Lege Artis Medicinae

[In the focus: Dermatology]

HORVÁTH Attila

MAY 20, 2005

Lege Artis Medicinae - 2005;15(05)

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Lege Artis Medicinae

[NEUTROPENIA AND SUBSEQUENT INFECTION IN HEMATOLOGICAL DISEASES]

SINKÓ János

[Neutropenia is an immunocompromised state commonly occurring in hematological practice. The underlying disorder responsible for a critical drop in absolute granulocyte count can either be of congenital or acquired nature. Neutropenic patients frequently develop serious, at times even fatal infections. Severity of illness, outcome, type of infecting organisms are markedly influenced by additional risk factors such as impaired T- or B-lymphocyte function as well as the injury of biological barriers. Neutropenic infections should generally be treated according to evidence-based guidelines. However, in certain groups of patients, where randomized trials are lacking, all identified components of immunodeficiency should be taken into account and antimicrobial treatment or prophylaxis should individually be tailored.]

Lege Artis Medicinae

[Who should provide the definitive care of the patient with osteoporotic fracture?]

BÁLINT Géza

Lege Artis Medicinae

[CURRENT PRACTICE AND PROBLEMS IN RESUSCITATION]

DIÓSZEGHY Csaba

[Cardiopulmonary resuscitation is the progressive management of sudden cardiac arrest with the goal of restoring spontaneous circulation and preserve vital organ functions. Sudden cardiac death occuring out of hospital is still one of the major causes of death among otherwise healthy and young population however, approppriate management - certainly including resuscitation as the first step - might provide a reasonably good quality of life. Long term outcome of resuscitation is mainly determined by the links of the Chain of Survival, eg. early access, early CPR, early defibrillation and early advanced care. The aim of this review is to present the upto- date concepts for the best management of these survival links.]

Lege Artis Medicinae

[THE ROLE OF REGISTRY ANALYSIS IN THE FORMING OF THERAPEUTIC GUIDELINES IN RENAL TRANSPLANTATION]

PERNER Ferenc, HERCZEG Balázs, SZENOHRADSZKY Pál, ASZTALOS László, KALMÁR-NAGY Károly, JÁRAY Jenő

[The authors assess the two main outcomes of the immunosuppressive therapy after renal transplantation: graft and patient survival. According to their view, evidence from randomised clinical trials results can be well complemented by the several unique transplant registries and outcome research based upon these databases. The comparison of evidence from these two sources addresses the question of achievable outcome under ideal (controlled) versus real life conditions. Based on a systematic review of the relevant clinical trials and registries it can be stated, that in the case of some immunosuppressants (mycophenolate mofetil vs azathioprine, microemulsified cyclosporin vs cyclosporin and tacrolimus vs cyclosporin) the improvement in the intermediate outcome can lead to improved graft and patient survival, while in the case of other drugs no significant difference in hard endpoints were detected (tacrolimus vs microemulsified cyclosporin). Evidence on graft and patient survival differences could not be derived from traditional randomised clinical trials, only from transplant registries. For the sake of improved evidence based therapeutic guidelines in renal transplantation, authors call for further development of the Hungarian transplant registry.]

Lege Artis Medicinae

[Providing emergency care in the field]

SZEGECZKY Dezső

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Neuroscience highlights: Main cell types underlying memory and spatial navigation

