[Various phenotypes of postpartum atypical hemolytic uremic syndrome: the role of genetic testing in determining prognosis. Case report].

We report a case of atypical hemolytic uremic syndrome (aHUS) that occurred after childbirth in a patient with a history of numerous recurrent episodes of TMA with nephrotic proteinuria and impaired renal function. At 33 weeks of the first spontaneous pregnancy, proteinuria up to 0.8 g/l was first registered, at 38 weeks she was hospitalized with proteinuria, reaching a maximum of 13 g/l, she was delivered promptly, after which progressive thrombocytopenia was noted over the next few days (up to 44×109/l) and anemia and severe arterial hypertension, which could not be corrected by several groups of antihypertensive drugs. Initiated plasma therapy had no effect. After exclusion of all other causes of TMA, therapy with eculizumab was initiated, which made it possible to quickly and completely stop the phenomena of TMA. The presented observation demonstrates the successful treatment of recurrent course of aHUS with eculizumab with the achievement of complete recovery of kidney function in a patient with a homozygous mutation in the MCP gene. It is worth noting the importance of genetic research even in those situations where clinically aHUS is beyond doubt.

[INFLAMMATION AND CARDIAC INSUFFICIENCY ASSOCIATED WITH INFECTIOUS ENDOCARDITIS].

The study included 62 patients with uncomplicated primary and secondary infectious endocarditis admitted to S.PBotkin city hospital from 2011 to 2014. The emphasis is laid on diagnostic significance of dynamic measurements of the levels of C-reactive protein, tumour necrosis factor and highly sensitive troponin-1 for the evaluation of activity of the infectious/toxic process, severity of the disease, and detection of complications. The study revealed the relationship of the enhanced level of troponin-1 with changes of inflammation markers, morphofunctional characteristics of myocardium, and circulatory failure. Morphologicl study demonstrated inflammatory and dystrophic changes in myocardium, focal and diffuse cardiofibrosis suggesting development of non-coronarogenic myocardial lesions that play an important role in the progress of cardiac failure associated with infectious endocarditis.

Umbilical cord blood for autologous transfusion in the early postnatal ontogeny: analysis of cell composition and viability during long-term culturing.

Changes in cell composition and viability as well as the content and functional activity of hemopoietic progenitor cells were analyzed during long-term (up to 1 month at 4°C) storage of human umbilical cord blood cells. No significant quantitative changes in erythrocytes were found during this period. The total content and viability of leukocytes changed, which resulted in the prevalence of mononuclear cells (lymphocytes and monocytes). Analysis of functional activity of hemopoietic stem cells in semisolid culture revealed a decrease in the relative content of CFU during the first week of storage [corrected] and inability of cells to colony formation after 2 weeks.

[Clinical and morphological aspects of infectious endocarditis].

Comparative analysis of anamnestic, clinical, laboratory and instrumental data involved 106 patients with infectious endocarditis treated in S.P. Botkin City Clinical Hospital in 2000-2011 and 92 ones admitted in 1985-1977. The results give evidence of ongoing pathomorphosis of infectious endocarditis as is apparent from the growing number of male patients, increased frequency of primary cases and previous invasive or intravascular manipulations, thromboembolic complications including pulmonary thromboembolism, right heart chamber endocarditis. Over half of the patients are socially unadapted C-peptide level is of diagnostic value for the assessment of inflammation activity, precursors of natriuretic peptide can be used to detect preclinical and clinical stages of cardiac failure related to infectious endocarditis. There is correlation between severity of inflammation and myocardial dysfunction. Early surgical intervention in the absence of effect of combined antibacterial therapy improves prognosis. Morphological studies of inflammation-related changes in myocardium, destruction of cardiomyocytes, dystrophic processes, and fibrosis play an important role in the development of cardiac insufficiency and prognosis of infectious endocarditis.

[Role of the genetic factors, detoxication systems and oxidative stress in the pathogenesis of endometriosis and infertility (review)].

