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Vadood Javadi Parvaneh, Mohsen Jari, Saeideh Qhasemi, Mohammad Mahdi Nasehi, Khosro Rahmani, Reza ShiariMofid Children’s Hospital, Shahid Beheshti Medical University of Sciences, Tehran, IranBackground: Systemic lupus erythematosus (SLE) is an autoimmune disease which involves multiple organs, including peripheral nervous system.Case presentation: We describe a 12-year-old boy with progressively worsening neurological symptoms as first manifestation. Legs pain, loss of balance, and lower extremity weakness were the reason for his admission in neurologic ward. The patient was started on intravenous immunoglobulin therapy due to the possibility of Guillain–Barre syndrome and acute inflammatory demyelinating polyneuropathy (AIDP). However, there was no appropriate response and he developed recurrent attacks of polyneuropathy again with diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Then, he received intravenous pulse of methylprednisolone for 5 consecutive days followed by oral prednisolone for 3 months. One month after withdrawal of corticosteroid he admitted again with the same manifestations. Rheumatologic workup revealed the presence of leukopenia, hemolytic anemia, hematuria, proteinuria, positive antinuclear antibodies, and ds-DNA antibodies. On the basis of the American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria for SLE, the patient had underlying diagnosis of SLE. Eventually, he was treated by the pulse of methylprednisolone and cyclophosphamide, and oral hydroxychloroquine and prednisolone. His neurological and physical symptoms improved and complete neurological recovery occurred several months later.Conclusion: SLE and AIDP/CIDP are different entities, but ADP/CIDP can be part of the neurologic manifestations of the SLE. Although the association between AIDP/CIDP and SLE is very rare especially as a first manifestation of SLE, it should be early recognized for rapid appropriate treatment.Keywords: Guillain–Barre syndrome, chronic inflammatory demyelinating polyneuropathy, systemic lupus erythematosus, nerve disorders

Objective Data on the relationship between iron deficiency anemia and febrile convulsions are controversial. The aim of this study was to determine the association between iron deficiency anemia and febrile convulsions among children Materials & Methods This case-control study was conducted during 2006-2007, on 90 children with febrile seizures (case) and 90 febrile children without seizures (control) referred to the Amirkola children hospital (a referral hospital in the north of Iran). Two groups were matched for age and sex. In all children hemoglobin (Hb) level, hematocrit (Hct), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH) and plasma ferritin (PF) were determined and the data collected were analyzed statistically using the t-test. Results The mean PF and TIBC levels were not significantly different in the febrile seizure compared to the reference group; neither were differences in Hb levels statistically significant between two groups. However MCV and MCH were significantly higher in the febrile seizure group (p<0.05) compared to reference group. Conclusion Plasma ferritin levels were not significantly lower in children with febrile seizures in comparison with the children in control group. It seems that iron insufficiency does not play a role in pediatric febrile seizures. [ABSTRACT FROM AUTHOR]

Objectives: Claims data covers a large population and can be utilized for various epidemiological and economic purposes. However, the diagnosis of prescriptions is not determined in the claims data of many countries. This study aimed to develop a decision rule algorithm using prescriptions to detect patients with hypertension in claims data., Methods: In this retrospective study, all Iran Health Insurance Organization (IHIO)-insured patients from 24 provinces between 2012 and 2016 were analyzed. A list of available antihypertensive drugs was generated and a literature review and an exploratory analysis were performed for identifying additional usages. An algorithm with 13 decision rules, using variables including prescribed medications, age, sex, and physician specialty, was developed and validated., Results: Among all the patients in the IHIO database, a total of 4,590,486 received at least one antihypertensive medication, with a total of 79,975,134 prescriptions issued. The algorithm detected that 76.89% of patients had hypertension. Among 20.43% of all prescriptions the algorithm detected as issued for hypertension, mainly were prescribed by general practitioners (55.78%) and hypertension specialists (30.42%). The validity assessment of the algorithm showed a sensitivity of 100.00%, specificity of 48.91%, positive predictive value of 69.68%, negative predictive value of 100.00%, and accuracy of 76.50%., Conclusion: The algorithm demonstrated good performance in detecting patients with hypertension using claims data. Considering the large-scale and passively aggregated nature of claims data compared to other surveillance surveys, applying the developed algorithm could assist policymakers, insurers, and researchers in formulating strategies to enhance the quality of personalized care., Supplementary Information: The online version contains supplementary material available at 10.1007/s40200-024-01519-y., Competing Interests: Conflict of interestThe authors declared that they have no conflict of interest., (© The Author(s), under exclusive licence to Tehran University of Medical Sciences 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Background: The inappropriate use and overprescription of antibiotics pose a global health threat, particularly contributing to antimicrobial resistance. This study aims to evaluate antibiotic prescription prevalence in Iranian outpatients using the defined daily doses (DDD) and Access, Watch, and Reserve classification systems., Methods: This retrospective study analyzed electronic prescriptions for systemic antibiotics in Tehran, Iran, from March 2022 to March 2023. The data were obtained from the Iranian Health Insurance Organization and processed using the Cross-Industry Standard Process. Descriptive statistics and DDD per 1,000 inhabitants per day were calculated., Results: A total of 817,178 antibiotic prescriptions were analyzed, with a sex distribution of 57.43% female and a median age of 48 years. On average, each patient received 1.89 antibiotics per prescription. Over 63% of antibiotics were classified in the "Watch" category, with Azithromycin being the most commonly prescribed (27.56%). The total DDD per 1,000 inhabitants per day was 4.99, with general practitioners accounting for 58.02% of the prescriptions, primarily prescribing Azithromycin., Conclusions: The study emphasizes the high use of Watch group antibiotics, indicating a need for improved prescribing practices. Education on antibiotic stewardship and stricter guidelines are necessary to combat antimicrobial resistance. Continuous monitoring is crucial to optimize antibiotic use in outpatient settings in Iran., (Copyright © 2024 Association for Professionals in Infection Control and Epidemiology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Three medications have been approved for spinal muscular atrophy (SMA) treatment. No head-to-head clinical trials have directly compared the efficacy of nusinersen and risdiplam. We compare the efficacy of them in Type 2 and 3 SMA patients, with 6 months of follow-up., Methods: A multicenter cohort study was conducted. Demographic, genetic and clinical findings containing Hammersmith Functional Motor Scale Expanded (HFMSE) and revised upper limb module (RULM) scales were gathered. An increase of at least 3 points in HFMSE and RULM scores was considered a positive response., Results: 73 (58.4%) children received risdiplam, and 52 (41.6%) received nusinersen non-randomly, based on clinical decision. The difference in HFMSE and RULM scores compared to the baseline was significant in both groups (P-value <0.001). However, there was no significant difference between mean difference changes in HFMSE and RULM scores between the two groups. 80.4% of patients in the risdiplam group and 72% in the nusinersen group achieved the 3-point cutoff after 6 months, and there is no significant difference between the two groups (P-Value:0.33)., Conclusions: This study showed that both medications significantly changed the HFSME and RULM after 3 and 6 months of follow-up. However, there was no significant difference between the two drugs according to the HFSME.

