SciCombinator

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Journal: Orvosi hetilap

168

Behcet’s disease is a multisystem autoimmune disease with variable clinial manifestations. The diagnosis may pose a difficult challenge for the clinician, who has to be familiar with the wide spectrum and combination of the symptoms of Behcet’s disease. It is considered a rare disease in Hungary, and there are only few reports on Behcet’s disease in the Hungarian literature. However, the past history of Hungary, the worldwide growing incidence of the disease, and the authors' experience raise the possibility that the occurrence of the disease is higher than previously thought. In this review the authors present and discuss literature data on the pathogenesis and pathomechanism, as well as their own experience concerning the symptomatology of Behcet’s disease in order to promote diagnosis and offer adequate therapy for the patients. The authors presume that the importance of the disease is underestimated in Hungary due to a considerable number of unrecognized cases and they propose to establish a national registry for Behcets disease. Orv. Hetil., 2013, 154, 93-101.

Concepts: Epidemiology, Medical terms, Autoimmune diseases, Prevalence, Hungary, Behçet's disease, Hungarian language, Kingdom of Hungary

167

Hairy cell leukemia is a mature B-cell non-Hogkin lymphoma characterized by unique clinical, morphological and immunhistochemical features. Patients with hairy cell leukemia usually present with splenomegaly, progressive pancytopenia and a relative indolent clinical course. The diagnosis does not always indicate immediate treatment, as treatment depends on the clinical stage of the leukemia. Asymptomatic disease without progression requires a watchful waiting policy, while other categories usually need treatment. The treatment of choice is purin nucleosid analogues (pentostatin, cladribine) which can achieve complete remission even for decades. Interferon and monoclonal CD20 antibodies can also significantly prolong tevent free survival. Unfortunately, only the latter two therapies are easily available in Hungary. Splenectomy, which was suggested as first line treatment before the era of purin nucleosid analogues, is only recommended as ultimum refugium. Although hairy cell leukemia is a well-defined lymphoproliferative disease, sometimes it is difficult to differentiate it from other similar entities such as hairy cell leukema variant, splenic marginal zone lymphoma, small lymphocytic lymphoma etc. Making the correct diagnosis is of utmost importance because of the great difference in treatment modalities. Recently, a somatic mutation was found in all analysed hairy cell leukemia samples, but not in other splenic B-cell lymphomas. This article reviews the significance of this observation and presents the different types of methods for the detection of this mutation. Orv. Hetil., 2013, 154, 123-127.

Concepts: Lymphocyte, Cancer, Types of cancer, Hematology, Leukemia, Lymphoma, Blood disorders, Hairy cell leukemia

27

The “gold standard” of the prevention of atrial fibrillation related thromboembolic events is anticoagulation therapy with oral vitamin K antagonists. A certain proportion of high-risk patients with atrial fibrillation are not receiving effective antithrombotic therapy because of problems associated with its use. Resolution of subsequent left atrial appendage thrombi is quite a great challenge in patients who are not tolerating “standard” antithrombotic drugs. According to the knowledge of the authors, this is the first report of a patient with non-valvular persistent atrial fibrillation and high stroke risk, who was intolerant to “standard” anticoagulant therapy and had persistent left atrial appendage thrombi following the use of a wide variety of “standard” anticoagulants. Successful resolution of left atrial appendage thrombi with dabigatran and successful percutaneous left atrial appendage closure were performed in this case. Orv. Hetil., 2013, 154, 262-265.

Concepts: Stroke, Atrial fibrillation, Thrombosis, Pulmonary embolism, Warfarin, Anticoagulant, Dabigatran, Left atrial appendage

27

The aim of the authors is to present two cases which raise the possibility of an association between polycystic ovarian syndrome/hyperandrogenism and ovarian cyst torsion in peripubertal girls. Androgen excess may cause more frequently ovarian cyst formation in premenarcheal or young adolescents with undiagnosed polycystic ovarian syndrome than in adults. The authors recommend that polycystic ovarian syndrome as wel as late onset congenital adrenal hyperplasia should be considered in peripubertal adolescents with ovarian cyst torsion. In case polycystic ovarian syndrome is confirmed, adequate management according to age and pubertal development of the patients should be commenced. Orv. Hetil., 2013, 154, 113-117.

