Ngawaphi la matye ezintso nawe owakhayo? (Amatye eCystine) Masifunde ngawo ngokulula!

Ngaba wakha wangeniswa esibhedlele ngenxa yamatye ezintso kunye nentlungu enganyamezelekiyo emqolo wakho, esiswini esisezantsi, okanye ecaleni? Okanye wakha weva ngomntu omaziyo oye waba nengxaki enjalo? Amatye ezintso ngamava abuhlungu ngokwenene. La matye anokwakheka ngeendlela ezahlukeneyo. Namhlanje siza kuthetha ngohlobo lwelitye lezintso olungaqhelekanga, kodwa olunokubangela iingxaki ukuba alunyangwa ngokufanelekileyo, ingakumbi phakathi kwabantu abaselula, olubizwa ngokuba ngamatye e -cystine .

Zithini ezi litye ze-cystine?

Ngamafutshane, amatye e-cystine luhlobo lwelitye lezintso. Amatye ezintso ziingqokelela eziqinileyo ezenzeka xa iikhemikhali ezahlukeneyo kumchamo wakho ziqokelelana, njengeengqalutye ezincinci. Amatye e-cystine enziwe yikhemikhali ebizwa ngokuba yi-cystine . Le cystine yi- amino acid efumaneka emizimbeni yethu. Ngokwesiqhelo, okufuneka kwenzeke kukuba le cystine ibuyiselwe emzimbeni zizintso. Kodwa ngamanye amaxesha, xa oku kungenzeki kakuhle, i-cystine iqokelelana kumchamo ize yenze amatye.

La matye e-cystine adla ngokuba makhulu kakhulu . Ngoko ke ngamanye amaxesha kufuneka utyando ukuze asuswe ukuze kuthintelwe ukonakala kwezintso okanye kwindlela yomchamo. Into ebuhlungu kukuba, abantu abaninzi abafumana amatye e-cystine banokuvelisa la matye amaxesha amaninzi ebomini babo. Kodwa ungakhathazeki, kukho iindlela zokunyanga le meko ngaphandle kotyando. Umzekelo, ungayilawula le meko ngokutshintsha ukutya kwakho okanye ukusebenzisa amayeza alinganisela i-pH yomchamo wakho.

Ngoko ke yintoni iCystinuria?

I-Cystinuria yimeko engaqhelekanga, ezuzwe njengelifa . Oku kwenzeka xa ikhemikhali ebizwa ngokuba yi-cystine iqokelelana kumchamo wakho. Malunga nomntu omnye kwabalishumi lamawaka unale meko. Ukuba une-cystinuria, umzimba wakho unengxaki yokunyibilikisa i-cystine. Oku kwenzeka xa iqokelelana ize yenze amatye e-cystine. I-Cystinuria inokubangela ukuba ube namatye ezintso amaxesha amaninzi ebomini bakho.

Oku kubangelwa luhlobo lwejini ebizwa ngokuba yi- (autosomal recessive gene) . Oku kuthetha ukuba ukuba umntwana uzuze esi jini kumama nakutata, umntwana uya kuba ne-cystinuria. Le yimeko yobomi bonke kwaye ayinakunyangwa ngokupheleleyo. Nangona kunjalo, inokulawulwa kakuhle ngonyango olufanelekileyo .

Zinqabile kangakanani ii-cystine kidney stones?

Xa kuthelekiswa nezinye iintlobo zamatye ezintso, amatye e-cystine ayenzeka kuphela kwi-1% ukuya kwi-2% yamatyala. Oko kunqabile kakhulu. Kodwa ngokumangalisayo, i-6% ukuya kwi-8% yamatye ezintso ebantwaneni ngamatye e-cystine . Enyanisweni, i-80% yabantu abavelisa amatye e-cystine bavelisa ilitye labo lokuqala ngaphambi kokuba bafikelele kwiminyaka engama-20.Nangona kunjalo, kunokwenzeka ukuvelisa elinye okanye ngaphezulu la matye nangaliphi na ixesha.

Zithini iimpawu zamatye e-cystine?

Iimpawu zamatye e-cystine zifana kakhulu nezo zamanye amatye ezintso. Uqala ukuva iimpawu xa kuvela ilitye.

