Konke Odinga Ukukwazi Nge-ADPKD

-Delile

• Izimpawu ze-ADPKD
• Ukwelashwa kwe-ADPKD
• Imiphumela emibi yokwelashwa kwe-ADPKD
• Ukuhlolisiswa kwe-ADPKD
• Ukuxilongwa kwe-ADPKD
• Izimbangela ze-ADPKD
• Izinkinga
• Isikhathi sokuphila nombono

I-Autosomal ephezulu ye-polycystic kidney disease (ADPKD) yisimo esingelapheki esenza ukuthi ama-cysts akhule ezinso.

INational Institute of Diabetes and Digestive and Kidney Diseases ibika ukuthi ithinta umuntu olinganiselwa kwabangu-400 kuya ku-1 000.

Funda ukuze ufunde kabanzi mayelana ne:

• izimpawu
• izimbangela
• ukwelashwa

Izimpawu ze-ADPKD

I-ADPKD ingadala izimpawu ezahlukahlukene, kufaka phakathi:

• ikhanda
• ubuhlungu emhlane wakho
• ubuhlungu ezinhlangothini zakho
• igazi emchameni wakho
• usayizi wesisu okhuphukile
• umuzwa wokugcwala esiswini sakho

Izimpawu zivame ukukhula lapho usumdala, uphakathi kweminyaka yobudala engama-30 nengama-40 ubudala, yize zingavela naseminyakeni esezingeni eliphakeme kakhulu. Kwezinye izimo, izimpawu zivela ebuntwaneni noma ebusheni.

Izimpawu zalesi simo zivame ukuba zimbi ngokuhamba kwesikhathi.

Ukwelashwa kwe-ADPKD

Alikho ikhambi elaziwayo le-ADPKD. Kodwa-ke, ukwelashwa kuyatholakala ukusiza ukuphatha lesi sifo kanye nezinkinga zaso ezingaba khona.

Ukusiza ukubambezela ukukhula kwe-ADPKD, udokotela wakho angakunikeza i-tolvaptan (Jynarque).

Wukuphela kwemithi i-Food and Drug Administration (i-FDA) evume ngokukhethekile ukwelapha i-ADPKD. Lo muthi ungasiza ukubambezela noma ukuvimbela ukwehluleka kwezinso.

Ngokuya ngesimo sakho esithile kanye nezidingo zokwelashwa, udokotela wakho angangeza futhi okukodwa noma okuningi kokulandelayo kuhlelo lwakho lokwelashwa:

• ushintsho lwendlela yokuphila ukusiza ukwehlisa umfutho wegazi nokukhuthaza impilo yezinso
• imithi yokusiza ukunciphisa umfutho wegazi, ukudambisa izinhlungu, noma ukwelapha izifo ezingenzeka ezinso, emgudwini wokuchama, noma kwezinye izindawo
• ukuhlinzwa ukuze kususwe ama-cysts abangela ubuhlungu obukhulu
• ukuphuza amanzi usuku lonke nokugwema i-caffeine ukunciphisa ukukhula kwama-cysts (abacwaningi bacwaninga ukuthi i-hydration iyithinta kanjani i-ADPKD)
• ukudla izingxenye ezincane zamaprotheni aphezulu
• ukukhawulela usawoti, noma i-sodium ekudleni kwakho
• ukugwema i-potassium eningi ne-phosphorus ekudleni kwakho
• ukunciphisa ukusetshenziswa kotshwala

Ukuphatha i-ADPKD nokunamathela kuhlelo lwakho lokwelashwa kungaba yinselele, kepha kubalulekile ekunciphiseni ukuqhubeka kwesifo.

Uma udokotela wakho ekuyalela i-tolvaptan (Jynarque), kuzodingeka uhlolwe njalo ukuze uhlole impilo yesibindi sakho ngoba umuthi ungadala ukulimala kwesibindi.

Udokotela wakho uzophinda aqaphe impilo yezinso zakho ukubona ukuthi isimo sizinzile noma siyaqhubeka yini.

Uma uba nokuhluleka ukusebenza kwezinso, kuzodingeka uthole i-dialysis noma ukufakelwa izinso ukukhokhela ukulahleka kokusebenza kwezinso.

Khuluma nodokotela wakho ukuze ufunde kabanzi mayelana nezinketho zakho zokwelashwa, kufaka phakathi izinzuzo ezingaba khona, ubungozi, nezindleko zezindlela ezahlukahlukene zokwelashwa.

Imiphumela emibi yokwelashwa kwe-ADPKD

Iningi lemithi udokotela wakho angayibheka ukusiza ukuphatha noma ukuphatha i-ADPKD inengozi ethile yemiphumela emibi.

