Thrombotic Thrombocytopenic Purpura, Moschcowitz Syndrome

The authors present a case of a 16-year-old boy, who was referred to the hospital due to thrombocytopenia, anemia, proteinuria and hyperbilirubinemia. Based on the clinical picture and the laboratory data, thrombotic thrombocytopenic purpura (TTP) was diagnosed. The adequate therapy was immediately started. TTP is quite a rare entity. The etiology and the pathogenesis are not well defined. The authors summarize the different pathomechanisms, which may play a role in the development of TTP. Similarity to the hemolytic uremic syndrome (HUS), therapeutic possibilities, prognosis and the outcome are also discussed. The importance of the early diagnosis of TTP in childhood, and life-saving effect of the adequate treatment are emphasized

Szántás geometriai modellezése, elemzése : Geometric modeling and analysis of plowing

Ploughing is basic tillage; the machine is the plough. During ploughing, the plough rotates while shredding, loosening and mixing the cultivated soil layer. Accurate knowledge of the operation of the plough is very important from the point of view of the soil and the favourable energy consumption, and thus also indirectly from the point of view of the environment. In the dissertation we illustrate the work of the plough body with the help of mathematical models. We study the relationship between working depth and working width and the effect of the geometrical characteristics of the parts forming the plough body on the ploughing process. Kivonat A szántás alapvető talajmunka, munkagépe az eke. A szántás során az eke forgatja, miközben aprítja, lazítja, keveri a megmunkált talajréteget. Az eke működésének pontos ismerete nagyon fontos a talaj, a kedvező energiafelhasználás, így közvetve a környezet szempontjából is. A dolgozatban matematikai modellek segítségével szemléltetjük az eketest munkáját. Vizsgáljuk a munkamélység és munkaszélesség közti összefüggést, valamint az eketestet alkotó részek geometriai jellemzőinek hatását a szántás folyamatára.   &nbsp

Improper Supplementation Habits of Folic Acid Intake by Hungarian Pregnant Women: Improper Recommendations

Background: Neural tube defects (NTDs) are some of the most common congenital anomalies. Proper folic acid supplementation is a dominant risk factor, which has been shown to decrease the incidence of NTDs. In Canada, the incidence of neuroblastoma has presented a considerable decrease of 60% as a result of enrichment cereal grain flours with synthetic folic acid. The aim of this study was to investigate the effect of folic acid intake by pregnant women on the incidence of NTDs and neuroblastoma. Methods: Regular folic acid intake has been recommended to pregnant women in Hungary since the eighties of the last century by health visitors eventually raking effect as an official protocol which had been released in 1997. During 2001, 2002 and 2003. folic acid intake habits of pregnant women were evaluated by health visitors, proving to be successful in collecting data front 95.06% of the pregnant women. The incidence of NTDs has been registered by the Hungarian National Centre of Epidemiology, Department of Human Genetics and Teratology. The Pediatric Cancer Registry provided the incidence of neuroblastoma in children. Results: Consistent findings revealed a regular intake of supplementary folic acid products by 68.71% of the pregnant women. Out of these. 93.13% of pregnant women who were taking folic acid, started the supplementation after their 7 weeks of pregnancies, a time designated as the completion period of the development of the neural tube. The dose of folic acid supplementation was evaluated as less than 5 mg/day in 84.75% of the pregnant women. In Hungary, the incidence of NTDs has remained constant, while the incidence of neuroblastoma has shown constant slight increase in spite of the introduction of folic acid supplementation in 1997. Conclusions: Based on our experience, folic acid supplementation was initiated after the recognition of pregnancy and its application in a dose of lower than 5 mg/day neither decreased the incidence of NTDs nor did it have an effect on the neuroblastoma incidence. It is implicated that proper folic acid supplementation, which is started front the conception. can be achieved only with the enrichment of cereal grain flours

Identification of near-surface saline water in the Lake Kelemenszék area, Danube-Tisza Interfluve, Hungary

