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概述
视网膜色素变性（Retinitis Pigmentosa，RP）是一种罕见的眼部遗传疾病 that causes progressive vision loss:
症状
RP 会导致视网膜细胞随着时间推移而损坏，从而导致以下症状：
- 夜间视力减退（夜盲）
- 视野减退，导致管状视野
- 对强光敏感
- 色觉丧失
Progression
Symptoms usually begin in childhood and worsen over years or decades. Central vision is eventually affected, making it difficult to read, drive, or recognize faces.
Causes
RP is usually caused by genetic changes passed down from parents to children. Other causes include some medicines, infections, and eye injuries.
Treatment
There's no cure for RP, but medications can help treat complications. A medical professional can diagnose RP and manage the condition to improve symptoms. Retinitis Pigmentosa | National Eye Institute
Age
问 At what age does retinitis pigmentosa start?
答 Symptoms of RP usually develop between the ages of 10 and 30, although some people experience symptoms during childhood. Symptoms vary depending on what part of the retina is affected. RP is a progressive disease, but the rate at which vision deteriorates varies for each person.
https://www.ucsfhealth.org/conditions/retinitis-pigmentosa
Progression time
问 How long does retinitis pigmentosa progress?
答 Retinitis pigmentosa is a disabling disease that is currently incurable. It typically starts at the early teenage years and progresses to severe visual impairment during the 4th and the 5th decade.
https://www.nature.com/articles/eye2016315
Advanced symptoms
问 What are the advanced symptoms of retinitis pigmentosa?
答 Later retinitis pigmentosa signs and symptoms may include:
- Having a sensation of twinkling or flashing light.
- Having tunnel vision (only central vision).
- Being sensitive to or uncomfortable in bright light (photophobia).
- Losing the ability to see color.
- Having very low vision.
https://my.clevelandclinic.org/health/diseases/17429-retinitis-pigmentosa
Severity
问 How serious is retinitis pigmentosa?
答 Retinitis pigmentosa symptoms usually begin in childhood or adolescence. However, each person may experience symptoms differently. Some people with the problem have a slow, very progressive loss of eyesight. Others lose their eyesight much more quickly and severely.
https://www.hopkinsmedicine.org/health/conditions-and-diseases/retinitis-pigmentosa
Cure
问 Is there a cure for retinitis pigmentosa?
It is caused by the death of cells that detect light signals, known as photoreceptor cells. There is no known cure for RP, and the development of new treatments for this condition relies on cell and gene therapies.
Prognosis
问 What is the prognosis for retinitis pigmentosa?
Retinitis pigmentosa (RP) is an inherited retinal dystrophy leading to progressive loss of the photoreceptors and retinal pigment epithelium and resulting in blindness usually after several decades.
https://www.orpha.net/en/disease/detail/791
Stages
问 Stages of retinitis pigmentosa？
Typical form
- In the early stage, night blindness is the main symptom. ...
- In the mid stage, the clinical picture is complete. ... -- In the end stage, patients can no longer move autonomously, as a result of peripheral vision loss (classical tunnel vision), with few degrees of remaining visual field around the fixation point.
https://pmc.ncbi.nlm.nih.gov/articles/PMC1621055/
Vision
问 What does a person with retinitis pigmentosa see?
Most forms of retinitis pigmentosa affect the rod cells. This causes night blindness and poor peripheral vision (tunnel vision). When the cone cells are affected, central vision and color vision are affected. The vision loss is usually gradual, and progresses over years.
https://uvahealth.com/services/eye-care/retinitis-pigmentosa
Most likely to get
问 Who is most likely to develop retinitis pigmentosa?
RP is typically diagnosed in children, adolescents and young adults. It is a progressive disorder. The rate of progression and degree of visual loss varies from person to person.
https://www.fightingblindness.org/diseases/retinitis-pigmentosa
Rarity
问 How rare is retinitis pigmentosa?
Retinitis pigmentosa is one of the most common inherited diseases of the retina (retinopathies). It is estimated to affect 1 in 3,500 to 1 in 4,000 people in the United States and Europe.
https://medlineplus.gov/genetics/condition/retinitis-pigmentosa/
https://www.msdmanuals.cn/professional/eye-disorders/retinal-disorders/retinitis-pigmentosa
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