Genetics, zviratidzo, uye kuongororwa kwePKD
Polycystic Kidney Disease, kana kuti PKD, inoratidzira maitiro ehutano hwezvirwere zvepso. Sezvo izwi iri rinoratidza, "poly" -cystic inoreva kuvapo kwe-multiple cysts (yakavharwa, isina zviputi, dzimwe nguva izere nemvura) muinso. Iropi cysts muhuwandu haisi kuwanikwa kusingawanzoitiki, asi kuongororwa kwe-cysts muinso hakusi iyo PKD.
PKD, chaizvoizvo, inongova chimwe chezvikonzero zvakasiyana siyana nei munhu achigona kuvaka ma cysts muinso.
Icho chinonzi genetic nhaka uye iyo yePKD inoita kuti ive chinhu chaiyo chaiyo. Icho hachisi chirwere chinorwara, uye chikamu chikuru chevarwere vanogona kuona kuti itsvo dzavo dzinorega kukanganisa, zvichida dialysis kana kuchinja kwepvo.
Dzimwe Mhando dzeCysts
Imwe mhando yeimpso cysts (iyo isiri ye-cysts yakasangana nePKD) inosanganisira:
- Zvirwere zviri nyore zvinokonzera, izvo zvinowanzova chigumisiro chinokonzerwa nekukwegura. Vanenge gumi nezviviri kubva muzana zvevanhu vane makore 50 kusvika ku70 uye 22.1 muzana yevanhu vose vane makore anopfuura makumi manomwe vachave nekrisiti imwe chete muinso.
- Zvinosvibisa (apo cysts inogona kunge ichimiririra kenza mukati meitsvo, dzimwe nguva inonzi maitiro akaoma).
- Yakagadziriswa, sekune varwere vane chirwere chisingaperi chepfungwa (CKD).
Nokudaro, kamwe ma-cysts akaonekwa muinso, danho rinotevera nderokusiyanisa kana iri kushaya makore ane chokuita nekuwana, PKD, kana chimwe chinhu.
Genetics
PKD inowanzojairika yeve genetic disorder, inokonzera vanenge 1 muvanhu 500, uye inoramba iine chinokonzera chinokonzera mafungiro kukanganisa .
Chirwere chacho chinowanzopiwa nhaka kubva kune mumwe wevabereki (90 muzana yematambudziko), kana, kazhinji kazhinji, inova "de-novo"
Kunzwisisa ruzivo rwePKD kunokosha kuti uchinzwisise chirwere 'zviratidzo uye zvidzidzo. Nzira yemhaka kubva kumubereki kusvika kumwana inoparadzanisa pakati pemhando mbiri dzePKD.
Autosomal Inonyanya Kuita PKD (AD-PKD) ndiyo inowanikwa yakawanda inowanikwa nhaka uye 90 muzana yePKD maitiro ndiwo rudzi urwu. Zviratidzo zvinowanzokura mushure mehupenyu hwemazera emakore makumi matatu kusvika ku40, kunyange zvazvo hukuru huri muhuduku husingazivikanwi.
Imajini asina kukwana anogona kunge ari anonzi PKD1, PKD2, kana maGeni ePKD3. Ndeupi weiwo majeni ane chigadziro uye chimiro chechimiro chinogona kunge chine simba guru pamigumisiro inotarisirwa yePKD. Somuenzaniso, chirwere chePKD1, chinowanikwa pa chromosome 16, ndiyo inonyanya kufanana nenzvimbo yekutengesa inoonekwa mu 85 muzana yematambudziko eAdPKD. Kukanganisa mujeni (sezvinoitika kune dzimwe shanduko )wo kunotungamirira kuwedzera kukura kwemasero epithelial muinso uye yakatevera cyst formation.
Kushandisa Autosomal Recessive PKD (AR-PKD) yakawanda uye inogona kutanga mangwanani, kunyange mwana paanenge achikura pamuviri. Chimwe chikonzero nei rudzi urwu rwePKD rushoma ndeyekuti varwere vanorwara havawanzorarama kwenguva yakakwana yekubereka uye kupfuurira kuchinja kwevana vavo.
Zvakare, kuisa muchidimbu, 90 muzana yematambudziko ePKD akagara nhaka, uye yemhando dzakagara nhaka, 90 kubva muzana ndiyo inotenderera yakawanda. Nokudaro, varwere vane PKD vachawanzove vane autosomal inotakura PKD (AD-PKD).
Kusuruvara uye Kuchinja Kunzvimbo
Nzvimbo yekutendeuka ichave nemigumisiro pachirwere chechirwere.
