Ingabe imisele yakho yenyongo ikhulisiwe? Ake sixoxe ngesifo sikaCaroli!

Ingabe ngezinye izikhathi uzizwa sengathi umzimba wakho uphenduka ophuzi, noma uzizwa sengathi uthola ubuhlungu ohlangothini lwesokudla lwesisu sakho? Abanye abantu bangase babe nomkhuhlane nokuhlanza kanye nalezi zimpawu. Esinye isizathu esingaba khona ngemuva kwalokhu yisimo esizokhuluma ngaso namuhla esibizwa ngokuthi 'isifo sikaCaroli.' Ake sibone ukuthi siyini, kungani senzeka, sitholakala kanjani, nokuthi ukwelashwa kuyini.

Kalula nje, siyini isifo sikaCaroli?

Cabanga nje, ngaphakathi kwesibindi sethu kukhona amapayipi amancane abizwa ngokuthi amapayipi e-bile . Lawa mapayipi athwala uketshezi lokugaya ukudla olubizwa ngokuthi i-bile olukhiqizwa esibindini luye emathunjini amancane. Manje, lesi sifo sikaCaroli yisimo lapho amapayipi e-bile eba makhulu kunokujwayelekile, okungukuthi, avuleka . Lesi yisifo sofuzo , okungukuthi, sibangelwa ushintsho kumajini ethu. Lapho la mapayipi eba makhulu kanje, i-bile iqala ukuqongelela esikhundleni sokugeleza kahle. Uma lokhu kuqongelela, amatshe epayipi le-bile angakheka, izifo zingavela, futhi ngezinye izikhathi ama-cyst angakheka ngisho.

Kalula nje, inyongo kufanele ikwazi ukugeleza kalula ngaphandle kokubhajwa. Kodwa-ke, lapho imisele yenyongo iba mikhulu kakhulu, inyongo ingaqongelela kalula. Ngokuhamba kwesikhathi, lenyongo eqongelelene ingakha amatshe amancane.

Uma unesifo iCaroli, ungase ube nezimpawu eziningi zalesi simo impilo yakho yonke. Izimpawu zivame ukuqala cishe eminyakeni engama-30 , kodwa zingenzeka kunoma yimuphi ubudala. Ungase uqaphele isikhumba sakho kanye namehlo akho amhlophe ephenduka aphuzi (lokhu sikubiza ngokuthi i-jaundice ), futhi ungase uqale ukuthola izimpawu ezifana nomkhuhlane kanye ne-flu kaningi. Nakuba lesi simo singathinta impilo yakho kanye nekhwalithi yokuphila, kunezindlela zokwelapha ezingasiza ukulawula izimpawu zakho.

Ingabe zikhona izinhlobo zesifo sikaCaroli?

Yebo, kunezinhlobo ezimbili eziyinhloko zalokhu:

• Isifo sikaCaroli: Lolu hlobo luthinta kuphela imigudu yakho yenyongo. Alubangeli ezinye izinkinga ngesibindi sakho.
• I-Caroli syndrome: Lolu uhlobo olubi kakhulu. Kulokhu, isibindi siba nezibazi, esizibiza ngokuthi i-fibrosis .

Sivame kangakanani lesi sifo?

Isifo sikaCaroli siyisifo esingavamile kakhulu . Kulinganiselwa ukuthi umuntu oyedwa kuphela kwabayisigidi aba naso . Ngakho-ke akusona isifo esithinta abantu abaningi.

Ngakho-ke, ziyini izimpawu zesifo sikaCaroli?

Ngokuvamile, amatshe amancane akheka emigudwini yenyongo awabangeli zimpawu ezinkulu. Kodwa-ke, uma itshe livimba umgudwini wenyongo, lingavuvukala. Lokhu sikubiza ngokuthi i-cholangitis . Yilapho kuvela khona izimpawu zesifo sikaCaroli:

• Ubuhlungu ohlangothini lwesokudla lomzimba wakho , ngokuvamile esiswini esingaphezulu.
• Ukuhlanza .
• Umkhuhlane kanye nokubanda .
• Ukuphuzi kwesikhumba kanye nokumhlophe kwamehlo (i-jaundice) .
• Ukuluma .

Akuvamile kakhulu, lokhu kunqwabelana kwenyongo kungaholela ekukhuleni kohlobo lwesimila esinomdlavuza esibizwa ngokuthi i-cholangiocarcinoma emigudwini yenyongo. Yingakho kubalulekile ukuqaphela ngalokhu.

Kungani isifo sikaCaroli sivela? Ziyini izimbangela?

Isifo sikaCaroli siyisifo esibangelwa izakhi zofuzo . Lokhu kusho ukuthi sibangelwa ushintsho ezakhini zofuzo emizimbeni yethu. Esikhathini esiningi, singavela ngezikhathi ezithile, okusho ukuthi asidluliselwa ezizukulwaneni ngezizukulwane. Kodwa-ke, kweminye imindeni, kubikwe ukuthi siwuhlobo olulawulayo lwe-autosomal , okusho ukuthi uma umzali oyedwa enaso, nezingane zingasithola.

