viteliform macular dystrophy

viteliform macular dystrophy anedi kramarahita autosomal dominant kanti rugmata, idhi pragatiseela drushti nashtaniki kaaranamavutundi. eee rugmata retina, mukhyamgaa macula anie piluvabade retina kendraaniki sameepamlo unna oka chinna praantamlooni kanaalanu prabhaavitam chestundi. macula padunaina kendra drushtiki baadhyata vahistundi, idhi chadavadam, driving cheyadam mariyu mukhalanu gurtinchadam vanti vivaranaatmaka panulaku avasaram. eee paristhiti pasupu (ledhaa narinja), koddigaa ettaina, guddu pacchasona (latin "vitellus") maadirigaane gundrani nirmaanaalatoo vargeekarinchabadutundi.
janyusaastram.
"uttama vyaadhi" anedi autosomal aadhipatya namoonaalo vaarasatvamgaa vastundi, antey prati kanamlo marchabadina janyuvu yokka oka coppy rugmataku kaaranamavutundi. chaalaa sandarbhaalalo, baadhita vyaktiki eee paristhiti unna tallidandrulalo okaru untaaru.
vayojana-prarambha vitreliform macular dystrophy yokka vaarasatva namuunaa khachitamgaa autosomal aadhipatyam kaligi untundi. ayithe, chaalaa mandhi baadhitulaku vaari kutumbamlo eee rugmata charitra ledhu mariyu koddi sankhyalo baadhita kutumbaalu matrame nivedinchabaddaayi. endukante paristhiti yokka chochukupovadam asampuurnamgaa undhi; anduvalla, oka vyakti utparivartana yugmavikalpam yokka kaapeeni kaligi undatam mariyu vmd samalakshanaanni pradarsinchakapovadam saadhyamavutundi. purushulu mariyu streela nishpatti sumaru 1:1 gaa undhi.
pathophysiology.
rds" mariyu mariyu janyuvulalo utparivartanalu vitreliform macular dystrophyki kaaranamavutaayi. kaaranamavutaayi janyuvulooni utparivartanalu uttama vyaadhiki kaaranamavutaayi. panichestundi ledhaa "rds" janyuvulo maarpulu vayojana-prarambha roopam yokka vitelliform macular dystrophyki kaaranamavutaayi; ayithe, eee rendu janyuvulalo utparivartanala phalitamgaa naaluginta oka vanthu kante takkuva kesulu sambhavistaayi. chaalaa sandarbhaallo, vayojana-prarambha roopam yokka kaaranam teliyadu.
teliyadu janyuvu bestrophine anae proteinnu tayaaru cheyadaaniki suuchanalanu andistundi. daani khachitamaina paniteeru anischitamgaa unnappatikee, eee protein retiinaalooni kanaalaloki ledhaa velupala pratikuulamgaa chaarj cheyabadina chlorin anuvula (chloride ayanlu) kadalikanu niyantrinche chaanelgaa panichestundi. panichestundi janyuvulo utparivartanalu bahusha chloride pravahanni niyantrinchaleni asaadhaarana aakarapu chaanal utpattiki daariteestaayi. eee panicheyakapovadam valla maculalo lipophasine nirmaanam mariyu pragatiseela drushti nashtaniki elaa sambandham undho parisoedhakulu nirnayinchaledu.
rds" janyuvu periphirin anae protein tayaareeki suuchanalanu andistundi. retiinaalooni kaanti-sensing (photoreceptor) kanaala saadhaarana paniteeruku eee protein avasaram. rds janyuvulooni utparivartanalu kaanti-sensing varnadravyam kaligina eee kanaalalooni nirmaanaalanu debbateestaayi, idhi drushti nashtaniki daariteestundi. vayojana-prarambha vitelliform macular dystrophy unnavaarilo rds utparivartanalu kendra drushtini matrame enduku prabhaavitam chestayo aspashtamgaa undhi.
rooga nirdhaarana.
viteliform macular dystrophy valla macula antarleena kanaalalo kovvu pasupu varnadravyam (lipophusine) erpadutundi. retina pigment epithelium kooda ksheenistundi. kalakramena, eee padaardham asadhaaranamgaa perukupovadam spashtamaina kendra drushti kosam keelakamaina kanaalanu debbateestundi. phalitamgaa, eee rugmata unna vyaktulu tarachugaa vaari kendra drushtini kolpotaru mariyu aspashtamaina ledhaa vakreekruta drushtini anubhavinchavacchu, mariyu nashtam arudugaa samaruupamgaa untundi. modata errati kaantitoe, aapai aakupaccha rangulo scotomata kanipistundi; chivaraku, saapeksha (ledhaa marinta teevramaina sandarbhaallo, sampoorna) scotomata tellati kaantitoe sambhavistundi. vitelliform macular dystrophy side (paridheeya) drushtini ledhaa raatri samayamlo chuse saamarthyaanni prabhaavitam cheyadu.
parisoedhakulu saaruupya lakshanaalatoo rendu rakaalaina viteliform macular dystrophyni vivarinchaaru. prarambha-prarambha roopam (uttama vyaadhi anie pilustaaru) saadhaaranamgaa baalyamlo kanipistundi. kanipistundi vyaadhi yokka vividha dasalato jeevistunna vamsapaaramparyaanni nirvachinchina german netra vaidyudu friedrich nundi eee vyaadhi daani paerunu pondindi. lakshanaala prarambham mariyu drushti nashtam yokka teevrata vistrutamgaa maarutuu untaayi. vayojana-prarambha roopam taruvaata praarambhamavutundi, saadhaaranamgaa madhya vayassulo, mariyu saapekshamgaa telikapati drushti nashtanni kaligistundi. rendu rakaalaina viteliform macular dystrophy okkokkati maculalo lakshana maarpulanu kaligi untaayi, veetini kanti pareeksha samayamlo gurtinchavacchu.
uttama vyaadhiloo, fundus floresin angiography ledhaa indocyanin green angiography vanti paraclinic anchanaalu, baseline mariyu final electrocolography (eeojee) phalitaalato paatu, vyaadhi purogatipai velugunivvavacchu.

