Adem Aydin

Efficacy of continuous midazolam infusion and mortality in childhood refractory generalized convulsive status epilepticus

PURPOSE Continuous midazolam infusion is commonly used for the management of status epilepticus (SE). The purpose of this study was to assess the efficacy of midazolam and mortality in childhood refractory generalized convulsive SE. METHODS We included 27 children with refractory generalized convulsive SE. Midazolam was given 0.2 mg/kg as bolus, followed by 1-5 microg/kg/min as continuous infusion. Clinical data and response to treatment were recorded for each patient. RESULTS Acute symptomatic SE accounted for 52%, and central nervous system (CNS) infections were the most frequently associated etiologic condition (44%). Complete control of seizures was achieved with midazolam infusion in the 26 (96%) children within 65 min; at a mean midazolam infusion rate of 3.1 microg/kg/min. Adverse effects such as hypotension, bradycardia or respiratory depression did not occur during midazolam infusion. In one (4%) patient with acute meningoencephalitis, SE could not be controlled. Five (19%) patients died; four had acute symptomatic aetiology and one had progressive encephalopathy. CONCLUSION Midazolam is effective and safe in the control of refractory generalized convulsive SE. The response to treatment and mortality were related to the underlying aetiology.

Associations of IGF-I, IGFBP-1 and IGFBP-3 on Intrauterine Growth and Early Catch-Up Growth

Fetal cord blood IGF-I, IGFBP-1 and IGFBP- 3 levels of appropriate-for-gestational-age (AGA) and intrauterine growth retardation (IUGR) babies are studied and followed up for 6–9 months, reevaluated for anthropometric measures and the effects of IGF-I, IGFBP-1 and IGFBP-3 on fetal growth and early catch-up growth is investigated. 23 AGA and 21 IUGR babies, totally 44 newborns, were included in the study protocol. IGF-I and IGFBP-3 levels were found to be high in AGAs with respect to IUGR babies and IGFBP-1 is found to be high in IUGR with respect to AGAs. IGF-I was significantly lower in IUGR babies without catch-up growth (group 2b) with respect to AGAs (group 1) and neonates with IUGR and catch-up growth (group 2a) and group 2a infants had higher IGF-I values than group 2b infants (p < 0.05). IGFBP-3 levels in group 1 were significantly higher than in the other two groups (p < 0.05), but not significantly different in group 2a with respect to group 2b (p > 0.05). IGFBP-1 values showed no statistically significant difference with respect to the three different groups (p > 0.05). A good correlation was found between birth weight, postnatal weight and postnatal height and IGF-I and IGFBP-3 levels (p < 0.05) but not with IGFBP-1 levels. Aside from the height of the 3 groups of infants which were similar to each other after the follow-up period, IGF-I was significantly high in IUGR infants with catch-up growth with respect to IUGR infants without catch-up growth, indicating its importance in early catch-up growth of IUGR babies.

Sturge-Weber syndrome without facial nevus

An 11-month-old patient with Sturge-Weber syndrome with the absence of facial angioma and normal mental development is presented. Noncontrast computed tomography revealed left parieto-occipital atrophy with heavy gyriform calcifications. Axial T(2)-weighed magnetic resonance imaging confirmed the presence of low-signal areas corresponding to the gyral calcifications evident on computed tomography. Contrast-enhanced T(1)-weighted axial and coronal images exhibited high signals in the left parieto-occipital cortical and subcortical areas, representing angiomatous malformations. The clinical appearance and pathologic features of the reported patient were compared with those of similar patients described in published reports.

Effects of sociodemographic factors on febrile convulsion prevalence

Background: The aim of the present study was to determine the lifetime risk (LTR) of febrile convulsion and the effects of socioeconomic factors affecting this risk among school children in Izmir province, Turkey.

Limb girdle muscular dystrophy type 2A presenting with cardiac arrest.