Background: The burden of disease based on disability-adjusted life years (DALYs) is one of the internationally accepted metrics for assessing the impact of a disease or injury on population health. This study aimed to provide evidence of the burden of COVID-19 on health in Iran based on hospital-level data from the Iran Health Insurance Organization (IHIO), which covers almost half of the country's population., Methods: The data of all IHIO enrollees who were referred to hospitals across the country from the beginning of the COVID-19 pandemic (February 2020) to December 30, 2021, with assigned diagnosis codes of COVID-19, were extracted from the hospital information processing system. The DALYs due to COVID-19 were estimated using the standard approach of the World Health Organization and the European Burden of Disease Network guideline., Results: In the years 2020 and 2021, among a population of about 42 million people, 1,040,367 individuals were admitted to the hospital due to COVID-19 infection, of whom 73% were hospitalized (760,963 patients). The total estimated DALYs for these two years were 665,823 and 928,393, respectively (1,603 and 2,234 per 100,000 population). 99.7% of DALYs were attributed to years of life lost due to premature death (YLLs). The share of the disease burden in the age groups of under 20 years, 20-49 years, 50-80 years, and over 80 years was 6.6%, 26.4%, 58.4%, and 8.6%, respectively., Conclusions: Based on the hospital-level data estimates, COVID-19 has had a significant burden on health in Iran. COVID-19 was identified as the fifth leading cause of disease burden in Iran during the study period, ranking after cardiovascular diseases, psychological disorders, neoplasms, and musculoskeletal disorders. Additionally, COVID-19 was the third major cause of death, following cardiovascular diseases and neoplasms. Policymaking and the implementation of comprehensive programs to enhance the response of the health system and society to outbreaks of emerging and re-emerging infectious diseases are of utmost importance., (© 2024. The Author(s).)

Head computed tomography (CT) is the preferred imaging modality for mild traumatic brain injury (mTBI). The routine use of head CT in low-risk individuals with mild TBI offers no clinical benefit but also causes notable health and financial burden. Despite the availability of related guidelines, studies have reported considerable rate of non-indicated head CT requests. The objectives were to provide an overall estimate for the head CT overutilization rate and to identify the factors contributing to the overuse. A systematic review of PubMed, Scopus, Web of Science, and Embase databases was conducted up to November 2023, following PRISMA and MOOSE guidelines. Two reviewers independently selected eligible articles and extracted data. Quality assessment was performed using a bias risk tool, and a random-effects model was used for data synthesis. Fourteen studies, encompassing 28,612 patients, were included, with 27,809 undergoing head CT scans. Notably, 75% of the included studies exhibited a moderate to high risk of bias. The overutilization rate for pediatric and adult patients was 27% (95% CI: 5-50%) and 32% (95% CI: 21-44%), respectively. An alternative rate, focusing on low-risk pediatric patients, was 54% (95% CI: 20-89%). Overutilization rates showed no significant difference between teaching and non-teaching hospitals. Patients with mTBI from falls or assaults were less likely to receive non-indicated scans. There was no significant association between physician specialty or seniority and overuse, nor between patients' age or sex and the likelihood of receiving a non-indicated scan. Approximately one-third of head CT scans in mTBI cases are avoidable, underscoring the necessity for quality improvement programs to reduce unnecessary imaging and its associated burdens., (© 2024. The Author(s), under exclusive licence to American Society of Emergency Radiology (ASER).)

Objectives: Therapeutic plasma exchange (TPE) is a plasmapheresis procedure whose Safety data for pediatric neuro-immunological disorders (PNID) is confined. The present research documents TPE's safety and feasibility data in these conditions., Materials & Methods: The current study involved six distinct groups of patients with PNID undergoing TPE: neuromyelitis optic spectrum disorder (NMOSD), autoimmune encephalitis (AIE), acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), Guillain-Barre syndrome (GBS), and optic neuritis (ON). This study documented complications related to each TPE process. In addition, TPE's efficacy was studied in these patients., Results: The present study recorded adverse effects in 18 patients with PNID that received 121 TPE cycles: five cycles (4.13%) in MS, three (2.48%) in AIE subgroup, one (0.82%) in ADEM, and two (1.65%) in GBS. No severe complications were observed among the patients., Conclusion: Patients with PNID tolerated therapeutic plasma exchange, which was a safe process., Competing Interests: The authors have no conflicts of interest to declare., (© 2023 The Authors.)

