Rationale for the German recommendations for phenylalanine level control in phenylketonuria 1997

Burgard, Peter; Bremer, H. J.; Bührdel, P; Clemens, P.; Mönch, E; Przyrembel, Hildegard; Trefz, Friedrich K.; Ullrich, Kurt

doi:10.1007/s004310051008

Cited by 129 publications

(83 citation statements)

References 78 publications

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“…In addition brain Phe levels Ͻ200 M seem to allow a normal cognitive development. Our results support the recommendations of the German Working Group for Metabolic Diseases from 1999, supposing that only patients with HPA caused by phenylalanine hydroxylase (PAH) deficiency and serum Phe levels of Ն600 M on a free nutrition must be treated (2). Because of a possible late-onset PKU, untreated patients should be monitored very carefully at least during the first year of life (4).…”

Section: Discussionsupporting

confidence: 84%

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Normal Clinical Outcome in Untreated Subjects with Mild Hyperphenylalaninemia

et al. 2001

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Section: Discussionsupporting

confidence: 84%

“…In their study they included patients with Phe levels Ͻ900 M. Most German patients with mild HPA are not treated, as a normal outcome was anticipated (2,3).…”

mentioning

confidence: 99%

Normal Clinical Outcome in Untreated Subjects with Mild Hyperphenylalaninemia

et al. 2001

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“…This includes even those that are not typically regarded as protein providers, such as fruits and vegetables. It is therefore common practice in Germany and many other, but not all, countries to calculate phe intake from all foods to achieve tolerable phe plasma concentrations (Burgard et al, 1999;Stemerdink et al, 2000;Anon, 2001).…”

Section: Introductionmentioning

confidence: 99%

Unrestricted consumption of fruits and vegetables in phenylketonuria: no major impact on metabolic control

Rohde¹,

Mütze²,

Weigel³

et al. 2012

Eur J Clin Nutr

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Background/Objectives: The treatment of phenylketonuria (PKU) requires consistent restriction of protein intake from natural sources. Therefore, protein from all foods has to be accounted for, even the small amounts in fruits and vegetables. We studied whether free consumption of fruits and vegetables containing less than 75 mg phenylalanine (phe) per 100 g affects metabolic control in children with PKU. Subjects/Methods: Fourteen children (2-10 years) were included in a cross-over study, with a two-week period of conventional treatment (accounting for protein from fruits and vegetables) and a two-week period with free fruit and vegetable consumption. The instruction to follow liberal fruit and vegetable consumption in the first or second study period was randomized. Detailed daily dietary records were obtained throughout the study. Phe and nutrient content was calculated. Dried-blood phe concentration was monitored daily. Results: Although total phe intake increased by an average of 58 mg per day (P ¼ 0.037) during the 2 weeks of free fruit and vegetable consumption, dried-blood phe concentrations were unchanged. Total intake of fruits and vegetables did not increase, but patients instead used the higher phe tolerance to consume more of other foods, which were calculated and accounted for. Conclusion: Free consumption of fruits and vegetables does not impair metabolic control in PKU patients over a 2-week period.

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“…Several collaborative PKU studies demonstrated a continual increase in mean Phe levels in those older than 4 years of age in relation to liberalization of diet restrictions and decreased frequency of blood Phe monitoring (Smith and Beasley 1989;Wendel et al 1990;Burgard et al 1997). Among an observational study of 330 patients under 20 years of age, 30% of blood Phe levels were above the maximum recommended value in those less than 4 years, 50% in those 5 to 10 years, and 80% in those 15 to 19 years of age .…”

Section: Introductionmentioning

confidence: 99%

Correlation of age‐specific phenylalanine levels with intellectual outcome in patients with phenylketonuria

Viau

Wengreen

Ernst

et al. 2011

J of Inher Metab Disea

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This study includes data collected from a retrospective chart review of patients with phenylketonuria treated at the University of Utah Metabolic Clinic. Among the approximately 175 patients treated for phenylketonuria at this clinic, 55 patients (28 female) met the inclusion criteria for the present study. Based on the diagnostic Phe level, 66.7% were classified as having classic phenylketonuria, 18.5% with moderate phenylketonuria, and 14.8% with mild phenylketonuria.

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Rationale for the German recommendations for phenylalanine level control in phenylketonuria 1997

Cited by 129 publications

References 78 publications

Normal Clinical Outcome in Untreated Subjects with Mild Hyperphenylalaninemia

Normal Clinical Outcome in Untreated Subjects with Mild Hyperphenylalaninemia

Unrestricted consumption of fruits and vegetables in phenylketonuria: no major impact on metabolic control

Correlation of age‐specific phenylalanine levels with intellectual outcome in patients with phenylketonuria

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