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Zentrum für Nephrologie und Stoffwechsel
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Hereditäre Amyloidose

Die Gruppe der hereditären Amyloidosen fasst Erkrankungen mit Amyloidablagerungen in verschiedenen Organen zusammen. Bei Ablagerungen in viszeralen Organen, wie der Niere können vitale Funktionen gefährdet sein.

Einteilung

Die Einteilung der Amyloidosen erfolgt in hereditäre und erworbene Formen. Letztere können inflammatorisch oder neoplastisch verursacht sein. Unterschieden werden können ferner lokale und systemische Formen. Die klassische Einteilung der Amyloidosen folgt den biochemischen Eigenschaften der Fibrillen. Da diese Webseite nur hereditäre Formen behandelt liegen der Einteilung die verursachenden Gene zugrunde.

Gliederung

Erbliche metabolische Nierenerkrankungen
Hereditäre Amyloidose
ATTR-Amyloidose
TTR
Amyloidose vom Finnischen Typ
GSN
Cryopyrin-assoziiertes periodisches Fieber-Syndrom
CINCA-Syndrom
NLRP3
Familiäres kälteinduziertes autoinflammatorisches Syndrom 1
NLRP3
Muckle-Wells-Syndrom
NLRP3
Familiäres Mittelmeerfieber
MEFV
NOD2
SAA1
TNFRSF1A
Nierenamyloidose
APOA1
Apolipoprotine A2-Amyloidose
APOA2
B2M
CST3
FGA
LYZ
Zerebroarterielle Amyloidose
Zerebroarterielle Amyloidose vom Britischen Typ
ITM2B
Zerebroarterielle Amyloidose vom Dänischen Typ
ITM2B
Zerebroarterielle Amyloidose vom Holländischen Typ
APP
Zerebroarterielle Amyloidose vom Isländischen Typ
CST3
Hyperoxalurie
Lysinurische Proteinintoleranz
Morbus Fabry

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Orphanet article

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Update: 14. August 2020
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