87 results on '"Wildgoose J"'
Search Results
2. Weaning infants with phenylketonuria: a review
- Author
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MacDonald, A., Evans, S., Cochrane, B., and Wildgoose, J.
- Published
- 2012
- Full Text
- View/download PDF
3. An assessment of the principal factors affecting dairy product supply and demand in the united kingdom with reference to the implementation of specific policies
- Author
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Wildgoose, J. K.
- Subjects
338.1 - Published
- 1978
4. Ion mobility mass spectrometry of proteins in a modified commercial mass spectrometer
- Author
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Thalassinos, K., Slade, S.E., Jennings, K.R., Scrivens, J.H., Giles, K., Wildgoose, J., Hoyes, J., Bateman, R.H., and Bowers, M.T.
- Published
- 2004
- Full Text
- View/download PDF
5. Weaning practices in phenylketonuria vary between health professionals in Europe.
- Author
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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. 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
7. Eccentric exercise and neuroleptic malignant syndrome
- Author
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Gralton, E, Wildgoose, J, Donovan, W M, and Wilkie, J
- Published
- 1998
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. Practices in prescribing protein substitutes for PKU in Europe : No uniformity of approach
- Author
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Aguiar, A., Ahring, K., Almeida, M. F., Assoun, M., Belanger Quintana, A., Bigot, S., Bihet, G., Malmberg, Karin Blom, Burlina, A., Bushueva, T., Caris, A., Chan, H., Clark, A., Clark, S., Cochrane, B., Corthouts, K., Dalmau, J., Dassy, M., De Meyer, A., Didycz, B., Diels, M., Dokupil, K., Dubois, S., Eftring, K., Ekengren, J., Ellerton, C., Evans, S., Faria, A., Fischer, A., Ford, S., Freisinger, P., Gizewska, M., Gokmen-Ozel, H., Gribben, J., Gunden, F., Heddrich-Ellerbrok, M., Heiber, S., Heidenborg, C., Jankowski, C., Janssen-Regelink, R., Jones, I., Jonkers, C., Joerg-Streller, M., Kaalund-Hansen, K., Kiss, E., Lammardo, A. M., Lang, K., Lier, D., Lilje, R., Lowry, S., Luyten, K., MacDonald, A., Meyer, U., Moor, D., Pal, A., Robert, M., Robertson, L., Rocha, J. C., Rohde, C., Ross, K., Saruhan, S., Sjoqvist, E., Skeath, R., Stoelen, L., Ter Horst, N. M., Terry, A., Timmer, C., Tuncer, N., Vande Kerckhove, K., van der Ploeg, L., van Rijn, M., van Spronsen, F. J., van Teeffelen-Heithoff, A., van Wegberg, A., van Wyk, K., Vasconcelos, C., Vitoria, I., Wildgoose, J., Webster, D., White, F. J., Zweers, H., Aguiar, A., Ahring, K., Almeida, M. F., Assoun, M., Belanger Quintana, A., Bigot, S., Bihet, G., Malmberg, Karin Blom, Burlina, A., Bushueva, T., Caris, A., Chan, H., Clark, A., Clark, S., Cochrane, B., Corthouts, K., Dalmau, J., Dassy, M., De Meyer, A., Didycz, B., Diels, M., Dokupil, K., Dubois, S., Eftring, K., Ekengren, J., Ellerton, C., Evans, S., Faria, A., Fischer, A., Ford, S., Freisinger, P., Gizewska, M., Gokmen-Ozel, H., Gribben, J., Gunden, F., Heddrich-Ellerbrok, M., Heiber, S., Heidenborg, C., Jankowski, C., Janssen-Regelink, R., Jones, I., Jonkers, C., Joerg-Streller, M., Kaalund-Hansen, K., Kiss, E., Lammardo, A. M., Lang, K., Lier, D., Lilje, R., Lowry, S., Luyten, K., MacDonald, A., Meyer, U., Moor, D., Pal, A., Robert, M., Robertson, L., Rocha, J. C., Rohde, C., Ross, K., Saruhan, S., Sjoqvist, E., Skeath, R., Stoelen, L., Ter Horst, N. M., Terry, A., Timmer, C., Tuncer, N., Vande Kerckhove, K., van der Ploeg, L., van Rijn, M., van Spronsen, F. J., van Teeffelen-Heithoff, A., van Wegberg, A., van Wyk, K., Vasconcelos, C., Vitoria, I., Wildgoose, J., Webster, D., White, F. J., and Zweers, H.
- Abstract
Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU). Methods: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. Results: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n = 24 centres) (infants <1 year, >2-3 g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n = 10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n = 4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n = 25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). Conclusions: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population.
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- 2015
- Full Text
- View/download PDF
12. How strict is galactose restriction in adults with galactosaemia? International practice
- Author
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Adam, Siddig Momin, Akroyd, R., Bernabei, S., Bollhalder, S., Boocock, S., Burlina, A., Coote, T., Corthouts, K., Dalmau, Judith, Dawson, S., Defourny, S., Meyer, A., Desloovere, A., Devlin, Y., Diels, M., Dokoupil, K., Donald, S., Evans, S., Fasan, I., Ferguson, C., Ford, S., Forga, M., Gallo, G., Grünert, S.C., Heddrich-Ellerbrok, M., Heidenborg, C., Jonkers, C.F., Lefebure, K., Luyten, K., MacDonald, A., Meyer, U., Micciche, A., Müller, E., Portnoi, P., Ripley, S., Robert, C.M., Robertson, L.V., Rosenbaum-Fabian, S., Sahm, K., Schultz, Erica S, Singleton, K., Sjöqvist, E., Stoelen, L., Terry, A., Thompson, S., Timmer, C., Vande Kerckhove, K., van den Ploeg, L., Van Driessche, M., van Rijn, M., van Teeffelen-Heithoff, A., Vitoria, I., Voillot, C., Wenz, J., Westbrook, M., Wildgoose, J., Zweers, H., Adam, Siddig Momin, Akroyd, R., Bernabei, S., Bollhalder, S., Boocock, S., Burlina, A., Coote, T., Corthouts, K., Dalmau, Judith, Dawson, S., Defourny, S., Meyer, A., Desloovere, A., Devlin, Y., Diels, M., Dokoupil, K., Donald, S., Evans, S., Fasan, I., Ferguson, C., Ford, S., Forga, M., Gallo, G., Grünert, S.C., Heddrich-Ellerbrok, M., Heidenborg, C., Jonkers, C.F., Lefebure, K., Luyten, K., MacDonald, A., Meyer, U., Micciche, A., Müller, E., Portnoi, P., Ripley, S., Robert, C.M., Robertson, L.V., Rosenbaum-Fabian, S., Sahm, K., Schultz, Erica S, Singleton, K., Sjöqvist, E., Stoelen, L., Terry, A., Thompson, S., Timmer, C., Vande Kerckhove, K., van den Ploeg, L., Van Driessche, M., van Rijn, M., van Teeffelen-Heithoff, A., Vitoria, I., Voillot, C., Wenz, J., Westbrook, M., Wildgoose, J., and Zweers, H.
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- 2015
13. Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach
- Author
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UCL - (SLuc) Service d'endocrinologie et de nutrition, Aguiar, A., Ahring, K., Almeida, M.F., Assoun, M., Belanger Quintana, A., Bigot, S., Bihet, G., Blom Malmberg, K., Burlina, A., Bushueva, T., Caris, A., Chan, H., Clark, A., Clark, S., Cochrane, B., Corthouts, K., Dalmau, J., Dassy, Martine, De Meyer, A., Didycz, B., Diels, M., Dokupil, K., Dubois, S., Eftring, K., Ekengren, J., Ellerton, C., Evans, S., Faria, A., Fischer, A., Ford, S., Freisinger, P., Gizewska, M., Gokmen-Ozel, H., Gribben, J., Gunden, F., Heddrich-Ellerbrok, M., Heiber, S., Heidenborg, C., Jankowski, C., Janssen-Regelink, R., Jones, I., Jonkers, C., Joerg-Streller, M., Kaalund-Hansen, K., Kiss, E., Lammardo, A.M., Lang, K., Lier, D., Lilje, R., Lowry, S., Luyten, K., MacDonald, A., Meyer, U., Moor, D., Pal, A., Robert, M., Robertson, L., Rocha, J.C., Rohde, C., Ross, K., Saruhan, S., Sjöqvist, E., Skeath, R., Stoelen, L., Ter Horst, N.M., Terry, A., Timmer, C., Tuncer, N., Vande Kerckhove, K., van der Ploeg, L., van Rijn, M., van Spronsen, F.J., van Teeffelen-Heithoff, A., van Wegberg, A., van Wyk, K., Vasconcelos, C., Vitoria, I., Wildgoose, J., Webster, D., White, F.J., Zweers, H., UCL - (SLuc) Service d'endocrinologie et de nutrition, Aguiar, A., Ahring, K., Almeida, M.F., Assoun, M., Belanger Quintana, A., Bigot, S., Bihet, G., Blom Malmberg, K., Burlina, A., Bushueva, T., Caris, A., Chan, H., Clark, A., Clark, S., Cochrane, B., Corthouts, K., Dalmau, J., Dassy, Martine, De Meyer, A., Didycz, B., Diels, M., Dokupil, K., Dubois, S., Eftring, K., Ekengren, J., Ellerton, C., Evans, S., Faria, A., Fischer, A., Ford, S., Freisinger, P., Gizewska, M., Gokmen-Ozel, H., Gribben, J., Gunden, F., Heddrich-Ellerbrok, M., Heiber, S., Heidenborg, C., Jankowski, C., Janssen-Regelink, R., Jones, I., Jonkers, C., Joerg-Streller, M., Kaalund-Hansen, K., Kiss, E., Lammardo, A.M., Lang, K., Lier, D., Lilje, R., Lowry, S., Luyten, K., MacDonald, A., Meyer, U., Moor, D., Pal, A., Robert, M., Robertson, L., Rocha, J.C., Rohde, C., Ross, K., Saruhan, S., Sjöqvist, E., Skeath, R., Stoelen, L., Ter Horst, N.M., Terry, A., Timmer, C., Tuncer, N., Vande Kerckhove, K., van der Ploeg, L., van Rijn, M., van Spronsen, F.J., van Teeffelen-Heithoff, A., van Wegberg, A., van Wyk, K., Vasconcelos, C., Vitoria, I., Wildgoose, J., Webster, D., White, F.J., and Zweers, H.
