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Familial renal amyloidosis | |
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This condition is inherited in an autosomal dominant manner | |
Classification and external resources | |
Specialty | endocrinology |
ICD-10 | E85.0 |
ICD-9-CM | 277.3 |
OMIM | 105200 |
DiseasesDB | 33335 |
eMedicine | med/3379 |
Familial renal amyloidosis (or familial visceral amyloidosis, or hereditary amyloid nephropathy) is a form of amyloidosis primarily presenting in the kidney.[1]
It is associated most commonly with congenital mutations in the fibrinogen alpha chain and classified as a dysfibrinogenemia (see Hereditary Fibrinogen Aα-Chain Amyloidosis).[2][3] and, less commonly, with congenital mutations in apolipoprotein A1[4] and lysozyme.[5][6]
It is also known as "Ostertag" type, after B. Ostertag, who characterized it in 1932 and 1950.[7][8]
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