How Do I Choose the Best Retinitis Pigmentosa Treatment?
Retinitis pigmentosa (RP) refers to a disease that affects the retina and causes retinal function to deteriorate.
Pigmented retinitis
- RP is the abbreviation of Retinitis Pigmentosa. Retinitis pigmentosa is a chronic, progressive, hereditary, dystrophic retinal degeneration. Because the prognosis of this disease is not good, the Hong Kong Oriental Daily has the title of "horrible retinal pigment lesions" and its cause is unknown. It cannot be treated and should not be detected. The subtitles of genetic offspring remind people to pay attention to the severity of retinal pigment degeneration. The Hong Kong Wen Wei Po under the headline "Retinal Eye Diseases Don't Make Night Blindness" and experts warned that under the heading of retinal pigment degeneration will unknowingly take away vision, people are warned to understand how retinal pigment degeneration is a serious problem for patients' eyes.
(RP) The meaning of retinitis pigmentosa (RP)
- Retinitis pigmentosa (RP) refers to a disease that affects the retina and causes retinal function to deteriorate.
- Director of the Department of Ophthalmology, Director of the Beijing Armed Police Second Hospital, Director of the Department of Ophthalmology Yang Chunhua, and Director of Director Zhang Zhenyi, after repeated clinical practice for nearly thirty years, adopted "Heller's artery ring and vortex vein collateral circulation to establish retinal pigment degeneration with an effective rate of 95.6%. Prof. Cai Haoran, a doctoral supervisor of Peking University's visual research center, confirmed that the electro-physiological contrast test before and after surgery confirmed that the curative effect is significant. The establishment of Heller's arterial annular vortex vein collateral circulation is based on the pathological changes of retinal pigment degeneration, and the outer choroid layer The circulation metabolism of the large blood vessels, the optic nerve meningeal vascular network, the anterior and ethmoidal regions of the optic papillae, and the retinal sector have been significantly improved and the visual function has been reversed. Early (electroretinal unextinguished) vision can be greatly improved after surgery, Widen the field of vision, and the electrogram of the visual network gradually returned to normal. In the middle and advanced patients (the retinal diagram was extinguished, the computer vision remained above 10 degrees, and the central vision was above 0.1), the vision improved slightly after surgery, and the visual field improved. Changes, a follow-up visit one year after surgery, the electroencephalogram light peak dark valley Adenby improved.
- After 27 years of intensive research and unremitting exploration, a special research group led by Director Yang Chunhua and Director Zhang Zhenyi has carried out surgical intervention for the important intermediate link of retinal pigment degeneration and choroidal microcirculation abnormality. Improved the academic argument of "establishment of collateral circulation from autologous blood vessels" in the treatment of retinal pigment degeneration. The surgical design does not damage the original vision and field of vision, and is a safe and effective surgical treatment option at this stage. In the course of basic theoretical research and clinical practice, he was supported by Professor Cai Haoran, a doctoral tutor of the Peking University School of Medicine and an expert in bioelectricity. Through strict visual physiology before and after surgery: electroretinogram (F-ERG), electrooculogram ( The comparison of the parameters of EOG and visual evoked potential (P-VEP) fully demonstrates the effectiveness of this surgery in the clinical treatment of patients with retinal pigment degeneration. According to the medical health information of the Ministry of Health, it is concluded that the treatment methods that can cause visual electrophysiological improvement in patients with retinal pigment degeneration have not been reported in the same literature at home and abroad. It is confirmed that this special medical service project is at the leading level in the field of ophthalmology at home and abroad.
