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Endocrine - DVR > 178 endo polyautoimmune syndromes > Flashcards

178 endo polyautoimmune syndromes Flashcards

1
Q

autoimmune polyglandular syndromes - what should be given first?

A

cortisol

then thyroid hormones

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2
Q

APS1

A

AR; autoimmune regulator AIRE gene -> regulates T cell self tolerance during thymic development via expressing tissue-specific antigens

present < 5 years old

chronic candidiiasis –> mucous
chronic hypoPT
autoimmune Adrenal insufficiency

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3
Q

APS2

A

multigenetic

autoimmune adrenal insufficiency
Autoimmune thyroid disease +/- DM1
Stiff man sydnrome - rigidity and alopecia

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4
Q

APS3

A

thyroid autoimmune disease + others (DM1, pernicious anemia, vilitigo) but not AI, hypoPT, candidiasis

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5
Q

what causes APS?

A

autoreactive t cells

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6
Q

IPEX rx

A

bone marrow transplant

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Endocrine - DVR (22 decks)

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