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Open Access 01-12-2016 | Case report

Acute cholangitis in an old patient with Crigler-Najjar syndrome type II - a case report

Authors: Samuel Raimundo Fernandes, Carlos Miguel Moura, Beatriz Rodrigues, Luís Araújo Correia, Helena Cortez-Pinto, José Velosa

Published in: BMC Gastroenterology | Issue 1/2016

Abstract

Background

Crigler-Najjar syndrome (CN) is a very rare genetic disorder characterized by an inability to conjugate bilirubin. Contrary to CN type I, patients with CN II exhibit residual capacity to conjugate bilirubin and may present a normal life expectancy.

Case presentation

We report an unusual late diagnosis of CN type II in an 80-year-old female admitted with severe acute cholangitis. While the patient present typical clinical and radiologic signs of bile duct obstruction and cholangitis, her blood analysis showed severe unconjugated hyperbilirubinemia. Endoscopic retrograde cholangiopancreatography confirmed the diagnosis and allowed therapeutic intervention. The anatomopathologic examination of her gallbladder following cholecystectomy showed signs of chronic cholecystitis.

Conclusion

The risk of gallstone disease may be increased in patients with CN syndrome. While unusual, we alert to this curious and potential life-threatening presentation.

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Title: Acute cholangitis in an old patient with Crigler-Najjar syndrome type II - a case report
Authors: Samuel Raimundo Fernandes
Carlos Miguel Moura
Beatriz Rodrigues
Luís Araújo Correia
Helena Cortez-Pinto
José Velosa
Publication date: 01-12-2016
Publisher: BioMed Central
Published in: BMC Gastroenterology / Issue 1/2016
Electronic ISSN: 1471-230X
DOI: https://doi.org/10.1186/s12876-016-0449-9

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