Ali Baykan

Macrosomic newborns of diabetic mothers are associated with increased aortic intima-media thickness and lipid concentrations

Background/Aims: Exposure to diabetes in utero has been established as a significantrisk factor for some of components of metabolic syndrome. A few studies have examined relationship between the metabolic syndrome and echocardiographic left ventricular (LV) mass. We aimed to investigate relationship between abdominal aortic intima-media thickness (aIMT), LV mass and lipid profile in macrosomic newborns. Methods: Abdominal aIMT was measured in 30 macrosomic neonates of diabetic mothers (group A), 30 macrosomic neonates of healthy mothers (group B) and 30 healthy neonates (group C). Lipid profile and LV mass were determined. Result: Mean aIMT was significantly higher in groups A and B (0.489 ± 0.015, 0.466 ± 0.019 mm, respectively) than in controls (0.375 ± 0.024 mm). Weight-adjusted aIMT in group A was significantly higher than in groups B and C. Macrosomia was associated with increased lipid concentrations. Both LV mass indexed for BSA (body surface area) and birth weight measurements were significantly increased in group A compared with control. Conclusions: Macrosomic neonates of diabetic mothers have significant aIMT and LV mass indexed for BSA and birth weight with lipid alterations. It might play a role in the pathogenesis of atherosclerosis in adult life.

Arrhythmogenic right ventricular cardiomyopathy (Naxos disease): report of a Turkish boy.

Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair. This disease is a heart muscle disorder causing life‐threatening ventricular arrhythmias, heart failure, and sudden cardiac death. The pathological hallmark of the disease is the progressive replacement of myocardial cells by fat and fibrous tissue. It appears in families descending from the Hellenic island of Naxos. We presented a 13‐year‐old Turkish boy with Naxos disease associated with ventricular tachycardia because of its rarity, and reviewed the literature. (PACE 2003; 26:2326–2329)

Carotid artery thickness in children and young adults with end stage renal disease

Atherosclerosis is a major cause of morbidity and mortality for ESRD patients and we have little knowledge about the presence and risk factors of atherosclerosis in children with CRF. The measurement of carotid artery intima-media thickness (cIMT) using high-resolution ultrasonography is suggested as an excellent marker of subclinical atherosclerosis. In this study, we aimed to investigate the presence of atherosclerosis and to determine the relationship between atherosclerosis and some risk factors in children and young adults with ESRD. Thirty-four patients with ESRD and 20 controls were included in this study. The measurement of cIMT was performed by using a linear B-mode 7.5-MHz ultrasound transducer. We determined anemia, abnormal calcium/phosphate metabolism, hyperhomocysteinemia, hypertriglyceridemia and increased lipoprotein (a) levels in the ESRD group. The cIMT in the ESRD group was higher than in the control group (P<0.05). SBP, DBP, MAP, LVMI and LVH prevalence were statistically higher in the ESRD group (P<0.05). There were significant positive correlations between cIMT and LVMI, MBP, whereas a significant negative correlation was determined between cIMT and PTH in the ESRD group (P<0.05). When a multiple linear regression analysis was performed with cIMT as a dependent variable and LVMI, MBP, PTH, as independent variables, a significant positive correlation was determined between cIMT and LVMI (P<0.05). In conclusion, we think that arteriopathy occurs in children with ESRD. Left ventricular hypertrophy and hypertension may associate with vascular changes in children and young adults with ESRD. Further investigations are necessary to explain association of LVMI index with cIMT.

Increased endothelial microparticles in obese and overweight children.

Abstract Background: Obesity in children increases the risk of atherosclerosis. Endothelial dysfunction is an important factor in the pathogenesis of atherosclerosis, and endothelial microparticles (EMPs) are considered as markers of endothelial dysfunction. In this study, we aimed to evaluate circulating EMPs in obese and overweight children and to disclose the measure of obesity with the strongest relation with circulating microparticles and carotid atherosclerosis. Methods: This prospective study included 55 obese and overweight children and 23 healthy controls. Insulin resistance was studied. Both in vivo and in vitro human umbilical vein endothelial cell evaluations were used for the study. Circulating EMPs (CD144 and CD146) were measured by flow cytometry. The carotid artery intima-media thickness (cIMT) and left ventricular mass index (LVMI) were measured using ultrasound and echocardiography, respectively. Study groups were compared for anthropometric measurement, insulin resistance, circulating EMP, cIMT, and LVMI. The relationship among overweight, obesity, and circulating EMPs were investigated. Results: Blood pressure, CD144+EMP levels, and LVMI were statistically higher in the patients group than in the control group. The multiple logistic regression analysis and the backward elimination method showed that CD144+EMP and systolic blood pressure had a linear relationship with overweight and obesity. Conclusion: Our results suggest that endothelial damage starts in the early stage of childhood obesity and that obese and overweight children have increased circulating CD144+EMPs, showing that endothelial dysfunction and increased CD144+EMPs may be related to obesity.

The factors that affect milk-to-serum ratio for iron during early lactation.

To determine the factors that affect milk iron content at the second week of lactation and whether the supplementation to lactating mother with iron might increase breast milk iron content between 2 weeks and 4 months postpartum. Healthy mothers were enrolled 10 to 20 days postpartum, if their babies were term, normal gestational age, and exclusive breastfed. Maternal blood samples for hemoglobin (Hb), iron, iron binding capacity, serum ferritin, and breast milk samples for iron and zinc were taken and mothers were randomized into iron supplemented and placebo groups. At the end of the fourth month, blood and milk samples were taken again. Forty-seven healthy mothers were included into the study. Milk iron content was lower; however, milk-to-serum iron ratio was higher in cases with low maternal iron reserves than cases with adequate iron reserves. Mothers with low Hb level (<12 g/dL) had higher milk zinc content and lower milk iron/zinc content than mothers with normal Hb value. Iron supplementation to lactating nonanemic mothers did not change milk iron content and the decline in milk iron content and milk-to-serum iron ratio. Milk iron content and milk-to-serum iron ratio of iron could be regulated by active transport in cooperation with maternal iron status.

