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The aim of this study was to evaluate the feasibility, efficacy and safety of transcatheter closure of patent ductus arteriosus (PDA) with the Occlutech duct occluder (ODO) in children. We reviewed the clinical records of 71 patients who underwent percutaneous closure of PDA with an ODO between September 2014 and August 2016. The Occlutech duct occluder was applied to 71 patients during the study period (September 2014-August 2016), and the results were analyzed in this study. Forty-two of the patients were female and 29 male. The median age was 20.5 months (range, 6-194 months) and median weight was 16 kg (range, 6-68 kg). The PDA was classified as type A in 54 patients (76.1%), type E in 14 (19.7%), type C in 2 (2.8%) and type B in 1 (1.4%) based on the Krichenko classification. A standard ODO device was used for the transcatheter closure procedure in 66 patients and the long-shank ODO device in 5. In the echocardiographic measurement of PDA, the median smallest diameter was 2.7 mm (range, 1.5-7.0 mm), and in the angiographic measurement, the median smallest diameter was 2.5 mm (range, 1.5-6.5 mm). All 71 patients underwent successful PDA closure with the ODO. Angiography following the procedure showed complete closure in 47 patients (66.2%), mild residual shunt in 13 patients (18.3%) and a trivial shunt in 11 patients (15.5%). Color flow Doppler echocardiogpaphy at 24 h post-implantation showed that complete closure was achieved in 65 patients (91.5%), and 6 patients (8.5%) had mild residual shunt. All patients (100%) had complete closure at 30 days of follow-up. The results of this study showed that the Occlutech PDA occluder device is safe and effective in the closure of PDA. As the pulmonary artery side of the device is wider than the aortic side, protrusion toward the aortic side and embolization are prevented, but there is residual shunt in the early period, although this residual shunt disappeared after a few months.

Sabaz MN, Akin A, Bilici M, Demir F, Ture M, Balik H. Factors affecting mortality in children with dilated cardiomyopathy. Turk J Pediatr 2019; 61: 485-492. Dilated cardiomyopathy (DCMP) is a heart disease with high mortality rates that is often seen in children. Genetic and infectious reasons are primary in the etiology. The aim of this study was to investigate the etiology of DCMP and the parameters predicting mortality. A retrospective examination was made of 37 patients diagnosed with DCMP between January 2012 and October 2016. Data were recorded from the patient files of age, gender, complaints on presentation, findings of the physical examination, laboratory test results, echocardiography and electrocardiography findings at the time of diagnosis. These parameters were then compared between the surviving and nonsurviving patients. The patients comprised 21 males with a mean age of 27.50±50 months. Diagnosis was made at the age of < 12 months in 67.6% patients. Within mean 8 months of diagnosis, 16.2% of the patients were lost to mortality and 83.8% of the patients survived. In 83.3% of the non-surviving patients and in 29% of the surviving patients, sinus tachycardia was present at the time of diagnosis (p=0.023). Corrected QT (QTc) at the time of diagnosis was longer in the non-surviving patients (p=0.007). On ECG, the rate of ST-T wave change was higher in the non-surviving patients (80% vs. 17.8%, p=0.012). In conclusion, a significant proportion of the patients were diagnosed below the age of one year. In the non-surviving patients, as sinus tachycardia and ischaemic changes on ECG were seen more often and the QTc was longer, these findings could be considered to be predictors of mortality.

Bilici M, Fidanci-Dedeoglu Z, Demir F, Akin A, Ture M, Balik H, Tan I, Ertugrul S. Prolonged QT dispersion is associated with pediatric syncope. Turk J Pediatr 2019; 61: 85-91. We aimed to find out whether QT dispersion can be used as a diagnostic marker in terms of syncope type, and recurrence risk. Fifty-two patients (28 male, 53.8%) admitted to the pediatric cardiology clinic with syncope were compared with 50 healthy controls (28 male, 56%; mean age: 13.8±2.3 years, range: 11-17 years) for QT dispersion (QTd) and other electrocardiographic findings. Gender, age, echocardiography, blood pressure while standing and sitting, electrolyte levels, liver and kidney function tests, and QTd and QTcd (calculated with corrected QT: QTc) in electrocardiography (ECG) of patients were all evaluated. Patients` ages varied between 7-17 years and the mean age at first syncope was 13.9±2.4 years. Mean follow-up duration of the patients was 10±5 months (range: 5-18 months). Mean number of syncope attacks was 2.8±2.2. QTd (72±46 ms vs. 34±14 ms) and QTcd (77±45 ms vs. 33±14 ms) values of the patients were significantly longer, compared to control group (p < 0.001). The age, gender, QTd, and QTcd values did not differ between the subtypes of syncope based on pathogenetic mechanism. Additionally, these parameters did not differ in terms of the number of syncope recurrence and tilt test. Patients` complaints reduced after cardiologic evaluation and advice regarding prevention of syncope. We think that in follow-up period, education and preventive precautions that can be taken in the daily life must be emphasized, and drug treatment can be started in unresponsive cases.

