amyloid polyneuropathy-nephropathy Iowa type; amyloidosis van Allen type; familial amyloid polyneuropathy type III (AMYLIOWA)

Pathology

• hereditary generalized amyloidosis due to deposition of amyloid mainly constituted by apolipoprotein A1

Genetics

• result of a mutation in APOA1

Clinical manifestations

• neuropathy in the early stages of the disease
• nephropathy late in the course
• severe peptic ulcer disease can occur
• hearing loss is frequent
• cataracts may be present
• vitreous opacities are not observed

Complications

• death is due in most cases to renal amyloidosis

More general terms

• amyloidosis