Atypical haemolytic uremic syndrome (aHUS) is an extremely rare pathology with the development of complement-mediated thrombotic microangiopathy (TMA). Before eculizumab, a humanized IgG monoclonal antibody to the complement component C5, the prognosis of total and renal survival with aHUS was unfavourable due to the high probability of death and the development of end-stage chronic renal failure in surviving patients. This article presents a clinical observation of a patient with aHUS who had an identified heterozygous factor H (CFH) mutation — c.3653G>A(p.Cys1218Tyr), and two heterozygous variants of polymorphism in the same gene — c.2016A>G; c.2808G>T. Despite the achievement of haematological remission of TMA against the background of plasma therapy, the child developed dialysis-dependent renal failure. Treatment with eculizumab in a patient with chronic kidney damage provided a significant improvement in their function, maintaining a stable remission and improving the quality of life of the patient with aHUS.

Kh. М. Emirova, Е. S. Stolyarevich take part in educational events for doctors as lecturers with the support of Alexion Pharma. The other contributors confirmed the absence of a reportable conflict of interest.

Dear friends, colleagues!

The article presents updated data on the problem of premature ventricular contractions in children based on the clinical guidelines of the Russian Society of Cardiology and the Union of Pediatricians of Russia for the diagnosis, treatment and management of pediatric patients with premature ventricular contraction. The issues of diagnosis and treatment based on the principles of evidence-based medicine as well as important aspects of prevention of exacerbations and follow-up have been clarified in detail. The criteria for assessing the quality of care for patients with premature ventricular contractions have been presented.

CONFLICT OF INTEREST. Not declared.

Background. In recent years, the opinion has been established that skin manifestations of psoriasis are associated with the damage of other organs and systems, and that psoriatic lesions have a systemic nature, and therefore the term ‘psoriatic disease’ is increasingly used in scientific literature. Among psoriasis comorbidities, metabolic disorders and cardiovascular diseases are of particular clinical importance. Our aim was to study the effect of systemic cytostatic therapy with methotrexate on the remodelling processes of large vessels in children with psoriasis who have normal and increased body mass index. Methods. The primary data was collected by extracting data from medical records and case follow-up records. The subsequent accumulation, storage, grouping and primary sorting of research data were carried out using a personal computer and Excel application package. Clinical evaluation of the severity of psoriasis and skin lesions was assessed by calculating the PASI index. Non-invasive oscillometric arteriography was performed on a portable ArterioGraph (TensioMed Ltd, Hungary). Results. A direct significant correlation has been established between the revealed impairments of the function of large arteries and the indicators of fat metabolism, the severity of psoriasis and body mass index. During the systemic pathogenetic cytostatic therapy with methotrexate, a significant positive dynamics of the function parameters of large arteries was revealed in children with psoriasis who have both normal and increased body mass index. Conclusion. The undertaken studies showed that methotrexate is an effective and safe agent for treating psoriasis in children aged from 3 to 18 years, has a positive effect on the function of large vessels.

Nikolay N. Murashkin — receiving research funding from pharmaceutical companies Jansen, Eli Lilly. Receiving fees for scientific advice from Galderma, Pierre Fabre, Bayer, Astellas, Libriderm.

Alexander I. Materikin — receiving research funding from pharmaceutical company Eli Lilly. Receiving fees for scientific advice from Bioderma, Libriderm.

