Evaluation of pediatricians' awareness about anaphylaxis.

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.

Comparison of blood brain barrier permeability in normal and ovariectomized female rats that demonstrate right or left paw preference.

We explored the relations among paw preference, cerebral asymmetry and asymmetrical disruption of blood-brain barrier (BBB) permeability in normal and ovariectomized female rats with known paw preference. A high dose of pentylenetetrazol was used to disrupt the BBB and induce acute hypertension. To determine the areas of macroscopic infarct, samples were stained with 2,3,5-triphenyltetrazolium chloride. Histological staining techniques were used to show the areas of infarct microscopically on paraffin sections. Sixty-two percent of the rats demonstrated right paw preference, 24% demonstrated left paw preference and 14% were ambidextrous. Areas of infarct, which indicated destruction of the BBB, were determined microscopically and macroscopically in rats that demonstrated right and left paw preference. We found a relation between permeability of the BBB and paw preference. There may be a relation between paw preference, cerebral asymmetry and asymmetrical destruction of the BBB in rats. Asymmetrical destruction of the BBB in experimental rats was similar to the control group, which had asymmetrically disrupted BBB with respect to paw preference. Like the control rats, asymmetrical areas of infarct consistent with cerebral asymmetry were observed in ovariectomized rats.

Differences in the cellular and humoral immune system between middle-aged men with different intensity and duration of physically training.

AIM: The effects of acute exercise on immune system and serum magnesium and iron have been investigated in recent years. However, data related to the comparisons of long-term physical training with different intensity and duration are limited. METHODS: The association between long-term physical training and cellular (lymphocyte phenotyping) and humoral immune parameters (serum immunoglobulins) and serum magnesium and iron values in the middle-aged men was investigated. Eleven male master athletes (MA) performing high intensity and long duration training, 11 male recreational athletes (RA) performing moderate intensity and duration training (>10 years) participated. Eleven male sedentary individuals were enrolled as control group (CG). RESULTS: The percentages of total CD3+ T cells, CD4+ T helper, CD8+ T suppressor/cytotoxic, CD19+ B cells, natural killer cells, HLA-DR+ active T cells and CD4/CD8 ratios did not show any significant difference among 3 groups. In MA, VO2max values showed a significant negative correlation with CD4+ T helper cells. There were no significant differences among MA, RA and CG in terms of IgG, IgA, and IgM concentrations. There was a significant correlation between VO2max and IgG in RA. Iron, iron binding capacity and ferritin were found similar in all groups, but serum magnesium level in MA was significantly lower than RA and CG. CONCLUSION: No exact data to support immunosuppression or immunostimulation could be obtained except a significant negative correlation between CD4+ T helper cells and VO2max values in MA and a positive correlation between serum IgG and VO2max ivalues in RA. These findings may be the indirect markers of cellular immune system suppression by intensive exercises and stimulation of IgG production by moderate exercises.

Protective role of alpha-tocopherol and caffeic acid phenethyl ester on ischemia-reperfusion injury via nitric oxide and myeloperoxidase in rat kidneys.

BACKGROUND: The aim of this study was to determine the acute effects of antioxidant caffeic acid phenethyl ester (CAPE) and alpha-tocopherol (vitamin E) on nitric oxide (NO) production, neutrophil infiltration, and antioxidant enzyme activities on an in vivo model of renal ischemia-reperfusion injury. METHODS: Rats were divided into five equal groups each consisting six rats: sham operation, ischemia, ischemia-reperfusion (I/R), I/R plus CAPE, and I/R plus vitamin E groups. CAPE or vitamin E was administered intraperitoneally before reperfusion. After experimental procedure, rats were sacrificed and both ipsilateral and contralateral kidneys were removed and prepared for NO concentrations, myeloperoxidase (MPO), catalase (CAT) and superoxide dismutase (SOD) activities. RESULTS: Acute administration of vitamin E decreased NO concentrations in both ipsilateral and contralateral renal tissues compared to I/R group. SOD activity was increased in I/R and I/R + CAPE groups compared to sham operation group. The most prominent results were encountered in MPO activities, which did not change in contralateral kidneys in both ischemia and I/R groups. There was a significant decrease in ipsilateral MPO activity in ischemia group and a significant increase in I/R group compared to sham operation group. Pretreatment with intraperitoneal CAPE significantly diminished the tissue MPO activity indicating the prevention of the neutrophil sequestration into the kidney. CONCLUSION: There is a role for CAPE in attenuation in renal damage after I/R injury of the kidney, in part at least by inhibition of neutrophil sequestration.

