Uri kusangana nematambudziko nemaburi emudumbu here? Ngatidzidzei nezvePrimary Sclerosing Cholangitis (PSC)!

Dzimwe nguva tinonzwa kuneta pasina chikonzero, tinorwadziwa zvishoma mudumbu redu, kana kuti pamwe tinongonzwa sekunge tiri kuzvikwenya. Kunyangwe tisinganyatso teerera zvinhu izvi muhupenyu hwedu hwezuva nezuva, dzimwe nguva izvi zvinogona kuva zviratidzo zvechirwere chakakomba mumuviri wedu. Nhasi tichataura nezvechirwere chakaoma zvishoma, asi zvakakosha kuti munhu wese azive. Icho chinonzi Primary Sclerosing Cholangitis , kana kuti PSC muchidimbu.

Chii chinonzi primary sclerosing cholangitis (PSC)?

Zvichitaurwa zviri nyore, PSC chirwere chinoitika mumigero ye bile mumuviri wedu. Sezvamungayeuka, bile rinogadzirwa muchiropa. Bile iri rinobatsira mukugaya. Saka, kune hurongwa hwemachubhu madiki anotakura bile iri kubva kuchiropa kuenda muura. Tinodana machubhu iwayo kuti machubhu e bile.

MuPSC, kune kuzvimba kwenguva refu , kana kuzvimba, mumaburi aya enyongo. Vanachiremba vanodanawo izvi kuti cholangitis . Kana kuzvimba uku kukaramba kuripo kwenguva yakareba, mavanga anotanga kuumbwa mumaburi enyongo. Mavanga aya anoita kuti maburi enyongo ave akaomarara uye akatetepa. Izvi zvinonzi sclerosis .

Fungidzira pombi yemvura yakavharika nevhu uye ichitetepa, zvichiita kuti mvura isafambe zvakanaka, handiti? Saizvozvowo, kana nzira dzenyongori dzikava diki, kufamba kwenyongori kunodzikira. Izvi zvinonzi biliary stricture . Saka, kana nyongori isingakwanise kuyerera zvakanaka, inotanga kudzokera kuchiropa. Kuunganidzwa kwenyongori muchiropa (cholestasis) uku kunokuvadza chiropa zvishoma nezvishoma.

Nyaya iyi yePSC yakakomba here?

Ehe, PSC chirwere chakakomba . Ungasava nezviratidzo pakutanga. Asi chirwere chinoenderera mberi. Kana bile ravharika mumigero yebile (cholestasis), humwe huturu huri mubile hunogona kuungana muropa. Wobva watanga kunzwa kusagadzikana. Izvi zvinokonzerawo kukuvara kwechiropa nguva dzose.

Kazhinji, kukuvara uku kunogona kukonzera kutadza kushanda kwechiropa zvachose mushure memakore angangoita gumi kusvika gumi nemashanu. Sezvamunoziva, hatigone kurarama tisina chiropa. Kunyangwe mishonga iripo ichigona kudzora zviratidzo, hapasati pave nemushonga wePSC. Mhinduro chete ndeyekuisirwa chiropa .

Ndeupi musiyano uripo pakati pechirwere che sclerosing cholangitis chekutanga nechipiri?

Izwi rekuti "primary" pano rinoreva kuti chirwere ichi ndicho chinokonzera kuzvimba uye mavanga emaburi enyongo. Kureva kuti, chirwere ichi chinokonzerwa nechirwere ichi chega, pasina chimwe chikonzero.

Asi,Muchirwere chinonzi secondary sclerosing cholangitis, kuzvimba (cholangitis) uye mavanga (sclerosis) ema ducts e bile kunoitika nekuda kwechimwe chikonzero. Kureva kuti, chirwere ichi chinokonzerwa nechimwe chirwere kana chiitiko. Heano mimwe mienzaniso:

• Kukuvara kwemutsinga dzenyongo (semuenzaniso, panguva yekuvhiyiwa).
• Utachiona mumigero ye bile.
• Kurapa gomarara (Chemotherapy).
• Matombo emunduru anovhara ducts dzenyongo.
• Kurwara kwepancreatitis kunowanzoitika.

