فهرست مطالب

  • Volume:14 Issue: 2, 2020
  • تاریخ انتشار: 1399/01/10
  • تعداد عناوین: 10
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  • Farin Soleimani*, Nadia Azari, Hesam Ghiasvand, Shiva Fatollahierad Pages 7-15

    Objectives:

    The 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.

    Keywords: Preterm infants, developmental care, NICU, Interventions, Bayley Scales of Infant, Toddler Development
  • Afshin Fayyazi*, Reihane Eslamian, Ali Khajeh, maryam Dehghani Pages 17-25
    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.

    Keywords: Infantile spasms, ACTH therapy, dosage, Side effects
  • Marjan Shakiba*, Mohammadreza Alaei, Hedyeh Saneifard, Asieh Mosallanejad Pages 27-39
    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.

    Keywords: Phenylketonuria, Child
  • Shakiba Ghaffari*, Minoo Kalantari, Mehdi Rezaee, Alireza AkbarzadehBaghban Pages 41-57
    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 has shown most significant direct effect on participation, after that higher gross motor function (-.26), higher manual ability (-.19), communication function (-.17), higher cognitive level (-.16), more brothers and sisters in family (.15) and less emotional- behavioral problems (-.15) have shown direct effect on intensity of participation. Family Structures & Relationships (.17) and unsupportive environment constructs (-.14) have shown indirect but significant effect (P< .05). The relationship between family education level and income with participation were not significant (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.

    Keywords: Participation, Leisure, Cerebral Palsy, Structural Equation Modeling
  • Pegah Katibeh*, Soroor Inaloo, Peyman Jafari, Fahimeh fattah, Samaneh Mazloomi Pages 59-68
    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.

    Keywords: epilepsy, quality of life, KIDSCREEN-27
  • Javad Akhondian*, Farah Ashrafzadeh, Hossein Eslamieh Pages 69-77

    Focal epilepsy is one of the most prevalent type of seizures in childhood. In this study we will compare the effect of new approved drug leveitiracetam versus carbamazepine in the treatment of focal epilepsy.

    Methods and Materials:

     we studied  newly diagnosed  children with focal epilepsy (in the age range of 1-16). The children were treated randomly with two drug levetiracetam and carbamazepine. Patients have been followed for seizure and drugs side effects at intervals of 1 month and six months. We checked liver function tests and complete blood count for all patients and they asked about any significant side effects such as drowsiness، restlessness and skin reaction. Eventually, they were classified in two groups of 25 consuming levetiracetam and 25 consuming carbamazepine.

    Results

    In our study, two cases were excluded in levetiracetam group because of sever agitation and 3 patients (13%) had relapsing seizures. In the group of carbamazepine,10 patients(40%) had relapse. Seizure was not repeated in 15 (60%) and 20 (87%) cases in  carbamazepin and levetiracetam  groups, respectivelly.chi square examination  showed that the responses to treatment had significant differences (P=0.03). Agitation and drowsiness  were the most prevalent complication in levetiracetam and carbamazepine groups, respectivelly. Fortunately ،liver enzyme dysfunction and blood cell disturbance were observed in none of the groups.

    Conclusion

     According to findings, there were significant differences in controlling  seizures  between two groups that implicate much more influence of levetiracetam (87%) in suppression of focal seizure .

    Keywords: focal epilepsy, carbamazepine, levetiracetam, Effectiveness
  • Parvaneh Karimzadeh*, Parinaz Habibi Pages 79-86

    Objective:

    Although 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 methods:

    Two 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.

    results:

    Seizure 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. 

    Conclusion:

    Neurometabolic 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

    Keywords: inborn errors of metabolism, neurometabolic disorders, epilepsy
  • Farzad Ahmadabadi*, Sonia Ruhollahi, Reza Maskani, Narjes Jafari, Sanaz KarimiDardashti Pages 87-92

    Acute necrotizing encephalopathy of childhood (ANEC) is a disease characterized by post infectious (respiratory or gastrointestinal) encephalopathy accompanying with fever and  rapid alteration of consciousness and seizures. Acute encephalopathy following viral infection with seizure and altered of consciousness and absence of CSF pleocytosios with occasional increased level of protein are clinical characteristics of ANEC (1). This disease is nearly exclusively in East Asian infants and children who had been previously healthy (2). Serial magnetic resonance imaging examinations have demonstrated symmetric lesion involving the thalami, brainstem, cerebellum, and white matter in this disease (8). The condition accompanies a poor prognosis with high morbidity and mortality rates.We introduce 3 cases with ANE those referred to our hospital during 2 weeks and created this idea that we have an epidemic.

    Keywords: Acute Necrotizing Encephalopathy, seizure, pediatric
  • Rahmaneh Moosavi*, Niloofar JahangirSoltani, Massoud Houshmand Pages 93-100

    CMT4 is an autosomal recessive form of Charcot-Marie-tooth disease which has shown more severity and earlier age of onset compared to other types of it, furthermore; CMT4C andCMT4D are the more prevalent types in Mediterranean countries due to higher incidence of consanguineous marriages. The most important aim of this study is to illuminate the rate of p.R148X mutation in NDRG1 gene and p.R1109X in SH3TC2 gene which are responsible genes for CMT4D and CMT4C respectively in Iranian population, Furthermore; this study investigated the probable other nucleotide changes in exon 14 of SH3TC2 gene and exon 7 of NDRG1 gene. In order to study this disease, 24 CMT4 affected individuals that they referred to Iran Special Medical Center, were clinically and electrophysiologically evaluated and selected for this study. The patients’ DNA was extracted from blood samples and after PCR, the products were sequenced and analyzed by Finch TV software. None of the founder mutations we were searching for were seen in this study. Sequencing of SH3TC2 gene showed SNP rs1025476 (g.57975C>T) in 21 patients (87.5%) that 7 individuals were homozygous and 14 individuals were heterozygous for this variant. Despite of high rate of considered mutations in some specific populations it seems that these mutations are very rare in Iranian CMT4 affected individuals. To clarify the association of SNP rs1025476 with CMT4, further assesments are needed and it could be helpful in knowing the Iranian population genetic markers and their genetic features.

    Keywords: SH3TC2 gene, NDRG1 gene, CMT4, Charcot-Marie-tooth, Iran
  • Asieh Mosallanejad*, Mohammadreza Alaei, SaeedReza Ghaffari, maryam Rafati, Hedyeh Saneifard Pages 101-106

    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.

    Keywords: : Mucopolysaccharidosis, Sly syndrome, GUSB gene, Hydrops fetalis