title: Acute cholangitis in an old patient with Crigler-Najjar syndrome type II - a case report.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Serviço de Gastrenterologia e Hepatologia, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Avenida Professor Egas Moniz, Lisboa, 1649-035, Portugal. Samuelrmfernandes@gmail.com.|, Avenida Tomás Fonseca n° 36, 13, Lisbon, B 1600-275, Portugal. Samuelrmfernandes@gmail.com.
authors.forename: Samuel Raimundo
authors.initials: SR
authors.lastname: Fernandes
authors.affiliation: Serviço de Gastrenterologia e Hepatologia, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Avenida Professor Egas Moniz, Lisboa, 1649-035, Portugal.
authors.forename: Carlos Miguel
authors.initials: CM
authors.lastname: Moura
authors.affiliation: Serviço de Gastrenterologia e Hepatologia, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Avenida Professor Egas Moniz, Lisboa, 1649-035, Portugal.
authors.forename: Beatriz
authors.initials: B
authors.lastname: Rodrigues
authors.affiliation: Serviço de Gastrenterologia e Hepatologia, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Avenida Professor Egas Moniz, Lisboa, 1649-035, Portugal.
authors.forename: Luís Araújo
authors.initials: LA
authors.lastname: Correia
authors.affiliation: Serviço de Gastrenterologia e Hepatologia, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Avenida Professor Egas Moniz, Lisboa, 1649-035, Portugal.
authors.forename: Helena
authors.initials: H
authors.lastname: Cortez-Pinto
authors.affiliation: Serviço de Gastrenterologia e Hepatologia, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Avenida Professor Egas Moniz, Lisboa, 1649-035, Portugal.
authors.forename: José
authors.initials: J
authors.lastname: Velosa
mesh_terms: D000208:Acute Disease; D000369:Aged, 80 and over; D002760:Cholangiopancreatography, Endoscopic Retrograde; D002761:Cholangitis / Q000150:complications* / Q000175:diagnosis; D017081:Cholecystectomy, Laparoscopic; D002764:Cholecystitis / Q000150:complications* / Q000601:surgery; D002769:Cholelithiasis / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D002779:Cholestasis / Q000150:complications* / Q000175:diagnosis; D002908:Chronic Disease; D003414:Crigler-Najjar Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D014463:Ultrasonography
keywords: Acute cholangitis; Choledocolitiasis; Crigler Najjar; Hyperbilirubinemia; Uridine diphosphate-glucuronosyl-transferase deficiency