Search results

Clinical Oncology

APRIL 10, 2019

[Metals and cancer]

VETLÉNYI Enikő, RÁCZ Gergely

[We often tend to forget about our environment when looking for the origin of a disease. Inhaled air, drinking water and food, substances in contact with the skin all have an effect on the human body. Metals are indispensable parts of our everyday lives, their mining, processing and use cause a continuous exposure to them. Metal exert their effects on the body in various ways. Many of them are essential for maintaining homeostasis, but excessive or harmful metal intake can lead to health damage, including tumour formation through multiple attack points. Metals substitute each other during different transport processes and in the structure of proteins, they cause oxidative stress and bind to DNA, thereby damaging it. Applying them appropriately, the proapoptotic effect of the metal compounds is brought to the fore, thus becoming a therapeutic tool for tumours. Nowadays, platinum(II) compounds are widely used as chemotherapeutic agents and there are many ongoing studies to fi nd metal compounds with an ideal therapeutic and side-effect profi le. The aims of this article were to draw the attention to the dangers of metals in relation to cancer and to highlight their diverse application possibilities in current and future cancer therapy and diagnostics.]

Clinical Neuroscience

SEPTEMBER 30, 2020

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEN Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

Clinical Neuroscience

JULY 30, 2019

A case report of Morvan syndrome

AYTAC Emrah, ACAR Türkan

Morvan syndrome is a rare disease characterized by peripheral nerve hyperexcitability, encephalopathy, dys­autonomia and significant insomnia. The patient, who was included in the present study, was followed-up at our clinics for confusion, myokymia, hyperhidrosis, epileptic seizures, tachycardia, agitation, hypokalemia, and hyponatremia. The cranial MRI of the patient demonstrated hyperintensities at the T2 and FLAIR sections of the medial temporal lobe and insular lobes. Electromyography and neurotransmission examination results were concordant with peripheral nerve hyperreactivity. Contactin-associated protein-like 2 antibodies and leucine-rich glioma inactivated protein 1 antibodies were detected as positive. The patient was diagnosed with Morvan syndrome; intravenous immunoglobulin and corticosteroid treatment was started. Almost full remission was achieved. This very rare syndrome implies challenges in diagnosis and treatment; however, remission can be achieved during the follow-up. In addition, caution is needed in the long-term follow-up of these patients regarding the development of malignancies.

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.

Hypertension and nephrology

SEPTEMBER 10, 2017

[Systemic ANCA-associated vasculitis. Induction immunosuppression therapy, complications and outcome. Part 2]

HARIS Ágnes, POLNER Kálmán

[The present review is compiled of two parts, the first part aims to summarize the induction immunosuppressive therapy, the second part delineates the outcome and complications of ANCA-associated vasculitis. ANCA-associated vasculitis is a systemic disease, accompanied with rapidly progressive glomerulonephritis and severe, often life-threatening extrarenal complications. By early diagnosis and immediate initiation of immunosuppressive therapy both patient and renal outcome have been substantially improved. The major aims of modern therapeutic protocols are, besides improving survival, to decrease immunosuppressive drug toxicity and avoid infections. Immunosuppression is based on the combination of large dose of corticosteroid and cyclophosphamide, which is advisable to supplement by plasma exchange. The B-cell depleting anti-CD20 monoclonal antibody rituximab, which has already been available in Hungary, has been proved to be similarly effective in newly diagnosed ANCA-vasculitis, and even more effective in a relapsing disease, compared to cyclophosphamide. Amongst rituximab’s further indications in this disease is the preservation of young women’s fertility, and it also has priority in some other special cases. Early diagnosis and prompt immunosuppressive treatment have resulted that ANCAvasculitis became a treatable disease with reasonably good clinical outcome, yet both the disease and the immunosuppressive medications frequently cause complications, which necessitate continuous alertness of the attending nephrologists.]

Hypertension and nephrology

MAY 20, 2017

[Systemic ANCA-associated vasculitis. Induction immunosuppression therapy, complications and outcome. Part 1]

