Primary sclerosing cholangitis: Izimpawu, Sifo Nokuselapha

Sclerosing cholangitis - isifo Amapayipi isibindi Bile lapho izindonga zawo ukuvuvukala ezingamahlalakhona iqala. Umphumela yomsuka kukhona izinqubo sclerosis m. E. yomiselela nge isibanda izicubu. Lokhu zokugula alinaso ubuhlobo esiyimbangela nezinye izifo zesibindi, kodwa ngokuvamile kuholela ukubonakala ngenxa yezinkinga walo mzimba. Esihlokweni lanamuhla, sizobe uchaze ukuthi kungani uthuthukisa sclerosing cholangitis. Izimpawu kanye nokwelashwa isifo uzophinde wethulwe ukunakwa kwakho.

inkomba yokwakheka

Bile kuyingxenye ebalulekile yenqubo yokugaya ukudla. Yena ehilelekile ukuwohloka kwamafutha, kwandisa umsebenzi enzyme pancreatic kudaleke peristalsis. Bile ngokuqhubekayo ukukhiqiza amaseli isibindi - hepatocytes. Ngelanga ngenxa yomsebenzi wabo olunzulu kutholakala mayelana 1 ilitha uketshezi. Ngemva kwalokho, le Bile ingena esinyeni kanye duodenum.

Outflow uketshezi ihlelwe ngokusebenzisa Amapayipi ekhethekile. Njengoba bengena intrahepatic futhi extrahepatic kuye indawo. Ngenxa yalokho izinqubo amile, Ukungena kwamagciwane noma ezinye izizathu, le Amapayipi ingavuthi avuvukele. Ngesikhathi esifanayo ukukhuluma mayelana nokuthuthukiswa izifo ezifana cholangitis. inqubo sokugembula njalo has a omsakazo ezahlukene. Ngakho-ke, sikude zinhlobo ezilandelayo: anobuthi, igciwane, helminthic, sclerosing. Kugcine kukalutshwana kakhulu, kodwa libhekene ezinzima.

Sclerosing cholangitis ihlukaniswe izinhlobo ezimbili: zamabanga aphansi naphezulu. Ngamunye wabo libhekene isethi ezithile izimpawu kanye Yiqiniso. Esimweni sokuqala kusho isifo esingelapheki ukuthi kuhambisana ukucinana Bile hhayi ubovu ukuvuvukala Amapayipi, ukubhujiswa kwabo futhi esikhundleni nge ezicutshini. Secondary ifomu zokugula eba ngaphansi isenzo ezinobuthi. Ezimweni ezingavamile, lapho ivela khona kubangelwa enganele liningi igazi. Kulesi sihloko sizogxila kabanzi kwi okufana eyinhloko isifo.

Incazelo emfushane isifo

Primary sclerosing cholangitis libhekwa njengoba pathologies ezingavamile kohlelo biliary. Ngokusho idatha kwezibalo, ke sitholakale umuntu oyedwa kwabane ayizinkulungwane 100 labantu ngalinye. Mechanism ukuthuthukiswa isifo kunciphisa ukubukeka ukuvuvukala kule Amapayipi encane hepatic. Ngakho kukhona ukuqina wabo. Kancane kancane kuyendlulela neziteshi sikhubazekile okuyothi Bile agelezela bubble. izinqubo Emgodini onomthombo zisebenze isikhala intercellular kwesibindi, okuholela isifo sokusha.

Ngisho ekupheleni kwekhulu leminyaka elidlule kwakunokwenzeka ukuxilonga izifo kuphela emva kokuhlinzwa noma kwesidumbu. Siyabonga ekuthuthukiseni imithi namuhla ukugula kungatholakala ngisemncane kakhulu. Ngokwezinga elikhulu ukungenwa ke ngabantu abaneminyaka engu-25 kuya kwengu-40. Lezi imingcele ngesimo kakhulu, ngoba isifo kungaba asymptomatic isikhathi eside. Ngezinye izikhathi izimpawu ukuvuvukala aphanjaniswe isifo sokuhlasela kwamasosha omzimba, izilonda emathunjini bese-cystic fibrosis.

Izimbangela cholangitis

Izimbangela ngqo lesi sifo akwaziwa. Odokotela isehlane iqembu izici ezandisa amathuba nesenzakalo yayo. Phakathi kwabo kukhona:

• yofuzo;
• Virus umsebenzi emzimbeni;
• propensity yezifo sokuhlasela kwamasosha omzimba;
• ukuchayeka ezinobuthi.

Phakathi kwalezi zici unikezwa igunya izindlela zofuzo. ubufakazi obuqand 'ikhanda leli qiniso izifundo eziningi zesifo phakathi kwamalungu omkhaya.

emitholampilo isithombe

On the isifo iminyaka eminingi kungenzeka asymptomatic noma nge unezimpawu ezikahle. Iziguli ngokuvamile abakwazi ukusho ngokuthe ngqó lapho kwakubonakala sengathi ekhombisa sclerosing izimpawu cholangitis. Pathology ngokuvamile ukutholwa ithuba ukubona udokotela mayelana nezinye izinkinga zempilo. Ngesikhathi uphethwe wembula lesibonakaliso sokuqala zokugula - anda amanani enzyme hepatic.

