{"id":1134,"date":"2013-01-29T20:10:29","date_gmt":"2013-01-29T18:10:29","guid":{"rendered":"http:\/\/www.hvtd.org\/?p=1134"},"modified":"2013-01-29T20:10:29","modified_gmt":"2013-01-29T18:10:29","slug":"bir-pilot-adayinda-retinitis-pigmentoza","status":"publish","type":"post","link":"https:\/\/www.hvtd.org\/?p=1134","title":{"rendered":"Bir Pilot Aday\u0131nda RET\u0130N\u0130T\u0130S P\u0130GMENTOZA"},"content":{"rendered":"

\"retinitispigmentosa\"<\/a>Retinitis pigmentosa (RP), insanda genetik k\u00f6kenli olarak k\u00f6rl\u00fck yapabilen ve retinadaki fotoresept\u00f6r h\u00fccrelerinin progresif dejenerasyonu ile karakterize bir hastal\u0131kt\u0131r ve bir grup kal\u0131tsal g\u00f6z hastal\u0131\u011f\u0131na verilen ortak add\u0131r.<\/p>\n

Klinik \u00f6yk\u00fc<\/b>: 23 ya\u015f\u0131nda, Amerikan Deniz K. pilot aday\u0131 bayan \u00f6\u011frenci. T-34C u\u00e7a\u011f\u0131nda 39 saat ve genel havac\u0131l\u0131k u\u00e7aklar\u0131nda 25 saatlik bir u\u00e7u\u015f e\u011fitimini tamamlam\u0131\u015f. Pilotaj muayenesinde sol g\u00f6z\u00fcn\u00fcn g\u00f6rmesi, 8\/10’dan daha fazla artmad\u0131\u011f\u0131 belirlenmi\u015f; optometrist taraf\u0131ndan makula dejenerasyonu \u00f6n tan\u0131s\u0131 konularak ileri tetkik i\u00e7in u\u00e7u\u015f doktoru olan g\u00f6z hekimine sevk edilmi\u015f. Anamnezde \u00f6\u011frencinin, ak\u015fam saatlerinde araba s\u00fcrerken ve gece e\u011fitimleri s\u0131ras\u0131nda g\u00fc\u00e7l\u00fck \u00e7ekti\u011fi ve RP ye ait aile \u00f6yk\u00fcs\u00fc (babas\u0131nda ve anneannesinde de RP) oldu\u011fu anla\u015f\u0131lm\u0131\u015f… Oftalmolojik muayenede, Acetazolamide tedavisine yan\u0131t veren kistoid makula \u00f6demi bulunmu\u015f; RP \u00f6n tan\u0131s\u0131 konulmu\u015f; kesin tan\u0131 i\u00e7in Elektroretinografi (ERG) yap\u0131lm\u0131\u015f ve RP tan\u0131s\u0131n\u0131 kesinle\u015ftirmi\u015ftir. Bunun \u00fczerine \u00f6\u011frencinin Donanma Hava Kuvvetleriyle ili\u015fkisi kesilmi\u015f, 3. s\u0131n\u0131f sa\u011fl\u0131k sertifikas\u0131 da FAA taraf\u0131ndan iptal edilmi\u015ftir.<\/p>\n

Sonraki s\u00fcre\u00e7te \u00f6\u011frenci, oftalmoskopik muayene ile takip s\u00fcrecine al\u0131nm\u0131\u015f. G\u00f6rme, her iki g\u00f6zde 10\/10’a ula\u015fm\u0131\u015f, kistoid mak\u00fcla \u00f6demi azalm\u0131\u015ft\u0131r. Acetazolamide ile birlikte A vitamini de verilmi\u015ftir.<\/p>\n

Klinik tablo<\/b>: RP’nin semptom ve bulgular\u0131 1855\u2019ten beri literat\u00fcrde yer almaktad\u0131r. Klinik g\u00f6r\u00fcn\u00fcm de\u011fi\u015fken olabilir ancak gece k\u00f6rl\u00fc\u011f\u00fc semptomlar\u0131 ile birlikte kon ve batonlar\u0131n artm\u0131\u015f karanl\u0131k adaptasyon e\u015fi\u011fi bulgular\u0131 mevcut olabilir. Genelde hastalar, pozitif aile hikayesi, adaptasyon bozuklu\u011fu ve adolesan \u00e7a\u011fda orta perifer g\u00f6rme alan\u0131nda g\u00f6rme azalmas\u0131 tan\u0131mlarlar. Fundus muayenesinde s\u0131kl\u0131kla anormal retinal pigmentasyonu, orta periferde kemik k\u0131ym\u0131klar\u0131 tarz\u0131nda g\u00f6r\u00fcn\u00fcmler, arteriollerde daralma ve optik sinir soluklu\u011fu g\u00f6r\u00fcl\u00fcr; ayr\u0131ca miyopiye yatk\u0131nl\u0131k, arka subkaps\u00fcler katarakt ve vitre de\u011fi\u015fiklikleri s\u0131kt\u0131r. Oftalmoskopla g\u00f6r\u00fclen kemik-k\u0131ym\u0131\u011f\u0131 tarz\u0131nda olu\u015fumlar, periferik k\u00fcmeler i\u00e7inde ve morfolojik g\u00f6r\u00fcn\u00fcmlerine g\u00f6re n\u00f6ral retinada biriken pigment gran\u00fclleridir.<\/p>\n

