Calaamadaha 'Stickler syndrome' waa xaalad dhif ah ama hidda-wata oo saameysa unugyada isku xiran ee jirka. Si gaar ah, shakhsiyaadka qaba Stickler syndrome guud ahaan waxay leeyihiin isbadel ku dhaca unugyada soo saaro kolajka. Isku-dhafashadaan hidaha ee bukaan-socodka waxay keeni karaan qaar ama dhammaan sifooyinka astaamaha ah ee Stickler syndrome:
- Dhibaatooyinka indhaha ah - oo ay ka midka yihiin dhererka, isbedelka vitreoretinal, glaucoma , cataracts , iyo godadka retina ama xakameyn. Xaaladaha qaarkood, dhibaatooyinka indhaha ee la xiriira Stickler Syndrome waxay keeni karaan indho la'aan.
- Dhibaatooyinka dhegta dhexe ee u horseedi kara isku dhafka, dareen- celinta ama maqalka, luminta dhegta , dheecaanka dhagaha ama laf-dhabarka dhaqdhaqaaqa lafaha dhegta.
- Dhibaatooyinka Skeletal oo ay ku jiraan nabarro xuubka ah (scoliosis ama kyphosis), xuubka isgoysyada, wareegga hore ee arthritis, laabta laabta, suuxdinta muruqyada carruurnimada (oo loo yaqaan cudurka Legg-Calve-Perthe), iyo dhibaatooyinka wadajirka ah ee guud.
- Shakhsiyaadka qaba Stickler syndrome ayaa laga yaabaa in ay sii kororto marxaladda mitral-mareenka marka loo eego guud ahaan dadweynaha, sida laga soo xigtay cilmibaadhista qaarkood.
- Dhibaatooyinka ilkaha oo ay ku jiraan shucaac.
- Muuqaal waji oo jajaban ayaa mararka qaarkood loo yaqaan 'Pierre Robin'. Tani waxay inta badan ku jirtaa waxyaabo ay ka mid yihiin gogol dillaac , gogol dhaadheer, geedka sare ee cufan, carrabka oo ka soo noqda sidii caadiga ahayd, garka yar yar, iyo daanka hoose ee hoose. Iyada oo ku xiran darnaanta shakhsiyaadka wajiga (taas oo aad ugu kala duwan shakhsiyaadka), xaaladahaas waxay keeni karaan dhibaatooyin quudin, gaar ahaan inta ay yar yihiin.
- Qaababka kale waxaa ka mid noqon kara hypertonia , cagaha cagaha, iyo faraha dheer.
Xanuunka Stickler wuxuu aad ula mid yahay xaalad la xiriirta oo loo yaqaan 'Marshall syndrome', inkasta oo shakhsiyaadka qaba 'Marshall Syndrome' ay badanaa leeyihiin cillad gaaban oo lagu daray calaamadaha badan ee Stickler syndrome. Cudurka Stickler wuxuu u qaybsan yahay shan nooc oo kala duwan iyadoo ku xiran calaamadaha horay loo soo sheegay.
Calaamadaha iyo darnaanta way kala duwan yihiin kuwa u dhexeeya dadka qaba Stickler syndrome xitaa qoysas isku mid ah.
Sababaha
Cudurka Stickler syndrome waxaa lagu qiyaasay in ku dhowaad 1 qof 7,500 dhalmo. Si kastaba ha noqotee, xaaladda ayaa la rumeeysan yahay in lagu baaro. Calaamadaha Stickler waxaa waalidiinta u gudbiya carruurta oo ku jira qaabka ugu hooseeya ee autosomal. Khatarta waalidka leh Stickler syndrome-ka ee ku gudbaya cunuggu waa 50 boqolkiiba uurka. Calaamadaha 'Stickler syndrome' waxay ku dhacaan labada ragga iyo dumarka.
Ciladeynta
Calaamadaha Stickler waxaa laga yaabaa in laga shakiyo haddii aad leedahay astaamo ama calaamadaha cudurkaan, gaar ahaan haddii qof qoyskaaga ka mid ah lagu ogaado cudurka Stickler syndrome. Baaritaanka genetic waxaa laga yaabaa in uu ku caawiyo baarista cudurka Stickler syndrome laakiin hadda ma jirto shuruudo lagu ogaado heerka cudurka dabiiciga ah ee ay bixiyaan bulshada caafimaadka.
Daaweynta
Ma jiro wax daawo ah oo loo yaqaan Stickler syndrome laakiin daweyno badan iyo daweyno ayaa jira oo lagu maareeyo calaamadaha cudurka Stickler syndrome. Aqoonsiga hore ama calaamadaha cudurka Stickler systrome waa muhiim sidaa darteed xaaladaha la xidhiidha waa la baadhi karaa isla markiiba la daaweyn karaa. Qalitaanka qalliinka ee isbedelka wajiga sida fardaha roogga ayaa laga yaabaa inay lagama maarmaan noqoto si looga caawiyo cunista iyo neefsashada.
Muraayadaha saxda ah ama qaliinka waxay faa'iido u yeelan karaan daaweynta dhibaatooyinka indhaha. Qalabka maqalka ama qaliinka sida sida meeleynta tuubbooyinka neefta ayaa sixi kara ama daaweyn kara dhibaatooyinka dhegta. Marmarka qaarkood dawooyinka anti-inflammatory ayaa ku caawin kara daaweynta arthritis-ka ama dhibaatooyinka wadajirka ah, marka ay jiraan xaalado halis ah beddelaada qaliinka ee isku-xigga ayaa lagama maarmaan ah.
Ilaha:
Tixraaca Guud ee Hoyga. Xinjirowga Xinjirowga. https://ghr.nlm.nih.gov/condition/stickler-syndrome
Marfan Foundation. Xinjirowga Xinjirowga. https://www.marfan.org/stickler-syndrome.
Urur Qaran ee Cudurka Naafada. Xinjirowga Xinjirowga. http://rarediseases.org/rare-diseases/stickler-syndrome/.