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European Journal of Heart Failure Feb 2021
Topics: Amyloid Neuropathies, Familial; Heart Failure; Humans; Prealbumin
PubMed: 33342016
DOI: 10.1002/ejhf.2080 -
Innere Medizin (Heidelberg, Germany) Sep 2023Transthyretin amyloid cardiomyopathy (ATTR-CM) is an underdiagnosed cause of heart failure and arrhythmia. This differential diagnosis should particularly be considered... (Review)
Review
Transthyretin amyloid cardiomyopathy (ATTR-CM) is an underdiagnosed cause of heart failure and arrhythmia. This differential diagnosis should particularly be considered in older patients with left ventricular hypertrophy (LVH) who are also suffering from heart failure with preserved ejection fraction (HFpEF) or aortic valve stenosis. ATTR-CM is caused either by a genetic variation or by aging processes. The extracellular accumulation of amyloid fibrils in the heart causes a restrictive cardiomyopathy, which leads to typical heart failure symptoms as well as cardiac conduction and repolarization disturbances. Extracardiac problems such as a carpal tunnel syndrome can also be indicative for ATTR-CM. The disease can be diagnosed either by a myocardial biopsy or alternatively by a positive bone scintigraphy with the simultaneous exclusion of monoclonal proteins in blood and urine. Besides a symptomatic treatment, the transthyretin (TTR) stabilizer tafamidis is now available, which can significantly delay the disease progress. In the coming years, the approval of further drugs for the treatment of ATTR-CM is to be expected.
Topics: Aged; Humans; Amyloid Neuropathies, Familial; Cardiomyopathies; Heart Failure; Prealbumin; Stroke Volume; Tomography, X-Ray Computed; Benzoxazoles
PubMed: 37540258
DOI: 10.1007/s00108-023-01569-z -
Annals of Clinical and Laboratory... Jan 2021Prealbumin is a blood component tested for nutrition monitoring, which could be affected during inflammation. This study aimed to investigate the relationships between...
OBJECTIVE
Prealbumin is a blood component tested for nutrition monitoring, which could be affected during inflammation. This study aimed to investigate the relationships between prealbumin and C-reactive protein (CRP) in inflammatory rheumatic musculoskeletal diseases (RMDs), including Takayasu arteritis, antineutrophil cytoplasmic antibody-associated vasculitis, systemic lupus erythematosus, Behcet's disease and polymyositis/dermatomyositis.
METHODS
A total of 52 healthy controls and 508 RMD patients were included. We collected 3714 clinical and laboratory records from June 2011 to August 2019, and the longest follow-up period was eleven years. The associations between prealbumin and CRP, the globulin gap, the albumin-to-globulin ratio, and IgG were evaluated.
RESULTS
Prealbumin had a high correlation coefficient (r=-0.497, <0.001), consistent changes over time with CRP, and a high area under the curve [AUC=0.777 (95% CI 0.76-0.795)] for CRP. The statistical relationship between the prealbumin and CRP was not affected by sex, ethnicity or age. Among RMDs, prealbumin showed the strongest correlation with CRP in Takayasu arteritis (r=0.607, <0.001). In addition, a moderate relationship was observed between prealbumin and IgG, the globulin gap and the albumin-to-globulin ratio.
CONCLUSION
Prealbumin is closely related to CRP in Chinese patients with five chronic inflammatory RMDs, which may be due to the influence of chronic inflammation during the course of disease.
Topics: Adult; Biomarkers; C-Reactive Protein; Case-Control Studies; China; Female; Humans; Inflammation; Male; Middle Aged; Musculoskeletal Diseases; Prealbumin; ROC Curve; Rheumatic Diseases
PubMed: 33653784
DOI: No ID Found -
Heart Failure Reviews Mar 2024Transthyretin cardiac amyloidosis (ATTR-CA) is a progressive disease characterized by the deposition of abnormal transthyretin protein fibrils in the heart, leading to... (Review)
Review
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 significant role in various steps of ATTR-CA, including clinical presentation, diagnostic challenges, disease progression, and treatment outcomes. ATTR-CA predominantly affects men, whereas women are older at presentation. Women generally present with a history of heart failure with preserved ejection fraction and/or carpal tunnel syndrome. When indexed, left ventricular (LV) wall thickness is equal, or even increased, than men. Women also have smaller LV cavities, more preserved ejection fractions, and apparently a slightly worse right ventricular and diastolic function. Given the under-representation on women in clinical trials, no data regarding sex influence on the treatment response are currently available. Finally, it seems there are no differences in overall prognosis, even if premenopausal women may have a certain level of myocardial protection. Genetic variations, environmental factors, and hormonal changes are considered as potential contributors to observed disparities. Understanding sex differences in ATTR-CA is vital for accurate diagnosis and management. By considering these differences, clinicians can improve diagnostic accuracy, tailor treatments, and optimize outcomes for both sexes with ATTR-CA.
