2010年8月7日星期六

Vampire Disease

Vampire Disease




Abstract correcting edit summary

Vampire disease is rare in the world, strange disease, the sick must be a very extreme way to avoid the sun. Exposure to sunlight. The skin will blister, but also feel pain and burning.

Vampire Disease - Introduction

Vampire Disease

The so-called vampire disease is congenital erythropoietic porphyria (CEP), the tragic fate of its patients are suspected to be the origin of vampire story. Severe congenital erythropoietic porphyria patients addicted to drinking blood, the blood increased by added hemoglobin; afraid of the sun, can only live in the dark; hate garlic; due to severe anemia makes the face pale; toxin in patients with lip and gum erosion, so that patients sharp teeth like a wolf; skin covered with scars, look particularly old; body with bones and urine red, black and brown teeth and so on. These symptoms are related to the legendary vampire is very similar to the disease so people will call it vampire disease. Now, even very serious scientists also believe that modern movies are made into a vampire legends are drawn from the Middle Ages, these legends has its basis in reality.

Vampire Disease - Symptoms and signs

Porphyria has many manifestations, more common form is acute intermittent porphyria (AIP), the British "mad king" George III is one of the victims of this disease. The most severe porphyria congenital erythropoietic porphyria (CEP), the tragic fate of its patients are suspected to be the origin of vampire story. Although porphyria is usually caused by the genetic mutation, but excessive drinking and environmental pollution will also induce the disease. The most notorious example occurred in the last century 50's Turkey. About 4,000 people in the consumption of the sprayed fungicides hexachlorobenzene wheat had contracted a type of porphyria, hundreds of people died. Soon after, hexachlorobenzene fungicides on the disabled worldwide. [1]

Addicted to drink blood:: For most patients with porphyria, blood transfusion and hemoglobin can effectively relieve symptoms, and still is now mainstream treatment. Hemoglobin is very tenacious, through the digestive tract can still be absorbed by the small intestine. This means that, at least in theory, the ancient porphyria patients can smoke or drink blood to help you feel comfortable - this is probably the origin of vampire story.



Vampire Disease

Fear of the sun:: is a photosensitive pigment porphyrin, which will gather in human skin, bones and teeth. Most of the porphyrin in the dark was benign, not causing any harm to the body, but once exposed to sunlight can be transformed into a dangerous toxins, eating people's muscles and tissues. Therefore, patients with porphyria, as the legendary vampire so they can only live in the world of darkness, the can not see the light.

Disgust of garlic: the film "Blade Runner" inside, "Blade" with piles of garlic against vampires bullets and razor, invincible. In most of the vampire legend, as garlic and crosses have been described as the vampire killer. Interestingly, patients with porphyria also hate garlic. Simple reason, some of the chemical composition of garlic will worsen their condition, bringing pain and other symptoms.

Face pale: Most patients with porphyria are associated with severe anemia, not only because they usually only live in the dark, more importantly, their bodies will affect the porphyrin in hematopoietic function, undermining the formation of hemoglobin . Typically, patients with porphyria with large areas of the body but also the pigment deposition - often purple.

Sharp teeth like a wolf: porphyrin sun exposure will be swallowed into the muscle and tissue of toxins, one of the main performance is that it will corrode the lips and gums were so sharp they are exposed, like the wolf teeth. Decay always look bloody gums, inevitably reminiscent of the blood of vampires taking.

Immortality: the role of the toxins, porphyria patient's ears and nose will be the corrosion, but the skin will be covered with scars, so they look particularly old. Legend in Europe, immortality will often be described as similar to the way, repeated the baseless assertion, and there will be a "vampire with immortality itself." In fact, the life span of patients with porphyria are usually very short. [1]

Vampire Disease - etiology

Vampire Disease

Porphyria is probably a genetic disease, because some genes can influence the damage caused by the skin pigment.

Porphyria also known as porphyria, is a ferrous hemoglobin disorder characterized by genetic disease. Porphyrin to hemoglobin, myoglobin, peroxidase and cytochrome synthesis process such as an intermediate, mainly in the synthesis of red bone marrow and liver. Is generally believed that glycine and succinic acid in a series of enzymes, the first synthesis of δ-aminolevulinic acid, culminating in the bile of the original porphyrin, followed by derived for the uroporphyrinogen, urine porphyrins, fecal porphyrin and the original porphyrin, which protoporphyrin and ferrous (Fe2 +) together to form ferrous hemoglobin (Hb). Normally, fecal porphyrin content in urine very little negative common qualitative method, the original bile porphyrin was also found negative. If a large number of porphyrins in urine, turns red in the sun shall be porphyria. [2]

谢尔盖瓦西里耶夫 that serious attacks in porphyria when the damaged tissue will become deformed. Whole body, especially the extremities will twist and facial deformation, skin darkening, gums are corrosion Xianchu fangs, and then give it a kind of porphyrin blood red, giving the impression of a kind of bloody fangs. Such people fear the sun, the sun often appear under the skin ulcer with pain of burns. Only the night before they go out, mentally began to change.

