Ngaba unengxaki ngemibhobho yakho yenyongo? Masifunde ngePrimary Sclerosing Cholangitis (PSC)!

Ngamanye amaxesha siziva sidiniwe ngaphandle kwesizathu, sifumana iintlungu ezincinci kwicala eliphezulu lasekunene lesisu sethu, okanye mhlawumbi siziva ngathi siyazikrwempa. Nangona singazinaki kakhulu ezi zinto kubomi bethu bemihla ngemihla, ngamanye amaxesha ezi zinokuba ziimpawu zemeko embi kakhulu emzimbeni wethu. Namhlanje siza kuthetha ngemeko enzima kancinci, kodwa kubaluleke kakhulu ukuba wonke umntu ayiqaphele. Leyo yiPrimary Sclerosing Cholangitis , okanye iPSC ngamafutshane.

Yintoni i-primary sclerosing cholangitis (PSC)?

Ngamafutshane, i-PSC sisifo esenzeka kwimibhobho yenyongo emzimbeni wethu. Njengoko unokukhumbula, inyongo iveliswa esibindini. Le nyongo inceda ekugayweni kokutya. Ngoko ke, kukho inkqubo yeetyhubhu ezincinci ezithwala le nyongo ukusuka esibindini ukuya emathunjini. Sizibiza ezo tyhubhu ngokuthi yimibhobho yenyongo.

Kwi-PSC, kukho ukudumba okungapheliyo , okanye ukudumba, kwezi ducts zenyongo. Oogqirha bakwabiza oku ngokuba yi-cholangitis . Xa oku kudumba kuqhubeka ixesha elide, kuqala ukwakheka kwamanxeba kwiducts zenyongo. Oku kubangelwa kukuba amanxeba abangela ukuba iducts zenyongo zibe nzima kwaye zimxinwa. Oku kubizwa ngokuba yi -sclerosis .

Khawucinge ngombhobho wamanzi ovalwe ngumhlaba kwaye uncipha, nto leyo eyenza kube nzima ukuba amanzi adlule kuwo, akunjalo? Ngokufanayo, xa imibhobho yenyongo incipha, ukuhamba kwenyongo kuyathintelwa. Oku kubizwa ngokuba yi-biliary stricture . Ngoko ke, xa inyongo ingakwazi ukuhamba kakuhle, iqala ukubuyela esibindini. Oku kuqokelelana kwenyongo esibindini (i-cholestasis) kuyawonakalisa kancinci kancinci isibindi.

Ingaba le meko ye-PSC imbi kakhulu?

Ewe, i-PSC sisifo esibi kakhulu . Usenokungabi nazimpawu kwizigaba zokuqala. Kodwa sisifo esiqhubekayo. Xa inyongo ivalekile kwimibhobho yenyongo (i-cholestasis), ezinye zeetyhefu kwinyongo zinokuqokelelana egazini. Emva koko uqala ukuziva ungakhululekanga. Kwakhona, oku kubangela umonakalo oqhubekayo kwisibindi.

Ngokwesiqhelo, lo monakalo unokukhokelela ekusileleni ngokupheleleyo kwesibindi emva kweminyaka eli-10 ukuya kweli-15. Njengoko usazi, asinakuphila ngaphandle kwesibindi. Nangona unyango lwangoku lunokulawula iimpawu, akukho nyango lwe-PSC. Isisombululo kuphela kukufakelwa kwesibindi .

Yintoni umahluko phakathi kwe-cholangitis ye-sclerosing yokuqala neyesibini?

Igama elithi "primary" apha lithetha ukuba esi sifo sesona sizathu siphambili sokudumba kunye nokuqhekeka kwemibhobho yenyongo. Oko kukuthi, le meko ibangelwa sesi sifo sodwa, ngaphandle kwesinye isizathu.

Kodwa,Kwimeko ebizwa ngokuba yi-secondary sclerosing cholangitis, ukudumba (i-cholangitis) kunye nokukrweleka (i-sclerosis) kwe-bile ducts kwenzeka ngenxa yesinye isizathu. Oko kukuthi, le meko ibangelwa sisifo okanye isiganeko esithile. Nazi ezinye imizekelo:

• Ukulimala kwimibhobho yenyongo (umzekelo, ngexesha lotyando).
• Iintsholongwane kwimibhobho yenyongo.
• Unyango lokulwa nomhlaza (iChemotherapy).
• Amatye enyongo avala imibhobho yenyongo.
• I-pancreatitis ephindaphindayo.

