Amyloidosis is a heterogeneous group of disorders affecting a single-or multiple-organ system and presents as generalized or localized disease. Both generalized amyloidosis and localized amyloidosis can be primary or secondary. Localized amyloidosis affects organs like urinary bladder, lung, larynx, skin, tongue and the region around the eye, producing detectable nodular masses which are clinically suspected as malignancy. We present six cases of localized urinary bladder amyloidosis that were clinically and cystoscopically suspected as bladder tumor or cystitis, which occurred over a period of last 10 years. Histology in all cases revealed diagnosis of primary amyloidosis. None of them had any stigmata of secondary disease. The cases were treated by simple transurethral resection of bladder. Two out of the six cases recurred after 3 to 5 years of initial presentation and were asymptomatic thereafter. Amyloidosis of the bladder is a rare condition which often mimics bladder neoplasm clinically and cystoscopically and histological examination is a must for definite diagnosis and proper management.
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http://dx.doi.org/10.4103/0377-4929.41671 | DOI Listing |
Neurology
January 2025
Neurology Unit, Department of Clinical and Experimental Sciences, University of Brescia, Italy.
Objectives: Cerebral amyloid angiopathy (CAA) is the main driver of amyloid-related imaging abnormalities (ARIAs) in Alzheimer disease (AD). We compared different versions of the Boston criteria for CAA diagnosis in AD.
Methods: This article presents a single-center analysis (outpatient neurodegenerative clinic) of patients with AD with mild cognitive impairment (MCI) or early dementia, meeting NIA-AA criteria and having biological amyloid confirmation (CSF or imaging).
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 PDFJAMA 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.
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 PDFExpert 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.
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