Investigation of the effect of sensorimotor exercises on proprioceptive perceptions among children with spastic hemiplegic cerebral palsy.

STUDY DESIGN: This is a blinded randomized clinical trial. INTRODUCTION: Sensorimotor exercises may be an effective, noninvasive treatment modality for treating patients with pediatric spastic hemiplegic cerebral palsy (CP). PURPOSE OF THE STUDY: We aim to evaluate the effect of sensorimotor exercises on the proprioceptive capability among children with spastic hemiplegic CP. METHODS: This randomized clinical trial was performed on children with spastic hemiplegic CP. Thirty children aged 8 to 12 years old, with spastic hemiplegic CP, were randomly selected and equally divided into experimental and control groups (N = 15 for each). A joint positioning test was used to measure the patients' baseline proprioceptive ability. The exercise program included sensory and motor exercises which lasted for 8 weeks (three 60-min sessions per week). The data were analyzed by using Welch and paired-sample t-test at the significance level of P < .05. RESULTS: The results indicated that the proprioceptive capability of the upper limb could significantly improve (P = .001, effect size = 0.41, observed power = 0.99) by using simultaneous exercising of the sensorimotor complex. The mean increased from 8.53 ± 1.6 to 10.53 ± 1.19 in the experimental group, whereas it changed from 6.66 ± 3.44 to 6.73 ± 3.15 in the control group. DISCUSSION: In consistent with other studies, the exercises used in the present study enhanced the proprioceptive capability but not sensory performance of the hands of children with hemiplegic. Synchronous sensory and motor training in children with hemiplegic CP may improve the function and organization of the somatosensory cortex and reduce sensory disturbances. Although parents were subjectively satisfied with the outcome of the exercises, such as independency and life style, however these recordings were not measured in this study. CONCLUSION: Implementing simultaneous sensorimotor exercises can improve the proprioceptive capability of the hand. Therefore, these exercises can be used in the rehabilitation programs to meet the children's needs with hemiplegic CP.

Efficacy and safety of levetiracetam in the management of seizures in neonates.

OBJECTIVE: To evaluate the efficacy and safety of levetiracetam (LEV) in the management of seizures in neonates. METHODS: A prospective non-blind, single arm clinical trial conducted in the Department of Neonatology and Pediatric Intensive Care, Mohamad Kermanshahi, and Imam Reza Hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran from May 2014 to December 2014. Fifty out of 60 newborns with gestational age >/=30 weeks with clinically diagnosed seizures were included. Levetiracetam was administered orally with an initial dose of 10 mg/kg twice a day. The patients were observed continuously by Neuro Intensive Care nurses, and visited daily by a neuropediatrician in the first 7 days and then at days 14, 30, and 90 after the start of LEV administration. Clinical examination was performed for every patient, and seizure number, antiepileptic medication, and adverse events were detailed at every visit. RESULTS: 47 infants were seizure free under LEV at the end of the first week, 47 remained seizure free at 4 weeks, and 46 remained seizure free at 11 weeks. No immediate and long-term side effects were noted in our patients. CONCLUSION: This study investigated the efficacy and safety of LEV in neonatal seizure control but confirmation with further randomized controlled trials is required.

The First Report of Iranian Registry of Patients with Spinal Muscular Atrophy.

BACKGROUND: Insufficient amounts of survival motor neuron protein is leading to one of the most disabling neuromuscular diseases, spinal muscular atrophy (SMA). Before the current study, the detailed characteristics of Iranian patients with SMA had not been determined. OBJECTIVE: To describe the key demographic, clinical, and genetic characteristics of patients with SMA registered in the Iranian Registry of SMA (IRSMA). METHODS: IRSMA has been established since 2018, and the demographic, clinical, and genetic characteristics of patients with SMA were recorded according to the methods of treat neuromuscular disease (TREAT-NMD) project. RESULTS: By October 1, 2022, 781 patients with 5q SMA were registered. Of them, 164 patients died, the majority of them had SMA type 1 and died during the first 20 months of life. The median survival of patients with type 1 SMA was 23 months. The consanguinity rate in 617 alive patients was 52.4%, while merely 24.8% of them had a positive family history. The most common type of SMA in live patients was type 3. Morbidities were defined as having scoliosis (44.1%), wheelchair dependency (36.8%), tube feeding (8.1%), and requiring mechanical ventilation (9.9%). Most of the registered patients had a homozygous deletion of SMN1, while the frequency of patients with higher copy numbers of SMN2, was less in more severe types of the disease. Earlier onset of the disease was significantly seen in patients with lower copy numbers of SMN2. The neuronal apoptosis inhibitory protein (NAIP) gene deletion was associated with a higher incidence of more severe types of SMA, higher dependency on ventilators, tube feeding, and earlier onset of the disease. CONCLUSIONS: The IRSMA is the first established Iranian nationwide registry of patients with SMA. Using this registry, decision-makers, researchers, and practitioners can precisely understand the epidemiology, characteristics, and genetics of patients with SMA in Iran.

