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Phenylalanine hydroxylase deficiency in children
- Monitoring and evaluating the impact of the guideline.
The following are three performance measures or indicators for implementing this adapted CPG for PKU in children:
1. Adherence to PKU Guidelines
▪️ Numerator: Number of children with PAH deficiency who received treatment as per guideline recommendations.
▪️ Denominator: Total number of children diagnosed with PAH deficiency.
▪️ Data Source: Metabolic outpatients/ clinic patient records.
2. Neurocognitive outcome
▪️ Numerator: Total number of children with PAH deficiency with average IQ and neurocognitive functions.
▪️ Denominator: Total number of children diagnosed with PAH deficeinc.
▪️ Data Source: Metabolic outpatients/ clinic patient records..
These key performance indicators are designed to measure the effectiveness and adherence to the guidelines, the efficiency of the treatment in terms of resource utilization and the success of the treatment in preventing further complications (neurocognitive outcome).