Background: Different populations and areas of the world experienced diverse COVID-19 hospitalization and mortality rates. Claims data is a systematically recorded source of hospitalized patients' information that could be used to evaluate the disease management course and outcomes. We aimed to investigate the hospitalization and mortality patterns and associated factors in a huge sample of hospitalized patients., Methods: In this retrospective registry-based study, we utilized claim data from the Iran Health Insurance Organization (IHIO) consisting of approximately one million hospitalized patients across various hospitals in Iran over a 26-month period. All records in the hospitalization dataset with ICD-10 codes U07.1/U07.2 for clinically/laboratory confirmed COVID-19 were included. In this study, a case referred to one instance of a patient being hospitalized. If a patient experienced multiple hospitalizations within 30 days, those were aggregated into a single case. However, if hospitalizations had longer intervals, they were considered independent cases. The primary outcomes of study were general and intensive care unit (ICU) hospitalization periods and case fatality rate (CFR) at the hospital. Besides, various demographic and hospitalization-associated factors were analyzed to derive the associations with study outcomes using accelerated failure time (AFT) and logistic regression models., Results: A total number of 1 113 678 admissions with COVID-19 diagnosis were recorded by IHIO during the study period, defined as 917 198 cases, including 51.9% females and 48.1% males. The 61-70 age group had the highest number of cases for both sexes. Among defined cases, CFR was 10.36% (95% CI: 10.29-10.42). The >80 age group had the highest CFR (26.01% [95% CI: 25.75-26.27]). The median of overall hospitalization and ICU days were 4 (IQR: 3-7) and 5 (IQR: 2-8), respectively. Male patients had a significantly higher risk for mortality both generally (odds ratio (OR) = 1.36 [1.34-1.37]) and among ICU admitted patients (1.12 [1.09-1.12]). Among various insurance funds, Foreign Citizens had the highest risk of death both generally (adjusted OR = 2.06 [1.91-2.22]) and in ICU (aOR = 1.71 [1.51-1.92]). Increasing age groups was a risk of longer hospitalization, and the >80 age group had the highest risk for overall hospitalization period (median ratio = 1.52 [1.51-1.54]) and at ICU (median ratio = 1.17 [1.16-1.18]). Considering Tehran as the reference province, Sistan and Balcuchestan (aOR = 1.4 [1.32-1.48]), Alborz (aOR = 1.28 [1.22-1.35]), and Khorasan Razavi (aOR = 1.24 [1.20-1.28]) were the provinces with the highest risk of mortality in hospitalized patients., Conclusion: Hospitalization data unveiled mortality and duration associations with variables, highlighting provincial outcome disparities in Iran. Using enhanced registry systems in conjunction with other studies, empowers policymakers with evidence for optimizing resource allocation and fortifying healthcare system resilience against future health challenges., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Mehrizi et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background: Different medication prescription patterns have been associated with varying course of disease and outcomes in COVID-19. Health claims data is a rich source of information on disease treatment and outcomes. We aimed to investigate drug prescription patterns and their association with mortality and hospitalization via insurance data for a relatively long period of the pandemic in Iran., Methods: We retrieved hospitalized patients' data from Iran Health Insurance Organization (IHIO) spanning 26 months (2020-2022) nationwide. Included were patients with ICD-10 codes U07.1/U07.2 for confirmed/suspected COVID-19. A case was defined as a single hospitalization event for an individual patient. Multiple hospitalizations of a patient within a 30-day interval were aggregated into a single case, while hospitalizations with intervals exceeding 30 days were treated as independent cases. The Anatomical Therapeutic Chemical (ATC) was used for medications classification. The two main study outcomes were general and intensive care unit (ICU) hospitalization periods and mortality. Besides, various demographic and clinical associate factors were analyzed to derive the associations with medication prescription patterns and study outcomes using accelerated failure time (AFT) and logistic regression models., Results: During the 26 months of the study period, 1,113,678 admissions with COVID-19 diagnosis at hospitals working in company with IHIO were recorded. 917,198 cases were detected from the database, among which 51.91% were females and 48.09% were males. Among the main groups of medications, antithrombotics (55.84% [95% CI: 55.74-55.94]), corticosteroids (54.14% [54.04-54.24]), and antibiotics (42.22% [42.12-42.32]) were the top used medications among cases with COVID-19. Investigation of the duration of hospitalization based on main medication groups showed antithrombotics (adjusted median ratio = 0.94 [0.94-0.95]) were significantly associated with shorter periods of overall hospitalization. Also, antithrombotics (adjusted odds ratio = 0.74 [95%CI, 0.73-0.76]), corticosteroids (0.97 [0.95-0.99]), antivirals (0.82 [0.80-0.83]), and ACE inhibitor/ARB (0.79 [0.77-0.80]) were significantly associated with lower mortality., Conclusion: Over 2 years of investigation, antithrombotics, corticosteroids, and antibiotics were the top medications for hospitalized patients with COVID-19. Trends in medication prescription varied based on various factors across the country. Medication prescriptions could potentially significantly impact the trends of mortality and hospitalization during epidemics, thereby affecting both health and economic burdens., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Mehrizi, Golestani, Malekpour, Karami, Nasehi, Effatpanah, Ranjbaran, Shahali, Sari and Daroudi.)

