Hemocromatose Hereditária Flashcards

1
Q

Patogenia.

A

Mutado no gene hFE ↑ absorção de ferro

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2
Q

Epidemiologia.

A

Caucasiano 40-50 anos , comum 1:250

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3
Q

Doença dos 6H

A
Hepatomegalia 95%
Hepatocarcinoma 100x
Heart (Ic , arrtmias)
Hiperpigmentacao "dm bronzeado"
Hipogonadismo (atrofia testicular)
Hartrite (pseudo-gota)
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4
Q

Qual o agente que causa maior propensão a infecções ?

A

Vibrio vulnificus

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5
Q

Como diagnosticar?

Parâmetros da ferritina

A

Ferritina > 200 mcg/l e saturado de transferrina 50/60%

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6
Q

Como confirmar o diagnóstico?

A

C282Y e H63D

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7
Q

Em dúvida diagnóstica:

A

Biopsia hepática

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8
Q

Como fazer o tratamento?

A

Flebotomia (500 ml; seriada ; alvo ferritina < 50 mcg/l

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