Till date, there had been no reported case of dialysis-related amyloidosis (DRA) associated with a β2-microglobulin variant. We report here a 41-year-old haemodialysis patient with systemic amyloidosis, exhibiting macroglossia and swelling salivary glands, uncommon clinical manifestations for DRA. Molecular analysis showed that the patient had a new variant of β2-microglobulin (V27M). Extracted amyloid protein was predominantly composed of variant β2-microglobulin. In vitro analysis revealed that this variant β2-microglobulin had a strong amyloidogenic propensity, probably owing to the decreased stability caused by a bulky methionine residue. Our data clearly show that V27M variant is amyloidogenic and this mutation results in unusual clinical manifestations. To date, only one amyloidogenic β2-microglobulin variant (D76N) has been reported in non-dialysis patients. It is noteworthy that the V27M and D76N variants show substantial differences in both clinical phenotypes and pathomechanical features. This is the first case of DRA associated with a naturally occurring β2-microglobulin variant.
summary The purpose of this study was to examine the translucency of glass-fibre-reinforced composite framework materials. Vectris and FibreKor, as well as an experimental material, were the glass-fibre-reinforced framework materials used. Targis, Sculpture and Estenia were the types of particulate filler composites veneered onto frameworks. Specimens were fabricated from each material, 0.5 and 1.0 mm thick. In addition, laminate specimens, 1.5 mm thick, were fabricated. The translucency of each specimen was evaluated by determining its contrast ratio. The laminate specimens were examined for colour differences. The experimental framework material was more translucent than the enamel composite when it was not coloured, and was nearly as translucent as the dentine composite when coloured. The commercial tooth-coloured framework materials were nearly as translucent as the dentine composite. It was found that it was possible to reproduce the same colour as the veneering dentine composite, when the framework thickness was 0.5 mm, except in the case of FibreKor. Within the limitations of this study, tooth-coloured, glass-fibre-reinforced framework materials are nearly as translucent as the veneering dentine composite, but these materials affect the colour of the prosthesis if the thickness of the framework material is increased beyond a certain point.
