Showing papers in "Iranian journal of child neurology in 2020"

Bilirubin Induced Encephalopathy.

Abstract: Hyperbilirubinemia is one of the most common neonatal disorders. Delayed diagnosis and treatment of the pathologic and progressive indirect hyperbilirubinemia lead to neurological deficits, defined as bilirubin induced encephalopathy (BIE) (2). The incidence of this disorder in underdeveloped countries is much more than developed areas. All neonates with the risk factors for increased the blood level of indirect bilirubin are at risk for BIE, especially preterm neonates which are prone to low bilirubin kernicterus . BIE can be transient and acute (with early, intermediate and advanced phases)or be permanent, chronic and lifelong ( with tetrad of symptoms including visual (upward gaze palsy), auditory (sensory neural hearing loss), dental dysplasia abnormalities, and extrapyramidal disturbances (choreoathetosis cerebral palsy).Beside the abnormal neurologic manifestations of the jaundiced neonates ,brain MRI is the best imaging modality for the confirmation of the diagnosis. Although early treatment of extreme hyperbilirubinemia by phototherapy and exchange transfusion can prevent the BIE, unfortunately the chronic bilirubin encephalopathy does not have definitive treatment.

Emotional and Behavioral Disorders in Pediatric Cancer Patients.

Abstract: Objectives Childhood malignancies raise a range of medical, psychological and social concerns. Identifying psychiatric disorders along with providing mental health services to prevent the emergence and aggravation of mental health problems in children seems necessary in pediatric hospitals. We aimed to find out the frequency of probable emotional and behavioral disorders among children and adolescents with malignancy. Materials & Methods This was a cross sectional study conducted at Hematology-Oncology Ward of Mofid Hospital, Tehran, Iran in 2017-2018. Emotional and behavioral disorders was assessed in 399 pediatric patients with malignancy, aged 5 to 12 years, using the Parent Checklist of CSI-4. All data analyses performed using the Stata 14 at 95% sig-value. Results: In all, 89.2% of sample met diagnostic criteria for at least one disorder. The most prevalent psychiatric disorders were special phobia (57%) enuresis (41.9%) Obsessive-Compulsive disorder (45.6%) and Separation Anxiety disorder (30.3%). Our results did not show significant gender and disease type differences in prevalence of psychiatric disorders. Conclusion: Prevalence of emotional and behavioral disorders in pediatric patients with malignancy admitted in the children’s hospital is common. These disorders affect on the treatment and quality of life of pediatric patients. Therefore, these findings may guide parents, nurses and clinicians to become more careful about identification and management of them.

Relationship Between Parental Communication Patterns and Self-Efficacy in Adolescents with Parental Substance Abuse

Abstract: Objectives Since an individual’s personality and behavior are mainly shaped in the family environment, parental substance abuse and its consequences can lead to confusion and instability in the family environment and reduce child-parent relationship. This study was conducted to investigate the relationship between different aspects of parental communication patterns and self-efficacy in adolescents with parental substance abuse. Materials & Methods In this descriptive correlational study, a total of 100 adolescents with parental substance abuse were selected via random sampling. The data collection tools included the parental communication patterns scale and self-efficacy questionnaire. Data were analyzed in SPSSversion 20, using linear regression analysis and Pearson’s correlation coefficient test. Results The mean age of the adolescents was 14.5±2.5 years. Pearson’s correlation coefficient showed that the adolescents’ self-efficacy score was directly correlated with the score of conversation orientation dimension (a parental communication pattern) and inversely correlated with the dimension of conformity orientation (P<0.001). Conclusion: Development of educational programs to promote communication skills, positive family interactions, decision-making, and flexibility can change the unhealthy family environment into a healthy and productive one, which promotes self-efficacy beliefs in children.

Sensory Processing Patterns and Sleep Quality in Primary School Children

Abstract: Objectives: Sensory processing and sleep quality affect children's academic performance and their quality of life. The purpose of this study was to investigate the relationship between sensory processing patterns and sleep quality in primary schoolchildren.Materials & Methods: In this cross-sectional study, 231 primary school students aged 7 to 12 years old (133 girls and 98 boys, mean age of 8.68±1.51) who were studying in schools in Tehran were randomly selected through cluster sampling. Researchers distributed a questionnaire for children's sleep habits to assess the quality of sleep and a Sensory Profile Questionnaire to assess the sensory processing patterns.Results: In this study, we found a meaningful moderate relationship between sensory processing patterns and the general scores of sleep habits (p <0.001) and each of the patterns of sensory processing (avoidance, sensitivity, seeking, and registration) had a negative relationship with areas of sleep habits (p =.005). Also, there was a significant difference between children who had more challenges with sleep and children with normal sleep patterns in sensory processing; mean differences in all four sensory quadrants were significant (p <001).Conclusion: Sensory processing patterns and sleep habits in primary school children have moderate correlation. Occupational therapists should consider the relationship between sensory challenges and sleep habits during their practice decisions with sensory challenges and sleep problems. Better sleep may occur with attention to sensory needs within the sleep routines. When sleep is better, this may lead to improved quality of life for the family and student performance at school.

