Background: Awareness of Kawasaki disease (KD) is emerging in Russia but the diagnosis is still often missed.Methods: This is a retrospective study of 303 children with KD who received care at a single center in Moscow over the period from 2004 to 2016.Results: Overall, coronary artery aneurysms were documented in 91 (30,0%) of 303 patients and transient ectasia in 40 (13,2%). Intracoronary thrombi were found in 12 of 15 patients with giant aneurysms and in 3 patients with medium-sized aneurysms.Conclusion: The patients with KD in the Moscow region had typical features of the disease described in the literature but the proportion of patients with coronary artery aneurysms was higher than reported from other countries. We assume that this is due to delayed treatment, which has gradually improved over time. Increased awareness of KD in Russia is critical to ensure timely diagnosis and treatment.

The purpose of this research is to study the characteristics of the change in the circadian rhythm of macrophage colony-stimulating factor (M-CSF) content in the peripheral blood serum of patients with stage II essential hypertension (EH) based on 5 time points (8:00, 14:00, 20:00, 2:00, and 8:00) and analyze its connection with the frequency of cardiovascular events. Materials and methods Identified levels of M-CSF in the peripheral blood serum of 60 patients with stage II EH, before and after 1 year of antihypertensive therapy using enzyme-linked immunoassays (at 8:00, 14:00, 20:00, 2:00, and 8:00). Results The research demonstrated that stage II EH patients with a medical case history lasting 10–14 years have a greater content of M-CSF in their peripheral blood serum (p > 0.001). Before the start of antihypertensive therapy, they also have an increased variability in the circadian rhythm of M-CSF content in the bloodstream (when compared with healthy individuals) due to an increase at 20:00, decrease at 2:00 and recovery at 8:00. In 70% of those patients taking antihypertensive medication and have reached their target arterial blood pressure, the cytokine decrease stabilizes at 2:00 but the increase at 20:00 remains unchanged. Thirty percent of patients retained the rhythm characteristics of M-CSF content in the blood serum typical of patients before the start of therapy. This is a predictor of an increase in the five-year risk of developing cardiovascular complications, particularly myocardial infarction and acute cerebrovascular accident, in individuals with a comparable risk of cardiovascular complications or death on the Framingham risk score.

BackgroundJuvenile localized scleroderma (JLS) is a chronic inflammatory fibrosing disorder with numerous disease subtypes, results in deep tissue atrophy, limited functional capacity. Treatment of jLS is still contraversal, ranges from topical skin management to immunosuppressive therapy (IST).ObjectivesTo estimate the resons for IST and its efficacy in JLS,MethodsWe detected antibodies to collagen I-IV types (AbC) (IFA, IMTEC); antinuclear antibodies (IFI, Orgentesc) anti dsDNA, anti-topoisomerase (SCL-70), anticentromere (ACA), antinuclear antibodies (ANA), rheumatoid factor (RF); serum level of gyaluronic acid (GA) (IFA, Corgenix), fibronectin (FN) (IFI, Technoclone) in 150 JLS, 35 juvenile systemic sclerosis (JSS) patients and 100 healthy control children.Efficacy of IST was estimated retrospectively in 385 JLS pts. by skin score, skin thickness (Durometr), joint immobility, laboratory tests.ResultsPenicillanine (PA) monotherapy 8-10 mg/rg/d for 3-4 years we used in 76 pts (40 - single plague morphea, 15 - generalized scleroderma (scld), 10 - “en coup de sabre” JLS, 11 - linear morphea. PA was effective in 98% of - single plague morphea, 20% of generalized and linear scld, 2% of “en coup de sabre” (ECDS) pts.PA 8-10 mg/kg/d for 3-5 years + Prednisone (Pr) 1mg/kg/day for 6-10 weeks, then taped and stopped in 12 mo. Were given to 135 JLS pts (46 - linear scld, 45 - hemitype scld, 44 - generalized scld, 30 - ECDS. PA+Pr was effective in 90% of linear, generalized and- hemitype scld pts, 48% ECDS pts.Methotrexate (MTX) monotherapy 10-15 mg/m2/wk for 2-5 years we used in 100 JLS pts (58 - linear scld, 25 - generalized scld, 17 - ECDS. MTX was benefit in 92% linear and generalized scld pts, 50% ECDS.MTX 10-15 mg/m2/wk for 2-5 years + Pr 1mg/kg/day for 8-10 weeks, then taped and stopped in 12 mo. We used in 75 JLS (35 hemitype scld pts, linear scld – 25 pts, 1 with ECDS). MTX +Pr was effective in 96% of hemitype & linear scld pts, in 60% ECDS.Average efficacy of IST for different JLS subtypes varied from 2 to 60% in ECDS scld, from 90% to 96% in hemitype & linear scld, decreasing in pts with disease duration more than 34 months. JLS pts received IST have stopped skin progression and musle atrophy, skin fibrosis and joint contractures have been partly reversed. In contrast to JLS pts (N=120) without IST, observed at our department in 1960-1980, who showed progression of fibrotic skin and joint damage in 70% and organ involvement in 5% of cases in 3 year follow up.ConclusionsOur study shows that autoantibodies and some markers of fibrosis are increased in JLS pts, that makes indroduction of IST proven in scld children without organ involvement, but rapid spread progression of skin,musle and joint desease. Indications for IST regimes for each scld subtype are not clear now. Our experience suggests that pts with single plaque morphea have good response to PA monotherapy, the possibility to avoide cytostatics is important. The most resistant is ECDS type.ReferencesZulian F, Martini G, Vallongo C, et al. Methotrexate in j...