Mankhwala osokoneza bongo amatha kuchiritsa mankhwala a PKD
Matenda a impso a Polycystic ( PKD ) ndi matenda a chibadwa omwe amadziwika ndi kukhalapo ndi kukula kwa mphuno mu impso. Mosiyana ndi zomwe zimatchedwa cysts , PKD si matenda a benign ndipo kachilombo kakang'ono ka odwala PKD ali pangozi ya impso kulephera, kufuna dialysis kapena kusintha kwa impso.
Pamene wodwalayo aphunzira za momwe amawadziwira PKD, funso loyamba limene limabwera ndiloti limachiritsidwa.
Tisanamvetsetse kuti chithandizo chingagwiritse ntchito bwanji kuchepetsa matendawa, nthawi yayitali yomwe imakhala ndi hormone yotchedwa ADH, kapena hormonic anti-diuretic (yotchedwanso vasopressin) ndi yofunikira.
Udindo wa ADH mu PKD
ADH inathandiza moyo kuchoka ku nyanja kupita ku nthaka, zaka zapitazo. Ngati sizinali za ADH, zamoyo zambiri sizikanatha kulimbana ndi chikoka chakuda cha nthaka yomwe imatenthetsa pansi pa dzuwa!
Zopangidwa ndi mbali ya ubongo wotchedwa "hypothalamus," ADH ndi hormone yomwe imagwira pa impso ndikuyisunga ndi kusunga madzi. Ndichomwe chimapangitsa mkodzo kuwoneka mdima ndikuwunika kwambiri pamene simunakhale ndi madzi okwanira kapena mumakhala kunja kunja kutentha. Choncho, zimatha kuwonetsa kuti madzi amafunika kuchulukitsidwa ndi kuchuluka kotani kuti "zisinthidwe" kuti tikwanitse zosowa zathu (malingana ndi zinthu zina, kuphatikizapo madzi omwe timadya komanso nyengo yozungulira).
Kodi ADH ikugwirizana bwanji ndi zokambirana za CKD? Kafukufuku wasonyeza kuti ADH ndi mmodzi wa akuluakulu othandiza kukula kwa kansalu (chifukwa cha impso kulephera) mu PKD. Mwa kuyankhula kwina, ngati mutatha kuchepetsa zigawo za ADH, kapena kulepheretsa zomwe zikuchitika pa mphutsi, zingakhale zotheka kuchepetsa kukula kwa kstasi ndi kupititsa patsogolo kwa PKD.
Zotsatira Zamakono Zamakono
Kumvetsetsa udindo wa ADH kumathandiza kumvetsetsa njira zomwe angapezere mankhwala komanso chifukwa chake angagwire ntchito, kuchokera kuwonjezeka kwa madzi kuti adziwe mankhwala osokoneza bongo.
- Kuwonjezeka kwa madzi : Monga zophweka ngati izi zikumveka, madzi akumwa ndi njira yowonetsera kuti zigawo za ADH zikhale pansi. Ma ADH amapita pamene mukuyamba kutaya madzi. Izi zidzakuthandizani kumvetseratu ludzu kuti mumwe madzi, zomwe zidzatsikira kuchepetsa zigawo za ADH. Pachifukwa ichi, lingaliro ndikuteteza ADH nthawi zonse pansi poyesa ADH kukwera. Zinalembedwa kuti izi zikhoza kuchepetsa kukula kwa PKD. Ndizothandiza komanso zogwira mtima zomwe ziri zenizeni komabe ziribe zodabwitsa ngakhale.
- Kulimbana ndi mavuto: Ngati palibe mankhwala enaake omwe alipo pakalipano, sitingathe kuthetsa mavuto a PKD. Izi zimaphatikizapo kuthamanga kwa magazi, matenda a impso, miyala ya impso, ndi electrolytes osadziwika. Kuthamanga kwa magazi kumachiritsidwa pogwiritsa ntchito mankhwala enieni otchedwa ACE inhibitors kapena ARBs . Kuwonjezeka kwa madzi kumathandizanso kuchepetsa chiopsezo cha mavuto ena akuluakulu a PKD: matenda a impso ndi miyala ya impso.
Tsogolo Labwino Njira
Kumvetsetsa kwathu kwa udindo wa ADH pakuwonjezeka kwa PKD kwachititsa kuti pakhale kafukufuku wowonjezera omwe angapereke njira zowonjezera zowonjezera kupyolera muzinthu za "band-aid" zomwe tazitchula pamwambapa.
Kafukufuku wamakono akugwiritsidwa ntchito popeza mankhwala omwe angalepheretse kuchitapo kanthu kwa ADH ndipo potero amachititsa kuti mphukira zisakulire zazikulu (popeza kukula kwa cyst kukula ndi matenda a impso odwala PKD).
