Amyloidosis is characterized by the systemic or localized extra cellular deposition of amyloid, a proteinaceous fibrillar material in various tissues and organs, mainly classified into primary (AL) or secondary (AA) groups according to the biochemical nature of the fibril forming protein. The histopathological diagnosis was made on Congo red stained biopsy specimens using polarized light and th...

Amyloid is identified microscopically as an amorphous extracellular hyaline material that exhibits "apple-green" birefringence with Congo red stains. Amyloid is not a chemically distinct entity, and currently available molecular methods are capable of identifying over 20 amyloidogenic precursor proteins. Some of the more common diseases associated with amyloidosis include plasma cell dyscrasias...

In March 1999, a 49-year-old woman presented with acute right hip pain and no history of trauma. She had been diagnosed with rapidly progressive glomerulonephritis in 1966 leading to complete renal failure in 1973. Treatment included multiple failed attempts at transplantation and long-term hemodialysis. She was diagnosed with beta-2 microglobulin amyloidosis in 1986. She had a parathyroidectom...

Objectives FMF is the most common autoinflammatory disease characterized by recurrent febrile polyserositis. The gravest consequence of FMF is nephropatic amyloidosis of AA type, which may progress to affect other organs, including the lungs[]. Pulmonary hypertension (PH) in FMF related amyloidosis is rare; only a few cases have been reported so far[,]. We aim to elucidate development of PH in ...

OBJECTIVE To evaluate the long-term impact of liver transplantation on ocular manifestations of familial amyloid polyneuropathy (FAP) in Japanese patients. METHODS Medical records were retrospectively reviewed in a long-term follow-up study. Of 52 patients with FAP amyloidogenic transthyretin Val30Met, 22 patients underwent liver transplantation. We assessed ocular manifestations, including a...

A 69-year-old man presented with a few years’ history of nodules on the buttocks. Past history included chronic renal failure controlled with haemodialysis for 19 years and a decompression operation for carpal tunnel syndrome. Physical examination revealed a red-brown pediculated nodule 50×20 mm in diameter on the sacral region (Fig. 1A). In addition, 120×80 mm elastic, hard subcutaneous masses...

Central sulcus hemorrhage is a rare imaging finding that can be related to cerebral amyloidosis in a normotensive non-traumatic elderly patient and present as an isolated finding or in association with other areas of involvement. We report a case presenting with an isolated central sulcus hemorrhage on computed tomography. Further imaging work-up excluded other potential causes of peripheral he...