Ni ayahe mabuye y'impyiko nawe utera? (Amabuye ya Cystine) Reka tuyamenye mu buryo bworoshye!

Ese wigeze ujya mu bitaro ufite amabuye y'impyiko n'ububabare bukabije mu mugongo, mu nda yo hasi, cyangwa ku ruhande? Cyangwa se wigeze wumva umuntu uzi wagize ikibazo nk'icyo? Amabuye y'impyiko ni ikintu kibabaza cyane. Aya mabuye ashobora kubaho mu buryo butandukanye. Uyu munsi tugiye kuvuga ku bwoko bw'amabuye y'impyiko akunze kugaragara gake, ariko ashobora guteza ibibazo iyo adavuwe neza, cyane cyane mu rubyiruko, yitwa amabuye ya cystine .

Aya mabuye ya cystine ni ayahe?

Mu magambo make, amabuye ya cystine ni ubwoko bw'amabuye y'impyiko. Amabuye y'impyiko ni ibikomere bikomeye bibaho iyo imiti itandukanye mu nkari zawe ikusanyije, nk'utubuye duto. Amabuye ya cystine agizwe n'ikinyabutabire cyitwa cystine . Iyi cystine ni aside amine iboneka mu mibiri yacu. Ubusanzwe, icyagombye kubaho ni uko iyi cystine yongera kwinjizwa mu mubiri n'impyiko. Ariko rimwe na rimwe, iyo ibi bitagenze neza, cystine irundanya mu nkari ikarema amabuye.

Utubuye twa cystine akenshi tuba tunini cyane . Hari ubwo biba ngombwa ko tubagwa kugira ngo dukurweho kugira ngo hirindwe kwangirika kw'impyiko cyangwa inzira y'inkari. Ikibabaje ni uko abantu benshi barwara cystine bashobora kurwara utu tubuye inshuro nyinshi mu buzima bwabo. Ariko ntugahangayike, hari uburyo bwo kuvura iyi ndwara utabazwe. Urugero, ushobora kuyirwanya uhindura indyo yawe cyangwa ukoresheje imiti ipima pH y'inkari zawe.

None se Cystinuria ni iki?

Cystinuria ni indwara idakunze kugaragara, umuntu avukana . Iki ni igihe ikintu cyitwa cystine cyiyongera mu nkari zawe. Abantu bagera ku bihumbi icumi barwaye iyi ndwara. Niba ufite cystinuria, umubiri wawe ugira ikibazo cyo gushonga kwa cystine. Iki ni cyo gihe yiyongera ikarema amabuye ya cystine. Cystinuria ishobora gutuma ugira amabuye y'impyiko inshuro nyinshi mu buzima bwawe.

Ibi biterwa n'ubwoko bwa gene yitwa (autosomal recessive gene) . Ibi bivuze ko iyo umwana azunguye iyi gene kuri nyina na se, umwana azarwara cystinuria. Iyi ni indwara ihoraho kandi ntishobora gukira burundu. Ariko, ishobora kugenzurwa neza iyo habayeho kuvurwa neza .

Ni gake gashoboka gute kugira amabuye y’impyiko ya cystine?

Ugereranyije n'ubundi bwoko bw'amabuye y'impyiko, amabuye ya cystine aboneka gusa muri 1% kugeza kuri 2% by'abarwayi. Ibyo ni gake cyane. Ariko igitangaje ni uko 6% kugeza kuri 8% by'amabuye y'impyiko mu bana ari amabuye ya cystine . Mu by'ukuri, 80% by'abantu bakura amabuye ya cystine bakura ibuye ryabo rya mbere mbere y'imyaka 20.Ariko, birashoboka gutera rimwe cyangwa menshi muri aya mabuye mu gihe icyo ari cyo cyose.

Ni ibihe bimenyetso by'amabuye ya cystine?

Ibimenyetso by'amabuye ya cystine bisa cyane n'iby'andi mabuye yo mu mpyiko. Utangira kumva ibimenyetso gusa iyo ibuye rivutse.