Nazi zitsanzo zingapo:
- Tolvaptan: Awa ndi mankhwala omwe poyamba adavomerezedwa kuti azitha kulandira ma sodium otsika ndi zochita poletsa malowa (otchedwa V2 receptor) omwe ADH angalowe nawo ku impso (taganizirani za V2 receptor ngati "chimbudzi" kuti chimene ADH chiyenera kuyikapo, pamene tolvaptan ndi "fake fake" kuti pakali pano iteteze izo kuchitika).
Chidziwitso chabwino cha TEMPO chawonetsa chithandizo cha mankhwala cha tolvaptan chomwe chimachepetsa kuchepa kwa ntchito ya impso mu PKD. Mankhwalawa akuwoneka kuti akuchedwa kukula kwa impso ', zomwe zimapangitsa kuti ntchito yaing'ono yaing'ono ichepe pa zaka zitatu. Tolvaptan, komabe, sanalandire madalitso a FDA ku US kwa mankhwala a PKD, makamaka chifukwa cha nkhawa za zotsatira za chiwindi. Zavomerezedwa kale kuti zithandize PKD kumadera ena a dziko lapansi).
- Octreotide: Imeneyi ndiyo njira yamakono yotchedwa hormone yotchedwa somatostatin. Chiyeso mu 2005 choyamba chinanena kuti chithandizo cha miyezi isanu ndi umodzi ndi somatostatin chikhoza kuchepetsa kukula kwa kansalu. Ngakhale tikudziwa kuti kugwira ntchito kwa impso mu PKD kumatsatira kukula kwa cyst, kuphunzira sikungakhalenso kunena kuti kuchepetsa kukula kwa nthendayi, pakali pano, kungatanthauzire ku chitetezo cha impso.
Kenaka, mu 2013 tinawona zotsatira za mayesero a ALADIN ofalitsidwa ku Lancet. Phunziroli linali ndi nthawi yayitali yotsatilapo kusiyana ndi maphunziro apitalo ndipo anawonetsa mphuno yotsika kwambiri kwa odwala opatsidwa ndi octreotide pakatha chaka chimodzi, koma osati zaka zitatu.
Chifukwa cha deta yomwe takhala nayo pakalipano, zikuoneka kuti octreotide ikhoza kukhala ndi mwayi wothandizira PKD. Pazifukwa zina, zikuwoneka kuti octreotide imachepetsanso kukula kwa impso kwa chaka chimodzi, koma zotsatira zake zimakhala zopanda phindu kwa nthawi yaitali. Mwachiwonekere, kufufuza kwakukulu kwambiri kuyang'ana pa zotsatira zowopsya zotsatila deta ndizofunika.
Ngakhale amithenga onsewa asonyeza malonjezo mpaka pano (kuphatikizapo otsutsa ena monga mTOR inhibitors ndi mankhwala ena mu mayesero a zamankhwala), mtengo ndi chinthu chodetsa nkhawa kwambiri. Zonsezi zikhale zofanana, octreotide ikhoza kukhala yotsika mtengo kusiyana ndi tolvaptan zomwe zingathe kukhala chithandizo chamoyo wonse. Mu 2017, mapiritsi a tolvaptan amatha masiku 30 (15 mg) kwa tolvaptan mapiritsi amtengo wapatali pa $ 11,000 mpaka $ 12,000 ku US, pamene 90 amps a octreotide (100 mcg injection) amathamanga madola 300 mpaka $ 400!
> Zotsatira:
> Nagao S, Kazuhiro N, Makoto K, et al. Kuwonjezeka kwa Mavitamini a Madzi Kumachepetsa Kuwonjezeka kwa Matenda a Polycystic Impso mu PCK Rat. J Am Soc Nephrol. 2006 Aug, 17 (8): 2220-7. Epub 2006 Jun 28.
> Higashihara E, Nutahara K, Tanbo M, et al. Kodi kuchuluka kwa madzi kumathandiza kupewa matenda opatsirana pogwiritsa ntchito matenda a impso? Nephrology Dialysis Transplantation. 2014 Sept; 29 (9): 1710-9.
> Torres V, Chapman A, Devuyst O, et al. Tolvaptan mwa Odwala ndi Autosomal Dominant Polycystic Impso Matenda. N Engl J Med 2012; 367: 2407-2418, 2012. DOI: 10.1056 / NEJMoa1205511
> Caroli A, Perico N, Perna A, et al. Zotsatira za longacting somatostatin analogue pa impso ndi cyst kukula mu autosomal kwambiri polycystic matenda a impso (ALADIN): randomized, placebo ankalamulira, multicentre mayesero. Lancet. 2013 Nov 2; 382 (9903): 1485-95. lembani: 10.1016 / S0140-6736 (13) 61407-5.