Menene cutar Marfan, alamomi da magani

Wadatacce

• Babban bayyanar cututtuka
• Dalilin cutar Marfan
• Yadda ake ganewar asali
• Zaɓuɓɓukan magani

Marfan Syndrome cuta ce ta kwayar halitta wacce ke shafar ƙwayoyin haɗi, waɗanda ke da alhakin tallafi da ruɓaɓɓen gabobi da yawa a cikin jiki. Mutanen da ke fama da wannan ciwo suna da tsayi sosai, sirara kuma suna da yatsu da yatsu na ƙwarai kuma ƙila suna da canje-canje a zuciya, idanu, ƙasusuwa da huhu.

Wannan ciwo yana faruwa ne saboda lahani da aka samu a cikin kwayar halittar fibrillin-1, wanda shine babban jigon jijiyoyi, ganuwar jijiyoyi da haɗin gwiwa, wanda ke haifar da wasu sassa da gabobin jiki zama masu rauni. Babban likita ko likitan yara ne suka gano cutar ta hanyar tarihin lafiyar mutum, gwaje-gwajen jini da hoto kuma maganin yana ƙunshe da tallafawa alamun da cutar ta haifar.

Babban bayyanar cututtuka

Cutar Marfan wata cuta ce ta kwayar halitta wacce ke haifar da canje-canje a cikin tsarin jiki daban-daban, wanda ke haifar da alamomi da alamomin da za su iya bayyana a lokacin haihuwa ko ma a duk tsawon rayuwa, tsananin sa ya bambanta daga mutum ɗaya zuwa wancan. Wadannan alamun zasu iya bayyana a wadannan wurare:

• Zuciya: babban sakamakon cututtukan Marfan sune canje-canje na zuciya, wanda ke haifar da asarar tallafi a cikin bangon jijiya, wanda zai iya haifar da rashin kuzarin jijiyoyin jiki, kumburin iska da kuma mitral valve prolapse;
• Kasusuwa: wannan ciwo yana haifar da ƙasusuwa suyi girma sosai kuma ana iya ganin su ta ƙaruwa da girman mutum da ta hanu, yatsu da yatsu. Kirjin da aka tono, kuma ana kiransapectus excavatum, shi ne lokacin da wani damuwa ya samu a tsakiyar kirji;
• Idanu: abu ne na yau da kullun ga mutanen da suke da wannan cutar su sami hijirar kwayar ido, glaucoma, cataract, myopia kuma suna iya samun mafi ƙarancin ɓangaren ido mafi ƙanƙanta;
• Kashin baya: bayyanuwar wannan ciwo na iya kasancewa bayyane a cikin matsalolin kashin baya kamar scoliosis, wanda shine karkatar da kashin baya zuwa gefen dama ko hagu. Haka kuma yana yiwuwa a lura da ƙaruwa a cikin jakar dural a cikin yankin lumbar, wanda shine membrane wanda ke rufe yankin kashin baya.

Sauran alamomin da za su iya bayyana saboda wannan ciwo su ne sakin jiki da jijiyoyin jiki, nakasawa a cikin leda, wanda aka sani da rufin baki, da kuma kafafun kafa, wadanda ke dauke da dogayen kafa, ba tare da tafin tafin kafa ba. Duba ƙarin menene ƙafafun kafa da yadda ake yin magani.

Dalilin cutar Marfan

Ciwon Marfan ya samo asali ne daga lahani a cikin kwayar halittar da ake kira fibrillin-1 ko FBN1, wanda ke da aikin tabbatar da tallafi da kuma samar da zaren roba na wasu gabobin jiki, kamar ƙashi, zuciya, idanu da kuma kashin baya.

A mafi yawan lokuta, wannan lahani na gado ne, wannan yana nufin cewa ana ɗaukarsa daga uba ko uwa zuwa ga yaron kuma zai iya faruwa a cikin mata da maza. Koyaya, a wasu mawuyacin yanayi, wannan lahani a cikin kwayar halitta na iya faruwa kwatsam kuma ba tare da sanannen dalili ba.

Yadda ake ganewar asali

Ganewar cutar Marfan ta kasance daga babban likita ko likitan yara dangane da tarihin dangin mutum da canje-canje na zahiri, kuma ana iya ba da umarnin gwaje-gwajen hotunan, kamar su kwayar halittar jini da wutan lantarki, don gano yiwuwar matsaloli a cikin zuciya, kamar rarraba aortic. Learnara koyo game da rarraba aortic da yadda ake gane shi.

Hakanan ana nuna rayukan X, lissafin hoto ko hoton maganaɗisu don bincika canje-canje a cikin wasu gabobin da gwajin jini, kamar gwajin kwayar halitta, waɗanda ke iya gano maye gurbi a cikin kwayar halittar da ke da alhakin bayyanar wannan ciwo. Bayan sakamakon gwaje-gwajen ya fito, likita zai bayar da shawara kan kwayoyin halitta, inda za a bayar da shawarwari kan kwayoyin halittar dangin.

Zaɓuɓɓukan magani

Maganin cutar Marfan ba'a nufin magance cutar, amma yana taimakawa rage alamun don inganta rayuwar mutanen da ke fama da wannan ciwo da nufin taimakawa rage ƙarancin nakasa, inganta motsin mahaɗa da rage yiwuwar raguwa.

Sabili da haka, marasa lafiya da cutar Marfan ya kamata su riƙa yin bincike akai-akai game da zuciya da jijiyoyin jini, kuma su sha magunguna kamar beta-blockers, don hana lalacewar tsarin zuciya da jijiyoyin jini. Bugu da ƙari, yin aikin tiyata na iya zama dole don gyara raunuka a cikin jijiyar aortic, misali.