Concepts: Ovarian cyst, Testosterone, Polycystic ovary syndrome, Mineralocorticoid, Congenital adrenal hyperplasia, Congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency, Adrenal insufficiency, Virilization

25

The authors review the history and risk factors of thrombophlebitis of the lower limb, and describe the main points of surgical and conservative treatment of varicophlebitis. They present the case of a 71-year-old woman who had ascending varicophlebitis and bilateral pulmonary embolism. The authors draw attention to important points: patients must be followed after phlebitis of the lower limb, and their thrombotic factors must be examined to prevent the new thromboembolic events. Orv. Hetil., 2015, 156(16), 644-649.

Concepts: Pulmonary embolism

24

In many developed countries the prevalence of venous disorders and its consequences are higher than that of arterial diseases. Thus it is very important to understand the exact physiological and pathophysiological function of small veins and their control mechanisms. Small veins and venules have an important role in the regulation of capillary fluid exchange, as well as return of the venous blood into the heart. However, there is only limited knowledge available regarding the role of local mechanisms controlling the vasomotor tone and diameter of small veins. In the last decade the authors focused on the elucidation of these mechanisms in isolated skeletal muscle venules of rats. Their results suggest that the tone of small veins is controlled by the integration of several mechanisms, activated by the intraluminal pressure and flow/wall shear stress, in addition to numerous local mediators synthesized and released from the smooth muscle and endothelium. These mechanisms are involved - in a complex manner - in the control of postcapillary resistance, thus regulation of tissue blood supply, venous return and consequently in the modulation of the cardiac output, as well. Orv. Hetil., 2016, 157(21), 805-812.

Concepts: Blood, Heart, Blood vessel, Cardiac muscle, Artery, Vein, Cardiovascular system, Smooth muscle

22

L. J. Thénard and J. L. Gay-Lussac discovered hydrogen peroxide in 1818. Later, Thénard noticed that animal and plant tissues decompose hydrogen peroxide. The substance which is responsible for this reaction was named as catalase by O. Loew in 1900. The catalase enzyme was regarded as a diagnostic and a tumour marker in the late years of the 19th century and in the early years of the 20th century. Acatalasemia, an inherited deficiency of enzyme catalase, was studied in Japan, Switzerland and Hungary. The recent findings on catalase are focusing on the effects of reactive oxygen species and on the association of acatalasemia and diabetes mellitus. Orv Hetil. 2018; 159(24): 959-964.

4

Introduction: In gastrointestinal diseases folk medicine usually recommends herbs and teas with antiinflammatory activities, especially mucilage and bitter agent containing plants. The use of stomatics as adjuvant therapy in medical practice is frequent. Aim: The authors explored the mineral element content of some herbs, because mineral elements may have significant role in the development and treatment of gastrointestinal diseases, and a close connection between the presence or absence of mineral elements and inflammatoric mediators was noted. Methods: The mineral content in Trigonellae foenugraeci semen, Farfarae folium et flos, Taraxaci radix and Cichorii radix and teas prepared from these herbs was determined using inductively coupled plasma optical emission spectrometry. The results were evaluated from nutritional and biomedical points of view. Results:Farfarae folium et flos tea was found to be a rich source of calcium, chromium, manganese and molybdenum. Trigonellae foenugraeci semen, Taraxaci radix and Cichorii radix displayed favourable calcium to magnesium ratio (1-1.5) and they contained other elements (manganese, iron, copper, zinc) which may have significant role in the immune defence system. Conclusion: The beneficial therapeutic effect of the studied herbs may be related to their mineral element content. Orv. Hetil., 2013, 154, 538-543.