Efficacy of Topiramate Alone and Topiramate Plus Vitamin D3 in the Prophylaxis of Pediatric Migraine: A Randomized Clinical Trial

Abstract: Objective Topiramate is effective in the prevention of pediatric migraine, and studies show that vitamin D3 supplementation might also be useful in the treatment of adult migraineurs with a normal vitamin D3 level.The present study aimed at comparing the efficacy and safety of topiramate plus vitamin D3 and topiramate alone in the prophylaxis of pediatric migraine. Materials & Methods In a single-blinded, randomized, clinical trial, 5-15-year-old children with migraine headaches, referred to the Pediatric Neurology Clinic of Shahid Sadoughi Medical Sciences University, Yazd, Iran from January 2016 to January 2017, were randomly allocated to receive 2 mg/kg/day of topiramate or 2 mg/kg/day of topiramate plus one 500,000 IU vitamin D3 pearl weekly for two consecutive months. Primary outcomes were the reduction of monthly frequency, severity, duration, and the disability score of migraine, and the secondary outcomes included a good response to treatment (more than 50% reduction in monthly headache frequency) and a lack of clinical adverse events. Results Totally, 31 female and 26 male children with the mean age of 10.02±2.11 years were evaluated. Both drugs were effective in the reduction of monthly frequency, severity, duration, and disability for headaches. Nevertheless, the combination of topiramate and vitamin D3 was more effective than topiramate alone in reducing the monthly headaches frequency (6.12±1.26 vs. 9.87±2.44 times, P=0.01) and disability score (19.24±6.32 vs. 22.11±7.91, P=0.02). Good response to treatment was observed in 60.7% and 75.9% of the subjects in the topiramate alone and topiramate plus vitamin D3 groups, respectively, and topiramate plus vitaminD3 was more effective (P= 0.01). Transient mild side effects were observed in 14.3% and 17.2% of the subjects in the topiramate alone and topiramate plus vitamin D3 groups, respectively (P=0.8). Conclusion A combination of Topiramate and vitamin D3 might be considered safe and more effective than Topiramate alone in the prophylaxis of pediatric migraine.

Attention Deficit Disorder with Hyperactivity Symptoms in Early-Treated Phenylketonuria Patients.

Abstract: ObjectivesTo assess the presence of symptoms consistent with Attention Deficit Disorder with Hyperactivity (ADHD) in all patients with early-treated phenylketonuria (PKU) in the State ofSanta Catarina in southernBrazil.Materials & MethodsAll of the patients diagnosed with PKU by newborn-screening tests, with ages varying from 6 to 18 years and who started treatment before 60 days of life and presented phenylalanine levels consistently below 6 mg/dL throughout treatment, were included. The subjects were invited to complete a questionnaire that collected sociodemographic, gestational and clinical data. ADHD symptoms were assessed using the revision of the Swanson, Nolan and Pelham Questionnaire.Results: A total of 34 patients were evaluated, who were 53% male and 94% white and had an average age of 12 years, and 15% were born premature. According to the Swanson, Nolan and Pelham Questionnaire, 13 patients (38%) met the diagnostic criteria for ADHD, with 2 patients having the inattentive type, 6 patients having the hyperactive or impulsive type and 1 patient having the oppositional defiant disorder type.Conclusion:Although the patients with PKU were regularly treated from birth, there was a high prevalence of symptoms consistent with ADHD. A pathophysiological interface that involves the dopamine metabolic pathway may exist between the two conditions.

Social Determinants of Health, Maternal Involvement, and Child Development: Direct and Mediated Pathways.

Abstract: Objectives In the process of child development, a variety of factors are at play. In this regard, social determinants of health play a determining role in the development and growth of the child. This study aimed to design and test the model for social determinants of health for the development of 36-6o-month-old children in Tehran with the mediation of maternal Involvement. Materials & Methods This cross-sectional study was conducted among 1067 mothers and their 36-60-month-old children in childcare centers in Tehran, using multistage sampling. Data gathering tools consisted of a demographic questionnaire for mothers and children, a questionnaire on unhealthy behaviors, Ages and Stages Questionnaire, Economic and Social Status Questionnaire, Perceived Social Support Questionnaire, Perceived Stress Questionnaire, Spielberger Anxiety Inventory, Beck Depression Inventory, ENRICH: Marital Satisfaction Scale, and Participation Scale for Parents and Mothers. Results Model fit measures were suitable and goodness of fit (RMSEA = 0.031, GFI = 1) was satisfactory. In addition, the results of path analysis indicated that the participation of mothers in the development of children had a direct positive (s = 0.089) and increasing effect. Conclusion Findings indicated that depression, anxiety, stress, and marital satisfaction have both direct and indirect effects on the participation of mothers and child development. Moreover, the model fit measures indicated the utility and high proportionality of the model, as well as the logic of the adjusted relationships of variables based on theconceptual model.

The Impact of Antiepileptic Drugs on Thyroid Function in Children with Epilepsy: New Versus Old.

Abstract: Objectives To investigate the effects of traditional antiepileptic drugs (AEDs) versus newer AEDs on the thyroid hormone profile of children with epilepsy. Materials & Methods A total of 80 children with epilepsy were included in this study and were divided into two groups. Group 1 included 40 children with epilepsy on traditional AEDs, and group 2 included 40 children with epilepsy on newer AEDs. Forty healthy children were also included as the control group (group 3). We analyzed the serum levels of free triiodothyronine (FT3), free thyroxine (FT4), and thyroid stimulating hormone (TSH). Results: Epileptic children treated with traditional drugs showed a statistically significant decrease in the serum fT4 and increase in TSH concentrations compared to the control group ( P < 0.001). Whereas epileptic children treated with newer drugs showed no statistically significant changes in the serum fT3, fT4 and TSH concentrations compared to the control group Conclusions: Traditional AEDs have a significant impact in the thyroid hormone profile compared to the newer AEDs.

Effect of Midazolam Alone Versus Midazolam with Maternal Presence on Pain and Anxiety of Lumbar Puncture in 6 to 24-Month-Old Children.

Abstract: Objectives Midazolam at a dosage of 0.51 mg/kg induces anxiolytic effects in 90% of children. This study was performed to elucidate whether intravenous midazolam with maternal presence is more efficient than intravenous midazolam alone in the reduction of pain and anxiety of lumbar puncture (LP) in 6 to 24-month-old children. Materials & Methods In this not-blinded clinical trial, we included 60 children aged 6 to 24 months old undergoing LP in the Pediatric Ward of Shahid Sadoughi Hospital, Yazd, Iran, from September 2014 to March 2015. The participants were randomly assigned to two groups, and all of them received painless injection of 0.5 mg/kg midazolam five minutes before LP. In group I, LP was performed with maternal presence and in group II, the mothers were absent. The primary outcomes included anxiety and pain scores before LP and during needle insertion to the skin for LP. The secondary outcomes comprised of success rates in the reduction of anxiety (anxiety score of four and more) and pain (pain score of less than three) when the needle was inserted to the skin for LP. Results Twenty-eight girls and 32 boys were evaluated in the two groups. Maternal presence was found to be effective in the reduction of anxiety (2.7±0.65 vs. 3.83±0.87; P=0.001) and pain scores (3.8±1.75 vs. 6.1±1.63, P=0.001). In the maternal presence group, success rate in anxiety (76.7% vs. 16.6%; P=0.0001) and pain reduction (63.3 % vs. 6.7%; P=0.0001) was higher than in the midazolam alone group. Conclusion Maternal presence during lumbar puncture can reduce pain and anxiety among 6 to 24-month-old children.

Predictors of Leisure Participation in 6 to 14-Year-Old Children with Cerebral Palsy: Structural Equation Modeling.

Abstract: Objective: The aim of this study was to test a model of child,family and environment and identify factors affecting intensity of leisure participation by children with cerebral palsy (CP).Materials & Methods: in this cross-sectional study,232 children with cerebral palsy (141 boys and 91 girls), age range 6 to 14 years and their parents were selected from 4 schools with special needs and 5 rehabilitation center through available sampling in Shiraz city. To evaluate leisure participation, we used the Persian version of Children’s Assessment of Participation and Enjoyment (CAPE) completed by participants. Demographic questionnaires, Craig Hospital Inventory of Environmental Factors (CHIEF), Strengths and Difficulties Questionnaire (SDQ), Family Environmental Scale (FES), SPARCLE cognitive level and parent's version of Gross Motor Function Classification System, Manual Ability Classification System and Communication Function Classification System were send to parents with some necessary explanations and completed. Structural equation modeling was used to test the model hypothesis. SPSS18 and AMOS16 were used for statistical data analysis. Results: Comparative fit index indicated a moderate to good fit model. The presented model explains 44% of the variance for intensity of participation. Constructs such as Family Activity Orientation with standardized total effect of (.31) and path coefficients P .05).Conclusion: Intensity of CP children participation is influenced by child, family and environmental factors. Parent's knowledge of recreational activities and their preferences to participation in leisure and recreations give children more opportunities to participate. higher gross motor function, manual ability, and communication function have an important role in participation. Family structure means family cohesion, roles organization, and conflicts between family members and also encountering physical, attitudinal and structural barriers in the home and community change children participation pattern indirectly. This study suggests therapists to support children’s behavior, family relationships and involvement in community activities and optimizing physical function for children who have limitations in self-mobility to enhance their participation.

Effects of Developmental Care on Neurodevelopment of Preterm Infants.

