Search

Your search keyword '"A. Skarpalezou"' showing total 29 results
Start Over Author "A. Skarpalezou"
29 results on '"A. Skarpalezou"'

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

Author

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, and A. MacDonald

Subjects
Medicine (General), R5-920, Biology (General), QH301-705.5
Abstract

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®) 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. Keywords: Weaning, Infant, Phenylketonuria, Phenylalanine, Phe-free infant formula

Published
2019
Full Text
View/download PDF

2. Early feeding practices in infants with phenylketonuria across Europe

Author

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, and A. MacDonald

Subjects
Medicine (General), R5-920, Biology (General), QH301-705.5
Abstract

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® 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

Published
2018
Full Text
View/download PDF

3. Low Glycemic Index Diet for Epilepsy. A liberalized Ketogenic Diet or A Different Nutritional Therapeutical Process? Facts and Hypothesis

Author

Eftherpi Dalpa, Martha Spilioti, Astrinia Skarpalezou, Dimitrios Tsiptsios, Athanasios Evangeliou, Irene Katsanika, Olga Grafakou, and Maria Papadopoulou

Subjects
Pediatrics, medicine.medical_specialty, business.industry, medicine.medical_treatment, social sciences, General Medicine, Carbohydrate metabolism, medicine.disease, Low glycemic index, Epilepsy, Glycemic index, medicine, Ketone bodies, business, Intractable seizures, Ketogenic diet
Abstract

Over the last decades, the ketogenic diet (KD) has become a valuable weapon in the management of intractable seizures [1,2]. However not all of patients truly benefit from this therapy.

Published
2019

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

Author

Pinto, A., Adams, S., Ahring, K., Allen, H., Almeida, M.F., Garcia-Arenas, D., Arslan, N., Assoun, M., Altinok, Y. Atik, Barrio-Carreras, D., Quintana, A. Belanger, Bernabei, S.M., Bontemps, C., Boyle, F., Bruni, G., Bueno-Delgado, M., Caine, G., Carvalho, R., Chrobot, A., Chyz, K., Cochrane, B., Correia, C., Corthouts, K., Daly, A., Leo, S. De, Desloovere, A., Meyer, A, Theux, A. De, Didycz, B., Dijsselhof, M.E., Dokoupil, K., Drabik, J., Dunlop, C., Eberle-Pelloth, W., Eftring, K., Ekengren, J., Errekalde, I., Evans, S., Foucart, A., Fokkema, L., Francois, L., French, M., Forssell, E., Gingell, C., Goncalves, C., Ozel, H. Gokmen, Grimsley, A., Gugelmo, G., Gyure, E., Heller, C., Hensler, R., Jardim, I., Joost, C., Jorg-Streller, M., Jouault, C., Jung, A., Kanthe, M., Koc, N., Kok, I.L., Kozanoglu, T., Kumru, B., Lang, F., Lang, K., Liegeois, I., Liguori, A., Lilje, R., Lubina, O., Manta-Vogli, P., Mayr, D., Meneses, C., Newby, C., Meyer, U., Mexia, S., Nicol, C., Och, U., Olivas, S.M., Pedron-Giner, C., Pereira, R., Plutowska-Hoffmann, K., Purves, J., Dionigi, A. Re, Reinson, K., Robert, M., Robertson, L., Rocha, J.C., Rohde, C., Rosenbaum-Fabian, S., Rossi, A, Ruiz, M., Saligova, J., Gutierrez-Sanchez, A., Schlune, A., Schulpis, K., Serrano-Nieto, J., Skarpalezou, A., Skeath, R., Slabbert, A., Straczek, K., Gizewska, M., Terry, A., Wegberg, A.M.J. van, Zuvadelli, J., MacDonald, A., Pinto, A., Adams, S., Ahring, K., Allen, H., Almeida, M.F., Garcia-Arenas, D., Arslan, N., Assoun, M., Altinok, Y. Atik, Barrio-Carreras, D., Quintana, A. Belanger, Bernabei, S.M., Bontemps, C., Boyle, F., Bruni, G., Bueno-Delgado, M., Caine, G., Carvalho, R., Chrobot, A., Chyz, K., Cochrane, B., Correia, C., Corthouts, K., Daly, A., Leo, S. De, Desloovere, A., Meyer, A, Theux, A. De, Didycz, B., Dijsselhof, M.E., Dokoupil, K., Drabik, J., Dunlop, C., Eberle-Pelloth, W., Eftring, K., Ekengren, J., Errekalde, I., Evans, S., Foucart, A., Fokkema, L., Francois, L., French, M., Forssell, E., Gingell, C., Goncalves, C., Ozel, H. Gokmen, Grimsley, A., Gugelmo, G., Gyure, E., Heller, C., Hensler, R., Jardim, I., Joost, C., Jorg-Streller, M., Jouault, C., Jung, A., Kanthe, M., Koc, N., Kok, I.L., Kozanoglu, T., Kumru, B., Lang, F., Lang, K., Liegeois, I., Liguori, A., Lilje, R., Lubina, O., Manta-Vogli, P., Mayr, D., Meneses, C., Newby, C., Meyer, U., Mexia, S., Nicol, C., Och, U., Olivas, S.M., Pedron-Giner, C., Pereira, R., Plutowska-Hoffmann, K., Purves, J., Dionigi, A. Re, Reinson, K., Robert, M., Robertson, L., Rocha, J.C., Rohde, C., Rosenbaum-Fabian, S., Rossi, A, Ruiz, M., Saligova, J., Gutierrez-Sanchez, A., Schlune, A., Schulpis, K., Serrano-Nieto, J., Skarpalezou, A., Skeath, R., Slabbert, A., Straczek, K., Gizewska, M., Terry, A., Wegberg, A.M.J. van, Zuvadelli, J., and MacDonald, A.

