EVALUATION OF EFFICIENCY OF IMIGLUCERASE (CEREZYME) IN THE TREATMENT OF GAUCHER DISEASE (CASE REPORTS AND REVIEW OF THE LITERATURE).

The data on occurrence and pathogenetic mechanisms of glucosylceramide lipidosis (Gaucher disease), which is based on the hereditary deficiency of glucocerebrosidase activity -the enzyme involved in the processing of cellular metabolism products, is presented. Clinical and morphological manifestations of the disease, therapeutic tactics and prognosis are defined. A typical clinical case of Gaucher disease type 1 in two adult patients was analyzed. It is shown that timely diagnosis of Gaucher disease allows the prescription of adequate treatment by means of enzyme replacement therapy with cerezyme drug. That is why the correct and timely diagnosis of the disease can prolongate patient's life for many years. In this case, the patient should be under the care of doctors (pediatricians, general physicians, hematologists). Continued treatment of Gaucher disease with cerezyme stabilizes the pathological process completely, decreases significant changes in bones and parenchymal organs, improving patients' lives considerably. For that reason, the sooner the adequate therapy is started, the more effective the results will be.

LIVER FUNCTION DISORDERS IN PATIENTS WITH FIRST DIAGNOSED PULMONARY TUBERCULOSIS.

The patients with pulmonary tuberculosis suffer from hepatic dysfunction due to both hepatotoxic affection with anti-TB drugs and other factors (hypoxia, antioxidant deficiency, intoxication). Therefore, the aim of the research was to study the markers of cytolytic and cholestatic syndromes in cases of hepatic dysfunction in the first diagnosed patients with pulmonary tuberculosis before treatment and in two months of treatment with first-line antituberculous drugs. Three groups of persons were examined: the first group of almost healthy donors (control) - 31 persons. Also, 62 patients were examined for the first time diagnosed pulmonary tuberculosis before treatment and in two months of therapy with first-line antituberculous drugs, which were divided into two groups: the second - the patients (31 persons), who had a moderate intoxication syndrome; the third - the patients (31 persons), who had a severe toxicity syndrome when admitted to hospital. A significant hepatic dysfunction in the first diagnosed patients with pulmonary tuberculosis and severe intoxication syndrome has been established. In the patients with moderate intoxication syndrome, the markers of cytolysis and cholestasis were within normal range before treatment, but after the intensive phase of treatment, the levels of LDH, LF, and GGT were significantly increased. In patients with severe intoxication syndrome, even before treatment, the increase in markers of cytolysis and cholestasis was evidenced. After the intensive phase of treatment, the increase in these rates sustained. It is recommended for patients with pulmonary tuberculosis and severe intoxication syndrome to perform diagnostics of such hepatic dysfunction markers as LDH, ALP and GGT, and if any disorders revealed, to prescribe additional hepatoprotective therapy.

[CARDIOREABILITATION PECULIARITIES AND CORRECTION OF VIOLATIONS OF SISTOLIC, DIASOLIC FUNCTION AND HEART RATE VARIABILITY IN PATIENTS WITH ACUTE CORONARY SYNDROME AND CORONARY ARTERY REVASCULARIZATION].

In patients with acute coronary syndrome (ACS) who underwent coronary arteries revascularization, violations of hemodynamics, metabolism and heart rate variability often develop in the postoperative period, therefore, the goal of the study was to establish the features of disturbances and the effectiveness of correction of left ventricular systolic and diastolic dysfunction and heart rate variability in stages of cardiorehabilitation in patients with acute coronary syndrome who underwent coronary arteries revascularization. The experimental group included 40 patients with ACS in the postoperative period who underwent balloon angioplasty and stenting of the coronary arteries (25 patients with ST-segment elevation ACS and 15 patients without ST-segment elevation ACS). The age of examined patients was 37 to 74 years, an average of 52.6±6.7 years. The control group consisted of 20 patients, comparable in age and clinico-laboratory manifestations of ACS, who underwent drug treatment with direct anticoagulants, double antiplatelet therapy, ß-blockers, ACE inhibitors and statins. Clinical efficacy of cardiorespiratory process in patients of both groups was assessed by the dynamics of general clinical symptoms and parameters of natriuretic propeptide, systolic and diastolic function of the left ventricle and heart rate variability. In the initial state, clinical and laboratory-instrumental signs of myocardial ischemia disappear in patients with ACS undergoing surgical revascularization of the coronary arteries, but clinical and subclinical manifestations of heart failure were diagnosed. The use of the accelerated program of cardiac rehabilitation already during the first month of studies leads to a decreasement of the signs of systolic and diastolic dysfunction, the level of NT-proBNP and improve in the variability of the heart rhythm wich significantly improves the life quality of patients with ACS. To monitor the effectiveness and safety of cardiac rehabilitation in patients with ACS who underwent coronary arteries revascularization, in addition to the generally accepted methods (determination of heart rate, blood pressure, a 6-minute test), it is advisable to diagnose the subclinical stage of heart failure by determining the level of NT-proBNP, Doppler echocardiogram, parameters of the left ventricular systolic and diastolic function and heart rate variability.