Ndeupi musiyano uripo pakati pechirwere chePrimary biliary cirrhosis (PBC) nePSC?

Chirwere ichi, chaimbozivikanwa sePrimary Biliary Cirrhosis, ikozvino chinonzi Primary Biliary Cholangitis (PBC) .

PBC nePSC zvirwere zvinoramba zvichiitika zvinokanganisa chiropa, zvichikanganisa ducts dze bile. Zviratidzo nemigumisiro yazvo zvakafanana zvikuru. Zvirwere zvese izvi zvinoita kuti ducts dze bile dzishaye simba zvishoma nezvishoma. Kana ducts dze bile dzikakuvara, dzikava nemavanga, uye dzikava shoma, kufamba kwe bile kunovharika. Nyoro inobva yadzokera kuchiropa, zvichikuvadza chiropa zvakanyanya. Izvi zvinogona kukonzera mavanga pachiropa uye pakupedzisira cirrhosis yechiropa .

Asi pane misiyano mikuru pakati pezvirwere izvi zviviri:

• PBC inokanganisa chete ducts dze bile mukati mechiropa (intrahepatic). PSC inogona kukanganisa ducts dze bile mukati mechiropa neducts dze bile kunze kwechiropa (extrahepatic).
• PBC inowanikwa zvakanyanya muvakadzi (10:1 ratio), nepo PSC inowanikwa zvakanyanya muvarume (2:1 ratio).
• Vanenge 80% yevarwere vePSC vane chirwere chemudumbu chakagara chiripo (IBD) , kunyanya chirwere chemudumbu chinonzi ulcerative colitis . Zvisinei, izvi hazvina hukama hwakakura nePBC.
• Pane njodzi yakakura yekenza yemudumbu uchishandisa PSC. Hapana njodzi yakakura kudaro nePBC.
• Ursodeoxycholic acid (UDCA) inoshandiswa kurapa PBC, asi parizvino hapana mushonga chaiwo wePSC.

Chirwere ichi chinonzi PSC chakajairika zvakadii? Ndiani anowanzobatwa nacho?

PSC chirwere chisingawanzoitiki . Pasi rose, zvinofungidzirwa kuti munhu mumwe chete pavanhu zviuru gumi ndiye ane chirwere ichi.

Chirwere ichi chakajairika kaviri muvarume pane vakadzi. Chirwere ichi chinowanzoonekwa pazera remakore makumi mana. Vanhu vanosvika 80% vane PSC vanewo IBD (Inflammatory Bowel Disease) , kunyanya Ulcerative Colitis . Kana mumwe munhu mumhuri aine chirwere ichi, vamwe vari panjodzi yekuchibatawo.

Chii chinokonzera PSC?

Chikonzero chaicho chePSC hachisati chazivikanwa, asi chinofungidzirwa kuti chinokonzerwa nezvinhu zvakasiyana-siyana. Izvi zvinosanganisira:

• Majini
• Zvinhu zvakatipoteredza
• Masero ekudzivirira muviri

Vanachiremba vanofunga kuti PSC ingave rudzi rwechirwere che autoimmune . Izvi zvinoreva kuti masoja anofanirwa kutidzivirira kubva kuzvirwere, anotanga kurwisa masero edu ane hutano. Kuzvimba chimwe chezvombo zve immune system yedu. Kazhinji ndezvenguva pfupi, zvichirwisa muvengi. Asi kana kuzvimba kukava kwenguva refu, kazhinji chiratidzo chechirwere.

