Izidakamizwa eziphezulu futhi ezizayo zingabamba umuthi ku-PKD
Isifo se-Polycystic isifo (i- PKD ) isifo esiwumzimba esichazwa ubukhona nokukhula okuqhubekayo kwama-cysts ezinso. Ngokungafani ne-okuthiwa ama-cysts alula , i-PKD ayiyona isifo esibuhlungu futhi ingxenyana enkulu yeziguli ze-PKD zisengozini yokuhluleka kwezinso, okudinga i-dialysis noma ukufakelwa kwezinso.
Uma isiguli sifunda ngokuxilongwa kwabo kwe-PKD, umbuzo wokuqala ovela ukuthi ngabe uyaphathwa yini.
Ngaphambi kokuba siqonde ukuthi yini yokwelapha engase isetshenziselwe ukunciphisa lesi sifo, imfucuza emfushane eyingxenye yehomoni ebizwa nge-ADH, noma i-hormone ephikisayo (eyaziwa ngokuthi i-vasopressin) iyadingeka.
Indima ye-ADH ku-PKD
I-ADH yasize ukuphila kuguquke kusukela olwandle kuya ezweni, ama-eons adlule. Uma kwakungeyona ye-ADH, eziningi eziphilayo eziphilayo ngeke zikwazi ukulwa nomthelela onamandla wokudambisa amandla womhlaba ofudumalayo ngaphansi kwelanga elivuthayo!
Ekhiqizwa yingxenye yobuchopho ebizwa ngokuthi i- "hypothalamus," i-ADH iyi-hormone esebenza ezinso futhi ikugcina igcine futhi igcine amanzi. Yilokho okwenza umchamo ubonakale ubumnyama futhi ugxila lapho ungenalo amanzi anele okuphuza noma uchithe usuku ngaphandle kwelanga elishisayo. Ngakho-ke, kungaba nomthelela ekutheni amanzi adinga ukuxoshwa kanjani nokuthi kufanele "abuyele" kanjani ukuze ahlangabezane nezidingo zethu (kuye ngokuthi ezinye izici, kubandakanya ukutholakala kwamanzi kanye nokushisa okuncane).
I-ADH ingene kanjani ekuxoxweni nge-CKD? Ucwaningo lubonise ukuthi i-ADH ingomunye wabagqugquzeli abakhulu bokukhula kwe-cyst (isizathu sokuhluleka kwezinso) ku-PKD. Ngamanye amazwi, uma ungase uhlehlise amazinga e-ADH ngandlela-thile, noma uvimbele isenzo saso kuma-cysts, kungase kwenzeke ukunciphisa ukukhushulwa kwe-cyst nokuqhubekela phambili okungenakulinganiswa kwe-PKD.
Izinketho zamanje zokwelapha
Ukuqonda indima ye-ADH kusiza ekuqondeni izinketho zokwelashwa ezitholakalayo nokuthi kungani zingase zisebenze, kusukela ekukhuleni kwamanzi ekudleni kwezidakamizwa zokusika.
- Ukunwa kwamanzi okwandisiwe : Njengoba kulula lokhu lokhu, ukuphuza amanzi kuyindlela ephumelelayo yokugcina amazinga e-ADH phansi. Amazinga we-ADH akhuphuka lapho uqala ukuphelelwa amanzi. Lokhu kuzosusa impendulo yokoma okwenza uphuze amanzi, okuzoholela ekunciphiseni amazinga e-ADH. Kulesi simo, umqondo ukugcina i-ADH ihlale iphansi ngokumisa ukukhuphuka kwe-ADH. Kuhlelwe ukuthi lokhu kunganciphisa ukuqhubeka kwePKD. Yeka ukuthi kuphumelela kangakanani futhi kunenjongo ukuthi kungokwamaqiniso okwamanje kusasebenza.
- Ukusingatha izinkinga: Uma kungekho ezinye zokwelashwa okwamanje ezitholakalayo, sinqunyelwe ekulawuleni izinkinga ze-PKD. Lezi zihlanganisa ukucindezela kwegazi eliphezulu, izifo zezinso, amatshe ezinso, nama electrolyte angavamile. Umfutho wegazi ophezulu uphathwa ngokusebenzisa imithi ethile ebizwa nge- ACE inhibitors noma ama-ARB . Ukwandiswa kwamanzi okudla kungasiza ekunciphiseni ingozi yezinye izinkinga ezimbili ezihlobene ne-PKD: izifo zezinso nezinso zezinso.
Izinketho zokwelapha zesikhathi esizayo
Ukuqonda kwethu indima ye-ADH ekwenzeni ukuthi i-PKD ibe nzima kakhulu kuye kwaholela ekucwaningeni okuthembisayo okunganikeza izinketho zokwelashwa ezingaphezulu kuka "band-aid" ukungenelela okuchazwe ngenhla.
Ucwaningo lwamanje lugxile ekutholeni izidakamizwa ezingavimbela isenzo se-ADH futhi ngaleyo ndlela zivimbele ama-cyst ukuba angakhuli kakhulu (njengoba ukwanda kwe-cyst ukukhula kwe-cyst ukuhluleka kwezinso kumagciwane e-PKD).
