Molekulargenetische Diagnostik
Praxis Dr. Mato Nagel

Membranoproliferative Glomerulonephritis (MPGN)

Die MPGN ist eine seltene Form der Glomerulonephritis mit einem typischen histologischen Bild. Diese rein histologische Einteilung wird nun mehr oder weniger verlassen zugunsten der Einteilung C3-Glomerulopathie, bei welcher neben histomorphologischen Komponenten, wie der C3-Ablagerung und der elektronenmikroskopischen Lokalisation auch pathophysiologische Betrachtungen wie die abnormale Aktivierung des alternativen Complement-Weges eine Rolle spielen. Im Zusammenhang mit diesen pathophysiologischen Betrachtungen des Complementsystems spielt auch die genetische Diagnostik dieser Complementstörungen eine zunehmende Rolle.

Einteilung

Membranoproliferative glomerulonephritis (MPGN) I

Diskrete Immunkomplex-Ablagerungen im Mesangium und subepithelial. Sekundäre Aktivierung der klassischen Complement-Kasskade.

Membranoproliferative glomerulonephritis (MPGN) II

Kontinuierliche elektronendichte bandartige C3-reiche Ablagerungen entlang der glomerulären Basalmembran, der tubulären Basalmembran und der Bowmanschen Kapsel als Ausdruck einer Überaktivierung des alternativen Complement-Weges.

Membranoproliferative glomerulonephritis (MPGN) III

Burkholder Variante: Zahlreiche subepitheliale und subendotheliale Ablagerungen.
Strife und Anders Variante: Vornehmlich intramembranöse Ablagerungen, die zu einer Unterbrechung der glomerulären Basalmembran führen.

Gliederung

Glomerulonephritis
C3 Glomerulopathie
CFHR5 Nephropathie
Goodpasture-Syndrom
Lupus erythematodes Nephritis
Membranoproliferative Glomerulonephritis (MPGN)
ADAMTS13
C1QA
C1QB
C1QC
C3
CD46
CFB
CFD
CFH
CFHR1
CFHR2
CFHR3
CFHR4
CFHR5
CFI
CLU
CR1-Mangel
CR1
DGKE
Komplement C1q-Mangel
C1QA
C1QB
C1QC
Komplement C1s-Mangel
C1S
PIGA
THBD
Membranöse Glomerulonephritis
Mesangioproliferative Glomerulonephritis

Referenzen:

1.

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2.

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3.

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4.

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5.

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6.

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7.

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8.

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9.

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10.

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