Kodi muli ndi vuto ndi njira zanu zotulutsira ndulu? Tiyeni tiphunzire za Primary Sclerosing Cholangitis (PSC)!

Nthawi zina timamva kutopa popanda chifukwa, timamva kupweteka pang'ono kumtunda kwa dzanja lamanja la m'mimba mwathu, kapena mwina timangomva ngati tikudzikanda tokha. Ngakhale kuti sitisamala kwambiri ndi zinthuzi pa moyo wathu watsiku ndi tsiku, nthawi zina izi zitha kukhala zizindikiro za matenda oopsa kwambiri m'thupi lathu. Lero tikambirana za matenda ovuta pang'ono, koma ndikofunikira kwambiri kuti aliyense adziwe. Ndi matenda a Primary Sclerosing Cholangitis , kapena PSC mwachidule.

Kodi matenda oyamba a sclerosing cholangitis (PSC) ndi chiyani?

Mwachidule, PSC ndi matenda omwe amapezeka m'mitsempha ya ndulu m'thupi lathu. Monga mukukumbukira, ndulu imapangidwa m'chiwindi. Ndudu iyi imathandiza kugaya chakudya. Chifukwa chake, pali njira ya machubu ang'onoang'ono omwe amanyamula ndulu iyi kuchokera ku chiwindi kupita m'matumbo. Timatcha machubu amenewo kuti ndulu.

Mu PSC, pali kutupa kosatha , kapena kutupa, m'mitsempha ya ndulu iyi. Madokotala amatchanso cholangitis . Kutupa kumeneku kukapitirira kwa nthawi yayitali, zipsera zimayamba kupangika m'mitsempha ya ndulu. Zipsera izi zimapangitsa kuti mitsempha ya ndulu ikhale yolimba komanso yopapatiza. Izi zimatchedwa sclerosis .

Tangoganizirani chitoliro cha madzi chomwe chatsekedwa ndi dothi ndipo chikuchepa, zomwe zimapangitsa kuti madzi aziyenda movutikira, sichoncho? Mofananamo, pamene ndulu imachepetsa kuyenda, ndulu imachepa. Izi zimatchedwa biliary stricture . Chifukwa chake, ndulu ikalephera kuyenda bwino, imayamba kubwerera m'chiwindi. Kuchulukana kwa ndulu m'chiwindi (cholestasis) pang'onopang'ono kumawononga chiwindi.

Kodi vuto la PSC ili ndi lalikulu?

Inde, PSC ndi matenda oopsa kwambiri . Mwina simungakhale ndi zizindikiro zilizonse kumayambiriro. Koma ndi matenda omwe amapitirira. Ngati ndulu yatsekedwa mu ducts ya ndulu (cholestasis), poizoni wina mu ndulu amatha kudziunjikira m'magazi. Kenako mumayamba kumva kusasangalala. Komanso, izi zimayambitsa kuwonongeka kosalekeza kwa chiwindi.

Kawirikawiri, kuwonongeka kumeneku kungayambitse kulephera kwa chiwindi kwathunthu patatha zaka pafupifupi 10 mpaka 15. Monga mukudziwa, sitingakhale popanda chiwindi. Ngakhale kuti mankhwala omwe alipo pano amatha kuchepetsa zizindikiro, palibe mankhwala a PSC. Yankho lokhalo ndi kuyika chiwindi .

Kodi pali kusiyana kotani pakati pa cholangitis yoyambirira ndi yachiwiri ya sclerosing?

Mawu oti "choyamba" apa amatanthauza kuti matendawa ndiye chifukwa chachikulu cha kutupa ndi zipsera za ndulu. Izi zikutanthauza kuti vutoli limayamba chifukwa cha matendawa okha, popanda chifukwa china chilichonse.

