Cystinuria

Zvemukati

• Ndezvipi zviratidzo zvecystinuria?
• Chii chinokonzera cystinuria?
• Ndiani ari panjodzi yecystinuria?
• Ko cystinuria inoonekwa sei?
• 24-awa weti kuunganidzwa
• Intravenous pyelogram
• Abdominal CT scan
• Kuongorora urinal
• Ndeapi matambudziko ecystinuria?
• Kurapa cystinuria kunorapwa sei? | Kurapa
• Kuchinja kwekudya
• Kugadzirisa pH chiyero
• Mishonga
• Kuvhiya
• Ndeupi mutarisiro wenguva refu?
• Cystinuria ingadzivirirwa sei?

Chii chinonzi cystinuria?

Cystinuria chirwere chakagarwa nhaka chinokonzera matombo akagadzirwa neamino acid cystine kuti agadzire muitsvo, dundira, uye ureters. Zvirwere zvakagara nhaka zvinopfuudzwa kubva kuvabereki kuenda kuvana kuburikidza nehurema mumajini avo. Kuti uwane cystinuria, munhu anofanirwa kugara nhaka urema kubva kuvabereki vese.

Iko kuremara kuri mugene kunokonzera kuti cystine iungane mukati meitsvo, inova nhengo dzinobatsira kudzora zvinopinda nekubuda muropa rako. Itsvo dzine mabasa mazhinji, kusanganisira:

• kudzorerazve mamineral akakosha uye mapuroteni kumashure mumuviri
• kusefa ropa kubvisa tsvina ine chepfu
• kuburitsa weti yekudzinga tsvina kubva mumuviri

Mune mumwe munhu ane cystinuria, iyo amino acid cystine inovaka uye inogadzira matombo pane kudzoka muropa. Aya mabwe anogona kunamatira muitsvo, dundira, uye ureters. Izvi zvinogona kurwadza kwazvo kusvikira matombo apfuura nekuita weti. Matombo makuru kwazvo angangoda kuvhiyiwa.

Matombo anogona kudzoka kakawanda. Mishonga inowanikwa kugadzirisa marwadzo uye kudzivirira mamwe matombo kubva pakuumba.

Ndezvipi zviratidzo zvecystinuria?

Kunyangwe cystinuria iri yehupenyu hwese, zviratidzo zvinowanzoitika muvakuru vechidiki, zvinoenderana nekudzidza muEuropean Journal yeUrology. Pakave nezviitiko zvisingawanzoitika muvacheche nevechiri kuyaruka. Zviratidzo zvinogona kusanganisira:

• ropa mumurini
• kurwadziwa kwakanyanya parutivi kana kumashure, kazhinji nguva dzose kudivi rimwe
• kusvotwa uye kurutsa
• kurwadziwa padyo nekugomera, chiuno, kana dumbu

Cystinuria haina chiratidzo, zvichireva kuti inokonzeresa zviratidzo, kana pasina mabwe. Nekudaro, izvo zviratidzo zvinodzoka nguva yega yega mabwe paanoumba itsvo. Matombo anowanzoitika kanopfuura kamwe.

Chii chinokonzera cystinuria?

Kukanganisa, kunonziwo kuchinja, mumajeni SLC3A1 uye SLC7A9 kukonzera cystinuria. Aya majini anopa rairo kumuviri wako kuti uite imwe yekufambisa mapuroteni anowanikwa mune itsvo. Iyi protein inowanzo kudzora kudzoserwa kwemamwe amino acids.

Amino acids inogadzirwa kana muviri uchigaya nekutyora mapuroteni. Ivo anoshandiswa kuita akasiyana akasiyana emuviri mashandiro, saka iwo akakosha kumuviri wako uye haatariswe semarara. Naizvozvo, kana aya maamino acids akapinda muitsvo, anowanzo nyudzwa adzoka muropa. Mune vanhu vane cystinuria, urema hunokanganisa hutachiona hwehutakuri hwekugona kudzorerazve maamino acids.

Imwe yeamino acids - cystine - haina kunyungudika zvakanyanya mumuti. Kana isina kubatwazve, inozoungana mukati meitsvo yoita makristasi, kana matombo ecystine. Matombo akaoma-dombo anobva abatika muitsvo, dundira, uye ureters. Izvi zvinogona kurwadza kwazvo.

Ndiani ari panjodzi yecystinuria?

Iwe uri panjodzi yekuwana cystinuria chete kana vabereki vako vaine chakakanganisika mune yavo geni inokonzera chirwere. Zvakare, iwe unongowana chirwere kana iwe ukagara nhaka hurema kubva kuvabereki vako vese. Cystinuria inoitika mune angangoita 1 muvane vanhu zviuru gumi kutenderera pasirese, saka hazvishamise.

Ko cystinuria inoonekwa sei?