Abstract: ObjectivesThe aim of this study protocol is to systematically review the literature to examine the effects of developmental care on preterm infants’ neurodevelopment in the neonatal intensive care unit (NICU).Methods: We will retrieve the studies through searching the following databases: Web of Science, PubMed, EMBASE (Ovid), Cochrane Central Register of Controlled Trials (CENTRAL), CINAHL (EBSCO), and Scopus.We will include randomized controlled trials (RCTs) with randomization at either an individual or cluster level. Our primary outcome will be the effect of developmental care in NICU neonates on their mental and motor development. The secondary outcome will be weight gain in NICU and hospital stay length. The assessment tool of development should be The Bayley Scales of Infant and Toddler Development, in any of the first, second and third edition. This study will employ Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) to identify relevant articles and report the screening process. The agreement between two experts in developmental neonatology will be reached in all selecting studies. Two reviewers will be achieved data extraction and then they will be compared. Any discrepancies between the extracted data will be discussed to reach a consensus. The extracted data will be imported to Review Manager 5.3 by one reviewer. Two reviewers will independently evaluate the risk of bias for all included studies using the Cochrane Collaborations’ tool for assessing risk of bias in randomized trials.We will conduct a meta-analysis for assessing the possible quantitative impact of developmental interventions on interested primary and secondary outcomes. We will use random effect if the I-square statistics is equal or more than 75%, and fixed effect otherwise. Publication bias will be assessed using Egger’s test and illustration with the funnel plot. We will estimate the Standardized Mean Difference (SMD) with 95% confidence interval through Metan command in STATA 14. The method provided by the Cochrane hand book, will be used in this statistical analysis. The significance level for assessing these analyses will be 0.05. Discussion: According to the findings of this systematic review, by abstracting the results and presenting the conclusions, improvements in interventional decisions can be improved, and results of this systematic review will highlight which clinical practice could possibly be optimized to improve outcome and specifically neurodevelopment in this vulnerable population.

Autistic feature as a presentation of Inborn Errors of Metabolism.

Abstract: Autism spectrum disorder (ASD) is a category of neurodevelopmental disorders characterized by social and communication impairment and restricted or repetitive behaviors. The pathogenesis of ASD is not well understood and it's proved that genetic is strongly associated with ASD in 5 to 25% of cases. Inborn errors of metabolism(IEMs), defined by a vast array of disorders that are caused by specific enzyme deficiencies or transport protein defects, is as frequent as in 1 in 800 births. IEMs can manifest several psychiatric or behavioral manifestations such as self-injuriesincreased activity and aggression, personality changes, paranoia, depression, catatonia, and psychosis. IEMs underlie autistic symptoms in less than 5% of cases. The literature on the association between ASD and respiratory chain abnormalities is growing, including complex III/IV deficiency and MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes) syndrome, as well as glucose-6-phosphate dehydrogenase deficiency. Google Scholar, Pubmed, and SCOPUS databases were searched using a combination of the following keywords: "autism spectrum disorder", "autism spectrum", "autistic feature" and "inborn error of metabolism", " IEM", "congenital error of metabolism". Initially, 655 articles were found and our expert and methodologist altogether selected 187 articles based on the titles, relevance, and text language. After reading full texts, 37 studies were selected for review. We think it's best to consider IEMs in children with syndromic ASD and/or if there is a strong familial history of autism or parental consanguineous marriage.

Levetiracetam (levebel) Versus Carbamazepine Monotherapy for Focal Epilepsy in Children: A randomized clinical trial.

Abstract: Objective This study aimed at comparing the effect of a newly approved drug leveitiracetam (LEV) versus carbamazepine (CBZ) in the treatment of childhood focal epilepsy. Methods & materials The study population included newly diagnosed children with focal epilepsy (1-16 years old) referring to the Pediatric Neurology Ward of Quaem Hospital, Mashhad, Iran from May 2013 to March 2014. The subjects were randomly treated with LEV or CBZ. Patients were followed for seizure control and drug side effects throughout six months. We assessed liver function and complete blood count for all patients through one month and they were asked about significant side effects, such as drowsiness، restlessness, and skin reaction. Eventually, they were assigned in two groups (n=25) receiving LEV and CBZ. Results In our study, two cases in the LEV group were excluded because of severe agitation. Relapsing seizures were observed in 3 (13%) and 10 (40%) patients in LEV and CBZ groups, respectively. The seizure was not repeated in 15 cases (60%) in the CBZ group and 20 cases (87%) in the LEV group. The results of the Chi-squared test showed significant differences in the responses to treatment between the groups (P=0.03). Agitation was the most prevalent complication in the LEV group, whereas drowsiness was more common in the CBZ group. Fortunately, liver enzyme dysfunction and blood cell disturbances were not observed in the subjects. Conclusion According to the findings, there were significant differences in controlling seizures between two groups that indicated the effectiveness of LEV (87%) in the suppression of focal seizure.

A Predictive Model for Respiratory Failure and Determining the Risk Factors of Prolonged Mechanical Ventilation in Children with Guillain-Barre Syndrome.

