[HTML][HTML] Elective liver transplantation for the treatment of classical maple syrup urine disease
KA Strauss, GV Mazariegos, R Sindhi, R Squires… - American journal of …, 2006 - Elsevier
An 8.5-year-old girl with classical maple syrup urine disease (MSUD) required liver
transplantation for hypervitaminosis A and was effectively cured of MSUD over an 8-year
clinical follow-up period. We developed a collaborative multidisciplinary effort to evaluate
the effects of elective liver transplantation in 10 additional children (age range 1.9–20.5
years) with classical MSUD. Patients were transplanted with whole cadaveric livers under a
protocol designed to optimize safe pre-and post-transplant management of MSUD. All …
transplantation for hypervitaminosis A and was effectively cured of MSUD over an 8-year
clinical follow-up period. We developed a collaborative multidisciplinary effort to evaluate
the effects of elective liver transplantation in 10 additional children (age range 1.9–20.5
years) with classical MSUD. Patients were transplanted with whole cadaveric livers under a
protocol designed to optimize safe pre-and post-transplant management of MSUD. All …