- Retinitis pigmentosa is a difficult disease in ophthalmology, known as incurable disease. It is a hereditary chronic eye disease that originates from retinal dystrophy. It affects both eyes. There are about 300,000 domestic patients and about 3 million worldwide. Its clinical features are early night blindness, concentric constriction of the visual field, and finally a tubular visual field. Binocular blindness or frequent blindness occupies a considerable proportion of blindness. Retinitis pigmentosa (RP) is a hereditary rod and cone dystrophy disease. It is characterized by night blindness, reduced visual field, and fundus bone cell-like pigmentation. For decades, many basic studies in the ophthalmology field at home and abroad have confirmed that "retinitis pigmentosa is associated with reduced choroidal blood flow." The formation of night blindness and tubular visual field indicates that choroidal blood flow can no longer meet the metabolic needs of rod cells, and the area of choroidal capillaries. Corresponding decline in blood circulation function. After performing "autologous blood vessel collateral circulation establishment surgery" on more than 3,000 patients in China, Japan, the United States, Germany, Kazakhstan, Myanmar, Mongolia, the Philippines, Canada and other countries, our center confirmed that early and middle-term patients can be treated with surgery. Improve vision and widen the field of vision to varying degrees, and the total effective rate of improvement of the fundus reaches 96%; advanced patients can effectively prevent the progress of the disease and delay blindness. Basic medicine promotes the advancement of clinical medicine. The clinical practice of surgery has verified that this surgical design is consistent with the research results of ocular microcirculation theory at home and abroad. Retinal pigment degeneration is a congenital hereditary blindness disease that is primarily caused by omental choroidal dystrophy, with retinal pigment epithelium and retinal dysfunction as the main clinical manifestations. The population incidence in China is about 1/4000, with more than 300,000, and more than 3 million patients worldwide. The clinical manifestations are early night blindness, progressive narrowing of the visual field, and severe cases are tubular. Eventually, central vision declines and gradually blindness or frequent blindness occurs.
- Once the patient's conclusion of the disease (incurable disease) has caused great psychological damage to the patient, it also casts a shadow on the happy family and heals the treatment. The long medical career is exhausting, but eventually he cannot get rid of blindness. Doom. This project has achieved remarkable results through repeated clinical practice for up to 18 years, which is confirmed by contrast electrophysiological tests before and after surgery. Searched by the Institute of Medical Information of the Chinese Academy of Medical Sciences, it is concluded that the same domestic and foreign literatures have not been reported for the treatment methods that can cause ophthalmic electrophysiological improvement. Although retinal pigment degeneration is a hereditary disease, microcirculation disorders of the retina and choroid have played a vital role in the occurrence and development of the disease. The medicine of the motherland calls this disease "high wind cataract" and "high wind sparrow", which is due to lack of congenital endowment. In the 1970s, Soviet ophthalmologists proposed to improve choroidal microcirculation to treat this disease. In 1990, British ophthalmologists confirmed that retinal pigment degeneration is associated with decreased choroidal blood flow. This theory was confirmed again by Doppler tests with Yang Wenli and others at Beijing Tongren Hospital in 1999. . According to the pathological changes of retinal pigment degeneration, the center adopts the establishment of collateral circulation surgery, which significantly improves the circulation metabolism of the outer choroidal large blood vessels and the retinal fan zone, and the visual function is reversed. Breaking new ground for treating patients with retinal pigment degeneration.
- Due to the continuous improvement of surgical methods and the gradual improvement of adjuvant treatment, early patients can improve their vision after surgery, widen their field of vision, and the electroretinogram can gradually return to normal or receive normal; in the middle period, patients can improve their vision, and the visual field and electroretinogram cannot be changed. However, the light peak, dark valley, and Arden ratios of the electroencephalogram in 60% of the patients after one year improved or are close to normal; advanced patients can effectively prevent the disease from developing after surgery. The experts of the center advised that patients with retinal pigment degeneration should pay close attention to the changes of electroretinogram and computer visual field graphics once the diagnosis is made. Do not delay the treatment and cause irreversible visual function damage. So far, 550 patients have been successfully treated and all have received good results. Among them, 26 patients were observed for 5-15 years. The long-term effect was significantly better than the short-term effect. No complications were found. Once patients with retinal pigment degeneration are diagnosed, they should pay close attention to the changes in electroretinogram, computer vision, and fundus, and perform surgery in the early and middle stages to avoid delaying the treatment and causing irreversible visual function damage, so as to strive for better efficacy. All medical staff in our center will make unremitting efforts to provide the best possible medical services for patients with retinal pigment degeneration.
