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Embolization of a catheter fragment is a very rarely seen complication, and few cases have been reported in children. Catheter fragments must be urgently extracted due to life-threatening complications. Most catheter fragments are removed very soon after being lost in the cardiovascular system, including the venous system and right side of the heart. In our report, we describe a child with catheter fragment, which was removed from the left ventricle 32 days after embolization. This catheter fragment was successfully retrieved percutaneously using a gooseneck snare catheter through the femoral artery. [ABSTRACT FROM AUTHOR] more...

Objective: Abnormalities of the aortic root and ascending aorta are common in patients with a bicuspid aortic valve (BAV). The aim of this study was to evaluate the stiffness of the abdominal aorta in children with BAV. Methods: In this cross-sectional observational study, we evaluated 35 children with normally functioning or mildly regurgitant BAV and 35 healthy children as controls. All children were noninvasively evaluated with transthoracic echocardiography. Annulus of aorta and abdominal aorta diameters were measured. Aortic strain (S), pressure strain elastic modulus (Ep), pressure strain normalized by diastolic pressure (Ep*), aortic stiffness β index (βSI) and, aortic distensibility (DIS) were calculated using the measured data. In evaluation of the data Student's t-test, Chi-square test, Pearson's correlation and multivariate linear regression analysis were used. Results: Diameter of the aortic annulus was found significantly larger in the children with BAV than the control group (p<0.05). The abdominal aorta systolic and diastolic diameters were similar in the two groups (p>0.05). The children with BAV exhibited significantly lower S (0.210 ± 0.04/0.267 ± 0.07, p<0.001) and DIS (1.04 ± 0.2/1.4 ± 0.4 10(-6) cm(2) dyne(-1), p<0.001); and higher Ep (200 ± 39/153 ± 47 N/m(-2), p<0.001), Ep* (3.42 ± 0.9/2.5 ± 0.9, p<0.001) and βSI (1.1 ± 0.3/0.84 ± 0.3, p<0.001) than the control group. There was no correlation between the systolic and diastolic diameters of abdominal aorta and aortic elasticity parameters (p>0.05). Conclusion: Bicuspid aortic valve is associated with an increased abdominal aortic stiffness in children. However, impaired abdominal aortic elasticity is not due to abdominal aortic dilatation. These findings require validation by further studies. [ABSTRACT FROM AUTHOR] more...

Scimitar syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of scimitar syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of scimitar syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of scimitar syndrome. [ABSTRACT FROM AUTHOR] more...

Scimitar syndrome is a rare congenital cardiopulmonary malformation characterized by hypoplasia of the right lung and drainage of the right pulmonary veins into the vena cava inferior. It may also be associated with cardiac dextroversion and anomalies of the tracheobronchial system, cardiovascular system, and diaphragm. Some cases are asymptomatic with others diagnosed in early-childhood period with pulmonary hypoplasia and other associated malformations. We present here a patient whose venous return of the middle and lower lobes of the right lung is into the superior vena cava, which is a very unusual finding for this disorder. [ABSTRACT FROM AUTHOR] more...

Background: Much epidemiological evidence has linked low birthweight with late cardiovascular risk. Intrauterine growth retardation (IUGR) is associated with the increased risk of cardiovascular disease in adult life; it is unclear whether the relationship is present at younger ages. We evaluated whether abdominal aortic stiffness was altered in patients with IUGR (born at term with birthweight small for gestational age) in younger ages., Methods: Thirty-two (24 girls and eight boys) IUGR children aged 8.77 +/- 2.05 years were enrolled in the study. The birthweight was traced from the medical records. Their gestational ages were 38.9 +/- 0.85 weeks and birthweights 2130 +/- 198 g, respectively. Thirty-one healthy subjects who had normal gestational age and birthweight, matched for age and sex were recruited as a control group. Aortic strain, pressure strain elastic modulus (Ep), and normalized Ep and aortic distensibility were measured by a sphygmomanometer and transthoracic echocardiography in all subjects from the abdominal aorta., Results: There was no statistically significant difference between the study and the control groups in sex, mean age, body mass index, lipid profile, leptin, insulin-like growth factor-1 or insulin-like growth factor binding protein 3. In IUGR children, aortic strain (0.201 +/- 0.027 vs 0.254 +/- 0.031, P < 0.001) and aortic distensibility (1.08 +/- 0.19 vs 1.42 +/- 0.24, P < 0.001) were significantly lower compared with the control group. However Ep (188 +/- 36.2 vs 146 +/- 27.1, P < 0.001) and normalized Ep (2.97 +/- 0.40 vs 2.1 +/- 0.39, P < 0.001) were significantly higher in IUGR patients., Conclusions: This study demonstrates that abdominal aortic stiffness is increased in IUGR patients. These data suggest that prenatal events could be related to cardiovascular risk in later life. more...

