title: Children undergoing liver transplantation for treatment of inherited metabolic diseases are prone to higher oxidative stress, complement activity and transforming growth factor-β1.
abstract: BACKGROUND
  Main indications for liver transplantation in the pediatric population include biliary atresia and inherited metabolic diseases. The present study evaluated whether there are differences between pediatric patients undergoing living-related liver transplantation due to the two diseases in terms of their oxidative and immunological status during their regular outpatient follow-up visits.
  
  
  METHODS
  A clinical outpatient study measuring serum oxidative stress index (calculated as serum oxidant/antioxidant ratio, in the form of serum total hydroperoxide/serum biological antioxidative potential), serum terminal complement component 5a, as an indicator of complement activity and immunological status, and transforming growth factor-ß1, as a marker of liver fibrosis, in 16 patients (6 males and 10 females, 2.5-15 years old) who received living-related liver transplantation due to inherited metabolic diseases (n=6; in the form of propionic acidemia [n=1], methylmalonic acidemia [n=1], arginase deficiency [n=1], tyrosinemia [n=2], and glycogen storage disease type 1b [n=1], with an age range of 2.4-14.6 years old) and due to biliary atresia ([n=10], with an age range of 2.9-14.5 years old).
  
  
  RESULTS
  Serum oxidative stress index, complement component-5a, and transforming growth factor-ß1 were significantly higher in the inherited metabolic diseases group than in the biliary atresia group. In all patients, serum oxidative stress index correlated positively with complement component-5a and transforming growth factor-ß1.
  
  
  CONCLUSIONS
  Patients who receive living-related liver transplantation due to inherited metabolic diseases are prone to higher oxidative stress, complement activity, and serum transforming growth factor-ß1.
authors.affiliation: Department of Pediatric Surgery, Fujita Health University School of Medicine, Toyoake, Aichi, Japan. drmhamed@yahoo.com
authors.forename: Mohamed Hamed
authors.initials: MH
authors.lastname: Hussein
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suzuki
authors.forename: Ghada Abdel-Hamid
authors.initials: GA
authors.lastname: Daoud
authors.forename: Tatenobu
authors.initials: T
authors.lastname: Goto
authors.forename: Yoko
authors.initials: Y
authors.lastname: Nakajima
authors.forename: Takazumi
authors.initials: T
authors.lastname: Kato
authors.forename: Masahito
authors.initials: M
authors.lastname: Hibi
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Tomishige
authors.forename: Fujio
authors.initials: F
authors.lastname: Hara
authors.forename: Shin
authors.initials: S
authors.lastname: Kato
authors.forename: Hiroki
authors.initials: H
authors.lastname: Kakita
authors.forename: Michi
authors.initials: M
authors.lastname: Kamei
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Ito
authors.forename: Ineko
authors.initials: I
authors.lastname: Kato
authors.forename: Atsushi
authors.initials: A
authors.lastname: Sugioka
authors.forename: Hajime
authors.initials: H
authors.lastname: Togari
mesh_terms: D000293:Adolescent; D001656:Biliary Atresia / Q000097:blood* / Q000276:immunology / Q000601:surgery*; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D015936:Complement C5a / Q000378:metabolism*; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D008661:Metabolism, Inborn Errors / Q000097:blood* / Q000276:immunology / Q000601:surgery*; D018384:Oxidative Stress; D053773:Transforming Growth Factor beta1 / Q000097:blood*
chemical_list: D015415:Biomarkers; D053773:Transforming Growth Factor beta1; D015936:Complement C5a