Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis.

INTRODUCTION: This prospective study was planned to investigate the frequency and relationship of acquired von Willebrand syndrome (AVWS) with aortic and pulmonary stenosis in patients. METHODS: A total of 84 children, ranging from two to 18 years of age, were enrolled in this study. Of these, 28 had isolated aortic stenosis, 32 had isolated pulmonary stenosis and 24 were healthy. Children with aortic and pulmonary stenosis associated with other congenital heart diseases were excluded. Children with hypothyroidism, renal or liver disease, malignancy or autoimmune disease were also excluded. Wholeblood count, blood group, factor VIII level, prothrombin time (PT), activated partial thromboplastin time (aPTT), von Willebrand factor antigen (VWF:Ag), ristocetin co-factor (VWF:RCo), and bleeding time using a platelet-function analyser (PFA-100) were performed in all patients. All of the children in the study underwent a detailed physical examination and echocardiographic evaluation. RESULTS: A history of bleeding was positive in 18% of the aortic stenosis group, 9% of the pulmonary stenosis group, and 4% of the control group. Seven of 60 (12%) patients had laboratory findings that implied a diagnosis of AVWS, and two of these (28%) had a history of bleeding. The frequency of AVWS was 14% in patients with aortic stenosis and 9% in those with pulmonary stenosis. CONCLUSION: AVWS is not rare in stenotic obstructive cardiac diseases. A detailed history of bleeding should be taken from patients with valvular disease. Even if the history is negative, whole blood count, PT and aPTT should be performed. If necessary, PFA-100 closure time and further tests should be planned for the diagnosis of AVWS.

Does intrauterine tobacco exposure increase the pain perception of newborns?

BACKGROUND: This study aimed to assess whether there was a difference in the pain-perception levels of newborns born to mothers who smoked during pregnancy and newborns born to mothers who were not exposed to active or passive smoking during pregnancy. MATERIALS AND METHODS: A total of 60 newborns born by normal spontaneous vaginal birth between June 2013 and June 2014 were included in the study: 30 born to mothers who smoked during pregnancy, and 30 born to mothers not exposed to smoking. Mothers or newborns who had taken analgesics or sedative medications in the previous 24 hours, newborns not born at term, and sick newborns were not included in the study. During the routine hepatitis B-vaccination injection given at postnatal 48 hours, the newborns' behavior was monitored and recorded by video camera. The data obtained from the recordings were evaluated according to the Neonatal Pain, Agitation, and Sedation Scale and analyzed with SPSS 20. RESULTS: The median pain score of the group exposed to tobacco smoke in utero was 8.5, while the median pain score of the unexposed group was 6 (P<0.001). CONCLUSION: Exposure to tobacco smoke in utero may increase the pain-perception levels of newborns.

Relation between QT Variables and Left Ventricular Geometry in Athletes and Obese Children.

INTRODUCTION: We aimed to evaluate the relation between QT variables and left ventricular geometry in athletes and obese children. MATERIAL AND METHODS: This study included 209 children and adolescents. Thirty-eight of them were obese, 140 were athletes and 31 were normal weighted. Children with history of functional and structural cardiovascular diseases (acquired and congenital), chronic systemic diseases, hypertension, sleep apnea, endocrinological disorders were excluded. All subjects underwent detailed electrocardiographic and echocardiographic examination. RESULTS: QT dispersion was significantly higher in obese children, basketball players, football players and swimmers compared to control group (p < 0.05, p < 0.001, < 0.001 and < 0.01 respectively). Basketball players had the highest QT dispersion. Left ventricular endsystolic diameter (mm/m2) was higher in footballers, swimmers, wrestlers and tennis players compared to obese subjects. Interventricular septal thickness at diastole was highest in swimmers compared to control group (p < 0.001) and left ventricular posterior wall thickness was significantly higher in footballers and swimmers compared to healthy subjects (p < 0.01 and p < 0.001 respectively). Left ventricular geometry was normal in 84 patients (47.1%), but 34 patients (19.1%) had concentric remodelling, 20 (22.4%) had concentric hypertrophy and 40 (28.6%) had eccentric hypertrophy. No concentric and eccentric hypertrophy was observed in obese subjects. Left ventricle hypertrophy was prominent in dynamic and combined groups but it was not statistically significant (p = 0.204). QT dispersion was statistically higher in obese, dynamic sports and combined sports compared to control group (p < 0.05, p < 0.001 and p < 0.001 respectively). DISCUSSION: In this study we determined that QT dispersion is elevated in dynamic and combined sports types and obese children. CONCLUSION: QT dispersion is prolonged in obese and athletes who participate dynamic and combined sports.