Izinto ezinjalo zinokwenzeka:

• Intlungu ebukhali ehlabayo kwelinye icala lomzimba wakho (umqolo) .
• Ukuva iintlungu kwindawo yempumlo okanye ezantsi kwesisu.
• Ukubona igazi kumchamo .
• Ukugabha kunye nokuphazamiseka kwesisu (isicaphucaphu).
• Intlungu xa uchama (oku kwenzeka xa ilitye likufutshane nesinyi).

Ngamanye amaxesha, ilitye le-cystine linokunamathela lize libangele usulelo lwendlela yomchamo (UTI) . Ngenxa yokuba amatye e-cystine makhulu kancinci kunamanye amatye, anokubangela iingxaki ezinjalo.

Kutheni la matye e-cystine edalwa?

Njengoko besitshilo ngaphambili, amatye e-cystine anokwenzeka kakhulu kubantu abanesimo esingaqhelekanga esibizwa ngokuba yi-cystinuria . I-Cystinuria ibangelwa kukuguquka kwezakhi zofuzo i-SLC3A1 kunye ne-SLC7A9 . Le meko ibangela ukuba umzimba wakho ungakwazi ukuyisebenzisa ngokufanelekileyo i-amino acid cystine. Emva koko iyaqokelelana ize yenze amatye. La matye anokufumaneka naphi na kwindlela yakho yomchamo, njengakwisinye sakho okanye kwizintso.

Ngamafutshane, le yingxaki yemfuza. Oko kuthetha ukuba ayilotyala lakho.

Zenziwe ngantoni ii-cysteine ​​​​stones?

Amatye e-Cystine enziwe yi -cystine . Njengoko besitshilo ngaphambili, i-cystine yi- amino acid . Ii-amino acid luhlobo lweproteni. Zinceda imizimba yethu ilungise izicubu, yakhe izihlunu, kwaye ilwe nosulelo. Abantu abane-cystinuria baneengxaki zezintso zabo ezifunxa okanye ezisebenzisa le amino acid.

Ziziphi iingxaki ezinokubakho ngenxa yamatye e-cystine?

I-Cystinuria yimeko ehlala ihleli. Ugqirha wakho angakunceda ulawule le meko ngamayeza, utshintsho kwindlela otya ngayo, mhlawumbi notyando. Nangona kunjalo, unamathuba amaninzi okuba ne-cystine stones ubomi bakho bonke. La matye anokuba buhlungu kakhulu xa edlula.

Amatye e-cystine anokubangela iingxaki ezifana nezi:

• Usulelo lwezintso .
• Ukuvaleka kwe-ureter (umbhobho othwala umchamo ukusuka kwizintso ukuya kwisinyi).
• Iintsholongwane zoMchamo (UTIs) .
• Ukonakala kwezintso.

Uyazi njani ukuba une-cystine stones?

Ugqirha wakho uza kuxilonga amatye e-cystine ngokuphulaphula iimpawu zakho aze athathe imbali yakho epheleleyo yezonyango, kuquka nokuba kukho nabani na kusapho lwakho okhe waba nezi meko. Ukongeza, unokukwazi ukwenza oku kulandelayo:

• Uvavanyo lokufota , olufana nee-ultrasound scans kunye nee-CT scans , kunye novavanyo lomchamo.
• Ukuthumela ilitye kwilabhoratri ukuze livavanywe. Ukuba unako, qokelela ilitye eliye laphuma nomchamo wakho, kwaye ugqirha wakho angalithumela kwilabhoratri ukuze lihlolwe. Ngale ndlela, banokufumanisa ukuba lenziwe ngayiphi na ikhemikhali ilitye.

Iphathwa njani i-cystinuria?

Eyona nto ibalulekileyo xa unyanga amatye e-cystine kukuzama ukuwathintela ukuba angakheki kwasekuqaleni . Enye yezona ndlela zilungileyo zokwenza oku kukunciphisa inani le-cystine kumchamo wakho. Ugqirha wakho uya kusebenzisa indibaniselwano yamayeza kunye notshintsho kwindlela yokutya ukunceda ukunciphisa inani lamatye owakhayo. Ukuba amayeza kunye notshintsho kwindlela yokutya awathinteli ukwakheka kwamatye, okanye ukuba amatye makhulu kakhulu, utyando lolunye ukhetho.

