Lege Artis Medicinae

[I dissected the whole country]


JUNE 30, 1992

Lege Artis Medicinae - 1992;2(06)

[Every student hears a lot of interesting stories in the various lectures during their university years. Most of the stories are, of course, about the successes and failures of medicine. But the stories you hear in forensic medicine lectures stand out somewhat. ]



Further articles in this publication

Lege Artis Medicinae

[Immunological aspects of granuloma formation]

DANKÓ Katalin, SZEGEDI Gyula

[The process of inflammation can be classified as acute, subacute and chronic, as well as the special category of granulomatous inflammation. Classification of granulomatous inflammation based on morphologic criteria. These are epitheloid, histiocytic, foreign-body, necrobiotic and mixed inflammatory granuloma. The current knowledge suggests the following sequences for the mechanisms of granuloma formation in sarcoidosis (Fig 2): 1. T-cells are locally activated and proliferating 2. These activated T-cells release lymphokines that recruit and activate monocytes/macrophages 3. These activated monocytes/macrophages may differentiate into granuloma cells such as epithelioids cells and multinucleated giant cells and release pro-inflammatory profibrotic mediators that modulate the granulomatous and fibrotic process. It is hoped that further studies in sarcoidosis will help not only in clarifying the pathogenic mechanisms leading to granuloma formation, but also in eventually revealing the etiology of sarcoidosis. ]

Lege Artis Medicinae

[Transvaginal ultrasound examination of embryo development in early pregnancy (sono-embryology) II. central nervous system]

CSABAY László, SZABÓ István, NÉMET János, PAPP Zoltán

[In the second part of their paper, the authors look over the most important phases of the development of the central nervous system during the first trimester of pregnancy - especially considering the stages, which can be differentiated with a high resolution transvaginal transducer. The closed neural tube is developing at days 40 to 44 by the closure of the posterior neuropore, however at this time no brain structures can be detected by ultrasound. During the 7th week the three primary brain vesicles (prosencephalon, metencepha lon, myelencephalon) can be identified – a single ventricle can be seen by ultrasound. During the 8th week the secondary brain vesicles (telencephalon, diencephalon, mesencephalon, metencephalon, myelencephalon) can be separated. During the 9th week the lateral ventricles are filled out by the choroid plexus, which are retracted to the occipital horn by the 13th week. The appearance of the brain structures also takes place according to an accurate „calendar". The structures recognized by ultrasound can be compared to those of the central nervous system known from embryology. The problem of the primary prevention of the cerebrospinal malformations, which are among the most frequent congenital disorders, is not yet solved; therefore the early prenatal diagnosis of the structural anomalies has great importance. ]

Lege Artis Medicinae

[Laparoscopic varicocelectomy]

HOLMAN Endre, TÓTH Csaba, PÁSZTOR Imre, FICSÓR Ervin, PAPP Ferenc

[ With the spreading use of the laparoscope in the urological surgery laparoscopic varicocelectomy may become an alternative treatment of varicocele. The method can be advantageous mainly for patients with bilateral cases compared to the open operation. The authors report on two cases that have been the first urological laparoscopic operations in Hungary.]

Lege Artis Medicinae

[Vascular surgical aspects of small bowel ischaemia]

DÓBI István

[A brief account is given of acute and chronic diseases underlying the disorders of blood supply to the small bowel. In cases of acute disorders, considering the diagnostic difficulties involved, the author stresses the importance of commencing of a vasodilatator treatment immediately after the tentative diagnosis has been reached based on the first suspicious symptomes, and also requiring angiography to render the accurate diagnosis. This is the only possibility to increase (without irreversible tissue damages) the number of successful early vascular reconstructions which are the prerequisites to reducing in lethality. Finally, based on his own extensive experimental work, the author proposes to make allowances for the augmented local postischaemic vasoconstrictor trends of vascular reactivity in the complex therapeutic regimen of these diseases. ]

Lege Artis Medicinae

[Cardiovascular effects of sleep-related breathing disorders, a review]

VÁRDI Visy Katalin

[The acute and chronic cardiovascular effects of sleep related breathing disorders are summarized in this review. Obstructive sleep apnoea syndrome is characterized by recurrent upper airway collapse during inspiration. The resulting asphyxia causes hypoxia and arousal. The vigorous inspiratory efforts produce falls in blood pressure coinciding with each effort (pulsus paradoxus). A rise in blood pressure is seen concurrently with each termination of apnoea and arousal. Bradycardia induced by hypoxia in each obstructive period and increased frequency at time of arousals could lead to arrhythmias. This may result in a high prevalence of hypertension, acute myocardial infarction and arrhythmias in sufferers of obstructive sleep apnoea syndrome have. ]

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[Sleep habits among preschool- and schoolchildren]

FUSZ Katalin, RITECZ Bernadett, BALOGH Brigitta, TAKÁCS Krisztina, SOMLAI Eszter, RAPOSA L. Bence, OLÁH András

[Objective - Our aim is to evaluate sleep habits, sleep quality and influencing factors among preschool- and schoolchildren. Method - Two questionnaires were recorded. Questionnaire 1 dealt with sleeping habits, breastfeeding and health behavior of preschool children and infant, and it contained the abbreviated version of the Children’s Sleep Habits Questionnaire. Questionnaire 2 dealt with health behavior and the application of sleep hygiene rules, as well as it contained the Athens Insomnia Scale. Subjects - We assessed a total of 1063 questionnaires: 516 kindergarten children participated in our online survey across the country; 547 primary and secondary school students participated in the 2nd questionnaire survey in Szolnok. Results - Parents’ observation shows that the average nighttime sleeping time of kindergarten children is 10 hours 20 minutes on weekdays and 10 hours 36 minutes on weekends. The most popular sleeping habits in kindergarten age: teal reading (65.1%) and co-sleeping (42.8%). Parents of infants used breastfeeding (50.4%) and rocking (43.2%) most frequently before sleep. Co-sleeping has a positive influence on the length of lactation. Among the preschool sleeping habits we have proved a number of positive effects of teal reading, while watching television have negative effects. The sleep quality of school-age children according to the Athens Insomnia Scale is 6.11 points (SD: 4.11), 19% of the children are insomniac. Their sleep time is 7 hours 31 minutes on weekdays and 9 hours 30 minutes on weekends. The usage of good health behavior and sleep hygiene rules positively influence sleep quality and sleep duration. Conclusions - With our results, we would like to draw the attention of children and parents to the importance of sleeping and using sleep hygiene rules.]