The aim of this paper is to provide a systematic review of the role of the genetic factors, detoxication systems and oxidative stress in the pathogenesis of endometriosis and infertility. Endometriosis and infertility are still both the most uncommon diseases in gynecology. Many aspects of female reproductive function are strongly influenced by genetic factors, and numerous studies have attempted to identify susceptibility genes for disorders affecting female fertility such as polycystic ovary syndrome, endometriosis, fibroids, cancer (ovarian, vulvar, cervical), premature ovarian failure, recurrent pregnancy loss and pre-eclampsia. The most solid evidence linking specific polymorphisms to endometriosis is showed by the studies investigating a phase II detoxification enzyme. No data were found concerning influences of the genetic factors on the female infertility. Contrary, a lot of studies devoted to the genetic factors of male infertility are presented. It's known that endometriosis associated with increased systemic oxidative stress. The implication of increased systemic oxidative stress in disease progression or the association with other oxidative stress-related pathologic conditions needs to be addressed in further studies. The majority of studies suggest a reduced antioxidant capacity in infertile wome with endometriosis. In the present review we discussed the role of the genetic factors in the pathogenesis of endometriosis and infertility. NAT2 polimorphism, xenobiotic methabolism and exogenous factors are somehow related with these diseases. An altered balance between pro-oxidant antioxidant activities may have an impact on folliculogenesis and adequate embryo development.

[Injuries to myocardium in infectious endocarditis].

Clinical and morphological characteristics of myocardium in infectious endocarditis (IE) are described as exemplified by pathological changes in the vascular bed, stroma, and myofibrils. Multiple sclerotic changes in myocardium in the form of "sheath sclerosis" were revealed. It is shown that progress of cardiac insufficiency in IE may be due to specific changes in the heart muscle. The necessity of early diagnostics of myocardial lesions is discussed with reference to revision of antibiotic therapy and modern surgical treatment of lE.

[With long-term conservative treatment of infectious endocarditis after aortic valve replacement].

This clinical study revealed peculiarities of early and late infectious endocarditis (IE) of artificial aortic valves. Possible sources of bacteremia, pathogenic agents and clinical picture of this pathology, methods of its early and differential diagnostics are described. High efficacy of combined antibiotic therapy is demonstrated in case of impracticability of surgical treatment.

Experimental study of corrective effects of human umbilical blood nuclears under conditions of a posthemorrhagic state.

Intravenous transfusion of nucleated cells from human umbilical cord blood (10(6)/ml saline) to female rats 3 h after massive blood loss produced a pronounced corrective effect (day 5): alleviates posthemorrhagic changes and normalizes hemoconcentration parameters, metabolic disorders, and behavioral reactions.

[Diagnostics of inflammation activity in infectious endocarditis].

This work was designed to analyse dynamics of inflammation markers (C-reactive protein, TNF-alpha) and procalcitonin in 66 patients with infectious endocarditis (IE) followed up during 6 months. Comparison of clinical observations and laboratory data revealed specific trends in these parameters in patients with different clinical course of IE and at its different stages. Some of the changes are shown to be responsible for the unfavourable prognosis of IE. TNF-alpha levels are considered to be a sensitive and informative index of the patients" clinical conditions throughout the entire period of IE evolvement.

[Analysis of transfusion related acute lung injuries after donor plasma transfusion in obstetrics].

The article presents a clinical investigation of two incidents of Transfusion Related Acute Lung Injury (TRALI) which have occurred after the fresh frozen plasma (FFP) transfusion in puerperant. According to evidence, when in timely diagnosed, the TRALI treatment is not complicated and lies in symptomatic lung oedema treatment. The incidence of TRALI in V.I. Kulakov Research Center of Obstetrics, Gynaecology, and Perinatology is estimated as 1 event out of the 699.5 transfused FFP doses per year. Among of the most promising methods of reducing the risk of TRALI and other transfusion related complications are: reduction of donor blood transfusion, introduction of autohaemodonorship and education of physicians working in obstetrics.

[Specific features of the clincal course of infectious endocarditis in a multi-field hospital].

The clinical course of infectious endocardities (IE) was followed in 112 patients admitted to the Botkin City Hospital in 2000-2007 with reference to the age and social structure of the pathology, its clinical symptoms, results of laboratory and instrumental analysis, strategy of antibacterial therapy, and outcome of the disease. The data were compared with those obtained in the previous period (1987-1955). He study demonstrated an increased number of men with IE (up to 65%) in the recent years and an almost two-fold rise in the incidence of primary endocardities. The frequency of thromboembolic complications also increased (from 34 to 69%) involving the pulmonary artery system (24%). The same refers to the number of patients with multivalvular lesions (2, 3 or 4 heart valves) and affected right cardiac chambers (28.4%). Shortcomings at various stages of the treatment of patients with endocarditis have been revealed.

[Current technologies for blood loss prevention during abdominal hysterectomy].

