Ingabe unezinkinga ngemigudu yakho ye-bile? Ake sifunde nge-Primary Sclerosing Cholangitis (PSC)!

Ngezinye izikhathi sizizwa sikhathele ngaphandle kwesizathu, sizwa ubuhlungu obuncane engxenyeni ephezulu kwesokudla yesisu sethu, noma mhlawumbe sizizwa sengathi siyaziklwebha. Nakuba singazinaki kakhulu lezi zinto ekuphileni kwethu kwansuku zonke, ngezinye izikhathi lezi zingaba izimpawu zesimo esibucayi kakhulu emzimbeni wethu. Namuhla sizokhuluma ngesimo esiyinkimbinkimbi kancane, kodwa kubaluleke kakhulu ukuthi wonke umuntu asiqaphele. Leso yi -Primary Sclerosing Cholangitis , noma i-PSC ngamafuphi.

Iyini i-primary sclerosing cholangitis (PSC)?

Kalula nje, i-PSC yisifo esenzeka emigudwini ye-bile emzimbeni wethu. Njengoba ungase ukhumbule, i-bile ikhiqizwa esibindini. Le nyongo iyasiza ekugayweni kokudla. Ngakho-ke, kukhona uhlelo lwamapayipi amancane athwala le nyongo isuka esibindini iye emathunjini. Sibiza lawo mapayipi ngokuthi imigudwini ye-bile.

Ku-PSC, kukhona ukuvuvukala okungapheli , noma ukuvuvukala, kulezi zindawo zokudonsa inyongo. Odokotela babiza lokhu ngokuthi i-cholangitis . Uma lokhu kuvuvukala kuqhubeka isikhathi eside, kuqala ukwakheka kwezibazi emifuleni yenyongo. Lokhu kuzibazi kubangela ukuthi imigudu yenyongo ibe lukhuni futhi ibe mncane. Lokhu kubizwa ngokuthi i-sclerosis .

Cabanga ngepayipi lamanzi elivalekile ngothuli futhi lincipha, okwenza kube nzima ukuthi amanzi adlule, akunjalo? Ngokufanayo, lapho imisele yenyongo iba mincane, ukugeleza kwenyongo kuyavinjelwa. Lokhu kubizwa ngokuthi i-biliary stricture . Ngakho-ke, lapho inyongo ingakwazi ukugeleza kahle, iqala ukubuyela esibindini. Lokhu kuqongelela kwenyongo esibindini (i-cholestasis) kulimaza isibindi kancane kancane.

Ingabe lesi simo se-PSC sibucayi?

Yebo, i-PSC empeleni iyisifo esibi kakhulu . Ungase ungabi nazimpawu ezigabeni zokuqala. Kodwa yisifo esiqhubekayo. Uma i-bile ivinjiwe emigudwini ye-bile (i-cholestasis), ubuthi obuthile e-bile bunganqwabelana egazini. Bese uqala ukuzizwa ungakhululekile. Futhi, lokhu kubangela umonakalo oqhubekayo esibindini.

Ngokuvamile, lo monakalo ungaholela ekuhlulekeni kwesibindi ngokuphelele ngemva kweminyaka engaba yi-10 kuya kweyi-15. Njengoba wazi, asikwazi ukuphila ngaphandle kwesibindi. Nakuba ukwelashwa kwamanje kungalawula izimpawu, asikho ikhambi le-PSC. Isixazululo kuphela ukufakelwa kwesibindi .

Uyini umehluko phakathi kwe-cholangitis ye-sclerosis eyinhloko neyesibili?

Igama elithi "okuyinhloko" lapha lisho ukuthi lesi sifo siyimbangela eyinhloko yokuvuvukala kanye nokuqhekeka kwemisele yenyongo. Okusho ukuthi, lesi simo sibangelwa yilesi sifo sodwa, ngaphandle kwesinye isizathu.