Yintoni umahluko phakathi kwe-primary biliary cirrhosis (PBC) kunye ne-PSC?

Esi sifo, esasisaziwa ngokuba yiPrimary Biliary Cirrhosis, ngoku sibizwa ngokuba yiPrimary Biliary Cholangitis (PBC) .

Zombini i-PBC kunye ne-PSC zizifo eziqhubekekayo ezichaphazela isibindi, zichaphazela imibhobho yenyongo. Iimpawu kunye nemiphumo yazo zombini ziyafana kakhulu. Zombini ezi zifo zibangela ukuba imibhobho yenyongo ibe buthathaka kancinci kancinci. Xa imibhobho yenyongo yonakele, itshiswe, kwaye inciphe, ukuhamba kwenyongo kuyavalwa. Emva koko inyongo ibuyela esibindini, yonakalise ngakumbi isibindi. Oku kunokukhokelela ekuqhekekeni kwesibindi kwaye ekugqibeleni kube yi-cirrhosis yesibindi .

Kodwa kukho umahluko omkhulu phakathi kwezi zifo zimbini:

• I-PBC ichaphazela kuphela imibhobho yenyongo engaphakathi kwesibindi (intrahepatic). I-PSC inokuchaphazela zombini imibhobho yenyongo engaphakathi kwesibindi kunye nemibhobho yenyongo engaphandle kwesibindi (extrahepatic).
• I-PBC ixhaphake kakhulu kwabasetyhini (umlinganiselo we-10:1), ngelixa i-PSC ixhaphake kakhulu kwamadoda (umlinganiselo we-2:1).
• Malunga nama-80% ezigulane ze-PSC zinesifo samathumbu esibangelwa kukudumba (i-IBD) esele sikhona, ingakumbi i-ulcerative colitis . Nangona kunjalo, oku akunanto yakwenza ne-PBC.
• Kukho umngcipheko ophezulu wokuba nomhlaza we-bile duct nge-PSC. Akukho mngcipheko mkhulu kangako nge-PBC.
• I-Ursodeoxycholic acid (UDCA) isetyenziselwa ukunyanga i-PBC, kodwa okwangoku akukho nyango luthile lwe-PSC.

Ixhaphake kangakanani le meko ibizwa ngokuba yi-PSC? Ngubani odla ngokuba nayo?

I-PSC sisifo esingaqhelekanga . Ehlabathini lonke, kuqikelelwa ukuba ngumntu omnye kuphela kubantu abalishumi lamawaka abanaso esi sifo.

Esi sifo sixhaphake kabini kumadoda kunakwabasetyhini. Esi sifo sidla ngokufunyaniswa simalunga neminyaka engama-40 ubudala. Malunga nama-80% abantu abane-PSC nabo bane -IBD (Inflammatory Bowel Disease) , ingakumbi i-Ulcerative Colitis . Ukuba kukho umntu kusapho onale sifo, nabanye basengozini yokuba naso.

Yintoni ebangela i-PSC?

Isizathu esichanekileyo se-PSC asikaziwa okwangoku, kodwa kucingelwa ukuba sibangelwa zizinto ezahlukeneyo. Ezi ziquka:

• I-genetics
• Izinto ezisingqongileyo
• Iiseli zomzimba ezikhusela umzimba

Oogqirha bacinga ukuba i-PSC isenokuba luhlobo lwesifo sokuzikhusela komzimba . Oku kuthetha ukuba inkqubo yokuzikhusela yomzimba wethu, amajoni afanele ukusikhusela kwizifo, ngempazamo aqala ukuhlasela iiseli zethu eziphilileyo. Ukudumba sesinye sezixhobo zenkqubo yethu yokuzikhusela. Kudla ngokuba yinto yexeshana, isebenza nxamnye nomhlaseli. Kodwa ukuba ukudumba kuba yinto engapheliyo, kudla ngokuba luphawu lwesifo.

