Publications by authors named "P P Mangione"

Objective: Fournier's gangrene (FG) is a rare, life-threatening necrotizing fasciitis primarily affecting the perineal, genital, and perianal regions. This rapidly progressing bacterial infection predominantly affects middleaged and elderly men. This multicenter study aims to describe the management in a wide cohort of Fournier's gangrene cases that presented to three tertiary centers with early extensive surgical debridement.

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AL amyloidosis is a life-threatening disease caused by deposition of immunoglobulin light chains. While the mechanisms underlying light chains amyloidogenesis in vivo remain unclear, several studies have highlighted the role that tissue environment and structural amyloidogenicity of individual light chains have in the disease pathogenesis. AL natural deposits contain both full-length light chains and fragments encompassing the variable domain (V) as well as different length segments of the constant region (C), thus highlighting the relevance that proteolysis may have in the fibrillogenesis pathway.

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Article Synopsis
  • The text discusses the ongoing debate about how native human transthyretin (TTR) converts into amyloid fibrils in the body, emphasizing the differences in research conditions used for studying this process.
  • It focuses on two specific TTR variants, S52P and L111M, that cause severe systemic amyloidosis, particularly affecting the heart, and highlights their varying stability and susceptibility to proteolytic cleavage.
  • The study shows that while both variants are similar in stability, the L111M variant is more prone to cleavage and faster fibril formation when influenced by factors like heparin and fibrillar seeds, indicating complex mechanisms at play in amyloid formation.
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Background: Robotic spinal surgery may result in better pedicle screw placement accuracy, and reduction in radiation exposure and length of stay, compared to freehand surgery. The purpose of this randomized controlled trial (RCT) is to compare screw placement accuracy of robot-assisted surgery with integrated 3D computer-assisted navigation versus freehand surgery with 2D fluoroscopy for arthrodesis of the thoraco-lumbar spine.

Methods: This is a single-centre evaluator-blinded RCT with a 1:1 allocation ratio.

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β-microglobulin (β-m) is a plasma protein derived from physiological shedding of the class I major histocompatibility complex (MHCI), causing human systemic amyloidosis either due to persistently high concentrations of the wild-type (WT) protein in hemodialyzed patients, or in presence of mutations, such as D76N β-m, which favor protein deposition in the adulthood, despite normal plasma levels. Here we describe a new transgenic Caenorhabditis elegans () strain expressing human WT β-m at high concentrations, mimicking the condition that underlies dialysis-related amyloidosis (DRA) and we compare it to a previously established strain expressing the highly amyloidogenic D76N β-m at lower concentrations. Both strains exhibit behavioral defects, the severity of which correlates with β-m levels rather than with the presence of mutations, being more pronounced in WT β-m worms.

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