- Retinitis pigmentosa is one of the serious eye diseases that cause blindness today. Statistics from the American ophthalmology community show that in the 20--40 age group, retinal pigmentosis is the main blinding eye disease. According to rough statistics, there are about 1.5 million patients with retinal pigment degeneration around the world. This amazing number does not decrease year by year, but increases day by day. Isn't this enough to attract the attention of the world! In the early stages of retinal pigment degeneration, there are only symptoms of night blindness, which does not affect work, study and normal life at all, and the disease is unknown Impaired vision without realizing it. Especially for children in early childhood, it is more difficult to detect early. The retinal structure is precise and the function is complicated. It is the intraocular tissue of the posterior segment of the eyeball that cannot be seen with the naked eye. In 1851, Helmhotz invented the ophthalmoscope, and the fundus changes of retinal pigment degeneration were revealed. Despite this, treatment is still empty talk. Only in the 1970s did retinal pigment degeneration attract the attention of western developed countries. This disease was found in China in the early Qing Dynasty, and was called "sparrow cataract" at that time. As the name suggests, it is just right. The name of the disease is good, and the basis of dialectical treatment is also given. For example, it is mainly based on strengthening the spleen and replenishing qi, adding spleen and promoting blood circulation prescriptions, and cooperating with acupuncture, massage, qigong and nourishing drugs. currently using.
- Medical genetics is the main component of human genetics. It is a marginal science combining genetics and medicine. It explores the relationship between the occurrence and development of human diseases and genetic factors to provide the scientific basis for diagnosis, treatment, and prevention of genetic diseases. So as to contribute to improving the quality of hot mouth. With the continuous development of medicine, people's living standards continue to improve, most infectious diseases have been controlled, and some have disappeared. Some chronic non-infectious diseases have gradually increased and become a prominent problem in modern medicine. So far, people have realized that hereditary diseases have exceeded With 5,000 species, it poses a great threat to human life and health. However, with the rapid development of modern high technology, the research of basic medicine, especially immunology and molecular biology and genetics, has also advanced in depth and breadth. Cytogenetics and molecular genetics, which are studied from the cellular level and the molecular level The development is more rapid, making genetic diagnosis and gene therapy of some hereditary diseases more likely. For example: after taking out cells from a patient, genetically modifying them, and then reinjecting them into the body, the abnormal genes are artificially changed to achieve the purpose of preventing and treating genetic diseases; a research institution at Boston University in the United States injected a gene that controls the secretion of vascular endothelial growth factor This gene can instruct the human body to grow new blood vessels. Achievements in molecular genetics research and gains in manipulating human genetic combinations have made it possible to apply recombinant DNA technology to treat diseases. Although this genetic engineering is currently in the exploratory and experimental stage, it is expected that humans will be The sequence of 100,000 genes on the chromosome was figured out, and a complete human genome map was drawn-"The Blueprint of Life." I believe this is a directional and essential measure. With the advancement of immunogenetics and surgery, the prospects for some difficult-to-treat genetic diseases, such as the treatment of retinal pigment degeneration, are encouraging. The French poet Goethe said, "All I have to do is to reach out and harvest the crops that others have planted for me." What I do is to sort out the "fruits" that others have harvested and dedicate them to the reader. . Due to our limited level, less experience, and lack of information on hand, there will be many mistakes. I would like to thank your fellow eye doctors and readers for corrections. We are grateful for this. The purpose of our establishment of this scientific research website is to call on all sectors of the society to pay attention to the genetic disease of retinal pigment degeneration, to provide as much support and assistance to patients as possible, and to awaken people's understanding of retinal pigment degeneration. At the same time, I call for fellow ophthalmologists to carry out scientific research collaborations and website exchanges so that more RP patients can be effectively controlled and satisfied with their treatment. If the patients with retinal pigment degeneration can get some inspiration from our website, summon the courage of life, and realize that the colorful world will not leave you, then we will be very pleased. There are methods in the literature to try vasodilators, vitamins A and B1, tissue therapy, various hormones, Chinese herbs, acupuncture and other methods, or to avoid rapid deterioration of visual function.
Retinitis pigmentosa
- The retina is a precise thin layer of tissue located inside the eyeball. It contains multiple layers of light-sensing cells called "photoreceptors," which are connected to the brain through the optic nerve.
- Pigmented retinitis
- Below the retina is retinal pigment epithelium (RIPE). Retinal pigment epithelium supports the work of retinal photoreceptors.
- There are two types of photoreceptor cells on the retina: cone cells and rod cells.
- The cones are concentrated in the central part of the retina (called the macular area), responsible for central vision and distinguishing the colorful.
- Rod cells are distributed around the macula and are responsible for peripheral vision and scotopic vision.
