Lege Artis Medicinae

[The state of alternative medicine in Europe]

SZÁNTÓ Zsuzsa

MAY 27, 1992

Lege Artis Medicinae - 1992;2(05)

[In the last decade or two, alternative healing practices have emerged and spread rapidly throughout the "western world" and recently in Hungary. In almost all Western European countries, it is now legal - even for lay people - to practise healing activities that were previously forbidden. Legalisation has taken place formally in some places or is in the process of taking place, but in others the situation is similar to that in Hungary: the existing law has been implicitly repealed. ]

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Lege Artis Medicinae

[Treatment of endodermal thyroid cancers]

ÉSIK Olga, NÉMETH György

[Following staging procedures, differentiated thyroid cancers i. e. papillary and follicular carcinomas, are surgically treated with intra- or extracapsular total thyroidectomy (depending on the anatomical extent of the disease) and selective lymphnode dissection. Postoperative complementary use of adjuvant external and systemic radiotherapy is the general practice. External irradiation decreases local/regional relapses in all cases. Radioiodine treatment decreases local/regional/ distant recurrences in cases of tumour cells capable of iodine uptake. Life-long thyroxine medication is required, with a TSH-suppressive dose for relapse prevention and hormone substitution. This treatment regimen, a careful follow-up, and adequate rescue therapy are the prerequisites of the generally excellent patient prognosis, i. e. a survival probability measurable in decades. In anaplastic carcinoma following extracapsular total thyroidectomy and lymph node dissection, external irradiation is always indicated. Chemotherapy has a benefical effect on survival. Hormone replacement therapy is necessary to substitute for the missing metabolic effect of the thyroid. This combined modality treatment leads to some improvement with regard to the prognosis of this poor-risk type of cancer. ]

Lege Artis Medicinae

[Correspondence]

CSIZÉR Zoltán

["Programmed cell death" I read with great interest and attention Dr. Béla Szende's excellent communication on the biological and pathological significance of programmed cell death (apoptosis). Although the phenomenon of apoptosis (albeit under a different name) has been described for more than twenty years, its importance in the regulation of tissue homeostasis has only been recognized in the last few years. In 1991, for example, Jean Claude Ameisen and Andre Carpon of the Pasteur Institute in Lille put forward their hypothesis that apoptosis explains the qualitative and quantitative deficiency of helper T cells in HIV-infected patients.]

Lege Artis Medicinae

[Malnutrition syndrome in childhood part II.]

BAKSAI László, PHILIP W. H. Esker

[Malnutrition is a chronic nutritional disorder resulting from insufficient intake of caloric energy and/or some fundamental nutrients. Since it is the most common public health problem in the world it has a great impact on pediatric morbidity and mortality. The etiology is extremely complex. When one considers the less severe symptoms and signs caused by vitamin-, mineral-, and trace element deficiencies, it is apparent that not only children living in underprivileged countries are affected. In the course of making the diagnosis an assess ment of the nutritional status should also be made, especially considering the above-mentioned marginal nutritional deficiencies. The most common manifestation of nutritional disorders is anemia, which is mostly caused by lack of iron, vitamin B12, folic acid and can be complicated by infections. Nutritional requirements can be affected by several drugs. That is why this medication-nutrition interaction has to be kept in mind during treatment. The interaction of malnutrition and infection is very important, primarily for three reasons: mechanisms by which infection complicates the metabolic and nutritional status of the host; importance of suboptimal nutritional status on susceptibility and severity of intercurrent infections; clinical and therapeutic implication of these interactions. ]

Lege Artis Medicinae

[Operative Hysteroscopy]

BACSKÓ György

[Many thousands of hysterectomies are perfor med each year in women suffering from menorrhagia resistant to medical treatment but having, no demonstrable uterine abnormality. Hysteroscopy, the up to date diagnostic and therapeutic method can help to get advance in the diagnosis and therapy in these situations. In addition to permitting more precise diagnoses to be made, hysteroscopy allows the endometrium to be treated selectively under direct vision. The full thickness of the endometrium may be ablated using laser or resectoscope. The benefits of selective endometrial destruction versus hysterectomy are enormous. Patients undergoing endometrial ablation are frequently delighted with the short hospital stay, and the cost of this procedure is lower than the cost of hysterectomy. Many Müllerian fusion defects are amenable to hysteroscopic treatment. Patients with certain types of Müllerian defects undergoing hysteroscopic septotomy will enjoy as high a rate of reproductive success as those receiving more traditional procedures while avoiding the risks, the subsequent caesar sections, and the high cost. ]

Lege Artis Medicinae

[Transvaginal ultrasonography of embryo development in prematurity, sono-embryology I. The embryo and its appendages]