Objective: To determine the clinical and MRI characteristics of multiple sclerosis (MS) in the children and adolescents., Material & Methods: In this cross-sectional study, information of 95 MS patients was obtained from the Iranian MS registry. Disease characteristics and imaging data were collected using medical records., Results: Ninety-five patients including 64 female and 31 male subjects with mean age of 13.97±2.4 years (range, 8-18) years were enrolled. The most frequent signs and symptoms were ophthalmic symptoms (n=61, 64.2%), brainstem signs (n=44, 46.3%), cerebellar signs (n=32, 33.6%) and pyramidal signs (n=26, 27.3%). Blurred vision (n=21, 34.4%) was the most common ophthalmic symptom and ataxia (n=24, 75%) the most prevalent cerebellar sign. The most common brainstem signs/symptoms were motor symptoms and vertigo (each n=14, 31.8%) and the most common pyramidal sign/symptom was right upper monoparesis (n=14, 23.3%). Active demyelinating lesions were reported in brain MRI of all patients, mostly appeared as periventricular (n=91, 95.8%) and pericallosal (n=55, 57.9%) lesions. Acute demyelinating spinal lesions were presented in 38 patients (51.3%) with a prominent involvement of the cervical spine (n=33, 86.8%)., Conclusion: In our study, the most frequent signs and symptoms were eye symptoms, brainstem signs, cerebellar signs and pyramidal signs, respectively. Moreover, our results showed that MRI plays a critical role in the diagnostic evaluation of MS in children with presence of brain lesions in all patients and spinal lesion in a considerable portion of patients., Competing Interests: Authors declare that they have no conflicts of interest

Background & Aims: This study aimed to comprehensively investigate the effects of omega 3 supplementation on BDNF., Methods: Original databases were searched using standard keywords to identify all controlled trials that investigating the BDNF effects of omega 3 supplementation. Pooled weighted mean difference and 95% confidence intervals were achieved by random-effects model analysis for the best estimation of outcomes., Results: According to the results of a random-effects meta-analysis, omega 3 supplementation significantly raised BDNF levels compared to the control group (pooled WMD of 1.01 μmol/L; 95% confidence interval [CI] 0.35 to 1.67; P = 0.003) and this increase was even more pronounced for interventions >10 weeks and doses ≤1500 mg/day. Additionally, in individuals under 50 years of age, a greater increase in the effects of omega-3 supplements on this brain factor was observed., Conclusions: The present comprehensive review and meta-regression analysis generally showed that omega-3 supplementation can statistically significantly increase BDNF levels., Competing Interests: Declaration of competing interest We, the authors, declare that we had no competing interests., (Copyright © 2023 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

Objectives: Migraine is a common disorder in children, and its prophylaxis with minimal side effects is momentous. This study aimed to compare the efficacy of Pregabalin and Sodium Valproate in preventing migraine attacks., Material & Methods: Sixty-four children (aged 6-18) with migraines were recruited, as defined by Internation Headache Criteria (ICHD-III). They were randomly assigned to two groups: Sodium Valproate (n=32) and Pregabalin (n=32). The minimum dosage of drugs was prescribed in both groups. The patients were followed for four months. The parameters such as frequency, intensity, duration of migraine attacks, and the number of painkillers that the patients used monthly were recorded. The Spence Children's anxiety scale was also used to evaluate medications' effect on patients' anxiety levels., Results: Two medications were equally effective in reducing the intensity and duration of attacks. Additionally, their effect on reducing the anxiety level of patients was equal. There was a significant difference between the effect of drugs on the frequency of migraine attacks at the end of the first and fourth months and the number of painkillers used at the end of the fourth month. The frequency of attacks was decreased by more than 50% in twenty-eight patients (90%) of Pregabalin recipients and twenty-one patients (84%) of Sodium Valproate recipients., Conclusion: Considering the better effect of Pregabalin in the reduction of frequency of migraine attacks and pain-reducing medications consumption, Pregabalin could be a proper substitute for Sodium Valproate for prophylactic migraine treatment in children., Competing Interests: The authors declared no conflict of interest.

Neuromuscular diseases (NMDs) affect muscle function directly or indirectly by affecting nerves or neuromuscular junctions. One of the leading causes of death in patients with NMD is respiratory muscle weakness (RMW). Respiratory involvement in patients with NMD can manifest widely, from mild failure that may initially affect only sleep to severe failure that can be life-threatening. Care approaches include arranged and precise clinical follow-ups of signs of sleep-disordered breathing, daytime hypoventilation, coughing, and swallowing disturbances . This manuscript will review the mechanisms and abnormalities of respiratory function in patients with NMD and help optimize NMD management., Competing Interests: The authors declare no conflicts of interest.

Objectives: Pharmaceutical allergic reactions due to antiseizure medications (ASMs) are one of the major concerns in the management of patients. Finding an alternative ASM which does not cause allergic reactions and has acceptable effectiveness can be difficult. In this regard, the present study attempts to investigate the cross-reactivity between phenobarbital and levetiracetam in children under treatment for seizure control., Materials & Methods: The present study is a prospective, observational independent assessor study. 30 children with epilepsy who were hypersensitive to phenobarbital therapy were studied. In order to evaluate the cross-reactivity of the drugs, levetiracetam replaced phenobarbital to control seizure. Within 6 months, any allergic reactions and seizure recurrences were evaluated in the patients., Results: 53 % of the children in this study were female. The mean age of patients was 42.4 months. In patients' follow up no cross-reactive responses were observed in any of the patients. Seizure recurrence rate was 30 % in the first six months of follow up that with increasing dosage in the second six months of follow-up, decreased to 10 %., Conclusion: Based on the results of this study, in children with epilepsy controlled by phenobarbital if allergic reactions to phenobarbital occur, levetiracetam may be used as a suitable alternative medicine., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2023 The Authors.)

