retinal pigment degeneration overview >

　　Of rhegmatogenous retinal pigment degeneration (primarypigmentarydegeneratiooftheretina) in history has called retinitis pigmentosa, retinitispigmentosa). It is a more common carpet layer-retinal degeneration. According to some areas in China, the group survey data rates of about 1/3500. Retinal pigment degeneration is a rare genetic eye disease. The performance for chronic, progressive retinal degeneration, finally can lead to blindness.

　　Some patients retinal pigment degeneration for dominance heredity, both parents as long as there is a party with virulence genes, kids will come on. There are some patients retinal pigment degeneration for chain sex, only with genetic mother virulence genes, the children will come on. Another some cases at the same time with hearing impairment, this type of retinal pigment degeneration in men.

　　Some of the retina (depending on the stem cells photoreceptor cells) shall be responsible for the AnGuang vision. Depending on the stem cells if gradually degeneration, patients in AnGuang environment (night blindness is most noticeable decline in sight). Night blindness is most often in childhood that appear symptoms, over time, can appear progressive vision loss around. In a late cases, can only remaining a small center vision (tubular vision) and very narrow surrounding vision.

　　Through the ophthalmoscope check, doctor can be found on the retina has certain special change diagnostic value. There are also several items inspection can help further diagnosis. For family members can be set up to check the genetic pattern.
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　　 retinal pigment degeneration treatment measures

　　A trial in the literature vasodilators, vitamin A and B1, organization therapy, all kinds of hormone, Chinese herbal medicine and acupuncture methods, or can avoid visual function rapidly deteriorating.

　　1, shading of the glass lens selection may accelerate cells depends on the strong light, so must wear section degeneration with shading glasses. The color of the lens in theory, should use and apparent red with tonal red purple, but anything with gray, cloudy or beauty indoor use 0 ~ 1; Sunny or light in 2 ~ 3 under the grey lens. Deep black sunglasses doesn't fit. Green lens disabled.

　　2, avoid spirit and body overwrought excessive tension within the body fluids catecholamine (catecholamine) increase choroidal vascular therefore, shrink and low oxygen (hypoxia) state, the cells degeneration intensified. Depending on the Our traditional qigong (the static reactive), with their own will to high speed the cerebral cortex and the body organs activities, such as perseverance, to prevent the disease of visual function rapidly deteriorating may beneficial.


　　 retinal pigment degeneration etiology

　　The disease for genetic disease. The genetic means has autosomal recessive, dominant and recessive sexual chain of three. To most autosomal recessive; Dominant take second place; Sex chain recessive heredity minimum. Now think autosomal dominant type has at least two seats in the first gene chromosome with the third short arm of chromosome long arm. Sex chain genetic is located in the X chromosome short wall area area and two area area. About the mechanism, nearly 20 ~ 30 years, some of the disc clues. According to the electron microscope, chemical, electric physiology, organization eye examination such as blood vessels fluorescence imaging, think of the disease that material happened, mainly due to the retina pigment epithelium cells looked at the film festival plate cells engulfed, digestive function, the plate film recession disintegrating residues, rules form a layer of obstacles, interfere with nutrients from the rotation of the retina choroidal to, cause the cells of the progressive on malnutrition and degeneration and gradually disappear. This process has set up a file in a primary retinal pigment of rat retina RCS confirmed. As for pigment epithelium cells ingest function failure reason, it is not clear. May and abnormal gene, some or some of the lack of relevant enzymes. In immunology, in recent years, studies found that patients with the disease humoral immune cells, immune are abnormal, vitreous has activated T cells, B cells and macrophages, retinal pigment epithelium cells express HLA antigen, normal-DR, no such performance. It also found that patients with the disease has its own immune to this phenomenon, but if there is no enough autoimmune conditions based. In biochemistry, also found that patients with the disease has its own immune to this phenomenon, but if there is no enough autoimmune conditions based. In biochemistry, found that patients with the disease lipid metabolic abnormalities, retina have fat with brown particle accumulation; Zinc, copper, and trace elements like selenium and metabolic enzymes also have exception. To sum up, the disease may exist DuoZhong different pathogenesis.


