Liking and sensory description of protein substitutes in phenyilketonuria subjects: A case-study in Northern and Southern Italy

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 to amino acid formulations. These sensory attributes should be considered during product development as a key factor influencing subjects\u2019 satisfaction

Exploring drivers of liking of low-phenylalanine products in subjects with phenyilketonuria using check-all-that-apply method

The aim of the present study was to apply the Check-all-that-apply (CATA) method in an ambulatory context involving subjects with phenylketonuria (PKU) to obtain a sensory description and to find the drivers of liking of low-phenylalanine products (Glycomacropeptide vs. L-amino acids formulas). 86 subjects with PKU (age range: 8-55 years) evaluated 8 samples: 4 L-amino acid formulas and 4 Glycomacropeptide (GMP) formulas, flavored with neutral, chocolate, strawberry and tomato aromas. Participants were asked to indicate which sensory attributes characterized each formulations and to score the overall liking. Significant differences were found regarding liking scores (F = 65.29; p < 0.001). GMP samples flavored with chocolate and strawberry, described as sweets, with a mild and natural taste and odor, were the most appreciated. Overall, GMP formulas obtained higher liking scores compared to L-amino acid formulas. Tomato flavored samples, described as bitter, salty, with artificial color, with strong taste and odor, obtained the lowest scores. In conclusion, CATA questionnaire seems to be a suitable method also in ambulatory context since this approach suggested that different foods and beverages with GMP could be developed to improve dietary treatment compliance of subjects with PKU from school age onwards

Early feeding practices in infants with phenylketonuria across Europe

Background: In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe. Methods: We sent a cross sectional, survey monkey (R) questionnaire to European health professionals working in IMD. It contained 31 open and multiple-choice questions. The results were analysed according to different geographical regions. Results: Ninety-five centres from 21 countries responded. Over 60% of centres commenced diet in infants by age 10 days, with 58% of centres implementing newborn screening by day 3 post birth. At diagnosis, infant hospital admission occurred in 61% of metabolic centres, mainly in Eastern, Western and Southern Europe. Breastfeeding fell sharply following diagnosis with only 30% of women still breast feeding at 6 months. 53% of centres gave pre-measured Phe-free infant formula before each breast feed and 23% alternated breast feeds with Phe-free infant formula. With standard infant formula feeds, measured amounts were followed by Phe-free infant formula to satiety in 37% of centres (n = 35/95), whereas 44% (n = 42/95) advised mixing both formulas together. Weaning commenced between 17 and 26 weeks in 85% centres, >= 26 weeks in 12% and <17 weeks in 3%. Discussion: This is the largest European survey completed on PKU infant feeding practices. It is evident that practices varied widely across Europe, and the practicalities of infant feeding in PKU received little focus in the PKU European Guidelines (2017). There are few reports comparing different feeding techniques with blood Phe control, Phe fluctuations and growth. Controlled prospective studies are necessary to assess how different infant feeding practices may influence longer term feeding development.Peer reviewe

Weaning practices in phenylketonuria vary between health professionals in Europe

Background: In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe. Methods: A cross sectional questionnaire (survey monkey (R)) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis. Results: Weaning started at 17-26 weeks in 85% (n=81/95) of centres, > 26 weeks in 12% (n=11/95) and 26 weeks. First solids were mainly low Phe vegetables (59%, n=56/95) and fruit (34%, n=32/95). A Phe exchange system to allocate dietary Phe was used by 52% (n=49/95) of centres predominantly from Northern and Southern Europe and 48% (n=46/95) calculated most Phe containing food sources (all centres in Eastern Europe and the majority from Germany and Austria). Some centres used a combination of both methods. A second stage Phe-free L-amino acid supplement containing a higher protein equivalent was introduced by 41% (n=39/95) of centres at infant age 26-36 weeks (mainly from Germany, Austria, Northern and Eastern Europe) and 37% (n=35/95) at infant age > 1y mainly from Southern Europe. 53% (n=50/95) of centres recommended a second stage Phe-free L-amino acid supplement in a spoonable or semi-solid form. Conclusions: Weaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development.Peer reviewe

Liking of low-phenylalanine products in Phenyilketonuria patients: a case study comparing subjects from North and South of Italy

