 = 33.6, df = 3, p < 0.001). Conclusion The GEE model demonstrated that the move of the NFMHS improved self-reported environmental QOL. The cluster location made significant differences, as expected for a system of stratified therapeutic security, with a steady improvement in scores on all three atmosphere subscales.

Background and Objectives: Pediatric cancer poses significant challenges for physicians, giving rise to numerous ethical dilemmas during diagnosis and treatment of affected children. This study explored the ethical experiences of pediatric oncologists across various phases of patient care and their interaction with patients’ families. Materials and Methods: This qualitative study was performed by purposive sampling method, involving pediatric oncologists from different provinces of Iran. Research data were gathered through semi-structured interviews, continuing until saturation of codes was achieved among new participants. Research data were extracted using MAXQDA 10 software and categorized by qualitative content analysis with the agreement of the research team. Results: Through 11 interviews conducted with pediatric oncologists representing 10 provinces in Iran, 158 initial codes were extracted. Following code analysis and classification, three main themes were identified, encompassing a total had 10 sub-themes. The main themes included parental reactions, therapeutic challenges, family communication strategies, and the physicians' inner experiences. Conclusion: The findings of this study showed a broad spectrum of ethical and clinical experiences of pediatric oncologists in Iran, with influences from geographical, religious, and demographic factors. The study underscores the need for tailored educational programs, protocols, and guidelines to improve the ethical performance knowledge of these physicians in alignment with the cultural, geographical, and religious conditions of Iran.

Abstract Background Breath‐holding spells (BHS) are common in infancy and early childhood and may appear like seizures. Factors such as autonomic dysfunction and iron deficiency anemia are thought to contribute to the incidence of BHS. In this study, electrocardiographic (ECG) parameters of patients with BHS were compared to those of healthy, normal children. Logistic regression and machine‐learning (ML) models were then created to predict these spells based on ECG characteristics. Methods In this case–control study, 52 BHS children have included as the case and 150 healthy children as the control group. ECG was taken from all children along with clinical examinations. Multivariate logistic regression model was used to predict BHS occurrence based on ECG parameters. ML model was trained and validated using the Gradient‐Boosting algorithm, in the R programming language. Results In BHS and control groups, the average age was 11.90 ± 6.63 and 11.33 ± 6.17 months, respectively (p = .58). Mean heart rate, PR interval, and QRS interval on ECGs did not differ significantly between the two groups. BHS patients had significantly higher QTc, QTd, TpTe, and TpTe/QT (all p‐values

Background: Cytomegalovirus (CMV) is a pathogenic virus that is found everywhere. The serovirulance of this virus varies widely among different communities. The present study was conducted to investigate the frequency of CMV serovirulance in mothers and their very low birth weight (

Abstract POLR3A is a main subunit encoding RNA polymerase III, which is involved in transcription of many RNA structures. Here, we report a new presentation of c.1771‐6C > G intronic variant presenting as developmental regression, seizure, and dystonia in a 6‐year‐old boy associated with striatum involvement in the brain MRI.

Abstract Purpose An increasing number of studies have reported a significant association between long non-coding RNAs (lncRNAs) dysregulation and pancreatic cancers. In the present study, we aimed to gather articles to evaluate the prognostic value of long non coding RNA in pancreatic cancer. Experimental design We systematically searched all eligible articles from databases of PubMed, Web of Science, and Scopus to meta-analysis of published articles and screen association of multiple lncRNAs expression with clinicopathology and/or survival of pancreatic cancer. The pooled hazard ratios (HRs) and their 95% confidence intervals (95% CIs) were used to analysis of overall survival, disease-free survival and progression-free survival were measured with a fixed or random effects model. Results A total of 39 articles were included in the present meta-analysis. Our results showed that dysregulation of lncRNAs were linked to overall survival (39 studies, 4736 patients HR = 0.41, 95% CI 0.25 ± 0.58, random-effects in pancreatic cancer. Moreover, altered lncRNAs were also contributed to progression-free survival (8 studies, 1180 patients HR: 1.88, 95% CI (1.35–2.62) and disease-free survival (2 studies, 285 patients, HR: 6.07, 95% CI 1.28–28.78). In addition, our findings revealed the association between dysregulated RNAs and clinicopathological features in this type of cancer. Conclusions In conclusion, dysregulated lncRNAs could be served as promising biomarkers for diagnosis and prognosis of pancreatic cancer.

Abstract A 10‐year‐old boy who was referred due to acute hydrocephalus symptoms was diagnosed as the first case of pediatric DLGNT in Iran. The results suggested that using shunting for hydrocephaly and anti‐seizure medicines, as well as chemotherapeutic agents, can be an effective treatment strategy for DLGNT. Although the patient was stable without a tumor recurrence for a limited follow‐up period of 22 months, further studies are expected.