Aim Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO. Methods We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA. Results The median patient age was 2.5 years (6 months–35 years), and median PDA diameter was 2.5 mm (1.2–11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24–48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months. Conclusion Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325–331)

Kawasaki disease (KD) is a systemic febrile condition leading to the inflammation of vessels including coronary arteries (1). Coronary artery aneurysms, occuring in approximately 15–25% of the patients, are the most important and life-threatening complication of KD (2). KD mainly affects children younger than five years old. Although it has been known for years, the etiology of the disease remains largely unexplained (3,4). Due to the lack of specific tools, the diagnosis of KD is still based on clinical features. At least four of the following five clinical criteria together with fever lasting equal to or longer than five days should be present for the diagnosis: bilateral non-purulent conjunctivitis; extremity findings including erythema and/or indurative edema on the hands and feet, and membranous desquamation starting in the periungual regions; changes in the lips and oral cavity; polymorphous exanthema; and cervical lymphadenopathy (usually unilateral with a diameter of 1.5 cm or more). However, those patients with prolonged fever (≥5 days) together with two or three clinical criteria may be diagnosed as incomplete KD in the presence of some supplemental laboratory findings (5). It has been reported increasingly in recent years, especially among young infants and children. Because incomplete KD also results in coronary artery lesions (CALs), it should be diagnosed promptly (5,6). However, the diagnosis is still difficult and further laboratory studies are needed. Neutrophil to lymphocyte ratio (NLR) is a novel parameter, studied in the conditions with inflammatory pathogenesis including ischemic heart disease, stroke, and infections (7–9). It was reported to be a strong predictor of adverse cardiovascular outcome and severity of coronary artery ectasia in adult population (10–12). In this study, the usefulness of NLR in the diagnosis of KD or incomplete KD and the correlation between NLR and the presence of coronary artery lesions were evaluated. The usefulness of NLR has not been studied in patients with KD so far.

Although influenza is an acute and uncomplicated disease, that limits itself in the healthy children, it may lead to death by rarely forming the sickness. The most common complication of influenza is pneumonia and it is a rare complication which is developed together with myocarditis by influenza A and B viruses. A 32 months-old male patient was admitted for rapidly developed respiratory distress and tachycardia after fever, cough, vomiting, malaise and runny nose. His general status was medium, he had conscious and had hepatomegaly, tachycardia, dyspnea, tachypnea, intercostal-subcostal retractions and bilateral rhonchus. Cardiac enzyme levels and other laboratory parameters were found normal. Myocarditis and ejection fraction was determined as 42% in echocardiography. However, hospitalization hours between 24 and 48, the patient, whose significant respiratory compromise developed, was intubated and fastened to a mechanical ventilator. H1N1 is produce in nasopharyngeal swab culture at the sixth day of follow-up. Because we think H1N1 virus was responsible from current myocarditis, oseltamivir treatment was initiated. In the fourth day of the treatment the patient’s fever returned to normal, in the ninth day a dramatic recovery was observed. In tracking echocardiography, a significant improvement was observed in the ejection fraction and myocarditis picture compared with admission time. This case was presented in order to remind that in a patients, who present with influenza findings but have respiratory distress and tachycardia in addition to lower respiratory tract infection, myocarditis should also be considered in the differential diagnosis and to remind that promising results could be obtained with the early diagnosis and treatment.

Pediatric dysrhythmias are rare but important causes of admission to pediatric clinics and emergency departments. Due to the development of successful surgical treatment of congenital heart diseases and improvements in the diagnostic tools, pediatric dysrhythmias are more frequently diagnosed. Although pediatric dysrhythmias are may be asymptomatic, they may manifest with weakness, dizziness, decrease in the effort capacity, easy fatigability, irregularity in heartbeats, palpitations, syncope and cardiac arrest. Since dysrhythmias may give rise to significant hemodynamic outcomes, their recognition by pediatricians and family physicians is vital for the patients. This review aims to contribute to the correct diagnosis and management of the cases with frequently encountered pediatric dysrhythmias.

Karadeniz, Cem/0000-0003-0529-2391; Guvenc, Tolga/0000-0003-1468-3415 WOS: 000340836500006 PubMed: 25098616 Cardiac contusion is a potentially fatal complication of blunt chest trauma. The effects of a combination of quercetin and methylprednisolone against trauma-induced cardiac contusion were studied. Thirty-five female Sprague-Dawley rats were divided into five groups (n = 7) as follows: sham, cardiac contusion with no therapy, treated with methylprednisolone (30 mg/kg on the first day, and 3 mg/kg on the following days), treated with quercetin (50 mg.kg(-1).day(-1)), and treated with a combination of methylprednisolone and quercetin. Serum troponin I (Tn-I) and tumor necrosis factor-alpha (TNF-alpha) levels and cardiac histopathological findings were evaluated. Tn-I and TNF-alpha levels were elevated after contusion (P = 0.001 and P = 0.001). Seven days later, Tn-I and TNF-alpha levels decreased in the rats treated with methylprednisolone, quercetin, and the combination of methylprednisolone and quercetin compared to the rats without therapy, but a statistical significance was found only with the combination therapy (P = 0.001 and P = 0.011, respectively). Histopathological degeneration and necrosis scores were statistically lower in the methylprednisolone and quercetin combination group compared to the group treated only with methylprednisolone (P = 0.017 and P = 0.007, respectively). However, only degeneration scores were lower in the combination therapy group compared to the group treated only with quercetin (P = 0.017). Inducible nitric oxide synthase positivity scores were decreased in all treatment groups compared to the untreated groups (P = 0.097, P = 0.026, and P = 0.004, respectively). We conclude that a combination of quercetin and methylprednisolone can be used for the specific treatment of cardiac contusion.