Ibintu nk'ibi bishobora kubaho:

• Ububabare bukabije ku ruhande rumwe rw'umubiri wawe (umugongo) .
• Kumva ububabare mu gice cy'ikibuno cyangwa mu nda yo hasi.
• Kubona amaraso mu nkari .
• Kuruka no kubabara mu nda (isesemi).
• Kubabara iyo uri kunyara (ibi bibaho iyo ibuye riri hafi y'uruhago).

Hari igihe ibuye rya cystine rishobora gufatirwamo rigatera indwara yo mu nzira y'inkari (UTI) . Kubera ko amabuye ya cystine ari manini gato ugereranyije n'andi mabuye, ashobora gutera izo ngorane.

Kuki aya mabuye ya cystine avuka?

Nkuko twabivuze mbere, amabuye ya cystine akunze kugaragara ku bantu bafite indwara idakunze kugaragara yitwa cystinuria . Cystinuria iterwa n'impinduka mu turemangingo twa SLC3A1 na SLC7A9 . Iyi ndwara ituma umubiri wawe udashobora gukoresha neza aside amine cystine. Hanyuma irarundanya kandi igakora amabuye. Aya mabuye ashobora kuba ahantu hose mu nzira y'inkari zawe, nko mu ruhago rwawe cyangwa mu mpyiko.

Mu magambo make, iki ni ikibazo cy’uturemangingo. Ibyo bivuze ko atari wowe ubyiteye.

Amabuye ya cysteine ​​​​akorwa n'iki?

Amabuye ya Cystine agizwe na Cystine . Nkuko twabivuze mbere, Cystine ni aside amine . Aside amine ni ubwoko bwa poroteyine. Afasha umubiri wacu gusana ingirangingo, kubaka imitsi, no kurwanya indwara. Abantu bafite cystinuria bafite ibibazo by'uko impyiko zabo zifata cyangwa zigakoresha iyi aside amine.

Ni izihe ngorane zishobora guterwa n'amabuye ya cystine?

Cystinuria ni indwara ibaho ubuzima bwose. Muganga wawe ashobora kugufasha guhangana n'iyi ndwara akoresheje imiti, guhindura imirire, ndetse wenda no kubagwa. Ariko, ushobora kurwara amabuye ya cystine mu buzima bwawe bwose. Aya mabuye ashobora kubabaza cyane mu gihe arimo arashira.

Amabuye ya cystine ashobora gutera ingorane nka:

• Indwara z'impyiko .
• Kuziba k'umuyoboro w'inkari (umuyoboro utwara inkari ziva mu mpyiko zijya mu ruhago).
• Indwara zo mu nzira y'inkari (UTIs) .
• Kwangirika kw'impyiko.

Wamenya ute ko ufite amabuye ya cystine?

Muganga wawe azasuzuma utubuye twa cystine akurikije ibimenyetso byawe kandi agafata amateka yawe yose y’ubuzima, harimo niba hari umuntu wo mu muryango wawe wigeze kugira izi ndwara. Byongeye kandi, ushobora gukora ibi bikurikira:

• Ibizamini byo gufata amashusho , nka scan za ultrasound na CT scan , hamwe n'ibizamini by'inkari.
• Kohereza ibuye muri laboratwari kugira ngo ripimwe. Niba bishoboka, tora ibuye ryavuye mu nkari zawe, maze muganga wawe aryohereze muri laboratwari kugira ngo risesengurwe. Muri ubwo buryo, bashobora kumenya neza imiterere y'ibuye iryo buye rikozemo.

Indwara ya cystinuria ivurwa ite?

Ikintu cy'ingenzi cyane mu kuvura amabuye ya cystine ni ukugerageza kuyakumira mbere na mbere . Bumwe mu buryo bwiza bwo kubikora ni ukugabanya ingano ya cystine mu nkari zawe. Muganga wawe ashobora gukoresha imiti ivanze n'impinduka mu mirire kugira ngo bigabanye umubare w'amabuye ukora. Niba imiti n'impinduka mu mirire bitabujije amabuye gukora, cyangwa niba amabuye ari manini cyane, kubagwa ni ubundi buryo.

