Development and psychometric validation of measures to assess the impact of phenylketonuria and its dietary treatment on patients’ and parents’ quality of life: the phenylketonuria – quality of life (PKU-QOL) questionnaires

Regnault, Antoine; Burlina, Alberto; Cunningham, Amy; Bettiol, Esther; Moreau-Stucker, Flavie; Benmedjahed, Khadra; Bosch, Annet M

doi:10.1186/s13023-015-0261-6

Orphanet Journal of Rare Diseases

Table 4 Spearman correlation coefficients between child PKU-QOL and adolescent PKU-QOL and PedsQL scores

From: Development and psychometric validation of measures to assess the impact of phenylketonuria and its dietary treatment on patients’ and parents’ quality of life: the phenylketonuria – quality of life (PKU-QOL) questionnaires

PKU-QOL scores	Child evaluable population (n = 92)				Adolescent evaluable population (n = 110)
PKU-QOL scores	EF	PF	SoF	ScF	EF	PF	SoF	ScF
Self-rated health status	-	-	-	-	−0.26	−0.23	−0.22	−0.16
Headaches	−0.07	−0.25	−0.05	−0.22	−0.23	−0.28	−0.22	−0.26
Stomach aches	−0.33	−0.06	−0.06	−0.17	−0.16	−0.23	−0.25	−0.11
Tiredness	−0.13	−0.34	−0.21	−0.30	−0.23	−0.22	−0.15	−0.18
Angry	−0.38	−0.36	−0.24	−0.30	−0.30	−0.08	−0.27	−0.08
Aggressiveness	−0.08	−0.13	−0.00	−0.10	−0.31	−0.20	−0.21	−0.31
Moodiness	−0.13	−0.26	−0.11	−0.25	−0.28	−0.10	−0.10	−0.27
Sadness	−0.41	−0.33	−0.33	−0.23	−0.31	−0.17	−0.23	−0.16
Anxiety	−0.26	−0.05	−0.31	−0.12	−0.27	−0.18	−0.27	−0.10
Lack of concentration	−0.15	−0.32	−0.33	−0.36	−0.21	−0.19	−0.27	−0.48
Slow thinking	−0.24	−0.45	−0.37	−0.43	−0.32	−0.42	−0.42	−0.36
Emotional impact of PKU	−0.45	−0.22	−0.28	−0.33	−0.47	−0.19	−0.31	−0.28
Practical impact of PKU	−0.40	−0.24	−0.25	−0.09	−0.29	−0.19	−0.30	−0.28
Social impact of PKU	−0.39	−0.38	−0.41	−0.20	−0.35	−0.10	−0.20	−0.15
Overall impact of PKU	−0.54	−0.35	−0.41	−0.30	−0.49	−0.21	−0.31	−0.31
Anxiety – blood test	−0.13	−0.05	−0.04	0.05	−0.11	−0.16	−0.05	−0.03
Anxiety – blood Phe levels	−0.42	−0.25	−0.13	−0.37	−0.37	−0.14	−0.18	−0.25
Adherence to Phe-free protein supplements	−0.12	−0.11	0.00	−0.11	−0.13	−0.23	−0.24	−0.26
Practical impact of Phe-free protein supplements	−0.23	−0.18	−0.21	−0.16	−0.43	−0.31	−0.36	−0.25
Guilt if poor adherence to Phe-free protein supplements	0.14	0.09	0.01	0.18	−0.11	0.11	0.04	0.08
Relationships within family because of Phe-free protein supplements	−0.30	−0.18	−0.18	−0.11	−0.16	−0.11	−0.14	−0.37
Taste – Phe-free protein supplements	−0.28	−0.06	−0.10	−0.23	−0.06	0.00	0.08	−0.17
Food temptations	−0.32	−0.29	−0.35	−0.14	−0.36	−0.10	−0.24	−0.18
Adherence to dietary protein – restriction	−0.03	−0.14	−0.01	−0.11	−0.39	−0.09	−0.29	−0.18
Social impact of dietary protein restriction	−0.40	−0.45	−0.40	−0.47	−0.37	−0.19	−0.34	−0.34
Practical impact of dietary protein restriction	-	-	-	-	−0.44	−0.24	−0.30	−0.40
Overall impact of dietary protein restriction	-	-	-	-	−0.45	−0.27	−0.33	−0.35
Taste – specialty low-protein food	−0.12	0.08	0.13	−0.01	−0.19	−0.03	−0.09	−0.15
Food enjoyment	−0.19	−0.23	−0.26	−0.11	0.04	0.03	−0.08	−0.08
Guilt if dietary protein restriction not followed	0.01	−0.08	−0.10	−0.03	−0.24	−0.03	−0.16	−0.03
Overall difficulty following dietary protein restriction	−0.32	−0.23	−0.24	−0.21	−0.37	−0.16	−0.19	−0.32

PedsQL: Pediatric Quality-of-Life Inventory; Phe: phenylalanine; PKU: phenylketonuria; QOL: quality of life; EF: Emotional functioning; PF: Physical functioning; SoF: Social functioning; ScF: School functioning.
Moderate correlations (>0.4) are shown in bold.

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ISSN: 1750-1172

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