Determination of common organophosphorus pesticides in the blood of children with acute leukaemia using a double-solvent system as a novel extractant for dispersive liquid-liquid microextraction.

In this research, a new mode of dispersive liquid-liquid microextraction based on a double-solvent system (DLLME-DSS) was developed for the extraction and preconcentration of organophosphorus pesticides (OPPs) in the blood of children with acute leukaemia prior to determination by high-performance liquid chromatography-ultraviolet detection (HPLC-UV). In the present method, two long normal chain alcohols are mixed in a particular ratio, and then injected into the sample solution, which is magnetically stirred. In this case, the mixture of the two alcohols changes to a new aggregate extractant. This new double-solvent is used as an extractant, which has a higher extraction power than any of its components alone. Under the optimum conditions, the calibration graph was linear in the rage of 3-600 µg L-1 with detection limits of 1 to 2 µg L-1. Relative standard deviations (RSDs) including intra-day and inter-day of the method based on 7 replicate determinations of 100.0 µg L-1 for each analyte were in the range of 2.9-4.7% and 3.8-6.1%, respectively. The results proved that DLLME-DSS is a sensitive, very simple, inexpensive, environmentally friendly, rapid and efficient method for the preconcentration of trace amounts of OPPs in blood samples.

Comparison of three therapeutic interventions for chronic constipation in paediatric patients with cerebral palsy: a randomised clinical trial.

INTRODUCTION: Cerebral palsy (CP) is a permanent non-progressive movement disorder, which is due to brain injuries during brain development. Constipation is one of the prevalent conditions in children with CP that can adversely affect the psychological health, appetite, and overall health of these patients. AIM: To compare three therapeutic methods in the treatment of chronic constipation in CP children. MATERIAL AND METHODS: In this randomised clinical trial (RCT), paediatric CP patients with chronic constipation were randomly divided into three groups (groups A, B, and C). Group A received polyethylene glycol (PEG), group B received PEG with Motilium, and group C received Motilium for 2 weeks. Motilium was administered at 0.2 mg/kg/dose and PEG at 0.5 g/kg/dose three times daily. To compare the therapeutic effect the McNemar test was used and a significance level of 0.05 was considered. RESULTS: The highest rate of improvement in chronic constipation was seen in group B and the lowest rate belonged to group C. Satisfactory response frequency was seen in 10 patients in group A (58.8%), 17 patients in group B (94.4%), and 1 patient in group C (6.6%); p < 0.001. CONCLUSIONS: According to the findings, simultaneous use of PEG and Motilium had the best therapeutic effect for chronic constipation among CP children. However, Motilium alone was nearly ineffective, and PEG alone had a moderate therapeutic effect.

Glutaric AciduriaType 1: Clinical and Molecular Study in Iranian Patients, 3 Novel Mutations.

OBJECTIVE: Glutaricaciduria type 1 (GA1), is a rare, treatable neuro metabolic disease, due to glutaryl- CoA dehydrogenase (GCDH) gene mutation.In regions without neonatal blood screening (NBS), patients are diagnosed in symptomatic period. This study was carried out to assess patients with GA1 for clinical, biochemical, neuroimaging findings and GCDH gene mutations analysis. MATERIALS & METHODS: In this cross-sectional study, clinical manifestation, neuroimaging and metabolic findings of eleven Iranian GA1 patients of MofidChildren's Hospital, Tehran, Iranbetween 2001 and 2011,were evaluated.Mutational analysis of the GCDH gene was performed on genomic DNA. Genomic DNA was extracted from peripheral lymphocytes using QIAamp DNA Micro Kit (Qiagen). All 11 exons and flanking intronic regions of the GCDH gene were amplified by polymerase chain reaction (PCR). RESULTS: All patients were diagnosed before 32 months old. Clinical presentations of GA1 include acute encephalopathic crisis and/or developmental delay and macrocephaly. Seven GCDH gene mutations were detected in our patients. The most frequent GCDH mutations occurred in exon7 then exon8, 10 and11. G244 C in exon7, R294 Q in exon8 and N373 S in exon 10 were three novel mutations. There was no correlation between of genotype and phenotype in our patients. CONCLUSION: Physician must remember GA1 in differential diagnosis of acute encephalopathic crisis, macrocephaly, developmental delay, movement disorders such as dystonia and dyskinesia. Early detection, proper treatment and selective screening of patients' siblings can prevent neurologic disabilities.

Comparison of propranolol and pregabalin for prophylaxis of childhood migraine: a randomised controlled trial.