Hastal\u0131\u011f\u0131n erken d\u00f6nemlerinde g\u00f6z dibi, g\u00fcve yeni\u011fi ya da gran\u00fcler g\u00f6r\u00fcn\u00fcmdedir. Bunu depigmente fundus \u00fczerinde biriken kemik k\u0131ym\u0131klar\u0131 tarz\u0131nda pigmente gran\u00fcllerin olu\u015fumu izler. Hastal\u0131\u011f\u0131n seyri de\u011fi\u015fkendir. Erken tan\u0131 ve tedavi sonucunda, y\u0131llar ge\u00e7tik\u00e7e g\u00f6rme kayb\u0131 s\u0131kl\u0131kla minimal d\u00fczeyde kal\u0131r ya da hi\u00e7 bulunmayabilir. Ani \u015fekilde geli\u015fen bir g\u00f6rme kayb\u0131 ya da i\u015f g\u00f6rmezlik riskinin bulunmamas\u0131 dikkat \u00e7ekicidir.<\/p>\n

Tan\u0131<\/b>: RP\u2019nin tan\u0131s\u0131nda en \u00f6nemli ara\u00e7 ERG\u2019dir. Fotoresept\u00f6rlerin karanl\u0131k adaptasyondan sonra (skotopik ERG) veya belli \u015fiddette \u0131\u015f\u0131\u011fa adapte olmalar\u0131ndan sonra (fotopik ERG) k\u0131sa s\u00fcreli bir \u0131\u015f\u0131k kayna\u011f\u0131 ile uyar\u0131lmas\u0131 prensibine dayan\u0131r. Yan\u0131tlar, ekstraok\u00fcler bir kontakt lens elektrotu ile toplan\u0131r.<\/p>\n

Stereotipik RP, koni h\u00fccreleriyle baton h\u00fccrelerinin hastal\u0131\u011f\u0131d\u0131r; ilk \u00f6nce skotopik ERG\u2019de de\u011fi\u015fikliklerle ortaya \u00e7\u0131kar ve fotoresept\u00f6r h\u00fccreleri ile ERG’nin post-resept\u00f6r bile\u015fenlerinde nisbi bir kay\u0131p g\u00f6r\u00fcl\u00fcr. \u2018Kar\u015f\u0131t hastal\u0131k\u2019 denilen formda ise koni-baton distrofisi ve fotopik ERG de\u011fi\u015fiklikleri skotopik ERG de\u011fi\u015fikliklerinden \u00f6nce g\u00f6r\u00fcl\u00fcr. Sonu\u00e7 olarak baton yan\u0131t\u0131 etkilenir. Koni-baton distrofisi fotopik ERG\u2019de bozukluk ve yan\u0131tlar\u0131 de\u011fi\u015fken olmayan skotopik ERG ile karakterizedir.<\/p>\n

Oftalmolojik muayenede anormal retinal pigmentasyon, orta periferde kemik k\u0131ym\u0131klar\u0131 tarz\u0131nda g\u00f6r\u00fcn\u00fcmler, arteriollerde daralma, optik sinir soluklu\u011fu, miyopiye yatk\u0131nl\u0131k, arka subkaps\u00fcler katarakt ve vitre de\u011fi\u015fiklikleri s\u0131kt\u0131r. Atipik karanl\u0131k adaptasyonu, gece k\u00f6rl\u00fc\u011f\u00fc, g\u00f6rme alan kayb\u0131, retinal damarlar\u0131n silikle\u015fmesi ve optik sinir ba\u015f\u0131nda de\u011fi\u015fiklikler hastal\u0131\u011f\u0131n seyri boyunca g\u00f6r\u00fclebilse de kesin tan\u0131 i\u00e7in \u015fart de\u011fildir.<\/p>\n