Kawasaki disease (KD) is an inflammatory vasculitis. KD is classified into two groups based on clinical characteristics criteria, namely classic and incomplete. Cerebral vascular abnormality, especially arterial ischemic stroke (AIS) is very rare and unusual in KD. Here, we report a 4-year-old boy who was referred to our tertiary pediatric center with abrupt right hemiparesis and aphasia. At admission time, he had febrile illness and was toxic. On physical examination, we found unilateral left submandibular lymphadenopathy. On neurologic examination, we obtained right sided hemiparesis with hemiparetic gait and aphasia. His deep tendon reflexes (DTRs) of right extremities were exaggerated and his sensory system was intact. Based on these features, some differential diagnoses were suggested, such as acute encephalitis with focal signs, brain abscess, cerebral vasculitis, hemorrhagic insults, and ischemic stroke, etc. After a complete evaluation, especially brain MRI and MRA, our diagnosis was arterial ischemic stroke (AIS) following atypical KD. Based on these findings, we administered intravenous immunoglobulin (IVIG 2 gm/kg) and oral high dose aspirin (100 mg/kg/d). He responded to these anti-inflammatory treatments dramatically.

The purpose of this study was to investigate the effects of four types of diets containing different forms of selenium on the hematological blood parameters, reproduction and health of dairy cattle during the transition period. Twenty-four close-up dry cows with a mean of 259 ± 1days of pregnancy and expected 21 ± 1days prior to parturition were selected. The cows were fed four diets: 1) basal diet without selenium supplementation (C); 2) basal diet plus 0.5ppm selenium in the form of sodium selenite (Se-S) 3) basal diet plus 0.5ppm selenium in the form of selenium yeast (Se-Y); 4) basal diet plus 0.5ppm selenium in the form of selenomethionine (Se-M). Blood samples for measuring hematological blood parameters (including lymphocyte, red blood cells, hemoglobin, and hematocrit, MCV, MCH, MCHC and RDWc) were taken at 21 and 10 days before delivery, delivery date, 10 and 21 days after delivery. There was no significant difference in hematological parameters before and after delivery in experimental and control groups. However, in the prepartum period, MCH tended to increase significantly in selenium methionine treatment (p

The aim of this study was to determine the effects of supplementing close-up and fresh dairy cows’ diets with sodium selenite or organic sources of selenium (Se) on the dry matter intake (DMI), milk production and composition. Based on their body condition score (BCS), previous lactation milk yield and expected calving date, 24 multiparous Holstein dairy cows were balanced and randomly assigned to 4 experimental treatments. Experimental treatments were: control (basal diet without Se supplementation with 0.10 and 0.15 mg of Se kg-1 DM in close-up and fresh diets, respectively), sodium selenite supplementation (0.50 mg of Se kg-1 DM; Se-S), selenium yeast supplementation (0.50 mg of Se kg-1 DM; Se-Y) and selenomethionine supplementation (0.50 mg of Se kg-1 DM; Se-M). Neither Se supplementation nor Se source affected DMI pre- and postpartum. Milk production and composition were not different across Se sources, but concentrations of Se in milk were 14 and 10% greater for cows fed Se-M and Se-Y than for those fed control, respectively. Cows fed Se–M had lower somatic cell count than control (SCC; 173.11 vs. 318.89 cells × 103 Ml-1). The changes of BW and BCS pre- and postpartum were not affected by treatments over experimental period. The results of present study showed that selenomethionine supplementation compared to other sources decreased SCC in transition cows. In addition, milk Se concentrations increased in Se-Y and Se-M groups more significantly compared to the Se-S group. This indicates that organic Se had better replace inorganic Se for transition dairy cows.

The aim of this study was to determine the effects of supplementing close-up and fresh dairy cows’ diets with sodium selenite or organic sources of selenium (Se) on the serum biochemical parameters and antioxidant indicators and Se status. Twenty-four multiparous Holstein dairy cows were balanced by body condition score (BCS), previous lactation milk yield and expected calving date and randomly assigned to 1 of 4 experimental treatments. Treatments were: control (basal diet without Se supplementation), sodium selenite supplementation (0.50 mg of Se kg-1 DM; Se-S), selenium yeast supplementation (0.50 mg of Se kg-1 DM; Se-Y) and selenomethionine supplementation (0.50 mg of Se kg-1 DM; Se-M). Serum Se concentrations were higher for cows in Se-Y (72.34 µg dL-1) and Se-Met (72.34 µg dL-1) than control (59.93µg/dl) and Se-S (64.79µg/dl). The Se supplementation and sources did not affect serum metabolites or indices of antioxidant pre and postpartum, except serum total protein and albumin concentrations. Cows in Se-M had greater serum total protein and albumin concentrations than those in control. The results of present study showed that serum Se concentrations increased in Se-Y and Se–M more effectively than Se-S, indicating that selenomethionine could replace Se-S as an effective organic Se source for transition dairy cows.

