Related Experiment Video

Updated: Jun 2, 2026

A Treatment Package without Escape Extinction to Address Food Selectivity
04:23

A Treatment Package without Escape Extinction to Address Food Selectivity

Published on: August 21, 2015

Pre-attentive processing in children with early and continuously-treated PKU. Effects of concurrent Phe level and

Leo M J de Sonneville1, Stephan C J Huijbregts, Robert Licht

  • 1Leiden Institute for Brain and Cognition, and Dept. of Clinical Child and Adolescent Studies, Faculty of Social Sciences, Leiden University, Wassenaarseweg 52, 2333, AK, Leiden, The Netherlands. LdeSonneville@fsw.leidenuniv.nl

Journal of Inherited Metabolic Disease
|May 5, 2011
PubMed

Insights

Phenylketonuria (PKU) in children may impact brain processing, even with early treatment. Higher lifetime phenylalanine levels are linked to attention and neural processing deficits, suggesting dietary control is crucial.

Area of Science:

  • Neuroscience
  • Pediatrics
  • Metabolic Disorders

Background:

  • Phenylketonuria (PKU) is an inherited metabolic disorder requiring early dietary intervention.
  • Long-term effects of phenylalanine (Phe) levels on neurodevelopment in treated children are not fully understood.
  • Pre-attentive sensory processing may be a sensitive indicator of early brain development.

Purpose of the Study:

  • To compare visual evoked potentials (VEP) and auditory mismatch negativity (MMN) in children with early-treated PKU and controls.
  • To investigate the association between dietary control indices and VEP/MMN parameters in children with PKU.
  • To explore the impact of lifetime phenylalanine exposure on neurophysiological measures.

Main Methods:

  • Sixty-four children (7–14 years) with early-treated PKU and age-matched controls were assessed.
  • Visual evoked potentials (VEP) and auditory mismatch negativity (MMN) amplitudes and latencies were measured.
  • Lifetime and concurrent phenylalanine levels, and Index of Dietary Control (IDC) were analyzed.

Main Results:

  • No significant differences in VEP and MMN indices were found between PKU and control groups.
  • Higher lifetime Phe levels correlated with increased N75 amplitudes (attention) and longer P110 latencies (neural processing speed).
  • Elevated lifetime Phe and poorer Index of Dietary Control (IDC) were associated with decreased MMN amplitudes (stimulus change detection).

Conclusions:

  • Early dietary management in PKU may not fully normalize pre-attentive sensory processing.
  • Lifetime phenylalanine exposure, rather than concurrent levels, appears linked to subtle neurophysiological alterations.
  • Dietary control quality may influence the development of pre-attentive processes, highlighting potential windows of vulnerability to phenylalanine toxicity.

More Related Videos

P50 Sensory Gating in Infants12:55

P50 Sensory Gating in Infants

Published on: December 26, 2013

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring07:35

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring

Published on: June 23, 2015

Related Concept Videos

Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses a challenge in...
Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption01:23

Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption

Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
Overview of Protein Metabolism01:21

Overview of Protein Metabolism

Proteins are broken down into amino acids during digestion. Unlike fats and carbohydrates, which are stored for later use, proteins are not. Instead, amino acids are either used to produce ATP through oxidation or contribute to the creation of new proteins for the growth and repair of the body. Any surplus amino acids from the diet are converted into glucose or triglycerides rather than excreted.
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...
Pharmacokinetics in Pediatric Patients: Drug Excretion01:26

Pharmacokinetics in Pediatric Patients: Drug Excretion

In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
Chronic Kidney Disease III: Interprofessional Care01:28

Chronic Kidney Disease III: Interprofessional Care

Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...