CNS Structural Anomalies in Iranian Children with Global Developmental Delay

How to Cite This Article: Zamani GH, Shervin Badv R, Niksirat A, Alizadeh H. CNS Structural Anomalies in Iranian Children with Global Developmental Delay. Iran J Child Neurol. 2013 Winter; 7 (1):25-28. ObjectiveCentral Nervous system (CNS) malformations are one of the most important causes of global developmental delay (GDD) in Children. About one percent of infants with GDD have an inherited metabolic disorder and 3-10 percent have a chromosomal disorder. This study aimed to survey the frequency of brain structural anomalies and their subtypes among the variety of etiologic factors in children with GDD in our patients.Materials & MethodsThis study used the results of neuroimaging studies [unenhanced brain Magnetic Resonance Imaging (MRI)] of all children who had been referred for evaluation of GDD to outpatient Clinic of Pediatric neurology at Children’s Medical Center affiliated to Tehran University of Medical Science between September 2009 and September 2010.ResultsIn this study, unenhanced brain MRI was performed on 405 children, of which80 cases (20 percent) had brain structural anomalies. In 8.7 percent of the cases, previous history of brain structural disorders existed in other children of the family and 20 percent of mothers had inadequate consumption of folate during pregnancy.ConclusionBased on the results of this study, unenhanced cranial MRI seems to be a fundamental part of evaluation in all children with GDD. Adequate folate consumption as prophylaxis as well as genetic counseling can be worthy for high-risk mothers who have previous history of CNS anomaly or miscarriage to avoid repeated CNS anomalies in their next pregnancies. References1. Fenichel M. Clinical Pediatric Neurology: A Signs and Symptoms Approach. 6th ed. Philadelphia: Saunders; 2009. p. 119-52.2. A guide to investigation of children with developmental delay in East Anglia 2005Available from:http://www. phgfoundation.org/file/2366.3. Williams J. Global developmental delay–globally helpful? Dev Med Child Neurol 2010;52(3):227.4. Shevell M, Ashwal S, Donley D, Flint J, Gingold M, Hirtz D, et al. Practice parameter: Evaluation of the child with global developmental delay: Report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society. Neurology 2003;60(3):367-80.5. Whiting K. Investigating the child with learning difficulty.Current Pediatrics 2001;11(4):240-7.6. Aicardi J. The etiology of developmental delay. Semin Pediatr Neurol 1998;5(1):15-20.7. Von Wendt L, Rantakallio P. Congenital malformations of the central nervous system in a 1-year birth cohort followed to the age of 14 years. Childs Nerv Syst.1986;2(2):80-2.8. Kuzniecky R, Murro A, King D, Morawetz R, Smith J, Powers R, et al. Magnetic resonance imaging in childhood intractable partial epilepsy: Pathologic correlations. Neurology1993;43:681-7.9. Massimi L, Paternoster G, Fasano T, et al: On the changing epidemiology of hydrocephalus. Childs Nerv Syst. 2009;25(7):795-800.10. Warkany J, Lemire RJ, Cohen Jr MM. Mental retardation and congenital malformations of the central nervous system. Chicago: Year Book Medical Publishers; 1981.11. Petrini J, Damus K, Johnston RB Jr. An overview of infant mortality and birth defects in the United States. Teratology. 1997;56(1-2):8-10.12. Rosano A, Botto LD, Botting B, Mastroiacovo P. Infant mortality and congenital anomalies from 1950 to 1994: an international perspective. J Epidemiol Community Health 2000; 54(9):660-6.13. Cordero JF. Finding the causes of birth defects. The New England Journal of Medicine. 1994;331(1):48-9.14. Srour M, Mazer B, Shevell MI. Analysis of Clinical Features Predicting Etiologic Yield in the Assessment of Global    Developmental    Delay.Pediatrics    2006 ;118(1):139-45.15. Meral O, Burak T, Nur A. Etiologic evaluation in 247 children with GDD at Istanbul, Turkey. J Trop Pediatr 2005;51(5):310-3.16. World Health Organization. Weekly Iron-Folic Acid Supplementation (WIFS) in women of reproductive age: its role in promoting optimal maternal and child health. Geneva, World Health Organization, 2009. WHO/NMH/ NHD/MNM/09.2. p. 2. 