[Saprophytic microflora of Kurgazak mineral water].

Samples of weakly mineralized hydrocarbonate-calcium-magnesium water from the spring of Kurgazak were analyzed during 3 successive years for the purpose of investigating seasonal dynamics of trophic microorganisms.

[Change in indicators of metabolism and kidney function in intact rats after parenteral administration of sodium hypochlorite]. / Izmenenie pokazatelei metabolizma i funktii pochek u intaktnykh krys posle parenteral'nogo vvedeniia gipokhlorita natriia.

The changes of kidney metabolism, function and enzymuria activity of nephron epithelium cells after 4 days of parenteral administration of sodium hypochlorite 0.02% and 0.06% solutions were investigated in the experiments on 15 intact non-inbred rats of 200-280 g body weight. It was revealed that sodium hypochlorite induced metabolic damage to the kidney similar to ischemic one. The 0.02 and 0.06% solutions of sodium hypochlorite administered parenterally produced no negative effect on renal function. There was no significant rise of enzymuria activity in experimental groups compared to the controls. Moreover, enzymuria of four from six nephron epithelium cell enzymes decreased significantly to day 5 of the experiment. Metabolic damage and enzymuria activity changes were more pronounced in rats given parenteral 0.06% solution of sodium hypochlorite.

[Increase in structural-functional tolerance of kidney to ischemia upon parenteral administration of sodium hypochlorite in the pre-ischemic period]. / Povyshenie strukturo-funktsional'noi tolerantnosti pochek k ishemii pri parenteral'nom vvedenii gipokhlorita natriia v predyshemicheskom periode.

The opportunity of raising kidney structural and functional tolerance to ischemia with parenteral injections of 0.06% solution of sodium hypochlorite for 4 days in preischemia period was studied in experiment on 22 non-inbred rats of 200-280 g body weight. 90 minute ischemia was created by ligating the left and right kidney arteries, veins, ureters. Morphological and functional kidney data, enzymuria activity of nephron epithelium cells were registered, 80 and 33% of the rats survived 7 days after ischemia in the study and control groups, respectively. The control animals showed deterioration of the tubules function, high enzymuria. Thus, it is possible to raise kidney tolerance to ischemia by preischemic parenteral injection of sodium hypochlorite solution.

Compact myelin dictates the differential targeting of two sodium channel isoforms in the same axon.

Voltage-dependent sodium channels are uniformly distributed along unmyelinated axons, but are highly concentrated at nodes of Ranvier in myelinated axons. Here, we show that this pattern is associated with differential localization of distinct sodium channel alpha subunits to the unmyelinated and myelinated zones of the same retinal ganglion cell axons. In adult axons, Na(v)1.2 is localized to the unmyelinated zone, whereas Na(v)1.6 is specifically targeted to nodes. During development, Na(v)1.2 is expressed first and becomes clustered at immature nodes of Ranvier, but as myelination proceeds, Na(v)1.6 replaces Na(v)1.2 at nodes. In Shiverer mice, which lack compact myelin, Na(v)1.2 is found throughout adult axons, whereas little Na(v)1.6 is detected. Together, these data show that sodium channel isoforms are differentially targeted to distinct domains of the same axon in a process associated with formation of compact myelin.

[Sodium hypochlorite in the treatment of bacteremic circulatory shock]. / Gipokhlorit natriia v lechenii infektsionno-toksicheskogo sosudistogo shoka.

17 patients with massive urinary infection and bacteriemic circulatory shock received a combined intensive treatment including indirect electrochemical blood oxidation (IEBO) with 0.06% solution of sodium hypochlorite (SHC). SHC intravenous infusions resulted in stabilization of arterial pressure within 24 hours. IEBO in the treatment of incipient bacteriemic shock improves prognosis.

[Puncture-drainage sanation of abdominal abscesses: is it sufficient treatment?]. / Punktsionno-dreniruiushchaia sanatsiia gnoinikov briushnoi polosti: ischerpyvaiushchii li metod lecheniia?.

