Acute Intermittent Porphyria Flashcards

1
Q

AIP

A

autosomal dominant condition causing a defect in porphobilinogen deaminase

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2
Q

what does porphobilinogen deaminase do

A

helps with making haemoglobin

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3
Q

signs

A

abdo pain
vomiting
neuro features - seizures
psychiatric features

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4
Q

investigations

A

urine goes red in light

raised urinary porphobilinogen

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5
Q

management

A

remove triggers

IV haematin

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6
Q

what are some triggers

A

pill

trimetrophin

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