Report of 3 Patients With Urea Cycle Defects Treated With Related Living-Donor Liver Transplant

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Ozcay F., Baris Z., Moray G., Haberal N., Torgay A., Haberal M.

EXPERIMENTAL AND CLINICAL TRANSPLANTATION, vol.13, pp.126-130, 2015 (SCI-Expanded)

• Publication Type: Article / Article
• Volume: 13
• Publication Date: 2015
• Doi Number: 10.6002/ect.tdtd2015.p69
• Journal Name: EXPERIMENTAL AND CLINICAL TRANSPLANTATION
• Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
• Page Numbers: pp.126-130
• Eskisehir Osmangazi University Affiliated: No

Abstract

Urea cycle defects are a group of metabolic disorders caused by enzymatic disruption of the urea cycle pathway, transforming nitrogen to urea for excretion from the body. Severe cases present in early infancy with life-threatening metabolic decompensation, and these episodes of hyperammonemia can be fatal or result in permanent neurologic damage. Despite the progress in pharmacologic treatment, long-term survival is poor especially for severe cases. Liver trans plant is an alternative treatment option, providing sufficient enzymatic activity and decreasing the risk of metabolic decompensation.