- Both cone cells and rod cells convert light stimuli into electrical impulses, which are transmitted to the optic nerve through nerve cells, and then to the brain via the optic nerve to "see" the actual image.
- RP patients' photoreceptor cells gradually degenerate and eventually lose function.
Retinitis pigmentosa
- Primary pigmentary degeneratio of the retina has historically been called retinitis pigmentosa. It is a relatively common type of blanket-retinal degeneration. Under the Ministry of
- Pigmented retinitis
- In some patients, retinal pigment degeneration is dominant. As long as one of the parents carries the disease-causing gene, the child will develop the disease. There are also some patients with retinal pigment degeneration in a chain-linked inheritance, only the mother with the disease-causing genes, children will develop the disease. Other cases are accompanied by hearing loss. This type of retinal pigment degeneration is more common in men.
- Some photoreceptor cells (rod cells) in the retina are responsible for vision in low light. If the rod cells gradually degenerate, the patient's vision is significantly reduced in night light (night blindness). Symptoms of night blindness often appear in childhood, and progressive peripheral vision loss may occur over time. In advanced cases, only a small central field of view (tubular field of view) and a narrow peripheral field of view may remain.
- Through ophthalmoscope, the doctor can find some special changes in the retina with diagnostic value. There are also several tests that can help with further diagnosis. Examination of family members can establish a genetic model.
Etiology of pigmented retinitis
- This disease is a hereditary disease. There are three hereditary modes: autosomal recessive, dominant and sexually linked recessive. Autosomal recessive heredity is the most; dominant is second; sex-linked recessive heredity is the smallest. Autosomal dominant genotypes are currently believed to have at least two loci, located on the short arm of chromosome 1 and the long arm of chromosome 3. Sex-linked genetic genes are located in the first wall and second wall of the short wall of the X chromosome. Regarding the pathogenesis, in the past 20 to 30 years, there have been some clues to the valve. According to the examination data of electron microscope, histochemistry, electrophysiology, fundus angiography and other examination data, it is speculated that the occurrence of this disease is mainly due to retinal pigment epithelial cells phagocytosis and degradation of digestive function of the extracellular disc membrane.
- Pigmented retinitis
- In terms of immunology, recent studies have found that patients with this disease have abnormal humoral immunity and cellular immunity. There are activated T cells, B cells and macrophages in the vitreous body, and retinal pigment epithelial cells express HLA-DR antigen, which is not the case in normal people. Kind of performance. At the same time, it is also found that patients with this disease have autoimmune phenomena, but there is no sufficient basis for whether the disease has autoimmune diseases. In terms of biochemistry, it is also found that patients with the disease have autoimmune phenomena, but there is no sufficient basis for whether the disease has an autoimmune disease. In terms of biochemistry, abnormal lipid metabolism was found in patients with this disease. Lipofuscin particles were accumulated in the retina; trace elements such as zinc, copper, selenium, and enzyme metabolism were also abnormal. In summary, there may be many different pathogenesis of this disease.
Pathological changes of retinitis pigmentosa
- Clinically obtained specimens were all advanced cases. The main changes seen under the light microscope are the progressive degeneration of the retinal neuroepithelial layer, especially rod cells, followed by the gradual atrophy of the retina from the outer to the inner layer of tissue, accompanied by glial hyperplasia. The pigment epithelium also undergoes degeneration and hyperplasia, showing loss or accumulation of pigment, and migration to the inner layer of the retina. The retinal vessel wall is thickened with hyaline degeneration, and even the lumen is completely occluded. Choroid blood vessels can harden to varying degrees, and capillaries disappear completely or partially. The optic nerve can completely atrophy, and there is often a glial hyperplasia on the optic ribs, forming a membrane block, which is connected to the glial membrane in the retina. The waxy yellow color of the optic disc seen under the ophthalmoscope is generally considered to be related to this.
Clinical manifestations of retinitis pigmentosa
Symptoms and functional changes of pigmented retina
- Night blindness: The earliest symptoms of this disease often begin in childhood or adolescence, and often occur before visible changes in the fundus. It starts lightly and gradually increases with age. Very few patients may have no night blindness in the early stages.
