[Down's syndrome with congenital acute leukemia and congenital heart disease]. / Sluchai sindroma Dauna s vrozhdennym ostrym leikozom i vrozhdennym porokom serdtsa.

OBJECTIVE: To report a rare case of Down's syndrome with congenital acute leukemia and congenital heart disease. MATERIAL AND METHODS: We have retrospectively analyzed an electronic database of patients aged 0-17 years who underwent inpatient treatment of congenital heart disease at the Federal Center for Cardiovascular Surgery for the period from January 1, 2010 to December 31, 2018. Incidence of Down's syndrome in children with congenital heart defects was 5.36% of the total number of children with congenital heart defects. No gender differences were identified. The most common congenital heart defects were ventricular septal defect, atrioventricular septal defect, atrial septal defect, patent ductus arteriosus, tetralogy of Fallot. Down's syndrome and congenital heart disease were diagnosed prenatally in 33 out of 319 patients. A rare case of Down's syndrome combined with congenital heart disease and acute leukemia is described. Treatment outcome was unfavorable. CONCLUSION. S: Evere concomitant diseases in a child with Down's syndrome determine high postoperative morbidity and mortality. Prenatal diagnosis of Down's syndrome via screening of pregnant women for ultrasound and biochemical markers of this pathology is required.

[THE REFERENCE VALUES OF AGGREGATION OF PLATELETS IN ADULT POPULATION OF THE ASTRAKHAN OBLAST USING AGGREGOMETER MULTIPLATE].

The modern international standards recommend each laboratory to develop or to confirm available in literature the reference intervalsfor every laboratory indicator In the Astrakhanskaia oblast, sampling of128 healthy males andfemales were examinedfor aggregation function of thrombocytes using impedance technique and applying aggregometer Multiplate ("Verum Diagnostica", Germany). The study used as inductors peptide activating receptor of thrombin; arachidonic and adenosine diphosphoric acids. The reference range of aggregation of thrombocytes with peptide activating receptor of thrombin, at aggregometer Multiplate, in healthy population of theAstrakhanskaia oblast made up to 815.2-1498.4 AU/min, with arachidonic acid--660-1341 AU/min. with adenosine diphosphoric acid--598-1120 AU/min.

[The reference intervals of antithrombin III in population of the Astrakhanskaia oblast at application of automated coagulometers.]

The foreign and national professional societies of laboratory diagnostic recommend to every laboratory to develop own or substantiate available in literature reference intervals for every laboratory index. The reference interval of antithrombin III was established for adult population of the Astrakhanskaia oblast according available standards on automated coagulometers "ACL 9000" (Instrumentation Laboratory, USA) and "STA Compact" (Diagnostica Stago, France). The received data were compared with data present in instructions to reactive kits for detection of antithrombin III and reference literature. The reference interval AT III in adult population of the Astrakhanskaia oblast has no dependencies on gender and age and can be compared with reference intervals mentioned in instructions to reactive kits for detection of activity of AT III of firms producers Instrumentation Laboratory (USA) and Diagnostica Stago (France) in the reference literature. The reference intervals AT III mentioned in instructions to reactive kits produced by Instrumentation Laboratory (USA) and Diagnostica Stago (France) and in reference literature can be applied for interpretation of results of detection of activity of AT III by automated coagulometers "ACL 9000" (Instrumentation Laboratory, USA) and "STA Compact" (Diagnostica Stago, France) in adult population of the Astrakhanskaia oblast.

[MARKERS OF SYSTEMIC INFLAMMATORY RESPONSE IN PATIENTS OPERATED FOR COMPLICATIONS OF INFECTIOUS ENDOCARDITIS].

The study of markers of systemic inflammatory response in patients with infectious endocarditis showed that at admittance they had elevated blood C-reactive protein and ferritin levels while leukocyte count and fibrinogen content remained normal. C-reactive protein and ferritin levels increased on day 1 of the postoperative period and tended to grow further on day 3 when both parameters reached maximum values; they started to decrease on day 6 These data indicate that C-reactive protein and ferritin levels can be used to characterise the postoperative conditions of the patients including manifestations of systemic inflammation and outcome of surgical treatment.

[REFERENCE RANGES OF ANTITHROMBINE III IN ASSOCIATION WITH THE USE OF A STA COMPACT AUTOMATED COAGULOMETER].

Domestic andforeign professional diagnostic communities recommend to develop new or confirm literature reference ranges for each laboratory parameter. Bearing in mind the importance of antithrombin-III levels for anticoagulation therapy, we determined their reference ranges for the adult population of the Astrakhan region and compared them with those obtained by other authors and presented in the instructions for use of commercial kits for measuring ant ithrombin-III. Our reference ranges differ from those cited in the said instructions.

[The significance of immature granulocytes in diagnostic of infectious inflammatory processes in cardiosurgery patients].

The evaluation was implemented concerning the values of automated parameter "immature granulocytes" in cardiosurgery patients. It is demonstrated that amount of immature granulocytes in peripheral blood exceeding 2% indicates development of infectious inflammatory process (sepsis). The monitoring of amount of immature granulocytes in cardiosurgery patients makes it possible to evaluate effectiveness of applied therapy, severity of patient condition, course of post-surgery period and outcome of disease.

[Changes in biochemical parameters of blood in patients with rheumatic heart failure following surgical intervention].

We analysed changes of biochemical parameters of blood in patients treated for chronic rheumatic heart problems. It was shown that biochemical parameters of blood changed within 12 days after surgical intervention; the changes were especially pronounced on days 1-3 whereas normalization occurs by days 9-12. Pathogenetically sound time periods for changes of biochemical characteristics of blood serum were established in the patients treated for chronic rheumatic heart problems. On the one hand, it will allow more accurate monitoring of development of complications in the postoperative period and on the other hand to reduce expenses by decreasing the number of unnecessary analyses.

[The importance of thrombocytic indices in patients operated for chronic rheumatic heart disease].

We studied the importance of thrombocytic indices in patients operated for chronic rheumatic heart disease and showed that a decrease in the platelet count is accompanied by increased thrombocytic indices including platelet width and mean volume, the relative number of large platelets in peripheral blood The increase was regarded as a compensatory response to the decrease in the platelet count; it suggests enhanced thrombocytopoiesis. It is concluded that deretmination of thrombocytic indices in cardiosurgical patients using automated hematological analyzers allows to evaluate the prcesses of thrombocytopoiesis.