Ngaba kukho ingxaki ngemibhobho yenyongo yesibindi sakho? Masifunde ngqo ngePrimary Biliary Cholangitis (PBC)!

Hayi bo, ngaba nawe uziva ngathi udiniwe kakhulu, udiniwe nangona ungenzi nto? Okanye ngamanye amaxesha uziva ngathi ukrwela ulusu lwakho ngaphandle kwesizathu? Ngamanye amaxesha, nangona singazinaki kakhulu ezi zinto, zinokuba luphawu lwengxaki yesibindi, esinye sezitho ezibalulekileyo emzimbeni wethu. Namhlanje siza kuthetha ngesifo esinjalo esichaphazela isibindi, kwaye nangona igama lisenokubonakala liyinkimbinkimbi kancinci, kulula ukuliqonda. Oko kukuthi (iPrimary Biliary Cholangitis) , ekwaziwa ngokuba yi- (PBC) ngamafutshane.

Yintoni iPrimary Biliary Cholangitis (PBC)? Masiyiqonde ngokulula!

Kulungile, eli gama libonakala lide kancinci, akunjalo? Masibone ukuba lithetha ukuthini.

"I-Cholangitis" kukudumba kwemibhobho yakho yenyongo . Uyazi, ulwelo olubizwa ngokuba yinyongo, oluveliswa esibindini sethu, luhamba ngenkqubo yeetyhubhu ezincinci. Ezo tyhubhu zibizwa ngokuba yimibhobho yenyongo. Igama elithi "Biliary" likwathetha into enxulumene nale mibhobho yenyongo. Igama elithi "Primary" lithetha "isiseko" okanye "isiqalo."

Emva koko, xa udibanisa konke oku, i-Primary Biliary Cholangitis ithetha ukuba, kungekhona ngenxa yesinye isifo, usulelo, okanye ukuvaleka, kodwa ngenxa yesi sifo, kukho ukudumba kwemisele yenyongo ngaphakathi kwesibindi sakho . Ekubeni oku kudumba kungenzeki ngenxa yento ethile, awazi ukuba kuya kuyeka nini. Ngoko ke yimeko eqhubeka kancinci kancinci.

Esi sifo sasibizwa ngokuba yiPrimary Biliary Cirrhosis kuba ekugqibeleni sinokubangela ukuba isibindi sibe lukhuni size sibe namanxeba, nto leyo ekhokelela kwimeko ebizwa ngokuba yi-cirrhosis .

I-(PBC) iyichaphazela njani imizimba yethu?

Khawucinge nje, kwenzeka ntoni ukuba inxalenye yomzimba wethu ihlala ivutha? Ngokwesiqhelo, ukudumba okwethutyana yinxalenye yenkqubo yokuphiliswa. Kodwa ukuba olu hlobo lokudumba okungapheliyo luyaqhubeka, endaweni yokuphiliswa, indawo iqala ukuba namanxeba.

Yiloo nto eyenzekayo kwimibhobho yenyongo engaphakathi kwesibindi sakho kwi-PBC. Le mibhobho iyancitshiswa ize ivalwe zizibazi . Emva koko inyongo eveliswa sisibindi ayikwazi ukuhamba kakuhle. Le nyongo iqala ukubuyela ngaphakathi kwesibindi. Xa le nyongo iqokelelana, yonakalisa iiseli zesibindi kwaye ibangele ukudumba kwisibindi. Ekuhambeni kwexesha, le zicubu zesibindi ezisempilweni zithathelwa indawo yizicubu zesibazi. Ekugqibeleni, isibindi asikwazi ukusebenza kakuhle, nto leyo esiyikhankanyileyo ngaphambili (i-cirrhosis) .

Ingaba i-PBC yimeko enzima?

Ewe, iPrimary Biliary Cholangitis sisifo esingapheliyo nesiqhubekayo . Oku kuthetha ukuba ayipheleli nje ekubeni ngcono, inokuba mandundu ngokuhamba kwexesha. Ayibi mandundu ngaxeshanye, iba mandundu kancinci, ngamanqanaba ahlukeneyo. Usenokungabi nazimpawu ekuqaleni.