Isibonelo, iJynarque ingadala ukoma ngokweqile, ukuchama kaningi, futhi kwezinye izimo, ukulimala kwesibindi okukhulu. Kuke kwaba nemibiko yokwehluleka okukhulu kwesibindi okudinga ukufakelwa kwesibindi kubantu abathatha iJynarque.

Ezinye izindlela zokwelapha ezibhekise kwizimpawu ezithile ze-ADPKD nazo zingadala imiphumela emibi. Ukuze ufunde kabanzi ngemiphumela emibi engaba khona yokwelashwa okuhlukile, khuluma nodokotela wakho.

Uma ucabanga ukuthi kungenzeka ukuthi ube nemiphumela emibi ekwelashweni, yazisa udokotela wakho ngokushesha. Bangancoma ushintsho ohlelweni lwakho lokwelashwa.

Udokotela wakho kungenzeka futhi ukuthi a-oda izivivinyo ezijwayelekile ngenkathi uselashwa okuthile ukuze uhlole izimpawu zokulimala kwesibindi noma eminye imiphumela emibi.

Ukuhlolisiswa kwe-ADPKD

Isifo sezinso sePolycystic (PKD) yisifo sofuzo.

Ukuhlolwa kwe-DNA kuyatholakala, futhi kunezinhlobo ezimbili zokuhlolwa:

• Ukuhlolwa kokuxhumana kwe-Gene. Lokhu kuhlolwa kuhlaziya omaka abathile ku-DNA yamalungu omndeni ane-PKD. Kudinga amasampula egazi avela kuwe kanye namalungu omndeni amaningana athintekile futhi angathinteki yi-PKD.
  
• Ukuhlaziywa kokuguqulwa okuqondile / ukulandelana kwe-DNA. Lokhu kuhlolwa kudinga isampula eyodwa kuphela evela kuwe. Ihlaziya ngqo i-DNA yezakhi zofuzo ze-PKD.

Ukuxilongwa kwe-ADPKD

Ukuthola i-ADPKD, udokotela wakho uzokubuza mayelana:

• izimpawu zakho
• umlando wezempilo yomuntu siqu
• umlando wezokwelapha womndeni

Bangayalela ukuhlolwa kwe-ultrasound noma okunye ukuhlola ukubheka ama-cysts nezinye izimbangela zezimpawu zakho.

Bangaphinde ba-oda ukuhlolwa kofuzo ukuze ufunde ukuthi unokuguquka kwezakhi zofuzo okubangela i-ADPKD. Uma unesakhi sofuzo esithintekile futhi unezingane, zingabakhuthaza ukuthi nabo bathole ukuhlolwa kofuzo.

Izimbangela ze-ADPKD

I-ADPKD yisimo sofuzo esizuzwe njengefa.

Ezimweni eziningi, kubangelwa ukuguqulwa kohlobo lwe-PKD1 noma uhlobo lwe-PKD2.

Ukuthuthukisa i-ADPKD, umuntu kufanele abe nekhophi eyodwa yesakhi sofuzo esithintekile. Ngokuvamile bathola isakhi sofuzo esithintekile komunye wabazali babo, kepha ezimweni ezingavamile, ukuguquka kofuzo kungenzeka ngokuzenzakalela.

Uma une-ADPKD futhi umlingani wakho engenayo futhi unquma ukuqala umndeni ndawonye, ​​izingane zakho zingaba namathuba angama-50 okuthuthukisa lesi sifo.

Izinkinga

Isimo futhi sikubeka engcupheni yezinkinga, ezinjenge:

• umfutho wegazi ophakeme
• izifo zomgudu womchamo
• ama-cysts esibindi sakho noma amanyikwe
• ama-valve wenhliziyo angajwayelekile
• i-aneurysm yobuchopho
• ukwehluleka kwezinso

Isikhathi sokuphila nombono

Isikhathi sakho sokuphila nesimo sakho se-ADPKD sincike ezintweni eziningana, kufaka phakathi:

• ukuguqulwa okuthile kwezakhi zofuzo okudala i-ADPKD
• noma iziphi izinkinga ozihlakulelayo
• ukwelashwa okutholayo nokuthi unamathela kangakanani kuhlelo lwakho lokwelashwa
• impilo yakho yonke kanye nendlela ophila ngayo

Khuluma nodokotela wakho ukuze ufunde kabanzi ngesimo sakho nangombono wakho. Lapho i-ADPKD itholakala kusenesikhathi futhi iphathwa ngendlela efanele, abantu maningi amathuba okuthi bakwazi ukugcina impilo ephelele, esebenzayo.

Isibonelo, abantu abaningi abane-ADPKD abasasebenza lapho kutholakala ukuthi bayakwazi ukuqhubeka nemisebenzi yabo.

Ukwenza imikhuba enempilo nokulandela uhlelo lokwelashwa olunconyiwe lukadokotela wakho kungasiza ekuvimbeleni izinkinga futhi kugcine izinso zakho ziphilile isikhathi eside.