title: Outcomes of Liver Transplantation During Adulthood After Kasai Portoenterostomy Due to Biliary Atresia.
abstract: Biliary atresia (BA) is a neonatal progressive cholangiopathy of unknown etiology and one of the most common reasons for liver transplantation (LT) in children. Kasai portoenterostomy (KP) improves survival of the native liver, although LT remains the only ultimate treatment. In some cases KP makes it possible to defer the ultimate LT until adulthood. We report our experience regarding 5 cases of BA treated with LT during adulthood. KP was performed in all patients at an average age of 176 days (range, 60-280), which allowed an average survival of the native liver of 19.01 years (range, 14.06-22.32). Five-year survival rate was 100%. Ten-year survival rate did not reach 100% because of a death 9.55 years after LT due to chronic graft rejection, in a patient who was already prepared for a new LT. Our results corroborate that KP remains the first-line treatment of BA. Early performance of the KP provides children with the best chance of survival, allowing the delay of the LT to adulthood. LT during adulthood in these patients achieves good post-LT survival rate; we have not found any data regarding this group of patients in the literature.
authors.affiliation: General and Digestive Surgery, Son Espases University Hospital, Palma de Mallorca, Spain; General and Digestive Surgery, Virgen del Rocío University Hospital, Seville, Spain. Electronic address: segusamjj@gmail.com.
authors.forename: J J
authors.initials: JJ
authors.lastname: Segura-Sampedro
authors.affiliation: General and Digestive Surgery, Virgen del Rocío University Hospital, Seville, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Bernal-Bellido
authors.affiliation: General and Digestive Surgery, Virgen del Rocío University Hospital, Seville, Spain.
authors.forename: L M
authors.initials: LM
authors.lastname: Marín-Gómez
authors.affiliation: General and Digestive Surgery, Virgen del Rocío University Hospital, Seville, Spain.
authors.forename: G
authors.initials: G
authors.lastname: Suárez-Artacho
authors.affiliation: General and Digestive Surgery, Virgen del Rocío University Hospital, Seville, Spain.
authors.forename: J
authors.initials: J
authors.lastname: Serrano-Díez-Canedo
authors.affiliation: General and Digestive Surgery, Virgen del Rocío University Hospital, Seville, Spain.
authors.forename: J M
authors.initials: JM
authors.lastname: Álamo-Martínez
authors.affiliation: General and Digestive Surgery, Virgen del Rocío University Hospital, Seville, Spain.
authors.forename: F J
authors.initials: FJ
authors.lastname: Padillo-Ruiz
authors.affiliation: General and Digestive Surgery, Virgen del Rocío University Hospital, Seville, Spain.
authors.forename: M Á
authors.initials: MÁ
authors.lastname: Gómez-Bravo
mesh_terms: D000293:Adolescent; D000328:Adult; D001656:Biliary Atresia / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000601:surgery; D016031:Liver Transplantation / Q000379:methods / Q000401:mortality*; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000009:adverse effects*; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult