Primary sclerosing cholangitis: Iimpawu, diagnosis and Treatment

Sclerosing cholangitis - esigulisa imibhobho zesibindi bile duct apho kweendonga zawo nokudumba ezingapheliyo iqala. Isiphumo imvelaphi yayo neenkqubo sclerosis, m. E. Ukuthathwa kunye amabala kwithishyu. Le ngezifo akanalo ulwalamano kobude kunye nezinye izifo zesibindi, kodwa kukhokelela ukubonakala iingxaki kulo mzimba. Kwinqaku namhlanje, siza kuchaza isizathu sokuba asakhasayo sclerosing cholangitis. Iimpawu kunye nonyango esi sifo uya kuthiwa thaca ukuze bakuthathele ingqalelo.

reference yokwakheka

Bile wenkqubo yinxenye ebalulekileyo kwinkqubo yokwetyisa. Yena obandakanyeka yokuwa amafutha, kwandisa umsebenzi enqanda pancreatic kudaleke peristalsis. Bile iyaqhubeka ukuvelisa iiseli zesibindi - hepatocytes. Ngosuku ngenxa yomsebenzi wabo kakhulu ifunyenwe malunga 1 ilitha ulwelo. Emva koko, lo inyongo engena esinyini kunye duodenum.

Ephuma secretion eziququzelelwe ngokusebenzisa imibhobho ezizodwa. Njengoko beza intrahepatic kunye extrahepatic kuxhomekeka indawo. Ngenxa yeenkqubo elingakhuliyo, umthondo iintsholongwane okanye ezinye izizathu, ukuba imibhobho libe ebushushwini babo. Ngelo xesha uthethe kuphuhliso izifo ezifana cholangitis. inkqubo yezifo usoloko a umyalezo eyahlukileyo. Ngoko ke, oko uyazimela iintlobo zilandelayo: ezingenabungozi, yintsholongwane, helminthic, sclerosing. Last inqabile kakhulu, kodwa lubonakala kakhulu.

cholangitis Sclerosing yahlulwe iintlobo ezimbini: eziziprayimari nezizisekondari. Ngamnye kubo iphawulwa iseti ethile iimpawu kunye khosi. Kwimeko yokuqala oko kuthetha ukuba isifo esinganyangekiyo into ihamba kwingxinano ngayo ibile hayi ubovu imibhobho nangokurhala, intshabalalo yabo ozothatha kwizicwili. ifomu Secondary ngezifo kuphuhliswa phantsi isenzo izinto eziyityhefu. Kwiimeko ezinqabe kakhulu, livela kubangelwa ubonelelo igazi elanele. Kweli nqaku liza siya kugxininisa ngakumbi phezu uhlobo iphambili sifo.

Inkcazelo emfutshane ye sifo

Primary sclerosing cholangitis zihlelwe ngolu ezimbi ezinqabileyo kwinkqubo biliary. Ngokutsho kwedatha yeenkcukacha-manani, oko banesifo omnye kwabane nganye 100 amawaka abantu. Indlela yophuhliso sifo kunciphisa imbonakalo ukudumba kwi imibhobho ezincinane hepatic. Ngenxa yoko kukho ukuqaqadeka kwabo. Ngcembe zilala phezu nemijelo likhubazeke obuthi inyongo lungena ngokukhululekileyo kwiqamza. iinkqubo amile isicelo kwisithuba intercellular kwesibindi, nto leyo ukuqina.

Nokuba ekupheleni kwenkulungwane yokugqibela kwaba lula ukufumanisa isifo kuphela emva utyando okanye yotyando. Ngenxa kuphuhliso amayeza namhlanje isigulo kungabonwa kakhulu ngaphambili. Kangangoko enkulu basengozini kuyo abantu abaneminyaka eyi-25 ukuya kuma-40. Le mida ngesimo kakhulu, ngenxa yokuba esi sifo kuba nazimpawu ixesha elide. Ngamanye amaxesha iimpawu ukudumba iphutha ukuba isifo samathambo, sisilonda sikabhobhosi kunye abanesigulo esinganyangekiyo.

Izinto ezibangela cholangitis

Iimbangi ngqo kwesi sifo ayaziwa. Oogqirha bodwa iqela izinto ezandisa amathuba okuba ukwenzeka kwayo. Phakathi kwazo kukho ezi:

• yofuzo;
• umsebenzi ntsholongwane emzimbeni;
• wayevuma kwizigulo sokuhlasela;
• Ukwazi izinto eziyityhefu.

Phakathi kwezi meko zinikwa ingqwalaselo iindlela yemfuza. bubungqina obubonakalayo oku izifundo ezininzi sifo phakathi kwamalungu osapho.

picture clinical

On lesi sifo iminyaka emininzi inokuba nazimpawu okanye uneempawu eziphakathi. Izigulane amaninzi akakwazi kuthi ncam xa wabonakala ebonisa sclerosing iimpawu cholangitis. Pathology idla ukubonwa ithuba ukubona ugqirha malunga nezinye iingxaki zempilo. Ngexesha kwaveza lowokuqala umqondiso lokugula - amazinga ukwanda enqanda hepatic.

