Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis

Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis.

Transthyretin amyloidosis is a fatal disorder that is characterized primarily by progressive neuropathy and cardiomyopathy. It occurs in both a mutant form (with autosomal dominant inheritance) and a wild-type form (with predominant cardiac involvement). This article guides clinicians as to when the disease should be suspected, describes the appropriate diagnostic evaluation for those with know...

Amyloidosis: diagnosis and prognosis

Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis.

BACKGROUND Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopathy for which several promising therapies are in development. The diagnosis is frequently delayed or missed because of the limited specificity of echocardiography and the traditional requirement for histological confirmation. It has long been recognized that technetium-labeled bone scintigraphy tracers can...

Systemic light-chain amyloidosis: advances in diagnosis, prognosis, and therapy.

Systemic light-chain amyloidosis (AL) is caused by misfolded immunoglobulin light-chain proteins that aggregate and deposit as unique fibrils, ultimately leading to organ failure and death. Recent developments that have significantly aided the management of patients with AL include diagnostic techniques for definitive typing of amyloid deposits by use of laser microdissection with mass spectrom...

Incidence, Diagnosis and Prognosis of Cardiac Amyloidosis

BACKGROUND AND OBJECTIVES Cardiac involvement is frequent in systemic amyloidosis and is the most important determinant of the clinical outcome. The aims of this study were to assess the incidence and prognosis of cardiac amyloidosis and discuss the diagnostic issues related to cardiac amyloidosis. SUBJECTS AND METHODS We retrospectively studied all patients diagnosed with systemic amyloidosi...