Yimaphi la matshe ezinso nawe owakhayo? (Amatshe e-Cystine) Ake sifunde ngawo kalula!

Wake wangeniswa esibhedlela ngenxa yamatshe ezinso kanye nobuhlungu obungabekezeleleki emhlane wakho, esiswini esingezansi, noma ohlangothini? Noma wake wezwa ngomuntu omaziyo oye waba nenkinga enjalo? Amatshe ezinso ayinto ebuhlungu ngempela. Lawa matshe angakheka ngezindlela eziningi ezahlukene. Namuhla sizokhuluma ngohlobo lwetshe lezinso olungavamile, kodwa olungabangela izinkinga uma lungelashwa kahle, ikakhulukazi phakathi kwentsha, olubizwa ngokuthi amatshe e-cystine .

Ayini la matshe e-cystine?

Kalula nje, amatshe e-cystine awuhlobo lwetshe lezinso. Amatshe ezinso ayizinqwaba eziqinile ezakha lapho amakhemikhali ahlukahlukene emchameni wakho eqoqana, njengamatshe amancane. Amatshe e-cystine akhiwa yikhemikhali ebizwa ngokuthi i-cystine . Le cystine iyi- amino acid etholakala emizimbeni yethu. Ngokuvamile, okufanele kwenzeke ukuthi le cystine ibuyiselwe emzimbeni yizinso. Kodwa ngezinye izikhathi, uma lokhu kungenzeki kahle, i-cystine iqoqana emchameni bese yakha amatshe.

Lawa matshe e-cystine avame ukuba makhulu kakhulu . Ngakho-ke ngezinye izikhathi kudingeka ukuhlinzwa ukuze asuswe ukuze kuvinjelwe ukulimala kwezinso noma emgudwini womchamo. Into edabukisayo ukuthi, abantu abaningi abathola amatshe e-cystine bangawathola la matshe izikhathi eziningana empilweni yabo. Kodwa ungakhathazeki, kunezindlela zokwelapha lesi simo ngaphandle kokuhlinzwa. Isibonelo, ungalawula lesi simo ngokushintsha ukudla kwakho noma ukusebenzisa imithi elinganisela i-pH yomchamo wakho.

Ngakho-ke iyini i-Cystinuria?

I-Cystinuria yisimo esingavamile, esizuzwe njengefa . Lokhu kwenzeka lapho ikhemikhali ebizwa ngokuthi i-cystine inqwabelana emchameni wakho. Cishe umuntu oyedwa kwabayizinkulungwane eziyishumi unalesi simo. Uma une-cystinuria, umzimba wakho unenkinga yokuncibilikisa i-cystine. Yilapho inqwabelana khona futhi yakhe amatshe e-cystine. I-Cystinuria ingabangela ukuthi uhlakulele amatshe ezinso izikhathi eziningana empilweni yakho.

Lokhu kubangelwa uhlobo lwezakhi zofuzo olubizwa ngokuthi (i-autosomal recessive gene) . Lokhu kusho ukuthi uma ingane izuza leli zakhi zofuzo kumama nakubaba, ingane izoba ne-cystinuria. Lesi yisimo sokuphila konke futhi asinakwelapheka ngokuphelele. Kodwa-ke, singalawulwa kahle ngokwelashwa okufanele .

Angavamile kangakanani amatshe ezinso e-cystine?

Uma kuqhathaniswa nezinye izinhlobo zamatshe ezinso, amatshe e-cystine ayenzeka kuphela ku-1% kuya ku-2% wamacala. Lokho akuvamile kakhulu. Kodwa ngokumangazayo, u-6% kuya ku-8% wamatshe ezinso ezinganeni angamatshe e-cystine . Eqinisweni, abantu abangu-80% abakha amatshe e-cystine bakha itshe labo lokuqala ngaphambi kweminyaka engu-20.Kodwa-ke, kungenzeka ukuthuthukisa elilodwa noma ngaphezulu lala matshe kunoma yimuphi ubudala.

Ziyini izimpawu zamatshe e-cystine?

Izimpawu zamatshe e-cystine zifana kakhulu nezezinye izinso. Uqala ukuzwa izimpawu kuphela lapho kwakheka itshe.