HARIS Ágnes, POLNER Kálmán

[The present review is compiled of two parts, the first part aims to summarize the induction immunosuppressive therapy, the second part delineates the outcome and complications of ANCA-associated vasculitis. ANCA-associated vasculitis is a systemic disease, accompanied with rapidly progressive glomerulonephritis and severe, often life-threatening extrarenal complications. By early diagnosis and immediate initiation of immunosuppressive therapy, both patient and renal outcome have been substantially improved. The major aims of modern therapeutic protocols are, besides improving survival, to decrease immunosuppressive drug toxicity and avoid infections. Immunosuppression is based on the combination of large dose of corticosteroid and cyclophosphamide, which is advisable to supplement by plasma exchange. The B-cell depleting anti-CD20 monoclonal antibody rituximab, which has already been available in Hungary, has been proved to be similarly effective in newly diagnosed ANCA-vasculitis, and even more effective in a relapsing disease, compared to cyclophosphamide. Amongst rituximab’s further indications in this disease is the preservation of young women’s fertility, and it also has priority in some other special cases. Early diagnosis and prompt immunosuppressive treatment have resulted that ANCAvasculitis became a treatable disease with reasonably good clinical outcome, yet both the disease and the immunosuppressive medications frequently cause complications, which necessitate continuous alertness of the attending nephrologists.]

Clinical Neuroscience

NOVEMBER 30, 2016

NMDA-receptor associated encephalitis in a woman with mature cystic ovarian teratoma

VANYA Melinda, FÜVESI Judit, KOVÁCS A. Zoltán, GORGORAPTIS Nikos, SALEK-HADDADI Afram, KOVÁCS LÁSZLÓ, BÁRTFAI György

Introduction - N-methyl-D-aspartate receptor (NMDA-R) antibody-associated encephalitis has been reported in the international neurological literature to be associated with mature or immature ovarian teratomas (OTs). However, few cases of encephalitis were diagnosed in Hungary. In 2011 Hollody et al. described the first case of anti-NMDA receptor associated encephalitis in Hungary. Objective - Our aim was to present a case of NMDA-R antibody-mediated encephalitis in a woman with OT thereby providing information facilitating diagnosis of OT in women, who present with symptoms of encephalitis. Case - We report the case of a 25 year-old women, who developed NMDA-R -antibody associated autoimmune encephalitis and who displayed confusion, disorientation, a behavioural disturbance with agitation and features of paranoia and at least one reported generalized tonic clonic seizure and orofacial dyskinesia. Magnetic resonance imaging revealed a functional ovarian cyst measuring 3.3 cm, which was removed surgically and demonstrated histologically to be a mature cystic OT. The serum was positive for antibodies to NMDA receptors. Following intravenous immunoglobulin treatment, oophorectomy and a 5-day course of plasma exchange, followed by corticosteroid and azathioprine immunosuppressive therapy, the patient displayed a significant clinical improvement. Conclusion - Cystic teratomas are common benign ovarian lesions in women of reproductive age. Although the association of OTs and NMDA-R antibody-associated encephalitis has been described in the international neurological literature, this relationship needs to be considered from on the interdisciplinary aspect by the health care providers.

Lege Artis Medicinae

JULY 20, 2015

[Treatment of chronic Pseudomonas aeruginosa lung infections in cystic fibrosis with inhaled tobramycin]

NAGY Béla, HOLICS Klára

[BACKGROUND - Intermittent or chronic pulmonary infections caused by Pseudo­monas aeruginosa (Pa) deteriorate clinical status and worsen lung function in patients with cystic fibrosis (CF). The prognosis of the disease and life expectancy of patients are substantially dependent on lung infections and inflammation; therefore the primary goal of the treatment is the early termination of the infection. PATIENTS AND METHODS - Efficacy of tobramycin (TOBI® 300 mg/5 mL solution for inhalation, henceforth TOBI®) inhalation in Pa pulmonary infections was studied in a non-interventional, observational, open-label, single-arm trial in subjects with CF. Fifty-three patients aged six to 31 years (averaged 15.8 years) were enrolled into the study. Three treatment cycles of TOBI® inhalation (28 days on drug, 28 days off drug) plus 6 months observational period were evaluated. Primary endpoint was changing in the predictive values of forced expiratory volume in 1 sec (FEV1) compared to the initial values after three cycles of the treatment. Secondary endpoints were changing in the FEV1 predictive values at the end of the complete study compared to the initial values; ratios of patients with decreased density of originally Pa-positive result of sputum culture; as well as safety and tolerability of the TOBI® treatment. RESULTS - FEV1 and FEV1% results were unchanged comparing to the initial values at the end of the treatment and after the observational period in the whole study population. However, sputum cultures became negative in 47.2% of all subjects as a result of the treatment, and the FEV1 values were gradually increased in these patients: after the third treatment cycle plus 160 mL, and at the end of the study plus 110 mL comparing to the initial values. Similar considerable increase was detected in the predictive FEV1% in this sub-group: after the first cycle plus 5.2%, at the end of third cycle plus 7%, after the observational period above 3.8% considering the starting results. CONCLUSIONS - TOBI® inhalation proved to be effective in improvement of lung function results and eradication of infection in our patients with Pa positive CF. The treatment was well tolerated and safe.]

