We report on a patient with Sedaghatian type spondylometaphyseal dysplasia (SSMD) who presented with metaphyseal dysplasia, congenital atrioventricular block, simplified gyral pattern, hypogenesis of corpus callosum, and severe cerebellar hypoplasia. We want to emphasize that in this rare congenital lethal skeletal dysplasia with unknown etiology, central nervous system malformations might be a major component of the disorder and should be evaluated in detail to possibly uncover the underlying pathophysiology.

During acute phase, patients with KD have reduced global left ventricular S and SR which may be more sensitive indicators of myocardial inflammation. This study showed gradual improvements in left ventricular myocardial function during treatment for KD.

Urinary LT-E4 and PG-F2a were both significantly higher in children with migraine during headache than during non-headache periods. The elevation in the levels of these inflammatory mediators was compatible with the hypothesis relating neuroinflammation in trigeminal vascular blood vessels with migraine pathophysiology. Leukotriene antagonists may be effective in the prophylaxis of migraine attacks.

The objective was to compare the efficacy and safety of naproxen (NXN) to acetylsalicylic acid (ASA) in the treatment of acute rheumatic fever (ARF). The data of 338 children were retrospectively analyzed. The patients were grouped according to joint and valve involvement and also drug chosen [methyl prednisolone (mPSL), ASA or NXN]. The treatment results and adverse events in each group were compared. The mean age was 10.3 years and the median follow-up was 62 months. Median time for normalization of acute phase reactants was 1 week in patients given steroids and 2 weeks in patients given ASA or NXN. ASA was replaced with NXN in 18 patients (10.2%) due to hepatic toxicity. The rate of rebound, recurrence and the prevalence of rheumatic valve disease were not different in patients given NXN, ASA or mPSL. In conclusion, NXN is a safe and effective alternative to ASA in the treatment of ARF in children.

Objectives The aim was to evaluate the role of tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE), to identify myocardial dysfunction, and to evaluate myocardial segmental deformation in acute viral myocarditis. Methods Twenty‐one patients and twelve healthy children were studied prospectively. The TDI and STE were performed before and after treatment. The myocardial velocities (Sm, Em, and Am) and time intervals (isovolumic contraction, isovolumic relaxation, and ejection times [ET]) at interventricular septum (IVS), left, and right ventricular basal segments were examined by TDI. The left ventricular global longitudinal strain (LVGLS) and strain rate (LVGLSR), left ventricular global circumferential strain (LVGCS) and strain rate (LVGCSR), and right ventricular global longitudinal strain (RVGLS) and strain rate (RVGLSR) were examined by STE. Results Sm and Em at IVS and at LV, ET at IVS, ET at RV, ET at LV were significantly lower in patients before treatment than controls. LVGLS, LVGLSR, LVGCS, LVGCSR, RVGLS, RVGLSR were significantly decreased in patients before treatment than controls. There was significant improvement for LVGLS, LVGLSR, LVGCS, LVGCSR, and RVGLS in patients after treatment. Sm, Em, and Am at LV were significantly lower in patients before treatment than in patients after treatment. In spite of improvements, Sm, Em, and ET at IVS, LVGLS, LVGLSR, LVGCS, LVGCSR were significantly lower in patients after treatment than controls. Conclusions The TDI and STE were useful methods for detection of early myocardial dysfunction and evaluation of treatment outcomes in acute viral myocarditis.

3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency, an inborn error of ketone body synthesis and leucine degradation, is a rare autosomal recessive disease. There are a few reports demonstrating clinical and neuroradiologic findings of this condition. The authors report case of an 8-mo-old infant with HMG-CoA lyase deficiency, who presented with macrocephaly, left ventricular noncompaction, recurrent pulmonary infections, nonketotic hypoglycemia, seizure and metabolic acidosis. There was no significant difference in brain magnetic resonance imaging after leucine-restricted diet and carnitine therapy and neurologic deterioration was not observed. Left ventricular noncompaction is an interesting finding for HMG-CoA lyase deficiency which has not been reported in the literature. The genetic analysis revealed a novel homozygote deletion in exon 3 and 4 in HMGCL gene. HMG-CoA lyase deficiency should be thought in the patients with hypoketotic hypoglycemia, hyperammonemia, elevated liver function tests, noncompaction left ventricle and characteristic white matter changes and in the differential diagnosis of macrocephaly.

Objective:Thyroid hormones have an important role in the regulation of the cardiovascular system. The aim of this study was to investigate the presence of subclinical myocardial dysfunction in children with euthyroid Hashimoto’s thyroiditis (eHT) without evident heart disease using tissue doppler imaging (TDI) and speckle tracking echocardiography (STE) methods.Methods:TDI and STE were peformed in 50 children with eHT and in 35 healthy children. To assess myocardial velocities and time intervals, including peak systolic velocity (Sm), peak early diastolic velocity (Em), peak late diastolic velocity (Am), isovolumetric contraction time (IVCT), isovolumetric relaxation time (IVRT) and ejection time (ET), TDI was performed at the base of the interventricular septum (IVS) and in the left and right ventricles (LV and RV, respectively). Analysis of myocardial deformation by STE including strain (S) and strain rate (SR) was performed globally in two planes, longitudinal (L) and mid-circumferential (C) in LV [LV global longitudinal strain (LVGLS), LV global longitudinal strain rate (LVGLSR), LV global circumferential strain (LVGCS), LV global circumferential strain rate (LVGCSR)] and RV [(RV global longitudinal strain (RVGLS), RV global longitudinal strain rate (RVGLSR)].Results:Among TDI parameters, ET at LV and IVS were significantly lower, IVRT and myocardial performance index at LV and IVS were significantly higher in the eHT group compared to controls (p=0.001). There were no significant differences in Sm, Em, Am and IVCT values between patients and controls. LVGLS, LVGLSR, LVGCS and LVGCSR values were significantly lower in patients than controls (p=0.01). There was a negative correlation between thyroid antibody levels and LV global longitudinal and circumferential strain and strain rate values (TPO-Ab and Tg-Ab between LVGLS, LVGLSR, LVGCS and LVGCSR; r=-411, p<0.001; r=-541, p<0.001; r=-430, p<.0.001; r=-502, r<0.01 and r=-397, p<0.001; r=-473, p<0.001; r=-519, p<0.001; r=-421, p<0.00, respectively).Conclusion:The results show that myocardial function in children with eHT is impaired in the absence of any clinical symptoms and that conventional echocardiography is inadequate to determine these changes.

Infective endocarditis (IE) is an infection of the endocardium and/or heart valves that involves thrombus formation (vegetation). This condition might damage the endocardial tissue and/or valves. An indwelling central venous catheter is a major risk factor for bacteremia at-risked pediatric populations such as premature infants; children with cancer and/or connective tissue disorders. Herbaspirillum huttiense is a Gram-negative opportunistic bacillus that may cause bacteremia and pneumonia rarely in this fragile population. Herein we report the very first case of bacteremia and IE in a pediatric oncology patient caused by H. huttiense.