CSABAY László, SZABÓ István, NÉMETH János, PAPP Zoltán

[The authors performed serial examinations by a high resolution transvaginal transducer during the first trimester of pregnancy to show the most important stages of the early embryonal development. This is a preliminary study to a prospective, detailed screening programme in the first trimester of pregnancy. The earliest sign of pregnancy can be detected at the 29–31st days of menstrual age using vaginal transducer. During the 5th week the (secondary) yolk sac can be seen, which is an unambiguous evidence of an intrauterine pregnancy. The living embryo can be de monstrated on 36–38th days with the help of the pulsation of the embryonic heart tube. Certain embryonic structures appear in a defined order (developmental „calendar"). The physiologic midgutherniation can be observed during 7–11th weeks. Transvaginal sonography has gained popularity only recently due to technical advances. The main fields of use became gynaecology and early pregnancy, for there is no need to have a full bladder, for the lack of disturbing tissue layers. This method is essentially a combination of the bimanual gynaecological and the ultrasound examination. ]

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[LAM 30: 1990–2020. Facing the mirror: Three decades of LAM, the Hungarian medicine and health care system]

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Lege Artis Medicinae

[A short chronicle of three decades ]

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[Hungarian professional periodicals started quite late in European context. Their publish­ing, editing and editorial philosophy were equally influenced by specific historical and political situations. Certain breaking points of history resulted in termina­tion of professional journals (War of In­de­pendence 1848-1849, First and Se­cond World Wars), however there were pe­riods, which instigated the progress of sciences and founding of new scientific journals. Both trends were apparent in years after the fall of former Hungarian regime in 1990. The structure of book and journal publishing has changed substantially, some publishers fell “victim” others started successfully as well. The latters include the then-established publishing house Literatura Medica and its own scientific journal, Lege Artis Me­di­cinae (according to its subtitle: New Hun­garian Medical Herald) issued first in 1990. Its appearance enhanced significantly the medical press market. Its scientific publications compete with articles of the well-established domestic medical journals however its philosophy set brand-new trends on the market. Concerning the medical community, it takes on its problems and provides a forum for them. These problems are emerging questions in health care, economy and prevention, in close interrelation with system of public health institutions, infrastructure and situation of those providing individual health services. In all of them, Lege Artis Medicinae follows consequently the ideas of traditional social medicine.]

Lege Artis Medicinae

[Focus on Lege Artis Medicinae (LAM)]

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[Three decades ago, LAM was launched with the goal of providing scientific information about medicine and its frontiers. From the very beginning, LAM has also concerned a special subject area while connecting medicine with the world of art. In the palette of medical articles, it remained a special feature to this day. The analysis of the history of LAM to date was performed using internationally accepted publication guidelines and scientific databases as a pledge of objectivity. We examined the practice of LAM if it meets the main criteria, the professional expectations of our days, when publishing contents of the traditional printed edition and its electronic version. We explored the visibility of articles in the largest bibliographic and scientific metric databases, and reviewed the LAM's place among the Hun­ga­rian professional journals. Our results show that in recent years LAM has gained international reputation des­pite publishing in Hungarian spoken by a few people. This is due to articles with foreign co-authors as well as references to LAM in articles written exclusively by foreign researchers. The journal is of course full readable in the Hungarian bibliographic databases, and its popularity is among the leading ones. The great virtue of the journal is the wide spectrum of the authors' affiliation, with which they cover almost completely the Hungarian health care institutional sys­tem. The special feature of its columns is enhanced by the publication of writings on art, which may increase Hungarian and foreign interest like that of medical articles.]

Lege Artis Medicinae

[Second game, 37th move and Fourth game 78th move]

VOKÓ Zoltán

[What has Go to do with making clinical decisions? One of the greatest intellectual challenges of bedside medicine is making decisions under uncertainty. Besides the psychological traps of traditionally intuitive and heuristic medical decision making, lack of information, scarce resources and characteristics of doctor-patient relationship contribute equally to this uncertainty. Formal, mathematical model based analysis of decisions used widely in developing clinical guidelines and in health technology assessment provides a good tool in theoretical terms to avoid pitfalls of intuitive decision making. Nevertheless it can be hardly used in individual situations and most physicians dislike it as well. This method, however, has its own limitations, especially while tailoring individual decisions, under inclusion of potential lack of input data used for calculations, or its large imprecision, and the low capability of the current mathematical models to represent the full complexity and variability of processes in complex systems. Nevertheless, clinical decision support systems can be helpful in the individual decision making of physicians if they are well integrated in the health information systems, and do not break down the physicians’ autonomy of making decisions. Classical decision support systems are knowledge based and rely on system of rules and problem specific algorithms. They are utilized widely from health administration to image processing. The current information revolution created the so-called artificial intelligence by machine learning methods, i.e. machines can learn indeed. This new generation of artificial intelligence is not based on particular system of rules but on neuronal networks teaching themselves by huge databases and general learning algorithms. This type of artificial intelligence outperforms humans already in certain fields like chess, Go, or aerial combat. Its development is full of challenges and threats, while it presents a technological breakthrough, which cannot be stopped and will transform our world. Its development and application has already started also in the healthcare. Health professionals must participate in this development to steer it into the right direction. Lee Sedol, 18-times Go world champion retired three years after his historical defeat from AlphaGo artificial intelligence, be­cause “Even if I become the No. 1, there is an entity that cannot be defeated”. It is our great luck that we do not need to compete or defeat it, we must ensure instead that it would be safe and trustworthy, and in collaboration with humans this entity would make healthcare more effective and efficient. ]

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.