Njengoba kuqhubeka isifo futhi lwasemtholampilo izinguquko isithombe. Phakathi izimpawu zaso eyinhloko okulandelayo kumele ezigqanyisiwe:

• ukukhathala, ukozela njalo;
• imnyama;
• yellowing we nolwelwesi lwamafinyila kanye isikhumba;
• nokuvusa lokushisa ukuze subfebrile amagugu;
• ezidabukile ubuhlungu ngakwesokudla endaweni subcostal, asinikeze entanyeni noma emahlombe;
• ukulunywa;
• xanthomas amaningi;
• kabi waya engxenyeni kwesokunxele engenhla, okubangelwa kweNdikimba ubende;
• lesikhumba sithuthukiswe kwesikhumba.

Ngezinye izikhathi, sclerosing eyinhloko cholangitis ezihambisana ukuvuvukala wamathumbu isifo. Lezi zihlanganisa izilonda emathunjini, isifo Crohn.

izindlela ukuxilongwa

Uma usola isifo kufanele ngokushesha ukufuna usizo lukadokotela. Ukuhlola nesineke iqala ekutadisheni yayo umlando, izikhalazo futhi izimpawu eziyinhloko. Ngemva kwalokho, uqhubekele behlolwa. Isiguli singase sibe imihuzuko esikhumbeni, jaundice ezinzima. Palpation ngokuvamile itholakala nokwanda kokungabi isibindi ubende.

Ukuze uqinisekise uphethwe owandulelayo nesineke wathumela ukuhlola okunzulu. Kuhlanganisa imisebenzi elandelayo:

• Ukuhlolwa kwegazi (okweqa izinkomba leukocyte futhi ESR kubonisa ukuvuvukala);
• ultrasound besisu;
• elastography isibindi (ivumela ukulinganisa umzimba kuyaqina);
• cholangiopancreatography retrograde (esimweni usebenzisa Ngokuphambene);
• igazi oyisazi samakhemikhali ezinto eziphilayo (e sclerosing eyinhloko cholangitis waphawula enzyme isibindi ngokweqile);
• MRI;
• Isibindi biopsy (le ndlela yocwaningo kusiza ukuhlonza izindawo cystic).

Lezi zindlela avumele ucwaningo ukuqinisekisa eyinhloko sclerosing cholangitis. Ukuxilongwa yesifo kungasiza ukucacisa kobukhulu inqubo sokugembula. Sekukonke kunezinhlobo ezine:

1. Portal. It libhekene ukubukeka puffiness futhi cystic hepatic mapayipi.
2. Periportal. Izimpawu esigabeni sokuqala kuthiwa kwahambisana ngumuntu cystic evelele nokubhujiswa icubungula mapayipi.
3. Septal. Ngalesi nesigaba lesi sifo kukhona izimpawu kokuqala ngokoma kwesibindi.
4. Cirrhotic. Elinesimo ukuthuthukiswa agcwele isifo sokusha biliary.

Ngokusekelwe imiphumela luhlolo eziyinkimbinkimbi udokotela ekunika yokwelapha.

izinkombandlela ukwelashwa

Ukwelashwa kule isifo kuhloswe zokubopha ukuvuvukala, nokuvulwa Bile ukugeleza futhi ngokukhishwa kobuthi egazini zomzimba. Kuze kube yimanje, izindlela ngoludala futhi op ukwelashwa kusetshenziswa imithi yesimanje. Esimweni sokuqala kusho imithi ukunamathela ukudla okuqinile. Ukuhlinzwa kuboniswa ezimweni ezimbi kakhulu, lapho ukwelashwa ngoludala ehluleka. Ukukhethwa indlela ethile yokwelashwa isesikhathini kudokotela.

Ukusetshenziswa kwezidakamizwa

Kuye kobukhulu isifo, kungaba zabelwe embhedeni ukuphumula futhi ukhiphe amanye umsebenzi. Uma isiguli bakhathazekile sebuhlungwini obunzima, wagunyaza antispasmodics ( "Cha-spa", "Spasmobru").

Ukumisa ukuvuvukala ukusiza izidakamizwa ezilandelayo:

1. Immunosuppressants ( "Azathioprine"). Bona zivimbela umsebenzi wamasosha omzimba.
2. Antifibrogennye kusho. Isenzo sabo esiyinhloko kuhloswe nokuqedwa cystic nokuvinjelwa ukuthuthukiswa kwabo okwengeziwe.
3. i-hormone Glucocorticosteroid ( "Prednisolone"). Asiza ukunciphisa ukuvuvukala.