Oogqirha baqaphele nokuba abantu abane-PSC banamathuba amaninzi okufumana ezinye izifo ezizikhuselayo emzimbeni. Umzekelo:

• Izifo zokudumba kwamathumbu
• Isifo seCeliac
• Isifo se-thyroid
• Uhlobo loku-1 lweswekile
• I-hepatitis ye-autoimmune
• I-autoimmune pancreatitis

Kucingelwa ukuba olu hlobo lokusabela komzimba lunokwenzeka xa abantu abaneempawu ezithile zemfuza bechaphazeleka zizinto ezithile ezingqongileyo (umz., ukuvezwa kwiikhemikhali ezinobungozi).

Zithini iimpawu ze-PSC?

Njengoko i-PSC iqhubeka, iimpawu ziyatshintsha. Malunga nama-50% abantu basenokungabi nazo iimpawu konke konke xa isifo sifunyaniswa. I-PSC idla ngokufunyaniswa ngengozi, ngexesha lovavanyo lwenye imeko. Iimpawu zokuqala azibonakali ngokucacileyo. Ziquka:

• Ukudinwa
• Intlungu yesisu esisezantsi ngasekunene
• Ulusu olurhawuzelelayo (ukurhawuzelela)

Iimpawu ezinokwenzeka njengoko isifo siqhubeka ziquka:

• Isisu esidumbileyo
• Isibindi esikhulu
• I-spleen ekhulisiweyo
• I-Jaundice - Oku kuthetha ukuba tyheli kwamhlophe emehlweni naselusu.
• Ifiva
• Ukunciphisa umzimba okungafunekiyo

Zithini iingxaki ze-PSC enzima?

I-Primary sclerosing cholangitis sisifo esiqhubeka kancinci kancinci, kodwa njengoko i-bile ducts kunye nesibindi sakho ziba namabala kwaye ukusebenza kwazo kuyehla, iingxaki ezahlukeneyo zinokwenzeka.

Izifo zeMetabolic

Ukuba imibhobho yenyongo yakho ivalekile kakhulu, ayikwazi ukupompa inyongo ngokufanelekileyo emathunjini akho amancinci. Inyongo ibalulekile ekugayeni ukutya, ingakumbi amafutha, nasekufunxeni iivithamini ezinyibilikayo kumafutha (A, D, E, kunye no-K). Ngaphandle kwenyongo, inkqubo yakho yokugaya ukutya ayinakukwazi ukucubungula amafutha ngokufanelekileyo, kwaye ayinakukwazi ukufunxa ezi vithamini zibalulekileyo ekutyeni. Oku kunokukhokelela kwiingxaki ezongezelelweyo:

• Indle enamafutha kunye norhudo
• Ukungafunxwa kokutya kunye nokungondleki kakuhle
• Ukukruquka nokuphuma kwegazi lula (ngenxa yokunqongophala kwevithamini K)
• Ubuthathaka bamathambo - i-Osteomalacia kunye ne-osteoporosis (ngenxa yokunqongophala kwevithamini D)
• Ukubona kakubi ebusuku (ngenxa yokunqongophala kwevithamini A)

Uxinzelelo lwegazi oluphezulu lwePortal

Njengoko isifo sesibindi siqhubeka, ukuhamba kwegazi esibindini kuyancipha. Oku kwandisa uxinzelelo kwi-portal vein, edlula kwinkqubo yakho yokugaya ukutya. Olu xinzelelo lunokubangela ukuba eminye imithambo kwi-esophagus yakho nasesiswini ikhule ize iqhume. Oku kunokukhokelela ekuphumeni kwegazi ngaphakathi .

Iintsholongwane

Imibhobho yenyongo evalekileyo idla ngokusuleleka, nto leyo ebangela iimeko ezifana nomkhuhlane, iintlungu zesisu, kunye nokutyhefwa ligazi (sepsis).

Umhlaza

Njengoko i-PSC iba mandundu ngakumbi, umngcipheko wokufumana iintlobo ezahlukeneyo zomhlaza uyanda:

• Umhlaza we-bile duct (i-cholangiocarcinoma) - Umngcipheko ophakathi kwe-5% kunye ne-20%.
• Umhlaza wenyongo
• Umhlaza wesibindi (i-hepatitis)
• Umhlaza we-Coloral Cancer - ukuba nawe une-IBD.