Acquired partial lipodystrophy (Barraquer-Simons syndrome) is a rare condition with onset in childhood, and it is characterized by progressive loss of subcutaneous fat in a cephalocaudal fashion. Although it is known that acquired partial lipodystrophy usually follows acute febrile illness, it is very rarely reported to occur in association with varicella. In this case report, we present a seven-year-old girl with progressive loss of fat in her face just after varicella who was diagnosed as acquired partial lipodystrophy. more...

Although genetic and environmental factors contribute to the pathogenesis of juvenile rheumathoid arthritis (JRA), the etiology and pathogenesis remain controversial. The objective of this study was to investigate genotypic and allelic frequencies of monocyte chemoattractant protein-1 (MCP-1) gene -2518 (G/A) polymorphism in the healthy Turkish population and patients with JRA. Genomic DNA was collected from 66 JRA patients and 150 healthy individuals. To evaluate the association of the -2518 (G/A) MCP-1 gene polymorphism with the outcome of JRA, we analyzed the types of JRA and the score on the childhood health assessment questionnaire (C-HAQ score). In the healthy Turkish population, the frequencies of A and G alleles were 71 and 29%, respectively. No significant difference was observed between the JRA patients and healthy subjects in the distribution allelic and genotypic frequencies of the -2518 (G/A) MCP-1 gene polymorphism (p>0.05). However, the AG genotype was found to be higher and the AA genotype was found to be lower in the patients with systemic type JRA compared to those with the other types of JRA (p=0.019). When the JRA patients were evaluated according to the C-HAQ score, we found that the -2518 (G/A) MCP-1 gene polymorphism did not relate the prognosis (p>0.05). AG genotype was found to be higher in the systemic type of JRA. The results indicate that MCP-1 gene polymorphism might slightly associate with patients with systemic JRA. Further studies are needed to elucidate the role of this polymorphism in the pathogenesis of JRA in various populations because this polymorphism has a functional significance and an ethnic difference. more...

The objectives of this study were to determine genotypic and allelic frequencies of macrophage migration inhibitory factor (MIF) gene -173 G/C polymorphism in patients with juvenile rheumatoid arthritis (JRA) and to evaluate the association of the MIF -173 C allele with the outcome of JRA. Genomic DNA was collected from 67 JRA patients and 153 healthy individuals. To evaluate the association of the MIF -173 polymorphism with the outcome, we analyzed the data concerning the treatment regimen, duration of glucocorticoid treatment, score on the childhood health assessment questionnaire (C-HAQ) and the number of joints with active arthritis. Nonsignificant differences were observed between the study and control groups in the distribution of genotype and allele frequencies of the MIF gene -173 G/C polymorphism. In JRA patients, carrying a MIF -173 C allele, the number of disease modifying antirheumatic drugs required for the treatment was more, the duration of glucocorticoid treatment was significantly longer, and at the last visits the C-HAQ scores and the number of joints with active arthritis were significantly higher. MIF gene -173 C allele frequency did not differ between the controls and JRA patients. MIF -173 C allele did not confer increased susceptibility to JRA in our study group. Carriage of the MIF -173 C allele was found to be a strong predictor of poor outcome in all types of JRA. more...

Objectives: Anorexia nervosa is a life-threatening eating disorder, with significant risk for sudden death due to severe cardiac complications. The aim of this prospective study was to evaluate the cardiac abnormalities in female adolescents with anorexia nervosa and to examine the long-term results and reversibility of the detected cardiac abnormalities., Methods: We prospectively studied eleven female adolescents (13.5-17 years old) with anorexia nervosa diagnosed according to DSM IV criteria. On admission they were all on a weight-losing course with a mean body mass index of 13.71 +/- 1.54 (11.38-17.05) kg/m2. The mean follow-up duration was 2.45 +/- 1.17 (1-4.5) years. All patients reached normal weight after treatment. The control group was composed of 12 healthy, age-matched, adolescent girls of normal weight. The patients with anorexia nervosa and the control group underwent a complete clinical examination, electrocardiographic and echocardiographic evaluations. These evaluations were repeated one year after refeeding., Results: Patients with anorexia nervosa had a lower heart rate and blood pressure than the control group and they increased to normal levels as found in the control group after refeeding. QT and QTc were significantly longer and R wave amplitudes in V6 were significantly lower in the patients with anorexia nervosa than in the control group. QT and QT'c dispersions were significantly greater in anorexia nervosa patients compared to the control group. Left ventricular mass and left ventricular mass index were significantly lower in the anorexia nervosa group. One year after refeeding, there was a significant decrease in QT, QTc, QTd and QTcd. Although in anorexia nervosa patients, R wave amplitudes in V6 increased after refeeding; they did not reach the levels found in the control group. Control echocardiograms of anorexia nervosa patients after refeeding showed an increase in LV diameters and cardiac mass. There was a strong correlation between QT dispersion and left ventricular mass index., Conclusions: The adolescent girls with anorexia nervosa had significant structural and functional cardiac abnormalities in comparison to the control group. All these abnormalities were reversible except low R wave amplitude in V6. more...

