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, Beh├žet'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

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

According to the international literature, DNA methylation analysis of the promoter region of SNRPN locus is the most efficient way to start genetic investigation in patients with suspected Prader-Willi syndrome.

Concepts: DNA, Gene, Gene expression, Promoter, Transcription, Transcription factor, Syndromes, DNA methylation

0

A main determinant of professional identity is the integrity of the discipline. The complexity of psychiatry in biological, psychological and sociological aspects is a typical instance of the necessity for integration.

Concepts: Psychology, Sociology, Vector space, Philosophy of science, Clinical psychology, Psychiatry, Social sciences, Mental health professional

0

The management of thoracic and abdominal organ injuries has very thorough and extensive literature, including evidence-based protocols. Pancreatic trauma stands as an exception. Blunt or penetrating trauma of the pancreas is rather rare (less than 2% of all trauma cases, approximately 3-12% of all abdominal trauma), leading to the lack of high-level evidences regarding its treatment. Damage of the pancreas parenchyma can cause substantial morbidity and mortality, therefore it is essential to separate cases where conservative treatment suffices from those that need surgical approach. This study aims to review the conclusions of relevant articles of the past decades concerning the management of both adult and childhood pancreatic trauma. Classifications and their reliability are revised. We enlist scaling systems that can help in making decision whether to operate or to treat conservatively, from physical examination to diagnostic measures and complications. To date, the treatment principles of pancreatic trauma are not based either on prospective or on randomised trials. The database search of studies retrieved only retrospective and/or small case cohorts, case reports and expert opinions (levels 4 and 5 of evidence). However, it is a generally accepted conviction that the damage of the main pancreatic duct determines if the pancreatic injury is of low or high grade. Available classifications are based on the same principle. Conservative treatment is feasible given that the patient is hemodinamically stable and the pancreatic duct is unimpaired. If duct lesion is discovered, adult cases are to be treated with minimally invasive (percutaneous or endoscopic) measures or surgically (including reconstruction, resection and drainage). The management of childhood injuries has controversial literature. Many arguments can be enumerated on the operative as also on the non-operative approach, this confusion is to be clarified in the future. The highest morbidity rates are derived from the late diagnosis of the pancreatic duct, while increased mortality is seen in the polytrauma patient groups. Levels 1-2 evidence-based recommendations are needed, but planning of strong trials is critically limited due to the small number of cases and the heterogeneity of the relevant patient groups. Orv Hetil. 2018; 159(2): 43-52.

Concepts: Pancreas

0

Thoracic aortic endograft implantation has become a widespread procedure in recent years, yet no report is available about Hungarian outcomes. Examination of our results is crucial to define further treatment strategies. Analysis of perioperative data from Hungarian thoracic endograft implantations based on the experience of 5 years is presented. Our retrospective, multicentric study analysed voluntarily reported data from all Hungarian institutions where thoracic endograft implantations are performed. Information was collected from every procedure performed in 5 years. Between 2012 and 2016, 131 thoracic stent graft implantations were performed in Hungary (67.18% male, mean age 62.80 years). 25.19% of the procedures were acute. 13.74% of the patients were diabetic. Indications for the procedure were aneurysm (64.89%), dissection (17.56%), aortic trauma (6.87%) and other conditions (10.69%). 73.91% of the dissection cases were acute. 16.47% of repaired aneurysms were ruptured. Additional preoperative revascularization (debranching) was performed in 26.72% of the cases. Postoperative stroke occured in 4.58%, temporary hemodialysis was needed in 1.53%, bowel ischaemia was present in 2.29% and reoperation within 30 days was needed in 5.34% of all cases. Thirty-day mortality of the procedure was 9.92%, 5-year long-term mortality reached 16.03%. Endovascular repair of the thoracic aorta is an effective procedure and our national data comfirmed its advantages compared to open thoracic surgery. Further use of the procedure in Hungary depends on the centralised care in vascular surgery and financial matters. Multidisciplinary cooperation and proper logistics are needed to provide patients with optimal treatment. Orv Hetil. 2018; 159(2): 53-57.

Concepts: Stroke, Stent, Abdominal aorta, Aortic aneurysm, Aneurysm, Aortic dissection, Aorta, Vascular surgery