Some anatomical grounds of postoperative extravisceral abscess formation, methods and results of their treatment, therapeutic policy in abscesses of parenchymatous organs are presented. 681 patients with abdominal abscess of various location were examined. Detailed analysis of ultrasonic and computed tomographic semiotics of postoperative extravisceral suppurative focus was carried out in 164 patients. The risk zones of extravisceral abscess formation in the conditions of changed visceral syntopy and destroyed ligamentic structures were determined. The efficiency of puncture-drainage treatment in different variants of subdiaphragmatic abscess was 89.4%, in abscess of omental bursa--83.3%. Intracavital prolonged proteolysis by immobilized proteinases (imosimase) was applied. The injection of imosimase in abscess cavity led to its content lysis, including the thick products of inflammation, improving their secretion through the drainage. In the majority of the patients, the puncture-drainage treatment of abscesses of various etiology and pathogenesis is an adequate method of sanation.

[Medichronal lowers blood ethanol and acetaldehyde and restores the concentration of catecholamines in rat tissues]. / Medikhronal znyzhuie vmist etanolu ta atsetal'dehidu v krovi i vidnovliuie kontsentratsiiu katekholaminiv u tkanynakh shchuriv.

Variation of ethanol and acetaldehyde concentrations in blood, catecholamines in hypothalamus, brain stem and hemispheres, heart and adrenal glands, serotonin in the same structures of the brain, thin intestine and blood in rats was studied. Isolated action of medichronal during 10 days against the background of prolonged administration of moderate doses of ethanol significantly lowered ethanol and acetaldehyde concentration in the animal blood. Medichronal increased the level of noradrenaline, lowered under the conditions of ethanol intoxication in the hypothalamus, and increased adrenalina level in the heart; noradrenaline level in adrenal glands is restored. The amount of serotonin in the blood and tissues increased under the conditions of ethanol intoxication did not vary under the action of medichronal. The obtained results indicate to pronounced detoxication influence of medichronal. One of the mechanisms of its action is normalizing the catecholamine changes caused by the ethanol intoxication in tissues.

[The conditions for performing extracorporeal lithotripsy with different physicochemical compositions of the urinary stones]. / Usloviia provedeniia distantsionnoi litotripsii pri razlichnom fiziko-khimicheskom sostave mochevykh kamnei.

Remote impulse lithotripsy (RIL) parameters were assessed when crushing uroliths with the use of URAT-II lithotriptor. The results were referred to the concrement size, location and physicochemical properties. A total of 365 examinations were made. Disintegration of large concrements, including coral calculi occurred in response to less number of impulses than it was necessary for destruction of concrements up to 1.5 cm in size. The hardest were vitlokite and cystine stones. The lowest traumaticity was observed in disintegration of the concrements composed of struvite and carbonatapatite.

[The new anti-alcohol preparation medikhronal: changes in the level of ethanol, acetaldehyde and monoamines during the treatment of alcoholism patients]. / Novyi antialkogol'nyi preparat medikhronal: izmenenie soderzhaniia étanola, atsetal'degida i monoaminov pri lechenii bol'nykh alkogolizmom.

Treating patients with stage-II alcoholism with medichronal and unithiol sharply decreases blood levels of alcohol and acetaldehyde. Medichronal restores the decreased level of norepinephrine excretion in 25% of patients and the increased level of epinephrine in 43%. The high baseline concentration of serotonin dropped in 41%, however, its level remained higher than in the controls. There was a negative correlation between the changes of ethanol concentrations and the excretion of norepinephrine. There was a direct dependence between the changes in the norepinephrine/epinephrine ratio and the serotonin concentration.

[The action of medikhronal (glucose-glycine-formate) on catecholamine excretion in alcoholics]. / Deistvie medikhronala (gliuzkozo-glitsin-formiata) na ékskretsiiu katekholaminov u bol'nykh alkogolizmom.

The application of the novel antialcoholic drug Medichronal mainly results in normalization of dyscatecholaminemias, by lowering or elevating noradrenaline or adrenaline levels. This effect is similar to that produced by unitiol that is conventionally used in nacrological care. The changes in catecholamine levels occur in various dyscatecholaminemias characterized by a high or low excretion of biogenic monoamines. Before and after therapy, in some patients, the concentrations of catecholamines do not differ from those in healthy persons.

[The bacteriological and immunological efficacy of biosporin in nonspecific ulcerative colitis]. / Bakteriologicheskaia i immunologicheskaia éffektivnost' biosporina pri nespetsificheskom iazvennom kolite.

