Protein Requirements in Adults With Phenylketonuria (PKU)

University of British Columbia

Status: Completed

Conditions: Phenylketonuria

Treatments: Dietary Supplement: Free amino acids intake

Study type: Interventional

Funder types: Other; Industry

Identifiers

NCT03939052

H18-03464

Details and patient eligibility

About

Phenylketonuria (PKU) is an inherited inborn error of phenylalanine (PHE) metabolism caused by decreased activity of phenylalanine hydroxylase (PAH) enzyme. Therefore, PHE accumulates in plasma leading to mental problems. Treatment is a phenylalanine-restricted diet with sufficient protein. However, the optimum protein requirements are still unknown and compliance with diet is not satisfactory in PKU adults. A Previously established technique called indicator amino acid oxidation (IAAO) will be used to determine protein requirements from amino acid based formula vs. glycomacropeptide (GMP) in adults with PKU (≥ 19y). This study will help treat adults with enough protein ensuring maintenance of health.

Enrollment: 6 patients

Sex: All

Ages: 19 to 50 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

-Adults more than 19 years of age who are diagnosed with PKU and clinically stable with no acute illness

Exclusion criteria

• Adults with PKU under age 19 year
• Adults diagnosed with PKU but are currently ill with a fever or cold

Trial design

Primary purpose: Supportive Care

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

---

6 participants in 1 patient group

Protein intake

Experimental group

Description:

Free amino acids vs. Glycomacropeptide (GMP)

Treatment:

Dietary Supplement: Free amino acids intake