Vanachiremba vakaonawo kuti vanhu vane PSC vane mukana wakakura wekuwana zvimwe zvirwere zve autoimmune. Semuenzaniso:

• Zvirwere zvematumbu zvinozvimba
• Chirwere cheCeliac
• Chirwere chethyroid
• Chirwere cheshuga cherudzi rwekutanga
• Chiropa chinozvirwisa mumuviri
• Kuzvirwisa kwemuviri pancreatitis

Zvinofungidzirwa kuti rudzi urwu rwemhinduro yekuzvidzivirira kwemuviri runogona kuitika kana vanhu vane mamwe maitiro emajini vakasangana nezvimwe zvinhu zvezvakatipoteredza (semuenzaniso, kusangana nemakemikari ane chepfu).

Ndezvipi zviratidzo zvePSC?

Sezvo PSC ichifambira mberi, zviratidzo zvinochinja. Vanhu vanosvika 50% vanogona kunge vasina zviratidzo zvachose kana chirwere ichi chaonekwa. PSC inowanzoonekwa netsaona, panguva yekuongororwa kwechimwe chirwere. Zviratidzo zvepakutanga hazviwanzoonekwa zvakanyanya. Zvinosanganisira:

• Kuneta
• Marwadzo emudumbu echikamu chepamusoro cherudyi
• Ganda rinokwenya (pruritus)

Zviratidzo zvinogona kuitika kana chirwere chichikura zvinosanganisira:

• Dumbu rakazvimba
• Chiropa chakakura
• Chirwatata chakakura
• Jaundice - Izvi zvinoreva kuti machena emaziso neganda anotsvukira.
• Kupindwa nechando
• Kuderedza uremu kusingatarisirwi

Ndezvipi zvinetso zvePSC yakanyanya?

Chirwere chePrimary sclerosing cholangitis chirwere chinokura zvishoma nezvishoma, asi sezvo ducts dze bile nechiropa zvinova nemavanga uye mashandiro azvo achiderera, matambudziko akasiyana-siyana anogona kuitika.

Zvirwere zvemuviri

Kana nzira dzemudumbu wako dzakavharika zvakanyanya, hadzigone kupomba bile zvakanaka muura hwako hudiki. Bile rinokosha pakugaya chikafu, kunyanya mafuta, uye pakutora mavhitamini anonyunguduka mumafuta (A, D, E, naK). Pasina bile, kugaya kwako hakugone kugadzirisa mafuta zvakanaka, uye hakugone kutora mavhitamini aya akakosha kubva muchikafu. Izvi zvinogona kukonzera mamwe matambudziko:

• Zvituru zvine mafuta uye manyoka
• Kusanyoroveswa kwezvinwiwa mumuviri uye kushaya chikafu chakakwana
• Kutsvuka uye kubuda ropa zviri nyore (nekuda kwekushomeka kwevhitamini K)
• Kushaya simba kwemapfupa - Osteomalacia uye osteoporosis (nekuda kwekushaikwa kwevhitamini D)
• Kusaona zvakanaka usiku (nekuda kwekushomeka kwevhitamini A)

Kukwira kweropa kwakanyanya pa portal

Sezvo cirrhosis ichienderera mberi, kuyerera kweropa muchiropa kunoderera. Izvi zvinowedzera kumanikidzwa kwetsinga yemudumbu, iyo inofamba nemudumbu. Kumanikidzwa uku kunogona kukonzera kuti dzimwe tsinga dziri mugurokuro nemudumbu dzikure uye dziputike. Izvi zvinogona kukonzera kubuda ropa mukati memuviri .

Utachiona

Mapaipi enyongo akavharika anogona kutapukirwa, zvichikonzera zvirwere zvakaita sefivha, kurwadziwa mudumbu, uye chepfu muropa (sepsis).

Kenza

Sezvo PSC ichiwedzera kuipa, njodzi yekubatwa nekenza yemhando dzakasiyana inowedzera:

• Gomarara remudumbu (cholangiocarcinoma) - Njodzi iri pakati pe5% ne20%.
• Kenza yenyongo
• Kenza yechiropa (hepatoma)
• Kenza yemudumbu - kana uinewo IBD.

PSC inoonekwa sei?