The effects of dexmedetomidine on early stage renal functions in pediatric patients undergoing cardiac angiography using non‐ionic contrast media: a double‐blind, randomized clinical trial

In this study we aimed to investigate the effects of dexmedetomidine on early stage renal function in pediatric patients undergoing cardiac angiography.

Guillain–Barré Syndrome Associated with Legionnella Infection

This is the first report of Guillain-Barré syndrome (GBS) related to Legionnella pneumophilia infection. A 13-year-old boy presented with acute dysphagia and dyspnea. He lived in a rural area and had a history of drinking potable deep-hole water. The patient was intubated because of increased respiratory distress. A positive direct fluoresein antigen test confirmed L. pneumophilia infection in BAL. One week after the first admission, acute weakness was noticed including the lower extremities and was more prominent in the distal than the proximal portions. GBS was considered as the initial diagnosis. Tests for all causes known to trigger GBS were negative. Specific serology for L. pneumophilia IgG was positive. He was treated with intravenous immunoglobulins and discharged with minor weakness and difficulty in walking in the second month. On the basis of this case, L. pneumophilia should be included in the etiologic spectrum of GBS.

Cheatham platinum stent implantation in children with coarctation of the aorta: single-centre short-term, intermediate-term, and long-term results from Turkey.

OBJECTIVES Our aim was to evaluate patients who were treated by percutaneous stent implantation. METHODS Patients with aortic coarctation (n = 35) who had been treated with 38 stents - 12 bare and 26 covered - were evaluated. The demographics and procedural and follow-up data were recorded from hospital registers and compared according to patient specifications, for example, weight and coarctation nature. RESULTS The procedure was successful in all patients. There was a statistically significant difference between the patients with native coarctation (n = 17) and those with recurrent coarctation (n = 18) in terms of pre-procedural blood pressures, systolic gradients, coarctation diameters, and the ratio of the coarctation site diameter to the descending aorta diameter. Although all patients received antihypertensive drugs before the procedure, the drug was discontinued in 26 patients during follow-up (p < 0.001). Stent migration was observed in four patients with recurrent coarctation (11.4%), and peripheral arterial injury was seen in three patients (8.5%). The mean follow-up time was 34 ± 16 months. On average, 21 (6-42) months after the procedure, six patients underwent cardiac catheterisation. At least 2 years after the procedure, tomography was performed in 20 patients (57.2%). Patients who were evaluated by multi-slice computerised tomography revealed no pathologies. There was no statistically significant difference between the five patients weighing less than 20 kg and the other 30 patients in terms of demographic and procedural characteristics, procedure success and complication rates, and follow-up data. CONCLUSION Stent implantation for aortic coarctation is a method yielding satisfactory results in reducing coarctation gradients, efficient enlargement of the lesion area, and resolution of hypertension for children, including those weighing less than 20 kg.

Different clinical presentations of Naxos disease and Carvajal syndrome: Case series from a single tertiary center and review of the literature.

Objective Naxos disease is an autosomal recessive, inherited, cardiocutaneous disorder, characterized by arrhythmogenic right ventricular cardiomyopathy, woolly hair, and palmoplantar keratoderma. Carvajal syndrome is characterized by palmoplantar keratoderma, curly hair, dilated cardiomyopathy, especially on the left ventricle side, and early morbidity. The aim of this study was to evaluate the cutaneous and cardiac findings and genotype-phenotype relationship of six patients diagnosed with Naxos/Carvajal syndrome. Methods A retrospective review of six cases diagnosed with Naxos/Carvajal syndrome at our institution from 2002 to 2012 was performed. Demographic data; presenting complaints; cutaneous and cardiac findings; electrocardiography, echocardiography, and genetic analysis results; and treatment data were obtained from patient files. Results The patient group was composed of 4 males and 2 females, ranging from 1.5 to 13 years, with a mean age 6.4 years. Typical cutaneous and hair findings were present in all patients. Two cases presented with ventricular tachycardia attack, and 2 cases presented with severe heart failure. Two cases had only cutaneous findings without cardiac involvement at diagnosis. An implantable cardioverter-defibrillator was implanted in one case due to ongoing recurrent ventricular tachycardia attacks despite various antiarrhythmic treatments. Three of the 6 patients died during the follow-up. Conclusion For cases with woolly hair and palmoplantar keratoderma, the physician should provide a cardiac assessment, considering Naxos/ Carvajal disease associated with cardiomyopathy. When an early diagnosis is made, the life expectancy may be increased by treatment of heart failure and arrhythmias; also, genetic counseling should be performed.

Aorta-right atrial tunnel closure using the transcatheter technique: a case of a 3-year-old child.

Aorta-right atrial tunnel is a rare congenital defect. If it is not treated, critical complications can occur. Surgical closure is the treatment of choice, but with increasing experience in transcatheter techniques and the introduction of new occluder devices the percutaneous technique is now a successful modality for closure. In this paper, we report the case of a 3-year-old child with aorta-right atrial tunnel whose defect was closed with the vascular plug 4 device.