The results from examination of intestinal microflora and immune status in 75 patients with nonspecific ulcerative colitis with different degree of disease seriousness are presented. The deviations in the composition of normal microflora were primarily expressed in a decrease of the number of bifidobacteria. In 64.9% of patients the disease proceeded against the background of deficit of T-cellular immunity link. The sufficiently expressed bacteriological and immunological efficiency of complex therapy including preparation from spore-forming bacteria as a normalizer of microflora is shown.

[The effect of protamine on cholesterol, triglycerides and blood serum proteins during the dynamics of experimental hypercholesterolemia]. / Deistvie protamina na kholesterin, triglitseridy i belki syvorotki krovi v dinamike éksperimental'noi giperkholesterinemii.

Content of total proteins, cholesterol, and triglycerides was studied in the blood serum and lipoprotein fractions of rabbits after administration of protamine within 1, 3 and 7 months of hypercholesterolemia development. The protamine effect was accompanied by "equalization" of cholesterol and triglyceride concentrations, as compared with their alteration in hypercholesterolemia, by stabilization of their content in various periods of the disease, but at the higher level than that of intact animals. The phenomenon observed occurred mainly in lipid components of LDL and VLDL subfractions. Analysis of alterations in the content of cholesterol and proteins in HDL and apo B-containing fractions showed that administration of protamine during long-term hypercholesterolemia led to development of antiatherogenic symptoms. Proatherogenic alterations in lipoprotein composition, developed during hypercholesterolemia, appear to be inhibited by protamine.

[Enterosorption in the treatment of ulcerative colitis patients]. / Enterosorbtsiia v lechenii bol'nykh iazvennym kolitom.

Enterodes and splenin were used in the treatment of patients with ulcerative colitis (idiopathic proctocolitis) in association with routine drug treatment while steroids were not employed. Results indicate increase of the therapeutic effect due to improvement of regeneration of the intestine and improvement of the immunity status.

[The action of teturam and acetaldehyde on the tissue catecholamines of rats]. / Deistvie teturama i atsetal'degida na katekholaminy tkanei krys.

The results of the isolated and combined effects of teturam and acetaldehyde indicate that a decrease of noradrenaline levels in the brain and heart is induced by teturam. An increase of adrenaline content in the hypothalamus is determined by acetaldehyde. In the adrenals the agents produce the oppositely directed changes in catecholamine content. Against the background of chronic administration of acetaldehyde teturam potentiates its influence on changes in noradrenaline levels in all tissues except the adrenals and adrenaline levels in the hypothalamus and brain stem.

[Effect of protamine on the composition of blood lipoproteins at various periods of hypercholesterolemia in rabbits]. / Diia protaminu na sklad lipoproteïniv krovi u rizni periody giperkholesterynemiï u kroliv.

Protamine has been studied for its effect on changes in the protein-lipid composition of main fractions of blood lipoproteins in different periods (1- and 7-months) of hypercholesterolemia in rabbits. Daily introduction of protamine to intact animals for 30 days did not change the studied parameters. Results of analysis of the protamine action against the background of the 1-month hypercholesterolemia testify to an increase of the protein and cholesterin concentration in apoB-containing lipoproteins. A moderate increase of the cholesterin concentration in the HDL composition has been observed, the protein concentration remaining at high level. The atherogenic diet for 7 month has induced in animals subjected to its dyslipoproteinemia and atherosclerosis of vessels. Under these conditions protamine has inhibited "proatherogenic" alterations in the composition of lipoproteins. The concentration of protein, cholesterin and triglycerides in the apoB-containing fractions decreased and the concentration of total cholesterin and triglycerides in the blood serum fell considerably.

[Acetaldehyde in the cardiotoxic mechanism of action of alcohol: effects on cardiac and adrenal catecholamines]. / Atsetal'degid v mekhanizmakh kardiotoksicheskikh éffektov alkogolia: deistvie na katekholaminy serdtsa i nadpochechnikov.

How the levels of rat cardiac, adrenal, and epinephrine and norepinephrine change with acetaldehyde and ethanol given alone or in combination during their acute and chronic administration was examined. In some series of experiments, acetaldehyde and ethanol were used in combination with teturam. The findings suggest that the coupling of two negative processes--a reduction in cardiac norepinephrine levels, which is associated mainly with the effects produced by ethanol, and an increase in epinephrine concentrations, which is apparently induced by acetaldehyde--may be one of the mechanisms responsible for cardiovascular abnormalities in alcoholic intoxication. Acetaldehyde modifies the regulatory effect of the sympathoadrenal system on cardiac function, causing adrenal catecholamine imbalance.