Pathogenesis of Henoch-Schönlein purpura nephritis

The severity of renal involvement is the major factor determining the long-term outcome of children with Henoch-Schönlein purpura (HSP) nephritis (HSPN). Approximately 40% children with HSP develop nephritis, usually within 4 to 6 weeks after the initial onset of the typical purpuric rashes. Although the pathogenetic mechanisms are still not fully delineated, several studies suggest that galactose-deficient IgA1 (Gd-IgA1) is recognized by anti-glycan antibodies, leading to the formation of the circulating immune complexes and their mesangial deposition that induce renal injury in HSPN

Perceived Conflict of Interest in Health Science Partnerships

University scientists conducting research on topics of potential health concern often want to partner with a range of actors, including government entities, non-governmental organizations, and private enterprises. Such partnerships can provide access to needed resources, including funding. However, those who observe the results of such partnerships may judge those results based on who is involved. This set of studies seeks to assess how people perceive two hypothetical health science research collaborations. In doing so, it also tests the utility of using procedural justice concepts to assess perceptions of research legitimacy as a theoretical way to investigate conflict of interest perceptions. Findings show that including an industry collaborator has clear negative repercussions for how people see a research partnership and that these perceptions shape people’s willingness to see the research as a legitimate source of knowledge. Additional research aimed at further communicating procedures that might mitigate the impact of industry collaboration is suggested

Impaired production of interleukins in patients with cell-mediated immunodeficiencies.

The poor mitogen response to phytohaemagglutinin (PHA) of lymphocytes from three patients with cell-mediated immunodeficiencies was restored to normal when supernatants containing interleukin 2 (IL-2) were added. One of three children with severe combined immunodeficiency also showed a partial response. There was no improvement in the normal mitogenic response of the lymphocytes from patients with either the X linked or common variable forms of hypogammaglobulinaemia. All three patients with cell-mediated immunodeficiencies showed gross imbalance in the ratio of helper/inducer (OKT4+) to suppressor/cytotoxic (OKT8+) T cells. The PHA stimulated culture supernatant from one of these patients failed to induce proliferation of a cytolytic continuous T cell line. Our data suggests that the underlying defect in these patients may be a failure in production of interleukins but not in the acquisition of IL-2 receptors

AN ADDITIONAL NSII RFLP AT THE X-LINKED CHRONIC GRANULOMATOUS-DISEASE (CYBB) LOCUS

We prove Anderson localization in a disordered photonic crystal waveguide by measuring the ensemble-averaged localization length which is controlled by the dispersion of the photonic crystal waveguide. In such structures, the localization length shows a 10-fold variation between the fast- and the slow-light regime and, in the latter case, it becomes shorter than the sample length thus giving rise to strongly confined modes. The dispersive behavior of the localization length demonstrates the close relation between Anderson localization and the photon density of states in disordered photonic crystals, which opens a promising route to controlling and exploiting Anderson localization for efficient light confinement.Comment: 4 pages, 4 figure

Dual meningoencephalitis with echovirus type 11 and adenovirus in combined (common variable) immunodeficiency

We report here two siblings with common variable immunodeficiency who both contracted a meningoencephalitis with isolates of echovirus and adenovirus. One sibling recovered after treatment; his older sister, however, died from her virus meningoencephalitis.link_to_subscribed_fulltex

Epstein-Barr-virus-transformed lymphoblastoid cell lines derived from patients with X-linked agammaglobulinaemia and Wiskott-Aldrich Syndrome: responses to B cell growth and differentiation factors

Epstein-Barr-virus-transformed B lymphoblastoid cell lines (EBV-transformed LCL) from three patients with X-linked agammaglobulinaemia (XLA), six patients with Wiskott-Aldrich Syndrome (WAS), and seven normal donors, were tested for growth and differentiation in response to human recombinant IL-4, a commercially available, low molecular weight B cell growth factor (BCGF(low)), and B cell differentiation factor (BCDF) secreted by the T24 cell line, now known to be IL-6. Proliferation (3H-TdR uptake) by EBV-transformed LCL from both XLA and WAS patients in response to BCGF(low) was similar to that obtained with the normal cell lines. In addition, three normal and three WAS, but none of the XLA EBV-transformed LCL, proliferated a little in response to IL-4. All the normal B cell lines secreted IgM, and six out of the seven secreted IgG in response to BCGF(low) and BCDF. A similar pattern of response was obtained with the WAS EBV-transformed LCL (6/6 secreted IgM and 4/6 secreted IgG). Several of the normal and WAS EBV-transformed LCL also secreted IgM and IgG in response to IL-4. In contrast, the lines from the XLA patients were abnormal. One secreted large amounts of IgM and two secreted small amounts, but none of the XLA lines secreted IgG constitutively or in response to any of the factors (IL-4, BCGF(low), BCDF). The lack of detectable IgG secretion by the XLA lines was probably due to an absence of precommitted IgG B cell precursors transformed by EBV rather than an intrinsic inability to respond to BCGF and BCDF. All of the lines, including those derived from XLA patients, were shown to secrete B cell growth and differentiation factors detected on indicator B cell lines. These results suggest that the abnormal X-linked genes responsible for XLA and WAS do not interfere with B cell responses to B cell growth and differentiation factors.link_to_subscribed_fulltex