Lege Artis Medicinae

[Decidual endometriosis causing bowel perforation]

CSERNI Gábor, REZNYÁK Béla, CINKE Klára, FARMOSI Sándor

JUNE 10, 2009

Lege Artis Medicinae - 2009;19(04-05)

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Lege Artis Medicinae

[Dilemmas of ‘off-label’ therapy]

SIMON László

Lege Artis Medicinae

[Focus on the diagnosis and therapy of chronic cough]

SZILASI Mária

[Cough by itself is not a disease but a part of a complex defense mechanism protecting from harmful materials entering the airways and cleaning the lungs and airways from potentially harmful materials. Normally, cough is accompanied by other defense mechanisms (bronchoconstriction and secretion of sputum) that increase the effectiveness of cough. Cough generally is caused by intrapulmonary disorders, but may be related to extrapulmonary lesions that are not easy to diagnose. In every case, an etiological diagnosis has to be the aim because this is the only way to proper treatment. From the abundance of reasons for cough, upper airway cough syndrome, asthma, chronic obstructive pulmonary disease, and gastro-esophageal reflux disease are discussed in detail.]

Lege Artis Medicinae

[Tumor necrosis factor-alpha blockade: adverse clinical consequences and safety issues]

MŰZES Györgyi

[The pleiotropic cytokine tumor necrosis factor (TNF)-α seems to be fundamentally involved in the pathogenesis of a variety of immune-mediated (partly autoimmune) chronic inflammatory disorders; therefore, its blockade has allowed a remarkable advance in treatment strategies. Safety and tolerability profile of TNF-antagonists is generally favorable, their overall risk/benefit ratio is definitely positive. Possible adverse consequences related to TNF-α blocking monoclonal antibodies and soluble receptors can be classified as class-specific side effects that are related to their mode of action, and individual, molecule-specific effects. Immunogenic potential of immunoglobulins (Ig) eliciting an anti-(Ig-) antibody immune response may reduce or eliminate their therapeutic benefit, increase the risk of resistance or intolerance to biologic agents, and also lead to other adverse clinical effects. The immunogenicity profile of TNFantagonists is mainly related to their hetero- (xeno-), allo- (iso-)genic or idiotypic antigen character. By means of generating fully human monoclonal antibodies, more tolerable drugs could be introduced into clinical practice.]

Lege Artis Medicinae

[Necessity of a shift in attitudes in the judgment and therapy of skin and soft-tissue infections]

SZALKA András

[Skin and soft-tissue infections are defined as infections of the epidermis, dermis, or subcutan tissue. They are among the most common human bacterial infections observed in clinical practice. It has also been shown that the incidence of skin and soft-tissue infections is increasing. This has been attributed to several factors, including increasing population age, surgical wounds related to more invasive surgery in the ageing population, obesity, malnutrition, diabetes, peripheral vascular disease and decreasing immunocompetence. There has been a major increase in the occurrence of Staphylococcus aureus infections, and communityacquired methicillin-resistant S. aureus (MRSA) infections in particular. Although many cases of skin and soft-tissue infections can be successfully treated using empirical antimicrobial therapy, changing resistance patterns of S. aureus isolates necessitate new treatment strategies.]

Lege Artis Medicinae

[Gastroenterological diseases during pregnancy in GPs’ practice]

BÁLINT Levente

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Clinical Neuroscience

Positive airway pressure normalizes glucose metabolism in obstructive sleep apnea independent of diabetes and obesity

KABELOĞLU Vasfiye, SENEL Benbir Gulçin, KARADENIZ Derya

The relationship among obstructive sleep apnea syndrome (OSAS), type 2 diabetes mellitus (DM2) and obesity is very complex and multi-directional. Obesity and increased visceral fat are important perpetuating factors for DM2 in patients with OSAS. On the other hand, OSAS itself leads to obesity by causing both leptin and insulin resistance as a consequence of activation of the sympathetic nervous system. Risk for developing DM2 further increases in patients with OSAS and obesity. Data regarding effects of positive airway pressure (PAP) therapy, gold standard treatment for OSAS, on glycemic control were inconsistent due to variability in duration of and adherence to PAP therapy. In our cohort study we investigated effects of PAP treatment on glucose metabolism in normal-weighted non-diabetic OSAS patients, in obese non-diabetic OSAS patients, and in OSAS patients with DM2. We prospectively analyzed 67 patients diagnosed with OSAS and documented to be effectively treated with PAP therapy for three months. Apnea-hypopnea index was highest in the diabetic group, being significantly higher than in the normal-weighted group (p=0.021). Mean HOMA values were significantly higher in obese (p=0.002) and diabetic group (p=0.001) than normal-weighted group; the differences were still significant after PAP therapy. HbA1c levels were significantly higher in diabetic group compared to those in normal-weighted (p=0.012) and obese (p=0.001) groups. After PAP treatment, decrease in HbA1c levels were significant in normal-weighted (p=0.008), obese (p=0.034), and diabetic (p=0.011) groups. There was no correlation with the change in HbA1c levels and age (p=0.212), BMI (p=0.322), AHI (p=0.098) or oxygen levels (p=0.122). Our study showed that treatment of OSAS by PAP therapy offers beneficial effect on glucose metabolism, not only in diabetic patients, but also in obese and normal-weighted OSAS patients. Although data regarding overall effects of PAP therapy on glycemic control present contradictory results in the literature, it should be emphasized that duration and adherence to PAP therapy were main determinants for beneficial outcome of treatment.

