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The PROPHECI trial: a phase II, double-blind, placebo-controlled, randomized clinical trial for the treatment of pseudoxanthoma elasticum with oral pyrophosphate.
Trials
January 2025
Université Côte d'Azur, CNRS, LP2M, Nice, France.
Background: /aims. Pseudoxanthoma Elasticum (PXE, OMIM 264800) is an autosomal, recessive, metabolic disorder characterized by progressive ectopic calcification in the skin, the vasculature and Bruch's membrane. Variants in the ABCC6 gene are associated with low plasma pyrophosphate (PPi) concentration.
View Article and Find Full Text PDFThe question of strains in AA amyloidosis.
Sci Rep
January 2025
Department of Immunology, Genetics and Pathology, Uppsala University, Rudbeck Laboratory, C11, 75185, Uppsala, Sweden.
The existence of transmissible amyloid fibril strains has long intrigued the scientific community. The strain theory originates from prion disorders, but here, we provide evidence of strains in systemic amyloidosis. Human AA amyloidosis manifests as two distinct clinical phenotypes called common AA and vascular AA.
View Article and Find Full Text PDFSurgical and oncological implications of the presence of hepatic artery anatomical variations in patients undergoing pancreaticoduodenectomy: a single center experience.
Updates Surg
January 2025
Pancreatic and Endocrine Surgical Unit, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Bologna, Italy.
The presence of an aberrant right hepatic artery (a-RHA) could influence the oncological and postoperative outcomes after pancreaticoduodenectomy (PD). A comparative study was conducted, including patients who underwent PD with a-RHA or with normal RHA anatomy. The primary endpoints were R1 resection in all margins (pancreatic, anterior, posterior, superior mesenteric artery, and portal groove), overall survival (OS), and disease-free survival (DFS).
View Article and Find Full Text PDFSuperior Outcomes with Ultrasound-Guided Hyaluronidase for Impending Filler-Induced Facial Skin Necrosis: A Systematic Review and Pilot Meta-Analysis.
Aesthetic Plast Surg
January 2025
Department of Plastic and Reconstructive Surgery, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
Background: Hyaluronidase remains the mainstay treatment for impending filler-induced facial skin necrosis. Complete resolution of impending skin necrosis following hyaluronidase injection is estimated to be around 77.8%.
View Article and Find Full Text PDFPhoton-counting detector CTA to assess intracranial stents and flow diverters: an in vivo study with ultrahigh-resolution spectral reconstructions.
Eur Radiol Exp
January 2025
Department of Neuroradiology, University hospital RWTH Aachen, Aachen, Germany.
Background: To define optimal parameters for the evaluation of vessel visibility in intracranial stents (ICS) and flow diverters (FD) using photon-counting detector computed tomography angiography (PCD-CTA) with spectral reconstructions.
Methods: We retrospectively analyzed consecutive patients with implanted ICS or FD, who received a PCD-CTA between April 2023 and March 2024. Polyenergetic, virtual monoenergetic, pure lumen, and iodine reconstructions with different keV levels (40, 60, and 80) and reconstruction kernels (body vascular [Bv]48, Bv56, Bv64, Bv72, and Bv76) were evaluated by two radiologists with regions of interests and Likert scales.
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