　　 retinal pigment degeneration pathological changes

　　The specimens are clinical get late cases. Optical microscope have seen major change to the retina neural stem cells in the cortex, especially the progressive degeneration, followed by the retina by extroversion inside the layer of tissue gradually atrophic, with hair nerve tissue hyperplasia. Pigment epithelium also produces denaturation and hyperplasia, visible disfiguring or accumulate, and to the retina lining migration. Retinal blood sample degeneration and glass wall happen thickening, and even the lumen totally occluded. Choroidal vascular can have different degree sclerosis, capillary completely or partially disappear. The optic nerve can complete atrophy, depending on the rib usually glial hyperplasia, form of membrane, and retina tissue is connected to the film. Ophthalmoscope see videodisc wax yellow, general and think about this.


　　 retinal pigment degeneration clinical manifestations of

　　1, symptoms and functional changes

　　(1) for the disease appears the earliest night blindness is most: the symptoms, often begins with children or teenagers, and occurs in eye visible change have before. At the beginning of the light, along with the age and gradually increase hyperplasia. A few patients can also be no night blindness is most early colon.

　　(2) the dark adapt to check: early cone cells is normal, stem cell function function fell, the stem cells will not increase threshold value curve, cause light color difference to narrow. Late stem cells function is lost, the cone threshold value increases, the formation and high single-phase curve.

　　(3) field of vision and central vision: there is early ring the dark spots, the position and the equator is the lesions. After the dark spots to the center and ring around slowly expand and become tubular vision. Central vision normal or close to normal, early with disease development and gradually decreases, finally completely blind.

　　(4) visual electrophysiology: no reaction, especially ERG b wave this disease is typical of the disappeared, and the change in the fundus changes often appear change. Early EOGLP/DT obviously decrease or put out, even if in the early days, when the field of vision, dark adaptation, even ERG, change is unclear, already can be found out. So in this than ERG EOG diagnosis more sensitive.

　　⑸ color: most patients childhood faded after normal color, abnormal. Typical for blue color blindness, change the red and green barriers less sleep.

　　2, the fundus examination have seen the disease early for night blindness is most, although can be completely normal eye. After the disease progresses and application with gradually appear fundus changes. The typical change are:

　　1) the retina pigmentation: the equator, the pigment began in the sudden little point, have increased and then change, a bone cells, sometimes a sample irregular line around the equator into shape, the width of the annular ranging alignment. Pigment is located in the retina, blood vessels near the more special in the front of the vein, can cover part of the blood vessels, or vascular distribution in blood vessels, the more densely. In branch Later, pigmentation since the equator and around the back of a gradually expanded, finally be full of the eye. At the same time, the retina pigment epithelium disfiguring, exposed a leopard grain shape choroidal vascular and travelers. Choroidal vascular sclerosis also late, a yellowish-white stripes. Vitreous generally clear, sometimes I see a patch or linear cloudy.

　　2) change: retinal blood vessels consistency with progression of narrow, and worse, especially in the artery for significant. In a late, artery to fine line, in a distance away from the DVDS that is difficult to recognize and like disappeared, but the same from the white line, and also have no white scabbard around the bag. 3) fluorescence vascular eye seen imaging: background fluorescence without fluorescence area, hints of choroidal capillary layer atrophy. Retinal vascular occlusion can have, and sometimes visible to the posterior polar or peripheral part of mottled spot. Fluorescent

　　3, special clinical types

　　(1) the monocular sex of rhegmatogenous retinal pigment degeneration: very, very rare. For this type of diagnosis, must have of rhegmatogenous retinal pigment is a typical degeneration changes, and the other eye totally normal (including electricity physical check), after 5 years of follow-up is still more than did not come on, to be sure. This type of patients in middle age adults, general without familial history.

　　(2) the quadrant sex of rhegmatogenous retinal pigment degeneration: what is rare. Features for lesions involving only eyes the same quadrant, and a clear demarcation between the normal area. The view of the corresponding change, eyesight is better, for low wave ERG. Fluorescence imaging showed lesions area than ophthalmoscope see range. This type of often volatile, but also for autosomal dominant and recessive and sex chain recessive heredity report.

