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Porphyria – A modernized Vampire Disease?

Posted Jul 13 2012 10:28am

Do you know how many teenie bopper sites I had to go through to find this image? The answer is too many. But it was well worth it, because I am *sooooo* Team Edward (twirls hair, giggles).

But no, seriously, I am.
My mom is Team Jacob.
We’ve had some heated discussions about it.
And have watched the movies.
Don’t judge.

On a more serious note, there actually is a condition that is attributed to what is nicknamed, “the Modernized Vampire Disease,” generally known as porphyria. The term “porphyria” comes from the Greek word porphura, meaning “purple.” The general pathology of this medical condition includes a disordered heme synthesis and breakdown pathway, resulting in clinical symptoms that manifest (mostly associated with the skin, but can also affect the liver and the nervous system as well).

Generally, there are 3 different categories of porphyria, which include Neuro-Psychological
(1) Acute Intermittent
(2) Plumbo

Mixed
(1) Copro variegate

Cutaneous
(1) Cutanea-tarda
(2) Congenital Erythropoietic
(3) Erythropoietic

The diagnosis most associated with what we can attribute to a “vampire” is porphyria cutanea tarda, the type of porphyria that mainly affects skin and results in extreme photosensitivity, often resulting in rashes when exposed to sunlight (directly leading to sleeping in a coffin, obviously). The whole drinking-blood thing is not associated with this real medical condition, although may be filed under the general term of hematophagy (act of an animal drinking another animal’s blood).

Heme, within our red blood cells, is synthesized from succinyl CoA and glycine within the body, and uses Vitamin B12 as a coenzyme. It is then combined with globin chains, to make the hemoglobin molecule, also known generally as a porphyrin ring. It is a complex biochemical pathway, which will not be discussed here, with many enzymes involved. If there is any type of deficiency or malfunction with an enzyme (due to congenital reasons or from taking specific medications), intermediate byproducts may build up, and deposit in body tissues, such as the skin, liver, or nervous system, leading to the diagnosis of porphyria, in general. Toxicity may be due to iron overload in the tissues, similar to that of hemochromatosis.

Historically speaking, King George III of England had this congenital disease, resulting in red urine, psychotic symptoms, and skin rashes. Besides these symptoms, other common signs can include abdominal pain, muscle weakness, and other neurologic defects.

The main diagnostic criteria of any type of porphyria includes urine studies, looking at aminolevulinic acid (ALA) and porphobilinogen (PBG), which are heme synthesis intermediates that build up due to enzyme deficiencies in the pathways, and leak into the urine.

Actually, this might be more like my kind of “Team Edward” anyway, since one of my favorite movies is Edward Scissorhands , rather than Twilight

References > http://www.nytimes.com/1985/05/31/us/rare-disease-proposed-as-cause-for-vampires.html
> http://www.mdconsult.com/books/page.do?eid=4-u1.0-B978-1-4377-0755-7..00085-3&isbn=978-1-4377-0755-7&uniqId=345170843-5#4-u1.0-B978-1-4377-0755-7..00085-3
> http://www.mdconsult.com/books/page.do?eid=4-u1.0-B0-443-07300-7..50212-9–cesec6&isbn=0-443-07300-7&sid=1331715423&uniqId=345170843-6#4-u1.0-B0-443-07300-7..50212-9–cesec6

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