Vampire Disease - classification

Hematoporphyrin disease is actually a pigment called porphyrin accumulation in the skin, bones and teeth related diseases caused by collectively. Many porphyrins are harmless in the dark, but sunlight can be converted to meat-type corrosive toxin. If you do not treat the disease, the most serious symptoms (such as red blood cell formation of congenital disorder of blood porphyrin) will lead to deformation of the body and eventually into people's imagination in the resurrection of the zombies as the terrorist of the malformations - ears and nose of patients to be " take ", and her lip and gum erosion, exposed root red, dense scar the skin, such as the zombie-like pale (reflecting the potential anemia). Because of anemia through blood transfusions, some historians speculate that the dark ages in Europe, the Middle Ages, the blood in patients with porphyria may be trying to drink the blood of such prescriptions to treat. Leaving aside the veracity of this assertion how the blood of those with congenital erythropoietic porphyria patients can not definitely recognized the risks involved in the day outdoors. They probably know as much as possible to avoid the garlic, because garlic in certain chemical substances are thought to increase blood porphyria symptoms, so that the incidence of mild pain became unbearable.

Porphyria can be divided into two categories: erythropoietic porphyria and hepatic porphyria.

Congenital erythropoietic porphyria (CEP) of the patients were suspected to be the origin of vampire story.

Porphyria cutanea tarda is the most common porphyria in a, also known as acquired porphyria. The clinical features are light sensitive dermatitis, skin pigmentation, liver disease and hirsutism. The patients mostly after the age of 30, initially as face, neck, back and other parts exposed to light more than light hair and pigmentation, easily overlooked. With the extension of disease, illness worsened after the acute onset of summer light symptoms such as blisters, bullae, or even when blood blisters attention. Old patients face wrinkling, face, neck, chest, etc. have scleroderma-like performance. In addition, patients often accompanied by liver, and diabetes. Variety of chemicals or drugs such as 666, estrogen, busulfan, phenobarbital, chlorpromazine, phenytoin, etc., especially alcohol, easy to cause acute attack of this disease.

Vampire Disease - Differential Diagnosis

Vampire

1. Erythropoietic porphyria characterized by blood:

① blood reticulocytes increased, changes may have hemolytic anemia. Was the phenomenon of bone marrow erythroblastic hyperplasia, containing a large number of porphyrin;

② large number of urine and a small amount of urine porphyrin faecal porphyrin Ⅰ Ⅰ;

③ Fecal droppings in a small amount of urine porphyrins and porphyrin Ⅰ Ⅰ;

④ protoporphyrin type, may have increased protoporphyrin;

⑤ red blood cells containing a large number of porphyrins in urine and feces porphyrins and protoporphyrin.

2. Hepatic porphyria characterized by blood:

① blood and bone marrow with normal porphyrin, acute abdominal pain, increased white blood cells of peripheral blood;

② Urine often has a red, colorless and when there are emitted by sunlight or boiling acid turns red after 30min. Acute intermittent urine containing a large number of porphyrin precursors (δ-aminolevulinic acid, porphyrins bile original), and urine and faeces porphyrin;

③ acute intermittent and delayed skin type, and fecal porphyrin normal or slightly more, while the mixed type of fecal porphyrins and protoporphyrin were significantly increased, hereditary fecal porphyrin to discharge increased fecal porphyrin Ⅲ;

④ have liver dysfunction, protein and albumin and globulin to reduce the proportion of inverted; ⑤ cutaneous delayed increase of serum iron, acute intermittent may have electrolyte imbalance (especially sodium), serum protein bound iodine and cholesterol increased. Have renal dysfunction, oliguria and azotemia occur.

3. Acute abdomen acute intermittent porphyria attacks of abdominal pain, often misdiagnosed as acute abdomen. However, the latter with permanent abdominal tenderness, rebound tenderness and muscle tension, urine unchanged after heat exposure or acidified bile of the original red and porphyrin test was negative.

4. Lead poisoning can cause abdominal pain Erzhi porphyrin metabolism disorders, and acute intermittent porphyria similar. But the former has the history of lead exposure, and the gall of the original normal porphyrin.

