Acute intermittent porphyria

Question 1

What are some SSx of acute intermittent porphyria?

Answer 1

Severe abdominal pain
Neuropathic pain
Neuropsychiatric symptoms
Constipation or diarrhoea
Urine which turns red on exposure to sunlight/standing

Question 2

Complications of AIP?

Answer 2

Hepatocellular carcinoma
Chronic renal failure
Hypertension

Question 3

Drug triggers for AIP?

Answer 3

Antidepressants
Barbituates
Azoles
Alcohol
OCP
HRT
Anabolic steroids

Question 4

What enzyme is deficient in AIP?

Answer 4

Porphobilinogen deaminase

Question 5

What build up is there is acute intermittent porphyria?

Answer 5

Urinary Porphobilinogen
Delta aminolaevulinic acid

Question 6

What is the treatment for acute intermittent porphyria?

Answer 6

IV Haem arginase (Creates negative feedback preventing production of haem)

Question 7

What should be monitored in AIP?

Answer 7

Urinary porphobilinogen
Control HTN: monitor hepatic and renal fxn
Liver imaging (porphobilinogen can cause liver Ca)