Topics: Humans; Female; Male; Cardiomyopathies; Prealbumin; Sex Characteristics; Amyloidosis; Heart; Amyloid Neuropathies, Familial
PubMed: 37566193
DOI: 10.1007/s10741-023-10339-w -
The American Journal of the Medical... Aug 2023
Topics: Humans; Prealbumin; Amyloidosis; Cardiomyopathies
PubMed: 37001694
DOI: 10.1016/j.amjms.2023.03.023 -
Current Heart Failure Reports Oct 2022Transthyretin cardiac amyloidosis (ATTR-CM) is an infiltrative cardiomyopathy and an increasingly recognized cause of morbidity and mortality. There remains substantial... (Review)
Review
PURPOSE OF REVIEW
Transthyretin cardiac amyloidosis (ATTR-CM) is an infiltrative cardiomyopathy and an increasingly recognized cause of morbidity and mortality. There remains substantial delay between initial symptoms and diagnosis. With the recent emergence of various targeted therapies proven to reduce morbidity and mortality, there is an imperative to diagnose subclinical disease. Biomarkers may be well-suited for this role.
RECENT FINDINGS
Conventional markers of heart failure, such as natriuretic peptides and cardiac troponins, and estimated glomerular filtration rate are associated with risk in ATTR-CM. Circulating transthyretin (TTR) levels parallel TTR kinetic stability, correlate with disease severity, and may serve as indirect markers of ATTR-CM disease activity and response to targeted treatment. There is also growing evidence for the correlation of TTR to retinol-binding protein 4, a biomarker which independently associates with this disease. The rate-limiting step for ATTR pathogenesis is dissociation of the TTR homotetramer, which may be quantified using subunit exchange to allow for early risk assessment, prognostication, and assessment of treatment response. The protein species that result from the dissociation and misfolding of TTR are known as nonnative transthyretin (NNTTR). NNTTR is quantifiable via peptide probes and is a specific biomarker whose reduction is positively correlated with improvement in neuropathic ATTR amyloidosis. Neurofilament light chain (NfL) is released into the blood after axonal damage and correlates with neuropathic ATTR amyloidosis, but its clinical use in ATTR-CM is uncertain. Conventional markers of heart failure, transthyretin, retinol-binding protein 4, transthyretin kinetic stability, nonnative transthyretin, peptide probes, and neurofilament light chain have potential as biomarkers to enable early, subclinical diagnosis in patients with transthyretin cardiac amyloidosis.
Topics: Amyloid Neuropathies, Familial; Biomarkers; Cardiomyopathies; Heart Failure; Humans; Prealbumin; Troponin
PubMed: 35930129
DOI: 10.1007/s11897-022-00570-1 -
American Journal of TherapeuticsDeposition of wild-type or mutant transthyretin (TTR) amyloid fibrils in the myocardium causes TTR amyloid cardiomyopathy (ATTR-CM). Targeted therapeutics for ATTR-CM...
BACKGROUND
Deposition of wild-type or mutant transthyretin (TTR) amyloid fibrils in the myocardium causes TTR amyloid cardiomyopathy (ATTR-CM). Targeted therapeutics for ATTR-CM include TTR stabilizers (tafamidis and diflunisal) and oligonucleotide drugs (revusiran, patisiran, and inotersen). TTR stabilizers prevent dissociation of transthyretin tetramers. Transthyretin monomers can misfold and form amyloid fibrils. TTR stabilizers thereby limit amyloid fibrils development and deposition. Oligonucleotide drugs inhibit hepatic synthesis of transthyretin, which decreases transthyretin protein levels and thus the amyloid fibril substrate.
AREAS OF UNCERTAINTY
To study the safety and efficacy of targeted therapeutics in patients with ATTR-CM, we performed a pooled analysis. A random-effects model with the Mantel-Haenszel method was used to pool the data.
