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JIMD Reports, Volume 32

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Cover of 'JIMD Reports, Volume 32'

Table of Contents

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    Book Overview
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    Chapter 537 Newborn Screening Programmes in Europe, Arguments and Efforts Regarding Harmonisation: Focus on Organic Acidurias
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    Chapter 541 Whole Exome Sequencing Identifies the Genetic Basis of Late-Onset Leigh Syndrome in a Patient with MRI but Little Biochemical Evidence of a Mitochondrial Disorder
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    Chapter 547 Hydroxysteroid 17-Beta Dehydrogenase Type 10 Disease in Siblings
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    Chapter 553 Endurance Exercise Training in Young Adults with Barth Syndrome: A Pilot Study
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    Chapter 556 Newborn Screening for Vitamin B6 Non-responsive Classical Homocystinuria: Systematical Evaluation of a Two-Tier Strategy
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    Chapter 560 Establishing New Cut-Off Limits for Galactose 1-Phosphate-Uridyltransferase Deficiency for the Dutch Newborn Screening Programme
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    Chapter 561 Management of an LCHADD Patient During Pregnancy and High Intensity Exercise
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    Chapter 562 Rare Case of Hepatic Gaucheroma in a Child on Enzyme Replacement Therapy
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    Chapter 564 Reliable Diagnosis of Carnitine Palmitoyltransferase Type IA Deficiency by Analysis of Plasma Acylcarnitine Profiles
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    Chapter 566 Low Protein Formula: Consequences of Quantitative Effects of Pre-analytical Factors on Amino Acid Concentrations in Plasma of Healthy Infants
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    Chapter 567 Relationships Between Childhood Experiences and Adulthood Outcomes in Women with PKU: A Qualitative Analysis
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    Chapter 568 A Multiplatform Metabolomics Approach to Characterize Plasma Levels of Phenylalanine and Tyrosine in Phenylketonuria
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    Chapter 570 Japanese Male Siblings with 2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase Deficiency (HSD10 Disease) Without Neurological Regression
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    Chapter 571 The Effect of S-Adenosylmethionine on Self-Mutilation in a Patient with Lesch–Nyhan Disease
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    Chapter 572 Four Years of Diagnostic Challenges with Tetrahydrobiopterin Deficiencies in Iranian Patients
Attention for Chapter 566: Low Protein Formula: Consequences of Quantitative Effects of Pre-analytical Factors on Amino Acid Concentrations in Plasma of Healthy Infants
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Chapter title
Low Protein Formula: Consequences of Quantitative Effects of Pre-analytical Factors on Amino Acid Concentrations in Plasma of Healthy Infants
Chapter number 566
Book title
JIMD Reports, Volume 32
Published in
JIMD Reports, June 2016
DOI 10.1007/8904_2016_566
Pubmed ID
Book ISBNs
978-3-66-254384-9, 978-3-66-254385-6
Authors

Claude Bachmann, Alexander Kainz, Elisabeth Haschke-Becher

Editors

Eva Morava, Matthias Baumgartner, Marc Patterson, Shamima Rahman, Johannes Zschocke, Verena Peters

Abstract

Quantifying pre-analytical effects of postprandial sampling delay and daily protein intake on plasma amino acid concentrations in healthy infants fed formula with low protein content (1.8-1.9 g/100 kcal). Intake of formula with higher protein content bears a risk for later obesity (Kirchberg, J Clin Endocrinol Metab 100(1):149-158, 2015). Formulas containing less than 1.8 g protein might be adequate but not safe (Fomon, J Pediatr Gastroenterol Nutr 28:495-501, 1999). With on-demand feeding reproducible controls of indispensible amino acid concentration cannot be made at trough level. Data of 102 healthy infants aged 1 month and 79 aged 4 months fed formula with low protein content were obtained from a previous study (Haschke-Becher, J Inherit Metab Dis 39(1):25-37, 2016). They were analysed by multiple regression. Independent variables were the postprandial sampling delay from 2.25 to 4.5 h and the daily protein intake. Dependant variables were the amino acid concentrations. The combined effect was calculated with the natural logarithm of the amino acid concentration. Most amino acids fitted a significant exponential decrease due to the sampling delay, except of aspartate, citrulline, glutamine, glutamate, histidine, tryptophan and tyrosine at 1 month; and at 4 months except of citrulline, glutamine, glutamate, glycine and ornithine. Significant effects of protein intake were found for lysine and serine at 1 month and for glutamate at 4 months of age. Lowest limits of significant amino acid concentrations were calculated by extrapolation of sampling delay to 5 h and using the 10th percentile after back-transformation to μmol/L. A procedure to avoid the pitfall of overestimating amino acid concentration is presented.

Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 2 Mendeley readers of this research output. Click here to see the associated Mendeley record.

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Demographic breakdown

Readers by professional status Count As %
Professor > Associate Professor 1 50%
Student > Postgraduate 1 50%
Readers by discipline Count As %
Nursing and Health Professions 1 50%
Unknown 1 50%