Lege Artis Medicinae

[Autoimmune pancreatitis]

JAKAB Lajos

NOVEMBER 19, 2006

Lege Artis Medicinae - 2006;16(11)

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Lege Artis Medicinae

[Understanding Pathogenesis Better]

CZAKÓ László

Lege Artis Medicinae

[THE PHENOMENON OF BURNOUT IN VIEW OF THE HUNGARIAN AND INTERNATIONAL LITERATURE]

KOVÁCS Mariann

[There is plethora of international publications on the topic of burnout among health professionals. The most comprehensive study in Hungarian on this issue was written fifteen years ago by psychiatrist Sandor Fekete. In the past fifteen years there has been a growing interest among both the scientific community and the public in studies on health psychology, behavioural sciences, sociology of health and quality of life as well as in the harmful effects of work stress. The first part of the study presents clarification of the concept of burnout, its development, course, assessment and treatment, whereas the second part gives a brief overview of the history of burnout research in the past thirty years with special focus on the most recent international and Hungarian literature, as well as on changes in research trends.]

Lege Artis Medicinae

[In Memoriam Sándor Gerő (1904-1992)]

EMED Alexander

Lege Artis Medicinae

[Sexual disturbances in hepatological disease]

GYOVAI Gabriella

Lege Artis Medicinae

[A NEW WAY TO TREAT ALLERGIC ASTHMA: ANTI-IGE THERAPY]

MAGYAR Pál

[Immunoglobulin E (IgE) plays a central role in the pathogenesis of the inflammation of the bronchial mucosa and airway hyperreactivity, which in turn produces the symptoms of allergic bronchial asthma. Omalizumab, the recently developed anti-IgE monoclonal antibody binds to the Cε3 region of the IgE molecule and thus prevents binding of the IgE to the surface of FCεRI receptor bearing cells (mast cells, basophils and antigen presenting dendritic cells). In the absence of cell-bound IgE, these cells are not activated and thus do not release inflammatory mediators and proinflammatory cytokines upon allergen exposition. Treatment with omalizumab significantly decreases the number of bronchial mucosal eosinophils and FCεRI positive cells, and the FCεRI receptor expression of the latter. Double blind controlled clinical studies have demonstrated that omalizumab treatment reduces the number of exacerbations and emergency room visits, the β2-agonist requirement and the dose of inhaled steroids, improves exspiratory airflow limitation, asthmatic symptoms and asthma-related quality of life in patients with moderate to severe allergic asthma. Low baseline FEV1, the use of high dose inhaled corticosteroids and a history of emergency asthma treatment in the past year are significant predictors of a better response to omalizumab. Omalizumab is tolerated well by patients. With the exception of local skin reactions, no significant difference in adverse events between patients taking omalizumab and control groups have been reported. According to the GINA (Global Initiative for Asthma) stepwise therapy protocol of asthma, omalizumab is indicated for severe asthmatics whose symptoms can not be controlled by inhaled corticosteroids and long-acting β2 agonists.]

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Isolated hypoglossal nerve palsy due to a jugular foramen schwannoma

ÖZTOP-CAKMAK Özgür, VANLI-YAVUZ Ebru, AYGÜN Serhat, BASTAN Birgül, EGEMEN Emrah, SOLAROGLU Ihsan, GURSOY-OZDEMIR Yesemin

Introduction – Although the involvement of the hypoglossal nerve together with other cranial nerves is common in several pathological conditions of the brain, particularly the brainstem, isolated hypoglossal nerve palsy is a rare condition and a diagnostic challenge. Case presentation – The presented patient arrived to the hospital with a history of slurred speech and an uncomfortable sensation on his tongue. Neurological examination showed left-sided hemiatrophy of the tongue with fasciculations and deviation towards the left side during protrusion. Based on the clinical and MRI findings, a diagnosis of hypoglossal nerve schwannoma was made. Discussion – Hypoglossal nerve palsy may arise from multiple causes such as trauma, infections, neoplasms, and endocrine, autoimmune and vascular pathologies. In our case, the isolated involvement of the hypoglossal nerve was at the skull base segment, where the damage to the hypoglossal nerve may occur mostly due to metastasis, nasopharyngeal carcinomas, nerve sheath tumors and glomus tumors. Conclusion – Because of the complexity of the region’s anatomy, the patient diagnosed with hypoglossal nerve schwannoma was referred for gamma knife radiosurgery.

Clinical Neuroscience

Myasthenia gravis, Guillain-Barré syndrome, or both?

ERDOGAN Cagdas, TEKIN Selma, ÜNLÜTÜRK Zeynep, GEDIK Korkut Derya

Myasthenia gravis (MG) and Guillain-Barré syndrome (GBS) are autoimmune disorders that may cause weakness in the extremities. The coexistence of MG and GBS in the same patient has rarely been reported previously. A 52-year-old male presenting with ptosis of the left eye that worsened with fatigue, especially toward evening, was evaluated in our outpatient department. His acetylcholine receptor antibody results were positive, supporting the diagnosis of MG. His medical history revealed a post-infectious acute onset of weakness in four extremities, difficulty in swallowing and respiratory failure, which was compatible with a myasthenic crisis; however, his nerve conduction studies and albuminocytologic dissociation at the time were compatible with GBS. With this case report, we aimed to mention this rare coincidental state, discuss possible diagnoses and review all other similar cases in the literature with their main features.