Izinto ezinjengalezi zingenzeka:

• Ubuhlungu obuhlabayo obubukhali ohlangothini olulodwa lomzimba wakho (emhlane) .
• Ukuzwa ubuhlungu endaweni yesisu noma esiswini esingezansi.
• Ukubona igazi emchameni .
• Ukuhlanza kanye nokuphazamiseka kwesisu (isicanucanu).
• Ubuhlungu lapho uchama (lokhu kwenzeka lapho itshe liseduze nesinye).

Ngezinye izikhathi, itshe le-cystine lingabhajwa bese libangela ukutheleleka komgudu womchamo (UTI) . Ngenxa yokuthi amatshe e-cystine makhulu kancane kunamanye amatshe, angase abangele izinkinga ezinjalo.

Kungani la matshe e-cystine akheka?

Njengoba sishilo ngaphambili, amatshe e-cystine cishe akhula kubantu abanesimo esingavamile esibizwa ngokuthi i-cystinuria . I-Cystinuria ibangelwa izinguquko ezakhiweni zezakhi zofuzo i-SLC3A1 kanye ne-SLC7A9 . Lesi simo sibangela ukuthi umzimba wakho ungakwazi ukusebenzisa kahle i-amino acid cystine. Bese iqoqana futhi yakhe amatshe. Lawa matshe angahlala noma kuphi emgudwini wakho womchamo, njengasesinyeni sakho noma ezinso.

Kalula nje, lokhu kuyinkinga yofuzo. Lokho kusho ukuthi akulona iphutha lakho.

Amatshe e-cysteine ​​​​enziwe ngani?

Amatshe e-Cystine akhiwe yi -cystine . Njengoba sishilo ngaphambili, i-cystine iyi- amino acid . Ama-amino acid awuhlobo lweprotheni. Asiza imizimba yethu ukulungisa izicubu, ukwakha imisipha, nokulwa nezifo. Abantu abane-cystinuria banezinkinga ngezinso zabo ezimunca noma ezisebenzisa le amino acid.

Yiziphi izinkinga ezingaba khona ngamatshe e-cystine?

I-Cystinuria yisimo esihlala isikhathi eside. Udokotela wakho angakusiza ukuthi uphathe lesi simo ngemithi, ukushintsha ukudla, futhi mhlawumbe nokuhlinzwa. Kodwa-ke, kungenzeka kakhulu ukuthi ube namatshe e-cystine impilo yakho yonke. Lawa matshe angaba buhlungu kakhulu lapho edlula.

Amatshe e-cystine angabangela izinkinga ezifana nalezi:

• Ukutheleleka kwezinso .
• Ukuvaleka kwe-ureter (ipayipi elithwala umchamo lisuka ezinso liye kwesinye).
• Ukutheleleka Kwendlela Yomchamo (UTIs) .
• Ukulimala kwezinso.

Wazi kanjani ukuthi unamatshe e-cystine?

Udokotela wakho uzohlonza amatshe e-cystine ngokulalela izimpawu zakho nokubhala umlando wakho wezokwelapha ophelele, okuhlanganisa nokuthi ngabe kukhona yini emndenini wakho oke waba nalezi zimo. Ngaphezu kwalokho, ungase ukwazi ukwenza okulandelayo:

• Ukuhlolwa kwezithombe , njengokuskena kwe-ultrasound kanye nokuskena kwe-CT , kanye nokuhlolwa komchamo.
• Ukuthumela itshe elabhorethri ukuze lihlolwe. Uma ungakwazi, qoqa itshe eliye laphuma nomchamo wakho, bese udokotela wakho elithumela elabhorethri ukuze lihlaziywe. Ngaleyo ndlela, bangathola ukuthi itshe lenziwe ngamakhemikhali angakanani.

Iphathwa kanjani i-cystinuria?