Abstract: Objective: Determining the predictors of respiratory failure and duration of intubation in children with Guillain-Barre Syndrome (GBS).Material and methods: Children diagnosed with GBS at Tabriz Children’s Hospital were studied. Factors associated and influencing respiratory failure and duration of intubation were determined using both univariate and multiple analysis.Results: Three hundred twenty-four children were enrolled in the study, of whom 54.0% were males. Thirty-one (9.6%) patients underwent mechanical ventilation, the patients under 5 years old were more prone to requirement of mechanical ventilation (11.3% vs. 6.9%). The cases who are hospitalized in winter were more likely to need ventilation compared to the cases hospitalized in the spring (OR =7.00 ; 95% CI:1.51 - 32.53 ). Also autonomic involvement (OR= 8.88 ,95% CI :4.03 - 19.58 ,p<0.001) and cranial nerves involvement (OR =9.88 ,95% CI:3.68 - 26.52, p<0.001) emerged as risk factors for mechanical ventilation requirement.Overall, 16.1% of patients with axonal electrophysiologic pattern required mechanical ventilation compared to 7.4% among those with demyelinating type, (OR:2.15, 95% CI: 1.01 - 4.69). In univariate analysis, the only variable which has a correlation with the duration of intubation is axonal electrophysiologic pattern p= 0.028.Conclusion: Approximately 10% of patients required mechanical ventilation. Season, cranial nerve involvement, autonomic dysfunction, and the electrophysiologic pattern is more important variables for prediction and duration of respiratory failure and mechanical ventilation.

Comparison of the effect of high and low doses of adrenocorticotropic hormone (acth) in the management of infantile spasms.

Abstract: Introduction:Infantile spasmscan have irrecoverable adverse effects on a child’s brain. Adrenocorticotropic hormone (ACTH) is the most common first line medication for its treatment; however, the suitable dose and duration of treatment continue to be debated among specialists. The common high doses of this drug can produce more side effects; thus, lower doses are preferred. The aim of this study is to determine the effect and extent of complicationscaused by high and low doses of ACTH for children suffering from infantile spasms.Materials and methods:This study is a clinical trial in which 32 infants from 1.5 and 18 months of age suffering from infantile spasms were divided into high and low dose ACTH groups. Treatment lasted for two months. The therapeutic effects and their complications were then compared over the course of 18 months.Results:The results indicate thatthere were no significant differences between groups for short-term prognosis of controlling convulsions, final prognosis of patients for spasm relapse, EEG changes after treatment, post-treatment evolution and development of hypertension. However, there was a significant difference in the frequency distribution for intensity of restlessness and becoming Cushingoid, which were more frequent in the high dose group.Conclusion:These results indicate that high and low dose ACTH are equally effective in terms of controlling spasms, yet the low dose brings about fewer side effects.

Serum Level of Vitamin D and Febrile Seizure? A Clinical Study.

Abstract: Objective:To evaluate the serum level of vitamin D in children aged six to 60 months with febrile seizure and febrile children without the seizureMaterials & MethodsFebrile children aged six to 60 months with or without seizure were studied. Demographic characteristics, serum level of vitamin D, and other laboratory findings were recorded.ResultsAmong the 104 children, 51 patients had fever without a seizure and 53 patients had a febrile seizure. The mean subjects’ age was significantly more in the febrile seizure group compared to the without seizure group (16.26 ± 11.87 versus 26.36 ± 14.11 months, p = 0.001). The mean serum level of vitamin D in the with and withouts eizure groups was 41.92 ± 22.42 and 48.41 ± 15.25 microgram perd eciliter, respectively (p = 0.08). There was no significant correlation between serum level of vitamin D and seizure occurrence (p = 0.07).The mean serum sodium and potassium levels, and platelet count were significantly lower in the febrile seizure group compared to the without seizure group (p 0.05).ConclusionThe serum level of vitamin D in febrile children with or without seizure was normal. The serum level of vitamin D was lower inp atients with the seizure but not statistically significant. More clinical studies are needed to evaluate the relationship between febrile seizurea nd the serum level of vitamin D.

"Neonatal seizure: how reliable is its diagnosis and treatment? A mini review of previous knowledge".

Abstract: Seizure is the most common neurologic event in the neonatal period when the immature, growing brain is vulnerable to various injuries. Seizure might be present as an atypical feature in neonates, which makes diagnosis a challenge. A vast majority of seizures are symptomatic due to an underlying cause, searching for etiology to remove them leads to more effective therapy. However, there is doubt about the diagnosis of seizures and the best treatment for them. Here, we reviewed articles related to diagnosis and treatment of neonatal seizures to evaluate the available evidence. The results showed that despite numerous studies on the topic, neither an implicit diagnosing method nor a therapeutic regime was proposed. It was also observed that phenobarbital prescription was reduced while newer anti-seizure medication such as levetiracetam was further prescribed. Seizure, the most common neurologic disorder in neonates, is a challenging topic for both neonatologists and neurologists. Since patients are critically ill, designing a randomized clinical trial appears not to be easy for neonates admitted to a neonatal intensive care unit. Moreover, both the diagnosis and treatment of seizures should be re-evaluated based on neonatal characteristics. In the recent decade, new less harmful anti-seizure medications are being replaced with old ones.

A Rare Case Report of Acute Necrotizing Encephalopathy of Childhood

Abstract: Background: Acute Necrotizing Encephalopathy in childhood (ANEC) is a kind of fast growing illness accompanied with progressive Encephalopathy. The aim of this article is to report a rare case of ANEC in a 4-year-old boy with bilateral thalamic necrosis and a nonfatal outcome. Case Report: The patient was a 4-year-old Iranian boy who had no prior history of health problems and hospitalization except for a period of jaundice and phototherapy as a neonate. In the neurological consultation a brain MRI was requested for the patient to analyze the possibility of brain damage, which showed the involvement of cerebellum, thalamus and the existence of basal ganglia which led to the diagnosis of ANEC. Conclusion: In conclusion, although ANEC is a rare disease, it should not be underestimated.