Umuti

Niba umuganga wawe atekereza ko kunywa amazi menshi bizafasha ibuye kwikiza ubwaryo, ashobora kukwandikira imiti y’ububabare itangwa na muganga (OTC) ku bubabare. Bashobora kandi kukwandikira imiti nka tiopronin na d-penicillamine kugira ngo ifashe mu gukumira kuremwa kw’amabuye mu gihe kizaza.

Gahunda yo kurya

Muganga wawe ashobora kukugira inama yo guhindura uburyo bwo kurya kugira ngo wirinde ko amabuye avuka. Bimwe muri ibyo birimo:

• Kunywa amazi menshi. Ibi bishobora gufasha gushonga amabuye ya cystine. Muganga wawe ashobora kukubwira kunywa ounces 96 (hafi litiro 2.8) z'amazi ku munsi, kunywa ikirahure cy'amazi buri saha, cyangwa kunywa amazi igihe ukangutse nijoro.
• Gabanya ingano ya sodiyumu (umunyu) urya. Ibyo bivuze ko ugomba kureka kurya ibiryo bikonje cyangwa ibikonje. Si umunyu dushyira mu muceri gusa, ahubwo harimo n'umunyu wihishe mu bintu nk'ibiryo bitose, ibiryo bitose, isosi, ibiryo bipfunyitse, n'ibiryo bigurwa mu maduka. Bityo rero ugomba kubyitondera.
• Poroteyine y'amatungo , bivuze kurya inyama nkeya ahubwo ukarya poroteyine ikomoka ku bimera. Urugero, ushobora kongeramo ibintu nk'ibishyimbo, lentile na soya mu mirire yawe aho kongeramo inyama n'amafi.

Kubaga

Amabuye manini ashobora kwangirika iyo adakoreshejwe mu nzira y'inkari. Aya mabuye ashobora kubabaza cyane kandi agashobora kubuza inkari gutembera mu mubiri. Hari uburyo butandukanye muganga wawe ashobora gukoresha kugira ngo akureho ibuye:

• Kamera nto kandi yoroshye inyuzwa mu muyoboro w'inkari (urethra), inyuze mu ruhago no mu muyoboro w'inkari (ureteroscopy), kugira ngo haboneke ibuye rigakurwamo (ureteroscopy) .
• Uruhu rw'umugongo rucibwa gato, umuyoboro unyuramo ujya mu mpyiko, hanyuma ibuye rikavanwaho.(impyiko zo mu ruhu).

Wamenya ute niba ubuvuzi bwagenze neza?

Muganga wawe ashobora gutegeka ko ukorerwa isuzuma ry'inkari buri gihe kugira ngo arebe urugero rw'uburwayi mu nkari yawe. Iki kizamini kizamufasha kumenya niba gahunda yawe yo kuvura ikora cyangwa idakora.

Ese amabuye ya cystine ashobora gushonga?

Yego, ibuye rya cystine rishobora kwanduzwa. Ariko bisaba gukurikiranwa witonze no gukomeza kuvurwa . Aho gushonga burundu, rishobora gukorwa rito kandi byoroshye kwanduzwa.

Ni ibihe biribwa bifasha mu gihe cystinuria?

Ubushakashatsi bwagaragaje ko abantu barwaye cystinuria bashobora kungukirwa no guhindura imirire. Bimwe muri ibyo birimo kunywa amazi menshi, kurya imbuto n'imboga nyinshi, no kurya indyo irimo umunyu muke. Imbuto z'amacunga nk'amacunga n'indimu, imboga z'icyatsi, n'imboga z'amababi ni byiza cyane.

Ese amabuye ya cystine ashobora kwirindwa?