I-Caroli syndrome ibangelwa ukuguquka kwezakhi zofuzo esakhiweni esibizwa ngokuthi i-PKHD1 . Lesi sakhi sofuzo sinomthwalo wokukhiqiza iphrotheni esiza imisele yenyongo kanye nezinso ukukhula ngesikhathi sokukhulelwa, okungukuthi, lapho umntwana esesibelethweni. Ngenxa yokuthi isifo sikaCaroli kanye ne-Caroli syndrome zihlobene, abacwaningi bakholelwa ukuthi isakhi sofuzo se-PKHD1 sihilelekile esifweni sikaCaroli. Kodwa-ke, kudingeka ucwaningo olwengeziwe ukuze kutholakale lokhu.

Odokotela basithola kanjani isifo sikaCaroli?

Uma ubona udokotela, bazoqala ngokukuhlola ngokomzimba bese bebuza ngezimpawu zakho. Ngemuva kwalokho, bazo-oda ukuhlolwa kwegazi, njenge -Comprehensive Metabolic Panel (CMP), ehlanganisa ama-enzyme esibindi . Uma kukhona iphethini yokuphakama kula ma-enzyme, kungabonisa ukuthi i-bile duct ivaliwe.

Ngaphezu kokuhlolwa kwegazi, udokotela wakho cishe uzoyala ukuhlolwa okuningana kwezithombe/i-radiology , okuyizivivinyo ezibonakala ngaphakathi emzimbeni wakho. Lokhu kufaka phakathi:

• I-ultrasound yesisu
• I- CT scan (i-computed tomography scan)
• I- MRI scan (i-magnetic resonance imaging scan)

Ngaphezu kwalokho, udokotela wakho angase akucele ukuthi wenze ukuhlolwa kwe-ERCP (endoscopic retrograde cholangiopancreatography) . Lokhu kuhilela ukufaka ithubhu elincane, eliguquguqukayo (elibizwa ngokuthi i-endoscope ) ngomlomo wakho bese wehla esiswini sakho ungene emigudwini yakho ye-bile. Ngale ERCP, udokotela wakho angabheka emigudwini yakho ye-bile nesibindi ukuze abone ukuthi kukhona yini izimila noma izibazi.

Okunye ukuthi, udokotela wakho angakucela nokuthi wenze uhlobo olukhethekile lwe-MRI olubizwa ngokuthi i-MRCP (magnetic resonance cholangiopancreatography) . Lokhu kungalinganisa ukuthi imisele yakho ye-bile ibanzi kangakanani. Kungahlola namatshe enyongo.

Yiziphi izindlela zokwelapha lokhu? Ingabe kungelapheka?

Uma ngikhuluma iqiniso, isifo sikaCaroliAkukho ukwelashwa okwamanje. Kodwa-ke, kunezindlela zokwelapha ezingasiza ekulawuleni izimpawu nasekuvimbeleni izinkinga. Lokhu kuzodinga ukuthi usebenzisane nethimba lodokotela abangochwepheshe emikhakheni eyahlukene. Lokhu kufaka phakathi:

• Isazi sezakhi zofuzo
• Udokotela Wesisu
• Uchwepheshe wesibindi/udokotela wezifo zesibindi
• Udokotela wezifo zomchamo ( njengoba nezinso nazo zithinteka)
• Udokotela ochwepheshe bezifo zezinso (i-Nephrologist)
• Isazi semisebe ( sezinto ezifana nokuskena)
• Udokotela ohlinzayo wezitho zokufakelwa (uma kudingeka)

Ukwelashwa kwesifo sikaCaroli kuncike ekutheni i-bile duct evulekile ikuphi esibindini.

• Ngezinye izikhathi ukuhlinzwa: Uma i-bile duct ekhulisiwe isehlangothini lwesobunxele noma lwesokudla sesibindi, udokotela wakho angasusa leyo ngxenye yesibindi ngokuhlinzwa. Lokhu kubizwa ngokuthi i-hemi-hepatectomy . Abantu abasuswe ingxenye yesibindi ngale ndlela ngokuvamile ababi nezimpawu futhi.
• Imithi: Kodwa-ke, uma imisele yenyongo evulekile isakazeke esibindini sonke, udokotela wakho angakunika ama-antibiotic ukuvimbela ukutheleleka okubizwa ngokuthi i-cholangitis . Futhi, umuthi obizwa ngokuthi i-ursodeoxycholic acid ungasiza kwezinye zezinkinga ezibangelwa yisifo sikaCaroli.
• Okunye ukuhlinzwa: Ngezinye izikhathi, udokotela wakho angase ancome i-internal biliary bypass ukusiza ukukhipha i-bile esibindini.
• Ukufakelwa kwesibindi: Ezimweni ezimbi kakhulu, ukufakelwa kwesibindi kuyindlela engcono kakhulu yokwelapha.

Ungaphila isikhathi esingakanani unesifo sikaCaroli?

