Comparative efficacy of risdiplam and nusinersen in Type 2 and 3 spinal muscular atrophy patients: A cohort study using real-world data.

Background: Three medications have been approved for spinal muscular atrophy (SMA) treatment. No head-to-head clinical trials have directly compared the efficacy of nusinersen and risdiplam. We compare the efficacy of them in Type 2 and 3 SMA patients, with 6 months of follow-up.

QT Interval Changes in Children With Febrile Convulsion Compared With Healthy.

Introduction: Febrile seizure is a temperature-related seizure that affects the QT interval. The purpose of this study was to evaluate the changes in the QT interval caused by febrile convulsion (FC) compared with healthy children.

Methods: This case-control study examined 180 children equally distributed between patients and controls.

Comparing Prophylactic Effect of Levetiracetam, Sodium Valproate, and Propranolol in Pediatric Migraine: A Randomized Clinical Trial.

Objectives: Epidemiologic studies point to an increased prevalence of migraine in children in recent decades. Migraine treatment involves acute and prophylactic therapy. Recently, such anti-epileptic drugs as Levetiracetam have been used to treat adult migraines.

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).

Identification of a Q278X Mutation in an Iranian Patient with Neuronal Ceroid Lipofuscinosis 2: Literature Review and Mutations Update.

Neuronal ceroid lipofuscinoses type 2 (CLN2), the most common form of Batten disease, is caused by TPP1 loss of function, resulting in tripeptidyl peptidase-1 enzyme deficiency and cerebral accumulation of lipopigments. Clinical hallmarks include epileptic seizures, vision loss, progressive movement disorder, ataxia, and eventually death. Diagnosis is often delayed due to the rarity of the conditions.

Tunable broadband polarization converters based on coded graphene metasurfaces.

In this paper, two optimization algorithms (randomly initialized hill climbing and genetic algorithms) are considered to design broadband polarization converters based on coded metasurfaces. A pixeled graphene patch with an elliptic structure is proposed for the initial solution. Each pixel can be 1 and 0 which represents the presence and absence of the graphene.

Fulminant and Diffuse Cerebral Toxoplasmosis as the First Manifestation of HIV Infection: A Case Presentation and Review of the Literature.

BACKGROUND One of the most common causes of central nervous system (CNS) opportunistic infections in immunocompromised patients is toxoplasmosis. It can cause focal or disseminated brain lesions leading to neurological deficit, coma, and death. Prompt management with optimal antibiotics is vital.

Prevalence of Febrile Seizures in Children in Zahedan, South East of Iran.

Objectives: Febrile seizure is the most common seizure disorder in childhood and a common cause of hospitalization in hospitals. We aimed to investigate the prevalence of febrile seizures in children in Zahedan, south-east of Iran.

Materials & Methods: In this cross-sectional study, 600 children under 7 yr were examined for positive history of febrile seizure in 2014.

Comparison of the Effect of Phenobarbital versus Sodium Valproate in Management of Children with Status Epilepticus.

Objectives: Acute prolonged seizure is the most common neurological emergency in children. This research was conducted to compare the effect of intravenous phenobarbital and Sodium valproate in control of seizure in children with status epilepticus, referred to emergency ward from Mar to Nov 2013.

Materials & Methods: In this randomized clinical trial, registered with the code number IRCT2015051722300N1, 80 children aged 6 months to 10 years with prolonged seizure and with no response to one dose of diazepam (0.

Molecular genetic study of Calpainopathy in Iran.

Introduction: Calpainopathy is an autosomal recessive form of limb girdle muscular dystrophies (LGMDs) caused by mutations in the CAPN3 gene. CAPN3 is a Ca-dependent cystein protease consisting of 821 amino acids. LGMD is a highly heterogeneous disorder and mutation identification of this disease by Sanger sequencing of all genes is expensive and time consuming.

Chronic Myelogenous Leukemia Presenting with Facial Nerve Palsy in an Infant.

When a child presents with cranial nerve palsy and a bulging fontanel, a pediatric neurologist is often consulted to determine the cause of increased intracranial pressure. This report describes an infant with chronic myelogenous leukemia (CML) referred to Ali-bin-Abitaleb Hospital, Zahedan, eastern Iran in 2013 who presented with seventh cranial nerve palsy and bulging fontanel. Chromosomal analysis and peripheral blood smear confirmed the diagnosis of CML.

