Systemic amyloidosis is a fatal disorder caused by pathological extracellular deposits of amyloid fibrils that are always coated with the normal plasma protein, serum amyloid P component (SAP). The small-molecule drug, miridesap, [(R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC)] depletes circulating SAP but leaves some SAP in amyloid deposits. This ...

Apolipoprotein A-I amyloidosis is a rare, late-onset, autosomal dominant condition characterized by systemic deposition of amyloid in tissues, the major clinical problems being related to renal, hepatic, and cardiac involvement. Described is the clinical and histologic picture of renal involvement as a result of apolipoprotein A-I amyloidosis in five families of Italian ancestry. In all of the ...

purpose : primary localized amyloidosis of eyelid is a localized type of amyloidosis without evidence of systemic involvement, which is a quite rare clinical condition. here we report a case of primary localized amyloidosis of eyelid. case report : a 40-year-old woman presented with a four-month history of swelling of the left upper eyelid resulting in a mechanical ptosis. the mass had a firm b...

RATIONALE Hereditary amyloidosis is diagnosed worldwidely with an increasing incidence. As the most common form, transthyretin-related hereditary amyloidosis (ATTR amyloidosis) is an autosomal dominant inherited disease due to mutations of TTR. Over the past several decades, more than 130 mutations have been reported. Previous studies suggested that ATTR amyloidosis initially showed polyneuropa...

Familial Mediaterranian Fever (FMF) is an ethnically related genetic disease, characterized by spontaneously resolving episodes of fever and pain. Amyloidosis of the amyloid A type is the most important manifestation of FMF, affecting many organs, including the kidneys, adrenal glands, intestines, spleen, thyroid, heart, lungs and liver. There is an increasing concern about the association of n...

A variety of biliary and hepatocellular diseases occur with increased incidence in patients with inflammatory bowel disease. These include fatty infiltration of the liver, cholelithiasis, pericholangitis-primary sclerosing cholangitis, cirrhosis, chronic active hepatitis, liver abscess, amyloidosis, granulomatous hepatitis, and bile duct carcinoma. Radiography is essential in accurate diagnosis.

Secondary amyloidosis is a common disease of water fowl and is characterized by the deposition of extracellular fibrils of amyloid A (AA) protein in the liver and certain other organs. Neither the normal role of serum amyloid A (SAA), a major acute phase response protein, nor the causes of secondary amyloidosis are well understood. To investigate a possible genetic contribution to disease susce...

Immunoglobulin light chain systemic amyloidosis (AL) is a progressive disease caused by monoclonal light chains with specific mutations that confer a unique propensity to misfold from their native structure to less stable, partially folded intermediates that selfaggregate into oligomers and then into the highlyordered cross β-sheet structure which defines amyloid fibrils. At least 11 additional...

BACKGROUND Amyloidosis is a systemic disease known to affect a vast range of organs, including the liver, heart, and kidney. When infiltrating the liver, amyloidosis typically does not present with cirrhosis. Typical presentation includes hepatomegaly with some mild laboratory abnormalities. CASE REPORT A 72-year-old man presented with a 2-week history of worsening abdominal, scrotal, and ext...