Concepts: Medicine, Iron, Magnesium, Dietary mineral, Aluminium, Metal, Copper, Chemical elements

0

A characteristics of mediastinal disorders is that the high anatomical density of vital structures in this region represents a challenge for diagnosis and surgical treatment. Space-occupying lesions can grow without causing overt manifestations - or can progress symptom-free - hence they can reach an extreme size by the time of surgery. A 58-year-old male patient was hospitalized for pleural effusion and an extensive, space-occupying mediastinal lesion, which had been causing respiratory symptoms for 15 years. Cytology of the pleural effusion did not confirm malignancy. The CT scan depicted progression manifested as an increase in the size of the lesion with a likely site of origin in the left adrenal gland. According to the MRI, by contrast, the lesion might have originated in the region of vertebrae Th9-10, as suggested by the lack of dural continuity. However, its adrenal origin could not be excluded either; endocrine activity was not detected. An operation was performed with a neurosurgeon included in the surgical team. A spinal tumor of the size of 20.2 by 11.1 by 10.8 cm was removed through thoraco-laparotomy, and reconstruction of the diaphragm was performed. Histology confirmed a schwannoma. Postoperatively, the expansion of the lung was only partial, because the patient discontinued respiratory rehabilitation. The follow-up CT scan depicted local recurrence. In the lack of alternative therapeutic modalities, surgical resection is usually the sole option for the management of large, mediastinal space-occupying lesions diagnosed at an advanced stage. Such operations should only be performed in specialist surgical centers and with multidisciplinary collaboration. Orv Hetil. 2019; 160(37): 1476-1479.

0

Introduction: The inadequate, inappropriate sinus-node tachycardia is not a rare clinical syndrome, defined as a non-paroxysmal, increased sinus-rate at rest, and/or inadequate response to physical and/or emotional stress, and palpitations. Aim: The aim of this study was to describe our experiences with the investigations of our inappropriate sinus-node tachycardia patients. Method: In the last years, 104 patients (92 women, 12 men, mean age: 31 ± 10 years) were treated with this cardiac arrhythmia entity. All patients underwent 12 leads ECG, chest X-ray, echocardiography, Holter-monitoring and transtelephonic ECG observations. The quality of life score was estimated by using the European Heart Rhythm Association scale. Results: Patients had no structural heart disease (physical examination ECG, chest X-ray, echocardiography were normal), the laboratory values (TSH, blood count) were within normal limits, but the resting heart rates were repeatedly high (102 ± 8/min). The results of Holter recording (expressed as minimal-maximal [average] heart rate/min) without drug therapy showed high heart rate values (59 ± 8, 160 ± 14 [94 ± 6]/min). The standard bicycle ergometry showed an average loading capacity of 124 ± 23 watt (heart rate: control: 99 ± 12/min, top: 167 ± 13/min) with early, inadequate sinus tachycardia. To disclose the episodes of paroxysmal supraventricular tachycardia, beside the Holter-monitoring transtelephonic ECG system was used. This diagnostic modality was very useful for the exclusion of paroxysmal supraventricular tachycardia episodes during the palpitation symptoms. Out of 104 patients, 4 patients (3.8%) showed familiar occurrence, another 16 patients (15.2%) had previous slow-pathway radiofrequency ablation due to atrioventricular nodal reentry tachycardia. Conclusions: Based on our clinical observations, it can be pointed out that inappropriate sinus-node tachycardia syndrome (1) occurs mainly in young women, mostly in students, inducing decreased quality of life scores (EHRA score: 2.3 ± 0.4); (2) the prevalence in our outpatient clinic was 0.7%; (3) the patient population is not homogeneous: familiar or postablation occurrence is possible in some patients; (4) transtelephonic ECG has been proved to be very useful to disclose episodes of paroxysmal supraventricular tachycardia in these patients. Orv Hetil. 2019; 160(37): 1464-1470.