Isifo sikaWilson

-Delile

• Izimpawu nezimpawu zesifo sikaWilson
• Okuhlobene nesibindi
• Yezinzwa
• Izindandatho zeKayser-Fleischer kanye ne-sunflower cataract
• Ezinye izimpawu
• Yini imbangela futhi ubani osengozini yesifo sikaWilson?
• Sitholakala kanjani isifo sikaWilson?
• Ukuhlolwa ngokomzimba
• Ukuhlolwa kwelebhu
• Selashwa kanjani isifo sikaWilson?
• Isigaba sokuqala
• Isigaba sesibili
• Isigaba sesithathu
• Uyini umbono wesifo sikaWilson?
• Ungakwazi yini ukuvimbela isifo sikaWilson?
• Izinyathelo ezilandelayo

Siyini isifo sikaWilson?

Isifo sikaWilson, esaziwa nangokuthi ukwehla kwe-hepatolenticular kanye nokuwohloka kwe-lenticular okuqhubekayo, kuyisifo esingajwayelekile sezakhi zofuzo esidala ubuthi bethusi emzimbeni. Ithinta cishe umuntu oyedwa kwabangu-30,000 emhlabeni jikelele.

Emzimbeni ophilile, isibindi sikhipha ithusi eliningi bese silikhipha ngomchamo. Ngesifo sikaWilson, isibindi asikwazi ukukhipha ithusi elengeziwe kahle. Ithusi elengeziwe bese lakha ezithweni ezifana nobuchopho, isibindi namehlo.

Ukuxilongwa kusenesikhathi kubalulekile ekunqandeni ukuqhubeka kwesifo sikaWilson. Ukwelashwa kungabandakanya ukuthatha imithi noma ukufakelwa isibindi. Ukubambezeleka noma ukungatholi ukwelashwa kungadala ukwehluleka kwesibindi, ukulimala kobuchopho, noma ezinye izimo ezisongela impilo.

Khuluma nodokotela wakho uma umndeni wakho unomlando wesifo sikaWilson. Abantu abaningi abanalesi simo baphila impilo ejwayelekile, enempilo.

Izimpawu nezimpawu zesifo sikaWilson

Izimpawu nezimpawu zesifo sikaWilson ziyahlukahluka kakhulu, kuya ngokuthi yisiphi isitho esithintekile. Bangaphanjaniswa nezinye izifo noma izimo. Isifo sikaWilson singatholakala kuphela kudokotela nangokuhlolwa kokuxilongwa.

Okuhlobene nesibindi

Izimpawu ezilandelayo zingakhombisa ukuqongelela kwethusi esibindini:

• ubuthakathaka
• ngizizwe ngikhathele
• ukuncipha komzimba
• isicanucanu
• ukuhlanza
• ukulahlekelwa isifiso sokudla
• ukulunywa
• i-jaundice, noma ukuphuzi kwesikhumba
• i-edema, noma ukuvuvukala kwemilenze nesisu
• ubuhlungu noma ukuqunjelwa esiswini
• spider angiomas, noma imithambo yegazi ebonakalayo efana negatsha esikhunjeni
• amajaqamba

Eziningi zalezi zimpawu, njenge-jaundice ne-edema, ziyefana nezinye izimo ezinjengokwehluleka kwesibindi nezinso. Udokotela wakho uzokwenza izivivinyo eziningi ngaphambi kokuqinisekisa ukutholakala kwesifo sikaWilson.

Yezinzwa

Ukuqoqwa kwethusi ebuchosheni kungadala izimpawu ezinjenge:

• inkumbulo, inkulumo, noma ukukhubazeka kombono
• ukuhamba ngokungajwayelekile
• migraines
• ukumunca amathe
• ukuqwasha
• ubulukhuni ngezandla
• izinguquko zobuntu
• ukushintsha kwemizwa
• ukudana
• izinkinga esikoleni

Ezigabeni ezithuthukile, lezi zimpawu zingafaka ukuphazamiseka kwemisipha, ukuquleka, nobuhlungu bemisipha ngesikhathi sokunyakaza.

Izindandatho zeKayser-Fleischer kanye ne-sunflower cataract

Udokotela wakho uzophinde ahlole izindandatho zeKayser-Fleischer (KF) nokhakhayi lwe-sunflower emehlweni. Izindandatho ze-KF zingumbala wegolide onsundu ngokungajwayelekile emehlweni abangelwa yimali yethusi eyeqile. Amasongo e-KF akhombisa cishe abantu abangamaphesenti angama-97 abanesifo sikaWilson.