DATA SOURCES
A literature search was performed using PubMed, Cochrane CENTRAL, and Embase databases using the search terms "cardiac amyloidosis" AND "tafamidis" OR "patisiran" OR "inotersen" OR "revusiran" OR "diflunisal."
THERAPEUTIC ADVANCES
We identified 6 studies that compared targeted therapeutics with placebo. One study was stopped prematurely because of increased mortality in the targeted therapeutics arm. Pooled analysis included 1238 patients, of which 738 patients received targeted therapeutics and 500 patients received placebo. When compared with placebo, targeted therapeutics significantly reduced all-cause mortality [OR 0.39, 95% confidence interval (CI): 0.16-0.97, P = 0.04]. Only 2 studies reported the effect on cardiovascular-related hospitalizations. There was a trend toward an improvement in global longitudinal strain (mean difference -0.69, 95% CI: -1.44 to 0.05, P = 0.07). When compared with placebo, there was no increase in serious adverse events with targeted therapeutics (OR 1.06, 95% CI: 0.78-1.44, P = 0.72).
CONCLUSION
Evidence from the pooled analysis revealed targeted therapeutics improve survival and are well-tolerated. These findings suggest a potential role for targeted therapeutics in the treatment of patients with ATTR-CM.
Topics: Humans; Amyloid Neuropathies, Familial; Prealbumin; Diflunisal; Oligonucleotides; Cardiomyopathies
PubMed: 37713689
DOI: 10.1097/MJT.0000000000001296 -
Heart Failure Clinics Jan 2022Transthyretin cardiac amyloidosis (ATTR-CA) is a systemic disorder resulting from the extracellular deposition of amyloid fibrils of misfolded transthyretin protein in... (Review)
Review
Transthyretin cardiac amyloidosis (ATTR-CA) is a systemic disorder resulting from the extracellular deposition of amyloid fibrils of misfolded transthyretin protein in the heart. ATTR-CA is a life-threatening disease, which can be caused by progressive deposition of wild type transthyretin (wtATTR) or by aggregation of an inherited mutated variant of transthyretin (mATTR). mATTR Is a rare condition transmitted in an autosomal dominant manner with incomplete penetrance, causing heterogenous phenotypes which can range from predominant neuropathic involvement, predominant cardiomyopathy, or mixed. Diagnosis of ATTR-CA is complex and requires integration of different imaging tools (echocardiography, bone scintigraphy, magnetic resonance) with genetics, clinical signs, laboratory tests, and histology. In recent years, new therapeutic agents have shown good efficacy and impact on survival and quality of life in this subset of patients, nevertheless patients affected by ATTR-CA may still carry an unfavorable prognosis, thus highlighting the need for new therapies. This review aims to assess cardiovascular involvement, diagnosis, and management of patients affected by ATTR-CA.
Topics: Amyloid Neuropathies, Familial; Cardiomyopathies; Heart; Humans; Prealbumin; Quality of Life
PubMed: 34776085
DOI: 10.1016/j.hfc.2021.07.006 -
Journal of the American College of... Jul 2019
Topics: Amyloidosis; Cardiomyopathies; Humans; Prealbumin
PubMed: 31319911
DOI: 10.1016/j.jacc.2019.05.031 -
Global Heart 2023Transthyretin cardiac amyloidosis (ATTR-CA) has been traditionally considered a rare and inexorably fatal condition. ATTR-CA now is an increasingly recognised cause of...
Transthyretin cardiac amyloidosis (ATTR-CA) has been traditionally considered a rare and inexorably fatal condition. ATTR-CA now is an increasingly recognised cause of heart failure and mortality worldwide with effective pharmacological treatments. Advances in non-invasive diagnosis, coupled with the development of effective treatments, have transformed the diagnosis of ATTR-CA, which is now possible without recourse to endomyocardial biopsy in around 70% of cases. Many patients are now diagnosed at an earlier stage. Echocardiography and cardiac magnetic resonance have enabled identification of patients with possible ATTR-CA and more accurate prognostic stratification. Therapies able to slow or halt ATTR-CA progression and increase survival are now available and there is also evidence that patients may benefit from specific conventional heart failure medications. A wide horizon of possibilities is unfolding and awaits discovery.
Topics: Humans; Prealbumin; Amyloidosis; Heart Failure; Prognosis; Cardiomyopathies
PubMed: 38028963
DOI: 10.5334/gh.1275