Hanifi Soylu

Buccal midazolam for treatment of prolonged seizures in children

Midazolam is a relatively new anticonvulsive agent in the benzodiazepine group. It has a short onset of duration and is practical for use, providing several alternatives such as intravenous, intramuscular, and intranasal routes. The buccal route could be an alternative choice for seizure control in an emergency setting. However, no sufficient reports are available on buccal midazolam administration. The present study was designated to examine the efficacy of buccal midazolam in children at different ages with seizures of more than 5 min duration. Nineteen previously unreported children, aged from 1 month to 15 years, were treated with a 0.3 mg/kg dose of buccal midazolam; 13 had prolonged seizures, and six had status epilepticus, with a duration of 5-45 min (mean 22 min). Sixteen of 19 seizures (84.2%) stopped within 10 min of buccal midazolam being given. The drug efficacy in patients with status epilepticus was 50%. However, all patients with convulsions shorter than 30 min showed a perfect response (100%). Convulsion episodes stopped within 3.89+/-2.22 min (median time 3 min). Seizure duration was correlated with cessation of seizure (r=0.76, P<0.001). No clinically important side effects were seen in any patient. On the basis of this experience, we concluded that a 0.3 mg/kg dose of buccal administration of midazolam might offer an effective treatment in all ages of children.

Morgagni hernia: an unexpected cause of respiratory complaints and a chest mass.

Morgagni hernia (MH) is the least common type of congenital diaphragmatic hernias. Although its course is often asymptomatic, it may be associated with various respiratory and gastrointestinal symptoms. We describe 7 children with MH during a 5‐year period in three pediatric centers in Turkey.

Rupture of membranes before the age of viability and birth after the age of viability: comparison of outcomes in a matched cohort study.

Objective:To compare composite adverse outcome rate of infants <32 weeks gestational age (GA) who were born after preterm premature rupture of membranes (PPROM) at previable gestation to those born without PPROM.Study Design:Retrospective review of prospective collected data for infants discharged between 2004 and 2007 was conducted. Cases were infants with >7 days of PPROM that occurred before 24 weeks. Matched cohort consisted of infants born without PPROM (matched for GA, sex and admission date). Composite adverse outcome was assessed considering death or any of the following three severe morbidities (severe neurological injury, severe retinopathy of prematurity or chronic lung disease).Result:The 29 cases had higher mean severity of illness score compared with 74 matched infants. Mean duration of ROM was 45 vs 2 days and mean GA at the ROM was 21 vs 27 weeks, respectively. Logistic regression confirmed significantly higher risk of composite adverse outcome rates for cases (69 vs 47%; P=0.02, adjusted odds ratio 4.0, 95% CI 1.2, 13.6).Conclusion:The survival rate for infants born at <32 weeks following PPROM at previable age has improved significantly; however, these infants had a higher rate of adverse composite neonatal outcome.

Regulation of adenosine levels during cerebral ischemia

Adenosine is a neuromodulator with its level increasing up to 100-fold during ischemic events, and attenuates the excitotoxic neuronal injury. Adenosine is produced both intracellularly and extracellularly, and nucleoside transport proteins transfer adenosine across plasma membranes. Adenosine levels and receptor-mediated effects of adenosine are regulated by intracellular ATP consumption, cellular release of ATP, metabolism of extracellular ATP (and other adenine nucleotides), adenosine influx, adenosine efflux and adenosine metabolism. Recent studies have used genetically modified mice to investigate the relative contributions of intra- and extracellular pathways for adenosine formation. The importance of cortical or hippocampal neurons as a source or a sink of adenosine under basal and hypoxic/ischemic conditions was addressed through the use of transgenic mice expressing human equilibrative nucleoside transporter 1 (hENT1) under the control of a promoter for neuron-specific enolase. From these studies, we conclude that ATP consumption within neurons is the primary source of adenosine in neuronal cultures, but not in hippocampal slices or in vivo mice exposed to ischemic conditions.

Leukoencephalopathy with a mild clinical course : a case report

Infantile-onset leukoencephalopathy of van der Knaap type is manifested by initially normal or near normal neurological findings despite infantile-onset megalencephaly and magnetic resonance imaging evidence of severe white matter affection. Until this entity was recently described, these cases were usually presented under the heading of atypical variants of Alexander disease. To date 63 individuals have been reported in English literature. We report a four-year-old boy presented in the first months of life with progressive megalencephaly, delay in walking, clumsiness, convulsions and magnetic resonance imaging evidence of diffuse swelling of white matter, cystic cavitations in frontal, temporal and parietal lobes.

Intracortical injection of endothelin-1 induces cortical infarcts in mice: effect of neuronal expression of an adenosine transporter

BackgroundActivation of adenosine A1 receptors has neuroprotective effects in animal stroke models. Adenosine levels are regulated by nucleoside transporters. In vitro studies showed that neuron-specific expression of human equilibrative nucleoside transporter 1 (hENT1) decreases extracellular adenosine levels and adenosine A1 receptor activity. In this study, we tested the effect of hENT1 expression on cortical infarct size following intracerebral injection of the vasoconstrictor endothelin-1 (ET-1) or saline.MethodsMice underwent stereotaxic intracortical injection of ET-1 (1 μl; 400 pmol) or saline (1 μl). Some mice received the adenosine receptor antagonist caffeine (25 mg/kg, intraperitoneal) 30 minutes prior to ET-1. Perfusion and T2-weighted magnetic resonance imaging (MRI) were used to measure cerebral blood flow (CBF) and subsequent infarct size, respectively.ResultsET-1 reduced CBF at the injection site to 7.3 ± 1.3% (n = 12) in hENT1 transgenic (Tg) and 12.5 ± 2.0% (n = 13) in wild type (Wt) mice. At 48 hours following ET-1 injection, CBF was partially restored to 35.8 ± 4.5% in Tg and to 45.2 ± 6.3% in Wt mice; infarct sizes were significantly greater in Tg (9 ± 1.1 mm3) than Wt (5.4 ± 0.8 mm3) mice. Saline-treated Tg and Wt mice had modest decreases in CBF and infarcts were less than 1 mm3. For mice treated with caffeine, CBF values and infarct sizes were not significantly different between Tg and Wt mice.ConclusionsET-1 produced greater ischemic injury in hENT1 Tg than in Wt mice. This genotype difference was not observed in mice that had received caffeine. These data indicate that hENT1 Tg mice have reduced ischemia-evoked increases in adenosine receptor activity compared to Wt mice.

