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Porphyria Educational Services


PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 1 No. 24                                       June 13, 1999
FOCUS: Symptomology of the Acute Porphyrias


        Symptomology of patients exacerbating during acute attacks
of porphyria can be quite varied from one patient to the next patient.
Such symptomology will also vary from one exacerbation to the next.

        While most porphyrics with a diagnosis of a acute form remain
latent most of their life,  about 60% will experience some forms of
acute episode in their lifetime.

        Women will on the average have a greater preponderance of acute
attacks  than will men.  Most of this is due to the hormonal differences
among the sexes.  However studies show that only sven percent of acute
attacks are directly caused by the onset of menses, and only six percent are
due to pregnancy.

        The age of onset for the majority of porphyrics with acute types
runs between ages 20 and 40 with the next onset beginning between ages 10-19
years of age.

        In a study published in 1996 71 percent of porphyrics with acute
types have a positive family history of porphyria.

        Looking at the precipitating factors of an acute attack,
pharmaceuticals still remains as the number one reason cause for triggering
acute attacks. About 54 percent of all acute attacks are triggered by use of drugs.

        While alot is said about not dieting or fasting because of bringing
on an acute attack, the same study published in 1996 shows that only three
percent of acute ayttacks are precipitated by fasting.  This runs the same
as those attacks caused by alcohol.

        Having other illness is responsible for triggering about nine
percent of acute attacks.  Such illness  can be something minor as a urinary tract
infection, strep or bronchial infection.  Almost 20 percent of acute attacks
go undetermined as to what caused them to be triggered.

        The general symptomology of an acute attack does not follow any
one set pattern.  However there is a large number of signs and symptoms that
are a part of the majority of acute attacks.  In 90 % of all acute attack
there is severe abdominal pain present.  Anout 87% of the porphyria patients have an
abdominal tenderness.    In addition about 66 % demonstrate other pain of
the limbs, joints and back.

        In the 1996 published study it indicated that 72 of all porphyria
patients will experience red urine.

        While 56 percent of porphyrics experience extreme constipation
during acute attacks, only nine percent have diarrhea as a part of their
acute attack.

        A big factor is that of nausea and vomiting in acute attacks which
is experienced by about 61 percent of the patients. This can lead to other
symptomology due to the loss of electrolytes which in turn cause mental
changes  during the attack, such as behavorial change [56 %], irritability
or anxiety by 40 percent, hallucinations or and confusion by 30 percent.
Seizures will occur in about 12 percent of the acute attacks. Often there is
cranial nerve involvement which has been found in 28 percent of the
acute attacks.

        In lab work obtained at the onset of an acute attack an iron
deficiency will be noted in roughly ten percent of the patients. 
Leukocytosis is found in roughly 15% of porphyrics.

        Looking at actual physicial finding during an acute attack  a fast
pulse otherwise noted as tachycardia, is observed in 50% of all porphyrics.
Labile hypotension [low blood pressure less than 90 diastolic] is found in
about 44 percent.  The use of propranolol for covering exacerbation of
symptoms during the acute attack will also add to the possibility of labile
hypotension.

       Profused sweating is found in 12 percent of acute attacks and eight
percent will experience pigmentation changes in their skin.  Some 31 percent
will run a fever during onset of an attack.

        Distended bowels and decreased motility are other factors often
noted by about half of all porphyrics.   Elevated liver panels may also be
found.

        While most of the statistics in this bulletin reflect those of the
published porphyria study published in teh 1996 Scientific American,
other studies have been completed over the years with similar results.

        One of the earliest studies on porphyria was undertaken by the
Swedish physician Waldenstrom in the year 1957.   Waldenstron cited that 14
percent of porphyria patients in an acute attack will experience respiratory
paralysis. Respiratory paralysis is the number leading cause of death
for porphyrics during an acute attack.

        In another study of symptomology undertaken by Dr. Goldberg in 1959,
just two years later, he found that 68 percent of porphyria patients
experience extreme muscle weakness.  Drs. Stein and Tschudy noted in another
study in 1970 that muscle weakness quite often  leading to perifpheral
neuropathy [PN] affects about 60 percent of patients during an acute attack.

        In the study by Stein and Tschudy they report that mental symptoms
are observed in about 40 percent of porphyria patients during acute attacks.
The mental symptoms include anxiety, restlessness, insomnia, paranoia, and
depression with such subjects last from a few hours to a few days mostly.