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Phenylalanine-free Diet for Patients With Secondary Hyperphenylalaninemia in ICU
Sponsor: Chang Gung Memorial Hospital
Summary
Hyperphenylalaninemia is not only present in patients with congenital phenylketonuria. In adults with critical illness, hyperphenylalaninemia is noted in some patients and is associated with high mortality rate. Hyperphenylalaninemia can cause metabolic acidosis, brain dysfunction, and metabolic disturbance. The investigators would like to see whether the phenylalanine-free milk for congenital phenylketonuria is also effective in lowering the blood phenylalanine concentrations in patients with critical illness in intensive care unit and hyperphenylalaninemia.
Official title: Phenylalanine-free Diet Lowers the Blood Phenylalanine Levels in Patients With High Phenylalanine Level in the Intensive Care Unit
Key Details
Gender
All
Age Range
20 Years - Any
Study Type
INTERVENTIONAL
Enrollment
100
Start Date
2021-07-30
Completion Date
2026-12-31
Last Updated
2026-03-20
Healthy Volunteers
No
Interventions
Phenylalanine-free milk
Once a plasma PHE level of ≥ 95 μM is noted, phenylalanine-free diet (Phenex-2®, Abbott Nutrition, Ohio, USA; or Phenyl-Free 2®, Mead Johnson Nutrition, Minnesota, USA) is started as the only nutrition source for enteral feeding, as per the recommendation of dieticians, in the following 4 days. The phenylalanine-free diet is free of PHE but is enriched with protein, potassium, tyrosine, and antioxidant micronutrients. After the completion of the study, diet will be back to normal diet for all patients.
Locations (1)
Chang Gung Memorial Hospital
Keelung, Taiwan