Репозиторий Университета

© 2018 Publishing House of the Union of Pediatricians. All rights reserved. Background. Tumour necrosis factor alpha inhibitors are widely used in the treatment of juvenile idiopathic arthritis (JIA). To achieve maximum efficiency of genetically engineered biologic drugs, it is necessary to study predictors of the response to therapy. Objective. Our aim was to identify early predictors of the response to adalimumab therapy in patients with JIA without systemic manifestations. Methods. A prospective cohort study analysed treatment results of patients with JIA without systemic manifestations, who were prescribed adalimumab for the period from August 2008 to August 2014. We studied the relationship between baseline demographic indicators as well as baseline and registered after one month of treatment clinical and laboratory parameters and the best (remission according to the Wallace criteria) response to therapy after one year. Results. In the first year of therapy, 94 (43.9%) of 214 patients achieved remission according to the Wallace criteria. In a multivariate analysis, predictors of achieving remission after one year of adalimumab therapy were the improvement according to the ACR70 criterion after one month of therapy [odds ratio (OR) 3.3; 95% confidence interval (CI) 1.7-6.7], a history of uveitis (OR 1.86; 95% CI 1.03-3.33), a decrease in the number of joints with active arthritis after one month of therapy (OR 1.09; 95% CI 1.02-1.16). During therapy, injection reactions in the form of pain were observed in 36 (16.8%) of 214 patients, infectious diseases of ENT organs - in 85 (39.7%), of the respiratory tract - in 17 (7.9%), and tubinfection - in 13 (6.1%) children. Conclusion. The presence of uveitis, rapid reduction in the number of joints with active arthritis and a high level of response to treatment after one month of adalimumab therapy are predictors for achieving remission during the first year of treatment.

© 2018, Dynasty Publishing House. All rights reserved. Chronic pancreatitis, being a complex multifactor clinico-genetic phenomenon, is a significant medical, social and economic problem. Study of pancreatitis remains a topical theme also for the paediatric cohort, especially taking into account practically similar morbidity rates in adults and children. The review presents information and analyzes main genetic risk factors of the development of pancreatitis. Mutations and polymorphisms of the CFTR, CTRC, SPINK1, CPA1, PRSS1, PRSS2 genes that are significant for functioning of the pancreas are discussed in detail. Examples of genetic variants associated with increased or decreased risks of developing disease are given. Special attention is paid to difficulties of interpreting the results of molecular-genetic testing associated with certain gene homology, presence of pseudogenes and disease cases conditioned by spontaneous mutation. Detection of genetic risk factors of chronic pancreatitis allows to early prevent the development of disease in the proband’s relatives, and also to take a more personalized approach to the patient’s treatment.

© 2018 Publishing House of the Union of Pediatricians. All rights reserved. Background. To assign genetically engineered biologic drugs, we need data on the predictors for response to therapy. Prognostic factors for the response to tocilizumab in patients with juvenile idiopathic arthritis (JIA) without systemic symptoms are poorly studied. Objective. Our aim was to reveal early predictors for the response to tocilizumab therapy in patients with JIA without systemic symptoms. Methods. A retrospective cohort study enrolled patients with JIA without systemic symptoms who received tocilizumab therapy between July 2009 and August 2017. We assessed the association between the initial demographic, clinical, and laboratory parameters in patients and the best response (according to the ACR90 criteria) to treatment after a year. Results. The study included 95 (girls 85%) patients; the mean age was 10.3 (6.0; 13.6). During the first year of therapy, 71 (75%), 55 (58%), 38 (40%), and 22 (23%) patients achieved the improvement according to ACR30/50/70/90 criteria, respectively; 22 (23%) patients reached disease inactive stage according to the Wallace criteria. When performing multivariate analysis, the following improvement predictors were revealed based on the ACR90 criteria after a year of treatment: decrease in serum C-reactive protein level during the first month of therapy [odds ratio (OR) 1.024; 95% confidence interval (CI) 1.007-1.051], decrease in disease activity score on the visual analogue scale according to the parent/patient assessment (OR 1.048; 95% CI 1.005-1.105), early onset of the disease (OR 0.38; 95% CI 0.16-0.72), persistent oligoarthritis according to the ILAR (OR 9.9; 95% CI 1.5-109.3). During the first year of tocilizumab administration, neutropenia was registered in one patient, leukopenia - in three cases, and urticaria - in one case. Conclusion. The variant of JIA, the age at the disease onset, and the disease course pattern in the first month of tocilizumab therapy are the predictors of treatment efficacy throughout the year.

© 2018 Russian Transplant Society. All Rights Reserved. Lung transplantation (LT) for idiopathic pulmonary arterial hypertension (IPAH) now is the only radical treatment of this disease. Aim: to analyze own experience of performing LT in patients with IPAH. Materials and methods. 8 adult IPAH patients, who underwent LT between 2014 and october 2018, were included. In 7 of 8 patients undergoing bilateral lung transplantation on intraoperative venoarterial extracorporeal membrane oxygenation (VA ECMO) with prolongation into the postoperative period. Results. VA ECMO support was prolonged into postoperative period 6 and 7 days respectively in 2 (25,0%) patients and 3 days in 6 (75,0%) patients. Hospital mortality in IPAH patients was 1. Conclusions. Own experience demonstrates that LT is an effective method of treatment in patients with IPAH. Hospital, 1- and 3-year survival rates for the patient collective were 87.5, 75.0 and 75.0% respectively.