Sleep Inducing for EEG Recording in Children: A Comparison between Oral Midazolam and Chloral Hydrate

How to Cite This Article: AshrafiMR, Azizi Malamiri R, Zamani GR, Mohammadi M, Hosseini F. Sleep Inducing for EEG Recording in Children: A Comparison between Oral Midazolam and Chloral Hydrate. Iran J Child Neurol. 2013 Winter;7(1):15-19.ObjectiveElectroencephalography (EEG) recording is a long duration procedure that needs patient’s cooperation for device setup and performing the procedure. Many children lose their cooperation during this procedure. Therefore, sedation and sleep are frequently induced using a few agents as pre procedure medication in children before EEG recording. We aimed to compare the sedative effects of oral midazolam versus chloral hydrate before the procedure along with their impacts on EEG recording in children.Materials & MethodsA randomized trial was carried out to compare the sedative effects of oral midazolam versus chloral hydrate and their impacts on EEG recording in children. A total of 198 children (100 in the midazolam group and 98 in the chloral hydrate group) were enrolled in the study and randomly allocated to receive either oral moidazolam or chloral hydrate.ResultsOral midazolam had superiority neither in sleep onset latency nor in sleep duration when compared to chloral hydrate. Moreover, the yield of epileptiform discharges in the chloral hydrate group was more than the midazolam group.ConclusionThe results of this study showed that both chloral hydrate 5% (one ml/kg) and oral midazolam (0.5 mg/kg) could be administered as a pre medication agent for EEG recording in children. However, oral midazolam at this dose had no advantage compared with chloral hydrate.ReferencesAshrafi MR, Mohammadi M, Tafarroji J, Shabanian R, Salamati P, Zamani GR. Melatonin versus chloral hydrate for recording sleep EEG. Eur J Paediatr Neurol 2010;14(3):235-8.Slifer KJ, Avis KT, Frutchey RA. Behavioral intervention to increase compliance with electroencephalographic procedures in children with developmental disabilities. Epilepsy Behav 2008;13 (1):189-95.Gauillard J, Cheref S, Vacherontrystram MN, Martin JC. [Chloral hydrate: a hypnotic best forgotten?]. Encephale 2002;28(3 Pt 1):200-4.Cote CJ, Karl HW, Notterman DA, Weinberg JA, McCloskey C. Adverse sedation events in pediatrics: analysis of medications used for sedation. Pediatrics 2000;106(4):633-44.Greenblatt DJ, Ehrenberg BL, Culm KE, Scavone JM, Corbett KE, Friedman HL, et al. Kinetics and EEG effects of midazolam during and after 1-minute, 1-hour, and 3-hour intravenous infusions. J Clin Pharmacol 2004;44(6):605-11.Gurakan F, Yuce A, Ozen H, Saltic IN. Midazolam and pethidine for the sedation of children undergoing gastrointestinal endoscopy. Crit care med 2000;28(6):2176-7.Karl HW, Cote CJ, McCubbin MM, Kelley M, Liebelt E, Kaufman S, et al. Intravenous midazolam for sedation of children undergoing procedures: an analysis of age- and procedure-related factors. Pediatr Emerg Care 1999;15(3):167-72.Lightdale JR, Mitchell PD, Fredette ME, Mahoney LB, Zgleszewski SE, Scharff L, et al. A Pilot Study of Ketamine versus Midazolam/Fentanyl Sedation in Children Undergoing GI Endoscopy. Int J Pediatr 2011; 2011:623710.Massanari M, Novitsky J, Reinstein LJ. Paradoxical reactions in children associated with midazolam use during endoscopy. 1997;36(12):681-4.Scott RC, Besag FM, Boyd SG, Berry D, Neville BG. Buccal absorption of midazolam: pharmacokinetics and EEG pharmacodynamics. Epilepsia 1998;39(3):290-4.Loewy J, Hallan C, Friedman E, Martinez C. Sleep/sedation in children undergoing EEG testing: a comparison of chloral hydrate and music therapy. Am J electroneurodiagnostic technol 2006;46(4):343-55.Rodriguez E, Jordan R. Contemporary trends in pediatric sedation and analgesia. Emerg Med Clin North Am 2002;20(1):199-222.Sisson DF, Siegel J. Chloral hydrate anesthesia: EEG power spectrum analysis and effects on VEPs in the rat. Neurotoxicol Teratol 1989;11(1):51-6.Thoresen M, Henriksen O, Wannag E, Laegreid L. Does a sedative dose of chloral hydrate modify the EEG of children with epilepsy? Electroencephalogr Clin Neurophysiol 1997;102(2):152-7

Clonidine Versus Chloral Hydrate for Recording Sleep EEG in Children

ObjectiveOne 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 & MethodsA 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).ConclusionThe 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.

The Effect of Different Treatments on Seed Dormancy Breaking of Weed Stalked Bur Grass Tragus racemosus (L.) AlI.

