Comparison of deflazacort and prednisone in duchenne muscular dystrophy

Duchenne muscular dystrophy [DMD] is a degenerative disease that usually becomes clinically detectable in childhood as progressive proximal weakness. No cure is yet available for DMD, but the use of steroids improves muscle strength and function. This study has been carried out to select the best steroid for the management of DMD. This study is a single-blind, randomized clinical trial with a sample volume of 34 DMD patients. Half of these patients were treated with deflazacort [0.9 mg/kg daily] and the other half with prednisone [0.75 mg/kg daily] for a period of 18 months. The motor function score and excess body weight were registered one year after the start and also at the end of the study and compared between the two groups. Deflazacort was more effective in the improvement of motor function after one year, but there was no significant difference between the two drugs at the end of the study [18 months after start]. Weight gain after one year and at the end of the study was higher in prednisone group and steroid treatment with deflazacort appears to cause fewer side effects than prednisone regarding weight gain. Deflazacort seems to be more effective than prednisone in the improvement of motor function causing fewer side effects, particularly weight gain. This medication may be important for the improvement of motor function and could be used as the best steroidal treatment for Duchenne muscular dystrophy

Efficacy of continuous high dose midazolam infusion in childhood refractory generalized convulsive status epilepticus

Prolonged and uncontrolled refractory status epilepticus [SE] is a life-threatening medical emergency in children [1, 2, 3]. There is no consensus on the optimal therapy for refractory status epilepticus [1]. The aim of this study was to develop a new method for treating patients with refractory status epilepticus. Ten children with refractory status epilepticus in Mofid Hospital, who did not respond to 10 microg/kg per min of intravenous midazolam, had their dose of midazolam increased to 30 microg/kg per min. All children were monitored for the development of side effects. Ten children with no response to low-dose midazolam were given a higher dose of midazolam, and 5 [50%] children had a good response. These patients had significantly different response to high-dose midazolam. One patient in the high-dose midazolam group was intubated and required mechanical ventilation. The duration of stay in the hospital and PICU and on mechanical ventilation in patients with no response to low-dose midazolam following with other drugs was longer than in the high-dose midazolam group. No death occurred in high dose midazolam group. High-midazolam dose drip infusion is a safe and effective protocol for refractory status epilepticus in children

survey of pediatrics resident knowledge of growth and development

The primary objective of this study was to evaluate pediatric residents' knowledge about the milestones of growth and development in children. Seventy-one pediatric residents from 3 main Children's Centers in Tehran were surveyed to evaluate their knowledge on growth and development of children. Included in the survey was based upon filling up a questionnaire containing questions about four different categories of development [motor, cognitive, speech and social interactions]. The scores that the residents got from the test were summed up and were compared together. 71 resident participants completed the questionnaire. The distribution of residents by year was 26 post graduate year 1 [PGY1], 26 PGY2, and 23 PGY3.The knowledge of 14 [19.7%] of the participants about child's development was ranked as "Less than expected", 47 [66.2%] as "Unacceptable", 10 [14.1%] as "Acceptable" and none [0%] as "Ideal", according to the total score they got from their correct answers to the questions. This study describes that most pediatric residents lack the acceptable knowledge on children's growth and development. It seems that their knowledge did not improve through their years of residency

effect of the ketogenic diet on the growth and biochemical parameters of the children with resistant epilepsy

The aim of this study was to evaluate the effect of the ketogenic diet on the growth parameters of the children with resistant epilepsy. A total of 36 children with resistant epilepsy who were 2 to 7 year old were put on the ketogenic diet. Their growth and biochemical parameters were studied at the beginning of the study and after 3 months. Weight decreased in all patients. Serum levels of hemoglobin, calcium, and blood sugar decreased significantly but remained in the normal range. Creatinine did not change, but BUN showed a significant increase. We can lower the complications of ketogenic diets by using more unsaturated fat, more water, and more minerals

Risk factors and prognosis of epilepsy in children with hemiparetic cerebral palsy

Epilepsy is reported in 15-90% of the children with Cerebral Palsy [CP] but its clinical course is not well defined. We conducted a retrospective study on children with hemiparetic CP who were referred to Pediatric Neurology Department of Mofid Hospital. The aim of our study was to evaluate the risk factors and prognosis of epilepsy in children with hemiparetic CP. We evaluated 64 children with hemiparetic CP who were referred to Pediatric Neurology Department of Mofid Hospital 2006 and 2008. according to our protocol, patients were divided into two roups: 34 children in the case group [hemiparetic patients with epilepsy] and 30 children in the control group [hemiparetic patients without epilepsy]. Prenatal, perinatal and postnatal events, maternal age at the time of delivery, socioeconomic status of the family, familial history of epilepsy, neuroimaging findings, side of the hemiparesia and age at diagnosis of hemiparesis were not considered as risk factors for epilepsy in hemiparetic children, but microcephaly, severity of hemiparetic CP and mental retardation were significantly associated with an increased risk of epilepsy in children with hemiparetic CP. Our study showed that microcephaly, severity of hemiparesis and mental retardation were risk factors for developing epilepsy in children with hemiparetic CP; furthermore, they had negative effects on rehabilitation outcome in these patients

