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Porphyria Types
Hereditary Coproporphyria (HCP)
This is an autosomal dominant form of hepatic porphyria that is
similar to AIP, except that some patients develop skin photosensitivity.
The deficient enzyme is coproporphyrinogen oxidase. The diagnosis
is established by finding excess coproporphyrin (especially coproporphyrin
type III) in urine and stool. Other types of porphyrins show little
or no increase. Urinary ALA and PBG are increased during acute
attacks but may become normal on recovery. Reliable assays for
the deficient enzyme are not generally available. If the enzyme
is measured, red blood cells should not be used. The enzyme is
found in mitochondria which are not present in red blood cells.
Precautions and treatment for acute attacks are as described for
AIP.
For more information please see AIP,
HCP, VP, & ADP.
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