Write about Acute Porphyria here.

• Generally neuropsychiatric problems

• Associated with excess production and urinary excretion of �-ALA and porphobilinogen (not increased in non-acute porphyrias)

• Depressed or normal porphobilinogen deaminase

Acute Intermittent Porphyria

• Hepatic and acute

• Autosomal dominant

• Mean age 30 years

• F>M

• May be precipitated by EtOH or durgs

• Presents with:

• abdo pain, v and constipation (90%)

• polyneuropathy (M occasionally S) (70%)

• hypertension and tachycardia (70%)

• psychiatric disorders (depression, anxiety, frank psychosis) (50%)

• Urine + Ehrlich's aldehyde = pink

Supportive management, high carb intake (indirect effect on production).  Analgesia.  Remission Rx = avoidance of precipitants.

Variegate porphyria

Cutaneous porphyria - bullous eruptions on exposure to sunlight.

Hereditary Coproporhyria

Extremely rare, broadly similar to variegate porphyria (distinction based on biochem assay).