Koma,Mu vuto lotchedwa secondary sclerosing cholangitis, kutupa (cholangitis) ndi zipsera (sclerosis) za ndulu kumachitika chifukwa cha chifukwa china. Izi zikutanthauza kuti vutoli limayamba chifukwa cha matenda ena kapena chochitika china. Nazi zitsanzo zina:

• Kuvulala kwa ndulu (monga, panthawi ya opaleshoni).
• Matenda m'mitsempha ya ndulu.
• Chithandizo cha khansa (Chemotherapy).
• Miyala ya ndulu yomwe imatseka njira zotulutsira ndulu.
• Pancreatitis yobwerezabwereza.

Kodi kusiyana pakati pa matenda oyamba a biliary cirrhosis (PBC) ndi PSC ndi kotani?

Matendawa, omwe kale ankadziwika kuti Primary Biliary Cirrhosis, tsopano amatchedwa Primary Biliary Cholangitis (PBC) .

Matenda onse a PBC ndi PSC ndi matenda omwe amakhudza chiwindi, omwe amakhudza njira zotulutsira ndulu. Zizindikiro ndi zotsatira zake zonse ndi zofanana kwambiri. Matenda onsewa amachititsa kuti njira zotulutsira ndulu zifooke pang'onopang'ono. Pamene njira zotulutsira ndulu zawonongeka, zipsera, komanso zachepa, kayendedwe ka ndulu kamatsekedwa. Kenako ndulu imabwerera m'chiwindi, zomwe zimawononga chiwindi kwambiri. Izi zingayambitse zipsera pachiwindi komanso matenda a chiwindi .

Koma pali kusiyana kwakukulu pakati pa matenda awiriwa:

• PBC imakhudza ma duct a ndulu mkati mwa chiwindi (intrahepatic). PSC imatha kukhudza ma duct a ndulu mkati mwa chiwindi ndi ma duct a ndulu kunja kwa chiwindi (extrahepatic).
• PBC imapezeka kwambiri mwa akazi (chiŵerengero cha 10:1), pomwe PSC imapezeka kwambiri mwa amuna (chiŵerengero cha 2:1).
• Pafupifupi 80% ya odwala a PSC ali ndi matenda otupa m'mimba (IBD) omwe analipo kale, makamaka ulcerative colitis . Komabe, izi sizikugwirizana kwambiri ndi PBC.
• Pali chiopsezo chachikulu cha khansa ya ndulu ndi PSC. Palibe chiopsezo chachikulu chotere ndi PBC.
• Ursodeoxycholic acid (UDCA) imagwiritsidwa ntchito pochiza matenda a PBC, koma pakadali pano palibe chithandizo chapadera cha PSC.

Kodi vuto limeneli lotchedwa PSC ndi lofala bwanji? Ndani nthawi zambiri amadwala nalo?

PSC ndi matenda osowa kwambiri . Padziko lonse lapansi, akuti munthu m'modzi yekha mwa anthu 10,000 ali ndi matendawa.

Matendawa amapezeka kawiri mwa amuna kuposa akazi. Matendawa nthawi zambiri amapezeka ali ndi zaka pafupifupi 40. Pafupifupi 80% ya anthu omwe ali ndi PSC alinso ndi IBD (Inflammatory Bowel Disease) , makamaka Ulcerative Colitis . Ngati wina m'banjamo ali ndi matendawa, ena ali pachiwopsezo chotenga matendawa.

Kodi n’chiyani chimayambitsa PSC?

Choyambitsa chenicheni cha PSC sichinadziwikebe, koma akuganiziridwa kuti chimayambitsidwa ndi zinthu zosiyanasiyana. Izi zikuphatikizapo:

• Majini
• Zinthu zachilengedwe
• Maselo oteteza thupi ku matenda

Madokotala amaganiza kuti PSC ikhoza kukhala mtundu wa matenda odziteteza okha . Izi zikutanthauza kuti chitetezo cha thupi lathu, asilikali omwe akuyenera kutiteteza ku matenda, molakwika amayamba kuukira maselo athu athanzi. Kutupa ndi chimodzi mwa zida za chitetezo chathu cha mthupi. Nthawi zambiri kumakhala kwakanthawi, komwe kumagwira ntchito motsutsana ndi wolowa m'thupi. Koma ngati kutupa kumakhala kosatha, nthawi zambiri kumakhala chizindikiro cha matenda.

