Related Experiment Video

Updated: Mar 22, 2026

Quantitative Autoradiographic Method for Determination of Regional Rates of Cerebral Protein Synthesis In Vivo
11:01

Quantitative Autoradiographic Method for Determination of Regional Rates of Cerebral Protein Synthesis In Vivo

Published on: June 28, 2019

7.5K

Relationship between age and white matter integrity in children with phenylketonuria

Erika Wesonga1, Joshua S Shimony2, Jerrel Rutlin3

  • 1Department of Psychological & Brain Sciences, One Brookings Drive, Campus Box 1125, Washington University, St. Louis, MO 63130, USA.

Molecular Genetics and Metabolism Reports
|April 27, 2016
PubMed

Insights

Children with phenylketonuria (PKU) show accelerated white matter development differences compared to healthy children. This study found a stronger age-related decrease in mean diffusivity (MD) in the brains of children with PKU.

Area of Science:

  • Neuroimaging
  • Developmental Neuroscience
  • Metabolic Disorders

Background:

  • Diffusion tensor imaging (DTI) reveals impaired white matter integrity in children with phenylketonuria (PKU).
  • Mean diffusivity (MD) is decreased in children with PKU compared to healthy controls.
  • The relationship between age and white matter integrity in PKU requires further investigation.

Purpose of the Study:

  • To examine age-related differences in white matter integrity (measured by MD) between children with PKU and healthy controls.
  • To explore group differences in the trajectory of white matter development across brain regions.

Main Methods:

  • Diffusion tensor imaging (DTI) was used to assess white matter integrity.
  • Hierarchical linear regression and Pearson correlation analyses explored relationships among MD, age, and group.
  • 31 children with early- and continuously-treated PKU and 51 healthy controls were studied.

Main Results:

  • A stronger age-related decrease in mean diffusivity (MD) was observed in children with PKU compared to healthy children.
  • This effect was significant in 4 out of 10 brain regions examined.
  • Findings suggest an altered trajectory of white matter development in children with PKU.

Conclusions:

  • The white matter development trajectory appears abnormal in children with phenylketonuria.
  • Longitudinal studies are recommended to further understand white matter development in this population.
  • Early and continuous treatment may influence these developmental patterns.

Keywords:
BrainChildrenDiffusion tensor imagingPhenylketonuriaWhite matter

More Related Videos

Probing the Brain in Autism Using fMRI and Diffusion Tensor Imaging12:21

Probing the Brain in Autism Using fMRI and Diffusion Tensor Imaging

Published on: September 12, 2011

26.0K
3D Modeling of the Lateral Ventricles and Histological Characterization of Periventricular Tissue in Humans and Mouse15:26

3D Modeling of the Lateral Ventricles and Histological Characterization of Periventricular Tissue in Humans and Mouse

Published on: May 19, 2015

14.9K

Related Concept Videos

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...
996
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...
315
Pharmacokinetics in Pediatric Patients: Drug Distribution01:17

Pharmacokinetics in Pediatric Patients: Drug Distribution

Drug distribution in the pediatric population exhibits unique challenges and considerations due to the physiological differences between children, particularly neonates and infants, and adults. A crucial aspect of pediatric pharmacology is understanding how these differences impact the pharmacokinetics of various drugs, necessitating age-specific dosing strategies to ensure efficacy and safety.Neonates and infants have a higher total body water content, ~75%–90% of their body weight,...
441
Biological Influences on Intelligence01:30

Biological Influences on Intelligence

Intelligence is often thought to be linked to brain size, but the relationship is more complex than that. While brain size does correlate modestly with some abilities, like verbal skills, the connection is weaker for others, such as spatial reasoning. Other factors, like brain structure, also play crucial roles. For instance, despite Einstein's smaller-than-average brain, his parietal cortex, which is involved in spatial reasoning, was 15% wider, suggesting that neural density might matter...
647
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...
669
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...
4.6K