Food Neophobia in Patients With Phenylketonuria

Abstract

Background: In phenylketonuria (PKU), only a limited range of natural foods, such as fruits, vegetables, oils and sugars, can be consumed without restrictions. The present study aimed to study if food neophobia (i.e. an unwillingness to eat novel food) occurs in patients with PKU and to compare in non-PKU healthy controls and to identify any associated factors. Methods: This cross-sectional case-control study used a convenience sampling strategy to recruit patients diagnosed with PKU and healthy controls matched by gender and age. Patients and controls were invited to complete the food neophobia scale (FNS) questionnaire, and clinical and treatment data were collected through a review of medical records. Results: Twenty-five patients (mean age 19.3 ± 4.7 years, 13 women) with PKU and 25 controls (mean age 19.9 ± 4.9 years; P = 0.676, 13 women) were evaluated. The mean age of treatment onset in patients with PKU was 52.8 ± 29.7 days. The mean phenylalanine (Phe) level in the 12-month preceding neophobia study was 710.5 ± 346 µmol/L. The mean FNS score of patients with PKU was significantly higher (47.2 ± 9.7) than controls (29.4 ± 12.5, P < 0.001). Food neophobia was associated more with male gender (P = 0.039). Conclusions: Food neophobia is frequent in patients with PKU. It does not appear to be associated with female gender, with Phe levels and not just in children.

Burden of phenylketonuria in Latin American patients: a systematic review and meta-analysis of observational studies

“…Sixty-four of the 79 included studies reported at least one patient-important outcome at individual or population level, and they are displayed in Table 1 for study characteristics. Regarding study design, 18 were case series [ 22 , 23 , 33 , 34 , 40 – 42 , 45 , 54 , 55 , 58 , 60 , 70 , 84 – 87 ], 47 cross-sectional studies [ 6 , 7 , 13 – 15 , 17 – 21 , 24 , 26 , 29 , 30 , 35 – 39 , 43 , 46 , 47 , 53 , 56 , 59 , 61 , 62 , 67 , 68 , 71 , 73 , 75 – 77 , 79 – 81 , 88 – 91 ], 13 case reports [ 34 , 44 , 48 , 51 , 63 , 64 , 69 , 74 , 84 , 87 , 92 – 94 ], and two cohort studies [ 57 , 95 ].…”

“…3 A Osteopenia Silveira et al 2021 [ 71 ] $ Brazil 101 14.0 45.5 Classic (56.4) and mild (43.6) Patients with late diagnosis and patients diagnosed with tetrahydrobiopterin (BH4) deficiency Individual Overweight and obesity – Early NR NR NA Included in Fig. 4 A, and B Teruya, 2019 [ 79 , 82 ] Brazil 23 18 39.0 Classic (47.8) and mild (52.2) NA Not included as quantitative data on outcome of interest not provided in paper Patient adherence to clinical recommendations Poor current adherence to Phe-restricted diet; poor current median adherence to Phe-restricted diet Early (65.2%) Phe-restricted diet and special formula Up to 90 days after born Tonon et al 2019 [ 73 ] £ Brazil 25 19.3 52.0 Classic (52.0) and mild (48.0) NR Individual Overweight or obese – Early Phe-restricted diet and special formula 52.8** NA Not included as quantitative data on outcome of interest not provided in paper Vieira, 2010 [ 29 ] $ Brazil 5...…”

The Impact of the Quality of Nutrition and Lifestyle in the Reproductive Years of Women with PKU on the Long-Term Health of Their Children

“…Eating disorders : There is increasing evidence of eating disorders, food neophobia, and adverse attitudes towards food in adults with PKU [ 24 , 30 , 31 , 32 ]. Disordered eating refers to abnormal behaviours focused on eating or feeding, but it does not fit the pattern of a specific eating disorder [ 33 ].…”

Burden of phenylketonuria in Latin American patients: A systematic review and meta-analysis of observational studies

“…The case series and cross-sectional studies sample size ranged from two [21] to 420 patients [44,45]. Patients' ages ranged from a mean of 3.11 [35] to 19.3 [102] years old (Table 1).…”

“…Seven further studies[24, 26,31, 89,91,100,105] were published initially as a thesis followed by a full-text publication[25,27,32,90,92,101, 106]. When information regarding risk of bias or other aspects related to study criteria were unavailable in the methods, we attempted to contact study authors for additional information.Forty of the included studies were conducted inBrazil [18,20,[24][25][26][27][31][32][33]40,42,43,49,50,52,55,59, 60, 62, 63,[65][66][67][68][70][71][72][78][79][80][86][87][88][89][90][91][92][93][94][95]97,[99][100][101][102]105, 106], seven in Argentina[19, 22, 23, 28-30, 34, 44, 45, 57, 58], ve in Chile[35, 36, 38,39,77], three each in Colombia [46,53,...…”