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Case Reports
. 2016 Mar 11:16:33.
doi: 10.1186/s12876-016-0449-9.

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

Samuel Raimundo Fernandes  1   2 , Carlos Miguel Moura  3 , Beatriz Rodrigues  3 , Luís Araújo Correia  3 , Helena Cortez-Pinto  3 , José Velosa  3
Affiliations
Case Reports

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

Samuel Raimundo Fernandes et al. BMC Gastroenterol. .

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.

Keywords: Acute cholangitis; Choledocolitiasis; Crigler Najjar; Hyperbilirubinemia; Uridine diphosphate-glucuronosyl-transferase deficiency.

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Figures

Fig. 1
Fig. 1
Abdominal ultrasound showing gallbladder wall thickening, pericholecystic fluid and cholelithiasis (a). The common bile duct is dilated (b)
Fig. 2
Fig. 2
Endoscopic retrograde cholangiopancreatography showing multiple stones in the gallbladder (arrow)
See this image and copyright information in PMC

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