Isifo sikaCaroli ngokwaso asinqumi ngokuqondile isikhathi sakho sokuphila. Isikhathi sokuphila sithinteka yizinkinga ezingabangelwa yiso. Lezi yizi:

• I-Cholangitis: Le cholangitis ebukhali, engabangelwa amatshe enyongo emigudwini yenyongo, idinga ukwelashwa okuphuthumayo futhi ingabulala phakathi kwamahora noma izinsuku.
• Umdlavuza we-bile duct (i-cholangiocarcinoma): Lo mdlavuza ungavela ngenxa yokuvuvukala kwesikhathi eside kanye nokuvaleka kwe-bile ducts. Ukutholwa kusenesikhathi kanye nokwelashwa kubalulekile ukuze kube nemiphumela emihle.
• Ukwehluleka kwesibindi:Uma ukuvuvukala kwemigudu yenyongo kuqhubeka, kungagcina kuholele ezifweni ezinkulu njengokwehluleka kwesibindi.

Into ebaluleke kakhulu ukuphathwa. Uma i-cholangitis yelashwa kahle futhi izinkinga zihlolwa njalo ngudokotela, abantu abanesifo i-Caroli bangaphila impilo ephelele.

Ingabe ikhona indlela yokuvimbela lesi sifo sikaCaroli?

Isifo sikaCaroli siyisifo esibangelwa izakhi zofuzo , ngakho-ke asinakuvinjelwa ngoba siyinto eza nezakhi zofuzo zethu.

Kufanele ngimbone nini udokotela?

Uma uba nezimpawu zesifo sikaCaroli – njengobuhlungu ohlangothini lwesokudla lwesisu sakho, ukuhlanza, umkhuhlane, kanye ne-jaundice – kufanele nakanjani ubone udokotela. Ungalibali.

Yimiphi imibuzo okufanele ngiyibuze udokotela?

Kungumqondo omuhle ukubuza le mibuzo uma ubona udokotela:

• Ingabe nginesifo sikaCaroli noma i-Caroli syndrome?
• Isimo sami sibi kangakanani ngokusekelwe emiphumeleni yokuskena kwami?
• Yiluphi uhlelo lokwelashwa olungcono kakhulu kimi?
• Ingabe ikhona imithi engasiza ekulawuleni izimpawu zami noma ekuvimbeleni izinkinga?
• Ngingakwazi yini ukuhlinzwa? Uma kunjalo, yiluphi uhlobo lokuhlinzwa okufanele ngilwenze?
• Ziyini izingozi nezinzuzo zokuhlinzwa?
• Yiziphi izimpawu zezinkinga okufanele ngizazi?

Uyini umehluko phakathi kwesifo sesibindi i-Polycystic (PCLD) nesifo sikaCaroli?

Isifo sesibindi i-Polycystic (PCLD) kanye nesifo i-Caroli zombili zithinta imisele ye-bile esibindini. Kodwa kunomehluko omkhulu phakathi kwalokhu okubili. Ku-PCLD, ama-cyst amaningi agcwele uketshezi akha isibindi sonke. Lawa afana nama-sacs angavamile. Nakuba ama-cyst angakheka futhi esifweni i-Caroli, ikakhulukazi kuhilela ukwandiswa kwemisele ye-bile ekhethekile ngaphakathi kwesibindi.

Ngakho-ke buyini ubudlelwano phakathi kwesifo sikaCaroli nama-cysts e-choledochal?

Isifo sikaCaroli sithinta imigudu yenyongo yesibindi, okuyimigudu yenyongo engaphakathi kwesibindi . Ama-cyst akhiwa kulezi migudu yenyongo yesibindi awuhlobo lwe -choledochal cyst . Ngakho-ke, uma unezinhlobo eziningana zalezi cyst, lokho kubizwa ngokuthi isifo sikaCaroli. Kalula nje, isifo sikaCaroli uhlobo lwe-choledochal cyst.

Okokugcina, izinto ezibaluleke kakhulu okudingeka uzikhumbule

Isifo sikaCaroli singaba yinto eyesabekayo ukuzwa ngaso. Kodwa-ke, ngokuthuthuka kwesayensi yezokwelapha, manje sekunezindlela ezisebenzayo zokuphatha lesi simo. Ngokuhlolwa njalo, wena nodokotela wakho ningaqapha isimo sakho eduze. Abantu abaningi abaneSifo sikaCaroli bathola impumuzo ezimpawini zabo ngemithi , kanyeNjengoba zingenawo umonakalo omkhulu, okusho ukuthi azikho izimbobo ezinkulu, izinqubo zezokwelapha zivame ukuba yindlela yokukhetha.

Khumbula, ukugxila lapha kukugcina uphilile futhi usebenza isikhathi eside ngangokunokwenzeka. Ngakho-ke, ungesabi, vele ulandele imiyalelo kadokotela wakho ngokucophelela. Ungesabi ukubuza imibuzo uma unayo.

Isifo sikaCaroli , Isibindi, Amapayipi Enyongo, Izifo Zofuzo, I-Jaundice, Amatshe Enyongo