Madokotala awonanso kuti anthu omwe ali ndi PSC ali ndi mwayi waukulu wopeza matenda ena odziteteza ku matenda ena. Mwachitsanzo:

• Matenda otupa m'matumbo
• Matenda a Celiac
• Matenda a chithokomiro
• Matenda a shuga amtundu woyamba
• Matenda a chiwindi odziteteza okha
• Matenda a autoimmune pancreatitis

Akuganiza kuti mtundu uwu wa yankho la autoimmune ungachitike pamene anthu omwe ali ndi majini enaake akumana ndi zinthu zina zachilengedwe (monga kukhudzana ndi mankhwala oopsa).

Kodi zizindikiro za PSC ndi ziti?

Pamene PSC ikupita patsogolo, zizindikiro zimasintha. Pafupifupi 50% ya anthu sangakhale ndi zizindikiro konse matendawa akapezeka. PSC nthawi zambiri imapezeka mwangozi, panthawi yoyezetsa matenda ena. Zizindikiro zoyambirira nthawi zambiri sizimaonekera bwino. Izi zikuphatikizapo:

• Kutopa
• Kupweteka kwa m'mimba kwa gawo lakumanja la chigawo chakumanja
• Khungu loyabwa (pruritus)

Zizindikiro zomwe zingachitike pamene matendawa akupita patsogolo ndi izi:

• Mimba yotupa
• Chiwindi chachikulu
• Nsungu yokulirapo
• Jaundice - Izi zikutanthauza chikasu cha maso ndi khungu.
• Malungo
• Kuchepetsa thupi kosayembekezereka

Kodi mavuto a PSC yoopsa ndi ati?

Matenda oyamba a sclerosing cholangitis ndi matenda omwe amakula pang'onopang'ono, koma pamene ndulu yanu ndi chiwindi chanu zikuwonongeka ndipo ntchito yawo ikuchepa, mavuto osiyanasiyana amatha kuchitika.

Matenda a kagayidwe kachakudya

Ngati njira zanu zotulutsira ndulu zatsekeka kwambiri, sizingathe kupopera ndulu bwino m'matumbo anu ang'onoang'ono. Ndudu ndi yofunika kwambiri pogaya chakudya, makamaka mafuta, komanso poyamwa mavitamini osungunuka mafuta (A, D, E, ndi K). Popanda ndulu, dongosolo lanu logaya chakudya silingathe kukonza mafuta bwino, ndipo silingathe kuyamwa mavitamini ofunikirawa kuchokera ku chakudya. Izi zingayambitse mavuto ena:

• Chimbudzi chonenepa komanso kutsegula m'mimba
• Kusamwa bwino chakudya ndi kusowa zakudya m'thupi
• Kutupa ndi kutuluka magazi mosavuta (chifukwa cha kusowa kwa vitamini K)
• Kufooka kwa mafupa - Osteomalacia ndi osteoporosis (chifukwa cha kusowa kwa vitamini D)
• Kusawona bwino usiku (chifukwa cha kusowa kwa vitamini A)

Kuthamanga kwa magazi kwa portal

Pamene matenda a chiwindi akupita patsogolo, magazi oyenda m'chiwindi amachepa. Izi zimawonjezera kupanikizika mu mtsempha wa portal, womwe umadutsa m'mimba mwanu. Kupanikizika kumeneku kungayambitse mitsempha ina ya m'mero ​​ndi m'mimba kukula ndi kuphulika. Izi zingayambitse kutuluka magazi mkati .

Matenda

Ma ducts a ndulu otsekeka nthawi zambiri amatha kutenga matenda, zomwe zimayambitsa matenda monga malungo, kupweteka m'mimba, komanso poizoni m'magazi (sepsis).

Khansa

Pamene PSC ikukulirakulira, chiopsezo chotenga mitundu ingapo ya khansa chimawonjezeka:

• Khansa ya duct ya bile (cholangiocarcinoma) - Chiwopsezo cha pakati pa 5% ndi 20%.
• Khansa ya ndulu
• Khansa ya chiwindi (hepatoma)
• Khansa ya m'matumbo - ngati muli ndi IBD.