Objectives: Due to a lack of data on pediatric multiple sclerosis (MS) epidemiology in Iran, this study aimed to determine the incidence rate of pediatric MS in Iran., Materials & Methods:  All the data of the patients with MS registered in the Ministry of Health and Medical Education of Iran for 20 years were collected in this study; those born in 1982 and diagnosed with the disease and treated since 2000 were included in this study. Therefor The collected variables were patients' age at the time of diagnosis, gender, year of diagnosis, urban or rural residency, and province of residence. Additionally, age-specific incidence rates per 100,000 of the population were calculated., Results: This study was performed on 4544 cases of pediatric MS within 2000-2019, of which 997 patients (21.9%) were male. The mean age of the patients with MS at the time of diagnosis was 14.3±4.6 years, and 4414 children (97.1%) lived in urban areas. The incidence rate of pediatric MS in Iran during 20 years increased from 0.26 per 100,000 of the population in 2000 to 1.53 in 2019., Conclusion: The incidence of pediatric MS in Iran is high, and the development of diagnostic practices in the past decade in Iran has contributed to the detection of this high incidence., Competing Interests: None

Objectives: Neuroimaging in high-risk neonates and infants is done to help child neurologists predict the future neurodevelopmental outcome of these children. In this study, we assessed high-risk neonates and infants admitted to the NICU or neonatal wards of Mofid children's Hospital, especially regarding clinical development and brain imaging., Materials & Methods: This cross-sectional study was conducted on 170 patients admitted to the neonatal and NICU ward of Mofid children's Hospital. Considering the inclusion criteria, 112 patients were included in this project. Brain ultrasonography was performed on almost all of these babies by a single radiologist. Some patients underwent a brain CT scan, and brain MRI without contrast was done on the others. These images were interpreted and compared by a single pediatric neuro-radiologist blinded to clinical data. All of these babies were followed up until 18 months of age., Results: In this study, 57.1% of the patients were male and 42.9% were female. Of 44 patients who obtained Electroencephalogram (EEG) during the hospitalization period with probable seizure, 25 (56.8%) had normal EEGs. Of 89 babies who were examined by ultrasound, 19 (21.3%) had abnormal findings; ventriculomegaly and then germinal matrix hemorrhage (GMH) were the most common abnormalities. Also, 27 cases (71.1%) of 38 patients undergoing a CT scan had abnormal findings. The most common findings were a hypodense area in the white matter and ventriculomegaly. Of 41 patients who underwent MRI between 1 and 27 months, 34 cases (82.9%) had an abnormal MRI. The most common findings were periventricular hyperintensities in 17 cases (41.5%), mildly delayed myelination in 15 cases (36.6%), and severe brain atrophy or thinning of corpus callosum or white matter volume loss in seven cases (17.1%). During the follow-up period, which was 18.55 ± 6.56 months, 79 (70.5%) of the children had normal development and 33 (29.5%) were suffering from a global neurodevelopmental delay. More precisely, 49 (43.7%) and 35 (31.2%) patients had motor development delay and delayed verbal development, respectively. The abnormal findings of brain imaging in the ultrasound, CT scan, and MRI were all significantly associated with an adverse neurodevelopmental outcome (P <0.001, P = 0.02, and P <0.001, respectively)., Conclusion: In this study, we showed that at any time before six months or after one year of age, the result of brain MRI was a strong predictor of the patient's outcome., Competing Interests: None, (© 2022 The Authors.)

Objectives: To determine the effectiveness of Rituximab (RTX) therapy as the first therapeutic choice for the long-term prevention of secondary relapse in children with AIND that had relapse after primary treatment with immunosuppressive agents other than RTX., Materials & Methods: We conducted a single-center retrospective study of 9 consecutive pediatric patients (≤ 18 years old) registered on Autoimmune and Demyelinating Disorders Database (ADDD) of Mofid Children Hospital, from 2012 to 2016 and experienced relapse following therapeutic interventions with immunosuppressive agents other than RTX., Result: A remarkable reduction of 94.13% (p=0.015) occurred in annualized relapse rate (ARR) as a clinical indicator of therapeutic efficacy comparing before and after initiating RTX therapy., Conclusion: Rituximab is an effective drug in relapse prevention of AIND when administrated to patients for whom initial treatment with other immunosuppressive agents fail.POWER OF EVIDENCE: This study represents Class IV evidence that RTX therapy significantly reduces ARR in pediatric AIND including DDCNS., Competing Interests: None

Pediatric inflammatory bowel disease (PIBD) is a multisystem disorder characterized by intestinal and extraintestinal manifestations and complications. Cerebrovascular events (CVE) are rare extraintestinal complications in patients with PIBD. Statistics show that 3.3% patients with PIBD and 1.3-6.4% adult patients with inflammatory bowel disease (IBD) experience CVE during the course of the disease. Therefore, this study aimed to review the records of children with IBD who developed CVE during the course of the disease. We retrospectively reviewed 62 cases of PIBD complicated by CVE. The mean patient age at the time of thrombotic events was 12.48±4.13 years. The incidence of ulcerative colitis was significantly higher than that of Crohn's disease (43 [70.5%] vs. 13 [21.3%] patients). Most patients (87.93%) were in the active phase of IBD at the time of CVE. The mean time interval between the onset of IBD and CVE was 20.84 weeks. Overall, 11 (26.83%) patients showed neurological symptoms of CVE at disease onset. The most frequent symptom on admission was persistent and severe headaches (67.85%). The most common site of cerebral venous thrombosis was the transverse sinuses (n=23, 53.48%). The right middle cerebral artery (n=3, 33.34%) was the predominant site of cerebral arterial infarction. Overall, 41 (69.49%) patients who were mostly administered unfractionated heparin or low-molecular-weight heparin (56.09%) recovered completely. Patients with IBD are at a risk of thromboembolism. CVE may be the most common type of thromboembolism. Based on these findings, the most common risk factor for CVE is IBD flares. In patients with CVE, anticoagulant therapy with heparin, followed by warfarin, is necessary., Competing Interests: Conflict of Interest: The authors have no financial conflicts of interest., (Copyright © 2022 by The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition.)