Nangona kunjalo, eyona nto iyingozi kakhulu ngale nto kukuba ekugqibeleni inokukhokelela ekusileleni ngokupheleleyo kwesibindi kwanokufa ukuba ukufakelwa isibindi akwenziwanga.

Kodwa, ngethamsanqa, kukho amayeza anokunciphisa ukuqhubela phambili kwesi sifo . Kwaye ayinguye wonke umntu ofikelela kweli nqanaba lokugqibela. Ngoko ke ungakhathazeki.

Yintoni umahluko phakathi kwe (PBC) kunye ne (PSC)?

Usenokuba ukhe weva ngesinye isifo esibizwa ngokuba yiPrimary Sclerosing Cholangitis, okanye iPSC. Ezi zimbini ziyafana kakhulu, kodwa kukho umahluko omncinci obalulekileyo.

• Kwi-PBC , yimijelo yenyongo engaphakathi kwesibindi sakho (i-intrahepatic bile ducts) kuphela echaphazelekayo.
• Nangona kunjalo, kwi-PSC, imibhobho yenyongo engaphakathi kwesibindi kunye nangaphandle kwesibindi (imibhobho engaphandle kwesibindi) iyachaphazeleka .
• Kukwakho umahluko othile kumaqela apho ezi zifo zimbini zixhaphake khona.
• Into ebalulekileyo kukuba, nangona kukho amayeza okunyanga (PBC), akukho nyango luphumeleleyo olufunyenweyo (PSC) .

Ngubani onokuthi abe nesi sifo (PBC)?

Nangona oku kungabonakala kumangalisa, abafazi banamathuba aphindwe kalishumi okufumana isifo sentliziyo kunamadoda (10:1) . Ngokwezibalo eMelika, malunga nama-60 kwabasetyhini abayi-100,000 banesi sifo, xa kuthelekiswa namadoda ayi-15 kwayi-100,000.

Uninzi lwabantu lufunyaniswa lunesifo emva kweminyaka engama-40. Esi sifo sixhaphake kakhulu eScotland, kumazwe aseScandinavia, nakumpuma-mpuma weNgilani.

Kwakhona, ukuba wena okanye umntu kusapho lwakho unesifo esithile sokuzikhusela komzimba, kusenokwenzeka ukuba ube nePBC . Oku kuthetha ukuba kunokubakho unxibelelwano lwemfuza .

Zithini iimpawu zesifo (i-PBC)?

Abantu abaninzi abane-PBC abanazo iimpawu kwasekuqaleni. Kuphela xa isifo siqhubeka apho iimpawu ezinxulumene nemibhobho yenyongo ziqala ukubonakala. Iimpawu zokuqala neziqhelekileyo ezibonwa kubantu abane-PBC zezi:

• Ukudinwa kakhulu (malunga nama-65% abantu)
• Ulusu olurhawuzelelayo (malunga nama-55% abantu)

Ezi mpawu zichaphazela wonke umntu ngokwahlukileyo. Abanye abantu banokuba nazo kwangethuba kwesi sifo, ngelixa abanye banokuba nazo kamva. Zinokuba nzima kakhulu nakweyiphi na inqanaba lesi sifo. Akubonakali ngathi kukho unxibelelwano olukhulu phakathi kokuba iimpawu zakho zinzima kangakanani kunye nokuba isifo siqhubele phambili kangakanani. Nangona kunjalo, uphando oluthile lubonisa ukuba abantu abaneempawu ezinzima kwangethuba banokuba nokukhula ngokukhawuleza kwesi sifo .

Ziziphi iingxaki zezifo ze-bile duct?

Kwinqanaba lokuqala, usenokungaqapheli ukuba unengxaki ngemibhobho yakho yenyongo. Kodwa njengoko isifo siqhubeka, kuvela iingxaki ezahlukeneyo. Inyongo engakwaziyo ukuhamba kakuhle,Iqala ukuvuza egazini, nto leyo ebangela izifo. Kwakhona, xa inyongo ingakwazi ukufikelela kwinkqubo yokugaya ukutya ngokufanelekileyo, ukugaya ukutya kuyaphazamiseka. Kuba inyongo ibalulekile ekugayeni ukutya.

Kamva, ukuvaleka kwesibindi kunokuchaphazela nemithambo yegazi edlula esibindini, nto leyo ekhokelela kwimeko ebizwa ngokuba yi-portal hypertension , nto leyo ethetha uxinzelelo lwegazi oluphezulu kuloo mithambo.

