P013: Glutaric acidemia type 1 (GA-1) in Wisconsin Menominee Nation

The same ROC analysis was applied to Phe and Phe/Tyr ratio values from plasma at the time of confirmation of diagnosis.The Phe/Tyr ratio cut-off in plasma was 4.8 with an efficiency of 95%, sensitivity of 98%, and specificity of 90%.Plasma Phe was similarly effective as a prognostic marker (efficiency=94%; sensitivity=96%; specificity=90%). Conclusion:The NBS Phe/Tyr ratio appears to be a good early predictor of severity of PKU.This can be helpful to facilitate communication with families, determine urgency of follow-up, and guide counseling.