Cardiac Amyloidosis – An Underdiagnosed Cause of Heart Failure with Preserved Ejection Fraction – Updated Diagnosis and Treatment Options [PDF]
Cardiac amyloidosis (CA) still represents a frequently missed cause of heart failure with preserved ejection fraction (HFpEF). In the light of many new and effective therapies for immunoglobulin light chain amyloidosis (AL) and for transthyretin ...
Roxana Cristina RIMBAS +3 more
doaj +1 more source
Senile Systemic Amyloidosis: Clinical Features at Presentation and Outcome [PDF]
Background Cardiac amyloidosis is a fatal disease whose prognosis and treatment rely on identification of the amyloid type. In our aging population transthyretin amyloidosis (ATTRwt) is common and must be differentiated from other amyloid types.
Banypersad, SM +15 more
core +1 more source
Monoclonal gammopathy of renal significance: Diagnostic workup [PDF]
The clinical spectrum of diseases associated with monoclonal gammopathies is wide and they are most commonly the consequence of renal deposition of monoclonal immunoglobulin or its components.
Cabrita, A. +5 more
core +1 more source
Chronic myopathy due to immunoglobulin light chain amyloidosis [PDF]
Amyloid myopathy associated with a plasma cell dyscrasia is a rare cause of muscle hypertrophy. It can be a challenging diagnosis, since pathological findings are often elusive. In addition, the mechanism by which immunoglobulin light-chain deposition stimulates muscle overgrowth remains poorly understood. We present a 53-year old female with a 10-year
Manoli, Irini +9 more
openaire +3 more sources
Light Chain Stabilization: A Therapeutic Approach to Ameliorate AL Amyloidosis
Non-native immunoglobulin light chain conformations, including aggregates, appear to cause light chain amyloidosis pathology. Despite significant progress in pharmacological eradication of the neoplastic plasma cells that secrete these light chains, in ...
Gareth J. Morgan +2 more
doaj +1 more source
A common beta-sheet architecture underlies in vitro and in vivo beta(2)-microglobulin amyloid fibrils [PDF]
Misfolding and aggregation of normally soluble proteins into amyloid fibrils and their deposition and accumulation underlies a variety of clinically significant diseases.
Jahn, T.R., Radford, S.E., Tennent, G.A.
core +2 more sources
Longer survival using modern therapies has increased the number of patients with immunoglobulin light-chain amyloidosis receiving kidney transplantation.
C. Heybeli +24 more
semanticscholar +1 more source
Localized Lymph Node Light Chain Amyloidosis
Immunoglobulin-derived light chain amyloidosis can occasionally be associated with localized disease. We present a patient with localized lymph node light chain amyloidosis without an underlying monoclonal protein or lymphoproliferative disorder and ...
Binod Dhakal +3 more
doaj +1 more source
Duodenal biopsy: an unexpected confirmatory test for a patient with al-amyloidosis
SSystemic AL- amyloidosis is a disorder of protein folding in which there is extra-cellular accumulation as β pleated fibrillar deposits of monoclonal immunoglobulin light chain fragments. AL- amyloidosis is a rare clinical entity.
S. Pirasath +4 more
doaj +1 more source
Skeletal Myopathy as the Initial Manifestation of Light Chain Multiple Myeloma
Monoclonal gammopathies due to plasma cell dyscrasias can cause various rare neuromuscular disorders. The peripheral nervous system is most commonly affected, while muscle diseases associated with monoclonal gammopathies are rare. Skeletal myopathy, as a
Mohamed Reda Belkhribchia +5 more
doaj +1 more source

