Kobadwa nako amaurosis Leber: zimayambitsa, zizindikiro ndi mbali chithandizo

Matenda a zida za zithunzi nthawi zonse kwa wodwala kwambiri za mavuto ndiponso mavuto. Maso - ndicho kwenikweni limba. Atatero, anthu akhoza kuona dziko ndi kwathunthu oyendetsa okha m'malere. The kudwala kowopsya imatengedwa imfa ya masomphenya chapakati. Mu yochizira matenda, izo zimapezeka otchedwa amaurosis Leber a. Chithandizo, zizindikiro, amachititsa matenda - awa ndi ena mwa mavuto kuti m'nkhani lero.

Kufotokozera za matenda ndi mitundu yake

Amaurosis ndi ocular kudwala wagawo mmene muli tsankho kapena okwana khungu. chikhalidwe ichi si kugwirizana ndi view mwachindunji kuwonongeka limba. Ndi zinchito wina. matenda chifukwa cha kusokonezeka kwa mitsempha ya zida za zithunzi. Izi ziri chimodzimodzi choncho pamene, pa kuunikidwa ophthalmic, dokotala saona zolakwika iliyonse yaikulu. Pankhaniyi, wodwalayo anadandaula za kulimbikira zithunzi kuwonongeka.

Amaurosis ndi mitundu iwiri: chosakhalitsa (chosakhalitsa) ndi kobadwa nako. Mlandu amakhala ndi imfa kanthawi la masomphenya. Chitukuko cha matenda, kawirikawiri chifukwa cha ischemia mu chigwa cha mtsempha wamagazi ndi carotid. Mothandizidwa ndi mankhwala kapena kudzikonda masomphenya abwezeretsedwa posachedwa. Ngati maziko zizindikiro chosakhalitsa khungu la ischemia m'kati, opaleshoni imagwiridwa. mtundu uwu ntchito zambiri kuteteza sitiroko odwala.

Kobadwa nako amaurosis Leber n'chothandiza kwambiri kwa madokotala ndi asayansi. Choncho, m'pofunika kuganizira kudwala ili payokha.

NKHANI Leber kobadwa nako amaurosis

Cholowa mawonekedwe a matenda, madokotala chikugwirizana ndi matenda majini rhodopsin kupanga. Ndi udindo kufala kwa mudziwe pamodzi mitolo mitsempha mu ubongo. Ngati rhodopsin mokwanira, diso anali bwinobwino amasintha kuwala mu nyemba zomwe zithunzi zithunzi anapanga kenako. Malinga ndi kafukufuku wa posachedwapa pankhaniyi, kuwonjezera rhodopsin pathological ndondomeko kumakhudzanso zinthu zina - ndodo ndi cones.

Leber kobadwa nako amaurosis nthawi yoyamba anatchulidwa 1867. Wasayansi, dzina lake sazidzagwiritsa matenda, kuzindikiridwa mawonetseredwe waukulu. Izi makamaka khungu, nystagmus, ndi maonekedwe a mawanga ukalamba fundus lapansi. The kudwala n'chosoŵa (milandu 3 pa 100 zikwi. Population). Mofanana amakhudza amuna ndi akazi.

The zifukwa

Chifukwa pali kobadwa nako amaurosis Leber? The zimayambitsa matenda adakali sakudziwika. madokotala ambiri amanena za izo chifukwa chosowa zinthu bwino kulunjika pigment epithelium (rhodopsin) ndi maselowa. Ndi chifukwa cha kuvutika zakuthupi chibadwa. Kudwala ali autosomal recessive akafuna HIV. Ndi kumuopa pokhapokha ngati makolo onse DNA alipo mu jini mutated.

Tikuyankhula zokhudza RPE 65 jini mu khromozomu choyamba. Pa wasayansi lero, pali pafupifupi 80 mitundu cha masinthidwe, lomwe odana angapo mitundu ya retinal abiotrophy. Mapuloteni ndiyotengera ndi mbewu izi ndi udindo kagayidwe wa retinol mu pigment epithelium. Pamaso pa chilema majini anafotokoza kwalephera. Chifukwa, kaphatikizidwe wa rhodopsin mabasi zotsatira mu maonekedwe zizindikiro khalidwe la matenda.

chithunzi matenda

Cholowa Leber a kobadwa nako amaurosis ndi wapezeka ana aang'ono. Ana ndi matenda kawirikawiri palibe bongo zithunzi. Iwo zosamveka ndi zina kuvulaza okha. Dziwani kuti khungu ndi malire akangobadwa. Pa moyo wa mwana iye m'kati, koma pafupi ndi msinkhu kumayambitsa malizitsani imfa ya masomphenya. Komabe, pali kuchotserapo.