Background There is increasing evidence of clustering of certain cardiac anomalies in some families. The frequency and echocardiographic features of such anomalies among the relatives of patients with bicuspid aortic valve (BAV) or other left-sided cardiovascular anomalies (LSCA) were evaluated. Methods The patients with BAV or any other LSCA and their relatives were enrolled in the study. They underwent an echocardiographic examination. The probands were assessed in three groups: BAV, BAV + coarctation of aorta (CoA), and other LSCA. Their relatives were also grouped and evaluated accordingly. The echocardiographic measurements were standardized by Z-scores. Results Eighty-six probands and 261 relatives were evaluated. The numbers of the patients in the BAV, BAV + CoA, and other LSCA group were 52, 14, and 20, respectively. Any LSCA was determined in 17 (6.5%) of the relatives. Thirteen (5%) had aortic dilatation and the remainder (1.5%) had BAV. Accordingly, BAV incidence among relatives of patients with BAV was found to be 1.9%. A second individual with an LSCA was observed in 12.8% of 86 families investigated. The frequencies of aortic stenosis, aortic regurgitation, aortic stenosis + aortic regurgitation, and aortic dilatation in the patients with BAV were found to be 37.9%, 53%, 25.8% and 48.5%, respectively. In contrast to previous reports, no enlargement was observed in the pulmonary arteries of BAV patients. Conclusions BAV and other LSCA are of clinical significance. Because the clustering of LSCA in some families is observed, we recommend echocardiographic screening of those relatives. If this is not possible, at least it should be achieved for BAV patients.

We present a rare case of spontaneous closure of a fistula between the left coronary artery and the right ventricle (RV) within a few days of newborn period. A 14-day-old male newborn was referred to our clinic for investigation of tachypnea and cardiac murmur. A color flow Doppler echocardiography revealed turbulent flow of a large coronary artery fistula (CAF) between the left coronary artery and the RV. Tachypnea was regressed and repeat echocardiogram showed no CAF related to cardiac chambers after 4 days of admission. The spontaneous closure of CAF was found to be more likely in cases younger than 2 years with small-sized fistulas opening into the right-sided structures, especially into the RV. Nevertheless, the spontaneous closure is very rare in cases with significant shunt. To the best of our knowledge, this is the first case with documented spontaneous closure of CAF just within the newborn period.

Objective: To investigate the effects of carbamazepinetherapy on thyroid function tests in childrenMethods: The carbamazepine group consisted of 58 childrenunder observation for epilepsy, and the control groupof 54 healthy children. Age of onset, length of drug use,drug dosage and laboratory parameters including free triiodothyronine(FT3), free thyroxin (FT4) and thyrotropin(TSH) were recorded. These data were then comparedagainst those from the control group.Results: In the carbamazepine group, FT3 was 3.86 ±0.43 pg/mL, FT4 was 1.15 ± 0.18 mg/dL and TSH: 2.58 ±1.33 ml U/L. In the control group, FT3 was 4.13 ± 0.59 pg/mL, FT4 1.34 ± 0.13 mg/dL and TSH was 2.06 ± 0.89 mlU/L. No statistically significant difference between rates ofsubclinical hypothyroidism was determined between thetwo groups (p=0.196).Conclusion: Although, carbamazepine reduces thyroidhormone concentrations, rarely causes hypothyroidism.Key words: Child, carbamazepine, thyroid function tests

Background: The clinical spectrum of Brucella infection is quite diverse and characterized by multi-system involvement. Patients present with myocarditis, endocarditis, or pericarditis. Infective endocarditis is the most common cardiovascular complication in patients with brucellosis. Although conduction abnormalities are seen in cases with endocarditis, they are reported very rarely in the setting of cardiac Brucella infection. Case Report: An eight and a half-year-old male patient was referred to our clinic due to inadequate response to cotrimaxazole plus streptomycin treatment at the 15th day of admission. Although local hospital records on the patient showed a heart rate of 80 bpm, we determined a heart rate of 46 bpm. The electrocardiogram showed complete atrioventricular (AV) block. The average heart rate was determined as 48 bpm with 24-hour Holter electrocardiogram (ECG) monitoring. The echocardiographic examination showed normal-sized heart chambers and the absence of valvular involvement. An agglutination test for brucellosis was found to be positive with a titer of 1/320. High fever, arthralgia, and splenomegaly regressed following doxycycline plus rifampicin therapy, but there was no improvement in the AV block. A permanent pacemaker was implanted because of the detection of an average heart rate of 48 bpm. Conclusion: Because cardiac failure and rhythm abnormalities are reported in the course of Brucella infection and may be associated with significant outcomes, cases with brucellosis should be evaluated carefully in terms of cardiac involvement. This report aims to draw attention to complete AV block as an extremely rare complication of Brucella infection.

Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO.We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA.The median patient age was 2.5 years (6 months-35 years), and median PDA diameter was 2.5 mm (1.2-11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24-48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months.Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325-331).

Ture M, Bilici M, Akin A, Demir F, Balik H, Darakci SM. Complete atrioventricular block associated with clozapine intoxication: case report. Turk J Pediatr 2019; 61: 618-621. Clozapine is one of the atypical anti-psychotic drugs used in the treatment of resistant schizophrenia. Although cardiac side-effects are rare, it has been reported that there may be development of myocarditis, dilated cardiomyopathy, postural orthostatic hypotension and prolonged QT duration. Complete atrioventricular (AV) block is characterized by the inability to transmit all of the atrial signal to the ventricles. Causes may be congenital, idiopathic or acquired which are associated with surgery, infection, or muscle disease. AV block is extremely serious and permanent pacemaker insertion is usually necessary for all patients. Complete AV block may develop due to clozapine intoxication through increase in vagal tonus, sinoatrial node (SN) and the inhibition of atrioventricular node signalling. The case presented here is of a 15-year old female patient who developed AV total cardiac block associated with the taking of clozapine in a suicide attempt.

