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Porphyria Educational Services

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

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

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.