Open AccessAsociación entre enfermedad de Crohn y colangitis esclerosante primaria en una niña de 10 años
Author(s) -
Ana Muñoz Lozón,
C. Iglesias Blázquez,
Cristina Menéndez Arias,
Patricia Domínguez Sánchez
Publication year - 2016
Publication title -
archivos argentinos de pediatría
Language(s) - Spanish
Resource type - Journals
SCImago Journal Rank - 0.236
H-Index - 19
eISSN - 1668-3501
pISSN - 0325-0075
DOI - 10.5546/aap.2016.e187
Subject(s) - medicine , gastroenterology , mesalazine , abdominal pain , primary sclerosing cholangitis , inflammatory bowel disease , disease
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.