UOC di Pediatria e Neonatologia, Ospedale di Ravenna, AUSL della Romagna
Indirizzo per corrispondenza: email@example.com
Key words: Inflammatory bowel disease, Sclerosing cholangitis, Children, Case report
A picture of the onset of “hepatocolangitis” especially if with dilatation of the biliary tract must necessarily make one think of primary sclerosing cholangitis (PSC). The suspicion of PSC must always make one think of concomitant chronic inflammatory bowel disease (IBD) even if asymptomatic or paucisymptomatic. In fact, in 70-90% of PSCs it is associated with IBD, generally with an ulcerative rectocolitis phenotype. In these cases the dosage of faecal calprotectin can be an indicator of suspicion in case of positivity. In the same way, in a child/adolescent diagnosed with IBD (especially if they present with URC), transaminases and especially GGTs should always be monitored because one in 10 cases may have a concomitant PSC picture during their lifetime. Sometimes PSC can overlap clinical, biochemical and histological features with autoimmune hepatitis and configure a framework known as autoimmune sclerosing cholangitis (ASC). The prognosis of PSC is more benign in the child than in the adult (although burdened with complications), but the diagnosis (cholangio-MRI and liver biopsy), as well as the treatment (with ursodeoxycholic acid and immunosuppressants), must be timely. The paper reports the case of a 9-year-old child with an initial diagnosis of infectious hepatocolangitis which turned out to be a primary sclerosing cholangitis, with an associated picture of ulcerative rectocolitis little expressed from clinical and biopsy points of view.
Vuoi citare questo contributo?