Into ebaluleke kakhulu lapho welapha amatshe e-cystine ukuzama ukuwavimbela ukuthi akheke kwasekuqaleni . Indlela ephumelela kakhulu yokwenza lokhu ukunciphisa inani le-cystine emchameni wakho. Udokotela wakho cishe uzosebenzisa inhlanganisela yemithi kanye nezinguquko zokudla ukusiza ukunciphisa inani lamatshe owakhayo. Uma imithi kanye nezinguquko zokudla zingavimbeli ukwakheka kwamatshe, noma uma amatshe amakhulu kakhulu, ukuhlinzwa kungenye indlela.

Imithi

Uma udokotela wakho ecabanga ukuthi ukuphuza amanzi amaningi kuzosiza itshe lizidlulele lodwa, angase akunike imithi yobuhlungu ethengiswa ngaphandle kwemithi kadokotela (OTC). Angase akunike nemithi efana ne -tiopronin kanye ne-d-penicillamine ukusiza ekuvimbeleni ukwakheka kwamatshe esikhathini esizayo.

Uhlelo Lokudla

Udokotela wakho angase akucebise ukuthi ushintshe imikhuba yakho yokudla ukuze uvimbele ukwakheka kwamatshe. Ezinye zalezi zifaka:

• Ukuphuza amanzi amaningi. Lokhu kungasiza ekuncibilikiseni amatshe e-cystine. Udokotela wakho angakutshela ukuthi uphuze ama-ounces angu-96 (cishe amalitha angu-2.8) amanzi ngosuku, uphuze ingilazi yamanzi njalo ngehora, noma uphuze amanzi uma uvuka ebusuku.
• Nciphisa inani le-sodium (usawoti) odlayo. Lokho kusho ukuthi kufanele uyeke ukudla ukudla okuqandisiwe noma okusemathinini. Akuyona nje usawoti esiwufaka elayisini esilidlayo, kodwa futhi kukhona nosawoti ofihliwe ezintweni ezifana nama-pickle, ama-pickle, amasoso, ukudla okupakishwe, kanye nokudla okuthengwa esitolo. Ngakho-ke kudingeka uqaphele nangalokho.
• Iphrotheni yezilwane , okusho ukudla inyama encane bese udla iphrotheni esuselwa ezitshalweni. Isibonelo, ungangeza izinto ezifana noshizi, ubhontshisi, kanye nesoya ekudleni kwakho esikhundleni senyama nenhlanzi.

Ukuhlinzwa

Amatshe amakhulu angonakala uma engadluliswanga emgudwini womchamo. Lawa matshe angaba buhlungu kakhulu futhi angavimba ukugeleza komchamo emzimbeni. Kunezindlela eziningana udokotela wakho angazisebenzisa ukususa itshe:

• Ikhamera encane, eguquguqukayo idluliselwa kwi-urethra yakho, ngesinyi kanye ne-urethra, ukuze kutholakale itshe bese lisuswa (ureteroscopy) .
• Kwenziwa ukusika okuncane esikhumbeni somhlane wakho, kufakwe ipayipi ngalo liye ezinso, bese kususwa itshe.(i-percutaneous nephrolithotomy).

Wazi kanjani ukuthi ukwelashwa kuphumelele?

Udokotela wakho angase acele ukuhlolwa komchamo njalo ukuze kuhlolwe amazinga e-cystine yakho. Lokhu kuhlolwa kuzomsiza ukuthi anqume ukuthi uhlelo lwakho lokwelashwa luyasebenza noma cha.

Ingabe amatshe e-cystine angancibilika?

Yebo, itshe le-cystine lingadluliswa. Kodwa lidinga ukuqapha ngokucophelela nokwelashwa okuqhubekayo . Esikhundleni sokuncibilika ngokuphelele, lingenziwa libe lincane futhi kube lula ukulidlulisa.

Yikuphi ukudla okusiza nge-cystinuria?

Ucwaningo lukhombisile ukuthi abantu abane-cystinuria bangazuza ngokwenza izinguquko ekudleni. Ezinye zalezi zinguquko zifaka phakathi ukuphuza amanzi amaningi, ukudla izithelo nemifino eminingi, kanye nokudla ukudla okunosawoti omncane. Izithelo ze-citrus ezifana nama-oranges nolamula, imifino eluhlaza, kanye nemifino eluhlaza enamaqabunga zinhle kakhulu.

