[Cardiovascular diseases are the leading cause of mortality and morbidity in children with chronic kidney disease (CKD). Similar to adults, children with CKD experience a high burden of traditional and uremia-associated risk factors. Recent years, several studies were published in connection with cardiovascular risk factors, patomechanism, and early markers of cardiovascular diseases. Early signs of cardiomyopathy, such as left ventricular hypertrophy or dysfunction, and markers of atherosclerosis, such as increased intima-media thickness of the carotid artery or increased wall stiffness of the aorta are frequently present in early stages of CKD in children. As prevention is important in pediatrics, the evaluation of subtle changes of the cardiovascular system provide opportunity for early treatment and that enables children to develop normally and have a better long-term quality of life. Recently, newer non-invasive cardiovascular imaging modalities have been emerged to diagnose subclinical alterations of the heart and vessels in this specific population with kidney disease. In this review, we provide an overwiev of the emerging imaging techniques used to detect early subclinical organ damage in pediatric chronic kidney disease patients.]

[Pulmonary arterial hypertension is a rare disease, but it occurs more often in systemic autoimmune diseases, where it represents one of the most severe, life-threatening complications. Its development is due to an immunoregulatory disorder characteristic to systemic diseases, persistent inflammation and the subsequent endothelial dysfunction, the presence of pathogenic autoantibodies, smooth muscle cell dysfunction and complex angiogenetic disorder. As a consequence of endothelial cell dysfunction, the balance between regulatory factors of vasoconstriction and vasodilation is disrupted. Intimal hyperplasia, endothelial cell proliferation, media hypertrophy and local thrombus formation can be observed and one of the main pathomorphological characteristic features, plexiform lesion develops, leading to obliterative vasculopathy. A more severe form of the disease develops in systemic sclerosis, which is explained by the main pathophysiological elements of scleroderma, namely immunoregulatory disorder, vasculopathy and fibroblast dysfunction. It is not easy to monitor the disease in these cases, because the deterioration can be caused by many other factors as well. Therefore, beseides the usual examinations, biomarkers and screening methods have a significant role. Treatment is not simple either, since no wellapplicable algorithms are available. In many disorders (systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis), effective immunosuppressive therapy started in time is crucial, whereas in case of systemic sclerosis, the principles of therapy applied for the idiopathic form should be followed.]

[INTRODUCTION - Mixed connective tissue disease (MCTD) is an inflammatory autoimmune disease with multiple organ involvement. Immune- inflammatory processes play a crucial role in the pathogenesis of atherosclerosis. The connection between inflammatory parameters and atherosclerosis in MCTD has not yet been studied. Lipid abnormality is an important risk factor of atherosclerosis. Among the lipids, HDL is protective, which is in part due to the antioxidant effect of paraoxonase. In this paper, the lipid profiles and paraoxonase activities of MCTD patients were studied and the factors causing abnormalities were investigated. PATIENTS AND METHODS - Thirty-seven patients with MCTD, who had not taken any lipid lowering drugs in the past 2 months, were enrolled in the study. Thirty healthy individuals served as controls. At the time of the study the mean age of the MCTD patients was 51.2 ± 9.5 years, and the mean disease duration was 11.0 ± 7.2 years. Paraoxonase activity was determined by spectrophotometry, lipid profiles were determined by a Cobas Integra 700 Analyser, the von Willebrand factor antigen (vWFAg) was measured by turbidimetry in platelet-poor plasma and the thrombomodulin and anti-endothelial cell antibody (AECA) measurements were carried out by ELISA methods. RESULTS - Paraoxonase activity in the MCTD patients was lower than in the control population (118.5 ± 64.6 U/l vs. 188.0 ± 77.6, p<0.001). The arylesterase activity was also significantly lower in the patients (p<0.001). The reduction of paraoxonase activity was in correlation with the age of the patients, the duration of the disease and with vascular (eye, cardiac, cerebral) disorders. The total cholesterol and triglicerid levels of the patients were significantly increased compared to the control group, while in the apoA1 levels a significant reduction was seen. A very strong correlation was observed between the reduction of paraoxonase activity and the increase of endothelial cell activation markers (thrombomodulin, vWFAg, AECA). There was no difference in the values of patients with or without corticosteroid treatment. CONCLUSIONS - The results suggest that in MCTD there is an increased risk for atherosclerosis. Apart from an elevated cholesterol and triglicerid level, a reduced paraoxonase level and activity may also play a role in the development of atherosclerosis,. Therefore, in patients with MCTD, due to the increased oxidative processes and the impaired elimination of free radicals, a sustained damage to the endothelial cells occurs, which is indicated by increased levels of thrombomodulin, vWFAg, and anti-endothelial antibody.]

[INTRODUCTION - Large hiatal hernias may result in cardiac space-occupying lesions. Our case study discusses the clinical aspects and the possible diagnostic approaches for such large hiatal hernias that cause cardiac compression. CASE REPORT - In an elderly patient with no cardiovascular symptoms, transthoracic echocardiography revealed a left atrial space-occupying mass. The diagnosis regarding the origin of the cardial mass was clarified by a glass of soda water drunk by the patient during echocardiography, as it resulted in a decrease in the inhomogenous echodensity of the mass and the appearance of “contrast bubbles” within it. This phenomenon suggested the presence of a large hiatal hernia compressing the heart, which was confirmed by gastroscopy and upper gastrointestinal barium examination. CONCLUSION - Drinking soda water during transthoracic echocardiography can help to diagnose lesions that look like intracardial tumours.]

[INTRODUCTION - The functional and morphological changes of oesophagus occur in two third of mixed connective tissue disease patients according to the literature. CASE REPORT - We report three cases of 27, 39 and 48 year old women suffering of lateral pharyngoesophageal, epibronchial and epiphrenic diverticula associated with connective tissue disease. Diverticula had an average diameter of 3.8 cm (maximal diameters: 7.2-8 cm). The esophageal pouches produced dysphagia, dystonia, motility disorders, food stagnation and vomiting, retrosternal burning sensation and tachyarrythmia after 5-16 month's latency period. In one patient pneumoesophagus also evolved. The high serum enzyme levels and proximal electromyogram proved the presence of polymyositis. CONCLUSION - The localization, number and the size of esophageal diverticula are determined by the interstitial myopathy.]