Amayeza

Ukuba ugqirha wakho ucinga ukuba ukusela amanzi amaninzi kuya kunceda ilitye lizidlulele lodwa, unokukunika amayeza entlungu athengiswa ngaphandle kwe-odolo (OTC) ukuze uncede intlungu. Banokukunika amayeza anjenge- tiopronin kunye ne -d-penicillamine ukunceda ukuthintela ukwakheka kwamatye kwixesha elizayo.

Isicwangciso sokutya

Ugqirha wakho unokukucebisa ukuba utshintshe indlela otya ngayo ukuze uthintele ukwakheka kwamatye. Ezinye zezi ziquka:

• Ukusela amanzi amaninzi. Oku kunokunceda ekunyibilikiseni amatye e-cystine. Ugqirha wakho angakuxelela ukuba usele ii-ounces ezingama-96 (malunga neelitha ezi-2.8) zamanzi ngosuku, usele iglasi yamanzi rhoqo ngeyure, okanye usele amanzi xa uvuka ebusuku.
• Nciphisa ubungakanani besodium (ityuwa) oyityayo. Oko kuthetha ukuba kufuneka uyeke ukutya ukutya okukhenkcezisiweyo okanye okusemathinini. Akupheleli nje kwityuwa esiyibeka kwirayisi esiyityayo, kodwa kukho netyuwa efihliweyo kwizinto ezifana neepickles, iipickles, iisosi, ukutya okupakishweyo, kunye nokutya okuthengwe evenkileni. Ngoko ke kufuneka ulumke nakwelo tyala.
• Iproteni yezilwanyana , oko kuthetha ukutya inyama encinci endaweni yokutya iproteni esekwe kwizityalo. Umzekelo, ungongeza izinto ezifana neetships, iilentils, kunye nesoya ekutyeni kwakho endaweni yenyama nentlanzi.

Utyando

Amatye amakhulu angonakala ukuba awadluliswanga kwindlela yomchamo. La matye anokuba buhlungu kakhulu kwaye anokuthintela ukuhamba komchamo emzimbeni. Kukho iindlela ezahlukeneyo ugqirha wakho anokuzisebenzisa ukususa ilitye:

• Ikhamera encinci, eguquguqukayo idluliselwa kwi-urethra yakho, ngesinyi kunye ne-urethra, ukuze kufunyanwe ilitye kwaye lisuswe (ureteroscopy) .
• Kwenziwa umngxuma omncinci eluswini lomqolo wakho, kufakwe ityhubhu ngayo iye kwizintso, kwaye ilitye liyasuswa.(i-percutaneous nephrolithotomy).

Uyazi njani ukuba unyango luphumelele?

Ugqirha wakho unokuyalela ukuba kwenziwe uvavanyo lomchamo rhoqo ukuze kuhlolwe amanqanaba e-cystine yakho. Olu vavanyo luya kumnceda ukuba aqonde ukuba isicwangciso sakho sonyango siyasebenza okanye akunjalo.

Ngaba amatye e-cystine anokunyibilika?

Ewe, ilitye le-cystine lingadluliselwa. Kodwa lifuna ukujongwa ngononophelo kunye nonyango oluqhubekayo . Endaweni yokunyibilika ngokupheleleyo, linokwenziwa libe lincinci kwaye kube lula ukulidlulisela.

Ziziphi ukutya ezinceda kwi-cystinuria?

Uphando lubonise ukuba abantu abane-cystinuria banokungenelwa ngokwenza utshintsho kwindlela yokutya. Ezinye zezi zinto ziquka ukusela amanzi amaninzi, ukutya iziqhamo nemifuno emininzi, nokutya ukutya okunetyuwa encinci. Iziqhamo ze-citrus ezifana nee-orenji kunye neelamuni, imifuno eluhlaza, kunye nemifuno eluhlaza enamagqabi zilungile kakhulu.