Introdução: O objetivo deste trabalho foi avaliar a relação entre variáveis QT e geometria do ventrículo esquerdo em atletas e crianças obesas. Material e Métodos: Foram incluídos no estudo 209 crianças e adolescentes. Trinta e oito deles eram obesos, 140 foram atletas e 31 tinham peso normal. Crianças com antecedentes funcionais e estruturais de doenças cardiovasculares (adquiridas e congénitas), doenças sistémicas crónicas, hipertensão arterial, apneia do sono, doenças endocrinológicas foram excluídos. Todos os sujeitos rea-lizaram detalhados exames eletrocardiográficos e ecocardiográficos. Resultados: A dispersão do QT foi significativamente maior em crianças obesas, jogadores de basquete, jogadores de futebol e nadadores, por comparação com o grupo controlo (p < 0,05, p < 0,001, < 0,001 e < 0,01, respetivamente). Os jogadores de basquetebol tiveram a maior dispersão do QT. O di'metro ventricular esquerdo endosistólico (mm/m2) foi maior nos jogadores, nadadores, lutadores e jogadores de ténis por comparação com indivíduos obesos. A espessura do septo interventricular na diástole foi maior em nadadores por comparação com o grupo controlo (p < 0,001) e a espessura da parede posterior do ventrículo esquerdo foi significativamente maior nos jogadores e nadadores por comparação a indivíduos saudáveis (p < 0,01 e p < 0,001, respetivamente). A geometria do ventrículo esquerdo foi normal em 84 pacientes (47,1%), mas 34 pacientes (19,1%) tinham remodelação concêntrica, 20 (22,4%) apresentaram hipertrofia concêntrica e 40 (28,6%) apresentaram hipertrofia excêntrica. Não foi observada hipertrofia concêntrica e excêntrica em indivíduos obesos. A hipertrofia do ventrículo esquerdo era proeminente nos grupos din'mico e combinado mas a diferença não foi estatisticamente significativa (p = 0,204). A dispersão do QT foi significativamente maior nos obesos e praticantes de desporto din'mico e combinado em relação ao grupo controlo (p < 0,05, p < 0,001 e p < 0,001, respetivamente). Discussão: Neste estudo determinámos que a dispersão do QT é elevada em tipos de desporto din'mico e combinado, e em crianças obesas. Conclusão: A dispersão do QT é prolongada em pacientes obesos e atletas que participam em desporto din'mico e combinado.

Serum ischemia-modified albumin levels in adolescent smokers.

BACKGROUND: It is known that the biochemical marker linked to tissue ischemia, ischemia-modified albumin (IMA), is related to oxidative stress. Cigarette smoking is a situation with increased oxidative stress causing cell damage and it is thought that many of the negative effects linked to smoking may occur after the biological material in the body is exposed to oxidative damage. This study aimed to identify variability in serum IMA levels in adolescents who smoke. METHODS: This case-control study comprised 60 adolescents without any chronic disease. The smoking group was 30 adolescents between the ages of 14 and 17 years who smoked, while the control group was 30 healthy adolescents who did not smoke. Blood samples were collected from all subjects and serum IMA levels and serum nicotine metabolites were determined. RESULTS: The serum IMA levels in the adolescents who smoked were 0.452±0.094 absorbance unit (ABSU), while the control group had ASBU levels of 0.427±0.054. There was no significant difference between the groups in terms of serum IMA levels (p=0.210). There was a significant difference between the control and smoking groups in terms of serum nicotine metabolite levels (p<0.001). CONCLUSIONS: Among adolescents who smoke, serum IMA levels may not be a good marker for oxidative stress.