Abstract

Contains fulltext : 205374.pdf (publisher's version ) (Open Access), 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 6months 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 17-26weeks in 85% (n=81/95) of centres, >26weeks in 12% (n=11/95) and<17weeks in 3% (n=3/95). Infant's showing an interest in solid foods, and their age, were important determinant factors influencing weaning commencement. 51% (n=48/95) of centres introduced Phe containing foods at 17-26weeks and 48% (n=46/95) at >26weeks. 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 26-36weeks (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

Published
2019

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

Author

UCL - (SLuc) Service d'endocrinologie et de nutrition, Pinto, A, Adams, S, Ahring, K, Allen, H, Almeida, M F, Garcia-Arenas, D, Arslan, N, Assoun, M, Atik Altınok, Y, Barrio-Carreras, D, Belanger Quintana, A, Bernabei, S M, Bontemps, C, Boyle, F, Bruni, G, Bueno-Delgado, M, Caine, G, Carvalho, R, Chrobot, A, Chyż, K, Cochrane, B, Correia, C, Corthouts, K, Daly, A, De Leo, S, Desloovere, A, De Meyer, A, De Theux, A, Didycz, B, Dijsselhof, M E, Dokoupil, K, Drabik, J, Dunlop, C, Eberle-Pelloth, W, Eftring, K, Ekengren, J, Errekalde, I, Evans, S, Foucart, Audrey, Fokkema, L, François, L, French, M, Forssell, E, Gingell, C, Gonçalves, C, Gökmen Özel, H, Grimsley, A, Gugelmo, G, Gyüre, E, Heller, C, Hensler, R, Jardim, I, Joost, C, Jörg-Streller, M, Jouault, C, Jung, A, Kanthe, M, Koç, N, Kok, I L, Kozanoğlu, T, Kumru, B, Lang, F, Lang, K, Liegeois, I, Liguori, A, Lilje, R, Ļubina, O, Manta-Vogli, P, Mayr, D, Meneses, C, Newby, C, Meyer, U, Mexia, S, Nicol, C, Och, U, Olivas, S M, Pedrón-Giner, C, Pereira, R, Plutowska-Hoffmann, K, Purves, J, Re Dionigi, A, Reinson, K, Robert, M, Robertson, L, Rocha, J C, Rohde, C, Rosenbaum-Fabian, S, Rossi, A, Ruiz, M, Saligova, J, Gutiérrez-Sánchez, A, Schlune, A, Schulpis, K, Serrano-Nieto, J, Skarpalezou, A, Skeath, R, Slabbert, A, Straczek, K, Giżewska, M, Terry, A, Thom, R, Tooke, A, Tuokkola, J, van Dam, E, van den Hurk, T A M, van der Ploeg, E M C, Vande Kerckhove, K, Van Driessche, M, van Wegberg, A M J, van Wyk, K, Vasconcelos, C, Velez García, V, Wildgoose, J, Winkler, T, Żółkowska, J, Zuvadelli, J, MacDonald, A, UCL - (SLuc) Service d'endocrinologie et de nutrition, Pinto, A, Adams, S, Ahring, K, Allen, H, Almeida, M F, Garcia-Arenas, D, Arslan, N, Assoun, M, Atik Altınok, Y, Barrio-Carreras, D, Belanger Quintana, A, Bernabei, S M, Bontemps, C, Boyle, F, Bruni, G, Bueno-Delgado, M, Caine, G, Carvalho, R, Chrobot, A, Chyż, K, Cochrane, B, Correia, C, Corthouts, K, Daly, A, De Leo, S, Desloovere, A, De Meyer, A, De Theux, A, Didycz, B, Dijsselhof, M E, Dokoupil, K, Drabik, J, Dunlop, C, Eberle-Pelloth, W, Eftring, K, Ekengren, J, Errekalde, I, Evans, S, Foucart, Audrey, Fokkema, L, François, L, French, M, Forssell, E, Gingell, C, Gonçalves, C, Gökmen Özel, H, Grimsley, A, Gugelmo, G, Gyüre, E, Heller, C, Hensler, R, Jardim, I, Joost, C, Jörg-Streller, M, Jouault, C, Jung, A, Kanthe, M, Koç, N, Kok, I L, Kozanoğlu, T, Kumru, B, Lang, F, Lang, K, Liegeois, I, Liguori, A, Lilje, R, Ļubina, O, Manta-Vogli, P, Mayr, D, Meneses, C, Newby, C, Meyer, U, Mexia, S, Nicol, C, Och, U, Olivas, S M, Pedrón-Giner, C, Pereira, R, Plutowska-Hoffmann, K, Purves, J, Re Dionigi, A, Reinson, K, Robert, M, Robertson, L, Rocha, J C, Rohde, C, Rosenbaum-Fabian, S, Rossi, A, Ruiz, M, Saligova, J, Gutiérrez-Sánchez, A, Schlune, A, Schulpis, K, Serrano-Nieto, J, Skarpalezou, A, Skeath, R, Slabbert, A, Straczek, K, Giżewska, M, Terry, A, Thom, R, Tooke, A, Tuokkola, J, van Dam, E, van den Hurk, T A M, van der Ploeg, E M C, Vande Kerckhove, K, Van Driessche, M, van Wegberg, A M J, van Wyk, K, Vasconcelos, C, Velez García, V, Wildgoose, J, Winkler, T, Żółkowska, J, Zuvadelli, J, and MacDonald, A