- Abstract
Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU). Methods: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. Results: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n. =. 24 centres) (infants <. 1. year, >. 2-3. g/kg/day; 1-3. years of age, >. 2-3. g/kg/day; 4-10. years of age, >. 1.5-2.5. g/kg/day) and Southern Europe (n. =. 10 centres) (infants <. 1. year, 2.5. g/kg/day, 1-3. years of age, 2. g/kg/day; 4-10. years of age, 1.5-2. g/kg/day), than by Eastern Europe (n. =. 4 centres) (infants <. 1. year, 2.5. g/kg/day, 1-3. years of age, >. 2-2.5. g/kg/day; 4-10. years of age, >. 1.5-2. g/kg/day) and with Western Europe (n. =. 25 centres) giving the least (infants <. 1. year, >. 2-2.5. g/kg/day, 1-3. years of age, 1.5-2. g/kg/day; 4-10. years of age, 1-1.5. g/kg/day). Total protein prescription was similar in patients aged >. 10. years (1-1.5. g/kg/day) and maternal patients (1-1.5. g/kg/day). Conclusions: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population.
- Published
- 2015
14. How strict is galactose restriction in adults with galactosaemia? International practice
- Author
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Adam, S., Akroyd, R., Bernabei, S., Bollhalder, S., Boocock, S., Burlina, A., Coote, T., Corthouts, K., Dalmau, J., Dawson, S., Defourny, S., Meijer, A, Desloovere, A., Devlin, Y., Diels, M., Dokoupil, K., Donald, S., Evans, S., Fasan, I., Ferguson, C., Ford, S., Forga, M., Gallo, G., Grunert, S.C., Heddrich-Ellerbrok, M., Heidenborg, C., Jonkers, C., Lefebure, K., Luyten, K., Macdonald, A., Meyer, U., Micciche, A., Muller, E., Portnoi, P., Ripley, S., Robert, M., Robertson, L.V., Rosenbaum-Fabian, S., Sahm, K., Schultz, S., Singleton, K., Sjoqvist, E., Stoelen, L., Terry, A., Thompson, S., Timmer, C., Kerckhove, K. Vande, Ploeg, L. van der, Driessche, M. Van, Rijn, M. van de, Teeffelen-Heithoff, A. van, Vitoria, I., Voillot, C., Wenz, J., Westbrook, M., Wildgoose, J., Zweers, H.E., Adam, S., Akroyd, R., Bernabei, S., Bollhalder, S., Boocock, S., Burlina, A., Coote, T., Corthouts, K., Dalmau, J., Dawson, S., Defourny, S., Meijer, A, Desloovere, A., Devlin, Y., Diels, M., Dokoupil, K., Donald, S., Evans, S., Fasan, I., Ferguson, C., Ford, S., Forga, M., Gallo, G., Grunert, S.C., Heddrich-Ellerbrok, M., Heidenborg, C., Jonkers, C., Lefebure, K., Luyten, K., Macdonald, A., Meyer, U., Micciche, A., Muller, E., Portnoi, P., Ripley, S., Robert, M., Robertson, L.V., Rosenbaum-Fabian, S., Sahm, K., Schultz, S., Singleton, K., Sjoqvist, E., Stoelen, L., Terry, A., Thompson, S., Timmer, C., Kerckhove, K. Vande, Ploeg, L. van der, Driessche, M. Van, Rijn, M. van de, Teeffelen-Heithoff, A. van, Vitoria, I., Voillot, C., Wenz, J., Westbrook, M., Wildgoose, J., and Zweers, H.E.
- Abstract
Item does not contain fulltext, Dietary management of 418 adult patients with galactosaemia (from 39 centres/12 countries) was compared. All centres advised lactose restriction, 6 restricted galactose from galactosides +/- fruits and vegetables and 12 offal. 38% (n=15) relaxed diet by: 1) allowing traces of lactose in manufactured foods (n=13) or 2) giving fruits, vegetables and galactosides (n=2). Only 15% (n=6) calculated dietary galactose. 32% of patients were lost to dietetic follow-up. In adult galactosaemia, there is limited diet relaxation.
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- 2015
15. Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach
- Author
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Afdeling Dietetiek, Other research (not in main researchprogram), Aguiar, A., Ahring, K., Almeida, M.F., Assoun, M., Belanger Quintana, A., Bigot, S., Bihet, G., Blom Malmberg, K., Burlina, A., Bushueva, T., Caris, A., Chan, H., Clark, Rachael A, Clark, Sue, Cochrane, B., Corthouts, K., Dalmau, Judith, Dassy, M., Meyer, A., Didycz, B., Diels, M., Dokupil, K., Dubois, S., Eftring, K., Ekengren, J., Ellerton, C., Evans, S., Faria, A., Fischer, A., Ford, S., Freisinger, P., Gizewska, M., Gokmen-Ozel, H., Gribben, J., Gunden, F., Heddrich-Ellerbrok, M., Heiber, S., Heidenborg, C., Jankowski, C., Janssen-Regelink, R.G., Jones, C.I., Jonkers, C.F., Joerg-Streller, M., Kaalund-Hansen, K., Kiss, E., Lammardo, A.M., Lang, K., Lier, D., Lilje, R., Lowry, S., Luyten, K., MacDonald, A., Meyer, U., de Moor, J., Pal, A., Robert, C.M., Robertson, L., Rocha, J.C., Rohde, C., Ross, K., Saruhan, S., Sjöqvist, E., Skeath, R., Stoelen, L., ter Horst, N.M., Terry, A., Timmer, C., Tuncer, N., Vande Kerckhove, K., van den Ploeg, L., van Rijn, M., van Spronsen, Francjan J., van Teeffelen-Heithoff, A., van Wegberg, A., van Wyk, K., Vasconcelos, C., Vitoria, I., Wildgoose, J., Webster, D., White, F.J., Zweers, H., Afdeling Dietetiek, Other research (not in main researchprogram), Aguiar, A., Ahring, K., Almeida, M.F., Assoun, M., Belanger Quintana, A., Bigot, S., Bihet, G., Blom Malmberg, K., Burlina, A., Bushueva, T., Caris, A., Chan, H., Clark, Rachael A, Clark, Sue, Cochrane, B., Corthouts, K., Dalmau, Judith, Dassy, M., Meyer, A., Didycz, B., Diels, M., Dokupil, K., Dubois, S., Eftring, K., Ekengren, J., Ellerton, C., Evans, S., Faria, A., Fischer, A., Ford, S., Freisinger, P., Gizewska, M., Gokmen-Ozel, H., Gribben, J., Gunden, F., Heddrich-Ellerbrok, M., Heiber, S., Heidenborg, C., Jankowski, C., Janssen-Regelink, R.G., Jones, C.I., Jonkers, C.F., Joerg-Streller, M., Kaalund-Hansen, K., Kiss, E., Lammardo, A.M., Lang, K., Lier, D., Lilje, R., Lowry, S., Luyten, K., MacDonald, A., Meyer, U., de Moor, J., Pal, A., Robert, C.M., Robertson, L., Rocha, J.C., Rohde, C., Ross, K., Saruhan, S., Sjöqvist, E., Skeath, R., Stoelen, L., ter Horst, N.M., Terry, A., Timmer, C., Tuncer, N., Vande Kerckhove, K., van den Ploeg, L., van Rijn, M., van Spronsen, Francjan J., van Teeffelen-Heithoff, A., van Wegberg, A., van Wyk, K., Vasconcelos, C., Vitoria, I., Wildgoose, J., Webster, D., White, F.J., and Zweers, H.