The paper deals with the development and use of original adequate transfusion-infusion programs to compensate for blood loss in abdominal hysterectomies for uterine myomas of varying sizes, by applying the current blood-supplying technologies that permit avoidance of transfusion of donor blood components. Four blood loss-replenishing programs in abdominal hysterectomy for large-sized myomas were studied for impact on the basic functions in premenopasal women. These included: (1) preoperative blood storage of blood autocomponents; (2) normovolemic hemodilution with hydroxyethyl starch (HES) solution being used as a dilutant; (3) administration of HES solutions as an independent solution compensating for blood loss, and (4) intraoperative reinfusion of autoerythrocytes. The proposed transfusion-infusion programs in abdominal hysterectomy for large-sized myomas by applying blood-preserving methods are effective in avoiding the use of donor blood components and accordingly in reducing the frequency of complications associated with transfusions of donor blood components, and improving the postoperative period.

[Angiotensin converting enzyme activity in the clinical course and forming of cor pulmonale in patients with chronic obstructive pulmonary diseases].

The aim of the study was to evaluate the influence of ACE in various biological media (blood, sputum, and lung tissue) on the clinical manifestations and morphofunctional cardiac variables in 137 patients with chronic obstructive pulmonary disease (COPD), of whom 17% had mild, 70% had medium, and 13% had severe degree of the disease according to Federal program, 1999. Seventy percent of the patients had stage II pulmonary hypertension according to Paleyev, 1986, while the number of patients with stage I and III was 14 and 16%, respectively. Circulatory insufficiency was mild (NYHA I to II) in 124 patients, and medium (NYHA III) in 13 patients only. The results of the study demonstrate that COPD exacerbation is accompanied by an increase in ACE activity, mostly in the lung tissue, induced sputum (IS), and, to a lesser degree, in blood serum; ISA CE activity is almost the same as that in the lung tissue. The study established a direct correlation between IS ACE activity and the level of C-reactive protein, an inflammatory process activity marker, and a reverse correlation between IS ACE activity and respiratory function variables, the latter characterizing bronchial obstruction. The study shows a strong direct correlation between IS ACE activity and the degree of right ventricular (RV) and left ventricular (LV) hypertrophy, as well as diastolic function disturbances according to RV isovolumetric relaxation time and LV early diastolic filling delay time. There is a strong reverse correlation between IS ACE activity and the disbalance of the ratio of blood flow velocities during early and late diastolic ventricular filling. The study found no significant correlations between ACE activity and the variables of ventricular systolic function. The study demonstrates a significant role of ACE activity changes in the progression of obstruction, inflammation, and myocardial remodeling.

[Potential of antiinflammatory therapy in patients with chronic obstructive lung disease].

The aim of the study was to investigate the effects of erespal (fenspirid) on the clinical manifestations and parameters of inflammation in patients with chronic obstructive lung disease (COLD). The two stages of the study included 3-weak treatment during exacerbation periods and a 3-month outpatient follow-up during clinical remission periods. Erespal was administered twice a day in a total dose of 160 mg/day. The study included evaluation of clinical symptoms, respiratory function, bronchoscopic data, laboratory indices of inflammation, antioxydative status, life quality (LQ), as well as cytological and cytochemical analysis of induced sputum (IS). During exacerbation periods of COLD the researchers observed early and noticeable antitussive and mucolytic action of erespal, which were associated with lessening of bronchial obstruction and inflammation, significant fall of C-reactive protein level, cytosis in IS, proportion of neutrophiles (p < 0.01), elevation of lysosomal cation proteins in neutrophiles (p < 0.01), increase of total oxidant serum level (in 46.2% of the patients vs. 26.3% of the patients in the conventional therapy group). Long term (3 months) treatment with erespal led to further positive dynamics of clinical and laboratory indices of inflammation, and improved the results of cytological and cytochemical study of IS. The results of the study demonstrate that inclusion of erespal in complex therapy of COLD increases efficacy of treatment, due to its anti-inflammatory action during the periods of exacerbation and relative remission, prevents augmenting of bronchial obstruction, and improves patients' LQ.

[Clinical and hemodynamic efficacy of selective beta-blocker bisoprolol and cytoprotector trimetazidine in the treatment of chronic cardiac failure in patients with ischemic heart disease]. / Kliniko-gemodinamicheskaia éffektivnost' selektivnogo beta-blokatora bisoprolola i tsitoprotektora trimetazidina pri lechenii khronicheskoi serdechnoi nedostatochnosti u bol'nykh ishemicheskoi bolezn'iu serdtsa.