Non-progressive Non-immune Hydrops Fetalis Caused by a Novel Mutation in GUSB Gene

Abstract: Mucopolysaccharidosis type VII or Sly syndrome is a rare autosomal recessive disorder caused by deficiency of β Glucuronidase enzyme, which is involved in degradation of glycosaminoglycans. The lack of β Glucuronidase in this lysosomal storage disorder is characterized by various manifestation such as non-immune hydrops fetalis, spinal deformity, organomegaly, multiplex dysostosis, intellectual disability, and eye involvement. It has been found to be caused by a mutation in GUSB gene on chromosome 7 q11. Here we reported an Iranian girl with Mucopolysaccharidosis type VII and a novel mutation (C. 542G>T, P.Arg181Leu) in GUSB gene.

Increasing Mothers' Confidence and Ability by Creating Opportunities for Parent Empowerment (COPE): A Randomized, Controlled Trial

Abstract: Objective: Premature neonates are at great risk for cerebral palsy, developmental delays, hearing problems and visual impairments. Interventions to reduce the morbidities and adverse health outcomes in these neonates and improve parent-infant interaction are highly important. This study was conducted to determine the effect of the Creating Opportunitiesf or Parent Empowerment (COPE) program on the perceived mat ernal parenting self-efficacy of premature parents Materials and Methods: This was a randomized controlled trial with equal randomization (1:1:1 for 3 groups) and parallel group design. Forty-five preterm neonates were randomly allocated into treatment (n=15), supervision (n=15), and control (n=15) groups. COPE program was provided for a 4-phase educational-behavioral intervention program to the treatment and supervision groups. Primary outcome was parental self-efficacy, which was assessed with the Perceived Maternal Parenting Self-Efficacy . All measurements were taken before and after completion of the study with the valid equipment and by the blind assessors. Results: COPE mothers reported significantly stronger beliefs regarding their parental role and have more confidence to their ability in caring of neonates compared with control mothers ( P -value <0.001). Conclusion : An educational-behavioral intervention program would strengthen mothers’ beliefs and knowledge about their neonates and would remove barriers to enhance their ability for caring of neonates and enhance parent-infant interaction.

Assessment of anthropometric indices in patients with phenylketonuria

Abstract: Background: Dietary phenylalanine restriction is the main treatment of phenylketonuria (PKU, OMIM 261600). There are studies which have demonstrated growth retardation in these patients, and some are in contrast. This study is done to assess the growth parameters of treated PKU patients. Method : 105 treated PKU patients were compared to 105 controls matched in age, sex and birth weight. Weight, height, head circumference, weight for height and BMI (weight/height2) were measured and transformed into Z-scores. Correlations between pretreatment plasma Phenylalanine concentrations mean plasma Phenylalanine concentrations, and anthropometric parameters were analyzed in patients. Findings: There was no significant difference between weight Z-scores, weight/height and BMI means, in patients and controls (P: 0.842, p: 0.257, p: 0.622 respectively). There was a significant decrease in height Z-scores of patients more than 2 years old (P: 0.005). Also, there was a significant decrease in patient's Head circumference than controls'(P: 0.03), however this significance got more in patients above 3 years old (P: 0.001). There was not a significant correlation between weight and height Z-scores, and patients' pretreatment plasma phenylalanine (P: 0.054 and 0.15), however this correlation was positively significant in HC Z-scores (r= -0.445, P: 0.01).Moreover,no correlation was found between growth parameters and mean plasma phenylalanine concentrations. Conclusions: Growth parameters are not impaired in early treated PKU patients; however height in patients more than 2 years old and Head circumference in patients more than 3 years old were retarded. Disease severity is a more effective factor in HC growth than dietary adherence.

Evaluation of the Quality of Life in Epileptic Children of Shiraz, Southern Iran.

Abstract: Introduction: People suffer chronic disease like epilepsy are highly prone to debilitating changes in factors that affect the quality of life such as physical capacity, self-esteem, relationships with others and fulfillment of their daily life activities. In this study, we decided to evaluate the quality of life in children with epilepsy in Shiraz, South Iran. Methods: Epileptic patients referred to epilepsy clinic of Shiraz University of Medical Sciences and had no first time episode of seizures in the previous 6 months and no febrile-seizure were included in the study. Patients were evaluated using the standard KIDSCREEN-27 questionnaire. Data were analyzed using the statistical software SPSS 21, Man Whitney and Chi-square tests and reported in terms of descriptive statistics. The significance level was considered less than 0.05. Results: In this case-control study, 229 children with epilepsy were compared with a control group of 400 normal individuals. The mean age was 12.44±3.16 and 12.10±2.69 years. The tonic-clonic seizure had the highest prevalence . Being a boy, older age and having more seizures per year were associated with lower quality of life; in general, epileptic children had significantly lower QOL compared to normal cases. Conclusion: In general, epileptic children had an overall lower QOL while factors such as older age, male gender, and higher number of seizures over the years reduced the quality of life of these patients.