Kodwa,Esimweni esibizwa ngokuthi i-secondary sclerosing cholangitis, ukuvuvukala (i-cholangitis) kanye nezibazi (i-sclerosis) ze-bile ducts kwenzeka ngenxa yesinye isizathu. Okusho ukuthi, lesi simo sibangelwa esinye isifo noma isenzakalo. Nazi ezinye izibonelo:

• Ukulimala emigudwini yenyongo (isibonelo, ngesikhathi sokuhlinzwa).
• Ukutheleleka emigudwini yenyongo.
• Ukwelashwa okulwa nomdlavuza (i-Chemotherapy).
• Amatshe enyongo avimba imigudu yenyongo.
• I-pancreatitis ephindaphindayo.

Uyini umehluko phakathi kwe-primary biliary cirrhosis (PBC) kanye ne-PSC?

Lesi sifo, esaziwa ngokuthi yi-Primary Biliary Cirrhosis, manje sesibizwa ngokuthi i-Primary Biliary Cholangitis (PBC) .

Zombili i-PBC kanye ne-PSC ziyizifo eziqhubekayo ezithinta isibindi, ezithinta imigudu ye-bile. Izimpawu nemiphumela yazo zombili zifana kakhulu. Zombili lezi zifo zibangela ukuthi imigudu ye-bile ibuthaka kancane kancane. Lapho imigudu ye-bile yonakala, iba nezibazi, futhi incipha, ukugeleza kwe-bile kuyavaleka. I-bile bese ibuyela emuva esibindini, okulimaza isibindi kakhulu. Lokhu kungaholela ekuqhekekeni kwesibindi kanye nokuqina kwesibindi .

Kodwa kunezinhlobo eziningana zomehluko omkhulu phakathi kwalezi zifo ezimbili:

• I-PBC ithinta kuphela imisele ye-bile engaphakathi kwesibindi (intrahepatic). I-PSC ingathinta kokubili imisele ye-bile engaphakathi kwesibindi kanye nemisele ye-bile engaphandle kwesibindi (extrahepatic).
• I-PBC ivame kakhulu kwabesifazane (isilinganiso esingu-10:1), kuyilapho i-PSC ivame kakhulu kwabesilisa (isilinganiso esingu-2:1).
• Cishe ama-80% eziguli ze-PSC anesifo samathumbu esivuvukalayo (i-IBD) esivele sikhona, ikakhulukazi i-ulcerative colitis . Kodwa-ke, lokhu akulona ihlobana elibalulekile ne-PBC.
• Kunengozi enkulu yokuba nomdlavuza we-bile duct nge-PSC. Ayikho ingozi enkulu kangaka nge-PBC.
• I-Ursodeoxycholic acid (UDCA) isetshenziselwa ukwelapha i-PBC, kodwa okwamanje akukho ukwelashwa okuqondile kwe-PSC.

Sivame kangakanani lesi simo esibizwa ngokuthi i-PSC? Ubani ovame ukusithola?

I-PSC iyisifo esingavamile kakhulu . Emhlabeni wonke, kulinganiselwa ukuthi umuntu oyedwa kuphela kwabayizinkulungwane eziyishumi unalesi sifo.

Lesi sifo sivame kabili kwabesilisa kunakwabesifazane. Lesi sifo sivame ukutholakala sineminyaka engaba ngu-40. Cishe abantu abangu-80% abane-PSC nabo bane -IBD (Inflammatory Bowel Disease) , ikakhulukazi i-Ulcerative Colitis . Uma othile emndenini enalesi sifo, nabanye basengozini yokusithola.

Yini ebangela i-PSC?

Imbangela eqondile ye-PSC ayaziwa okwamanje, kodwa kucatshangwa ukuthi ibangelwa yinhlanganisela yezici. Lezi zifaka:

• Izakhi zofuzo
• Izici zemvelo
• Amaseli omzimba

Odokotela bacabanga ukuthi i-PSC ingaba uhlobo lwesifo sokuzivikela komzimba . Lokhu kusho ukuthi uhlelo lokuzivikela lomzimba wethu, amasosha okufanele asivikele ezifweni, ngephutha luqala ukuhlasela amangqamuzana ethu aphilile. Ukuvuvukala kungenye yezikhali zesimiso sethu sokuzivikela komzimba. Ngokuvamile kuba okwesikhashana, kusebenza ngokumelene nomhlaseli. Kodwa uma ukuvuvukala kuba okungamahlalakhona, kuvame ukuba uphawu lwesifo.