AIM: To assess the effect of a long-term (6 months) treatment with selective beta-blocker bisoprolol, cytoprotector trimetazidine and their combination on the clinical course, left ventricular morphofunctional parameters and quality of life of patients with ischemic heart disease (IHD) with chronic heart failure (CHF) functional class III-IV. MATERIAL AND METHODS: A total of 71 IHD patients with CHF (FC III-IV) entered the study. In group 1 basic therapy was combined with bisoprolol, in group 2--with trimetazidine, in group 3--with bisoprolol + trimetazidine. The initial dose of bisoprolol was 1.25 mg with subsequent dose titration to individually tolerated. Trimetazidine was given in a dose 20 mg 3 times a day. Clinical tests and echocardiography parameters were assessed monthly. RESULTS: CHF functional class decreased in all the groups. End diastolic and systolic volumes of the left ventricle reduced, while ejection fraction increased. The best treatment results were achieved in combination of bisoprolol with trimetazidine (LV EF increased by 42.6%, while Ve/Va decreased by 59%). CONCLUSION: Clinicohemodynamic benefit obtained after long-term administration of bisoprolol, trimetazidine and their combination in IHD patients with CHF (Fc III-IV) justifies inclusion of these drugs as monotherapy and combined treatment of CHF in IHD.

[Potentialities of cytoprotection in the treatment of chronic heart failure in patients with coronary heart disease]. / Vozmozhnosti tsitoprotektsii v lechenii khronicheskoi serdechnoi nedostatochenosti u bol'nykh ishemicheskoi bolezn'iu serdtsa.

The study was undertaken to evaluate the effects of long-term (6-month) therapy with the selective beta-blocker bisoprolol, the cytoprotector trimetazidine and their combination on the clinical course of disease, the morphofunctional parameters of the left ventricle (LV) and life quality in 71 patients with coronary heart disease (CHD) concurrent with functional classes (FC) III-IV chronic heart failure (CHF) (ejection fraction (EF) < 35%, as evidenced by echoCG). In Groups 1, 2, and 3, basic therapy was supplemented by bisoprolol, trimetazidine, and bisoprolol plus trimetazidine, respectively. The initial dose of bisoprolol was 1.25 mg with its subsequent titration to an individually tolerable. Trimetazidine was given in a dose of 20 mg thrice daily. The patients' clinical status and echoCG were monthly assessed. There was a decrease in FC of CHD in all the groups, a reduction in LV end diastolic and systolic volumes, and an increase in shortening faction, LV EF, and an improvement of LV diastolic function. There was evidence that it should be expedient and safe to use the cytoprotector trimetazidine in the treatment of CHF in patients with CHD. In patients with CHD concurrent with CHF, therapy using a combination of bisoprolol and trimerazidine was found to have the most pronounced impact, which yielded the maximum clinical and hemodynamic effect (LV EF increased by 42.6% of the baseline values, the rate of early and late ventricular diastolic filling decreased by 59% of the baseline values). The revealed regularities of the positive effect on the clinical and hemodynamic parameters of long-use of bisoprolol, trimetazidine, and their combination in patients with CHD concurrent with FC III-IV CHF show it expedient to include these drugs and their combination into therapy for CHF that develops in the presence of CHD.

[The stomach and duodenum condition in patients with chronic obstructive lung diseases]. / Sostoianie zheludka i dvenadtsatiperstnoi kishki u bol'nykh s khronicheskimi obstruktivnymi bolezniami legkikh.

Examining the gastroduodenal mucosa (GDM) in 61 patients with chronic obstructive pulmonary diseases (COPD) showed changes in the areas studied in all cases. GDM atrophy increased with the severity of COPD. Helicobacter pylori are essential in developing gastric pathology in patients with mild bronchopulmonary disease. The severity of atrophic gastritis increases with the longer duration of COPD and with more severity of hypoxia and bronchial obstruction.

[Change in activity of angiotensin-converting enzyme in pneumonia and chronic obstructive lung diseases]. / Izmenenie aktivnosti angiotenzinprevrashchaiushchego fermenta pri pnevmonii i khronicheskikh obstruktivnykh bolezniakh legkikh.

AIM: To study the activity of angiotensin-converting enzyme (ACE) in patients with pneumonia and chronic obstructive lung diseases (COLD). MATERIAL AND METHODS: Sixty nine patients with pneumonia and 77 with COLD were examined. The activity of ACE in the serum and bronchoalveolar lavage (BAL) and the effects of leukocytic elastase and concentrations of zinc, endogenous inhibitors, and activators were studied. RESULTS: The patients with pneumonia in the acute phase of the disease have been found to have low ACE activity in both blood and BAL. As the inflammatory process comes to an end, ACE activity normalizes. In the patients with COLD, the activity of ACE is primarily decreased at remission. When COLD aggravates, the activity of ACE in blood and BAL increases. In pneumonia and COLD, the changes in ACE activity are more profound in BAL than in blood. CONCLUSION: The only cause of the altered activity of ACE in patients with COLD and pneumonia is a change in the concentration of the enzyme.