Abstract

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®) 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 < 17 weeks in 3% (n = 3/95). Infant's showing an interest in solid foods, and their age, were important determinant factors influencing weaning commencement. 51% (n = 48/95) of centres introduced Phe containing foods at 17-26 weeks and 48% (n = 46/95) at >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 throu

Published
2019

6. Early Feeding Practices in Infants with Phenylketonuria Across Europe

Author

Karit Reinson, F. Lang, M.E. Dijsselhof, J. Żółkowska, K. Dokoupil, T.A.M. van den Hurk, W. Eberle-Pelloth, Anita MacDonald, Carolyn Dunlop, María A. Ruiz, D. Barrio-Carreras, T. Kozanoğlu, K. Vande Kerckhove, I. Jardim, Andrea Schlune, L. François, J. Wildgoose, C. Correia, A. Re Dionigi, A. De Theux, Bozena Didycz, S. De Leo, A. Skarpalezou, P. Manta-Vogli, K. Straczek, K. Chyż, A. Chrobot, H. Gokmen Ozel, Clara Vasconcelos, Maria Gizewska, Alex Pinto, Karen Corthouts, V. Velez García, M. Jörg-Streller, A. Belanger Quintana, C. Meneses, Barbara Cochrane, M.F. Almeida, K. Schulpis, C. Pedrón-Giner, R. Lilje, A. Grimsley, A.M.J. van Wegberg, T. Winkler, R. Hensler, Júlio César Rocha, G. Bruni, Louise Robertson, K. Plutowska-Hoffmann, M. Bueno-Delgado, N. Koç, Anne Daly, L. Fokkema, R. Pereira, K. Ahring, D. Garcia-Arenas, Andreas Jung, Martine Robert, S.M. Olivas, J. Serrano-Nieto, J. Saligova, S.M. Bernabei, Ulrike Och, E. Forssell, Jetta Tuokkola, R. Thom, I. Liegeois, J. Ekengren, C. Jouault, A. Gutiérrez-Sánchez, K. Lang, Camille Newby, Nur Arslan, U. Meyer, C. Joost, Moira French, C. Bontemps, H. Allen, M. Kanthe, Juri Zuvadelli, E. van Dam, A. Foucart, M. Van Driessche, I.L. Kok, A. De Meyer, J. Drabik, Carmen Rohde, Rachel Skeath, Sharon Evans, An Desloovere, C. Gingell, E.M.C. van der Ploeg, D. Mayr, E. Gyüre, Y. Atik Altınok, B. Kumru, O. Ļubina, A. Slabbert, Stefanie Rosenbaum-Fabian, G. Gugelmo, Claire Nicol, G. Caine, I. Errekalde, A. Liguori, Sandra Adams, A. Rossi, A. Tooke, R. Carvalho, J. Purves, C. Heller, M. Assoun, Carolina Gonçalves, K. Eftring, F. Boyle, A. Terry, S. Mexia, K. van Wyk, Ege Üniversitesi, HUS Children and Adolescents, Clinicum, University of Helsinki, HUS Internal Medicine and Rehabilitation, Children's Hospital, MUMC+: TPZ Dietetiek (9), RS: FHML non-thematic output, and UCL - (SLuc) Unité d'endocrinologie pédiatrique

