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Biomedical subjects

Z Balogh

Publications and source records attributed to Z Balogh.

At least 19 recordsLinked to original sources

Health-related quality of life of patients with juvenile idiopathic arthritis coming from 3 different geographic areas. The PRINTO multinational quality of life cohort study.

OBJECTIVES: To compare health-related quality of life (HRQL) and to identify clinical determinants for poor HRQL of patients with juvenile idiopathic arthritis (JIA) coming from three geographic areas. METHODS: The HRQL was assessed through the Child Health Questionnaire (CHQ). A total of 30 countries were included grouped in three geographic areas: 16 countries in Western Europe; 10 in Eastern Europe; and four in Latin America. Potential determinants of poor HRQL included demographic data, physician's and parent's global assessments, measures of joint inflammation, disability as measured by Childhood Health Assessment Questionnaire (CHAQ) and erythrocyte sedimentation rate. Poor HRQL was defined as a CHQ physical summary score (PhS) or psychosocial summary score (PsS) <2 S.D. from that of healthy children. RESULTS: A total of 3167 patients with JIA, younger than 18 yrs, were included in this study. The most affected health concepts (<2 S.D. from healthy children) that differentiate the three geographic areas include physical functioning, bodily pain/discomfort, global health, general health perception, change in health with respect to the previous year, self-esteem and family cohesion. Determinants for poor HRQL were similar across geographic areas with physical well-being mostly affected by the level of disability while the psychosocial well-being by the intensity of pain. CONCLUSION: We found that patients with JIA have a significant impairment of their HRQL compared with healthy peers, particularly in the physical domain. Disability and pain are the most important determinants of physical and psychosocial well-being irrespective of the geographic area of origin.

Adolescent↗

Richter's and prolymphocytic transformation of chronic lymphocytic leukemia are associated with high mRNA expression of activation-induced cytidine deaminase and aberrant somatic hypermutation.

Chronic lymphocytic leukemia (CLL) is an indolent B-cell non-Hodgkin's lymphoma that may transform into higher-grade lymphoma. The transformation involves an increased number of prolymphocytic cells, termed prolymphocytic transformation (PLT) or the development of diffuse large B-cell lymphoma (DLBL), also referred to as Richter's transformation (RT). To analyze whether activation-induced cytidine deaminase (AID), which is essential for somatic hypermutation (SHM) of normal B-cells, and malfunction of SHM termed aberrant somatic hypermutation (ASHM) are associated with higher-grade transformation of CLL, AID mRNA expression and the mutation pattern of c-MYC, PAX-5 and RhoH genes were analyzed in eight cases of CLL without transformation and in 21 cases that showed RT or PLT. Chronic lymphocytic leukemia cases, which showed no transformation or eventually transformed into higher-grade lymphoma, showed low levels of AID mRNA expression and low frequency of mutations of c-MYC, PAX-5 and RhoH genes. In both RT and PLT, high-levels of AID mRNA expression and high-frequency mutations of c-MYC, PAX-5 and RhoH genes were detected. These results indicate that AID expression and ASHM are associated with higher-grade transformation of CLL and provide further evidences that AID expression and ASHM may be activated during the clonal history of B-cell lymphomas.

Cell Transformation, Neoplastic↗

National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary--clinical characteristics and disease course of 44 patients with juvenile dermatomyositis.

Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs. Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM). A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Although we found favourable survival probability, further investigations are needed to assess functional outcome.

Adolescent↗

Prospective study to evaluate the influence of FAST on trauma patient management.

