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Lege Artis Medicinae

OCTOBER 21, 2020

[Gene modified immune cells: New weapons not exclusively against cancer]

SZÖŐR Árpád

[The oncological breakthrough of the last decade was the application of CD19-specific CAR T cells in different hematologic diseases. Experience gained by clinical trials, coupled with investments of the private stakeholders and the pharmaceutical industry resulted not only in commercial release to the public of already developed CAR T cell products, but drew the atten­tion of many researchers to the potentials of new type immune cells, and their non-oncological administration. This study aims to present briefly those preclinical applications, which approved successfully the administration of CAR T cells in autoimmune and infectious diseases.]

Lege Artis Medicinae

JULY 01, 2020

[Persisting Hashimoto’s thyroiditis converting to Graves’ disease]

TÓTH Géza

[Graves’disease and Hashimoto’s thyroiditis are the two most important types of autoimmune thyroid dis­eases. Autoimmune hyperthyroidism commonly leads later on to hypothyroidism. The conversion from persisting Hashimoto-thyroiditis to hyperthyroidism is rare in the literature. The author presents the cases of two patients, whose Hashimoto’s thyroiditis treated with thyroxin for years, changed into Graves’ hyperthyroidism sponta­neously. CASE REPORT – The patients had been diagnosed with autoimmune hypothyroidism since several years. The clinical symp­­toms, the low peripheral hormone le­vels, the high level of antithyroid antibo­dies and the ultrasound imaging confirmed the hypertrophic form of Hashimoto’s thy­roiditis. After several years of high dose le­vothyroxin treatment, clinical symptoms of hyperthyroidism have appeared. After omit­ting the substitution, the thyroid hormone levelled off at high values and the level of anti-TSH receptor antibodies raised too. The diffuse, obviously increased blood flow of the thyroid glands, and in one of the patients the thyroid scan, confirmed the Graves’ disease. During the thyreostatic treatment, the symptoms of the patients disappeared, they became euthyreoid and the antibody levels decreased as well. The Graves’ disease and the Hashimoto’s thyroiditis have many common features. These immunological, ge­netic and other common features enable the mutual transition of these two diseases.]

Clinical Neuroscience

JANUARY 30, 2020

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.

Lege Artis Medicinae

JULY 20, 2019

[Severe polymyositis associated with multiplex pulmonary abscesses]

SZABÓ Katalin, VINCZE Anett, NAGY-VINCZE Melinda, DANKÓ Katalin, GRIGER Zoltán

[INTRODUCTION – Idiopathic inflammatory myopathies are heterogeneous autoimmune diseases characterized by immune mediated inflammation of the skeletal muscles. CASE REPORT – A case of a 62-year-old male patient with severe proximal muscle weakness, elevated creatine kinase and swallowing difficulity is presented. Electromyography showed myogenic pattern, thus probable polymyositis was diagnosed. Radiological examination has confirmed bilateral multiplex lung lesions, which were caused by the possibility of tumor, tuberculosis, vasculitis and abscess as well. The condition of the patient deteriorated, nasogastric feeding, high dose steroid treatment was initiated, which reduced the patient's creatinine kinase values, but muscle strength was not changed. Based on the results of various investigations, the condition of the patient was finally confirmed by the development of myositis, resulting dysphagia, chronic aspiration, and multiplex lung abscess. Antibiotic therapy, steroid treatment was continued and finally intravenous immunoglobulin treatment was administered. The condition of the patient gradually improved, the swallowing dysfunction disappeared, and the lung abscesses were resolved. As a result of physiotherapy and rehabilitation treatment, the patient could walk again. CONCLUSIONS – Nasogastric feeding is recommended to prevent aspiration in the case of myositis-associated dysphagia. In case of steroid refractory therapy, the use of intravenous immunoglobulin may be effective. ]

Clinical Neuroscience

JULY 30, 2019

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.

Lege Artis Medicinae

OCTOBER 20, 2018

[Gene modified T cells against cancer]

SZÖŐR Árpád

[Chimeric antigen receptor modified (CAR) T cells are hailed as a revolutionary breakthrough in the field of oncology. CAR T cells were first applied, with outstanding success, in the treatment of various leukaemias, yielding unprecedented antitumor activity and long periods of disease free survival. Following the success of CAR T cell therapy in leukaemias, solid tumors should now be targeted. These are more complex targets, therefore CAR T cell therapy needs to be further optimized for this purpose. Also, some unfortunate side effects, including the potentially deadly global inflammation called cytokine storm have to be minimized and possibly even eradicated. The next decade will be an exciting time to define whether this therapy which is yet exclusively used for cancer patients is also successful in the treatment of other diseases. In a recent study, T cells reengineered with CAR derived chimeric autoantibody receptors (CAAR) efficiently prevented disease progression in pre-clinical animal models simulating the serious autoimmune disorder, pemphigus vulgaris. Therapy was based on CAAR constructs which have the unique ability to selectively recognize and eliminate the B-cell clones secreting autoantibodies against a self-protein, thus playing a key role in disease pathogenesis and progression. In this review, we would like to give an overview about the history of the CAR T-cell concept, summarize briefly the currently running clinical trials, and discuss the challenges and future prospects of CAR T-cell therapy.]