Introduction: Stalked Bur Grass (Tragus racemosus L.) is an angiosperm annual plant with C4 photosynthesis pathway and stolon. It grows in hot and dry summers. This plant spreaded throughout the world from hot regions of Africa. It is regularly seen in barren lands or in between the generations with sequential initial stages with light-texture soils. Seed dormancy is in fact a physiological phenomenon which is observed in the seeds of most crops, pasture plants, medicinal herbs and weeds. Dormancy allows the plant to guarantee its germination and survival for long years and to survive through adverse environmental conditions through its spatial and temporal spread. Materials and Methods: In order to evaluation dormancy break of Tragus racemosus L. seeds, an experiment was carried out based a Randomized Complete Block Design with four replications in research laboratory of Department of Agriculture, Birjand University during 2013. The initial experiments showed that the seeds of Stalked Bur Grass had initial dormancy and were unable to germinate at normal conditions, so that less than 5% of the seeds germinated. The studied treatments for breaking seeds dormancy included control (seeds disinfection by distilled water), wet chilling at 4°C for 1, 2, 3 and 4 weeds, treatment with H2SO4 at 97% for 20, 40, 60 and 80 seconds, treatment with KNO3 at 0.2, 0.4, 0.6 and 0.8% for 24 hours and treatment with Gibberellic acid (GA3) at 50, 100, 200 and 400 ppm. In this study, 25 seeds of Stalked Bur Grass were uniformly placed in petri dishes with the diameters of 9 cm on Watmann filter papers and were applied with 5 mL distilled water. The number of germinated seeds was counted on a daily basis for 21 days. In the end, germination percentage and rate was determined. Results and Discussion: The results revealed that the effect of all studied levels of all treatments were significant on germination percentage and rate. The highest germination percentage (76%) was observed under wet chilling treatment at 4°C for 4 weeds and the lowest one (6%) was observed in control. The highest germination percentage under H2SO4 treatment was 41% obtained at the level of 80 seconds, under KNO3 treatment was 69% obtained at the level of 0.8%, and under GA3 treatment was 62% obtained at the level of 400 ppm. The highest germination rate (18.24 seeds per day) was observed at KNO3 treatment (0.8%) and the lowest one (0.91 seeds per day) was observed in control. In addition, the highest germination rate under H 2SO4 treatment was 15.28 seeds per day obtained at the level of 80 seconds, under wet chilling was 13.25 seeds per day obtained at the level of 3 weeks, and under GA3 treatment was 12.08 seeds per day obtained at the level of 200 ppm. Wet chilling enhances the production of such stimulants as gibberellins. On the other hand, chilling treatment may reduce ABA amount or the sensitivity of embryo to ABA which can play a role in seeds dormancy breaking. KNO3 is likely to increase the sensitivity of germinating seeds to light acting as a complement factor for phytochrome which results in higher germination of the seeds. Most researchers believe that dormancy is broken by the balance between growth inhibitors like abscisic acid and growth stimulators like gibberellins. In addition, gibberellins activate a special signaling pathway that reduces abscisic acid in seeds and in contrast, auxins and cytokinins of the seeds are increased to a level enough for inducing dormancy break. Conclusion: In the present study, the germinated seeds were counted for 21 days. The highest germination percentage (76%) was observed under wet chilling treatment for 4 weeks and the lowest one (6%) was observed under control treatment. The highest germination rate (18.24 seeds per day) was observed under KNO3treatment (0.8%) and the lowest rate (0.91 seeds per day) was reported under control treatment. According to the results it can be concluded that the dormancy of Stalked Bur Grass seeds belongs to physiological dormancy type

Clinical Short Term Outcome of Guillain-Barré Syndrome in Children

Objective: Several factors are useful in predicting the prognosis of Guillain-Barre syndrome (GBS). The objective of this study was to determine the role of clinical presentation scaling to predict patient's short-term outcome. Material & Methods: Forty five patients with the confirmed diagnosis of GBS, according to international diagnostic criteria, were enrolled in this study. All children who were not able to walk unaided (i.e., ordinal disability score=ODS ≥3) were treated with intravenous immunoglobulin (IVIg) alone or with corticosteroid.The primary outcome measures were the degree of disability at discharge, length of hospital stay, need to intensive care setting and mortality. Findings: Male to female ratio was 1.05: 1 with mean age of 5.9 years. The most common manifestation was limb weakness (71.1%). Absent or decreased deep tendon reflexes were seen in 44% and 53.3% patients, respectively. All children experienced some degree of pain, with moderate to severe intensity (pain faces score ≥3) in 91.2% patients. Cranial nerve involvement was found in 46.7% children, most commonly as bulbar weakness (40%). Ten (22.2%) patients were admitted in PICU, and ventilation support was needed for 2 (4.4%) of them. Clinical response was regain of unaided walking (ODS≤2) which was achieved in 62.2% patients. After treatment all patients developed significant improvement of functional disability which was assessed by ODS and arm function scores. A higher ODS at presentation was associated significantly with a longer hospital stay (P=0.03) and higher arm function score (P<0.001). Absent tendon reflexes and cranial nerve involvement were associated with higher functional scores, longer hospital stay and admission in PICU. Also, higher arm function scores were associated significantly with intensive care unit admission (P=0.01). Conclusion: These results indicate that the ODS and arm function scores can be applied as prognostic factor for clinical short-term outcome among GBS patients

