title: Intrathecal baclofen therapy after liver transplant in a patient with Crigler-Najjar syndrome.
abstract: Crigler-Najjar syndrome (CNS) is described as severe infantile, nonhemolytic, unconjugated hyperbilirubinemia and is divided into type I and type II according to the patient's response to phenobarbital treatment. Patients with type I CNS usually require a liver transplant. These patients often have spasticity and dystonia, both of which can be treated with intrathecal baclofen therapy. We present the case of a patient with CNS type I who underwent a liver transplant followed years later by intrathecal baclofen therapy. To our knowledge, this article provides the first report of a patient with CNS being treated for dystonia with an intrathecal baclofen pump. Despite his complicated history, this patient has remained medically stable after both interventions.
authors.affiliation: Children's Hospital and Research Center, 747 52nd St, Oakland, CA 94609(∗). Electronic address: napala2@hotmail.com.
authors.forename: Napala R
authors.initials: NR
authors.lastname: Pratini
authors.affiliation: Children's Hospital and Research Center, Oakland, CA(†).
authors.forename: Jacob A
authors.initials: JA
authors.lastname: Neufeld
mesh_terms: D001418:Baclofen / Q000008:administration & dosage*; D003414:Crigler-Najjar Syndrome / Q000628:therapy*; D004421:Dystonia / Q000188:drug therapy*; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D009125:Muscle Relaxants, Central / Q000008:administration & dosage*; D055815:Young Adult
chemical_list: D009125:Muscle Relaxants, Central; D001418:Baclofen