Ifunyaniswa njani i-PSC?

Amaxesha amaninzi, esi sifo sifunyanwa ngengozi, xa kuvavanywa enye into. Malunga nama-50% abantu basenokungabi nazimpawu xa isifo sifunyenwe. Iimpawu zokuqala zesifo zinokubonwa ngovavanyo lwegazi okanye uvavanyo lomfanekiso. Iiskeni zemibhobho yenyongo enePSC zibonisa iimpawu ezikhethekileyo. Ukuba uvavanyo lwegazi lubonisa amanqanaba aphezulu e-enzyme ebizwa ngokuba yi-alkaline phosphatase , okanye ukuba kukho ii-antibodies ezithile ezibonisa impendulo yomzimba kwimibhobho yenyongo, kunokuba luphawu lwePSC. Ukwanda kweeseli ezimhlophe zegazi kudla ngokuba luphawu losulelo lwesibindi.

Ukuqinisekisa ukuxilongwa, ugqirha wakho unokucebisa ezinye iimvavanyo ezithile:

• Uvavanyo lokusebenza kwesibindi (LFT): Olu vavanyo lwegazi lukhangela amanqanaba aphezulu ee-enzyme ezithile esibindini. Amanqanaba aphezulu e-alkaline phosphatase anokubonisa i-PSC.
• I-Magnetic resonance cholangiopancreatography (MRCP) : Olu luvavanyo lwe-MRI (Magnetic Resonance Imaging) . Lungathatha imifanekiso eneenkcukacha zenkqubo yakho yenyongo (isibindi, inyongo, kunye ne-bile ducts). Olu luvavanyo lokuqala lokufumanisa i-PSC. Oku kungenxa yokuba alungenisi kwaye alubandakanyi ukuvezwa kwimitha. Nangona kunjalo, ngamanye amaxesha, ukuba isifo sisekutsha okanye asibi kakhulu, sisenokungakwazi ukusibona ngokuchanekileyo. Emva koko kunokufuneka olunye uvavanyo.

Iphathwa njani i-PSC?

Okwangoku, akukho nyango lunokuthintela okanye lubuyisele umva ukuqhubela phambili kwe-primary sclerosing cholangitis. Nangona kunjalo, iimpawu kunye neengxaki zinokunyangwa ngokuthe ngqo. Umzekelo, ugqirha wakho unokukunika izinto ezifana nezi:

• Amayeza okunyanga ulusu olurhawuzelelwayo (ukurhawuzelelwa)
• Izongezo zokunyanga ukungabikho kweevithamini
• Ii-antibiotics zokunyanga izifo

Ugqirha wakho uza kujonga isibindi sakho kunye nemijelo yenyongo. Njengoko isifo siqhubeka, ngamanye amaxesha banokuvula umjelo wenyongo ovalekileyo. Oku kungenziwa ngovavanyo olubizwa ngokuba yi -ERCP (endoscopic retrograde cholangiopancreatography) . Le yinkqubo ekuvumela ukuba ubone imijelo yenyongo yakho ngaphandle kotyando.

I-ERCP luhlobo lwe -endoscopy . Ibandakanya ukuhlola amalungu akho usebenzisa i-endoscope, ityhubhu ende, eguquguqukayo enekhamera encinci kunye nokukhanya kuyo. Emva kokuba uhlanjululwe, ugqirha wakho uza kufaka le tyhubhu ezantsi emqaleni wakho nasesiswini sakho. Izixhobo ezincinci zingadluliselwa kule tyhubhu ukunyanga imibhobho yakho yenyongo. Ingcali ingasebenzisa ibhaluni ukwandisa umbhobho wakho wenyongo, okanye ifake i-stent ukuze ihlale ivulekile. Ukuba i-ERCP ayikwazi ukufikelela ekuvalekeni, inkqubo ebizwa ngokuba yi-'percutaneous trans-hepatic cholangiography (PTHC)' ngamanye amaxesha iyasetyenziswa.