Odokotela baphinde baphawula ukuthi abantu abane-PSC banamathuba amaningi okuthola ezinye izifo ezihlasela amasosha omzimba. Isibonelo:

• Izifo zamathumbu ezivuvukalayo
• Isifo se-Celiac
• Isifo se-thyroid
• Isifo sikashukela sohlobo 1
• I-hepatitis yokuzivikela komzimba
• I-autoimmune pancreatitis

Kucatshangwa ukuthi lolu hlobo lokusabela kwe-autoimmune lungenzeka lapho abantu abanezimpawu ezithile zofuzo bevezwa ezicini ezithile zemvelo (isib., ukuchayeka kumakhemikhali anobuthi).

Ziyini izimpawu ze-PSC?

Njengoba i-PSC iqhubeka, izimpawu ziyashintsha. Cishe abantu abangu-50% bangase bangabi nazimpawu nhlobo lapho lesi sifo sitholakala. I-PSC ivame ukutholakala ngengozi, ngesikhathi sokuhlolwa kwesinye isimo. Izimpawu zokuqala ngokuvamile azibonakali kahle. Zihlanganisa:

• Ukukhathala
• Ubuhlungu besisu esingaphezulu kwesokudla
• Isikhumba esilumayo (ukucasuka)

Izimpawu ezingase zivele njengoba isifo siqhubeka zifaka:

• Isisu esivuvukile
• Isibindi esikhulu
• Ubende obukhulu
• I-Jaundice - Lokhu kusho ukuphuzi kwamehlo nesikhumba.
• Imfiva
• Ukwehlisa isisindo okungahlosiwe

Yiziphi izinkinga ze-PSC enzima?

I-primary sclerosing cholangitis iyisifo esiqhubeka kancane kancane, kodwa njengoba imigudu ye-bile nesibindi sakho siba nezibazi futhi ukusebenza kwazo kuwohloka, kungaba nezinkinga ezahlukahlukene.

Izifo ze-Metabolic

Uma imigudu yakho yenyongo ivalekile kakhulu, ayikwazi ukupompa inyongo kahle ingene emathunjini akho amancane. Inyongo ibalulekile ekugayeni ukudla, ikakhulukazi amafutha, kanye nokumunca amavithamini ancibilikayo emafutheni (A, D, E, kanye no-K). Ngaphandle kwenyongo, uhlelo lwakho lokugaya ukudla alukwazi ukucubungula amafutha kahle, futhi alukwazi ukumunca la mavithamini abalulekile ekudleni. Lokhu kungaholela ezinkingeni ezengeziwe:

• Indle enamafutha kanye nesifo sohudo
• Ukungamuncwa kokudla kanye nokungondleki kahle
• Ukuphuka kalula nokopha (ngenxa yokuntuleka kwevithamini K)
• Ubuthakathaka bamathambo - i-Osteomalacia kanye ne-osteoporosis (ngenxa yokuntuleka kwevithamini D)
• Ukungaboni kahle ebusuku (ngenxa yokuntuleka kwevithamini A)

Umfutho wegazi ophezulu we-portal

Njengoba i-cirrhosis iqhubeka, ukugeleza kwegazi esibindini kuyancipha. Lokhu kwandisa ingcindezi emthanjeni we-portal, odlula ohlelweni lwakho lokugaya ukudla. Lokhu kucindezela kungabangela ukuthi eminye imithambo emphinjeni nasesiswini sakho ikhule futhi iqhume. Lokhu kungaholela ekuphumeni kwegazi ngaphakathi .

Izifo

Imigudu yenyongo evalekile ivame ukungenwa yigciwane, okubangela izimo ezifana nomkhuhlane, ubuhlungu besisu, kanye noshevu wegazi (i-sepsis).

Umdlavuza

Njengoba i-PSC iba nzima kakhulu, ingozi yokuthola izinhlobo eziningana zomdlavuza iyanda:

• Umdlavuza we-bile duct (i-cholangiocarcinoma) - Ingozi ephakathi kuka-5% no-20%.
• Umdlavuza wenyongo
• Umdlavuza wesibindi (i-hepatitis)
• Umdlavuza we-Coloral Rectum - uma nawe une-IBD.

I-PSC ihlolwa kanjani?

