Vachiberekwa amaurosis Leber: zvinokonzera, zviratidzo uye kurapwa mamiriro

Zvirwere kuona midziyo anogara akapa murwere akawanda kuzvuzvungudza uye matambudziko. Eyes - ndiyo huru kutsinga dzinouya nenyere. With nayo, vanhu vanogona kuona nyika uye dzokuMabvazuva zvakazara pachavo vari muchadenga. Chinonyanya anotyisa zvokurwara kunoonekwa sechinhu kurasikirwa nechepakati chiratidzo. In okurapa, inowanikwa rinonzi Leber kuti amaurosis. Treatment, zviratidzo, hunokonzera chirwere - izvi zvinongova zvimwe nyaya kuti munyaya yanhasi nyaya.

Description of chirwere uye ayo dzakasiyana

Amaurosis chinhu ocular zvokurwara chikwata umo mune kukwana kana zvachose upofu. Izvi ezvinhu hakuna chokuita zvakananga nemaonero kukuvara nenyere. Chinhu dzichipfekeka mumwe. A chirwere yakakonzerwa nokuvhiringidzirwa manzwiro ari kutarisana midziyo. Izvi ndozvazvakaita apo, panguva pakuongorora ophthalmic, chiremba haaoni chero chinokosha zvarakasiya chaizvo. Panyaya iyi, murwere anonyunyuta tichinzwa kutadza kuona zvakanaka.

Amaurosis ndirwo mhando mbiri: zvinopfuura (zvinopfuura) uye vachiberekwa. nyaya yokutanga dzinoratidzwa pfupi kurasikirwa zvaakaratidzwa. Kukura chirwere, kazhinji kunokonzerwa ischemia mumudziyo pamusoro carotid tsinga. Achibatsirwa mishonga kana anozvipira chiono anodzorerwa nokukurumidza. Kana pakanyorwa zvinopfuura upofu zviratidzo ischemia mberi, kuvhiya rinoitwa. mhando iyi kushanda kazhinji kudzivirira sitiroko pavarwere.

Vachiberekwa amaurosis Leber ari kufarira n'anga uye masayendisiti. Naizvozvo, zvinokurudzirwa kuti funga zvokurwara ichi voga.

Features Leber vachiberekwa amaurosis

Nhaka chimiro chirwere, vanachiremba chokuita nemagene zvinetso rhodopsin kugadzirwa. Hazvina basa nokupfuuridzirwa mashoko achitevedza etsinga mitundu muuropi. Kana rhodopsin zvakakwana, iyo the retina yakanga yabudirira kunochinja chiedza mukati pulses izvo Mifananidzo vari akaumba vakazochinja. Maererano achangopfuura zveBhaibheri yacho, kuwedzera rhodopsin pathological muitiro uyewo kunosvika kune dzimwe nzvimbo - pematanda uye pasikweya.

Leber vachiberekwa amaurosis yokutanga nguva akanga vanotaurwa muna 1867. The musayendisiti, ainzi akazonzi chirwere, akazivisa kuonekwa huru. Izvi kunyanya upofu, nystagmus, uye chitarisiko zera makwapa pamusoro fundus. The zvokurwara iri dererei (3 nyaya pa 100 zviuru. Population). It zvakafanana inobata zvose varume uye vakadzi.

The zvikonzero

Sei pane vachiberekwa amaurosis Leber? Zvinokonzera chirwere vachiri kuzivikanwa. vanachiremba vakawanda vanoti nezvazvo nokuda kushaya pachena zvinhu specificity ruvara epithelium (rhodopsin) pamwe photoreceptors. It kunokonzerwa abnormalities yokugara zvinhu. Zvokurwara ane autosomal recessive kwadzinoita hutachiwana. It kutyiwa chete kana vabereki vose DNA aripo ari mutated Gene.

Tiri kutaura RPE-65 Gene rokutanga chromosome. On musayendisiti nhasi, kune anenge 80 akasiyana kunotouraya ayo, izvo predispose wandei siyana retinal abiotrophy. Protein encoded ne Gene iri anokonzera muviri kuti retinol ari ruvara epithelium. In pamberi majini urema akatsanangura urongwa rotsva. Somugumisiro, mashoko kwetunhu rhodopsin rinomira chinoguma chimiro zviratidzo yo chirwere.

zvekuchipatara mufananidzo

Enhaka Leber kwakaita vachiberekwa amaurosis iri dzakaonekwa mu muduku. In vana vane chirwere ichi kazhinji hapana ziso hunenge. Vane zveufende uye neapo kuvadza pachavo. Zvinokosha kucherechedza kuti upofu haasi Mhedziso pakuzvarwa. Mumakore upenyu mwana iye mberi, asi pedyo pakuyaruka inotungamirira apedzise kurasikirwa wezvakaonekwa. Zvisinei, pane kunze.

