Porphyria Educational Services
PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 1 No.
25
June 20, 1999
Focus: Porphyria Terminology
When begins reading medical journals and documents on
the disease "porphyria" when encounters all kinds of
new words
and special terminology unqiue to this disease which in itslf is
very unique and very complex. Likewise the terminology
becomes
very complex.
porphyria: A group of inborn
disorders in which there is an abnormal
increase in biological
colorings or pigmentation.
erythropoietic porphyria: A type of
porphyria in which large amounts of
porphyrins are
manufactured i the bone marrow.
hepatic porphyria: A type of porphyria in
which large amoubnts of
porphyrins are
produced in the liver.
plumboporphyria : Porphyria from the Latin
"plumbum" meaning "lead".
triggers: The precipitating
factors in an acute attacks which cause the
symptoms to
exacerbate. Precipitating factors must be searched
for and if found,. at
once eliminated.
familial: A disease in some families
and not found in others.
hypokalemia: A condition in which too
little potassium is found in the
the bloodstream.
hyponatremia: A condition in which too
little sodium is found in the
the bloodstream.
ALA:
The abbreviation for delta-aminolevulinic acid.
ALAD: The abbreviation for
delta-aminolevulinic acide dehydratase
which is one type of
porphyria although considered relatively rare.
autosomal dominant: A pattewrn of
inheritance attributed to genes
located on chromosones
other than X and Y and sex chromosones.
The trait can show up
in successive generations of a family.
chromosone: THe circular DNA
molecule containing the entire set of
genetic instructions
essential for life of the cells.
DNA:
The abbreviationfor deoxyribonucleic acid. a thread-like
molecule that is
genetic material and is located in the chromosones
within an organism's
cells.
carbohydrates: A large group of sugars,
starches and the main source for
energy for all body
functions. Lack of carbohydrates can result in fatigue
and depression and
also can trigger porphyria acute attacks.
BMR:
Abbreviation for basic metabolism rate.
Erthrohepatic porphyria: ANother name for
the EPP type of porphyria.
familial PCT: An inherited type of
PCT as opposed to acquired PCT.
fecal:
Pertaining to the feces which is mostly solid waste from the
digestive tract
system.
hepatic:
Having to do with the liver.
hypertension: High blood
pressure
hypotension: Low blood
pressure.
intermittent:
To alternate between periods of attacks and periods of
remission.
intervention therapy: An act to prevent
harm to a patient and to improve the
mental,emotional and
physical functions of the patient.
metabolism: The sum
total of all of the biochemical reactions in the
cells and systems of
the human boday.
lymphocytes: One of two types of small white
bloods cells.
CBC:
Complete blood count.
RBC:
Red blood cells
WBC:
White blood cells
hgm:
Hemoglobin
Ig:
Immunoglobin
bulbar paralysis: A condition
characterized by the parlysis of the
lips, tongue, throat,
mouth nad vocal cords.
PN:
Abbreviation for peripheral neuropathy.
dioxin: An
ingredient in herbicides linked to a tyope of acne [chloracne]
and with PCT [porphyria cutaneous tarda]. Dioxin was also
an
ingredient in Agent Orange and has also been linked to many
cancers.
DRG:
Abbreviation for "diagnosis related group"
cogenital: Meaning present at birth.
IV:
Meaning intravenous or going through the blood veins.
marker:
A region of a chromosone that can be identified and
followed as inherited.
pallative:
Treatment given only to lessen symptoms rather than to
cure them.
Intervention therapy is a form of pallative care.
PDR:
Physicians Desk Reference.
PBG:
Porphobilinogen.
PCT:
The abbreviation for Porphyria CUtaneous Tarda, a skin porphyria.