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Hypertension and nephrology

SEPTEMBER 30, 2020

[Post-career development of cardiometabolic changes and hypertension in competitive athletes]

LELBACH Ádám, KÁNTOR Márk, KOLLER Ákos

[Regular physical activity is essential in delaying the aging processes (e.g. arterial remodelling – stiffening, metabolism, bodyweight), the beneficial effects of competitive sports – especially strength sports – according to the recent data of the literature are questionable. The beneficial effects of physical activity on the cardiovascular (CV) system are well known, however less is known regarding the delayed impacts of high intensity competitive sports on the CV system, especially after the sport career is over. This review summarizes the effects of active competitive sport and the post-career period on the cardiometabolic system with special attention to the systemic blood pressure and the development of metabolic syndrome. After sport career, the welldeveloped high performance cardiovascular- and metabolic system suddenly is much less used, but still supported by sport-level diet. It is well known that hypertension is a significant pathogenic factor in the development of cardiovascular diseases, characterized – among others – by reduced elasticity of large- and medium- sized vessels thereby importantly contributing to the development of systolic hypertension. Inflammation and thrombus formation both play an important role in the development of vascular injury and atherosclerosis. The increased tone of microvessels can impair the blood supply of certain organs, including the coronary circulation. It has been ample shown, that regular non-competitive, aerobic exercise activities are important factors in preventing hypertension. Such pathological changes become more evident after the development of post-career obesity, as well as the development of hypertension due to the activation of the renin-angiotensin system through sodium retention and other metabolic changes (increased glucose tolerance, insulin resistance, type II diabetes mellitus). It has been ample shown, that regular non-competitive, dynamic aerobic exercise activities are important factors in preventing hypertension. The frequency, intensity, type, and time (FITT) principle of exercise prescription is the first and common therapeutic approach, which represents the translation of cardiovascular basic science research results into hypertension treatment, thus can provide a personalized physical activity program/therapy according to medical needs not just for the post-career sportspersons, but the wide range of patients.]

Clinical Neuroscience

MAY 30, 2020

[The long-term follow-up of enzyme replacement treatment in late onset Pompe disease]

MOLNÁR Mária Judit, BORSOS Beáta, VÁRDI Visy Katalin, GROSZ Zoltán, SEBÕK Ágnes, DÉZSI Lívia, ALMÁSSY Zsuzsanna, KERÉNYI Levente, JOBBÁGY Zita, JÁVOR László, BIDLÓ Judit

[Pompe disease (PD) is a rare lysosomal disease caused by the deficient activity of acid alpha-glucosidase (GAA) enzyme due to mutations in the GAA gene. The enzymatic deficiency leads to the accumulation of glycogen within the lysosomes. Clinically, the disease has been classically classified in infantile and childhood/adult forms. Presently cc. close to 600 mutations distributed throughout the whole gene have been reported. The c.-32-13T>G splice mutation that is very common in patients of Caucasian origin affected by the childhood/adult form of the disease, with an allelic frequency close to 70%. Enzyme replacement treatment (ERT) is available for the patients with Pompe disease (Myozyme). In this paper, we are presenting the long term follow up of 13 adult onset cases treated more than 5 years. The longest follow up was 15 years. To evaluate the treatment efficacy, the 6 minutes walking test (6MWT) and the respiratory functions were monitored annually. The analysis revealed that at the beginning of ERT for 3-4 years the 6MWT had been generally increasing, then it declined, and after 10 years it was lower in 77% of the cases than it had been at the start of the treatment. In 23% of the cases the 6MWT increased during the follow up time. Only one of the patients become wheelchair dependent during the follow-up period. The respiratory function showed similar results especially in supine position. A high degree of variability was observed among patients in their responses to the treatment, which only partially associated with the antibody titer against the therapeutic protein. The efficacy of the ERT was associated with the type of the disease causing mutation, the baseline status of the disease, the lifestyle and the diet of the patient. The long-term follow up of the patients with innovative orphan drugs is necessary to really understand the value of the treatment and the need of the patients.]

Hypertension and nephrology

APRIL 24, 2020

[Possibilities and limitations. Dietary difficulties of chronic renal failure in childhood]

REUSZ György, SZABÓ Adrienn

[In chronic kidney disease (CKD), the role of the kidney in assuring homeostasis is gradually deteriorating. Besides fluid, electrolyte and hormonal disturbances, detoxification and control of blood pressure is insufficient without external help. In children, in addition to achieving equilibrium it is also essential to ensure optimal physical and cognitive/psychological development. Adequate calorie intake is a major determinant of growth during infancy. Among the therapeutic options it is essential to ensure a proper diet. In addition to reflecting the special needs of renal failure in its composition, it must be palatable for the child. Children with kidney disease should have a normal energy diet. Protein intake should not be reduced from the baseline recommendation, but lower phosphorus and high bioavailability should be preferred. A low sodium and potassium diet is recommended for a significant proportion of patients and is based on dietary advice. Further, diet planning may be problematic if the child has special dietary requirements and is in need of nasogastric tube feeding. Because diet planning is a complex task, it is difficult to achieve optimal protein supply and mineral restriction along with high energy intake. In such cases, enteral nutrition with special formulas/ drinks developed for pediatric nutrition may provide a solution.]