Association between the serum vitamin D level and disability of patients with multiple sclerosis (MS) has been investigated during several researches. However, these studies reported different results. The current study aims to estimate the correlation between the concentrations of 25 (OH) vitamin D and the level of disability among MS patients. Using Mesh and non-Mesh terms related to MS, disability level and vitamin D, different data banks were searched. Required information was extracted from the selected eligible primary articles. Stata version 11 software was applied for combining the primary correlation coefficients using random effect model. The effect of MS type and patients' age was assessed using meta-regression models. Sensitivity analysis was performed to investigate the role of each primary study in the pooled estimate. Egger test was applied to find any publication bias. Of 14 eligible studies, the total correlation coefficient (95% confidence interval) between 25 (OH) vitamin D level and disability in both sexes as well as among female was estimated as of -0.29 (-0.40, -0.17) and -0.35 (-0.46, -0.24) respectively. Two articles carried out among male did not report significant results. Our meta-analysis showed a significant negative correlation between 25 (OH) vitamin D level and disability of MS patients so that the disability reduces with increasing the 25 (OH) vitamin D level., Competing Interests: Conflict of interests: The authors declare no conflict of interest., (© 2021 the Author(s), licensee AIMS Press.)

Competing Interests: The authors report no sources of funding and no conflicts of interest.

This report is about a female child born to a multiple sclerosis mother who continued fingolimod during her first trimester of pregnancy. The child was born with normal full-term delivery but showed neurodevelopmental retardation in the following years. it is recommended that multiple sclerosis patients undergoing treatment with fingolimod use contraceptive methods for at least two months after discontinuation of the medication., (Copyright © 2019. Published by Elsevier B.V.)

Movement disorders are common neurologic disturbances in childhood. There are two major types of movement disorders. Hypokinetic disorders are with paucity of voluntary movements and are very uncommon in pediatric age group. Hyperkinetic movement abnormalities are very common in children and defined as abnormal repetitive involuntary movements. Movement disorders in childhood and even in adolescents are different in etiology, timing, treatment and prognosis versus adulthood movement abnormalities. In this brief article, we reviewed common types of hyperkinetic abnormal movements in children and adolescents with emphasis on etiologies, new classifications and recent treatment strategies., Competing Interests: The authors declare that there is no conflict of interest.

Objective: This study was conducted to predict the response to treatment in patients treated with anti-epilepsy drugs., Material and Methods: This analytical questionnaire-based study was conducted in 2014 among 128 patients with epilepsy admitted to Mofid Children's Hospital, Tehran, Iran. The inclusion criteria were children 2 months to 12 yr of age with epilepsy and patients who experienced fever and seizure attacks at least once were excluded from the study. Patients were followed up for 6 months and the response to their treatment was recorded. The good response to treatment was defined as the absence of seizure with two drugs during follow up., Results: Seventy-two patients (56.3%) were boys. The age of the first seizure was under 2 yr old in 90 patients (70.3%). History of febrile convulsion, family history of epilepsy and history of asphyxia was found in 16 (12.5%), 41 (32%), and 27 (21.1%) patients, respectively. Seizure etiology was idiopathic in 90 patients (70.3%), and the number of seizures was 1-2 in 36 patients (28.1%). Overall, 57 patients (44.5%) had cerebral lesion according to CT scan or MRI, and EEG was abnormal in 101 patients (78.9%). In 6-month follow-up, 40 patients (31.3%) responded well to the treatment and 88 patients (68.8%) responded poorly to the treatment. History of asphyxia (OR = 6.82), neonatal jaundice (OR = 2.81) and abnormal EEG (OR = 0.19) were effective factors in response to treatment., Conclusion: Abnormal EEG is an effective factor in treatment response in the children studied., Competing Interests: The authors declare that there is no conflict of interest.

Objective: Neurometabolic disorder is one of the important groups of diseases that prominently has presentation early infantile period. In this study, we evaluated the result of metabolic screening of the patient with seizure, developmental delay and/or regression in development, demographic disease clinical and radiological findings on admitted and outpatient visited children., Materials & Methods: Two-year retrospective review of 187 children with seizure, developmental delay and/or regression in the Mofid Children Hospital, Tehran, Iran was performed. The diagnosis was based on observation, findings of EEG and history of the patient, besides evaluation of patient milestones. The result of metabolic screening with Tandem mass spectrometry was evaluated using SPSS (ver.18.0) Statistical software., Results: Totally, 187 children with seizure, regression and/or developmental delay were evaluated by metabolic screening with tandem mass spectrometry method. The results of laboratory examination had no relationship between positive results of metabolic screening and the mentioned disease. The relations between positive results of metabolic screening and seizure, regression and/or developmental delay were not statistically meaningful., Conclusion: Positive results of metabolic screening and seizure, regression and/or developmental delay were not statistically meaningful.