Njengoko kangakanani isifo kunye notshintsho umfanekiso lwezonyango. Phakathi iimpawu zayo eziphambili kwezi zinto zilandelayo kufuneka ziphawuliwe:

• ukudinwa, ukozela rhoqo;
• imnyama;
• yellowing ye kwiinwebu nesikhumba;
• ukuphakamisa iqondo lobushushu ukuze subfebrile amanani;
• nokuqaqamba iintlungu kwindawo ekunene subcostal, uqhebeke entanyeni okanye negxalaba;
• ukulunywa;
• xanthomas multiple;
• kabi kwi ikota yesangqa yasekhohlo ephezulu, ngenxa wawandisa udakada;
• nombala lokuwandisa ulusu.

Ngamanye amaxesha, cholangitis sclerosing zaseprayimari ezihamba isifo wamathumbu arhabaxa. Ezi ziquka okwenziwa sisilonda sikabhobhosi, isifo Crohn kaThixo.

iindlela isifo

Ukuba urhanela isifo kufuneka ngoko nangoko afune uncedo evela kugqirha. Uviwo lwe isigulane luqala ukufunda imbali yayo, izikhalazo kunye neempawu zaseprayimari. Emva koko, dlulela nokuxilongwa kwemizimba yabo. Isigulane ukuba imikrwelo kakhulu eluswini, senyongo kakhulu. Ngokucofa idla wafumana ukwanda wesibindi udakada.

Ukuze uqinisekise uxilongo lokuqala isigulane wathumela uviwo olunye. Oku kuquka le misebenzi ilandelayo:

• uvavanyo lwegazi (elingaphaya izalathi si zokunikwa leukocytes kunye ESR ibonisa nangokurhala);
• ultrasound esiswini;
• elastography kwesibindi (ivumela ukuba luthambe umzimba ukuqikelela);
• cholangiopancreatography retrograde (ray usebenzisa umahluko);
• eziphilayo igazi (in cholangitis sclerosing zaseprayimari waphawula enzyme zesibindi kakhulu);
• MRI;
• Isibindi biopsy (le ndlela yophando kunceda ukuchonga iinkalo abanesigulo).

Ezi ndlela sivumele uphando ukuqinisekisa cholangitis sclerosing zaseprayimari. Lokufunyaniswa kwesifo kwakhona kunceda ukufumanisa ubuzaza inkqubo yezifo. Xa iyonke kukho ezine:

1. Portal. Lubonakala kukuvela sivakale sidumbile kunye abanesigulo hepatic imibhobho.
2. Periportal. Iimpawu kweqonga wokuqala zincediswe a abanesigulo ucaca ngakumbi kwaye nentshabalalo kwiinkqubo imibhobho.
3. Septal. Kweli nqanaba sifo kukho iimpawu zokuqala ngokoma.
4. Cirrhotic. Lubonakala ngokuba uphuhliso ngokupheleleyo-epheleleyo kwesibindi biliary.

Ngokusekelwe kwiziphumo zeemviwo ezimbaxa ugqirha imisela unyango.

Izikhokelo zonyango

Therapy kule sifo ijonge nokunqanda inkqubo kwiintetho ezikralayo, nokuhlawulwa kwemali duct ezigulela ngaphandle komzimba. Ukuza kuthi ga ngoku, iindlela emithethweni futhi ukusebenza zonyango ezisetyenziswa yesilungu. Kwimeko yokuqala kuthetha amayeza kunye nokubambelela ukutya engqongqo. Surgery iboniswe kwiimeko kakhulu, xa unyango elondoloza lutshitshe. Ukukhetha indlela ethile kunyango ukuya kwagqirha.

Ukusetyenziswa kweziyobisi

Kuxhomekeka ubungqongqo sifo, oko eyabelwe alale kunye bucala nayiphi na umthambo. Ukuba isigulane ochaphazelekayo malunga iintlungu eziqatha, azimiselweyo antispasmodics ( "No-Spa", "Spasmobru").

Ukumisa inkqubo yokukrala ukunceda amachiza zilandelayo:

1. Immunosuppressants ( "Azathioprine"). Bona zinqanda umsebenzi kwamajoni omzimba.
2. Antifibrogennye ithetha. isenzo zazo eziphambili ijolise ekupheliseni abanesigulo, nokuthintelwa uphuhliso lwabo ngakumbi.
3. hormone Glucocorticosteroid ( "Prednisolone"). Zinceda ukunciphisa komtshiso oko.

