Adrenoleukodystrophy
Taƙaitaccen bayani
Adrenoleukodystrophy (uh-dree-noh-loo-koh-DIS-truh-fee) cutace ce iri na rashin lafiya na gado (gene) wanda ke lalata membrane (myelin sheath) wanda ke rufe sel na jijiyoyi a cikin kwakwalwarka.
A cikin adrenoleukodystrophy (ALD), jikinka ba zai iya rushe ƙwayoyin mai masu tsayi sosai ba (VLCFAs), wanda ke haifar da ƙaruwar VLCFAs a cikin kwakwalwarka, tsarin jijiyoyin jikinka da kuma gland na adrenal.
Nau'in ALD da aka fi sani da shi shine X-linked ALD, wanda aka haifar da lahani na kwayoyin halitta akan chromosome X. X-linked ALD yana shafar maza fiye da mata, wadanda ke dauke da cutar.
Nau'o'in X-linked ALD sun hada da:
- Yarancin ALD. Wannan nau'in X-linked ALD yawanci yana faruwa tsakanin shekaru 4 zuwa 10. Farin abu na kwakwalwa yana lalacewa a hankali (leukodystrophy), kuma alamomi suna kara muni a hankali. Idan ba a gano shi da wuri ba, yarancin ALD na iya haifar da mutuwa a cikin shekaru biyar zuwa 10.
- Cututtukan Addison. Glands masu samar da homon (gland na adrenal) sau da yawa ba sa samar da isasshen steroids (adrenal insufficiency) a cikin mutanen da ke da ALD, wanda ke haifar da nau'in X-linked ALD da ake kira cututtukan Addison.
- Adrenomyeloneuropathy. Wannan nau'in X-linked ALD na manya shi ne nau'in da bai yi muni ba kuma yana ci gaba a hankali wanda ke haifar da alamomi kamar tafiya mai tauri da kuma rashin aiki na fitsari da hanji. Mata masu dauke da ALD na iya kamuwa da nau'in adrenomyeloneuropathy mai sauki.
Gano asali
Don don ga ALD, likitanki zai binciki alamun cutar da kuma tarihin lafiyarki da na danginku. Likitanka zai yi gwajin jiki kuma ya ba da umarnin yin gwaje-gwaje da dama, ciki har da:
- MRI. Magnets masu ƙarfi da raƙuman rediyo suna haifar da hotuna masu dalla-dalla na kwakwalwarki a cikin gwajin MRI. Wannan yana ba likitoci damar gano abubuwan da ba su da kyau a cikin kwakwalwarki wanda zai iya nuna adrenoleukodystrophy, gami da lalacewar nama (fari) na kwakwalwarki. Likitoci na iya amfani da nau'ikan MRI da dama don ganin hotunan kwakwalwar da suka fi dalla-dalla da gano alamun farkon leukodystrophy.
- Gwajin gani. Auna amsoshin gani na iya sa ido kan ci gaban cutar a maza waɗanda ba su da sauran alamun cutar.
- Biopsy na fata da kuma al'adun ƙwayoyin fibroblast. Ana iya ɗaukar ƙaramin samfurin fata don bincika ƙaruwar matakan VLCFA a wasu lokuta.
Gwajin jini. Wadannan gwaje-gwajen suna bincika matakan ƙwayoyin mai masu tsayi sosai (VLCFAs) a cikin jininka, wanda shine alamar adrenoleukodystrophy.
Likitoci suna amfani da samfuran jini don gwajin kwayoyin halitta don gano lahani ko canje-canje waɗanda ke haifar da ALD. Likitoci kuma suna amfani da gwaje-gwajen jini don tantance yadda glandon adrenal ɗinku ke aiki.
Jiyya
Adrenoleukodystrophy ba ta da magani. Duk da haka, dashen ƙwayoyin ƙashin baya zai iya dakatar da ci gaban ALD idan an yi shi lokacin da alamun cutar kwakwalwa suka fara bayyana. Likitoci za su mayar da hankali kan rage alamun cutar da rage ci gabanta.
Zabuka na magani na iya haɗawa da:
- Dashen ƙwayoyin ƙashin baya. Wannan na iya zama zaɓi don rage ko dakatar da ci gaban adrenoleukodystrophy a yara idan an gano ALD kuma an yi magani da wuri. Ana iya ɗaukar ƙwayoyin ƙashin baya daga ƙashin ƙugu ta hanyar dashen ƙwayoyin ƙashin ƙugu.
- Maganin rashin aikin adrenal gland. Mutane da yawa da ke da ALD suna kamuwa da rashin aikin adrenal gland kuma suna buƙatar gwajin adrenal gland akai-akai. Rashin aikin adrenal gland ana iya magance shi sosai tare da magungunan steroid (maganin maye gurbin corticosteroid).
- Magunguna. Likitanka na iya rubuta maka magunguna don taimakawa wajen rage alamun cutar, ciki har da ƙarfi da fitsari.
- Jiyya ta jiki. Jinyar jiki na iya taimakawa wajen rage tashin hankalin tsoka da rage ƙarfin tsoka. Likitanka na iya ba da shawarar kujerun guragu da sauran kayan aikin motsa jiki idan ya zama dole.
A cikin gwajin likita na kwanan nan, an yi wa yara maza da ke da ALD na kwakwalwa a farkon mataki magani tare da maganin gene a matsayin madadin dashen ƙwayoyin ƙashin baya. Sakamakon farko daga maganin gene yana da kyau. Ci gaban cutar ya tsaya a kashi 88 cikin 100 na yaran maza da suka shiga cikin gwajin. An buƙaci ƙarin bincike don tantance sakamakon dogon lokaci da amincin maganin gene na cerebral ALD.
Sanarwa: Agusta dandamali ne na bayanan kiwon lafiya kuma amsoshinsa ba su ƙunshi shawarar likita ba. Tabbatar da tuntuɓar ƙwararren likita mai lasisi kusa da ku kafin yin kowane canji.