Ngokuvamile, lesi sifo sitholakala ngengozi, lapho kuhlolwa okunye. Cishe abantu abangu-50% bangase bangabi nazimpawu lapho lesi sifo sitholakala. Izimpawu zokuqala zesifo zingabonakala ngokuhlolwa kwegazi noma ukuhlolwa kwesithombe. Ukuskena kwemisele ye-bile ene-PSC kubonisa izici ezikhethekile. Uma ukuhlolwa kwegazi kubonisa amazinga aphezulu e-enzyme ebizwa ngokuthi i-alkaline phosphatase , noma uma kukhona ama-antibodies athile abonisa impendulo yomzimba kumamisele ye-bile, kungaba uphawu lwe-PSC. Ukwanda kwamaseli amhlophe egazi ngokuvamile kuyisibonakaliso sokutheleleka kwesibindi.

Ukuqinisekisa ukuxilongwa, udokotela wakho angase aphakamise ezinye izivivinyo eziningana eziqondile:

• Ukuhlolwa kokusebenza kwesibindi (LFT): Lokhu kuhlolwa kwegazi kubheka amazinga aphezulu ama-enzyme athile esibindini. Amazinga aphezulu e-alkaline phosphatase angabonisa i-PSC.
• I-Magnetic resonance cholangiopancreatography (MRCP) : Lesi isikeni se -MRI (Magnetic Resonance Imaging) . Singathatha izithombe ezinemininingwane zesistimu yakho yenyongo (isibindi, inyongo, kanye nemisele yenyongo). Lesi isikeni sokuqala sokuthola i-PSC. Lokhu kungenxa yokuthi asihlaseli futhi asihileli ukuchayeka emisebeni. Kodwa-ke, ngezinye izikhathi, uma lesi sifo sisesencane noma singesibi kakhulu, singase singakwazi ukusithola ngokunembile. Ngemuva kwalokho kungadingeka esinye isikeni.

Iphathwa kanjani i-PSC?

Okwamanje, akukho ukwelashwa okungamisa noma kubuyisele emuva ukuqhubeka kwe-primary sclerosing cholangitis. Kodwa-ke, izimpawu nezinkinga zingelashwa ngqo. Isibonelo, udokotela wakho angase akunike izinto ezifana nalezi:

• Imithi yokwelapha isikhumba esilumayo (i-pruritus)
• Izithasiselo zokwelapha ukuntuleka kwamavithamini
• Ama-antibiotic okwelapha izifo

Udokotela wakho uzoqapha isibindi sakho kanye nemisele ye-bile. Njengoba lesi sifo siqhubeka, ngezinye izikhathi bangase bavule imisele ye-bile evalekile. Lokhu kungenziwa ngokuhlolwa okubizwa ngokuthi i-ERCP (endoscopic retrograde cholangiopancreatography) . Lena inqubo ekuvumela ukuthi ubone ngaphakathi kwemisele yakho ye-bile ngaphandle kokuhlinzwa.

I-ERCP uhlobo lwe -endoscopy . Ihilela ukuhlola izitho zakho usebenzisa i-endoscope, ithubhu elide, eliguquguqukayo elinekhamera encane bese likhanyisa. Ngemva kokuba ulaliswe i-anesthesia, udokotela wakho uzofaka le tyhubhu phansi emphinjeni wakho nasesiswini sakho. Izinsimbi ezincane zingadluliselwa ngaleli tyhubhu ukwelapha imigudu yakho ye-bile. Uchwepheshe angasebenzisa ibhaluni ukukhulisa imigudu yakho ye-bile, noma afake i-stent ukuze ayigcine ivulekile. Uma i-ERCP ingakwazi ukufinyelela ekuvinjweni, inqubo ebizwa ngokuthi i-`percutaneous trans-hepatic cholangiography (PTHC)' ngezinye izikhathi isetshenziswa.

Kodwa lokhu kuyisixazululo sesikhashana nje. Esikhathini esiyiminyaka eyi-10 kuya kwengama-20, i-primary sclerosing cholangitis iya ngokuya iba yimbi, isifo sesibindi siba sibi kakhulu, futhi ekugcineni isibindi siyahluleka ukusebenza. Udokotela wakho uzoqapha umonakalo esibindini sakho futhi anqume ukuthi uzocabangela nini ukufakelwa isibindi . Udinga futhi ukuhlangabezana nezindinganiso ezithile ukuze ube sohlwini lokulinda ukufakelwa isibindi.

