Porphyry is a rare group of diseases caused by metabolic disorders substances called porphyrins, which are important in creating the Hema, who is part of the hemoglobin molecule (and myoglobin).
The most common forms of porphyria, such as the late cutaneous porphyria, which occurs in some liver diseases (alcoholic, hepatitis C and other types of cirrhosis) are not hereditary. Other forms of porphyria are inherited disorders that are inherited by dominant or recessive.
What develop symptoms Porphyry?
Due to the lack of enzymes that are responsible for the installation of porphyrin in the hem, porphyrins builds up in blood and tissues, resulting in manifestations of the disease. Because of the accumulation in the liver Razija the liver, accumulation in the central nervous system leads to the occurrence of epileptic seizures, hallucinations and depression. Porifirini accrued in the skin react with visible light, which leads to the formation of toxic products that damage the skin, enhancing skin fragility, and creating bubbles and blisters that hard to heal and leave scars.
How is Porphyry manifested?
There is a basic division of acute porphyria or hepatic (liver) Porphyry, which is manifested in the form of attacks of severe pain in the abdomen, dizziness and seizures similar to epilepsy, hallucinations and heart work faster. If attacks are frequent and always followed by depression. These attacks can be caused by certain drugs that affect metabolism or hormonal variations during the menstrual cycle in women, and most infections. Bearing in mind that this disease is rare, it is difficult to identify correctly and that patients carry the card with you or find a physician of whom suffer because of neurological symptoms and can not explain what it is about the attack.
In skin or eritropetskih porphyria develop sensitivity to the sun with the appearance of tiny bubbles, High school musical that hard to heal and leave scars. When you pass the sun patients feel soreness of the skin, and then develop bubbles that shoot and leave blisters. Own patients learn to avoid the sun because they know that they cause change. Also, with the skin, there is swelling and bleeding gums and increased hair on the face and hands after a long disease duration, and due to porphyrin excretion, urine turns dark in the sun, or getting even purple or red. When skin has the appearance of acute porphyria attacks.
In eritropetske Porphyry, from childhood appears severe hypersensitivity to the sun, and dark teeth that fluoresce in UV exposure due to porphyrin accumulation in dental enamel, and also develops and anemia. This and other types of Porphyry is considered to be the explanation for the stories about vampires and werewolves: sallow face due to anemia, necrosis and right red urine, is what is described in ledendama Western civilization, and rejection by society of these people were much more widespread than today.
How is porphyria diagnosed?
The diagnosis of porphyria is set to ispitvanaj the existence of porphyrin in the urine and red blood cells. This analysis is done in specialized centers. The simplest test for some forms Discovers Porphyry that the urine expensive and expose the light, and if it turns dark porifirije diagnosis of one type is set.
Basis of acute porphyria are drugs that can reduce the length of the attack, diet with high carbohydrate content and hours of glucose infusion solution. You should also identify drugs that cause attacks and advise patients about their avoided, and women are sometimes resorting to termination of menstrual cycle when attacks are related to hormones. In late tarda antimalarici the method of assembly, and beta carotene in some cases. Occasional venesection (bloodletting) were applied in late tarda to reduce iron accumulation that occurs with this form of the disease.
What is pseudoPorphyry?
Unlike the “real” Porphyry, at pseudoPorphyry the sensitivity to the sun, bubbles and blisters on the skin with lagging scars occur although there is no porphyrin accumulation. Some other substances are likely responsible for the development of these symptoms. Pseudoporfirija occurs in patients on hemodialysis with a long and continuous use of solariums that cause skin damage. Also, some drugs may uzazvu pseudoporfiriju (naproxen, some antibiotics, diuretics, etc.).