The occurence of respiratory failure in progressive neuromuscular disorders is well recognized. This failure is observed most commonly in Duchenne dystrophy but sometimes occurs in Beckers, limb-girdle, and facioscapulohumeral dystrophies. Patients usually present acutely or subacutely with cyanosis and cor pulmonale, with severe decompensation often being precipitated by an acute intercurrent infection. However, cardiopulmonary arrest is an uncommon presentation. A male diagnosed with limb-girdle muscular dystrophy type 2A who presented with cardiopulmonary arrest that was precipitated by an upper respiratory tract infection is presented. The nocturnal application of noninvasive intermittent positive pressure ventilation with a bilevel positive airway pressure (Bi-PAP) device improved his symptoms and quality of life without resorting to more-invasive and more-restrictive forms of support. This report demonstrates an unusual presentation of limb-girdle muscular dystrophy and documents that nocturnal nasal administration of continuous airway pressure using the Bi-PAP device may be sufficient to maintain adequate ventilation in such patients.The occurrence of respiratory failure in progressive neuromuscular disorders is well recognized. This failure is observed most commonly in Duchenne dystrophy but sometimes occurs in Beckers, limb-girdle, and facioscapulohumeral dystrophies. Patients usually present acutely or subacutely with cyanosis and cor pulmonale, with severe decompensation often being precipitated by an acute intercurrent infection. However, cardiopulmonary arrest is an uncommon presentation. A male diagnosed with limb-girdle muscular dystrophy type 2A who presented with cardiopulmonary arrest that was precipitated by an upper respiratory tract infection is presented. The nocturnal application of noninvasive intermittent positive pressure ventilation with a bilevel positive airway pressure (Bi-PAP) device improved his symptoms and quality of life without resorting to more-invasive and more-restrictive forms of support. This report demonstrates an unusual presentation of limb-girdle muscular dystrophy and documents that nocturnal nasal administration of continuous airway pressure using the Bi-PAP device may be sufficient to maintain adequate ventilation in such patients.

Relationship between serum unconjugated bilirubin levels and the autofluorescence of white blood cells in neonatal jaundice.

In this study, using flow cytometry, we investigated the autofluorescence emitted by lymphocytes, monocytes and neutrophils exposed to different unconjugated bilirubin concentrations and investigated the relationship between these parameters. Different unconjugated bilirubin concentrations were prepared from a newborn serum with an unconjugated bilirubin concentration of 800 m mumol/l. The same concentrations of unconjugated bilirubin have been prepared from pure bilirubin. 10 microliters of cord blood were incubated at room temperature for 15 min with 90-microliter solutions with different bilirubin concentrations prepared from both serum and pure bilirubin. After incubation, cells were washed three times with PBS, erythrocytes were lysed by lysing buffer and run through flow cytometry immediately. Autofluorescence was measured by recording mean fluorescence channels for lymphocytes, monocytes and neutrophils. There was a good correlation between serum concentrations of unconjugated bilirubin that cells were exposed to and the autofluorescence intensity of neutrophils (r = 0.904, p < 0.005), monocytes (r = 0.759, p < 0.05) and lymphocytes (r = 0.766, p < 0.01). Results obtained with pure bilirubin were also similar. Autofluorescence emitted by lymphocytes was lower than that of monocytes (p < 0.01) or neutrophils (p < 0.0005).

Thyroxine-induced hypermotor seizure

Thyroxine-induced epilepsy is a very rare condition occurring in epileptic patients. Here we report a boy with thyroxine-induced hypermotor seizure (HMS) following thyroxine administration for his central hypothyroidism secondary to surgery and cranial radiation for his brain tumor. After 3 years seizure-free period, he had repeated HMS, seven to eight attacks per day, after initiation L-thyroxine treatment. Following reduction of the daily thyroxine dose, his seizures decreased in frequency. To our knowledge, this is the first reported case of HMS associated with L-thyroxine administration.

Sociodemographic risk factors for febrile seizures: A school-based study from Izmir, Turkey

PURPOSE Despite the fact that socioeconomic and environmental factors of a population are changing over time, there are few studies focusing on the effects of sociodemographic factors on the prevalence of febrile seizures (FS). This study was designed to find out the prevalence of FS and to investigate the effect of socio-cultural and economic factors on this prevalence among the Turkish school children. METHODS A school-based, cross-sectional study was conducted in first and second-class children. Data were collected through a questionnaire from the parents who agreed to be involved in the study. The survey had questions about some socioeconomic and demographic features of the children and febrile seizure episodes. RESULTS 3806 children and parent pairs accepted to participate in the survey. Febrile seizure prevalence was 4.8%. It was found that the prevalence of FS was significantly associated with the chronic illnesses of a child that requires continuous medication, developmental delay of a child, NICU history, gestational hypertension history of a mother, and lower educational level of a mother. Recurrence of FS was observed in 32.9% of children. Children whose first FS was seen below the 39 °C had 1.9 times more recurrence risk. CONCLUSION FS prevalence rate has declined from 9.7% to 4.3% in our study population within ten years. It was thought that advancing healthcare systems in our country might be decreased the prevalence. Our study enabled us to find out sociodemographic risk factors of FS, but further studies are needed in order to confirm the effect of sociodemographic factors on FS prevalence.