Albinism

Albinism is a rare inherited disorder in which little or no skin pigment melanin is formed. It may
affect the skin, hair, eyes, or sometimes just the eyes.

 Hair and skin are usually white, and eyes may be pink or pale bluish-grey.
 Doctors usually make the diagnosis of albinism by examining the skin and eyes.
 There is no cure, but if you have albinism, you should protect yourself from
sunlight to prevent sunburn and reduce your risk of skin cancer.
Albinism is a disorder of skin pigmentation  of all races and worldwide . This not only causes
hypopigmentation (abnormally low amount of melanin) or depigmentation (complete loss of
pigment) in the skin, but also causes some eye problems, such as blurred vision, eye
misalignment ( strabismus ), and involuntary eye movements (nystagmus). It is caused by a
rare genetic disorder.
Eye albinism, a type of albinism, usually affects the eyes rather than the skin and hair.  There
are also other types of albinism in which bleeding disorders occur.

Albinism is a type of genetic mutation . It is said that in Korea in the old days , it was called
Baekja ( 白 者 ). It is a congenital genetic disease in which melanin synthesis is deficient in
melanocytes. Also called albino . Since it is a congenital disease, it is different from vitiligo,
which is an acquired disease. It is a genetic biochemical phenomenon that occurs mainly because
the formation of tyrosinase, an enzyme that oxidizes tyrosine to produce melanin, becomes
impossible due to gene mutation . Albinism refers to the absence of pigment in the eyes and hair,
so it can be caused by several causes. # Prevalent in whites , rare in yellows and Native
Americans . However, the proportion of blacks is rather higher than that of whites in Europe ,
and the incidence of some subtypes varies according to race and region.
Literally, the lack of pigment in the body turns white. The hair also turns white , such as silver
hair, and pigment is lost from all the hair on the body, including the eyebrows and eyelashes as
well as the hair on the head . Because there is no pigment in the iris of the eye, the blood vessels
of the eye are reflected as it is, so it is red - eyed . However, in reality , albinism, the silver eye ,
is a very serious case and is just as rare. In the case of red eyes, only 0.001% of the world's
population is usually white or black . Sleeping is also possible. The hair is also rarely completely
gray, and more often it is a form with a little color left, such as light blonde .
In female albinism, the fat layer under the skin is relatively thicker than in males, resulting in
bright white skin, but in male albinism, the fat layer is thin, resulting in pink skin with slightly
visible blood vessels. However, this is also relative, not only females are white and males are
pinkish. Because the blood vessels are visible through the skin, when you cry or get hot with
emotions, you get very red hot, not just normal people.
From an anthropological point of view, it is a disease suffered by the ancestors of modern whites
and yellows.
Similar to but different from albinism is Leucism . albinismIn the case of it looks white like
albinism, but the ability to produce melanin is normal.
Contrary to albinism, there is also melanoma, in which the skin darkens in certain areas. #
Worldwide, the incidence of albinism is 1 in 17,000.
There is currently no cure. The current research stage is the level of research on the nucleotide
sequence of the disease.

The Physical Weaknesses of Albinism

His eyesight is extremely low and he is likely to be strabismus .

The color of her skin is white like the color of paper, not human
skin. In reality, it feels quite incongruent, and has been the
subject of painful discrimination since ancient times. Also,
because they lack melanin and are completely exposed to UV
rays , the possibility of getting skin cancer is extremely high.
Since it is difficult for the eyes to withstand the ultraviolet rays
of sunlight, the visual acuity is the same as that of ordinary
people at first, but as the eyes are exposed to strong sunlight
and ultraviolet rays, it deteriorates until the eyes can withstand
it. Depending on the degree of ocular albinism and how much
strong sunlight and ultraviolet rays are blocked from infancy, the final visual acuity differs. They
say that it is inconvenient because they are forced to wear sunscreen every time they go outside ,
wear long clothes even on hot days, and wear sunglasses on sunny days .

The problem is particularly big in terms of vision. In case of red eye albinism, the iris choroid
Since there is no melanin in the back, it is impossible to control the amount of incident light, and
since the eyeballs cannot be kept in a dark box, glare in bright places, symptoms such as blurred
vision appear. For this reason, it is necessary to wear sunglasses outdoors as much as possible,
and when you go to an ophthalmologist , you can often see parents with albinism children.

You can hide the color of your hair, for example, by dyeing your hair. It's very difficult to adapt
to society, but that doesn't mean you have to look at it like a monster. The gaze is a problem, but
in fact, compared to other incurable diseases, it does not physically hinder social life. It is not a
fatal disease in the first place, and it has a much higher incidence of skin cancer than others, but
that is also a matter of probability. My skin is weak, so the sun is more stinging, but short
exposure is not a big problem, it just dazzles my eyes a little more, but I don't necessarily have to
wear sunglasses. Depending on the severity, in summer, only sunscreen is applied and short
sleeves or clothes that expose the body are worn. There are people who model with these
characteristics . #Russian model Nastya Жидкова , Brazil 's 12-year-old sisters, for example,
have many dreamy photos that make use of the unique impression of albinism. # , and twin
sisters from Kazakhstan . # , there is also Chinese albinism singer Gong Zeyi ( 龚泽艺), though
not a model.