Three Cases of Acute Necrotizing Encephalopathy: Is It an Epidemic or Only Incidental?

Abstract: Acute necrotizing encephalopathy of childhood (ANEC) is a disease, characterized by a respiratory or gastrointestinal infection, accompanied with fever, rapid alteration of consciousness, and seizures. The clinical characteristics of ANEC include acute encephalopathy following a viral infection, seizure, altered consciousness, and absence of cerebrospinal fluid (CSF) pleocytosis, with an occasional increase in the level of proteins. This disease is almost exclusively seen in previously healthy infants and children from East Asia. Serial magnetic resonance imaging (MRI) examinations have demonstrated symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. ANEC has a poor prognosis with high morbidity and mortality rates. Herein, we present three cases of ANEC, who were referred to Bu-Ali Hospital of Ardabil, Iran during two weeks. Report of these three cases promoted the idea of an epidemic. The purpose of this case series was to raise the issue that ANEC may occur as an epidemic.

Approach to Patients with Neurometabolic Diseases Who Show Characteristic Signs and Symptoms.

Abstract: Neurometabolic disorders are hereditary conditions mainly affect the function of the brain and the nervous system. The prevalence of these disorders is 1 in 1,000 live births. Such disorders, at different ages, could manifest as sepsis, hypoglycemia, and other neurologic disorders. Having similar manifestations leads to delayed diagnosis of neurometabolic disorders. A number of neurometabolic disorders have known treatments; however, to prevent long-term complications the key factors are early diagnosis and treatment. Although a large number of neurometabolic diseases have no treatment or cure, the correct and on-time diagnosis before death is important for parents to have plans for prenatal diagnosis. Different diagnostic procedures could be offered to parents, enzymatic procedures, and determining metabolites in plasma, urine, and CSF, and molecular genetic diagnosis. Molecular genetic diagnostic procedures are expensive and could not be offered to all parents. Therefore, we aimed to design algorithms to diagnose neurometabolic disorders according to some frequent and characteristic signs and symptoms. By designing these algorithms and using them properly, we could offer diagnostic enzymatic panels. These enzymatic panels are inexpensive; thereby reducing the financial burden on the parents. Also, having an early diagnosis according to these panels could lead to offering more accurate and less expensive molecular genetic tests.

Language representation and presurgical language mapping in pediatric epilepsy: A narrative review

Abstract: As one of the most common neurological diseases in children, epilepsy affects 0.9-2% of children. Complex interactions among the etiologies of epilepsy, interictal discharges, seizures, and antiepileptic drugs lead to cognitive impairments in children with epilepsy. Since epilepsy is considered as a network disorder, in which seizures have a widespread impact on many parts of the brain, childhood epilepsy can even affect the normal development of language. About 25% of children with epilepsy do not respond to medications; therefore, brain surgery is considered as a treatment option for some of them. Presurgical neuropsychological evaluations including language mapping are recommended to preserve cognitive and language abilities of patients after surgery. Functional magnetic resonance imaging as a non-invasive technique for presurgical language mapping has been widely recommended in many epileptic centers. The present study reviewed language representation and presurgical language mapping in children with epilepsy. Mapping language in children with epilepsy helps to localize the epileptogenic zone, and also, to predict the cognitive outcome of epilepsy surgery and possible cognitive rehabilitation. This review collected information about language representation and language mapping in pediatric epilepsy settings.

Effect of Baby Walker Use on Developmental Status based on Ages and Stages Questionnaire Score (ASQ).

Abstract: Objective Baby walker is a popular device which parents use for entertainment, keeping infants safe and walking promotion. Some studies showed developmental delay in baby walker users though others reported no significant effect on development in this group. We designed this study to determine whether baby walker usage has any effect on development by using Ages and Stages Questionnaire (ASQ). Materials & Methods We evaluated 107 one-year-old infants in each baby walker user group and non-users (total number of 214 participants) by using ASQ test of 12-month. We re-evaluated 168 infants at age 18-month by using ASQ test of 18-month. The data of these groups was compared. Results Girls use baby walker more frequently (p=0.02). Baby walker usage was not significantly associated with parent’s educational state, mother employment, birth rank of infant and delivery method. The starting age of baby walker use was 6.61 ± 1.46 months. ASQ results in area of gross movement and fine movement were not significantly different in users and non- users at age 12 and 18 month. Conclusion Most parents believe baby walker can promote earlier walking, but based on current evidences this belief might not be true. Although most studies showed no developmental delay in baby walker users, parents should become aware about their possible negative effects and hazards.

An Approach to Neurometabolic Epilepsy in Children with an Underlying Neurometabolic Disorder.

