Ingaba imibhobho yenyongo yakho ikhulisiwe? Masithethe ngesifo sikaCaroli!

Ngaba ngamanye amaxesha uziva ngathi umzimba wakho ujika ube tyheli, okanye uziva ngathi ufumana intlungu kwicala lasekunene lesisu sakho? Abanye abantu banokuba nomkhuhlane kunye nokuhlanza kunye nezi mpawu. Esinye isizathu esinokubangela oku yimeko esiza kuthetha ngayo namhlanje ebizwa ngokuba yi-'Caroli's disease.' Makhe sibone ukuba yintoni, kutheni isenzeka, ifunyaniswa njani, kunye nonyango.

Ngamafutshane, yintoni iCaroli Disease?

Khawucinge nje, ngaphakathi kwesibindi sethu kukho iityhubhu ezincinci ezibizwa ngokuba zii-bile ducts . Ezi tyhubhu zithwala ulwelo lokugaya ukutya olubizwa ngokuba yi-bile eveliswa esibindini ukuya emathunjini amancinci. Ngoku, esi sifo sikaCaroli sisifo apho ii-bile ducts ziba nkulu kunesiqhelo, oko kukuthi, ziyavuleka . Esi sisifo semfuza , oko kukuthi, sibangelwa kukutshintsha kwezakhi zofuzo zethu. Xa ezi tyhubhu ziba nkulu ngolu hlobo, i-bile iqala ukuqokelelana endaweni yokuhamba kakuhle. Xa oku kuqokelelana, amatye e-bile duct anokwenziwa, usulelo lunokwenzeka, kwaye ngamanye amaxesha ii-cysts zinokuvela.

Ngamafutshane, inyongo ifanele ikwazi ukuhamba ngokulula ngaphandle kokunamathela. Nangona kunjalo, xa imibhobho yenyongo iba mikhulu kakhulu, inyongo inokuqokelelana ngokulula. Ekuhambeni kwexesha, le nyongo iqokelelanayo inokwenza amatye amancinci.

Ukuba unesifo sikaCaroli, unokufumana iimpawu ezininzi zale meko ubomi bakho bonke. Iimpawu zihlala ziqala malunga neminyaka engama-30 ubudala , kodwa zinokwenzeka nakweyiphi na iminyaka. Usenokubona ulusu lwakho kunye namehlo akho amhlophe ejika abe tyheli (esikubiza ngokuba yi- jaundice ), kwaye ungaqala ukufumana iimpawu ezifana nomkhuhlane kunye nomkhuhlane rhoqo. Nangona le meko inokuchaphazela impilo yakho kunye nomgangatho wobomi bakho, kukho unyango olunokukunceda ukulawula iimpawu zakho.

Ngaba kukho iintlobo zesifo sikaCaroli?

Ewe, kukho iintlobo ezimbini eziphambili zale nto:

• Isifo sikaCaroli: Olu hlobo luchaphazela kuphela imibhobho yenyongo yakho. Alubangeli naziphi na ezinye iingxaki kwisibindi sakho.
• I-Caroli syndrome: Olu lolona hlobo lubi kakhulu. Kule meko, isibindi siba namanxeba, esiwabiza ngokuba yi-fibrosis .

Ixhaphake kangakanani le sifo?

Isifo sikaCaroli sisifo esingaqhelekanga kakhulu . Kuqikelelwa ukuba ngumntu omnye kuphela kwisigidi esiba naso . Ngoko ke asisosifo esichaphazela abantu abaninzi.

Ngoko ke, zithini iimpawu zesifo sikaCaroli?

Ngokwesiqhelo, amatye amancinci aqukuqela kwimibhobho yenyongo awabangeli zimpawu zinkulu. Nangona kunjalo, ukuba ilitye livala umbhobho wenyongo, linokudumba. Oku sikubiza ngokuba yi -cholangitis . Kulapho iimpawu zesifo sikaCaroli zibonakala khona:

• Intlungu kwicala lasekunene lomzimba wakho , rhoqo kwisisu esingaphezulu.
• Ukuhlanza .
• Umkhuhlane kunye nokubanda .
• Ukutyheli kolusu kunye namhlophe emehlweni (i-jaundice) .
• Ukurhawuzelela .

Kunqabile kakhulu, oku kuqokelelana kwenyongo kunokukhokelela ekukhuleni kohlobo lwethumba lomhlaza elibizwa ngokuba yi-cholangiocarcinoma kwimibhobho yenyongo. Yiyo loo nto kubalulekile ukuba ulumke ngale nto.

Kutheni isifo sikaCaroli sisenzeka? Zithini izizathu?

Isifo sikaCaroli sisifo esibangelwa yimfuza . Oku kuthetha ukuba sibangelwa kukutshintsha kwezakhi zofuzo emizimbeni yethu. Uninzi lwexesha, sinokwenzeka ngamaxesha athile, oko kuthetha ukuba asidluliselwa kwizizukulwana ngezizukulwana. Nangona kunjalo, kwezinye iintsapho, kuye kwaxelwa ukuba sisifo esilawulayo , oko kuthetha ukuba ukuba omnye umzali unaso, nabantwana banokusifumana.

