Nutrition status of adults with phenylketonuria treated with pegvaliase

Viau, Krista; Wessel, Ann; Martell, Leslie; Sacharow, Stephanie; Rohr, Fran

doi:10.1016/j.ymgme.2021.06.002

Cited by 22 publications

(19 citation statements)

References 33 publications

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“…Sapropterin can liberate a woman's diet and increase natural food sources and nutrient intake in sub-groups of responsive women, but education and careful monitoring is needed, as changes in food patterns may have a negative impact on nutrient adequacy [32,103].…”

Section: Use Of Sapropterinmentioning

confidence: 99%

“…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 ].…”

Section: Nutritional Vulnerability Of Women With Pku In Their Reprodu...mentioning

confidence: 99%

“…Bilder et al reported that 3.4% of patients ( n = 128/3714) with PKU had an eating disorder compared with 0.9% in the general population [ 31 ]. Viau et al discussed that 53% of adults ( n = 9/18) on pegvaliase therapy had food neophobia with low enjoyment of food which did not appear to improve with a relaxed protein intake [ 32 ]. Luu et al [ 33 ] found that in a group of adults with PKU ( n = 15) aged 12–35 y, patients with poor metabolic control had symptoms of disordered eating at a higher frequency than those with good metabolic control.…”

Section: Nutritional Vulnerability Of Women With Pku In Their Reprodu...mentioning

confidence: 99%

“…Women may have unpleasant memories of the taste, smell, and texture of protein substitute from childhood, or they may associate it with causing gastrointestinal symptoms such as reflux and constipation [ 24 ]. The absence of protein substitute intake may lead to the thinning of hair and poor skin condition associated with inadequate nutritional status [ 32 ]. There are reports of reduced or low normal serum urea levels [ 40 ].…”

Section: Nutritional Vulnerability Of Women With Pku In Their Reprodu...mentioning

confidence: 99%

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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

et al. 2022

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A woman’s nutritional status before and during pregnancy can affect the health of her progeny. Phenylketonuria (PKU), a rare disorder causing high blood and brain phenylalanine (Phe) concentrations, is associated with neurocognitive disability. Lifelong treatment is mainly dietetic with a Phe-restricted diet, supplemented with a low-Phe protein substitute. Treatment adherence commonly decreases in adolescence, with some adults ceasing dietary treatment. In maternal PKU, elevated blood Phe is harmful to the fetus so a strict Phe-restricted diet must be re-established preconception, and this is particularly difficult to achieve. A woman’s reproductive years introduces an opportunity to adopt healthier behaviours to prepare for successful pregnancies and positive health outcomes for both themselves and their children. Several factors can influence the health status of women with PKU. Political, socioeconomic, and individual food and lifestyle choices affect diet quality, metabolic control, and epigenetics, which then pre-condition the overall maternal health and long-term health of the child. Here, we reflect on a comprehensive approach to treatment and introduce practical recommendations to optimize the wellbeing of women with PKU and the resultant health of their children.

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Section: Use Of Sapropterinmentioning

confidence: 99%

Section: Nutritional Vulnerability Of Women With Pku In Their Reprodu...mentioning

confidence: 99%

Section: Nutritional Vulnerability Of Women With Pku In Their Reprodu...mentioning

confidence: 99%

Section: Nutritional Vulnerability Of Women With Pku In Their Reprodu...mentioning

confidence: 99%

See 2 more Smart Citations

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

et al. 2022

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“…There was greater variation in participant age for studies including biochemical measurements of protein status than anthropometric measurements of protein studies. Six studies were conducted in children (age range 13.7 days to 10 years) [33,38,42,84,85,89], ten studies were in children and adolescents (age range 2.4 monthse18 years) [44,56,62,69,74,76,77,83,86,90], 15 in adolescents and adults (11e49 years) [7,39,40,45,46,57,63,64,70,72,75,79,91e93] and two in adults only [41,82]. Seven studies included mixed-age cohorts (age range 7 monthse54 years) [26,36,37,80,81,94,95].…”

Section: Study Characteristicsmentioning

confidence: 99%