First results with the boloSource() algorithm: Photometry of faint standard stars observed by Herschel/PACS

The boloSource() algorithm is a tool to separate the signal of compact sources from that of the diffuse background in the timeline of far-infrared measurements performed by the PACS camera of the Herschel Space Observatory. An important characteristic and quality indicator of this method is that how well it can reproduce the flux of faint standard stars which have reliable flux estimates. For this propose we selected a few calibrator targets and constructed light curves by extracting point source flux for each repetition of the measurements independently using standard aperture photometry methods. These were compared with the light curves obtained using the boloSource() method on the same dataset. The results indicate that boloSource() provides a similar level of photometric accuracy and reproducibility as the usual flux extraction and photometry methods. This new technique will be developed further and also tested against other methods in more complex fields with the goal to make it usable for large-scale studies in the future.Comment: 10 pages, 7 figures, 4 table

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

A magyar kormányzati modell = The Hungarian Governmental Model

A kutatás a rendszerváltással kialakított magyar kormányzati berendezkedés vizsgálatát célozta, a kormányzat szervezeti és működési viszonyait, illetve az ezekhez kapcsolódó intézmények fejlődéstörténetét és – az új alkotmány utáni helyzettel befejezve – jelenlegi állapotát dolgozza fel. Tárgykörei: a parlamentáris kormányzati viszonyok keletkezése és fejlődése, a kormány felépítése és belső viszonyai, testületi működése és döntési mechanizmusa, a miniszterelnök szerepe, a miniszterek helyzete, a kormányzás szervezeti kérdései, a kormányzati koordináció és a miniszterelnök szervezeti háttere, valamint politika és közigazgatás kapcsolatával összefüggésben az eddig alkalmazott államtitkári rendszerek. Külön téma a miniszteri felelősség alkotmányos intézményének vizsgálata, amely elsősorban annak két fő fajtájára az „alkotmányos jogi” és a „politikai” alakzatokra terjedt ki. A kutatás az egyes parlamenti ciklusokban kialakított megoldások és a gyakorlat értékelésével, összehasonlításával, az állandóság és a változások bemutatásával tárgyilagos kép adására törekszik a magyar kormányzati viszonyokról és problémáiról. A kutatás, amely a releváns jogi és politikai dokumentumok, illetve a közjogi és a politikatudományi szakirodalom, valamint a kormányzati gyakorlat feldolgozására és a külföldi megoldások figyelembevételére is támaszkodik, alapvetően jogias megközelítésű, de nem mellőzi a politikai és szociológiai összefüggéseket sem. | The research aimed at the examination of the Hungarian governmental structure shaped by the system change, the organisational and functional relations of the government and the development history of the related institutions and its actual state, finished by the new situation following the new constitution. The subjects are: formation and development of the parliamentary governmental relations, structure and internal relations of the government, its corporative functioning and decision-making mechanisms, role of the prime minster, the situation of the ministers, organisational issues of the governance, organisation background of the governmental coordination and the prime minister and the applied secretary of state systems until now in relation to the relations of politics and administration. The examination of the constitutional institution of ministerial responsibility is a separate issue which covers primarily its two main types, the “constitutional law” form and the “political form”. The research seeks to draw an objective picture of the Hungarian governmental relations and problems by appreciating and comparing the solutions and practice of each parliamentary term and by presenting the consistencies and changes. The research based on the relevant legal and political documents, public law and political sciences literature and the adaptation of governmental practice and referring to foreign solutions follows primarily a legal approach but does not disregard the political and sociological aspects

Incidence and Paris Classification of pediatric inflammatory bowel disease

New epidemiological data suggest that the incidence of inflammatory bowel disease (IBD) is increasing. As a result the burden of disease accounts for more strains to the health care system. The clinical variability queries whether disease characteristics are related to clinical outcome. Our aim was to delineate the latest results of incidence trends in pediatric IBD and to compare the first experiences with Paris Classification. Incidence of pediatric IBD has been increasing in Western Europe and in Eastern Europe. To better characterize IBD, Paris Classification was introduced and validated recently. Ileocolonic involvement is the most characteristic disease location in Crohn's disease (CD) based on applying Paris Classification. The rate of perianal disease and complicated behaviour in CD was similar. It is of interest that CD patients with colonic involvement were less likely to have stricturing disease compared with patients with ileal involvement. In addition, pancolitis dominated in ulcerative colitis (UC). However, most countries lack prospective, nationwide epidemiological studies to estimate incidence trends. This review emphasizes the importance of nationwide registries that enroll all pediatric IBD cases serving reliable data for "everyday practice." These first reports have shown that Paris Classification is a useful tool to determine the pediatric IBD phenotype

Szén-monoxid aktiválása: átmenetifém-karbonil komplexek és alkalmazásuk homogénkatalitikus karbonilezési reakciókban = The activation of carbon monoxide: transition metal complexes and their application in homogeneous catalytic carbonylation reactions

Az egyszerű, egy szénatomot tartalmazó "C1-egységek" különböző vegyületekbe történő beépítésének egyik legegyszerűbb módja szén-monoxid alkalmazása átmenetifém-katalizált reakciókban. A pályázat keretében a következő részterületeket vizsgáltuk. - Prokirális olefinek hidroformilezése ródium- és platinakomplexek jelenlétében. - Enol-triflátok és szintetikus analógjaik, a jód-alkének karbonilezési reakciói. - Ionfolyadékok szintézise és alkalmazása homogén katalízisben. - Platina-katalizált hidroformilezés reakció-mechanizmusának vizsgálata. | One of the most efficient ways of the selective introduction of a simple 'C1-unit' into various compounds is the application of carbon monoxide in transition metal catalysed reactions. Within the framework of the project the following topics have been investigated: - Hydroformylation of (prochiral) olefins in the presence of rhodium and platinum complexes. - Carbonylation of enol-triflates and their synthetic analogues, iodo-alkenes. - The application ionic liquids in homogeneous catalysis. - Mechanistic investigation of the platinum-catalysed hydroformylation

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

Foreign body impaction in the sigmoid colon

Foreign body ingestion is a common clinical problem in early childhood. However, it may occur even in adults, unknowingly. Most ingested foreign bodies entering the stomach pass through the gastrointestinal tract uneventfully. Here we report on a 13-year-old boy who presented with chronic abdominal pain, weight loss and occult gastrointestinal bleeding for 6 mo. Colonoscopy was negative; however, a ballpoint pen was impacted in the sigmoid region. Subsequently, the child admitted swallowing a pen as a 20-euro bet 6 mo previously. Crohn's disease is a chronic relapsing inflammatory gastrointestinal disease. It is often difficult to diagnose due to the fact that there is no single pathognomonic sign or symptom. This case is a description of an adolescent with chronic gastrointestinal symptoms due to a foreign body. Therefore, an ingested foreign body should be included in the differential diagnostic procedure related to gastrointestinal symptoms