Clinical Neuroscience

SEPTEMBER 30, 2018

Acute motor and sensory axonal neuropathy associated with Sjögren’s syndrome

ETHEMOGLU Ozlem, KOCATÜRK Özcan, TARINI Zeynep Emine

Sjögren’s syndrome (SS) is an autoimmune disease with mononuclear cell infiltration and destruction of the lacrimal gland and salivary glands, which cause dryness of the eyes and mouth. The most common neurological condition seen in SS is peripheral neuropathy. Initial manifestation of SS as an acute fulminant peripheral neuropathy is extremely rare. We report a 42-year-old patient presenting with acute motor sensory-axonal neuropathy in the presence of SS. She showed partial response to intravenous immunoglobulin but favourable clinical improvement was seen after initiation of corticosteroid treatment.

Lege Artis Medicinae

AUGUST 30, 2018

[How to diagnose idiopathic pulmonary fibrosis. Part 2]

HORVÁTH Ildikó, KERPEL-FRONIUS Anna, HARKÓ Tünde

[Idiopathic pulmonary fibrosis is a severe irreversible lung disease with a progressive course. The disease onset is hard to discover due to the unspecific signs and symp­toms. It occurs mainly in elderly people. In the past decades its prevalence has increased continuously. Physical examination, restrictive pattern on lung function test with decreased diffusion capacity are characteristic features of the disease. Chest X-ray showing fibrotic pattern also points toward the diagnosis of idiopathic pulmonary fibrosis. Differential diagnosis is based on high resolution komputertomográfy. Diag­nosis of IPF is based on the appearance of usual interstitial pneumonia pattern together with the lack of external risk factors and autoimmune or other diseases also known to cause this pattern seen on chest imaging. If no firm diagnosis can be built lung biopsy is required. Multidis­ciplinary teams from clinician, radiologist and pathologist are set in predefined centres that could provide care with novel antifibrotic drugs. These can slow disease progression and are in the frontline in the treatment of the disease. Further research is required to understand the pathomechanism and foster the discovery of further treatment options. ]

Lege Artis Medicinae

JULY 20, 2017

[Eosinophil granulomatous polyangiitis]

WITTMANN Zsófia, KARDOS Magdolna, FINTHA Attila, KERKOVITS Lóránt, AMBRUS Csaba

[Due to the high prevalence of kidney involvement, patients with systemic autoimmune disorders, also including small vessel vasculitides are frequently seen in neph-rology centers. Activated neutrophils attacking the wall of various blood vessel are key features in these diseases, leading to bleeding, occlusion, ischaemia and tissue necrosis. This latter finding is reflected in the term necrotising vasculitis. In this paper, we present a case of eosinophil ganulomatous polyangiitis (EGPA, formerly called Churg-Strauss syndrome), the least common form of ANCA associated small vessel vasculitides. We found it very interesting but not uncommon that our patient was admitted to the nephrology ward with vague symptoms that became more and more suggestive and typical for vasculitis during our observation, guiding us to the right diagnosis. Timely and appropriate immunosuppressive therapy based on immunology lab report and histology findings resulted in good response and remission of the disease in our patient. ]

Lege Artis Medicinae

MAY 20, 2017

[Plasmaferesis for the treatment of acute pancreatitis related to extreme hypertriglyceridemia]

BELOPOTOCZKY Gábor, ORENTSÁK Áron, PÁRTOS Gergely, ARÁNYI József, HARIS Ágnes, PENYIGE József, SIKE Róbert, DEMETER Pál

[We present the case of a 43-year-od wo­man, with pancreatitis related to extreme - higher than 100 mmol/L - secondary hy­pertriglyceridemia. Fast clinical improvement and rapid regression of pancreatitis were achieved by appropriate therapy of pancreatitis, the diabetic metabolic disturbance, correcting microcirculation and treating hypertriglyceridemia with plasmapheresis. The authors underline, that the complex therapeutic approach and early plasmapheresis in pancreatitis related to hypertriglyceridemia may prevent necrosis and more severe, even fatal, outcome of the disease. ]