Abstract: ObjectiveAlthough in born errors of metabolism are rare, but total prevalence is . approximately 40-60% of IEM (inborn errors of metabolism)may present with epilepsy as one of the main clinical presentations and substantial number of them have specific treatment and obligates timely and appropriate diagnosis to prevent irreversible insults.Materials of methodsTwo years retrospective review of 128 documented neurometabolic patients, selected and evaluated in the mofid children hospital, tehran, Iran according to pre-prepared questionnaire to investigate epilepsy prevalence and seizure phenotype.the results were evaluated using spss version 23.resultsSeizure was reported in 49% (63/128) of all patients. The seizure was single episode in 7 patients (7%) .epilepsy prevalence was 42%(54/128) .The most common seizure types were GTC (43%), tonic (22%) and myoclonic (10%) respectively. Epilepsy was medically refractory in 30% () and mean Anti -seizure drug number administered for refractory cases was 3.2. 50% of refractory cases had mixed type seizures and 25% GTC and myoclonic seizures. ConclusionNeurometabolic disorders are rare but treatable etiology of epilepsy and considerable percent of them 42%(current study) present epilepsy as part of clinical featureKey words: inborn errors of metabolism,neurometabolic disorders,epilepsy

Serum Zinc Level in Children with Febrile Seizure.

Abstract: Objectives Febrile seizure (FS) is the most common type of seizure in children. FS is a genetic age-limited seizure, which only occurs with febrile illness. Today, it is known that genetic factors play a major role in the occurrence of FS. Nevertheless, some trace elements, such as zinc, may play an important role in the occurrence of FS. In this study, we investigated the serum level of zinc in patients with FS and febrile children without seizure (control group). Materials &methods This prospective case-control study was conducted on 41 patients with simple and complex FS as the case group and 41 febrile children without seizure as the age- and sex-matched control group. The participants were admitted to Best Hospital of Hamadan, Iran between January 2013 and January 2014 . The children's age ranged from six months to five years. Serum zinc levels were measured by atomic absorption spectroscopy in these groups. Results The mean serum zinc levels were 70.41±20.46 and 92.73±17.62 mcg/dL in the case and control groups, respectively (P=0.001). The results showed that the serum zinc level in children with FS was significantly lower than that of the control group. Conclusion Based on the present results, serum zinc level was lower in children with febrile seizure. However, further basic research is needed to examine the efficacy of zinc supplements in the prevention of FS.

Clonidine Versus Chloral Hydrate for Recording Sleep EEG in Children

Abstract: Objective One of the difficulties for conduct electroencephalography (EEG) in pediatric patient population is that they are not always cooperative during the procedure. Different medications have been used to induce sedation during EEG recording. In order to find a medication with least adverse effects and high efficacy, we aimed to compare clonidine and chloral hydrate as a premedication prior EEG performing in pediatric population. Materials & Methods A prospective, randomized, single-blinded, controlled trial was carried out over 198 children (9 to 156 months) to investigate the sedative and adverse effects of clonidine and chloral hydrate. Patients, partially sleep-deprived the night before, were randomly divided in two groups of clonidine (100 patients) and chloral hydrate (98 patients), on an alternative day basis. Results The average sleep onset latency was significantly longer in the clonidine group than chloral hydrate group (Mann-Whitney test, p < 0.0001). Sleep duration ranged between 15-150 minutes and it was not significantly different between two groups (Mann-Whitney test p = 0.2). Drowsiness with chloral hydrate terminated faster than with clonidine. Drowsiness after arousal was seen in 58% and 26.1% of patients in the clonidine and chloral hydrate groups respectively that was significant (Mann-Whitney test, p = 0.058). EEG results were reported normal in 77 subjects in the chloral hydrate group (77%) and in 69 subjects (69%) in the clonidine group (p = 0.161). Generalized epileptiform discharges reported significantly in the clonidine group (Mann-Whitney test , p = 0.006). Conclusion The results of this study showed that both chloral hydrate 5% (one ml/kg)and clonidine (4 μg/kg)could be administered as a pre medication agent for EEG recording in children , although drowsiness after arousal of clonidine is greater than chloral hydrate . However, the yield of generalized epileptiform discharges in the clonidine group was more than the chloral hydrate group.

Evaluation of the Efficacy of LevetiracetamPlus Iron in Comparison With Iron Alone in Controlling and Reducing the Frequency of Breath-Holding Spells in Children Aged 6 Months to 5 Years.

Abstract: Ojective A breath-holding spell (BHS) is defined as an apnea attack following an initial stressful event like anger, sadness, and fear, a painful event like falling or head trauma or any stressful psychology event. This study was designed to assess the comparative efficacy of levetiracetam plus iron and iron alone in reducing the BHS frequency in children aged 6 months to 5 years Materials & Methods This study was designed as a double-blinded randomized clinical trial. Sixty patients aged 6 months to 5 years were assigned into two groups, with the first group (A) receiving only iron and the second group (B) receiving levetiracetam plus iron. At the end of the study, the efficacy of therapy was analyzed comparatively in these groups Results In this study, the mean number of attacks was 3.94 ± 2.69 before treatment and 1.71 ± 1.99 after treatment in the group A, while it was 6.39 ± 5.7 before treatment and 0.37 ± 1.03after treatment in the group B. The mean number of attacks after treatment was lower in group B than in group A. In fact, there was a significant difference between the two groups in terms of the number of attacks after treatment (P = 0.003). Conclusion Levetiracetam plus iron is more effective than iron alone in reducing breath-holding spells in children aged 6 months to 5 years.