Kolestatske bolesti jetre i njihove komplikacije

master's thesis

• Title (english): Cholestatic liver diseases and their complications
• Author: Vedrana Vlahović
• Mentor: Lucija Virović Jukić (mentor)
• Committee member: Davor Hrabar (predsjednik povjerenstva)
• Committee member: Jasenka Markeljević (član povjerenstva)
• Committee member: Lucija Virović Jukić (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Internal Medicine); Zagreb
• Defense date and country: 2019-07-12, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Internal Medicine

Abstract

Primarne kolestatske bolesti jetre predstavljaju rijetke bolesti koje zahvaćaju jetrene
stanice, izvan jetrene (ekstrahepatalne) ili unutar jetrene (intrahepatalne) žučne
vodove, a dovode do smetnji u stvaranju ili otjecanju žuči. Primarni bilijarni kolangitis
ili primarna bilijarna ciroza (PBC), kako se do nedavno bolest zvala, jedan je od
uzroka unutar jetrene kolestaze. Progresivno oštećenje malih žučnih vodova unutar
jetre može dovesti do ciroze i zatajenja jetrene funkcije. Primarni sklerozirajući
kolangitis (PSC) može uzrokovati unutar i izvan jetrenu kolestazu, ovisno o tome koje
žučne kanale zahvati upalni proces. Obilježava ga kronična upala praćena
oštećenjem i fibrozom žučnih vodova s posljedičnom segmentalnom obliteracijom
lumena. U obje bolesti pacijenti se mogu prezentirati svrbežom i žuticom kože, a od
laboratorijskih nalaza povišenim vrijednostima alkalne fosfataze (ALP) i gamaglutamil
transpeptidaze (GGT). PBC ima i povišena antimitohondrijska protutijela, dok
se PSC dokazuje kolangiografijom magnetskom rezonancijom. Komplikacije bolesti
mogu biti posljedica kolestaze, upalnog (autoimunog) zbivanja te ciroze jetre ili
razvoja tumora. Ursodeoksikolna kiselina je terapija izbora za PBC, jer usporava tijek
bolesti, dok obetikolna kiselina predstavlja drugi izbor. Za PSC ne postoji dokazano
djelotvorna medikamentoza terapija, ali se primjenjuje endoskopsko i operativno
liječenje suženja žučnih vodova. Komplikacije dugotrajne kolestaze u vidu
malapsorpcije i svrbeža zahtijevaju simptomatsko liječenje, a svrbež je ponekad
tvrdokoran na terapiju. Transplatacija jetre predstavlja metodu izbora u završnom
stadiju obje bolesti.

Abstract (english)

Primary cholestatic liver diseases represent rare diseases which affect liver cells
(hepatocytes), outside liver (extra-hepatic) or inside liver (intra-hepatic) bile ducts and
lead to disturbance in production or flow of bile. Primary biliary cholangitis or primary
biliary cirrhosis (PBC), how the disease was called until recently, is one of the causes
of liver cholestasis. Progressive damage to small bile ducts inside the liver can lead
to cirrhosis and liver function failure. Primary sclerosing cholangitis (PSC) can cause
both intra-hepatic and extra-hepatic damage, depending on which bile ducts
inflammatory process affects. It is characterized by chronic inflammation followed by
fibrosis of the bile ducts with consequent segmental obliteration of the lumina. In both
diseases, patients may present with itch and skin jaundice, and out of laboratory
findings with elevated alkaline phosphatase (ALP) and gamma-glutamyl transferase
(GGT). In PBC, anti-mitochondrial antibodies are also elevated, while the PSC is
determined by magnetic resonance cholangiopancreatography. Diseases can be
complicated by development of cholestasis, inflammatory (autoimmune) events, liver
cirrhosis or tumor development. Due to its ability to slow down the course of the
disease, ursodeoxycholic acid is therapy of choice for PBC, while obeticholic acid is
the second line treatment. There is no proven effective drug therapy for the PSC, but
endoscopic and operative treatment are used. Complications of long-term cholestasis
in the form of malabsorption and itching require symptomatic treatment, although
itching is sometimes difficult to treat successfully.
Liver transplantation is the method of choice in the final stage of both diseases.

• Keywords: kolestatske bolesti jetre; primarni bilijarni kolangitis; primarni sklerozirajući kolangitis
• Keywords (english): cholestatic liver diseases; primary biliary cholangitis; primary sclerosing cholangitis
• Language: croatian
• URN:NBN: urn:nbn:hr:105:338353
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2019-10-18 07:24:01