Autoimmune hepatitis in childhood

master's thesis

• Title (croatian): Autoimunosni hepatitis u djece
• Author: Sadaf Khan
• Mentor: Jurica Vuković (mentor)
• Committee member: Duška Tješić-Drinković (predsjednik povjerenstva)
• Committee member: Marija Jelušić (član povjerenstva)
• Committee member: Jurica Vuković (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Pediatrics); Zagreb
• Defense date and country: 2018-07-13, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Pediatrics

Abstract

Autoimmune hepatitis (AIH) is an inflammatory process, where autoantibodies attack and damage liver cells. Like many autoimmune diseases, aetiology is unknown and it affects females more than males. It is characterised by both the presence of autoantibodies in serum and by high activity hepatic infiltrate pathohistology. There are two types of pediatric AIH identified; type I and type II. The types are differentiated on serological basis mainly: type I is positive for smooth muscle antibody (SMA) and/or antinuclear antibody (ANA); whereas, type II is positive for antibody to liver kidney microsome type 1 (ALKM1). Moreover, type II clinical presentation is more acute, usually at younger age as well as lower levels of IgA. In comparison to type I pediatric AIH, often present at older age/puberty. Nevertheless, the response to treatment and prognosis are comparable in both types. Genetic predisposition based on HLA typing has been found among different ethnicities. The treatment for both types is long- term immunosuppression classically with steroids and azathioprine; the treatment aims to normalise serology and liver histology – remission. Non-classic treatment is reserved for patients that are resistant to classic treatment; i.e. immunosuppression with budesonide, mycophenolate mofetil and cyclosporine. Relapses can occur; complete remission without the need of long-term
immunosuppression is only achieved in 1 in 5 patients. Pediatric AIH has shown a significant association with other autoimmune diseases like autoimmune sclerosing cholangitis, inflammatory bowel disease, autoimmune thyroiditis, psoriasis and rheumatoid arthritis; some share clinical features and are collectively called overlap syndrome.

Abstract (croatian)

Autoimuni hepatitis (AIH) je upalni proces, gdje autoprotutijela napadaju i oštećuju jetrene stanice. Poput mnogih autoimunih bolesti, etiologija je nepoznata, a žene su više zahvaćene nego muškarci. Karakterizira ga prisutnost autoprotutijela u serumu i patohistologija visoke aktivnosti hepatičkog infiltrata. Postoje dvije vrste dječjeg AIH: tip I i tip II. Tipovi se uglavnom razlikuju serološki: tip I je pozitivan za glatko mišićna antitijela (SMA) i/ili antinuklearno antitijelo (ANA); dok je tip II pozitivan za protutijela na mikrosome jetre i bubrega tipa 1 (ALKM1). Štoviše, klinička prezentacija tipa II je akutnija, obično u mlađoj dobi, a IgA je nižih vrijednosti. U usporedbi s tipom I pedijatrijskog AIH, često prisutnog u starijoj dobi/pubertetu. Ipak, odgovor na liječenje i prognoza je usporediva u oba tipa. Genetska predispozicija na temelju HLA tipizacije pronađena je među različitim nacionalnostima. Liječenje oba tipa je dugotrajna imunosupresija, tipično - steroidima i azatioprinom; liječenje nastoji normalizirati serološki nalaz i histologiju jetre, odnosno postići remisiju. Netipičan tretman je rezerviran za bolesnike koji su otporni na tipičan tretman; tj. imunosupresija s budesonidom, mikofenolat mofetilom i ciklosporinom. Može doći do recidiva; potpuna remisija bez potrebe za dugotrajnom imunosupresijom postiže se samo u jednoj od 5 bolesnika. Dječji AIH je pokazao značajnu povezanost s drugim autoimunim bolestima kao što su autoimuni sklerotični kolangitis, upalna bolest crijeva, autoimuni tireoiditis, psorijaza i reumatoidni artritis; neki dijele kliničke osobine i zajednički se nazivaju sindrom preklapanja.

• Keywords: autoimmune hepatitis; immunosuppression; pediatric
• Keywords (croatian): autoimuni hepatitis; imunosupresija; pedijatrija
• Language: english
• URN:NBN: urn:nbn:hr:105:424139
• Study programme: Title: Medicine (in English language); 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: 2019-04-18 08:45:25