Kodi cystinosis ndi zizindikiro zazikulu ndi ziti?

Zamkati

• Zizindikiro zazikulu
• Cystinosis mu impso
• Cystinosis m'maso
• Zomwe zimayambitsa cystinosis
• Momwe mankhwalawa amachitikira

Cystinosis ndimatenda obadwa nawo momwe thupi limapezera cystine yochulukirapo, amino acid yomwe, ikapitirira mkati mwa maselo, imapanga timibulu tomwe timalepheretsa magwiridwe antchito a maselo, chifukwa chake, matendawa amatha kukhudza ziwalo zingapo za thupi , Kugawidwa m'magulu atatu:

• Nephropathic cystinosis: imakhudza impso ndipo imawonekera mwa khanda, koma imatha kusintha kupita mbali zina za thupi monga maso;
• Wapakatikati cystinosis: ndi ofanana ndi nephropathic cystinosis koma imayamba kukula unyamata;
• Ocular cystinosis: ndi mtundu wocheperako womwe umangofika m'maso.

Ichi ndi matenda amtundu omwe amatha kupezeka mumkodzo ndikuyesa magazi ngati khanda, pafupifupi miyezi isanu ndi umodzi. Makolo ndi dokotala wa ana atha kukayikira matendawa ngati mwanayo nthawi zonse ali ndi ludzu kwambiri, amakodza ndikusanza kwambiri ndipo samalemera bwino, ndikumakayikira matenda a Fanconi.

Zizindikiro zazikulu

Zizindikiro za cystinosis zimasiyana malinga ndi chiwalo chomwe chakhudzidwa, ndipo chitha kuphatikizira:

Cystinosis mu impso

• Kuchuluka kwa ludzu;
• Kufunitsitsa kukulira;
• Kutopa kosavuta;
• Kuchuluka kwa magazi.

Cystinosis m'maso

• Kupweteka m'maso;
• Kumvetsetsa kuunika;
• Kuvuta kuwona, komwe kumatha kukhala khungu.

Kuphatikiza apo, zizindikilo zina monga kuvutika kumeza, kuchedwa kukula, kusanza pafupipafupi, kudzimbidwa kapena zovuta monga matenda ashuga komanso kusintha kwa ntchito ya chithokomiro, mwachitsanzo, zitha kuwonekeranso.

Zomwe zimayambitsa cystinosis

Cystinosis ndi matenda omwe amayamba chifukwa cha kusintha kwa mtundu wa CTNS, womwe umayambitsa kupanga puloteni yotchedwa cystinosine. Puloteniyi nthawi zambiri imachotsa cystine m'maselo amkati, kuti isamangidwe mkati.

Izi zikachitika, maselo athanzi amawonongeka ndipo amalephera kugwira ntchito bwino, kuwononga chiwalo chonse pakapita nthawi.

Momwe mankhwalawa amachitikira

Chithandizochi chimachitika kuchokera nthawi yomwe matendawa amapezeka, kuyambira kugwiritsa ntchito mankhwala, monga cysteamine, omwe amathandiza thupi kuchotsa zina mwa zotupa. Komabe, sikutheka kupewa kufalikira kwa matendawa, chifukwa chake, nthawi zambiri kumakhala kofunikira kupatsirana impso, pomwe matendawa adakhudza kale chiwalocho mozama kwambiri.

Komabe, matendawa akakhala m'ziwalo zina, kumuika sikumachiritsa matendawa, chifukwa chake, kungakhale kofunikira kupitiriza kugwiritsa ntchito mankhwalawo.

Kuphatikiza apo, zina mwa zovuta ndi zovuta zimafunikira chithandizo chapadera, monga matenda ashuga kapena matenda a chithokomiro, kuti athandize ana kukhala ndi moyo wabwino.