Reduced physical fitness in children and adolescents with type 1 diabetes

SUMMARY Aims: To evaluate motor performance and cardiorespiratory function in youths with type 1 diabetes in comparison with age-matched control group; and to analyse the influence of physical activity level, anthropometric and physical fitness parameters on long-term metabolic control. Methods: 106 youths with diabetes and 130 healthy youths aged 8-18 were assessed by the Eurofit Test Battery regarding motor performances, cardiorespiratory fitness (VO2max), skinfold thickness, and body mass index. Physical activity level was assessed through the use of questionnaires. Predictors of physical fitness and metabolic control were determined with regression analysis. Results: There were no differences either in body composition or in physical activity level, but younger girls with diabetes had impaired results in speed of upper limb movement, abdominal muscle strength, upper body strength, running speed and VO2max; older girls with diabetes had poor results in speed of upper limb movement, abdominal muscle strength, upper body strength and VO2max. Younger boys with diabetes had impaired results in speed of upper limb movement, flexibility, static strength of the hand and abdominal muscle strength; and older boys with diabetes had poor results in speed of upper limb movement, flexibility, abdominal muscle strength, upper body strength and VO2max. Older age, female gender, higher skinfold thickness, lower physical activity level and higher HbA1c were significant independent predictors of poorer VO2max. Better VO2max proved to be the single predictor of favourable HbA1c. Conclusions: Youths with diabetes have reduced fitness parameters. Efforts should be carried out to improve physical fitness as part of treatment and care of children and adolescents with type 1 diabetes

Kontrollált stabilitású polimer nano- és biokompozitok kifejlesztése és ellenőrzött gyártástechnológiája = Development of polymer nano- and biocomposites of designed stability and controlled technology for their production

A projekt megvalósítása során új nano- és biostruktúrákat (nano-antacid, bio/szénnanoszál) állítottunk elő, melyek hatóanyag-hordozóként, segédanyagként alkalmazhatók. Hatóanyagok morfológiáját (pl. amorfizálás) segédanyagok jelenlétében szabályoztuk. Korszerű (részben újonnan kifejlesztett) analitikai és matematikai (kemometria, kvantumkémiai) módszerekkel segítettük elő a fáziskölcsönhatások/felületmódosítások hatásának megértését. Így optimális technológiákat és receptúrákat választhattunk ki égésgátló hatású (pl. foszforszármazékok, nanorészecskék) és gyógyszer (pl. antacid, diuretikum, béta-blokkoló és Alzhimer-kór ellenes) hatóanyagok formulálására. Szabályozott mini/szuperkritikus-extrúzióval terjesztettük ki ezeket az eredményeket (a jól biohasznosuló) amorf gyógyszer-készítmények stabilitásának biztosítására. Kontrollált electrospinning technikával előállított nanoszálakal egy évet meghaladó stabilitást bizonyítottuk. Nanostruktúrált nedvességzáró bevonatok kifejlesztésével hidrolízis-érzékeny hatóanyagok stabilitását sikerült megőrizni. Az alkalmazástechnikai lehetőségek biogyógyszerészeti és égésgátlási irányba is bővültek (pl. probiotikum nanoformulálása, önkioltó PP és TP-PUR előállítása). Az ipari léptékű kontrollált gyártás előkészítésére in-line analitikai módszerek, szabályozott reaktor és extrúder alkalmazásával megnövelt léptékű kísérletekre került sor. Az eredményeket 51 közlemény, 2 szabadalom, PhD, OTDK, TDK dolgozatok formájában dokumentáltuk. | The realization of the project resulted in preparation of new nano/biostructures (nano-antacide, bio/carbonfiber), which can be applied as drug delivery and auxiliary additives. The morphology of active ingredients (amorpization) was controlled in presence of excipients. We promoted the understanding of the influences of interfacial interactions and surface modifications by modern/new analytical and mathematical (chemometry, quantum chemistry) methods. Thus optimal technologies and recipes could be chosen for formulation of active flame retardant (phosphorous derivatives, nanoparticles) and pharmaceutical (antacid, diuretic, beta-blocker and anti-Alzhimer- disease) ingredients. We extended these results to the stabilization of amorphous pharmaceuticals of good bioavailability by controlled mini/supercritical extrusion. In the case of nanofibers prepared by controlled electrospinning more than one year stability could be proven. The stability of hydrolysis-sensitive actives could be preserved by developing nanostructured humidity-barrier coating. The applicability of the results could be extended to biopharmacy and fire safety (nanoformulation of probiotics, preparation of self-extinguishing PP and TP-PUR) direction. In order to prepare the industrial scale production we applied in-line analytical methods, controlled reactor and extruder at larger scale experiments. We documented the results in 51 scientific papers, 2 patent claims, PhD theses, OTDK, TDK reports

