Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10834111 | Molecular Genetics and Metabolism | 2005 | 4 Pages |
Abstract
Neonatal hyperammonemia secondary to X-linked ornithine transcarbamylase (OTC) deficiency carries a high risk of morbidity and mortality. Results of medical therapy are less than satisfactory. Experience with liver transplantation in very young affected infants is limited. We report a male newborn with severe OTC deficiency who underwent successful orthotopic, cadaveric liver transplantation at the age of 60 days. Although technically challenging in the neonatal period, liver transplantation should be considered early as the most promising treatment approach currently available.
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Authors
Regina Ensenauer, Mendel Tuchman, Mounif El-Youssef, Suresh Kotagal, Michael B. Ishitani, Dietrich Matern, Dusica Babovic-Vuksanovic,