The acute porphyrias include AIP ("Acute Intermittent Porphyria"), HC ("Hereidatary Coproporphyria"), "Variegated Porphyria" (VP), and plumboporphyria. CLINICAL: Attacks of abdominal pain, limb weakness, and neuropsychiatric changes. Porphyria produces increase skin photosensitivity. Attacks are variable, but usually end with complete remission of Sx. Acute attacks can be precipitated by hormonal change and menstruation. Because of the association with female hormones, there is a 4:1 female predilection. Many attacks are produced by medications and other drugs, especially those that induce liver enzymes (e.g. BCP's, barbiturates, cigarette smoking, and ETOH). There are published lists of all of the "safe" and "precipitating" drugs. SYMPTOMS: Abdminal pain is almost universal (~ 100%), vomiting, constipation are very frequent (>75%). There is a neuropathy in 2/3 of pts, usually limb and girdle with UE and proximal mm affected most. Respiratory compromise is a common, and significant complication. Acute seizures and psychotic personality changes can occur. Metabolic changes include low Na, as a result of SIADH. Cardiovascular changes include tachycardia and hypertension in up to 70% of cases. URINE: Black urine (after standing/ exposure to light) from porphobilinogen (PBG) polymerizing into uroporphyrin and the brown-red pigment porphobilin. Analysis of urine for PBG is critical in making the diagnosis
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Disease Topic 331 
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