Porphyria Educational Services
PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol 1 No. 43
October 17, 1999
FOCUS: Management of the Acute Porphyrias: AIP, VP &
HCP
Three hepatic porphyrias--acute intermittent porphyria,
hereditary
coproporphyria and variegate porphyria--are characterized by
episodic acute
attacks that consist of such as abdominal pain,nausea,
vomiting,
constipation, hypertension, massive sweating, bulbar paralysis,
respiratory
difficulty, tachycardia, and various neuro-psychiatric
symptoms along with
associated with increased excretion of porphyrins and porphyrin
precursors. Sound
familiar? We all know more symptoms as well, but will not
elaborate here.
Peripheral neuropathy is manifested as pain in the extremities,
and it may
progress to a severe motor neuropathy. Often porphyrics
experience a
loss of gait similar to that of MS patients. Another exacerbation
is that of a foot drop.
Measurement of porphobilinogen [PBG] in the urine gives a prompt
diagnosis during acute attacks. According to Dr.R.
Kauppinen of the
University Hospital of Helsinki, Finland, "attacks are often
induced by
precipitating factors such as drugs, alcohol, infection, fasting
or changes
in sex-hormone balance,fungicides, herbicides, insecticides,
fungicides,
pesticides,some paint chemicals, resins, other known toxic
chemicals,
and they all should be eliminated when a patient is treated
during an attack."
Intervention therapy for restoration of porphyrin biosynthesis to
normal
levels is necessary.It is usually infused. [300-400 gm per
24 hours]
Adequate calories are necessary and parenteral nutrition with
carbohydrates
may be necessary. Glucose intravenous feeding is the best and
most often
used immeduiate intervention. It is also becomoing more and
more a
preventative treatment for eliminating acute attacks today.
In addition attacks may also require therapy for hypertension,
pain and epileptic seizures. Propranolol has been used
effectively for
onset of acute attacks. Compazine is a leading medication
for the
treating of nausea and vomiting,. Neurontin [Gabapentin} is
emerging
as the formost medication for the control seizure activity, if
such activity
is not covered by the propranolol.
In addition to the use of glucose iv therapy for prevention, the
strict avoidance of all precipitating factors may not be
necessary in the
asymptomatic phase.
For more on the chemical toxins and other triggering factors of
the
acute porphyrias, read Dr. Kauppinen's article, "Management
of the
Porphyrias" which can be found in the 1998 Apr;14(2):48-51
issue of Photodermatology Photoimmunology and Photomedicine.