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 6 Spearman correlation coefficients between Adult PKU-QOL and SF-36 scores in the adult evaluable population (n = 104)

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	SF-36 scores
PKU-QOL scores	PF	RP	BP	GH	VT	SF	RE	MH
Self-rated health status	−0.37	−0.22	−0.30	−0.58	−0.57	−0.36	−0.29	−0.50
Headaches	−0.20	−0.26	−0.39	−0.31	−0.29	−0.41	−0.30	−0.35
Stomach aches	−0.17	−0.19	−0.40	−0.30	−0.34	−0.26	−0.33	−0.37
Tiredness	−0.28	−0.34	−0.30	−0.40	−0.52	−0.40	−0.32	−0.39
Lack of concentration	−0.25	−0.59	−0.37	−0.40	−0.52	−0.51	−0.56	−0.60
Slow thinking	−0.27	−0.36	−0.29	−0.36	−0.32	−0.44	−0.47	−0.43
Trembling hands	−0.16	−0.21	−0.19	−0.31	−0.31	−0.19	−0.25	−0.28
Irritability	−0.23	−0.44	−0.32	−0.29	−0.20	−0.31	−0.40	−0.39
Aggressiveness	−0.22	−0.36	−0.21	−0.24	−0.22	−0.31	−0.31	−0.24
Moodiness	−0.21	−0.40	−0.45	−0.38	−0.41	−0.54	−0.50	−0.53
Sadness	−0.21	−0.44	−0.48	−0.49	−0.39	−0.61	−0.56	−0.66
Anxiety	−0.16	−0.31	−0.26	−0.31	−0.29	−0.37	−0.43	−0.47
Emotional impact of PKU	−0.41	−0.31	−0.35	−0.43	−0.34	−0.35	−0.31	−0.39
Practical impact of PKU	−0.05	−0.38	−0.18	−0.38	−0.38	−0.38	−0.47	−0.38
Social impact of PKU	−0.34	−0.32	−0.30	−0.40	−0.30	−0.48	−0.35	−0.39
Overall impact of PKU	−0.27	−0.42	−0.42	−0.53	−0.32	−0.53	−0.45	−0.48
Anxiety – blood test	0.05	−0.03	−0.27	−0.16	−0.12	−0.32	−0.22	−0.25
Anxiety – Phe levels	−0.19	−0.11	−0.21	−0.28	−0.19	−0.25	−0.17	−0.15
Anxiety – Phe levels during pregnancy	−0.11	0.11	−0.03	−0.02	−0.00	−0.05	0.06	−0.13
Financial impact of PKU	−0.11	−0.05	−0.05	0.14	0.03	0.01	0.03	0.08
Information on PKU	−0.08	−0.11	−0.15	−0.12	−0.08	−0.17	−0.22	−0.19
Adherence to Phe-free protein supplements	−0.10	−0.28	−0.25	−0.14	−0.29	−0.35	−0.38	−0.18
Practical impact of Phe-free protein supplements	−0.13	−0.13	−0.11	−0.41	−0.43	−0.33	−0.25	−0.37
Guilt if poor adherence to Phe-free protein supplements	−0.12	−0.16	−0.23	−0.22	−0.13	−0.30	−0.13	−0.23
Relationships within family because of Phe-free protein supplements	−0.02	−0.01	−0.20	−0.15	−0.13	−0.13	0.01	−0.09
Taste – Phe-free protein supplements	0.07	0.12	0.04	−0.24	−0.25	−0.18	−0.00	0.00
Food temptations	−0.14	−0.40	−0.19	−0.21	−0.25	−0.37	−0.31	−0.33
Adherence to dietary protein restriction	−0.03	−0.29	−0.13	−0.06	−0.17	−0.24	−0.29	−0.29
Social impact of dietary protein restriction	−0.12	−0.40	−0.20	−0.43	−0.44	−0.50	−0.50	−0.49
Practical impact of dietary protein restriction	−0.21	−0.25	−0.11	−0.38	−0.59	−0.44	−0.39	−0.46
Overall impact of dietary protein restriction	−0.23	−0.33	−0.17	−0.45	−0.57	−0.54	−0.44	−0.49
Taste – specialty low-protein food	−0.09	−0.31	−0.27	−0.33	−0.25	−0.34	−0.41	−0.33
Food enjoyment	−0.19	−0.28	−0.16	−0.36	−0.34	−0.41	−0.38	−0.29
Guilt if dietary protein restriction not followed	−0.17	−0.15	−0.21	−0.30	−0.10	−0.33	−0.12	−0.16
Overall difficulty following dietary protein restriction	−0.33	−0.47	−0.21	−0.38	−0.46	−0.46	−0.34	−0.33

Phe: phenylalanine; PKU: phenylketonuria; QOL: quality of life; SF-36: 36-item Short Form; PF: Physical functioning; RP: Role Physical; BP: Bodily Pain; GH: General health; VT: Vitality; SF: Social Functioning; RE: Role Emotional; MH: Mental Health.
Correlations between Adult PKU-QOL and SF-36 scores were low to moderate. Moderate correlations (>0.4) are shown in bold.

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ISSN: 1750-1172

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