Nekuchinja kwePKD2, cysts inovandudza zvizhinji gare gare, uye kukundikana kwechirwere kazhinji hazviitike kusvika pakupera semakore ekuma70s. Kusiyana neizvi nePKD1 kuchinja kwejena, apo varwere vanogona kukura kusveta kwetsvo mumakore ekuma50s.
Varwere vane mutsauko wePKD2 havawanzozivi kunyange chero nhoroondo yemhuri yePKD. Muchiitiko ichi, nguva dzose zvinogoneka kuti baba vedu vakatakura mutumbi vakafa vasati chirwere chacho chakanga chakaoma kwazvo kukonzera zviratidzo kana kuti chinoda dialysis.
Zviratidzo
Zviratidzo zvakasiyana siyana zvinogona kuonekwa muPKD. Mienzaniso inowanzosanganisira:
- Flank kurwadziwa nemhaka yekuwedzera mafungiro
- Urinary tract infections
- Ipi mabwe (nekuda kwekuderera kwemuti kumhanya mumasikisi)
- Tsanga dzinogona kuve mune dzimwe nhengo dzakadai seropa uye pancreas zvakare
- Varwere vanowanzova nehutano hwepamusoro yeropa hupi dzakapiwa maitsvo 'basa muropa rekugadzirisa mutemo
Kuziva
Kunyange zvazvo kuchinja kwePKD kunowanzovapo pakuberekwa, itsvo dzinenge dzisina kuonekwa panguva iyoyo. Aya majekiti anowedzera kuva mashizha akazara anozadza pamusoro pemakore ekutanga makumi emakore, panguva iyo angatanga kuisa zviratidzo kana zviratidzo nenguva apo mumwe munhu anosvika pamakore makumi matatu. Zvisinei, kufambira mberi kwezvirwere zvepfungwa kusvika pakuguma kunogona kutora makumi emakore kubvira ipapo zvichienda.
Vanhu vakawanda vanoziva nezvehupenyu hwemhuri yePKD vane chidziro chepasi chekuonekwa kuti vane PKD kubva vari vaviri varwere uye vanachiremba vanoziva zvakanaka mhuri yakasimba yechirwere ichi. Muzviitiko apo nhoroondo yemhuri isingagoni kuzivikanwa kana inoita se "yakarongeka," kuongororwa kwakaoma zvikuru uye kunoda kuongororwa nevephrologist. Muchiitiko ichi, mubereki akabatwa angadai akafa asati chirwere ichi chikava nemukana wekufambira mberi kusvika pakuguma kwepfungwa yepfungwa. Pakupedzisira, kana iri nyaya ye "kuchinja mutsauko," pangave pasina PKD iripo mune chero mubereki.
Chirwere chepakutanga chePKD chinoshandiswa uchishandisa zvidzidzo zvekufungidzira sa ultrasound kana CT scan. Zvisinei, nokuti mumwe munhu ane majekiti akawanda muitsvo hazvirevi kuti vane PKD. Inogona kungova yerimwe-zvakare-dzakawanda krissts, kana dzimwe mikana yakafanana neyamarullary cystic chirwere chepfungwa (kwete zvakafanana nePKD).
Kana kuongororwa kuri kusava nechokwadi, kuongorora mazita ekugadzira kunogona kusimbisa kana kuramba kufungidzirwa. Kuenzanisa kwemajeni kunowanzove yakadhura asi uye yakanyanya kushandiswa apo kuongororwa kwakafanana.
Dendaase Course
Inguva yakareba sei avo vane PKD vanotora kukura kwepvo kusakona? Iyi ingangodaro mubvunzo mumwe chete uyo vanhu vachangobva kuongororwa kuti vane PKD vane. Mumamiriro ezvinhu akaipisisa apo varwere vanofambira mberi kuzozadzisa kusagadzikana kwemazino, zvinoda dialysis kana kutapurika, mafungiro epfungwa (GFR) inogona kuderera neanenge ma 5 points pagore. Nokudaro, mumwe munhu anotanga neGFR wevana 50 angasvika kuGFR yevashanu mumakore anoda kusvika mapfumbamwe, panguva iyo dialysis kana kutemwa kunogona kudiwa.
Cherechedza kuti havasi murwere wose ane PKD achazorega kupedzisira kupera. Zvichiri kudikanwa kusimbiswa ndezvokuti havasi vose vane PKD vanozofambira mberi kusvika pavanenge vachida dialysis. Varwere vane PKD2 gene mutation zviri pachena kuti vanomira mukana wakanaka wekudzivisa kukanganisa kwakakwana kwemasimba. Ichi ndicho chikonzero, semunhu wose, pasi pehafu yePKD mazamu achaonekwa munguva yemararamiro ehutano, sezvo chirwere chacho chingave chetachiona chinyararire.
> Sources:
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