Lege Artis Medicinae

[Interdisciplinary forum of MOTESZ - Budapest, 23-25th March, 2006.]

KRAMER Imre

JULY 20, 2006

Lege Artis Medicinae - 2006;16(07)

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Lege Artis Medicinae

[WEGENER’S GRANULOMATOSIS PRESENTING AS MASTOIDITIS: A DIAGNOSTIC CHALLENGE]

NAGY Pál, SZABÓ László, DOMJÁN Gyula, GADÓ Klára, BALOGH Károly

[INTRODUCTION - Wegener's granulomatosis has an uncertain pathomechanism, but is probably autoimmune in origin. In typical cases the mucosa of the nose, paranasal sinuses and of the lower respiratory tract, as well as the lungs and the kidneys are affected. Patients present with sinusitis, recurrent pneumonia or renal disease associated with microhaematuria, pyuria or azotaemia. Fever, polyarthralgia or polyarthritis may also occur. The underlying pathologic changes are necrotizing vasculitis, granulomas and parenchymal necrosis. The diagnosis is based on a combination of the clinical picture, microscopic findings and immunofluorescent demonstration of cANCA. CASE REPORT - A 27-year-old woman presented with symptoms of unilateral mastoiditis. In the following 7 months she underwent 7 operations in 4 hospitals for a locally progressive, destructive process of uncertain etiology showing a septic course. The clinical picture was not specific, the cANCA test was not definitive, and the histologic findings were initially interpreted as nonspecific inflammation. Repeated biopsies, multiple reviews of the microscopic specimens, consultations, differential diagnostic considerations, and, finally, the success of the treatment with corticosteroids and cyclophosphamide led to the diagnosis of Wegener’s granulomatosis. Currently the patient has been in remission for 32 months. CONCLUSION - The definitive diagnosis of Wegener’s granulomatosis, particularly of its localized or limited form, may be problematic despite well-defined diagnostic criteria. Setting up the correct diagnosis may take months or years. In case of unusual respiratory or otological symptoms, and in view of ineffective medical or surgical treatment, Wegener’s granulomatosis has to be considered. An atypical clinical picture, inconclusive histologic, radiologic and laboratory findings warrant the need for close collaboration of various specialists. This is particularly important since state-of-the-art therapy of Wegener’s granulomatosis promises a favourable prognosis.]

Lege Artis Medicinae

[NONINVASIVE MECHANICAL VENTILATION IN CHRONIC OBSTRUCTIVE LUNG DISEASES WITH ACUTE OR CHRONIC RESPIRATORY FAILURE]

BALIKÓ Zoltán

[Over the past decade, the use of noninvasive ventilation in the setting of acute exacerbations of chronic obstructive pulmonary disease (COPD) has gained popularity, and is recommended by evidence-based guidelines. The evidence that it should be effective in chronic COPD is much weaker, and large, prospective, randomised, controlled studies that would also provide a guide for the selection of the best candidates, are still lacking. It has bee established, however, that home mechanical ventilation is certainly beneficial for a well-defined subgroup of patients. This includes the so called “blue bloater” patients (with hypercapnia and polyglobulia), other cases with increased hypercapnia, and patients with "overlapping" syndrome (COPD accompanied by sleep apnoe). Also, for patients with acute respiratory failure who refuse intratracheal intubation noninvasive mechanical ventilation remains the only choice. The past years have unequivocally proved the superiority of the positive pressure ventilation mode for either short term or long term use.]

Lege Artis Medicinae

[Medicine and Literature The Opus of Ferenc Toldy]

KAPRONCZAY Károly

Lege Artis Medicinae

[CONTROVERSIAL ISSUES IN THE TREATMENT OF OPERABLE CERVICAL CANCER]

