Porphyria Educational Services

PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 1 No. 40                                    September 20, 1999
FOCUS:  Incidence of Acute Hepatic PorphyriaSymptomology

 Incidence of of Symptoms and Signs of Acute Porphyrias has been relatively
 steady over the years since the first major studies were undertaken and
 later published.

In the  basic medical testbook entitled: "The Metabolic Basis of Inherited
Disease" in  Chapter 66 which is labeled "The Porphyrias" Dr. A. KappasM.D.
et.al. are the authors of the articles presented in the 7th Edition, Vol II published in 1995.

The reason for the popularity of this  presentation of incidences is
because it compares the early studies and one can compare them with the 1997
study which show that there is not a change really, just more ability to
diagnose because of advanced technology...and yet we still find it very hard
to get adequate---uncompromised diagnosis  testing done today  with all the
latest scientific breakthroughs.

The Symptomsand Signs of the acute porphyrias below are compared  with
studuies done by  Waldenstrom in 1957;   Goldberg in 1959; a dual study by
Stein and Tschudy in 1970  and a study completed in 1997 of known accute
porphyria patients.

Abdominal pain  ranked     85% ,   94%, 95%  and 96% respectively.
Vomiting and Nausea   ranked  59% ,  88%, 43 %    and  84%.
Constipation  was found to be  48% ,  84%, 48%    and   52%.
Diarrhea  ranked  9 % , 12 %, 5 %   and  7%.
Limb, head, neck, or chest pain  ranked  52%, 50% , 54% and 57 %.
Muscle  weakness  42%,   68%, 60% and   67%.
Sensory loss  ranked 9 %, 38%, 26%  and  21%.
Convulsions  10%, 16%, 20% and  18% respectively.
Respiratory paralysis  came in 14%, 10%,  9 %   and  12%.
Mental symptoms   ranked 55% ,  58%, 40%   and 54%.
Hypertension ranked  40% , 54%, 36% and  33%.
Tachycardia  was ranked 28%,  64%, 80% and  67%.
Fever and also Profused sweating both ranked 37% ,14%,  9 % and  8%.

Additionally, the 1997 study goes  on to include chronic paresthesia,
numbness,  abnormal urinary bladder function, decrease or loss of tendon
reflexes, tremor and seizures, abdominal distention, anxiety, insomnia,
restlessness, and depression. All of these symptomology show significant
presence in the  case histories studied.