Reversible white matter changes following a 4-week high phenylalanine exposure in adults with phenylketonuria.

Alterations in brain structure are frequently observed in adults with early-treated phenylketonuria (PKU) compared to healthy controls, with cerebral white matter (WM) being particularly affected. The extent to which temporary elevation of phenylalanine (Phe) levels impacts WM remains unclear. We conducted a double-blind, randomised, placebo-controlled crossover trial to investigate the effects of a 4-week high Phe exposure on cerebral WM and its relationship to cognitive performance and metabolic parameters in adults with PKU.

Do early-treated adults with phenylketonuria sense high phenylalanine levels?

This study aimed to analyze whether early-treated adults with phenylketonuria (PKU) can subjectively sense high phenylalanine (Phe) concentrations and whether a possible impact of Phe on objective measures of cognitive performance and mood reflects patients' self-perception. Data from the PICO study, a randomized, placebo-controlled, double-blind, crossover trial, were analyzed. Twenty-eight adults with PKU received either Phe capsules or placebo in two 4-week intervention periods in a randomized order, with a 4-week washout in between.

Development of working memory, processing speed, and psychosocial functions in patients with pediatric cancer.

Many patients after pediatric cancer suffer from long-term cognitive difficulties. This study investigates the development of cognitive and psychosocial functions between diagnosis and one year after cancer treatment and reveals insight into the association between cognitive and psychosocial development and various risk factors. This retrospective clinical record review included fifty-seven patients, aged 4-16 years, that were examined at the beginning of the cancer treatment (T1) and one year after cancer treatment (T2) to evaluate the development of working memory (WM), processing speed (PS), psychosocial functions, and quality of life (QoL).

Effect of a four-week oral Phe administration on neural activation and cerebral blood flow in adults with early-treated phenylketonuria.

Background: Phenylketonuria (PKU) is a rare inborn error of metabolism characterized by impaired catabolism of the amino acid phenylalanine (Phe) into tyrosine. Cross-sectional studies suggest slight alterations in cognitive performance and neural activation in adults with early-treated PKU. The influence of high Phe levels on brain function in adulthood, however, remains insufficiently studied.

Cerebral blood flow and structural connectivity after working memory or physical training in paediatric cancer survivors - Exploratory findings.

Paediatric cancer survivors often suffer from cognitive long-term difficulties. Consequently, strengthening cognition is of major clinical relevance. This study investigated cerebral changes in relation to cognition in non-brain tumour paediatric cancer survivors after working memory or physical training compared to a control group.