Subjects
0301 basic medicine, Pediatrics, PKU, Phenylketonuria, Breastfeeding, lnfectious Diseases and Global Health Radboud Institute for Molecular Life Sciences [Radboudumc 4], IMD, Inherited Metabolic Disorders, PROTEIN, 030105 genetics & heredity, Phe-Free Infant Formula, Endocrinology, IMD, Standard infant formula, HDE PED, Medicine, Phenylketonuria, lcsh:QH301-705.5, 2. Zero hunger, lcsh:R5-920, 1184 Genetics, developmental biology, physiology, Inherited Metabolic Disorders, 3. Good health, PKU, GROWTH, lcsh:Medicine (General), DIETARY-MANAGEMENT, Research Paper, Phe, medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, Phe, Phenylalanine, Phenylalanine, Breast milk, 03 medical and health sciences, Phe-free infant formula, Infant practices, Genetics, Weaning, Molecular Biology, Newborn screening, Infant Practices, business.industry, Dietary management, nutritional and metabolic diseases, lcsh:Biology (General), Infant formula, Human medicine, business, Breast feeding
Abstract

WOS: 000442229500021, PubMed ID: 30101073, 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., Vitaflo, We thank Vitaflo for supporting the publication cost of this paper.

Published
2018

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

Author

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

Published
2019
Full Text
View/download PDF

8. Early feeding practices in infants with phenylketonuria across Europe

Author

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

Published
2018

9. Early feeding practices in infants with phenylketonuria across Europe

Author

Afdeling Dietetiek, Other research (not in main researchprogram), Pinto, A., Adams, S., Ahring, K., Allen, H., Almeida, M. F., Garcia-Arenas, D., Arslan, N., Assoun, M., Atik Altınok, Y., Barrio-Carreras, D., Belanger Quintana, A., Bernabei, S. M., Bontemps, C., Boyle, F., Bruni, G., Bueno-Delgado, M., Caine, G., Carvalho, R., Chrobot, A., Chyż, K., Cochrane, B., Correia, C., Corthouts, K., Daly, A., De Leo, S., Desloovere, A., De Meyer, A., De Theux, A., Didycz, B., Dijsselhof, M. E., Dokoupil, K., Drabik, J., Dunlop, C., Eberle-Pelloth, W., Eftring, K., Ekengren, J., Errekalde, I., Evans, S., Foucart, A., Fokkema, L., François, L., French, M., Forssell, E., Gingell, C., Gonçalves, C., Gökmen Özel, H., Grimsley, A., Gugelmo, G., Gyüre, E., Heller, C., Hensler, R., Jardim, I., Joost, C., Jörg-Streller, M., Jouault, C., Jung, A., Kanthe, M., Koç, N., Kok, I. L., Kozanoğlu, T., Kumru, B., Lang, F., Lang, K., Liegeois, I., Liguori, A., Lilje, R., Ļubina, O., Manta-Vogli, P., Mayr, D., Meneses, C., Newby, C., Meyer, U., Mexia, S., Nicol, C., Och, U., Olivas, S. M., Pedrón-Giner, C., Pereira, R., Plutowska-Hoffmann, K., Purves, J., Re Dionigi, A., Reinson, K., Robert, M., Robertson, L., Rocha, J. C., Rohde, C., Rosenbaum-Fabian, S., Rossi, A., Ruiz, M., Saligova, J., Gutiérrez-Sánchez, A., Schlune, A., Schulpis, K., Serrano-Nieto, J., Skarpalezou, A., Skeath, R., Slabbert, A., Straczek, K., Giżewska, M., Terry, A., Thom, R., Tooke, A., Tuokkola, J., van Dam, E., van den Hurk, T. A.M., van der Ploeg, E. M.C., Vande Kerckhove, K., Van Driessche, M., van Wegberg, A. M.J., van Wyk, K., Vasconcelos, C., Velez García, V., Wildgoose, J., Winkler, T., Żółkowska, J., Zuvadelli, J., MacDonald, A., Afdeling Dietetiek, Other research (not in main researchprogram), Pinto, A., Adams, S., Ahring, K., Allen, H., Almeida, M. F., Garcia-Arenas, D., Arslan, N., Assoun, M., Atik Altınok, Y., Barrio-Carreras, D., Belanger Quintana, A., Bernabei, S. M., Bontemps, C., Boyle, F., Bruni, G., Bueno-Delgado, M., Caine, G., Carvalho, R., Chrobot, A., Chyż, K., Cochrane, B., Correia, C., Corthouts, K., Daly, A., De Leo, S., Desloovere, A., De Meyer, A., De Theux, A., Didycz, B., Dijsselhof, M. E., Dokoupil, K., Drabik, J., Dunlop, C., Eberle-Pelloth, W., Eftring, K., Ekengren, J., Errekalde, I., Evans, S., Foucart, A., Fokkema, L., François, L., French, M., Forssell, E., Gingell, C., Gonçalves, C., Gökmen Özel, H., Grimsley, A., Gugelmo, G., Gyüre, E., Heller, C., Hensler, R., Jardim, I., Joost, C., Jörg-Streller, M., Jouault, C., Jung, A., Kanthe, M., Koç, N., Kok, I. L., Kozanoğlu, T., Kumru, B., Lang, F., Lang, K., Liegeois, I., Liguori, A., Lilje, R., Ļubina, O., Manta-Vogli, P., Mayr, D., Meneses, C., Newby, C., Meyer, U., Mexia, S., Nicol, C., Och, U., Olivas, S. M., Pedrón-Giner, C., Pereira, R., Plutowska-Hoffmann, K., Purves, J., Re Dionigi, A., Reinson, K., Robert, M., Robertson, L., Rocha, J. C., Rohde, C., Rosenbaum-Fabian, S., Rossi, A., Ruiz, M., Saligova, J., Gutiérrez-Sánchez, A., Schlune, A., Schulpis, K., Serrano-Nieto, J., Skarpalezou, A., Skeath, R., Slabbert, A., Straczek, K., Giżewska, M., Terry, A., Thom, R., Tooke, A., Tuokkola, J., van Dam, E., van den Hurk, T. A.M., van der Ploeg, E. M.C., Vande Kerckhove, K., Van Driessche, M., van Wegberg, A. M.J., van Wyk, K., Vasconcelos, C., Velez García, V., Wildgoose, J., Winkler, T., Żółkowska, J., Zuvadelli, J., and MacDonald, A.