Hungarian Immunology

[Paraneoplastic diseases of the locomotor system]

ANDRÁS Csilla, SZÁNTÓ János, SZEKANECZ Zoltán, CSIKI Zoltán, ILLÉS Árpád, SZEKANECZ Éva, DANKÓ Katalin

[Paraneoplasias in rheumatology can be present in different forms of arthropathies, myopathies. In addition, we often see atypical forms of systemic autoimmune diseases. Vasculitis is mainly associated with lymphoproliferative diseases. The direct invasion of bones and joints is not present in paraneoplasia, as this entity is a collection of symptoms generated by the tumour itself by producing biologic mediators, hormones, peptides, antibodies, cytotoxic lymphocytes, autocrine and paracrine mediators. Recognition of paraneoplasia is of outmost importance because it attires attention to the presence of tumour in the organism and thus enables us for early treatment of the malignancy. Monitoring of the severity of paraneoplastic symptoms serves as a marker for determination of efficacy of the targeted oncological therapy. On the other side, because these severe symptoms affect the patient's quality of life and can lead even to death their in time recognition and treatment is extremely important.]

Lege Artis Medicinae

[Idiopathic retroperitoneal fibrosis (Morbus Ormond) associated with severe cardiomyopathy in a young man: a case report]

KOVÁCS Erzsébet, ZEHER Margit, SZÁNTÓ János, PÁSZTOR Éva, TÓTH László, PFLIEGLER György

[INTRODUCTION - The cause of idiopathic retroperitoneal fibrosis is unknown, but probably involves a chronic, nonspecific, autoimmune inflammation. This rare disease mostly affects middle-aged or elderly people, it hardly ever occurs in young people. It causes obstruction of the urinary tract, postrenal insufficiency as well as functional disorder of vital organs in the retroperitoneum and its surrounding area. The diagnosis is based on diagnostic imaging and histological examinations. Surgical treatment of the urinary obstruction is only efficient in the long-term if combined with immunomodulatory treatment. CASE REPORT - A young, 34-year-old man who presented with oedema in his legs was diagnosed with retroperitoneal fibrosis. Autoimmune serology was negative. He needed temporary nephrostomy on the right side. Treatment with steroid and colchicine was initially successful. His severe cardiomyopathy improved gradually. No regression occurred with tamoxifen, so we started to administer azathioprine with methylprednisolone. The control abdominal CT showed stagnation of the process. CONCLUSIONS - Because of the rarity of this disease, no randomised controlled trials are available regarding its treatment. The aim of treatment is to eradicate the symptoms, solve the urinary obstruction, maintain renal function and prevent progression of the disease. Improvement may be achieved by corticosteroids, cyclophosphamide, azathioprine, leflunomide, mycophenolate mofetil and tamoxifen. To our knowledge, this case is unique as no similar case with severe cardiomyopathy and idiopathic retroperitoneal fibrosis has been reported in the literature previously.]

Lege Artis Medicinae

[ENTERAL ADMINISTRATION OF N-3 POLYUNSATURATED FATTY ACIDS IN ACUTE PANCREATITIS]

LÁSZTITY Natália, HAMVAS József, BÍRÓ Lajos, NÉMETH Éva, MAROSVÖLGYI Tamás, DECSI Tamás, PAP Ákos, ANTAL Magda

[BACKGROUND - The main determinant of outcome in acute pancreatitis is the extent of inflammation and pancreatic necrosis. Early administration of n-3 polyunsaturated fatty acids (PUFAs) may prevent the development of severe complications through modulation of eicosanoid synthesis and cytokine release. PATIENTS AND METHODS - In the prospective, randomised clinical trial 14 patients with acute pancreatitis received n-3 PUFAs (3.3 g/day for 5- 7 days) as a supplement to their enteral formula in the form of fish oil, and another 14 patients receiving enteral nutrition served as a control group. Measurements of erythrocyte superoxidedysmutase activity, serum total antioxidant status, C-reactive protein and praealbumin concentrations were performed at admission and at day 3, 7 and 14. Beside routine laboratory and imaging examinations, the fatty acid and vitamin A and E concentrations of the serum lipid fractions were also determined at admission and at day 7 of the jejunal nutrition. The endpoints of the study were the duration of hospitalisation, the duration of jejunal nutrition and the frequency of complications. RESULTS - A significantly higher superoxidedysmutase activity was observed in patients receiving n-3 fatty acids at day 3 of the treatment. The n-3 to n-6 long chain PUFA ratio increased significantly in the serum lipids of the patients receiving n-3 PUFA supplementation, whereas remained unchanged in the controls. Supplementation resulted in a significant decrease in the length of hospitalisation (13.1±6.7 vs. 19.3±7.2 days, p<0.05) and jejunal feeding (10.6±6.7 vs. 17.6±10.5, p<0.05). Complications developed in 6/14 (42%) of the treated group and in 9/14 (64%) of the control patients. CONCLUSION - Enteral administration of n-3 PUFAs in acute pancreatitis may promote earlier recovery by moderating inflammation.]