Vachiberekwa amaurosis Leber inozivikanwa nokuva anotevera zviratidzo:

1. Involuntary ziso kufamba.
2. Vadzidzi vanoita havaratidzi kuti chiedza.
3. The mwana zvakaoma zvakakwana kuti pfungwa yake anopenya rimwe chinhu. It kushanda asingadi kusiyanisa zvinhu pedyo.
4. Tarirai nguva dzose achitsauka.
5. Zvokurwara kunowanzoguma keratoconus. urema ichi apo cornea alters ari kwakajairika configuration. It anova cône serin'i.

Leber kwakaita vachiberekwa amaurosis, kazhinji, ndiko systemic zvakasikwa uye ari chokuita nezvimwe zvirwere. Vana vakadaro vane retardation mupfungwa. Ivo kazhinji murombo kana pasina kunzwa. Maperembudzi kuzadzikiswa nemamwe abnormalities pakati zvomukati.

nzira nechirwere

Nemhaka yokuwanda chirwere zvinoratidza siyana hurongwa dzimwe nguva zvakaoma nenguva vanofungidzirwa Leber vachiberekwa amaurosis. Zvinokonzera, zviratidzo chirwere ichi uye voruzhinji vabereki handizivi. Naizvozvo, havaiti pakarepo kutsvaka rubatsiro kubva chiremba.

Diagnostics iri paaivatambudza optometrist. Dzimwe nguva unofanira kubatsirwa kunze vanachiremba :. wetsinga, Angiology, etc. Kuongorora murwere rinotanga nokudzidza nhoroondo yacho. Panyaya iyi, chiremba panguva kuongororwa angabvunza kugadzirisa mibvunzo. Somuenzaniso, apo zviratidzo hosha wokutanga akazviratidza, zviitiko amaurosis vamwe vomumhuri rakazivikanwa.

Zviri fanira kuva shoma murwere nyanzvi inoongorora fundus weziso, chiratidzo macheki, unhu kutarisana dzepanguva. Kana zvakakodzera, izvozvo anorayirwa kuti MRI, uyewo kugadza wokuwedzera kubvunza ane geneticist.

kurapa Features

Leber kuti vachiberekwa amaurosis hakusi rinorapika. Zvakakodzera, maererano nedzimwe ophthalmologists vaiona kugadzwa nazvo mishonga. kosi kwakadaro kunosanganisira kushandisa vhitamini zvivako, intraocular jekiseni uye vasodilator zvinodhaka.

Kazhinji, varwere dzakadaro, apo kuramba zvakakwana azvipire maonero, chiremba prescribes magirazi. Zvisinei, kunyange nokubatsira kwavo zvakaoma chaizvo kusiyanisa pakati chiso chedu nevamwe. Child compulsorily pfekai nhoroondo panguva neuropathologist.

Vabereki, akavapindurawo, vanofanira kutaridzwa daro uko mwana anokwanisa kusiyanisa pakati kutaura nevamwe vanhu. Nokuti zvakakodzera paumbwe nechepakati tsinga zviri nani kuva kuwanda wainyatsotaura spacers. Izvi zvichaita kuti mwana kubvuma zviso, chiso chedu kutevedzera uye kuonana chinotevera hama.

The fungidziro varwere

To musi, vanachiremba havagoni ape zvakanangwa uye kubudirira kukurira amaurosis. Zvisinei, pasi pose, masayendisiti kuramba kuitisa miedzo kurapwa uye kutsvaka mushonga nokudenga. Vamwe izvi zvinoratidza migumisiro yakanaka. Somuenzaniso, apo dzakabatwa ari the retina pamusoro RPE65 Gene varwere ziso vanoratidza chinokosha kuvandudza zvaakaratidzwa.

Kana maperembudzi fungidziro, zviri kakawanda zvisingafadzi.. Inenge 95% wevarwere makore 10 pane kurasikirwa wakakwana wezvakaonekwa.