Can Mean Platelet Volume Value Be Used as Inflammation Marker in Children with Familial Mediterranean Fever?

Aim:Familial Mediterranean Fever (FMF) is disrupted response to inflammation by the organism as a result of mutations in the MEFV gene. It has been shown that the mean platelet volume could be used as a marker in many inflammatory diseases. In this study, it was aimed to determine inflammation by observing the changes in the mean platelet volume in the complete blood count.Materials and Methods:The study included 570 children diagnosed with the FMF based on the Tel-Hashomer Criteria. We also included 73 healthy children as a control group. Demographic features, results of genetic analyses, complete blood count parameters, mean platelet volume levels, and C-reactive protein results were evaluated retrospectively. Data of the patients were recorded separately based on the acute attack and attack-free periods under treatment.Results:The mean platelet volume was found to be 8.4±2.4 fL in the attack period and 9.5±1.9 fL in the attack-free period. The mean platelet volume was significantly lower in patients with an attack period compared to the patients at an attack-free period. Leukocyte count and C-reactive protein were statistically significantly higher in the attack period compared to the attack-free period.Conclusion:The mean platelet volume decreases in patients with FMF with the effect of inflammation during the attack period. We think that it is important to consider mean platelet volume while evaluating the blood count

Left ventricular non-compaction in children and adolescents: Clinical features, treatment and follow-up

Background: Left ventricular non-compaction (LVNC) is a specific cardiomyopathy that occurs following a disruption of endomyocardial morphogenesis. This study presents clinical findings, diagnostic features, treatment and follow-up of pediatric patients diagnosed with LVNC. Methods: Patients with LVNC who were followed from January 2006 to March 2010 were included in this study. Diagnosis was made with the use of characteristic findings of magnetic resonance imaging and echocardiography. Holter electrocardiography and metabolic screening tests were also performed in all patients. Results: A total of 24 patients were studied (18 male, six female). Patient age at diagnosis was 50 ± 60 months (eight days to 15 years). Average follow-up period was 22 ± 12 months (four months to four years). Findings at diagnosis were as follows: eight (33%) patients had heart failure, five (20%) had rhythm abnormalities, five (20%) had cardiomegaly, two had murmurs, two had cyanosis, and two presented with fatigue. Ten (41%) patients had been followed previously with other diagnoses. In 21 (87.5%) patients, electrocardiographic abnormalities were noted, especially left ventricular hypertrophy and ST-T changes. Patients had an average ejection fraction of 46% (18-73%) and three of them had additional congenital heart disease (patent ductus arteriosus, aortopulmonary window and complex cyanotic heart disease). Scanning for metabolic diseases revealed fatty acid oxidation disorder in one patient, and mitochondrial disease in another. During follow-up, a permanent pacemaker was implanted in a patient with severe bradycardia and ventricular dysfunction, and three patients died. Conclusion: LVNC can be diagnosed at any age from newborn to adolescent and has a variable clinical course. Closer study of patients with cardiomegaly and heart failure can reduce delays in diagnosis of LVNC. (Cardiol J 2011; 18, 2: 176-184

Antinuclear antibody testing in a Turkish pediatrics clinic: is it always necessary?

Introduction: the term anti-nuclear antibody (ANA) is used to define a large group of autoantibodies which specifically bind to nuclear elements. Although healthy individuals may also have ANA positivity, the measurement of ANA is generally used in the diagnosis of autoimmune disorders. However, various studies have shown that ANA testing may be overused, especially in pediatrics clinics. Our aim was to investigate the reasons for antinuclear antibody (ANA) testing in the general pediatrics and pediatric rheumatology clinics of our hospital and to determine whether ANA testing was ordered appropriately by evaluating chief complaints and the ultimate diagnoses of these cases. Methods: the medical records of pediatric patients in whom ANA testing was performed between January 2014 and June 2016 were retrospectively evaluated. Subjects were grouped according to the indication for ANA testing and ANA titers. Results: ANA tests were ordered in a total of 409 patients during the study period, with 113 positive ANA results. The ANA test was ordered mostly due to joint pain (50% of the study population). There was an increased likelihood of autoimmune rheumatic diseases (ARDs) with higher ANA titer. The positive predictive value of an ANA test was 16% for any connective tissue disease and 13% for lupus in the pediatric setting. Conclusion: in the current study, more than one-fourth of the subjects were found to have ANA positivity, while only 15% were ultimately diagnosed with ARDs. Our findings underline the importance of an increased awareness of correct indications for ANA testing

Signal-averaged electrocardiogram may be a beneficial prognostic procedure in the postoperative follow-up tetralogy of fallot patients to determine the risk of ventricular arrhythmias

Early detection of arrhythmias after congenital heart disease surgery is important because it can help decrease morbidity and mortality. Standard electrocardiograms (ECGs) contain frequencies between 0.05 and 100 Hz, but higher frequencies are also present. Using high-resolution technology, the highest amplitudes of these high-frequency components within the QRS complex can be recorded and analyzed. We studied the relationship between ventricular late potentials, ventricular arrhythmias and right ventricular systolic pressure in 22 patients who underwent tetralogy of Fallot repair (mean follow-up, 40.1 +/- 33.5 months). Holter ECG monitoring and signal-averaged electrocardiograms (SAECGs) were performed. SAECG parameters studied included the duration of the filtered QRS, the duration of terminal QRS below 40 mu V, and the root mean square amplitude of the terminal 40 msec. Cardiac catheterization was performed on 19 patients. Eighteen healthy volunteers were studied as a control. Ventricular arrhythmias were found in 13 patients; right ventricular systolic hypertension was found in 1 patient. No significant residual ventricular septal defects were detected. Eight patients had ventricular late potentials. Right ventricular systolic pressure did not differ significantly between patients with or without late potentials. There were significant differences between patients with ventricular arrhythmias and healthy volunteers; filtered QRS duration was significantly longer in patients with ventricular arrhythmias. SAECG may be beneficial in determining ventricular arrhythmia risk in tetralogy of Fallot patients postoperatively