5. Pellagra often glossitis, stomatitis and nutritional disorder history, urine porphyrins and their precursors, not much given to nicotinic acid treatment is effective.

6. Symptomatic urinary porphyrin this disease is found in liver disease, connective tissue disease, a variety of blood diseases, to fecal and urinary porphyrin porphyrin-based increase was not significant, did not increase porphyrin precursors and porphyrins in urine test was negative primary biliary .

7. Other persons with psychiatric syndrome, which require and polio, encephalitis, schizophrenia and other similar identification. [2]

Vampire Disease - Treatment and processing



Cause of treatment



Vampire person with a beautiful, clean, elegant, psychedelic, mystical temperament, but also with the slightest breath of terror

Family should be investigated for patients who have increased porphyrin and its precursors, who should be treated with bone marrow lesions of their liver disease to avoid all kinds of triggers.



Symptomatic treatment

1. Erythropoietic porphyria treatment of blood

Mainly for skin damage and hemolytic anemia processing.

(1) Skin damage: avoid sunlight and trauma can be 15% of the amino acid ointment. 30mg daily oral administration of β-carotene or riboflavin 20 ~ 40mg, or every other day service A flat 50mg, a light flu allergy prevention may be effective.

(2) hemolytic anemia: the implementation of splenectomy on hemolysis may have good results, those who are allergic to certain light perception have a lasting effect.

(3) nourish the liver and gallbladder: 4g cholestyramine resin taken before meals, 3 times / day.

2. Porphyria hepatic blood

(1) to avoid incentives: such as fatigue, mental stimulation.

(2) diet: high carbohydrate diet to prevent attacks, and the drinking should be taboo.

(3) hormone: daily service prednisone 30 ~ 60mg, some cases may be strange effect.

(4) abdominal pain attacks: ① sedation, chlorpromazine per 12.5 ~ 25mg, 3 ~ 4 times / d can reduce psychotic symptoms, aspirin can alleviate the abdominal pain; ② tetraethylammonium chloride 500 ~ 1000mg intramuscular or 200 ~ 250mg intravenous injection; ③ cAMP AMP125mg intramuscular injection, 2 / d; ④ EDTA in 1g, 4 / d, or 0.25 ~ 0.5g normal saline or glucose solution infusion, 2 / d . 3 to 5 days for a treatment interval of 2 to 4 repeat applications can be 3 to 4 courses.

(5) hemoglobin: hemoglobin severe treatment should be given every 3 ~ 6mg/kg, 24h total <6mg/kg. Diluted with normal saline intravenous injection or infusion (<40mg/min), 6 ~ 8min Note End. 12h intervals for at least another 3 to 5 days for treatment. It should be noted, a large number of injections can lead to kidney damage.

(6) of chloroquine: Each oral 125mg, 2 times per week. When the urinary porphyrin excretion fell to <100μg / d, stop taking it.

(7) fluid: glucose inhibits δ-aminolevulinic acid (porphyrin precursors) synthetase activity decreased porphyrin formation can be intravenous infusion of glucose 10 ~ 20g / h, the daily volume of close to 300g / d, while correcting hyponatremia, hypertriglyceridemia and low chlorine and other hypomagnesemia.

(8) Other: Available calcium gluconate, liver extract, a lot of vitamin B and C treatment, bleeding may also be appropriate (each 300 ~ 500ml, 2 to 3 times per week to the hemoglobin up to 100g / L).



Surgery and anesthesia

1. Surgical issues related to this disease itself without surgery. Coexisting surgical diseases necessitating surgery to avoid the acute stage implementation, and preoperative preparation should be strengthened. Control of hypertension and psychiatric symptoms, to correct abnormal liver function and water and electrolyte imbalance.

2. Anesthesia porphyria can be life threatening because of drug inspired and therefore should be very cautious of narcotic drugs. Select type of anesthesia and the surgical site should be determined, as far as possible use local anesthesia, regional block anesthesia. Avoid spinal anesthesia to prevent postoperative neuropsychological changes. More major surgery with general anesthesia, induction is the most dangerous stage. The basis inspire porphyria attacks can be divided into three types of narcotic drugs: ① non-priming drug; ② controversial drugs; ③ trigger drug. It goes without saying, should try to use non-narcotic medication drug priming, drug caution controversial drug without priming, to ensure safety.