Published
2018

10. Early feeding practices in infants with phenylketonuria across Europe

Author

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

Published
2018
Full Text
View/download PDF

11. Bone mineral status in children with phenylketonuria under treatment

Author

Irini Voskaki, Helen Athanasopoulou, A. Al-Qadreh, Kleopatra H. Schulpis, A Skarpalezou, and C Mengreli

Subjects
Bone mineral, medicine.medical_specialty, Bone disease, Bone density, Phenylketonurias, business.industry, Osteoporosis, General Medicine, medicine.disease, Bone remodeling, Osteopenia, Endocrinology, Internal medicine, Pediatrics, Perinatology and Child Health, medicine, Hypercalciuria, business
Abstract

Bone mineral status was assessed in 48 children with phenylketonuria (PKU) (20M, 28 F, aged 2.5-17 y). Bone density was measured in the distal third of the right forearm using single photon absorptiometry and was expressed as +/-SD with respect to age- and gender-matched controls. Serum calcium (Ca), magnesium (Mg), phosphorus (P), alkaline phosphatase (ALP), parathyroid hormone and 25-hydroxyvitamin D were measured in morning samples. The ratios of urinary Ca/creatinine (UCa/UCr), UP/UCr, UMg/UCr and hydroxyproline (OH-Pr)/UCr were calculated in urine samples collected over a period of 3 h. Patients' data were compared with those of 50 controls (22 M, 28 F, aged 3-15 y). The data showed severe osteopenia (below -2SD) in 22/48 patients. Bone loss was more prominent in patients over 8 y old. Bone density correlated significantly with age (r=-0.56,p 10mgdl(-1), in contrast to 21/26 in the older group. It is clear from the data that osteopenia is commonly found in PKU patients from early life. The biochemical data indicate a metabolic state of low bone turnover in PKU patients. In conclusion, a better, more restricted diet may correct osteopenia.

Published
2007

14. Body Composition Profile of Young Patients With Phenylketonuria and Mild Hyperphenylalaninemia

Author

Artemis Doulgeraki, Astrinia Skarpalezou, Ioannis Monopolis, Areti Theodosiadou, and Kleopatra H. Schulpis

Subjects
Bone mineral, medicine.medical_specialty, Newborn screening, Bone density, business.industry, Phenylalanine, Endocrinology, Diabetes and Metabolism, Standard score, medicine.disease, Obesity, Kowsar, Endocrinology, Hyperphenylalaninemia, Internal medicine, Lean body mass, Muscle, Medicine, Bone, business, Children, Research Article
Abstract