Kardiyak fibrom, rabdomiyomdan sonra en sık görülen benign kardiyak tümör olmasına rağmen insidansı oldukça düşüktür. Çıkış yolu obstrüksiyonuna, aritmiye veya ciddi kapak disfonksiyonuna yol açtıklarında cerrahi müdahale gerektirirler. Bu yazıda oldukça büyük olmasına rağmen asemptomatik seyir gösteren fibromlu bir süt çocuğu sunuldu. Although fibroma is the second most common benign cardiac tumor following rabdomyoma, its incidence is quite low. Fibromas causing ventricular outflow tract obstruction, arrhythmia, and/or severe valvular dysfunction necessitate surgical resection. An infant with asymptomatic clinical course, despite having a giant right ventricular fibroma was reported herein.

Electric shock is a condition that may affect various organ systems and potentially cause death. Cardiac findings vary from asymptomatic mild injury to fatal myocardial involvement. Herein we present a five-year-old boy with a cardiac thrombus developing after an accidental electrical shock. Cardiac arrhythmias and evidence of ischemia have been reported after electric shock; we were, however, unable to identify an earlier case report of intracardiac thrombosis related to electric shock. Findings such as elevated cardiac enzymes and systolic dysfunction, which indicate myocardial damage following electric shock, were present in our patient. We think that the cardiac thrombus might have resulted from the myocardial damage and the slowed intracardiac blood flow related to systolic dysfunction. As the thrombus was thought to have been formed through known mechanisms, it was treated traditionally. However, further data regarding the etiology and management of such thrombi is needed.

Lightning strikes may cause injury to the heart, ranging from slight electrocardiographic changes to fatal damage. As heart injury is the most important cause of mortality in these patients, cardiac monitoring is crucial. Even though various ECG changes have been reported, published data on pathologic ST-segment changes is scarce. Herein, we present a seven-year old patient with ST-segment elevation following a lightning strike. There is not sufficient data regarding lightning-related myocardial ischemia. However, because of the similar effects of lightning strikes and high-voltage electric shocks, we believe myocardial injury related to lightning may be managed in the same manner as is cardiac involvement associated with electric shock.

Although influenza is an acute and uncomplicated disease, that limits itself in the healthy children, it may lead to death by rarely forming the sickness. The most common complication of influenza is pneumonia and it is a rare complication which is developed together with myocarditis by influenza A and B viruses. A 32 months-old male patient was admitted for rapidly developed respiratory distress and tachycardia after fever, cough, vomiting, malaise and runny nose. His general status was medium, he had conscious and had hepatomegaly, tachycardia, dyspnea, tachypnea, intercostal-subcostal retractions and bilateral rhonchus. Cardiac enzyme levels and other laboratory parameters were found normal. Myocarditis and ejection fraction was determined as 42% in echocardiography. However, hospitalization hours between 24 and 48, the patient, whose significant respiratory compromise developed, was intubated and fastened to a mechanical ventilator. H1N1 is produce in nasopharyngeal swab culture at the sixth day of follow-up. Because we think H1N1 virus was responsible from current myocarditis, oseltamivir treatment was initiated. In the fourth day of the treatment the patient’s fever returned to normal, in the ninth day a dramatic recovery was observed. In tracking echocardiography, a significant improvement was observed in the ejection fraction and myocarditis picture compared with admission time. This case was presented in order to remind that in a patients, who present with influenza findings but have respiratory distress and tachycardia in addition to lower respiratory tract infection, myocarditis should also be considered in the differential diagnosis and to remind that promising results could be obtained with the early diagnosis and treatment, İnfluenza, sağlıklı çocuklarda genellikle kendi kendini sınırlayan, akut ve komplikasyonsuz bir hastalık olsa da nadiren ciddi hastalık tablosu oluşturup ölüme neden olabilir. Enfeksiyonun en sık komplikasyonu pnömoni olup, miyokardit influenza A ve B virüsüne bağlı olarak gelişebilen nadir bir komplikasyondur. 32 aylık erkek hasta ateş, öksürük, kusma, halsizlik, burun akıntısı, sonrasında hızlı gelişen solunum sıkıntısı ve taşikardi şikâyetleri ile kabul edildi. Genel durumu orta, bilinci açık, hepatomegali, taşikardi, dispne, takipne, interkostal-subkostal çekilme ve bilateral ronküs mevcuttu. Kardiak enzim düzeyleri ve diğer laboratuvar parametreler normaldi. Ekokardiyografide miyokardit ve ejeksiyon fraksiyonu %42 olarak bulundu. Ancak takiplerinin 24-48. saatinde belirgin solunum sıkıntısı gelişen hasta entübe edilip mekanik ventilatöre bağlandı. Hastanın nazofarinks sürüntü kültüründe takiplerinin altıncı gününde H1N1 üredi. Mevcut myokardit tablosunun H1N1virüsüne bağlı olabileceği düşünülerek Oseltamivir tedavisi başlandı. Tedavinin dördüncü gününde ateşi normale dönen hastanın dokuzuncu gününde kliniğinde dramatik bir iyileşme görüldü. Ekokardiyografi takiplerinde ise ejeksiyon fraksiyonu ve myokardit tablosunda başvuru anına göre belirgin düzelme gözlendi. Gribal enfeksiyon bulgularıyla başvurup solunum sıkıntısı ve taşikardi gelişen hastalarda, alt solunum yolu enfeksiyonu yanında miyokarditin de ayırıcı tanıda düşünülmesi ve erken tanı ve tedavi ile yüz güldürücü sonuçların alınabileceğini hatırlatmak amacıyla sunuldu.Anahtar Kelimeler: Çocuk, H1N1, influenza virüsü, myokardit, oseltamivir

