Children and adolescents with type 1 diabetes mellitus have a sixfold greater risk for prolonged QTc interval.

BACKGROUND: QT-wave abnormalities have been detected in type 1 diabetes mellitus (T1DM). Prolongation of the heart rate corrected QT interval (QTc) has been associated with cardiovascular mortality. We evaluated how often QT/QTc abnormalities are present in youth with T1DM and if they are associated with disease parameters. METHODS: Sixty-two T1DM youngsters and equal age- and gender-matched controls were studied. Demographic, anthropometric, and laboratory data were determined. QT was measured on a 12-lead resting electrocardiogram. QTc was calculated using Bazett's formula. RESULTS: T1DM patients had significantly longer QT/QTc than controls, but significance disappeared after adjustment for confounders. Abnormally prolonged QTc≥440 ms was observed six times more frequently in those with T1DM. QT was correlated with age, age at disease onset, but not with glycated hemoglobin or diabetes duration; QTc was only correlated with pubertal stage. CONCLUSIONS: T1DM youths have a sixfold increased risk for QT/QTc prolongation and should have regular follow-up for cardiac autonomic dysfunction.

Correlation of plasma B-type natriuretic peptide with shunt volume in children with congenital heart disease involving left-to-right shunt.

INTRODUCTION: Concentrations of B-type natriuretic peptide (BNP) are recognised as a reliable marker of ventricular dysfunction in adults. In this study, plasma levels of BNP were determined in children with congenital heart disease (CHD) involving a left-to-right shunt, and were correlated with the shunt volume. METHODS: Seventy-six children (38 boys/38 girls, mean age 22.4 months) with CHD (Group A: 31 with atrial septal defect [ASD], 23 with ventricular septal defect [VSD], 8 with ASD and VSD, 14 with patent ductus arteriosus [PDA]) and 34 healthy children (group B) were studied. BNP was measured by chemiluminescent microparticle immunoassay in all children. The amount of shunt (the ratio of pulmonary blood flow/systemic blood flow: Qp/Qs) was measured using Doppler velocimetry and two-dimensional echocardiography. A haemodynamically significant left-to-right shunt was defined as Qp/Qs>1.5. Correlations were evaluated between all patient groups and healthy subjects and BNP was compared with echocardiographic data reflecting right and left ventricle volume overload. RESULTS: Thirty-four children of group A had Qp/Qs>1.5 (group A1) and 42 Qp/Qs<1.5 (group A2). BNP levels were higher in group A1 than group A2 (p=0.015), while there were no significant differences in BNP between group A2 and group B (p=0.79). BNP 24.4 pg/ml was determined as the cut-off point to identify patients with Qp/Qs>1.5. BNP values were similar among patients with ASD and VSD, but they were significantly higher in patients with PDA. BNP was positively correlated with Qp/Qs (r=0.59, p<0.001), and with the pulmonary artery velocity (r=0.27) and gradient (r=0.49), while there was a negative correlation with ejection fraction (r=-0.14). BNP levels were significantly higher in 10 infants with clinical signs of heart failure (p=0.025). CONCLUSION: These results, which are consistent with previous reports, suggest a possible role of BNP as an early diagnostic marker of the significance of shunt in children with CHD.

Evaluation of Oral Glucose Tolerance Test in Children With Epilepsy.

Glucose metabolism of children with drug-resistant epilepsy, controlled by antiepileptic drugs epilepsy, and first-time nonfebrile seizures was studied through the performance of an oral glucose tolerance test and through insulin, C-peptide, and glycosylated hemoglobin measurements. In the refractory epilepsy group, there were more abnormal oral glucose tolerance test results (62.07%) in comparison to the controlled epilepsy group (25%) and the group of first-time seizures (21.21%). There was a significant difference between the group of refractory epilepsy and every other group concerning the abnormality of the oral glucose tolerance test (P < .05). The mean values of insulin, HbA1c, and C-peptide levels were normal for all groups. The results of the present study suggest that there is a distinction of refractory epilepsies from the drug-controlled ones and the first-induced seizures relating to their metabolic profile, regardless of the type of seizures.

Skills of primary healthcare physicians in paediatric cardiac auscultation.

