SciCombinator

Discover the most talked about and latest scientific content & concepts.

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

Introduction: During liver transplantation, haemostasis is typically assessed by means of standard laboratory tests and viscoelastic tests, while dynamic monitoring of coagulation factor specific blood losses is an unusual, yet established approach. Aim: Our aim was to evaluate the volume-based haemostasis reserves in blood product free liver transplants in the first perioperative 48 hours, in association with the Child-Pugh score. Method: Data of 59 blood product free liver transplanted patients' coagulation factor levels, viscoelastic parameters and coagulation factor specific blood losses according to Gross methodological, baseline and ‘coagulopathic’ trigger levels were analysed. The haemostasis reserves were estimated according to the Child-Pugh classification. Laboratory tests and the calculation of haemostasis reserves were carried out before liver transplantation (T1), at the end of the surgery (T2) and also 12-24-48 hours postoperatively (T3-T4-T5). The viscoelastic tests were performed before liver transplantation (T1) and at the end of the surgery (T2). Results: Fibrinogen levels decreased by 1.2 g/L. Factor II, V, VII, X levels decreased by 26-40%. From T2 to T4, fibrinogen increased by 0.9 ± 0.6 g/L over 24 h (p<0.001). Factor II, V, VII, X levels increased by 12-30% between T3 to T5 (p<0.001). The viscoelastic parameters remained in the normal range during liver transplantation (T1-T2). Haemostasis reserves decreased by 61% at the end of surgery (p<0.001), but reached 88% of the preoperative value on the second postoperative day. The initial reserves of Child B and C groups were 36-41% lower than Child A, nevertheless, these differences were not significant at 48 hours. Conclusion: The volume-based haemostasis approach supplements the standard laboratory and viscoelastic tests. This unusual approach dynamically indicates the actual reserve of haemostasis and shows the 'weakest link' within the system. Orv Hetil. 2020; 161(7): 252-262.

0

Introduction: Osteoid osteoma is a rare benign bone tumor displaying typical clinical symptoms and radiological signs in most cases. Characteristic symptoms are nocturnal pain alleviated by non-steroid antiinflammatory drugs. Radiological findings are also characteristic, the central osteolytic ‘nidus’ is surrounded by reactive sclerosis. These lesions are rare in the hand, typical symptoms may be absent, furthermore, atypical symptoms may occur. Characteristic radiographic signs may also be missing. Therefore, diagnosis may prove difficult. In the case of radiological/clinical suspicion, HRCT (high-resolution CT) is recommended. Aim: Our aim is to summarize the pathophysiology, occurrence and clinical features of these lesions and also the difficulties that accompany diagnosis on the hand. Treatment options will also be discussed. Method: Retrospective analysis was performed at Semmelweis University, Department of Orthopedics, between March 2014 and December 2018. Inclusion criteria were: patients who had undergone treatment for osteoid osteoma during this period. Data from the 6 patients who have undergone open surgery for osteoid osteoma of the hand will be presented as case reports. Results: During this period, a total of 112 patients were treated for osteoid osteoma at our Department, 8 lesions were found on the hand (7%). Among the cases presenting on the hand, typical nocturnal pain was absent in 3 cases, and in 1 case the pain did not respond to non-steroid antiinflammatory drugs. Open surgery and curettage was performed in 6 cases with good results. Conclusion: Osteoid osteoma on the hand is rare, typical symptoms may be missing, and atypical symptoms may occur. Radiographic findings may be nondescript, HRCT is recommended in the case of clinical suspicion. Performing radiofrequency ablation for osteoid osteoma of the hand may pose difficulties, curettage is the gold standard of treatment. Curettage alleviates symptoms well. Orv Hetil. 2020; 161(7): 263-268.