Lege Artis Medicinae

[Autoimmune pancreatitis]

JAKAB Lajos

NOVEMBER 19, 2006

Lege Artis Medicinae - 2006;16(11)

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Lege Artis Medicinae

[In Memoriam Sándor Gerő (1904-1992)]

EMED Alexander

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

[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.]

Lege Artis Medicinae

[I Still Have So Much to Do... A Discussion with Éva Csatár MD]

VARGA Brigitta

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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.

Clinical Neuroscience

[LADA type diabetes, celiac diasease, cerebellar ataxia and stiff person syndrome. A rare association of autoimmune disorders]

SOÓS Zsuzsanna, SALAMON Mónika, ERDEI Katalin, KASZÁS Nóra, FOLYOVICH András, SZŰCS Anna, BARCS Gábor, ARÁNYI Zsuzsanna, SKALICZKI József, VADASDI Károly, WINKLER Gábor

[Celiac disease - in its typical form - is a chronic immunemediated enteropathy with typical clinical symptoms that develops against gliadin content of cereal grains, and is often associated with other autoimmune diseases. In cases of atypical manifestation classic symptoms may be absent or mild, and extra-intestinal symptoms or associated syndromes dominate clinical picture. The authors present a longitudinal follow-up of such a case. A 63-years old woman was diagnosed with epilepsy at the age of 19, and with progressive limb ataxia at the age of 36, which was initially thought to be caused by cerebellar atrophy, later probably by stiff person syndrome. At the age 59, her diabetes mellitus manifested with type 2 diabetic phenotype, but based on GAD positivity later was reclassified as type 1 diabetes. Only the last check-up discovered the celiac disease, retrospectively explaining the entire disease course and neurological symptoms. By presenting this case, the authors would like to draw attention to the fact that one should think of the possibility of celiac disease when cerebellar ataxia, progressive neurological symptoms and diabetes are present at the same time. An early diagnosis may help to delay the progression of disease and help better treatment.]

Lege Artis Medicinae

[AUTOIMMUNE PANCREATITIS - AN UNDERDIAGNOSED DISEASE?]

CZAKÓ László

[Autoimmune pancreatitis is a recently recognized type of chronic pancreatitis that is clearly distinct from alcoholic chronic pancreatitis. Its clinical symptoms include jaundice, abdominal pain, weight loss and diabetes mellitus. It may be associated with other autoimmune diseases. IgG levels are elevated and autoantibodies can be detected. Pancreatic imaging reveals a diffuse enlargement of the pancreas and irregular narrowing of the main pancreatic duct. The characteristic histological features are lymphoplasmacytic infiltration and fibrosis. Autoimmune pancreatitis responds dramatically to steroid therapy, in contrast to other types of chronic pancreatitis, which hardly respond to any of the various therapies. It is important to be aware of this disease because it may be mistaken for other forms of chronic pancreatitis or pancreatic cancer, which leads to pancreatic resection when steroid treatment would be sufficient. This review discusses the clinical, laboratory, histological and imaging findings that are seen in autoimmune pancreatitis with particular focus on diagnosis. With the improvement of the diagnostic work-up less unnecessary pancreatic resections are expected to happen in patients with autoimmune pancreatitis.]

Lege Artis Medicinae

[ACUTE DERMATOMYOSITIS ASSOCIATED TO THE CARCINOMA OF THE PROSTATE]

TÁLLAI Béla, MORSHED Ali Salah, FLASKÓ Tibor, PONYI Andrea, DANKÓ Katalin, TÓTH Csaba

[INTRODUCTION - In some cases of polymyositis/ dermatomyositis (PM/DM) of autoimmune origin, different malignant tumours can initiate the difficult cascade mechanisms at cell level leading to the rapid weakness of the skeletal muscles. Till now, in the international literature only four cases of PM/DM associated with cancer of prostate has been reported. CASE REPORT - Authors present a case of a 57 yearold male patient, where weakness in patient's movements developed leading to total immobility in 3 months. Purple discoloration developed on his hand and face. Significantly elevated creatin kinase (CK) levels and blood sediment rate with mild anaemia were observed during laboratory examinations. Dysphagia and lack of appetite resulted in the loss 10 kgs in body weight. Both clinical evaluation, elevated serum CK level, skin symptomes, positive electromyography and muscle biopsy confirmed acute definitive dermatomyositis. Urological examination revealed a palpable hard area at the right lobe of the prostate. Prostate biopsy confirmed the presence of carcinoma in the right lobe of the prostate. There was no sign or symptome referring to either local propagation or distant manifestation. Therefore, radical prostatectomy was performed, the tumorous prostate and both seminal vesicles were removed. Histological examination proved malignant focus in the right lobe of the prostate. After the operation patient gradually became stronger, corticosteroid medication were decreased then stopped. Patient's original muscle power and movement recovered and his previous body weight was regained. During the regular control examinations all results of laboratory tests are in normal range. CONCLUSION - In the background of some autoimmune diseases malignant tumours can be revealed. It is rare when urological neoplasms initiative the process. In men with PM/DM commencing beyond 50 years of age it is necessary to think of the presence of prostate cancer, which can be cured by performing radical operation in appropriate time.]