Objectives: This study evaluated the opinion of high school students about anti-tobacco law by questioning whether adolescents have access to sufficient information about the hazards of smoking and the resources available to help quit. Methods: The study population included all 11th grade students studying at eight different schools in Ankara. 1266 students were evaluated by a questionnaire intented to secure information about their smoking habits and concerning their opinions on smoking. Results: Totally 1244 students (697 females and 547 males) enrolled the study. The mean age was 17.1 ± 0.6 years (15-20). We evaluated each of the school groups and observed that the highest smoking rate (24%) was among the vocational school students, and the lowest rate (7.5%) was among medical vocational high school students. We found that 47.7% of the students knew the content of the anti-smoking laws and approximately 81.8% of them approved the punishments. About 60% of students who were participated in study mentioned that they were impressed with the informative short films broadcast in Visual Media about the harmful effects of cigarettes. Conclusion: The results of the study showed that young population are aware of the new laws about smoking however this awareness don9t effects their smoking behaviors. The barriers preventing young smokers from seeking professional help should be identified and they should be managed accordingly. The more active roles of educators and parents in tobacco control programs may be effective to be more successful in the fight against smoking.

Objective To evaluate the epidemiologic, clinical and laboratory characteristics of a group of children with type 1 diabetes mellitus (T1DM) living in a Turkish city. Methods The records of 395 (boys/girls: 199/196) children with newly diagnosed T1DM hospitalized in the years 1985-2004 were evaluated retrospectively. The data were assessed by gender and age subgroups (≤5, 6-10 and ≥11 years). Results Mean age of children at diagnosis was 8.1±4.1 years. At T1DM onset, the number of children ≤5, between 6-10 and ≥11 years old was 110 (27.9%), 147 (37.2%) and 138 (34.9%), respectively. The patients were mostly diagnosed at ages 6-8 years (24.1%), followed by cases aged 3-5 years (22.0%). Polyuria and polydipsia were the most common symptoms (94.7%). Mean duration of symptoms was 21.5±18.6 days. Although the patients mostly presented in autumn (30.7%), no season-related significant differences were found. The frequency of ketoacidosis was relatively high (48.5%). When compared to boys, the girls experienced higher rates of ketoacidosis (55.1% vs. 41.7%, p=0.042); had a higher frequency of anti-thyroid peroxidase antibodies (11.7% vs. 4.2%, p=0.049) and higher insulin requirement (0.89±0.41 vs. 0.77±0.36 IU/kg, p=0.005). Cases with a family history of T1DM were more likely to have anti-endomysial antibodies (42.9% vs. 8.1%, p=0.027) and higher initial blood glucose levels (510.5±145.0 vs. 436.1±156.5 mg/dL, p=0.005). Conclusion The findings possibly indicate a decreasing age of T1DM onset. The high frequency of ketoacidosis at presentation is noteworthy. Girls had higher rates of ketoacidosis, higher frequency of anti-thyroid antibodies and higher insulin requirements as compared to boys. Patients with a family history of T1DM had higher initial glucose levels and higher frequency of anti-endomysial antibodies.

Cytotoxin-associated gene A (CagA) product is a bacterial virulence factor contributing to the pathogenicity of Helicobacter pylori (HP) infection in humans. Host factors, which vary in different countries, interact with bacterial factors to determine the disease state. Our objective was to investigate the frequency of CagA-positive HP strains and evaluate the contribution of CagA positivity to symptoms and development of mucosal lesions in HP-infected Turkish children. We conducted a prospective clinical trial in 240 consecutive Turkish children undergoing endoscopy (110 girls, 130 boys; mean age, 8.7 ± 4.3 years). HP infection was diagnosed on the basis of a positive rapid urease test and histology of the mucosal specimens. HP IgG and CagA IgG antibodies were measured by enzyme-linked immunosorbent assay in HP-positive children. The HP positivity rate was 50.4% in our study group (51 girls, 70 boys; mean age, 9.9 ± 3.9 years). CagA was positive in 74.4%. HP infection was less common in children with vomiting (25.9%, P < 0.05). CagA positivity was not associated with any clinical symptom. HP positivity was higher in children with duodenal ulcer (80% vs. 49.1%, P = 0.05); while CagA positivity was similar. Antral nodularity was strongly associated with HP positivity and CagA positivity (30.6% vs. 3.4% and 36.7% vs. 12.9%, respectively, P < 0.05). A negative association between CagA positivity and esophagitis was observed (20% vs. 76.7%, P < 0.05). CagA positivity is common in HP-infected Turkish children. Esophageal lesions are less common in children infected with CagA-positive strains. Although HP is associated with duodenal ulcer disease, CagA positivity does not seem to contribute to development of ulcers in children in our series.

We report 32 biotinidase-deficient patients detected by family studies in the index cases. The study group consisted of 10 mothers, 4 fathers and 18 siblings. There were 17 individuals (3 mothers, 4 fathers and 10 siblings) with profound biotinidase deficiency (BD) (< 10% of mean normal activity) and 15 (7 mothers and 8 siblings) with partial BD (10–30% of mean normal activity). In the profound BD group, only three siblings were symptomatic. Dermatitis, microcephaly, developmental delay and convulsions were observed. The patients with partial BD did not have any clinical symptoms except one sibling with borderline IQ score. None of the parents was symptomatic. Family investigation of patients with BD is very important for the detection of asymptomatic patients who are at risk of exhibiting symptoms at any age. Careful evaluation of these untreated individuals with BD is important to obtain additional information about the natural history of this disorder and may provide clues to phenotype–genotype relationships and treatment regimes.