Kazhinji, chirwere ichi chinowanikwa netsaona, kana munhu achiongororwa chimwe chinhu. Vanhu vanosvika 50% vangasava nezviratidzo kana chirwere ichi chaonekwa. Zviratidzo zvekutanga zvechirwere zvinogona kuonekwa nekuongororwa kweropa kana kuongororwa kwemifananidzo. Kuongororwa kwenzira dzemudumbu dzine PSC kunoratidza zvinhu zvakakosha. Kana kuongororwa kweropa kuchiratidza huwandu hwakawanda hwe enzyme inonzi alkaline phosphatase , kana paine mamwe ma antibodies anoratidza mhinduro yekudzivirira muviri kuma ducts emudumbu, zvinogona kuva chiratidzo chePSC. Kuwedzera kwemasero machena eropa kunowanzova chiratidzo chehutachiona hwechiropa.

Kuti asimbise chirwere ichi, chiremba wako anogona kukupa mimwe miedzo yakati wandei:

• Kuongororwa kwebasa rechiropa (LFT): Kuongororwa kweropa uku kunotsvaga huwandu hwakakwira hwema enzymes chaiwo muchiropa. Kuwanda kwe alkaline phosphatase kunogona kuratidza PSC.
• Magnetic resonance cholangiopancreatography (MRCP) : Iyi iMRI (Magnetic Resonance Imaging) scan. Inogona kutora mifananidzo yakadzama ye biliary system yako (chiropa, gallbladder, uye bile ducts). Iyi ndiyo scan yekutanga yekuongorora PSC. Izvi zvinokonzerwa nekuti haipindi uye haisanganisire kuratidzwa nemwaranzi. Zvisinei, dzimwe nguva, kana chirwere ichi chikangotanga kana kuti chisina kunyanya kuipa, chingasakwanise kuchiona nemazvo. Zvadaro imwe scan ingangodiwa.

PSC inorapwa sei?

Parizvino, hapana mushonga unogona kumisa kana kudzora kufambira mberi kwechirwere cheprimary sclerosing cholangitis. Zvisinei, zviratidzo nematambudziko zvinogona kurapwa zvakananga. Semuenzaniso, chiremba wako anogona kukunyorera zvinhu zvakaita se:

• Mishonga yekurapa ganda rinokwenya (pruritus)
• Mishonga yekurapa kushomeka kwemavhitamini
• Mishonga inorwisa mabhakitiriya yekurapa hutachiona

Chiremba wako acharamba achinyatsoongorora chiropa chako nemidziyo yemudumbu. Sezvo chirwere ichi chichienderera mberi, dzimwe nguva vanogona kuvhura duct yemudumbu yakavharika. Izvi zvinogona kuitwa kuburikidza nebvunzo inonzi ERCP (endoscopic retrograde cholangiopancreatography) . Iyi inzira inokubvumira kuona mukati memidziyo yemudumbu yako pasina kuvhiyiwa.

ERCP rudzi rwe endoscopy . Inosanganisira kuongorora nhengo dzako uchishandisa endoscope, chubhu refu, inochinjika ine kamera diki uye chiedza pairi. Mushure mekunge warapwa, chiremba wako achaisa chubhu iyi pasi pehuro pako uye mudumbu mako. Zvishandiso zvidiki zvinogona kupfuudzwa nepachubhu iyi kurapa ducts dze bile. Nyanzvi inogona kushandisa bharumu kuwedzera ducts dze bile, kana kuisa stent kuti irambe yakavhurika. Kana ERCP isingakwanise kusvika pakuvharika, dzimwe nguva nzira inonzi `percutaneous trans-hepatic cholangiography (PTHC)' inoshandiswa.

Asi iyi ingori mhinduro yenguva pfupi. Mukati memakore gumi kusvika makumi maviri, chirwere chepfungwa chinonzi primary sclerosing cholangitis chinowedzera kuipa, chirwere chechiropa chinowedzera kuipa, uye pakupedzisira chiropa chinokundikana kushanda zvakanaka. Chiremba wako achaongorora kukuvara kwechiropa chako uye achasarudza kuti ungafunga riini nezvekuisirwa chiropa . Unofanirawo kuzadzisa zvimwe zvinodiwa kuti uve pane runyorwa rwevanhu vakamirira kuisirwa chiropa.

