title: Liver biopsy complicated by hemorrhage in a patient with ARC syndrome.
abstract: ARC syndrome is a rare disorder consisting of arthrogryposis, renal tubular acidosis and cholestatic liver disease. We report the case of a 5-week-old patient who underwent a percutaneous liver biopsy complicated by hemorrhage, and was subsequently diagnosed with ARC syndrome. A review of the literature demonstrates that these patients are at increased risk of bleeding caused by platelet dysfunction. The evaluation and management of unexpected hemorrhage in pediatric patients as a result of undiagnosed congenital bleeding problems is discussed.
authors.affiliation: Department of Anesthesia, Hospital for Sick Children, University Avenue, Toronto, Ontario, Canada. jason.hayes@sickkids.ca
authors.forename: Jason A
authors.initials: JA
authors.lastname: Hayes
authors.forename: Walter H A
authors.initials: WH
authors.lastname: Kahr
authors.forename: Bryan
authors.initials: B
authors.lastname: Lo
authors.forename: Bruce A
authors.initials: BA
authors.lastname: Macpherson
mesh_terms: D000015:Abnormalities, Multiple; D000141:Acidosis, Renal Tubular / Q000150:complications* / Q000175:diagnosis; D058186:Acute Kidney Injury / Q000150:complications; D001176:Arthrogryposis / Q000150:complications* / Q000175:diagnosis; D001706:Biopsy / Q000009:adverse effects / Q000379:methods; D001778:Blood Coagulation Disorders / Q000150:complications; D001803:Blood Transfusion / Q000379:methods; D001919:Bradycardia / Q000188:drug therapy / Q000209:etiology; D001993:Bronchodilator Agents / Q000627:therapeutic use; D002780:Cholestasis, Intrahepatic / Q000150:complications* / Q000175:diagnosis; D003937:Diagnosis, Differential; D004837:Epinephrine / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006470:Hemorrhage / Q000150:complications*; D006801:Humans; D007022:Hypotension / Q000188:drug therapy / Q000209:etiology; D007223:Infant; D008099:Liver / Q000473:pathology*; D013577:Syndrome
chemical_list: D001993:Bronchodilator Agents; D004837:Epinephrine