Környezetkímélő égésgátló rendszerek új szintézisirányainak és hatásmechanizmusának vizsgálata = Investigation of new synthetic pathways for environmental flame retardants and their mechanism of action

Új reaktív égésgátló szerves foszforszármazékokat előállítására számítógéppel irányított szintézismódszert dolgoztunk ki. Az adalékot nano-részecskékkel is kombináltuk és az eljárásra szabadalmi oltalmat kaptunk. Nanokompozitok vizsgálatára FTIR gázanalízissel kombinált LP-Raman-IR mikroszkópiás módszert fejlesztettünk ki, majd a korábbiaknál teljesebb matematikai modellt állítottunk fel. Az eredmények a gápjármű-, építő- és villamosiparban kerülnek hasznosításra. Előny, hogy égésgátolt kompozitokat másodnyersanyagból is sikerült kifejleszteni. | New reactive fire retardant phosphorus derivatives have been synthesized using computer controlled method. The additive has been combined with nanoparticles. A relevant patent has been filed. A microscopic method of LP-Raman-IR, combined with FTIR gas analysis, has been developed for the analysis of nanocomposites. It led us to establish a mathematical model of improved applicability. The utilization of the results in the automotive, construction and electrical industry is in progress. Fire retarded composites utilizing secondary resources has been developed as well

A SHOX géndeletio előfordulása idiopathiás alacsonynövésben

INTRODUCTION: The isolated haploinsufficiency of the SHOX gene is one of the most common cause of short stature determined by monogenic mutations. The heterozygous deviation of the gene can be detected in 2-15% of patients with idiopathic short stature (ISS), in 50-90% of patients with Leri-Weill dyschondrosteosis syndrome (LWS), and in almost 100% of patients with Turner syndrome. AIM: The aim of our study was to evaluate the frequency of SHOX gene haploinsufficiency in children with ISS, LWS and in patients having Turner syndrome phenotype (TF), but normal karyotype, and to identify the dysmorphic signs characteristic for SHOX gene deficiency. METHOD: A total of 144 patients were included in the study. Multiplex Ligation-dependent Probe Amplification (MLPA) method was used to identify the SHOX gene haploinsufficiency. The relationships between clinical data (axiological parameters, skeletal disorders, dysmorphic signs) and genotype were analyzed by statistical methods. RESULTS: 11 (7.6%) of the 144 patients showed SHOX gene deficiency with female dominance (8/11, 81% female). The SHOX positive patients had a significantly higher BMI (in 5/11 vs. 20/133 cases, p<0.02) and presented more frequent dysmorphic signs (9/11vs 62/133, p = 0.02). Madelung deformity of the upper limbs was also significantly more frequent among the SHOX positive patients (4/11, i.e. 36%, vs. 14/133, i.e. 10%, p = 0.0066). There were no statistically significant differences between the mean age, mean height and auxological measurements (sitting height/height, arm span/height) between the two groups of patients. CONCLUSIONS: The occurrence of SHOX gene haploinsufficiency observed in our population corresponds to the literature data. In SHOX positive patients, in addition to short stature, the dysmorphic signs have a positive predictive value for SHOX gene alterations. However, the SHOX deletion detected in a patient with idiopathic short stature without dysmorphic signs suggest that SHOX deletion analysis can be recommended in patients with ISS. Orv Hetil. 2017; 158(34): 1351-1356

Macroscopic Manifestation of Domain-wall Magnetism and Magnetoelectric Effect in a N\'eel-type Skyrmion Host

We report a magnetic state in GaV$_4$Se$_8$ which emerges exclusively in samples with mesoscale polar domains and not in polar mono-domain crystals. Its onset is accompanied with a sharp anomaly in the magnetic susceptibility and the magnetic torque, distinct from other anomalies observed also in polar mono-domain samples upon transitions between the cycloidal, the N\'eel-type skyrmion lattice and the ferromagnetic states. We ascribe this additional transition to the formation of magnetic textures localized at structural domain walls, where the magnetic interactions change stepwise and spin textures with different spiral planes, hosted by neighbouring domains, need to be matched. A clear anomaly in the magneto-current indicates that the domain-wall-confined magnetic states also have strong contributions to the magnetoelectric response. We expect polar domain walls to commonly host such confined magnetic edge states, especially in materials with long wavelength magnetic order

A levoszimendán perioperatív alkalmazása a szívsebészetben. Magyar ajánlás = Perioperative use of levosimendan in cardiac surgery. Hungarian recommendation