- Pigmented retinitis
- (3) Visual field and central vision: There are circular dark spots in the early stage, and the position is consistent with the lesions in the equator. Later, the circular dark spot gradually expanded toward the center and the periphery to form a tubular field of vision. Central vision is normal or near normal in the early stage, gradually decreases with the course of the disease, and finally completely blind.
- Visual electrophysiology: ERG has no response, especially the disappearance of b-waves is a typical change of the disease, and the change is often earlier than the fundus. EOG LP / DT is significantly reduced or extinguished, even in early stages, when changes in visual field, dark adaptation, and even ERG are not obvious, it can be detected. Therefore, the diagnosis of EOG is more sensitive than that of ERG.
- Color vision: Most patients have normal color vision during childhood, and then gradually become abnormal. Typical changes are blue blindness and less red and green vision disturbances.
Typical changes seen in fundus examination of pigmented retinitis
- Although the night is early blinded, the fundus can be completely normal. With the progress of the disease, the fundus changes gradually. Typical changes are:
- 1) Retinal pigmentation: Beginning at the equator, the pigments are small dots with protrusions, and then increase and become larger. They are osteocyte-like, sometimes irregular lines, and are arranged in a wide and narrow circular arrangement around the equator. The pigment is mostly located near the retinal blood vessels, especially in front of the veins. It can cover part of the blood vessels, or be distributed along the blood vessels, and is more dense at the branch of the blood vessels. Later, the pigmentation gradually expanded from the equator to the posterior pole and the periphery, and finally covered the entire fundus. At the same time, the retinal pigment epithelium was depigmented, exposing the choroidal blood vessels to a leopard-like fundus. The late choroidal blood vessels are also hardened with yellow-white stripes. The vitreous body is generally clear, and occasionally a few dot-like or linear turbidity is occasionally seen.
- 2) Retinal vascular changes: Consistent stenosis of the blood vessels, which worsens as the disease progresses, especially the arteries. In the late stage, the arteries are thinly lined, and after a certain distance from the optic disc, they are illegible and disappear, but they do not change from the white line or the white sheath. 3) Fluorescence fundus fundus angiography: There is no fluorescent area in the background fluorescence, suggesting that the choroid capillary layer is atrophic. Retinal blood vessels may be occluded, and sometimes mottled fluorescent spots in the posterior pole or peripheral areas can be seen.
Special clinical types of pigmented retinitis
- Monocular primary retinal pigment degeneration: very rare. The diagnosis of this type must be one eye with the typical changes of primary retinal pigment degeneration, and the other eye is completely normal (including electrophysiological examination), and can not be determined after more than five years of follow-up. This type of disease occurs in middle age and generally has no family history.
- Quadrant primary retinal pigment degeneration: Rarely. The characteristic is that the lesion only involves the same quadrant of both eyes, and is clearly demarcated from the normal area. Corresponding visual field changes, better vision, ERG is low wave. Fluorescence imaging showed that the lesion area was larger than that seen under the ophthalmoscope. This type is often sporadic, but also reports of autosomal dominant, recessive, and sex-linked recessive inheritance.
- (3) Central or paracentric primary retinal pigment degeneration: also known as reverse progressive retinal pigment degeneration. From the beginning, there is vision loss and color vision impairment. Fundus examination showed atrophic lesions of the macula, osteoblast-like pigment accumulation, and low ERG or unrecordable. In the early stage, cone cell damage was the main factor. In the late stage, the peripheral retina is involved and vascular changes occur.
- Non-pigmented retinal pigment degeneration: It is a kind of examination with various symptoms and visual function of typical retinal pigment degeneration. There are also changes under the ophthalmoscope, such as dimness of the entire fundus, thinning of retinal blood vessels, and waxy atrophy of the late optic disc. There is no pigmentation, or only a few osteocyte-like pigmentation spots appear in the surrounding fundus. . Some people believe that this type is an early manifestation of pigment degeneration, and typical pigments will still appear after the disease develops. Therefore it cannot constitute a separate clinical type. But there are also those who have no pigment changes. This type of heredity is the same as typical pigment degeneration, with dominant, recessive, and sex-linked recessive inheritance three types.
Differential diagnosis of pigmented retinitis
- According to the above history, symptoms, visual function and ophthalmoscope, the diagnosis was not too difficult. But when distinguished from some congenital or acquired choroidal retinal inflammation, secondary retinal pigment degeneration should be distinguished.