I-Caroli syndrome ibangelwa kukuguquka kwezakhi zofuzo kwi-gene ebizwa ngokuba yi-PKHD1 . Le gene inoxanduva lokuvelisa iproteni enceda i-bile ducts kunye nezintso ukuba zikhule ngexesha le-embryonic stage, oko kukuthi, xa umntwana esesibelekweni. Ngenxa yokuba isifo sikaCaroli kunye ne-Caroli syndrome zihlobene, abaphandi bakholelwa ukuba i-PKHD1 gene nayo ibandakanyeka kwisifo sikaCaroli. Nangona kunjalo, uphando oluthe kratya luyadingeka ukuze kuchongwe oku.

Oogqirha basixilonga njani isifo sikaCaroli?

Xa ubona ugqirha, baya kuqala benze uvavanyo lomzimba baze babuze ngeempawu zakho. Emva koko, baya kuyalela uvavanyo lwegazi, olufana neComprehensive Metabolic Panel (CMP), equka ii-enzymes zesibindi . Ukuba kukho indlela yokuphakama kwezi enzymes, oko kunokubonisa ukuba i-bile duct ivaliwe.

Ukongeza kwiimvavanyo zegazi, ugqirha wakho usenokuyalela iimvavanyo ezininzi ze-imaging/radiology , eziziimvavanyo ezijongeka ngaphakathi emzimbeni wakho. Ezi ziquka:

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

Ukongeza, ugqirha wakho angakucela ukuba wenze uvavanyo lwe-ERCP (endoscopic retrograde cholangiopancreatography) . Oku kuquka ukufaka ityhubhu encinci, eguquguqukayo (ebizwa ngokuba yi-endoscope ) ngomlomo wakho ize yehle esiswini sakho iye kwimibhobho yakho yenyongo. Ngale ERCP, ugqirha wakho unokujonga imibhobho yakho yenyongo kunye nesibindi ukuze abone ukuba kukho na iithumba okanye amanxeba.

Enye into kukuba, ugqirha wakho angakucela ukuba wenze uhlobo oluthile lwe-MRI olubizwa ngokuba yi -MRCP (magnetic resonance cholangiopancreatography) . Oku kunokulinganisa ububanzi bemibhobho yakho yenyongo. Kunokujonga namatye enyongo.

Zithini iindlela zokunyanga oku? Ngaba kunokunyangwa?

Ukuthetha inyani, isifo sikaCaroliAkukho nyango okwangoku. Nangona kunjalo, kukho unyango olunokunceda ukulawula iimpawu kunye nokuthintela iingxaki. Oku kuya kufuna ukuba usebenzisane neqela loogqirha abaziingcali kwiinkalo ezahlukeneyo. Ezi ziquka:

• Ingcali yemfuza
• Ingcali yezifo zesisu
• Ingcali yesibindi/Ingcali yeHepatologist
• Ugqirha wezifo zomchamo ( njengoko nezintso nazo zichaphazeleka)
• Ugqirha ogxile kwizifo zezintso (i-Nephrologist)
• Ingcali ye-radiology ( kwizinto ezinje ngeeskeni)
• Ugqirha wotyando lokufakelwa izityalo (ukuba kuyimfuneko)

Unyango lwesifo sikaCaroli luxhomekeke ekubeni iphi i-bile duct evulekileyo esibindini.

• Ngamanye amaxesha utyando: Ukuba i-bile duct ekhulisiweyo ikwicala lasekhohlo okanye lasekunene lesibindi, ugqirha wakho unokususa loo nxalenye yesibindi ngotyando. Oku kubizwa ngokuba yi-hemi-hepatectomy . Abantu abasuswe inxalenye yesibindi ngale ndlela badla ngokungabi nazo iimpawu kwakhona.
• Amayeza: Nangona kunjalo, ukuba imibhobho yenyongo evulekileyo isasazeke kwisibindi sonke, ugqirha wakho unokukunika amayeza okubulala iintsholongwane ukuthintela usulelo olubizwa ngokuba yi-cholangitis . Kwakhona, iyeza elibizwa ngokuba yi -ursodeoxycholic acid linokunceda kwezinye zeengxaki ezibangelwa sisifo sikaCaroli.
• Ezinye iindlela zotyando: Ngamanye amaxesha, ugqirha wakho unokucebisa ukuba kwenziwe i-internal biliary bypass ukunceda ekukhupheni i-bile esibindini.
• Ukufakelwa kwesibindi: Kwiimeko ezinzima kakhulu, ukufakelwa kwesibindi lolona khetho lungcono lonyango.

Ungaphila ixesha elingakanani unesifo sikaCaroli?

