Matenda a chiberekero amachititsa kuti pakhale kusokoneza mkuwa
Matenda a Wilson, omwe amadziwikanso kuti hepatolenticular degeneration, ndi matenda a chibadwa omwe amachititsa kuti mkuwa uwonjezeke kwambiri m'thupi. Ndi matenda osadziwika omwe amakhudza mmodzi mwa anthu 30,000. Kuti munthu asakhudzidwe, iye ayenera kulandira kusintha kwasinthika kuchokera kwa mmodzi koma makolo onse awiri.
Kupeza mkuwa kosasangalatsa kwenikweni kumakhudza chiwindi, ubongo, impso, ndi maso komanso kungakhudze mtima ndi dongosolo la endocrine.
Zizindikiro za matenda a Wilson zimakhala zikuwonekera makamaka kumayambiriro kwa moyo, makamaka pakati pa zaka zisanu ndi zisanu ndi zitatu. Zovuta za matendawa zimaphatikizapo kulephera kwa chiwindi, mavuto a impso, ndipo nthawi zina zimakhala zovuta kwambiri.
Zimayambitsa
Matenda a Wilson ndi matenda obadwa nawo muzolowera zamtundu wa autosomal . Izi zikutanthawuza kuti makolo onse awiri amakhala ndi zonyamulira za genetic mutation, ngakhale kuti sipadzakhalanso zizindikiro kapena mbiri ya banja ya matendawa. Anthu amene ali ndi zonyamula katundu angakhale ndi umboni wosakanizika wamkuwa wamkuwa koma nthawi zambiri sali okwanira kuti athandizidwe.
Matenda a Wilson ndi amodzi mwa matenda osiyanasiyana omwe mkuwa amamanga molakwika, nthawi zambiri m'chiwindi. Zimaphatikizapo jini yotchedwa ATP7B yomwe thupi limagwiritsa ntchito popanga mkuwa mu bile . Kusinthika kwa jini kumathandiza kuti izi zisokonezeke ndipo zimapangitsa kuti thupi lisapitirire.
Pamene mitsempha ya mkuwa ikuyamba kudwalitsa chiwindi, thupi lidzayesa kuwaswa ndi kutseka chinyontho cha hydrochloric ndi chitsulo chachitsulo kuti imitsitsire mamolekyu amkuwa. M'kupita kwa nthawi, izi zimachititsa kuti chiwindi chikhale chiwindi (fibrosis), chiwindi cha chiwindi , ndi chiwindi.
Chifukwa mkuwa ndizofunikira pakati pa mapangidwe a collagen ndi kuyamwa kwa chitsulo, kuwonongeka kulikonse kwa njirayi kungapweteke msinkhu.
Ichi ndi chifukwa chake matenda a Wilson angayambitse matenda a chiwindi m'zaka zitatu zoyambirira za moyo ndi chibwibwi (zomwe zimagwirizanitsidwa ndi akuluakulu) achinyamata ndi achinyamata.
Zizindikiro Zogwirizana ndi Chiwindi
Zizindikiro za matenda a Wilson zimasiyana malinga ndi kuwonongeka kwa minofu. Popeza kuti mkuwa umayamba kuwonjezeka pachiwindi ndi ubongo poyamba, zizindikiro za matendawa zimawonekera kwambiri mu ziwalo izi.
Zizindikiro zoyambirira za chiwindi chopweteka nthawi zambiri zimakhala zofanana ndi zomwe zimawoneka ndi matenda a chiwindi . Kupititsa patsogolo kwa fibrosis kungayambitse ku chidziwitso chotchedwa portal hypertension chomwe chiwopsezo cha magazi mkati mwa chiwindi chimayamba kuwuka. Ngati chiwindi chimawonjezeka, munthu akhoza kukumana ndi zochitika zoopsa kwambiri, kuphatikizapo kutuluka kwa magazi ndi chiwindi .
Zina mwa zizindikiro zokhudzana ndi chiwindi zomwe zimapezeka mu Wilson's disease:
- Kutopa
- Nausea
- Kuthamanga
- Kutaya njala
- Mitsempha ya minofu
- Khungu la khungu ndi maso ( jaundice )
- Anagwiritsira madzi m'miyendo ( edema )
- Kupezeka kwa madzi m'mimba ( ascites )
- Nkhumba zotsekemera pamtambo (angiomas a kangaude)
- Ululu kapena umphumphu kumtunda kumanzere pamimba chifukwa cha nthenda yotambasula
- Kuwombera magazi kapena malo otayira chifukwa cha zovuta zowonongeka
Ngakhale kuti matendawa amafala kwambiri kwa anthu omwe ali ndi matenda aakulu, osatengeredwa, amayamba kudwala khansa ya chiwindi (mosiyana ndi matenda a chiwindi omwe amapezeka ndi matenda a chiwindi kapena uchidakwa ).
Zisonyezo za m'maganizo
Kulephera kwa chiwindi chachikulu kumadziwika ndi kukula kwa mtundu wamagazi wamagazi wotchedwa hemolytic anemia momwe maselo ofiira a m'magazi adzaphulika ndi kufa. Popeza maselo ofiira amapezeka katatu kuchuluka kwa ammonia monga plasma (chigawo chimodzi cha magazi), kuwonongedwa kwa maselowa kungachititse kuti ammonia ndi ma poizoni ena alowe mwamsanga m'magazi.
