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Clinical Neuroscience

MARCH 30, 2021

hirdetés

[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

BOCZÁN Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette, SZÉLL Márta, KARCAGI Veronika, ZÁDORI Dénes, MOLNÁR Mária Judit

[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]

Lege Artis Medicinae

OCTOBER 21, 2020

[Atherosclerosis: an ancient process in a new interpretation]

REIBER István

[The progress of atherosclerosis starts in childhood and lasts until the body dies. Most cardiovascular diseases and deaths can be traced back to atherosclerotic vascular changes. The process is thousands of years old, but its complex pathophysiology becomes recognized and realised only nowadays. Based on the evidence available today, atherosclerosis is such a chronic inflammatory disease of large- and medium-sized arteries, which is characterized by lipoproteins and immune cells transformed through oxidative and other changes and subendothelial accumulation of extracellular matrix. Innate and adaptive immunity provide a complex regulating system of atherogenesis, which while directing specifically the pro-atherogenic inflammatory and atheroprotective anti-inflammatory processes intensify plaque progression or even stabilize them respectively. With our growing knowledge about the pathology of atherogenesis, we can further improve the identification of cardiovascular risk conditions and apply more personalized therapeutic strategies.]

Clinical Oncology

APRIL 10, 2019

[Metals and cancer]

VETLÉNYI Enikő, RÁCZ Gergely

[We often tend to forget about our environment when looking for the origin of a disease. Inhaled air, drinking water and food, substances in contact with the skin all have an effect on the human body. Metals are indispensable parts of our everyday lives, their mining, processing and use cause a continuous exposure to them. Metal exert their effects on the body in various ways. Many of them are essential for maintaining homeostasis, but excessive or harmful metal intake can lead to health damage, including tumour formation through multiple attack points. Metals substitute each other during different transport processes and in the structure of proteins, they cause oxidative stress and bind to DNA, thereby damaging it. Applying them appropriately, the proapoptotic effect of the metal compounds is brought to the fore, thus becoming a therapeutic tool for tumours. Nowadays, platinum(II) compounds are widely used as chemotherapeutic agents and there are many ongoing studies to fi nd metal compounds with an ideal therapeutic and side-effect profi le. The aims of this article were to draw the attention to the dangers of metals in relation to cancer and to highlight their diverse application possibilities in current and future cancer therapy and diagnostics.]

Lege Artis Medicinae

NOVEMBER 15, 2019

[The association between advanced age and peripheral arterial disease]

KOLOSSVÁRY Endre, FARKAS Katalin

[The high-income countries are characterized by the aging of the residents (epidemiological transition) and the change of the disease patterns that are recognized in the population (epidemiological transition). In that sense, considering the cardiovascular diseases in the last few decades, a decline of mortality of acute, fatal conditions (stroke, myocardial infarction) is notable. All these factors contributed to the recognition of the importance of peripheral arterial disease and related problems in the aging popula­tion of the affected people. The high prevalence, the decline of quality of life associated with compromised lower limb circula­tion, the risk of the limb loss, the challenge of rehabilitation and the high mortality represent a significant and increasing burden to the healthcare. The review aims to analyse the relation of the aging population and peripheral arterial disease, addressing the aspects of epidemiology, diagnostics, and therapy. ]

Hypertension and nephrology

APRIL 29, 2021

[Covid-19 and peripheral arterial disease]

FARKAS Katalin

[Patients with peripheral vascular disease (PAD) are negatively affected by the coronavirus epidemic in several ways. Fewer-than-usual doctor-patient encounters make it more difficult to detect disease or disease progression. Outbreaks due to the epidemic reinforce a sedentary lifestyle that can mask the symptoms of PAD through lack of exercise. Another risk is that patients with cardiovascular disease are at risk for severe Covid-19 disease, and have a significantly increased risk of mortality. In the case of home treatment of Covid-19 infection in a patient with PAD, close observation is required to make a timely decision on the need for hospitalization. Vaccination, which is gradually available to all PAD patients, could be the solution to prevent the disease.]

