We aimed to assess if the same cognitive batteries can be used cross-nationally to monitor the effect of Phenylketonuria (PKU). We assessed whether a battery, previously used with English adults with PKU (AwPKU), was also sensitive to impairments in Italian AwPKU. From our original battery, we selected a number of tasks that comprehensively assessed visual attention, visuo-motor coordination, executive functions (particularly, reasoning, planning, and monitoring), sustained attention, and verbal and visual memory and learning. When verbal stimuli/or responses were involved, stimuli were closely matched between the two languages for psycholinguistic variables. We administered the tasks to 19 Italian AwPKU and 19 Italian matched controls and compared results from with 19 English AwPKU and 19 English matched controls selected from a previously tested cohort. Participant election was blind to cognitive performance and metabolic control, but participants were closely matched for age and education. The Italian AwPKU group had slightly worse metabolic control but showed levels of performance and patterns of impairment similar to the English AwPKU group. The Italian results also showed extensive correlations between adult cognitive measures and metabolic measures across the life span, both in terms of Phenylalanine (Phe) levels and Phe fluctuations, replicating previous results in English. These results suggest that batteries with the same and/or matched tasks can be used to assess cognitive outcomes across countries allowing results to be compared and accrued. Future studies should explore potential differences in metabolic control across countries to understand what variables make metabolic control easier to achieve.

Cognitive Outcomes and Relationships with Phenylalanine in Phenylketonuria: A Comparison between Italian and English Adult Samples / Romani, Cristina; Manti, Filippo; Nardecchia, Francesca; Valentini, Federica; Fallarino, Nicoletta; Carducci, Claudia; DE LEO, Sabrina; Macdonald, Anita; Palermo, Liana; Leuzzi, Vincenzo. - In: NUTRIENTS. - ISSN 2072-6643. - (2020). [10.3390/nu12103033]

Cognitive Outcomes and Relationships with Phenylalanine in Phenylketonuria: A Comparison between Italian and English Adult Samples.

Cristina Romani
Primo
Writing – Original Draft Preparation
;
Filippo Manti
Secondo
Writing – Review & Editing
;
Francesca Nardecchia
Writing – Review & Editing
;
Federica Valentini
Data Curation
;
Nicoletta Fallarino
Data Curation
;
Claudia Carducci
Data Curation
;
Sabrina De Leo
Data Curation
;
Liana Palermo
Penultimo
Writing – Review & Editing
;
Vincenzo Leuzzi
Ultimo
Writing – Review & Editing
2020

Abstract

We aimed to assess if the same cognitive batteries can be used cross-nationally to monitor the effect of Phenylketonuria (PKU). We assessed whether a battery, previously used with English adults with PKU (AwPKU), was also sensitive to impairments in Italian AwPKU. From our original battery, we selected a number of tasks that comprehensively assessed visual attention, visuo-motor coordination, executive functions (particularly, reasoning, planning, and monitoring), sustained attention, and verbal and visual memory and learning. When verbal stimuli/or responses were involved, stimuli were closely matched between the two languages for psycholinguistic variables. We administered the tasks to 19 Italian AwPKU and 19 Italian matched controls and compared results from with 19 English AwPKU and 19 English matched controls selected from a previously tested cohort. Participant election was blind to cognitive performance and metabolic control, but participants were closely matched for age and education. The Italian AwPKU group had slightly worse metabolic control but showed levels of performance and patterns of impairment similar to the English AwPKU group. The Italian results also showed extensive correlations between adult cognitive measures and metabolic measures across the life span, both in terms of Phenylalanine (Phe) levels and Phe fluctuations, replicating previous results in English. These results suggest that batteries with the same and/or matched tasks can be used to assess cognitive outcomes across countries allowing results to be compared and accrued. Future studies should explore potential differences in metabolic control across countries to understand what variables make metabolic control easier to achieve.
2020
PKU; Phe associations; cognitive outcomes; cross-countries; cross-cultural.
01 Pubblicazione su rivista::01a Articolo in rivista
Cognitive Outcomes and Relationships with Phenylalanine in Phenylketonuria: A Comparison between Italian and English Adult Samples / Romani, Cristina; Manti, Filippo; Nardecchia, Francesca; Valentini, Federica; Fallarino, Nicoletta; Carducci, Claudia; DE LEO, Sabrina; Macdonald, Anita; Palermo, Liana; Leuzzi, Vincenzo. - In: NUTRIENTS. - ISSN 2072-6643. - (2020). [10.3390/nu12103033]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1442914
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