Nutrient intake and food consumption of adolescents and young adults with phenylketonuria

Schulz, Benjamin L.; Bremer, H. J.

doi:10.1111/j.1651-2227.1995.tb13748.x

Cited by 48 publications

(31 citation statements)

References 17 publications

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“…Likewise, the fat supply did not change compared to classical dietary treatment, but stayed below the recommendations (DACH 2000). A low fat intake in PKU patients under Phe restricted diet has already been described (Schulz andBremer 1995, Rohde et al 2012). Many of the protein rich foods, which must be avoided in the PKU diet, are simultaneously sources rich in fat.…”

Section: Discussionmentioning

confidence: 99%

Nutritional Changes and Micronutrient Supply in Patients with Phenylketonuria Under Therapy with Tetrahydrobiopterin (BH4)

et al. 2012

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Background: Since 2008 patients with BH 4 -sensitive phenylketonuria can be treated with sapropterin dihydrochloride (Kuvan®) in addition to the classic phenylalanine (Phe) restricted diet. The aim of this study was to evaluate the nutritional changes and micronutrient supply in patients with phenylketonuria (PKU) under therapy with tetrahydrobiopterin (BH 4 ).Subjects and Methods: 19 children with PKU (4-18 years) and potential BH 4 -sensitivity were included, 14 completed the study protocol. Dried blood Phe concentrations as well as detailed dietary records were obtained throughout the study at preassigned study days.Results: Eight patients could increase their Phe tolerance from 629 AE 476 mg to 2131 AE 1084 mg (P ¼ 0.006) under BH 4 while maintaining good metabolic control (Phe concentration in dried blood 283 AE 145 mM vs. 304 AE 136 mM, P ¼ 1.0), therefore proving to be BH 4 -sensitive. They decreased their consumption of special low protein products and fruit while increasing their consumption of high protein foods such as processed meat, milk and dairy products. Intake of vitamin D (P ¼ 0.016), iron (P ¼ 0.002), calcium (P ¼ 0.017), iodine (P ¼ 0.005) and zinc (P ¼ 0.046) significantly declined during BH 4 treatment while no differences in energy and macronutrient supply occurred.Conclusion: BH 4 -sensitive patients showed good metabolic control under markedly increased Phe consumption. However, the insufficient supply of some micronutrients needs consideration. Long-term multicenter settings with higher sample sizes are necessary to investigate the changes of nutrient intake under BH 4 therapy to further evaluate potential risks of malnutrition. Supplementation may become necessary.

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Section: Discussionmentioning

confidence: 99%

Nutritional Changes and Micronutrient Supply in Patients with Phenylketonuria Under Therapy with Tetrahydrobiopterin (BH4)

et al. 2012

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“…This recommendation, at that time very strict, can nowadays be found in general recommendations for treating PKU [15,25]. Details of the design and protocol as well of results have been reported elsewhere [3,5,19,[21][22][23]28] and in the present issue of the European Journal of Pediatrics. The data presented in this paper concentrate on the development of intelligence of the patients from 4 "to 9 years of life in relation to their blood Phe levels during the first 9 years of life.…”

Section: Introductionmentioning

confidence: 95%

Intellectual development of the patients of the German Collaborative Study of children treated for phenylketonuria

et al. 1996

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In a multicentric and interdisciplinary approach the German Collaborative Study of Children Treated for Phenylketonuria (PKU) investigates prospectively the effects of early started strict dietary treatment on the growth and development of 140 patients. The present paper focuses on longitudinal intelligence data from 4, 5 and 9 years of age of 89 patients in relation to the quality of dietary control in comparison to 200 healthy children tested by the same protocol. Cluster analysis of phenylalanine (Phe) levels distinguished a cluster of good dietary control with Phe levels according to the recommendation of maintaining Phe levels below 360 mumol/l, a cluster of poor dietary control with Phe levels greater than 600 mumol/l after the age of 3 years, and a cluster of intermediate control. Intelligence quotients (IQ) and Phe clusters were inversely related with non-significant differences between the clusters good and intermediate. On average, all three clusters scored significantly lower than healthy age peers. Mean IQ scores decreased for patients as well as for healthy children due to different tests used at different measurement occasions. Patients with poor dietary control showed a steeper decrease between 4 and 5 years than patients with better dietary control. Between 5 and 9 years IQ differences between patients and healthy children remained stable. Verbal IQs were higher than performance IQs for patients as well as for healthy children. It is concluded that after early and strict treatment during the pre-school years Phe levels, in the range observed, do not influence IQ development until the age of 9 years. IQ subscale pattern indicate that PKU results in a generalized reduction of IQ instead of disturbing specific abilities. It remains to be investigated whether higher Phe levels are also innocuous and/or may result in late effects.

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“…Some AA products are devoid of vitamin and mineral supplements to improve taste and acceptability with the assumption that separate vitamin and mineral supplements will be taken each day. Nutrient intake, specifically of vitamin B 6 , B 12 [28] , calcium, folate and iron [29] will be deficient if the AA formula and/or vitamin and mineral supplements are not consumed each day. Interestingly, even with adequate iron intake, studies have shown iron deficiency in those with PKU [30] .…”

Section: Vitamin Mineral and Essential Fatty Acid Statusmentioning

confidence: 99%

Nutritional Management of Phenylketonuria

MacLeod

Ney²

2010

Ann Nestlé [Engl]

119

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Phenylketonuria (PKU) is caused by deficient activity of the enzyme phenylalanine hydroxylase, needed to convert the essential amino acid (AA) phenylalanine (phe) to tyrosine. In order to prevent neurological damage, lifelong adherence to a low-phe diet that is restricted in natural foods and requires ingestion of a phe-free AA formula to meet protein needs is required. The goal of nutritional management for those with PKU is to maintain plasma phe concentrations that support optimal growth, development, and mental functioning while providing a nutritionally complete diet. This paper reviews developing a lifelong dietary prescription for those with PKU, outcomes of nutritional management, compliance with the low-phe diet across the life cycle, and new options for nutritional management. An individualized dietary prescription is needed to meet nutrient requirements, and the adequacy of phe intake is monitored with assessment of blood phe levels. Elevated phe concentrations may occur due to illness, excessive or inadequate phe intake, or inadequate intake of AA formula. Although normal growth and development occurs with adherence to the low-phe diet, it is important to monitor vitamin, mineral and essential fatty acid status, especially in those who do not consume sufficient AA formula. Given the growing population of adults with PKU, further research is needed to understand the risks for developing osteoporosis and cardiovascular disease. There are promising new options to liberalize the diet and improve metabolic control such as tetrahydrobiopterin therapy or supplementation with large neutral AAs. Moreover, foods made with glycomacropeptide, an intact protein that contains minimal phe, improves the PKU diet by offering a palatable alternative to AA formula. In summary, continued efforts are needed to overcome the biggest challenge to living with PKU – lifelong adherence to the low-phe diet.

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Nutrient intake and food consumption of adolescents and young adults with phenylketonuria

Cited by 48 publications

References 17 publications

Nutritional Changes and Micronutrient Supply in Patients with Phenylketonuria Under Therapy with Tetrahydrobiopterin (BH4)

Nutritional Changes and Micronutrient Supply in Patients with Phenylketonuria Under Therapy with Tetrahydrobiopterin (BH4)

Intellectual development of the patients of the German Collaborative Study of children treated for phenylketonuria

Nutritional Management of Phenylketonuria

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