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I-Polycystic Kidney Disease

Isifo sezinso se-Polycystic sithinta izigidi emhlabeni wonke, okusenza sibe esinye sezifo ezivame kakhulu ezizuzwe njengefa ukuphazamiseka kwezinso. Kusukela ekushintsheni kwemithi nendlela yokuphila kuya ezinkambisweni ezikhethekile, odokotela manje sebengakwazi ukunikeza izindlela ezihlukahlukene zokulawula izimpawu nokuvikela ukusebenza kwezinso. Ukuqonda ukuthi isifo sezinso ze-polycystic siyithinta kanjani impilo yonke kuyisinyathelo sokuqala sokwenza izinqumo ezinolwazi mayelana nokunakekelwa kanye nezinketho zokwelashwa. 

Iyini I-Polycystic Kidney Disease? 

Isifo sezinso i-Polycystic (PKD) isifo sofuzo esibonakala ngokwakhiwa kwamasaka agcwele uketshezi okuthiwa ama-cysts ezinso. Ngokungafani nama-cysts ezinso alula angase akhule ngokukhula, ama-PKD cysts angashintsha kakhulu kokubili ukuma nobukhulu bezinso, okuholela ezinkingeni ezinkulu zempilo. 

Lesi simo sidala ukuthi izinso zikhule njengoba lezi zimila zikhula futhi zanda ngokuhamba kwesikhathi. Imvelo ye-PKD ikwenza ithinteke ikakhulukazi, njengoba inciphisa kancane kancane amandla ezinso okuhlunga udoti egazini. Ama-cyst ku-PKD anezici ezimbalwa ezihlukile: 

  • Zingahluka kakhulu ngosayizi 
  • Aqukethe uketshezi futhi ayaqhubeka nokukhula 
  • Angathuthuka kuzo zombili izinso kanyekanye 
  • Zingathinta ezinye izitho, ikakhulukazi isibindi kanye kwamanyikwe 

I-PKD ihlukaniswa njengohlobo lwesifo sezinso esingamahlalakhona esingase sithuthukele ekuhlulekeni kwezinso uma singalawulwa. Ubunzima besimo buyahlukahluka phakathi kwabantu, abanye baba nezimpawu ezithambile kuyilapho abanye bebhekana nezinkinga ezinkulu kakhulu. Nakuba umthelela oyinhloko usezinso, i-PKD ingathinta izinhlelo eziningi zomzimba, ezingase zibangele umfutho wegazi ophakeme kanye nezinkinga ngemithambo yegazi ebuchosheni nasenhliziyweni. 

Izinhlobo ze-Polycystic Kidney Disease (PKD) 

Odokotela bahlukanisa izifo zezinso ze-polycystic zibe izinhlobo ezimbili ezihlukene, ngayinye enezici ezihlukile namaphethini efa. 

  • I-Autosomal Dominant Polycystic Kidney Disease (ADPKD): I-ADPKD imele ifomu elivame kakhulu, elithinta cishe abantu abayisi-9 kwabayi-10 abane-PKD. Lolu hlobo luvame ukuvela phakathi kweminyaka engama-30 no-40, nakuba ama-cyst angase aqale ukwakheka ngaphambi kwesikhathi. I-ADPKD yenzeka lapho umuntu ezuza njengefa ikhophi eyodwa yofuzo olushintshiwe kunoma yimuphi umzali, okunikeza ingane ngayinye yomzali othintekayo ithuba elingu-50% lokuthuthukisa isimo. 
  • I-Autosomal Recessive Polycystic Kidney Disease (ARPKD): I-ARPKD ayijwayelekile kakhulu, ithinta cishe umuntu oyedwa kwabayi-20,000 kuya ku-40,000. Leli fomu livela ekuqaleni kwempilo, ngokuvamile lithinta izingane ezisanda kuzalwa noma izingane ezincane. Bobabili abazali kufanele baphathe ukuguqulwa kofuzo lwe-PKHD1 ukuze ingane yakhe i-ARPKD. 

