Phenylalanine-free Diet for Patients With Secondary Hyperphenylalaninemia in ICU
Phenylalanine-free Diet Lowers the Blood Phenylalanine Levels in Patients With High Phenylalanine Level in the Intensive Care Unit
Chang Gung Memorial Hospital
100 participants
Jul 30, 2021
INTERVENTIONAL
Conditions
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.
Eligibility
Inclusion Criteria4
- had Acute Physiology And Chronic Health Evaluation (APACHE II) scores of ≥15;
- were expected to stay in the ICU >48 hours;
- patients with baseline PHE levels of ≥95 uM;
- were over 20 years old.
Exclusion Criteria2
- patients with comorbid disorders other than the main cause for admission that might compromise their survival within three months, such as terminal stage cancer;
- patients with expected survival <3 days.
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Interventions
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)
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NCT04896281