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Hypertension and nephrology

NOVEMBER 04, 2020

[Wearing a face mask: effect on a doctor – patient relationship. Complicating factors and their compensations]

VONYIK Gabriella, FARKAS Martin, TURNER Andrea, FINTA Ervin, BORSZÉKI Judit

[Wearing face masks plays an important role to effectively decrease the chance of transmitting respiratory diseases. Face masks commonly worn during the Covid-19 pandemic to shield the mouth and the nose, cover about 60- 70% of the area of the face that is crucial for the effective verbal and nonverbal communication and perception of mental states. Face masks may complicate social interaction especially in the medical setting where communication skills and doctor-patient relationship are essential to primary care consultations. Literature was reviewed on the impact of such face masks on effective doctor and patient communication as well as useful alternative ways are suggested to compensate them in order to maintain the effective doctor-patient interaction.]

Clinical Neuroscience

SEPTEMBER 30, 2020

[Pompe disease treated with enzyme replacement therapy in pregnancy]

GROSZ Zoltán, VÁRDI Visy Katalin, MOLNÁR Mária Judit

[Pompe disease is a rare lysosomal storage disease inherited in a recessive manner resulting muscular dystrophy. Due to the lack of the enzyme alpha glucosidase, glycogen accumulates in the cells. In the infantile form of Pompe disease hypotonia and severe cardio-respiratory failure are common leading to death within 2 years if left untreated, while the late-onset form is characterized with limb-girdle and axial muscle weakness accompanied with respiratory dysfunction. Pompe disease has been treated with regular intake of the missing enzyme since 2006, which significantly improved the survival and severity of symptoms in patients of both subtypes. The enzyme replacement therapy (ERT) is safe and well tolerated. However, limited data are available on its use in pregnancy. Our goal is to share our experience and review the literature on the safety of enzyme replacement therapy for Pompe disease during pregnancy and post partum.]

Hypertension and nephrology

JUNE 24, 2020

[Treatment of hypertension in patients with chronic renal failure]

SZLOVÁK Edina, SZILVESZTER Dolgos

[The prevalence of chronic renal failure and hypertension is steadily increasing worldwide. The risk of possible cardiovascular death in patient with advanced renal failure is greater than the risk of progression to end-stage renal failure. Therefore treating and achieving target blood pressure is important in order to slow renal function decline in parallel with cardiovascular risk reduction. However, guidelines do not specify a single blood pressure target to be achieved in patients with renal failure, but suggest evidence based, reno- and cardioprotective therapy. This paper summarizes the clinical practice of treating hypertension (drug and nondrug treatment, therapeutic algorithm, target value, effectiveness of therapy) in patients with chronic renal failure.]

Hypertension and nephrology

JUNE 24, 2020

[Hypertension and Covid-19 – Part I. Significance of age, underlying diseases, and ACEI/ARB therapy in hypertension and co-morbidities during SARS-Cov2 infection]

KÉKES Ede, SZÉKÁCS Béla, NAGY Judit, KOVÁCS Tibor

[The appearance of the Covid-19 epidemic in different continents shows specific clinical features. Confirmed infected patients are detectable from approximately 30 years, with a maximum between 40 and 70 years of age. At the same time, however, a significant proportion of those who die from the infection come from patients over 65 years. The prevalence and mortality rates of the hypertensive population show a very similar formation. Based on the data collected, it is not surprising that hypertension as the underlying disease in the Covid- 19 epidemic is the first in all analysis. A more precise analysis clarified that it is not hypertension per se, but co-morbidities and complications of hypertension that play a primary role behind large-scale mortality in old age, such as diabetes, coronary heart disease, stroke, heart failure, and chronic kidney disease. Data from China, North America, and Italy suggest that hypertension and diabetes – and in North America, pathological obesity – in infected patients actually only reflect the prevalence of these diseases in a given population. The presence of comorbidities (coronary artery disease, stroke, heart failure, arrhythmia, chronic kidney disease) – based on multivariate logistic regression analysis – presents a more risk for severe clinical course and mortality. Some recent analyses have provided strong evidence that ACEI/ARB treatment does not pose a higher risk for the course or outcome of infection. Their administration is constantly needed in hypertension and comorbidities due to their organ protective and slowing the progression of diseases.]

Hypertension and nephrology

JUNE 24, 2020

[Not all young, athletic university students have optimal blood pressure. Results of the May 2019 Measurement Month (MMM19)]

PATÓ Anna, NÉMETH Zoltán, JÁRAI Zoltán, KOLLER Ákos

[For about 100 years, it has been known that persistently high blood pressure (hypertension) is the leading cause of cardiovascular disease deaths, such as major risk factors for heart failure, myocardial infarction, stroke, kidney and eye disease. Unfortunately, one of the characteristics of hypertension is that it remains undetected for a long time until damage to one or more organs has already occurred. Therefore, it is very important that systemic blood pressure is monitored from time to time, especially above 45 years of age. The prevalence of hypertension is more than 35% in the Hungarian population. Recent data have pointed out that hypertension may occur at a younger age, although at a lower frequency. Our studies were conducted in the context of the May 2019 Measurement Month (MMM19), an international campaign that draws attention to hypertension and the lack of systematic screening at population level. We hypothesized that hypertension may occur in young, apparently healthy university students. The studies were conducted among students of the University of Physical Education (n = 33; 25 males, 8 females). The mean age was 24.3 years (20–34 years). To measure blood pressure, an automatic blood pressure monitor (Omron MIT5) was used, placed on individuals’ upper arms, as specified in the guidelines, in a relaxed, sitting position. The measurements were repeated three times at one-minute intervals and the average of the three measurements was statistically analyzed. Blood pressure values (mean ± SEM, averaged over 3 measurements) in the student population were systolic 121.9 ± 14.4 mmHg, diastolic 75.1 ± 9.1 mmHg, mean 90.7 ± 10.9 mmHg, and heart rate: 46.8 ± 5.3 mmHg. Although mean blood pressure was within the normal range, higher values were also found in some individuals. Systolic blood pressure fell by 33% due to elevated normal blood pressure, respectively of Grade 1 hypertension. These data confirmed our hypothesis that hypertension may occur at a young age, despite the fact that members of the study population exercise regularly, which is an important preventive factor in the development of cardiovascular disease. These results underscore the importance of regular blood pressure measurement in adolescents, which may reduce the global spread of hypertension and prevent the development of a number of cardiovascular diseases, including stroke, heart attack, renal failure and mental decline.]