Background: There is evidence in support of low bone density in young patients with disorders of phenylalanine metabolism; however, little is known about muscle and fat mass in these patients, especially in those with mild hyperphenylalaninemia (mHPA). Objectives: We aimed to evaluate body composition of children and adolescents with early-diagnosed disorders of phenylalanine metabolism. Patients and Methods: The study was conducted in the Institute of Child Health, which is the national center that performs newborn screening. Bone, muscle, and fat mass of 48 patients with phenylketonuria (PKU) and 32 patients with mild mHPA, aged five to 18 years, were compared to 57 age- and sex-matched controls. Dual energy X-ray absorptiometry was used for this purpose. Results: Compared to controls, bone mineral density (BMD) was lower in patients with PKU (mean total body BMD z score, 0.11; P = 0.03) and in those with mild mHPA (mean lumbar BMD z score, -0.34; P = 0.01). Lean body mass and fat mass were not significantly affected in the study group. Comparison between the two patients groups did not reveal any difference in body composition profiles; however, pubertal status appeared important for within-group comparisons. Fat mass was significantly increased in teenagers with PKU, which was more evident in those with poor dietary compliance irrespective of gender (fat mass z score, 0.66; P = 0.018). Finally, positive correlations were found not only between bone, muscle, and fat mass in both groups, but also between fat mass and Phenylalanine levels in patients with PKU (r, 0.46; P = 0.001). Conclusions: Bone mineral density appears suboptimal in young patients with disorders of phenylalanine metabolism. Adolescents seemed more prone to obesity, especially when their dietary adherence was poor, whereas muscle mass was not considerably affected. To ensure healthier bones and less fat content, close follow-up as well as proper lifestyle advice is needed.

Published
2014

17. Branched chain amino acids as adjunctive therapy to ketogenic diet in epilepsy: pilot study and hypothesis

Author

Anestis Ilias, Sotirios Varlamis, Evangelos Pavlou, Nikolaos Nikolaidis, Panagiota Kalaidopoulou, Vai Doulioglou, Martha Spilioti, Ilias Chatziioanidis, Kyriakos Garganis, Konstantina Vasilaki, Irini Katsanika, Astrinia Skarpalezou, Serafia Kalamitsou, and Athanasios Evangeliou

Subjects
medicine.medical_specialty, Additional Therapy, medicine.medical_treatment, Seizure reduction, Pilot Projects, Models, Biological, Epilepsy, Seizures, Internal medicine, Medicine, Humans, Prospective Studies, Seizure activity, Prospective cohort study, Child, chemistry.chemical_classification, business.industry, Ketosis, medicine.disease, Amino acid, Endocrinology, Treatment Outcome, chemistry, Child, Preschool, Pediatrics, Perinatology and Child Health, Anticonvulsants, Neurology (clinical), business, Diet, Ketogenic, Amino Acids, Branched-Chain, Ketogenic diet, Follow-Up Studies
Abstract

A pilot prospective follow-up study of the role of the branched chain amino acids as additional therapy to the ketogenic diet was carried out in 17 children, aged between 2 and 7 years, with refractory epilepsy. All of these patients were on the ketogenic diet; none of them was seizure free, while only 13 had more or less benefited from the diet. The addition of branched chain amino acids induced a 100% seizure reduction in 3 patients, while a 50% to 90% reduction was noticed in 5. Moreover, in all of the patients, no reduction in ketosis was recorded despite the change in the fat-to-protein ratio from 4:1 to 2.5:1. Although our data are preliminary, we suggest that branched chain amino acids may increase the effectiveness of the ketogenic diet and the diet could be more easily tolerated by the patients because of the change in the ratio of fat to protein.

Published
2009

19. Application of a ketogenic diet in children with autistic behavior: Pilot study

Author

Evangeliou, A Vlachonikolis, I Mihailidou, H Spilioti, M and Skarpalezou, A Makaronas, N Prokopiou, A Christodoulou, P and Liapi-Adamidou, G Helidonis, E Sbyrakis, S Smeitink, J

Abstract

A pilot prospective follow-up study of the role of the ketogenic diet was carried out on 30 children, aged between 4 and 10 years, with autistic behavior. The diet was applied for 6 months, with continuous administration for 4 weeks, interrupted by 2-week diet-free intervals. Seven patients could not tolerate the diet, whereas five other patients adhered to the diet for I to 2 months and then discontinued it. Of the remaining group who adhered to the diet, 18 of 30 children (60%), improvement was recorded in several parameters and in accordance with the Childhood Autism Rating Scale. Significant improvement (> 12 units of the Childhood Autism Rating Scale) was recorded in two patients (pre-Scale: 35.00 +/- 1.41 [mean +/- SD]), average improvement (> 8-12 units) in eight patients (pre-Scale: 41.88 +/- 3.14[mean SD]), and minor improvement (2-8 units) in eight patients (pre-Scale: 45.25 +/- 2.76 [mean +/- SD]). Although these data are very preliminary, there is some evidence that the ketogenic diet may be used in autistic behavior as an additional or alternative therapy.

