ESC: Tafamidis Cuts Deaths, Admissions in TTR Cardiac Amyloidosis
(MedPage Today) -- Plus clear advantage seen in 6-minute walk test
(Shinshu University) Small gauge vitrectomy for vitreous amyloidosis and subsequent management of secondary glaucoma in patients with hereditary transthyretin amyloidosis.
Abstract Many metabolic and neurodegenerative diseases are associated with protein misfolding and aggregation. Insulin a key hormone, under certain conditions aggregates and forms pathological amyloid fibrils. Several polyphenols have been studied extensively to elucidate their inhibitory effect on amyloid formation. In the present study, we used insulin as an amyloid model to test the mechanism and efficacy of rutin as an anti-amyloidogenic molecule. By using electron microscopy, dynamic light scattering and circular dichroism spectroscopy, we show that rutin inhibits the insulin aggregate and fibril formation. F...
Hereditary transthyretin (ATTRv) amyloidosis is an adult-onset, systemic disorder caused by mutations in the transthyretin (TTR) gene. As ATTRv amyloidosis is inherited in an autosomal dominant manner, family members of the patients are at risk of developing the disease.
The wrist is not commonly aspirated in the emergency department, but emergent arthrocentesis may be indicated for extreme or concerning cases, and tapping the wrist to determine the underlying pathology or relieve pain may be of great value. The synovial fluid from the joint space can be analyzed for crystals, infection, and blood. This information may help determine the overall plan and aid in decision-making and consultation. The ultimate treatment plan may include admission, intravenous antibiotics, multiple aspirations, and even surgical washout.A swollen, painful wrist that is hot to the touch is concerning for sep...
Amyloid transthyretin (ATTR) cardiac amyloidosis (CA) is an increasingly recognized cause of restrictive cardiomyopathy and associated heart failure with preserved ejection fraction (HFpEF). Despite improved diagnostic techniques to identify this condition, many patients experience delayed diagnosis. Interpretation of routine cardiac biomarkers (i.e. troponins and brain natriuretic peptide (BNP)) and echocardiography in ATTR-CA is limited, particularly in relation to pyrophosphate (PYP) scintigraphy.
Medical treatment for cardiac amyloidosis (CA) is evolving rapidly. Heart transplantation can be a valid option when followed by transplantation of bone marrow or liver, dependent on the type and origin of the amyloid protein. Thus, accurate typing of amyloidosis has implications for treatment, prognosis, and genetic counseling. Although non-invasive diagnostic techniques can type CA, endomyocardial biopsy (EMB) may be needed in the case of equivocal imaging findings or discordant data. We aimed to define the role of mass spectrometry (MS) for diagnosis and subtyping of CA.
After initial efforts at heart transplantation (HT) in patients with light chain amyloidosis were fraught with poor outcomes, emerging experience with modern chemotherapy regimens has led to a major improvement in outcomes in select centers. Given the systemic nature of the disease and the added immunosuppression in the form of chemotherapy, this study sought to analyze if AL patients undergoing HT had a higher burden of readmissions and infections non-amyloid heart transplant patients.
Advanced amyloid cardiomyopathy (ACM) patients have high waitlist (WL) mortality. Given the greater emphasis and clarity for status exceptions for ACM patients in the new allocation system, we sought to assess whether this change in the allocation policy would affect the WL and post-transplant outcomes in ACM pts.
Limited data exists on the use continuous-flow left ventricular assist device (CF-LVAD) in patients with cardiac amyloidosis (CA). The largest single center analysis on CF-LVAD support in patients with CA reports a 50% mortality at 6-months. Our primary aim was to examine the survival and morbidity of our patients with diagnosed CA and supported by CF-LVAD.
Advances in amyloidosis management have improved patient longevity. Coupling potential increased transplantation for amyloid patients with graft scarcity, we intend to assess the use of extended-donor criteria (EDC) hearts for amyloidosis.