title: Improved neurologic prognosis for a patient with propionic acidemia who received early living donor liver transplantation.
abstract: Despite medical therapy, patients with propionic academia (PA) still display a tendency to develop epilepsy. Patients with neonatal-onset PA who have received early living donor liver transplantation (LDLT) are limited in number, and the effect on neurologic prognosis, including epilepsy, is not clear. We report a patient with PA whose EEG findings improved dramatically after undergoing LDLT at age 7 months. The patient's neurologic development and brain MRI findings were quite satisfactory at age 2 years and 3 months. LDLT is effective not only in preventing metabolic decompensation, but also in improving neurologic function to ensure better quality of life.
authors.affiliation: Department of Pediatrics, National Hospital Organization, Hokkaido Medical Center, Sapporo, Japan. nagaom@hok-mc.hosp.go.jp
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Nagao
authors.forename: Toju
authors.initials: T
authors.lastname: Tanaka
authors.forename: Mayuko
authors.initials: M
authors.lastname: Morii
authors.forename: Shuji
authors.initials: S
authors.lastname: Wakai
authors.forename: Reiko
authors.initials: R
authors.lastname: Horikawa
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
mesh_terms: D004827:Epilepsy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation; D019520:Living Donors; D008279:Magnetic Resonance Imaging; D011379:Prognosis; D056693:Propionic Acidemia / Q000503:physiopathology / Q000601:surgery*