Umehluko omkhulu phakathi kwalezi zinhlobo uhlanganisa: 

  • Ukuvama: I-ADPKD ithinta umuntu oyedwa kwabangu-400 kuya ku-1,000, kuyilapho i-ARPKD iyivelakancane kakhulu 
  • Iminyaka Yomthelela: Izimpawu ze-ADPKD zivame ukuvela lapho umuntu esekhulile, kuyilapho i-ARPKD ibonakala ebuntwaneni noma ebuntwaneni 
  • Ukubandakanyeka kwesitho: I-ARPKD ivamise ukuphatha izinso nesibindi, kuyilapho i-ADPKD ithinta kakhulu izinso. 
  • Ukuqhubeka Kwezifo: I-ARPKD ivamise ukukhombisa izimpawu ezinzima kakhulu ekuqaleni kwempilo uma kuqhathaniswa ne-ADPKD 
  • Izinga Lokusinda: Iziguli ze-ADPKD zivame ukuphila zibe abantu abadala, kuyilapho i-ARPKD ingaba yingozi empilweni isencane 

Izimpawu ze-Polycystic Kidney Disease 

Iziguli zingase zithole ukubonakaliswa okuhlukahlukene ngokomzimba. Lezi zimpawu zingabandakanya: 

  • Umfutho wegazi ophakeme ugqama njengenkomba evamile yasekuqaleni yalesi simo 
  • Ubuhlungu bomhlane noma ohlangothini, ikakhulukazi emhlane ongezansi 
  • Igazi emchameni (hematuria) 
  • Ukwanda kobukhulu besisu ngenxa yezinso ezikhulisiwe 
  • Ukutheleleka njalo kwesinye isinye noma izinso 
  • Umuzwa wokugcwala esiswini 
  • Ikhanda elibuhlungu elihlobene nomfutho wegazi ophezulu 
  • Amatshe ezinso 
  • Izimpawu ezihlobene nenhliziyo, okuhlanganisa ukuzwa ukuphaphazela noma ukushaya esifubeni 

Izimbangela Nezingozi Zezinso Ze-Polycystic 

Ukuguqulwa kofuzo kusenhliziyweni yesifo sezinso se-polycystic, ngezakhi zofuzo ezithile ezidlala indima ebalulekile ekuthuthukisweni kwaso. Ososayensi bahlonze ukuguqulwa kofuzo ezakhini zofuzo ezintathu eziyinhloko, i-PKD1, PKD2, ne-PKHD1, ezithinta ukuthuthukiswa okujwayelekile nokusebenza kwamaseli ezinso. 

Kunezici ezimbalwa ezikhulisa ubungozi bokukhula noma ukuhlangana nezinkinga ezivela ku-polycystic kidney disease: 

  • Umlando oqinile womndeni walesi simo 
  • Ukuqhubeka kweminyaka, ikakhulukazi ezimeni zama-cyst alula 
  • Ukwelashwa kwe-dialysis yesikhathi eside 
  • Ifa likamama, elivame ukuholela ekwandeni kobunzima besifo 
  • Ukuba khona kokuguqulwa kwezakhi zofuzo ezithile 

Nakuba izimo eziningi zesifo sezinso ze-polycystic zibangelwa izimbangela zofuzo, abanye abantu bahlakulela ifomu elitholiwe. Lokhu kuhluka, okwaziwa ngokuthi isifo sezinso esitholwa yi-polycystic, ngokuvamile kwenzeka kubantu asebethole ukwelashwa nge-hemodialysis iminyaka eminingana ngenxa yezinye izimo zezinso. 

Izinkinga ze-Polycystic Kidney Disease 

Izinkinga ezibaluleke kakhulu zihlanganisa: 

  • Izinkinga zenhliziyo nemithambo yegazi: Cishe bonke abantu abane-PKD bahlakulela umfutho wegazi ophakeme, okungaholela esifweni senhliziyo kanye unhlangothi. Lesi simo sidinga ukuqapha nokuphathwa ngokucophelela ukuze kuvinjelwe ukulimala kwezinso. Ukungajwayelekile kwe-valve yenhliziyo kwenzeka cishe ku-25% wabantu abanesifo sezinso ze-polycystic futhi baba nesimo esaziwa ngokuthi "i-floppy valve" enhliziyweni. 
  • I-Brain Aneurysms: Cishe u-10% wabantu abane-PKD uba nobuchopho i-aneurysms. Lawa maqhuqhuva afana nebhaluni emithanjeni yegazi adinga ukunakekelwa ngokushesha uma ebanga ikhanda elibuhlungu noma ezinye izimpawu zemizwa. 
  • Izinkinga Zesibindi: Ama-cysts esibindi amelela inkinga evame kakhulu engeyona eyezinso, ikakhulukazi ethinta abesifazane. Nakuba la ma-cysts engavamile ukuba nomthelela ekusebenzeni kwesibindi, angabangela ubuhlungu besisu nokuvuvukala. 
  • Izinkinga Zesistimu Yokugaya: Kuhlanganisa i-diverticulosis, lapho kwakheka khona izikhwama ezincane odongeni lwamakholoni. Lokhu kungase kubangele izinguquko ekuhambeni kwamathumbu noma ukungakhululeki kwesisu. 
  • Ukutheleleka kwe-Urinary Tract: I-UTI ivame kakhulu njengoba lesi sifo siqhubeka, okungase kuholele ezifweni zezinso ezingase zilimaze izitho zomzimba. 