AIM: To evaluate the performance of primary healthcare physicians in paediatric cardiac auscultation and the impact of a multimedia-based teaching intervention. METHODS: A total of 106 primary healthcare physicians (77 paediatricians, 14 general practitioners and 15 medical graduates) attended four paediatric cardiac auscultation teaching courses based on virtual patients' presentation (digital phonocardiography). Their auscultatory performance was documented at the beginning of each course and at the end of two of the courses. RESULTS: Participants initially detected 73% of abnormal murmurs and 17% of additional sounds, while 22% of innocent murmurs were interpreted as abnormal. Overall cardiac auscultation performance, assessed by a combined auscultation score, was low and independent of training level (graduates: 39.5/trainees: 42.8/board certified: 42.6, p = 0.89) or specialty (paediatricians: 42.7/general practitioners: 43.1, p = 0.89). Multimedia-based teaching was associated with a significant improvement in abnormal murmur (92.5%) and additional sound (40%) detection (p < 0.001), while 25% of innocent murmurs were still interpreted as abnormal (p = 0.127). CONCLUSION: Clinical skills of primary healthcare physicians in paediatric cardiac auscultation, independent of training level or specialty, still leave potential for improvement. Multimedia-based teaching interventions represent an effective means of improving paediatric cardiac auscultatory skills.

Cardiac complications and immunophenotypic profile of infectious mononucleosis syndrome in children.

OBJECTIVE: To investigate cardiac complications in infectious mononucleosis patients and to associate them with biochemical and immunological parameters, as well as with spleen ultrasound findings. DESIGN: Cross-sectional study with follow-up. SETTING: Tertiary care pediatric unit, in the city of Thessaloniki, Greece. PARTICIPANTS AND INTERVENTIONS: Twenty-five children (15 boys, aged 1-11.6 years) suffering from infectious mononucleosis were studied during the acute phase and after 3-6 months. Cardiac evaluation comprised of electrocardiogram, echocardiogram, and measurement of creatine phosphokinase, creatine phosphokinase cardiac isoenzyme, and troponin levels. Biochemical and immunological tests included serum transaminases, serum amylase, CD3+/CD8+ T-lymphocytes subpopulation and CD4+/CD8+ T-lymphocytes ratio. RESULTS: During acute phase, all children had splenomegaly and normal serum amylase values. 17 patients had elevated serum transaminases. Percentages of CD3+/CD8+ T-lymphocytes subpopulation were elevated and CD4+/CD8+ ratio was decreased in all patients. Echocardiography revealed mild pericardial effusion in 13 patients (10/21 with Epstein-Barr infection, 3/4 with cytomegalovirus infection), but none presented with myocarditis. Four out of these 13 patients also had markedly elevated liver enzymes, 10/13 had significant splenomegaly and 12/13 presented very low CD4+/CD8+ T-lymphocytes ratio. Pericardial effusion demonstrated a statistically significant association solely with very low CD4+/CD8+ T-lymphocytes ratio (<0.5). Repetition of laboratory tests 3-6 months post-discharge detected persistent mild pericardial effusion in five patients, along with decreased CD4+/CD8+ ratio in 1/5. CONCLUSIONS: In infectious mononucleosis syndrome, asymptomatic pericardial effusion could be associated with very low CD4+/CD8+ ratio (<0.5). Further studies would extend and confirm such an association.

Ghrelin and growth hormone serum levels during the clonidine test in children with short stature and variable growth hormone status.

OBJECTIVE: The exact role of ghrelin in the control of growth hormone (GH) secretion has not been completely clarified as yet. The aim of the present study was 1) to investigate the effect of a substance promoting GH secretion (clonidine) on ghrelin levels in children with short stature with growth hormone deficiency (GHD) and normal growth hormone (NGH), and 2) to assess possible correlations between GH and ghrelin values during the clonidine test. DESIGN: Eighteen prepubertal children with short stature were included in the study. Using the results of two GH-provocative tests (glucagon and clonidine), the participants were divided into two groups: GHD and NGH. In both groups, ghrelin levels were determined during the clonidine stimulation test. RESULTS: Different responses regarding ghrelin levels during the clonidine stimulation test were observed in the two study groups (GHD and NGH). A decrease in ghrelin levels was observed in the NGH children accompanied by a rise in the circulating GH levels, whereas the GHD children demonstrated a rise in both ghrelin and GH levels. CONCLUSIONS: The data indicate an inverse relationship between circulating ghrelin and GH in NGH children, suggesting the presence of a negative feedback loop between ghrelin and GH. Analogous changes were not observed in GHD children.

