Children with inflammatory bowel disease already have an altered arterial pulse wave.

Adults with inflammatory bowel disease (IBD) have an increased risk for vascular events. This study aims to evaluate arterial parameters in paediatric IBD. Carotid intima-media thickness (CIMT) was measured by ultrasound, and Arteriograph was used to assess aortic pulse wave velocity (PWVao), brachial and aortic augmentation indexes (AixBrach, AixAo), central systolic blood pressure (SBPao), and heart rate (HR). A total of 161 children were included; 55 (34%) children with newly diagnosed IBD (median age 14.35 (11.88-16.31) years, 53% males), 53(33%) in remission (median age 15.62 (13.46-16.70) years, 66% males), and 53 (33%) controls (median age 14.09 (11.18-14.09) years, 55% males) were recruited into a case-control study. Compared to controls, patients with active disease and those in clinical remission had significantly lower AixBrach and AixAo (P < 0.001, P = 0.009; P < 0.001, P = 0.003). PWVao and CIMT were still normal. HR was higher in both IBD groups than in controls (P < 0.001; P = 0.006). HR positively correlated with disease duration (P = 0.001). In the ordinary least squares regression models, anti-tumour necrosis factor (TNF) α treatment predicted lower peripheral and central systolic blood pressures, in contrast to aminosalicylates and methotrexate. Aminosalicylate treatment predicted increased HR.  Conclusion: Children with IBD have an increased heart rate, a lower augmentation index and, therefore, an altered pulse waveform. In paediatric IBD, arterial stiffness and CIMT are still normal, indicating the potential for adequate IBD treatment to preserve arterial health. What is Known: • Adult patients with inflammatory bowel disease (IBD) have increased carotid intima-media thickness and arterial stiffness, which positively correlates with cardiovascular risk and predicts mortality. Adequate treatment, especially anti-tumour necrosis factor (TNF) α medications, lower these risks. • Children with IBD have impaired endothelial function and reduced heart rate (HR) variability. What is New: • Children with IBD have impaired endothelial function and reduced heart rate (HR) variability. • Anti-TNFα treatment in children and adolescents with IBD lowers systolic pressure, whereas methotrexate and aminosalicylates have the opposite effect. Amiynosalyiciylate treatment also increases HR.

[RIGHT VENTRICULAR ARRHYTMOGENIC CARDIOMYOPATHY ­ HAVE WE AVOIDED A FAMILY TRAGEDY BY APPLYING CONTEMPORARY DIAGNOSTIC AND TREATMENT APPROACH?].

Arrhytmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by fibrofatty replacement, dominantly in the right, but often also in the left ventricle. It is a significant cause of sudden death in children and adolescents. A thorough family history and contemporary diagnostic and treatment approach are prerequisites for the prevention of the sudden death syndrome. The diagnosis is more often established in adults than in children. Patients: We present a family with four members affected and diagnosed with ARVC already in childhood/adolescence. The average age of symptom presentation was 12 years (10­14 years). The importance of family tree isemphasized and contemporary diagnostic and treatment methods, with the aim of sudden death prevention, are described. Conclusions: Family history is essential for the early ARVC diagnosis. There is a need for revision of current diagnostic criteria in pediatric population. In the future progression of fibrous tissue proportion in relation to age and the stage of the disease should be taken in account. Furthermore, the repolarisation abnormality is inappropriate as a criterion in children less than 14 years old, so in that age group less of present diagnostic criteria can be applied. A combination of modern implantable cardioverter defibrillator and radiofrequency ablation assures an excellent life quality in our patients. However, the longterm prognosis for our patients, because of a progressive course of the disease, remains uncertain.