What are target phenylalanine levels for PKU patients?

Target blood phe is <360 μmol/L (6 mg/dL) for all ages, with stricter targets of 120-360 μmol/L recommended during pregnancy to prevent maternal PKU syndrome.

How is sapropterin responsiveness tested?

A BH4 loading test gives 20 mg/kg sapropterin; a ≥30% reduction in blood phe at 24-48 hours indicates responsiveness (seen in ~25-50% of PKU patients).

What is the dietary phenylalanine tolerance calculation?

Tolerance is determined empirically by adjusting natural protein intake while maintaining blood phe in target range, typically 200-500 mg phe/day for classical PKU.

How does maternal PKU affect the fetus?

Maternal blood phe >600 μmol/L causes microcephaly, congenital heart disease, intellectual disability, and growth restriction in the non-PKU fetus.

What is the role of large neutral amino acids in PKU?

LNAA supplements compete with phe for blood-brain barrier transport, reducing brain phe levels; used as adjunct or alternative in adults with difficulty maintaining diet.

Phenylketonuria (PKU) Monitoring Calculator

Calculates dietary phenylalanine tolerance, target blood phe levels by age, and sapropterin (BH4) responsiveness assessment for phenylketonuria management.

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Frequently Asked Questions

What are target phenylalanine levels for PKU patients?

How is sapropterin responsiveness tested?

What is the dietary phenylalanine tolerance calculation?

How does maternal PKU affect the fetus?

What is the role of large neutral amino acids in PKU?