Lege Artis Medicinae

[Neurological complications of COVID-19]

LAMBERTUS Iván, DOBRONYI Levente, BERECZKI Dániel, KOVÁCS Tibor

[The SARS-CoV-2 virus of COVID-19 is present in all countries of the world by 2021 causing primarily respiratory symptoms by penumonia with severe respiratory failure. Since the early stages of the pandemic, there were published case reports and comprehensive clinical studies about the neurological symptoms and complications of the infection (e.g. myalgia, anosmia, ageusia, encephalitis, encephalopathy, cerebrovascular conditions, Guillain-Barré syndrome and specific neuropathies). As it is well known, drugs used in therapeutic research may also have neurological adverse effects. Our summary aims to provide a practical overview of domestic and international literature about the already known neurological complications of SARS-CoV-2 infection.]

Clinical Neuroscience

[The long-term follow-up of enzyme replacement treatment in late onset Pompe disease]

MOLNÁR Mária Judit, BORSOS Beáta, VÁRDI Visy Katalin, GROSZ Zoltán, SEBÕK Ágnes, DÉZSI Lívia, ALMÁSSY Zsuzsanna, KERÉNYI Levente, JOBBÁGY Zita, JÁVOR László, BIDLÓ Judit

[Pompe disease (PD) is a rare lysosomal disease caused by the deficient activity of acid alpha-glucosidase (GAA) enzyme due to mutations in the GAA gene. The enzymatic deficiency leads to the accumulation of glycogen within the lysosomes. Clinically, the disease has been classically classified in infantile and childhood/adult forms. Presently cc. close to 600 mutations distributed throughout the whole gene have been reported. The c.-32-13T>G splice mutation that is very common in patients of Caucasian origin affected by the childhood/adult form of the disease, with an allelic frequency close to 70%. Enzyme replacement treatment (ERT) is available for the patients with Pompe disease (Myozyme). In this paper, we are presenting the long term follow up of 13 adult onset cases treated more than 5 years. The longest follow up was 15 years. To evaluate the treatment efficacy, the 6 minutes walking test (6MWT) and the respiratory functions were monitored annually. The analysis revealed that at the beginning of ERT for 3-4 years the 6MWT had been generally increasing, then it declined, and after 10 years it was lower in 77% of the cases than it had been at the start of the treatment. In 23% of the cases the 6MWT increased during the follow up time. Only one of the patients become wheelchair dependent during the follow-up period. The respiratory function showed similar results especially in supine position. A high degree of variability was observed among patients in their responses to the treatment, which only partially associated with the antibody titer against the therapeutic protein. The efficacy of the ERT was associated with the type of the disease causing mutation, the baseline status of the disease, the lifestyle and the diet of the patient. The long-term follow up of the patients with innovative orphan drugs is necessary to really understand the value of the treatment and the need of the patients.]

Clinical Neuroscience

[The Expanded Disability Status Scale scoring in patients with multiple sclerosis]

FÜVESI Judit

[Gait disturbance is a major symptom in patients with multiple sclerosis. The Expanded Disability Status Scale (EDSS) was first used in clinical trials of multiple sclerosis for the assessment of disability, however it has become more and more widely used in clinical practice as well. Nowadays its use is essential in application of the new diagnostic criteria, the new clinical form classification and in monitoring the efficacy of therapies. EDSS is based on a standardised neurological examination, but focuses on those symptoms that are frequent in multiple sclerosis. Based on the examination it assesses seven functional systems: visual, brainstem, pyramidal, cerebellar, sensory, bowel-bladder and cerebral functions. EDSS scores can be determined based on the scores given in the functional systems and on testing the walking distance. In newer versions the “Ambulation score” has been added. This chapter clarifies the scores based on the maximal walking distance and the need for a walking aid to walk this distance. The Neurostatus/EDSS training method improves the reproducibility of the standardised neurological examination that forms the basis of the EDSS scoring. Of the tests assessing walking, the Timed-25 Foot Walk Test and the self-administered 12-Item Multiple Sclerosis Walking Scale are suitable for routine evaluation of walking performance. An increase of more than 20% in the Timed-25 Foot Walk may be considered a significant change in gait. ]

Clinical Neuroscience

[In memoriam Prof. Dr. Ferenc Garzuly (1937-2021)]

ILLÉS Zsolt, HAHN Katalin, KÁLMÁN Bernadette

[Ferenc Garzuly passed away after a long and productive life at the age of 84. He worked for almost 60 years at the Markusovszky University Teaching Hospital, where primarily led the laboratory of neuropathology and the department of neurology, but transferred to the department of pathology after his retirement. By authoring several books on rare diseases, he greatly enriched the case-based tea­ching approach in medicine. He described the Hun­garian type of transthyretin mutation causing the familial me­nin­go­cerebrovascular amyloidosis phenotype. The presentation of a special phenotype of Fabry disease associated with megadolichobasilar anomaly and a novel mutation in the alpha-galactosidase-A gene is also associated with his name. ]