Relationship between Abdominal Aortic Intima Media Thickness and Central Obesity in Children.

BACKGROUND: Childhood obesity is one of the important risk factors for early atherosclerosis. We aimed to evaluate the effect of obesity on abdominal aortic intima media thickness (aaIMT) in children. METHODS: We consecutively recruited 60 obese and 28 healthy children from the outpatient clinic of pediatrics. In all patients, BMI, waist circumference (WC) and hip circumference (HC) were measured, and fasting serum lipid profile, plasma glucose and plasma insulin were studied. Homeostasis model assessment-estimated insulin resistance (HOMA-IR) was calculated for the obese group. All children underwent ultrasonography to evaluate hepatosteatosis and to measure aaIMT. RESULTS: There was a significant difference between the groups in terms of aaIMT, insulin, glucose, HOMA-IR, total cholesterol, low-density lipoprotein, triglyceride and hepatosteatosis. aaIMT was 1.12 ± 0.25 and 0.61 ± 0.18 mm in the obese and nonobese groups, respectively (p < 0.001). In the obese group, there was a positive correlation between aaIMT and WC, WC/height ratio, BMI, HC and hepatosteatosis (p < 0.05 for all), with the highest significance for WC. CONCLUSIONS: This study showed that aaIMT was significantly higher in obese children than in nonobese children, and that WC was a strong predictor for aaIMT. Early detection of an increased aaIMT in obese children may guide the follow-up of these patients in terms of developing atherosclerosis and its complications.

Effect of delivery mode on postpartum neonatal body temperatures.

OBJECTIVE: It is known that general and local anesthesia practices disrupt the delicate balance of thermoregulation center which is already sensitive to very tiny differences of temperatures in a normal subject. We aimed to evaluate and compare the rectal temperatures of newborns born with normal vaginal delivery and cesarean section. METHODS: We performed a prospective study of 106 term newborn - 40 born with normal vaginal delivery (group 1) and 66 born with cesarean section [51 spinal anesthesia (group 2), 15 general anesthesia (group 3)]. Only term babies were included in the study. Babies of eclamptic, pre-eclamptic and diabetic mothers and babies with chronic systemic diseases were excluded. Pregnants who underwent elective cesarean section were included in the study. Adolescent pregnants, pregnants with increased risks and pregnants with complicated operations were excluded. Mothers' temperatures were measured before and after the interventions. Rectal temperatures of the babies were measured immediately after birth. RESULTS: Environmental temperature was maintained at 22-24 °C. Pre-operative mother temperatures were 36.31 ± 0.30 °C in group 1, 36.36 ± 0.26 °C in group 2 and 36.39 ± 0.19 °C in group 3 (p = 0.414). Post-operative mother temperatures were 36.39 ± 0.27 °C in group 1, 36.29 ± 0.31 °C in group 2 and 36.25 ± 0.28 °C in group 3 (p = 0.215). Rectal temperatures of the babies born with normal vaginal delivery were significantly higher than the others. It was lowest in the general anesthesia group (37.5 ± 0.6 °C, 37.2 ± 0.2 °C and 36.8 ± 0.4 °C in group 1, 2 and 3, respectively). The temperature differences between groups were statistically significant p < 0.001). CONCLUSIONS: In conclusion, it is worthy to note that temperatures of the newborns can differ according to the delivery mode. Physicians and health professionals that take care of the newborns should be aware of this difference.

Prenatal maternal risk factors for infantile colic.