Etyoloji<\/b>: RP, bir dizi birbiriyle ili\u015fkili retinal fonksiyon bozuklu\u011funa verilen genel add\u0131r. Bu hastal\u0131klar\u0131n pek \u00e7ok formu, literat\u00fcrde retinitis pigmentoza teriminin k\u0131smi ya da tam e\u015f anlaml\u0131 s\u00f6zc\u00fckleri ile ifade edilirler. Bu, hastal\u0131klar\u0131n ortak grupland\u0131r\u0131lmalar\u0131na yol a\u00e7an benzerlikler, klinik semptomlar, ERG fenotipleri ya da genetik \u00f6zelliklerinden kaynaklanmaktad\u0131r. Koni-baton\u00a0 RP diye de bilinen tipik olgularda a\u011f\u0131rl\u0131kl\u0131 olarak batonlar etkilenir. Bu ise erken ya\u015fta ya da hastal\u0131\u011f\u0131n erken d\u00f6neminde g\u00f6r\u00fclen gece k\u00f6rl\u00fc\u011f\u00fc ve iki tarafl\u0131 simetrik orta-periferik g\u00f6rme alan kayb\u0131 ile karakterize bir dizi klinik semptom olu\u015fturur.<\/p>\n

Epidemiyoloji<\/b>: RP, en s\u0131k g\u00f6r\u00fclen retinal distrofilere \u00f6rnek olan heterojen bir kal\u0131tsal g\u00f6z hastal\u0131\u011f\u0131 grubudur. D\u00fcnyadaki prevalans\u0131, 1\/3.000-1\/5.000 aras\u0131ndad\u0131r. Retinal dejeneresanslar\u0131n kal\u0131tsal olanlar\u0131, fotoresept\u00f6rler ya da retinal pigment h\u00fccreleri i\u00e7inde gen mutasyonlar\u0131 ile karakterizedir; bu da g\u00f6rsel fonksiyonun yayg\u0131n olarak kayb\u0131yla sonu\u00e7lan\u0131r. Hastal\u0131k, fonksiyonel k\u00f6rl\u00fckle sonu\u00e7lanabilir. S\u0131kl\u0131kla olgular, ge\u00e7 adolesan \u00e7a\u011fda g\u00f6rsel semptomlarla ba\u015fvurabilir.<\/p>\n

Prognoz:<\/strong> Hastal\u0131k ilerledik\u00e7e, mavi \u0131\u015f\u0131k k\u00f6rl\u00fc\u011f\u00fc, uzak periferde g\u00f6rme alan kayb\u0131 ve sonu\u00e7ta santral g\u00f6rme kayb\u0131 da ortaya \u00e7\u0131kabilir. Erken d\u00f6nemlerde fundus g\u00f6r\u00fcn\u00fcm\u00fc normal olabilir. Daha ileri d\u00f6nemlerde silikle\u015fmi\u015f retinal damarlar, retina i\u00e7i pigment birikimi, optik sinirin mumsu soluk g\u00f6r\u00fcn\u00fcm\u00fc dikkat \u00e7eker. Retinan\u0131n i\u00e7 tabakalar\u0131ndaki pigmentler, batonlar\u0131n en yo\u011fun oldu\u011fu orta periferde \u00e7evresel olarak g\u00f6r\u00fcl\u00fcr. \u00c7o\u011fu olguda katarakt geli\u015febilir ve baz\u0131 olgulara kistoid mak\u00fcler \u00f6dem e\u015flik edebilir. Baz\u0131 hastalarda, 30\u2019lu ya\u015flar gibi erken ya\u015flarda legal k\u00f6rl\u00fck geli\u015febilir. \u00c7o\u011funlukla 60 ya\u015flar\u0131nda g\u00f6rme merkezinde 20 derecenin alt\u0131nda bir g\u00f6rme alan\u0131 kal\u0131r ve hastalar legal k\u00f6rl\u00fck s\u0131n\u0131r\u0131na ula\u015f\u0131rlar.<\/p>\n

Tedavi<\/b>: Tedavi se\u00e7enekleri, oftalmolojinin yan dallar\u0131nda tart\u0131\u015fmal\u0131 olsa da ortak g\u00f6r\u00fc\u015f, A vitamininin faydal\u0131 g\u00f6rme s\u00fcresini 7 y\u0131la kadar uzatt\u0131\u011f\u0131 y\u00f6n\u00fcndedir. A ve E vitaminlerinin kar\u015f\u0131la\u015ft\u0131r\u0131ld\u0131\u011f\u0131 bir \u00e7al\u0131\u015fmada, g\u00fcnde 15.000 \u00dcnite A vitamininin RP ilerlemesini az fakat istatistiksel olarak anlaml\u0131 derecede yava\u015flatt\u0131\u011f\u0131 ortaya \u00e7\u0131kar\u0131lm\u0131\u015ft\u0131r. Ayn\u0131 \u00e7al\u0131\u015fmada g\u00fcnl\u00fck 400 \u00fcnite E vitamini ise zararl\u0131 bulunmu\u015ftur. Korunma, ilerlemenin durdurulmas\u0131 ya da hastal\u0131\u011f\u0131n geriye d\u00f6nd\u00fcr\u00fclmesi y\u00f6n\u00fcnde etkin bir yakla\u015f\u0131m yoktur.<\/p>\n