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[Neuropsychological rehabilitation following acquired brain injury]

TAMÁS Viktória, KOVÁCS Noémi, TASNÁDI Emese

[Neuropsychological rehabilitation or rehabilitation neuropsychology is a field within applied neuropsychology. It originally diverges from applied clinical and functional neuropsychology, although it could not be entirely differed from them. The unique nature of this area over the complexity is given by its process-controlled and system-approach aspects. In Hungary the number of neurorehabilitation centres and departments requiring neurocognitive rehabilitation has been continually increasing. Nevertheless, the number is still low; accordingly in our country this field is relatively young and isn’t well known. Authors of this review would like to draw attention to the importance of rehabilitation of patients with acquired brain injury and improvement of their quality of life with the theoretical and practical knowledge, as well as the necessity of future alterations and challenges emphasizing the need of a significant change of this narrow domain. ]

Clinical Neuroscience

[Advanced Parkinson’s disease characteristics in clinical practice: Results from the OBSERVE-PD study and sub-analysis of the Hungarian data]

TAKÁTS Annamária, ASCHERMANN Zsuzsanna, VÉCSEI László, KLIVÉNYI Péter, DÉZSI Lívia, ZÁDORI Dénes, VALIKOVICS Attila, VARANNAI Lajos, ONUK Koray, KINCZEL Beatrix, KOVÁCS Norbert

[The majority of patients with advanced Parkinson’s disease are treated at specialized movement disorder centers. Currently, there is no clear consensus on how to define the stages of Parkinson’s disease; the proportion of Parkinson’s patients with advanced Parkinson’s disease, the referral process, and the clinical features used to characterize advanced Parkinson’s disease are not well delineated. The primary objective of this observational study was to evaluate the proportion of Parkinson’s patients identified as advanced patients according to physician’s judgment in all participating movement disorder centers across the study. Here we evaluate the Hungarian subset of the participating patients. The study was conducted in a cross-sectional, non-interventional, multi-country, multi-center format in 18 countries. Data were collected during a single patient visit. Current Parkinson’s disease status was assessed with Unified Parkinson’s Disease Rating Scale (UPDRS) parts II, III, IV, and V (modified Hoehn and Yahr staging). Non-motor symptoms were assessed using the PD Non-motor Symptoms Scale (NMSS); quality of life was assessed with the PD 8-item Quality-of-Life Questionnaire (PDQ-8). Parkinson’s disease was classified as advanced versus non-advanced based on physician assessment and on questions developed by the Delphi method. Overall, 2627 patients with Parkinson’s disease from 126 sites were documented. In Hungary, 100 patients with Parkinson’s disease were documented in four movement disorder centers, and, according to the physician assessment, 50% of these patients had advanced Parkinson’s disease. Their mean scores showed significantly higher impairment in those with, versus without advanced Parkinson’s disease: UPDRS II (14.1 vs. 9.2), UPDRS IV Q32 (1.1 vs. 0.0) and Q39 (1.1 vs. 0.5), UPDRS V (2.8 vs. 2.0) and PDQ-8 (29.1 vs. 18.9). Physicians in Hungarian movement disorder centers assessed that half of the Parkinson’s patients had advanced disease, with worse motor and non-motor symptom severity and worse QoL than those without advanced Parkinson’s disease. Despite being classified as eligible for invasive/device-aided treatment, that treatment had not been initiated in 25% of these patients.]

Lege Artis Medicinae

[Mechanism of mucosal defence and options to reduce virus invasion during the COVID pandemic]


[The portal of entry for coronavirus is the mucous membrane of the respiratory tract. Severity, organ manifestations and out­come of COVID-19 are determined by the viral load, burdening the attacked organism. Condition of the respiratory tract and gastrointestinal mucosa and the capacity of their defence system are crucial for virus penetration, fusion with epithelial cells and replication. Direct neural spread, penet­ra­tion into the deeper airways and spread through the lymph nodes depend on these functions. Virus binding and engulfment is an active process. The virus penetrates the endosomes of the epithelial cell, by enzymatic transfer where it is recognised by natural defence agents and triggers the first defence responses. These alarm the entire immune system and trigger a whole chain of inflammatory and enzymatic defence processes (cytokine and bradykinin storm) proportional to the viral load. The severity endpoint of COVID-19 pathology is alveolitis, cerebral vasculitis and intestinal da­mage, often with fatal outcome. The airway mucosa defends itself by secreting surface factors and recruiting and activating cells of the adaptive immune system. An important element of the latter is the early ap­pearance of secretory IgA in the mucosa. The viral invasion can be prevented by application of a nasal spray containing carrageenan, which engages the virus and prevents its attachment by the gel-forming property of carrageenan. This effect has worked in previous virus epidemics and the first COVID-19 experiences confirm its pro­tec­tive role. ]

Clinical Neuroscience

[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.]