Correction: Corrigendum: Phenylalanine hydroxylase deficiency: diagnosis and management guideline

Phenylketonuria is an inborn error of metabolism that historically has been treated with a strict phenylalanine-restricted diet where all foods are weighed and measured. This is cumbersome and difficult for patients and caregivers, especially patients with high phenylalanine blood concentrations who often have neurocognitive deficits. The Simplified Diet is an alternative approach that allows for increased flexibility, promotes healthy food choices, and is easier to manage than a traditional diet for PKU. This paper describes the implementation of the Simplified Diet and outlines education, counseling strategies, and challenges encountered by three metabolic clinics in the United States.

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Multiclinic Observations on the Simplified Diet in PKU

Bernstein

Burns

Sailer-Hammons

et al. 2017

Journal of Nutrition and Metabolism

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Morning specimen is not representative of metabolic control in Tunisian children with phenylketonuria: a repeated cross-sectional study

Abdelaziz

Tangour

Chehida

et al. 2020

Journal of Pediatric Endocrinology and Metabolism

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Objective and methodsTo evaluate variation of capillary phenylalanine concentrations over the day in patients treated for phenylketonuria and the reliability of the morning sample to assess metabolic control, we conducted a repeated cross-sectional study in 25 Tunisian patients on phenylalanine-low diet. For each patient, we collected nine capillary samples over the day. Phenylalanine was dosed by fluorimetry.ResultsThere was a wide variability of phenylalanine concentrations over the day (p<0.001). Compared to morning sample, phenylalanine concentration was significantly lower before lunch (p=0.038), after lunch (p=0.025), before dinner (p<0.001), after dinner (p=0.035) and at 4:00 a.m. (p=0.011). Compared to the 24 h sampling, the morning sample had a 68% to identify unbalanced patients. 60% of patients, had peak phenylalanine concentration after the morning. Half of the patients with normal morning phenylalanine concentration had low phenylalanine values over 8–20 h. Percentages of high phenylalanine concentrations over the last semester were higher in patients with poor metabolic control over the 24 h (21% ± 43 vs. 0% ± 9%); p=0.043.ConclusionA single morning sample gives an incomplete information on metabolic control in phenylketonuric patients. Using four pre-prandial samples on the day should be considered as alternative in patients with good metabolic control.

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Correction: Corrigendum: Phenylalanine hydroxylase deficiency: diagnosis and management guideline

Cited by 2 publications

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Multiclinic Observations on the Simplified Diet in PKU

Multiclinic Observations on the Simplified Diet in PKU

Morning specimen is not representative of metabolic control in Tunisian children with phenylketonuria: a repeated cross-sectional study

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