KRABOTH Zoltán, KÁLMÁN Bernadette

Interest in the hippocampal formation and its role in navigation and memory arose in the second part of the 20th century, at least in part due to the curious case of Henry G. Molaison, who underwent brain surgery for intractable epilepsy. The temporal association observed between the removal of his entorhinal cortex along with a significant part of hippocampus and the developing severe memory deficit inspired scientists to focus on these regions. The subsequent discovery of the so-called place cells in the hippocampus launched the description of many other functional cell types and neuronal networks throughout the Papez-circuit that has a key role in memory processes and spatial information coding (speed, head direction, border, grid, object-vector etc). Each of these cell types has its own unique characteristics, and together they form the so-called “Brain GPS”. The aim of this short survey is to highlight for practicing neurologists the types of cells and neuronal networks that represent the anatomical substrates and physiological correlates of pathological entities affecting the limbic system, especially in the temporal lobe. For that purpose, we survey early discoveries along with the most relevant neuroscience observations from the recent literature. By this brief survey, we highlight main cell types in the hippocampal formation, and describe their roles in spatial navigation and memory processes. In recent decades, an array of new and functionally unique neuron types has been recognized in the hippocampal formation, but likely more remain to be discovered. For a better understanding of the heterogeneous presentations of neurological disorders affecting this anatomical region, insights into the constantly evolving neuroscience behind may be helpful. The public health consequences of diseases that affect memory and spatial navigation are high, and grow as the population ages, prompting scientist to focus on further exploring this brain region.

Clinical Neuroscience

Coexistence of cervical vertebral scalloping, pedicle deficiencies and dural ectasia in type I neurofibromatosis

YALDIZ Mahizer

Neurofibromatosis type 1 (NF-1; also known as Von Recklinghausen’s disease) is a common autosomal dominant disease that occurs in the general population at the rate of 1 in 3000. Many NF-1 patients present with spinal malformations. A 54-year-old female patient was admitted to the Outpatient Clinic of Dermatology with gradually increasing swelling and spots on the body that had been present for a long period of time. Cervical vertebral scalloping, pedicle deficiencies and dural ectasia (DE) were also detected. She was diagnosed with NF-1. NF-1 is routinely seen in dermatology practice. Coexistence of NF-1 with vertebral scalloping, pedicle deficiencies and DE rarely occurs. Our case is the second reported instance in the literature of NF-1 with a spinal anomaly in the cervical region, and the first reported instance of the coexistence of NF-1 with cervical vertebral scalloping, pedicle deficiencies and DE.

Clinical Neuroscience

The methylation status of NKCC1 and KCC2 in the patients with refractory temporal lobe epilepsy

UNAL Yasemin, KARA Murat, GENC Fatma, OZTURK Aslan Dilek, GÖMCELI Bicer Yasemin, KAYNAR Taner, TOSUN Kursad, KUTLU Gülnihal

Purpose - Methylation is a key epigenetic modification of DNA and regarding its impact on epilepsy, it is argued that “DNA methylation may play an important role in seizure susceptibility and maintenance of the disorder”. DNA methylation status of KCC2 (SCL12A5) and NKCC1 (SCL12A2) associated with refractory temporal lobe epilepsy was investigated in our study. Materials and methods - Thirty-eight patients with temporal lobe epilepsy (TLE) who were diagnosed by video EEG monitoring and 32 healthy control subjects were included in the study. Twenty-three patients in TLE group were men and the remaining 15 were women. Among them, 27 had unilateral temporal focus (9 with right; 18 with left) and 11 patients had bilateral TLE. We analyzed promoter region methylation status of the KCC2 (SCL12A5) and NKCC1 (SCL12A2) genes in the case and control groups. Gene regions of interest were amplified through PCR and sequencing was accomplished with pyro-sequencing. Results - We found a significant relationship between TLE and methylation on the NKCC1. However, there was no association between TLE and methylation on the KCC2 gene. Also, we found no association between right or left and unilateral or bilateral foci of TLE. There was no relationship between TLE and methylation on the NKCC1and KCC2 genes in terms of mesial temporal sclerosis in cranial MRI, head trauma or febrile convulsions. Conclusion - The methylation of NKCC1 can be a mecha­nism of refractory temporal lobe epilepsy. There are limited findings about DNA methylation in TLE. Therefore, further studies with large sample sizes are necessary.

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[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

Lege Artis Medicinae

[In the focus: malignant lung diseases]

MAGYAR Pál