Ungaphila isikhathi esingakanani ne-PSC?

Uma sekutholakale ukuthi unesifo, isilinganiso sobude bempilo siyahlukahluka phakathi kweminyaka eyi-10 nengama-20. Ukufakelwa isibindi kunganikeza ithuba elisha lokuphila. Kodwa-ke, ezimweni eziyi-15% kuya kwezingama-20%, i-PSC ingabuya ngisho nangemva kokufakelwa isibindi. Uma lokho kwenzeka, isibindi esisha singase sihluleke. Ezimweni ezinjalo, isilinganiso sobude bempilo singaba yizinyanga eziyisishiyagalolunye.

Esinye isici esingathinta isikhathi sokuphila umdlavuza. Uma uba nomdlavuza njengenkinga ye-PSC, ungase ungabi umuntu ofanele ukufakelwa isibindi. Ezimweni ezikhethwe ngokucophelela, odokotela bangase baqale baphathe umdlavuza ngemisebe noma nge-chemotherapy, bese bezama ukufakelwa isibindi.

Kufanele ngizinakekele kanjani uma nginalesi sifo?

Ukwenza izinguquko endleleni yokuphila kungasiza ekulawuleni ukukhathala okuvamile nge-PSC futhi kuvimbele ukulimala kwesibindi okwengeziwe. Isibonelo:

• Gwema utshwala ngokuphelele.
• Yidla ukudla okucutshungulwe kancane bese udla ukudla okuphelele.
• Lawula ukucindezeleka.
• Bhala kahle.
• Zivocavoce nsuku zonke.

I-primary sclerosing cholangitis yisifo esingavamile, esingalindelekile, futhi esingavinjelwa. Siqhubeka kancane, futhi ngokuvamile asinazo izimpawu ezigabeni zokuqala. Kodwa uma ubona udokotela wakho njalo futhi uthola ukuhlolwa okuvamile, lesi sifo singatholakala ngaphambi kokuba siqale ukuthinta impilo yakho. Yilapho-ke ungenza khona izinguquko zendlela yokuphila ezingasiza ukugcina isibindi sakho siphilile isikhathi eside. Uzothunyelwa futhi ukuze uthole ukunakekelwa kwezokwelapha kwesikhathi eside, okusho ukuhlolwa njalo kanye nokungenelela uma kudingeka.

Okokugcina, izinto okufanele uzikhumbule

Kulungile, ngakho-ke ngithemba ukuthi lokho esikhulume ngakho nge-PSC kukusizile ukukhumbula ezinye zezinto ezibaluleke kakhulu. Kuyisimo esiyinkimbinkimbi kancane, kodwa kubaluleke kakhulu ukuqaphela.

• I-PSC yisifo esithinta imigudu yenyongo futhi siba sibi kakhulu ngokuhamba kwesikhathi. Singalimaza isibindi.
• Kungase kungabi khona zimpawu ezigabeni zokuqala, ngakho-ke kubalulekile ukuhlolwa njalo kwezokwelapha.
• Okwamanje akukho ukwelashwa kwalesi sifo, kodwa kunezindlela zokwelapha zokulawula izimpawu nokuthuthukisa ikhwalithi yokuphila.
• Ukufakelwa kwesibindi kuyisisombululo esiyinhloko ezimweni ezinzima.
• Ukulandela indlela yokuphila enempilo kusiza ekuvikeleni isibindi.

Uma uneminye imibuzo mayelana ne-PSC, noma uma ucabanga ukuthi unanoma yiziphi zalezi zimpawu, qiniseka ukuthi ubona udokotela ukuze akunike iseluleko . Ungakhathazeki, kunezinto ongazenza ukuze uzilawule uma zitholakala kusenesikhathi.

I- Sclerosing Primary Cholangitis, i-PSC, Isifo Somsele Wenyongo, Isifo Sesibindi, I-Jaundice, Ubuhlungu Besisu, I-Sclerosing Primary Cholangitis