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Clinical Neuroscience

NOVEMBER 30, 2018

[Effects of neural therapy on quality of live in patients with inoperable lower extremity artery disease ]

MOLNÁR István, DEÁK Botond Zsolt, HEGYI Gabriella, KOVÁCS Zoltán, KAPÓCS Gábor, SZŐKE Henrik

[Objectives - Our aim was to evaluate the effects of percutaneous neurolysis of lumbal sympathetic ganglions on pain and the resulting changes in quality of life with validated objective and subjective methods. To follow the adverse effects and complications of the procedure. Materials and methods - A prospective, non-randomized, interventional, clinical cohort study under real life conditons was conducted. The time of the observation was 6 months. Palliative neural therapy was performed to reduce the ischemic pain of the affected leg of the patients involved in the study. Prior to treatment and after 35 days, Visual Analogue Scale (VAS) was used to measure the intensity of lower limb pain. The related changes in the quality of life were followed by a general 36-Item Short-Form Health Survey (SF-36) questionnaire. We measured the changes of the patients’ skin temperature and ankle/arm index. The post-treatment results were compared to the pre-treatment results. We compared the results of objective and subjective measures. We followed the side effects and complications of the pain therapy. Each of the examined subjects had obliterative (Fontaine II/b stage) arterial disease of the lower limbs, in which no revascularization intervention was feasible and their ischemic pain was of VAS≥7. Results - Data of 124 patients (69 male, 55 female) could be evaluated. The decrease in intensity of limb pain in the post-treatment period was significant (p=0.001). Quality of life also indicated a significant improvement (p=0.004). Changes in skin temperature and ankle/arm index demonstrated significant improvement (p≤0.005): skin temperature increased from 27.6°C to 31.2°C, the ankle/arm index inceased from 0.67 to 0.83 on average. Changes in objective and subjective measures correlated with each other. No worthening of symptoms, serious adverse events or complications were observed. Conclusion - The chemical denervation of the lumbar sympathetic ganglions with percutaneous application is a minimally invasive intervention, useful in outpatient care, which can be well tolerated by the patient without any significant side effect or complication. Its hyperaemic effect and the pain reduction of the leg can improve the quality of life of the patients.]

Lege Artis Medicinae

OCTOBER 20, 2011

[Pulmonary arterial hypertension in systemic autoimmune diseases]

VÉGH Judit, ZEHER Margit

[Pulmonary arterial hypertension is a rare disease, but it occurs more often in systemic autoimmune diseases, where it represents one of the most severe, life-threatening complications. Its development is due to an immunoregulatory disorder characteristic to systemic diseases, persistent inflammation and the subsequent endothelial dysfunction, the presence of pathogenic autoantibodies, smooth muscle cell dysfunction and complex angiogenetic disorder. As a consequence of endothelial cell dysfunction, the balance between regulatory factors of vasoconstriction and vasodilation is disrupted. Intimal hyperplasia, endothelial cell proliferation, media hypertrophy and local thrombus formation can be observed and one of the main pathomorphological characteristic features, plexiform lesion develops, leading to obliterative vasculopathy. A more severe form of the disease develops in systemic sclerosis, which is explained by the main pathophysiological elements of scleroderma, namely immunoregulatory disorder, vasculopathy and fibroblast dysfunction. It is not easy to monitor the disease in these cases, because the deterioration can be caused by many other factors as well. Therefore, beseides the usual examinations, biomarkers and screening methods have a significant role. Treatment is not simple either, since no wellapplicable algorithms are available. In many disorders (systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis), effective immunosuppressive therapy started in time is crucial, whereas in case of systemic sclerosis, the principles of therapy applied for the idiopathic form should be followed.]

Lege Artis Medicinae

SEPTEMBER 21, 2009

[Up-to-date management of systemic sclerosis]

SZŰCS Gabriella

[Systemic sclerosis is a chronic autoimmune disease characterized by three major features: widespread fibrosis in the skin and internal organs, a non-inflammatory small vessel obliterative vasculopathy and immunological activation with disease-specific autoantibodies. It is necessary to take a systematic approach to the diagnosis and evaluation of each case in order to provide appropriate treatment. Disease-modifying approaches can be classified according to the underlying pathogenic process. Thus vascular therapies include agents used for Raynaud’s phenomenon, critical digital ischaemia and organ-based vascular complications such as scleroderma renal crisis and pulmonary hypertension. Immunosuppressive drugs are used in lung involvement or rapid skin progression. The results of different anti-fibrotic therapies are controversial. Finally in managing organ-based manifestations and complications a multidisciplinary approach to the therapy is useful with patient education as an integral component of successful management.]