PETE Imre

[Wertheim hysterectomy as a baseline surgical treatment for patients with FIGO stage 1a2-2a cervical cancer was introduced in 1989 at the National Institute of Oncology, Budapest, Hungary. From then until 2002 when the results were first evaluated 308 such operations were performed. The average 5- and 10-year survival rates were 80% and 75%, respectively. Although these results are comparable to literature data, several issues concerning this group of patients remain to be solved. The participation rate in the Pap test screening programme covered by the national health insurance is only 30%. There is no national database on the current treatment of these patients that could help identify the necessary steps to be taken in order to improve the results. The number of radical hysterectomies performed a year in Hungary, or their outcome, is not known. The treatment scheme varies among the institutes and so does surgical experience. There is no consent regarding treatment policy, preoperative irradiation or chemotherapy, surgical technique, or postoperative management. The improvement of the participation rate in the screening programme, the use of prognostic factors to determine the most appropriate treatment, the role of minimally invasive and fertility-preserving surgery, the preservation of ovaries and the use of neoadjuvant chemotherapy are some of the further issues that need to be discussed. The first step should be data acquisition from all institutes in the country in order to develop and apply uniform treatment guidelines.]

Lege Artis Medicinae

[THE POTENTIAL ROLE OF THE SEMICARBAZIDESENSITIVE AMINE OXIDASE ACTIVITY IN THE DEVELOPMENT OF DIABETIC RETINOPATHY]

DURA Eszter

[Recent data suggest that the copper-containing semicarbazide-sensitive amine oxidase enzyme (SSAO) may play a role in vascular endothelial damage through conversion of certain endogenous monoamines, such as methylamine, into cytotoxic aldehydes, hydrogen peroxide and ammonia. SSAO is present in various human tissues and in the serum. Elevated SSAO activities have been reported in patients with both types of diabetes mellitus. We have demonstrated that the activity of serum SSAO is significantly higher in type 2 diabetic patients with high-risk proliferative diabetic retinopathy compared to those without retinopathy. Our clinical results support the hypothesis that elevated SSAO activity may be involved in the pathogenesis of microvascular diabetic late complications, such as retinopathy. The enzymatic conversion of the endogenous monoamines (e.g. methylamine, aminoacetone) into toxic aldehydes and hydrogenperoxide may be one of the possible mechanisms of the development of microangiopathy. Also, the vascular adhesion protein-1 (VAP-1) function of the molecule can cause leukostasis and leukocyte activation through increased leukocyte adhesion, resulting in worsening of the capillary circulation and hypoxia. Further prospective, larger studies are needed to elucidate the role of the possible association between serum SSAO activity and highrisk proliferative retinopathy in patients with type 2 diabetes. The pharmacological manipulation of SSAO activity might be an interesting new concept for prevention and treatment of diabetic retinopathy.]

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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

[Interdisciplinary approach of vestibular system impairment]

PONGRÁCZ Endre

[In the first part of this review the definition of vertigo/dizziness was discussed. The major difference between the two signs is the exsistence of the direction, which is specific for vertigo. Dizziness is a frequent complaint in the clinical practice. Its frequency is increasing with advance of age, to intimate the play of declining cognitive process in the pathogenesis of its. The popular health significance of vertigo is in the rowing number of the patients. The onset of the most cases with acute vertigo appears between secundums and minutes so the patients will be provided in circumstances of emergency department. First of all three form schould be take into account: neuronitis vestibularis, benign paroxysmal positional vertigo and Meniere syndrome. Without tipical periferal signs of vertigo, central cause should be searched, principally stroke (lysis possibility). The differential diagnose of the different dizzeness/vertigo forms according to the elapsed time of the onset or congenital and acquired nystagmus was created in tables. The recommendations of the therapy of acute and chronic dizziness/ vertigo syndroms are, lack of results of evidence based trials doubtful. The more often used drugs based on clinical trials are discussed as vinpocetine, betahistine and piracetam. The in vitro and in vivo data suggest that the last molecule is eligible to use both in periferal and central type of vertigo syndroms.]

Hungarian Radiology

[The Spring Event of the Society of Hungarian Radiologists Ultrasonography Section Budapest, 28 March 2003]

HARKÁNYI Zoltán, MORVAY Zita

Lege Artis Medicinae

[Nonerosive Reflux Disease – An Interdisciplinary Congress]

VARRÓ Vince

Clinical Neuroscience

[Abstracts of the IBRO International Workshop Budapest, 29–31 January, 2004 ]