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Porphyria Educational Services



PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 2 No. 11                                           March 12, 2000
FOCUS:  Variegate porphyria: past, present and future.


Variegate porphyria, one of the acute hepatic porphyrias.  It is
usually refered to as VP.

VP  is characterized by a partial reduction in protoporphyrinogen oxidase.
Protoporphyrin oxidase is the seventh enzyme of the heme biosynthetic
pathway.

VP in most aspects resembles AIP [acute intermittent porphyria} and in
addiiton has the cutaneous symptoms.

For a long time, this disease has caused confusion among the porphyrias
because it presents with clinical symptoms and biochemical findings that
can be similar to those found in other types of porphyriasm such as the AIP.


Frank J, Christiano of Columbia University's Department of Dermatology,
has been a leader in reviewing the biochemical, clinical, historial and
genetical aspects of VP.


Paul Roberts PA, Guest Columnist