Midterm Results of Implantable Cardioverter Defibrillators in Children and Young Adults from a Single Center in Turkey.

BACKGROUND: Despite concerns about complications with the implantable cardioverter defibrillator (ICD), it is effective for the prevention of sudden cardiac death (SCD). We aimed to analyze our midterm experience with ICD in children and young adults. METHODS: This retrospective study included patients who were implanted with an ICD between 2001 and 2014. Demographic characteristics, clinical information, shock features, and complications for all patients with ICD were analyzed. The study population was divided into two groups: early-era patients implanted before 2008, and late-era patients implanted after 2008. RESULTS: Sixty-nine patients (median age: 12 years, median follow-up: 52 months) were implanted with an ICD. Diagnostic categories were channelopathy (56.6%), cardiomyopathy (36.2%), congenital heart disease (5.8%), and other (1.4%). We performed implantation for primary prevention in 66.6% (39.3% in early-era patients and 85.4% in late-era patients). Thirty-one (44.9%) received 139 appropriate shocks (66% of total shocks) while 14 (20.2%) received 71 inappropriate shocks. However, there was no statistically significant difference in the use of appropriate shocks in the primary (66.7%) versus the secondary (72.2%) prevention groups. The incidence of appropriate and inappropriate shock was 66.7% and 33.3% in the primary prevention group, and 72.2% and 27.8% in the secondary prevention group, respectively. Two patients died, although only one death was the result of a lead problem. CONCLUSIONS: Although lead integrity problems, inappropriate shocks, and infections are significant issues, ICD therapy appears to be a safe, effective, and necessary option for the prevention of SCD in both children and young adults.

Adrenomedullin predicts high risk and culture positivity in children with solid tumors suffering from neutropenic fever.

AIM OF STUDY: Neutropenic fever is a source of morbidity and mortality in children with cancer. It is not possible to detect the causative agent in cultures in most cases; the research for a marker that can show the severity of the disease is ongoing. We evaluated the role of adrenomedullin (ADM) at predicting prognosis on patients with febrile neutropenia, which has been proven to be a good prognostic marker for diseases with high morbidity and mortality, such as heart failure, ischemic ventricular dysfunction, sepsis, and systemic inflammatory response syndrome. MATERIALS AND METHODS: We recorded the 36 febrile episodes of 14 children receiving chemotherapy due to solid tumors. There were 10 events with unknown origin in the low-risk group, while in the high-risk group, there were 17 events with unknown origin, 8 events with microbiological origin and 1 event with clinically proven infection. Cultures were positive only in the high-risk group. However, the changes of ADM levels through time periods (first, second, third, and seventh days) were not significant. RESULTS: The first-day plasma ADM levels significantly predicted the presence of culture positivity (AUC 0.628, 95% CI 0.40-0.85, p = 0.303) and high-risk patients with neutropenic fever (AUC 0.76, 95% CI 0.56-0.97, p = 0.016). CONCLUSION: Our study showed that increased plasma ADM was correlated with high-risk neutropenic fever and culture positivity. The ADM levels in the high-risk group were clearly high at the diagnosis and continued to the end of the treatment.

Comparison of Transesophageal and Intracardiac Electrophysiologic Studies for the Diagnosis of Childhood Supraventricular Tachycardias.