Unogona kugara kwenguva yakareba sei nePSC?

Kana munhu aonekwa kuti ane chirwere ichi, avhareji yehupenyu hwake inosiyana pakati pemakore gumi kusvika makumi maviri. Kuisirwa chiropa kunogona kupa nguva itsva yekurarama. Zvisinei, mu15% kusvika 20% yezviitiko, PSC inogona kudzoka kunyangwe mushure mekuisirwa chiropa. Kana izvozvo zvikaitika, chiropa chitsva chinogonawo kutadza kushanda. Muzviitiko zvakadaro, avhareji yehupenyu hunotarisirwa imwedzi mipfumbamwe.

Chimwe chinhu chinogona kukanganisa makore ekurarama igomarara. Kana ukaita kenza sedambudziko rePSC, ungasave munhu akanaka wekuisirwa chiropa. Muzviitiko zvakasarudzwa zvakanaka, vanachiremba vanogona kutanga varapa kenza nemwaranzi kana chemotherapy, vozoedza kuisirwa chiropa.

Ndinofanira kuzvitarisira sei kana ndine chirwere ichi?

Kuchinja mararamiro kunogona kubatsira kudzora kuneta kunowanzoitika nePSC uye kudzivirira kukuvara kwechiropa. Semuenzaniso:

• Dzivisa doro zvachose.
• Idya chikafu chisina kugadzirwa zvakanyanya asi idya chikafu chakazara.
• Dzora kushushikana.
• Rara zvakanaka.
• Ita maekisesaizi zuva rega rega.

Chirwere chePrimary sclerosing cholangitis chirwere chisingawanzoitiki, chisingafungidzike, uye chinogona kudzivirirwa. Chinokura zvishoma nezvishoma, uye kazhinji hachina zviratidzo pakutanga. Asi kana ukaona chiremba wako nguva dzose uye ukaongororwa nguva dzose, chirwere ichi chinogona kuonekwa chisati chatanga kukanganisa hupenyu hwako. Ndipo paunokwanisa kuchinja mararamiro ako izvo zvinogona kubatsira kuchengetedza chiropa chako chine hutano kwenguva yakareba. Uchatumirwawo kunorapwa kwenguva refu, zvinoreva kuongororwa nguva dzose uye kupindira kana zvichidikanwa.

Pakupedzisira, zvinhu zvekurangarira

Zvakanaka, saka ndinovimba zvatataura nezvazvo nezvePSC zvakubatsira kurangarira zvimwe zvezvinhu zvakakosha. Mamiriro acho akaoma zvishoma, asi zvakakosha kuti uzvizive.

• PSC chirwere chinokanganisa ducts dze bile uye chinotowedzera kuipa nekufamba kwenguva. Chinogona kukuvadza chiropa.
• Panogona kunge pasina zviratidzo pakutanga, saka zvakakosha kugara uchiongororwa nachiremba.
• Parizvino hapana mushonga wechirwere ichi, asi kune mishonga yekudzora zviratidzo uye kuvandudza hupenyu.
• Kuisirwa chiropa ndiyo mhinduro huru muzviitiko zvakakomba.
• Kutevedzera mararamiro ane hutano kunobatsira kuchengetedza chiropa.

Kana uine mimwe mibvunzo nezvePSC, kana kuti uchifunga kuti une chimwe chezviratidzo izvi, iva nechokwadi chekuona chiremba kuti akupe mazano . Usazvidya mwoyo, pane zvaunogona kuita kuti uzvigadzirise kana zvikaonekwa nekukurumidza.

` Kuomarara Kweganda Kwekutanga, PSC, Chirwere Chemudumbu Mudumbu, Chirwere Chechiropa, Jaundice, Marwadzo Emudumbu, Kuomarara Kweganda Kwekutanga