Tart\u0131\u015fma<\/b>: FAA y\u00f6nergelerine g\u00f6re retina tabakas\u0131, retina ve koroid kolobomu, koroidit, retina dekolman\u0131, diyabetik retinopati, retinit, retinitis pigmentosa, retinal t\u00fcm\u00f6r, makula ve di\u011fer retina dejenerasyonlar\u0131, toksoplazmozis, vs. a\u00e7\u0131s\u0131ndan incelenmelidir. S\u00f6z konusu y\u00f6nergede de belirtildi\u011fi \u00fczere bu yaz\u0131da bahsedilen vaka, art\u0131k 3. s\u0131n\u0131f havac\u0131l\u0131k sertifikas\u0131na sahip olamaz. \u00d6\u011frencinin\u00a0 halen 10\/10 g\u00f6rme d\u00fczeyine sahip olmas\u0131 ve hastal\u0131\u011f\u0131n do\u011fal seyri g\u00f6z \u00f6n\u00fcne al\u0131nd\u0131\u011f\u0131nda, sadece g\u00fcnd\u00fcz u\u00e7mas\u0131 \u015fart\u0131yla 3. s\u0131n\u0131f u\u00e7ucu sertifikas\u0131 verilmesi akla yatk\u0131n olabilir. FAA taraf\u0131ndan g\u00f6rme yetene\u011fi ile ilgili bir belge istenmesi mant\u0131kl\u0131 olabilir, \u00e7\u00fcnk\u00fc RP\u2019de g\u00f6rme keskinli\u011findeki azalma on y\u0131llar boyunca ve yava\u015f\u00e7a olur; yani ani olarak g\u00f6rme kayb\u0131 ve i\u015f g\u00f6rmezli\u011fe neden olmaz.<\/p>\n

Kaynak<\/b>: Sean Hollonbeck, Thomas Webster. Retinitis Pigmentosa in a Naval Aviator. Case Report. Federal Air Surgeons’ Medical Bulletin 2008; Vol 46\/4;8-9<\/p>\n

\u00c7eviri<\/b>: Prof.Dr. Sarper Karak\u00fc\u00e7\u00fck. (Erciyes \u00dc. T\u0131p Fak. G\u00f6z Hast. AD. Kayseri)<\/p>\n","protected":false},"excerpt":{"rendered":"

Retinitis pigmentosa (RP), insanda genetik k\u00f6kenli olarak k\u00f6rl\u00fck yapabilen ve retinadaki fotoresept\u00f6r h\u00fccrelerinin progresif dejenerasyonu ile karakterize bir hastal\u0131kt\u0131r ve bir grup kal\u0131tsal g\u00f6z hastal\u0131\u011f\u0131na verilen ortak add\u0131r. Klinik \u00f6yk\u00fc: 23 ya\u015f\u0131nda, Amerikan Deniz K. pilot aday\u0131 bayan \u00f6\u011frenci. T-34C u\u00e7a\u011f\u0131nda 39 saat ve genel havac\u0131l\u0131k u\u00e7aklar\u0131nda 25 saatlik bir u\u00e7u\u015f e\u011fitimini tamamlam\u0131\u015f. Pilotaj muayenesinde sol g\u00f6z\u00fcn\u00fcn g\u00f6rmesi, 8\/10’dan daha […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_eb_attr":"","_jetpack_memberships_contains_paid_content":false,"footnotes":""},"categories":[6],"tags":[],"class_list":["post-1134","post","type-post","status-publish","format-standard","hentry","category-bulten"],"jetpack_featured_media_url":"","jetpack_sharing_enabled":true,"jetpack-related-posts":[],"_links":{"self":[{"href":"https:\/\/www.hvtd.org\/index.php?rest_route=\/wp\/v2\/posts\/1134","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.hvtd.org\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.hvtd.org\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.hvtd.org\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.hvtd.org\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=1134"}],"version-history":[{"count":1,"href":"https:\/\/www.hvtd.org\/index.php?rest_route=\/wp\/v2\/posts\/1134\/revisions"}],"predecessor-version":[{"id":1136,"href":"https:\/\/www.hvtd.org\/index.php?rest_route=\/wp\/v2\/posts\/1134\/revisions\/1136"}],"wp:attachment":[{"href":"https:\/\/www.hvtd.org\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=1134"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.hvtd.org\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=1134"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.hvtd.org\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=1134"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}