Short-term Effects of Good Metabolic Control on Cognitive Function, Wellbeing, and Metabolic Parameters in Adult Patients With Phenylketonuria

Klaus Parhofer13 enrolled

Overview

The goal of this study is to evaluate if in adult patients with phenylketonuria (PKU) without strict metabolic control during the last 12 months, strict metabolic control for 8 weeks results in an improvement of cognitive function.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Enrollment

Conditions

Phenylketonurias

Interventions

PHE-dietOTHER

phenylalanine-restricted diet with phenylalanine free supplements for 8 weeks

Eligibility

Sex: ALLMin age: 18 YearsMax age: 59 Years

Medical Language ↔ Plain English

Inclusion Criteria: * Men and women with classical PKU * Age ≥ 18 years * Good metabolic control during childhood (at least until 12 years of age) * Plasma Phe levels ≥ 15 mg/dl for ≥ 1 year * Written informed consent prior to study participation Exclusion Criteria: * Reduced cognitive function (unability to perform test battery) * Drug and or alcohol abuse * Treatment with BH4 * Treatment with drugs known to affect cognitive function

Locations (1)

University Munich

Munich, Germany

Outcomes

Primary Outcomes

Spatial working memory strategy index

cognitive function; scores range from 4 to 28, with lower scores representing better outcome

Time frame: 8 weeks

Secondary Outcomes

Reaction time

cognitive function; milliseconds, with faster times representing better outcome

Time frame: 8 weeks

Paired Associates Learning

cognitive function; scores range from 0 to 70, with lower scores indicating better outcome

Time frame: 8 weeks

Multitasking Test

cognitive function; scores range from 0 to 279, with lower scores indicating better outcome

Time frame: 8 weeks

Data from ClinicalTrials.gov

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