Lege Artis Medicinae

[Immunologic prevention in type 1 diabetes mellitus]

KIS János, ENGELMANN Péter, HEYAM Jalahej, ORBÁN Tihamér

SEPTEMBER 15, 2006

Lege Artis Medicinae - 2006;16(08-09)

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Lege Artis Medicinae

[A new simple tool for tonometric determination of the pCO2 in the gastrointestinal tract]

BODA Domokos, TÁLOSI Gyula, KASZAKI József

Lege Artis Medicinae

[STATE-OF-THE-ART DIAGNOSIS AND TREATMENT OF PITUITARY ADENOMAS SECRETING GROWTH HORMONE]

CZIRJÁK Sándor

[The history of the treatment of pituitary adenomas that cause acromegaly is as long as that of neurosurgery. While in the first half of the past century the aim of surgery was to save the patient's life, later the radical removal of the tumour was coupled with an effort to decrease complications, morbidity and mortality to the minimum. Today, beside all these, the complete sparing of the remaining hypophyseal substance and restoration of normal pituitary function are also important goals. The achievement of these goals is efficiently served by recent advances in microscopy, the minimally invasive methods of craniotomy, the availability of endoscopy in neurosurgery, three-dimensional computerguided neuronavigation, intraoperative colour Doppler sonography, as well as intraoperative real-time MRI. Recent developments in pharmacological research have created new promising conservative treatment modalities that supplement surgery, including somatostatin analogues and growth hormone receptor agonists. Also as supplementary treatment to surgery, occasionally replacing it, new radiosurgical methods, such as stereotaxic radiation, gamma knife, and heavy particle irradiation have gained grounds in neurosurgical practice.]

Lege Artis Medicinae

[BRONCHUS ASSOCIATED LYMPHOID TISSUE LYMPHOMA]

HERTEL Katalin, ZSIRAY Miklós, SOLTÉSZ Ibolya

[INTRODUCTION - Primary lymphomas rarely occur in the lung. CASE REPORT - The authors present the case of an asymptomatic 61-year-old man. The patient was identified on routine chest X-ray having a streak infiltrate in the upper lobe of the right lung, which did not respond to antibiotics. On histological examination of the bronchoscopic specimen BALT- (bronchus associated lymphoid tissue) lymphoma was presumed. Since staging showed the disease to be localized, lobectomy was performed. The patient is symptom-free 16 months after surgery and there is no relapse. CONCLUSION - Although BALT-lymphomas are of low-grade malignancy in most of the cases, relapses can develop in the original organ or in other extra-nodal locations years later and BALTlymphomas may also transform into large-cell lymphomas of more aggressive behaviour.]

Lege Artis Medicinae

[DIAGNOSIS, DIFFERENTIAL DIAGNOSIS AND TREATMENT OF HYPERPROLACTINAEMIA]

IVÁN Gabriella, GÓTH Miklós

[Hyperprolactinaemia is one of the most common endocrine diseases. Besides hypothyroidism- associated hyperprolactinaemia and that arising as a side effect of certain drugs, the most common cause of hyperprolactinaemia is a pituitary microadenoma (<10 mm in diameter) or macroadenoma (≥10 mm) that produces prolactin (prolactinoma). In addition, several physiological conditions can elevate (mostly temporarily) the serum prolactin level, therefore, setting up the precise diagnosis requires careful evaluation of the patient’s history and the laboratory, clinical, and imaging findings. Moreover, macroprolactinaemia, which is usually not a pituitary tumour-related disease, should also be ruled out. Prolactinomas represent the most common form of functioning pituitary adenomas, accounting for 30-40% of such tumours. The typical clinical symptoms of hyperprolactinaemia may be modulated by the mass effect of macroadenomas. In women the disease typically manifests as menstrual disturbance of various degree, including primary or secondary amenorrhoea, oligomenorrhoea, short luteal phase, infertility and galactorrhoea. In men reduced libido, impotence, infertility, gynaecomastia and, rarely, galactorrhoea are the typical symptoms. Dopamine agonist therapy is the first choice of treatment. Dopamine agonist therapy (bromocriptine and the recently developed quinagolide and cabergoline) successfully lowers the serum prolactine level in nearly 90% of cases, and, importantly, it also reduces the size of the tumour in the majority of cases. This explains why today surgery and radiotherapy are only used in rare special cases of prolactinoma.]

