Lege Artis Medicinae

[Expedition to the land of origin]

MOHOS Márta

FEBRUARY 20, 2001

Lege Artis Medicinae - 2001;11(02)

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Lege Artis Medicinae

[The role of steroid hormones in the protection against oxidative stress]

BÉKÉSI Gábor, FEHÉR János

[The incidence of vascular diseases increases in postmenopausal women. This negative trend seems to be controllable favourably by hormone replacement therapy (HRT). Based on the free radical mediated mechanism of atherogenesis the authors try to find the reasons for these observations on the basis of their and others’ data. They consider the increase of activity and amount of neutrophil enzyme myeloperoxydase by certain steroid particulars one of the most important elements of the vasoprotective effect of HRT. This increase diminishes the production of superoxide anion by the inhibition of the NADPH-oxidase enzyme. The observation proved firstly by the authors, that the plasma levels of myeloperoxydase - and so the ability for the inhibition of superoxide anion production by neutrophils - decreases in elderly people, might explain the higher incidence of some free radical mediated illnesses in the elderly. The authors also give a short review of antioxidant effects of steroids in general.]

Lege Artis Medicinae

[The importance of vitrectomy in the management of diabetic retinopathy in proliferative stage]

BERECZKI Árpád

[Vitrectomy is a closed microsurgical technique, by which intraocular tissue, blood and foreign bodies can be removed through a small incision in the pars plana with an automated suction-cutting device (vitrectom), while maintaining normal or slightly elevated intraocular pressure. This technique requires many accessory instruments (microscope, endoillumination, intraocular instruments, endolaser). The main application of vitrectomy is the treatment of severe complications in diabetes mellitus: diabetic retinopathy in the proliferative stage. Vitrectomy is indicated in diabetic retinopathy for the removal of intraocular bleeding and for the treatment of complications of diabetic proliferative vitreoretinopathy (recurring bleeding, tractional and rhegmatogenous retinal detachment). In the case of intraocular bleeding, early vitrectomy is better than delayed, providing good visus (V: 0,5) in 25% of all patients (based on literature data). In retinal detachment cases of tractional origin, surgery provides satisfactory visus for self-care in 2/3rd of all patients.]

Lege Artis Medicinae

[Alphabetic index 1991-2000]

Lege Artis Medicinae

[„There should be someone to hold the hand of the dying!” Deep interviews on the difficulties of caring for the dying]

BOGNÁR Tamás, KOLOSAI Nedda, HEGEDÛS Katalin, PILLING János

Lege Artis Medicinae

[Gastrointestinal stromal tumor]

GONDA Gábor

[Gastrointestinal stromal tumors were subject of much controversy in the last decades. This type of tumors was delineated from the leiomyoma - leiomyosarcoma group. GISTs show variable histological picture, moreover, they are capable of dual (neurogen and myogen) differentiation as it is proved by immunohistochemical and ultrastructural studies. These tumors have relatively good prognosis, only 10-30% of them is malignant, although it is difficult to predict their behaviour in a given case. The most reliable signs of malignancy - cytological pleoimorphism, high mitotic activity, proliferation index above 10 %, and aneuploid DNA content - are generally accepted. The treatment of choice is excision, but not enucleation. Radical surgery is not necessary, since lymph node metastasis is exceptionally rare. Further investigations revealed electronmicroscopical and immunohistochemical (CD 34, CD 117) similarities of tumor cells and interstitial cells of Cajal (ICC) located in the wall of the bowel. The results of these investigations led to the theory that cells of GIST and ICCs are of the same stem cell origin. Molecular genetic studies are also of great help in the differential diagnosis and in predicting the prognosis of GISTs. Mutations in the c-kit gene can not be detected in leiomyomas, so they are thougt to be specific for GISTs. Mutation of exon 11. of chromosome 4. is observed only in malignant GISTs.]

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[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.]