In this study, we aimed to compare the results of transesophageal electrophysiologic studies (TEEPS) and intracardiac electrophysiologic studies (IEPS) in a cohort of pediatric patients with SVTs. The medical records of children aged between 0 and 18 years who underwent TEEPS between January 2007 and June 2012 were systematically reviewed, and those without pre-excitation and who underwent subsequent IEPS were identified. Post-procedural diagnoses were compared for compatibility. A total of 162 patients were included in the study with a mean age at diagnosis 11.6 ± 3.6 years. Tachycardia was induced in 152 patients by TEEPS and in 154 patients by IEPS. Overall, in 147 patients, tachycardia was induced by both TEEPS and IEPS. Diagnoses were compatible in 135 out of 147 patients (91.8 %). Nine out of the 12 patients with discrepant results were diagnosed with atrioventricular-reentrant tachycardia (AVRT) and three with atrioventricular nodal reentrant tachycardia (AVNRT) after TEEPS. Following IEPS, TEEPS diagnosis of AVRT was revised to typical AVNRT in 5 patients and atypical AVNRT in 4 patients. Two of the 3 patients who were diagnosed as having AVNRT following TEEPS were confirmed to have atrial tachycardia after IEPS, while the other patient was diagnosed with AVRT. Tachycardia terminated spontaneously in 3 patients, while overdrive pacing was attempted to terminate the tachycardia in 149 patients, with a success rate of 93.2 % (139/149). The diagnostic compatibility between TEEPS and IEPS is quite high. A diagnostic discrepancy mostly occurs in patients diagnosed with AVRT by TEEPS, and the possibility of atypical AVNRT should be considered in patients with a VA ≥70 ms to avoid such discrepancies.

Transjugular approach for radiofrequency ablation of permanent junctional reciprocal tachycardia in a newborn with bilateral femoral vein thrombosis.

Although radiofrequency ablation is the first line therapy in some children with supraventricular tachycardia, its application in small children is still limited. Herein, we presented a premature newborn diagnosed as multidrug-resistant permanent junctional reciprocal tachycardia, and treated by radiofrequency ablation via the jugular vein approach because of bilateral femoral vein thrombosis. We think that when there is limited vascular access, the transjugular route for radiofrequency ablation might be considered as an alternative treatment in newborns with multidrug-resistant supraventricular tachycardia.

Sensitivity of transesophageal electrophysiologic study in children with supraventricular tachycardia on electrocardiography.

INTRODUCTION: The aim of this study was to evaluate the inducibility of tachycardia by transesophageal electrophysiologic study (TEEPS) in patients with documented supraventricular tachycardia (SVT) on electrocardiography and to investigate the accuracy of TEEPS records by comparing with intracardiac electrophysiologic study (IEPS). MATERIAL AND METHODS: The TEEPS records of patients having documented electrocardiography during SVT were reviewed. The results of TEEPS in 43 of 85 patients were compared with results of IEPS for compatibility of diagnosis. RESULTS: A total 85 patients, 46 male and 39 female, mean weight 35.1 kg (36-87), aged 1 month-17 years, were included. Tachycardia was induced by TEEPS in 79 of 85 patients with documented electrocardiography (sensitivity 92.9%). IEPS for diagnosis or ablation was conducted in 40 patients having inducible tachycardia and three of six who had no inducible tachycardia by TEEPS. Tachycardia was induced by IEPS in 39 of 40 (97.5%) patients who had inducible tachycardia and two of three who had no inducible tachycardia by TEEPS. Mechanisms of tachycardias were similar in 97.5% of patients (37/39) who had inducible tachycardia in TEEPS and IEPS. One of the patients with atrioventricular reentry tachycardia by TEEPS was diagnosed as atrioventricular nodal reentry tachycardia (AVNRT) and the other one was diagnosed as atypical AVNRT and atrial tachycardia by IEPS. CONCLUSION: The rates of inducibility and mechanisms of tachycardias by TEEPS in children having documented SVT were similar with those obtained from IEPS.

Prevalence and risk factors of tinea capitis and tinea pedis in school children in Turkey.