Lege Artis Medicinae

JANUARY 20, 2011

[Autoimmune pancreatitis in Hungary: a national multicenter study]

CZAKÓ László, GYÖKERES Tibor, TAKÁCS Tamás, TOPA Lajos, SAHIN Péter, DUBRAVCSIK Zsolt, SZEPES Attila, PAP Ákos, FÖLDESI Imre, TISZLAVICZ László, WITTMANN Tibor

[BACKGROUND - Autoimmune pancreatitis (AIP) is an increasingly recognised, special form of chronic pancreatitis, which greatly differs from other forms of chronic pancreatitis. Most papers on this condition have been published in Japan. METHODS - In our multicenter study, we aim to present the characteristics (demographics, clinical symptoms, laboratory and morphological findings, extrapancreatic symptoms, response to therapy, remission) of the first 13 Hungarian cases of AIP. RESULTS - The mean age at presentation was 44.2 years (range: 19-74); 54% of patients were women. New-onset mild abdominal pain (77%), weight loss (38%) and jaundice (31%) were the most common symptoms, with ulcerative colitis as the most frequent (38%) extrapancreatic manifestation. Diffuse pancreatic swelling was seen in 7 patients (54%), and a focal mass in 5 (38%). Pancreatic duct strictures were present in all patients. Serum immunoglobulin-G4 level was elevated in 71% of the patients in whom it was measured. All percutaneous core biopsies (4 patients) and surgical specimens (2 patients), and 2 of the 4 biopsies of the papilla of Vater revealed hystological findings typical for AIP: periductal, diffuse lymphoplasmacytic infiltration, marked interstitial fibrosis, and obliterative phlebitis. Immunostaining revealed IgG4-positive plasma cells in 57% of the patients in whom it was examined. Granulocytic epithelial lesions (GEL) were detected in 3 patients. These patients were younger (mean age 34 years), 66% were women and 6% had ulcerative colitis, whereas the mean age of patients without GEL was 65 years, and the majority of them were men. Steroid treatment resulted in remission of the symptoms in all patients. Because of suspicion of pancreatic tumour, 2 patients with focal AIP underwent partial pancreatectomy. Symptoms relapsed in one patient, but an increased dose of steroid resulted in a remission, which was maintained by azathioprine therapy. CONCLUSIONS - In our first Hungarian cases, we have confirmed previously reported characteristics of AIP. AIP with GEL is frequent among our patients; this condition is more frequent among women and younger patients, and is often associated with ulcerative colitis. If AIP is suspected, the performance of percutaneous biopsy is highly recommended. The therapeutic response to steroid therapy was excellent.]

Lege Artis Medicinae

JULY 14, 2008

[UNSOLVED THERAPY - COMPLEX TREATMENT OF BUERGER’S DISEASE]

SZOMJÁK Edit, DÉR Henrietta, KEREKES György, VERES Katalin, DEZSŐ Balázs, TAKÁCS István, TÓTH Judit, MÓZES Péter, SOLTÉSZ Pál

[INTRODUCTION - Buerger’s disease is a nonatherosclerotic, segmental, inflammatory, progressive obliterative disease of the blood vessels that most commonly affects the small and medium-sized arteries and also the veins of the lower limb. It is strongly associated with tobacco use. CASE REPORT - A 37-year-old heavy smoker forest worker presented with severe gangrenous changes on the fingers as the upper limb manifestations of Buerger’s disease, which required acral amputation. After complex medical treatment, pharmacological sympathectomy on the left side and surgical sympathectomy on the right side, bilateral conserving necrectomy, cessation of smoking and 2 cycles of cyclophosphamid, the patient's status significantly improved, his pain diminished and, although to a limited extent, he could use both hands. CONCLUSIONS - Buerger’s disease is a disorder with unclear aethiology and is difficult to diagnose. Despite of a good prognosis, a causal treatment is not yet possible, so any antithrombotic, haemorheological, vasodilator or immunosuppressive therapy that have been reported to have beneficial effect should be considered for treatment.]

Lege Artis Medicinae

JANUARY 20, 2007

[PATHOLOGICAL FEATURES OF SYSTEMIC SCLEROSIS]

VARJÚ Cecília, KUMÁNOVICS Gábor, CZIRJÁK László

[Systemic sclerosis is characterized by fibrosis and subsequent atrophy of the skin and several internal organs as well as by generalized obliterative vasculopathy. The ethiology of systemic sclerosis is not quite clear yet, but the role of certain environmental factors, genetic properties and microchimaerism has been proven. Vasculopathy is a key feature that includes both functional changes (Raynaud's phenomenon) and morphological alterations (lesion of the endothel). The triggering event is the activation of endothelial cells. This is followed by an autoimmune inflammatory process causing vascular lesion, which will eventually lead to progressive pathologic fibrosis with increased deposition of collagen and intercellular matrix proteins. Normal tissues of vital internal organs will gradually loose structure, become atrophic and irreversibly damaged. In the treatment of systemic sclerosis the most significant achievements of the past decade have been made in the therapy and prevention of scleroderma renal crisis, pulmonary arterial hypertension and other vascular complications, resulting in higher survival rates and better quality of life. In pulmonary fibrosis the beneficial effect of cyclophosphamide therapy has been proven. Today, research focuses on new therapeutic approaches based on the recently clarified molecular pathological processes, as well as on laboratory and clinical markers that predict the activity of the disease or the efficiency of therapy. The aim of the present paper is to review current knowledge on the pathology of systemic sclerosis and provide help in the diagnosis, therapy and follow-up of the disease.]

