Juvenile Localized Scleroderma (JLS) and Juvenile Systemic Sclerosis (JSSc) form a group of rare pediatric diseases that can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physician’s experience. Consequently, treatment regimens differ throughout Europe. In 2012, a European initiative called SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases.

Childhood-onset systemic lupus erythematosus (cSLE) represents 15-20% of all SLE cases and is in general associated with a more aggressive disease course and more rapid damage accrual than adult-onset SLE. Disease expression varies according to ethnicity, with more severe disease course in non-Caucasian ethnic groups. The majority of patients with cSLE develop damage within 5-10 years of disease onset, most frequently involving the musculoskeletal, ocular, renal and central nervous systems. Premature atherosclerosis and osteoporosis have become increasingly prevalent comorbidities in cSLE patients. Treatment of cSLE is challenging and is further complicated by an unpredictable disease course, adolescent noncompliance and long requirement for therapy. New therapeutic regimens combining immunosuppressive agents and targeted B-cell depletion often provide improved disease control and follow the oncologic model of remission induction and maintenance therapy. Management of children with SLE must include also prevention of medication side effects on growth, delayed puberty, development and fertility. Optimal management of an adolescent with SLE should take into account also patient’s quality of life, psychosocial development and organization of successful transition from pediatric to adult care. The antiphospholipid antibody syndrome (APS) is a multisystemic autoimmune disease characterized by thromboembolic events, pregnancy morbidity, hematologic, dermatologic, neurologic and other manifestations in the presence of elevated titers of antiphospholipid antibodies (aPL). APS may occur as an isolated clinical entity (primary APS) or in association with autoimmune diseases, infections and malignancies. Multiple pathogenic mechanisms have been proposed by which aPL may predispose to thrombosis including interaction between aPL and endothelial cells, platelets, monocytes, activation of the complement system, and interaction with the proteins involved in the regulation of the coagulation cascade. Management in all patients with APS include avoidance of additional risk factors for thrombosis. Patients with persistently positive aPL, in particular those with lupus anticoagulants (LA), have a high risk for recurrent thrombosis and should receive long-term anticoagulation with warfarin. The standard treatment in APS patients with venous or non-cerebral arterial thromboembolism consist of oral anticoagulation at a target INR of 2.0-3.0. However, it is essential to individualize treatment according to the presence of additional thrombophilic risk factors and the aPL profile (multiple aPL antibodies, high titers of aCL and/or anti-β2GPI, presence of LA). An improved understanding of the pathogenic mechanisms by which aPL induce thrombosis has suggested some innovative treatments such as new anticoagulant and antiplatelet drugs, hydroxychloroquine, statins, complement inhibitors, rituximab and other targeted therapies.

Childhood-onset systemic lupus erythematosus (cSLE) is a rare multisystem autoimmune disease that often leads to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physician’s experience. Consequently, treatment regimens differ throughout Europe. In 2012, a European initiative called SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases such as cSLE.

Summary Turos cheese belongs to the group of fresh, acidic, dried cheeses, fl avoured with dried red pepper and cone shaped, produced in Međumurje region of Croatia and Hungary by Croats from Pomurje. Th e goal of this paper was to investigate the production procedure, physico-chemical properties and microbiological qulity of Turos cheese, whose production takes place on the family farms and in small dairy plants. Due to skimming the sour cream during the production at family farms a signifi cant part of milk fat was removed that resulted in a lower content of milk fat in traditional Turos cheese (P<0.01) in comparison to the Turos cheese produced at small scale dairy plant. A signifi cantly higher salt content (P<0.001) and pH value (P<0.05) was found in Turos cheese produced at family farms. Th e investigations have shown a signifi cant diff erence in height (P<0.0001) and in weight (P<0.05) in favor of the cheeses produced in small scale dairy plants. Microbiological analysis showed that all the analyzed cheeses met the requirement of hygienic conditions. Further analyses have shown the presence of yeasts and molds in the cheeses.

Polyarteritis Nodosa (PAN) is a systemic, necrotizing vasculitis that affects medium-sized and small muscular arteries resulting in microaneurysm formation. In the presteroid era, mortality was high and the diagnosis was exclusively made postmortem.

macrophage activation syndrome (MAS) is life-threatening complication of rheumatic diseases and is most frequent seen in systemic juvenile idiopathic arthritis (sJIA). Prompt recognition and immediate therapy is life saving.

Kawasaki disease (KD) typically presents in children younger then 5y as a febrile illness with mucocutaneous changes. If untreated, KD can result in coronary aneurisms in 25% patients.

There is a critical need for population-based prospective cohort studies because they follow individuals before the onset of disease, allowing for studies that can identify biomarkers and disease-modifying effects and thereby contributing to systems epidemiology. This paper describes the design and baseline characteristics of an intensively examined subpopulation of the LifeLines cohort in the Netherlands. For this unique sub-cohort, LifeLines DEEP, additional blood (n=1387), exhaled air (n=1425), fecal samples (n=1248) and gastrointestinal health questionnaires (n=1176) were collected for analysis of the genome, epigenome, transcriptome, microbiome, metabolome and other biological levels. Here, we provide an overview of the different data layers in LifeLines DEEP and present baseline characteristics of the study population including food intake and quality of life. We also describe how the LifeLines DEEP cohort allows for the detailed investigation of genetic, genomic and metabolic variation on a wealth of phenotypic outcomes. Finally, we examine the determinants of gastrointestinal health, an area of particular interest to us that can be addressed by LifeLines DEEP.

Future smart distribution grids will apart from a large number of measurement instruments, communication infrastructure, intelligent software etc., also require the appropriate techniques for analysis of the available signals. Various disturbances of different intensities constantly occur in real distribution systems. Many of them are just temporary while others cause the tripping of protection devices and the suspension of electricity supply. For distribution network operators, timely identification and adequate analysis of disturbances represent a very important segment of operation of electricity distribution networks. In this paper, the disturbance registered in the real distribution system of Bosnia and Herzegovina is analysed using four different time-frequency analysis techniques (Short-Time Fourier Transform (STFT), Continuous Wavelet Transform (CWT), Wigner-Ville Distribution (WVD) and Hilbert-Huang Transform (HHT)). The results show that all the applied techniques successfully identified the disturbance which is reflected in changes in frequency during the observed time period. These techniques could be suitable to be applied as a part of power quality monitoring systems, which provide the required measurement signals. The utilization of these techniques can provide distribution system operators with additional, a very important information about the distribution system.