Absztrakt: Az alacsony perctérfogat szindróma jelentősen emeli a szívműtétek szövődményeit és a halálozást, megnyújtja az intenzív osztályos és kórházi tartózkodási időt. A kezelésére alkalmazott katecholaminterápiának nemkívánatos szisztémás és kardiális mellékhatásai lehetnek. A levoszimendán érzékenyebbé teszi a szívizom kalciumcsatornáit kalciumra, és megnyitja az adenozin-trifoszfát (ATP)-szenzitív káliumcsatornákat (KATP) is. Ennek köszönhetően javítja a szív teljesítményét, nem növeli a szívizom oxigénigényét, valamint védőhatást fejt ki a szívre és számos egyéb szervre is. A korábbiakban megjelent irodalom és szakértői vélemények alapján 2015-ben publikálták a szakértői véleményeket tartalmazó európai dokumentumot a levoszimendán szívsebészeti perioperatív alkalmazásáról. Ennek figyelembevételével, továbbá a hét magyar szívcentrum és a gyermekszívcentrum (szívsebész, aneszteziológus és kardiológus képviselőinek) bevonásával kidolgoztuk a magyar ajánlást, melynek két meghatározó pillére van: az irodalmi evidenciák és a magyar centrumok képviselőinek tapasztalatai. Az áttekintett területek: koszorúérműtétek, billentyűműtétek, keringéstámogató eszközök és szívtranszplantáció, mind felnőtt, mind gyermek szívsebészeti beavatkozások vonatkozásában. Orv Hetil. 2018; 159(22): 870–877. | Abstract: Low output syndrome significantly increases morbidity and mortality of cardiac surgery and lengthens the durations of intensive care unit and hospital stays. Its treatment by catecholamines can lead to undesirable systemic and cardiac complications. Levosimendan is a calcium sensitiser and adenosine triphosphate (ATP)-sensitive potassium channel (IK,ATP) opener agent. Due to these effects, it improves myocardium performance, does not influence adversely the balance between O2 supply and demand, and possesses cardioprotective and organ protective properties as well. Based on the scientific literature and experts’ opinions, a European recommendation was published on the perioperative use of levosimendan in cardiac surgery in 2015. Along this line, and also taking into consideration cardiac surgeon, anaesthesiologist and cardiologist representatives of the seven Hungarian heart centres and the children heart centre, the Hungarian recommendation has been formulated that is based on two pillars: literature evidence and Hungarian expert opinions. The reviewed fields are: coronary and valvular surgery, assist device implantation, heart transplantation both in adult and pediatric cardiologic practice. Orv Hetil. 2018; 159(22): 870–877

A levoszimendán perioperatív alkalmazása a szívsebészetben

Low output syndrome significantly increases morbidity and mortality of cardiac surgery and lengthens the durations of intensive care unit and hospital stays. Its treatment by catecholamines can lead to undesirable systemic and cardiac complications. Levosimendan is a calcium sensitiser and adenosine triphosphate (ATP)-sensitive potassium channel (IK,ATP) opener agent. Due to these effects, it improves myocardium performance, does not influence adversely the balance between O2 supply and demand, and possesses cardioprotective and organ protective properties as well. Based on the scientific literature and experts' opinions, a European recommendation was published on the perioperative use of levosimendan in cardiac surgery in 2015. Along this line, and also taking into consideration cardiac surgeon, anaesthesiologist and cardiologist representatives of the seven Hungarian heart centres and the children heart centre, the Hungarian recommendation has been formulated that is based on two pillars: literature evidence and Hungarian expert opinions. The reviewed fields are: coronary and valvular surgery, assist device implantation, heart transplantation both in adult and pediatric cardiologic practice. Orv Hetil. 2018; 159(22): 870-877

Prolonged activity of the transposase helper may raise safety concerns during DNA transposon-based gene therapy

DNA transposon-based gene delivery vectors represent a promising new branch of randomly integrating vector development for gene therapy. For the side-by-side evaluation of the piggyBac and Sleeping Beauty systems—the only DNA transposons currently employed in clinical trials—during therapeutic intervention, we treated the mouse model of tyrosinemia type I with liver-targeted gene delivery using both transposon vectors. For genome-wide mapping of transposon insertion sites we developed a new next-generation sequencing procedure called streptavidin-based enrichment sequencing, which allowed us to identify approximately one million integration sites for both systems. We revealed that a high proportion of piggyBac integrations are clustered in hot regions and found that they are frequently recurring at the same genomic positions among treated animals, indicating that the genome-wide distribution of Sleeping Beauty-generated integrations is closer to random. We also revealed that the piggyBac transposase protein exhibits prolonged activity, which predicts the risk of oncogenesis by generating chromosomal double-strand breaks. Safety concerns associated with prolonged transpositional activity draw attention to the importance of squeezing the active state of the transposase enzymes into a narrower time window