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The role of multi-modality imaging for the assessment of left atrium and left atrial appendage. A clinical consensus statement of the European Association of Cardiovascular Imaging (EACVI), European Heart Rhythm Association (EHRA) of the European Society of Cardiology (ESC).
Eur Heart J Cardiovasc Imaging
January 2025
Department of Cardiology, University of Rennes, CHU Rennes, Inserm, LTSI - UMR 1099, Rennes, France.
Structural, architectural, contractile or electrophysiological alterations may occur in the left atrium (LA). The concept of LA cardiopathy is supported by accumulating scientific evidence demonstrating that LA remodeling has become a cornerstone diagnostic and prognostic marker. The structure and the function of LA and left atrial appendage (LAA) which is an integral part of the LA, are key elements for a better understanding of multiple clinical conditions, most notably atrial fibrillation (AF), cardioembolism, heart failure and mitral valve diseases.
View Article and Find Full Text PDFFive-Year Results With Patisiran for Hereditary Transthyretin Amyloidosis With Polyneuropathy: A Randomized Clinical Trial With Open-Label Extension.
JAMA Neurol
January 2025
Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy.
Importance: There is a lack of long-term efficacy and safety data on hereditary transthyretin amyloidosis with polyneuropathy (hATTR-PN) and on RNA interference (RNAi) therapeutics in general. This study presents the longest-term data to date on patisiran for hATTR-PN.
Objective: To present the long-term efficacy and safety of patisiran in adults with hATTR-PN.
Localized light chain amyloidosis of the stomach that regressed after eradication of Helicobacter pylori over 15 years of follow-up: a case report and new hypothesis.
Clin J Gastroenterol
January 2025
Department of Gastroenterology, Saiseikai Kanazawa Hospital, Ni-13-6 Akatsuchimachi, Kanazawa, Ishikawa, 920-0353, Japan.
Localized light chain amyloidosis is considered to be a plasmacytic B-cell lymphoproliferative disorder caused by antigenic induction. A hypothesis has been proposed that antigen-induced local plasmacytic B cells produce amyloidogenic proteins that are processed into amyloid fibrils in giant cells leading to amyloid fibril deposition. However, the inciting antigen exposure or immune response that signals plasmacytic B-cell infiltration, activation, and selection, is unknown.
View Article and Find Full Text PDFA retrospective observational analysis of the real-world care pathway of people with hereditary transthyretin amyloidosis with polyneuropathy in Italy.
Expert Rev Pharmacoecon Outcomes Res
January 2025
ANMCO Research Center, Heart Care Foundation, Firenze, Italy.
Background: This retrospective observational study described the epidemiology and the burden on the Italian healthcare service (SSN) of patients with polyneuropathy (PN) associated with hereditary transthyretin amyloidosis (ATTRv).
Research Design And Methods: From the Fondazione ReS (Ricerca e Salute) administrative healthcare database (~5.5 million inhabitants in 2021), patients were identified as having ATTRv-PN in 2021 if they had received treatments for ATTRv-PN under SSN reimbursement (i.
Primary localized bladder amyloidosis with negative postoperative Congo red staining: A case report.
Urol Case Rep
January 2025
Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Article Synopsis
- Localized bladder amyloidosis is a rare condition that can be mistaken for a bladder tumor, with only over 200 cases documented.
- A 67-year-old woman experienced painless blood in urine, leading to tests that suggested bladder amyloidosis, including a Congo red staining potentially indicating the presence of amyloid.
- The excised tissue from her procedure showed unexpected pink-staining patterns but did not confirm amyloidosis through Congo red staining, highlighting the importance of thorough evaluation for diagnosis despite negative results.
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