Zinthu zimenezi zikakhumudwitsa ubongo, munthu akhoza kukhala ndi ubongo wambiri , kutaya kwa ubongo kumachita chifukwa cha matenda a chiwindi. Zizindikiro zingakhalepo:
- Migraines
- Kusagona
- Kutaya kwaiwala
- Kulankhulidwa
- Kusintha kwa masomphenya
- Matenda osokonezeka komanso kusawonongeka
- Nkhawa kapena kupanikizika
- Kusintha kwa umunthu (kuphatikizapo kusaganizira ndi kukhumudwa)
- Parkinsonism (kukhwima, kunjenjemera, kuthamangitsidwa)
- Psychosis
Chifukwa chakuti zifukwa zomwe zingayambitse zizindikirozi ndi zazikulu, matenda a Wilson sapezeka kawirikawiri pazinthu za neuropsychiatric zokha.
Zizindikiro Zina
Kupezeka kopanda thupi kwa mkuwa m'thupi kumakhudza mwachindunji kapena mosiyana ndi ziwalo zina za thupi.
- Pomwe zikuchitika m'maso, matenda a Wilson angayambitse chizindikiro chodziwika bwino monga mphete za Kayser-Fleisher. Izi ndizo mitundu yozungulira ya golide yofiira kwambiri yomwe ili moyandikana ndi mapiri a iris omwe amachitidwa ndi zipilala zamkuwa. Zimapezeka pafupifupi 65 peresenti ya anthu omwe ali ndi matenda a Wilson.
- Mukapezeka mu impso, matenda a Wilson angayambitse kutopa, kufooka kwa minofu, kusokonezeka, miyala ya impso, ndi mitsempha m'magazi chifukwa cha kuchuluka kwa magazi m'magazi. Mkhalidwewu ukhozanso kuchititsa kuti calcium yochuluke kwambiri mu impso ndipo, chodabwitsa, kufooketsa mafupa chifukwa cha kugawidwa ndi kutaya kashiamu.
Nthawi zambiri, matenda a Wilson angayambitse matenda a mtima (kufooka kwa mtima) komanso kusabereka komanso kubwereka mobwerezabwereza chifukwa cha kuwonongeka kwa chithokomiro.
Kudziwa
Chifukwa cha zizindikiro zosiyana siyana, matenda a Wilson nthawi zambiri amakhala ovuta kuwazindikira. Makamaka ngati zizindikirozo n'zosavuta, matendawa amatha kulakwitsa zinthu zonse kuchokera ku poizoni wolemera kwambiri ndi chiwindi cha matenda a hepatitis C kupita ku mankhwala omwe amachititsa kuti lupus ndi cerebral palsy zisokonezeke.
Ngati matenda a Wilson akudandaula, kufufuza kudzaphatikizapo kubwereza zizindikiro za thupi limodzi ndi mayesero angapo oyeza, kuphatikizapo:
- Mayeso a enzyme ya chiwindi
- Kuyezetsa magazi kuti muone ngati mchere ndi wotsika kwambiri wa ceruloplasmin (mapuloteni omwe amabweretsa mkuwa kudzera mwazi)
- Magazi a shuga amayeza kuti aone ngati shuga akuchepa kwambiri
- Mphindi wa maola 24 kuti muyesedwe kwa acidity ndi calcium
- Chiwindi chimagwiritsidwa ntchito poyeza kukula kwa mkuwa
- Kuyezetsa magazi kumatsimikizira kusintha kwa kusintha kwa ATB7B
Chithandizo
Matenda oyambirira a Wilson a matenda amapereka zotsatira zabwino. Anthu omwe amapezeka ndi matendawa amachizidwa m'njira zitatu:
- Chithandizo chimayamba ndi kugwiritsa ntchito mankhwala amkuwa kuti achotse mkuwa wambiri. Penicillamine kawirikawiri ndi mankhwala oyamba omwe angasankhe. Zimagwira ntchito pomangirira ndi mkuwa, zomwe zimapangitsa kuti chitsulo chikhale chosavuta kwambiri mumtambo. Nthawi zina zotsatira zake zimakhala zofunikira ndipo zimaphatikizapo kufooka kwa minofu, kuthamanga, ndi kupweteka. Pakati pa iwo omwe akuwona zizindikiro, 50 peresenti adzakhala ndi zizindikiro zowonjezereka zowonjezereka. Zikatero, mankhwala achiwiri angapangidwe.
- Kamodzi ka mkuwa kamakhala kosavomerezeka, nthaka imatchulidwa ngati mtundu wa chithandizo chamakono. Zinc zotengedwa pamlomo zimateteza thupi kuti lisamve mkuwa. Mphuno ya m'mimba ndiyo zotsatira zoyipa kwambiri.
- Kusintha kwa zakudya kumatsimikizira kuti mumapewa kugwiritsira ntchito mkuwa wopanda nsalu. Izi zimaphatikizapo zakudya zamkuwa monga chifuwa, chiwindi, mtedza, bowa, zipatso zouma, batala wa kirimba, ndi chokoleti chamdima. Zophatikizira zamphongo, monga multivitamins ndi zomwe zimagwiritsidwa ntchito pochizira matenda a m'mimba, zingathenso kulowera m'malo.
Anthu omwe ali ndi matenda aakulu a chiwindi omwe amalephera kuthandizira kuchipatala angafunike kuika chiwindi .
> Zotsatira:
> Borges Pinto, R .; Reis Schneider, A .; ndi da Silveira, T. "Cirrhosis kwa ana ndi achinyamata: Mwachidule." World Journal ya Hepatology. 2015; 7 (): 392-405.
> Patil, M .; Chihema, K.; Krishnamurthy, A. et al. "Kubwereza ndi Zomwe Zilipo Panopa pa Matenda a Wilson." Journal of Clinical and Experimental Hepatology. 2013; 3 (4): 321-336.