Lege Artis Medicinae

[Report on the 1st Debrecen Medical Days]

BRÚGÓS Boglárka, JUHÁSZ Mária

JULY 20, 2004

Lege Artis Medicinae - 2004;14(07)

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Lege Artis Medicinae

[THE GENETICS OF DIABETES MELLITUS]

KORÁNYI LÁSZLÓ, PÁNCZÉL Pál

[The number of diabetic patients will be doubled in the coming decades reaching 300 million for year 2025. The number of type 1 diabetics will also be increased but the majority of it will result from the increased number of type 2 diabetics. All types of diabetes are the consequence of a combination of genetic susceptibility and environmental factors, meaning that the prevention of diabetes epidemic cannot be done without the clarification of the genetic background. Significant progression has happened in the discovery of the genetic background of type 1 diabetes mellitus. It was helped by the etiologic classification of the disease: with the new classification the patient groups became more homogeneous. The HLA system is responsible for about 50-70% of the genetic risk while the effects of other genetic factors contribute 1-2% of the genetic susceptibility, respectively. Presently 25 gene regions are known as the different genetic factors of type 1 diabetes mellitus. Regarding the HLA system, the genes and pathomechanism causing the disease are not known. The classification of diabetes mellitus can be based on the HLA type while the predictability of type 1 diabetes mellitus is helped by the HLA type and the INS-VNTR. Much less is known about the genetic background of the polygenic type 2 diabetes mellitus. Its manifestation is now happening at younger age before. The best-fit genetic model consists of only a few genes with moderate effect superimposed on a polygenic background. Several „candidate” genes participating in the impaired insulin secretion and insulin action have already been investigated as the genes responsible for type 2 diabetes. These data showed the specificity in the population and most showed mild or modest association with the disease. Genomewide scans have resulted a number of significant diabetes susceptibility genes specific for a variety of populations, but these investigations have only resulted in the isolation of one gene (calpain 10) that is thought to contribute to type 2 diabetes. Most recent genomewide scans found loci on chromosome 20 in two different populations with significant segregation of type 2 diabetes. These loci are near to the region harboring the transcription factor hepatocyte nuclear factor genes. The transcription regulator HNF family is responsible for the regulation of the expression of several genes participating in the function of liver and pancreatic islet becoming a strong candidate for being a diabetes gene.]

Lege Artis Medicinae

[FIRST EXPERIENCES WITH INFLIXIMAB THERAPY IN CROHN’S DISEASE - RESULTS OF A HUNGARIAN MULTICENTRE STUDY]

SALAMON Ágnes, SIMON László, NEMESÁNSZKY Elemér

[INTRODUCTION - In recent years a number of clinical trials have been proved that infliximab, a monoclonal chimeric antibody against tumour necrosis factor (one of the proinflammatory cytokines) is an effective induction and maintenance therapy in severe and fistulizing Crohn’s disease. The authors summarize the results of the first Hungarian, open, multicenter clinical trial with biological treatment of Crohn’s disease. PATIENTS AND METHODS - Overall 74 patients in 9 study-centers were enrolled and allocated into the following two groups: 29 patients (31%) with severe, active, therapy-resistent clinical picture of Crohn’s disease and 45 individuals (61%) showing fistulizing forms of the disease. Infliximab was administered iv. in a dose of 5 mg/kg body mass one single occasion in patients formed the first group and altogether three times in cases suffering of fistulizing form of the disease. RESULTS - In active Crohn cases infliximab exerted its beneficial effect within 4 weeks (58% of patients improved) which was most pronounced at week 8th (79% of the patients was in better condition). However, in cases of fistulizing form longer period is neccessary for developing the effect. In 76% of treated patients proved to be efficacious within 14 weeks but later on flare-ups were observed. Concomittantly administered immunsuppressive agents did not altered the beneficial effect of infliximab. CONCLUSIONS - The results are proving the benefit of infliximab induction therapy both in cases of severe, active or in fistulizing forms of the disease. The article reviews the indication of infliximab treatment and provides a „user’s guide” for practizing clinicians.]

Lege Artis Medicinae

[LATE COMPLICATION OF EXTRANODAL LYMPHOMA FOLLOWING DERMATOMYOSITIS]

VÁNCSA Andrea, PONYI Andrea, CONSTANTIN Tamás, GERGELY Lajos, DANKÓ Katalin

[INTRODUCTION - Intramuscular follicular Bcell lymphoma is a rare entity of malignant lymphomas complicating the disease course of dermato/polymyositis. CASE REPORT - The authors report a female patient who was diagnosed with dermatomyositis in 1963 (age of 36). She was given steroid and hydroxychloroquine therapy. In 1973 she was treated with steroid for Boeck sarcoidosis. In 1999, she was treated for steroid therapy again for reoccurence of dermatomyositis. In 2000, a histological diagnosis of primary intramuscular follicular lymphoma was established from the right thigh region. She was given several courses of polychemotherapy and radiation therapy, but the lymphoma was difficult to control, and she died of progressive disease in 2003. CONCLUSION - It is an interesting case as 37 years elapsed since the diagnosis of dermatomyositis before the lymphoma has started. We could not state a definite relation between the myositis and the lymphoma but the increased frequency of autoimmune diseases is probably due to immune regulatory problems, that have eventually led to the appearance of the rapidly growing indolent lymphoma.]

