Hungarian Immunology

[History of immunology in Hungary Part V]

KARASSZON Dénes, CSABA Béla

MAY 10, 2004

Hungarian Immunology - 2004;3(04)

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Hungarian Immunology

[Immunology of Felty’s syndrome]

BÁLINT Géza, BÁLINT Péter

[Felty’s syndrome can be regarded as “super-rheumatoid” disease. Immungenetically the syndrome is much more homogenous, than rheumatoid arthritis. HLA-DRB1*0401 antigen is present in 83% of the patients. Felty’s syndrome develops usually after a longer course of rheumatoid arthritis, in 1% of rheumatoid patients. Rheumatoid arthritis patients with long lasting unexplained neutropenia can be diagnosed having Felty’s syndrome, even without detectable splenomegaly. On the contrary, rheumatoid arthritis with splenomegaly, but without present or previous neutropenia with unexplained origin cannot be regarded as having Felty’s syndrome. Inspite of the fact, that the arthritis of Felty’s syndrome can be inactive, because of the neutropenia and increased risk of recurrent infections, the patients should be kept under tight supervision, and should be properly treated, if required. Immunologically Felty’s syndrome is characterized by rheumatoid factor positivity in 95-100%, ANA positivity in 50-100%, antihistone positivity in 63-83%. Antibodies against dsDNA rarely, but against ssDNA frequently occur. No anti Sm and interestingly no anti Ro and anti La antibodies can be detected inspite of the high incidence of associated Sjögren’s syndrome. Immunoglobulin levels are higher and complement levels are lower, than in rheumatoid arthritis. Circulating immuncomplex level is usually high. Non-specific antineutrophil anticitoplasmatic antibodies can be found in high percentage. The neutropenia of Felty’s syndrome can be either caused by increased IgG neutrophilic binding activity or by inhibition of the granulocytes colony growing in the bone marrow, by peripheral blood mononuclear cells. Expansion of large granular lymphocytes can be seen in 30-40% of patients with Felty’s syndrome. Large granular lymphocyte syndrome is not rarely associated with rheumatoid arthritis. The neutrophil account is normal or elevated in this syndrome, but splenomegaly occurs. These cases are called as pseudo Felty’s syndrome. The patients with Felty's syndrome suffering from recurrent infections required treatment even if the arthritis is inactive. Methotrexate treatment should be started first, if this treatment fails, other disease modifying drugs or colony stimulating factor can be given. There is no experience with other biological treatments. In treatment of resistant cases splenectomy is indicated. Non-steroid anti-inflammatory drugs should be better avoided.]

Hungarian Immunology

[Changes of immune system in the elderly]

MEKKEL Gabriella, BARTA Zsolt, BAKÓ Gyula

[”Immunosenescence” means the change of the immune system with ageing. Ageing of the immun system is a physiological process, based on complex immunregulatory alterations, which make the elderly more susceptible for infections, malignant and autoimmun diseases. It’s important to differentiate the primary (physiological) and secondory (caused by diseases) modifications of immunregulatory system. After reviewing the strict Senieur protocoll, which makes a basis for gerontological/geriatry studies, the authors survey in details the changes of the each cell types of the immune system. The impaired functions of the NK (natural killer) and PMN (polymorphonuclear) cells and macrophags (as the parts of innate immunity), the decreased numbers of T lymphocyts, and the less specific antibodies produced by B lymphocyts (as the part of the acquired immunity) together are responsible for the increased susceptibility of the elderly to infections, and for the higher prevalence of malignancies in elderly patients. The imbalance of pro- and anti-inflammatoric cytokines and the TH1- TH2 shift also play role in this process. Appearance of autoantigens and the increased autoreactivity are in the background of the frequency autoimmun diseases in the elderly. The development of malignant diseases is a complex process, caused by on the one hand the changes in innate immunity, on the other the decreased activity of suppressor mechanisms. These changes are not correctly known, but the literature grows increasingly. The good command of the molecular mechanisms provides the facilities to improve remedial interventions in the future.]

Hungarian Immunology

[Autumn international conferences]

SZEKANECZ Zoltán

Hungarian Immunology

[Investigation of activated T-cells by non-Hodgkin’s lymphoma patients]

VÁRÓCZY László, GERGELY Lajos, ALEKSZA Magdolna, MILTÉNYI Zsófia, ILLÉS Árpád

[BACKGROUND - The immune system has several mechanisms to fight against developing malignant cell clones in the host, one of them is the activated T-cell response. Both CD4+ helper and CD8+ cytotoxic T-cells bear CD69 and HLA-DR molecules as important surface activation markers. AIM - Our aim was to determine, how the ratio of activated T-cells change in the peripheral blood of non-Hodgkin-lymphoma patients during the periods of polychemotherapy. PATIENTS AND METHODS - We used the peripheral blood samples of 43 non-Hodgkin-lymphoma’s patients (20 females, 23 males, mean age 52.4 years). We determined the level of CD3+/HLADR+ and CD3+/CD69+ T-cell subsets before, during and after the periods of polychemotherapy, using the methods of immunofluorescence stain and flow cytometry. RESULTS - We found the ratio of CD3+/HLA-DR+ cells significantly higher in non-Hodgkin-lymphoma’s patients before treatment compared to healthy controls (10.63% vs. 2.97%, p<0.001). During the period of polychemotherapy, this ratio began to increase significantly (16.94% vs. 10.63%, p=0.006). The level of CD3+/CD69+ cells did not change significantly. After treatment, the ratio of activated T-cells decreased, however, we detected significantly higher rate of CD3+/HLA-DR+ lymphocytes in patients who relapsed within one year than in those who stayed in remission (9.55% vs. 20.62%, p<0.001). CONCLUSION - Investigation of CD3+/HLA-DR+ activated T-cells might be a promising method to determine the immune defence and this way the prognostics of lymphoma patients.]

