Association between rapidly progressive glomerulonephritis and the properdin factor BfF and different HLA-D region products

AbstractAssociation between rapidly progressive glomerulonephritis and the properdin factor BfF and different HLA-D region products. Frequencies of the HLA antigens ABC, DR and MT, as well as of the properdin factor alleles were determined in 24 unrelated patients presenting with immune complex mediated idiopathic rapidly progressive glomerulonephritis (RPGN) type II. As in Goodpasture syndrome (RPGN type I with pulmonary hemorrhage), a significant association with the B-cell alloantigen HLA-DR 2 was demonstrated (relative risk for HLA-DR 2 positive individuals was 3.54; P < 0.01). In addition a marked increase of the HLA specificity MT 3 was shown, which is supposed to belong to an antigen system of a second HLA-D region locus. The highest relative risk of 14.67 (P < 0.00001), however, was calculated for all patients carrying the BfF phenotype. Increased numbers of patients positive for HLA-DR 2 and -MT 3, as well as BfF suggested immune response genes or disease-related mutations on different haplotypes responsible for a MHC (major histocompatibility complex) associated predisposition of RPGN type II.Association entre la glomérulonéphrite rapidement progressive et le BfF du facteur de la properdine et les produits des différentes régions HLA-D. Les fréquences des antigènes HLA, ABC, DR et MT, ainsi que les allèles du facteur de la properdine ont été déterminées chez 24 malades non consanguins ayant une glomérulonéphrite rapidement progressive idiopathique médiée par des immunescomplexes (RPGN) de type IL Comme dans le syndrome de Goodpasture (RPGN de type I avec hémorragie pulmonaire), une association significative avec l'alloantigène cellulaire B HLA-DR 2 a été démontrée (le risque relatif pour des individus positifs à HLA-DR 2 était 3,54; P < 0,01). En plus, une augmentation marquée de la spécificité HLA-MT 3 a été montrée, laquelle est supposée appartenir à un système antigénique d'un second locus HLA-D. Le risque relatif le plus élevé, 14,67 (P < 0,00001), cependant, était calculé pour tous les malades porteurs du phénotype BfF. Les nombres augmentés de malades positifs pour HLA-DR 2 et-MT3, comme pour BfF suggéraient une réponse immune de gènes ou de mutations induites par la maladie sur différents haplotypes responsables d'une MHC (complexe principal d'histocompatibilité) associés à la prédisposition à une RPGN de type II

Urinary matrix calculi consisting of microfibrillar protein in patients on maintenance hemodialysis

Urinary matrix calculi consisting of microfibrillar protein in patients on maintenance hemodialysis. In seven patients on maintenance hemodialysis,de novo recurrent renal stone formation was observed. In all patients, the underlying disease was glomerulonephritis, with or without the nephrotic syndrome. All patients had considerable persistent proteinuria. The stones consisted predominantly of protein, as revealed by amino acid analysis, and had a negligible carbohydrate and lipid content. Only in some specimens, X-ray diffraction and scanning electron microscopy revealed the presence of small amounts of whewellit (calcium oxalate monohydrate) and/or uric acid. In semithin sections, the stones had a laminated texture and exhibited structural anisotropy under polarized light. With transmission electron microscopy, they were found to consist of peculiar microfibrils. The proteinaceous material differed from fibrin or Tamm-Horsfall-protein, as indicated by ultrastructure, carbohydrate analysis, and amino acid analysis. Symptomaticde novo matrix stone formation constitutes another complication of dialyzed patients which has not been reported so far.Calculs urinaires constitués de protéines microfibrillaires chez des malades en hémodialyse chronique. Chez sept malades en hémodialyse chronique, la formation récidivante de calculs urinaires a été observée. Chez tous les malades, la maladie initiale était une glomérulonéphrite avec ou sans syndrome néphrotique. Tous les malades avaient une protéinurie importante. Les calculs étaient essentiellement constitués de protéine, comme l'indiquait l'analyse des acides aminés, et avaient un contenu négligeable en hydrates de carbone et lipides. Dans quelques échantillons seulement la diffraction X et la microscopie électronique à balayage ont révélé la présence de faibles quantités d'oxalate de calcium monohydrate et/ou d'acide urique. Sur les coupes semifines les calculs avaient un aspect laminé et étaient anisotropiés en lumière polarisée. En microscopie électronique à transmission il a été observé des microfibrilles particulières. La substance protéique diffère de la fibrine ou de la protéine de Tamm-Horsfall d'après l'étude ultrastructurale, l'analyse des hydrates de carbone, et l'analyse des acides aminés. La formation de novo de calculs, accompagnée de syndromes de lithiase, constitue une complication non encore rapportée à ce jour au cours de la dialyse chronique

Performance of students with Special Educational Needs in Germany

The reporting of findings from PISA (Programme for International Student Assessment) is an important part of educational monitoring in Germany. However, until now the subsample of students with Special Educational Needs (SEN) had been too small to single out this group and report findings. In PISA 2012, the sample of ninth-grade students in Germany was thus expanded by students with SEN in inclusive settings and students with SEN in an oversample of 49 special schools. This article describes and compares the proficiency of students with SEN in inclusive settings and in special schools. In all three PISA domains assessing Literacy, Reading, Mathematics, and Science, students with SEN in inclusive settings achieve proficiency level 2, while students with SEN in special schools achieve proficiency level 1. It turns out that students with SEN have a lower average socioeconomic status than regular students, especially those in special schools. Possible explanations for the higher achievement of students with SEN in inclusive settings are discussed

Virus particle assembly into crystalline domains enabled by the coffee ring effect

International audienceTobacco mosaic virus particles can be rapidly assembled into 3D-domains by capillary flow-driven alignment at the triple contact-line of an evaporating droplet. Virus particles of similar to 150 angstrom diameter can be resolved within individual domains at the outer rim of the "coffee-ring" type residue by atomic force microscopy. The crystalline domains can also be probed by X-ray microdiffraction techniques. Both techniques reveal that the rod-like virus particles are oriented parallel to the rim. We further demonstrate the feasibility of collection of hk0 reflection intensities in GISAXS geometry and show it allows calculating a low-resolution electron density projection along the rod axi