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AApoAIV amyloidosis

MedGen UID:
Concept ID:
Synonyms: Apolipoprotein A-IV amyloidosis; apolipoprotein A-IV amyloidosis
Modes of inheritance:
Not genetically inherited
MedGen UID:
Concept ID:
Source: Orphanet
clinical entity without genetic inheritance.
Monarch Initiative: MONDO:0018589
Orphanet: ORPHA439232


A rare, systemic amyloidosis characterized by slowly progressive renal dysfunction, increased serum creatinine, mostly normal urine analysis with no significant proteinuria and associated heart disease. Cardiac involvement presents as hypertrophic obstructive cardiomyopathy, left ventricular outflow tract obstruction, coronary artery disease and conduction system abnormalities. Histology reveals renal tubular atrophy, interstitial fibrosis, glomerular sclerosis, and medullar amyloid deposits. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAApoAIV amyloidosis

Recent clinical studies


Dasari S, Amin MS, Kurtin PJ, Vrana JA, Theis JD, Grogg KL, Alexander MP, Nasr SH, Fervenza FC, Leung N, Sethi S
Kidney Int 2016 Sep;90(3):658-64. Epub 2016 Jun 2 doi: 10.1016/j.kint.2016.04.003. PMID: 27262366

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