Hungarian Immunology

MARCH 20, 2006

[Clinical and immunoserological characteristics of mixed connective tissue disease (MCTD) associated with pulmonary arterial hypertension (PAH)]

VÉGH Judit, CSÍPŐ István, UDVARDY Miklós, KAPPELMAYER János, LAKOS Gabriella, ALEKSZA Magdolna, ZEHER Margit, SZEGEDI Gyula, BODOLAY Edit

[INTRODUCTION - The authors investigated the clinical characteristics, survival, accumulated damage index and immunoserological abnormalities in patients with mixed connective tissue disease (MCTD) associated with pulmonary arterial hypertension (PAH). PATIENTS AND METHODS - Anti-U1RNP autoantibodies, anti-endothelial cell antibodies, anti-cardiolipin antibodies and serum trombomodulin as well as von Willebrand factor antigen concentrations were measured in 25 patients with MCTD associated with PAH (11 right heart catheterization and 14 Doppler echocardiography) and in 154 MCTD patients without PAH. Changes in arterial pulmonary pressure were followed up by echocardiography. RESULTS - In the 25 patients PAH followed MCTD diagnosis in the average 11.6±4.5 years of the diseases. The probability of survival was lower in MCTD patients with PAH than in the 154 non-PAH MCTD patients (five years survival rate in MCTD with PAH: 73.39%, vs. 96.43% in non PAH MCTD; p<0.01; 10 years survival rate 86.74% vs. 93.25%; p<0.01). Anti-endothelial cell antibodies were more frequently present in MCTD patients sera with PAH than in non PAH MCTD (p<0.001). Serum trombomodulin and vWFAg levels were higher in MCTDPAH patients than in non PAH MCTD patients (trombomodulin:34.2±15.3 ng/ml vs. 11.8±6.5 ng/ml; p<0.001; vWFAg: 311.1±147% vs. 172.5± 141%. Significant correlations were noticed between the quantity of anti-endothelial cell antibodies and serum trombomodulin level (r=0.466) as well as the quantity of anti-endothelial cell antibodies and vWFAg serum level (r=0.550). CONCLUSION - Survival probability was worse for MCTD patients with PAH than for non PAH MCTD patients. Our results suggest that in MCTD the presence of anti-endothelial cell antibodies and endothelial cell activation may play a role in the development of pulmonary arterial hypertension and in the maintenance of obliterative vascular processes.]

Lege Artis Medicinae

SEPTEMBER 18, 2004

[THE VASCULAR BACKGROUND OF ERECTILE DYSFUNCTION]

TÓTH Károly

[With the new era of the medicaments, erectile dysfunction has become of great interest among sexual problems. Earlier, psychogenic origin was thought to be the main background, but today vasculogenetic reasons are have become equally important factors. Experimental data and clinical investigations demonstrate that erectile dysfunction is part and consequence of the same vascular process which is observed in aging and in atherosclerotic processes of the well-known arterial localisations (coronary arteries, cerebral arteries etc.). It was also common previously to associate erectile dysfunction with obliterative atherosclerotic disease but today endothel dysfunction, vascular remodelling and atherosclerosis are thought to be main pathogenetic factors affecting the whole arterial vascular system causing erectile dysfunction. Classic risk factors can also be observed in these patients, such as hypertension, dyslipidaemia, smoking and diabetes. Neurogenic factors are also involved in the development of erectile dysfunction. Based on this, erectile dysfunction is considered as the predictor of cardiovascular diseases preceding the general manifestation of the disease by 2-3 years.]

Hungarian Immunology

MARCH 20, 2009

[Features of serum carnitine ester profile in systemic sclerosis]

TALIÁN Csaba Gábor, KISS György Csaba, MELEGH Béla, CZIRJÁK László

[AIMS - Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, fibrosis, inflammatory changes, and late stage atrophy with obliterative vasculopathy. Clinically it has two distinct subtypes, limited (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). Carnitine is an essential metabolite with ester forming capability. We investigated the possible relationship between carnitine metabolism and systemic sclerosis, stratified by disease subgroups. PATIENTS AND METHODS - We performed comparative measurements of carnitine profile in 107 systemic sclerosis patients (78 lcSSc and 29 dcSSc) and 47 healthy controls using triple quadrupol ESI mass spectrometry. RESULTS - C2-, C3-, C8-, C10:1- and C10-carnitine ester levels were moderately decreased in the serum of the patients, while C5- and C6-carnitine ester concentrations increased in both types of the disease compared with controls; as a summary, these changes altogether led to reduced total carnitine ester concentrations. Free carnitine level remained unaffected. CONCLUSIONS - These results show that both types of SSc have a moderate influence on shortand medium chain carnitine ester metabolism, but likely do not affect the quantity of the free carnitine reserves.]