Abstract In the Danube Valley, a chain of saline lakes and a salinized area are situated in the discharge zone of two different waters. The two watertype were followed till the near surface by regional chemical and hydraulic investigations (Mádlné Szőnyi and Tóth 2009). The goals of this study were to prove the presence of these different waters in the near surface zone around the Lake Kelemenszék, and allocate their connection to the lake and to the salinized ground surface. Near-surface geophysical measurements (VES, RMT) supported the regional hydraulic and chemical results. The sediments are saturated by saline water down to 110 m beneath the lake, and to the east the fresh water is prevalent. The local chemical investigations strengthened this distribution. Highly saline water with Na-HCO3-Cl-type discharges into and around the lake. To the east there is a continuous change towards a (Ca,Mg)-(HCO3)2-type fresh water discharge. The interface between the two water types is a 2–3 km wide transition zone. The results of the investigations proved that the saline water of the deep flow system rises to the surface and provide salt for the salinization of the area

Primer immundefektus esetén előforduló onkohematológiai kórképek = Primary immunodeficiencies and haemato-oncology

Absztrakt: A primer immundefektusok (PID) ritka, veleszületett, többnyire öröklődő immunhiányos állapotok. Körülbelül 350 gén érintettsége mutatható ki ezen betegségeknél, amelynek következménye az immunsejtek számbeli és/vagy funkcionális csökkenése. A primer immunhiány gyakorisága napjainkban egyre nő. A PID kezelésében jelentős eredményeket értek el az immunglobulinpótlás és az őssejtterápia bevezetésével, ennek köszönhetően ezen betegek hosszú távú túlélése is megnövekedett. Mivel a PID-es betegek tovább élnek, mint néhány évtizeddel ezelőtt, több rosszindulatú daganatos megbetegedést is diagnosztizálunk náluk. PID-ben szenvedő gyermekek és felnőttek körében is a malignitás áll a fertőzések után a halálokok között a második helyen. Jelen munkánk során bemutatjuk a PID-es betegségekben előforduló gyakoribb rosszindulatú betegségeket és azok klinikai jellemzőit. Orv Hetil. 2018; 159(49): 2073–2078. | Abstract: Primary immunodeficiencies (PID) are rare, congenital disorders, often associated with genetic defects in the immune system. According to our current knowlegde, about 350 genes are involved in distinct immunodeficiency disorders. In PIDs at least one, and often more, immune component is impaired, missing, or has an inappropriate function. The prevalence of PID has been increasing. Due to advances in the treatment of PID, especially immunoglobulin replacement therapy and stem cell transplantation, the life expectancy of patients is longer. As patients with PID live longer, malignancies are diagnosed more commonly. Patients with PID are at an increased risk of malignancy compared with the normal population. Malignancy is the second most common cause of death in these patients after infections. The aim of this article is to review the malignancies and their clinical relevance in patients with PID. Orv Hetil. 2018; 159(49); 2073–2078

Major basic protein, but not eosinophil cationic protein or eosinophil protein X, is related to atopy in cystic fibrosis.

Increased eosinophil granule proteins have been described in serum and sputum samples of patients with cystic fibrosis (CF). It has been assumed that eosinophil degranulation is enhanced in atopic subjects - as in asthmatics. Since in CF no differences in eosinophil cationic protein (ECP), eosinophil protein X (EPX), and eosinophil peroxidase between atopic and nonatopic subjects have been detected, we investigated whether major basic protein (MBP) is increased in serum and sputum samples derived from atopic (n = 14) compared with nonatopic CF subjects (n = 26). In CF patients, high mean serum (sputum) levels of ECP 29.7 microg/l (2.7 mg/l), EPX 53.7 microg/l (7.9 mg/l), and MBP 984.6 microg/l but low sputum MBP levels (57.4 microg/l) were measured. In addition, in serum and in sputum samples, a significant correlation between MBP and ECP (P<0.03 and P<0.0001, respectively) or EPX (P<0.05 and P<0.0004, respectively) was detected. By subdivision of the patients into allergic and nonallergic subjects, significant differences were found for serum MBP values only(mean 1382.2 microg/l vs. 770.5 microg/l; P<0.0001), but not for ECP or EPX serum levels or for eosinophil proteins in sputum. Although no differences between atopic and nonatopic CF patients in ECP and EPX were found, serum MBP levels were higher in patients sensitized to inhalant allergens than in nonsensitized subjects. These results indicate differential release of eosinophil granule proteins in peripheral blood from eosinophils, and they also indicate that MBP in serum likely is to be a better discriminator of atopy in CF