Iranian Journal of Child Neurology

Abstract
This review presented the current literature on coping mechanisms among parents of children with autism spectrum disorder (ASD), focusing on types of coping mechanisms among parents and different coping mechanisms between mothers and fathers of children with ASD.
A search of published literature in English was conducted using Google Scholar, PsycINFO, Medline, Scopus, CINAHL, EBSCO, Springer, Ovid, PubMed, and Cochrane Library up to February 2020. Overall, 18 articles were relevant to the review. The review included thirteen studies for types of coping mechanisms among parents of children with ASD and five studies for different coping mechanisms between mothers and fathers. Coping mechanisms demonstrated by parents when caring for their child include problem-focused and emotion-focused coping. A comparison between fathers and mothers in our review showed that mothers used emotion-focused coping more than fathers, while fathers used problem-focused coping more frequently than mothers.
The review provides an exciting opportunity to advance our knowledge on types of coping mechanisms and gender difference in using coping mechanisms among parents of children with ASD. The review also sheds new light on developing supportive interventions by healthcare providers to improve coping mechanisms among parents of children with ASD.

Abstract

Thalassemia syndromes are the most prevalent monogenic hemoglobinopathy in the world. In Iran, thalassemia is a public health problem because this country has been located on the thalassemia belt. In recent decades, considering that the life expectancy of patients with thalassemia has dramatically improved, some unrecognized complications have emerged in these individuals. One of these complications is a hypercoagulable state that may lead to thromboembolic events (TEE). The TEE may involve any organ in the body, including the central nervous system. Ischemic cerebrovascular events in thalassemic patients have been divided into two categories, namely overt stroke and silent cerebral infarcts (SCI). Overt stroke often develops in patients with beta-thalassemia major; however, patients with thalassemia intermedia usually suffer from SCI. This review article discusses brain vascular involvement.

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; therefor; those born in 1982 and diagnosed with the disease and treated since 2000 were included in this study. The collected variables were patients’ age at the time of diagnosis, gender, year of diagnosis, urban or rural residency, and province of residence. Additionally, agespecific 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.

Abstract
Objectives
The profile of mental disorders has been changing over time. The aim of this study was to investigate the prevalence of mental disorders in children and adolescents in the Lorestan province of Iran.
Materials & Methods
This community-based cross-sectional study was done on 1001 children and adolescents aged 6-18 years from Lorestan province randomly selected by multistage cluster sampling method. Children and their parents were interviewed using the Kiddie-Sads-Present and Lifetime Version (K-SADS-PL). Also, the comorbidities of psychiatric disorders were evaluated.
Results
According to obtained results, 25.1% of participants were diagnosed to have at least one psychiatric disorder. The prevalence of psychiatric disorders was not significantly different between the two sexes (OR=0.876, P=0.378) and three age groups (P>0.05). this prevalence was significantly higher in rural areas than in urban areas (OR=1.997, P=0) and was significantly lower in children of fathers with a high school diploma or higher education compared to children of less-educated fathers (P<0.05). On contrary, it was significantly more prevalent in children of mothers with high school and bachelor’s degrees compared to illiterate mothers. (p<0.05). The most prevalent psychiatric disorders were oppositional defiant disorder (ODD) (5.9%), separation anxiety disorder (5.3%), and depressive disorders (5%). The most prevalent groups of psychiatric disorders included anxiety disorders (11.2%), behavioral disorders (9.4%), and mooddisorders (5%). Behavioral disorders were highly comorbid with anxiety disorders (20.7%).
Conclusion
Mental disorders affect a high proportion of children and adolescents in Lorestan province.There is a need for psychiatric facilities to provide for the needs of families to child mental health services.

Abstract

Objectives

The Present study aimed to analyze the Impact of “Care for Child Development" program on the sensitivity and responsiveness skills of mothers of children who are at risk of developmental delay.

Materials & Methods

 This study is a quasi-experimental research planned in pretest-posttest with experimental and control groups. The statistical population included all mothers with children at risk of developmental delay in Tabriz. 50 mothers were selected through purposive sampling. Then, they were randomly divided into two groups of 25 (one experimental group and one control group). The experimental group received training about the program of “Care for child Development “ in 4 group session, while the control group did not receive the same training . The social-emotional assessment/evaluation measure family profile (SEAM TM  family profile)  and maternal caregiving quality scale were the research measurement tools. The obtained data were evaluated by analysis of covariance (ANCOVA) and independent t-test using SPSS software version 20.

Results

There was a significant difference between the experimental and control groups in maternal caregiving quality and responsiveness, provision of appropriate activities, predictable programs, and provision of play environment and safe home (P <0.05).

Conclusions

 The results showed that the ‘care for child development’ program has positive effect on sensitivity and responsiveness skills of mothers of at risk children and could be considered and applied as a practical plan in national health policies.