Ukusetshenziswa lezi imithi evumela ukuba unqobe esigabeni sokuqala ekuthuthukiseni sclerosing eyinhloko cholangitis. Izimpawu zesifo ngokuvamile kuvinjelwe phambili ekuphileni okuvamile iziguli. isikhumba silume, izinkinga ipheshana wokugaya ukudla bese dyspepsia - zonke lezi ukwephula abe nomthelela omubi empilweni yabo. Ngakho-ke ngaphezu kwalokho babelwe yokwelapha lingaze. Kuhlanganisa nokwamukela hepatic ( "Essentiale"), ama-enzyme esiswini ( "Creon") kanye nemithi ukuqeda ukulunywa. Izidakamizwa njalo kukhethwe ngamunye, ikhumbula jikelele isithombe emitholampilo isimo lesiguli.

Izici ukudla

Iziguli babelwa amandla "inombolo etafuleni 5". Uma lokhu Ukudla kufanele kube nomkhawulo ukusetshenziswa okunamafutha, okuthosiwe izitsha okubabayo. Ezilwane Kungcono esikhundleni isitshalo. Ngaphezu kwalokho, kubalulekile ukuba aluqede ngokuphelele kusukela khekhe Ukudla namaswidi, izithelo omuncu kanye amajikijolo, ushokoledi, utshwala, inyama kanye marinades.

Livu pha zisebenzisa mafutha inyama / inhlanzi, ezinye izinhlobo isinkwa, insima phezu kwamanzi. Ungase futhi udle imikhiqizo yobisi, uju, pasta isobho yemifino umhluzi.

Lapho sitholakele "sclerosing eyinhloko cholangitis" ukwelashwa nge imithi nokudla kunikeza yi emihle kuphela esigabeni sokuqala. Uma ngalesi sikhathi is aphuthelwe lokho kuzodinga ukuhlinzwa.

kokungenela kuhlinzwa

zokwelapha okulondoloza elisetshenziswa Umumo ayinzima yenqubo sokugembula. Ngisho ireferensi esifike ngesikhathi udokotela akusho ngaso sonke isikhathi ukunikeza umphumela omuhle ukuziphatha yokwelapha okwalandela. Lapho ngemithi aliholeli ukuzinza isimo noma ehluleka ukubuyisela zamanje ejwayelekile Bile, iphendukele kokungenela kuhlinzwa.

Namuhla odokotela uthanda ukusebenza endoscopic. Basikisela okwenza zonke manipulations ngokusebenzisa sebeziqhumbuza ezincane esikhumbeni. Nokho, lezo zinqubo kukhona ezimweni eziningi ukunikeza umphumela wangaleso sikhathi futhi zigcwele ukuxakeka. Futhi ukuqhuba ibhaluni dilatation futhi stenting mapayipi. Ngesikhathi inqubo, udokotela wenabisa iziteshi amasilinda ekhethekile bese ubeka anezikhala, ukuvimbela nabo kusuka olugcwele. Uma kukhona ukuba umkhankaso sclerosing cholangitis, impatho isibindi ukufakelwa kusho.

yezinkinga kungenzeka

Lesi sifo libhekene ukugeleza kancane. It esabela kahle yokwelapha, kanye nokuchichima kokuthula ukubonakaliswa letindzala eshubisa inqubo kuphela. Phakathi nezinkinga ezivamile zalo yilezi ezilandelayo:

1. nomfutho wegazi ophezulu Portal. Lokhu zokugula kuhambisana ukwanda ukugandeleleka hepatic ukugeleza kwegazi. elisebenza ngayo main kubhekwa ascites.
2. Cholestasis syndrome. Ngaphansi kwesimo ukuqina Amapayipi Bile kancane kancane Taper e zabo cross-kahle. Lokhu kuchaza ukubukeka jaundice kanye pruritus. Njengoba ukuqhubeka mafutha anciphisa isikhala esingaphakathi isifo unciphise ezingasho. Steatorrhea kwenzeka, okuyinto kuhambisana kwamathambo.
3. sclerosing Amagcikwane cholangitis isibindi.
4. pancreatitis Okungalapheki.
5. Holangiokartsionoma (isimila Bile Amapayipi).
6. Cholelithiasis.

yezinkinga ezinjalo zenzeka 3-4 isigaba ohlelweni sokugembula.

Ukubikezela kanye nokuvinjelwa izinyathelo

Primary sclerosing cholangitis libhekwa njengoba kancane ethuthuka isifo. yi lakhe ezimweni eziningi ungongapheliyo yokwehluleka kwesibindi. Kuyaphawuleka isimo sibe sibi Izibikezelo ukuguga esiguli, khona pathologies concomitant emathunjini, okuvela kuyo ukuxakeka. Ngokuvamile, ne ukubonakala izimpawu kokuqala isifo phambi esigabeni sokugcina elisukela iminyaka 7 kuya ku-12.

Kungenzeka yini ukuthi luvimbele eyinhloko sclerosing cholangitis? Uphenyo odokotela bathi ngenxa cwaningo enganele lokuvikela ezithile isifo engakaze athuthukile.