Interstitial lung disease (ILD) is a large group of diseases with a severe course and, as a rule, an unfavorable prognosis, since irreversible pulmonary fibrosis forms in the outcome of most ILD. Among ILD in children and adolescents, hypersensitivity pneumonitis (HP) is of significant clinical importance. The clinical observation presented in the article demonstrates the first successful experience in our country (2018) of transplanting both lungs to an adolescent with pulmonary fibrosis resulting from the HP chronic course. Lung transplantation has become the only way to prolong the patient's life.

COVID-19 infection usually occurs in children in a mild form, but some of them in a delayed period (one or several weeks after acute infection with COVID-19) may develop a severe inflammatory disease with clinical manifestations similar to toxic shock syndrome (Kawasaki disease), classified as multisystem inflammatory syndrome in children (MISC). It is possible that the syndrome has only a temporary connection with the COVID-19 infection. In the future, new associations of such clinical manifestations with other infectious (or non-infectious) diseases may appear. But currently, all children in the described cohorts with MISC have an association with COVID-19 infection. It is believed that the syndrome is initiated by an excessive adaptive immune response with the formation of autoantibodies. Treatment is based on anti-inflammatory, including steroid therapy, the possible use of intravenous immunoglobulin, aspirin, interleukin 1 and 6 receptor antagonists. The article analyzes current views on Kawasaki-multisystem inflammatory syndrome in children in the delayed period of COVID-19 coronavirus infection in the aspects of diagnosis, pathogenesis, clinical manifestations (with a discussion of foreign and Russian studies) and approaches to therapy and possible prevention, including the possibility of using plasmapheresis in complex therapy.

Приведена методика обследования больных с гипертрофической кардиомиопатией, основанная на исследовании радиальной посегментарной деформации миокарда в сопоставлении данных с толщиной миокардиального сегмента с целью определения критической толщины миокарда, при которой впервые в сегменте возникает систолическая миокардиальная дисфункция. Данный метод может быть применен для определения показаний к началу терапии у асимптоматичных больных с гипертрофической кардиомиопатией.

No abstract

Pulmonary hypertension (PH) in pediatrics is a polygenic multifactorial condition with extremely adverse prognosis. Selection of optimal management is a severe task. In absence of treatment the mean life duration in children is not higher one year. Last two decades, revolution in approaches to treatment improved the survival of this patients group. Recently, pediatricians and pediatric cardiologists have three drugs groups that act on the main pathogenetic chains of PH: endothelin pathway, nitric oxide pathway and prostacyclin pathway. At the moment, approaches to pediatric PH are based on the data obtained in the trials on adult patients. However, not long ago there were first randomized trials on children performed. The group of authors of current article presents a modern view on the problem of PH in children, and expert recommendations on children management. Class of recommendations and evidence level were set by the data obtained in pediatric population or on adult population with at least 10% of children included. To the strategy, developed by the Russian clinicians, laid the analysis of experience of the pathology treatment in Russian Federation, as the current practics and clinical guidelines on pediatric PH in Europe, and the recent trials published.