Published
2003

20. Application of a ketogenic diet in children with autistic behavior: pilot study

Author

Jan A.M. Smeitink, S Sbyrakis, Martha Spilioti, Athanasios Evangeliou, Ioannis G. Vlachonikolis, Panagiotis Christodoulou, Nikolaos Makaronas, Emmanouel Helidonis, Ahilleas Prokopiou, Astrinia Skarpalezou, Helen Mihailidou, and Georgia Liapi-Adamidou

Subjects
Male, Pediatrics, medicine.medical_specialty, Alternative therapy, medicine.medical_treatment, Treatment outcome, Pilot Projects, Ketone Bodies, Severity of Illness Index, 03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, Severity of illness, Dietary Carbohydrates, Humans, Medicine, Autistic Disorder, Child, business.industry, Clinical trial, Treatment Outcome, Genetic defects of metabolism [UMCN 5.1], Child, Preschool, Pediatrics, Perinatology and Child Health, Childhood Autism Rating Scale, Female, Dietary Proteins, Neurology (clinical), business, Cellular energy metabolism [UMCN 5.3], 030217 neurology & neurosurgery, Ketogenic diet
Abstract

A pilot prospective follow-up study of the role of the ketogenic diet was carried out on 30 children, aged between 4 and 10 years, with autistic behavior. The diet was applied for 6 months, with continuous administration for 4 weeks, interrupted by 2-week diet-free intervals. Seven patients could not tolerate the diet, whereas five other patients adhered to the diet for 1 to 2 months and then discontinued it. Of the remaining group who adhered to the diet, 18 of 30 children (60%), improvement was recorded in several parameters and in accordance with the Childhood Autism Rating Scale. Significant improvement (> 12 units of the Childhood Autism Rating Scale) was recorded in two patients (pre-Scale: 35.00 ± 1.41 [mean ± SD]), average improvement (> 8—12 units) in eight patients (pre-Scale: 41.88 ± 3.14[mean ± SD]), and minor improvement (2—8 units) in eight patients (pre-Scale: 45.25 ± 2.76 [mean ± SD]). Although these data are very preliminary, there is some evidence that the ketogenic diet may be used in autistic behavior as an additional or alternative therapy. ( J Child Neurol 2003; 18: 113—118).

Published
2003

21. Bone mineral status in children with phenylketonuria under treatment

Author

A, Al-Qadreh, K H, Schulpis, H, Athanasopoulou, C, Mengreli, A, Skarpalezou, and I, Voskaki

Subjects
Male, Adolescent, Bone Density, Child, Preschool, Phenylketonurias, Age Factors, Humans, Female, Child
Abstract

Bone mineral status was assessed in 48 children with phenylketonuria (PKU) (20M, 28 F, aged 2.5-17 y). Bone density was measured in the distal third of the right forearm using single photon absorptiometry and was expressed as +/-SD with respect to age- and gender-matched controls. Serum calcium (Ca), magnesium (Mg), phosphorus (P), alkaline phosphatase (ALP), parathyroid hormone and 25-hydroxyvitamin D were measured in morning samples. The ratios of urinary Ca/creatinine (UCa/UCr), UP/UCr, UMg/UCr and hydroxyproline (OH-Pr)/UCr were calculated in urine samples collected over a period of 3 h. Patients' data were compared with those of 50 controls (22 M, 28 F, aged 3-15 y). The data showed severe osteopenia (below -2SD) in 22/48 patients. Bone loss was more prominent in patients over 8 y old. Bone density correlated significantly with age (r=-0.56,p0.001) and with Phe (r=-0.49, p0.007) but did not correlate with the other biochemical indices studied. Comparing PKU children with controls, significantly higher serum calcium and magnesium (p=0.04, p0.001, respectively), lower ALP (p=0.01), higher UCa/UCr ratio (p0.001), lower UP/UCr (p0.001) and lower UOH-Pr/UCr (p0.001) were found. Dietary compliance was poor in patients over the age of 8y, as only 3/22 ofor = 8y had mean serum phenylalanine10mgdl(-1), in contrast to 21/26 in the older group. It is clear from the data that osteopenia is commonly found in PKU patients from early life. The biochemical data indicate a metabolic state of low bone turnover in PKU patients. In conclusion, a better, more restricted diet may correct osteopenia.

Published
1998

23. Application of a ketogenic diet in children with autistic behavior: pilot study.

Author

Evangeliou, A., Vlachonikolis, I., Mihailidou, H., Spilioti, M., Skarpalezou, A., Makaronas, N., Prokopiou, A., Christodoulou, P., Liapi-Adamidou, G., Helidonis, E., Sbyrakis, S., Smeitink, J.A.M., Evangeliou, A., Vlachonikolis, I., Mihailidou, H., Spilioti, M., Skarpalezou, A., Makaronas, N., Prokopiou, A., Christodoulou, P., Liapi-Adamidou, G., Helidonis, E., Sbyrakis, S., and Smeitink, J.A.M.

