LINKING AND SENSORY DESCRIPTION OF PROTEIN SUBSTITUTES IN PHENYLKETONURIA SUBJECTS: A CASE-STUDY IN NORTHERN AND SOUTHERN ITALY

Main Article Content

C. PROSERPIO
E. VERDUCI
I. SCALA
P. STRISCIUGLIO
J. ZUVADELLI
E. PAGLIARINI

Keywords

acceptability, CATA, food development, food formulations, odor, taste

Abstract

Nowadays, it is important to make effort to develop new formulations for subjects affected by rare diseases who need to follow a lifetime diet to maintain a good health.


The purpose of the study was to evaluate the acceptability and to obtain a sensory descriptive analysis of protein substitutes (glycomacropeptide GMP formulas vsL-amino acid formulas) involving subjects affected by phenylketonuria in Northern and  Southern Italy. Results demonstrated in both groups of subjects a  greater acceptability of GMP samples, characterized by sweet and mild taste, mild odor, and natural color, compared with amino acid formulations. These sensory attributes should be considered during product development as a key factor influencing subjects’ satisfaction.

Abstract 154 | pdf Downloads 170

References

Aguiar A., Ahring K., Almeida M.F., Assoun M., Quintana A.B., Bigot S., Caris A. et al. 2015. Practices in prescribing protein substitutes for PKU in Europe: no uniformity of approach. Mol. Genet. Metab. 115(1):17-22.

Blau N. 2016. Genetics of phenylketonuria: then and now. Hum Mutat. 37(6):508-515.

Cox D. N., Hendrie G.A. and Carty, D. 2015. Sensitivity, hedonics and preferences for basic tastes and fat amongst adults and children of differing weight status: a comprehensive review. Food Qual. and Preference, 41, 112-120.

Feillet F. and Agostoni C. 2010. Nutritional issues in treating phenylketonuria. J Inherit Metab Dis. 33(6):659-664.

Giovannini M., Verduci E., Salvatici E., Paci S. and Riva E. 2012. Phenylketonuria: nutritional advances and challenges. Nutr Metab. 9(1):7.

ISTAT. Indagine Multiscopo “Aspetti della vita quotidiana-2015”. http://dati.istat.it.

Jaeger S.R., Beresford M.K., Paisley A.G., Antúnez L., Vidal L., Cadena R.S., Ares G. et al. 2015.Check-all-that-apply (CATA) questions for sensory product characterization by consumers: Investigations into the number of terms used in CATA questions. Food Qual. Prefer. 42:154-164.

Köster E.P. 2009. Diversity in the determinants of food choice: A psychological perspective. Food Qual. Prefer. 20(2):70-82.

Lawless H.T., Popper R. and Kroll BJ. 2010. A comparison of the labeled magnitude (LAM) scale, an 11-point category scale and the traditional 9-point hedonic scale. Food Qual. Prefer. 21(1):4-12.

LimK., van Calcar S.C., Nelson K.L., Gleason S.T. and Ney D.M. 2007. Acceptable low-phenylalanine foods and beverages can be made with glycomacropeptide from cheese whey for individuals with PKU.Mol.Genet.Metab. 92(1):176-178.

MacDonald A., Gokmen-Ozel H., van Rijn M. and Burgard P. 2010. The reality of dietary compliance in the management of phenylketonuria. J. Inherit. Metab. Dis. 33(6):665-670.

Ney D.M., Gleason S.T., Van Calcar S.C., MacLeod E.L., Nelson K.L., Etzel, M.R., Wolff J. et al. A. 2009. Nutritional management of PKU with glycomacropeptide from cheese whey. J. Inherit. Metab. Dis. 32(1):32-39.

Meyners M., Castura J.C. and Carr B.T. 2013. Existing and new approaches for the analysis of CATA data. Food Qual. Prefer. 30(2):309-319.

Plaehn D. 2012. CATA penalty/reward. Food Qual. and Prefer. 24:141-152.

Proserpio C., Pagliarini E., Zuvadelli J., Paci S., Re Dionigi A., Banderali G., Verduci E. et al. 2018. Exploring drivers of liking of low-phenylalanine products in subjects with phenyilketonuria using check-all-that-apply method. Nutrients. 10(9):1179.

Ruggiero G.M., Hannöver W., Mantero M. and Papa R. 2000. Body acceptance and culture: A study in northern and southern Italy. Eur. Eat. Disorders Rev. 8(1):40-50.

Solverson P., Murali S.G., Brinkman A.S., Nelson D.W., Clayton M.K., Yen C.L.E. and Ney D.M. 2012. Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria. Am. J. Physiol. Endocrinol. Metab. 302(7):885-895.

Van Calcar S.C. and Ney D.M. 2012. Food products made with glycomacropeptide, a low-phenylalanine whey protein, provide a new alternative to amino acid–based medical foods for nutrition management of phenylketonuria. J. Acad. Nutr. Diet. 112(8):1201-1210.

Van Spronsen F.J. and Burgard P. 2008. The truth of treating patients with phenylketonuria after childhood: the need for a new guideline. J. Inherit. Metab Dis.31(6):673-679.

van Spronsen F.J., van Wegberg A.M., Ahring K., Bélanger-Quintana A., Blau N., Bosch A.M., Huijbregts S.C. et al. 2017. Key European guidelines for the diagnosis and management of patients with phenylketonuria. Lancet. Diabetes Endocrinol. 5:743-756.

Varela P. and Ares G. 2012. Sensory profiling, the blurred line between sensory and consumer science. A review of novel methods for product characterization. Food Res. Int. 48(2):893-908.