OBJECTIVE: To evaluate the prevalence and risk factors of tinea capitis and tinea pedis in school children in Turkey. METHODS: The study included 8122 students from 24 schools in the rural and urban areas around Kayseri,Turkey. We asked every student for their personal identification and also for their sanitation in order to get an idea about dermatophytosis. Samples taken from suspicious lesions were collected and inoculated onto Sabouraud dextrose agar slants. For identification of grown fungi, macroscopic appearance of colonies, microscopic examination and biochemical tests were used. RESULTS: There were 41 (0.5%) suspicious lesions in feet and 31 (0.3%) in scalp and 22 (0.2%) students were diagnosed as tinea pedis and 9 (0.1%) as tinea capitis by fungal culture. The predominant etiologic agents in feet were Trichophyton rubrum 8 (36%), Trichophyton mentagrophytes 1 (4%), Rhodotorula 8 (36%), Trichosporon 2 (9%), Candida glabrata 2 (9%), Candida albicans 1 (4%), while Trichophyton verrucosum 8 (88%) and Trichophyton mentagrophytes 1 (12%) were identified in scalp samples. School settlement was found as risk factors on the frequency of tinea pedis and capitis. Age and gender were also found as risk factors on the frequency of tinea pedis. CONCLUSION: The results of this study demonstrate a low prevalence of tinea capitis and tinea pedis in school children of central Anatolia of Turkey. School settlement is a very important factor affecting the prevalence of tinea capitis and pedis in school children in central Anatolia of Turkey.

Pediculosis capitis: prevalence and its associated factors in primary school children living in rural and urban areas in Kayseri, Turkey.

OBJECTIVE: The aim of this study was to investigate the prevalence and risk factors of pediculosis capitis in schoolchildren living in rural and urban areas in Kayseri, a city located in central Anatolia in Turkey. METHODS: This cross-sectional school-based study was performed in 24 randomly selected public schools. A total of 8,122 schoolchildren aged 5-16 years, from kindergarten to eighth grade, were examined for the presence of pediculosis capitis. A child was defined as being infested by the presence of live or dead lice or eggs/nits. The results were analyzed using the chi-squared test and logistic regression analysis. RESULTS: The overall prevalence of head lice infestation was 13.1%. Pediculosis was more frequent in girls (25.2%) than in boys (0.86%) (p < 0.001). The prevalence was lower in children aged 5-8 years than in those aged 9-11 or 12-16 years (p < 0.001). In multiple regression analyses, the variables demonstrating statistically significant association with pediculosis were: being a girl (OR = 40.93; 95% Cl = 29.06-57.66), being 9-11 years old (OR = 1.54; 95% Cl = 1.25-1.89), residing with > or = 3 siblings (OR = 1.98; 95% Cl = 1.57-2.50), having a mother with no education (OR = 1.73; 95% CI = 1.29-2.33), having a father with no education (OR = 1.45; 95% Cl = 1.08-1.94), living in a rural area (OR = 2.34; 95% Cl = 2.02-2.71) and living in a one-room house (OR = 2.39; 95% CI = 1.41-4.08). CONCLUSIONS: Pediculosis capitis remains a health problem in schoolchildren in Kayseri, Turkey. In addition to improvement in socioeconomic status, collaborative and participation efforts among physicians, nurses, teachers, and parents are necessary to maintain effective epidemiological surveillance and provide treatment.

Cardiac autonomic regulation is disturbed in children with euthyroid Hashimoto thyroiditis.

Hashimoto thyroiditis (chronic autoimmune thyroiditis) is the most common form of thyroiditis in childhood. Previous studies have found autonomic dysfunction of varying magnitude in patients with autoimmune diseases, which is considered a cardiovascular risk factor. We aimed to evaluate the heart rate variability (HRV), a measure of cardiac autonomic modulation, in children with euthyroid Hashimoto thyroiditis (eHT). The study included 32 patients with eHT (27 girls and 5 boys; mean age 11 ± 4.1 years, range 8-16; body mass index 0.47 ± 0.69 kg/m(2)), as judged by normal or minimally elevated serum TSH levels (normal range: 0.34-5.6 mIU/l) and normal levels of free thyroid hormones (FT4 and FT3) and 38 euthyroid age-matched controls. Patients with eHT and control subjects underwent physical examination and 24-hour ambulatory ECG monitoring. Time-domain parameters of HRV were evaluated for cardiac autonomic functions. Children with eHT displayed significantly lower values of time-domain parameters of SDANN (standard deviation of the averages of NN intervals), RMSSD (square root of the mean of the sum of the squares of differences between adjacent NN intervals), NN50 counts (number of pairs of adjacent NN intervals differing by more than 50 ms) and PNN50 (NN50 count divided by the total number of all NN intervals) for each 5-min interval, compared to healthy controls (p < 0.05 for each), indicating the decreased beat-to-beat variation of heart rate. In conclusion, eHT is associated with disturbed autonomic regulation of heart rate. Hence, the children with eHT are at higher risk for developing cardiovascular diseases.

