Lege Artis Medicinae

[A Man of Spirit – Remembering Béla Buda]

KAPÓCS Gábor

AUGUST 20, 2013

Lege Artis Medicinae - 2013;23(07-08)

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Lege Artis Medicinae

[ARBITER 6-HALTS]

MATOS Lajos

Lege Artis Medicinae

[“War of numbers” and the facts about pegylated interferon based treatment of chronic hepatitis C]

MAKARA Mihály, HUNYADY Béla

[Further to the traditional pegylated interferon and ribavirin products, used since 2001 in the treatment of chronic hepatitis C, two new direct antiviral protease inhibitors were licensed in 2011: telaprevir and boceprevir. Added to the traditional dual combination either of these drugs increase significantly the rate of sustained viral response. Discussions over the relative efficacy of two different bands of interferons with different pharmacokinetic properties as well as of the different protease inhibitors are arising regularly. After critical review of the relevant literature the authors did not identify any clinically meaningful differences in efficacy of the two pegylated interferons (α 2a or α 2b) neither in dual nor in triple combination therapies. There has been no convincing evidence found to support superiority of one protease inhibitor over the other, either: Generally both drugs can be recommended for patients previously or actually not responding to dual therapy. However, there is a tendency towards a potential difference between the relapse rates after treatments with the two pegylated interferons in dual therapy, attributable potentially to the difference of their pharmacokinetic profiles. In special cases the choice of protease inhibitors can be influenced by cost-effectiveness and side effect profile.]

Lege Artis Medicinae

[The Face of Impressionism]

NAGY Zsuzsanna

Lege Artis Medicinae

[Legal status and regulation of complementary and alternative medicine in Europe]

HEGYI Gabriella, VINJAR Fønnebø, TORKEL Falkenberg, HÖK Johanna, SOLVEIG Wiesener

[OBJECTIVE - This study aims to review the current legal status of complementary and alternative medicine (CAM) in the 27 member states and 12 associated states of the European Union (EU). METHODS - We contacted national Ministries of Health and educational institutions of all participating countries, international, local and regional CAM associations and members of the EU project CAMbrella. Literature search was performed in governmental, scientific and popular science websites as well as the web sites/databases of health ministries and EU and Hungarian law documents. RESULTS - All 39 nations have different legislative frameworks and different regulations of CAM. CAM activities are regulated by health legislation in 17 member states, 11 of which have created a specific CAM law, and 6 of which include sections related to CAM in their general health laws. Some countries only regulate several CAM treatments. Preparations, agents and herbal products used in CAM are subject to similar authorization requirements in all countries, comparably to other medicinal products. One exception is the requirement for documentation of efficacy studies. The Directives, Regulations and Resolutions of the EU will affect the conditions that might influence CAM treatment(s) in Europe. CONCLUSION - We experienced an extraordinary diversity in EU countries with regard to the regulation and practice of CAM, but did not find differences in the regulation of herbal products and medicines used in CAM. This motivates patients, practitioners as well as researchers when crossing the borders of European countries. In the current legislative environment we think that harmonisation of law is possible within the EU: individual states within culturally similar regions should harmonise their CAM legislation and regulation. This would probably safeguard against inadequately justified, over- or underregulated practice at national levels. In Hungary, modifications of the decree regulating on CAM are currently being prepared following professional recommendations. This outdated decree needs to be reconsidered, as 17 years of practice have shown which practices are worthy to be maintainted, taught and applied within the healthcare system and which should be omitted.]

Lege Artis Medicinae

[The Remedy for Physicians – About Béla Buda]

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Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

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Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

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To handle the HaNDL syndrome through a case: The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis

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The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a rare entity. This disease has been related to migrainous headaches. It is a benign, self-limited disorder, which is characterized by fluctuating neurological symptoms and cerebrospinal fluid lymphocytosis. We describe a case of a 47 years old man with acute onset of headache and aphasia. Cerebrospinal fluid analysis revealed a lymphocytic pleocytosis (25 cells/μl, 100% lymphocytes). Electroencephalogram showed moderate slow rhythm in the left hemisphere, with temporoparietal predominance, and without epileptiform activity. His blood tests as well as magnetic resonance imaging (MRI) results were normal. With the diagnosis of HaNDL syndrome the patient was accepted in the Department of Neurology and discharged with full recovery.

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CANOMAD syndrome with respiratory failure

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CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

Lege Artis Medicinae

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

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[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. ]