Lege Artis Medicinae

[The Painter’s Revolt against the Father, or Jesus Chastised ]

GEREVICH József

OCTOBER 20, 2018

Lege Artis Medicinae - 2018;28(10)

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Further articles in this publication

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[General questions and bioethical aspects of handling mistakes in health care]

KOVÁCS József

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[A rebellion against the father - the chastened Jesus]

MARJAI Kamilla

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[Juvenile idiopathic arthritis: from diagnosis to treatment]

MOSDÓSI Bernadett

[Juvenile idiopathic arthritis is a heterogeneous group of diseases that is one of the most common chronic diseases of childhood. It begins before 16 years of age, the etiology is unknown, it is persisting for at least 6 weeks and other pathologic conditions could be excluded. Juvenile idiopathic arthritis includes several diseases categories, each of them have distinct methods of presentation, clinical symptoms, pathogenesis and treatment options. Recent advances in the understanding of the immunological pathogenesis of the diseases have led to dramatic improvements in treatment. Although the management is using a multidisciplinary approach, the disease often persist into adulthood and can cause significant long-term morbidity and physical disability. Our article discusses the classification, clinical manifestations, differential diagnosis, treatment and complications of juvenile idiopathic arthritis. ]

Lege Artis Medicinae

[Gene modified T cells against cancer]

SZÖŐR Árpád

[Chimeric antigen receptor modified (CAR) T cells are hailed as a revolutionary breakthrough in the field of oncology. CAR T cells were first applied, with outstanding success, in the treatment of various leukaemias, yielding unprecedented antitumor activity and long periods of disease free survival. Following the success of CAR T cell therapy in leukaemias, solid tumors should now be targeted. These are more complex targets, therefore CAR T cell therapy needs to be further optimized for this purpose. Also, some unfortunate side effects, including the potentially deadly global inflammation called cytokine storm have to be minimized and possibly even eradicated. The next decade will be an exciting time to define whether this therapy which is yet exclusively used for cancer patients is also successful in the treatment of other diseases. In a recent study, T cells reengineered with CAR derived chimeric autoantibody receptors (CAAR) efficiently prevented disease progression in pre-clinical animal models simulating the serious autoimmune disorder, pemphigus vulgaris. Therapy was based on CAAR constructs which have the unique ability to selectively recognize and eliminate the B-cell clones secreting autoantibodies against a self-protein, thus playing a key role in disease pathogenesis and progression. In this review, we would like to give an overview about the history of the CAR T-cell concept, summarize briefly the currently running clinical trials, and discuss the challenges and future prospects of CAR T-cell therapy.]

Lege Artis Medicinae

[Acetylsalicylic acid (ASA) 75 or 100 mg? ]

KIS János Tibor, ZSIGMOND Kálmán

[The acetylsalicylic acid (ASA) is one of the oldest and most widely used drugs in the world. Currently, it is the most commonly used for the treatment and prophylaxis of cardiovascular diseases. Today, there has been consensus that the risk of ASA’s side effects in primary prevention is greater than the expected benefits. However, it maintains its leading role in the secondary prevention of cardiovascular diseases. There is also a consensus that small doses are recommended for preventive purposes, but there is no agreement whether it should be 75 or 81 or 100 mg. The authors summa­rize the publications published in this topic. ]

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[A growing body of evidence suggests that sleep plays an essential role in the consolidation of different memory systems, but less is known about the beneficial effect of sleep on relational memory processes and the recognition of emotional facial expressions, however, it is a fundamental cognitive skill in human everyday life. Thus, the study aims to investigate the effect of timing of learning and the role of sleep in relational memory processes. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. Our results suggest that the timing of learning and sleep plays an important role in the stabilizing process of memory representation to resist against forgetting.]

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[Chronic administration of antiepileptic drugs without history of unprovoked epileptic seizures are not recommended for epilepsy prophylaxis. Conversely, if the patient suffered the first unprovoked seizure, then the presence of epileptiform discharges on the EEG, focal neurological signs, and the presence of epileptogenic lesion on the MRI are risk factors for a second seizure (such as for the development of epilepsy). Without these risk factors, the chance of a second seizure is about 25-30%, while the presence of these risk factors (for example signs of previous stroke, neurotrauma, or encephalitis on the MRI) can predict >70% seizure recurrence. Thus the International League Against Epilepsy (ILAE) re-defined the term ’epilepsy’ which can be diagnosed even after the first seizure, if the risk of seizure recurrence is high. According to this definition, we can start antiepileptic drug therapy after a single unprovoked seizure. There are four antiepileptic drugs which has the highest evidence (level „A”) as first-line initial monotherapy for treating newly diagnosed epilepsy. These are: carbamazepine, phenytoin, levetiracetam, and zonisamide (ZNS). The present review focuses on the ZNS. Beacuse ZNS can be administrated once a day, it is an optimal drug for maintaining patient’s compliance and for those patients who have a high risk for developing a non-compliance (for example teenagers and young adults). Due to the low interaction potential, ZNS treatment is safe and effective in treating epilepsy of elderly people. ZNS is an ideal drug in epilepsy accompanied by obesity, because ZNS has a weight loss effect, especially in obese patients.]

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[History of vaccine production in Hungary ]

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[This study presents the complete history of the Hungarian vaccine production, partly in association with the process of fighting vaccine-preventable infectious diseases, and underlines the fact that every government actively contributed to the age-adjusted mandatory vaccination schedule of the past 140 years. It demonstrates the various achievements from the smallpox lymph production through the launch of diphtheria serum production at Phylaxia and the establishment of the National Public Health Institute (OKI) with its vaccine production and the later institutional transformation of OKI into Humán as economic corporation to its closure. Among all OKI’s vaccine production activities, this study focuses on the production of influenza vaccines, due to its international importance in the 1960s and 1970s. The vaccine production against diphtheria tetanus and pertussis stands out from Humán’s activities, and the tetanus component of this vaccine is still used in the products of a multinational vaccine manufacturer. ]

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[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.]