Сравнительная оценка эффективности базисных препаратов при лечениидетей и подростков с различными вариантами ювенильного идиопатического артрита

Objective. To specify the diagnosis of juvenile idiopathic arthritis (JIA) and to define the significance of pathogenetic changes, the authors have studied cellular and humoral immunological parameters, which are of the greatest informative value in this pathology: CD4+, CD4+/CD8+, CD8+, CD16+, CD95+ in the serum, as well as a spectrum of interleukins (IL): tumor necrosis factor-а, IL-6, IL-1, and IL-4. Subjects and methods. An optimal basic drug was selected for a specific form of JIA. Preference is given to cyclosporine and methorexate (MT) in its polyarticular form with the high activity of an inflammatory process, to MT, cyclosporine A, and auranofin in the disseminated form of oligoarthritis, and to auranofin and sulfasalazine in the persistent form. Results. None of the basic drugs is superior while individually choosing a treatment and this or that drug has to be very frequently chosen empiricallyЦель исследования. Для уточнения диагноза ювенильного идиопатического артрита (ЮИА) и определения значения патогенетических изменений исследовали показатели клеточного и гуморального иммунитета, наиболее информативные при данной патологии: CD4+, CD4+/CD8+, CD8+, CD16+, CD95+ в сыворотке крови, а также спектр интерлейкинов (ИЛ): ФНО а, ИЛ 6, ИЛ 1, ИЛ 4. Материал и методы. Для конкретной формы ЮИА подбирали оптимальный базисный препарат. При полиартикулярной форме с высокой степенью активности воспалительного процесса предпочтение отдавали циклоспорину и метотрексату (МТ); при распространяющемся варианте олигоартрита - МТ, циклоспорину А (ЦсА), ауранофину, при персистирующем варианте - ауранофину, сульфасалазину. Результаты исследования. Ни один из базисных препаратов не являлся исключительным при индивидуальном подборе лечения, и очень часто в ходе лечения приходилось эмпирически подбирать тот или иной препарат

Reoperations after the correction of partial abnormal venous return: a clinical case series

Rationale: The surgical procedure for partial abnormal pulmonary venous return (PAPV) implies the formation of a tunnel between the orifices of the right pulmonary veins and the left atrial cavity with the interatrial septum repair. To avoid any obstruction of the tunnel, a direct anastomosis between the superior vena cava (SVC) and the right atrial appendage is placed in a number of cases. However, the reparative procedure is potentially associated with such complications as SVC stenosis and/or pulmonary vein stenosis. Aim: To present our experience of reoperations for stenosis of systemic and pulmonary veins after primary PAPV repair. Materials and methods: From 2014 to 2018, seven patients (boys, 4) with SVC stenosis and/or with stenosis of the right pulmonary veins have been admitted to the FCHMT (Kaliningrad). Their mean bodyweight was 29.1 kg (± 12.5 kg), mean age 9.3 years (± 3.6 years). Three patients had an isolated SVC stenosis. In two patients, there was a combination of SVC stenosis and pulmonary vein obstruction (from stenosis to complete occlusion); in one patient the right pulmonary vein stenosis had been caused by thrombosis. SVC stenosis with subaortal obstruction after the repair of double-outlet right ventricle was diagnosed in one case. In six patients, various procedures for correction of SVC and/or pulmonary vein stenosis were performed. Only in one case the obstruction of systemic veins, confirmed by cardiac catheterization, turned out to be non-significant, making a reoperation unnecessary. Results: There were no deaths in this patient group. Endovascular procedures were performed in two patients. In one case, there was an unsuccessful attempt of transluminal balloon angioplasty for SVC stenosis, with subsequent Warden procedure. In another case, a 16-year old patient with SVC stenosis and high pulmonary hypertension was admitted after two previous surgeries had failed. We used a homograft as SVC prosthesis; however, after one year, a re-stenosis was identified. In this case, a SVC stent placement was successful. Due to sick sinus syndrome at one year after the Warden procedure and the repair of pulmonary veins by a homograft, an implantation of electric cardiac pacemaker was necessary in one patient. Conclusion: The curative repair of partial abnormal venous return is a well-established surgical technique with satisfactory shortand long-term results. A potential complication of the procedure is an obstruction of systemic and pulmonary veins. To prevent such complications during primary defect repair, as well as with reoperations for vein stenosis, the Warden procedure seems effective and highly reproducible technique associated with lower surgical risks