Hungarian Immunology

[Therapeutic treatment of rheumatoid arthritis by gene therapy-induced apoptosis]

WOODS M. James, VOLIN V. Michael

[Gene therapy was initially conceptualized as a treatment for individuals with genetic disorders, where defective genes would be replaced with functional ones. This concept was eventually broadened to include the use of gene therapy as a delivery mechanism for gene products effective in the treatment of diseases. The latter use of gene therapy, essentially as a drug delivery mechanism, was recognized to be particularly useful in the treatment of rheumatoid arthritis because it may have many advantages over traditional therapies. Two groups of target genes that are potentially useful for gene transfer include soluble inflammatory mediators that in theory could suppress the inflammatory process, and apoptotic mediators that may induce cell death, thereby suppressing the accumulation of inflammatory cells in the joint. To date the former group of target genes has received most of the attention, but it is the latter group of apoptosis-inducing targets that will be discussed in this review. We will focus our discussion on target genes that have shown success at inducing apoptosis in animal models of arthritis and will also include discussion of the apoptotic pathways that are altered in the attempts to reduce inflamed synovial tissue.]

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[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

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[The connection between the socioeconomic status and stroke in Budapest]

VASTAGH Ildikó, SZŐCS Ildikó, OBERFRANK Ferenc, AJTAY András, BERECZKI Dániel

[The well-known gap bet­ween stroke mortality of Eastern and Western Euro­pean countries may reflect the effect of socioeconomic diffe­rences. Such a gap may be present between neighborhoods of different wealth within one city. We set forth to compare age distribution, incidence, case fatality, mortality, and risk factor profile of stroke patients of the poorest (District 8) and wealthiest (District 12) districts of Budapest. We synthesize the results of our former comparative epidemiological investigations focusing on the association of socioeconomic background and features of stroke in two districts of the capital city of Hungary. The “Budapest District 8–12 project” pointed out the younger age of stroke patients of the poorer district, and established that the prevalence of smoking, alcohol-consumption, and untreated hypertension is also higher in District 8. The “Six Years in Two Districts” project involving 4779 patients with a 10-year follow-up revealed higher incidence, case fatality and mortality of stroke in the less wealthy district. The younger patients of the poorer region show higher risk-factor prevalence, die younger and their fatality grows faster during long-term follow-up. The higher prevalence of risk factors and the higher fatality of the younger age groups in the socioeconomically deprived district reflect the higher vulnerability of the population in District 8. The missing link between poverty and stroke outcome seems to be lifestyle risk-factors and lack of adherence to primary preventive efforts. Public health campaigns on stroke prevention should focus on the young generation of socioeconomi­cally deprived neighborhoods. ]

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[The Comprehensive Aphasia Test in Hungarian]

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[In this paper we present the Comprehensive Aphasia Test-Hungarian (CAT-H; Zakariás and Lukács, in preparation), an assessment tool newly adapted to Hungarian, currently under standardisation. The test is suitable for the assessment of an acquired language disorder, post-stroke aphasia. The aims of this paper are to present 1) the main characteristics of the test, its areas of application, and the process of the Hungarian adaptation and standardisation, 2) the first results from a sample of Hungarian people with aphasia and healthy controls. Ninety-nine people with aphasia, mostly with unilateral, left hemisphere stroke, and 19 neurologically intact control participants were administered the CAT-H. In addition, we developed a questionnaire assessing demographic and clinical information. The CAT-H consists of two parts, a Cognitive Screening Test and a Language Test. People with aphasia performed significantly worse than the control group in all language and almost all cognitive subtests of the CAT-H. Consistent with our expectations, the control group performed close to ceiling in all subtests, whereas people with aphasia exhibited great individual variability both in the language and the cognitive subtests. In addition, we found that age, time post-onset, and type of stroke were associated with cognitive and linguistic abilities measured by the CAT-H. Our results and our experiences clearly show that the CAT-H provides a comprehensive profile of a person’s impaired and intact language abilities and can be used to monitor language recovery as well as to screen for basic cognitive deficits in aphasia. We hope that the CAT-H will be a unique resource for rehabilitation professionals and aphasia researchers in aphasia assessment and diagnostics in Hungary. ]

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Cases of inborn errors of metabolism diagnosed in children with autism

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Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

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[Thiazide- or thiazide-like diuretics should be used in the treatment of patients with hypertension? Particularities of the situation in Hungary]

VÁLYI Péter

[Diuretics have remained the cornerstone of the antihypertensive treatment since their widespreading in the 1960s. According to the 2018 ESC/ESH Guidelines for the management of arterial hypertension, in the absence of evidence from direct comparator trials and recognizing that many of the approved single-pill combinations are based on hydrochlorothiazide, this drug and thiazide-like indapamide can be considered suitable antihypertensive agents. In the 2018 Hungarian guidelines indapamide is named as the most efficacious diuretic in the treatment of patients with hypertension. The aim of the publication is redefining thiazide- and thiazide-like diuretic use in the treatment of hypertensive patients, with particular attention to presently available hydrochlorothia­zide and indapamide, and their combination drugs in Hungary.]