Abstract

Phenylketonuria (PKU) requires tight control to prevent neurocognitive impairment but reports show that patients may present mild cognitive defects related to higher impulsivity. We hypothesize that chronic intervention may influence the parents and child bonding and the child´s resources to face problems. To describe the PKU parenting styles perceived by the children (PS) and their coping strategies (CS) assessing their relationship with impulsivity, 30 early diagnosed and adequately treated PKU children and 30 non PKU aged-paired controls (CG) were compared. The Argentine Children´s Coping Questionnaire, Argentine Scale Perception of the Relationship with Parents, WISC IV Comprehension Subtest, and CPT II test were administered. PKU PS were based on control: strict to pathologic in the mother and acceptable in the father (both p<0.05 vs. CG). Children significantly sought greater support and showed less emotional control when facing conflicts. These characteristics positively correlated with maternal control r:.383 and r:.398 (both p<0.05). Impulsivity was higher in PKU (p<0.05) but didn´t associate with PS or CS. Maternal strict control wasn´t linked to the higher impulsivity found (possibly neurobiologically based). Nevertheless, if both factors are present, patients may develop a psychological and/or behavioral trait of greater dependency and impulsivity that must be considered in their follow-up.

Highlights

  • Phenylketonuria (PKU) is a chronic metabolic disease that needs to be treated and tightly controlled since its neonatal detection to prevent neurocognitive impairment

  • On another note, coping strategies are defined as the resources that the individual uses to face conflictive situations with either cognitive and/or emotional effort. [4,5] how stressors are faced depends on the available personal resources, the environmental limitations to exert them, and the ability to put them into practice.[6,7,8]

  • This study aimed to investigate the impact of the PKU chronic condition on the parenting styles perceived by the affected children and their coping strategies

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Summary

Introduction

Phenylketonuria (PKU) is a chronic metabolic disease that needs to be treated and tightly controlled since its neonatal detection to prevent neurocognitive impairment. This follows the WHO definition, which states that chronic diseases are conditions that lasting one year or more require ongoing medical attention and/or limit activities of daily living.[1] They include organic, psychological, and/or social diseases. On another note, coping strategies are defined as the resources that the individual uses to face conflictive situations with either cognitive and/or emotional effort. It has been reported that chronically ill children may have fewer resources to solve everyday problems. Even with a normal IQ, cognitive deficiencies in executive functions have been consistently reported. [1,2] Among them, defects in the inhibitory impulse control and impaired cognitive flexibility were described. [1,3,4,5]

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