Familial amyloidotic polyneuropathy (FAP) is an inherited disease characterized by an abnormal systemic deposition of a mutant protein called transthyretin (TTR) with elective involvement of the peripheral nervous system, but often determining cardiac, gastrointestinal, and urinary tract dysfunction. FAP commonly affects the liver and the heart until end-organs failure. Transthyretin amyloidosi...

BACKGROUND Patients with end-stage-liver-disease (ESLD) require orthotopic liver transplantation (OLT) as treatment. However, cirrhotic cardiomyopathy can be clinically revealed during OLT, with the possible development of a transient overt congestive heart failure. A number of patients require a combined procedure of liver transplantation and heart surgery, which includes heart transplantation...

AA amyloidosis results from the pathologic deposition in the kidneys and other organs of fibrils composed of N-terminal fragments of serum amyloid A protein (SAA). Given that there are only limited means to visualize these deposits, we have developed a series of mAbs, 2A4, 7D8, and 8G9, that bind specifically with nanomolar affinity to a carboxy-terminal epitope generated following proteolysis ...

The association of the presence of an abnormal serum protein fraction between P and y globulins in reticuloendothelial disease and lymphatic leukaemia is a Ml recognized phenomenon (Rundles et al., 1954; Buffa and Rappaport, 1957; Owen e* 1959). The combination of leukaemia and amyloidosis is also recognized, but he presence of a coagulation defect in amyloidosis must be relatively uncommon. CA...

Systemic amyloidosis commonly affects the heart. Indeed, cardiac symptoms may be the first clinical indicator of underlying amyloid deposition. Using two case studies, this article reviews the latest evidence regarding cardiac amyloidosis. The diagnosis of cardiac involvement can be established through imaging with echocardiography and magnetic resonance. Supportive evidence may be gained from ...

Amyloidosis in adult rheumatoid arthritis is well documented (Table I). Rosenberg, Baggenstoss, and Hench (1944) reported an incidence of 6-6 per cent. in a study of thirty post mortem cases and Unger, Zuckerbrod, Beck, and Steele (1948a) recorded a similar incidence (6 -9 per cent. in 58 cases), but Teilum and Lindahl (1954) found it in 60-7 per cent. of 28 post mortem cases. Missen and Taylor...

Amyloidosis results from extracellular deposition of fibril-forming proteins and currently ~30 different proteins have been found to be amyloidogenic. Recently, a novel type of amyloidosis with a high incidence on Hispanic population has been described to be derived from leukocyte chemotactic factor 2 (ALECT2). The objective of the present article is to raise awareness on the presence of this e...

Authors describe the case of a 60-year-old diabetic man who presented with jaundice, ascites and significant weight loss over a period of 2 months. Physical examination revealed firm hepatomegaly with ascites. On evaluation, nephropathy, axonal neuropathy, carpal tunnel syndrome and decompensated cryptogenic liver disease with portal hypertension were found fitting with the diagnosis of diabeti...

Introduction: We report the first case of coexistent systemic amyloidosis, plasma cell myeloma (MM), and myelodysplastic syndrome (refractory anemia with ringed sideroblasts:MDS-RARS). Case Report: Our patient was diagnosed with amyloidosis of the liver, stomach and tongue following which a bone marrow biopsy was performed. Review of the bone marrow biopsy revealed the presence of two distinct ...

Background While transthyretin (TTR) amyloidosis is usually characterized by peripheral and autonomic neuropathy, a majority of patients also have evidence of restrictive cardiomyopathy. In addition, amyloid cardiomyopathy may occur in the elderly without the presence of a mutant form of TTR. Both the hereditary and wild-type TTR amyloidosis are characterized by progressive restrictive cardiomy...