High dose calcitriol in osteopetrorickets.

Osteopetrorickets is a rare autosomal recessive disorder of osteoclast function characterized by abnormally dense bone and failure of resorption of calcified cartilage. Rickets is a paradoxical complication of osteopetrosis, resulting from the inability of the osteoclasts to maintain a normal calcium-phosphorus balance in the extracellular fluid. We report a patient with an unusual case of infantile osteopetro-rickets who was admitted with anterior fontanel bulging and was treated with haploidentical bone marrow transplantation.

Oral and dental findings in osteopetrorickets.

While dental findings of both rickets and osteopetrosis have been reported, there is no published report on the oral and dental findings of osteopetrorickets. In this paper dental findings of osteopetrorickets were presented. A two-year-old female child was referred to the pedodontics clinic for dental examinations before bone marrow transplantation. Her teeth showed severe mobility and the eruption of the teeth were delayed. The dental findings of the patient were different from that of osteopetrosis and rickets.

Assessment of autonomic functions in children with familial Mediterranean fever by using heart rate variability measurements.

AIM: The aim of this study is to analyze possible autonomic nerve system alterations and assess the efficacy of heart rate variability (HRV) analysis in anticipation of cardiovascular risks in pediatric patients with familial Mediterranean fever (FMF). METHOD: In this study, cardiac autonomic functions were investigated in children with FMF by analyzing HRV and its other probable cardiac effects by echocardiography. We studied 70 pediatric patients with FMF and 50 healthy controls. RESULTS: The time-domain parameters of HRV were compared between the FMF and control groups. SDNN (standard deviation of all NN intervals) was significantly decreased in patients with FMF as compared to control subjects. The other time-domain parameters of HRV and the frequency-domain parameters of HRV were similar in both groups. Frequency-dependent HRV parameters were similar in both groups, as were conventional echocardiographic parameters. CONCLUSION: HRV is a convenient and reliable technique for evaluation of autonomic functions. There are only a few studies on the assessment of autonomic functions by means of HRV in adult FMF patients but not in pediatric patients. Further studies are required to assess whether there is autonomic dysfunction in children with FMF.

Percutaneous closure of a coronary fistula with single Amplatzer Vascular Plug II in a five-month-old female: The youngest case report.

Coronary artery fistula (CAF) is a rare clinical abnormality characterized by a connection between one or more coronary arteries and an adjacent cardiac chamber or vascular structure. Although CAF complications are more common in older children over time, there is still no consensus in terms of treatment indications in children with asymptomatic fistula. We describe an asymptomatic infant diagnosed with a severely dilated right coronary artery and a coronary-cameral fistula treated successfully by transcatheter device closure. The Amplatzer Vascular Plug II appears to be safe, effective and relatively easy to use for CAF closure in infants.

Stent Placement in a Neonate with Sano Modification of the Norwood using Semi-Elective Extracorporeal Membrane Oxygenation.

Extracorporeal membrane oxygenation (ECMO) is a well-established tool of cardiopulmonary circulatory support for cardiopulmonary failure in children and adults. It has been used as a supportive strategy during interventional procedures in neonates with congenital heart disease. Herein, we describe a neonate with hypoplastic left heart syndrome who underwent stenting of the Sano shunt and left pulmonary artery after Norwood Sano operation using intra-procedural ECMO support. The use of ECMO as a bridge to recovery might be a feasible and reasonably safe adjunctive approach in the treatment of complications in selective case of neonates having undergone the Norwood Sano procedure.