Lege Artis Medicinae

[Epilepsy in coronavirus pandemic]

SZŰCS Anna, HALÁSZ Péter, NARULA Lalit

[We aim to review the impact of COVID-19 pandemic on epilepsy and epilepsy-care. While the virus has no specific link with epilepsy, it may affect the nervous system both directly and indirectly, leading to seizures in several ways. The hyper-coagulable state occurring with the infection may cause strokes leading to seizures. The infection may first manifest in the form of disturbances of consciousness and behaviour, seizures, and even status epilepticus. The interactions of antiviral/antiepileptic drugs need to be taken into account during treatment. The hypercoagulable state induced by COVID-2 infection may cause stroke, which leads to seizures. The infection can occur also as an impaired consciousness of non-epileptic origin. Interactions of antiviral/antiepileptic drugs have also to be taken into account. The pandemic itself as well as quarantines and social distancing may cause anxiety and insomnia, challenge continuous antiepileptic supply; each one carrying the risk of seizing. Young epilepsy patients with learning disabilities and mental health issues are most vulnerable, justifying their hyper-protection. The danger of infection has highlighted the role of telemedicine. Internet-based video communication may ensure full care for chro­nic patients. Those methods favour bes­­ted patients with higher education. Epilepsy does not increase directly the risk of infection, but its comorbidities may worsen the course of the disease. Brain lesions and hypoxia, stress, insomnia and fever joining the infection increase seizure susceptibility. Because the danger of infection ma­de telemedicine an essential tool of pa­tient care, education and better computer supply for those in need is crucial. ]

Clinical Neuroscience

[Paradigm shift in management of patients with vertigo and imbalance]

MIKE Andrea, TAMÁS T. László

[Dizziness is one of the most common causes of medical visits. Management of the dizzy patient may be challenging both for the general practitioner, in emergency departments, and special clinics, as behind a seemingly homogeneous clinical presentation several very different etiologies may occur. Research of the last two century enriched our knowledge about physiology and pathophysiology of the vestibular system. Much knowledge is now available about the labyrinth being able to sense head motions and gravity, processing of afferent vestibular stimuli, reflectory oculomotor and postural control, or recovery of the vestibular system. Based on scientific results new beside tests have been introduced including provocation maneuvers for detecting ectopic otoliths in different semicircular canals, head impulse test to examine function of the vestibulo-ocular reflex, and the HINTS+ battery for differentiation of peripheral or central origin of an acute vestibular syndrome. Technical innovations like videooculography and vestibular evoked myogenic potentials enable us to selectively and side-specifically examine the function of all six semicircular canals and two otolith organs. Pathomechanism of disorders with vertigo and dizziness became more clear resulting in the development or amendment of diagnostic criteria of several vestibular disorders including vestibular migraine, Menière’s disease, benign paroxysmal positional vertigo, persistent postural-perceptual dizziness, bilateral vestibulopathy. Broader knowledge about the pathomechanism promoted the development of new therapeutic methods like different repositioning maneuvers in benign paroxysmal positional vertigo, pharmaceutical therapies, vestibular rehabilitation, and psychotherapy. We aimed to summarize the novelties in the field of oto-neurology.]

Lege Artis Medicinae

[Treatment options for localized and widespread post-COVID pain]

VERECKEI Edit

[Data on the new coronavirus caused di­s­ease and its treatment have been accumulating for more than a year. There are four main disease courses: no or mild symp­toms, unavoidable hospitalisation, severe or lethal outcome, and permanent or post-hoc manifestations. The last course is usually referred to as post-COVID syndrome. As a part of the acute and post-COVID symptomatology there were published pain perceptions with frequent but heterogeneous localisation. It is practical to classify them by the origin of pain: nociceptive/inflammatory, peripheral neuropathic or central. Additionally, we have to clear which phase is the post-COVID patient in i.e. the patient has the prolonged COVID- or persistent post-COVID syndrome. However, in addition to an acute general inflammatory reaction, a true inflammation of joints and muscles is very rare in the musculoskeletal system. The diffuse musculoskeletal pain, chronic fatigue, generalised anxiety and depressive disposition manifest themselves in both acute and persistent forms. Their explanation is essentially of neurological nature: there are para-infectious “neuro-inflammation”, i.e. neuropathic and central mechanisms in the background. Accor­ding­ly, therapeutic options must be chosen while concerning the neuropharmacological action mechanisms of analgesics. El­derly patients at high iatrogenic risk and with multiple co-morbidities may be treated transdermal instead of oral drug administration.]