Zvinodhaka-uye-zvinodhaka zvinogona kubata chirwere chePKD
Chirwere chePycycystic chinonzi kidney ( PKD ) chirwere chinokonzerwa nehutachiona hunoratidzwa nekuvapo uye kukura kunowedzera kwekriss muitsvo. Kusiyana neainzi inonzi cysts iri nyore , PKD haisi chirwere chinorwara uye chikamu chakakura chevarwere vePKD vari pangozi yehvo kusakundikana, zvichida kubata dialysis kana kuchinja kwepvo.
Apo murwere anodzidza nezvehutano hwavo hwePKD, mubvunzo wokutanga unouya ndewekuti unorapwa.
Tisati tanzwisisa zvinogona kurapwa mushonga unogona kuderedza chirwere ichi, pfupi pfupi pane basa rehomoni inonzi ADH, kana kuti anti-diuretic hormone (inozivikanwawo sevasopressin) inokosha.
Basa reAdH muPKD
ADH yakabatsira upenyu kuchinjwa kubva kumhenderekedzo yegungwa kuenda kune imwe nyika, makore gumi apfuura. Kana zvisiri zveAdh, zvipenyu zvakawanda zvingadai zvisingakwanisi kurwisa simba rakaoma rekuita simba remhepo pasi penyika inopisa zuva!
Yakabudiswa nechikamu cheuropi chinonzi "hypothalamus," ADH inonzi hormone inoshanda painso uye ichiita kuti ichengetedze uye kuchengetedza mvura. Ndicho chinoita kuti urini ione yakajeka uye yakanyanyisa paunenge usina mvura yakakwana yekunwa kana kupedza zuva kunze kwezuva rinopisa. Izvi zvinogona, saka, zvinokonzera kuti mvura yakawanda inoda kuve yakasunungurwa uye kuti yakawanda sei inofanira "kuchinjwa" kuti iite zvatinoda (zvichienderana nezvimwe zvinhu, kusanganisira kutora mvura uye kunyange kutonhora kwemazuva).
Ko ADH inokodzera sei mukukurukurirana kweCKD? Zvidzidzo zvakaratidza kuti ADH ndomumwe wevanhu vanokurudzira kukura kwechistst (chikonzero chetsvo kusakundikana) muPKD. Mune mamwe mazwi, kana iwe unogona kuita zvakaderera mazinga ADH, kana kudzivisa chiito chayo pamakristi, zvingave zvichigoneka kuderedza kukura kwechistst uye kufambira mberi kusingagoneki kwePKD.
Current Treatment Options
Kunzwisisa basa re ADH kunobatsira pakunzwisisa nzira dzekurapa dzinowanikwa uye nei vachigona kushanda, kubva pakuwedzera kwemvura kunotapira zvinodhaka.
- Kuwedzerwa kwekudya kwemvura : Sezvakanzwisiswa sezvo izvi zvinonzwika, kunwa mvura inzira inobatsira yekuchengetedza zvikamu zveADH pasi. Nhamba dze ADH dzinokwira apo iwe unotanga kuoma. Izvi zvichabvisa mhinduro ye nyota ichiita kuti unwe mvura, iyo inotungamirira kuderedza mazinga ADH. Muchiitiko ichi, pfungwa ndeyekuti kuchengetedza ADH nguva dzose kuve nekuderera nekuregererwa kweAdh kusimuka. Inotsanangurwa kuti izvi zvinogona kuderedza kufambira mberi kwePKD. Izvo zvinobudirira uye zvine chinangwa izvo zviri mumashoko chaiwo zvichiri kusakanganiswa zvakadaro.
- Kugadzirisa zvinetso: Mukusabatwa kweimwe mishonga chaiyo ikozvino inowanika, isu tisingakwanisi kugadzirisa zvinetso zvePKD. Izvi zvinosanganisira kuwedzera kweropa, zvirwere zvepvo, itsvo dzemabwe, uye kusagadzikana kwema electrolytes. High blood pressure inobatwa uchishandisa mishonga inonzi ACE inhibitors kana ARBs . Kuwedzerwa kwemvura kunwa kunogonawo kubatsira mukuderedza dambudziko rehumwe humwe humwe hutano hukuru hunokonzerwa nePKD: zvirwere zveputsvo neminyo yemabwe.
Future Treatment Options
Nzwisiso yedu yebasa raAdH pakuwedzera kwePKD yakatungamirira kukutsvakurudza kwakavimbiswa kunogona kupa mishonga yekodzero yakanyanyisa kunze kwebato re "band-aid" rinotsanangurwa pamusoro apa.
Ongororo yemazuva ano inonyanya kutsvaga kuwana mishonga inogona kudzivirira chiito che ADH uye nekudaro kudzivirira maitiro ekukura kukura (kubva pakuwedzera kwekristi ndiko crux yechiputsi kusakatika muvarwere vePKD).