BACKGROUND: Previous studies have concentrated on the accuracy of Focused Assessment with Sonography in Trauma (FAST), but evaluation of whether FAST changes subsequent management has not been fully assessed. METHODS: This prospective study compared 419 trauma admissions in two groups, FAST and no-FAST, for demographics, time of resuscitation, and action after resuscitation. The 194 patients undergoing FAST had their management plan specified before, and confirmed after, FAST was performed to assess for change in management. To ensure scan consistency and to minimize bias, criteria were established to define an adequate FAST. RESULTS: FAST was performed in 194 patients (46%), assessing for free fluid. Management was changed in 59 cases (32.8%) after FAST. Laparotomy was prevented in 1 patient, computed tomography was prevented in 23 patients, and diagnostic peritoneal lavage was prevented in 15 patients. Computed tomography rates were reduced from 47% to 34% and diagnostic peritoneal lavage rates were reduced from 9% to 1%. CONCLUSIONS: FAST plays a key role in trauma, changing subsequent management in an appreciable number of patients.

Adolescent↗

PRINTO/PRES international website for families of children with rheumatic diseases: www.pediatric-rheumatology.printo.it.

OBJECTIVE: To prepare a website for families and health professionals containing up to date information about paediatric rheumatic diseases (PRD). METHODS: Firstly, paediatric rheumatology centres and family self help associations were surveyed to characterise current clinical practice of physicians providing care for children with PRD, research activities, and training facilities of each centre. Secondly, international consensus was reached on the content of the website. Finally, the website was developed and the texts translated. RESULTS: The web page contains three main sections: (a) description for families of the characteristics of 15 PRD; (b) list of paediatric rheumatology centres; (c) contact information for family self help associations. A version for 45 countries in 52 languages (with another three in progress) is now available on the web. 291 surveys from 171 centres and 102 family associations were received from 42 countries. The median proportion of time spent in paediatric practice in the centres examined was 100%, with 70% of this time dedicated to paediatric rheumatology. 90% of the centres were willing to perform clinical trials in the future. CONCLUSIONS: The PRINTO/PRES website provides a well defined and competent set of information about PRD, with appropriate multiple translated versions and easy web navigational direction.

Child↗

Correlation between the activities of lipoprotein lipase and paraoxonase in type 2 diabetes mellitus.

UNLABELLED: In type 2 diabetes mellitus the decreased catabolism of triglyceride-rich lipoproteins as a consequence of mainly the decreased lipoprotein lipase activity results in hypertriglyceridaemia and other lipoprotein alterations promoting atherosclerosis. The high-density lipoprotein-associated enzyme, paraoxonase, prevents the oxidation of low-density lipoprotein, which is an antiatherogenic effect. AIM: to examine the relation between the activities of enzymes influencing HDL remodelling- LPL and PON- in type 2 diabetes mellitus. METHODS: 56 newly diagnosed type 2 diabetic patients and 39 healthy controls were involved in the study. The serum PON activity was measured spectrophotometrically using paraoxone as substrate. PON phenotype was determined by the dual substrate method, PON mass was measured by ELISA. The determination of lipoprotein lipase activity was performed using 3H-triolein. RESULTS: We noticed smaller PON activity decrease in our newly diagnosed diabetic subjects compared to the previous studies which investigated the alteration of enzyme activity after a longer duration of diabetes mellitus. The lipoprotein lipase activity showed a positive correlation with PON activity (r=0.43; P<0.02). Interestingly, the PON activity of the homozygous-low activity group did not correlate with the LPL activity, while in the heterozygous and homozygous-high activity groups there was a significantly positive correlation (r=0.51; P<0.05) between PON and LPL activity. CONCLUSION: Besides lipid alterations, the metabolic changes of type 2 diabetes mellitus influence the reduction of the antioxidant capacity of HDL by remodelling HDL and decreasing PON activity via modification of lipoprotein lipase activity, which might contribute to accelerated atherosclerosis.

Aryldialkylphosphatase↗

Effect of short term treatment with simvastatin and atorvastatin on lipids and paraoxonase activity in patients with hyperlipoproteinaemia.