LAM Extra for General Practicioners

DECEMBER 05, 2014

[Polyneuropathy as a first sign of microscopic polyangiitis]

ZÖLD Éva, HORVÁTH Ildikó Fanny, TARJÁN Péter, BARTA Zsolt, ZEHER Margit

[INTRODUCTION - Microscopic polyangiitis (MPA) is a systemic autoimmun disease characterized by necrotizing small vasculitis. MPA belongs to the ANCA-associated vasculitides. The disease can affect many of the body’s organ systems. Major organs involved are kidneys, skin, peripheral nerves and lungs. In addition, generalized symptoms such as fever and weight loss are very common. CASE REPORT - In January 2013 a 56-year old woman presented with weight loss, lower leg numbness, walking difficulty and petechiae on the lower legs. One month later, laboratory examinations showed progressive kidney dysfunction, anemia, hypersedimentation and elevated C reactive protein level, but further tests and investigations for potential bacterial infection and tumors were all negative. In sum, clinical signs and symptoms suggested systemic vasculitis, which was proved by the kidney biopsy and ENG examination. From these findings, microscopic polyangiitis was diagnosed with polyneuropathy and glomerulonephritis. The patient was a Hepatitis B (HBV) virus carrier, which can be provoking factor for vasculitis. Corticosteroid and six treatment cycles of intravenous pulse cyclophosphamide were performed for induction of remission. After treatment her symptoms improved and kidney function was normalized. Antiviral treatment was started because of HBV reactivation in October 2013. As a new manifestation of MPA, pulmonary symptoms were appeared in November 2013 and the patient was treated with synchronization of plasmapheresis and pulse cyclophosphamide with good clinical effectivity. Now, she is treated with methotrexate as immunosuppressive treatment and control examinations indicate the remission of the disease with proper renal function. CONCLUSION - We draw attention to a rare case of vasculitis and underline the importance of both the early diagnosis and the early and effective immunosuppressive therapy. Peripheral neuropathy may occur as a result of having systemic vasculitis. Nevertheless, the exploration and elimination of provoking factors are also must be part of the management and the regular follow-up is essential to recognize the disease relapses, thus avoid permanent organ damage.]

Lege Artis Medicinae

SEPTEMBER 21, 2014

[Polyneuropathy as a first sign of microscopic polyangiitis]

ZÖLD Éva, HORVÁTH Ildikó Fanny, TARJÁN Péter, BARTA Zsolt, ZEHER Margit

[INTRODUCTION - Microscopic polyangiitis (MPA) is a systemic autoimmun disease characterized by necrotizing small vasculitis. MPA belongs to the ANCA-associated vasculitides. The disease can affect many of the body’s organ systems. Major organs involved are kidneys, skin, peripheral nerves and lungs. In addition, generalized symptoms such as fever and weight loss are very common. CASE REPORT - In January 2013 a 56-year old woman presented with weight loss, lower leg numbness, walking difficulty and petechiae on the lower legs. One month later, laboratory examinations showed progressive kidney dysfunction, anemia, hypersedimentation and elevated C reactive protein level, but further tests and investigations for potential bacterial infection and tumors were all negative. In sum, clinical signs and symptoms suggested systemic vasculitis, which was proved by the kidney biopsy and ENG examination. From these findings, microscopic polyangiitis was diagnosed with polyneuropathy and glomerulonephritis. The patient was a Hepatitis B (HBV) virus carrier, which can be provoking factor for vasculitis. Corticosteroid and six treatment cycles of intravenous pulse cyclophosphamide were performed for induction of remission. After treatment her symptoms improved and kidney function was normalized. Antiviral treatment was started because of HBV reactivation in October 2013. As a new manifestation of MPA, pulmonary symptoms were appeared in November 2013 and the patient was treated with synchronization of plasmapheresis and pulse cyclophosphamide with good clinical effectivity. Now, she is treated with methotrexate as immunosuppressive treatment and control examinations indicate the remission of the disease with proper renal function. CONCLUSION - We draw attention to a rare case of vasculitis and underline the importance of both the early diagnosis and the early and effective immunosuppressive therapy. Peripheral neuropathy may occur as a result of having systemic vasculitis. Nevertheless, the exploration and elimination of provoking factors are also must be part of the management and the regular follow-up is essential to recognize the disease relapses, thus avoid permanent organ damage.]