[Clinical and morphological diagnosis of secondary hemophagocytic lymphohistiocytosis]. / Kliniko-morfologicheskaya diagnostika vtorichnogo gemofagotsitarnogo limfogistiotsitoza.

Secondary hemophagocytic lymphohistiocytosis (hemophagocytic syndrome, massive macrophage activation syndrome) is a rare, aggressive, severe syndrome with a frequent fatal outcome. The pathogenesis of the syndrome, the role of viral infection, and the development of an excessive, aberrant, and inflammatory response leading to multiple organ dysfunction and sepsis remain unclear so far. The paper presents the data available in the literature and describes the authors' own observation of secondary hemophagocytic lymphohistiocytosis in a 14-year-old girl who had Epstein-Barr virus infection and died of severe multiple organ dysfunction. The in vivo diagnosis was based on the typical clinical presentations of the disease, the detection of the virus by a polymerase chain reaction assay, and the determination of blood ferritin levels. Contemporary morphological diagnostic criteria (immunohistochemical reactions with anti-EBV and anti-CD68 monoclonal antibodies; Epstein-Barr virus encoded RNA (EBER) in situ hybridization) are shown; and clinical and pathological comparisons have been made.

[The etiologic structure and biologic characteristics of agents of infections of bloodstream.]

As before, the infections of blood flow remain actual because of absence of universal approaches to their diagnostic and high lethality. Taking into account the fact that most frequently the sepsisogenic strains are selected in blood, studying of biological characteristics of hemocultures in comparison with strains isolated from other biotopes is very important for understanding the mechanisms of survival of pathogens in blood and prognosis development of septic complications. The article presents the results of microbiological analysis of blood carried out in multi-field clinic of Khanti-Mansiisk during 2007-2015. The following biological characteristics of S.aureus, E.coli, P.aeruginosa, C.albicans: hemolytic, catalase, anti-lysozyme, anti-complementary, biofilm forming, activity of hemocultures and strains isolated from other biotopes. The detection of hemolytic, catalase, anti-lysozyme, anti-complementary activities was implemented using photo-metric technique by capacity to lyse/inactivate corresponding substrate. The biofilm forming activity was evaluated according alteration of contact angle of moistening of surface of biofilm. The priority pathogens of infections of blood stream are coagulase-positive staphylococci - 38.3% (S.aureus), coagulase-negative staphylococci (S.epidermidis, S.hominis etc.), enterobacteria - 12.3% (E.coli, K.pneumonia), Pseudomonas aeruginosa - 4.1%, among fungi - C.albicans (2.5%). It is demonstrated that, in comparison with strains isolated from other biotops, hemocultures have statistically reliable higher values of catalase, anti-lysozyme, anti-complementary, biofilm forming activities. The conclusion is made concerning significance of these biological characteristics of pathogens for their survival in blood. It is proposed to use these indices as markers of sepsisogenic strains in prognosis of generalization of inflectional process.

[Complex treatment of generalized gingivitis with mineral therapy].

A comprehensive examination and treatment of 49 students aged 20-25 years diagnosed chronic generalized catarrhal gingivitis (CGCG) were held. Depending on the methods of treatment were created into two groups of observation. The main group comprised 24 patients who along with dental sanitation and treatment of gingivitis took the course of salt treatment for 17 days, as opposed to control group. Hygienic researches and mineralthcrapy were held in a special room (23.6 m2). equipped with sylvite blocks with a total reaction surface 5 m2, salt filters with air ducts filled with wooden plates with mineral fragments. The study of the effectiveness of a comprehensive treatment of young patients with CGCG allowsto gel an information of the positive impact of salt therapyon the clinical condition of marginal periodontal tissues and indeces of oral cavity local immunity. The main curative factorsforming the internal environment of silvinite structures are multicomponent highly dispersed salt aerosol with a defined particle size and aeroionization. Natural salts complex consisting of chlorides of potassium, sodium and magnesium has an anti-inflammatory and immuno-modulating effects.