Ngaba amatye e-cystine angathintelwa?

I-Cystinuria yimeko ezuzwe njengelifa, ngoko ke ayinakuthintelwa. Nangona kunjalo, ngokulandela isicwangciso sonyango esinikwa ngugqirha wakho , unokuthintela ukwakheka kwamatye e-cystine . Oko kukuthi, unokulawula ukwakheka kwamatye.

Ngaba i-cystinuria iyingozi ebomini?

Oku akudli ngokuba yingozi ebomini . Uninzi lwabantu alufumani zimo zinzima ezifana nokungasebenzi kakuhle kwezintso. Eyona nto ibalulekileyo kukusebenzisana nogqirha wakho ukuze ufumane unyango olusebenzayo kuwe. Uninzi lwabantu lusabela kakuhle kunyango. Kukwanokwenzeka ukufumana unyango olungenabungozi kwizintso, kwisinyi, okanye kwindlela yomchamo.

Iyintoni ikamva lamatye e-sistine?

Amatye e-Cystine anokwakheka izihlandlo ezininzi ebomini bakho. Ukuba une-cystinuria, kuya kufuneka uqhubeke nokwenza utshintsho kwindlela yokutya kwaye uthathe amayeza okuthintela ukwakheka kwamatye e-cystine. Ugqirha wakho uza kukujonga aze aqinisekise ukuba uwanyanga ngokufanelekileyo nawaphi na amatye amatsha e-cystine ayenzekayo. Oku kwenzelwa ukuthintela umonakalo kwindlela yakho yomchamo, izintso, kunye nesinyi.

Ndifanele ndimbone nini ugqirha?

Ukuba uneempawu zelitye lezintso, qiniseka ukuba ubona ugqirha . Nazi ezinye zezona mpawu zixhaphakileyo:

• Intlungu esiswini, ecaleni komqolo, okanye emqolo.
• Isicaphucaphu nokuhlanza.
• Intlungu xa uchama.

I-Cystinuria yimeko ehlala ubomi bonke ebangela ukuba kubekho amatye e-cystine. Sebenzisana nogqirha wakho ukuze uyile isicwangciso sonyango sokuthintela ukwenzeka kwamatye. Ngenxa yokuba le meko ibangelwa lufuzo, abantu abane-cystinuria banokufuna ukuqwalasela iingcebiso ngefuzo ukuba bacinga ngokuqalisa usapho.Kunokuba luncedo ukuyifumana. Iindaba ezimnandi zezokuba le meko idla ngokulawulwa ngonyango kwaye ayibangeli ukungasebenzi kakuhle kwezintso okanye umonakalo omkhulu kwindlela yomchamo.

Izinto ezibalulekileyo ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, masishwankathele ezinye zezinto ekufuneka uzikhumbule koko sithethe ngako:

• Amatye e-Cystine luhlobo lwelitye lezintso elenziwa ngenxa yesifo semfuza esibizwa ngokuba yi-cystinuria .
• Ezi zinokuba nkulu kakhulu kwaye zinokwenzeka izihlandlo ezininzi ebomini.
• Iimpawu zingabandakanya iintlungu eziqatha, igazi emchameni, kunye nokuhlanza .
• Unyango luquka ukusela amanzi amaninzi, ukunciphisa ityuwa kunye neprotheyini yezilwanyana, kwaye, ukuba kuyimfuneko, amayeza kunye notyando .
• Nangona i-cystinuria ingenakunyangwa ngokupheleleyo, ngonyango olufanelekileyo, ukwakheka kwamatye kunokulawulwa kwaye ubomi obuqhelekileyo bunokuphila.
• Ukuba uneempawu zamatye ezintso , musa ukuchitha ixesha uye kugqirha.

Nceda uqaphele ukuba olu lwazi lujolise ekunikeni ulwazi kuphela kwaye alufanele luthathwe njengengcebiso yezonyango. Kungcono rhoqo ukubonana nogqirha xa kukho naziphi na iingxaki zempilo.

Amatye ezintso , amatye e-cystine, i-cystinuria, amatye omchamo, izifo zezintso, izifo zofuzo, usulelo lwendlela yomchamo