[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.

[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.

[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.

[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.

[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.

[The late results of the surgical treatment of vesicorenal reflux in children]. / Otdalennye rezul'taty operativnogo lecheniia puzyrno-pochechnogo refliuksa u detei.

1-27-year follow-up results are available for 455 patients who at the age of 3 months-14 years were operated on vesicoureteral segments with reflux of the II, III and IV degree. Overall 648 interventions were made, of them 78 according to Grégoir-Lich (54 patients). Good results were obtained in 91.02% of cases (reflux degree II and III). Resection of distal ureter with its implantation into the bladder by Politano-Leadbetter technique was performed in 401 patients on 570 vesicoureteral segments. In reflux degree II and III good outcomes were reached in 97.37%, degree IV in 90.80% of the cases. Histological findings on the resected ureters showed that pathogenetically the reflux was initiated by structural changes in distal ureteral wall which occurred as a congenital or acquired condition. The removal of the destroyed segment contributed to advanced treatment effects. The reflux elimination favoured renal growth and clinical improvement of pyelonephritis. X-ray images of 150 kidneys (95 patients) 10-26 years after the reflux discontinuation reflected a 1.5-5-fold increase in the kidney size. Before stabilization parenchymal sclerosis developed the following 1-2 to 10-15 years.

[Corrective operations in ureterocele in children]. / Korrigiruiushchie operatsii pri ureterotsele u detei.

The authors analyze their experience in the treatment of 59 children aged 3 months--14 years with ureterocele of the single (22 patients) and double ureter (37 patients). Examination and surgical data showed that ureterocele in the operated on children was congenital and became a component of a complicated malformation of the distal ureter. Structural changes varied from minimal to total dysplasia reflected in the degree of the ureteral dilatation. These changes were characteristic for both normal and double ureters and served the base for treatment policy decision. In normal megaureter the authors performed resection of the distal ureter as well as ureterocele, modelling and neoplantation of the proximal segment according to the antireflux technique. Minor dilatation of the pelvic ureter (up to 1.0-1.5 cm dependent on the patient's age) indicated that structural alterations of its wall are confined to the submucosal segment. In view of this, surgical intervention involved only endovesicular electroperforation of ureterocele. The policy in the double ureter ureterocele was based on the same criteria.

[The nature of the major pathology in the perinatal period at the present time]. / Kharakter osnovnoi patologii perinatal'nogo perioda na sovremennom étape.

The main pathology leading to mortinatality has been determined. It is shown that in 57.8% of cases mortinatality is associated with placental insufficiency, the reason for which can be found out by the pathoanatomical study of the afterbirth. It is proposed to differentiate afterbirth disorders of implantation, placentation, circulation and inflammatory changes. Fifty per cent of neonates are shown to die from the respiratory failure associated with the formation of atelectasis, hyaline membranes and edema with pulmonary hemorrhages. Next in frequency are cephalic ventricle extravasations. The main pathogenetic factor of both neonatal pathologies is prematurity with functional and morphological tissue immaturity.

[Gaucher's disease combined with congenital developmental defects of the urinary system]. / Bolezn' Goshe v sochetanii s vrozhdennymi porokami razvitiia mochevyvodiashchei sistemy.

Two cases of Gaucher's disease of the juvenile type are described, in a boy of 1 year and 7 months and in a girl of 8 years. The juvenile type of Gaucher's disease is documented by detection in the internal organs of typical Gaucher's cells formed mainly of histiocytes and macrophages, PAS-positive and weakly positive with Sudan III, and by the absence of the brain gangliocytes impairments. The peculiarity of these cases consists in the combination of glucocerebroside with congenital developmental defects of the urinary system: in one case with bilateral megaureter and hydronephrosis and in the second one with developmental defect of the renal arteries and bilateral cystic renal dysplasia.

[Current aspects of the problem of thesaurismoses]. / Sovremennye aspekty problemy tezaurismozov.

The main changes occuring in the theory of thesaurismoses are presented briefly. Main groups of thesaurismoses characterized by disorders of one or another kind of metabolism are dealt with. Most frequently occuring forms of thesaurismoses are described and by their examples it is shown how new nosological entities are distinguished by the nature of metabolic disorders, by the defect of the enzyme, and by the degree of severity of the enzymatic block. The main criteria for morphological diagnosis of thesaurismoses are presented including the presence of accumulation cell, their localization in different organs, histochemical analysis of accumulation products and electron microscopic findings. Biochemical examinations are considered to be essential for diagnosis.