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Metaboliske levertilstande

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Debut af α 1<br />

-AT-mangel<br />

Neonatal debut:<br />

• Konjugeret hyperbilirubinæmi, svær kolestase<br />

• Kolestase forsvinder oftest inden 6 mdr., et mindre antal<br />

dog kronisk leversygdom (bl.a. paucity of bile ducts)<br />

Adult debut (prs. med mutation: % syge):<br />

• 41-50-årige: 5% (mand:kvinde 2:1)<br />

• 51-60-årige: 15% mænd, 0 % kvinder

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