Objective: Overall, 2%-5% of patients with multiple sclerosis (MS) experienced the first episode of disease before the age 18 years old. Since the age of onset among children is not similar to that in general population, clinicians often fail to early diagnose the disease. This study aimed to determine the epidemiological and clinical patterns of MS among Iranian children., Materials & Methods: In this cross-sectional study carried out in Iran in 2014-2015, information was collected using a checklist with approved reliability and validity. Method sampling was consensus. Data were analyzed using frequency, mean and standard deviation indices by means of SPSS ver. 20 software., Results: Totally, 177 MS children were investigated. 75.7% of them were female. Mean (SD), minimum and maximum age of subjects were 15.9 (2), 7 and 18 yr, respectively. The most reported symptoms were sensory (28.2%), motor (29.4%), diplopia (20.3%) and visual (32.8%). Primary MRI results showed 91.5% and 53.1% periventricular and spinal cord lesions, respectively., Conclusion: MS is significantly more common among women. The most common age of onset is during the second decades. Sensory and motor problems are the most symptoms, while, periventricular and spinal cord lesions are the most MRI results.

Objective: Several factors are involved in the etiology of febrile seizure (FS), among them is zinc (Zn), which has been discussed in various studies. The present systematic review compares Zn levels in children with FS and a control group., Materials & Methods: We searched keywords of febrile seizure, febrile convulsion, children, childhood, fever, trace elements, risk factor, predisposing, zinc, Zn, and epilepsy in the following databases: SCOPUS, PubMed, and Google Scholar. The quality of research papers was assessed using a checklist. Data was extracted from primary studies based on demographic variables and amounts of Zn in case and control groups., Results: Twenty primary studies were entered in the present study. Of which, eighteen studies, reported that Zn serum levels were significantly lower in the case group (patients with FS) than the control group., Conclusion: The present systematic review indicated that Zn is one factor for predicting FS. A low level of this element among children can be regarded as a contributing factor for FS, a conclusion with a high consensus among different studies carried out in different parts of the world.

Objective: Canavan's disease is a lethal illness caused by a single gene mutation that is inherited as an autosomal recessive pattern. It has many different clinical features especially in the non-Ashkenazi Jewish population., Material & Methods: 45 patients were referred to the Pediatric Neurology Department of Mofid Children's Hospital in Tehran-Iran from 2010-2014 with a chief complaint of neuro developmental delays, seizures, and neuroimaging findings of leukodystrophy were included in this study. Magnetic Resonance Spectrometry (MRS) and neuro metabolic assessment from a referral laboratory in Germany confirmed that 17 patients had Canavan's disease., Results: Visual impairment, seizure, hypotonia, neuro developmental arrest, and macrocephaly were the most consistent findings in the patients in this study. Assessments of neuro developmental status revealed that 13 (76%) patients had neuro developmental delays and 4 (24%) patients had normal neuro development until 18 months of age and then their neuro developmental milestones regressed. In this study, 100% of cases had macrocephalia and 76% of these patients had visual impairment. A history of seizures was positive in 8 (47%) patients and began around 3 months of age with the most common type of seizure was tonic spasm. EEGs were abnormal in all epileptic patients. In ten of the infantile group, we did not detect elevated level of N-acetylaspartic acid (NAA) in serum and urine. However, the MRS showed typical findings for Canavan's disease (peaks of N-acetylaspartic acid)., Conclusion: We suggest using MRS to detect N-acetylaspartic acid as an acceptable method for the diagnosis of Canavan's disease in infants even with normal serum and urine N-acetylaspartic acid levels.

Vigabatrin is an antiepileptic drug that results in higher gamma-aminobutyrate levels in the brain and retina. Vigabatrin-induced visual field defects are usually asymptomatic and only detectable by perimetry. Further, perimetry requires good cooperation, and children aged under 10 years cannot do it. Electroretinogram response amplitude to full-field 30-Hz flicker shine has been offered to be more specific in predicting visual field defects. This study is scheduled to investigate the vigabatrin-associated visual complications in 67 epileptic children taking vigabatrin using full-field electroretinogram. Electroretinographic surveys showed normal range parameters despite 3 months of vigabatrin treatment, and just 3 (4.47%) children had been visually impaired at the end of 6-month treatment. Among these 3 cases, 1 patient had persistent electroretinogram abnormality despite vigabatrin discontinuation. Our study suggests that vigabatrin is secure for short-term pediatric antiepileptic treatment, with few cases of visual impairments and that are often reversible., (© The Author(s) 2013.)

Objective: Approximately one-third of all children with epilepsy do not achieve complete seizure improvement. This study evaluated the efficacy of Vigabatrin in children with intractable epilepsy., Methods: From November 2011 to October 2012, 73 children with refractory epilepsy (failure of seizure control with the use of two or more anticonvulsant drugs) who were referred to the Children's Medical Center and Mofid Children's Hospital were included in the study. The patients were treated with Vigabatrin in addition to their previous medication, and followed-up after three to four weeks to determine the daily frequency, severity, and duration of seizures in addition to any reported side effects., Findings: Of the 67 children, 41 (61.2%) were males and 26 (38.8%) females, their age ranging from three months to 13 years with an average of 3.1 [standard deviation (SD), 2.6] years. The mean daily frequency of seizures at baseline was 6.61 (SD, 5.9) seizures per day. Vigabatrin reduced the seizure frequency ≤2.9 (SD, 5.2) (56% decline) and 3.0 (SD, 5.3) (54.5% decline) per day after three and six months of treatment, respectively. A significant difference was observed between seizure frequencies at three (P<0.001) and six months (P<0.001) after Vigabatrin initiation compared with the baseline. Somnolence [3 (4.5%)], horse laugh [1 (1.5%)], urinary stones [1 (1.5%)], increased appetite [1 (1.5%)], and abnormal electroretinographic pattern [3 (4.5%)] were the most common side effects in our patients., Conclusion: This study confirms the short-term efficacy and safety of Vigabatrin in children with refractory epilepsies.