Lege Artis Medicinae

MAY 01, 2000

[Hemodynamic changes during simulated orthostasis]

LÁSZLÓ Zoltán, RÖSSEL Andreas, HINGHOFER-SZALKAY G. Helmut

[INTRODUCTION – „Lower body negative pressure" (LBNP, simulated orthostasis) is an experimental method to simulate orthostatic stress. METHODS – The aim of this study was to quantitate hemodynamic and thoracic bioimpedance changes with four levels of LBNP (-15, -35, -55, -65 mmHg) in 7 healthy men in supine position 20 min before (pre-LBNP), 30 min during, and 20 min after suction as well as without suction (rest control). RESULTS - Thoracic bioimpedance computed stroke volume index continuously decreased with time by the end of simulated orthostasis of -15, -35, -55 and -65 mmHg. The decrease of cardiac index was largest at LBNP of -15 and -35, where the highest relative increase of total peripheral resistance index occurred. Mean arterial pressure did not change in any systematic way with lower LBNP levels, but increased at the end of LBNP -55 and -65. Heart rate remained unchanged at LBNP-15, but increased continuously with simulated orthostasis duration at higher levels by the end of LBNP-35, -55, and -65. Having finished simulated orthostasis, heart rate fell below pre-stimulus levels and also below rest control levels for 25 min; computed stroke volume index transiently returned to pre-stimulus levels, whereas mean arterial pressure increased above pre-LBNP level after finishing the stimulus. CONCLUSIONS - Different levels of lower body negative pressure produced quantitatively different time course and dose-response patterns and subjects remained non-hypotensive up to -65 mmHg suction. The heart rate was depressed after simulated orthostasis, while arterial blood pressure and impedance computed total peripheral resistance exceeded pre-stimulus levels indicating an altered cardiovascular (neuro-humoral) state after 30 min of simulated orthostasis. ]

Hypertension and nephrology

APRIL 24, 2020

[Arterial hypertension and atrial fibrillation - the most important risk factors for stroke in clinical practice ]

LUDOVIT Gaspar, VESTENICKA Veronika, CAPRNDA Martin

[Vascular stroke is a very frequent cause of morbidity and mortality, and in patients who suffered stroke subsequent long-term neurological deficit of greater or lesser extent is an important factor. Numerous clinical and epidemiological studies confirmed that elevated systemic blood pressure is among the main risk factors of both ischemic and hemorrhagic vascular stroke, the effects of arterial hypertension being very complex including morphological and functional changes in vessels and vascular circulation. In our retrospective analysis of 218 patients hospitalized for stroke we found arterial hypertension in 91.2% of subjects and atrial fibrillation in 32.1% of subjects. 182 patients (83.5%) have been diagnosed with ischemic stroke and 36 patients (16.5%) with hemorrhagic stroke. In the group of patients with atrial fibrillation, only 33 patients (47.1%) were treated by anticoagulants, what points out an inadequate indication of anticoagulant treatment when considering the stroke risk calculation for atrial fibrillation (CHA2DS2- VASc Score) and bleeding risk (HAS-BLED Calculator for Atrial Fibrillation). It is also noteworthy that in the group of patients with anticoagulant therapy who have developed ischemic stroke in spite of this treatment, we found that in 48.5% the treatment was underdosed and therefore ineffective. Our work points to the need to improve the effective management of arterial hypertension and atrial fibrillation, the most common modifiable factors of vascular strokes.]

Hypertension and nephrology

APRIL 24, 2020

[Cardiovascular risk assessment in chronic kidney disease, significance of left ventricular myocardial mass index]

