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Archivos argentinos de pediatría

Print version ISSN 0325-0075On-line version ISSN 1668-3501


MUNOZ LOZON, Ana; IGLESIAS BLAZQUEZ, Cristina; MENENDEZ ARIAS, Cristina  and  DOMINGUEZ SANCHEZ, Patricia. Association between Crohn's disease and primary sclerosing cholangitis in a 10 year old girl. Arch. argent. pediatr. [online]. 2016, vol.114, n.3, pp.e187-e191. ISSN 0325-0075.

A 10 year old girl with diarrhea, abdominal pain, weight loss and fever of one month and a half of evolution. Analytical and sonographic findings raised the possibility of inflammatory bowel disease. Endoscopy and histology showed findings consistent with Crohn's disease. Treatment was initiated with mesalazine and exclusive enteral nutrition. Later corticosteroid treatment, immunosuppressive drugs and ursodeoxycholic acid were added due to cholestasis and persistent hypergammaglobulinemia. Magnetic resonance cholangiography and liver biopsy confirmed the diagnosis of concomitant primary sclerosing cholangitis. The association between Crohn's disease and primary sclerosing cholangitis is rare, predominantly in males between 20 and 40 years old and it presents a great clinical variability. The confirmation of the diagnosis requires magnetic resonance cholangiography or endoscopic retrograde cholangiopancreatography. The prognosis is poor and there is no treatment to slow the progression of the disease.

Keywords : Crohn's disease; Primary sclerosing cholangitis; Inflammatory bowel diseases.

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