What is the prevalence of such an ailment as vampirism? The disease is more common in representatives of the white race living in South Africa, in descendants who have migrated from Holland. A similar pathology is common in Finland.
The types by which the disease is inherited, and the characteristics of the disease are described in this article.
Vampires: reality or fiction?
Are there vampires in real life? History testifies that yes. Modern researchers believe that when hunting for vampires, a lot of people were injured, and, as a rule, these 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 had a terrifying appearance.
Scientists believe that those accused of such a terrible crime had a rare genetic blood disease, which was called "porphyria" from the Greek word porphyros (purple).
It is believed that the manifestation of the disease was facilitated by the birth of a child from close relatives. Porfiry became widespread in the small villages of Transylvania (the birthplace of the famous Count Dracula) about a thousand years ago.
Cases of vampirism were also found in the famous royal dynasties. For example, researcher Andrew Wilson in his book "Victorians" mentions the genetic form of porphyria, which was common among representatives of the British throne. According to him, this disease was the cause of dementia of Queen Victoria's grandfather - King George the Third.
Disease mechanisms
So, vampires in real life do not exist. With this pathology, the production of the gemma-non-protein part of hemoglobin is disrupted, which leads to an excess of toxic porphyrins in the blood that bind metals in the body, in particular iron and magnesium. Excessive substances poison almost the entire body.
There are several types of porphyria, and all of them, except for the latest form that affects the skin, are genetic.
The types of inheritance of each species vary, but the autosomal dominant mechanism comes to the fore.
The main provocateurs
Most often, the role of provocateurs of the disease are:
- adherence to a strict diet;
- nerve overload;
- barbiturates, tetracyclines, oral contraceptives;
- contact with toxic chemicals;
- a change in the hormonal background of a woman;
- infectious diseases ;
- the use of alcoholic beverages.
Symptoms of erythropoietic urophorphyria
How is vampirism manifested? The disease is quite rare and is transmitted autosomally, in a recessive manner. It can be observed in one generation in several children, as well as parents, who are considered heterozygous carriers of 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 a bright red urine, photosensitivity. In this process, uroporphyrinogen released from red blood cells undergoes oxidation and is transmitted to urophorphin, deposited on the skin.
Some time after birth, the child becomes covered with vesicles that transform into ulcers. With antibiotics, ulcers turn into scars. In this case, areas of sclerotic skin appear on the face and other parts of the body.
At the last stage, the child appears contracture of the joints and loss of vision, there is a lack of hair and nail growth.
How is porphyria manifested? Her symptoms are varied:
- the spleen of patients increases in size;
- hemolytic anemia appears with hemolysis inside the cells;
- the level of free serum bilirubin and the rate of reticulocytes increase;
- the erythronormoblastic bone marrow becomes expanded;
- erythrocyte life expectancy is sharply reduced;
- the level of uroporfin and coprporfin increases.
The disease often results in death in early childhood.
How is erythropoietic protoporphyria manifested?
Vampirism is a disease inherited in an autosomal dominant way. The basis of the disease is a violation of the synthesis of protoporphyrin heme, which affects part of the red blood cells. It is also possible to increase the level of synthesis of aminolevulinic acid.
Patients have an increased sensitivity to sunlight, the skin swells, itching appears, blisters appear on the burn sites, which subsequently ulcerate.
Scar formation is usually not observed. The level of uroporphyrin and coproporphyrin in erythrocytes and in the urine remains normal, and protoporphyrin IX in erythrocytes increases. Plasma protoporphyrin may also be high.
Anemia against the background of hemolysis is rare, due to the presence of two types of red blood cells in the bone marrow. The appearance of hemorrhage is sometimes noted, which is associated with the deposition of hyaline on the vascular walls and their subsequent rupture. An enlarged spleen is very rare.
Erythropoietic coproporphyria
This is an extremely rare pathology inherited in an autosomal dominant way. According to the clinical picture, it is similar to erythropoietic protoporphyria.
The content of coproporphyrin in red blood cells is 30-80 times higher than normal; excretion of large amounts of coproporphyrin III with urine and feces is noted. Mild photosensitization appears. The acute form of the disease can be caused by taking barbiturates.
An acute intermittent form of porphyria
This is one of the hepatic forms of the disease. Heavily leaking neurological signs of vampirism can be fatal. Sometimes their manifestation becomes less pronounced and a stable 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 synthetase delta-aminoleaulinic acid, which poisons nerve cells.
