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The patient
with PKU should be monitored regularly depending upon age. The following
monitoring guidelines have been developed for medical/nutritional management
of children with PKU:
Birth
to 1 Year:
- Serum
phenylalanine and tyrosine levels (including report to family and
appropriate adjustment of formula) according to schedule established
by "New Infant Protocol,"
then once per month when levels are stable.
- RD
contacts (nutritional assessment, including measurement of length,
weight, head circumference, and review of diet and blood levels, and
counseling) once per month.
- Hemoglobin
or hematocrit measurement once, at 6 months of age.
- PKU
Clinic visit once per month.
1 - 3
Years:
- Serum
phenylalanine and tyrosine levels (including report to family and
appropriate adjustment of formula and phenylalanine from food) once
per month; mid-month levels when serum phenylalanine is less than 1
mg/dL or greater than 6 mg/dL.
- RD
contacts (nutritional assessment, including measurement of length,
weight, head circumference, and review of diet and blood levels, and
counseling) once per month.
- Hemoglobin
or hematocrit measurement once per year.
- Albumin/prealbumin
measurement once every 1-2 years.
- PKU
Clinic visit once per month.
- Psychometric
assessment one evaluation at age 3, according to neuropsychological
testing protocol.
3 - 5
Years:
- Serum
phenylalanine and tyrosine levels (including report to family and
appropriate adjustment of formula and phenylalanine from food) once
per month; mid-month levels when serum phenylalanine is less than 1
mg/dL or greater than 6 mg/dL.
- RD
contacts (nutritional assessment, including measurement of height
and weight, review of diet and blood levels, and counseling) once
per month.
- Hemoglobin
or hematocrit measurement once per year.
- Albumin/prealbumin
measurement once every 1-2 years.
- PKU
Clinic visit once per month.
- Psychometric
assessment one evaluation every 3 years, according to neuropsychological
testing protocol.
5 Years
and older:
- Serum
phenylalanine and tyrosine levels (including report to family and
appropriate adjustment of formula and phenylalanine from food) once
per month; mid-month levels when serum phenylalanine is less than 1
mg/dL or greater than 6 mg/dL.
- RD
contacts (nutritional assessment, including measurement of height
and weight, review of diet and blood levels, and counseling) once
per month.
- Hemoglobin
or hematocrit measurement once per year.
- Albumin/prealbumin
measurement once every 1-2 years.
- PKU
Clinic visit once per month.
- Psychometric
assessment one evaluation every 3 years, according to neuropsychological
testing protocol.
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