Introduction: Acute cerebellitis (AC) is a rare potentially life-threatening condition in children. Some viral infections, vaccines and neuroimmunologic disorders are the most common causes of AC. Opium poisoning is an unusual cause of this condition. Case presentation: A 2-year-old girl was referred with loss of consciousness. She was ataxic just a few minutes after opium ingestion and after 1 hour, she became unconscious. We only found pinpoint pupils. After naloxone drip, her condition had been better but she was still obtunded and her urine was positive for opium products (morphine). MRI of the brain showed marked bilateral cerebellar swelling that respond to high-dose steroid dramatically. Conclusion: This case shows that opium intoxication should be considered as a rare cause of acute cerebellitis in children.

Introduction: cerebral vein thrombosis is a rare complication of nephrotic syndrome (NS). We report a known case of NS with hemorrhagic thrombosis. Case report: A boy with previous history of NS was admitted with headache and decrease of level of consciousness and his brain images were revealed hemorrhagic thrombosis Conclusions: cerebral vein thrombosis must be considered in patients with history of NS (especially in new cases and during of relapses) and prevention of hemoconcentration is very important to decrease thrombosis risk.

Drug-induced hypersensitivity syndrome (DHS), also named drug rash with Eosinophilia and systemic symptoms (DRESS) is a potentially dangerous side effect of some drugs, especially antiepileptic drugs (AEDs) such as phenobarbital, phenytoin, carbamazepine, lamotrigine, primidone, etc. It can also be caused by other drugs, such as sulfonamides and captopril. Diagnosis of DHS may be difficult because of the variety of clinical and laboratory abnormalities and manifestations and because the syndrome may mimic infectious or collagen vascular disorders. Management includes immediate withdrawal of the culprit drug, supportive care and systemic steroids or Immunoglobulins (IVIG). Here, we briefly reviewed the literature, followed by a case report that had all of the criteria of DRESS without eosinophilia.

Introduction: Exposure to 3, 4- methylenedioxymethamphetamine (MDMA) could lead to serotonergic system toxicity in the brain. This system is responsible for learning and memory functions. Studies show that MDMA causes memory impairment dose-dependently and acutely. The present study was designed to evaluate the chronic and acute effects of MDMD on spatial memory and acquisition of passive avoidance. Methods: Adult male Wistar rats (200-250 g) were given single or multiple injections of MDMA (10 mg/kg, IP). Using passive avoidance and Morris Water Maze (MWM) tasks, learning and spatial memory functions were assessed. The data were analyzed by SPSS 16 software and one- way analysis of variance (ANOVA) test. Results: Our results showed that there were significant differences in latency to enter the dark compartment (STL) between sham and MDMA- treated groups. Acute group significantly showed more STL in comparison with chronic group. Furthermore, MDMA groups spent more time in dark compartment (TDS) than the sham group. Administration of single dose of MDMA significantly caused an increase in TDS compared with the chronic group. In the MWM, MDMA treatment significantly increased the traveled distance and escaped latency compared to the sham group. Like to passive avoidance task, percentage of time spent in the target quadrant in MDMA- treated animals impaired in MWM compared with sham group. Discussion: These data suggest that MDMA treatment impairs learning and memory functions that are more extensive in acute- treated rats.

Objective: Stroke is most important cause of death and disability in adults. The hippocampal CA1 and sub-ventricular zone neurons are vulnerable to ischemia that can impair memory and learning functions. Although neurogenesis normally occurs in the dentate gyrus (DG) of the hippocampus and sub-ventricular zone (SVZ) following brain damage, this response is unable to compensate for severely damaged areas. This study aims to assess both neurogenesis and the neuroprotective effects of transforming growth factor-alpha (TGF-α) on the hippocampus and SVZ following ischemia-reperfusion. Materials and Methods: In this experimental study, a total of 48 male Wistar rats were divided into the following groups: surgical (n=12), phosphate buffered saline (PBS) treated vehicle shams (n=12), ischemia (n=12) and treatment (n=12) groups. Ischemia was induced by common carotid occlusion for 30 minutes followed by reperfusion, and TGF-α was then injected into the right lateral ventricle. Spatial memory was assessed using Morris water maze (MWM). Nestin and Bcl-2 family protein expressions were studied by immunohistochemistry (IHC) and Western blot methods, respectively. Finally, data were analyzed using Statistical Package for the Social Sciences (SPSS, SPSS Inc., Chicago, USA) version 16 and one-way analysis of variance (ANOVA). Results: TGF-α injection significantly increased nestin expression in both the hippocampal DG and SVZ areas. TGF-α treatment caused a significant decrease in Bax expression and an increase in Bcl-2 anti-apoptotic protein expression in the hippocampus. Our results showed a significant increase in the number of pyramidal neurons. Memory also improved significantly following TGF-α treatment. Conclusion: Our findings proved that TGF-α reduced ischemic injury and played a neuroprotective role in the pathogenesis of ischemic injury.