OBJECTIVE: High-density lipoprotein (HDL)-associated paraoxonase (PON) activity may play an important role in the inhibition of low-density lipoprotein (LDL) oxidation. Previous studies have demonstrated that serum PON activity is decreased in patients with hyperlipoproteinaemia and coronary heart disease. The study presented here examined the effect of short-term treatment with simvastatin and atorvastatin on lipids and PON activity in patients with hyperlipoproteinaemia. RESEARCH DESIGN AND METHODS: A prospective, non-blinded, single-group, cross-over, comparative trial was performed. Following an 8-week dietary run-in period, 49 patients (23 men and 26 women, mean age: 59.8 +/- 7.9 years) with Fredrickson type IIa. and IIb. hyperlipoproteinaemias were randomized to receive either simvastatin 20 mg/day or atorvastatin 10 mg/day for 3 months. Following an 8-week washout period, patients were crossed-over to receive the other drug for a further 3 months. Serum lipids were measured and serum PON activity was determined spectrophotometrically using paraoxon as a substrate. RESULTS: Simvastatin treatment significantly reduced serum cholesterol, LDL-cholesterol (LDL-C) and apolipoprotein (apo) B levels (p < 0.001). Atorvastatin had a more pronounced cholesterol, LDL-C- and apo B-lowering effect (p < 0.001) compared with simvastatin. Both statins also significantly reduced serum triglyceride levels (p < 0.01). Simvastatin and atorvastatin caused no significant change in the levels of HDL-cholesterol (HDL-C) and apo A1. HDL-associated PON activity did not change significantly after simvastatin therapy, but significantly increased after atorvastatin treatment (p < 0.05). CONCLUSIONS: Short-term administration of simvastatin did not increase PON activity. Atorvastatin treatment had a favourable effect on lipid profile and increased the activity of HDL-associated PON.

Adult↗

The effect of micronised fenofibrate on paraoxonase activity in patients with coronary heart disease.

OBJECTIVE: To evaluate the effect of micronised fenofibrate on serum paraoxonase (PON) and lipoprotein levels in coronary heart disease patients with type IIb hyperlipidemia. PATIENTS AND METHODS: Fifty-two patients were investigated for the three-month effect of 200 mg per day micronised fenofibrate on the serum enzyme activity and concentration of PON and their relationship with serum lipids, high-density lipoprotein (HDL-C) parameters. RESULTS: Serum paraoxonase activity was lower in CHD patients with type IIb hyperlipoproteinemia. During the three-month study it was observed that following treatment with micronised fenofibrate, serum triglyceride and cholesterol levels decreased, while HDL-C increased significantly (p<0.001). Low-density lipoprotein (p<0.05) and apolipoprotein B-100 (p<0.01) decreased, while HDL constituent apolipoprotein A-I (p<0.05) increased after micronised fenofibrate treatment. The HDL-associated paraoxonase specific activity increased significantly (p<0.05). To assess whether the increased PON activity was due to elevated HDL and apoA-I level, we standardized PON activity for HDL and apoA-I concentrations. The standardized values for HDL (PON/HDL) increased (p<0.05) while the PON/apoA-I ratio did not change significantly. CONCLUSION: Three months of treatment with micronised fenofibrate is thought to normalize lipid profile and improve antioxidant status by increasing serum paraoxonase activity in these patients.

Adult↗

Disturbed regulation of cholesterol synthesis in monocytes of obese patients with hypercholesterolemia.

