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OBJECTIVE: Anaphylaxis is a severe hypersensitivity reaction with a rapid onset and is potentially life-threatening if not treated promptly. This study aimed to determine the level of knowledge of pediatricians in Turkey in recognizing and treating the clinical symptoms of anaphylaxis, compare the previous studies conducted in Turkey chronologically, and show the current trends on awareness of anaphylaxis in developed and developing countries in the world. SUBJECTS AND METHODS: Pediatric residents and specialists from all over Turkey were included in the study. A questionnaire was prepared by compiling the current literature. Questions were sent to pediatricians via online applications. Statistical tests were used to analyze the data. RESULTS: A total of 524 pediatricians participated in the study. All participants accepted that anaphylaxis was a life-threatening condition. Almost all suggested epinephrine as the primary drug used in anaphylaxis. The proportion of pediatricians who knew the appropriate dose, route of administration, and place of administration of epinephrine was 82.8%, 88.9%, and 89.7%, respectively. The rate of pediatricians who recognized the clinical features of anaphylaxis was over 90%. The proportion of pediatricians who knew the epinephrine auto-injector and dose was 74.4% and 53.1%, respectively. Pediatricians with less than 10 years of experience and those working in public hospitals had better knowledge about atypical symptoms of anaphylaxis. CONCLUSIONS: Although there are still inadequacies in identifying atypical symptoms and treating anaphylaxis, our study revealed that the level of awareness of anaphylaxis had shown an increasing trend in Turkey over time. On the other hand, the knowledge on diagnosing and treating anaphylaxis still needs to be improved, especially for physicians working in rural areas of developing countries.

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Oxygen-derived free radicals have been implicated in the pathogenesis of renal injury after ischemia-reperfusion. Caffeic acid phenethyl ester (CAPE), an active component of propolis extract, exhibits antioxidant and anti-inflammatory properties. To determine whether CAPE offers any advantage over alpha-tocopherol, we compared their effects on an in vivo model of renal ischemia-reperfusion injury in rats. CAPE at 10 micromol/kg or alpha-tocopherol at 10 mg/kg was administered intraperitoneally before reperfusion. Acute administration of CAPE suppressed ischemia-reperfusion induced renal lipid peroxidation and tissue injury more than alpha-tocopherol. CAPE may therefore offer a therapeutic advantage in acute injury settings.

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Infantile-onset megalencephalic leucoencephalopathy (IML) is a recently recognized autosomal recessive white matter disorder. Unlike other megalencephalic leucoencephalopathies, in patients with IML a mild clinical course, a slowly progressive delay in motor development and mild mental deterioration are typical. We report on two affected siblings who have typical clinical and radiological findings of IML. Cranial magnetic resonance imaging showed involvement of the capsula externa, extrema and interna, nucleus dentatus, crus cerebri, periventricular and subcortical white matter. In addition, bilateral cystic changes were determined predominantly in the temporal lobes. There were no clear biochemical or metabolic disturbances. In the present paper, we discuss the clinical and neuroimaging findings of IML.

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Children with valvar pulmonary stenosis have right ventricular diastolic filling abnormalities that may be due to either right ventricular hypertrophy or right ventricular outflow obstruction. In order to investigate the reason for this abnormality, 23 consecutive cases with pulmonary stenosis (mean age 7.94 +/- 3.33 years) undergoing transluminal pulmonary balloon valvuloplasty without significant tricuspid or pulmonary valvar regurgitation were studied prospectively. Right ventricular diastolic filling indices and pulmonary valvar systolic gradients were measured in these children one day before and after pulmonary balloon valvuloplasty and were re-examined six months later. Right ventricular diastolic indices based on rapid early diastolic filling peak velocity (peak E), peak velocity during atrial contraction (peak A), and ratio of E/A were determined by pulsed Doppler echocardiography. In conclusion, right ventricular diastolic filling indices in patients with pulmonary stenosis did not improve after pulmonary balloon valvuloplasty in the first day but when re-examined by the sixth month there was a significant improvement. These data suggest that diastolic filling abnormalities are more likely a result of right ventricular hypertrophy than of right ventricular outflow obstruction.

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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. All children had acute or chronic respiratory symptoms; cough was the most frequent. The diagnosis was made by posterior-anterior (PA) and lateral chest X-rays. The PA chest X-rays showed a homogenous mass in 2 and a gas-filled cystic image in 3 children in the right cardiophrenic angle. A retrocardiac homogeneous density in one child, and bilateral consolidation in lower lung areas in another child were also seen. All lateral chest X-rays showed gas-filled bowel loops above the diaphragm. The diagnosis was confirmed by barium-contrast radiograph. Four patients had five additional anomalies, i.e., ventricular septal defect, right inguinal hernia, congenital hip dislocation, pectus carinatum, and obstruction of the uretero-pelvic junction. All of the hernias were repaired by an abdominal approach. There were no complications or recurrences during follow-up. In conclusion, MH should be considered in the differential diagnosis of cases of long-standing respiratory symptoms and/or when an unexplained radiological image, especially on the right cardiophrenic area, is present.

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In order to determine the efficiency of intranasal midazolam in prolonged convulsive episodes, we conducted a prospective study in children with various types of seizures. Nine patients (six boys, three girls; age range 6 months to 9 years) with prolonged convulsions lasting more than 10 min were treated with intranasal midazolam, 0.3 mg/kg. The success rate was 100% with only one case requiring a second dose. Estimated duration of seizures was 12-30 min (mean 18.6) while mean time elapsed until cessation of seizures was 139.6 s (range 60-480). No significant adverse effects were noted except for one patient who had seizures secondary to serious CNS infection and respiratory depression after intranasal midazolam.

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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.

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BACKGROUND: Accidental intrathecal vincristine (VCR) administration results in severe neurotoxicity, usually fatal in outcome. No specific therapy for initrathecal VCR toxicity has been reported so far. In our recent report, complete in vitro degradation of VCR by hypochlorous acid (HOCl) was demonstrated. METHODS: In this comparative study, we examined the in vivo effectiveness of HOCl in the cerebrospinal fluid of 24 New Zealand rabbits following intracisternal VCR administration. RESULTS: There were no significant clinical or histopathologic abnormalities in the control and HOCl groups; however, multiple necrotic foci on histopathological examination of brain sections in the VCR group were determined. There were significantly lower numbers of necrotic foci in brain sections of rabbits which received HOCl administration than those without therapy. CONCLUSION: Our results indicate that HOCl may reduce VCR neurotoxicity.

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Brucellosis can present initially with its haematological findings including anaemia, leukopenia, and thrombocytopenia and may mimic primary haematological diseases. We present two patients with complaints of severe epistaxis and isolated thrombocytopenia which was initially diagnosed as idiopathic thrombocytopenic purpura but which was finally attributed to brucellosis. Their platelet count reverted to normal within 2-3 weeks of initiating antibrucellosis treatment with recovery from the disease.

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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.

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