- Published
- 2015
16. How strict is galactose restriction in adults with galactosaemia? International practice
- Author
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Afdeling Dietetiek, Other research (not in main researchprogram), Adam, Siddig Momin, Akroyd, R., Bernabei, S., Bollhalder, S., Boocock, S., Burlina, A., Coote, T., Corthouts, K., Dalmau, Judith, Dawson, S., Defourny, S., Meyer, A., Desloovere, A., Devlin, Y., Diels, M., Dokoupil, K., Donald, S., Evans, S., Fasan, I., Ferguson, C., Ford, S., Forga, M., Gallo, G., Grünert, S.C., Heddrich-Ellerbrok, M., Heidenborg, C., Jonkers, C.F., Lefebure, K., Luyten, K., MacDonald, A., Meyer, U., Micciche, A., Müller, E., Portnoi, P., Ripley, S., Robert, C.M., Robertson, L.V., Rosenbaum-Fabian, S., Sahm, K., Schultz, Erica S, Singleton, K., Sjöqvist, E., Stoelen, L., Terry, A., Thompson, S., Timmer, C., Vande Kerckhove, K., van den Ploeg, L., Van Driessche, M., van Rijn, M., van Teeffelen-Heithoff, A., Vitoria, I., Voillot, C., Wenz, J., Westbrook, M., Wildgoose, J., Zweers, H., Afdeling Dietetiek, Other research (not in main researchprogram), Adam, Siddig Momin, Akroyd, R., Bernabei, S., Bollhalder, S., Boocock, S., Burlina, A., Coote, T., Corthouts, K., Dalmau, Judith, Dawson, S., Defourny, S., Meyer, A., Desloovere, A., Devlin, Y., Diels, M., Dokoupil, K., Donald, S., Evans, S., Fasan, I., Ferguson, C., Ford, S., Forga, M., Gallo, G., Grünert, S.C., Heddrich-Ellerbrok, M., Heidenborg, C., Jonkers, C.F., Lefebure, K., Luyten, K., MacDonald, A., Meyer, U., Micciche, A., Müller, E., Portnoi, P., Ripley, S., Robert, C.M., Robertson, L.V., Rosenbaum-Fabian, S., Sahm, K., Schultz, Erica S, Singleton, K., Sjöqvist, E., Stoelen, L., Terry, A., Thompson, S., Timmer, C., Vande Kerckhove, K., van den Ploeg, L., Van Driessche, M., van Rijn, M., van Teeffelen-Heithoff, A., Vitoria, I., Voillot, C., Wenz, J., Westbrook, M., Wildgoose, J., and Zweers, H.
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- 2015
17. Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach
- Author
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Aguiar, A., primary, Ahring, K., additional, Almeida, M.F., additional, Assoun, M., additional, Belanger Quintana, A., additional, Bigot, S., additional, Bihet, G., additional, Blom Malmberg, K., additional, Burlina, A., additional, Bushueva, T., additional, Caris, A., additional, Chan, H., additional, Clark, A., additional, Clark, S., additional, Cochrane, B., additional, Corthouts, K., additional, Dalmau, J., additional, Dassy, M., additional, De Meyer, A., additional, Didycz, B., additional, Diels, M., additional, Dokupil, K., additional, Dubois, S., additional, Eftring, K., additional, Ekengren, J., additional, Ellerton, C., additional, Evans, S., additional, Faria, A., additional, Fischer, A., additional, Ford, S., additional, Freisinger, P., additional, Giżewska, M., additional, Gokmen-Ozel, H., additional, Gribben, J., additional, Gunden, F., additional, Heddrich-Ellerbrok, M., additional, Heiber, S., additional, Heidenborg, C., additional, Jankowski, C., additional, Janssen-Regelink, R., additional, Jones, I., additional, Jonkers, C., additional, Joerg-Streller, M., additional, Kaalund-Hansen, K., additional, Kiss, E., additional, Lammardo, A.M., additional, Lang, K., additional, Lier, D., additional, Lilje, R., additional, Lowry, S., additional, Luyten, K., additional, MacDonald, A., additional, Meyer, U., additional, Moor, D., additional, Pal, A., additional, Robert, M., additional, Robertson, L., additional, Rocha, J.C., additional, Rohde, C., additional, Ross, K., additional, Saruhan, S., additional, Sjöqvist, E., additional, Skeath, R., additional, Stoelen, L., additional, Ter Horst, N.M., additional, Terry, A., additional, Timmer, C., additional, Tuncer, N., additional, Vande Kerckhove, K., additional, van der Ploeg, L., additional, van Rijn, M., additional, van Spronsen, F.J., additional, van Teeffelen-Heithoff, A., additional, van Wegberg, A., additional, van Wyk, K., additional, Vasconcelos, C., additional, Vitoria, I., additional, Wildgoose, J., additional, Webster, D., additional, White, F.J., additional, and Zweers, H., additional
- Published
- 2015
- Full Text
- View/download PDF
18. How strict is galactose restriction in adults with galactosaemia? International practice
- Author
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Adam, S., primary, Akroyd, R., additional, Bernabei, S., additional, Bollhalder, S., additional, Boocock, S., additional, Burlina, A., additional, Coote, T., additional, Corthouts, K., additional, Dalmau, J., additional, Dawson, S., additional, Defourny, S., additional, De Meyer, A., additional, Desloovere, A., additional, Devlin, Y., additional, Diels, M., additional, Dokoupil, K., additional, Donald, S., additional, Evans, S., additional, Fasan, I., additional, Ferguson, C., additional, Ford, S., additional, Forga, M., additional, Gallo, G., additional, Grünert, S.C., additional, Heddrich-Ellerbrok, M., additional, Heidenborg, C., additional, Jonkers, C., additional, Lefebure, K., additional, Luyten, K., additional, MacDonald, A., additional, Meyer, U., additional, Micciche, A., additional, Müller, E., additional, Portnoi, P., additional, Ripley, S., additional, Robert, M., additional, Robertson, L.V., additional, Rosenbaum-Fabian, S., additional, Sahm, K., additional, Schultz, S., additional, Singleton, K., additional, Sjöqvist, E., additional, Stoelen, L., additional, Terry, A., additional, Thompson, S., additional, Timmer, C., additional, Vande Kerckhove, K., additional, van der Ploeg, L., additional, Van Driessche, M., additional, van Rijn, M., additional, van Teeffelen-Heithoff, A., additional, Vitoria, I., additional, Voillot, C., additional, Wenz, J., additional, Westbrook, M., additional, Wildgoose, J., additional, and Zweers, H., additional
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- 2015
- Full Text
- View/download PDF
19. Dietary practices in pyridoxine non-responsive homocystinuria: A European survey.
- Author
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Adam, S., Almeida, M.F., Carbasius Weber, E., Champion, H., Chan, H., Daly, A., Dixon, M., Dokoupil, K., Egli, D., Evans, S., Eyskens, F., Faria, A., Ferguson, C., Hallam, P., Heddrich-Ellerbrok, M., Jacobs, J., Jankowski, C., Lachmann, R., Lilje, R., Link, R., Lowry, S., Luyten, K., Macdonald, A., Maritz, C., Martins, E., Meyer, U., Muller, E., Murphy, E., Robertson, L.V., Rocha, J.C., Saruggia, I., Schick, P., Stafford, J., Stoelen, L., Terry, A., Thom, R., Hurk, T. van den, Rijn, M. van de, Teefelen-Heithoff, A. van, Webster, D., White, F.J., Wildgoose, J., Zweers, H., Adam, S., Almeida, M.F., Carbasius Weber, E., Champion, H., Chan, H., Daly, A., Dixon, M., Dokoupil, K., Egli, D., Evans, S., Eyskens, F., Faria, A., Ferguson, C., Hallam, P., Heddrich-Ellerbrok, M., Jacobs, J., Jankowski, C., Lachmann, R., Lilje, R., Link, R., Lowry, S., Luyten, K., Macdonald, A., Maritz, C., Martins, E., Meyer, U., Muller, E., Murphy, E., Robertson, L.V., Rocha, J.C., Saruggia, I., Schick, P., Stafford, J., Stoelen, L., Terry, A., Thom, R., Hurk, T. van den, Rijn, M. van de, Teefelen-Heithoff, A. van, Webster, D., White, F.J., Wildgoose, J., and Zweers, H.
- Abstract
1 december 2013, Item does not contain fulltext, BACKGROUND: Within Europe, the management of pyridoxine (B6) non-responsive homocystinuria (HCU) may vary but there is limited knowledge about treatment practice. AIM: A comparison of dietetic management practices of patients with B6 non-responsive HCU in European centres. METHODS: A cross-sectional audit by questionnaire was completed by 29 inherited metabolic disorder (IMD) centres: (14 UK, 5 Germany, 3 Netherlands, 2 Switzerland, 2 Portugal, 1 France, 1 Norway, 1 Belgium). RESULTS: 181 patients (73% >16years of age) with HCU were identified. The majority (66%; n=119) were on dietary treatment (1-10years, 90%; 11-16years, 82%; and >16years, 58%) with or without betaine and 34% (n=62) were on betaine alone. The median natural protein intake (g/day) on diet only was, by age: 1-10years, 12g; 11-16years, 11g; and >16years, 45g. With diet and betaine, median natural protein intake (g/day) by age was: 1-10years, 13g; 11-16years, 20g; and >16years, 38g. Fifty-two percent (n=15) of centres allocated natural protein by calculating methionine rather than a protein exchange system. A methionine-free l-amino acid supplement was prescribed for 86% of diet treated patients. Fifty-two percent of centres recommended cystine supplements for low plasma concentrations. Target treatment concentrations for homocystine/homocysteine (free/total) and frequency of biochemical monitoring varied. CONCLUSION: In B6 non-responsive HCU the prescription of dietary restriction by IMD centres declined with age, potentially associated with poor adherence in older patients. Inconsistencies in biochemical monitoring and treatment indicate the need for international consensus guidelines.