1. Ukungafunxwa kwamafutha

Ukuba inkqubo yakho yokugaya ukutya ayifumani nyongo yaneleyo, ayinakukwazi ukuwohloka kakuhle nokufunxa amafutha ekutyeni kwakho. Oku kuncipha kokufunxwa kwamafutha kunokubangela oku kulandelayo:

• I-cholesterol ephezulu egazini .
• Amafutha agcinwa phantsi kolusu .
• Indle enamafutha okanye isisu esirhudayo .
• Ukuhla ukusinda .
• Ukwehla kwamanqanaba eevithamini ezinyibilikayo amafutha u-A, u-D, u-E, kunye no-K.
• I-Osteoporosis sisifo sokuncitshiswa kwamathambo esibangelwa kukuncipha kokufunxwa kwe-vitamin D enyibilikayo emafutheni.

2. Uxinzelelo lwegazi oluphezulu lwesango

Xa imithambo yegazi edlula esibindini ivalekile ngenxa yamanxeba, kuvela uxinzelelo lwegazi olukhankanyiweyo apha ngasentla, oko kuthetha ukuba uxinzelelo lwegazi kuloo mithambo kunye neminye imithambo edibene nayo luyanda. Olu xinzelelo lwegazi oluphezulu lunokubangela ezi meko zilandelayo:

• Isibindi esikhulu .
• I-spleen ekhulisiweyo .
• Umkhuhlane oqhelekileyo kunye nezinye izifo (ukunciphisa amajoni omzimba).
• Ukukruquka okulula nokopha (iiplatelets zegazi ezisezantsi - (thrombocytopenia) ).
• Ukubonakala kwemithambo yegazi ebomvu efana nesigcawu (spider angiomas) kumphezulu wesikhumba.
• Imithambo evuvukileyo okanye enamaqhina kwi-usophagus nakwisisu (imithambo evuvukileyo - ii-varices) .
• Ukopha kwamathumbu .
• Ukuqokelelana kolwelo esiswini (i-ascites) .
• Ukudumba (ukudumba) komzimba ongezantsi.
• Ukudideka kwengqondo ngamanye amaxesha kwenzeka ngenxa yokuqokelelwa kweetyhefu egazini.

Zithini izizathu ze (PBC)?

Kwi-PBC, inkqubo yakho yokuzikhusela komzimba ihlasela iiseli ezikwimibhobho yenyongo yesibindi sakho . Kungathi inkqubo yokuzikhusela yomzimba ibona ezi seli njengezihlaseli zangaphandle. Oku sikubiza ngokuba sisifo sokuzikhusela komzimba., okanye izifo ezizikhuselayo emzimbeni. Ngaphandle kwesizathu esicacileyo, inkqubo yomzimba yokuzikhusela itshabalalisa iiseli eziphilileyo, kwaye akukho lawulo lokuyithintela. Oku kuqhambuka okuqhubekayo kuko okubangela amanxeba.

Asazi kakuhle ukuba kutheni ezi zifo zokuzikhusela komzimba zikhula. Nangona kunjalo, kubonakala ngathi kukho into ebangela imfuza . Abantu abaninzi abafumana izifo zokuzikhusela komzimba banembali yentsapho yesi sifo, kwaye banokufumana iintlobo ezingaphezu kwesinye zezifo zokuzikhusela komzimba.

Kubonakala ngathi kukho into enxulumene nokusingqongileyo . Oko kukuthi, mhlawumbi into ethile ekwindalo inokubangela esi sifo. Isenokuba yikhemikhali ojongene nayo, okanye isenokuba lusulelo.

Ifunyaniswa njani i-PBC?

Ugqirha wakho uza kuqala akubuze ngembali yakho yezonyango kunye neempawu, enze uvavanyo lomzimba, aze emva koko athathe isampuli yegazi ukujonga i-PBC.

Olu vavanyo lwegazi lujonga ikakhulu ii-antibodies ezithile ezinxulumene ne-PBC, ngakumbi i-antibody ebizwa ngokuba yi -antimitochondrial antibody (AMA) . Lukwajonga nee-enzymes zesibindi eziphezulu, ngakumbi i-alkaline phosphatase (ALP), ezibonisa umonakalo wesibindi.