Kobadwa nako amaurosis Leber a amakhala ndi zizindikiro zotsatirazi:

1. Musanaganizire diso kayendedwe.
2. Ophunzira anatani bwino kuwala.
3. The mwana yovuta mokwanira kuganizira maso ake pa chinthu chimodzi. Ndi pafupifupi sakhala kusiyanitsa zinthu pafupi.
4. Yang'anani nthawi zonse Amayendayenda.
5. Kudwala nthawi zambiri limodzi ndi keratoconus. chilema izi zimene diso kumasintha kasinthidwe ochiritsira. Zimakhala phirilo zooneka.

Leber a kobadwa nako amaurosis, nthawi zambiri, ndi zokhudza zonse m'chilengedwe ndipo amagwirizana ndi matenda ena. ana oterowo retardation maganizo. Iwo kawirikawiri ndi osauka kapena ayi kumva. Matendawa lowonjezera ndi kuvutika zina ziwalo mkati.

njira matenda

Chifukwa cha kuchuluka kwa mawonetseredwe matenda a machitidwe osiyanasiyana ndi zina zovuta yake amaganiziridwa Leber kobadwa nako amaurosis. Zoyambitsa, zizindikiro za matenda ambiri a makolo sindikudziwa. Choncho, nthawi yomweyo kupempha thandizo kwa dokotala.

Diagnostics ndi chinkhoswe optometrist. Nthawi zina mumafunika thandizo la akatswiri kunja :. katswiriyu, Angiology, etc. Kudzifufuza wodwalayo akuyamba ndi kuphunzira za mbiri yake. Pankhaniyi, dokotala pa Kupenda angafunse kukambirana mafunso. Mwachitsanzo, pamene zizindikiro woyamba wa matenda anaonekera, milandu amaurosis ena m'banja wakhala kuzindikiridwa.

Ndi kuvomerezedwa ndi pang'ono mtima katswiri amasanthula fundus diso, macheke masomphenya, khalidwe zithunzi reflex. Ngati ndi kotheka, izo walangizidwa m'mutu ndipo anamupeza, komanso adzaika upo zina ndi zachibadwa a.

mankhwala Features

Leber a kobadwa nako amaurosis si mankhwala. Zoyenerera, monga ena ophthalmologists ankaona Kusankhidwa kwa mankhwala yokonza. Kodi khalidwe labwino zikuphatikizapo ntchito maofesi vitamini, intraocular jekeseni ndi mankhwala vasodilator.

Childs, odwala amenewa, pokhalabe mlingo okwanira view, dokotala n'kukulemberani magalasi. Komabe, ngakhale ndi thandizo lawo zovuta kusiyanitsa pakati pa nkhope ya anthu ena. Mwana mokakamiza kuvala nkhaniyi pa neuropathologist lapansi.

Makolo nawonso, ayenera mwachindunji mtunda pa zimene mwana amatha kusiyanitsa mawu a anthu ena. Mapangidwe moyenera chapakati mantha dongosolo ndi bwino kukhala mu osiyanasiyana mwachindunji spacers. njira imeneyi kuti mwana kuzindikira nkhope nkhope kukopera ndi kulankhula ndi wotsatira wa abale.

Kuti madokotala ananena zawo kwa odwala

Pakali pano, madokotala sangathe kupereka njira onse akulimbana ndi zothandiza kuthana ndi amaurosis. Komabe, kuzungulira dziko, asayansi kupitiriza kuchita mayesero matenda ndi kupeza mankhwala konsekonse. Ena mwa awa zotsatira zabwino. Mwachitsanzo, pamene kutumikiridwa mu diso la RPE65 jini odwala diso kuwonetsa kusintha kwambiri masomphenya.

Koma madokotala ananena zawo matenda, ndi nthawi zambiri zovuta. Pafupifupi 95% ya odwala zaka 10 pali imfa wathunthu wa masomphenya.