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jeudi 3 mai 2012

The Vampire Disease




Stories of Dracula and Vampires that rise after dark to stalk the innocent and drink their blood have been a part of popular folklore for centuries. What if this legend can be explained by medical science?

Porphyria, The Disease
Porphyria, sometimes called “the Vampire disease”, is a collection of rare, genetic blood disorders. 
The porphyrias are a classification of at least 8 different diseases which are caused by abnormalities in heme production. They manifest with either neurological complications ("acute") or skin problems ("cutaneous"), or occasionally both.
Heme is part of the hemoglobin in blood that has the ability to carry oxygen. It is a porphyrin molecule which contains iron and gives blood its red color. The common feature for all porphyria is an overproduction and accumulation of  porphyryns.
The worldwide prevalence has been estimated to be somewhere between 1 in 500 to 1 in 50,000 people.

Acute porphyrias symptoms: 
The acute, or hepatic, porphyrias primarily affect the nervous system, resulting in abdominal pain, vomiting, acute neuropathy, muscle weakness, seizures and mental disturbances, including hallucinations, depression, anxiety, and paranoia.
Hematin and  heme arginate are the drugs of choice for acute porphyra but they are not curative drugs.

Cutaneous porphyrias symptoms: 
The cutaneous, or erythropoietic, porphyrias primarily affect the skin, causing photosensitivity (photodermatitis), blisters, necrosis of the skin and gums, itching, and swelling, and increased hair growth on areas such as the forehead. Often there is no abdominal pain, distinguishing it from other porphyrias.
Blood transfusion is occasionally used to suppress innate heme production. There are no drugs to cure this form of the disease.

What started the legend?
Biochemist David Dolphin in 1985 proposed some theories by which vampires will be persons who were suffering of Porphyria. Here are his arguments:
  1. Sensitive to sunlight – When a porphyria sufferer (only on the 8 different diseases, 4 can induce sentivity to sunlight) is exposed to the rays of the sun, his skin and face get disfigured and blackened. 
  2. Fangs – The fangs that vampires have, are actually the appearance that a porphyria patient gets when connective tissues, including gums, are necrotic. This results into the exposed fang-like teeth that are quite gruesome and frightening in appearance.
  3. Garlic to ward-off vampires – It may come as a surprise but even this has basis. Porphyria patients have aversion to garlic since they are allergic to the chemicals contained in it (allicin).

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