Ukuba iziphumo zovavanyo lwakho zingqina ukuba une-PBC, ugqirha wakho uya kufuna ukujonga imifanekiso yesibindi sakho kunye nenkqubo ye-biliary . Oku kunokunceda ekususeni ezinye izizathu zeempawu zakho kwaye ubone ukuba isifo sihambe kangakanani. Ngokwesiqhelo, into elula njenge- ultrasound yesisu yenziwa kuqala. Kodwa ngamanye amaxesha, uvavanyo olufana ne -MRI (magnetic resonance imaging) lunokufuneka ukuze kufunyanwe imifanekiso ecacileyo.

Malunga ne-5% yabantu abane-PBC banokuba novavanyo lwe-AMA olubi, kodwa banokuba nezinye iimpawu. Ukuba oku kuyenzeka, ugqirha wakho unokufuna ukuthatha iqhekeza elincinci lesicwili esibindini sakho (i-liver biopsy) ukuqinisekisa ukuba une-PBC. Oku kudla ngokwenziwa ecaleni kwebhedi, ngenaliti efakwe esibindini sakho. Isampuli encinci yesicwili esithathwe kwinaliti ithunyelwa kwilebhu ukuze ihlolwe phantsi kwe-microscope.

Ziziphi iindlela zonyango lwe-Primary Biliary Cholangitis?

Oogqirha basebenzisa oku kulandelayo ukunyanga i-PBC:

Amayeza

I-PBC ayinakunyangwa ngokupheleleyo . Nangona kunjalo, amayeza anokulawula ukuqhubela phambili kwesi sifo aze aphucule imeko yakho .

(I-Ursodeoxycholic acid - UDCA)Luhlobo lwe-bile acid. Inceda ukususa i-bile esibindini kwaye inciphise umonakalo wesibindi. Eli yeza lisebenza kakuhle malunga nesiqingatha sabantu abane-PBC, ingakumbi kwizigaba zokuqala zesifo. Kubantu abangaphenduliyo kwi-UDCA, oogqirha ngamanye amaxesha bacebisa olunye uhlobo lwe-bile acid olubizwa ngokuba yi -obeticholic acid .

Kwakhona, iimpawu zakho zomntu ngamnye zinokunyangwa ngamayeza ahlukeneyo.

• Kwisikhumba esirhawuzelelwayo: ii-antihistamines (umz., i-diphenhydramine - efana neBenadryl®), unyango lokukhanya kwe-ultraviolet, okanye ii-bile acid sequestrants ezifana ne-cholestyramine zinokunikwa.
• Ukuthintela iziphumo ebezingalindelekanga ezifana nokungabikho kweevithamini kunye ne-osteoporosis: Izongezo zevithamini zinokunceda.
• Abanye abantu abadinwayo banokuzuza kwizinto ezivuselelayo ezifana ne-modafinil.

Utyando

Ukuba imeko yakho ayiphucuki ngonyango, kwaye ukusebenza kwesibindi sakho kuqhubeka nokuwohloka, ugqirha wakho angakubeka kuluhlu lokulinda ukufakelwa isibindi . Abantu abane-PBC bafumana iziphumo ezilungileyo kakhulu ngotyando lokufakelwa isibindi.

Njengezinye izifo ezihlasela amasosha omzimba, i-PBC inokuphinda ivele emva kokufakelwa isibindi, kodwa izinga lokuqhubela phambili kwesi sifo lihamba kancinci kakhulu okwesibini. Ubomi obulindelekileyo emva kokufakelwa isibindi buqhelekile.

Yintoni omele uyilindele xa uphila nale meko?

Kwiimeko ezininzi, iPrimary Biliary Cholangitis iqhubeka kancinci . Ukufunyaniswa kwangoko kunye nonyango olukhawulezileyo kunokuthintela okanye ubuncinane kulibazise amanqanaba okugqibela esi sifo kunye neengxaki zaso.

Uninzi lwabantu lunokuzilawula kakuhle iimpawu zabo ngamayeza. Nangona kunjalo, ukunyanga ukudinwa okwenzeka nge-PBC kusenokuba ngumceli mngeni. Abantu abaninzi baphila iminyaka emininzi ngaphandle kokuphazamiseka okukhulu kubomi babo bemihla ngemihla ngenxa ye-PBC.

