In October 2014 in Moscow at the XIII Russian Congress “Innovative Technologies in Pediatrics and Pediatric Surgery” was adopted the first part of the Russian recommendations “Congenital and multifactorial hereditary connective tissue disorders in children. Diagnostic algorithms. Tactics of treatment”. Multifactorial connective tissue dysplasia have a high prevalence in the population. We present the second part of the draft Guidelines dealing with multiple organ disorders in the connective tissue dysplasia. Despite the high level of modern molecular techniques, clarification of their nosology remains a distant prospect. Figuring out the incidence of connective tissue dysplasia hindered by the lack of common terminology, standardized diagnostic criteria, as well as the practical inaccessibility of modern molecular genetic techniques to identify the disease. In the first part of the Guidelines we could not find a place for all aspects of this complex issue, which bears an interdisciplinary approach. Later it was planned to develop recommendations for doctors of various specialties. During writing the second part it was taken into account the specialists and research teams from St Petersburg, Moscow, Tver, Omsk, Novosibirsk, Ivanovo, Chelyabinsk, Izhevsk, Orenburg, Smolensk, Petrozavodsk, Nalchik, Barnaul, Saratov, Rostov-on-Don, Voronezh, Stavropol, Yaroslavl. The core of the group works in active collaboration since 2008. The draft of the second part of the recommendation characterized especially multifactorial connective tissue dysplasia in infants, multiple organ disorders of the cardiovascular, respiratory, urinary system, gastrointestinal tract, hemostasis, nervous, musculoskeletal, upper respiratory tract and maxillodental apparatus. It sets out the course and tactics of various diseases with concurrent connective tissue dysplasia.
The paper presents the clinical and epidemiological characteristics of parvovirus infection (B-19) in Kyrgyzstan at the present stage. The main epidemiological factors identified in the process of collecting anamnesis are considered. The most typical clinical manifestations for this infection were also identified
BACKGROUND. Chronic kidney disease (CKD) is a common pathology influencing mortality risk in the world population. Calcification of aorta and heart structures (valves, coronary arteries) is a risk factor for cardiovascular complications. The influence of cytokines, integrin proinflammatory indices, acute phase proteins and other inflammatory factors on the risk of extravasal calcification is promising. THE AIM: to study the effect of cytokines, integrative proinflammatory indices, acute phase proteins and other inflammatory factors on the risk of extra-osseous calcification. PATIENTS AND METHODS. A one-stage, cohort study of 85 patients with CKD 5D treated with programmed hemodialysis was conducted. General clinical examination was carried out according to the protocol. Blood levels of C-reactive protein (CRP) were determined by immunoturbodimetry. A Glasgow Prognostic Score (GPS) risk index for systemic inflammation was calculated using CRP and plasma albumin concentrations. Interleukin-6 (IL-6), interleukin-3 (IL-3) were assessed by enzyme immunoassay. Blood leukocyte shift index (BLI) was calculated. Echocardioscopy was performed using Doppler mode. The presence of cardiac valve calcification (CAC) was registered, its severity was assessed. To estimate the abdominal aortic calcification, the abdominal radiography was carried out in the left lateral projection. The severity of manifestations of aortic calcification was assessed using the L.I. Kauppilla Calcification Scale. Statistical analysis was performed using STATISTICA 12.6. toolkit (StatSoft, USA). RESULTS. Systemic inflammatory factors negatively affected the severity of cardiovascular calcification. An increased GPS value was found to correlate with the severity of CAC and CSA. In the case of calcification severity analysis considering IL-3 and IL-6 values, it was also shown that high levels of these pro-inflammatory cytokines are associated with severe manifestations of anterior aortic wall calcification and aortic calcification at the L3 level. Inclusion of ISLC in the analysis had no effect on the severity of calcification of the aortic wall and no effect on the intensity of cardiac valve calcification in general, the aortic valve and the mitral valve in particular.
BACKGROUND. Currently, there is no doubt that there is a close relationship between the condition of the kidneys and the cardiovascular system. Neurohormonal systems various parts long-term hyperactivation is important in the development of pathological processes. Natriuretic peptides provide cardio and renoprotective effects, inhibit the activity of the renin-angiotensin-aldosterone system (RAAS). AIM. To establish the prognostic significance of determining the concentration of natriuretic peptides in comparison with the activity of RAAS in children with congenital malformations of the urinary system to assess the risk of cardiovascular complications.PATIENTS AND METHODS. 76 patients with congenital malformations OUS aged 3 to 18 years were examined: – 40 children with congenital vesicoureteral reflux, – 18 children with hydronephrosis, 18 children with other forms of OUS dysembryogenesis ( 10 patients with renal agenesis, 8-with hypoplasia). The control group consisted of 10 clinically healthy children of the appropriate age. The following parameters were determined in the blood by the enzyme immunoassay: renin, aldosterone, n-terminal propeptide of natriuretic hormone (NТ-рroВNР).RESULTS. Hyperproduction of NТ-рroВNР was observed in 61.8 % of patients, stimulation of renin and/or aldosterone production in 39.5 % of cases. Elevated levels of natriuretic peptides were found in 32.8 % of cases in 25 children with congenital OUS defects without signs of arterial hypertension (AH) and left ventricular hypertrophy (LVH). Significantly higher concentrations of (NТ-рroВNР), renin, and aldosterone are recorded in patients with AH and LVH.CONCLUSION. The increased level of natriuretic peptides is noted earlier than the clinical and instrumental signs of heart dysfunction are noticeable, it is important in identifying early stages of cardiovascular complications, evaluating the prognosis, and in justifying renoprotective treatment in pediatric Nephrology practice.
BACKGROUND. The role of inflammation and uremic intoxication in the development and progression of bone mineral disorders, including cardiovascular calcification, has been actively studied over the past decades. PATIENTS AND METHODS. A single-stage, cohort study of 85 patients with stage 5D CKD treated with programmatic hemodialysis was conducted. The blood concentrations of interleukin-3 (IL-3) and interleukin-6 (IL-6) were determined using the enzyme immunoassay, the level of fibrinogen - using the Rutberg method, and the level of p2-microglobulins - using the nephelometric method. The blood leukocyte shift index (ISLC) and the Glasgow Prognostic Score (GPS) risk index for systemic inflammation were also calculated, taking into account the level of C-reactive protein (CRP) and blood albumin. The presence of valvular calcification, its severity, and calcification of the abdominal aortic wall was recorded. Statistical analysis was performed using the program STATISTICA 12.6 ("StatSoft", USA). THE AIM: to evaluate the relationship between factors of systemic inflammation and cardiovascular calcification in patients with stage 5D chronic kidney disease. RESULTS. The risk of detecting calcification of the aorta and heart valves was influenced by the pro-inflammatory cytokines IL-3 and IL-6, as well as ISLK and GPS. However, inflammatory factors such as fibrinogen, p2-microglobulin, and CRP levels in the blood did not show a statistically significant effect. In the case when the predicted parameter was chosen not friendly calcification, but the presence of any of its components, the predictive significance of IL-3 decreased, but IL-6 remained. The 20% risk threshold was exceeded at IL-6 values of more than 33 pg/ml. The effect of ISLC on the probability of detection of calcification was shown both about friendly calcification and concerning isolated calcification of the aorta or valves. CONCLUSION. It was found that among the studied factors of inflammation, IL-6, ILK, and IL-3 demonstrate a relationship with the processes of cardiovascular calcification, GPS-only in relation to friendly calcification. Nomograms have been developed that allow predicting the detection of cardiovascular calcification in dialysis patients, depending on the state of the inflammatory circuit.
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