title: Total knee arthroplasty and Crigler-Najjar syndrome: a case report.
abstract: Crigler-Najjar (CN) syndrome is a rare genetic disease characterized by hyperbilirubinemia due to a deficiency in the hepatic enzyme UDP-glucuronosyl-transferase. We describe the first case of total knee arthroplasty in a patient with CN syndrome (type II). This procedure was complicated by kernicterus 1 week after hospital discharge. He also developed Klebsiella bacteremia and sepsis, requiring a brief ICU stay. He was discharged in good condition 2 months later. It is evident that physicians involved in the care of patients with CN syndrome in the peri-operative period need to have a high index of suspicion for the development of severe hyperbilirubinemia and kernicterus in order to appropriately manage and, possibly, prevent this complication. A literature review and intra-operative observations provide insight into the possible relationship between hyperbilirubinemia and osteoarthritis as well as the peri-operative considerations to be made for this group of patients.
authors.affiliation: Division of Orthopedic Surgery, Department of Surgery, University of Toronto, Toronto Western Hospital, Toronto, ON, Canada.
authors.forename: David
authors.initials: D
authors.lastname: Walmsley
authors.forename: Khalid
authors.initials: K
authors.lastname: Alzaharani
authors.forename: William J
authors.initials: WJ
authors.lastname: Coke
authors.forename: Rajiv
authors.initials: R
authors.lastname: Gandhi
mesh_terms: D019645:Arthroplasty, Replacement, Knee / Q000009:adverse effects*; D003414:Crigler-Najjar Syndrome / Q000150:complications*; D006801:Humans; D007647:Kernicterus / Q000209:etiology* / Q000628:therapy; D007710:Klebsiella Infections / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D010003:Osteoarthritis / Q000209:etiology / Q000601:surgery*; D054850:Osteophyte / Q000209:etiology / Q000601:surgery; D010951:Plasma Exchange; D020127:Recovery of Function; D012772:Shock, Septic / Q000150:complications / Q000209:etiology* / Q000382:microbiology