AIM: To examine maternal prenatal risk factors for infantile colic (IC). METHODS: Mothers were asked to complete a standard pre-coded questionnaire form. Inclusion in the study consisted of four criteria. The case group comprised healthy children with infantile colic according to Wessel's definition. The control group comprised healthy children with no history of IC. Prenatal period characteristics of mothers were examined to determine associations with IC. RESULTS: Children with mothers who had prophylactic iron supplementation therapy during pregnancy suffered more from IC. Postpartum depression was found to be more frequent in the case group than in the control group. Gynaecological history, including suffering from migraine, presence of any premenstrual symptoms, dysmenorrhea and high pre-pregnancy body mass index, was associated with development of IC. CONCLUSION: Pre-gestational gynaecological complaints and pregnancy characteristics may point to IC. These characteristics should be investigated and families should be informed.

Association between teething and independent walking in healthy children.

Developing teeth provide a reliable indication of maturation and biological age. The objective of this study was to establish whether there is any association between the time of emergence of the first primary tooth and the time when independent walking occurs. A cross-sectional study was conducted with a sample of 206 healthy children (95 girls and 111 boys) aged 12 to 60 (mean: 29.79±0.66) months who were able to walk independently. The study was conducted using a questionnaire that was filled out by the parents. The first primary tooth emerged at 6.86±0.14 (min: 3-max: 13) months; the mean independent walking time was 12.58±2.15 (min: 8.50-max: 24.00) months. There was no correlation between the first teething and independent walking times (r=0.045, p=0.523). Factors such as breastfeeding status, intake of vitamins, walker usage and body mass index were found not to affect the time of either emergence of the first deciduous tooth or independent walking. To the best of our knowledge, this is the first paper in literature to have researched the relationship between the time of emergence of the first deciduous tooth and that of independent walking. It should be explained to parents that there is no relationship between the two in order to resolve anxiety when their child acquires a tooth but does not walk, or vice versa.

Early detection of myocardial deformation by 2D speckle tracking echocardiography in normotensive obese children and adolescents.

OBJECTIVE: To evaluate the left venticular myocardial deformation parameters in normotensive obese children and adolescents by using 2-D speckle tracking echocardiography. METHODS: This observational cross-sectional study included 69 children and adolescents (aged between 10-18), 38 were normotensive obese and 31 were normal weighted. All children underwent detailed two- dimentional, Doppler and two-dimentional speckle tracking echocardiography. Student t-test, Mann-Whitney U test, chi-square test and Pearson's correlation were used in statistical analysis. Multiple linear regression analysis was used the determine independent variables on global longitudinal strain (GLS). RESULTS: While in normal limits, diastolic blood pressure was significantly higher in obese group. Left ventricular end-diastolic diameter (LVDd) and end-systolic diameter (LVDs), interventricular septal thickness (IVSd), left ventricular posterior wall thickness (LPWD) and left ventricular mass index (LVM)/height(2.7) were significantly higher in obese group compared to healthy peers (p=0.004, p=0.011, p<0.001, p=0.001, p<0.001) respectively. Obese subjects had reduced global longitudinal strain (GLS) values (p=0.001). Multiple linear regression analysis using the stepwise method were performed to assess the independent variables (age, body mass index, insulin resistance, systolic blood pressure, diastolic blood pressure, left ventricular diameters and LVM index (g/m(2.7)) affecting the dependent variable GLS. GLS was found significantly correlated with body mass index (BMI) (ß:0.440, p:0.001; 95% CI: 0.104-0.311). CONCLUSION: Left ventricular strain parameters obtained by two dimentional speckle tracking echocardiography were diminished in obese children compared to normal subjects indicating that obesity in childhood is linked to decreased myocardial deformation even in the absence of comorbidities in early stages.

Prolonged rooming-in in infancy is associated with generalized anxiety disorder in the adolescent period.