Insulin resistance is associated with at least threefold increased risk for prothrombotic state in severely obese youngsters.

UNLABELLED: Obesity in childhood increases the risk for early adult cardiovascular disease. However, the underlying mechanism is not fully known. The aims of this study were to measure levels of prothrombotic factors and examine their possible association with obesity and insulin resistance in obese children and adolescents. A total of 313 obese children and adolescents were recruited. In a cross-sectional design, we measured anthropometric parameters, plasminogen activator inhibitor-1-antigen (PAI-1-Ag), von Willebrand factor-antigen (vWF-Ag), fibrinogen (FB), lipids, fasting glucose, and insulin (FI) levels. Insulin resistance was estimated using the homeostasis model assessment for insulin resistance (HOMA-IR) index. Boys presented significantly higher PAI-1-Ag levels than girls (82.6 vs. 71.3 ng/ml, p = 0.01). Higher levels of PAI-1-Ag (96.8 vs. 69 ng/ml, p < 0.001), vWF-Ag (123.5 vs. 107.6%, p = 0.004) but not FB (353.1 vs. 337.6 mg/dl, p = 0.137) were found in insulin-resistant (IR) participants after adjusted for age, gender, and pubertal stage. IR patients were at 2.98 (CI: 1.084-8.193) and 4.86 (CI: 1.119-15.606) times greater risk for high PAI-1-Ag and vWF-Ag levels, respectively. All three prothrombotic factors were positively correlated with body mass index (BMI) and FI levels (p < 0.05), but only PAI-1-Ag and vWF-Ag were significantly correlated with HOMA-IR index (p ≤ 0.001). After adjustment for confounding factors, both BMI and HOMA-IR indices remained significantly associated with PAI-1-Ag (r2 = 0.225, p < 0.001) and vWF-Ag levels (r2 =0.077, p = 0.003). CONCLUSION: This study shows that obesity in youngsters, when accompanied with insulin resistance, is associated with at least threefold increased risk for elevated levels of prothrombotic factors, contributing to the early development of atherothrombosis. This impaired prothrombotic state may partially explain the increased risk for developing cardiovascular disease later in adulthood.

Anticonvulsant hypersensitivity syndrome closely mimicking Kawasaki disease.

Anticonvulsant hypersensitivity syndrome (AHS) is an acute, life-threatening, idiosyncratic drug reaction seen within 1-8 weeks after administration of an aromatic antiepileptic drug. The authors present the case of a 16-month-old boy who developed prolonged fever, a generalised pruritic rash and eosinophilia within 4 weeks after starting treatment with phenobarbital for complicated febrile seizures. He gradually fulfilled the diagnostic criteria for classical Kawasaki disease (KD), although the rash and the subsequent desquamation were atypical, he did not defervesce quickly with administration of corticosteroids and intravenous γ-globulin, and he had only two suggestive cardiac features of KD-that is, perivascular echogenicity of the coronary arteries and a small pericardial effusion. Other conditions considered in the differential diagnosis were excluded by appropriate extensive serological and microbiological studies. He recovered fully. This report shows that drugs such as phenobarbital may be responsible for febrile exanthematous illnesses that closely mimic KD.

A female infant with Silver Russell Syndrome, mesocardia and enlargement of the clitoris.

Silver Russell Syndrome (SRS) is a rare condition (1/3000 - 1/100,000 newborns). We present a female infant with SRS, cardiac malposition and asymmetric enlargement of the clitoris. She is the first child of Greek nonconsanguinous parents, born at 38 weeks gestation, following in vitro fertilisation (IVF). The patient had intrauterine growth retardation, body asymmetry, enlarged clitoris, hemihypertrophy of external genitalia and features characteristic of SRS. Electrocardiography and chest X-rays revealed a median position of the heart. The infant fulfilled the criteria proposed by Price et al for SRS. Genetic analysis did not reveal mUPD of chromosome 7. This is the first report of a patient with SRS presenting ìmesocardiaî and asymmetric enlargement of the clitoris. Our case constitutes another paradigm of SRS following IVF, which possibly supports the hypothesis that IVF may be associated with higher prevalence of SRS than natural fertilisation.

