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Porphyria Educational Services


PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 1 No. 26                                             June 27, 1999
Focus:  Porphyria Factoids

During acute attacks, the urinary excretion of PBG ranges from
20 to 200 mg daily.  Normally less than 2 is excreted in 24 hours.

Pyrroloporphyria is another name for AIP.

Swedish porphyria is also another name for AIP.

AIP is a dominant inherited disorder.

Correction of gross electolyte disturbances may control seizures in most
patients during acute attacks.

Drs. Dean and Barnes undetook the most extensive investigation of
VP in a study carried out in South Africa.

VP prevalence runs 1.3 per 100,000 in FInland however runs
1:400 in South Africa.

Another named for VP is "mixed porphyria".

In VP laboratory examination of both urine and stool is essential
confirm a diagnosis.

HCP is characterized by excessive fecal excretion of coproporphyrin II.
Protoporphyrin excretion remains normal or is only slightly increased.

HCP resembles AIP and VP and is an autosomal dominant trait.

Managment of HCP is the same as for AIP.

The primary genetic lesion in HCP is deficient activity of coporporhyrinogen
oxidase.

In the acute porphyrias the observation of tachycardia and the pulse rate
may be a good index for judging the activity of the disease.

Inappropriate sweating and bladder distention are thought to be
manifestations of autonomic neuropathy in porphyria.

Motor symptoms usually predominate in acute attacks however porphyric
neuropathy which is a polyneuropathy can be present.

Recurrent acute attacks lasting several days to several months may be
interspersed with long and variable asymptomatic or latent periods.
Some people may have as few as three attacks in a lifetime or as many as two
or three major attacks oper year, or become chronic with smoldering
symptomology.

The most common single symptom of porphyria attacks is that of abdominal
pain.  Such pain can be moderate to severe in degree and cramopy or colicky
in nature.

Many of the mental symptoms of porphyria acute attacks may result from
profound hyponatremia.  [Hyponatremia being a less than normal amount
of sodium in the blood.]

Porphyria patients usually require hospital care when an acute attack
occurs, because the clinical course of an acute attack is difficult to
predict.  Pain should always be treated with morphine.

If improvement is not observed within 24 hours with glucose therapy,
theraphy with intravenous hematin [ferric heme] should be initiated
except in patients with coagulation theraphy and those with a history
of blood clots.

VP is unaffected by treatment with chloroquine or repeated phlebotomies.

Protection against sun exposure is the only practical way to deal with
photosensitivity in any of at the acute porphyrias.

*Reference: Wintrobe's Clinical Hematology 9th Edition Volume 1 1993.