Two consecutive partial liver transplants in a patient with Classic Maple Syrup Urine Disease☆☆☆
Maple syrup urine disease is caused by a deficiency in the branched chain ketoacid dehydrogenase (BCKAD) complex. This results in the accumulation of branched chain amino acids (BCAA) and branched chain ketoacids in the body. Even when aggressively treated with dietary restriction of BCAA, patients...
| Main Authors: | , , , , , , |
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| Format: | Online |
| Language: | English |
| Published: |
Elsevier
2015
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| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4750575/ |