OBJECTIVE: This study aims to investigate whether anxiety disorders in adolescents have a link with the separation time of bed/bedroom sharing with parents. It also aims to raise awareness in society about the issue of minimizing anxiety disorders in childhood. METHODS: A case-control study was conducted in Sevket Yilmaz Training and Research Hospital between June 2013 and May 2014. The participants included 51 adolescents who were diagnosed as generalized anxiety disorder (GAD) with no-comorbidity and 71 healthy adolescents as the control group, who were chosen randomly. Diagnosis of GAD was based on the criteria from the Diagnostic and Statistical Manual of Mental Disorders (4th ed) by child and adolescent psychiatry doctors. The Turkish version of the State-Trait Anxiety Inventory (STAI) was used for the control group. A special survey about demographics and bed-sharing statements was constructed for the purpose of the study. The groups were analyzed in terms of duration of co-sleeping (bed-sharing) and rooming-in (keeping the mother and the baby in same room) with parents during infancy and the development of anxiety disorders in later period. RESULTS: Mean duration of rooming-in was significantly longer in the case group than in the control group (p=0.009). Similarly, mean duration of co-sleeping in the case group was longer than that of the control group. However, this difference was not statistically significant (p=0.529). CONCLUSION: Sleeping in the same room with children for a long time may result in anxiety disorders in later period due to possible difficulties in bonding and/or less self-confidence.

Prolonged pacifier usage in infancy does not cause eating behavior problem later.

The objective of this study conducted in children aged 3-7 years was to determine whether pacifier use during infancy is associated with eating problems in later periods. A total of 85 children (55.2 ±12.3 months) admitted to hospital with eating problems and with no organic pathology in scans, and 97 healthy children (52.24±10.97 months) without eating problems, were assigned to case and control groups, respectively. Eating problems were classified into five groups and investigated via a questionnaire. The presence of eating problems was analyzed for association with pacifier use. There was no significant difference between the two groups in terms of pacifier use (chi-square test: 0.141, p=0.707), and pacifier use is not related to a poor appetite in later periods. Pediatric healthcare providers and parents should be informed with regard to the subject.

Diagnosis, treatment and follow up of neonatal arrhythmias.

OBJECTIVE: This study aimed to evaluate the aetiology, spectrum, course and outcomes of neonates with arrhythmias observed in a tertiary neonatal intensive care unit from 2007 to 2012. METHODS: Neonates with rhythm problems were included. The results of electrocardiography (ECG), Holter ECG, echocardiography and biochemical analysis were evaluated. The long-term results of follow up were reviewed. RESULTS: Forty-five patients were male (68%) and 21 (32%) were female. Fifty-five patients (83.3%) were term, 11 (16.6%) were preterm, and 34% were diagnosed in the prenatal period. Twenty cases (30.3%) had congenital heart disease. Twenty-three patients (34.8%) were diagnosed during the foetal period. The most common arrhythmias were supraventricular ectopic beats and supraventricular tachycardia (SVT) at 39.3 and 22.7%, respectively. SVT recurred in five patients after the neonatal period. CONCLUSION: Supraventricular ectopic beats and SVT were the most common arrhythmias during the neonatal period. Although the prognosis of arrhythmias in the neonatal period is relatively good, regular monitoring is required.

Effects that different types of sports have on the hearts of children and adolescents and the value of two-dimensional strain-strain-rate echocardiography.

Whether the hypertrophy found in the hearts of athletes is physiologic or a risk factor for the progression of pathologic hypertrophy remains controversial. The diastolic and systolic functions of athletes with left ventricular (LV) hypertrophy usually are normal when measured by conventional methods. More precise assessment of global and regional myocardial function may be possible using a newly developed two-dimensional (2D) strain echocardiographic method. This study evaluated the effects that different types of sports have on the hearts of children and adolescents and compared the results of 2D strain and strain-rate echocardiographic techniques with conventional methods. Athletes from clubs for five different sports (basketball, swimming, football, wrestling, and tennis) who had practiced regularly at least 3 h per week during at least the previous 2 years were included in the study. The control group consisted of sedentary children and adolescents with no known cardiac or systemic diseases (n = 25). The athletes were grouped according to the type of exercise: dynamic (football, tennis), static (wrestling), or static and dynamic (basketball, swimming). Shortening fraction and ejection fraction values were within normal limits for the athletes in all the sports disciplines. Across all 140 athletes, LV geometry was normal in 58 athletes (41.4 %), whereas 22 athletes (15.7 %) had concentric remodeling, 20 (14.3 %) had concentric hypertrophy, and 40 (28.6 %) had eccentric hypertrophy. Global LV longitudinal strain values obtained from the average of apical four-, two-, and three-chamber global strain values were significantly lower for the basketball players than for all the other groups (p < 0.001).

