What is the prevalence of such an ailment as vampirism? The disease is more common in whites living in South Africa, in descendants who migrated from Holland. A similar pathology is common in Finland.
The types by which the disease is inherited and the features of the disease are described in this article.
Vampires: reality or fiction?
Do vampires exist in real life? History shows that yes. Modern researchers believe that a lot of people suffered during the hunt for vampires, and, as a rule, they were innocent people. Although the reason for the persecution still existed. Those people who were accused of vampirism did not drink other people's blood at all and were not werewolves, but at the same time they had a terrifying appearance.
Scientists believe that those accused of such a terrible crime had a rare genetic blood disease, which was named "porphyria" from the Greek word porphyros (purple).
It is believed that the manifestation of the diseasecontributed to the birth of a child from close relatives. Porfiria became widespread in the small villages of Transylvania (the birthplace of the famous Count Dracula) about a thousand years ago.
Cases of vampirism also occurred in famous royal dynasties. For example, researcher Andrew Wilson in his book "The Victorians" mentions the genetic form of porphyria, which was common among representatives of the British throne. According to him, it was this disease that caused the dementia of Queen Victoria's grandfather, King George III.
Mechanisms of disease
So, vampires don't exist in real life. With this pathology, the production of the gem-non-protein part of hemoglobin is disrupted, which leads to an excess in the blood of toxic porphyrins that bind metals in the body, in particular, iron and magnesium. Excess content of the substance poisons almost the entire body.
There are several types of porphyrias, and all but the latest form, which affects the skin, are genetic.
The inheritance patterns of each species vary, but the autosomal dominant mechanism comes to the fore.
Main provocateurs
Most often the role of disease provocateurs are:
- following a strict diet;
- nervous overload;
- barbiturates, tetracyclines, oral contraceptives;
- experience with contact with toxic chemicals;
- changing the hormonal background of a woman;
- infectious diseases;
- drinking alcohol.
Symptoms of erythropoietic uroporphyria
How does vampirism manifest itself? The disease is quite rare and is transmitted in an autosomal recessive manner. It can be observed in one generation in several children, as well as parents who are considered heterozygous carriers of the pathology. There are no clinical manifestations of the disease. The disease can manifest itself in infancy. What are the stages of vampirism?
At the first stage, newborns have bright red urine, the presence of photosensitivity. In this process, uroporphyrinogen released from red blood cells undergoes oxidation and is transferred to uroporphin, being deposited on the skin.
Some time after birth, the baby is covered with blisters that transform into ulcers. Against the background of taking antibiotics, ulcers turn into scars. In this case, areas of sclerosed skin appear on the face and other parts of the body.
At the last stage, the child develops contracture of the joints and loss of vision, there is a lack of growth of hair and nails.
How does porphyria manifest itself? Its symptoms are varied:
- the spleen of patients increases in size;
- manifested hemolytic anemia with hemolysis inside the cells;
- the level of free serum bilirubin and the reticulocyte count increase;
- becomes dilated erythronormoblastic germ of the bone marrow;
- lifespanerythrocytes is sharply reduced;
- uroporphin and coprporphin levels increase.
The disease is often fatal in early childhood.
How does erythropoietic protoporphyria manifest?
Vampirism is a disease inherited in an autosomal dominant manner. The basis of the disease is a violation of the synthesis of protoporphyrin heme, which concerns a part of erythrokaryocytes. It is also possible to increase the level of synthesis of aminolevulinic acid.
Patients have increased sensitivity to sunlight, skin swells, itching appears, blisters appear at the burn sites, which subsequently ulcerate.
Usually no scarring. The level of uroporphyrin and coproporphyrin in erythrocytes and in urine remains normal, and protoporphyrin IX in erythrocytes increases. Plasma protoporphyrin may also be high.
Anemia due to hemolysis is rare, due to the presence of two types of red blood cells in the bone marrow. Sometimes there is the appearance of hemorrhage, which is associated with the deposition of hyaline on the vascular walls and their subsequent rupture. Spleen enlargement is very rare.
Erythropoietic coproporphyria
This is an extremely rare pathology inherited in an autosomal dominant manner. It is clinically similar to erythropoietic protoporphyria.
The content of coproporphyrin in erythrocytes is 30-80 times higher than normal, there is an excretion of large amounts of coproporphyrin III with urine and feces. Mild photosensitivity appears. The acute form of the disease can be caused by taking barbiturates.
Acute intermittent porphyria
This is one of the hepatic forms of the disease. Severe neurological signs of vampirism can be fatal. Sometimes their manifestation becomes less pronounced and persistent remission occurs. The disease is inherited in an autosomal dominant manner. Its development is based on a violation of the activity of the enzyme uroporphyrinogen synthetase and an increase in the level of delta-aminoleaulic acid synthetase, which poisons nerve cells.
