interna medicina 5.8 - Biliiarna ciroza Posledica je...

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Biliiarna ciroza Posledica je dugotrajnih smetnji u oticanju žuči. 1. Primarna biliiarna ciroza Smetnje u oticanju žuči su u intrahepatičnim kanalima. Nastaje usled urođenih i stečenih mana intrahepatičnih kanala. Smatra se da je uzrok autoimuni poremećaj. Patološki proces počinje edemom zida intralobulusnih žučnih puteva i ćelijskim infiltratom. Kasnije dolazi do destrukcije holangiola koje zamenjuje fibrozno tkivo. Smatra se da tek posle 5 godina nastaje promene u jetri koje odgovaraju cirozi. Navedene promene ometaju tok žuči, pa se razvija HOLESTAZA (zastoj žuči). Zbog pritiska na okolne hepatocite, nastaje njihova nekroza, bujanje veziva sa stvaranjem regenerativnih čvorića. Portna hipertenzije javlja se u kasnijoj fazi bolesti. Bolest se javlja obično kod žena između 40. i 60. godine. Bolest traje 5-10 godina i završava se letalno. 2. Sekundarna biliiarna ciroza Smetnje u oticanju žuči nastaju usled promena na ekstrahepatičnim žučnim putevima, usled kamena, tumora, strikture, parazita, upale i tumora glave pankreasa. Klinička slika: postepen razvoj ikterusa progresivnog toka. Žutici obično duže prethodi svrab, koji je stalan simptom. Koža je mrka na kapcima, laktovima, dlanovima, tabanima. Vide se nataloženi holesterolski estri u obliku ploča = KSANTELAZMA. Vrhovi prstiju su maljičasti, a nokti bledi. Zbog slabog lučenja žuči - maldigestija - steatoreja. Hipovitaminoza A, D, E, K. Osteoporoza, krvarenja.
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Laboratoriia: hiperbilirubinemija, povišena alkalna fosfataza (obavezno!!!), povišen holesterol (10-20 puta), povišeni globulini (IgM), albumini su dugo normalni. Antitela protiv glatke muskulature žučnih puteva, antimitohondrijska antitela.
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