SÁGI Balázs, KÉSŐI István, VAS Tibor, CSIKY Botond, NAGY Judit, KOVÁCS Tibor

[Introduction: Earlier studies have shown that cardiovascular (CV) mortality and morbidity in chronic kidney disease (CKD) often exceed their average population, and left ventricular hypertrophy (LVH) is an independent risk factor for CV disease. However, in CKD, the relationship between LVH, arterial stiffness (AS) and renal function has not yet been fully elucidated. Little data is available on their prognostic role. Aims of our study a) cross-sectional examination of the relationship between left ventricular mass index (LVMI), arterial vascular stiffness, and renal function, b) in our follow-up study, clarification of the LVMI, the prognostic role of AS in patients with CKD, IgA nephropathy (IgAN). Methods: In our cross-sectional study, 79 IgAN patients were examined in our clinic. The myocardial mass index (LVMI) was determined using an estimation formula after echocardiographic measurements. Arterial stiffness was measured using a photoplethizmography technique (PulseTrace) and characterized by the stiffness index (SI). The MDRD formula was used to estimate renal function (GFR) (eGFR, ml/min/1.73 m2). In the prognostic study the primary combined endpoint was total mortality, the most important CV events (stroke, myocardial infarction or cardiovascular interventions such as revascularization) and end stage renal disease. Secondary endpoints were CV and renal endpoints separately. Results: Of the 79 patients included in our cross-sectional study, 50 were men, with an average age of 46 ± 11 years. The mean value of LVMI was 106.66 ± 22.98 g/m2. Patients were divided into groups of 115 g/m2 for males considered to be abnormal and 95 g/m2 for women. LVMI is closely correlated with SI and inversely with eGFR (corr. coeff: 0.358; p <0.05 or -0.526; p <0.001). In case of LVH, SI was significantly higher in both sexes (p = 0.005 in males, p = 0.04 in females). In case of higher LVMI, renal function was significantly lower (p = 0.002 in males, p = 0.01 in females). Metabolic syndrome occurred in several cases in both sexes with LVH, but the difference was only significant in male patients (males 6 vs. 10, p = 0.008; females 2 vs. 4, p = 0.29). In our follow-up study, the presence of LVH in men significantly reduced survival in both primary and secondary endpoints, whereas in women there was no significant difference. Conclusion: In IgAN decreasing of renal function is closely related to left ventricular hypertrophy and vascular stiffness, as well as a close relationship was found between LVMI and AS. Reduced renal function is associated with an increase in LVMI and an increase in AS, which may result in a worse prognosis for both CV and renal outcomes. The underlying role of all these can be assumed to be a common vascular and myocardial pathological remodeling.]

Clinical Neuroscience

MARCH 30, 2020

[Diseases with peripheral motor symptoms ]

DELI Gabriella, KOMÁROMY Hedvig, PÁL Endre, PFUND Zoltán

[Diseases with peripheral motor symptoms are a rare, but important subgroup of the all peripheral neuropathies, radiculopathies and neuronopathies. In these mostly progressive neuropathies, the clinical features include pure motor symptoms with weakness and wasting of the striated muscles. The differentiation of these diseases is frequently a challenge for qualified clinical neurologists. A careful history taking, the disease time course, the findings of routine clinical physical examination and the electrophysiological studies are all necessary in the diagnostic procedure. The aim of this publication is to overview the clinical characteristics of the pure motor peripheral neuropathies, to consider the diagnostic steps and the differential diagnosis, and finally to summarize the treatment options. ]

Hypertension and nephrology

DECEMBER 10, 2018

[Prognostic role of arterial stiffness in IgA nephropathy]

SÁGI Balázs, KÉSŐI István, VAS Tibor, CSIKY Botond, KOVÁCS Tibor, NAGY Judit

[Background: Arterial stiffness has a prognostic role in chronic cardiovascular diseases. Pulse wave velocity (PWV) determined by the carotid-femoral pulse detection is accepted as a gold standard method. Further diagnostic procedures are in use to assess the arterial stiffness including the finger photoplethysmography. The prognostic role of this method is limited in chronic renal diseases. The goal of our investigation was to determine the prognostic significance of the stiffness index (SIDVP) measured by the photoplethysmographic method in IgA nephropathy. Patients and methods: One hundred and three histologically proved IgA nephropathy patients with chronic kidney disease stage 1-4 were investigated (67 male, 36 female, 45 ± 11 years) and followed for an average 65 (6-107) months. The stiffness index was determined by the volume alteration of the digital artery during the cardiac cycle (Pulse Trace system, Micro Medical, Gilingham, Kent, UK). The primary combined end point was total mortality, major cardiovascular events (stroke, myocardial infarction or cardiovascular procedure, for example revascularisation) plus achieving end stage renal disease. The secondary end points were cardiovascular and renal end points alone. Results: The patients with increased stiffness index (> 10 m/s) had significantly more combined primary end point events (10/60 vs. 19/43, P = 0.015). In case of the secondary end points the renal end points were significantly more frequent in patients with higher stiffness index. Stiffness index has also proved to be an independent predictor on survival from other cardiovascular risk factors (age, hypertension, diabetes, obesity, lipid disturbances and decrease of renal function) using the Cox regression model in IgA nephropathy. Every 1 m/s increase in stiffness index resulted a 17% gain in the occurrence of the combined primary end point. Conclusions: Stiffness index determined by finger photoplethysmography is an eligible parameter to assess the prognosis in IgA nephropathy. Increased stiffness index in IgA nephropathy seems to be a good prognostic tool for identification of higher risk patients.]