Ingabe amatshe e-cystine angavinjelwa?

I-Cystinuria yisimo esizuzwe njengefa, ngakho-ke asinakuvinjelwa. Kodwa-ke, ngokulandela uhlelo lokwelashwa udokotela wakho akunikeza lona , ​​ungavimbela ukwakheka kwamatshe e-cystine . Okusho ukuthi, ungalawula ukwakheka kwamatshe.

Ingabe i-cystinuria isongela ukuphila?

Lokhu ngokuvamile akusongeli impilo . Iningi labantu alibi nezimo ezibucayi njengokwehluleka kwezinso. Into ebaluleke kakhulu ukusebenzisana nodokotela wakho ukuthola ukwelashwa okukusebenzelayo. Iningi labantu lisabela kahle ekwelashweni. Kungenzeka futhi ukuthola ukwelashwa okungeyona ingozi ezinso, esinyeni, noma emgudwini womchamo.

Iyini ikusasa lamatshe e-sistine?

Amatshe e-Cystine angakheka izikhathi eziningana empilweni yakho. Uma une-cystinuria, kuzodingeka uqhubeke nokwenza izinguquko ekudleni futhi uthathe imithi yokuvimbela ukwakheka kwamatshe e-cystine. Udokotela wakho uzokuqapha futhi aqinisekise ukuthi welapha kahle noma yimaphi amatshe amasha e-cystine akhekayo. Lokhu kwenzelwa ukuvimbela ukulimala komgudu wakho womchamo, izinso, kanye nesinye.

Kufanele ngimbone nini udokotela?

Uma unezimpawu zetshe lezinso, qiniseka ukuthi ubona udokotela . Nazi ezinye zezimpawu ezivame kakhulu:

• Ubuhlungu esiswini, ohlangothini lomhlane, noma embotsheni.
• Isicanucanu nokuhlanza.
• Ubuhlungu lapho uchama.

I-Cystinuria yisimo esihlala isikhathi eside esibangela ukwakheka kwamatshe e-cystine. Sebenzisana nodokotela wakho ukuze wakhe uhlelo lokwelapha ukuvimbela ukwakheka kwamatshe. Ngenxa yokuthi lesi simo singokwezakhi zofuzo, abantu abane-cystinuria bangase bafune ukucabangela ukwelulekwa ngokwezakhi zofuzo uma becabanga ukuqala umndeni.Kungasiza futhi ukukuthola. Izindaba ezinhle ukuthi lesi simo ngokuvamile singalawulwa ngokwelashwa futhi asibangeli ukwehluleka kwezinso noma umonakalo omkhulu endleleni yomchamo.

Izinto ezibaluleke kakhulu okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ake sifingqe ezinye zezinto okudingeka uzikhumbule kulokho esikhulume ngakho:

• Amatshe e-Cystine uhlobo lwetshe lezinso oluvela ngenxa yesimo sofuzo esibizwa ngokuthi i-cystinuria .
• Lezi zingaba zinkulu kakhulu futhi zingavela izikhathi eziningana empilweni yonke.
• Izimpawu zingase zihlanganise ubuhlungu obukhulu, igazi emchameni, kanye nokuhlanza .
• Ukwelashwa kuhlanganisa ukuphuza amanzi amaningi, ukunciphisa usawoti kanye namaprotheni ezilwane, kanye, uma kudingeka, imithi kanye nokuhlinzwa .
• Nakuba i-cystinuria ingelapheki ngokuphelele, ngokwelashwa okufanele, ukwakheka kwamatshe kungalawulwa futhi impilo evamile ingaphila.
• Uma unezimpawu zamatshe ezinso , ungachithi isikhathi uye kudokotela.

Sicela uqaphele ukuthi lolu lwazi lungolwezinjongo zolwazi kuphela futhi akufanele luthathwe njengeseluleko sezokwelapha. Kungcono ngaso sonke isikhathi ukubonisana nodokotela nganoma yiziphi izinkinga zempilo.

Amatshe ezinso , amatshe e-cystine, i-cystinuria, amatshe omchamo, izifo zezinso, izifo zofuzo, izifo zomgudu womchamo