Lege Artis Medicinae

[BREAST CANCER CARE: FROM PREVENTION TO SURVEILLANCE]

KAHÁN Zsuzsanna

[Breast cancer is the most common malignancy in women in developed countries. The development of most breast cancers is related to various hormonal effects, while 10% is associated with inherited gene mutations. Most of the primary prevention methods aim at decreasing the effects of hormones, but education on proper lifestyle is also an important risk-lowering method. The primary treatment of early breast cancer is usually breast-conserving surgery, either with the targeted removal of regional lymph nodes (by sentinel lymph node labelling) or with axillary block-dissection. The aim of postoperative radiotherapy is the eradication of the tumour cells left behind. Beside the locoregional tumour control this also plays a role in the prevention of recurrence or a secondary systemic dissemination. Adjuvant systemic treatments are used for the eradication of disseminated microscopic tumour foci. The use of modern adjuvant treatments may reduce death from the disease by up to 50%. The risks of relapse or death may be estimated based on established prognostic factors. While in low-risk patients it is not worth starting medical treatment, especially in view of the side effects, while in other cases chemo- or hormonal therapy may save the patient's life. The choice of the medical treatment should also depend on the patient's general health, the concomittant diseases and her preferences. The collaboration of the various specialists involved in the care of breast cancer patients can best take place at specialised breast centres that are equipped with the necessary technical basis, knowledge and professional experience.]

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Cases of inborn errors of metabolism diagnosed in children with autism

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.

Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

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.

Clinical Neuroscience

[The connection between the socioeconomic status and stroke in Budapest]

VASTAGH Ildikó, SZŐCS Ildikó, OBERFRANK Ferenc, AJTAY András, BERECZKI Dániel

[The well-known gap bet­ween stroke mortality of Eastern and Western Euro­pean countries may reflect the effect of socioeconomic diffe­rences. Such a gap may be present between neighborhoods of different wealth within one city. We set forth to compare age distribution, incidence, case fatality, mortality, and risk factor profile of stroke patients of the poorest (District 8) and wealthiest (District 12) districts of Budapest. We synthesize the results of our former comparative epidemiological investigations focusing on the association of socioeconomic background and features of stroke in two districts of the capital city of Hungary. The “Budapest District 8–12 project” pointed out the younger age of stroke patients of the poorer district, and established that the prevalence of smoking, alcohol-consumption, and untreated hypertension is also higher in District 8. The “Six Years in Two Districts” project involving 4779 patients with a 10-year follow-up revealed higher incidence, case fatality and mortality of stroke in the less wealthy district. The younger patients of the poorer region show higher risk-factor prevalence, die younger and their fatality grows faster during long-term follow-up. The higher prevalence of risk factors and the higher fatality of the younger age groups in the socioeconomically deprived district reflect the higher vulnerability of the population in District 8. The missing link between poverty and stroke outcome seems to be lifestyle risk-factors and lack of adherence to primary preventive efforts. Public health campaigns on stroke prevention should focus on the young generation of socioeconomi­cally deprived neighborhoods. ]

Clinical Neuroscience

[The Comprehensive Aphasia Test in Hungarian]

ZAKARIÁS Lilla, RÓZSA Sándor, LUKÁCS Ágnes

[In this paper we present the Comprehensive Aphasia Test-Hungarian (CAT-H; Zakariás and Lukács, in preparation), an assessment tool newly adapted to Hungarian, currently under standardisation. The test is suitable for the assessment of an acquired language disorder, post-stroke aphasia. The aims of this paper are to present 1) the main characteristics of the test, its areas of application, and the process of the Hungarian adaptation and standardisation, 2) the first results from a sample of Hungarian people with aphasia and healthy controls. Ninety-nine people with aphasia, mostly with unilateral, left hemisphere stroke, and 19 neurologically intact control participants were administered the CAT-H. In addition, we developed a questionnaire assessing demographic and clinical information. The CAT-H consists of two parts, a Cognitive Screening Test and a Language Test. People with aphasia performed significantly worse than the control group in all language and almost all cognitive subtests of the CAT-H. Consistent with our expectations, the control group performed close to ceiling in all subtests, whereas people with aphasia exhibited great individual variability both in the language and the cognitive subtests. In addition, we found that age, time post-onset, and type of stroke were associated with cognitive and linguistic abilities measured by the CAT-H. Our results and our experiences clearly show that the CAT-H provides a comprehensive profile of a person’s impaired and intact language abilities and can be used to monitor language recovery as well as to screen for basic cognitive deficits in aphasia. We hope that the CAT-H will be a unique resource for rehabilitation professionals and aphasia researchers in aphasia assessment and diagnostics in Hungary. ]

Lege Artis Medicinae

[A short chronicle of three decades ]

KAPRONCZAY Katalin

[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.]