The aim of the present study was to clarify the influence of obesity on the functions of low-density lipoprotein receptors (LDL-R) and 3-hydroxy-3-methylglutarate-coenyzme A (HMG-CoA) reductase both in healthy control subjects and in patients with hypercholesterolemia (HC). Experiments were performed on monocytes of 15 non-obese (C I) and 11 obese (C II) healthy control subjects and on 22 non-obese (HC I) and 26 obese (HC II) patients with HC. [(125)I]LDL was used to determine LDL-R activity by measuring binding and intracellular degradation. The rate of endogenous cholesterol synthesis was measured using [(14)C]acetate incorporation into the cholesterol fraction of monocytes. The binding ability of [(125)I]LDL was identical across all groups. The [(14)C]acetate incorporation in resting monocytes was increased only in obese HC group. The 50-microg/mL LDL protein-induced inhibition of [(14)C]acetate incorporation was significantly diminished (P <.001) in the same group. A strong positive correlation was detected between the [(14)C]acetate incorporation by resting cells and LDL-induced inhibition in all groups except the obese HC group, in which their correlation was negative (P <.001). Furthermore, in the obese HC group, a significant positive correlation was detected between body mass index (BMI) and the basal level of [(14)C]acetate incorporation, whereas a negative correlation was found between BMI and LDL-induced inhibition of [(14)C]acetate incorporation. The present data suggest that in patients with HC the concomitant obesity results in dysregulation of cholesterol homeostasis, which may contribute to the accelerated atherosclerosis.

Acetates↗

Gemfibrozil increases paraoxonase activity in type 2 diabetic patients. A new hypothesis of the beneficial action of fibrates?

OBJECTIVE: The constellation of elevated triglycerides and decreased high-density lipoprotein is recognised as a risk factor for CAD and constitutes the major dyslipidemia in type 2 diabetes. The high-density lipoprotein associated paraoxonase activity can inhibit low-density lipoprotein oxidation and has an antiatherogenic effect. To determine the effect of gemfibrozil on the dyslipidemia and serum paraoxonase activity in patients with type 2 diabetes. MATERIAL AND METHODS: Fifty-six type 2 diabetic patients with associated hypertriglyceridemia were involved. They were investigated for the effect of twice daily 600 mg of gemfibrozil on serum cholesterol, triglycerides, apolipoproteins, fibrinogen level, body mass index and glycated hemoglobin. Serum paraoxonase activity was measured spectrophotometrically. RESULTS: After three months, it was observed that under the effect of gemfibrozil, serum triglyceride level was significantly decreased (from median: 3.46 mmol/l quartiles: q1=2.92 q3=5.28 to median 2.20 mmol/l quartiles: q1=1.79 q3=3.65; p<0.001) while protective high-density lipoprotein (from 1.02 +/- 0.22 mmol/l to 1.13 +/- 0.28 mmol/l; p=0.05) and apolipoprotein A(1) (from 1.56 +/- 0.33 g/l to 1.72 +/- 0.29; p<0.01) levels were significantly increased. Serum paraoxonase activity was found to be significantly increased (from median: 100.2 U/l quartiles: q1=60.1 q3=152.7 to median 118.7 U/l quartiles: q1=80.1 q3=171.0; p<0.001) after gemfibrozil treatment. The total cholesterol, low-density lipoprotein, apolipoprotein B, lipoprotein (a), glycated hemoglobin and fibrinogen levels were not significantly changed during the three-month treatment period. The standardized paraoxonase activity for HDL and apo A(1) were not significantly changed. Paraoxonase activity did not correlate with the concentration of glycohemoglobin and the duration of diabetes. CONCLUSION: Twice daily administration of 600 mg of gemfibrozil is effective in type 2 diabetic patients with associated hypertriglyceridemia. It favorably lowers lipid levels, and improves antioxidant status by increasing serum paraoxonase activity.

Adult↗

[Various clinical patterns of relapsing polychondritis in six cases].

Relapsing polychondritis is a relatively rare disease characterized by episodic inflammation and progressive destruction of cartilage involving ears, nasal and laryngotracheal cartilage, cardiovascular system and the eyes. The increasing awareness of its clinically distinct has resulted in recognition of at least 550 reported cases. Six cases are reported to demonstrate the wide variety of clinical pattern. The most common features of the disease are auricular and nasal cartilage inflammation and nondeforming arthritis. Ocular symptoms and vasculitis is relatively rare. Two cases of relapsing polychondritis with laryngotracheobronchial manifestations illustrate the severe clinical features of the disease. Relapsing polychondritis may associate with diverse forms of connective tissue disease, such as rheumatoid arthritis. It seems interesting to note the onset in childhood. Treatment has been primarily symptomatic. In situations of mild symptoms, initial treatment is with nonsteroidal antiinflammatory drugs. For cases with serious manifestation, corticosteroids and immunosuppressants are indicated.