The level of porphyrins in red blood cells is normal. In 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.
An increased level of hepatic synthetase and delta-aminolevulinic acid is also noted, urinary porphobilinogen is increased.
Various complications may occur:
- abdominal pains that are 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. Patient death is usually due to respiratory paralysis. Many patients die in a coma or due to cachexia.
Exacerbation of the disease is often triggered by pregnancy, taking barbiturates, sulfonamides, analgin. Relatives of patients may have signs of the disease in the absence of vivid symptoms (erased form of the intermittent disease).
Inherited form
A vampire man inherits this disease in an autosomal dominant manner. Often, the disease develops covertly and appears only when the enzyme-propropyrinogen oxidase activity is disturbed and the level of synthesis of delta-aminolevulinic acid in the liver is increased.
According to the clinic, this form of the disease resembles such a pathology as acute intermittent porphyria. In acute urine, the level of delta-aminolevulinic acid and porphobilinogen does not reach such critical levels as in an alternating form. In urine and feces increased levels of coproporphyrin.
Variegate form
Also has an autosomal dominant type of inheritance. The development of the disease is provoked by a disturbed activity of the protoporphyrinogen oxidase enzyme and an increased level of synthesis of delta-aminolevulinic acid.
According to signs, the disease is similar to intermittent porphyria. Sometimes kidney failure develops. When taking barbiturates and analgin, acute pain in the abdomen and malfunction of the nervous system can occur.
Urocoproporphyria
This is the latest skin variety. It occurs in people who abuse alcohol, have had hepatitis, or have had contact with gasoline and poisons. Most patients have malfunctioning liver function. In urine, a high indicator of uroporphyrin is noted, the content of coproporphyrin is not increased much. The enzyme of the activity of urophorphinogen carboxylase is also detected.
At the same time, and the vast majority of patients' relatives, an increase in the level of uroporphyrin in the urine and coproporphyrin in the feces is detected. In some families, there are two to three carriers of this pathology.
A vampire man suffers from various skin pathologies:
- hypersensitivity to ultraviolet,
- mechanical injury
- thickening or thinning of the skin,
- the appearance of blisters on the back of the hand and face, followed by scarring.
Structural changes in the skin appear in the primary lesion of the dermis. Changes in the epidermis is secondary.
The leading clinical sign is an increase in liver size. Often its functionality suffers.
Principles of Therapy
How to cure vampirism? To date, no basic therapy methods have been developed that lead to the absolute relief of the disease. Therefore, the main goal of treatment is aimed at eliminating the symptoms of the disease. Protecting the body from ultraviolet rays can reduce the manifestations of the disease.
One of the main ways to reduce negative signs is to protect the body from exposure to the sun. It should exclude the use of drugs such as tranquilizers, analgesics, sulfonamides.
With sharp pain, the use of narcotic painkillers is indicated. For example, the use of chlorpromazine is indicated.
With a high blood pressure, appropriate therapy is selected. Commonly used is Inderal.
In order to reduce the content of porphyrins, the use of a drug such as Delagil is indicated. This drug promotes the binding of porphyrins to Delagil and Riboxin.
The course of vitamins, folic and nicotinic acid, riboflavin, retinol, nicotinic acid is shown.
For the treatment of skin lesions, the use of ointments, including resocin or corticosteroids, Shostakovsky balm is recommended.
It should be noted that treatment will be ineffective if the patient consumes alcohol.
Preventative measures
For the purpose of prevention, patients are recommended to exclude the use of drugs that increase the photosensitivity of the body. The use of such external agents as bergamot oil, eosin and rhodmin is also contraindicated.
Forecast
How to cure vampirism? With timely diagnostic procedures and correctly selected therapy, the treatment of porphyria gives good results. With the wrong choice of all appropriate measures, the course of the pathology can worsen.
Patients complain of acute pain in the abdomen, which do not have a specific location. Also, patients have nausea, vomiting and a violation of the digestive tract.
With an incorrect diagnosis and therapy, an acute form of porphyria in half the cases leads to death.
Conclusion
Are there any real vampires? Of course not. This is a myth that was born thanks to a pathology that deforms the appearance of a person.
Vampirism is a disease based on a metabolic disorder that has a genetic basis. Pathology contributes to the malfunction of the internal organs, and also violates the integrity of the skin.