Low-dose sirolimus in two cousins with autoimmune lymphoproliferative syndrome-associated infection

Autoimmune lymphoproliferative syndrome (ALPS) is characterized by non-malignant lymphoproliferation and autoimmunity, with a wide spectrum of clinical manifestations (OMIM 601859). Typical features include enlarged spleen and lymph nodes and autoimmune cytopenia. We describe a family with ALPS in which two cousins independently presented to their physicians with infection and discuss the therapeutic potential of sirolimus

Hyperspectral imaging as a technique for investigating the effect of consolidating materials on wood

The focus of this study was to investigate the potential of hyperspectral imaging (HSI) in the monitoring of commercial consolidant products applied on wood samples. Poplar (Populus spp.) and walnut (Juglans Regia L.) were chosen for the consolidant application. Both traditional and innovative products were selected, based on acrylic, epoxy, and aliphatic compounds. Wood samples were stressed by freeze/thaw cycles in order to cause material degradation without the loss of wood components. Then the consolidant was applied under vacuum. The samples were finally artificially aged for 168 h in a solar box chamber. The samples were acquired in the short wave infrared (1000 to 2500 nm) range by SISUChema XL™device (Specim, Finland) after 168 h of irradiation. As comparison, color measurement was also used as an economic, simple, and noninvasive technique to evaluate the deterioration and consolidation effects on wood. All data were then processed adopting a chemometric approach finalized to define correlation models, HSI based, between consolidating materials, wood species, and short-time aging effects