Nazi izibonelo ezimbalwa:
- I-Tolvaptan: Lena imithi eyayivunyelwe ekutholeni ukwelashwa kwamazinga aphansi e-sodium nezenzo ngokuvimbela isayithi (ebizwa ngokuthi i-V2 receptor) lapho i-ADH ingavame ukunamathela khona ezinso (cabanga nge-V2 receptor njenge "keyhole" ukuze okuyinto i-ADH idinga ukuyihlanganisa, kanti i-tolvaptan yiyona "ikhi lokukhohlisa" lapho okwamanje kuzokuvimbela ukuthi kungenzeki).
Isivivinyo se-TEMPO esiveziwe kahle sibonise uhlelo lokusebenza olusemtholampilo lwe-tolvaptan ekunciphiseni ukwehla kwezinso zezinso ku-PKD. Le ndlela ibonakala ihamba kancane ekukhuleni kwezinso, okuholela ekusebenzeni kwezinso ezincane esikhathini esiyiminyaka emithathu. Kodwa-ke, uTolvaptan, engakazuzi izibusiso ze-FDA e-US ngenqubo yokwelashwa kwe-PKD, ngenxa yalokho okukhathazayo ngemiphumela yayo esibindi. Sekuvunyelwe ukwelashwa kwe-PKD kwezinye izingxenye zomhlaba).
- I-Octreotide: Lena inguqulo ende yokwenza amahomoni okuthiwa i-somatostatin. Isivivinyo ngo-2005 saqala ukubika ukuthi ukwelashwa kwezinyanga eziyisithupha nge-somatostatin kunganciphisa ukwanda kwe-cyst. Nakuba siyazi ukuthi ukwehla kwezinso ze-PKD kulandela ukukhula kwe-cyst, isifundo sehlile sithi ukunciphisa ukukhula kwe-cyst, kulokhu, kuzohumusha ekuvikelweni kwezinso ezinempilo.
Khona-ke, ngo-2013 sabona imiphumela yesilingo se-ALADIN eshicilelwe ku-Lancet. Lolu cwaningo lunexesha elide lokulandelela kunezifundo zangaphambilini futhi libonise umthamo wezinso ophansi kakhulu ezigulini eziphathwe nge-octreotide ekulandeleni konyaka owodwa, kodwa hhayi eminyakeni emithathu.
Njengoba sinikezwe idatha esikude kakhulu, kubonakala sengathi i-octreotide ingaba nendima ebalulekile ekwelapheni kwe-PKD. Ngesizathu esithile, kubonakala sengathi i-octreotide inciphisa ukukhula kwevolumu yezinso ngaphezu konyaka owodwa, kodwa imiphumela yaba yinto engabalulekile ngaphezu kwesikhathi eside. Ngokusobala, ucwaningo olunzulu olunzulu olubheka idatha yesikhathi eside kanzima kuyadingeka.
Nakuba bobabili lezi zithunywa zibonise isithembiso kuze kube manje (ngaphezu kwamanye abaphikisi abanjengo-mTOR inhibitors nezinye izidakamizwa ezivivinyweni zomtholampilo), izindleko ukukhathazeka okukhulu. Zonke ezinye izinto zilingana, i-octreotide ingaba yindlela engabizi ngaphezu kwe-tolvaptan yalokho okungase kube ukwelashwa kwansuku zonke. Ngo-2017, ukunikezwa kwamaphilisi e-tolvaptan (ama-15 mg) we-tolvaptan izinsuku ezingu-30 kunenani lika-$ 11,000 kuya ku-$ 12,000 e-US, kanti ama-amps angu-90 we-octreotide (ama-100 mcg injection) agijima ku-$ 300 kuya ku-$ 400!
> Imithombo:
> Nagao S, Kazuhiro N, Makoto K, et al. Ukwanda Kwemanzi Okunciphisa Ukwanda Kwezifo Zezinso Zama-Polycystic kwi-PCK Rat. J Am Soc Nephrol. 2006 Aug; 17 (8): 2220-7. Epub 2006 Jun 28.
> Higashihara E, Nutahara K, Tanbo M, et al. Ingabe ukwanda kwamanzi ukuvimbela ukukhula kwesifo kwi-autosomal isifo se-polycystic esiphezulu se-autosomal? I-Nephrology Dialysis Transplantation. 2014 Sept; 29 (9): 1710-9.
> Torres V, Chapman A, Devuyst O, et al. I-Tolvaptan ezigulini ezine-Autosomal Dominant Polycystic Isifo Sezinso. N Engl J Med 2012; 367: 2407-2418, 2012. I-DOI: 10.1056 / NEJMoa1205511
> Caroli A, Perico N, Perna A, et al. Imiphumela ye-somatostatin analogue esebenzayo ekukhuleni kwezinso kanye ne-cyst kwi-autosomal ephakeme kakhulu ye-polycystic isifo sezinso (ALADIN): ukuhlolwa okungahleliwe, okuyi-placebo okulawulwayo, okuvivinya okuningi. I-Lancet. 2013 Nov 2; 382 (9903): 1485-95. i-doi: 10.1016 / S0140-6736 (13) 61407-5.