　　(3) of central or in the mind of rhegmatogenous retinal pigment degeneration. Also called inverse retinal pigment degeneration of progressive. Is the beginning of the vision loss and color barrier. Fundus examination visible macular lesion is shrinking, bone cells, a sample pigment accumulation ERG low wave or can't record. Early in the cone damage is given priority to, to stem cell damage later. Involvement of peripheral retinal late, and the emergence of vascular change.

　　(4) no pigment retinal pigment degeneration: a typical retinal pigment degeneration the symptoms and visual function seen the check. Ophthalmoscope also have the fundus retinal blood thinner, gray, later the DVDS wax yellow atrophy, etc, no pigmentation change, or just in the peripheral retinal appear a few bone cells, is called spot sample pigment without pigment retinal pigment degeneration. Some people think that the type of pigment degeneration is early findings, still can appear after illness development typical of the pigment. So do not make a single clinical types. But also have always the change without pigment. This type of genetic way and the typical pigment degeneration, have the same dominant and recessive, sexual chain recessive heredity three type.


　　 retinal pigment degeneration differential diagnosis

　　According to the history, symptoms, visual function and ophthalmoscope check see, diagnosis and without too much difficulty. But when the first born with some choroidal retinal tile or inflammation of the secondary retinal pigment after degeneration note identification.

　　Congenital syphilis and pregnant women in pregnancy 3 months after diagnosis with wind caused fetal eye disease, after birth seen eye with the disease are almost identical, ERG, views of visual function test results also hard to distinguish. Only in the determination of the parents of children of the serum syphilis reaction negative and mother during early pregnancy without rubella, ability is history diagnosis of primary pigment degeneration. When necessary to longer follow-up, congenital secondary pigment degeneration in which already exist, born illness static.

　　Syphilis and some acute infectious tile (such as smallpox, measles, mumps, scarlet fever etc), all can happen choroidal retinal infection, inflammation disappeared after the fundus changes, sometimes with primary pigment similar degeneration. When from the history, serologic test and fundus pigmentary spot big and position deep, form irregular bone cells (the samples), a retinal fovea atrophy spot, the DVDS atrophy tan-white (not wax yellow), night blindness is most to a lesser degree, so as to identify.


　　 retinal pigment degeneration complications >

　　Polar cataract after this disease is common complications. Commonly occur in late, crystal star in a muddy, were slow in the cortex, and, finally, can cause the whole crystal cloudy. About 1% ~ 3% of cases and glaucoma, many for the wide Angle, closed Angle sex rare. Someone from the statistics, the Angle that glaucoma is comorbid rather than with the disease complications. About 50% of cases with myopia. Myopia in autosomal recessive and sexual concatenate recessive heredity patients. But also in other members of the family. Deaf disease and with this person is as high as 19.4%. The retina and inner ear Corti organs are from the nerve, so both epithelial progressive degeneration may come from the same genes.

　　Pigment degeneration and deafness can happen at the same patients not only, also can occur in the same family respectively the different members, but not, it seems, both from different genes, and may have caused by the same gene for diversity. The disease can comorbid other genetic disease, the more common for pituitary area and also between the greater harm of Laurence-finds Bardt-Biedl syndrome.- The retinal pigment with typical degeneration and reproductive organs dysplasia, obesity, many fingers (toe) and intelligent defects five components. The syndrome appears in the early development, around 10 years old (or earlier) there has been a remarkable clinical manifestations, five part is not have, do not say the complete form. In addition, the disease, we are still a eye or other organs, concurrent or comorbid conditions, rare.


　　 retinal pigment degeneration prognosis >

　　The patient is sick recessive heredity disease, heavy early, and quick development is very poor prognosis. With 30 years old or so are highly visual function when bad, to the 50 or so close to completely blind. Dominance heredity patients, occasionally contrast also have development to a certain person, so after still tend to be relatively better prognosis recessive heredity type. So wait until normal barely learned and employment opportunities. The disease of the person, the recessive heredity fathers brought forth many Cousins united smoke history, banned Cousins united can make the loss occurred smoke about 22%. In addition, recessive genetic patients should try to avoid the family history of the disease and get married, couldn't be more with the disease also the marriage. Dominance heredity patients, its children the risk of the disease occurred is 50%.

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