Comparison of Effectiveness of Topiramate and Diazepam in Preventing Risk of Recurrent Febrile Seizure in Children under Age of 2 Years.

Objective: Febrile seizures are the most common type of convulsions. Medicinal prophylaxis is sometimes used for children at high risk of recurrent febrile seizure. In certain circumstances, conventional drugs such as diazepam and phenobarbital cannot be used and the need for alternative medicines is felt.

Bone Metabolism Disorder in Epileptic Children.

Objective: There are frequent anti-epileptic drugs used in management of epilepsy. Anti-epileptic drugs may have some complications on bone and vitamin D metabolism. This study aimed to comparison the bone metabolism disorder in epileptic children with healthy child in Zahedan, eastern Iran from Jul 2014 to Jun 2015.

Prevalence of G6PD deficiency in Children with Hepatitis A.

Hepatitis A virus is the most prevalent viral hepatitis. It is globally a major public health problem with different clinical symptoms. This study aimed at investigating the clinical findings and prevalence of glucose 6-phosphate dehydrogenase (G6PD) deficiency in children with hepatitis A.

Omega 3 in Childhood Migraines: a Double Blind Randomized Clinical Trial.

Objective: The effect of using omega-3 to prevent migraine attacks has been raised in recent studies. The majority of these studies have been conducted in adults. Conversely, other studies have yet to confirm the effect of omega-3.

Marital Status and Fertility in Adult Iranian Patients with β-Thalassemia Major.

Expecting a family is an important component and a great goal for better quality of life for most of adults with β-thalassemia major. The aim of the present study was to examine the marital status of adults with β-thalassemia major. This cross-sectional study examined the marital status of patients with transfusion-dependent β-thalassemia aged over 15 years.

Comparison of the Efficacy of Combination Therapy of Prednisolone - Acyclovir with Prednisolone Alone in Bell's Palsy.

Objective: Bell's palsy is a rapid onset, usually, unilateral paralysis of the facial nerve that causes significant changes in an individual's life such as a decline in personal, social, and educational performance. This study compared efficacy of combined prednisolone and acyclovir therapy with prednisolone alone.

Materials & Methods: This study is a randomized controlled trial conducted on 43 Children (2-18 years old) with Bell's palsy.

Comparison between the Ability of Glasgow Coma Scale and Full Outline of Unresponsiveness Score to Predict the Mortality and Discharge Rate of Pediatric Intensive Care Unit Patients.

Objective: Prediction of survival and mortality rates in costly environments such as the intensive care unit (ICU) is of great importance for the assessment of new treatments, resource consumption control, and improvement of quality control. This study aimed to determine the ability to predict mortality and discharge rate of patients using the FOUR score in the pediatric ICU (PICU) of Ali Ibn Abitalib Hospital, Zahedan and compare the results with those of Glasgow Coma Scale (GCS).

Methods: This prospective study was conducted on 200 patients admitted to the PICU.

A comparison of risperidone and buspirone for treatment of behavior disorders in children with phenylketonuria.

Objective: Many patients with late-diagnosed phenylketonuria (PKU) suffer from severe behavior problems. This study compares the effects of buspirone and risperidone on reducing behavior disorders in these patients.

Materials & Methods: In this crossover clinical trial study, patients with severe behavior disorders after medical examination were randomly divided into two groups of two 8-week crossover treatments with risperidone or buspirone.

Chronological variations of children poisoning causes in zahedan, South of iran.

Background: Poisoning is a common pediatric emergency among children and adolescents in the Emergency Department of Zahedan University of Medical Sciences hospital.

Objectives: The aim of this study was comparing the characteristics and variations of pediatric poisoning between two retrospective studies (1998 and 2008). We hypothesized that the epidemiology of pediatric patients admitted for poisoning is related to variations of environmental agents and drug usage.

Diagnosis of cystathionine beta-synthase deficiency by genetic analysis.

Intellectual disability like other common diseases is often complex because they are genetically heterogeneous, with many different genetic defects giving rise to clinically indistinguishable phenotypes. We present diagnosis of cystathionine beta-synthase (CBS) deficiency in a multiply affected Iranian family with obvious intellectual disability based on whole genome SNP homozygosity mapping. Diagnosis based on clinical presentations had not been made because of unavailability of appropriate medical services.