I-cataract ye-Sunflower ivela kubantu abangu-1 kwabangu-5 abanesifo sikaWilson. Lesi isikhungo esinezimpawu ezahlukahlukene ezinama-spokes akhanya ngaphandle.

Ezinye izimpawu

Ukwakheka kwethusi kwezinye izitho kungadala:

• ukushintsha kombala okuluhlaza okwesibhakabhaka kuzipikili
• amatshe ezinso
• i-osteoporosis ngaphambi kwesikhathi, noma ukungabi namandla kwamathambo
• isifo samathambo
• ukungahambi kahle esikhathini
• umfutho wegazi ophansi

Yini imbangela futhi ubani osengozini yesifo sikaWilson?

Ukuguquka kwesimo ku- I-ATP7B ufuzo, olukhomba ukuthuthwa ngethusi, lubanga isifo sikaWilson. Kufanele uzuze ifa kubazali bobabili ukuze ube nesifo sikaWilson. Lokhu kungasho ukuthi omunye wabazali bakho unaleso simo noma uphethe isakhi sofuzo.

Isakhi singeqa isizukulwane, ngakho-ke ungafuna ukubheka ukudlula abazali bakho noma wenze ukuhlolwa kofuzo.

Sitholakala kanjani isifo sikaWilson?

Isifo sikaWilson singase sibe nzima kodokotela ukuthi basheshe baxilonge. Izimpawu ziyefana nezinye izindaba zezempilo ezinjenge-heavy metal poisoning, hepatitis C, ne-cerebral palsy.

Kwesinye isikhathi udokotela wakho uzokwazi ukukhipha isifo sikaWilson uma sezivele izimpawu zemizwa futhi ayikho indandatho ye-K-F ebonakalayo.Kodwa lokhu akuhlali kunjalo kubantu abanezimpawu eziqondene nesibindi noma abangenazo ezinye izimpawu.

Udokotela uzobuza ngezimpawu zakho bese ebuza umlando wezempilo womndeni wakho. Bazophinde basebenzise izivivinyo ezahlukahlukene ukubheka umonakalo odalwe ukuqongelela kwethusi.

Ukuhlolwa ngokomzimba

Ngesikhathi somzimba wakho, udokotela wakho uzo:

• hlola umzimba wakho
• lalela imisindo esiswini
• hlola amehlo akho ngaphansi kokukhanya okukhanyayo ukuthola izindandatho ze-KF noma i-sunflower cataract
• ihlola amakhono akho wemoto nenkumbulo

Ukuhlolwa kwelebhu

Ukuhlolwa kwegazi, udokotela wakho uzodweba amasampula futhi awahlaziye elebhu ukuze ahlole:

• ukungajwayelekile kuma-enzyme akho wesibindi
• amazinga ethusi egazini
• amazinga aphansi e-ceruloplasmin, iphrotheni ethwala ithusi egazini
• isakhi sofuzo esiguqukile, esibizwa nangokuthi ukuhlolwa kofuzo
• ushukela wegazi ophansi

Udokotela wakho futhi angakucela ukuthi uqoqe umchamo wakho amahora angama-24 ukufuna ukuqongelela ithusi.

Selashwa kanjani isifo sikaWilson?

Ukwelashwa okuphumelelayo kwesifo sikaWilson kuncike esikhathini esingaphezu kwemithi. Ukwelashwa kuvame ukwenzeka ngezigaba ezintathu futhi kufanele kuhlale impilo yonke. Uma umuntu eyeka ukuthatha imishanguzo, ithusi lingakha futhi.

Isigaba sokuqala

Ukwelashwa kokuqala ukukhipha ithusi eleqile emzimbeni wakho ngokusebenzisa i-chelating therapy. Ama-Chelating agents afaka izidakamizwa ezinjenge-d-penicillamine ne-trientine, noma iSyprine. Le mithi izosusa ithusi elengeziwe ezithweni zakho bese ilikhiphela egazini. Izinso zakho zizobe sezihluza ithusi emchameni.

I-Trientine inemiphumela emibi embalwa ebikiwe kune-d-penicillamine. Imiphumela emibi engaba khona i-d-penicillamine ifaka phakathi:

• imfiva
• ukuqubuka
• izinkinga zezinso
• izindaba zomnkantsha

Udokotela wakho uzohlinzeka ngemithamo ephansi yemithi yokukhohlisa uma ukhulelwe, ngoba ingadala ukukhubazeka kokuzalwa.

Isigaba sesibili

Inhloso yesigaba sesibili ukugcina amazinga ejwayelekile ethusi ngemuva kokususwa. Udokotela wakho uzokunika i-zinc noma i-tetrathiomolybdate uma usuqedile ukwelashwa kokuqala noma ungakhombisi zimpawu kodwa unesifo sikaWilson.