Lege Artis Medicinae

[THE PSYCHO-SOCIAL BACKGROUND FACTORS OF INDUCED ABORTIONS BASED ON A REPRESENTATIVE NATIONAL SURVEY]

GYŐRFFY Zsuzsa, LŐRINCZ Jenő, ÁDÁM Szilvia, KOPP Mária

[INTRODUCTION - In Hungary the number of induced abortions is three times higher than in European Union countries. The exploration of the bio-psycho-social factors in the background of abortions may serve as an instrument to develop effective prevention. METHODS - The Hungarostudy 2002 national representative study included 12,634 interviewed subjects. The sample represents the Hungarian population above the age of 18, according to gender, age and geographical location. The aim of the health survey was to analyse the physical and psychological status of the Hungarian population as well as the psychosocial risk factors. The prevalence of abortion in the female sub-sample was 22%. We analysed the background factors of abortion with the help of a statistical analysis. RESULTS - The risk factors behind abortions can be grouped as: physical abuse on behalf of partners and/or parents, attachment disorder and early traumatisation, lack of social support, low financial status and inadequate social environment. These factors have all shown significant connection to abortions. Suicide attempts and suicidal thoughts, high Beck Depression (BDI) scores, smoking and alcohol consumption are also significant among those who had abortions. CONCLUSION - An important aim of the Hungarostudy 2002 survey and the follow-ups is to serve a more extensive and effective prevention by exploring the background factors of induced abortions.]

Lege Artis Medicinae

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MATOS Lajos

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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

[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

Clinical Neuroscience

Autonomic nervous system may be affected after carpal tunnel syndrome surgery: A possible mechanism for persistence of symptoms after surgery

ONDER Burcu, KELES Yavuz Betul

After carpal tunnel surgery, some patients report complaints such as edema, pain, and numbness. Purpose – The aim of this study was to evaluate autonomic nervous system function in patients with a history of carpal tunnel surgery using sympathetic skin response (SSR). Thirty three patients (55 ±10 years old) with a history of unilateral operation for carpal tunnel syndrome were included in the study. The SSR test was performed for both hands. Both upper extremities median and ulnar nerve conduction results were recorded. A reduced amplitude (p=0.006) and delayed latency (p<0.0001) were detected in the SSR test on the operated side compared to contralateral side. There was no correlation between SSR and carpal tunnel syndrome severity. Although complex regional pain syndrome does not develop in patients after carpal tunnel surgery, some of the complaints may be caused by effects on the autonomic nervous system.

Clinical Neuroscience

Simultaneous subdural, subarachnoideal and intracerebral haemorrhage after rupture of a peripheral middle cerebral artery aneurysm

BÉRES-MOLNÁR Anna Katalin, FOLYOVICH András, SZLOBODA Péter, SZENDREY-KISS Zsolt, BERECZKI Dániel, BAKOS Mária, VÁRALLYAY György, SZABÓ Huba, NYÁRI István

The cause of intracerebral, subarachnoid and subdural haemorrhage is different, and the simultaneous appearance in the same case is extremely rare. We describe the case of a patient with a ruptured aneurysm on the distal segment of the middle cerebral artery, with a concomitant subdural and intracerebral haemorrhage, and a subsequent secondary brainstem (Duret) haemorrhage. The 59-year-old woman had hypertension and diabetes in her medical history. She experienced anomic aphasia and left-sided headache starting one day before admission. She had no trauma. A few minutes after admission she suddenly became comatose, her breathing became superficial. Non-contrast CT revealed left sided fronto-parietal subdural and subarachnoid and intracerebral haemorrhage, and bleeding was also observed in the right pontine region. The patient had leucocytosis and hyperglycemia but normal hemostasis. After the subdural haemorrhage had been evacuated, the patient was transferred to intensive care unit. Sepsis developed. Echocardiography did not detect endocarditis. Neurological status, vigilance gradually improved. The rehabilitation process was interrupted by epileptic status. Control CT and CT angiography proved an aneurysm in the peripheral part of the left middle cerebral artery, which was later clipped. Histolo­gical examination excluded mycotic etiology of the aneu­rysm and “normal aneurysm wall” was described. The brain stem haemorrhage – Duret bleeding – was presumably caused by a sudden increase in intracranial pressure due to the supratentorial space occupying process and consequential trans-tentorial herniation. This case is a rarity, as the patient not only survived, but lives an active life with some residual symptoms.

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.