- Fetal fundus lesions caused by congenital syphilis and pregnant women in the third month of pregnancy
- Pigmented retinitis
- Acquired syphilis and certain acute infectious diseases (such as smallpox, measles, scarlet fever, mumps, etc.) can occur with chorioretinitis. Fundus changes after the inflammation subsides, sometimes similar to primary pigment degeneration. When applied from medical history, serological examination, large and deep pigmentation of the fundus, formation of irregular (non-osteocyte-like), choroidal retinal atrophy, optic disc atrophy was gray-white (not waxy yellow), light night blindness, etc. Identification.
Retinitis pigmentosa treatment
- There are methods in the literature to try vasodilators, vitamins A and B1, tissue therapy, various hormones, Chinese herbs, acupuncture and other methods, or to avoid rapid deterioration of visual function.
- 1. The selection of light-shielding spectacle lenses can accelerate the degeneration of the extracellular segment of the visual cells, so you must wear light-shielding glasses. The color of the lens should theoretically be reddish-purple with the same hue as the opto-red, but it is not suitable for beauty use gray, 0 to 1 for cloudy or indoor use; 2 to 3 gray lenses for sunny or strong light. Dark black sunglasses are not suitable. Green lenses are disabled.
- 2. Avoid excessive mental and physical tension. When catecholamine increases in body fluids, the choroidal blood vessels constrict and become hypoxia, which will increase the degeneration of visual cells. China's traditional qigong (static work), which can use its own will to accelerate the activity of the cerebral cortex and various organs of the body, if sustained, may be beneficial in preventing the rapid deterioration of visual function of the disease.
- 3. Supplement Lutein:
- Lutein is widely distributed in normal human retinas and has a protective effect on the retina. It is an important component of retinal tissue. Lutein has excellent ability to oxidize, resist light damage, and nutritional effects of visual cells. It can prevent oxygen. Damage of free radicals and harmful light to retinal visual cells and pigment epithelial cells, and increase the activity of visual cells. The human body cannot synthesize lutein itself, which is mainly derived from external intake. Once lacking, various retinal diseases will occur. Almost all retinopathy is related to the lack of lutein.
Retinitis pigmentosa complications
- Postpolar cataract is a common complication of this disease. It usually occurs in the late stage, and the crystal is cloudy and star-shaped. It is located in the subcortex of the posterior capsule, which progresses slowly, and finally can cause the entire crystal to be cloudy. About 1% to 3% of cases are complicated by glaucoma, most of which are wide-angle and angle-closing are rare. Some people study from a statistical perspective that glaucoma is associated with the disease rather than a complication. Myopia is associated with about 50% of cases. Myopia is more common in patients with autosomal recessive and sexually linked recessive inheritance. Also found in other members of the family. Deaf-mute patients with this disease also accounted for 19.4%. Corti organs of the retina and inner ear are derived from neuroepithelium, so the progressive degeneration of both may come from the same gene.
- Pigment degeneration and deafness can occur not only in the same patient, but also in different members of the same family, but they do not seem to originate from different genes and may be caused by the same gene's multi-direction. This disease can be accompanied by other genetic diseases. The more common one is the Laurence-Moon-Bardt-Biedl syndrome, which affects the pituitary region and the retina at the same time. The typical person has five components: retinal pigment degeneration, genital dysplasia, obesity, multi-finger (toe) and mental retardation. The syndrome occurs early in development and has significant clinical manifestations around age 10 (or earlier). The five components are not possessed and are called incomplete. In addition, the disease still has a congenital eye or other organs complicated or accompanied by disease, rare.
Retinitis pigmentosa prognosis
- Patients with recessive inheritance of this disease have early onset, severe illness, rapid development, and extremely poor prognosis. By 30 years of age, visual function has been highly poor, and by the age of 50, nearly blind. Dominant heredity patients, on the other hand, occasionally develop to a certain level and tend to stand still, so the prognosis is relatively better than recessive heredity. So wait until barely able to go to school and employment opportunities. Those with recessive inheritance of this disease often have a history of close relative smoking. Banning of close relative smoking can reduce the incidence of this disease by about 22%. In addition, patients with recessive inheritance should try to avoid marrying people with family history of the disease, let alone marrying people with this disease. Patients with dominant inheritance have a 50% risk of developing the disease in their children.