Implementation of Feed Efficiency in Iranian Holstein Breeding Program

This study aimed to evaluate the economic impact of improving feed efficiency on breeding objectives for Iranian Holsteins. Production and economic data from seven dairy herds were used to estimate the economic values of different traits, and a meta-analysis was conducted to analyze the genetic relationships between feed efficiency and other traits. Economic weights were calculated for various traits, with mean values per cow and per year across herds estimated at USD 0.34/kg for milk yield, USD 6.93/kg for fat yield, USD 5.53/kg for protein yield, USD −1.68/kg for dry matter intake, USD −1.70/kg for residual feed intake, USD 0.47/month for productive life, and USD −2.71/day for days open. The Iranian selection index was revised to improve feed efficiency, and the feed efficiency sub-index (FE$) introduced by the Holstein Association of the United States of America was adopted to reflect Iran’s economic and production systems. However, there were discrepancies between Iranian and US genetic coefficients in the sub-index, which could be attributed to differences in genetic and phenotypic parameters, as well as the economic value of each trait. More accurate estimates of economic values for each trait in FE$ could be obtained by collecting dry matter intake from Iranian herds and conducting genetic evaluations for residual feed intake

Factors affecting the outcome of community-acquired pneumonia among the patients hospitalized in Beheshti hospital (Kashan-Iran)

Background: Community-acquired pneumonia (CAP) is a common infectious disease with high morbidity and mortality. The goal of this study was to evaluate the factors affecting the outcome of pneumonia among the Beheshti hospital patients. Materials and Methods: This cohort study was done on pneumonia patients (n=140) in Kashan Beheshti hospital during 2014-2015. A questionnaire consisting the demographic, clinical and paraclinical findings and outcomes was filled-out. Results: Eighty three (59.3%) out of 140 patients were male and 57(40.7%) women. The majority of cases were ≥50 years old (mean age 60.02±1.70) .There was a history of diabetes in 54(38.6%) .The most common signs and symptoms were coughing and the lung rales. The ninty-one and 9% of the cases were improved and complicated condition, respectively. The complication were: pleural effusion (77%), empiyema (15%) and abscess (8%).There was positive CRP (100%); increased ESR (82%) and leukocytosis (80%). While, there was no statistical association between the sex, age and clinical symptoms with the disease complication and outcome, there was a significant correlation between the first BS, HbA1C, CRP, duration of hospitalization, radiographic pattern and diabetes with disease outcome. Conclusion: Considering the association between the diabetes in one side and some factors (outcome of pneumonia, duration of hospitalization, history of pneumonia, times of admission, BS at admission, HbA1c, bilateral involvement, leukocytosis, increased ESR, CRP and CURB 65, the diabetes should be considered as an important factor affecting the pneumonia outcome. The managed control of diabetes can improve the pneumonia outcome

Detection of Duchenne/Becker Muscular Dystrophy Carriers in a Group of Iranian Families by Linkage Analysis

This study determines the value of linkage analysis using six RFLP markers for carrier detection and prenatal diagnosis in familial DMD/BMD cases and their family members for the first time in the Iranian population. We studied the dystrophin gene in 33 unrelated patients with clinical diagnosis of DMD or BMD. Subsequently, we determined the rate of heterozygosity for six intragenic RFLP markers in the mothers of patients with dystrophin gene deletions. Finally, we studied the efficiency of linkage analysis by using RFLP markers for carrier status detection of DMD/BMD. In 63.6% of the patients we found one or more deletions. The most common heterozygous RFLP marker with 57.1% heterozygosity was pERT87.15Taq1. More than 80% of mothers in two groups of familial or non-familial cases had at least two heterozygous markers. Family linkage analysis was informative in more than 80% of the cases, allowing for accurate carrier detection. We found that linkage analysis using these six RFLP markers for carrier detection and prenatal diagnosis is a rapid, easy, reliable, and inexpensive method, suitable for most routine diagnostic services. The heterozygosity frequency of these markers is high enough in the Iranian population to allow carrier detection and prenatal diagnosis of DMD/BMD in more than 80% of familial cases in Iran