Xeroderma Pigmentosum: Children Of The Moon

Sufferers of autosomal recessive disorder (XP) are sensitive to UV light generated by the sun and develop pigmented spots, tumours and skin cancer after minimal exposure. MIMs are caused by mutations in genes that repair damaged DNA. People with XP are more likely to develop skin cancer than those without.

XP is characterized by a pronounced sensitivity to ultraviolet light and an inherited inability to repair damage caused by UV radiation from the sun. Molecular defects in XP cells lead to the development of pigment spots, tumours and skin cancer in sun-exposed skin. Unless precautions are taken to protect against sunlight, there is a risk of skin cancer and thus the occurrence of melanoma and other skin diseases.

According to the National Institutes of Health (NIH), nearly 25 percent of those affected are also involved in the central nervous system (CNS).

Xeroderma pigmentosum (XP) is a rare genetic disease characterized by a clinical phenotype that ranges from a mild, almost asymptomatic disease to severe, chronic and potentially fatal effects of UV radiation. Many patients can simultaneously suffer from other diseases such as melanoma, cystic fibrosis or multiple sclerosis (MS). Patients with XP often develop symptoms that begin very early in the sun - exposed areas of the body that lead to premature death from cancer, heart disease, stroke, kidney failure, diabetes, cardiovascular disease and other health problems.

We will focus on the eye disease associated with Laurence Moon syndrome (retinitis pigmentosa) and will distinguish between the overlapping features of XP and the already diagnosed Laurences-Moon-Bardet-Biedl syndrome. We differ in severity and frequency of the disease, see below for a detailed description of each disease and its clinical phenotype.

Retinitis pigmentosa (RP) is a term that indicates that a child suffers from a general hereditary disease that affects the light-sensitive nerve tissue lining the back of the eye.

The retina contains photoreceptor cells that convert incident light into electrical impulses that are transmitted to the brain and interpreted into visual images. Xeroderma pigmentosum is a rare disease transmitted by the skin tissue that covers the eye, which is extremely sensitive to ultraviolet light.

This means that you must have two copies of an abnormal gene for the disease trait to develop, and one copy of the normal gene for each copy.

UV light, such as sunlight, damages the genetic material (DNA) in the skin cells, making the skin very thin and causing patches of varying colour and blotchy pigmentation. Boikoketso has a very irritable and grim effect and his skin becomes very painful.

There is no cream or protection against the harsh rays of the sun, and there are no creams to protect against the harsh rays of the sun. This child was born with a very rare skin disease that makes her highly sensitive to sunlight and is described as sensitive to light.

Boikoketso is one of the South Africans diagnosed with XP, while only 50 others live with it. Although the original "XP" was chosen as the name for Microsoft's operating system, XP is actually a very rare and incurable skin disease that mainly occurs in children called Xeroderma Pigmentosum.

The nature of this incurable disease prevents skin cells from being repaired and repaired before they are damaged by the sun. In healthy people, skin and eye surfaces always repair themselves and heal after normal sunlight, which does not cause any damage. XP patients, often referred to as "children of the moon," cannot be exposed to normal sunlight and suffer from skin cancer.

X-ray and other diseases are caused by the failure of important DNA repair enzymes that normally protect against the harmful effects of UV radiation from sunlight. This can lead to accelerated premature aging in children and cause progressive neurodegenerative diseases resulting from a hereditary inability to repair ultraviolet (UV) induced DNA damage.

Although McCoy is one of 150 children in the United States who suffer from XP, a condition in which a person's body is unable to repair the damage caused by UV light, he differs from most other children. XP sufferers are known as Twilight or Moon Children, and they wear suits that Zwane calls "suits of the National Aeronautics and Space Administration" to limit exposure as much as possible. Patients are also provided with protective clothing such as caps, sunglasses and face protection to protect them from UV radiation.

The windows of his family home are stained with UV-resistant glass, as is his OSO, and he cannot go outside without a specially constructed hood to protect himself from the sun. He suffered third-degree burns because he had been exposed to sunlight at least three times in his life, most recently at the age of 12.

Cited Sources:

https://www.nairaland.com/3074237/xeroderma-pigmentosum.children-moon-photos 
https://familyconnect.org/after-the-diagnosis/browse-by-condition/laurence-moon-bardet-biedl-syndrome/123/ 
https://www.medindia.net/patientinfo/xeroderma-pigmentosum.htm 
https://www.ocregister.com/2007/11/19/profile-riley-mccoy-lives-with-xeroderma-pigmentosum/ 
https://www.iol.co.za/news/south-africa/gauteng/meet-boikoketso-the-night-child-1946781 
https://news.st-andrews.ac.uk/archive/volcanic-bug-aids-children-of-the-moon 
https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/xeroderma-pigmentosum/ 
https://www.nicklauschildrens.org/genetic/xeroderma-pigmentosum 
https://en.wikipedia.org/wiki/Xeroderma_pigmentosum