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Immunologie > Angeborene Immundefekte > Schwere Kombinierte B/T-Zell-Defekte (SCID)
T+ B+ SCID
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| Immunologie > Angeborene Immundefekte > Schwere Kombinierte B/T-Zell-Defekte (SCID) |
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T+ B+ SCID
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| Einleitung | ||
Synonym |
ZAP70-Mangel |
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Bare lymphocyte syndrome Typ 2 |
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IL-2-Mangel |
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Englisch |
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ICD10 |
D81.8: ZAP70-Mangel, IL2-Mangel |
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D81.7: Bare lymphozyte syndrome Type 2 |
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Definition |
T+B+ SCID |
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Klassifikationen |
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| Epidemiologie | ||
Inzidenz |
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Prävalenz |
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Alter |
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Häufigkeitsgipfel |
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Geschlecht |
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Ethnologie |
Bare lymphocyte syndrom Type 2: Nordafrika |
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| Pathologie | ||
Ätiologie |
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Risikofaktoren |
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Vererbung |
AR |
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Pathogenese |
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Molekularer Hintergrund |
ZAP70-Mangel: Mutation für die Tyrosinkinase ZAP70 Bare lymphocyte syndrome Type 2: MHCII-Expression vermindert IL2-Mangel |
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Makroskopie |
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Mikroskopie |
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| Diagnostik und Workup | ||
Kriterien |
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Diagnostik |
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Körperliche Untersuchung |
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Bildgebung |
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Blut |
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Weitere Diagnostik |
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Nachsorge |
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Meldepflicht |
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Pränataldiagnostik |
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| Symptome und Befunde | ||
ZAP70-Mangel |
Lymphozytose, CD8+ vermindert |
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| Verlauf und Prognose | ||
Stadien |
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Verlauf |
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Komplikationen |
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Prognose |
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Prophylaxe |
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| Differentialdiagnosen | ||
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| Therapien | ||
IL2-Mangel |
IL2-Substitution |
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| Referenzen | ||
Lehrbuch |
Herold 2003, S.56 |
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Reviews |
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Studien |
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Adressen |
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| Editorial | ||
Autor |
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Erstellt |
13.11.2002 |
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Reviewer |
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Linker |
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Status |
TRACK3 |
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Licence |
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