کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10834111 | 1065857 | 2005 | 4 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Management and outcome of neonatal-onset ornithine transcarbamylase deficiency following liver transplantation at 60 days of life
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: Management and outcome of neonatal-onset ornithine transcarbamylase deficiency following liver transplantation at 60 days of life Management and outcome of neonatal-onset ornithine transcarbamylase deficiency following liver transplantation at 60 days of life](/preview/png/10834111.png)
چکیده انگلیسی
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.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Molecular Genetics and Metabolism - Volume 84, Issue 4, April 2005, Pages 363-366
Journal: Molecular Genetics and Metabolism - Volume 84, Issue 4, April 2005, Pages 363-366
نویسندگان
Regina Ensenauer, Mendel Tuchman, Mounif El-Youssef, Suresh Kotagal, Michael B. Ishitani, Dietrich Matern, Dusica Babovic-Vuksanovic,