Isifo sikaCaroli asichazi ngokuthe ngqo ukuba uza kuphila ixesha elingakanani. Ubungakanani bobomi obulindelekileyo buchaphazeleka ziingxaki ezinokubangela. Nazi:

• I-Cholangitis: Le cholangitis ibukhali, enokubangelwa ngamatye enyongo kwimibhobho yenyongo, ifuna unyango olungxamisekileyo kwaye inokubulala kwiiyure okanye kwiintsuku.
• Umhlaza we-bile duct (cholangiocarcinoma): Lo mhlaza ungavela ngenxa yokudumba kwexesha elide kunye nokuvaleka kwe-bile ducts. Ukufunyanwa kwangethuba kunye nonyango kubalulekile ukuze kubekho iziphumo ezilungileyo.
• Ukungasebenzi kakuhle kwesibindi:Ukuba ukudumba kwemisele yenyongo kuyaqhubeka, ekugqibeleni kunokukhokelela kwizifo ezinzulu ezifana nokungasebenzi kakuhle kwesibindi.

Eyona nto ibalulekileyo kukulawula i-cholangitis. Ukuba i-cholangitis inyangwa ngokufanelekileyo kwaye iingxaki zijongwa rhoqo ngugqirha, abantu abanesifo sikaCaroli banokuphila ubomi obupheleleyo.

Ngaba ikho indlela yokuthintela esi sifo sikaCaroli?

Isifo sikaCaroli sisifo esibangelwa lufuzo , ngoko ke asinakuthintelwa kuba yinto eza nemfuza yethu.

Ndifanele ndimbone nini ugqirha?

Ukuba ufumana iimpawu zesifo sikaCaroli – ezinje ngentlungu kwicala lasekunene lesisu sakho, ukuhlanza, umkhuhlane, kunye ne-jaundice – ngokuqinisekileyo kuya kufuneka ubone ugqirha. Musa ukulibazisa.

Ndingayibuza yiphi imibuzo ugqirha?

Kungcono ukubuza le mibuzo xa ubona ugqirha:

• Ngaba ndinesifo sikaCaroli okanye isifo sikaCaroli?
• Ingxaki yam ingakanani ngokweziphumo zeskeni zam?
• Yeyiphi isicwangciso sonyango esifanelekileyo kum?
• Ngaba kukho amayeza anokunceda ukulawula iimpawu zam okanye ukuthintela iingxaki?
• Ndingatyandwa? Ukuba kunjalo, loluphi uhlobo lotyando endifanele ndilwenze?
• Zithini iingozi kunye neenzuzo zotyando?
• Zeziphi iimpawu zeengxaki ekufuneka ndizazi?

Yintoni umahluko phakathi kwePolycystic Liver Disease (PCLD) kunye nesifo sikaCaroli?

Isifo sesibindi sePolycystic (PCLD) kunye nesifo seCaroli zombini zichaphazela imibhobho yenyongo esibindini. Kodwa kukho umahluko omkhulu phakathi kwezi zimbini. KwiPCLD, ii-cysts ezininzi ezizaliswe lulwelo (ii-cysts) zenzeka kuso sonke isibindi. Ezi zifana neengxowa ezingaqhelekanga. Nangona ii-cysts zinokwakheka nakwisifo seCaroli, ikakhulu zibandakanya ukwandiswa kwemigudu yenyongo ekhethekileyo esibindini.

Ngoko ke yintoni ubudlelwane phakathi kwesifo sikaCaroli kunye ne-choledochal cysts?

Isifo sikaCaroli sichaphazela imibhobho yenyongo engaphakathi kwesibindi, eziziiduct zenyongo ezingaphakathi kwesibindi . Iicysts ezikhula kwezi duct zenyongo engaphakathi kwesibindi luhlobo lwe -choledochal cyst . Ngoko ke, ukuba unezinye zezi cysts, oko kubizwa ngokuba sisifo sikaCaroli. Ngamafutshane, isifo sikaCaroli luhlobo lwe-choledochal cyst.

Okokugqibela, ezona zinto zibalulekileyo ekufuneka uzikhumbule

Isifo sikaCaroli sinokuba yinto eyoyikisayo ukuyiva. Nangona kunjalo, ngokuhambela phambili kwesayensi yezonyango, ngoku kukho iindlela ezisebenzayo zokulawula le meko. Ngokujonga rhoqo, wena nogqirha wakho ninokujonga imeko yakho ngokusondeleyo. Abantu abaninzi abanesi sifo sikaCaroli bafumana isiqabu kwiimpawu zabo ngamayeza , kwayeAyichaphazeleki kangako, nto leyo ethetha ukuba akukho zisikelo zinkulu, iinkqubo zonyango zihlala ziyindlela yokukhetha.

Khumbula, ingqwalasela apha ikukugcina usempilweni kwaye usebenza ixesha elide kangangoko unako. Ngoko ke, ungoyiki, landela imiyalelo kagqirha wakho ngononophelo. Ungoyiki ukubuza imibuzo ukuba unayo.

Isifo sikaCaroli , Isibindi, Imibhobho yenyongo, Izifo zofuzo, i-Jaundice, amatye enyongo