- Published
- 2013
20. Dietary management of urea cycle disorders: European practice.
- Author
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Adam, S., Almeida, M.F., Assoun, M., Baruteau, J., Bernabei, S.M., Bigot, S., Champion, H., Daly, A., Dassy, M., Dawson, S., Dixon, M., Dokoupil, K., Dubois, S., Dunlop, C., Evans, S., Eyskens, F., Faria, A., Favre, E., Ferguson, C., Goncalves, C., Gribben, J., Heddrich-Ellerbrok, M., Jankowski, C., Janssen-Regelink, R.G., Jouault, C., Laguerre, C., Verge, S. Le, Link, R., Lowry, S., Luyten, K., Macdonald, A., Maritz, C., McDowell, S., Meyer, U., Micciche, A., Robert, M., Robertson, L.V., Rocha, J.C., Rohde, C., Saruggia, I., Sjoqvist, E., Stafford, J., Terry, A., Thom, R., nde Kerckhove, K. Va, Rijn, M. van de, Teeffelen-Heithoff, A. van, Wegberg, A.M.J. van, Wyk, K. van, Vasconcelos, C., Vestergaard, H., Webster, D., White, F.J., Wildgoose, J., Zweers, H., Adam, S., Almeida, M.F., Assoun, M., Baruteau, J., Bernabei, S.M., Bigot, S., Champion, H., Daly, A., Dassy, M., Dawson, S., Dixon, M., Dokoupil, K., Dubois, S., Dunlop, C., Evans, S., Eyskens, F., Faria, A., Favre, E., Ferguson, C., Goncalves, C., Gribben, J., Heddrich-Ellerbrok, M., Jankowski, C., Janssen-Regelink, R.G., Jouault, C., Laguerre, C., Verge, S. Le, Link, R., Lowry, S., Luyten, K., Macdonald, A., Maritz, C., McDowell, S., Meyer, U., Micciche, A., Robert, M., Robertson, L.V., Rocha, J.C., Rohde, C., Saruggia, I., Sjoqvist, E., Stafford, J., Terry, A., Thom, R., nde Kerckhove, K. Va, Rijn, M. van de, Teeffelen-Heithoff, A. van, Wegberg, A.M.J. van, Wyk, K. van, Vasconcelos, C., Vestergaard, H., Webster, D., White, F.J., Wildgoose, J., and Zweers, H.
- Abstract
1 december 2013, Item does not contain fulltext, BACKGROUND: There is no published data comparing dietary management of urea cycle disorders (UCD) in different countries. METHODS: Cross-sectional data from 41 European Inherited Metabolic Disorder (IMD) centres (17 UK, 6 France, 5 Germany, 4 Belgium, 4 Portugal, 2 Netherlands, 1 Denmark, 1 Italy, 1 Sweden) was collected by questionnaire describing management of patients with UCD on prescribed protein restricted diets. RESULTS: Data for 464 patients: N-acetylglutamate synthase (NAGS) deficiency, n=10; carbamoyl phosphate synthetase (CPS1) deficiency, n=29; ornithine transcarbamoylase (OTC) deficiency, n=214; citrullinaemia, n=108; argininosuccinic aciduria (ASA), n=80; arginase deficiency, n=23 was reported. The majority of patients (70%; n=327) were aged 0-16y and 30% (n=137) >16y. Prescribed median protein intake/kg body weight decreased with age with little variation between disorders. The UK tended to give more total protein than other European countries particularly in infancy. Supplements of essential amino acids (EAA) were prescribed for 38% [n=174] of the patients overall, but were given more commonly in arginase deficiency (74%), CPS (48%) and citrullinaemia (46%). Patients in Germany (64%), Portugal (67%) and Sweden (100%) were the most frequent users of EAA. Only 18% [n=84] of patients were prescribed tube feeds, most commonly for CPS (41%); and 21% [n=97] were prescribed oral energy supplements. CONCLUSIONS: Dietary treatment for UCD varies significantly between different conditions, and between and within European IMD centres. Further studies examining the outcome of treatment compared with the type of dietary therapy and nutritional support received are required.
- Published
- 2013
21. Dietary management of urea cycle disorders: European practice
- Author
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UCL - (SLuc) Centre de pathologie anorectale de l'enfant, Adam, S., Almeida, M.F., Assoun, M., Baruteau, J., Bernabei, S.M., Bigot, S., Champion, H., Daly, A., Dassy, Martine, Dawson, S., Dixon, M., Dokoupil, K., Dubois, S., Dunlop, C., Evans, S., Eyskens, F., Faria, A., Favre, E., Ferguson, C., Goncalves, C., Gribben, J., Heddrich-Ellerbrok, M., Jankowski, C., Janssen-Regelink, R., Jouault, C., Laguerre, C., Le Verge, S., Link, R., Lowry, S., Luyten, K., MacDonald, A., Maritz, C., McDowell, S., Meyer, U., Micciche, A., Robert, M., Robertson, L.V., Rocha, J.C., Rohde, C., Saruggia, I., Sjoqvist, E., Stafford, J., Terry, A., Thom, R., Vande Kerckhove, K., van Rijn, M., van Teeffelen-Heithoff, A., Wegberg, A.van, van Wyk, K., Vasconcelos, C., Vestergaard, H., Webster, D., White, F.J., Wildgoose, J., Zweers, H., UCL - (SLuc) Centre de pathologie anorectale de l'enfant, Adam, S., Almeida, M.F., Assoun, M., Baruteau, J., Bernabei, S.M., Bigot, S., Champion, H., Daly, A., Dassy, Martine, Dawson, S., Dixon, M., Dokoupil, K., Dubois, S., Dunlop, C., Evans, S., Eyskens, F., Faria, A., Favre, E., Ferguson, C., Goncalves, C., Gribben, J., Heddrich-Ellerbrok, M., Jankowski, C., Janssen-Regelink, R., Jouault, C., Laguerre, C., Le Verge, S., Link, R., Lowry, S., Luyten, K., MacDonald, A., Maritz, C., McDowell, S., Meyer, U., Micciche, A., Robert, M., Robertson, L.V., Rocha, J.C., Rohde, C., Saruggia, I., Sjoqvist, E., Stafford, J., Terry, A., Thom, R., Vande Kerckhove, K., van Rijn, M., van Teeffelen-Heithoff, A., Wegberg, A.van, van Wyk, K., Vasconcelos, C., Vestergaard, H., Webster, D., White, F.J., Wildgoose, J., and Zweers, H.
- Abstract
Background: There is no published data comparing dietary management of urea cycle disorders (UCD) in different countries. Methods: Cross-sectional data from 41 European Inherited Metabolic Disorder (IMD) centres (17 UK, 6 France, 5 Germany, 4 Belgium, 4 Portugal, 2 Netherlands, 1 Denmark, 1 Italy, 1 Sweden) was collected by questionnaire describing management of patients with UCD on prescribed protein restricted diets. Results: Data for 464 patients: N-acetylglutamate synthase (NAGS) deficiency, n=10; carbamoyl phosphate synthetase (CPS1) deficiency, n=29; ornithine transcarbamoylase (OTC) deficiency, n=214; citrullinaemia, n=108; argininosuccinic aciduria (ASA), n=80; arginase deficiency, n=23 was reported. The majority of patients (70%; n=327) were aged 0-16. y and 30% (n=137) >. 16. y. Prescribed median protein intake/kg body weight decreased with age with little variation between disorders. The UK tended to give more total protein than other European countries particularly in infancy. Supplements of essential amino acids (EAA) were prescribed for 38% [n=174] of the patients overall, but were given more commonly in arginase deficiency (74%), CPS (48%) and citrullinaemia (46%). Patients in Germany (64%), Portugal (67%) and Sweden (100%) were the most frequent users of EAA. Only 18% [n=84] of patients were prescribed tube feeds, most commonly for CPS (41%); and 21% [n=97] were prescribed oral energy supplements. Conclusions: Dietary treatment for UCD varies significantly between different conditions, and between and within European IMD centres. Further studies examining the outcome of treatment compared with the type of dietary therapy and nutritional support received are required. © 2013 Elsevier Inc.
