1,720,963 research outputs found
Immune mediated myasthenia gravis in children, current concepts and new treatments: A narrative review article
Myasthenia gravis (MG) is the most frequent transmission disease in the neuromuscular junction. Juvenile myasthenia gravis (JMG) is an autoimmune antibody-mediated disease of postsynaptic endplate defined as MG presentation in patients before the age of 18 years old. While many clinical features of JMG are identical to the adults, there are some significant differences between them regarding presentation, clinical course, antibody level, and thymus histopathology. In JMG, ocular symptoms are more frequent, the clinical course is comparably benign, and the outcome is better than adult MG. Antibodies attack the muscle endplate proteins in the postsynaptic membrane and interfere with transmission. These antibodies in most patients are against the acetylcholine receptors, but they may also be directed toward muscle-specific kinase, lipoprotein-related protein 4, and agrin. Findings show racial influences and genetic effects on the occurrence of JMG. The essential clinical symptom is fatigable weakness of muscles that can be in the form of isolated ocular type or more disseminated weakness. The diagnosis of JMG is essentially clinical, with fluctuating patterns of weakness and easy fatigability, but a series of diagnostic evaluations can confirm the diagnosis. Precise diagnostic evaluation and distinction from congenital myasthenic syndromes is critical. The treatment plan is conducted according to the clinical course (ocular or generalized), antibody type, and disease severity. The mainstay of treatment includes symptomatic therapy, long-lasting immunosuppressive treatment and treatment of myasthenic crisis. Novel medications are introduced and conducted to the specific pathophysiologic mechanisms of the disease, and they are used primarily in the refractory MG
Globoid Cell Leukodystrophy (Krabbe Disease)
How to Cite This Article: TAvasoli A. Globoid Cell Leukodystrophy (Krabbe Disease). Iran J Child Neurol. Autumn 2014;8;4(Suppl.1):14-15.pls see pdf
Going Beyond Counting First Authors in Author Co-citation Analysis
The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
acute tansverse myelitis in children
Acute transverse myelitis (ATM) is a rare inflammatory and demyelinating disorder of spinal cord with an immune – mediated basis (1). It is potentially a devastating disorder with variable outcomes that can present with subacute onset of motor, sensory and autonomic dysfunction.(2) TM can occur as an isolated condition, usually as a postinfectious disorder or may be the first presentation of relapsing acquired demyelinating syndromes (ADS) that include acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), and neuromyelitis optica (NMO). ATM also, must be distinguished from compressive and noninflamatory myelopathies. Diagnostic evaluation, prognosis, recurrence risk, and treatment plans are different among these entitie
Renal Function in Children with Febrile Convulsions
How to Cite This Article: Afsharkhas L, Tavasoli A. Renal Function in Children with Febrile Convulsions.Iran J Child Neurol. 2014 Autumn;8(4):57-61.AbstractObjectiveFebrile convulsions (FC) are the most frequent seizure disorder in children.Some studies have detected serum electrolyte disturbances in patients with FC.This study determines serum electrolytes, renal function tests, and frequency of urinary tract infection in hospitalized children with FC.Materials & MethodsIn this descriptive, cross sectional study, we evaluated 291 children with FC admitted to the Neurology ward of Ali-Asghar Children’s Hospital from 2008–2013. Data was recorded on age, sex, type (simple, complex), and recurrence of seizures, family history of FC and epilepsy, serum electrolytes, renal function tests, and urinary tract infections.ResultsA total of 291 patients with diagnosis of FC were admitted to our center. Of these 291 patients, 181 (62.2%) were male. The mean age was 24.4 ± 14.6 months.There were simple, complex, and recurrent FCs in 215 (73.9%), 76 (26.1%) and 61 (21%) of patients, respectively. Urinary tract infections (UTI) were found in 13 (4.5%) patients, more present in females (p-value = 0.03) and under 12 months of age (p-value = 0.003). Hyponatremia, hypocalcemia, and hypokalemia was detected in 32 (11%), 16 (5.5%), and 4 (1.4%) of cases, respectively. Twentyfour (8.2%) patients had a glomerular filtration rate less than 60 ml/min/1.73m2.There were no abnormalities in serum magnesium, BUN, and creatinine levels.ConclusionDuring FCs, mild changes may occur in renal function but a serum electrolyte evaluation is not necessary unless patients are dehydrated. In children with FC, urinary tract infections should be ruled out. ReferencesGhofrani M. Febrile Convulsion: Another look at an old subject. Iran J Child Neurology 2006 June:1(1):5-9.Swaiman K, Ashwal S, Ferriero D, Schor N. Swaiman’s Pediatric Neurology: Principles and Practice. 