(1) non-priming of drugs: the current view, the following drugs safe for patients porphyrin: propofol, midazolam, N2O, cyclopropane, ether, tube arrows poisoned arrow, succinylcholine, vecuronium amine, atropine, neostigmine, procaine, tetracaine, morphine, pethidine, fentanyl, buprenorphine, paracetamol, naloxone, nitrazepam, Cl hydroxyl to a stable , droperidol, phenothiazine, β blockers, epinephrine, phentolamine, corticosteroids, salbutamol, metoclopramide and domperidone.

(2) priming of drugs: a barbiturate medicines, etomidate, ketamine, enflurane, methoxy halothane, A library chloramines, mepivacaine, spray him?? Sim, on behalf of Lee, In addition to diazepam and midazolam, other than the benzodiazepine drugs, and Aetna ketone, verapamil, nifedipine, diltiazem, hydralazine, phenoxy benzyl amines, aminophylline, West Mi for small, nikethamide, imipramine, lysergic new base, phenytoin, aminopyrine, thiourea, chloride Nitrogen, sulfonamides and ethanol.

(3) controversial drugs: a halothane, isoflurane, atracurium, pancuronium, ammonium glycopyrrolate, lidocaine, prilocaine, bupivacaine, alfentanil, Shu Fen fentanyl, lidocaine, mexiletine, p-bromobenzyl amine, disopyramide, β agonists, α agonists, sodium nitroprusside and ranitidine.

(4) anesthesia management: maintain a suitable depth of anesthesia, should not be too shallow. Strengthen the respiratory management, to avoid hypoxia and (or) carbon dioxide accumulation. Hunger can trigger disease onset, intraoperative infusion of glucose (10 ~ 20g / h) for the prevention and treatment of porphyria important measures. Monitoring of urine output and whether the observed UV radiation red fluorescence, but also to prevent renal failure.

Blood in their efforts to seek treatment method for porphyria, scientists recognized that porphyrin is not only a target for treatment, while still a treatment tool. If injected into the porphyrin diseases such as cancer and other organizations, it can be light activated and destroy the disease tissue. This is called photodynamic therapy (PDT), which 70 years from the last century to become helpless on the cancer can cure a variety of malignant diseases, today a complex and effective means, including recent and often leads to macular degeneration and adults blind treatment of pathologic myopia, ongoing study also included coronary artery disease, AIDS, autoimmune diseases, transplant rejection and treatment of leukemia and other pioneering. [2]

Vampire disease - found that history

Ancient Greek physicians Hippocrates is generally considered the first recognized porphyria who, when he considered the disease a blood disease or lung disease. Until 1871, German biochemist 菲利克斯霍 Perot - Seiler porphyrin pigment was discovered with porphyria causal relationship between. 1889, BJ Stokvis a series of clinical symptoms collectively referred to as "porphyria," the name from this strange disease has been established.

Vampire Disease - Clinical Case

Vampire who suffer from disease, body with bones and urine red, black and brown teeth, and can only live in the dark, need to be supplemented to increase the blood hemoglobin.

2, according to ABC report, the United States, Pennsylvania, 5-year-old girl 卡西克纳夫 (pictured) born with a rare form of porphyria. She saw the sun, who will be blisters, or even ordinary light will hurt her, so Casey living in the dark all day, but from time to time to enter the same as the vampire blood hemoglobin added. Currently the world suffering from this rare porphyria no more than 500 people.



Vampire

April 10, 2001 Casey was born, doctors found that her skin a bit strange. Doctors began to suspect she had jaundice, bilirubin can be changed with a special light on her exposure, I did not expect Casey's condition worsened. Skin expert Howard Pride found Casey's skin is very sensitive to light and bilirubin light therapy has been severely burned Casey skin. Then doctors discovered that Casey's urine color is red.

Casey suffering the final expert diagnosis of a rare congenital red blood cells porphyria. This is a metabolic disease in vivo formation mechanism of heme disorder, leading to accumulate in patients with a kind of light-sensitive chemical porphyrin, bones and urine of patients to the color red, and teeth of patients become dark brown or purple.

Casey to cure the disease, her parents found a study porphyria 30 years Robert Mount Sinai School of Medicine geneticist? Daisinike. Casey Daisinike the laboratory to study DNA of parents found that they carry a rare "congenital porphyria red blood cells" variant gene and both pass the gene mutation Casey, made her very severe symptoms .

From birth newcomer, Casey can only live in the dark because sunlight and ultraviolet light are common at home bring harm to her, causing her body blisters and cause infection. If Casey's hemoglobin levels are too low, then she must accept blood transfusion. [3]

The mid-20th century, 80 in Virginia, there was a murder. Brownell is a 43-year-old victim of the bricklayer, before his death, he claimed to be vampires.

