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17 February 2013

Children undergoing liver transplantation for treatment of inherited metabolic diseases are prone to higher oxidative stress, complement activity and transforming growth factor-β1

Mohamed Hamed HusseinABCDEF, Takashi HashimotoABCDEFG, Tatsuya SuzukiAFG, Ghada Abdel-Hamid DaoudCDEF, Tatenobu GotoDEF, Yoko NakajimaDEF, Takazumi KatoBDF, Masahito HibiBDF, Hirokazu TomishigeBDF, Fujio HaraBDFG, Shin KatoDEF, Hiroki KakitaDEF, Michi KameiDEF, Tetsuya ItoCDEF, Ineko KatoACDE, Atsushi SugiokaBDF, Hajime TogariACDEFG

DOI: 10.12659/AOT.883820

Ann Transplant 2013; 18:63-68


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.

MATERIAL AND 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.

Keywords: Free Radicals, Cytokines, Biliary Atresia - immunology

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Annals of Transplantation eISSN: 2329-0358
Annals of Transplantation eISSN: 2329-0358