Heano mienzaniso mishomanana:
- Tolvaptan: Uyu mushonga waive wakagamuchirwa kurapwa kwemazinga epasi e-sodium uye unoita nekuvhara nzvimbo (inonzi V2 receptor) iyo ADH yaiwanzobatanidza nayo muinso (funga nezveV2 receptor se "keyhole" ku iyo ADH inoda kuisunga, asi tolvaptan ndiyo "fikisi inowanikwa" iyo apo iripo ichadzivirira izvozvo kuti zvirege kuitika).
Chirwere cheTEMPO chakanyatsoratidzwa chiratidze chinogona kliniki yekushandiswa kwe tolvaptan inononoka kuderera kwepfungwa basa muPKD. Izvo zvinoratidzika sezviri kunonoka kukura kweiyo itsvo 'volume, iyo inotungamirira kushoma kwepfungwa basa kunodarika kwemakore matatu emakore. Tolvaptan, zvisinei, haasati awana zvikomborero zveDAA muUnited States nokuda kwekurapwa kwePKD, pamwe nekuda kwekunetseka pamusoro pemigumisiro yayo pachiropa. Yatove yakagamuchirwa kurapwa kwePKD kune dzimwe nzvimbo dzenyika).
- Octreotide: Iyi ndiyo nguva yakareba yekuita maitiro ehomoni inonzi somatostatin. Chiyero muna 2005 chokutanga chakashuma kuti mwedzi mitanhatu yekurapa ne somatostatin inogona kukurumidza kukura. Kunyangwe tichiziva kuti kuderera kwehupenzi basa muPKD inotevera kukura kwechist, chidzidzo chacho chakamira pfupi kutaura kuti kuderedza kuwedzera kwechistst, munyaya iyi, kwaizoshandura muchipatara chinoreva kudzivirirwa kwepso.
Zvadaro, muna 2013 takaona migumisiro yechirwere che ALADIN chakabudiswa muLancet. Chidzidzo ichi chaiva nenguva yakareba yekutevera kupfuura zvidzidzo zvekare uye yakaratidza huwandu hwepfungwa yepamusoro kune varwere vanobatwa ne octreotide mugore rimwe chete kutevera, asi kwete mumakore matatu.
Tichipiwa nhoroondo yatakasvika kare, zvinoratidzika kuti octreotide inogona kuita basa mukurapa kwePKD. Nokuda kwechimwe chikonzero, zvinoratidzika kuti octreotide inononoka kukura kwepvovo yeminzwi pamusoro pegore rimwe chete, asi migumisiro inova isina basa pamusoro penguva refu. Zviri pachena, zvidzidzo zvakanyatsotarisa zvinotarisa kwenguva yakareba yemigumisiro yakaoma data inodiwa.
Kunyange zvazvo vaviri ava vashandi vakaratidza chivimbiso kusvikira zvino (kuwedzera kune vamwe vanokakavara seMTOR inhibitors nemimwe mishonga mumakirini emakirini), mari inonyanya kukosha. Zvimwe zvinhu zvose zvakaenzana, octreotide inogona kuva imwe nzira inodhura kupfuura tolvaptan nokuda kwechinhu chaicho chinogona kuva kurapwa kweupenyu hwose. Muna 2017, kushandiswa kwemazuva makumi matatu (15 mg) tolvaptan mapiritsi inotengeswa pa $ 11,000 kusvika $ 12,000 muU.S., asi 90 amps ectreotide (100 mcg injections) inotambira $ 300 kusvika $ 400!
> Sources:
> Nagao S, Kazuhiro N, Makoto K, et al. Kuwedzerwa Kwemvura Kunowedzera Kuwedzera kwePolycystic Disease Disease muPad Rat. J Am Soc Nephrol. 2006 Aug; 17 (8): 2220-7. Epub 2006 Jun 28.
> Higashihara E, Nutahara K, Tanbo M, et al. Ko mvura yakawanda inowanikwa inodzivirira chirwere chinowedzera mu autosomal inowanzokanganisika yepurocystic chirwere chepfungwa here? Nehrology Dialysis Transplantation. 2014 Sept; 29 (9): 1710-9.
> Torres V, Chapman A, Devuyst O, et al. Tolvaptan muVachipatendi vane Autosomal Dominant Polycystic Kidney Disease. N Engl J Med 2012; 367: 2407-2418, 2012. DOI: 10.1056 / NEJMoa1205511
> Caroli A, Perico N, Perna A, et al. Migumisiro yakareba yemainatostatin analogue pamusoro penzvo uye kukura kwechirst in autosomal inowanzobatwa nechirwere chepurocystic (ALADIN): randomized, placebo-controlled, multicentre trial. Lancet. 2013 Nov 2; 382 (9903): 1485-95. doi: 10.1016 / S0140-6736 (13) 61407-5.