Results 61 to 70 of about 35,531 (245)
Abstract Aims Transthyretin amyloid cardiomyopathy (ATTR‐CM) is an increasinglyrecognized cause of heart failure with preserved ejection fraction (HFpEF), which may be diagnosed non‐invasively using 99mTc 3,3‐diphosphono‐1,2‐propanodicarboxylic acid (DPD) scintigraphy‐based diagnostic criteria.
L. Healy +15 more
wiley +1 more source
Renal Amyloidosis Associated With 5 Novel Variants in the Fibrinogen A Alpha Chain Protein
Fibrinogen A alpha chain amyloidosis is an autosomal dominant disease associated with mutations in the fibrinogen A alpha chain (FGA) gene, and it is the most common cause of hereditary renal amyloidosis in the UK.
Dorota Rowczenio +18 more
doaj +1 more source
Púrpura. Manifestação de Amiloidose Sistémica Primária [PDF]
Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are
Apetato, M +4 more
core
Relationship between aetiology and left ventricular systolic dysfunction in hypertrophic cardiomyopathy [PDF]
Background: Hypertrophic cardiomyopathy (HCM) is a common cardiac disease caused by a range of genetic and acquired disorders. The most common cause is genetic variation in sarcomeric proteins genes.
Rosmini, Stefania <1981>
core +1 more source
A phenomap of TTR amyloidosis to aid diagnostic screening
Abstract Cardiac amyloidosis due to transthyretin (ATTR) remains an underdiagnosed cause of cardiomyopathy. As awareness of the disease grows and referrals for ATTR increase, clinicians are likely to encounter more atypical forms of the condition in clinical practice.
Alexios S. Antonopoulos +4 more
wiley +1 more source
Crystal structure of monomeric human β-2- microglobulin reveals clues to its amyloidogenic properties [PDF]
Dissociation of human β-2-microglobulin (β(2)m) from the heavy chain of the class I HLA complex is a critical first step in the formation of amyloid fibrils from this protein.
A. P. Kalverda +31 more
core +2 more sources
Abstract Aims Amyloid cardiomyopathy is caused by the deposition of light chain (AL) or transthyretin amyloid (ATTR) fibrils, that leads to a restrictive cardiomyopathy, often resulting in heart failure (HF) with preserved or reduced ejection fraction.
Robin Willixhofer +25 more
wiley +1 more source
Undiagnosed light chain systemic amyloidosis: does it matter to anesthesiologists? -a case report- [PDF]
Light chain systemic amyloidosis is rare but may accompany laryngeal or pulmonary involvement, which may increase the risk in airway management. We present a case of a patient planned for resection of cervical epidural mass. The patient had face and neck
Gwan Ho Kim +3 more
doaj +1 more source
The non-coding RNA landscape of plasma cell dyscrasias [PDF]
Despite substantial advancements have been done in the understanding of the pathogenesis of plasma cell (PC) disorders, these malignancies remain hard-to-treat.
Azab, Kareem A +3 more
core +1 more source
The aim of our study was to develop a more accurate staging system for hereditary ATTR patients currently receiving specific treatment. A staging system was created using as criteria: GLS ≤ −11%, NT‐proBNP ≤ 2,000 ng/L, and eGFR ≤ 65 mL/min. We managed to accurately stratify patients into three disease stages, significantly different in terms of ...
Gabriela Neculae +7 more
wiley +1 more source