PMID: 30325127 Akın A, Bilici M, Demir F, Yılmazer MM, İpek MŞ, Kara H. Percutaneous retrieval of umbilical vein catheter fragment in an infant two months after embolization. Turk J Pediatr 2018; 60: 191-193. Umbilical vein catheterization is frequently preferred and a safe route of venous access especially in newborns. However, some cases with breaking and embolization of those catheters have been rarely reported. Herein we present a two-and-a-half-month-old infant being catheterized within first postnatal week and diagnosed to have embolization of the catheter fragment to conjunction of hepatic vein and right atrium. Percutaneous withdrawal of broken catheter was achieved despite several months after the embolization took place. We suggest that transcatheter removal of catheter fragment embolizations may be safe even in late diagnosis cases.

Double-chambered left ventricle (DCLV) is a rare congenital abnormality in which the left ventricle is divided into two separate chambers by a septum or anomalous muscular structure. The chambers are observed mostly parallel to each other without stenosis, and less frequently in a superior-inferior arrangement. An asymptomatic girl is presented here who was diagnosed with DCLV on echocardiographic examination that was performed for the evaluation of cardiac murmur detected by a pediatrician. She has been followed up without treatment.

Reports state that Down syndrome (DS) patients with congenitally normal hearts might experience the development of cardiac abnormalities such as cardiac autonomic dysfunction, valvular lesions, bradycardia, and atrioventricular block. However, the presence of any difference in terms of P-wave dispersion (PWd) and QT dispersion (QTd) was not evaluated previously. This study prospectively investigated 100 DS patients with structurally normal hearts and 100 age- and sex-matched healthy control subjects. Standard 12-lead electrocardiograms were used to assess and compare P-wave and QT durations together with PWd and QTd. The median age of the DS patients and control subjects was 48 months. Heart rates and P-wave and QT dispersions were significantly greater in the DS group than in the control group (113 +/- A 22.9 vs 98.8 +/- A 16.6 bpm, p < 0.001; 31.3 +/- A 9.5 vs 24 +/- A 8.6 ms, p < 0.001; and 46.6 +/- A 15.9 vs 26 +/- A 9.1 ms, p < 0.001, respectively). A positive correlation was found between PWd and age in the DS patients (p < 0.05; r = 0.2). All children with DS should be followed up carefully with electrocardiography in terms of increased P-wave and QT dispersions even in the absence of concomitant congenital heart disease for management of susceptibility to arryhthmias.

Guvenc, Tolga/0000-0003-1468-3415 WOS: 000343078300003 PubMed: 25318911 Background: Cardiac contusion is an important cause of mortality after blunt chest trauma (BCT). The aim of this study was to investigate the therapeutic efficacy of the usage of aminoguanidine (AG), in myocardial damage occurring after BCT, alone and in combination with methylprednisolone (MP). Methods: Thirty-five female Wistar albino rats were randomly assigned to five groups (n = 7) including: sham controls (S); only cardiac contusion (CONT); cardiac contusion treated with methylprednisolone (CONT+MP); cardiac contusion treated with aminoguanidine (CONT+AG); and cardiac contusion treated with methylprednisolone and aminoguanidine (CONT+MP+AG). Seven days following the treatments, heart and serum specimens were evaluated histopathologically, immunohistochemically, and biochemically in all groups. Results: Serum AOPP and Tn-I levels increased significantly after cardiac contusions. Haemorrhage, tissue degeneration, and necrosis development was evident following contusions. Increased iNOS expression in myocardial tissue was significantly decreased in the CONT+AG+MP group compared to CONT+AG and CONT+MP groups (p = 0.001 and p = 0.011, respectively). The combined treatment of AG and MP increased Bcl-2 expression significantly after contusions compared to the other treatment groups. Conclusions: Combined usage of AG, a selective iNOS inhibitor, with MP, in cardiac contusions, showed a more powerful cardioprotective effect by increasing Bcl-2 expression and reducing iNOS expression (Tab. 3, Fig. 4, Ref. 33). Text in PDF www.elis.sk.

In this prospective study, we aimed to assess left and right ventricular function in terms of the presence of right bundle branch block (RBBB) in the cases with repaired ventricular septal defect (VSD). Fifty-three patients who had VSD surgery at least 1-year preceding admission and 52 healthy controls were enrolled into the study. All the participants underwent electrocardiographic and echocardiographic examination. The cases with RBBB were determined. The conventional and tissue Doppler echocardiographic measurements of the patients with and without RBBB were compared with each other and healthy controls. Twenty-eight of VSD repair groups were male and 25 were female. Control group consisted of 30 males and 22 females. The mean age of the study and control groups was 7.5 ± 5.0 and 6.9 ± 4.3 years, respectively. RBBB was detected in 20 of 53 (37.7 %) operated patients. The only significant difference between the cases with and without RBBB was decreased right ventricular fractional area change (%) in the former group (33 ± 7 vs. 39 ± 5 p0.05). When compared to controls, operated group had statistically lower [corrected] tricuspid annular plane systolic excursion (p0.05), lower systolic, early diastolic, and late diastolic myocardial velocities, higher left and right ventricular myocardial performance indices, irrespective of the presence of RBBB. The ratios of mitral or tricuspid inflow to left or right ventricular myocardial in early diastolic velocities measured from lateral annular levels were increased in operated group (all p values0.05). In conclusion, RBBB in the cases with surgical VSD repair might be associated with right ventricular dysfunction. Biventricular systolic and diastolic dysfunction may develop following VSD repair irrespective of the presence of RBBB. Tissue Doppler-derived myocardial performance indices are useful in detection of those subclinical dysfunctions.