Introduction Acute cerebellitis (AC) is a rare potentially life-threatening condition in children. Some viral infections, vaccines and neuroimmunologic disorders are the most common causes of AC. Opium poisoning is an unusual cause of this condition. Case presentation A 2-year-old girl was referred with loss of consciousness. She was ataxic just a few minutes after opium ingestion and after 1 hour, she became unconscious. We only found pinpoint pupils. After naloxone drip, her condition had been better but she was still obtunded and her urine was positive for opium products (morphine). MRI of the brain showed marked bilateral cerebellar swelling that respond to high-dose steroid dramatically. Conclusion This case shows that opium intoxication should be considered as a rare cause of acute cerebellitis in children.

Background: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder. The pathogenesis is unclear, but it is thought to be immune-mediated. The prognosis is favorable, with most children making a full recovery. Objectives: The present report analyzed different clinical presentations, response to treatment and outcome in a series of 11 patients with ADEM who referred to our tertiary center in north of Iran from 2010 to 2014. Materials and Methods: In this retrospective simple descriptive review, eleven cases with ADEM admitted in the neurology ward from 2010 to 2014 were enrolled. The clinical findings and laboratory and imaging results of patients were reviewed. All of these cases were evaluated with neurological examination, serologic tests for bacterial meningitis and viral encephalitis (especially, herpes simplex virus) and brain MRI without contrast. After discharge, patients were followed for at least six months (6 to 12 months) clinically and radiologically. Results: Of 11 children, 8 were male and 3 female. Their ages ranged between 4 and 10 years. The mean interval between the preceding infection and symptoms of encephalomyelitis was nine days. The most common presenting symptoms were ataxia in 45.4%, fever and headache in 36.4% and altered consciousness in 18.2% of patients. Neurological examination revealed pyramidal motor signs such as brisk deep tendon reflexes (hyperreflexia) (81.8%), cranial nerve involvement (18.2%), dysarthria (9.1%) and abnormal movements (9.1%). We followed up these patients in long-term for 6 to 12 months. Only in 1 child who received IVIG, mild ataxia had reminded. Conclusions: The prognosis of acute disseminated encephalomyelitis (ADEM) is favorable. Early diagnosis and prompt treatment of ADEM would probably reduce morbidity.

This study was conducted to determine nutritive value of common animal feed sources in East Azerbaijan province including second cutting of alfalfa hay ( N=35) and wheat straw (N=26). Chemical composition and mineral contents of alfalfa hay and wheat straw were determined according to standard methods. Then the average chemical compositions of these samples were compared with those of the Gilan, Kurdistan and Kermanshah samples and the National Research Council (NRC) data. The results demonstrated that the mean dry matter (DM) content of alfalfa hay and wheat straw were 942.4± 5.6 and 948.7 ± 6.5 g/kg, and mean crude protein (CP) concentrations were 158.6 ± 11.4 and 40.7 ± 4.8 g/kg of dry matter, respectively, that showed a significant difference with NRC data. Neutral Detergent Fiber (NDF) content of alfalfa hay was 347.5 ± 51.5 g/kg and Acid Detergent Fiber (ADF) of alfalfa hay and wheat straw were 447.3 ± 39.2 and 703.1 ± 5.55 g/kg, respectively; that showed no significant difference. The content of calcium in the alfalfa hay was 13.9 ± 3.5 g/kg that revealed no significant difference with the NRC data. The calcium concentration of wheat straw was 5.7 ± 1.4 g/kg, that differed significantly with the NRC data. The gross energy of alfalfa hay and wheat straw were 4100±154 and 3981± 126 kcal/ kg, respectively. In conclusion, it is recommended that agricultural managers in regional programming, farmers and feed manufacturers in balanced feed formulation use this data.

This paper synthesizes the important issues related to the post-completion audit of capital budgeting projects from a managerial prospective, with a special emphasis on the managerial control system governing the decision making process. The paper contemplates to explain the importance of such audits and also covers major areas in its systematic process. The paper has a normative orientation and draws from both the relevant fundamental concepts in operational auditing and capital budgeting literature.