Classification of albinism

Albinism is divided into three major categories.

Oculocutaneous Albinism
In addition to the various symptoms described above in the case of systemic symptoms,
nystagmus, teeth growth disorder, strabismus, deafness, mental weakness, etc. may accompany
the symptoms, and autosomal recessive inheritance is. It is very weak to sunlight and does not
cause pigmentation, but it easily causes erythema. The incidence of skin cancer is also very high.

In brief, systemic albinism is more likely to occur in a group with a lot of incestuous marriages ,
and an example is 'white Indians' living in the Panama region of the American continent.

Partial Albinism / Piebaldism

Localized albinism is divided into a skin type with partial albinism in the skin and hair and an
autosomal dominant inheritance, and an eye type with partial albinism in the eyes and X-
chromosome recessive inheritance (some autosomal recessive inheritance). there is.
Ocular Albinism
There is no abnormality in the skin or hair, but only the pigment deficiency in the eyes. Eye
symptoms are similar to systemic vitiligo, and glare and nystagmus can be seen, and there is
visual impairment.
Social aspects of albinism
In the old West, treatment for albinism was divided into extremes. If you were lucky and your
eyes were not red, you were praised as a child of a star , but if you had red eyes, you were
despised as a child of the devil and subjected to a witch hunt . Also , there were people who were
despised as vampires because they were weak against sunlight . There is a theory that one of the
universal images of vampires is silver eyes . The story of a boy with systemic albinism was
introduced on Curious Story Y , and the story of a woman with systemic albinism and a girl with
localized albinism were introduced on the national talk show Hello . Meanwhile , in the
mythology of the Inca Empire , there is a god who is presumed to be an albino.
black albinism
The incidence of albinism in blacks is 1 in 10,000, which
is greater than the probability of 1 in 36,000 in Europe .
Because Africa is exposed to a lot of ultraviolet rays , the
incidence of skin cancer is 1 in 3,000, which is higher than
1 in 10,000 of Caucasians. They live with contempt and
contempt in the dark-skinned black society. During 2008
alone, 19 or more albinism patients were killed in
Tanzania alone, and they live in much harsher conditions
than other races, such as being accused of adultery with a
white man. In Tanzania, there is a superstition that
albinism's body brings good luck, and limbs and parts of
the body are often cut off. A 17-year-old girl was having
dinner with her family when a group of gunmen cut off her leg. In some regions, the absurd
superstition

that eating the meat of patients with albinism cures Acquired Immune Deficiency Syndrome
spread, resulting in the death of many patients with albinism. In addition, albinism patients are
being hunted because of the superstition that if you put their hair in a net, it will attract fish or
bring good luck to the family. The price of one corpse of an albinism patient is around 500
million won, which is difficult to raise even in Korea.
So there are dedicated hunters who specialize in hunting albinos, and it is said that dozens of
albinos are killed. It is so serious that some African countries have even issued a policy that it is
possible to kill a hunter to protect the victim if a site that is trying to hunt a person with albinism
professionally is discovered.
Extrahuman Albinism
Albinism appears not only in humans but also in animals , but compared to humans, animals
have a very low survival rate because they have to live without being protected. Compared to
other mutations that are born, the survival rate is the lowest. In particular, the higher the species
on the food pyramid, the higher the survival rate, excluding humans, converges to 0%.
The reason why animals with albinism have a low survival rate is that carnivores have low
eyesight, resulting in lower aggression and agility compared to their own species. because it is
dangerous to Even in the case of snowy fields, UV rays can be more dangerous as UV rays are
directly reflected in the snow due to the nature of white reflecting light. That's why skiers wear
goggles.
Exceptionally, in the case of rodents such as rabbits and mice , albinism is not a problem because
they are mainly nocturnal or live in burrows. Red-eyed, white-furred rabbits are albinism species,
and some species have albinism dominant over normal rabbits, so it is easy to see such rabbits.
Of course, it is relatively easier to see than other creatures, but it is never easy to see an albino
rabbit when breeding real rabbits. Also, an albino killer whale, presumably the result of
inbreedingIn the case of , it was observed that they seemed to get along well with the group.
However, in normal cases, the odds of not surviving are higher. # Baeksa (white snake ) and
Baekrok (white reindeer) were regarded as spiritual creatures, especially in the East. Many
animals presumed to have albinism are also recorded in Samguk Sagi . However , white tigers
(white tigers ) were also considered spiritual creatures, but white tigers are not exactly albinism.
The white 'black bear' that lives in western Canada is not albinism, it is a recessive subspecies
with recessive traits matched. ( It's called luism . In the case of huskies and malamutes, etc.)
However, it is said that salmon are not wary of white, so they have an advantage in hunting
salmon and eat better than black bears.
The white elephant, which is also an albino object, is considered sacred in Buddhist countries
such as Burma , Thailand , and Cambodia .This is because the dream he had had was a dream in
which a white elephant came into his side. Because they are sacred animals, unlike other
elephants, they do not work and are treated with dignity. In ancient Thailand, when the king gave
a white elephant as a gift, the recipient could not throw it away and had to devote a huge amount
of money and time to the white elephant, and the king used this point to harass his servants who
did not like it. Therefore, the white elephant also means a small item bought and played at home,
a nuisance that is shiny on the outside and has no value in reality but cannot be disposed of. Its
actual skin color is reddish brown and turns pink when wet. In the case of Thailand, it is
managed by the royal family, and nine white elephants appeared at the former king's condolence
event in November 2016 .
There is also an albinism among humpback whales , which is called 'Migaloo'. First discovered
in 1991, migaloos are said to appear on Australia's Great Barrier Reef every June following a
school of humpback whales.
White individuals are often found among sperm whales, but this is not albinism, but bleaching
with age.
Among crows , there is a white crow. It is an example that refutes the assertion that “all crows
are black” and is also used as a basis for criticizing the fallacy of hasty generalization .
On the pet side, breeders and dealers deliberately cross breeds with recessive genes to improve
and sell albinism breeds. Like the red-eyed rabbit above, hamsters and ferrets, parrots , small
animals such as hedgehogs, snakes (white snakes), Pac-Man , clawed frogs ,[8] In addition to
Wooparoopa , there are numerous species such as guppies , selpin mollies , balloon mollies ,
Corydoras , tetras including red gobies , algae eaters , cichlids such as Discus , Oscar , and Negro
, catfish such as Clara and Genghis Khan , and polypterus . Small pets are improved into
albinism breeds and distributed in the market, and depending on whether the body color is made
only white or yellow, mixed white and yellow, or added with a tabby pattern, the albinism breeds
are also white, golden, OB , etc. The name is unique. There are many species, mainly fish, that
are improved and distributed with albinism. In addition, there is albinism among crustaceans, and
albinism in lobsters is also commonly caught. small animals ( mammals