The level of porphyrins in red blood cells is normal. In the urine, there is an increased content of uroporphyrin I and II, as well as coproporphyrin III. In the acute period, a precursor of porphyrins, porphobilinogen, is found in the urine.
There is also an increased level of hepatic synthetase and delta-aminolevulinic acid, increased urine porphobilinogen.
Various complications may occur:
- abdominal pain that is localized in various areas, which often requires surgical intervention;
- severe forms of polyneuritis;
- paresthesia;
- mental disorders;
- coma;
- high blood pressure;
- Pink urine.
Vampirism is a disease that can be fatal. The death of the patient is usually due to respiratory paralysis. Many patients die in a coma orcause of cachexia.
Exacerbation of the disease is often provoked by pregnancy, taking barbiturates, sulfonamides, analgin. Relatives of patients may show signs of the disease in the absence of vivid symptoms (erased form of intermittent disease).
Hereditary form
Vampire Man inherits this disease in an autosomal dominant manner. Often the disease develops latently and manifests itself only when the activity of the enzyme coproporphyrinogen oxidase is impaired and the level of synthesis of delta-aminolevulinic acid in the liver increases.
According to the clinic, this form of the disease resembles such a pathology as acute intermittent porphyria. In the urine with an acute course, the level of delta-aminolevulinic acid and porphobilinogen does not reach such critical levels as in the intermittent form. Increased levels of coproporphyrin in urine and feces.
Variegated form
Also has an autosomal dominant pattern of inheritance. The development of the disease is provoked by impaired activity of the protoporphyrinogen oxidase enzyme and an increased level of delta-aminolevulinic acid synthesis.
The signs of the disease are similar to intermittent porphyria. Sometimes kidney failure develops. When taking barbiturates and analgin, there may be acute pain in the abdomen and a malfunction of the nervous system.
Urocoproporphyria
This is the latest dermal variety. It occurs in people who abuse alcohol, have had hepatitis, or have had contact withgasoline and poisons. Most patients have liver failure. In the urine there is a high rate of uroporphyrin, the content of coproporphyrin is not much increased. The enzyme activity of uroporfinogen carboxylase is also detected.
At the same time, the vast majority of relatives of patients revealed an increase in the level of uroporphyrin in the urine and coproporphyrin in the feces. Some families have two or three carriers of this pathology.
Vampire Man suffers from various skin pathologies:
- UV sensitivity,
- mechanical injury,
- thickening or thinning of the skin,
- blistering on the back of the hand and face, followed by scarring.
Structural changes in the skin are manifested in the primary lesion of the dermis. Epidermal changes are secondary.
The leading clinical sign is an increase in the size of the liver. Its functionality often suffers.
Principles of Therapy
How to recover from vampirism? To date, the main methods of therapy leading to the absolute relief of the disease have not been developed. Therefore, the main goal of treatment is to eliminate the symptoms of the disease. Protecting the body from exposure to ultraviolet rays can reduce disease symptoms.
One of the main ways to reduce negative signs is to protect the body from sun exposure. Avoid taking drugs such as tranquilizers, analgesics, sulfonamides.
With sharp painthe use of narcotic painkillers is indicated. For example, the use of chlorpromazine is shown.
At high blood pressure, appropriate therapy is selected. Usually used "Inderal".
In order to reduce the content of porphyrins, the use of such a drug as "Delagil" is indicated. This drug promotes the binding of porphyrins "Delagil" and "Riboxin".
Indicated taking a course of vitamins, folic and nicotinic acid, riboflavin, retinol, nicotinic acid.
For the treatment of skin lesions, the use of ointments, including rezocin or corticosteroids, Shostakovsky's balm is recommended.
It should be noted that the treatment will be ineffective if the patient consumes alcoholic beverages.
Preventive measures
For the purpose of prevention, patients are recommended to exclude the use of drugs that increase the body's photosensitivity. The use of external agents such as bergamot oil, eosin and rodmine is also contraindicated.
Forecast
How to recover from vampirism? With timely diagnostic procedures and properly selected therapy, the treatment of porphyria gives good results. With the wrong choice of all appropriate measures, the course of the pathology may worsen.
Patients complain of acute pain in the abdomen, which do not have a specific localization. Patients also experience nausea, vomiting, andwork of the digestive tract.
With an incorrect diagnosis and treatment, the acute form of porphyria leads to death in half of the cases.
Conclusion
Are there real vampires? Of course not. This is a myth that was born due to pathology that disfigures the appearance of a person.
Vampirism is a disease, the basis of which is a violation of the metabolic nature, which has a genetic basis. Pathology contributes to the failure of the internal organs, and also violates the integrity of the skin.