Background. Therapy of psoriasis in children is an urgent problem of dermatology and paediatrics. The present-day knowledge of the disease pathogenesis defines the focus of therapeutic interventions causing rapid relief of disease symptoms, prevention of complications and the achievement of a complete skin resurfacing from psoriatic lesions as well as long-term retention of the effect. These requirements are largely met by therapy with genetically engineered biological drugs. Our aim was to study the clinical efficacy and safety of treating children suffering from moderate and severe psoriasis with genetically engineered biological drugs. Patients and Methods. The study included children with moderate and severe psoriasis. Four groups were formed: in the first and second groups, the children were treated with ustekinumab and etanercept, respectively, without prior methotrexate, in the third group — with ustekinumab after methotrexate, in the fourth group — with etanercept after methotrexate. The efficacy of therapy with ustekinumab and etanercept was evaluated using the PASI and CDLQI indices, their impact on the quality of life of patients was studied, the adverse events were registered. Results. The study involved 98 children aged from 6 to 17 years: 23 children in the first group, 26 — in the second group, 24 — in the third group, and 25 — in the fourth group. The arithmetic mean of the PASI index during the initial examination ranged from 23.7 ± 1.27 in patients of the second group to 31.9 ± 1.68 in the first group. The minimum value of the index was 10.5, the maximum one — 58.5. The CDLQI index at the first visit ranged from 14.8 ± 0.95 in patients of the fourth group to 17.0 ± 0.99 in the first group, which showed no statistically significant differences. Conclusion. Genetically engineered biological therapy with ustekinumab and etanercept is an effective and safe method for treating moderate and severe psoriasis in childhood, but ustekinumab compared to etanercept leads to a more pronounced decrease in the PASI index.

Nikolay N. Murashkin — receiving research funding from pharmaceutical companies Jansen, Eli Lilly. Receiving fees for scientific advice from Galderma, Pierre Fabre, Bayer, Astellas, Libriderm.

Eduard T. Ambarchian — receiving research funding from pharmaceutical company Eli Lilly. Receiving fees for scientific advice from Jansen, Libriderm.

Background. Currently, scleroderma is a rather rare disease, including among children. Despite the growing interest of investigators in this pathology, the problem of diagnosing oesophageal affection in various forms of systemic sclerosis remains under-investigated since endoscopic data does not provide a complete picture of the oesophageal function. Currently, mano-impedancemetry (MIP) is the leading method in Europe for determining the oesophageal function, including in children with scleroderma; in the Russian Federation, it is used only in adults. Proper and timely evaluation of the oesophageal motility state will allow not only to expand our understanding of the disease pathogenesis but also to determine the efficacy of the initiated therapy and to give practice suggestions in terms of treatment tactics in the early stages of the disease. Our aim was to determine the possibilities of mano-impedancemetry for diagnosing oesophageal affection in juvenile scleroderma. Patients and Methods. The study included children with juvenile scleroderma taken to the department of rheumatology and dermatology from June to August 2016. To identify oesophageal function disorders, all patients underwent high-resolution manometry in combination with impedancemetry. Results. A decrease in the distal oesophageal contraction amplitude in more than 30% of “wet” swallows was found in 2 of 7 children with juvenile systemic scleroderma and in one of 6 children with juvenile focal scleroderma. All 13 patients had peristaltic waves in the distal oesophagus with an amplitude of <30 mm Hg. They had interrupted peristalsis, in which the peristaltic wave did not go through the entire length of the oesophagus; one of these patients showed a simultaneous contraction with an amplitude of <30 mm Hg. All children (n=13) showed a positive correlation (r= 0.021) between the systemic disease and impaired motility (р=0.021). Conclusion. Despite the clinical and laboratory methods for diagnosing scleroderma, the data of oesophageal mano-impedancemetry is an important criterion, primarily for the differential diagnosis of systemic and focal scleroderma, and assists in the early initiation of therapy for oesophageal motility disorders.

Marina Ju. Stepanyan, Ekaterina I. Alexeeva, Elena V. Komarova confirmed the absence of a reportable conflict of interest.

Ekaterina I. Alexeeva — receiving research grants from Pfizer, Roche, Centocor, Novartis.

Nikolay N. Murashkin — receiving research funding from pharmaceutical companies Jansen, Eli Lilly. Receiving fees for scientific advice from Galderma, Pierre Fabre, Bayer, Astellas, Libriderm.

Resolution of the council of experts “Papillomavirus infection: the review of accumulated experience in the decision of multidisciplinary problem”.