Acute Intermittent Porphyria Flashcards
1
Q
What is porphyria?
A
Genetic disorder caused by error in haem biosynthesis causing toxic accumulation of porphyrin precursors
2
Q
What is porphyrin?
A
End product of haemoglobin production
3
Q
What is the inheritance pattern of acute intermittent porphyria?
A
Autosomal dominant
4
Q
What are features of an acute attack?
A
Colic Vomiting Fever Abdominal and back pain Raised WCC
5
Q
What drugs should you avoid in porphyria?
A
Alchohol
Barbituates
Tetracyclines
Contraceptive pill
6
Q
How do you manage an acute attack?
A
Remove causes
IV fluids
High carb diet
IV haematin