[Complex treatment of chronic periodontitis with balneopeloid therapy].

Complex investigation and treatment has been realized on 127 patients aged from 27 to 45 years with chronic generalized periodontitis. Patients were divided into two groups. In the fist group (68 patients) complex treatment included course of balneopeloid therapy with irrigation of high mineralized natural water (sanatorium "Tumentransgas" chink №1-95, Ugorsk) followed by application of sapropel mud (the Pake lake). The second group (59 patients) received only conventional periodontal treatment. Positive effect of balneopeloid therapy was identified, which was reflected in stabilizations of pathological process in the periodontal tissues in 78% of patients, as well as changes in physical and chemical properties of the oral gluid. Also the number of periodontopahogenic germs of gingival pockets was decreased.

[Coupling between the hemodynamic parameters and the morphological changes in the kidney in children with congenital hydronephrosis].

The data of renal ultrasonographic and Doppler studies were comparatively assessed in children with congenital hydronephrosis. The degree of renal B-mode ultrasound hemodynamic parameters was used as the basis for grouping of the children. The resistance index (RI) of all branches of renal arteries receives attention. Morphological studies were carried out on renal biopsy specimens from 29 children from different groups and on 12 removed kidneys; the expression of TGF beta1 and alphaSMA was revealed using the streptavidin-biotin-peroxidase method. Morphological changes as hypoplasia or dysplasia became more pronounced from Group 1 to Group 3, RI increasing to peak in Group 3. Vascular changes were confined to compensatory processes following the pattern of remodeling that was manifested by vascular wall thickening and a gradual increase in RI. Failing compensatory processes resulted in the development of renal functional and hormonal decompensation and in the elevation of RI. There was a coupling between the magnitude of morphological changes and RI increases. A set of the findings emphasizes the undoubtedly important role of renal ultrasound study that makes it possible to judge the state of the vascular bed and to suggest renal structural problems in congenital hydronephrosis.

[Physiological prerequisites for a successful child's interaction with the computer].

Were identified specific and meaningful for computer work physiological functions in children of 5-6 years: a functional motility of nervous processes, the function of the motor analyzer (accuracy of hand kinesthesia), the functional state of the eye accommodative system, mental capacity, volume and concentration, shortterm memory function. A considerable number of preschoolers (47%) set high enough level of development of organism functions necessary for successful interaction with the computer.

[Transforming growth factor as a marker beta of nephrogenetic disturbance in congenital obstructive uropathies].

The paper presents the results of morphological and immunohistochemical studies of the kidneys and ureters obtained at surgery in 25 children aged from 2 days to 3 years who had congenital obstructive uropathies. The morphological study revealed varying renal tissue dysplasia concurrent with dysplasia of the prepelvic or distal ureter. Immunohistochemically, the kidneys showed a pronounced expression of TGFbeta1, TGF(1R1 and R2 in the nephrocytes of non-differentiated and collecting tubules, in the tubular lumen, vascular walls, in the interstitial tissue and cell interstitial infiltrates, on the hypoplastic and disoriented ureteral smooth muscle fibers. The use of the ILIAS-method to determine urinary TGF levels before and after surgery indicated a direct correlation of these indices with the degree of renal tissue dysplasia.

alpha-Defensins from blood leukocytes of the monkey Papio hamadryas.

Three antimicrobial peptides named PHD1-3 (Papio hamadryas defensin) have been isolated from hamadryas baboon blood leukocytes using preparative electrophoresis and reverse-phase HPLC. The primary structures of these peptides have been determined by automated Edman degradation and mass-spectrometry. The results suggest that the peptides belong to the alpha-defensin family. Structural homology analysis reveals that among alpha-defensins from other animal species, PHD3 is the most closely related to RMAD5 (rhesus macaque alpha-defensin) (90% homology) from rhesus macaque leukocytes and also highly similar to human alpha-defensin HD5 (60% homology), which is produced by intestinal Paneth cells. The homology of PHD3 with human neutrophil alpha-defensin HNP1 (human natural peptide) was 30%. The primary structures of PHD1 and PHD2 are most similar to RED1 (rhesus enteral defensin), one of six enteral alpha-defensins of rhesus monkeys. PHD1-3 have been shown to be active against the Gram-positive bacteria Listeria monocytogenes and Staphylococcus aureus, the Gram-negative bacterium Escherichia coli, and the fungus Candida albicans, similarly to the human HNP1 defensin.

