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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.