We present a rare case of spontaneous closure of a fistula between the left coronary artery and the right ventricle (RV) within a few days of newborn period. A 14-day-old male newborn was referred to our clinic for investigation of tachypnea and cardiac murmur. A color flow Doppler echocardiography revealed turbulent flow of a large coronary artery fistula (CAF) between the left coronary artery and the RV. Tachypnea was regressed and repeat echocardiogram showed no CAF related to cardiac chambers after 4 days of admission. The spontaneous closure of CAF was found to be more likely in cases younger than 2 years with small-sized fistulas opening into the right-sided structures, especially into the RV. Nevertheless, the spontaneous closure is very rare in cases with significant shunt. To the best of our knowledge, this is the first case with documented spontaneous closure of CAF just within the newborn period.

There is increasing evidence of clustering of certain cardiac anomalies in some families. The frequency and echocardiographic features of such anomalies among the relatives of patients with bicuspid aortic valve (BAV) or other left-sided cardiovascular anomalies (LSCA) were evaluated.The patients with BAV or any other LSCA and their relatives were enrolled in the study. They underwent an echocardiographic examination. The probands were assessed in three groups: BAV, BAV + coarctation of aorta (CoA), and other LSCA. Their relatives were also grouped and evaluated accordingly. The echocardiographic measurements were standardized by Z-scores.Eighty-six probands and 261 relatives were evaluated. The numbers of the patients in the BAV, BAV + CoA, and other LSCA group were 52, 14, and 20, respectively. Any LSCA was determined in 17 (6.5%) of the relatives. Thirteen (5%) had aortic dilatation and the remainder (1.5%) had BAV. Accordingly, BAV incidence among relatives of patients with BAV was found to be 1.9%. A second individual with an LSCA was observed in 12.8% of 86 families investigated. The frequencies of aortic stenosis, aortic regurgitation, aortic stenosis + aortic regurgitation, and aortic dilatation in the patients with BAV were found to be 37.9%, 53%, 25.8% and 48.5%, respectively. In contrast to previous reports, no enlargement was observed in the pulmonary arteries of BAV patients.BAV and other LSCA are of clinical significance. Because the clustering of LSCA in some families is observed, we recommend echocardiographic screening of those relatives. If this is not possible, at least it should be achieved for BAV patients.

Rheumatic fever and rheumatic heart disease continue to be an important public health problem in developing countries. Doppler echocardiography is now widely used for early detection and recurrence of clinical evident carditis (CC) and silent (subclinical) carditis (SC). The aim of this study was to determine the frequency of SC and to compare clinical and echocardiographic features of CC and SC.A total of 156 consecutive patients diagnosed with acute rheumatic fever were included in the study. The patients without clinical evidence but with echocardiographic findings of carditis were diagnosed as having SC.Acute rheumatic fever was diagnosed in 156 patients, and 103 of these (66%) had carditis. The prevalence of SC was 28.2% among these 103 patients. Seventy-four of the patients with carditis were followed up for1 year, and 20 of those had SC. Valvular regurgitation disappeared completely in 18.5% and improved in 45.5% of the CC patients. The recovery and improvement rates in the SC group were 15% and 30%, respectively.It is suggested that Doppler echocardiography be performed in all patients with suspected acute rheumatic fever for early detection of SC. Echocardiography should be used as a diagnostic criterion in order not to miss a diagnosis of SC.

Varicella zoster virus (VZV) causes chickenpox in childhood and herpes zoster, associated with painful vesiculous skin lesions in adults (1-3). Varicella zoster virus pneumonia is uncommon among healthy children, and it is generally observed in immunosuppressed patients (1). Cardiac tamponade is a pediatric emergency characterized by accumulation of an excessive amount of fluid in the pericardial cavity and scarcely accompanies VZV infection. Here, a previously healthy girl who had pericardial tamponade following VZV pneumonia is presented.

Fontan operation is the connection of the systemic venous return to the pulmonary arteries, performed in patients with single ventricle physiology. The Fontan circuit is commonly fenestrated because of early postoperative risks such as high systemic venous pressure and low cardiac output. As it causes progressive cyanosis and increased risk of paradoxical embolism in the follow-up period, occlusion of these fenestrations is generally suggested. Successful closure of extracardiac Fontan fenestration with atrial septal occluder in a 10-year-old girl is reported herein. To our knowledge, this is the first such report from Turkey.

Noonan syndrome (NS; MIM #163950) is an autosomal dominant syndrome characterized by hypertelorism, downward slanting of the palpebral fissures, ptosis, low-set posteriorly angulated ears, short stature, and congenital heart disease, most commonly pulmonary valve stenosis, hypertrophic cardiomyopathy (HCM), and atrial septal defects (ASDs). We report a 6-day-old girl who had an unusual combination of supravalvular pulmonary stenosis (PS), obstructive HCM, secundum ASD, and abnormal mitral chordae. Diagnosis was based on characteristic dysmorphic features and presence of HCM. Subsequent cardiac catheterization confirmed the presence of hemodynamically significant PS, and surgical correction was planned. Supravalvular PS is an unusual cardiac phenotype in NS and its association with HCM and ASD has not been reported previously. A detailed echocardiographic examination is mandatory in NS patients for accurate diagnosis of cardiac phenotype and for further treatment plans.