Abstract

Item does not contain fulltext, A pilot prospective follow-up study of the role of the ketogenic diet was carried out on 30 children, aged between 4 and 10 years, with autistic behavior. The diet was applied for 6 months, with continuous administration for 4 weeks, interrupted by 2-week diet-free intervals. Seven patients could not tolerate the diet, whereas five other patients adhered to the diet for 1 to 2 months and then discontinued it. Of the remaining group who adhered to the diet, 18 of 30 children (60%), improvement was recorded in several parameters and in accordance with the Childhood Autism Rating Scale. Significant improvement (> 12 units of the Childhood Autism Rating Scale) was recorded in two patients (pre-Scale: 35.00 +/- 1.41[mean +/- SD]), average improvement (> 8-12 units) in eight patients (pre-Scale: 41.88 +/- 3.14[mean +/- SD]), and minor improvement (2-8 units) in eight patients (pre-Scale: 45.25 +/- 2.76 [mean +/- SD]). Although these data are very preliminary, there is some evidence that the ketogenic diet may be used in autistic behavior as an additional or alternative therapy.

Published
2003

24. Branched Chain Amino Acids as Adjunctive Therapy to Ketogenic Diet in Epilepsy: Pilot Study and Hypothesis

Author

Evangeliou, Athanasios, primary, Spilioti, Martha, additional, Doulioglou, Vai, additional, Kalaidopoulou, Panagiota, additional, Ilias, Anestis, additional, Skarpalezou, Astrinia, additional, Katsanika, Irini, additional, Kalamitsou, Serafia, additional, Vasilaki, Konstantina, additional, Chatziioanidis, Ilias, additional, Garganis, Kiriakos, additional, Pavlou, Evangelos, additional, Varlamis, Sotirios, additional, and Nikolaidis, Nikolaos, additional

Published
2009
Full Text
View/download PDF

27. Application of a Ketogenic Diet in Children With Autistic Behavior: Pilot Study

Author

Evangeliou, Athanasios, primary, Vlachonikolis, Ioannis, additional, Mihailidou, Helen, additional, Spilioti, Martha, additional, Skarpalezou, Astrinia, additional, Makaronas, Nikolaos, additional, Prokopiou, Ahilleas, additional, Christodoulou, Panagiotis, additional, Liapi-Adamidou, Georgia, additional, Helidonis, Emmanouel, additional, Sbyrakis, Stylianos, additional, and Smeitink, Jan, additional

Published
2003
Full Text
View/download PDF

28. Bone mineral status in children with phenylketonuria under treatment.

Author

Al-Qadreh, A, Schulpis, KH, Athanasopoulou, H, Mengreli, C, Skarpalezou, A, Voskaki, I, and Schulpis, K H

Subjects
BONES, JUVENILE diseases, PHENYLKETONURIA
Abstract

Bone mineral status was assessed in 48 children with phenylketonuria (PKU) (20M, 28 F, aged 2.5-17 y). Bone density was measured in the distal third of the right forearm using single photon absorptiometry and was expressed as +/-SD with respect to age- and gender-matched controls. Serum calcium (Ca), magnesium (Mg), phosphorus (P), alkaline phosphatase (ALP), parathyroid hormone and 25-hydroxyvitamin D were measured in morning samples. The ratios of urinary Ca/creatinine (UCa/UCr), UP/UCr, UMg/UCr and hydroxyproline (OH-Pr)/UCr were calculated in urine samples collected over a period of 3 h. Patients' data were compared with those of 50 controls (22 M, 28 F, aged 3-15 y). The data showed severe osteopenia (below -2SD) in 22/48 patients. Bone loss was more prominent in patients over 8 y old. Bone density correlated significantly with age (r=-0.56,p < 0.001) and with Phe (r=-0.49, p < 0.007) but did not correlate with the other biochemical indices studied. Comparing PKU children with controls, significantly higher serum calcium and magnesium (p=0.04, p < 0.001, respectively), lower ALP (p=0.01), higher UCa/UCr ratio (p < 0.001), lower UP/UCr (p < 0.001) and lower UOH-Pr/UCr (p < 0.001) were found. Dietary compliance was poor in patients over the age of 8y, as only 3/22 of < or = 8y had mean serum phenylalanine >10mgdl(-1), in contrast to 21/26 in the older group. It is clear from the data that osteopenia is commonly found in PKU patients from early life. The biochemical data indicate a metabolic state of low bone turnover in PKU patients. In conclusion, a better, more restricted diet may correct osteopenia. [ABSTRACT FROM AUTHOR]

Published
1998
Full Text
View/download PDF

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

Author

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

Abstract

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®) 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 < 17 weeks in 3% ( n  = 3/95). Infant's showing an interest in solid foods, and their age, were important determinant factors influencing weaning commencement. 51% ( n  = 48/95) of centres introduced Phe containing foods at 17-26 weeks and 48% ( n  = 46/95) at >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.

Published
2018
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results