The effect of caffeic acid phenethyl ester on ischemia-reperfusion injury in comparison with alpha-tocopherol in rat kidneys.

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.

Testosterone and nonverbal intelligence in right-handed men with successful and unsuccessful educational levels.

The relationship between serum total testosterone (T) concentration and fluid intelligence (nonverbal, spatial) was studied in consistently right-handed men with successful (S) or unsuccessful educational levels (NS). Hand preference was assessed by the Edinburgh Handedness Inventory. Nonverbal intelligence was measured by Cattell's Culture Fair Intelligence Test. Serum T level was determined using chemiluminescence enzyme-immunoassay on hormone autoanalyzer. There was no significant difference between the mean T levels of the S subjects and NS subjects, although S-men tended to have higher T levels than NS-men. The mean IQ was found to be significantly higher in S-men than NS-men. In the total sample (S + NS men), the correlation between T to IQ was best described by a polynomial regression (3rd order), exhibiting an inverse U-shaped regression. In S-men, the relationship between T and IQ was best described by a polynomial regression equation of the 3rd order; however, the relationship was not U-shaped, but rather a positive correlation (low T: low IQ and high T high IQ). In NS-men, there was an inverse U-shaped correlation between T and IQ (low and very high T: low IQ and moderate T: high IQ). The present data suggest that (i) very low and very high serum T concentrations may be disadvantageous, (ii) moderate T levels may be advantageous for general fluid intelligence, and (iii) a prewired cerebral organization may be essential for the T effects on cognitive abilities.

Infantile-onset megalencephalic leucoencephalopathy in two siblings.

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.

Ventricular diastolic filling indices in pulmonary stenosis.

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.

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. 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.

Intranasal midazolam for prolonged convulsive seizures.

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.

Potential salvage therapy for accidental intrathecal vincristine administration: a preliminary experimental study.

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.

Autosomal recessive multiple pterygium syndrome: a new variant?

Multiple pterygium syndromes include at least 15 different entities characterized by multiple pterygia or webs of the skin and multiple congenital anomalies. We describe a female infant who presented with a distinct constellation of multiple anomalies consisting of pterygia of the inguinal, intercrural and popliteal areas, flexion contractures and arthrogryposis of some joints, craniofacial anomalies including ectropion, medial canthal web, blepharophimosis, hypoplasia of nose, oral and nasopharyngeal cavities, vocal cords and tongue, micrognathia, orolabial synechiae secondary to pterygia, low set ears, alopecia, sad and expressionless face, short neck, asymmetric nipples, anal stenosis, rectal polyp, hypoplastic labia majora, complete syndactyly of all fingers and toes, pes equinovarus, bandlike web between feet, and absence of the nails and phalangeal-palmar creases. Radiological examination showed synostosis, absence or hypoplasia of metacarpal, metatarsal and phalangeal bones on feet and hands, and hypoplasia of pelvic bones and scapulae. This pattern of anomalies does not fit entirely any of the known multiple pterygium syndromes. Autosomal recessive inheritance is most likely due to the presence of three similarly affected siblings and normal parents.

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.

Severe epistaxis in brucellosis-induced isolated thrombocytopenia: a report of two cases.

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.

Roberts SC phocomelia with isolated cleft palate, thrombocytopenia, and eosinophilia.

The Roberts-SC (Pseudothalidomide) syndrome is a rare autosomal recessive disorder. We present a Roberts-SC Syndrome in a 20-day-old girl with phocomelia of the upper limbs, isolated cleft palate, micrognathia, prominent eyes, pectus excavatum, and pes equinovarus. Peripheral blood smear revealed thrombocytopenia and hypereosinophilia. Premature centromere separation in the child and also in her normal mother was noted.