A 6-year experience of the Ross procedure in children with congenital aortic valve disease

Background: The choice of an optimal method for correction of aortic valve stenosis or insufficiency remains the matter of debate. Some clinicians prefer transluminal balloon angioplasty, some would perform an open type commissurotomy, or aortic valve replacement, or to valve leaflets repair with various materials, while others opt for the Ross procedure. Each of these techniques has its advantages and disadvantages both in the early postoperative period and in the long-term.Aim: To assess the results of the Ross procedure, its complications, survival, and probability of reoperations in the long-term.Materials and methods: We retrospectively analyzed the results of 32 Ross procedures performed from the end of 2012 to the beginning of 2019 in children aged from 3 days to 18 years. Eight children were below 1 year of age, including one newborn. A pulmonary autograft was placed into the aortic position in all children. In 31 children, a homograft was placed into the pulmonary artery. In one patient, an aortic autograft was implanted instead of the pulmonary artery valve. The mean patient age was 6.8 years, mean bodyweight 27.7 kg (range, 3.9–74.6 kg).Results: Two patients died in the early postoperative period (mortality 6.25%). We examined 26 patients at the follow-up, with its mean duration of 2.5 years. There were no indications for replacement of the homograft during the follow-up. One patient, in whom we had implanted an aortic autograft into the pulmonary position, required its replacement after 4.5 years. We have no data on death rates in the long-term.Conclusion: The Ross procedure provides satisfactory results in the early and intermediate postoperative period. Continuous follow-up is needed to determine the time points and the proportion of repeated interventions with a pulmonary homograft and aortic valve

COMPARATIVE EVALUATION OF THE EFFICIENCY OF BASIC DRUGS IN THE TREATMENT OF CHILDREN AND ADOLESCENTS WITH DIFFERENT TYPES OF JUVENILE IDIOPATHIC ARTHRITIS

Objective. To specify the diagnosis of juvenile idiopathic arthritis (JIA) and to define the significance of pathogenetic changes, the authors have studied cellular and humoral immunological parameters, which are of the greatest informative value in this pathology: CD4+, CD4+/CD8+, CD8+, CD16+, CD95+ in the serum, as well as a spectrum of interleukins (IL): tumor necrosis factor-а, IL-6, IL-1, and IL-4. Subjects and methods. An optimal basic drug was selected for a specific form of JIA. Preference is given to cyclosporine and methorexate (MT) in its polyarticular form with the high activity of an inflammatory process, to MT, cyclosporine A, and auranofin in the disseminated form of oligoarthritis, and to auranofin and sulfasalazine in the persistent form. Results. None of the basic drugs is superior while individually choosing a treatment and this or that drug has to be very frequently chosen empiricall

ОПЫТ АМБУЛАТОРНОЙ РАБОТЫ ЦЕНТРА ЛЕГОЧНОЙ ГИПЕРТЕНЗИИ В САМАРСКОЙ ОБЛАСТИ

Pulmonary arterial hypertension (PAH) is chronic and progressive disease associated with poor prognosis despite advanced therapeutic options. The choice of adequate treatment requires medical examination as a potential misdiagnosis, particularly in patients present with post-capillary pulmonary hypertension, may result in death. Such patients should be followed up by an experienced healthcare specialist.OutpatientCenterwill allow identifying target patients with early-stage disease or minimizing their risk of complications. The article presents the experience of Outpatient Centre for Pulmonary Hypertension at the Regional Cardiology Dispensary in the Samara region.Легочная артериальная гипертензия (ЛАГ) – хроническое, прогрессирующее заболевание, ассоциируемое с плохим прогнозом, несмотря на современные достижения в лечении. Для верного выбора терапевтической тактики необходимо выполнить полное диагностическое обследование больных с подозрением на наличие ЛАГ, поскольку ложный диагноз, особенно при наличии пост-капиллярной формы легочной гипертензии, может привести к тяжелым осложнениям с риском фатального исхода. Такие пациенты должны находиться под постоянным наблюдением специалиста, обладающего должными знаниями по данной нозологии. Организованная служба амбулаторной помощи позволит выявить пациента на ранних стадиях заболевания или максимально уменьшить риск осложнений. В статье представлен опыт амбулаторной работы Центра легочной гипертензии на базе областного кардиодиспансера в Самарской области