Abstract

Objectives

West syndrome is a severe epileptic encephalopathy of young age. It is characterized by a clinico-electrical triad of infantile epileptic spasms, regression or arrest of psychomotor development, and hypsarrhythmia. In the last two decades, the large progress in the development of newer antiepileptic drugs has allowed us to have a vast choice of treatment options to control spasms, although they often fail to do so. Thus, there is a need to explore other treatment options.

Materials & Methods

Subjects in this open-labelled randomized control trial were included newly diagnosed children of age between 3 months and 5 years of both genders. A total of 52 children were recruited and randomized into two groups: an intervention group (n=30) and a non-intervention group (n=22). Magnesium sulphate was provided for the intervention group but not for the non-intervention one. Both groups received the rest of the treatments, including adrenocorticotropic hormone and antiepileptic drugs. The follow-up period was three months, at the end of which a per-protocol analysis was performed.

Results

There was no significant difference in seizure control and neurodevelopmental outcome between both groups, but electroencephalogram significantly improved in the intervention group compared to the control. Also, the clinical response was better in patients with normal initial serum magnesium levels in the intervention group (p=0.003) than in other patients.

Conclusion

Magnesium supplementation may be helpful in children with West syndrome

Abstract

Objectives

Perinatal hypoxic-ischemic brain injuries have been a major cause of mortality and neurodevelopmental morbidities in newborns. Citicoline and Piracetam have been used as nootropic agents in a number of studies. In this investigation, we aimed to determine the effects of these agents solely and in combination in hypoxicischemic brain damage in rabbit neonates.

Materials & Methods

Hypoxic-ischemic brain damage was induced by the occlusion of both uterine arteries of dams for eight minutes. The subjects were randomly divided into five groups as follows (n=6 per group): control group without hypoxia (C1), control group with hypoxicischemic damage (C2), the third group (P) received Piracetam (100 mg/kg), the fourth group (T) administered with Citicoline (250 mg/ kg), and the fifth (PT) received both. The preventive effects of the two drugs on hypoxic-ischemic brain damage were microscopically investigated by the rates of damage to the hippocampus.

Results

Neuronal destruction rates in C1, C2, P, T, and PT were 4%, 45%, 37.5%, 12.5% (P=0.01 vs. C2), and 20% (P=0.03 vs. C2), respectively. The total means of hypoxic-ischemic damage, cell edema, neuronal degeneration, and eosinophilic degeneration were lower in the T group compared to C2 (P<0.05).

Conclusion

According to our results and previous findings, Citicoline as a treatment for hypoxic-ischemic brain injuries could be beneficial, and it has priority over neuroprotective agents like Piracetam. Moreover, the combination of Citicoline and Piracetam showed no superior effect in contrast with Citicoline alone. However, experimental studies on larger populations and clinical trials are highly suggested

Abstract

Objectives

Fragile X syndrome (FXS) has been known as the most common cause of inherited intellectual disability and autism. This disease results from the loss of fragile X mental retardation protein expression due to the expansion of CGG repeats located on the 5’ untranslated region of the fragile X mental retardation 1 (FMR1) gene.

Materials & Methods

In the present study, the peripheral blood-mesenchymal stem cells (PB-MSCs) of two female full mutation carriers were differentiated into neuronal cells by the suppression of bone morphogenesis pathway
signaling. Then, the expression of genes adjacent to CGG repeats expansion, including SLIT and NTRK-like protein 2 (SLITRK2), SLIT and NTRK-like protein 4 (SLITRK4), methyl CpG binding protein 2 (MECP2), and gamma-aminobutyric acid receptor subunit alpha-3 (GABRA3), were evaluated in these cells using SYBR Green real-time polymerase chain reaction.

Results

The obtained results indicated that the expression of SLITRK2 and SLITRK4 were upregulated and downregulated in the neuron-like cells differentiated from the PB-MSCs of females with FMR1 full mutation, compared to that of the normal females, respectively. Furthermore, the expression of MECP2 and GABRA3 genes were observed to be related to the phenotypic differences observed in the female FMR1
full mutation carriers

Conclusion

The observed association of expression of genes located upstream of the FMR1 gene with phenotypic differences in the female carriers could increase the understanding of novel therapeutic targets for patients with mild symptoms of FXS and the patients affected by other FMR1-related disorders

Abstract

Objectives

In children suffering from febrile seizure, the likelihood of recurrence seems to be high in the early hours following the first episodes in the absence of proper interventions. The present study was aimed  at assessing and comparing the outcomes of different preventive interventions in the acute stage after febrile seizure in children.

Materials & Methods

This randomized clinical trial study was performed between September 2015 and September 2016. We enrolled patients aged between 6 and 60 months suffered from febrile seizure and referred to the Pediatric
Emergency Department at Besat Hospital in Hamadan. The eligible patients were randomly assigned to the following four receive one of the following groups: group 1 (not receiving any anti-seizure drugs), group 2 (receiving a single dose of phenobarbital) on admission, group 3 (receiving a single dose of phenobarbital on admission continued until the fever is resolved), and group 4 (receiving diazepam until the disappearance offever).