Severe digital necrosis in a 4-year-old boy: primary Raynaud's or jellyfish sting.

Raynaud's phenomena is a common disorder which may be primary or secondary to some connective tissue disorders such as systemic sclerosis and systemic lupus erythematosus. Jellyfish sting is a rare but life-threatening cause of Raynaud's phenomena. Digital gangrene is reported in 3% of children with secondary Raynaud's phenomena but does not occur in children with primary Raynaud's phenomena. We report a case of a 4-year-old boy who initially presented with episodes of pain and bluish to blackish discolouration and necrosis affecting the fingers on both hands after a jellyfish sting without any sign of connective tissue disorder.

A huge intracardiac thrombus developed in the presence of antithrombin III deficiency in a patient with end-stage renal failure.

In this study, we report a 15-year-old female with end-stage renal disease undergoing hemodialysis, who admitted with acute respiratory failure and generalized edema. Abdominal tomography detected thrombi in the right renal vein, in the hepatic segment of the inferior vena cava and in iliac veins. Levels of proteins C and S, antinuclear antigen, anti-dsDNA, C3, and C4 were in normal limits. The thrombi persisted despite treatment with nadroparin, heparin with fresh frozen plasma and warfarin. Due to heparin resistance, antithrombin III levels were measured and were found abnormally low. The first echocardiographic examination was in normal limits but the second echocardiography revealed a huge thrombus occluding the tricuspid valve. Urgent thrombectomy was planned but the patient died in the intensive care unit due to severe pulmonary edema.

Congenital left ventricular diverticulum associated with congenital ileo-jejunal atresia.

Congenital left-ventricular diverticulum (CVD) is a rare cardiac malformation. Echocardiography, magnetic resonance imaging, multislice computed tomography, and left-ventricular angiography are diagnostic tools. In this case report, we present a 5-month-old infant with CVD associated with congenital ileal atresia. The diverticulum appears to be of the left-ventricular type.

Echocardiographic and three-dimensional computed tomographic diagnosis of crossed pulmonary arteries: report of three cases.

Crossed pulmonary arteries are rare anomalies of the pulmonary trunk and its branches. In this anomaly, the ostium of the left pulmonary artery originates superiorly and to the right of the right pulmonary artery. This anomaly is usually associated with other congenital cardiac and extracardiac diseases. We report three neonates with congenital cardiac diseases who had this anomaly, which was detected first by echocardiography and then confirmed by cardiac three-dimensional computed tomography.

Hepatic adenomatosis in a 7-year-old child treated earlier with a Fontan procedure.

Patients who undergo a Fontan procedure experience some degree of liver disease. Hemodynamic changes such as central venous hypertension, depressed dynamic cardiac output, and late ventricular dysfunction combined with long-standing hypoxia preceding the Fontan procedure all are recognized risk factors for hepatic injury. The histopathologic changes associated with cardiac hepatopathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis. However, hepatic adenoma and hepatic adenomatosis (HA) are not well-known pathologies during the course of cardiac hepatopathy. This study focused on a 7-year-old girl with chronic hepatic changes and HA who had undergone a Fontan procedure. Hepatic adenomatosis was diagnosed on the basis of magnetic resonance imaging (MRI) and MRI-guided liver biopsy. To the best of the authors' knowledge, this case involved the youngest patient with hepatocellular adenomatosis documented in the literature. It was a unique case because the patient experienced HA after a Fontan procedure.