Lege Artis Medicinae

[ Health Law, Patient Rights, and the Experience of Jehovah's Witnesses]

HARASZTI Zoltán1

MARCH 01, 2000

Lege Artis Medicinae - 2000;10(03)

[According to Article 54 of the Constitution: “Every person in the Republic of Hungary is born he has the right to life and human dignity, from which no one shall be arbitrarily deprived. " Everyone has the right to the full enjoyment of the human dignity in the event of any illness or medical intervention. From the fundamental right of human dignity, the prohibition of all medical and health interventions that disregard the personality of the patient can be deduced.]

AFFILIATIONS

  1. Magyarországi Jehova Tanúi Egyház, Budapest

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Lege Artis Medicinae

[The role of coronary artery revascularization in the management of heart failure and systolic left ventricular disfunction]

LENGYEL Mária

[Ischemic heart disease accounts for about 70% of chronic systolic heart failure and severe systolic left ventricular dysfunction. Prognosis of ischemic heart failure is worse than that of non-ischemic heart failure and can only be moderately improved with optimal drug treatment. Further improvement may be expected from revascularization in the presence of reversible left ventricular dysfunction which is called myocardial viability. Viability associated with chronic left ventricular dysfunction is caused by hibernation. Low dose dobutamine stress echocardiography is the best imaging modality to predict the improvement of left ventricular function after revascularization. Myocardial contrast echocardiography can further improve the diagnostic value of dobutamine echocardiography. The disadvantage of nuclear imaging methods for the diagnosis of viability is their low specificity. Revascularization surgery improves left ventricular function and survival when the amount of viable myocardium is adequate. However, the non-revascularized viable myocardium is an unstable condition which can effect the prognosis adversely. Although there are no randomized studies, coronary bypass surgery is considered indicated for severe left ventricular dysfunction if the viability of the myocardium and the operability of the coronary arteries are estabilished, even in the absence of angina. To establish these criteria for revascularization imaging studies (preferably low dose dobu tamine stress echocardiography) and coronary angiography are required. ]

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[Action on Secondary Prevention through Intervention to Reduce Events]

MATOS Lajos

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[The microbiology pharmacokinetics and clinical use of carbapenems]

BÁN Éva, PRINZ Gyula

[ Imipenem and meropenem the two currently available carbapenems inhibit the synthesis of the cell wall similarly to other bactericidal B-lactam antimicrobials. These agents have excellent activity against the vast majority of aerobic and anaerobic Gram-positive and Gram-negative organisms. In addition to other B-lactam resistant microbes (e.g. Chlamydia, Mycoplasma) only Stenotrophomonas maltophilia and Enterococcus faecium bacteria are naturally resistant to carbapenems. Carbapenems are extremely stable compounds against nearly all types of B-lactamases: from the penicillinase of Staphylococcus to Class A and Class B types of B-lactamase enzymes of Gram-negative bacteria. Secondary resistance against carbapenems was described in case of the following bacteria: penicilline resistant S. pneumoniae, methicilline resistant Staphylococcus aureus, Pseudomonas aeruginosa, Enterobacter cloaceae, less frequently Enterobacter aerogenes, Serratia mercescens, Klebsiella pneumoniae and Acinetobacter baumannii. The pharmacokinetic profile of imipenem and meropenem are very similar. Carbapenems are valuable as empirical monotherapy due to their broad spectrum of antimicrobial activity and ß lactamase stability in the treatment of severe nosocomial infections, lower respiratory tract or intraabdominal infections and febrile neutropenia. The use of imipenem in central nervous system infection is not approved due to the high incidence of seizures. ]

Lege Artis Medicinae

[Confined placental mosaicism and uniparental disomy]

BODA Anikó, PAPP Zoltán

[Chromosomal mosaicism is defined as the presence of two or more cell lines having different chromosomal complements in the same individual. In the conceptus the extent of the mosaicism depends on the timing of chromosomal mutation occurance, the cell lineage affected, and the viability of the mutation. The resultant mosaicism can be either generalized, confined placental or confined embryonic. The process of the loss or removal of one of the three chromosomes from the trisomic conception, at least from the cells that will form the proper fetus is known as trisomic zygote rescue. As the result of this phenomenon, the embryo/fetus becomes disomic, while the placental compartment remains trisomic or mosaic. After losing a chromosome, the remaining pair might originate from the same parent. The presence of two chromosomes from one parent in a disomic cell line is termed uniparental disomy. Uniparental disomy is one form of aberrant origin for disomic cells, and the term „pseudodisomy" is also used. Uniparental disomy can involve homozygosity for the chromosome, and the term ,,isodisomy" has been suggested for this phenomenon. If the homozigosity for the chromosome is not complete, the term „heterodisomy" is used. Depending on the pathologic chromosome, the clinical consequences of the confined placental mosaicism and uniparental disomy can be intrauterine and/or postnatal growth restriction, spontaneous abortion. Increased perinatal morbidity and mortality, minor congenital malformations can result from the phenomena. Confined placental mosaicism and uniparental disomy are well known in syndromatology too. The connections of mosaic trisomy 7 and Silver-Russell syndrome, mosaic trisomy 15 and Angelman syndrome, mosaic trisomy 15 and Prader-Villi syndrome are described. Due to the presence of aneuploid cells in the placenta, confined placental mosaicism may cause placental dysfunction, hydropic degeneration of the placenta or „unexplained" highly increased serum hCG level. ]

Lege Artis Medicinae

[Home management of chronic obstructive pulmonary disease]

MÜHLBACHER Szilvia, VADÁSZ Imre

[Cessation of smoking is the primary condition for the successful management of COPD. The aim of pharmacotherapy is to diminish symptoms (dyspnoe, cough and expectoration) to increase cardiorespiratory performance and to improve the quality of life. Pharmacotherapy is based upon administration of bronchodilators (anticholinergic and beta-agonist preparations as well as theo phyllin). In the case of acute exacerbation anti- biotic treatment is indicated. Effectiveness of inhalative corticosteroids is not established well yet, whereas the oral and parenteral administration of corticosteroids is limited by severe side effects. In the advanced stage of disease long term oxygen therapy can increase survival and improve the quality of life. Respiratory rehabilitation is an essential component in the management, it facilitates expectoration and corrects respiratory technique, increases muscular force and performance, improves quality of life. Active cooperation of patients is necessary for the successful management of the desease; the patient should be informed on the particulars of the disease and on the required contribution.]

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CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

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Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

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