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amyloidosis type 8 (AMYL8); systemic non-neuropathic amyloidosis; Ostertag-type amyloidosis
Pathology:
- generalized amyloidosis due to deposition of:
a) apolipoprotein A1
b) fibrinogen
c) 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
General
amyloidosis
Database Correlations
OMIM 105200
References
OMIM :accession 105200