This is very strange murder case. Although Brownell throughout adulthood claim to vampire, but few of his words seriously. Brownell consecutive missing two weeks later, another neighbor, was found outside his apartment in the blood, thus giving rise to all the doubts. In his apartment, police found the body organs, tissue and more blood. Police first suspected Brownell killing people absconded. But after the blood analysis that is not the case, because it is Brownell own blood, that he was murdered. The most interesting is: After the body check, Brownell showed that he got liver disease Ye Lin. [4]

Reportedly, former German Chancellor Helmut Kohl's wife Hainiluola Cole, suffering from this incurable disease. Her 1993 fall ill, his condition is deteriorating, causing the burning sun for her unbearable. Eventually she was July 4, 2001 at home and committed suicide.

Vampire Disease - decrypt the legendary vampire

Vampire is a very popular characters in the world come almost every year there are several movie appearances.

Vampire, in English for the vampire, meaning zombie, meaning the demon blood draw. As a closely related human (vampire is usually hidden in the middle of the crowd) the devil image, it began several hundred years of legend has been with the strange and terrifying psychedelic colors. The past century, with the rise of Romantic literature and film, with a mysterious vampire aesthetic image, he became a rich and adhere to the cultural circumstances.

The first vampire story, about occurs at 1000 years ago. Middle Ages, there were a group of bloodthirsty wandering around. There are dozens of such people were arrested, and was burned alive, like the medieval Inquisition as witches who burned to death as sentenced. Members of the Inquisition wrote a lot about the vampire description, saying that they will revive after dark, leave the tomb, to taking the blood of sleeping people. They are cruel demon, with long sharp canine teeth, fear of the day.



Vampire person with a beautiful, clean, elegant, psychedelic, mystical temperament, but also with a trace atmosphere of terror.

14th century, the vampire superstition truly create a tradition, the main circulating in Central Europe, East Prussia (Prusse orientale), Silesia (silesie) and Bohemia (Boneme) range.

At that time the plague epidemic in Europe, this deadly infectious diseases so that people will be patient with the psychological fear in the yet to be determined hasty burial before death. Patients dying painfully struggling to come out, bloody body in the process damaged, often to see the activities of the so-called bodies, bloodshed and even self-devouring. In the shadow of death in Europe, with the psychological and panic spread quickly spread, zombie superstition be further strengthened.

The 18th century, under the reason in the Enlightenment was the victory, the various superstitions in this period suffered a serious setback. But the wave of vampire superstition is at this time it actually be an unprecedented development, but also behaved as a social phenomenon. This phenomenon has aroused the concern of religion, many of the vampire set to publish academic works.

Early lessons vampire is pure evil human blood, similar to the European legend of the werewolf. In the Middle Ages, and especially since the rise of Romanticism, the existence of vampires as its unique manner with a strong sexual, erotic aspects of color. The main attack for the vampire, the phenomenon of a small number of people eating there. As a vampire can often lure the opposite sex is not perceived in the win in each other's lives.



British doctor Liailisi is the first attempt to explain the scientific point of view of people vampire legends. In 1963, the Royal Medical Association, doctors have to submit an article entitled "On the porphyria and the vampire of the source of the disease," the paper put forward the theory of the bloodthirsty and porphyria associated with, that is a rare vampire genetic disease in which the body of the porphyrin metabolism, blood and urine in a large number of porphyrin, while the destruction of red blood pigment. Started no one believed him until the mid-80s have started to support his point of view.

Scientists have discovered that porphyria is a disease caused by certain genetic defects may affect skin pigment. Porphyrin is a nitrogen-containing organic compounds, commonly found in the body of all life, constitutes an integral part of hemoglobin. Porphyrins also form an integral part of chlorophyll. Genetic defects in porphyrin metabolism caused a problem, resulting in the aggregation of porphyrins in the skin, so skin sensitivity to light changes. Porphyrin has become the common oxygen transformed into the so-called pure oxygen as a catalyst, this oxygen can cause cell damage.



谢尔盖瓦西里耶夫 said: "porphyria patients with advanced facial deformation occurs, hand-foot terminal will be deformed, which makes them look very scary. Their skin color darker, with the erosion of the gums, they The teeth look very long. their front teeth will appear blood red, it seems that the blood stained above. In addition, porphyria patients are afraid to light, the light will lead to chronic inflammation and burning. so they only go out at night. a lot of mental patients problems may also occur. "



Now, even very serious scientists also believe that modern movies are made into a vampire legends are drawn from the Middle Ages, these legends has its basis in reality. [5]

没有评论:

发表评论

该小工具中存在错误
Loading...