- Published
- 2013
22. Dietary practices in pyridoxine non-responsive homocystinuria: A European survey
- Author
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Adam, S., primary, Almeida, M.F., additional, Carbasius Weber, E., additional, Champion, H., additional, Chan, H., additional, Daly, A., additional, Dixon, M., additional, Dokoupil, K., additional, Egli, D., additional, Evans, S., additional, Eyskens, F., additional, Faria, A., additional, Ferguson, C., additional, Hallam, P., additional, Heddrich-Ellerbrok, M., additional, Jacobs, J., additional, Jankowski, C., additional, Lachmann, R., additional, Lilje, R., additional, Link, R., additional, Lowry, S., additional, Luyten, K., additional, MacDonald, A., additional, Maritz, C., additional, Martins, E., additional, Meyer, U., additional, Müller, E., additional, Murphy, E., additional, Robertson, L.V., additional, Rocha, J.C., additional, Saruggia, I., additional, Schick, P., additional, Stafford, J., additional, Stoelen, L., additional, Terry, A., additional, Thom, R., additional, van den Hurk, T., additional, van Rijn, M., additional, van Teefelen-Heithoff, A., additional, Webster, D., additional, White, F.J., additional, Wildgoose, J., additional, and Zweers, H., additional
- Published
- 2013
- Full Text
- View/download PDF
23. Dietary management of urea cycle disorders: European practice
- Author
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Adam, S., primary, Almeida, M.F., additional, Assoun, M., additional, Baruteau, J., additional, Bernabei, S.M., additional, Bigot, S., additional, Champion, H., additional, Daly, A., additional, Dassy, M., additional, Dawson, S., additional, Dixon, M., additional, Dokoupil, K., additional, Dubois, S., additional, Dunlop, C., additional, Evans, S., additional, Eyskens, F., additional, Faria, A., additional, Favre, E., additional, Ferguson, C., additional, Goncalves, C., additional, Gribben, J., additional, Heddrich-Ellerbrok, M., additional, Jankowski, C., additional, Janssen-Regelink, R., additional, Jouault, C., additional, Laguerre, C., additional, Le Verge, S., additional, Link, R., additional, Lowry, S., additional, Luyten, K., additional, MacDonald, A., additional, Maritz, C., additional, McDowell, S., additional, Meyer, U., additional, Micciche, A., additional, Robert, M., additional, Robertson, L.V., additional, Rocha, J.C., additional, Rohde, C., additional, Saruggia, I., additional, Sjoqvist, E., additional, Stafford, J., additional, Terry, A., additional, Thom, R., additional, Vande Kerckhove, K., additional, van Rijn, M., additional, van Teeffelen-Heithoff, A., additional, Wegberg, A.van, additional, van Wyk, K., additional, Vasconcelos, C., additional, Vestergaard, H., additional, Webster, D., additional, White, F.J., additional, Wildgoose, J., additional, and Zweers, H., additional
- Published
- 2013
- Full Text
- View/download PDF
24. Weaning infants with phenylketonuria: a review
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MacDonald, A., primary, Evans, S., additional, Cochrane, B., additional, and Wildgoose, J., additional
- Published
- 2011
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25. The Exeter half-day release psychotherapy training scheme – a model for others?
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Wildgoose, J., primary, McCrindle, D., additional, and Tillett, R., additional
- Published
- 2002
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26. ChemInform Abstract: ALKYL N-PHENYLCARBAMOYLAZOFORMATES, A NOVEL CLASS OF UNSYMMETRICAL AZODICARBONYL ENOPHILES OF MODERATE REACTIVITY
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KNIGHT, G. T., primary, LOADMAN, M. J. R., additional, SAVILLE, B., additional, and WILDGOOSE, J., additional
- Published
- 1974
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27. An assessment of the clastogenic potential of 4-chloromethylbiphenyl in human lymphocytes in vitro
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Richardson, C.R. and Wildgoose, J.
- Published
- 1982
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28. Psychological and emotional problems in staff following assaults by patients
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Wildgoose, J.
- Published
- 2003
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29. Combining Enhanced Resolving Power with Duty Cycle Improvements on a Multi-Reflecting Time-of-Flight Mass Spectrometer.
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Johnson WJ, Palmer ME, Claude E, McCullagh M, Nixon P, and Wildgoose J
- Abstract
The combination of enhanced resolving power and improved duty cycle on a multireflecting time-of-flight mass spectrometer is described. Resolving power increases are achieved by extending the effective ion path length from 47 m to greater than 200 m. Path length increases are achieved through containment of ions within the analyzer for up to N = 5 passes using a pulsed deflection electrode. Resolving power was shown to increase from 220,000 to 402,000 (fwhm) at m / z 785 for N = 1 and N = 4 analyzer passes, respectively. Due to the timing of the pulsed deflection electrode, the approach is particularly suited to high resolution analysis over a targeted m / z range. Duty cycle enhancements are achieved for ions of the targeted m / z range via accumulation prior to orthogonal acceleration, providing signal improvements of 2 orders of magnitude. Achieving such high resolving powers at fast scan rates (30 Hz) can yield additional information such as fine isotope structure; when combined with ppb mass measurement accuracy, high confidence in analyte identification can be achieved. The technique is applied for N = 2 analyzer passes, demonstrating fine isotope structure for a typical UHPLC metabolite identification experiment at a 10 Hz acquisition rate. Additionally, mass spectrometry imaging data is acquired using DESI, demonstrating the improved image clarity achieved at >300,000 (fwhm).
- Published
- 2024
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- View/download PDF
30. Novel Hybrid Quadrupole-Multireflecting Time-of-Flight Mass Spectrometry System.
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Cooper-Shepherd DA, Wildgoose J, Kozlov B, Johnson WJ, Tyldesley-Worster R, Palmer ME, Hoyes JB, McCullagh M, Jones E, Tonge R, Marsden-Edwards E, Nixon P, Verenchikov A, and Langridge JI
- Abstract
A novel mass spectrometry system is described here comprising a quadrupole-multireflecting time-of-flight design. The new multireflecting time-of-flight analyzer has an effective path length of 48 m and employs planar, gridless ion mirrors providing fourth-order energy focusing resulting in resolving power over 200 000 fwhm and sub-ppm mass accuracy. We show how these attributes are maintained with relatively fast acquisition speeds, setting the system apart from other high resolution mass spectrometers. We have integrated this new system into both liquid chromatography-mass spectrometry and mass spectrometry imaging workflows to demonstrate how the instrument characteristics are of benefit to these applications.
- Published
- 2023
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31. Phenylalanine free infant formula in the dietary management of phenylketonuria.
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Yilmaz O, Cochrane B, Wildgoose J, Pinto A, Evans S, Daly A, Ashmore C, and MacDonald A
- Subjects
- Infant, Male, Humans, Prospective Studies, Phenylalanine, Proteins, Infant Formula, Phenylketonurias
- Abstract
Background: Phenylalanine-free infant formula is an essential source of safe protein in a phenylalanine restricted diet, but its efficacy is rarely studied. We report a multicentre, open, longitudinal, prospective intervention study on a phenylalanine-free infant formula (PKU Start: Vitaflo International Ltd.)., Results: This was a 2-part study: part I (28 days short term evaluation) and part II (12 months extension). Data was collected on infant blood phenylalanine concentrations, dietary intake, growth, and gastrointestinal tolerance. Ten infants (n = 8 males, 80%), with a median age of 14 weeks (range 4-36 weeks) were recruited from 3 treatment centres in the UK. Nine of ten infants completed the 28-day follow-up (one caregiver preferred the usual phenylalanine-free formula and discontinued the study formula after day 14) and 7/9 participated in study part II. The phenylalanine-free infant formula contributed a median of 57% (IQR 50-62%) energy and 53% (IQR 33-66%) of total protein intake from baseline to the end of the part II extension study. During the 12-month follow-up, infants maintained normal growth and satisfactory blood phenylalanine control. Any early gastrointestinal symptoms (constipation, colic, vomiting and poor feeding) improved with time., Conclusion: The study formula was well tolerated, helped maintain good metabolic control, and normal growth in infants with PKU. The long-term efficacy of phenylalanine-free infant formula should continue to be observed and monitored., (© 2023. The Author(s).)
- Published
- 2023
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- View/download PDF
32. British Thoracic Society Quality Standard for Clinically Significant Bronchiectasis in Adults 2022.
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Hill AT, Grillo L, Gruffydd-Jones K, Payne K, Souto M, Sullivan A, Wildgoose J, and Loebinger MR
- Subjects
- Adult, Humans, Respiratory Therapy, Societies, Medical, Bronchiectasis therapy
- Abstract
This British Thoracic Society Quality Standard for Clinically Significant Bronchiectasis in Adults 2022 aims to encourage good practice by setting standards of high-quality respiratory care that services should follow., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)
- Published
- 2022
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- View/download PDF
33. Surface-induced dissociation of protein complexes on a cyclic ion mobility spectrometer.
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Snyder DT, Jones BJ, Lin YF, Cooper-Shepherd DA, Hewitt D, Wildgoose J, Brown JM, Langridge JI, and Wysocki VH
- Subjects
- Mass Spectrometry, Proteins, Software
- Abstract
We describe the implementation of a simple three-electrode surface-induced dissociation (SID) cell on a cyclic ion mobility spectrometer (cIMS) and demonstrate the utility of multipass mobility separations for resolving multiple conformations of protein complexes generated during collision-induced and surface-induced unfolding (CIU & SIU) experiments. In addition to CIU and SIU, SID of protein complexes is readily accomplished within the native instrument software and with no additional external power supplies by entering a single SID collision energy, a simplification in user experience compared to prior implementations. A set of cyclic homomeric protein complexes and a heterohexamer with known CID and SID behavior were analyzed to investigate mass and mobility resolution improvements, the latter of which improved by 20-50% (median: 33%) compared to a linear travelling wave device. Multiple passes of intact complexes, or their SID fragments, increased the mobility resolution by an average of 15% per pass, with the racetrack effect being observed after ∼3 or 4 passes, depending on the drift time spread of the analytes. Even with modest improvements to apparent mobility resolving power, multipass experiments were particularly useful for separating conformations produced from CIU and SIU experiments. We illustrate several examples where either (1) multipass experiments revealed multiple overlapping conformations previously unobserved or obscured due to limited mobility resolution, or (2) CIU or SIU conformations that appeared 'native' in a single pass experiment were actually slightly compacted or expanded, with the change only being measurable through multipass experiments. The work conducted here, the first utilization of multipass cyclic ion mobility for CIU, SIU, and SID of protein assemblies and a demonstration of a wholly integrated SIU/SID workflow, paves the way for widespread adoption of SID technology for native mass spectrometry and also improves our understanding of gas-phase protein complex CIU and SIU conformationomes.