Results

The study population consisted of 248 children. The recurrence rate of seizure in the acute stage was 4.84%. Also, the impact of diazepam and phenobarbital (either as a single dose or as continuous) on the prevention of febrile seizure recurrence in the acute stage has been established. None of the patients had febrile status epilepticus.

Conclusion

Controlling seizures without prescribing anti-seizure drugs increases the risk for the recurrence of febrile seizure in the acute stage. Different drug regimens for controlling seizure, including diazepam and phenobarbital (as stat or maintenance), may play a similar role in preventing the occurrence of febrile seizure

Abstract

Objective

Migraine is recognized as a disease with unknown etiology andvarious pathophysiologic pathways which are not fully understood. Due to the relation between dairy intake and various chronic conditions in children and also the paucity of data on the probable role of dairy intake on pediatrics’ odds of having migraine, this study
was designed.

Materials & Methods

The present study was a population-based case-control design that was accomplished in a tertiary headache clinic.290 child (aged from7 to 14 years old) was included in this study. A definite diagnosis of migraine was performed by a neurologist; concerning the 2018 international classification of headache disorder 3 (ICHD3) criteria. Also, demographic and anthropometric characteristics were obtained. In addition, the usual dietary intake of participants was evaluated using a validated semi-quantitative food frequency questionnaire (FFQ).

Results

Those children in the case group significantly had higher age and BMI means (P.value:0.000). In the second regression model, odds of migraine were 48% (OR: 0.52; 95%CI:0.27-1.00) diminished in the second tertile and 53% (OR:0.47;95%CI:0.24-0.92) in the third tertile of low-fat dairy intake (P-trend:0.03). In the fully adjusted
model, the achieved migraine ORs were as followings:0.48 (95% CI:0.240.95) in the second tertile and 0.46 (95% CI:0.21-0.96) in the third tertile (P-trend:0.04), respectively. Children with more high fat dairy intake also consumed higher amounts of energy, pastries, simple sugar, unhealthy snacks, and hydrogenated oil (P<0.05).

Conclusion

This study results proposed that a greater amount of lowfat dairy intake may attenuate the odds of having migraine attacks in pediatrics and adolescents who might be at risk of headache, which can be attributed to the micronutrient and also to the bioactive content of these dietary components.

Abstract

Objectives

Bardet-Biedl syndrome (BBS) is an autosomal recessive pleiotropic ciliopathy, which includes multi-organ clinical manifestations. The known genes involved in the development of the disease account for the causality in about 80% of the examined cases.

Materials & Methods

We investigated two Iranian unrelated clinically diagnosed BBS patients, using a targeted next-generation sequencing panel consisting of 18 known BBS genes. The detected variants were investigated in the pedigree and studied using in silico tools for their pathogenicity. Patients’ phenotypes were also assessed.

Results

Novel homozygous variants were detected in BBS9 gene in each patient, c.2014C>T, p.Gln672Ter and c.673_674insAA, p.Gln225GlnfsX10. The variants were segregated in the corresponding pedigree and were authenticated to obtain enough evidence to be categorized as pathogenic variants.

Conclusion

Patients with truncating mutations in the same gene seem to show similar phenotypic features. Detection of novel and family-specific mutations is typically expected in the genetic hereditary diseases in
Iran, which can finally lead to prevent the recurrence of the disease in the consanguineous marriages.

Abstract

Hereditary sensory and autonomic neuropathies (HSAN) are rare genetic disorders that often manifest during childhood in the form of absence of pain sensation or self-mutilation. Patients often present significant oral self-mutilation manifestations, and biting of the lips, tongue, and cheeks have been frequently reported. This case report describes a case of hereditary sensory and autonomic neuropathy with oral and cutaneous ulcers.
Our patient was a 14-month-old girl with the chief complaint of a tongue ulcer, as stated by her parents, who were referred to our private dental clinic. Clinical examination revealed severe ulcers due to biting (Riga-Fede disease) on the ventral surface of the tongue and superficial ulcers on the dorsal surface of the tongue caused by the anterior maxillary teeth, along with some sores on fingers. The parents were healthy, with no congenital disease or familial history of a similar condition. The electrodiagnostic test revealed the absence of sensory nerve action potential response. However, the electromyographic findings and the compound muscle action
potential of the tibial and ulnar nerves were normal. Oral ulcers such as trauma to the lips and tongue, and self-mutilation trauma to the fingers can be used for early detection of Hereditary sensory and autonomic neuropathies. A multidisciplinary approach involving a professional dental team and a regular treatment protocol are imperative to prevent complications of Hereditary sensory and autonomic neuropathies.