Clinical Neuroscience

MARCH 30, 2020

[New applications of conventional EEG analysis ]

CLEMENS Béla, PUSKÁS Szilvia

[Neurophysiological research suggests that the so-called “standard” EEG analysis has been confronted with new diagnostic challenges. The findings mainly concern the occurrence, the neurophysiological and clinical significance of epileptiform EEG discharges in several neurological and psychiatric disorders. In addition to well-known interictal and ictal discharges, a growing number of recently recognized epileptiform phenomena have been described. The first reports suggested that they might be relevant for the comprehensive description of epileptic dysfunction and might contribute to diagnosis and treatment as well. However, considerable improvement of present-day “standard” EEG technique is necessary to give an appropriate answer to most challenges. Reliable registration and quantitative assessment of well-known epileptiform transients require extended electrode coverage of the head (high-density EEG) and long-term recordings including waking and sleep states to estimate frequency and dyna­mics of targeted activities. Computer-based automatic event detection is preferable to spare time and cost of the evaluation. The authors review recent progress concerning epidemiology, neurophysiology and clinical impact of well-known epileptiform transients and candidate epileptiform activities in neurological and psychiatric conditions. However, recent results need confirmation in large patient populations; therefore, research should not be restricted to a few central laboratories.]

Hypertension and nephrology

SEPTEMBER 10, 2019

[Kidney diet and the patient compliance issue ]

LADÁNYI Erzsébet

[There have been significant developments in the field of nephrology and dialysis as for the science and technology are concerned in the past decades. However, CKD patients still show high mortality and morbidity. From among the several factors determining the long-term outcome of CKD patients metabolic disorder and malnutrition play an important role. Malnutrition is often not diagnosed or is not paid enough attention to in the complex treatment of CKD patients. It is important to make both the patients and clinical staff more aware of proper nutrition and importance of prevention and treatment of malnutrition, respectively. The early diagnosis and treatment of malnutrition is of utmost importance in CKD patients. The long term renal failure and the accompanying malnutrition have a negative impact on their long term outcome and quality of life. Since the malnutrition causes a lot of complications, it is indispensable for dietitians and nephrologists to work closely together. Patient compliance is a determining factor in the successful implementation of renal diet.]

Lege Artis Medicinae

MARCH 20, 2019

[The modern therapy of lipid disorders in the daily practice ]

REIBER István

[Elevated LDL-C and/or triglycerides and decreased HDL-C levels contribute to development of vascular lesions. In the absence of detectable vascular disease the lipid values of providing vascular protection preferably with a healthy diet, regular physical activity and by trying to get out of harmful passions. If the healthy lifestyle is not effective enough, lipid-lowering medication may also be necessary in primary prevention. In the cases of inherited lipid disorders and in patients with high or very high cardiovascular risk it is necessary to take lipid lowering medications in effective dose. The Hun­ga­rian and the international guidelines have been formulated the targets of lipid values for vascular protection. The paper is trying to help for everyday medical practice, how to treat the dyslipidemic patients with the application of widely available lipid parameters and lipid lowering medications effectively. ]

Lege Artis Medicinae

MARCH 20, 2019

[Physiological-pathological muscle atrophy in elderly - interventions potencially inhibiting this progressive process ]

SZÉKÁCS Béla, MOLNÁR Andrea, BESENYEI Attila, MARTONY Zsuzsanna

[In old and very old age, one of the most prevalent signs of aged body’s decline is the progressive loss of muscle mass and function. First itself the physiological aging process can be dominant in the complex causative background but later it is usually intertwined with pathological mechanisms. The importance of muscle system is extremely high in the physiological regulation of various vital life processes The paper also points out the far-reaching consequences of sarcopenia syndrome that leads to general weakness, falls, traumas, acceleration of co-morbidities, rapidly declining self independence, ultimately frailty syndrome, and death. The initial body mass index has been recently replaced by a more adequate, more complex diagnostic approachment of sarcopenia that evaluates both muscle mass/strength and physical performance. Prevention or breaking the process of sarcopenia needs complex intervention which includes special fast protein rich diet with leucin and vitamin D combined with frequent physical exercise. ]