A five-year-old girl patient was admitted with cyanosis and dyspnea, which started from birth. She had small telangiectatic lesions on her face and cerebral arteriovenous malformation, but no family history of hereditary hemorrhagic telangiectasia. Contrast echocardiography and pulmonary angiography demonstrated diffuse pulmonary arteriovenous fistulas. The patient was diagnosed as polysplenia syndrome characterized with left atrial isomerism, interrupted inferior vena cava, azygous continuation to the superior vena cava, and hepatic veins draining to the right atrium. In contrast to the other polysplenia syndrome cases, in this patient, pulmonary arteriovenous fistulas were not associated with cavopulmonary anastomoses or liver disease. more...

The aims of this study were to investigate the effect of exogenous obesity on respiratory function tests, to define the relationship between the severity of obesity and respiratory function test parameters, and to detect the incidence of airway hyperresponsiveness and exercise-induced bronchospasm in an obese study group. This cross-sectional controlled study was done with 38 exogenous obese patients, aged 9 to 15 years, and 30 healthy children. Basal respiratory function test parameters were measured with spirometry. To display airway hyperresponsiveness, 4.5% hypertonic saline provocation test was used; exercise-induced bronchospasm incidence was defined with bicycle ergometry. Basal respiratory function test parameters were lower in the study group as compared with the control group. Exercise test was positive in 31.6% of the obese group and in 3.3% of the control group (P = 0.003). The provocation test with hypertonic saline test was positive in 18.4% of the obese group. There were strong negative correlations between body mass index (BMI), relative weight, skin fold thickness, waist/hip circumference ratio and basal forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and peak expiratory flow (PEF) values. The diagnosis and management of exercise-induced bronchospasm may improve exercise performance and physical activity, assist with weight loss, and break the vicious circle. more...

A three-month-old girl patient born at the 37th week of gestation as one of twins was admitted to our hospital with cardiac arrest. There was past medical history of multiple hospitalizations with symptoms of cyanosis, respiratory distress, and frequent and severe attacks of apnea since her birth. On nasopharyngeal computerized tomography a mass (25x24 mm) occupying the right side of the nasopharynx was detected. The pathological evaluation of the excised mass revealed benign teratoma. After the removal of the nasopharyngeal mass under general anesthesia, respiratory distress and attacks of apnea disappeared completely. In every neonate with unexplained stridor and recurrent apnea, beside the common causes like respiratory distress syndrome, sepsis, and asphyxia, the possibility of oropharyngeal and nasopharyngeal mass should be kept in mind. more...

Purpose: To measure the aortic stiffness in tobacco-smoking adolescents and to investigate its relationship to tobacco smoke., Methods: Aortic strain (S), pressure strain elastic modulus (E(p)), and normalized E(p) (E(p)*) in tobacco-smoking adolescents and the healthy control group were measured by a sphygmomanometer with cuff and transthoracic echocardiography. The study group consisted of 30 healthy cases (M/F: 27/3) as a control group and 30 tobacco-smoking volunteer adolescents (M/F: 28/2). Unpaired Student's t-test was used for comparison of these groups., Results: The mean ages were 16.1 +/- 1.8 years and 16.2 +/- 1.4 years, respectively. The number of cigarettes smoked per day was 31 +/- 7.1 and the duration of smoking was 3.4 +/- 1.1 years. S, E(p) and E(p)* measurements of tobacco smokers were different than the control groups' and this difference was statistically significant. S values were significantly higher in nonsmokers than in smokers; whereas E(p) and E(p)* values were significantly higher in smoker group., Conclusions: This study demonstrated that measurement of aortic stiffness with S, E(p), and E(p)* can be used as an early indicator of atherosclerosis in tobacco-smoking adolescents. more...