Midazolam efficacy and side effects in generalized and partial refractory status epilepticus in children

Midazolam is a significant and effective drug for control of a life-threatening condition, generalized and partial refractory convulsive status epilepticus. The goal of this study was evaluation of midazolam efficacy for management of this serious disease and its two side effects, hypotension and respiratory failure. Our study was done using a quasi experimental method; 22 children with generalized refractory convulsive status epilepticus and 13 with partial refractory convulsive status epilepticus were enrolled for the study. All patients received 0.2mg/kg/dose as a bolus intravenous midazolam followed by 1-6 mcg/kg/min continuous intravenous midazolam. Following this, termination of seizures as well as hypotension and respiratory failure were evaluated. midazolam ceased stop convulsions in 81.81% [18] patients with generalized seizures, and in 76.92% [10] patients with partial seizures, showing no significant difference between these two types of seizures [p=0.52]. Hypotension was induced in 18.18% [4] patients with generalized seizures and in 30.70% [4] patients with partial seizures, again difference not significant [p=0.14]. There was respiratory failure in 21.73% [5] patients with generalized seizure and in 7.69[1] patients with partial seizure, difference not significant [p=0.09]. There was no significant difference in efficacy and creation of hypotension and respiratory failure after continuous intravenous infusion of midazolam between generalized and partial refractory convulsive status epilepticus

Iron status in febrile seizure: a case-control study

Data on the relationship between iron deficiency anemia and febrile convulsions are controversial. The aim of this study was to determine the association between iron deficiency anemia and febrile convulsions among children. This case-control study was conducted during 2006-2007, on 90 children with febrile seizures [case] and 90 febrile children without seizures [control] referred to the Amirkola children hospital [a referral hospital in the north of Iran]. Two groups were matched for age and sex. In all children hemoglobin [Hb] level, hematocrit [Hct], mean corpuscular volume [MCV], mean corpuscular hemoglobin [MCH] and plasma ferritin [PF] were determined and the data collected were analyzed statistically using the t-test. The mean PF and TIBC levels were not significantly different in the febrile seizure compared to the reference group; neither were differences in Hb levels statistically significant between two groups. However MCV and MCH were significantly higher in the febrile seizure group [p<0.05] compared to reference group. Plasma ferritin levels were not significantly lower in children with febrile seizures in comparison with the children in control group. It seems that iron insufficiency does not play a role in pediatric febrile seizures

Spinal muscular atrophy from northern Iran: a clinical and genetic spectrum of ten patients

Autosomal recessive spinal muscular atrophy [SMA] is, after cystic fibrosis, the second most common fatal monogenic disorder and the second most common hereditary neuromuscular disease after duchenne dystrophy. The disease is characterized by degeneration of anterior horn cells leading to progressive paralysis with muscular atrophy. Depending on the clinical type [Werdnig-Hoffmann = type I, intermediate form = type II, Kugelberg-Welander = type III], some workers also have delineated an adult form of SMA [SMA type 4]. SMA causes early death or increasing disability in childhood. The aim of this investigation was to describe the clinical findings of patients with spinal muscular atrophy [SMA] with survival motor neuron [SMN] gene deletion. This is a descriptive study conducted on 10 patients of SMA, confirmed by deletion of the SMN gene. All 10 patients had symmetrical muscle weakness, which was diffuse in those with onset of symptoms up to 1 months of age, and either proximal or predominant in lower limbs. Frequency determination of positive clinical and laboratory data was done according to revised diagnostic criteria. It was found that all patients with SMA had homozygous deletions of exons 7 and 8 of the survival motor neuron 1 [SMNl] gene, which is one of the candidate genes identified within 5q13. Fasciculations, atrophy and decreased DTR were frequent findings. Laboratory metabolic tests and all brain CT scans were normal. EMC and NCV findings, all showed normal motor and Sensory NCV and denervation of muscles of upper and lower extremities were compatible with a diagnosis of spinal muscular atrophy. Our results confirm that SMN1 copy number analysis is an important parameter for identification of couples at risk of having a child affected with SMA and reduces unwarranted prenatal diagnosis for SMA