) are generally not only normal, but albinism is also common. Albinic puppies and albinistic cats
are not sold separately by breed. Although this is not particularly albinism, it is presumed that
breeds with white hair have been improved, and since albinism itself is a genetic disease, of
course it also exists in dogs and cats.
Albinism elk found in Changwon-si, Gyeongsangnam-do in 2010 Albinism elk found in Paju-si,
Gyeonggi-do in 2015 Sad truth of albinism animals Albinism elk found in Paju -si, Gyeonggi-do
in 2016 It molts to adapt to change or is a different form of mutation than albinism. In the case of
the former, wild rabbits and wolves shed their hair in white in winter. In the case of the latter, it
is a mutation that appears with a very rare probability even in nature, and the difference from
albinism is that there is color in parts other than body hair or skin because it is not pigment
deficiency. There are not a few of these mutants among the Baeksa and Baekrok mentioned
above. It is better not to make the mistake of simply looking at a creature with a white
appearance and calling it albinism. In addition, since albinism and vitiligo are distinctly different
diseases, care should be taken not to confuse them in using the terms.Although very rare, plants
can also get albinism. However, when plants suffer from albinism, their chlorophyll is destroyed
by ultraviolet rays and cannot photosynthesize, so they die shortly after germination. Only very
rare parasitic plants or chimeras survive.[9] Artificially, it can survive only when grafted with
other plants. Redwood albinism, which is rare among plant albinisms, can survive for a long time
and can grow up to 20 m in height, due to the nature of redwood roots that intertwine and share
nutrients. Although it is more of a parasitic plant with no function in redwood forests, recent
research suggests that it protects other trees from heavy metals by rapidly absorbing and storing
them. Redwood with albinism has broken pores, so transpiration and water absorption are much
faster. Plants that are trending in food technology such as Monstera and Spathiphyllum, which
are popular these days , are sold at a much higher price than 100% green plants. As for
individuals with some degree of green mixed in, be careful as 100% albinism will inevitably die.
cause
It is caused by a defect in several genes that are needed to make melanin pigment in
melanocytes. Ocular cutaneous albinism is inherited in an autosomal recessive fashion, so
parents usually have no symptoms and 25% of their children inherit it. Ocular albinism is
inherited in a sex chromosomal recessive manner.
 
Symptom
Symptoms vary depending on which gene is abnormal. In ocular skin albinism, people are born
with varying degrees of white skin and coat. It shows a decrease in skin color that varies from
slightly whiter than normal to light brown, and the color of hair can also vary from white to
brown. Since the eye lacks pigment in the retina and iris, the pupil appears red and exhibits a
variety of ophthalmic symptoms, including glare, nystagmus, and blurred vision.
As they grow up, sunburn from the sun easily occurs like Caucasian skin, and skin cancer
prodromal (actinic keratosis) or skin cancer may occur.
 
Diagnosis
Diagnosis is based on characteristic symptoms and family history. It can be confirmed by
identifying genetic abnormalities through molecular biological tests. In skin biopsy, melanocytes
can be observed, but melanin staining is negative.
 
cure
Since it is a genetic defect, there is no cure yet. Avoid excessive sun exposure, use sunscreen,
and have regular dermatological and ophthalmological examinations for skin lesions and eye
abnormalities.