Search PubMed⌕ Search

Biomedical subjects

L Nemes

Publications and source records attributed to L Nemes.

At least 19 recordsLinked to original sources
PubMed2007

Evaluation of pharmacokinetics, efficacy and safety of Immunate solvent detergent in previously treated patients with severe haemophilia A.

Immunate Solvent Detergent (S/D) is a plasma derived, purified, human factor VIII (FVIII) - von Willebrand factor (VWF) complex subjected to two virus inactivation/removal processes: S/D and vapor heat treatment. This prospective, multicentre, three-part clinical study evaluated the pharmacokinetics (in comparison to the predecessor product Immunate), efficacy and safety of Immunate S/D in 56 previously treated patients with severe haemophilia A. Subjects received Immunate S/D on-demand, as a prophylactic regimen or both. The results of the pharmacokinetic population demonstrate that Immunate and Immunate S/D were equivalent with respect to the FVIII - and to the retrospectively VWF - parameters assessed. A total of 623 bleeding episodes were reported in 47/56 subjects. The duration of prophylaxis ranged from 0.1-5.2 months with a total of 175.6 months. The median number of bleeds per month in subjects on prophylaxis was 0 (range 0-10). Ninety-six percent of bleeding episodes were rated as having an excellent or good response. For most bleeding episodes (89%), subjects required only one infusion with a mean dose of 29.6 IU kg(-1). No FVIII inhibitory antibodies were observed in any subject. No related serious adverse events were reported. Thus, the introduction of S/D treatment did not alter the PK characteristics and function of VWF and FVIII molecules of Immunate S/D which is effective and safe for treatment of bleeding episodes, management of surgical procedures, and prophylaxis.

Detergents↗
PubMed2004

Towards the goal of prophylaxis: experience and treatment strategies from Sweden, France and Hungary.

The only form of haemophilia treatment that is able to prevent arthropathy and other consequences of bleeding symptoms in patients with severe haemophilia is prophylaxis started at an early age (primary prophylaxis). It is also highly beneficial for the psychological and social wellbeing of patients and their families. Scientific institutions and international organizations such as WHO, the World Federation of Hemophilia (WFH) and the National Haemophilia Foundation (NHF) have recommended that prophylaxis be considered optimum therapy. This paper discusses the barriers to prophylaxis, such as the perceived need, costs and availability, and difficulty of venous access, and describes the authors' experiences with the therapy.

Blood Coagulation Factors↗
PubMed2004

Impact of environmental and hereditary risk factors on the clinical manifestation of thrombophilia in homozygous carriers of factor V:G1691A.

BACKGROUND: Limited data exist on the clinical manifestations of homozygous factor (F)V:G1691A mutation (FV Leiden) and the impact of environmental and genetic risk factors. OBJECTIVES: To assess the contribution of these factors on the thrombophilic phenotype. PATIENTS AND METHODS: In a retrospective multicenter cohort study 165 individuals with homozygous FV:G1691A mutation, of whom 129 had previous venous thromboembolism (VTE), were included. To study the role of environmental risk factors, patients were compared by the use of a standardized questionnaire to 165 sex- and age-matched individuals (reference group A); of these, two had previous VTE. To assess the role of genetic risk factors, factor (F)II:G20210A and MTHFR:C677T were determined in individuals homozygous for FV:G1691A and in 177 healthy individuals without previous VTE (reference group B). RESULTS: The first VTE occurred significantly earlier in women (median age 25 years) than men (35.5 years). In 81% of women and 29% of men an environmental risk factor was present before first VTE. Oral contraceptives increased the risk of thrombosis 4-fold [odds ratio (OR) 4.0, 95% confidence interval (CI) 1.7, 10.4] in women with homozygous FV:G1691A. Postoperative and post-traumatic VTE as first manifestation occurred in 13% and 15% of surgical/traumatic events in patients and in 0.7% and 1.8% in reference group A, respectively (OR 19.7, 95% CI 2.5, 154 and OR 9.2, 95% CI 1.1, 79.4). Heterozygous FII:G20210A was more prevalent in symptomatic patients (11.7%) compared with reference group B (2.8%, OR 4.6, 95% CI 1.6, 13.2). The prevalence of homozygous MTHFR:C677T genotype was similar in patients and reference group B. CONCLUSIONS: Our study supports the concept of thrombophilia as a multifactorial disorder. The knowledge of coexisting factors predisposing to VTE is useful for medical advice for primary and secondary prophylaxis in these patients.

Adolescent↗
PubMed2001

Familial occurrence of the May-Hegglin anomaly: is the accompanying renal failure part of a new subentity?