Cystinuria ni indwara umuntu avukana, bityo ntabwo ishobora kwirindwa. Ariko, ukurikije gahunda yo kuvura muganga wawe , ushobora gukumira ikorwa ry'amabuye ya cystine . Ni ukuvuga ko ushobora kugenzura ikorwa ry'amabuye.

Ese cystinuria ishyira ubuzima mu kaga?

Ibi akenshi ntabwo bishyira ubuzima mu kaga . Abantu benshi ntibarwara indwara zikomeye nko kunanirwa kw'impyiko. Icy'ingenzi ni ugukorana na muganga wawe kugira ngo ubone umuti ukubereye. Abantu benshi bakira neza ubuvuzi. Birashoboka kandi kubona umuti udafite ingaruka mbi ku mpyiko, uruhago, cyangwa inzira y'inkari.

Ni iki giteganijwe ku mabuye ya sistine mu gihe kizaza?

Amabuye ya Cystine ashobora kwirema inshuro nyinshi mu buzima bwawe. Niba ufite cystinuria, uzakenera gukomeza guhindura imirire no gufata imiti kugira ngo wirinde ko amabuye ya Cystine yirema. Muganga wawe azagukurikirana kandi arebe neza ko uvura neza amabuye mashya ya Cystine. Ibi ni ukugira ngo wirinde kwangirika k'inzira y'inkari, impyiko, n'uruhago.

Ni ryari ngomba kujya kwa muganga?

Niba ufite ibimenyetso by'ibuye ry'impyiko, menya neza ko wagiye kwa muganga . Dore bimwe mu bimenyetso bikunze kugaragara:

• Kubabara mu gifu, mu ruhande rw'umugongo, cyangwa mu kibuno.
• Isesemi no kuruka.
• Kubabara iyo uri kunyara.

Cystinuria ni indwara ibaho ubuzima bwose ituma amabuye ya cystine avuka. Fatanya na muganga wawe kugira ngo utegure gahunda yo kuvura kugira ngo amabuye adavuka. Kubera ko iyi ndwara ari iy’uturemangingo, abantu bafite cystinuria bashobora kubanza kugisha inama ku bijyanye n’uturemangingo niba batekereza gushinga umuryango.Bishobora kandi kuba ingirakamaro kubibona. Inkuru nziza ni uko ubusanzwe iyi ndwara ishobora kugenzurwa hakoreshejwe uburyo bwo kuvura kandi ntitera impyiko gucika intege cyangwa kwangirika gukomeye k'inzira y'inkari.

Ibintu by'ingenzi cyane ugomba kwibuka (Ubutumwa bwo kujyana mu rugo)

Noneho reka tuvuge muri make bimwe mu bintu ugomba kwibuka mu byo twavuze:

• Amabuye ya Cystine ni ubwoko bw'ibuye ry'impyiko rikorwa bitewe n'indwara y'uturemangingo yitwa cystinuria .
• Ibi bishobora kuba binini cyane kandi bishobora kubaho inshuro nyinshi mu buzima bwose.
• Ibimenyetso bishobora kuba birimo ububabare bukabije, amaraso mu nkari, no kuruka .
• Ubuvuzi burimo kunywa amazi menshi, kugabanya umunyu na poroteyine z'amatungo, ndetse nibiba ngombwa, imiti no kubagwa .
• Nubwo cystinuria idashobora gukira burundu, iyo ivuwe neza, kurema amabuye bishobora kugenzurwa kandi ubuzima busanzwe bugashobora kubaho.
• Niba ufite ibimenyetso by'utubuye mu mpyiko , ntugapfushe umwanya ngo urebe muganga.

Menya ko aya makuru ari ay'amakuru gusa kandi ntagomba gufatwa nk'inama z'abaganga. Ni byiza buri gihe ku bijyanye n'ibibazo by'ubuzima.

Amabuye y'impyiko , amabuye ya cystine, cystinuria, amabuye y'inkari, indwara z'impyiko, indwara z'uturemangingo, indwara z'inkari