Prune-belly syndrome and pulmonary hypoplasia: A potential cause of death

condition affecting mainly the abdominal wall and the genitourinary system.1 In addition, respiratory, cardiovascular, gastrointestinal and orthopedic anomalies may accompany PBS.2 Early recognition and treatment of PBS may prevent serious complications that may lead to fatalities. Herein, we report on a typical case of PBS in an infant, with a complex of genitourinary, respiratory, orthopedic and gastrointestinal anomalies, who died due to respiratory failure following a common respiratory infection.

Urinary excretion of deoxypyridinoline in 24-hour and first-void samples in healthy Turkish children

OBJECTIVES Collagen cross-links are formed during the maturation process of bone matrix. They have been considered as valuable markers in some metabolic, endocrinologic, and neoplastic bone disorders. As an advantage, it can be measured in urine as well as in serum samples. However, the excretion characteristics remains controversial. DESIGN AND METHODS We investigated urinary free deoxypyridinoline (f-Dpd) excretion in first-void urine samples and in 24-hour collections in healthy Turkish children. We also evaluated the possible correlations and gender-related differences in Dpd excretion between these sampling methods. Both urine samples of 62 subjects (aged from 31 to 120 months) were analyzed by Immulite chemiluminescent technique. RESULTS There were no remarkable differences in f-Dpd excretion between first-void and 24-hour urine samples, although f-Dpd values of the first-void samples were slightly higher (Dpd: creatinine, mean +/- SD, 20.5 +/- 5.8 nmol/mmol vs. 19.6 +/- 5.6 nmol/mmol, respectively, p > 0.05). A strong linear correlation was found between 24-hour and first-void urine f-Dpd excretions (r = 0.77, p < 0.05). In addition, f-Dpd showed no gender-related differences between boys and girls in either 24-hour or first-void urine samples (p > 0.05). CONCLUSIONS Because of difficulties in long-time urine collection in infants and young children, f-Dpd assessment in first-void single urine samples is an easy, safe, and non-invasive method.

Rosary-like giant coronary artery aneurysms in Kawasaki disease: Diagnosis with prospectively ECG-gated dual source CT angiography.

A seven-year-old boy was admitted to our hospital with fever, sore throat, cervical lymphadenomegaly and bilateral non-purulent conjunctivitis of 4 days’ duration. Laboratory results revealed: leukocyte count 19,700/mm3, hematocrit 31.5%, platelets 698,000/mm3, erythrocyte sedimentation rate 85 mm/h and C-reactive protein 171 mg/L. Echocardiography was unremarkable. A diagnosis of incomplete Kawasaki disease was made. Ten days later, a followup echocardiography revealed a proximal right coronary

SECKEL'S SYNDROME ASSOCIATED WITH CONGENITAL CYSTIC ADENOMATOID MALFORMATION OF THE LUNG

Sir, A 14-month-old girl was referred to our clinic with growth retardation, dyspnea and cough. She was the first child of parents who were first-degree relatives and was born following a full-term uncomplicated gestation and labor. Physical examination revealed a mild mentally retarded baby with microcephaly (head circumference 36 cm) and proportioned growth retardation (height 64 cm, weight 7200 8). Her face was characteristic with a receding forehead, large beaked nose, micrognathia, antimongoloid eyeslant and large eyes (Fig. 1). Furthermore, clinodactyly of the fifth finger and single palmar crease on the right were evident and the fontanelles were closed. Decreased breath sounds were heard on the right hemithorax. Laboratory studies yielded a hemoglobin concentration of 10.4 g/dl and white blood cells 9600/~1 with 56% segmented neutrophils and 44% lymphocytes. Routine biochemical screening, thyroid hormones, arterial blood-gases and chromosomal analyses were normal. Pulmonary function tests showed a restriction pattern. On skull graphy, narrowed sutures and fontanelles according to age were noted. Chest X-ray indicated two large radiolucent and thin-walled masses filled with gas on the right middle lobe. Thorax CT showed multiple thin-walled cysts filled with gas on the right middle lobe (Fig. 2). The patient was operated on and right middle lobectomy was performed. Macroscopic examination of the excised material showed multiple thin-walled cysts, 1 2cm in diameter. Microscopic examination of the material indicated multiple cysts resembling terminal bronchioles, lined by cuboidal epithelium. A thin fibromuscular layer underlay the larger cysts but was absent in the smaller ones (Fig. 3). A diagnosis of cystic adenomatoid malformation of the lung (CCAM), type 11, was made. After operation she showed near-normal total lung function. Seckel’s syndrome is a rare disorder with autosomal recessive transmission (1). It is characterized by failure to thrive, mental retardation, bird-like malformation of the face and further abnormalities (1, 2). These abnormalities are low-set malformed ears, clinodactyly of the fifth finger, absence of some phalangeal epiphyses, hypoplasia of the proximal radius, dislocation of the hip, hypoplasia of the proximal fibula, absent ribs, cryptorchidism, congenital heart disease and marrow hypoplasia (1 -3).