Association of EEG abnormality and developmental delay in phenylketonuria [PKU]: an analytic historical case-control study

Electroencephalogram [EEG] is an easy and non invasive evaluation method for diagnosis and early prognosis in children. Our aim was to assess the association between EEG and the patients' Developmental Quotient [DQ] level in phenylketonuria. In this study, 94 PKU patients [45 boys, 49 girls; mean age 8.5 +/- 6.2 years] who were diagnosed through newborn screening tests or later were followed. PKU was confirmed with a serum phenylalanie concentration above 6 mg/dl in untreated newborns. The patients were matched in gender, age and phenylalanine level. The ASQ [Age and Stage Questionnaire] was used for evaluation of the developmental stage of the case [abnormal EEG] and control [normal EEG] groups and the Wechsler Intelligence Scale was used to assess cognitive and intellectual abilities. Finally, one way ANOVA and chi square tests were used for analysis and P<0.05 was considered significant. The case group consisted of 63 patients [67%] with abnormal EEGs and the control group consisted of 31 patients [33%] with normal EEGs. In patients with abnormal EEGs, 34 [53%] had mile, nine [14% had moderate and 20 [33%] had severe EEG changes. Distribution of high and low DQ levels in the abnormal and normal EEG patients showed a significant difference [p=0.001]. There was no significant difference between phenylalanine level in case and control groups; therefore, EEG findings may affect patients' developmental scores despite a normal phenylalanine level in PKU patients

CNS involvement by novel influenza virus type A [H1N1], the first report from Iran

This is the first report of CNS involvement by the new influenza virus [influenza A [H1N1] in Iran. The patient was a 10-year-old boy with chief complaints of fever, malaise, and cranial nerve involvement, resulting in respiratory muscle paralysis and intubation. This shows that the new influenza virus, as well as the seasonal flu, can cause neurologic complications; however, the severity of the signs and symptoms is less and the disease may resolve without complications in the case of seasonal flu. Therefore, in each patient with neurological involvement and typical influenza signs and symptoms or a flu-like syndrome, diagnostic tests for H1N1 flu virus should be considered, especially during epidemics, and treatment with oseltamivir should be started

Relationship between celiac disease and refractory idiopathic epilepsy in children

Epilepsy occurs with a yearly incidence of 40 per 100,000 children, of which more than 25% are resistant to drug therapy. Epilepsy may occur in autoimmune diseases like lupus, celiac disease and myasthenia gravis. In this study, the relationship between celiac disease and refractory epilepsy was evaluated in children with idiopathic epilepsy. Hundred-fifty-five children [mean age, 6.7 +/- 3.3 years] with idiopathic and cryptogenic epilepsy referred to the neurology clinic were studied in two groups; drug controlled epilepsy [control, 82 patients] and refractory epilepsy groups [case,73 patients]. Both groups underwent serological tissue transglutaminase antibody measurement by ELISA. In seropositive cases, small intestine biopsy was conducted. Data analysis was performed using student's t test and 2 test. Seven [0.04%] patients had celiac disease based on a positive tissue transglutaminase antibody and three patients [0.01%] based on a positive biopsy. Three patients [2.4%] with drug controlled epilepsy [control group] and five with refractory epilepsy [case group] had seropositive celiac disease [p=0.255]. In the biopsy survey of six seropositive patients, one patient [1.2%] in the drug controlled epilepsy and two patients [2.7%] in the refractory epilepsy group had positive biopsy for celiac disease [p =0.604]. One seropositive patient did not cooperate for biopsy. If the relationship between celiac disease and epilepsy, especially in cases of symptomatic or oligosymptomatic celiac is proved, using gluten free diet increases the ability to control epilepsy particularly in refractory cases. We suggest celiac disease survey is not required in patients with idiopathic epilepsy

Acute inflammatory demyelinating polyneuropathy in children; clinical and electrophysiologic findings

The aim of this study was to evaluate the electrophysiologic findings of Guillain Barre Syndrome [GBS] in children and their relation with clinical progress of the disease. Twenty-three children with GBS were evaluated between 2005 and 2007. Electrophysiologic evaluations were performed at admission and one month later. Five patients needed respirator, 15 were bedridden, 1 developed recurrence 6 months later, and 2 experienced chronic GBS. The most common findings included: decreased amplitude of muscle action potential [CMAP] [96%], increased distal latency [74%], increased F wave latency [69%], and decreased nerve conduction velocity [NCV] [61%]. Sensory nerve conduction [evaluating sural nerve] was normal in 78% of the cases. These measures did not significantly change after 1 month. Electrodiagnostic evaluations are helpful at the primary stages of GBS for diagnosis. Fibrillation potentials and positive sharp waves showing denervation and axonal injury are presentative of longer duration of the disease and a worse prognosis