Kodi PSC imapezedwa bwanji?

Kawirikawiri, matendawa amapezeka mwangozi, akamayesa chinthu china. Pafupifupi 50% ya anthu sangakhale ndi zizindikiro zilizonse akapezeka ndi matendawa. Zizindikiro zoyambirira za matendawa zimatha kuwoneka poyesa magazi kapena poyesa kujambula. Kujambula kwa ducts za bile ndi PSC kumawonetsa mawonekedwe apadera. Ngati mayeso a magazi akuwonetsa kuchuluka kwa enzyme yotchedwa alkaline phosphatase , kapena ngati pali ma antibodies ena omwe akuwonetsa chitetezo cha mthupi ku ducts za bile, izi zitha kukhala chizindikiro cha PSC. Kuwonjezeka kwa maselo oyera amagazi nthawi zambiri ndi chizindikiro cha matenda a chiwindi.

Kuti atsimikizire matendawa, dokotala wanu angapereke mayeso ena apadera:

• Kuyesa kwa ntchito ya chiwindi (LFT): Mayeso a magazi awa amafufuza kuchuluka kwa ma enzyme enaake m'chiwindi. Kuchuluka kwa alkaline phosphatase kungasonyeze PSC.
• Magnetic resonance cholangiopancreatography (MRCP) : Iyi ndi MRI (Magnetic Resonance Imaging) . Imatha kujambula zithunzi za biliary system yanu (chiwindi, ndulu, ndi ndulu). Iyi ndi scan yoyamba yodziwira PSC. Izi zili choncho chifukwa siimayambitsa matenda ndipo siimayambitsa kuwala kwa dzuwa. Komabe, nthawi zina, ngati matendawa ali msanga kapena ayi kwambiri, sangathe kuwazindikira molondola. Kenako scan ina ingafunike.

Kodi PSC imachiritsidwa bwanji?

Pakadali pano, palibe chithandizo chomwe chingaletse kapena kusintha kupitirira kwa matenda oyamba a sclerosing cholangitis. Komabe, zizindikiro ndi zovuta zake zitha kuchiritsidwa mwachindunji. Mwachitsanzo, dokotala wanu angakupatseni zinthu monga:

• Mankhwala ochizira kuyabwa pakhungu (pruritus)
• Zakudya zowonjezera zochizira kusowa kwa mavitamini
• Maantibayotiki ochizira matenda

Dokotala wanu adzayang'anira chiwindi chanu ndi ducts za bile. Pamene matendawa akupitirira, nthawi zina angatsegule ducts za bile zotsekedwa. Izi zitha kuchitika kudzera mu mayeso otchedwa ERCP (endoscopic retrograde cholangiopancreatography) . Iyi ndi njira yomwe imakulolani kuwona ducts za bile popanda opaleshoni.

ERCP ndi mtundu wa endoscopy . Imafuna kufufuza ziwalo zanu pogwiritsa ntchito endoscope, chubu chachitali, chosinthasintha chokhala ndi kamera kakang'ono ndikuchiyika. Mukapatsidwa mankhwala oletsa ululu, dokotala wanu adzayika chubu ichi pakhosi panu ndi m'mimba mwanu. Zipangizo zazing'ono zitha kuperekedwa kudzera mu chubuchi kuti zichiritse ndulu yanu. Katswiri angagwiritse ntchito baluni kuti akulitse ndulu yanu, kapena kuyika stent kuti ikhale yotseguka. Ngati ERCP singafikire kutsekeka, njira yotchedwa `percutaneous trans-hepatic cholangiography (PTHC)' nthawi zina imagwiritsidwa ntchito.

Koma iyi ndi njira yothetsera vuto kwakanthawi kochepa. Pakatha zaka 10 mpaka 20, matenda oyamba a sclerosing cholangitis amakula pang'onopang'ono, matenda a chiwindi amakula kwambiri, ndipo pamapeto pake chiwindi chimalephera kugwira ntchito. Dokotala wanu adzayang'anira kuwonongeka kwa chiwindi chanu ndikusankha nthawi yoti muganizire zoyika chiwindi . Muyeneranso kukwaniritsa zofunikira zina kuti mukhale pamndandanda woyembekezera kuyika chiwindi.

