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Porphiria- a medical view on vampires

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Nina:

--- Quote ---acute intermittent porphyria  (AIP) hereditary hepatic porphyria due to a defect of pyrrole metabolism, with recurrent attacks of abdominal pain, gastrointestinal and neurologic disturbances, and excessive amounts of d-aminolevulinic acid and porphobilinogen in the urine.

congenital erythropoietic porphyria  (CEP) a form of erythropoietic porphyria, with cutaneous photosensitivity leading to mutilating lesions, hemolytic anemia, splenomegaly, excessive urinary excretion of uroporphyrin and coproporphyrin, and invariably erythrodontia and hypertrichosis.

porphyria cutaŽnea tarŽda  (PCT) a form characterized by cutaneous sensitivity that causes scarring bullae, hyperpigmentation, facial hypertrichosis, and sometimes sclerodermatous thickenings and alopecia; it is associated with reduced activity of an enzyme of heme synthesis.

erythropoietic porphyria  that in which excessive formation of porphyrin or its precursors occurs in bone marrow normoblasts, including congenital erythropoietic porphyria and erythropoietic protoporphyria.

hepatic porphyria  that in which the excess formation of porphyrin or its precursors occurs in the liver.

hepatoerythropoietic porphyria  (HEP) a severe form of porphyria cutanea tarda believed to result from a lack of activity of the enzyme catalyzing the conversion of uroporphyrinogen to coproporphyrinogen in the biosynthesis of heme.

variegate porphyria  (VP) a hereditary hepatic porphyria, with chronic skin manifestations, chiefly extreme mechanical fragility of the skin, mainly of areas exposed to sunlight, episodes of abdominal pain, neuropathy, and typically an excess of coproporphyrin and protoporphyrin in bile and feces.

http://medical-dictionary.thefreedictionary.com/Porphiria

--- End quote ---



The porphyrias are inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). They are broadly classified as hepatic porphyrias or erythropoietic porphyrias, based on the site of the overproduction and mainly accumulation of the porphyrins (or their chemical precursors). They manifest with either skin problems or with neurological complications (or occasionally both).

The term derives from the Greek πορΦυρα (porfura), meaning "purple". The name is likely to have been a reference to the purple discoloration of some body fluids in patients during an attack.

Signs and symptoms

Acute porphyria

The hepatic porphyrias primarily affect the nervous system, resulting in abdominal pain, vomiting, acute neuropathy, seizures, and mental disturbances, including hallucinations, depression, anxiety, and paranoia. Cardiac arrhythmias and tachycardia (fast heart rate) may develop as the autonomic nervous system is affected. Pain can be severe and can, in some cases, be both acute and chronic in nature. Constipation is frequently present, as the nervous system of the gut is affected, but diarrhea can also occur.

Given the many presentations and the relatively uncommon occurrence of porphyria the patient may initially be suspected to have other, unrelated conditions. For instance, the polyneuropathy of acute porphyria may be mistaken for Guillain-Barré syndrome, and porphyria testing is commonly recommended in those scenarios. Lupus erythematosus features photosensitivity, pain attacks and shares various other symptoms with porphyria.

Not all porphyrias are genetic, and patients with liver disease who develop porphyria as a result of liver dysfunction may exhibit signs of their conditions, such as jaundice.

Attacks of the disease can be triggered by drugs (e.g., barbiturates, alcohol, sulfa drugs, oral contraceptives, sedatives, and certain antibiotics), other chemicals and certain foods. Fasting can also trigger attacks.

Patients with hepatic porphyrias (PCT, AIP, HCP, VP) are at increased risk over their life for hepatocellular carcinoma (primary liver cancer) and may require monitoring. Other typical risk factors for liver cancer need not be present, such as hepatitis B or C, iron overload or alcoholic cirrhosis.

Cutaneous porphyria

The 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.

In some forms of porphyria, accumulated heme precursors excreted in the urine may change its color, after exposure to sunlight, to a dark reddish or dark brown color. Even a purple hue or pink urine may be seen. Heme precursors may also accumulate in the teeth and fingernails, giving them a reddish appearance.

Porphyrin studies

Porphyria is diagnosed through spectroscopy and biochemical analysis of blood, urine, and stool.[6] In general, urine estimation of porphobilinogen (PBG) is the first step if acute porphyria is suspected. As a result of feedback, the decreased production of heme leads to increased production of precursors, PBG being one of the first substances in the porphyrin synthesis pathway.
 In nearly all cases of acute porphyria syndromes, urinary PBG is markedly elevated except for the very rare ALA dehydratase deficiency or in patients with symptoms due to lead poisoning or hereditary tyrosinemia type I.

Repeat testing during an attack and subsequent attacks may be necessary in order to detect a porphyria, as levels may be normal or near-normal between attacks. The urine screening test has been known to fail in the initial stages of a severe life threatening attack of acute intermittent porphyria.

