Related Experiment Video
Updated: May 19, 2026
08:27
Single Synapse Indicators of Glutamate Release and Uptake in Acute Brain Slices from Normal and Huntington Mice
Published on: March 11, 2020
Processing speed and executive abilities in children with phenylketonuria
Alicia L Janos1, Dorothy K Grange, Robert D Steiner
1Department of Psychology, Campus Box 1125, Washington University, St. Louis, MO 63130, USA.
Neuropsychology
|August 8, 2012
Summary
Children with phenylketonuria (PKU) exhibit slower and more variable information processing. While processing speed improves with age, PKU impacts executive functions like working memory and inhibitory control.
Area of Science:
- Neuroscience
- Developmental Psychology
- Metabolic Disorders
Background:
- Phenylketonuria (PKU) is an inherited metabolic disorder.
- Early and continuous treatment is crucial but may not fully prevent neuropsychological deficits.
Purpose of the Study:
- To investigate processing speed and its variability in children with PKU.
- To examine the relationship between processing speed variables and executive functions in this population.
Main Methods:
- 42 children with PKU and 81 controls (ages 7-18) completed computerized reaction time tasks.
- Executive abilities were assessed using 7 tasks measuring working memory, inhibitory control, and strategic processing.
Main Results:
- Children with PKU demonstrated significantly slower and more variable processing speeds than controls.
- Processing speed improved with age similarly in both groups, but processing speed variability decreased more with age in the PKU group.
- Processing speed and variability influenced most executive tasks, with residual impairments in working memory and inhibitory control noted in the PKU group.
Conclusions:
- Information processing is demonstrably slower and less efficient in children with PKU.
- Processing speed and variability partially explain, but do not fully account for, executive function impairments in PKU.
Related Concept Videos
01:24Pharmacokinetics 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...
01:20Inborn 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...
01:30Information Processing Approach
The information-processing theory of cognitive development centers on fundamental mental processes, including attention, memory, and problem-solving skills. Researchers in this field examine how cognitive abilities, such as working memory, evolve and influence children's overall development. Studies indicate that children with stronger working memory tend to excel in reading comprehension, math, and problem-solving compared to peers with less efficient memory skills. Low working memory is also...
01:23Pharmacokinetics 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...
01:26Pharmacokinetics 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...
01:27Pharmacogenetics of Phase II Enzymes: N-acetyltransferase, Thiopurine S-methyltransferase, UDP-glucuronosyltransferase
Phase II biotransformation reactions are essential for detoxifying and eliminating xenobiotics, including many pharmaceutical compounds. These reactions typically involve conjugation, the covalent attachment of polar endogenous groups such as glucuronic acid, sulfate, methyl, or acetyl moieties to functional groups introduced during Phase I metabolism. The resulting conjugates are more water-soluble, enabling efficient renal or biliary excretion.The major classes of Phase II enzymes include...