Kodi mungakhale ndi moyo wautali bwanji ndi PSC?

Akapezeka ndi matenda, nthawi yoyembekezera kukhala ndi moyo imasiyana pakati pa zaka 10 ndi 20. Kuika chiwindi kungapereke moyo watsopano. Komabe, pa 15% mpaka 20% ya milandu, PSC imatha kubwerera ngakhale ataika chiwindi. Ngati zimenezo zitachitika, chiwindi chatsopanocho chingalepherenso. Pazochitika zotere, nthawi yoyembekezera kukhala ndi moyo imakhala pafupifupi miyezi isanu ndi inayi.

Chinthu china chomwe chingakhudze moyo wa munthu ndi khansa. Ngati mutakhala ndi khansa chifukwa cha PSC, simungakhale woyenera kuikidwa chiwindi. Muzochitika zosankhidwa mosamala, madokotala angayambe amachiza khansayo ndi radiation kapena chemotherapy, kenako n’kuyesa kuikidwa chiwindi.

Kodi ndiyenera kudzisamalira bwanji ngati ndili ndi matendawa?

Kusintha moyo kungathandize kuchepetsa kutopa komwe kumachitika ndi PSC komanso kupewa kuwonongeka kwina kwa chiwindi. Mwachitsanzo:

• Pewani mowa kotheratu.
• Idyani zakudya zochepa zokonzedwa kale koma idyani zakudya zonse.
• Lamulirani kupsinjika maganizo.
• Gonani bwino.
• Chitani masewera olimbitsa thupi tsiku lililonse.

Matenda oyamba a sclerosing cholangitis ndi matenda osowa, osayembekezereka, komanso opewedwa. Amakula pang'onopang'ono, ndipo nthawi zambiri sasonyeza zizindikiro kumayambiriro. Koma ngati mukaonana ndi dokotala nthawi zonse ndikuyesedwa nthawi zonse, matendawa amatha kuzindikirika asanayambe kukhudza moyo wanu. Pamenepo ndi pomwe mungasinthe moyo wanu zomwe zingathandize kuti chiwindi chanu chikhale chathanzi kwa nthawi yayitali. Mudzatumizidwanso kuchipatala kwa nthawi yayitali, zomwe zikutanthauza kuti mudzayezedwe nthawi zonse komanso kuchitapo kanthu ngati pakufunika kutero.

Pomaliza, zinthu zoti muzikumbukira

Chabwino, ndikukhulupirira kuti zomwe takambirana zokhudza PSC zakuthandizani kukumbukira zinthu zina zofunika kwambiri. Ndi nkhani yovuta pang'ono, koma ndikofunikira kwambiri kuidziwa.

• PSC ndi matenda omwe amakhudza ndulu ndipo amakula pakapita nthawi. Amatha kuwononga chiwindi.
• Pakhoza kukhala palibe zizindikiro kumayambiriro, choncho ndikofunikira kupita kuchipatala nthawi zonse.
• Pakadali pano palibe mankhwala a matendawa, koma pali mankhwala oletsa zizindikiro ndikuwongolera moyo wabwino.
• Kuika chiwindi ndi njira yaikulu yothetsera mavuto aakulu.
• Kutsatira moyo wathanzi kumathandiza kuteteza chiwindi.

Ngati muli ndi mafunso ena okhudza PSC, kapena ngati mukuganiza kuti muli ndi zizindikiro izi, onetsetsani kuti mwawonana ndi dokotala kuti akupatseni upangiri . Musadandaule, pali zinthu zomwe mungachite kuti muthane nazo ngati zapezeka msanga.

Cholangitis Yoyamba ya Sclerosing, PSC, Matenda a Mtsempha wa Bile, Matenda a Chiwindi, Jaundice, Ululu wa M'mimba, Cholangitis Yoyamba ya Sclerosing