Clinical Neuroscience

JULY 30, 2020

[The complex intensive care and rehabilitation of a quadriplegic patient using a diaphragm pacemaker]

FODOR Gábor, GARTNER Béla, KECSKÉS Gabriella

[A 21 year female polytraumatized patient was admitted to our unit after a serious motorbike accident. We carried out CT imaging, which confirmed the fracture of the C-II vertebra and compression of spinal cord. Futhermore, the diagnostic investigations detected the compound and comminuted fracture of the left humerus and femur; the sacrum and the pubic bones were broken as well. After the stabilization of the cervical vertebra, a tracheotomy and the fixation of her limbs were performed. She spent 1.5 years in our unit. Meanwhile we tried to fix all the medical problems related to tetraplegia and respiratory insufficiency. As part of this process she underwent an electrophysiological examination in Uppsala (Sweden) and a diaphragm pacemaker was implanted. Our main goal was to reach the fully available quality of life. It is worth making this case familiar in a wider range of public as it could be an excellent example for the close collaboration of medical and non-medical fields.]

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.]

Clinical Neuroscience

MAY 30, 2020

[Neurological aspects of the COVID-19 pandemic caused by the SARS-CoV-2 coronavirus]

BERECZKI Dániel, STANG Rita, BÖJTI Péter, KOVÁCS Tibor

[By the spring of 2020 the COVID-19 outbreak caused by the new SARS-CoV-2 coronavirus has become a pandemic, requiring fast and efficient reaction from societies and health care systems all over the world. Fever, coughing and dyspnea are considered the major signs of COVID-19. In addition to the involvement of the respiratory system, the infection may result in other symptoms and signs as well. Based on reports to date, neurological signs or symptoms appear in 30-50% of hospitalized COVID-19 patients, with higher incidence in those with more severe disease. Classical acute neurological syndromes have also been reported to associate with COVID-19. A drop in the volume of services for other acute diseases has been described in countries with healthcare systems focusing on COVID-19. During the COVID-19 epidemic it is also important to provide appropriate continuous care for those with chronic neurological disorders. It will be the task of the future to estimate the collateral damage caused by the COVID-19 epidemic on the outcome of other neurological disorders, and to screen for the possible late neurological complications of the SARS-CoV-2 coronavirus infection.]

Clinical Neuroscience

JANUARY 30, 2016

To treat or not to treat, cheyne-stokes respiration in a young adult with vascular encephalopathy

HUBATSCH Mihaela, ENGLERT Harald, WAGNER Ulrich

Cheyne-Stokes respiration (CSR) is a form of sleep-disordered breathing characterised by recurrent central sleep apnoea alternating with a crescendo-decrescendo pattern of tidal volume, relatively rare observation in sleep labs. It is mainly seen in severe heart failure and stroke. We report the case of a young man with CSR after sudden onset of seizure in the context of hypertensive exacerbation leading to the diagnosis of a leukoencephalopathy, and comment on differential diagnoses, prognostic and therapeutic outcomes. The very uniqueness of this case consists in the extremely young age for developing a vascular encephalopathy in the absence of genetic diseases and without previous diagnose of hypertension. There is no adequate explanation for the origin of vascular encephalopathy; also there is lack of evidence regarding the benefits and modality of treatment for CSR in neurologic diseases. Thus, we were forced to find the best compromise in a nocturnal oxygen therapy and follow-up.

Clinical Neuroscience

MARCH 30, 2016

The electrophysiological changes after 1 hz RTMS in ALS patients. A pilot study

MAJOR Zsigmond Zoltán, VACARAS Vitalie, MARIS Emilia, CRISAN Ioana, FLOREA Bogdan, MAJOR Andrea Kinga, MURESANU Fior Dafin

Motor neuron diseases are disabling poor prognostic conditions, with no successful treatment. Repetitive transcranial magnetic stimulation might offer a temporary functional improvement. Objective - We intended to evaluate the extent of the functional improvement using electrophysiological and clinical tests. Methods - Patients with motor neuron disease (amyotrophic lateral sclerosis) were included. Muscle strength and respiratory function assessment represents the clinical approach, and central motor conduction time, motor unit number estimation, blink reflex and H-reflex stands for electrophysiology. Two tests were performed using the whole battery prior and after low frequency repetitive transcranial magnetic stimulation, using 1 Hz stimulation frequency for five consecutive days, 20 minutes daily, at 80% of the individual resting motor threshold. Results - Central motor conduction time, muscle strength and pulmonary function showed no statistically significant differences, but a tendency towards improvement. Motor unit number estimation, blink reflex and H-reflex showed a significantly better outcome after the five day repetitive transcranial magnetic stimulation treatment. Conclusion - Low frequency repetitive transcranial magnetic stimulation influences beneficially electrophysiological parameters in amyotrophic lateral sclerosis, but with little clinical impact; further studies are needed to validate the extent of the effect.