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.

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.

• Authors: Mohamed Hamed Hussein, Takashi Hashimoto, Tatsuya Suzuki, Ghada Abdel-Hamid Daoud, Tatenobu Goto, Yoko Nakajima, Takazumi Kato, Masahito Hibi, Hirokazu Tomishige, Fujio Hara, Shin Kato, Hiroki Kakita, Michi Kamei, Tetsuya Ito, Ineko Kato, Atsushi Sugioka, Hajime Togari
• Journal: Annals of transplantation (Ann Transplant) Vol. 18 Pg. 63-8 (Feb 17 2013) ISSN: 2329-0358 [Electronic] United States
• PMID: 23792503 (Publication Type: Journal Article)

Chemical References

• Biomarkers
• Transforming Growth Factor beta1
• Complement C5a

Topics

• Adolescent
• Biliary Atresia (blood, immunology, surgery)
• Biomarkers (blood)
• Child
• Child, Preschool
• Complement C5a (metabolism)
• Female
• Humans
• Liver Transplantation
• Living Donors
• Male
• Metabolism, Inborn Errors (blood, immunology, surgery)
• Oxidative Stress
• Transforming Growth Factor beta1 (blood)