Cystinuria inowanzoonekwa kana mumwe munhu akasangana nechikamu cheitsvo matombo. Kuongororwa kunozoitwa nekuyedza matombo kuti aone kana akagadzirwa necystine. Kazhinji kuongororwa kwemajini kunoitwa. Kuwedzera kuongororwa kwekuongorora kunogona kusanganisira zvinotevera:

24-awa weti kuunganidzwa

Iwe uchazobvunzwa kuti utore weti yako mumudziyo mukati mezuva rese. Weti yacho inozotumirwa kurabhoritari kunoongororwa.

Intravenous pyelogram

Kuongororwa X-ray yeitsvo, dundira, uye ureters, nzira iyi inoshandisa dhayi muropa kubuda kubatsira kuona matombo.

Abdominal CT scan

Mhando iyi yeCT scan inoshandisa maX-ray kugadzira mifananidzo yezvivakwa mukati medumbu kutsvaga mabwe mukati meitsvo.

Kuongorora urinal

Uku ndiko kuongorora weti murabhoritari iyo inogona kusanganisira kutarisa muvara uye kutaridzika kweiyo weti, kutarisa weti pasi pe microscope, uye kuitisa bvunzo dzemakemikari kuti uone zvimwe zvinhu, zvakaita se cystine.

Ndeapi matambudziko ecystinuria?

Kana isina kurapwa zvakanaka, cystinuria inogona kurwadza zvakanyanya uye inogona kutungamira kumatambudziko akakomba. Aya matambudziko anosanganisira:

• itsvo kana dundira kukuvara kubva padombo
• Urinary turakiti hutachiona
• itsvo zvirwere
• ureteral mhingaidzo, kuvhara kweweureter, iyo chubhu inodonhedza weti kubva kuitsvo kuenda mudundira

Kurapa cystinuria kunorapwa sei? | Kurapa

Shanduko pakudya kwako, mishonga, uye kuvhiyiwa sarudzo dzekurapa matombo anoumba nekuda kwecystinuria.

Kuchinja kwekudya

Kuderedza kudyiwa kwemunyu kusvika isingasviki magiramu maviri pazuva kwakaratidzwa zvakare kubatsira mukudzivirira kuumbwa kwematombo, sekureva kwechidzidzo muEuropean Journal yeUrology.

Kugadzirisa pH chiyero

Cystine inonyungudika mumuti weti pane yakakwira pH, inova chiyero chekuti acidic kana yakakosha sei chinhu iri. Alkalinizing agents, zvakadai se potassium citrate kana acetazolamide, inowedzera pH yemuti kuti cystine iwedzere kusungunuka. Mimwe alkalinizing mishonga inogona kutengwa pamusoro pekambani. Iwe unofanirwa kutaura nachiremba wako usati watora chero rudzi rwekuwedzera.

Mishonga

Mishonga inozivikanwa seyekubiridzira inomiririra ichabatsira kupwanya cystine makristasi. Iyi mishonga inoshanda nekubatanidza nemakemikari ne cystine kuti iumbe yakaoma inogona kuzonyungudika mumuti. Mienzaniso inosanganisira D-penicillamine uye alpha-mercaptopropionylglycine. D-penicillamine inoshanda, asi ine yakawanda mhedzisiro.

Mishonga yemarwadzo inogona zvakare kunyorerwa kudzora marwadzo apo iwo matombo anopfuura nemu bladder uye kunze kwemuviri.

Kuvhiya

Kana matombo acho akakura uye achirwadza, kana kuvhara rimwe remachubhu anotungamira kubva kuitsvo, vangangoda kubviswa kuvhiya. Kune akatiwandei akasiyana marudzi ekuvhiya kuputsa matombo. Izvi zvinosanganisira zvinotevera maitiro:

• Extracorporeal kuvhunduka wave lithotripsy (ESWLMaitiro aya anoshandisa kuvhunduka kupwanya matombo makuru kuita zvidimbu zvidiki. Haisi inoshanda kumatombo ecystine senge mamwe marudzi ematombo eitsvo.
• Percutaneous nephrostolithotomy (kana nephrolithotomyMaitiro aya anosanganisira kupfuudza chimbo chakakosha kuburikidza neganda rako uye muitsvo yako kuti ubvise matombo kana kuapwanya.

Ndeupi mutarisiro wenguva refu?

Cystinuria mamiriro eupenyu hwese anogona kutarisirwa nemushonga. Matombo aya anowanzoonekwa kuvadiki vechidiki vari pasi pemakore makumi mana uye anogona kuitika zvisingaite nezera.

Cystinuria haina kukanganisa chero zvimwe zvikamu zvemuviri. Mamiriro acho haawanzo kukonzera kutadza kweitsvo. Kakawanda kugadzirwa kwematombo kunokonzera kuvhara, uye nzira dzekuvhiya dzinogona kudikanwa semhedzisiro, dzinogona kukanganisa itsvo kushanda nekufamba kwenguva, maererano neRare Diseases Network.

Cystinuria ingadzivirirwa sei?

Cystinuria haigone kudzivirirwa kana vese vabereki vakatakura kopi yehunhu hwesimba. Nekudaro, kunwa yakawanda mvura, uchideredza kunwa munyu, uye kutora mishonga kunogona kubatsira kudzivirira matombo kubva kuumbika muitsvo.