[Variants of formation of v. testicularts sinistra in children with varicocele].

Biopsies of veins of 20 boys with varicocele were studied morphologically. Three types of veins were observed. The first type was represented by veins with a wide lumen and the wall consisting of three layers of smooth muscle cells. The second morphological variant--veins of varying diameter of irregular shape and thin, frequently sclerosed wall. The third type were veins of a small diameter with well developed muscular layer. Immunohistochemical studies detected disturbances of vascular wall components suggesting the development of varicocele as a complex of congenital pathology with secondary alteration and destabilisation of compensatory processes.

[Renal development long after correction of primary non-refluxing forms of megaureter in children].

We studied the condition and development of 50 kidneys in 45 patients 10-30 years after surgical correction of megaureter. We made resection and neoimplantation of the ureter according to the antireflux technique. By x-ray findings, we estimated area of the kidney, renocortical index and compared them with visual assessment of the condition of the kidney and its pelvicocalicyal system. We have found that correction of the megaureter creates favourable conditions for development of the kidney as shown by good growth of the kidney (area increased 1.5-3.5 fold) in a stable renocortical index. This indicates stabilization of nephrosclerosis and large area of renal parenchyma. However, kidney growth was accompanied with developing nephrosclerosis in 7 cases, in 1 case nephrosclerosis terminated with secondary contraction and a decline of renal function. The condition of the kidney varied due to many factors such as the condition of the immune system, age of the patient, virulence of the microflora, etc., but the key role for the kidney belonged to condition of renal parenchyma before surgical intervention. The data of light optic (53 cases) and electron-microscopies (3 cases) examinations of renal biopsies demonstrated that renal lesions were characterized by congenital structural disorders such as low number of nephrons, the presence of canalicular and glomerular microcysts and other manifestations of hypoplastic dysplasia. Inflammatory infiltration of the cortical and medullary layer interstitium aggravated severity of renal lesion. Various correlation and severity of congenital and aquired lesions characterized condition of the kidney in each individual case. Thus it is necessary to conduct a long-term follow-up and update methods of therapy.

[Surgical correction of primary nonrefluxing megaureter in children and its remote results]. / Operativnaia korrektsiia pervichnykh nerefliuksiruiushchikh form megauretera u detei i ee otdalennye rezul'taty.

Correction of primary non-reflux megaureter (153 ureters) was made in 136 patients aged 3 months to 14 years. Bilateral disease was in 17 patients. Non-reflux non-obstructive megaureter was in 113 cases, obstructive in 40 cases including association with ureterocele in 23 cases. Resection of distal ureter with its neoimplantation into the urinary bladder according to the antireflux technique was made in 146 patients, endovesical electroperforation and resection of ureterocele were made in 5 and 2 patients, respectively. Good results were obtained in 88.3% (135 ureters), satisfactory in 2.6% (4 ureters), unsatisfactory in 9.1% (14 ureters). After effective correction of megaureter, the treatment should be focused on adequate therapy of pyelonephritis present in 90% examinees, on improvement of urodynamics and stabilization of sclerotic process in renal parenchyma. The patients need long-term follow-up and more effective treatment.

[Status and development of the kidney after surgical treatment of hydronephrosis in children]. / Sostoianie i razvitie pochek posle operativnogo lecheniia gidronefroza u detei.

