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Amyloidosis 

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  1. 11-04-2024 | Cardiac Amyloidosis | News | Article
    ACC 2024

    AI promising direction for cardiac amyloidosis detection

    AI models may be able to accurately detect cardiac amyloidosis in a range of diagnostic examinations. 

  2. Open Access 03-04-2024 | AA Amyloidosis | Online First

    AA Amyloidosis: A Contemporary View

    Disorders that create sustained inflammatory responses can result in extracellular deposition of amyloid A (AA) fibrils in various organs. These fibrils are derived from an acute-phase reactant, serum AA (SAA) protein, through a long process …

  3. 11-04-2024 | Amyloidosis | Online First

    Acute ischemic stroke as initial manifestation of cardiac amyloidosis

  4. Open Access 18-04-2024 | Cardiac Amyloidosis | Online First

    (Non)-Exertional Variables of Cardiopulmonary Exercise Testing in Heart Failure with and Without Cardiac Amyloidosis

    Cardiac amyloidosis (CA), either triggered by light chain (AL) or transthyretin (TTR) deposition in the heart, results in a restrictive relaxation pattern and heart failure (HF) with reduced prognosis [ 1 ]. Exercise limitations can be directly …

  5. 16-04-2024 | Amyloidosis | Online First

    Long-term treatment of hereditary transthyretin amyloidosis with patisiran: multicentre, real-world experience in Italy

    Hereditary transthyretin (ATTRv, v for variant) amyloidosis is a rare, progressive and ultimately lethal autosomal dominant disorder [ 1 ]. Transthyretin (TTR) circulates as a tetrameric protein produced by the liver. Mutations in the TTR gene …

  6. Open Access 01-04-2024 | AL Amyloidosis | OriginalPaper

    Minimal residual disease in systemic light chain amyloidosis: a systematic review and meta-analysis

    Systemic light chain (AL) amyloidosis is characterized by the secretion of monoclonal immunoglobulin light chains by abnormal clonal plasma cells, which are deposited in target organs, causing organ morphological abnormalities and dysfunction …

  7. 30-03-2024 | Amyloidosis | Online First

    A Case of Immunoglobulin Light Chain Hepatic Amyloidosis with Abdominal Hematoma

  8. 05-04-2024 | Cardiac Amyloidosis | Online First

    Serum alpha 1 antitrypsin potent act as an early diagnostic biomarker for cardiac amyloidosis

    Cardiac amyloidosis is a refractory cardiomyopathy with a poor prognosis and lacks effective treatments. N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin T are poor prognostic factors for myocardial amyloidosis. However, NT-proBNP …

  9. 24-04-2024 | Scintigraphy | Online First

    Letter to the editor concerning “Cardiac [99mTc]Tc-hydroxydiphosphonate uptake on bone scintigraphy in patients with hereditary transthyretin amyloidosis: an early follow-up marker?”

  10. Open Access 20-12-2023 | Amyloidosis | OriginalPaper

    Clinical and Genotype Characteristics and Symptom Migration in Patients With Mixed Phenotype Transthyretin Amyloidosis from the Transthyretin Amyloidosis Outcomes Survey

    Transthyretin amyloidosis (ATTR amyloidosis) is a progressive, multisystemic disease with hereditary (variant; ATTRv amyloidosis) and acquired (wild-type; ATTRwt amyloidosis) etiologies [ 1 ]. ATTR amyloidosis results from the deposition of …

  11. 17-04-2024 | Amyloidosis | Online First

    ALECT2 amyloidosis with concurrent IgG4-related interstitial nephritis, membranous nephropathy and diabetic kidney disease: a case report and literature review

    Leukocyte chemotactic factor-2 amyloidosis (ALECT2) is a recently described subtype of amyloidosis. IgG4-related disease is a rare fibroinflammatory condition characterized by dense interstitial lymphoplasmacytic infiltrates and fibrosis.

  12. Open Access 01-12-2024 | Alzheimer's Disease | Letter

    Beyond genetics: including the environmental dimension in amyloidosis mouse models for Alzheimer’s disease

    Environmental and lifestyle factors can promote peripheral inflammation, contributing to later cognitive decline [ 5 – 9 ]. Infections, even occurring years before dementia onset, elevate dementia risk through peripheral inflammatory and vascular …

  13. Open Access 23-03-2024 | Cardiac Amyloidosis | Online First

    Detection of cardiac amyloidosis on routine bone scintigraphy: an important gatekeeper role for the nuclear medicine physician

    Cardiac amyloidosis (CA)—mostly transthyretin-related (ATTR-CA)—has recently gained interest in cardiology. Bone scintigraphy (BS) is one of the main screening tools for ATTR-CA but also used for various other reasons. The objective was to …

  14. 25-01-2024 | Amyloidosis | OriginalPaper

    A case of intestinal intussusception with unique hemorrhagic polyps due to AL amyloidosis and excessive anticoagulation

    Most adult intussusceptions are secondary to various pathological conditions that serve as a lead point. Because of their serious nature, intussusceptions often require emergency surgery. We report a surgical case of amyloidosis associated with …

  15. 22-01-2024 | Scintigraphy | OriginalPaper

    Performance of 99mTc-PYP scintigraphy in the diagnosis of hereditary transthyretin cardiac amyloidosis

    Transthyretin amyloid cardiomyopathy (ATTR-CA) is caused by the destabilization of the transthyretin (TTR) protein, which promotes release and accumulation of monomers and the formation of amyloid fibrils deposited in the myocardium[ 1 ], leading …

  16. Open Access 01-12-2024 | Cardiac Amyloidosis | OriginalPaper

    Prognostic value of native T1 and extracellular volume in patients with immunoglubin light-chain amyloidosis

    Immunoglobulin light-chain amyloidosis (AL) is the most common systemic amyloidosis results from the deposition of protein derived from fragments of clonal immunoglobulin light chain by abnormally functioning plasma cells. Cardiac involvement is …

  17. Open Access 01-12-2024 | Respiratory Microbiota | OriginalPaper

    Sodium oligomannate alters gut microbiota, reduces cerebral amyloidosis and reactive microglia in a sex-specific manner

    It has recently become well-established that there is a connection between Alzheimer’s disease pathology and gut microbiome dysbiosis. We have previously demonstrated that antibiotic-mediated gut microbiota perturbations lead to attenuation of Aβ …

  18. Open Access 23-10-2023 | Amyloidosis | OriginalPaper

    Heterogenous electrophysiological features in early stage of hereditary transthyretin amyloidosis neuropathy

    Hereditary transthyretin amyloidosis (ATTRv, v for variant) is a multisystemic disorder caused by mutation in the transthyretin ( TTR ) gene with a progressive involvement of peripheral nervous system (PNS). The mutated TTR tetramer dissociates in …

  19. Open Access 11-08-2023 | Cardiac Amyloidosis | ReviewPaper

    Sex differences in transthyretin cardiac amyloidosis

    Transthyretin cardiac amyloidosis (ATTR-CA) is a progressive disease characterized by the deposition of abnormal transthyretin protein fibrils in the heart, leading to cardiac dysfunction. Recent evidence suggests that sex differences may play a …

  20. Open Access 01-12-2024 | Amyloidosis | OriginalPaper

    A case of bilateral amyloidosis localized to extraocular muscles mimicking thyroid eye disease

    Amyloidosis is a rare disease characterized by the accumulation of abnormal amyloid fibrillar protein, in various tissues [ 1 , 2 ]. Ocular amyloidosis has been documented in nearly all parts of the eye, including adnexal and orbital tissues [ 2 ].

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