5th edition.2012.Pp.790-798.Mohammadi M. Febrile Seizures: Four Steps Algorithmic Clinical Approach. Iranian Journal of Pediatrics 2010; 20(1):5-15.Commission on Epidemiology and Prognosis, International League against Epilepsy. Guidelines for epidemiologic studies on epilepsy. Epilepsia 1993;34(4):592–6.Saghazadeh A, Mastrangelo M, N. Genetic background of febrile seizures. Rev Neurosci 2014;25(1):129-61.NP. Evaluation of the child who convulses with fever. Pediatr 2003;5(7):457-61.Karimzadeh P, Fahimzad A, Poormehdi MS. Febrile Convulsions: The Role Played By Paraclinical Evaluation. Iran J Child Neurology 2008;2(4):21-24.Kwong KL, Tong KS, So KT. Management of febrile convulsion: scene in a regional hospital. Hong Kong Med J 2003 Oct; 9(5):319-22.Amiri M, Farzin L, Moassesi ME, Sajadi F. Serum trace element levels in febrile convulsion. Biol Trace Elem Res 2010; 135(1-3):38-44.Amouian S, Mohammadian S, Behnampour N Tizrou M. Trace Elements in Febrile Seizure Compared to Febrile Children Admitted to an Academic Hospital in Iran, 2011. Journal of Clinical and Diagnostic Research 2013 Oct;7(10): 2231-2233.Behrman E R. Nelson Textbook of Pediatrics . In: Bleyer A, Ritchey A.K. Nephrology section. 19th edition. 2011. P.1731-1845.Momen A, Monajemzadeh SM. the Frequency of Urinary Tract Infection among Children with Febrile Convulsion. Iranian Journal of Child Neurology 2011: 1(1):29-31.Sahib El-Radhi A, Carroll J, Klein N. Clinical Manual of Fever in Children. Springer. 2011. P.8-9.Heydarian, F, Ashrafzadeh F, Kam S. Simple Febrile Seizure: The Role of Serum Sodium Levels In prediction of Seizure Recurrence during the First 24 Hours. Iranian Journal of Child Neurology 2009;3(2):31-34.Nickavar A, Hassanpour H, Sotoudeh K. Validity of Serum Sodium and Calcium Screening in Children with Febrile Convulsion. Acta Medica Iranica 2009;47(3):229-231.Talebian A, Vakili Z, Talar SA, Kazemi SM, Mousavi GA. Assessment of the relation between serum zinc & magnesium levels in children with febrile convulsion. Iran J Pathol 2009; 4(4):157-160.Teach SJ, Geil PA. Incidence of bacteremia, Urinary Tract Infection & Unsuspected bacterial meningitis in children with febrile seizures. Pediatric Emergency care 1999; 15(1)9-12
The association between failure to thrive or anemia and febrile seizures in children between 6 months to 6 years old age
Objectives: Febrile seizure is the most common seizure disorder in childhood. Anemia or failure to thrive can predispose children to Febrile seizure by affecting the nervous system function. The current study investigates the association between febrile seizures and Anemia or failure to thrive.Materials and Methods: This case-control study was performed on 307children 6 months to 6 years old age hospitalized at the Ali Asghar children`s Hospital from 2011 to 2014 divided into two groups: a case group including 158 children with febrile seizures and a control group including 149 febrile children without seizure. The amount of Hgb, Hct, RBC count, MCV, MCH, and MCHC was recorded and weight-for-age and weight-for-height was calculated based on the WHO Z-Score charts.The date were compared between two groups.Results: There were no differences regarding age and sex between the groups. Statistically significant differences were found regarding the mean RBC count between the case group (4.38×106 ± 0.72×106) and the control group (4.24×106 ± 0.84×106) (p=0.013), as well as about the mean MCV that was 78.73 ± 0.97 and 76.78 ± 1.00 in the case and control groups respectively(p=0.005). Anemia was seen in 28.5% of the case and 42.3% of control group which was statistically significant (p=0.012). There was not statistically significant difference regarding failure to thrive between two groups.Conclusion: in children with febrile seizures anemia was lower comparing with febrile children without seizure. Moreover, there was not any association between failure to thrive and febrile seizures.
Variations on the Author
“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
We provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Dispelling the Myths Behind First-author Citation Counts
We conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
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