Upale žučnih vodova u djece liječene zbog bilijarne atrezije

master's thesis

• Title (english): Cholangitis in children treated for biliary atresia
• Author: Ana-Marija Novak
• Mentor: Tomislav Luetić (mentor)
• Committee member: Jurica Vuković (predsjednik povjerenstva)
• Committee member: Tomislav Meštrović (član povjerenstva)
• Committee member: Tomislav Luetić (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Surgery); Zagreb
• Defense date and country: 2019-07-12, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Surgery
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Internal Medicine

Abstract

Bilijarna atrezija rijetka je bolest neutvrđene etiologije koju karakterizira postojanje neprohodnih žučnih vodova, a može se javiti zasebno ili uz poremećaje drugih organskih sustava. Najčešće se manifestira u novorođenačkoj ili dojenačkoj dobi pojavom žutice, aholičnih stolica i tamnog urina, što upućuje na opstrukciju koja onemogućava fiziološki protok žuči u crijevo. U kasno prepoznatih slučajeva mogu biti prisutni znakovi uznapredovalog oštećenja jetre poput ascitesa, venskih varikoziteta i portalne encefalopatije. Kao primarni terapijski zahvat u većine oboljele djece izvodi se HPE po Kasaiju pri kojoj se anastomoziranjem slobodne vijuge tankog crijeva i portalnog dijela jetre formira put za protok žuči te se u slučaju uspješnog zahvata postiže nestanak znakova kolestaze. Unatoč ovoj operaciji, najvećem je broju oboljelih nakon određenog vremena ipak potrebna transplantacija jetre. Najčešća postoperativna komplikacija, kao i jedan od najznačajnijih faktora koji utječu na ishod bolesti nakon HPE, jest upala žučnih vodova ili kolangitis, za kojeg se smatra da nastaje kombinacijom više čimbenika koji pogoduju translokaciji i perzistenciji crijevnih bakterija u bilijarnom stablu. Ranija pojava i češće ponavljanje epizoda upale povezani su sa bržim propadanjem nativne jetre i ranijom potrebom za transplantacijom. U prevenciji kolangitisa koriste se različite kombinacije i režimi davanja antibiotika djelotvornih protiv najčešće izoliranih uzročnika, a primjenjuju se i kortikosteroidi zbog protuupalnih svojstava i povoljnog utjecaja na protok žuči te ursodeoksikolična kiselina koja također pokazuje koleretski efekt. Liječenje akutne upale temelji se na intravenskoj primjeni antibiotika širokog spektra uz eventualnu upotrebu intravenskih imunoglobulina.

Abstract (english)

Biliary atresia is a rare disease of undetermined etiology, characterized by the presence of impenetrable bile ducts, which may be found separately or joined with disruptions of other organ systems. It is most commonly presented in the newborn or infants by appearance of jaundice, acholic stools and dark urine, suggestive of an obstruction which disables the physiological flow of bile into the intestine. In tardy recognized cases, signs of advanced liver damage can be detected, such as ascites, venous varices and portal encephalopathy. In most patients Kasai HPE is performed as a primary intervention. By creating an anastomosis between an isolated intestinal loop and the liver hilum, a pathway allowing the flow of bile is established, which, in case of success, leads to disappearance of signs of cholestasis. In spite of the operation, the majority of patients eventually require a liver transplant. The most common postoperative complication, as well as one of the most significant factors affecting the outcome after HPE, is the inflammation of bile ducts or cholangitis, believed to develop as a consequence of multiple factors favoring translocation of bacteria from the intestine and their persistence in the biliary tree. Earlier and more common episodes of inflammation are associated with a more rapid native liver deterioration and an earlier need for transplantation. Various combinations and administration regimes of antibiotics effective towards most commonly isolated causative bacteria are used in order to prevent cholangitis. Additionally, corticosteroids are administered due to their anti-inflammatory nature and positive effect on bile flow, and ursodeoxycholic acid shows a choleretic effect as well. The treatment of acute inflammation is based on intravenous administration of wide-range antibiotics and possibly intravenous immunoglobulins.

• Keywords: bilijarna atrezija; hepatoportoenterostomija; kolangitis
• Keywords (english): biliary atresia; hepatoportoenterostomy; cholangitis
• Language: croatian
• URN:NBN: urn:nbn:hr:105:681049
• 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: 2020-03-06 09:56:02