We present two cases of the May-Hegglin anomaly discovered in a patient and one of her two sons. The female patient was known to have proteinuria from the age of 14 and was hospitalized in 1980, at the age of 25 years, because of hypertension and proteinuria (1.5 g/day). Thrombocytopenia was found with an abundance of megakaryocytes in the bone marrow. Both steroid treatment and splenectomy failed to ameliorate the thrombocytopenia, thought to be due to idiopathic thrombocytopenic purpura. Progressive renal failure, secondary hyperparathyroidism and uremic osteodystrophy were diagnosed in 1995. In January 1996, when she was hospitalized because of high-grade fever, we saw giant platelets and prominent blue inclusion bodies in almost all granulocytes in the peripheral blood smear. Electron microscopy confirmed the diagnosis of May-Hegglin anomaly in this patient and one of her sons, who at that time showed thrombocytopenia but no renal disease. Three years later, however, at the age of 15, the affected son was found to develop proteinuria. Coexpression of the May-Hegglin anomaly and renal disease, reported previously in a few other patients, may in fact represent a new subentity.

Adult↗
PubMed2001

A haemophilia A and B molecular genetic diagnostic programme in Hungary: a highly informative and cost-effective strategy.

Our aim was to set up a protocol in order to provide carrier and prenatal diagnosis to Hungarian haemophilia A (HA) and B (HB) patients and their relatives. For HA, a combination of direct mutation detection and some indirect marker analyses were used: the detection of the inversion mutation and analysis of three polymorphisms, BclI, IVS13 (CA)n and P39(CA)n. In severe cases, direct mutation detection was performed first. In inversion-negative severe cases and in moderate and mild cases, indirect methods were used. For carrier and prenatal diagnosis in HB, four polymorphisms, DdeI, TaqI, XmnI, and HhaI were examined. Our DNA bank contains samples from 50 HA families (34 severe, 15 moderate and one mild) and seven HB families from different parts of the country. In 100% of the HA cases either the gene inversion and/or at least one of the polymorphisms was found to be informative for carrier or prenatal diagnosis. In the HB cases, an informative marker was found in 95% of the cases (19 of 20). We conclude that these strategies are sufficient to make genetic diagnosis available to almost all HA and HB families in the region. This approach is highly informative and cost-effective, so it can be very useful in countries where direct sequencing of genes for factor VIII and IX is not available for routine diagnosis.

Chromosome Inversion↗
PubMed2000

Detection of CN radicals in DC nitrogen plasma used for deposition of CNx layers.

Emission spectra from a DC plasma discharge of nitrogen with a graphite cathode used for deposition of CNx layers were investigated in the visible range 350-900 nm. The spectra recorded at low and high resolution from both the negative glow and the positive column of the discharge were studied separately. All spectra are dominated by neutral and ionised N2 emission. In the positive column the violet band of the cyanogen (CN) radical was identified and analysed for vibrational structure. From a computer simulation of the rotationally resolved violet band, vibrational temperatures were derived and found to be in the intensity distribution for the nu = 0, 1 and 2 levels from thermal equilibrium. In the negative glow the strong N2+ features completely mask the spectral region of the violet band of CN. Conclusions were drawn concerning the CN formation by chemical sputtering, and the role of CN radicals in the formation of polymeric CNx layers of 1:1 = C:N stoichiometry.

Cyanides↗
PubMed2000

Pregnancy-associated risk for venous thromboembolism and pregnancy outcome in women homozygous for factor V Leiden.

INTRODUCTION: To evaluate the pregnancy-associated risk of venous thromboembolism and the risk of stillbirth and miscarriage a multicenter, retrospective and controlled study was conducted in women carrying the homozygous factor V Leiden mutation and in an agematched control group of women from the normal population. PATIENTS AND METHODS: In 64 homozygous (median age 44 years, range 21-75 years) and in 52 control women from five different centers data on venous thromboembolism and pregnancy outcome were obtained. RESULTS: The 64 homozygous women had in total 212 pregnancies, the 52 control women had 118 pregnancies. In homozygous women 65% of pregnancies ended with delivery of a viable infant, 15% with fetal loss (3.3% stillbirth, 12% miscarriage) and 20% by pregnancy termination. In the control women 75% of pregnancies ended with delivery of a viable infant, 12% with fetal loss (1.7% stillbirth, 10% miscarriage) and 13% by pregnancy termination. The differences were statistically not significant. Venous thromboembolism occurred significantly more often in the homozygous women, in 4.2% (9/212) during pregnancy and in 4.7% (10/212) after delivery or pregnancy termination. None of the control women had a thromboembolic episode. CONCLUSION: Our data indicate that women with homozygous factor V Leiden have a high probability for a favorable pregnancy outcome. The increased risk for venous thromboembolism during pregnancy and after delivery would favor heparin prophylaxis during and after pregnancy in women homozygous for factor V Leiden.