I-Zinc ethathwe ngomlomo njengosawoti noma i-acetate (Galzin) igcina umzimba ungamunci ithusi ekudleni. Ungaba nesisu esincane ngokucabanga ngokuthatha i-zinc. Izingane ezinesifo sikaWilson kodwa azikho izimpawu ezingase zithande ukuthatha i-zinc ukuvimbela isimo ukuthi singabi sibi noma sinciphise ukuqhubeka kwaso.

Isigaba sesithathu

Ngemuva kokuthi izimpawu zithuthuke futhi amazinga akho ethusi ajwayelekile, uzofuna ukugxila ekwelashweni kwesondlo isikhathi eside. Lokhu kufaka phakathi ukuqhubeka nokwelashwa kwe-zinc noma ukukhwabanisa nokuqapha njalo amazinga akho ethusi.

Ungaphatha futhi amazinga akho ethusi ngokugwema ukudla okunamazinga aphezulu, afana nalokhu:

• izithelo ezomisiwe
• isibindi
• amakhowe
• amantongomane
• izimbaza
• ushokoledi
• ama-multivitamini

Ungahle ufune ukuhlola amazinga akho wamanzi ekhaya, futhi. Kungaba nethusi elengeziwe emanzini akho uma ikhaya lakho linamapayipi wethusi.

Imithi ingathatha noma kuphi ukusuka ezinyangeni ezine kuya kweziyisithupha ukusebenza kumuntu ohlangabezana nezimpawu. Uma umuntu engaziphenduli lezi zindlela zokwelapha, zingadinga ukufakelwa isibindi. Ukufakelwa kwesibindi ngempumelelo kungaphilisa isifo sikaWilson. Izinga lokuphumelela kokufakelwa kwesibindi lingamaphesenti angama-85 ngemuva konyaka owodwa.

Uyini umbono wesifo sikaWilson?

Ekuqaleni uthola ukuthi unalo yini ufuzo lwesifo sikaWilson, kungcono ukubikezelwa kwakho kube ngcono. Isifo sikaWilson singakhula sibe ukwehluleka kwesibindi kanye nokulimala kobuchopho uma singalashwa.

Ukwelashwa ngaphambi kwesikhathi kungasiza ukuguqula izingqinamba zezinzwa nokulimala kwesibindi. Ukwelashwa esikhathini esizayo kungavimba ukuqhubeka kwesifo, kepha ngeke kubuyise umonakalo njalo. Abantu abasezigabeni ezithuthukile kungadingeka ukuthi bafunde ukuthi bangaziphatha kanjani izimpawu zabo phakathi nempilo yabo.

Ungakwazi yini ukuvimbela isifo sikaWilson?

Isifo sikaWilson yisakhi sofuzo esidluliselwe kubazali siye ezinganeni zabo. Uma abazali benengane enesifo sikaWilson, bangaba nezinye izingane ezinalesi sifo.

Yize ungeke ukwazi ukuvimbela isifo sikaWilson, ungalibazisa noma unciphise ukuqala kwesimo. Uma uthola ukuthi unesifo sikaWilson kusenesikhathi, ungakwazi ukuvimbela izimpawu ekuboniseni ngokuthatha imishanguzo efana ne-zinc. Uchwepheshe wezofuzo angasiza abazali ukuthi banqume ubungozi babo bokudlulisela isifo sikaWilson ezinganeni zabo.

Izinyathelo ezilandelayo

Yenza i-aphoyintimenti nodokotela wakho uma wena noma umuntu omaziyo enesifo sikaWilson noma ekhombisa izimpawu zokwehluleka kwesibindi. Inkomba enkulu yalesi simo umlando womndeni, kepha isakhi esiguqukile singeqa isizukulwane. Ungase ufune ukucela ukuhlolwa kofuzo kanye nezinye izivivinyo udokotela wakho azokuhlela.

Uzofuna ukuqala ukwelashwa kwakho ngokushesha uma uthola ukuxilongwa kwesifo sikaWilson. Ukwelashwa kusenesikhathi kungasiza ukuvimbela noma ukubambezela isimo, ikakhulukazi uma ungazikhombisi izimpawu okwamanje. Imithi ifaka ama-chelating agents kanye ne-zinc futhi kungathatha izinyanga eziyisithupha ukusebenza. Ngisho nangemva kokuba amazinga akho ethusi ebuyele kokujwayelekile, kufanele uqhubeke nokuphuza imithi, njengoba isifo sikaWilson siyisimo sempilo yonke.