- Published
- 2021
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34. Managing bronchiectasis in adults in primary care: a clinical update.
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Gruffydd-Jones K, Keeley D, Wildgoose J, and Hill A
- Subjects
- Adult, Humans, Primary Health Care, Bronchiectasis diagnosis, Bronchiectasis therapy
- Published
- 2021
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- View/download PDF
35. Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements.
- Author
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Evans S, Adam S, Adams S, Allen H, Ashmore C, Bailey S, Banks J, Churchill H, Cochrane B, Cook J, Dale C, Daly A, Dixon M, Dunlop C, Ellerton C, Emm A, Firman S, Ford S, French M, Gribben J, Grimsley A, Herlihy I, Hill M, Judd S, Lang K, Males J, McDonald J, McStravick N, Millington C, Newby C, Noble C, Pereira R, Pinto A, Robertson L, Robotham A, Ross K, Singleton K, Skeath R, Terry A, Van Wyk K, White F, White L, Wildgoose J, Woodall A, and MacDonald A
- Subjects
- Consensus, Delphi Technique, Diet, Protein-Restricted methods, Food Labeling standards, Humans, United Kingdom, Diet, Protein-Restricted standards, Dietary Proteins analysis, Dietetics standards, Phenylalanine analysis, Phenylketonurias diet therapy
- Abstract
In phenylketonuria (PKU), variable dietary advice provided by health professionals and social media leads to uncertainty for patients/caregivers reliant on accurate, evidence based dietary information. Over four years, 112 consensus statements concerning the allocation of foods in a low phenylalanine diet for PKU were developed by the British Inherited Metabolic Disease Dietitians Group (BIMDG-DG) from 34 PKU treatment centres, utilising 10 rounds of Delphi consultation to gain a majority (≥75%) decision. A mean of 29 UK dietitians (range: 18-40) and 18 treatment centres (range: 13-23) contributed in each round. Statements encompassed all foods/food groups divided into four categories based on defined protein/phenylalanine content: (1) foods high in protein/phenylalanine (best avoided); (2) foods allowed without restriction including fruit/vegetables containing phenylalanine ≤75 mg/100 g and most foods containing protein ≤0.5 g/100 g; (3) foods that should be calculated/weighed as an exchange food if they contain protein exchange ingredients (categorized into foods with a protein content of: >0.1 g/100 g (milk/plant milks only), >0.5 g/100 g (bread/pasta/cereal/flours), >1 g/100 g (cook-in/table-top sauces/dressings), >1.5 g/100 g (soya sauces)); and (4) fruit/vegetables containing phenylalanine >75 mg/100 g allocated as part of the protein/phenylalanine exchange system. These statements have been endorsed and translated into practical dietary management advice by the medical advisory dietitians for the National Society for PKU (NSPKU).
- Published
- 2020
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- View/download PDF
36. Mealtime Anxiety and Coping Behaviour in Parents and Children During Weaning in PKU: A Case-Control Study.
- Author
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Evans S, Daly A, Wildgoose J, Cochrane B, Ashmore C, Kearney S, and MacDonald A
- Subjects
- Adaptation, Psychological, Child, Preschool, Female, Health Surveys, Humans, Infant, Longitudinal Studies, Male, Phenylalanine blood, Prospective Studies, Surveys and Questionnaires, Weaning, Anxiety psychology, Meals psychology, Parents psychology, Phenylketonurias psychology
- Abstract
Solid food introduction may create anxiety for parents of children with phenylketonuria (PKU) due to the burden associated with protein substitute (PS) administration and natural protein restriction. In a longitudinal, prospective study, 20 mothers of children with PKU and 20 non-PKU control mothers completed 4 questionnaires (mealtime emotions, feed-time, Beck's anxiety inventory and the coping health inventory for parents), examining parent/child mealtime emotions, anxiety, stress and coping strategies at child ages: weaning start, 8 months (m), 12 m, 15 m, 18 m and 24 m. Overall, mothers of children with PKU cope well with solid food introduction when applying a low-phenylalanine diet, with comparable low levels of stress and anxiety reported in both PKU and non-PKU groups. However, mothers of children with PKU reported peak scores in anxiety for emotive/cognitive symptoms at a child age of 15 m, and higher use of coping strategies at 15 m and 24 m ( p < 0.05) of age. Generally, there was a trend that maternal anxiety regarding child rejection of PS increased with time, peaking between 12-24 m. In PKU, a child age of 12-18 m is identified as a key period when mothers feel most anxious/stressed with feeding, coinciding with raised blood phenylalanine levels probably associated with teething, illness and developing independence. Health professionals should be conscious of this vulnerable period and be prepared to offer more directional support as required., Competing Interests: B.C. received financial support from Nutricia Ltd and Vitaflo International Ltd to attend study days, meetings and conferences, and Nutricia Ltd for donating to patient events. A.D. received research funding from Vitaflo and financial support from Nutricia and Vitaflo to attend study days and conferences. S.E. received research funding from Nutricia and financial support from Nutricia and Vitaflo to attend study days and conferences. A.M. received research funding and honoraria from Nutricia, Vitaflo International and Merck Serono, Member of European Nutrition Expert Panel (Merck Serono international), Member of Sapropterin Advisory Board (Merck Serono international), Member of the Advisory Board Element (Danone-Nutricia). This was a study sponsored by industry (Nutricia Ltd), they contributed to the study design and monitored the study but the idea for the study was initiated by an independent researcher. Nutricia were blinded to the statistics. This was done by the lead researcher. Industry contributed to the NHS Trust for researchers’ time costs. The funders had no role in the collection, analyses, or interpretation of data, in the writing of the manuscript, or in the decision to publish the results.
- Published
- 2019
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37. A Cyclic Ion Mobility-Mass Spectrometry System.
- Author
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Giles K, Ujma J, Wildgoose J, Pringle S, Richardson K, Langridge D, and Green M
- Abstract
Improvements in the performance and availability of commercial instrumentation have made ion mobility-mass spectrometry (IM-MS) an increasingly popular approach for the structural analysis of ionic species as well as for separation of complex mixtures. Here, a new research instrument is presented which enables complex experiments, extending the current scope of IM technology. The instrument is based on a Waters SYNAPT G2-S i IM-MS platform, with the IM separation region modified to accept a cyclic ion mobility (cIM) device. The cIM region consists of a 98 cm path length, closed-loop traveling wave (TW)-enabled IM separator positioned orthogonally to the main ion optical axis. A key part of this geometry and its flexibility is the interface between the ion optical axis and the cIM, where a planar array of electrodes provides control over the TW direction and subsequent ion motion. On either side of the array, there are ion guides used for injection, ejection, storage, and activation of ions. In addition to single and multipass separations around the cIM, providing selectable mobility resolution, the instrument design and control software enable a range of "multifunction" experiments such as mobility selection, activation, storage, IMS
n , and importantly custom combinations of these functions. Here, the design and performance of the cIM-MS instrument is highlighted, with a mobility resolving power of approximately 750 demonstrated for 100 passes around the cIM device using a reverse sequence peptide pair. The multifunction capabilities are demonstrated through analysis of three isomeric pentasaccharide species and the small protein ubiquitin.- Published
- 2019
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- View/download PDF
38. Cyclic Ion Mobility Mass Spectrometry Distinguishes Anomers and Open-Ring Forms of Pentasaccharides.
- Author
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Ujma J, Ropartz D, Giles K, Richardson K, Langridge D, Wildgoose J, Green M, and Pringle S
- Abstract
There is increasing biopharmaceutical interest in oligosaccharides and glycosylation. A key requirement for these sample types is the ability to characterize the chain length, branching, type of monomers, and importantly stereochemistry and anomeric configuration. Herein, we showcase the multi-function capability of a cyclic ion mobility (cIM) separator embedded in a quadrupole/time-of-flight mass spectrometer (Q-ToF MS). The instrument design enables selective activation of mobility-separated precursors followed by cIM separation of product ions, an approach analogous to MS
n . Using high cIM resolution, we demonstrate the separation of three isomeric pentasaccharides and, moreover, that three components are present for each compound. We show that structural differences between product ions reflect the precursor differences in some cases but not others. These findings are corroborated by a heavy oxygen labelling approach. Using this methodology, the identity of fragment ions may be assigned. This enables us to postulate that the two main components observed for each pentasaccharide are anomeric forms. The remaining low abundance component is assigned as an open-ring form.- Published
- 2019
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39. Growth, Protein and Energy Intake in Children with PKU Taking a Weaning Protein Substitute in the First Two Years of Life: A Case-Control Study.