The bulk (up to 90%) of the genetic carriers of the more common, dominantly inherited acute hepatic porphyrias (acute intermittent porphyria, hereditary coproporphyria, variegate porphyria) have been noted in DNA tests to be latent for classic symptoms and may require DNA or enzyme testing. The exception to this may be latent postpuberty genetic carriers of hereditary coproporphyria.

As most porphyrias are rare conditions, general hospital labs typically do not have the expertise, technology or staff time to perform porphyria testing. In general, testing involves sending samples of blood, stool and urine to a reference laboratory.
 All samples to detect porphyrins must be handled properly. Samples should be taken during an acute attack, otherwise a false negative result may occur. Samples must be protected from light and either refrigerated or preserved.

Additional tests

Further diagnostic tests of affected organs may be required, such as nerve conduction studies for neuropathy or an ultrasound of the liver. Basic biochemical tests may assist in identifying liver disease, hepatocellular carcinoma, and other organ problems.

...

IN CULTURE AND HISTORY

Vampires and werewolves[/b]

Porphyria has been suggested as an explanation for the origin of vampire and werewolf legends, based upon a number of similarities between the condition and the folklore that was first speculated upon by biochemist David Dolphin in 1985. His ruminations gave rise to a popular urban legend which accepts this association as factual, though it is historically and factually baseless. Porphyria cutanea tarda presents clinically as a pathological sensitivity of skin exposed to light causing scarring, hair growth and disfiguration. Additionally, it was believed that the patients' missing heme could be absorbed through the stomach, correlating with the legends' hematophagy.[8]

Porphyrias have been detected in all races, multiple ethnic groups on every continent including Caucasians, Asians, Africans, Peruvian/Mexican Hispanics, Native Americans, Laplanders and Australian aborigines. There are high incidence reports of AIP in areas of India and Scandinavia and over 200 genetic variants of AIP, some of which are specific to families, although some strains have proven to be repeated mutations.


Historical patients

The insanity exhibited by King George III evidenced in the regency crisis of 1788 has inspired several attempts at retrospective diagnosis. The first, written in 1855, thirty-five years after his death, concluded he suffered from acute mania. M. Guttbacker, in 1941, suggested manic-depressive psychosis as a more likely diagnosis, The first suggestion that a physical illness was the cause of King George's mental derangements came in 1966, in a paper "The Insanity of King George III: A Classic Case of Porphyria", with a follow-up in 1968, "Porphyria in the Royal Houses of Stuart, Hanover and Prussia". The papers, by a mother/son psychiatrist team, were written as though the case for porphyria had been proven, but the response demonstrated that many, including those more intimately familiar with actual manifestations of porphyria, were unconvinced. The theory is treated in Purple Secret, which documents the ultimately unsuccessful search for genetic evidence of porphyria in the remains of royals suspected to suffer from it. Despite the lack of evidence, the notion that George III (and other members of the royal family) suffered from porphyria has achieved such popularity that many forget that it is merely a hypothesis.

Other commentators have suggested that Vincent van Gogh may have suffered from acute intermittent porphyria. Erik Vachon, the little-known builder whose character was combined with that of Svengali to create The Phantom of the Opera may have suffered from erythropoietic porphyria.

It has also been imagined that King Nebuchadnezzar of Babylon suffered from some form of porphyria (cf. Daniel 4). The symptoms of the various porphyrias are so wide-ranging that nearly any constellation of symptoms can be attributed to one or more of them.

The poet Robert Browning, also, notoriously wrote a poem called Porphyria's Lover, which aside from a literal interpretation of the word also compares love itself to a form of disorder.

http://encyclopedia.thefreedictionary.com/Porphiria


So, girls and boys, what do you say to this?

phyrrestar:
I say I already talked about this before.  :wink:

To discuss here, though, it sounds like it explains quite a bit about old vampires.  I definitely see this as an explaination of where a bunch of the folklore got started.  Granted, it doesn't explain the "non-classic" vampires, those from around China I think, but it's a wonderful look into where we got our traditional view of vampires.

This naturally doesn't explain modern vampires as those do not suffer the same symptoms as the "classic" ones.  All of the symptoms of modern vampires are very arguably completely psychological, born through desire or trauma most likely (more commonly trauma I think).  If physical symptoms show, it's generally (from my experience anyway) because the person suffers from some other disease or skin condition.  I've yet to meet a modern vampire who had physical symptoms that could not in anyway be simply psychological that later did not admit to either be lying for attention or had some other scientific explaination that had already been diagnosed.

Night Energy:
Then this can be said: the disease affect the skin or the internal system. I think is better to had the skin affected because its more painful to affect you inside.

Muerte:
  I would tend to agree, but I must ask what ever caused you to revive a topic which has laid dormant for over 3 years?  Would it not make more since to restart a new topic about the same subject?  In the future please try doing this instead of ignoring the rule about reviving dead topics.

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