Ukusetyenziswa kwezi amayeza kuvumela ukoyisa lokuqala uphuhliso cholangitis sclerosing ephambili. Iimpawu zesi sifo kwenza ubomi eliphambili eqhelekileyo kwizigulane. isikhumba sakho silume, iingxaki wokugaya kunye ukuqunjelwa - zonke ezi iintshukumo zokwaphulwa kwemithetho kuba nefuthe elibi empilweni yabo. Ngoko ke isebe owabelwe unyango nomqondiso. Oku kuquka ukufumana hepatic ( "Essentiale"), enzayim esiswini ( "Creon") kunye amayeza ukuphelisa ukurhawuzelela. Iziyobisi basoloko ezikhethiweyo ngumfundi, kuthathelwa ingqalelo epheleleyo zekliniki imeko yesigulane.

Iimpawu nokudla

Izigulane ziye zabelwa amandla "inombolo itheyibhile 5". Xa ukudla kufuneka ngcathu okunamafutha, okroqiweyo, kunye izitya zinongiwe. amafutha ezilwanyana Kukhethwa ukuba indawo isityalo. Ngaphezu koko, kuyimfuneko ukuba ecaleni ngokupheleleyo ukususela khekhe idayethi iilekese, iziqhamo ezimuncu kunye namaqunube, itshokolethi, utywala, inyama kunye marinades.

Imvume nikunikiwe ukusebenzisa inyama ndayame / intlanzi, ezinye iintlobo zezonka, isidudu phezu kwamanzi. Unako kwakhona ukutya iimveliso zobisi, nobusi, isuphu pasta umhluzi yemifuno.

Xa lokufunyaniswa unyango "zaseprayimari sclerosing cholangitis" ngamayeza nedayethi unika iziphumo ezidibanisayo kuphela isanda. Ukuba eli xesha oziphosileyo, kuya kufuneka uqhaqho.

ukungenelela kuhlinzwa

amayeza Conservative ezisetyenziswa kwiimilo lula yenkqubo yezifo. Nokuba ireferensi ngexesha ugqirha ayisoloko ukunika iziphumo ezidibanisayo ekuqhutyweni unyango olulandelayo. Xa yonyango ngamachiza ingakhokeleli kuqheleke imeko okanye uyasilela ukubuyisela ekhoyo okuqhelekileyo inyongo, ukubhenela ungenelelo yotyando.

Namhlanje oogqirha bakhetha imisebenzi endoscopic. Bacebise ngokwenza zonke zazo ngokusebenzisa ukusikwa ezincinane esikhumbeni. Nangona kunjalo, iinkqubo ezinjalo kwiimeko ezininzi ukunika isiphumo elifutshane elide yaye izaliswe iingxaki. Kwakhona ukuqhuba ibhaloni dilatation kunye imigudu stenting. Ngexesha inkqubo, ugqirha u imijelo cylinder ezizodwa kwaye icwangcisa umnatha, babavalela valiweyo. Ukuba kukho liza kuphehlelelwa sclerosing cholangitis, unyango isibindi ithetha.

iingxaki kunokwenzeka

Isifo luphawulwa ukuhamba kade. It iphendula kakuhle kuthomalaliso, nobuninzi ezibonisa lwenkqubo aggravates kuphela le nkqubo. Phakathi iingxaki zixhaphakileyo kwayo zezi zilandelayo:

1. BP Portal. Le ngezifo ihamba ukwanda koxinzelelo ukuhamba kwegazi hepatic. main nokubonakalaliswa kwaLo uthathwa ascites.
2. Cholestasis syndrome. Sizoku lwisana yokuqaqadeka le bile duct ngokuthe Taper in cross-kakuhle zabo. Oku kucacisa yimbonakalo senyongo kunye pruritus. Njengoko ukuqhubeka sayo sifo linciphise ngakumbi nangakumbi. Steatorrhea livela, nto leyo sikhatshwa kwamathambo.
3. sclerosing yebhaktiriya cholangitis esibindini.
4. pancreatitis Chronic.
5. Holangiokartsionoma (ithumba inyongo imibhobho).
6. Cholelithiasis.

iingxaki ezinjalo kwenzeka 3-4 nqanaba lenkqubo yezifo.

Ukuqikelela zothintelo amanyathelo

Primary sclerosing cholangitis zihlelwe ngolu ngokucothayo phambili izifo. ngenxa yakhe kwiimeko kakhulu ezingapheliyo sisibindi. Ngokuphawulekayo zimbi ALS ukuguga yomguli, ubukho ezimbi ehamba emathunjini, kukuvela iingxaki. Ngokuqhelekileyo, kunye ukuvela kweempawu okuqala izifo phambi kweqonga lokugqibela isusela kwiminyaka esi-7 ukuya kwi-12.

Ngaba kunokwenzeka ukuba ukuthintela cholangitis sclerosing primary? Izimvo oogqirha bathi ngenxa ukufunda zaneleyo yothintelo ezithile izifo sele iphuhlisiwe hayi.