Kodwa esi sisisombululo sexeshana nje kuphela. Kwiminyaka eli-10 ukuya kwengama-20, isifo se-sclerosis cholangitis esiqalayo siya sibi kancinci kancinci, isifo sesibindi siba sibi kakhulu, kwaye ekugqibeleni isibindi siyasilela. Ugqirha wakho uza kujonga umonakalo kwisibindi sakho aze agqibe ukuba uza kuqwalasela nini ukufakelwa isibindi . Kufuneka uhlangabezane nemigangatho ethile ukuze ube kuluhlu lokulinda ukufakelwa isibindi.

Ungaphila ixesha elingakanani ne-PSC?

Nje ukuba kufunyaniswe ukuba isifo sifunyenwe, umyinge wobomi uyahluka phakathi kweminyaka eli-10 ukuya kwengama-20. Ukufakelwa kwesibindi kunokunika ithuba elitsha lokuphila. Nangona kunjalo, kwi-15% ukuya kwi-20% yamatyala, i-PSC inokubuya nasemva kokufakelwa kwesibindi. Ukuba oko kuyenzeka, isibindi esitsha sinokungasebenzi. Kwiimeko ezinjalo, umyinge wobomi obulindelekileyo umalunga neenyanga ezilithoba.

Enye into enokuchaphazela ixesha lokuphila ngumhlaza. Ukuba unesifo somhlaza njengengxaki ye-PSC, usenokungabi ngumntu ofanelekileyo wokufakelwa isibindi. Kwiimeko ezikhethwe ngononophelo, oogqirha bangaqala ngokunyanga umhlaza ngemitha okanye i-chemotherapy, baze emva koko bazame ukufakelwa isibindi.

Ndingazinyamekela njani ukuba ndinesi sifo?

Ukwenza utshintsho kwindlela yokuphila kunokunceda ukulawula ukudinwa okuxhaphakileyo kwi-PSC kunye nokuthintela ukonakala kwesibindi. Umzekelo:

• Kuphephe ngokupheleleyo utywala.
• Yitya ukutya okucutshungulweyo kancinci kunokutya okupheleleyo.
• Lawula uxinzelelo.
• Lala kamnandi.
• Yenza umthambo yonke imihla.

I-Primary sclerosing cholangitis sisifo esingaqhelekanga, esingaqikelelweyo, nesinokuthintelwa. Siqhubeka kancinci, kwaye asibonakali zimpawu kwizigaba zokuqala. Kodwa ukuba ubona ugqirha wakho rhoqo kwaye ufumana uvavanyo oluqhelekileyo, esi sifo sinokufunyanwa ngaphambi kokuba siqale ukuchaphazela ubomi bakho. Kulapho unokwenza utshintsho kwindlela ophila ngayo olunokunceda ukugcina isibindi sakho siphilile ixesha elide. Uya kuthunyelwa kunyango lwexesha elide, oko kuthetha ukuvavanywa rhoqo kunye nokungenelela ukuba kuyimfuneko.

Okokugqibela, izinto ekufuneka uzikhumbule

Kulungile, ndiyathemba ukuba oko sithethe ngako nge-PSC kukuncede ukhumbule ezinye zezona zinto zibalulekileyo. Yimeko enzima kancinci, kodwa kubaluleke kakhulu ukuba uyiqaphele.

• I-PSC sisifo esichaphazela imibhobho yenyongo kwaye siba sibi ngokuhamba kwexesha. Singonakalisa isibindi.
• Kusenokungabikho zimpawu kwizigaba zokuqala, ngoko ke kubalulekile ukuba uhlolwe rhoqo ngugqirha.
• Okwangoku akukho nyango lwesi sifo, kodwa kukho unyango lokulawula iimpawu nokuphucula umgangatho wobomi.
• Ukufakelwa kwesibindi sisisombululo esiphambili kwiimeko ezinzima.
• Ukulandela indlela yokuphila esempilweni kunceda ekukhuseleni isibindi.

Ukuba uneminye imibuzo malunga ne-PSC, okanye ukuba ucinga ukuba unayo nayiphi na kwezi mpawu, qiniseka ukuba ubona ugqirha ukuze akunike ingcebiso . Ungakhathazeki, kukho izinto onokuzenza ukuze uyilawule ukuba ifunyenwe kwangethuba.

I- Sclerosing Primary Cholangitis, i-PSC, Isifo se-Bile Duct, Isifo sesibindi, i-jaundice, Intlungu yesisu, i-Sclerosing Primary Cholangitis