Sifo 

Odokotela basebenzisa amathuluzi amaningana okuxilonga ukuze baqinisekise i-PKD: 

  • Ukuhlolwa kwe-Ultrasound: Ithuluzi elivame kakhulu nelokuqala lokuhlola, kusetshenziswa amaza omsindo ukuthola ama-cysts 
  • I-MRI Scans: Ihlinzeka ngezithombe ezinemininingwane yesakhiwo sezinso futhi ingakala ivolumu yezinso 
  • I-CT Scans: Ibonisa ukubukwa okuphelele kwezinso futhi ikwazi ukubona ama-cysts amancane 
  • Ukuhlolwa Kwegazi Nomchamo: Isiza ukuhlola ukusebenza kwezinso nezinga lokuhlunga 

Ukwelapha I-Polycystic Kidney Disease 

Nakuba lingekho ikhambi le-PKD, intuthuko yezokwelapha yethule izindlela zokwelashwa ezithembisayo zesifo sezinso ze-polycystic. Ngo-2018, i-FDA igunyaze i-tolvaptan njengokwelashwa kokuqala okuqondiswe kubantu abadala abanesifo sezinso se-autosomal esiphezulu se-polycystic. Lo muthi, othathwa njengephilisi lomlomo kabili nsuku zonke, usiza ukubambezela ukukhula kwama-cysts ezinso futhi ugcine ukusebenza kwezinso. 

Izindlela zokwelapha zigxile ekulawuleni izimpawu nokuvimbela izinkinga ngokusebenzisa: 

  • Ukuphathwa Kwemithi: Ukulawula umfutho wegazi usebenzisa i-ACE inhibitors noma i-ARBS 
  • Ukulawula Izinhlungu: Ukusebenzisa izidambisi zinhlungu ezifanele njenge-paracetamol ngenkathi ugwema i-NSAIDS 
  • Ukwelashwa Kokutheleleka: Ukwelashwa ngokushesha kwama-antibiotic ezifweni ze-urinary tract 
  • Ukuphathwa Kwe-cyst: Ukukhipha ama-cysts amakhulu ukukhulula ingcindezi nobuhlungu Ukusekelwa Komsebenzi Wezinso: Ukugcina i-hydration efanele kanye nokulandela izincomo zokudla 

Uzoyibona Nini Udokotela 

Ukuqaphela isikhathi esifanele sokufuna usizo lwezokwelapha kungaba nomthelela omkhulu ekulawuleni isifo sezinso se-polycystic. Abantu kufanele bathintane nodokotela wabo ngokushesha uma bezwa ubuhlungu obungapheli beqolo, igazi emchameni, noma ukungenwa yizifo zomgudu womchamo njalo. 

Prevention 

Odokotela batusa izinyathelo ezimbalwa ezibalulekile zokuvikela ukuvikela ukusebenza kwezinso: 

  • Gcina umfutho wegazi ophakeme ungaphansi kuka-120/80 
  • Hlala unamanzi ngendlela efanele ngamalitha amanzi angama-2-3 nsuku zonke 
  • Landela ukudla okunosawoti omncane ukuze usekele ukulawula umfutho wegazi 
  • Ziqeqeshe njalo 
  • Gcina isisindo somzimba esinempilo 
  • Gwema ukubhema futhi unciphise ukuphuza utshwala 
  • Gada ukusebenza kwezinso njalo ngokuhlolwa kodokotela 
  • Ukucatshangelwa ngokudla kudlala indima ebalulekile kumasu okuvimbela. Abantu abanesifo sezinso ze-polycystic kufanele balinganisele usawoti wabo wansuku zonke ube amagremu angu-5. Ukudla okunomsoco kufanele kubandakanye ukusetshenziswa kwamaprotheni okusesilinganisweni, cishe amagremu angu-0.8-1.0 ngekhilogremu yesisindo somzimba nsuku zonke. 
  • Ukulawulwa kwe-hydration kudinga ukunakwa ngokucophelela, ikakhulukazi ezigabeni zokuqala zesifo. Nakuba kunganconywa ukuphuza uketshezi oluningi, ukuhlala uhlanzekile ngokwanele kusiza ukugcina ukusebenza kwezinso. 

