Measuring health-related quality of life in Hungarian children with heart disease: psychometric properties of the Hungarian version of the Pediatric Quality of Life Inventory™ 4.0 Generic Core Scales and the Cardiac Module

<p>Abstract</p> <p>Objectives</p> <p>The aim of the study was to investigate the psychometric properties of the Hungarian version of the Pediatric Quality of Life Inventory™ (PedsQL™) Generic Core Scales and Cardiac Module.</p> <p>Methods</p> <p>The PedsQL™ 4.0 Generic Core Scales and the PedsQL™ 3.0 Cardiac Module was administered to 254 caregivers of children (aged 2-18 years) and to 195 children (aged 5-18 years) at a pediatric cardiology outpatient unit. A postal survey on a demographically group-matched sample of the general population with 525 caregivers of children (aged 2-18 years) and 373 children (aged 5-18 years) was conducted with the PedsQL™ 4.0 Generic Core Scale. Responses were described, compared over subgroups of subjects, and were used to assess practical utility, distributional coverage, construct validity, internal consistency, and inter-reporter agreement of the instrument.</p> <p>Results</p> <p>The moderate scale-level mean percentage of missing item responses (range 1.8-2.3%) supported the feasibility of the Generic Core Scales for general Hungarian children. Minimal to moderate ceiling effects and no floor effects were found on the Generic Core Scales. We observed stronger ceiling than floor effects in the Cardiac Module. Most of the scales showed satisfactory reliability with Cronbach's α estimates exceeding 0.70. Generally, moderate to good agreement was found between self- and parent proxy-reports in the patient and in the comparison group (intraclass correlation coefficient range 0.52-0.77), but remarkably low agreement in the perceived physical appearance subscale in the age group 5-7 years (0.18) and for the treatment II scale (problems on taking heart medicine) scale of the Cardiac Module in children aged 8-12 years (0.39). Assessing the construct validity of the questionnaires, statistically significant difference was found between the patient group and the comparison group only in the Physical Functioning Scale scores (p = 0.003) of the child self-report component, and in Physical (p = 0.022), Emotional, (p = 0.017), Psychosocial Summary (p = 0.019) scores and in the total HRQoL (health-related quality of life) scale score (p = 0.034) for parent proxy-report.</p> <p>Conclusion</p> <p>The findings generally support the feasibility, reliability and validity of the Hungarian translation of the PedsQL™ 4.0 Generic Core Scales and the PedsQL™ 3.0 Cardiac Module in Hungarian children with heart disease.</p

A Drosophila melanogaster sejtes immunitása = The cellular immunity of Drosophila melanogaster

A Drosophila sejtközvetítette immunválaszának szabályozását vizsgáltuk valamint molekuláris immunológiai és genetikai ismeretek elsajátítását és projekt építését tettük lehetővé a tudomány iránt érdeklődő diákok és kutatók számára.Adult Drosophila vérsejtjeire, makrofágokra és a lamellocitákra jellemző markereket azonosítottunk.Az L5 antigén a lamellociták differenciálódásának a szuppresszora.A P1 antigénről (Nimród) megállapítottuk, hogy részt vesz a fagocitózisban.A P1 molekula jellegzetes motívumot hordoz (Nim-repeat),mely egy szupercsaládot határoz meg:tagjai a kódoló gén közvetlen környezetében helyezkednek el. Javaslatot tettünk a Nim repeat kialakulásának a modelljére,leírtuk a Nimród szuprcsalád lehetséges evolúcióját.A vérképzést és a vérsejtek funkcióit szabályozó géneket és molekulákat azonosítottunk.Egy epigenetikus regulátorról megállapítottuk,hogy a vérsejtek osztódását,egy mestergénről pedig,hogy a lamellociták differenciálódását szabályozza.RNSi mutánsgyűjteményben a szesszilis szövet kialakulásában résztvevő génterméket azonosítottunk.Megállapítottuk, hogy a lamellociták a szesszilis szövetből származnak,effektorsejtekké történő differenciálódásuk lépései immunológiai markerekkel nyomonkövethetők.Eddig nem ismert sejteket találtunk Drosophila fajokban és a jellemzésükre alkalmas immunológiai markereket azonosítottunk.Az általunk felismert markereket a Drosophila vérsejt-differenciálódását és funkcióit vizsgáló laboratóriumokban rutinszerűen használják. | We studied the regulation cellular immunity of Drosophila and developed a training-unit based on the research and teaching experience of our multidisciplinary team, with a broad interest in molecular immunology. Results: We identified markers for embryonic macrophages, lamellocytes and blood cells of the adult fly. We described the L5 antigen as a suppressor of lamellocyte development. We defined P1 antigen (Nimrod) as a phagocytosis receptor with a structurally and phylogenetically conserved characteristic unit, the Nim repeat. The repeat defines a superfamily with members located in the close proximity of the p1 gene. We suggested a model for the origin of the Nim repeat, and described the possible evolution of the superfamily. We defined an epigenetic regulator of blood cell differentiation and a master gene regulating the development of lamellocytes. We defined the sessile hematopoietic tissue as the source of lamellocytee precursors. We characterized sequential events in lamellocyte development from sessile stem cells to mature lamellocytes by sequential expression of lamellocyte antigens. We found so far undefined cell types in Drosophila species and developed immunological markers for them. The hemocyte markers defined in our laboratory are used worldwide routinely in studies of blood cell development and function in Drosophila