Development of the kidneys after correction of hydronephrosis was studied in 40 patients operated at the age of 8 months-12 years. Follow-up lasted 10-25 years. X-ray control showed efficiency of correction of the uretheropelvic segment (UPS). Roentgenoplanimetric study demonstrated a uniform 50-100% increase in renal area and its parenchyma in 28 patients (32 kidneys). In 9 patients parenchyma increased 2.5-4-fold, renal area by 50-100%. In three cases parenchyma diminished by 3-10% in growth of the kidney by 24-80%. This evidenced of nephrosclerosis progress. UPS correction favoured renal growth and development. There were only few cases when correction of UPS was positive but nephrosclerosis aggravated. Development of the kidney may depend on many factors such as preoperative condition of the kidney, severity of UPS obstruction, the patient's age, duration and severity of pyelonephritis, dysplasia and age-related immaturity of renal parenchyma, immune status of the organism, microorganism virulence, etc. But decisive was the condition of renal parenchyma before the operation. In 3 patients with progressive nephrosclerosis renal parenchyma affection was additionally characterized by advanced sclerosis and inflammatory infiltration of the intertitium which ended in a poor outcome. The above observations suggest the necessity of long-term postoperative follow-up of the patients to design effective measures aimed at the treatment and prevention of nephrosclerosis.

Circular minidefensins and posttranslational generation of molecular diversity.

We purified two new minidefensins (RTD-2 and RTD-3) from the bone marrow of rhesus monkeys. Both were circular octadecapeptides that contained three intramolecular disulfide bonds and were homologous to RTD-1, a circular (theta) defensin previously described by Tang et al. (Science, 286, 498-502, 1999). However, whereas the 18 residues of RTD-1 represent spliced nonapeptide fragments derived from two different demidefensin precursors, RTD-2 and -3 comprise tandem nonapeptide repeats derived from only one of the RTD-1 precursors. Thus, circular minidefensins are products of a novel posttranslational system that generates effector molecule diversity without commensurate genome expansion. A system wherein two demidefensin genes can produce three circular minidefensins might allow n such genes to produce (n/2)(n+1) peptides.

[Reconstructive surgeries for children with fused kidney hydronephrosis]. / Rekonstruktivnye operatsii pri gidronefroze podkovoobraznoi pochki u detei.

1-19-year follow-up results of plastic surgery for fused kidney hydronephrosis (FKH) in 12 children aged 3-14.5 years (a total of 15 hydronephroses, 3 cases of bilateral disease) were estimated as good. Correction of FKH is performed by resection of the affected ureteropelvic segment with bypass plastic reconstruction according to Andersen-Hynes-Kucera, compulsory total resection of the renal isthmus. Isthmotomy can be conducted only in the presence of connective tissue bridge between the renal poles. Often, renal isthmus consisting of parenchyma is to be removed together with resection of the renal pole (in joint circulation). In addition, for good outcome it is necessary to reconstruct pelvis and (or) perform nephropexy with muscular graft. The operated patients need long-term follow-up, control examinations of the kidney, updated treatment following elimination of the obstruction.

[The surgical correction of hydronephrosis in children (the late results)]. / Khirurgicheskaia korrektsiia gidronefroza u detei (otdalennye rezul'taty).

Surgical interventions on 368 pelviureteral segments were made in 348 hydronephrosis patients under 14 years old. Bilateral disease occurred in 20 patients. Resection of the pelviureteral segment was performed with ureteropyeloplasty according to Anderson-Hynes-Kucera procedure. Good results were observed in 97.82% of cases followed-up from 1 to 2 years after the operation. Histological examination of the resected segments revealed structural changes in the wall of prepelvic part of the ureter primarily of congenital genesis, irrespective of low-polar vessels, high origin of the ureter, its fixed kink or narrowing. Irreversibility of structural lesions necessitated conduction of resection ureteropyeloplasty with creation of morphofunctionally patent pelviureteral anastomosis. High efficacy of the operation supports its pathogenetic validity. The effect persisted at the follow-up. After operative correction of hydronephrosis medication should be aimed at adequate therapy of pyelonephritis and stabilization of sclerosis in renal parenchyma, at improvement of immune status and urodynamics. Further follow-up, control of the kidneys in search for advanced postobstruction treatment are needed.