Background A low and controlled dietary phenylalanine intakes is the mainstay of phenylketonuria therapy. Even if many amino acid mixtures have been developed these products are really poor liked by phenylketonuria patients. In this context, casein Glycomacropeptide (GMP) could be an interesting alternative protein source to usual amino acid mixtures. The aim of the present study was to evaluate sensory properties and liking of L-amino acid and GMP samples comparing patients from North and South of Italy. Methods 67 phenylketonuria patients (age range: 14-40 years), 34 admitted to San Paolo Hospital (Milan) and 33 admitted to A.O.U. Federico II of Naples, were recruited. Patients evaluated 8 low protein samples: 4 L-amino acid base samples and 4 GMP base samples, flavored with the same aromas (neutral, chocolate, strawberry and tomato) and with an equivalent protein intake of 5g/100ml. For each sample, participants were asked to indicate which sensory attributes characterize the formulations and to score the overall liking. Results The mean blood phenylalanine (SD) was 675,2 (307) and mean Body Mass Index was 23,4 (4,5). Significant differences were found between samples regarding liking scores in both groups (Milan: F= 42.48; p &lt;0.001; Naples: F=23.59, p &lt;0.001). GMP samples were generally preferred than amino acid mixtures, especially for samples flavored with chocolate and strawberry, described as sweets, with a mild and natural taste and odor. Patients from South of Italy gave significant higher liking scores to the amino acid base samples compared to patients from North suggesting different attitude toward products. Discussion The results of this study demonstrated that GMP-based formulas showed a greater acceptability compared to amino acid mixtures. In this context, different products could be developed using this cheese whey derived protein in order to improve compliance with dietary treatment of phenylketonuria patients

Sostituti proteici a base di aminoacidi vs glicomacropeptide: accettabilit&#224; e palatabilit&#224; in pazienti fenilchetonurici

Introduzione/background: Il trattamento della fenilchetonuria (PKU) prevede una dieta a basso e controllato apporto di fenilalanina (Phe). Lo scarso gradimento delle miscele aminoacidiche rappresenta un punto critico per la compliance. Lo scopo di questo studio \ue8 stato valutare propriet\ue0 sensoriali e gradimento di miscele di aminoacidi sintetici e formule a base di glicomacropeptide (GMP), proteina con basso contenuto di Phe, per selezionare le formulazioni pi\uf9 gradite. Metodi/pazienti: 53 pazienti PKU (et\ue0: 7-55 anni) hanno valutato in un\u2019unica seduta 8 campioni: 4 miscele di aminoacidi sintetici e 4 formule a base di GMP (Glytactin), aromatizzate con i medesimi aromi (neutro, cioccolato, pomodoro, fragola), e con apporto di proteine equivalenti pari a 5 g/100 ml. Per ciascun campione \ue8 stato chiesto di indicare quali descrittori, tra quelli proposti, caratterizzassero le formulazioni (CATA questionnaire) e il gradimento (scala lineare 100mm per adulti; scala figurata a 7 punti per bambini). Risultati: La Phe ematica media (DS) era di 256,7 (71,2) e di 501,6 (268,1) mol/L nei soggetti di et\ue0 inferiore e superiore a 12 anni, rispettivamente. Differenze significative sono state riscontrate tra i campioni per il gradimento (F: 28.06; p<0.001). I campioni di GMP aromatizzati con cioccolato e fragola, descritti come dolci, con gusto e odore delicato e naturale, sono risultati i pi\uf9 graditi. I campioni a base di aminoacidi sintetici sono risultati i meno graditi. I campioni al flavor pomodoro, descritti come salati, acidi, dal colore artificiale, con gusto e odore forte, hanno ottenuto i punteggi pi\uf9 bassi. Discussione: Dai risultati di questo studio emerge che le formule a base di GMP godono di una maggiore accettabilit\ue0 rispetto alle miscele aminoacidiche. Diversi alimenti e bevande, utilizzando prevalentamente gli aromi di cioccolato e fragola, potrebbero essere sviluppati partendo dal GMP per migliorare la compliance al trattamento dietetico dall\u2019et\ue0 scolare in poi

Early feeding practices in infants with phenylketonuria across Europe

Background: In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe

Weaning practices in phenylketonuria vary between health professionals in Europe

Background: In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe