Serum Lipid Concentrations in Subjects With Phenylketonuria and Their Families

DeClue, Terry; Davis, Jim; Schocken, Dawn; Kangas, Ruth; Benford, S A

doi:10.1001/archpedi.1991.02160110058020

Cited by 4 publications

(3 citation statements)

References 5 publications

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“…Enriching low protein breads and pasta with fiber should not only prevent future gastrointestinal problems which are associated with a low fiber intake but furthermore would achieve a longer feeling of satiety. The low fat content, high P/S ratio and extremely low cholesterol content are typical of the PKU diet (15,16). Our study confirmed this for adolescent and adult patients.…”

Section: Vitamins and Mineralsmentioning

confidence: 99%

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

Schulz

Bremer

1995

Acta Paediatrica

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Food and nutrient intake was assessed in 99 PKU patients (12-29 years old) by two food protocols (7 days and 4 days, respectively). Ninety-three patients completed at least one 7-day food record and 83 both records. Nineteen of 93 patients had already stopped taking the phenylalanine-free amino acid mixture (AAM), which is enriched with vitamins, minerals and trace elements. Plasma phenylalanine levels in this group were significantly higher than in patients who were still taking the AAM. Even without the AAM, protein intake still met the recommendations, but thiamin, riboflavin, folate, calcium and iron levels were below 80% of the US RDA in most patients. For those still taking the AAM, calorie, protein, vitamin and mineral intakes were above the recommendations. The diet was characterized by a low intake of fiber (median 14 (range 8-35) g/day), fat (27 (10-47) cal%) and cholesterol (75 (13-417) mg/day) as well as a high ratio of polyunsaturated/saturated fatty acids (0.7 (0.2-2.4)). Problems with dietary compliance in adolescents and young adults may lead to a combination of marginal nutrient intake and high phenylalanine levels.

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Section: Vitamins and Mineralsmentioning

confidence: 99%

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

Schulz

Bremer

1995

Acta Paediatrica

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“…The plasma lipid profile was reported in some studies with contradictory results. TCh level and lipoproteins were found to be lower compared to controls in some studies (Galluzzo et al ., ; Agostoni et al ., ) with an increase in plasma TG level (Schulpis & Scarpalezou, ; Colome et al ., ; Moseley et al ., ), whereas no difference was found in mean plasma TCh and lipoproteins in other studies (DeClue et al ., ; Galluzzo et al ., ; Hvas et al ., ; Schulpis et al ., , ). However, the data available so far are scarce with respect to the plasma lipid profile in patients with IEPM other than PKU.…”

Section: Discussionmentioning

confidence: 99%

Cardiovascular disease biomarkers in patients with inborn errors of protein metabolism: a pilot study

Karam

Majdalani

Daher

et al. 2014

J Human Nutrition Diet

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“…S128 The low Phe formula is replaced by a more concentrated source of amino acids towards the end of the 1 st year. Figure 1 shows the protein (and therefore Phe) as a percentage of energy intake from a series of studies carried out in different parts of the world with ages ranging from 0.6 to 30 years [10,15,24]. All studies except the Australian study show that PKU individuals had a lower protein intake than controls.…”

Section: Practical Management Strategiesmentioning

confidence: 99%

Recommendations for protein and amino acid intake in phenylketonuric patients

Cockburn

Clark

1996

Eur J Pediatr

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The methods for the determination of protein requirements are reviewed and the difficulties in achieving the recommendations of the dietary management of phenylketonuria proposed by a Medical Research Council Working Party on Phenylketonuria using currently available low phenylalanine (Phe) protein substitutes and low protein foods are examined. These recommendations are that all infants whose blood Phe concentrations exceed 600 mumol/l in the presence of a normal or low plasma tyrosine and an otherwise normal plasma amino acid profile while receiving a normal protein intake (2-3 g/kg/day), should start a low Phe diet immediately. Infants whose blood Phe concentrations remain persistently between 400 and 600 mumol/l for more than a few days should also start treatment. The diet should contain a protein substitute which is Phe free (or at least very low in Phe) and otherwise nutritionally complete with a composition sufficient to provide 100-120 mg/kg per day of tyrosine and a total amino acid intake of at least 3 g/kg per day in children under 2 years of age. In children over 2 years the intake of amino acids should be maintained at a level of 2 g/kg per day. The protein substitute should be spread as evenly as possible through the 24 h. Blood Phe concentrations should be maintained between 120 and 360 mumol/l. In children aged over 10 years it is suggested that the protein substitute should supply the protein reference nutrient intake + 50%. An upper blood Phe limit of 480 mumol/l rather than 360 mumol/l may be acceptable in school age children. Adults and adolescents should continue treatment with the aim to maintain blood Phe concentrations no higher than 700 mumol/l. During the period before conception and during pregnancy women should aim to have plasma Phe concentrations between 60-250 mumol/l.

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Serum Lipid Concentrations in Subjects With Phenylketonuria and Their Families

Cited by 4 publications

References 5 publications

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

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

Cardiovascular disease biomarkers in patients with inborn errors of protein metabolism: a pilot study

Recommendations for protein and amino acid intake in phenylketonuric patients

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