Migraine involves 5-10% of children and adolescents. Thirty percent of children with severe migraine attacks have school absence and reduced quality of life that need preventive therapy. The purpose of this randomised control trial study is to compare the effectiveness, safety and the tolerability of pregabalin toward Propranolol in migraine prophylaxis of children. From May 2011 to October 2012, 99 children 3-15 years referred to the neurology clinic of Mofid Children's Hospital with a diagnosis of migraine enrolled the study. Patients randomly divided into two groups (A&B). We treated children of group A with capsule of pregabalin as children of group B with tablet of propranolol for at least 8 weeks. In this study, 99 patients were examined that 91 children reached the last stage. The group A consistsed of 46 patients, 12(26.1%) girls, 34 (73.9%) boys and the group B consisted of 45 patients, 14(31.1%) girls, 31 (68.9%) boys. Basis of age, gender, headache onset, headache frequency, migraine type, triggering and relieving factors there was no significant difference among these groups (P>0.05). After 4 and 8 weeks of Pregabalin usage monthly headache frequency decreased to 2.2±4.5 and 1.76±6.2 respectively. Propranolol reduced monthly headache frequency up to 3.73±6.11 and 3.34±5.95 later 4 and 8 weeks respectively. There was a significant difference between these two groups according to headache frequency reduction (P=0.04). Pregabalin efficacy in reducing the frequency and duration of pediatric migraine headache is considerable in comparison with propranolol.

Low Glycemic Index Treatment in pediatric refractory epilepsy: the first Middle East report.

PURPOSE: Intractable epilepsy is a challenging aspects of pediatric epilepsy. This study was conducted to determine the efficacy and tolerability of Low Glycemic Index Treatment (LGIT) in pediatric patients referred to a Children's Hospital in Iran with intractable epilepsy. METHODS: We studied 42 children with refractory epilepsy aged between 1.5 and 17 years of age, from October 2009 to April 2011 in the pediatric neurology department of Mofid Children's Hospital. Patient information on clinical status, seizure type, and baseline frequency, blood and urine biochemistry, neuro-imaging and the EEG were collected. LGIT was initiated on an outpatient basis and the diet was composed of 65% fat, 25% protein and 10% carbohydrate (40-60 g), and the glycemic index of foods was limited to below 50. RESULTS: 84% of patients were categorized as having more than one seizure per day at study entry, with the remaining children as experiencing over one seizure per week. A greater than 50% seizure reduction was observed in 71.4% of the patients after the second week, in 73.8% at the end of the first month and in 77.8% at the end of the second month. In 30% of the patients a mild increase in blood urea nitrogen (BUN) was detected. The most important reasons for discontinuation of LGIT were restrictiveness, lack of satiation and excessive meat in this diet. No significant complications were observed during the administration of the diet. CONCLUSION: LGIT is a safe and effective adjuvant antiepileptic therapy and may be used as an alternative to the ketogenic diet in conditions when this diet cannot be used.

Crohn's disease and idiopathic thrombocytopenic purpura in a patient with ectodermal dysplasia and immunodeficiency.

In this case report we will describe a rare association between anhyrotic ectodermal dysplasia (AED) and immunodeficiency and autoimmunity [in our case: Idiopathic Thrombocytopenic Purpura (ITP) and Crohn disease]. AED is a rare congenital disorder characterized by sparse hair, abnormal teeth and anhidrosis due to lack of eccrine glands. The survey of 87 cases with (AED) revealed only one Irritable Bowel Disease (IBD). AED has only two relevancies with immunodeficiency: (EDA-ID: Ectodermal Dysplasia Anhyrotic with Immunodeficiency) and APE-CED (Autoimmune polyendocrinopathy, Candidiasis and Ectodermal Dysplasia) that in our case EDA-ID is strongly suspected.

Low magnesium concentration in erythrocytes of children with acute asthma.

Magnesium (Mg) is the second most abundant intracellular cation and is involved in numerous physiological functions, including protein folding, intracellular signaling and enzyme catalysis. It has been shown that magnesium deficiency exacerbates pulmonary airways hyper reactivity. Several studies suggest that magnesium level has no effect on asthma but others had shown a contributory effect. Because of its intracellular abundance the aim of this study was to determine if there was any difference in plasma and intracellular Mg concentrations of children with acute asthma compared to non asthmatic children. Twenty nine patients with acute asthma aged 2 to11 years admitted to the emergency department of hospital and 37 non asthmatic children with the same age were included in our study. 0.5 mL of heparinized whole blood samples of patients who were meeting inclusion criteria at the onset of admission with bronchoconstriction and before using any medication was drawn and it was immediately sent to the laboratory. Plasma and erythrocytes were separated and stored at -20C and later their Mg levels were quantified with atomic absorption spectrophotometry method. The average plasma and intracellular magnesium levels in patients were (0.79 +/- 0.098 mmol/L) and (1.17 +/- 0.27 mmol/L) respectively. Results of 37 non asthmatic persons [plasma (0.85 +/- 0.1 mmol/L ) and erythrocytes (1.33 +/- 0.21 mmol/L)] showed that there was no significant difference between plasma Mg levels in two groups (p 0.06) but intracellular magnesium level was significantly lower (p 0.03) in patients group. These results indicate that intracellular Mg level may be a more accurate method to assess Mg level in patients with asthma. Hence, determination of Mg concentration in erythrocytes may be used in evaluation of asthma pathophysiology. There are recommendations for using intravenous Mg sulfate in acute asthma, and this study supports the rational for using it in emergency departments for acute severe asthma.