Porphyria

Porphyria vampires

2007-07-14T21:35:00.000-07:00

An original reason of a werewolf and vampire myths was suggested by David Dolphin. He was a biochemist and for the first time published this theory in 1985. In this theory porphyria is suggested as an explanation for such myths. It sounds logically because there are many similarities in porphyria symptoms and the folklore descriptions of vampires. David Dolphin assumption gave a life to an urban legend, where assocation between vampires and porphyria is accepted as a fact.

Porphyria cutanea tarda is a disease, which lead to an hyper sensitivity of skin to a sunlight. Exposing areas of skin to light can cause an abnormal hair growth, scarring and disfigurements. As an additional prove of this theory spoke the fact, that the missing heme of a porphyria victims should be restored through the stomach. These thoughts correlate to the legends about vampires drinking human blood.

Porphyria

2007-07-11T23:02:00.003-07:00

A set of different disfunctions caused by irregularity in a chemical reaction of a heme production is called porphyria. Heme is very important for the health. It's concentrated in the blood and is responsible for oxygen transfer.

To produce heme human body need different enzymes. If some of then are abnormal the production of heme is broken and body synthesize not a heme, but some intermidiate composites, such as porphyrin.

There are two main types of a porphyria - it can affect either the skin or nervous system. If porphyria influence on skin - there will be itching and blisters and swelling. This happens if areas of a sking are exposed to light. The acute porphyria hit the nervous system. Among its symptoms are hallucinations, constipation, chest pain, tingling, paralysis etc.

Porphyria attacks can long for a long time: hours, days or even weeks. Among the main reasons of Porphyria are infections (ie hepatite), alcohol, stresses, drugs of a certain kind (oral contraceptives, sedative pills, tranquilisers), hormones.

The main methods of porphyria diagnosis are stool/urine/bloods thest. But it's rather difficult to certainly diagnose porphyria due to the fact that its symptoms are typical for a wide range of different diseases.

There are different methods of porphyria treatment. A most suitable is selected in according to the type of porphyria. Most commons are medicines (hematin) and giving heme. During attacks an opiates can be used to reduce the pain. Attacks are often accompanimented with severe pains, which leads to the hospitalization.

Porphyria king George

2007-07-11T22:07:00.001-07:00

The reason of the regency crisis of 1788 was in the King George III insanity. In our time there were performed several tries to diagnose the reason of this insanity. In 1966 some researches supposed that the roots of illness were hidden in a physical illness. These thoughts were descriped in a paper "The Insanity of King George III: A Classic Case of Porphyria". These paper wroten by a psychiatrist family (mother and son) are based on an assumption that the fact of porphyria in King George case was proven, but some facts were unconvinced. There were some additional researchs with goal to find a genetic evidence of a porphyria in the remains of King George, but they all failed to prove this. There are also a rather new theory (dated 2005) that the main reason of a porphyria was an arsenic given to King George. This poison is known as porphyrogenic, which can cause a porphyria.

In 1991 Alan Bennet presented his play "The Madness of George III", which became a base for 1994 movie with the same name. There is a remark in credits, where noticed that illness of King George has attributes of a porphyria.

There are also suspicions that some remarkable persons suffered from porphyria. Mary, Queen of Scots, Vincent van Gogh and King Nebuchadnezzar of Babylon are reffered among them.

Porphyria treatment

2007-07-11T21:33:00.001-07:00

Porphyria treatment (acute)
It often happens that empirical conduct is required. It's usually applied when there is a high suspicion of a porphyria. Acute assaults can be even fatal. Doctors widely advice a diet with a high content of a carbohydrate. Sometimes they use a 10% blend of a glucose.
In the United States and the United Kingdom the most popular drugs to treat porphyria are hematin and haem arginate. But these medicine must be taken in the very beggining of an attack to have a good effect. And the effect of these drugs can vary widely due to the individual person properties. Hematin and haem is not a panacea to treat porphyria, but can help greatly, because they reduce the length of attacks and its strength. Side-affects are rather serious, but happen very rarely. There medicines are heme-like, and in theory can be used a a toxical precursors. In the UK they can be bought only at 2 special centers, in the USA there is only one company producing Panhematin.

Hormone porphyria treatment
Cycle attacks in women can be contributed by hormonal fluctuations. In such cases attacks can be treated with luteinising hormones and oral contraceptives, because they influence on menstrual cycles. Though oral contraceptives sometimes itself can call a photosensitivity. Attacks can also can be triggered by the withdrawal of such contraceptives. Sometimes attacks are called by androgens and fertility.

Symptom control
During the attacks pain can be extremly severe. Sometimes it can be even fatal. In most cases an opiates should be used to reduce the pain. Pain should be reduced as soon as it's medically possible. Sometimes attacks are also cause a nausea. It can be temporarily reduced with the help of a hot shower.

Porphyria symptoms

2007-07-11T21:07:00.001-07:00

Prophyria mainly influence on the nervous system. Porphyria symptoms are commonly are stomach pains, sickness, mental instability, neuropathy. Sometimes there are are deppresions, abnormal agressy, hallucinations, paranoya. Due to the fact, thet autonomic nervous system is influenced by the disease, an additional symptoms can develop too. Such as tachycardia and cardiac arrhythmias. In some cases pain can be very harsh and can be a combination of acute and chronic. Sometimes also happens diarrhea and constipation. This happens because the nervous system of the gut is also affected. Porphyria symtoms may be rather different, it depends on the type of a porphyria.
We have the list of porphyria symtoms. It is a compilation from different sources and include almost all well-known and proved symtoms.

Cramps
Changes of an urine color
Paralysis
Nerve system disorders
Tingleing
Skin disorders
Mental upsets
Gastroenteritis
Change of personality
Blisters
Itches
Constipation
Skin became hypersensitive to sunlight
Vomiting
Pains in a chest area
Muscle deadness
Abdominal pain
Pain in back
Limb pains

Porphyria cutanea tarda

2007-07-11T20:44:00.000-07:00

Porphyria cutanea tarda (PCT) as a rule is the most common category of porphyria.
The disorder happens due to low levels of the enzyme accountable for the fifth step in heme creation. Heme is an important molecula for every body's organ. It's a part of hemoglobin and also facilitates transfer of oxygen in the blood.
Porphyria cutanea tarda fallout from underactivity of the enzyme uroporphyrinogen decarboxylase. Due to this begins store of porphyrins in the liver. Porhyrins manufactured in the liver are carried to the skin, using blood plasma as a transport.
Some precipitating factors can lead to the porphyria cutanea tarda. The most common of them are:
- an often alcohole use
- an extra iron in tissues
- using estrogens
- hepatitis c virus
Scientints think that all these thing influence on oxygen and iron in liver. So it can cause damage to the enzyme uroporphyrinogen decarboxylase.
In most cases people, who suffer from this disease, (something about 75-80%) do not have Porphyria cutanea tarda from heredity. Such form of PCT is called sporadic. In other cases the disease is hereditary and usually called familial. Both forms happens in an about 1 in 25 thouthands people.
The symptoms of Porphyria cutanea tarda usually become visible in maturity. The skin becoming overly precise to sunlight. Places of skin exposed to the sun become blisterring and scarring. It can also change pigmentation. Sometimes an augmented growth of hair occurs. Such areas of skin have a greater risk of an abnormal functionality of a liver and cancer.