Pandemic Influenza A (H1N1) virus-associated acute myopericarditis

Although pandemic influenza A (H1N1) virus may cause an upper respiratory tract infection similar to that caused by seasonal influenza, it can lead to serious complications such as pneumonia, encephalitis, and myocarditis. We describe a 2-year-old girl admitted with fever, cough, and vomiting followed by acute respiratory distress and diagnosed as H1N1-related myopericarditis. (Turk Arch Ped 2011; 46: 337-9

Pandemik İnfluenza A (H1N1) virüsü ile ilişkili akut miyoperikardit

Although pandemic influenza A (H1N1) virus may cause an upper respiratory tract infection similar to that caused by seasonal influenza, it can lead to serious complications such as pneumonia, encephalitis, and myocarditis. We describe a 2-year-old girl admitted with fever, cough, and vomiting followed by acute respiratory distress and diagnosed as H1N1-related myopericarditis. (Turk Arch Ped 2011; 46: 337-9

Results of surgical treatment for infective endocarditis in children

Objective: Infective endocarditis is uncommon condition, with a high degree of morbidity and mortality. It is less common in children, albeit tending to be associated with congenital cardiac malformations. We describe Our experience of the need for surgical treatment in children with infective endocarditis. Patients and methods: We analyzed retrospectively the records of 9 children aged below 16 years seen between May 2003 and March 2005 with infective endocarditis, reviewing the demographic details, clinical presentation, microbiological and echocardiographic data, operative findings, and outcome. Results: Apart from pre-existing renal insufficiency in 1. patient, congenital cardiac malformations were the predisposing factors. Blood cultures were positive in 3, but remained negative in the other 6 patients. The indications for Surgical treatment Included uncontrolled sepsis, congestive heart failure, recurrent endocarditis, patch or graft dehiscence, and pseudoaneursymal formation. Death due to uncontrolled sepsis resulting in multiorgan failure occurred in 1. patient, who had tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. Another patient died late postoperatively due to cardiac failure after relapse of the endocarditis in the setting of negative blood cultures. Conclusion: Despite advances in antimicrobial therapy, diagnosis, and measures of treatment for infective endocarditis, complications continue to be responsible for substantial morbidity and mortality. Since blood Cultures are frequently negative, clinical and echocardiographic findings should be the major determinants of strategies used for treatment. We believe that our small series of patients seen over the past two years in which Surgical treatment was performed will be helpful in guiding the clinical perspectives for children with infective endocarditis

The comparison a 16-year follow-up results of balloon angioplasty for aortic coarctation in children of different age groups: a single-center experience

Objective: Pediatric patients with different age groups who underwent balloon angioplasty for aortic coarctation were evaluated for recoarctation, aneurysm, peripheral arterial injuries and concomitant diseases

Electrocardiographic and echocardiographic findings in street children known to be substance abusers

Substance abuse is prevalent in adolescent street children, and death is reported as secondary to aspiration, accidental trauma, asphyxia, cardiac arrhythmia, anoxia, vagal inhibition and respiratory depression. In this study, we examined electrocardiographic and echocardiographic findings from 53 street male adolescents, comparing our findings to those obtained from 61 controls in the same age group. The street children smoked cigarettes (98.1%) and had used, or were using, thinner (73.6%), glue (75.5%), hashish (79.2%), morphine or its products (24.5%), ecstasy (37.7%), anti-emetics (13.2%) and alcohol (60.4%). On examination, their blood pressures were lower than the control group. Electrocardiographically, PR, QRS, QT were found to be longer (p less than 0.05) than the values for healthy controls. Although it was not statistically significant, QTc duration was also longer than the control group. Echocardiography revealed increased diameters of the left ventricle and atrium, the aorta, and the coronary arteries as compared to the healthy children (p less than 0.05)

Ultrasonic evaluation of early atherosclerosis in children and adolescents with type 1 diabetes mellitus

Objective: To assess early atherosclerosis using B-mode imaging of the carotid artery in children and adolescents with type 1 diabetes mellitus (T1DM) and to evaluate the relationship between various risk factors and intimal plus medial thickness (IMT) in this population.Methods: Fifty-two children and adolescents (aged 3-18 years) with uncomplicated T1DM and 43 age- and gender-matched healthy controls were examined. B-mode imaging was used to determine the intimal plus medial thickness (IMT) of the carotid artery in all subjects. Patients with T1DM and control subjects were divided into two groups according to age and gender. Furthermore, duration of DM was considered for comparison.Results: Patients and control subjects showed no association between IMT and sex, systolic blood pressure (sBP), diastolic blood pressure (dBP), serum lipid levels or left ventricular ejection fraction (LVEF). However, statistical Analysis indicated a good correlation between age and carotid arterial wall thickness in both diabetic and control groups. These findings were consistent with those in the literature. No correlation was found between IMT and the duration of DM.Conclusions: This study indicates that there is no association between T1DM and IMT in children and adolescents with T1DM