Adult↗

[Effect of fibrates on lipid metabolism and the blood coagulation system].

The authors summarised the complex effects of fibrates on atherogen dyslipidaemia and the blood coagulation system, as well as the results of previous intervention studies with fibrates. They also give an overview of the favourable molecular effects of fibrates and the role of peroxysoma proliferator activated receptor-alpha (PPAR-alpha).

Arteriosclerosis↗

Formation and inhibition of heterocyclic aromatic amines in fried ground beef patties.

The effect of vitamin E and oleoresin rosemary on heterocyclic aromatic amine (HAA) formation in fried ground beef patties was studied. Patties were fried at three temperatures (175 degrees C, 200 degrees C, 225 degrees C) for 6 and 10 min/side to determine the conditions for optimum HAA formation. HAAs were isolated by solid phase extraction and quantitated by HPLC. Greatest concentrations were generated when patties were fried at 225 degrees C for 10 min/side, 31.4 ng/g 2-amino-1-methyl-6-phenylimidazo[4,5-b]pyridine (PhIP) and 5.8 ng/g 2-amino-3,8-dimethylimidazo[4,5-f]quinoxaline (MeIQx). Vitamin E, when used at two concentrations (1% and 10% based on fat content) and added directly to the ground beef patties, reduced PhIP concentrations in the cooked patties by 69% and 72%, respectively. Smaller but more variable reductions were achieved for MeIQx. Comparable inhibition of HAA formation was achieved by the direct addition of vitamin E (1% based on fat content) to the surface of the patties before frying. Concentrations of five HAAs studied were all significantly reduced (P<0.006), with average reductions ranging from 45% to 75%. Oleoresin rosemary, when used at two concentrations (1% and 10% based on fat content), reduced PhIP formation by 44%.

Amines↗

NISS predicts postinjury multiple organ failure better than the ISS.

BACKGROUND: The Injury Severity Score (ISS) has been observed consistently to be a robust predictor of postinjury multiple organ failure (MOF). However, the ISS fails to account for multiple injuries to the same body region. Recently, the "new" ISS (NISS) has been proposed to address this shortcoming. Preliminary studies suggest the NISS is superior to the ISS in predicting trauma mortality. Our purpose was to determine whether the NISS is a better predictor of postinjury MOF than the ISS. METHODS: A total of 558 patients admitted to our Level I trauma center with ISS > 15, age > 15 years, and survival > 48 hours were prospectively identified; 101 (18%) developed postinjury MOF. Data characterizing postinjury MOF were collected, and the NISS was calculated retrospectively. The ISS and NISS were compared as univariate predictors of MOF. Multivariate analysis was used to determine whether substitution of NISS for ISS resulted in a superior predictive model. RESULTS: In 295 patients (53%), the NISS was greater than the ISS. This subgroup of patients experienced a greater frequency of MOF (26.7% vs. 8.3%, p < 0.0001), a higher mortality (12.8% vs. 4.9%, p < 0.001), and a higher early transfusion requirement (6.7 U vs. 3.6 U, p < 0.0001) compared with the group in which NISS equaled ISS. Moreover, the NISS yielded better separation between patients with and without MOF reflected by the greater difference in median NISS scores compared with ISS scores. The multivariate predictive model, including NISS, showed a better goodness of fit compared with the same model that included ISS. CONCLUSIONS: The NISS is superior to the ISS in the prediction of postinjury MOF. This measure of tissue injury severity should replace the ISS in trauma research.

Adult↗

Disturbed LDL and scavenger receptor functions in monocytes from chronic haemodialysed patients.