Isiphetho 

Impumelelo yokuphatha i-PKD incike kakhulu ekutholakaleni kusenesikhathi kanye nokunakekelwa kwezokwelapha okungaguquki. Iziguli ezilandela ukuguqulwa kwendlela yokuphila enconyiwe, zigcine amanzi ahlanzekile, futhi zisebenze eduze nethimba lazo lokunakekelwa kwezempilo ngokuvamile zithola imiphumela engcono. Ukuhlolwa njalo nokunaka izimpawu ngokushesha kusiza ukuvimbela izinkinga ezinkulu. 

Imibuzo Evame Ukubuzwa 

1. Ingabe izinso ze-polycystic zingasuswa? 

Odokotela ngokuvamile abancomi ukususa izinso ze-polycystic ngaphandle uma zibangela izinkinga ezinkulu. Ukususwa kungase kudingeke uma izinso zibangela ubuhlungu obukhulu noma izifo eziphindaphindayo noma lapho kudingeka isikhala sokufakelwa. Inqubo ye-nephrectomy ithwala izingozi futhi idinga ukucatshangelwa ngokucophelela kwezinzuzo ngokumelene nezinkinga ezingase zibe khona. 

2. Yini enhle ngesifo sezinso se-polycystic? 

Ukuguqulwa okuningana kwendlela yokuphila kungasiza ukuphatha ngempumelelo isifo sezinso se-polycystic. Inhlanganisela yokuphuza amanzi ngendlela efanele, ukulawula umfutho wegazi, nokuvivinya umzimba njalo kuba nenzuzo. I-Tolvaptan, egunyazwe yi-FDA, isiza ukukhula kancane kwesimila ezigulini ezifanele. Ukuhlolwa njalo kwezokwelapha kanye nokwelashwa ngokushesha kwezifo nakho kunomthelela ekulawulweni okungcono kwezifo. 

3. Isifo sezinso ze-polycystic siqala eminyakeni emingaki? 

Iminyaka yokuqala iyahlukahluka kuye ngohlobo lwe-PKD. I-ADPKD ivamise ukukhombisa izimpawu phakathi kweminyaka yobudala engama-30-40, noma ama-cysts angase akheke ngaphambi kwesikhathi. I-ARPKD, ifomu elingavamile, livela ebuntwaneni noma ngaphambi kwesikhathi ebuntwaneni. Abanye abantu bangase bangabi nezimpawu eziphawulekayo kuze kube kamuva ekuphileni. 

4. Ingabe isifo sezinso i-polycystic sibucayi? 

Yebo, isifo sezinso se-polycystic yisimo esibucayi esidinga ukuphathwa ngokucophelela. Cishe amaphesenti angu-50 eziguli aqala ukuhluleka kwezinso lapho eneminyaka engu-60 ubudala. Nokho, ukuqhubeka kwesifo kuyehluka kakhulu kubantu ngabanye, abanye baba nezimpawu ezithambile kuyilapho abanye bebhekana nezinkinga ezinzima kakhulu. 

5. Yini ongafanele uyiphuze uma unenkinga yezinso? 

Abantu abanenkinga yezinso kufanele bagweme noma banciphise: 

  • Amasoda anombala omnyama aphezulu ku-phosphorus  
  • Uphuzo oludakayo 
  • Iziphuzo zamandla 
  • Iziphuzo ezine-caffeine 
  • Iziphuzo ezinoshukela 
  • Amanani amaningi ejusi yezithelo 

6. Obani okungenzeka ukuthi babe ne-PKD? 

I-PKD ithinta kokubili amadoda nabesifazane ngokulinganayo, okwenzeka kuzo zonke izinhlanga nezinhlanga. Labo abanomlando womndeni babhekene nengozi enkulu kakhulu, njengoba isimo silandela amaphethini efa lofuzo. Amadoda ngokuvamile abhekana nokukhula ngokushesha kwesifo futhi angadinga ukwelashwa okushintshwa kwezinso esemncane kunabesifazane.

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