Adult↗
PubMed2000

New protocol for immune tolerance induction in acquired hemophilia.

BACKGROUND AND OBJECTIVES: Immune tolerance induction (ITI) regimens with human factor VIII concentrates are rarely if ever implemented in adult patients with auto-inhibitors, in contrast to alloantibody suppression, which is used primarily in young children with congenital hemophilia. On the basis of some earlier experience with synchronization of plasma exchange therapy of various autoimmune disorders we have developed a new aggressive protocol for the treatment of patients with acquired factor VIII (FVIII) antibody. We have evaluated the outcome of 14 consecutive nonhemophilic FVIII inhibitor patients treated in a single center with our ITI protocol between 1992 and 1999, comparing them to 6 historical control patients, treated with traditional immunosuppression therapy (steroid +/- cyclophosphamide) between 1988 and 1992. DESIGN AND METHODS: Our ITI protocol consists of three weeks of treatment with 1) human FVIII concentrates (30 U/kg/day for the 1st week, 20 U/kg/day for the 2nd, and 15 U/kg/day for the 3rd week), plus 2) iv. cyclophosphamide (200mg/day to a total dose of 2-3 grams), plus 3) methylprednisolone (100 mg/day iv. for one week and than tapering down the dose gradually over the next two weeks). The treatment of acute bleeding episodes in the two groups was not different. High purity and ultra-high purity factor VIII concentrates were used for the ITI. We performed aPTT and mixing tests before and after two hours of incubation, Bethesda inhibitor assay, porcine FVIII cross-reactivity, FVIII:C before and after FVIII administration (recovery), three times a week. The sex ratio and mean age (64 years for the ITI group versus 57 years for the controls), the initial and peak inhibitor titers, and residual FVIII: C values at the diagnosis were similar in the two groups. RESULTS: Eradication of the inhibitor occurred in 13/14 patients in the ITI vs. 4/6 patients in the control group. The main difference between the two groups was in the time needed for the complete disappearance of the inhibitor (4.6 weeks for ITI vs. 28.3 weeks for controls). In the ITI group we have observed only two relapses during the relatively long follow-up period (26 months), and in both cases the same re-induction protocol was successful again. No bleeding-related mortality occurred in this group in contrast to that of 33% in the controls. Apart from the well-known adverse effects of glucocorticoid therapy, we have observed only one patient with transient cytopenia. We have not seen any adverse event which could be attributed to the use of FVIII concentrates. INTERPRETATION AND CONCLUSIONS: We conclude that the ITI protocol described here is highly effective for the treatment of acquired hemophilia, induces quick therapeutic responses and favorably influences the underlying autoimmune disorder. We suggest that our ITI protocol is suitable for the eradication of idiopathic and autoimmune-associated FVIII autoantibodies in patients presenting with severe bleeding.

Adult↗
PubMed1999

Observation of the nu(6) + nu(9) Band of Ketene via Resonant Coriolis Interaction with nu(8).

We observed and analyzed a b-axis Coriolis resonance between higher J states of the nu(6) + nu(9) combination band and the nu(8) fundamental of ketene in the spectral region 940-970 cm(-1). The interaction resonantly couples K(a) = 1 states of the combination band to K(a) = 0 states of the fundamental and also affects K(a) = 1, 2 states in the fundamental. Due to the involvement of strongly asymmetry-split low K levels, the rotational constants and band origin of nu(6) + nu(9) could be accurately determined and are discussed in the light of high-quality anharmonic force fields. The Coriolis coupling parameter, zeta(b)(8,6+9), is very precisely determined. A smaller perturbation, which could not be fully analyzed, is tentatively attributed to K(a) = 2 upper states in the nu(5) + nu(9) band. Copyright 1999 Academic Press.

Journal Article↗
PubMed1999

May-Hegglin anomaly and pregnancy.

A hypertensive patients with thrombocytopenia is reported who had two pregnancies complicated by preeclampsia and cesarean deliveries without hemorrhage. During her first pregnancy corticosteroids were given for presumed autoimmune thrombocytopenia. Thereafter she was splenectomised. Ten years later May-Hegglin anomaly and renal failure were diagnosed. One of her children had easy bruising.

Adrenal Cortex Hormones↗
PubMed1999

[Not Available].