- Author
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Evans S, Daly A, Wildgoose J, Cochrane B, Chahal S, Ashmore C, Loveridge N, and MacDonald A
- Subjects
- Body Height physiology, Body Weight physiology, Case-Control Studies, Cephalometry, Child, Preschool, Energy Intake, Female, Head, Humans, Infant, Longitudinal Studies, Male, Nutrients analysis, Prospective Studies, Dietary Proteins administration & dosage, Dietary Supplements, Phenylketonurias diet therapy, Phenylketonurias physiopathology, Weaning
- Abstract
Growth issues have been observed in young children with phenylketonuria (PKU), but studies are conflicting. In infancy, there is an increasing trend to introduce a second-stage semi-solid weaning protein substitute (WPS) but there is concern that this may not meet energy requirements. In this longitudinal, prospective study, 20 children with PKU transitioning to a WPS, and 20 non-PKU controls were observed monthly from weaning commencement (4⁻6 months) to 12 m and at 15, 18 and 24 months of age for: weight, length, head circumference, body mass index (BMI), energy and macronutrient intake. Growth parameters were within normal range at all ages in both groups with no significant difference in mean z-scores except for accelerated length in the PKU group. No child with PKU had z-scores < -2 for any growth parameter at age 2 years. Total protein and energy intake in both groups were similar at all ages; however, from 12⁻24 months in the PKU group, the percentage of energy intake from carbohydrate increased (60%) but from fat decreased (25%) and inversely for controls (48% and 36%). In PKU, use of low volume WPS meets Phe-free protein requirements, facilitates transition to solid foods and supports normal growth. Further longitudinal study of growth, body composition and energy/nutrient intakes in early childhood are required to identify any changing trends.
- Published
- 2019
- Full Text
- View/download PDF
40. How Does Feeding Development and Progression onto Solid Foods in PKU Compare with Non-PKU Children During Weaning?
- Author
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Evans S, Daly A, Wildgoose J, Cochrane B, Chahal S, Ashmore C, Loveridge N, and MacDonald A
- Subjects
- Case-Control Studies, Dietary Proteins administration & dosage, Feeding Behavior, Female, Humans, Infant, Male, Foods, Specialized, Phenylalanine administration & dosage, Phenylketonurias, Weaning
- Abstract
Weaning is complex for children with phenylketonuria (PKU). Breastmilk/infant formula and phenylalanine (Phe)-free infant protein-substitute (PS) are gradually replaced with equivalent amounts of Phe-containing food, a semi-solid/spoonable weaning PS and special low-protein foods. In PKU, feeding patterns/practices during weaning in PKU have not been formally evaluated. In this longitudinal, prospective, case-control study ( n = 20) infants with PKU transitioning to a second-stage PS, were recruited at weaning (4⁻6 months) for a comparison of feeding practices and development with non-PKU infants. Subjects were monitored monthly to 12 months and at age 15 months, 18 months and 24 months for: feeding progression; food textures; motor skill development and self-feeding; feeding environment; gastrointestinal symptoms; and negative feeding behaviours. Children with PKU had comparable weaning progression to non-PKU infants including texture acceptance, infant formula volume and self-feeding skills. However, children with PKU had more prolonged Phe-free infant formula bottle-feeding and parental spoon feeding than controls; fewer meals/snacks per day; and experienced more flatulence ( p = 0.0005), burping ( p = 0.001), retching ( p = 0.03); and less regurgitation ( p = 0.003). Negative behaviours associated with PS at age 10⁻18 months, coincided with the age of teething. Use of semi-solid PS in PKU supports normal weaning development/progression but parents require support to manage the complexity of feeding and to normalise the social inclusivity of their child's family food environment. Further study regarding parental anxiety associated with mealtimes is required.
- Published
- 2019
- Full Text
- View/download PDF
41. Weaning practices in phenylketonuria vary between health professionals in Europe.
- Author
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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
42. Scanning Quadrupole Data-Independent Acquisition, Part B: Application to the Analysis of the Calcineurin-Interacting Proteins during Treatment of Aspergillus fumigatus with Azole and Echinocandin Antifungal Drugs.
- Author
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Juvvadi PR, Moseley MA, Hughes CJ, Soderblom EJ, Lennon S, Perkins SR, Thompson JW, Geromanos SJ, Wildgoose J, Richardson K, Langridge JI, Vissers JPC, and Steinbach WJ
- Subjects
- Antifungal Agents pharmacology, Aspergillus fumigatus chemistry, Aspergillus fumigatus genetics, Aspergillus fumigatus metabolism, Calcineurin genetics, Calcineurin metabolism, Caspofungin, Cell Membrane chemistry, Cell Membrane drug effects, Cell Membrane metabolism, Cell Wall chemistry, Cell Wall drug effects, Cell Wall metabolism, Chromatography, Liquid methods, Echinocandins pharmacology, Ergosterol biosynthesis, Fungal Proteins genetics, Fungal Proteins metabolism, Gene Expression, Gene Ontology, Genes, Reporter, Green Fluorescent Proteins genetics, Green Fluorescent Proteins metabolism, Immunoprecipitation, Lipopeptides pharmacology, Micafungin, Molecular Sequence Annotation, Protein Binding, Protein Interaction Mapping, Ribosomal Proteins genetics, Ribosomal Proteins metabolism, Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization methods, Aspergillus fumigatus drug effects, Calcineurin isolation & purification, Fungal Proteins isolation & purification, Ribosomal Proteins isolation & purification, Voriconazole pharmacology
- Abstract
Calcineurin is a critical cell-signaling protein that orchestrates growth, stress response, virulence, and antifungal drug resistance in several fungal pathogens. Blocking calcineurin signaling increases the efficacy of several currently available antifungals and suppresses drug resistance. We demonstrate the application of a novel scanning quadrupole DIA method for the analysis of changes in the proteins coimmunoprecipitated with calcineurin during therapeutic antifungal drug treatments of the deadly human fungal pathogen Aspergillus fumigatus. Our experimental design afforded an assessment of the precision of the method as demonstrated by peptide- and protein-centric analysis from eight replicates of the study pool QC samples. Two distinct classes of clinically relevant antifungal drugs that are guideline recommended for the treatment of invasive "aspergillosis" caused by Aspergillus fumigatus, the azoles (voriconazole) and the echinocandins (caspofungin and micafungin), which specifically target the fungal plasma membrane and the fungal cell wall, respectively, were chosen to distinguish variations occurring in the proteins coimmunoprecipitated with calcineurin. Novel potential interactors were identified in response to the different drug treatments that are indicative of the possible role for calcineurin in regulating these effectors. Notably, treatment with voriconazole showed increased immunoprecipitation of key proteins involved in membrane ergosterol biosynthesis with calcineurin. In contrast, echinocandin (caspofungin or micafungin) treatments caused increased immunoprecipitation of proteins involved in cell-wall biosynthesis and septation. Furthermore, abundant coimmunoprecipitation of ribosomal proteins with calcineurin occurred exclusively in echinocandins treatment, indicating reprogramming of cellular growth mechanisms during different antifungal drug treatments. While variations in the observed calcineurin immunoprecipitated proteins may also be due to changes in their expression levels under different drug treatments, this study suggests an important role for calcineurin-dependent cellular mechanisms in response to antifungal treatment of A. fumigatus that warrants future studies.
- Published
- 2018
- Full Text
- View/download PDF
43. Scanning Quadrupole Data-Independent Acquisition, Part A: Qualitative and Quantitative Characterization.
- Author
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Moseley MA, Hughes CJ, Juvvadi PR, Soderblom EJ, Lennon S, Perkins SR, Thompson JW, Steinbach WJ, Geromanos SJ, Wildgoose J, Langridge JI, Richardson K, and Vissers JPC
- Subjects
- Amino Acid Sequence, Chromatography, Liquid instrumentation, Chromatography, Liquid methods, Complex Mixtures chemistry, HeLa Cells, Humans, K562 Cells, Proteolysis, Proteomics instrumentation, Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization instrumentation, Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization methods, Blood Proteins isolation & purification, Escherichia coli chemistry, Proteome isolation & purification, Proteomics methods
- Abstract
A novel data-independent acquisition (DIA) method incorporating a scanning quadrupole in front of a collision cell and orthogonal acceleration time-of-flight mass analyzer is described. The method has been characterized for the qualitative and quantitative label-free proteomic analysis of complex biological samples. The principle of the scanning quadrupole DIA method is discussed, and analytical instrument characteristics, such as the quadrupole transmission width, scan/integration time, and chromatographic separation, have been optimized in relation to sample complexity for a number of different model proteomes of varying complexity and dynamic range including human plasma, cell lines, and bacteria. In addition, the technological merits over existing DIA approaches are described and contrasted. The qualitative and semiquantitative performance of the method is illustrated for the analysis of relatively simple protein digest mixtures and a well-characterized human cell line sample using untargeted and targeted search strategies. Finally, the results from a human cell line were compared against publicly available data that used similar chromatographic conditions but were acquired with DDA technology and alternative mass analyzer systems. Qualitative comparison showed excellent concordance of results with >90% overlap of the detected proteins.
- Published
- 2018
- Full Text
- View/download PDF
44. Lipid profiling of complex biological mixtures by liquid chromatography/mass spectrometry using a novel scanning quadrupole data-independent acquisition strategy.
- Author
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Gethings LA, Richardson K, Wildgoose J, Lennon S, Jarvis S, Bevan CL, Vissers JPC, and Langridge JI
- Subjects
- Animals, Cattle, Male, Metabolomics methods, Mice, Myocardium chemistry, Testis chemistry, Chromatography, High Pressure Liquid methods, Lipids analysis, Mass Spectrometry methods
- Abstract
Rationale: A novel data-independent acquisition method is detailed that incorporates a scanning quadrupole in front of an orthogonal acceleration time-of-flight (TOF) mass analyser. This approach is described and the attributes are compared and contrasted to other DIA approaches., Methods: Specific application of the method to both targeted and untargeted lipidomic identification strategies is discussed, with data from both shotgun and LC separated lipidomics experiments presented., Results: The benefits of the fast quadrupole scanning technique are highlighted, and include improvements in speed and specificity for complex mixtures providing high quality qualitative and quantitative data., Conclusions: In particular the high specificity afforded by the scanning quadrupole improves qualitative information for lipid identification., (Copyright © 2017 John Wiley & Sons, Ltd.)