Magnetic resonance angiography, function and viability evaluation in patients with Kawasaki disease.

OBJECTIVES: We evaluated the ability of magnetic resonance imaging to perform a noninvasive assessment of coronary arteries, function and viability in one examination in a population with Kawasaki disease. BACKGROUND: Magnetic resonance angiography (MRA) can identify coronary abnormalities in patients with Kawasaki disease (KD). Contrast enhanced cardiovascular magnetic resonance (CeCMR) is the current gold standard for scar detection. Steady-state, free precession (SSFP) cine is a reliable technique to evaluate myocardial function and wall motion. METHODS: Twenty patients with KD aged 7-12 yrs, were examined. Coronary MRA was performed using a 1.5 T system with two ECG-triggered pulse sequences. CeCMR images were acquired 15 minutes after the i.v. injection of 0.1 mmol/kg Gd-DTPA using an inversion recovery sequence. SSFP cines were acquired using 6-mm short-axis slices from the atrioventricular ring to the apex. RESULTS: Aneurysms of the coronary arteries were identified in 7 patients and coronary ectasia was present in the remaining 12 patients while 1 patient had both. Transmural anterior-apical scar was detected by ceCMR in two cases, while small inferior necrosis was identified in another 2 cases. Left ventricular function was deteriorated only in the two patients with antero-apical infarction. The presence of myocardial infarction was detected in the territory supplied by the involved coronary artery. CONCLUSION: In Kawasaki disease MRA, SSFP cine and ceCMR are able to perform noninvasive coronary artery evaluation, function and infarct detection in a single study.

Acute laryngotracheobronchitis and associated transient hyperphosphatasemia: a new case of transient hyperphosphatasemia in early childhood.

We report the case of a 20-month old boy with markedly elevated serum alkaline phosphatase (ALP) levels, documented during an episode of acute laryngotracheobronchitis. Biochemical investigations and imaging studies revealed no evidence of bone or liver disease. Transient hyperphosphatasemia (TH) was confirmed when serum ALP levels normalized within 2 months. Several theories were suggested for TH pathophysiology, viral infections among them; the exact causes, however, remain unclear. It is important to recognize TH and avoid misdiagnosis and unnecessary investigations.

Lipid profile of children with a family history of coronary heart disease or hyperlipidemia: 9-year experience of an outpatient clinic for the prevention of cardiovascular diseases.

The authors evaluated the lipid profile of children with a positive family history of coronary heart disease (CHD), cerebrovascular disease (CVD), or hyperlipidemia and compared them with controls in order to identify risk indicators for atherosclerosis. A group of 315 children (group A) aged more than 2 years old with a positive family history were evaluated for serum concentrations of total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), triglycerides (TG), apolipoprotein B100 (ApoB100), apolipoprotein A1 (Apo A1), and lipoprotein (a) (Lp[a]). These values were compared with the levels of a control group of 214 children of comparable age (group B). The median age of children in groups A and B was 10.6 (range 2.3-16) and 9.8 (range 3-13.7) years of age, respectively. Among these children, 196 (52%), 47 (12.5%), and 72 (19.1%) had a positive family history of CHD (group A1), cerebrovascular disease (CVD) (group A2), and hypercholesterolemia (group A3), respectively. We identified 8 children with genetically determined dyslipidemia: 2 children with homozygous and 6 with heterozygous familial hypercholesterolemia. Children in group A3 had significantly higher concentrations of TC, TG, LDL-C, and ApoB100 and lower concentrations of Apo A1 compared with controls, while no significant differences were found in concentrations of lipid variables among children of group A1, A2, and A3. Significant differences were also noted in the concentrations of TC, LDL-C, and Lp(a) between children of group A1 and controls. Screening the progeny of young patients with CHD or familial hypercholesterolemia can identify children at excessive risk for future vascular disease.