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.