In the forties, the life of Hungarian analysts was broken by two historical turning points. Both critical periods follow first an ascending, then an abruptly falling line. During faschism, psychoanalysis had in Hungary the longest life. At the end of 1944, one quarter of Hungarian analysts became victims of the Hungarian nazis. The year 1945 started with a hope of revival, with the social acceptance of psychoanalysis. After a couple of years, the communist regime disolved the Society, and forced the members into an inner emigration of 15 years.

Communism↗
PubMed1998

[Clinical evaluation of factors VIII and IX manufactured in Hungary, based on results of the first half year].

The development of anti-factor VIII/IX antibodies (inhibitor-induction) and the transmission of viral infections are the most significant complications of haemophilia treatment. The Humafactor-8 and Humafactor-9 are high-purity pasteurized factor VIII and IX concentrates, which are produced from pooled plasma of Hungarian donors by ion-exchange chromatography. The clinical study has been accomplished in two steps: first we have demonstrated the biological efficacy of the concentrates in a phase IV trial. After that we followed 13 patients with severe haemophilia for 6 months in respect of virus-safety and inhibitor-induction. According to our results the recently developed domestic FVIII/FIX concentrates display appropriate biological activities and they are safe as blood-borne virus-transmission and immunogenicity are concerned.

Factor IX↗
PubMed1998

[Indirect methods in the genetic diagnosis of hemophilia A].

In haemophilia A (HA), besides the direct detection of the most common mutation of the factor VIII gene (the gene inversion), it was necessary to establish indirect methods which are suitable to reveal the pattern of inheritance of the genes examined, regardless of the mutations they carry. This task can be achieved by the analysis of DNA polymorphisms located within and in the near proximity of the factor VIII gene. For diagnostic purposes we used an RFLP and two microsatellite polymorphisms. The aim of our program is to provide carrier and also prenatal diagnostics for affected families. So far we completed the analyses of 15 HA patients and 68 of their family members, and we gave prenatal diagnoses in 3 cases. According to the information content of the polymorphisms used, we expect to be able to provide DNA diagnoses to 95% of the Hungarian HA families requesting the test.

Carrier State↗
PubMed1996

Investigation of the relative infectivity and pathogenicity of different hepatitis C virus genotypes in hemophiliacs.

To assess the relative infectivity and pathogenicity of variants of hepatitis C virus (HCV) genotypes, the distribution of genotypes in hemophilic patients who had been treated with nonvirally inactivated factor concentrates or cryoprecipitates prepared from local blood donors was compared with those found in the respective blood donor populations. Genotype frequencies differed markedly in the four countries investigated (Scotland, Hungary, South Africa, and Thailand) but in each, the HCV genotype distributions in hemophiliacs and blood donors were similar. In addition, HCV genotypes in recipients of commercially manufactured concentrates were similar to those found in the US general population. These findings provide no evidence that HCV genotypes differ significantly from each other in replication rate, transmissibility, or infectivity.

Hemophilia A↗
PubMed1989

Cercopithecus aethiops monkey as a reliable model for in vitro study of T cell depletion of bone marrow with Campath-1 plus complement.

T-lymphocyte markers of peripheral blood mononuclear cells (PBMCs) and bone marrow mononuclear cells (BMMCs) of C. aethiops monkeys were studied by using anti-human monoclonal antibodies. The results show that C. aethiops T lymphocytes express surface markers which react specifically with anti-human MoAbs including CD3, CD4, CD8, CD2. However, very few CD3-positive cells were found, in contrast to the abundance in CD8+ cells. There is a high conservation of receptors forming E rosettes with AET-treated SRBCs, and antigens reacting with the anti-human T and B cell monoclonal antibody (Campath-1). The present findings indicate that C. aethiops can be used as a new experimental model for studies on T-cell depletion from bone marrow with Campath-1 MoAb + rabbit C.

Animals↗
PubMed1988

[Two historical changes in the vicissitudes of Hungarian psychoanalysts in the forties].

Between 1940 and 1950, Hungarian history struck two blows at psychoanalysts--as a movement and in their personal lives. The paper relates several episodes from this period and briefly traces the birth and development of the "Budapest School". Between the two world wars, the small group of Hungarian analysts played a relatively important role in the international movement. During the years of Fascism it was in Hungary that psychoanalysis was able to survive the longest. But in 1944, a quarter of all the Hungarian analysts perished. After the war the analysts' activity was notable chiefly for its extensive nature but after a few years the Association was dissolved. The paper describes the analysts' problems and fluctuating fortunes and also their incessant efforts, inspired by the hope of a theoretical and practical renewal of Hungarian psycho-analysis.

History, 20th Century↗