Nangona kunjalo, ayinguye wonke umntu ofunyaniswa ene-PBC kwisigaba sayo sokuqala. Kwaye abanye abantu bakhula uhlobo olunamandla ngakumbi lwesi sifo. Iimpawu ezifana nokudinwa kakhulu kunye namanqanaba aphezulu e-bilirubin egazini zinokubonisa ukuba esi sifo sinokukhula ngokukhawuleza siye kwisifo sesibindi. Ukuba ufika kwelo nqanaba, kuya kufuneka utyando lwesibindi ukuze usinde. Kodwa kwabo bane-PBC abaye batyando lwesibindi ngempumelelo, ingqikelelo ilungile kakhulu.

Ungaphila ixesha elingakanani ne (PBC)?

Ngokwesiqhelo kuthatha iminyaka eli-15 ukuya kwengama-20 ukuba esi sifo sifikelele kwinqanaba lokugqibela.

Inqanaba lokuqala, oko kuthetha ukuba nokuba uvavanyiwe waza wafunyaniswa une-PBC, usenokuba ungenazimpawu ixesha elide. Malunga nesiqingatha sabantu baza kuqala ukuba neempawu kwiminyaka emihlanu ukuya kwelishumi ezayo.Wakuba ubonakalise iimpawu, umyinge wobomi obulindelekileyo umalunga neminyaka eli-10. Ama-65% abantu abafakelwe isibindi ngempumelelo baphila iminyaka eli-10.

Ndingazinyamekela njani xa ndine (PBC)?

Ukutya ngendlela enempilo kunye nendlela yokuphila kuya kunceda ukugcina isibindi sakho sisempilweni kangangoko kunokwenzeka.

Umzekelo:

• Yeka ukutshaya, ukusela utywala, nokusebenzisa iziyobisi namayeza ngendlela engafanelekanga okanye ngenxa yolonwabo.
• Yitya ukutya okucutshungulweyo nokupakishwe kancinci, kodwa yitya ukutya okupheleleyo.
• Nciphisa amafutha agcweleyo kwaye utye amafutha angenawo agcweleyo asempilweni.
• Hamba-hamba ngokukhawuleza yonke imihla. Ukuzilolonga ngokunyusa ubunzima kunokunceda ukugcina amathambo ephilile.

Abantu abaninzi abane-Primary Biliary Cholangitis banokuphila iminyaka emininzi ngaphandle kweengxaki ezinkulu. Nangona kunjalo, ukuba nesifo esiqhubekayo, ngamanye amaxesha esibulalayo kunokukukhathaza. Thetha nogqirha wakho ngeendlela zokunciphisa uxinzelelo lwakho lomzimba, lwengqondo, nolweemvakalelo . Kwakhona, buza ngeenethiwekhi zenkxaso ezinceda abantu abaphila ne-PBC. Khumbula, impilo yakho iyonke inkulu kakhulu kunesifo nje kuphela.

Umyalezo Wokuya Ekhaya

I-Primary Biliary Cholangitis (PBC) sisifo esingapheliyo esichaphazela imibhobho yenyongo yesibindi. Kukholelwa ukuba ibangelwa yimeko yokuzikhusela komzimba . Nangona iimpawu zisenokungabonakali ekuqaleni, iimpawu ezinje ngokudinwa kunye nokurhawuzelela kolusu zinokuvela ngokuhamba kwexesha. Njengoko isifo siqhubeka , sinokukhokelela kwi-cirrhosis kunye nokungasebenzi kakuhle kwesibindi.

Nangona kunjalo, ngokufunyanwa kwangoko kunye nokusetyenziswa kwamayeza anjenge (UDCA), esi sifo sinokulawulwa . Abanye abantu banokufuna nokufakelwa isibindi.

Eyona nto ibalulekileyo kukufuna iingcebiso zonyango kunye nendlela yokuphila enempilo ukuba unale mpawu. Awuwedwa, ungoyiki ukuthetha ngale nto kwaye ufumane uncedo.

I- Cholangitis yeBiliary ephambili, i-PBC, isifo sesibindi, imibhobho ye-bile, i-cirrhosis, i-autoimmune