The aim of this study was to investigate the endothelial dysfunction (ED) and carotid intima-media thickness (IMT) in patients with glycogen storage disease (GSD) types Ia and III. In 22 patients with GSD (13, type Ia; 9, type III) and 18 healthy subjects, endothelial functions of the brachial artery and carotid IMT were evaluated by high-resolution ultrasound. Endothelial-dependent dilatation (EDD) was assessed by establishing reactive hyperemia. EDD and carotid IMTs were compared between the three groups. Mean cholesterol level was slightly higher in GSD type III patients but the difference was not significant. Triglyceride levels and cholesterol to high density lipoprotein (HDL) ratio were significantly higher in GSD type Ia patients. EDD was significantly impaired in GSD type Ia (13% +/- 8%, P = .001) and type III (15% +/- 6%, P = .005) patients when compared with the healthy subjects (22% +/- 4%). The carotid IMT was significantly higher in both GSD type Ia (0.23 +/- 0.03 mm, P =.005) and type III (0.26 +/- 0.05 mm, P = .001) patients when compared with the healthy subjects (0.20 +/- 0.02 mm). Both GSD type Ia and type III patients show significant ED and increased IMT, which are predictors of atherosclerosis.

A patient with a severe neonatal variant of 3-methylcrotonyl-CoA carboxylase (MCC) deficiency is reported. The first child of healthy consanguineous Turkish parents presented on the second day of life with dehydration, cyanosis, no sucking, generalized muscular hypotonia, encephalopathy, respiratory depression requiring mechanic ventilation, macrocephaly, severe acidosis and hypoglycaemia. Elevated C5-OH-carnitine in dried blood spot by tandem MS and elevated urinary excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine suggested MCC deficiency, confirmed by enzyme analysis in cultured fibroblasts. Cerebral ultrasonography and cranial CT findings revealed progressive changes such as disseminated encephalomalacia, cystic changes, ventricular dilatation and cerebral atrophy. Treatment with high-dose biotin and protein-restricted diet was ineffective and the patient died at the age of 33 days with progressive neurological deterioration. Mutation analysis revealed a homozygous mutation in the splice acceptor site of intron 15 in the MCC beta-subunit. Early-onset severe necrotizing encephalopathy should be included in the differential diagnosis of isolated MCC deficiency.

There are many kinds of glycoproteins that have sialic acid residues and it has been reported that these are elevated in some renal diseases and their significance in the pathogenesis of several renal diseases has been investigated. In the present study the serum and urine levels of sialic acid were measured in healthy controls and in children with either poststreptococcal acute glomerulonephritis (PSAGN) or minimal change nephrotic syndrome (MCNS) to test if there is any correlation with clinical and laboratory indices. In PSAGN and MCNS patients the serum and urine sialic acid concentrations at onset and relapse were significantly different from healthy controls (Mann-Whitney U-test P < 0.005). There was not a significant correlation between the clinical severity, serum creatinine and complement C3 levels and serum sialic acid concentrations in PSAGN patients. Also there was not a significant correlation between edema, serum albumin, IgG, transferrin, alpha-1-antitrypsin and serum sialic acid concentrations in MCNS patients. Although high serum and urine sialic acid levels were found in both PSAGN and MCNS patients, it does not have any clinical significance nor is it important as a diagnostic or prognostic marker.

Rhabdomyomas are the most common intracardiac tumours with an incidence of 60–80% in fetal life and are often associated with familial history of tuberous sclerosis (TS) (Chao et al. 2008). Rhabdom...

Hydroxyproline has the same integer molecular weight as leucine and isoleucine and is quantified with these by tandem mass spectrometry. An infant was diagnosed with hyperhydroxyprolinaemia following further evaluation of an elevated "leucine" level in newborn screening by tandem mass spectrometry.

Background Familial Mediterranean fever (FMF) is an inflammatory disease, which is suggested to be associated with increased risk of atherosclerosis. Epicardial adipose tissue (EAT) thickness and the mean platelet volume (MPV) are parameters used in prediction of atherosclerotic risk in various conditions. These parameters were evaluated in children with FMF and compared with healthy controls. Methods Forty-five patients with FMF and 54 age- and gender-matched healthy controls were assessed. Duration of symptoms, age at diagnosis, duration of delay in diagnosis, frequency and duration of FMF attacks, disease severity scores, response to colchicine therapy, MEditerraneanFeVer (MEFV) gene mutations, and MPV values were recorded. EAT thicknesses were measured by echocardiography. Results Epicardial adipose tissue thicknesses of the children with FMF were found to be significantly greater than that of controls (5.1 ± 1.4 vs. 4.5 ± 0.9 mm, p = 0.036). FMF patients had significantly higher MPV values compared with the controls (7.8 ± 1.1 vs. 7.3 ± 1.4 fl, p = 0.044). Age at diagnosis, duration of delay in diagnosis, and MPV values were found to be correlated with EAT thickness in the patient group (r = 0.49, p = 0.001 for the former parameters and r = 0.32, p = 0.04 for MPV). Conclusion Epicardial adipose tissue thickness and MPV values seem to be increased in children with FMF. These findings may indicate an increased risk of atherosclerosis in FMF patients.