Literature DB >> 17726313

Effects of various dietary amino acid preparations for phenylketonuric patients on the metabolic profiles along with postprandial insulin and ghrelin responses.

C Weigel1, M Rauh, C Kiener, W Rascher, I Knerr.   

Abstract

AIM: We investigated the metabolic profiles along with insulin and ghrelin responses following ingestion of various amino acid (AA) substitutes commonly used in the treatment of phenylketonuria to study the effects of added macronutrients.
METHODS: Twenty healthy and 6 phenylketonuric adults ingested AA mixtures with or without carbohydrates and fat (Anamix, Easiphen, or p-am 3; 0.35 g AA/kg body weight); milk powder shakes were used for control purposes. Serum AA, glucose, urea, insulin, and ghrelin were measured over 5 h.
RESULTS: Peak AA concentrations were achieved at around 60 min postprandially for supplemented AA powders and control shakes, significantly later than for pure AA. Of interest, the mean Phe/Tyr ratio declined by 40-50% in phenylketonuric patients following intake of Easiphen, Anamix, or p-am 3. The insulin peaks, up to 500% as compared with baseline, occurred at 30 min and were approximately 100% higher after intake of AA plus macronutrients. Glucose and urea remained constant. Ghrelin showed a nadir at 60 min, followed by a rise leading to a 30% increase of initial concentrations for pure AA as compared with more constant levels for preparations with macronutrients.
CONCLUSION: An oral AA bolus together with macronutrients retards hyperaminoacidemia, displays a higher insulin secretion, normoglycemia, and more stable ghrelin concentrations, whereas the pure AA tested here exerted weaker anabolic effects. Copyright (c) 2007 S. Karger AG, Basel.

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Year:  2007        PMID: 17726313     DOI: 10.1159/000107678

Source DB:  PubMed          Journal:  Ann Nutr Metab        ISSN: 0250-6807            Impact factor:   3.374


  6 in total

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Authors:  Erin L MacLeod; Murray K Clayton; Sandra C van Calcar; Denise M Ney
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2.  Carbohydrate status in patients with phenylketonuria.

Authors:  María L Couce; Paula Sánchez-Pintos; Isidro Vitoria; María-José De Castro; Luís Aldámiz-Echevarría; Patricia Correcher; Ana Fernández-Marmiesse; Iria Roca; Alvaro Hermida; Miguel Martínez-Olmos; Rosaura Leis
Journal:  Orphanet J Rare Dis       Date:  2018-06-27       Impact factor: 4.123

3.  Amino Acid Plasma Profiles from a Prolonged-Release Protein Substitute for Phenylketonuria: A Randomized, Single-Dose, Four-Way Crossover Trial in Healthy Volunteers.

Authors:  Mika Scheinin; Anna Barassi; Jouni Junnila; Zsófia Lovró; Giorgio Reiner; Essi Sarkkinen; Anita MacDonald
Journal:  Nutrients       Date:  2020-06-02       Impact factor: 5.717

4.  Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria.

Authors:  Anne Daly; Sharon Evans; Alex Pinto; Richard Jackson; Catherine Ashmore; Júlio César Rocha; Anita MacDonald
Journal:  Nutrients       Date:  2020-08-14       Impact factor: 5.717

5.  Optimising amino acid absorption: essential to improve nitrogen balance and metabolic control in phenylketonuria.

Authors:  Anita MacDonald; Rani H Singh; Júlio César Rocha; Francjan J van Spronsen
Journal:  Nutr Res Rev       Date:  2018-10-04       Impact factor: 7.800

6.  Weaning practices in phenylketonuria vary between health professionals in Europe.

Authors:  A Pinto; S Adams; K Ahring; H Allen; M F Almeida; D Garcia-Arenas; N Arslan; M Assoun; Y Atik Altınok; D Barrio-Carreras; A Belanger Quintana; S M Bernabei; C Bontemps; F Boyle; G Bruni; M Bueno-Delgado; G Caine; R Carvalho; A Chrobot; K Chyż; B Cochrane; C Correia; K Corthouts; A Daly; S De Leo; A Desloovere; A De Meyer; A De Theux; B Didycz; M E Dijsselhof; K Dokoupil; J Drabik; C Dunlop; W Eberle-Pelloth; K Eftring; J Ekengren; I Errekalde; S Evans; A Foucart; L Fokkema; L François; M French; E Forssell; C Gingell; C Gonçalves; H Gökmen Özel; A Grimsley; G Gugelmo; E Gyüre; C Heller; R Hensler; I Jardim; C Joost; M Jörg-Streller; C Jouault; A Jung; M Kanthe; N Koç; I L Kok; T Kozanoğlu; B Kumru; F Lang; K Lang; I Liegeois; A Liguori; R Lilje; O Ļubina; P Manta-Vogli; D Mayr; C Meneses; C Newby; U Meyer; S Mexia; C Nicol; U Och; S M Olivas; C Pedrón-Giner; R Pereira; K Plutowska-Hoffmann; J Purves; A Re Dionigi; K Reinson; M Robert; L Robertson; J C Rocha; C Rohde; S Rosenbaum-Fabian; A Rossi; M Ruiz; J Saligova; A Gutiérrez-Sánchez; A Schlune; K Schulpis; J Serrano-Nieto; A Skarpalezou; R Skeath; A Slabbert; K Straczek; M Giżewska; A Terry; R Thom; A Tooke; J Tuokkola; E van Dam; T A M van den Hurk; E M C van der Ploeg; K Vande Kerckhove; M Van Driessche; A M J van Wegberg; K van Wyk; C Vasconcelos; V Velez García; J Wildgoose; T Winkler; J Żółkowska; J Zuvadelli; A MacDonald
Journal:  Mol Genet Metab Rep       Date:  2018-11-25
  6 in total

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