Objective: Approximately one third of epileptic children do not achieve complete seizure improvement. Zonisamide is a new antiepileptic drug which is effective as adjunctive therapy in treatment of intractable partial seizures. The purpose of the current study was to evaluate the effectiveness, safety, and tolerability of Zonisamide in epileptic children., Materials & Methods: From November 2011 until October 2012, 68 children who referred to Children's Medical Center and Mofid Children Hospital due to refractory epilepsy (failure of seizure control with the use of two or more anticonvulsant drugs) entered the study. The patients were treated with Zonisamide by dose of 2- 12 mg/kg daily in addition to the previous medication. We followed the children every three to four-weeks intervals based on daily frequency, severity and duration of seizures. During the follow-up equal and more than fifty percent reduction in seizure frequency or severity known as response to the drug., Results: In this study 68 patients were examined that 61 children reached the last stage.35 (57.4%) were male and 26 (42.6%) patients were female. After first and six months of Zonisamide administration daily seizure frequency decreased to 2.95±3.54 and 3.73±3.5 respectively. There was significant difference between seizure frequency in first and six month after Zonisamide toward initial attacks. After six months ZNS therapy a little side effects were created in 10 patients (16.4%) including stuttering(4.9%), decreased appetite (4.9%), hallucination (1.6%), dizziness(1.6%), blurred vision(1.6%) and suspiring(1.6%) as all of them eliminated later dosage reduction., Conclusion: This study confirms the short term efficacy and safety of Zonisamide in children with refractory epilepsies.

Objective: Guillain-Barre syndrome (GBS) is an immune-mediated polyneuropathy that occurs mostly after prior infection. The diagnosis of this syndrome is dependent heavily on the history and examination, although cerebrospinal fluid analysis and electrodiagnostic testing usually confirm the diagnosis. This is a retrospective study which was performed to investigate the atypical features of GBS., Materials & Methods: Thirty three patients (21/63.6% males and 12/36.4% females) with GBS were retrospectively studied and prospectively evaluated at the Child Neurology institute of Mofid Children Hospital of Shahid Beheshti University of Medical Sciences between May 2011 and September 2012., Results: The mean age was 5.4 years (range, 1.5-10.5).Twenty one patients (87.9%) had previous history of infections. Eight patients (24.2%) admitted with atypical symptoms like upper limb weakness (3%), ptosis (3%), neck stiffness (3%), inability to stand (proximal weakness) (9.1%), headache (3%) and dysphagia (3%).According to disease process, weakness was ascending in 26 (78.8%), descending in 5 (15.2%) and static in 2 (6.1%) patients. Cranial nerve involvement was found in 8(24.3%) children, most commonly as facial palsy in 3 (9.1%)., Conclusion: In this study, 24.3% of our patients presented with atypical symptoms of GBS as upper limb weakness, ptosis, neck stiffness, inability to stand (proximal weakness), headache and dysphagia.

Chronic hepatitis B virus (HBV) infection is a major liver disease worldwide and its clinical manifestations are linked to immune response. The purpose of this study was to evaluate the relationship between selenium, copper, and zinc in comparison with transaminase level in chronic HBV patients. Serum samples of the HBV infected patients were obtained from Tooba medical center, Sari, Iran. Sixty patients were enrolled in this study (36 men and 24 women), mean age: 39.6 ± 12.2 years. The concentration of zinc, selenium, copper and transaminases were determined using an autoanalyzer system. Concentrations of selenium (0.273 ± 0.056 μg/dl) and zinc (2.1 ± 0.037) was elevated in patients with low transaminase levels as were significantly different in comparison with patients with high transaminase level (P<0.05). Serum copper concentration was similar in two groups of patients. Elevated levels of transaminase concentrations were independently associated with low zinc and selenium concentrations in chronic HBV patients. It is concluded that serum zinc and selenium levels are associated with less hepatic damage in chronic HBV patients and might have a protective role during liver injury.

Prevention programs are considered to be a top priority in Iran because beta-thalassemia (beta-thal) major (TM) is the most common autosomal disorder in Iran, and in the Mazandaran Province in particular. The main strategies comprise providing appropriate information for the public and professionals, screening and counseling of families at-risk and screening of general population prior to marriage. Providing laboratories for prenatal diagnosis was the most recent step in the program. We report the results of our prevention campaign for the period 1993-2006 in order to assess the effectiveness of all actions in controlling thalassemia major. In 1993, 500 TM patients were registered at the clinic of the Boo Ali Sina Hospital, Sari, Mazandaran, Iran. From 1993 to 1996, on average of 50 new cases were added to the cohort annually, whereas from 1995 to 2005 the number of new cases declined to 35 per year. Furthermore, the patients' average age increased. Overall, 51% of couples at-risk, who received genetic counseling, decided not to marry. All at-risk couples who are married were counseled for prevention of unplanned pregnancies. Currently, 64% are using safe contraceptive methods of family planning, and 14% are no longer at-risk for further pregnancies, the rest remained at-risk for unplanned pregnancies. In conclusion, at the relatively low cost of premarital screening and genetic counseling, we have offered at-risk couples the possibility of preventing the birth of at least 600 undesired TM patients. Thus, a great deal of suffering and an unbearable financial burden has been prevented to patients and their families.