BACKGROUND: The most frequent complication in patients with end-stage renal failure on chronic haemodialysis (HD) treatment is atherosclerosis, i.e. the different forms of heart and vascular diseases. The complete disorder of serum lipid and lipoprotein patterns is well demonstrated, whereas our knowledge about the low-density lipoprotein (LDL) and scavenger receptor expression and function are poorly understood. METHODS: In our current work, LDL and scavenger receptor expression and functions were simultaneously studied in monocytes obtained from 15 healthy male control subjects and from 11 chronic HD male patients applied with (125)I-labelled LDL, isolated from healthy volunteers. To study the scavenger LDL receptors, labelled acetylated LDL (acLDL) was used. RESULTS: LDL binding to the monocytes of the HD-group was found to be decreased in comparison to that of the controls. As a result, the 50 microg LDL protein-induced inhibition of endogenous cholesterol synthesis was also diminished. In contrast, acLDL binding was greatly increased, though it could trigger only a low apoE synthesis. Consequently the number of cholesterol inclusions in monocytes was increased. CONCLUSIONS: The disturbed expression and function of LDL and scavenger receptors both may play significant roles in pathogenesis of cardiovascular complications in chronic HD patients. Based on our present results, it can be assumed that dysfunction of scavenger receptors is at the centre of cardiovascular complications of HD patients with renal failure.

Anticholesteremic Agents↗

A study of increased levels of soluble vascular cell adhesion molecule-1 (sVCAM-1) in the cerebrospinal fluid of patients with multiple sclerosis and systemic lupus erythematosus.

The concentration of soluble vascular cell adhesion molecules (sVCAM-1) of serum and cerebrospinal fluid (CSF) was measured in clinically selected multiple sclerosis (MS) and systemic lupus erythematosus (SLE) patients, using an ELISA assay. The mean sVCAM-1 concentration in the serum of SLE patients was higher than normal. The mean CSF sVCAM-1 concentration was increased in the MS as in the SLE group. On analysis, the data suggests that there are some similarities in the immunological effects of these two different diseases of the central nervous system. A longitudinal analysis of the CSF is requested.

Adolescent↗

Effects of selective nitric oxide synthase inhibition in hyperdynamic endotoxemia in dogs.

OBJECTIVES: Our aims were to investigate the systemic hemodynamic effects of constitutive endothelial nitric oxide synthase (eNOS) and inducible NOS (iNOS) inhibitors in hyperdynamic endotoxemia. PATIENTS AND METHODS: Group 1 comprised sham-operated controls, while in group 2, 3 and 4, a hyperdynamic circulatory reaction was elicited by a 2-hour infusion of Escherichia coli endotoxin (ETX) in a dose of 5.3 microg/kg. The animals in group 3 were treated with 12. 5 mg/kg nonselective NOS inhibitor N-omega-nitro-L-arginine methyl ester (L-NAME), and those in group 4 with 2 mg/kg of the specific iNOS inhibitor S-methyl-isothiourea (SMT). Mean arterial pressure (MAP), cardiac output (CO) and myocardial contractility (MC) were measured, and total peripheral resistance (TPR) was calculated. The eNOS and iNOS activities were determined in myocardial biopsy samples taken after 8 h of endotoxemia. RESULTS: ETX induced significant decreases in TPR and MAP, a transient myocardial depression, and increased the myocardial eNOS and iNOS activities. L-NAME decreased the activities of both isoenzymes, increased MC but induced a fall in CO. SMT inhibited iNOS by 60%, without influencing the eNOS activity, increased MAP and contractility in the early phase of endotoxemia, and induced only a slight decrease in CO. CONCLUSIONS: Nonselective NOS inhibition restores the arterial pressure and exerts a positive inotropic effect, but decreases CO. SMT selectively decreases the iNOS activation without disturbing the vasoregulatory function of the eNOS-derived nitric oxide in hyperdynamic endotoxemia in the dog.

Animals↗