Lege Artis Medicinae

[Rosarium parvulorum Rosary for the Small Ones – Crown of Sonnets]

KIRÁLY Péter

DECEMBER 16, 2006

Lege Artis Medicinae - 2006;16(12)

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Lege Artis Medicinae

[The 48. Congress of the Hungarian Society of Gastroenterology - 17-21. June 2006. Szeged]

IZBÉKI Ferenc

Lege Artis Medicinae

[EFFECTS OF VITAMIN D AND THE EFFICACY OF VARIOUS VITAMIN D PRODUCTS]

BORS Katalin

[An increasing number of studies suggests that the effects of vitamin D is not limited to the regulation of calcium homeostasis, but it is also involved in several other physiologic processes. Vitamin D receptors are present on the surface and in the nucleus of most cells. Vitamin D as a steroid hormone has genomic and non-genomic effects. Vitamin D deficiency is an important problem worldwide, and so is in Hungary. Vitamin D promotes bone formation and mineralization and decreases the rate of bone absorption. Its extraskeletal effects include antitumour, muscle strength increasing, antiinflammatory, blood pressure lowering and insulin secretion increasing activites.]

Lege Artis Medicinae

[CLINICAL ASPECTS OF NOCTURNAL GASTRO-OESOPHAGEAL REFLUX]

DEMETER Pál

[Gastro-oesophageal reflux that occur at night has special clinical features and thus require extra attention. During sleep most anti-reflux mechanisms diminish, which results in prolonged contact between gastric acid and oesophageal mucosa compared to reflux during the day. Nighttime reflux symptoms adversely affect quality of life, vitality, physical and mental health. A further important consequence is the potential exacerbation of respiratory disorders such as asthma and sleep apnea. There is increasing interest in the association between nocturnal reflux and certain extra-oesophageal symptoms, including reflux laryngitis and chronic cough. An increased risk of erosive damage and adenocarcinoma of the oesophagus are also observed among patients who report nocturnal reflux symptoms. The primary goal of treatment is to improve quality of life and reduce the risk of complications by decreasing the time of acid contact with oesophageal mucosa. Nighttime reflux symptoms are much more difficult to control than daytime symptoms. Treatment guidelines generally recommend lifestyle changes as the initial approach in managing nocturnal symptoms, however, this is successful in only a small proportion of patients. Evidence-based reviews and meta-analyses favour the use of proton pump inhibitors in the treatment of gastro-oesophageal reflux disease and reflux-oesophagitis. Proton pump inhibitors are the most efficient acid-suppressing agents and thus diminish the harmful effect of acidic gastric reflux on the oesophageal mucosa. In addition, by decreasing the volume of gastric acid, they reduce the tendency to reflux.]

Lege Artis Medicinae

[In the focus: malignant lung diseases]

MAGYAR Pál

Lege Artis Medicinae

[SEVERE THERAPY RESISTANT BRONCHIAL ASTHMA]

HERJAVECZ Irén, BÁNKÚTI Beáta, CSOMA Zsuzsanna

[While severe asthma associated with high morbidity affects a relatively small proportion of all patients with asthma, it requires special attention and innovative treatment approaches. Although asthma is by definition characterized by reversible airflow obstruction, the obstruction becomes fixed to some extent and refractory to corticosteroids with the progression of the disease. The underlying change in this phenotype is airway wall remodelling. In allergic asthmatic patients who remain symptomatic despite highdose inhaled corticosteroid therapy, blockade of IgE with omalizumab confers appreciable clinical benefit. Chronic severe asthma is also accompanied by a marked increase in TNFproduction that might contribute to corticosteroid resistance. In accordance with this, TNF blockade with entanercept has been shown to improve asthma controll and at the same time to reduce bronchial hyperresponsiveness. Identification of new susceptibility genes, such as ADAM33, will provide further targets for therapy, which in turn can result in the development of treatment tools that modify the natural course of asthma and reverse the changes associated with airway remodelling, rather than simply suppress inflammation and dilate the airways.]

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[Focus on Lege Artis Medicinae (LAM)]

VASAS Lívia, GEGES József

[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.]

Clinical Neuroscience

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Clinical Neuroscience

[Tracing trace elements in mental functions]

JANKA Zoltán

[Trace elements are found in the living organism in small (trace) amounts and are mainly essential for living functions. Essential trace elements are in humans the chromium (Cr), cobalt (Co), copper (Cu), fluorine (F), iodine (I), iron (Fe), manganese (Mn), molybdenum (Mo), selenium (Se), zinc (Zn), and questionably the boron (B) and vanadium (V). According to the biopsychosocial concept, mental functions have biological underpinnings, therefore the impairment of certain neurochemical processes due to shortage of trace elements may have mental consequences. Scientific investigations indicate the putative role of trace element deficiency in psychiatric disorders such in depression (Zn, Cr, Se, Fe, Co, I), premenstrual dysphoria (Cr), schizophrenia (Zn, Se), cognitive deterioration/de­mentia (B, Zn, Fe, Mn, Co, V), mental retardation (I, Mo, Cu), binge-eating (Cr), autism (Zn, Mn, Cu, Co) and attention deficit hyperactivity disorder (Fe). At the same time, the excess quantity (chronic exposure, genetic error) of certain trace elements (Cu, Mn, Co, Cr, Fe, V) can also lead to mental disturbances (depression, anxiety, psychosis, cognitive dysfunction, insomnia). Lithium (Li), being efficacious in the treatment of bipolar mood disorder, is not declared officially as a trace element. Due to nutrition (drinking water, food) the serum Li level is about a thousand times less than that used in therapy. However, Li level in the red cells is lower as the membrane sodium-Li countertransport results in a Li efflux. Nevertheless, the possibility that Li is a trace element has emerged as studies indicate its potential efficacy in such a low concentration, since certain geographic regions show an inverse correlation between the Li level of drinking water and the suicide rate in that area. ]

Clinical Neuroscience

[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

Clinical Neuroscience

Extraskeletal, intradural, non-metastatic Ewing’s sarcoma. Case report

OTTÓFFY Gábor, KOMÁROMY Hedvig

Intracranial localization of Ewing’s sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing’s sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re­gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima­ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.