Outcome of early and long-term management of classical maple syrup urine disease.

@article{Clow1981OutcomeOE,
  title={Outcome of early and long-term management of classical maple syrup urine disease.},
  author={Carol L. Clow and Theresa M Reade and Charles R. Scriver},
  journal={Pediatrics},
  year={1981},
  volume={68 6},
  pages={856-62}
}
The outcome of 8,400 treatment days in the lives of four patients with classical maple syrup urine disease (MSUD) (present ages: 1 3/12, 5 7/12, and 8 11/12 years) are described. Each diagnosis was made by clinical signs rather than by newborn screening. Acute-phase treatment beginning on the 11th day of life comprised peritoneal dialysis, intravenous lipid, and early intestinal alimentation. Mean age at discharge from hospital was 29 days. There were 16 readmissions to the hospital for the… CONTINUE READING

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