amyloidosis type 8 (AMYL8); systemic non-neuropathic amyloidosis; Ostertag-type amyloidosis

Pathology

• generalized amyloidosis due to deposition of:
  • apolipoprotein A1
  • fibrinogen
  • lysozyme amyloids
• viscera are particularly affected
• no involvement of the nervous system
• renal amyloidosis

Genetics

• associated with defects in lysozyme-C (LYZ)
• associated with defects in APOA1

Clinical manifestations

• nephrotic syndrome
• arterial hypertension
• hepatosplenomegaly
• cholestasis
• petechial skin rash

More general terms

• amyloidosis

Database

• OMIM: https://mirror.omim.org/entry/105200