- Published
- 2017
- Full Text
- View/download PDF
45. Ion mobility tandem mass spectrometry enhances performance of bottom-up proteomics.
- Author
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Helm D, Vissers JP, Hughes CJ, Hahne H, Ruprecht B, Pachl F, Grzyb A, Richardson K, Wildgoose J, Maier SK, Marx H, Wilhelm M, Becher I, Lemeer S, Bantscheff M, Langridge JI, and Kuster B
- Subjects
- Flow Injection Analysis, HeLa Cells, Histone Deacetylase Inhibitors chemistry, Humans, Hydroxamic Acids chemistry, Ions, Phosphorylation, Proteomics methods, Sensitivity and Specificity, Tandem Mass Spectrometry methods, Time Factors, Trypsin chemistry, Histone Deacetylases analysis, Peptide Fragments analysis, Phosphoproteins analysis, Proteomics instrumentation, Tandem Mass Spectrometry instrumentation
- Abstract
One of the limiting factors in determining the sensitivity of tandem mass spectrometry using hybrid quadrupole orthogonal acceleration time-of-flight instruments is the duty cycle of the orthogonal ion injection system. As a consequence, only a fraction of the generated fragment ion beam is collected by the time-of-flight analyzer. Here we describe a method utilizing postfragmentation ion mobility spectrometry of peptide fragment ions in conjunction with mobility time synchronized orthogonal ion injection leading to a substantially improved duty cycle and a concomitant improvement in sensitivity of up to 10-fold for bottom-up proteomic experiments. This enabled the identification of 7500 human proteins within 1 day and 8600 phosphorylation sites within 5 h of LC-MS/MS time. The method also proved powerful for multiplexed quantification experiments using tandem mass tags exemplified by the chemoproteomic interaction analysis of histone deacetylases with Trichostatin A., (© 2014 by The American Society for Biochemistry and Molecular Biology, Inc.)
- Published
- 2014
- Full Text
- View/download PDF
46. Tyrosine supplementation for phenylketonuria.
- Author
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Webster D and Wildgoose J
- Subjects
- Humans, Intelligence drug effects, Neuropsychological Tests, Phenylalanine blood, Phenylketonurias blood, Phenylketonurias diet therapy, Randomized Controlled Trials as Topic, Tyrosine blood, Dietary Supplements, Phenylketonurias drug therapy, Tyrosine therapeutic use
- Abstract
Background: Phenylketonuria is an inherited disease for which the main treatment is the dietary restriction of the amino acid phenylalanine. The diet has to be initiated in the neonatal period to prevent or reduce mental handicap. However, the diet is very restrictive and unpalatable and can be difficult to follow. A deficiency of the amino acid tyrosine has been suggested as a cause of some of the neuropsychological problems exhibited in phenylketonuria. Therefore, this review aims to assess the efficacy of tyrosine supplementation for phenylketonuria., Objectives: To assess the effects of tyrosine supplementation alongside or instead of a phenylalanine-restricted diet for people with phenylketonuria, who commenced on diet at diagnosis and either continued on the diet or relaxed the diet later in life. To assess the evidence that tyrosine supplementation alongside, or instead of a phenylalanine-restricted diet improves intelligence, neuropsychological performance, growth and nutritional status, mortality rate and quality of life., Search Methods: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register which is comprised of references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. Additional studies were identified from handsearches of the Journal of Inherited Metabolic Disease (from inception in 1978 to 1998). The manufacturers of prescribable dietary products used in the treatment of phenylketonuria were also contacted for further references.Date of the most recent search of the Group's Inborn Errors of Metabolism Trials Register: 28 June 2012., Selection Criteria: All randomised or quasi-randomised trials investigating the use of tyrosine supplementation versus placebo in people with phenylketonuria in addition to, or instead of, a phenylalanine-restricted diet. People treated for maternal phenylketonuria were excluded., Data Collection and Analysis: Two authors independently assessed the trial eligibility, methodological quality and extracted the data., Main Results: Six trials were found, of which three trials reporting the results of a total of 56 participants, were suitable for inclusion in the review. The blood tyrosine concentrations were significantly higher in the participants receiving tyrosine supplements than those in the placebo group, mean difference 23.46 (95% confidence interval 12.87 to 34.05). No significant differences were found between any of the other outcomes measured., Authors' Conclusions: From the available evidence no recommendations can be made about whether tyrosine supplementation should be introduced into routine clinical practice. Further randomised controlled studies are required to provide more evidence.
- Published
- 2013
- Full Text
- View/download PDF
47. Tyrosine supplementation for phenylketonuria.
- Author
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Webster D and Wildgoose J
- Subjects
- Humans, Intelligence drug effects, Neuropsychological Tests, Phenylalanine blood, Phenylketonurias blood, Phenylketonurias diet therapy, Randomized Controlled Trials as Topic, Tyrosine blood, Dietary Supplements, Phenylketonurias drug therapy, Tyrosine therapeutic use
- Abstract
Background: Phenylketonuria is an inherited disease for which the main treatment is the dietary restriction of the amino acid phenylalanine. The diet has to be initiated in the neonatal period to prevent or reduce mental handicap. However, the diet is very restrictive and unpalatable and can be difficult to follow. A deficiency of the amino acid tyrosine has been suggested as a cause of some of the neuropsychological problems exhibited in phenylketonuria. Therefore, this review aims to assess the efficacy of tyrosine supplementation for phenylketonuria., Objectives: To assess the effects of tyrosine supplementation alongside or instead of a phenylalanine-restricted diet for people with phenylketonuria, who commenced on diet at diagnosis and either continued on the diet or relaxed the diet later in life. To assess the evidence that tyrosine supplementation alongside, or instead of a phenylalanine-restricted diet improves intelligence, neuropsychological performance, growth and nutritional status, mortality rate and quality of life., Search Strategy: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register which is comprised of references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. Additional studies were identified from handsearches of the Journal of Inherited Metabolic Disease (from inception in 1978 to 1998). The manufacturers of prescribable dietary products used in the treatment of phenylketonuria were also contacted for further references.Date of the most recent search of the Group's Inborn Errors of Metabolism Trials Register: 09 June 2010., Selection Criteria: All randomised or quasi-randomised trials investigating the use of tyrosine supplementation versus placebo in people with phenylketonuria in addition to, or instead of, a phenylalanine-restricted diet. People treated for maternal phenylketonuria were excluded., Data Collection and Analysis: Two authors independently assessed the trial eligibility, methodological quality and extracted the data., Main Results: Six trials were found, of which three trials reporting the results of a total of 56 participants, were suitable for inclusion in the review. The blood tyrosine concentrations were significantly higher in the participants receiving tyrosine supplements than those in the placebo group, mean difference 23.46 (95% confidence interval 12.87 to 34.05). No significant differences were found between any of the other outcomes measured., Authors' Conclusions: From the available evidence no recommendations can be made about whether tyrosine supplementation should be introduced into routine clinical practice. Further randomised controlled studies are required to provide more evidence.
- Published
- 2010
- Full Text
- View/download PDF
48. Dietary interventions for phenylketonuria.
- Author
-
Poustie VJ and Wildgoose J
- Subjects
- Humans, Phenylalanine blood, Phenylketonurias blood, Randomized Controlled Trials as Topic, Treatment Outcome, Phenylalanine administration & dosage, Phenylketonurias diet therapy
- Abstract
Background: Phenylketonuria is an inherited disease treated with dietary restriction of the amino acid phenylalanine. The diet is initiated in the neonatal period to prevent mental handicap; however, it is restrictive and can be difficult to follow. Whether the diet can be relaxed or discontinued during adolescence or should be continued for life remains a controversial issue, which we aim to address in this review., Objectives: To assess the effects of a low-phenylalanine diet commenced early in life for people with phenylketonuria. To assess the possible effects of relaxation or termination of the diet on intelligence, neuropsychological outcomes and mortality, growth, nutritional status, eating behaviour and quality of life., Search Strategy: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings.Most recent search of the Inborn Errors of Metabolism Trials Register: 05 March 2009., Selection Criteria: All randomised or quasi-randomised controlled trials comparing a low-phenylalanine diet to relaxation or termination of dietary restrictions in people with phenylketonuria., Data Collection and Analysis: Two authors independently assessed study eligibility and methodological quality, and subsequently extracted the data., Main Results: We included four studies in this review (251 participants), and found few significant differences between treatment and comparison groups for the outcomes of interest. Blood phenylalanine levels were significantly lower in participants with phenylketonuria following a low-phenylalanine diet compared to those on a less restricted diet, mean difference (MD) at three months -698.67 (95% confidence interval (CI) -869.44 to -527.89). Intelligence quotient was significantly higher in participants who continued the diet than in those who stopped the diet, MD after 12 months 5.00 (95% CI 0.40 to 9.60). However, these results came from a single study., Authors' Conclusions: The results of non-randomised studies have concluded that a low-phenylalanine diet is effective in reducing blood phenylalanine levels and improving intelligence quotient and neuropsychological outcomes. We were unable to find any randomised controlled studies that have assessed the effect of a low-phenylalanine diet versus no diet from diagnosis. In view of evidence from non-randomised studies, such a study would be unethical and it is recommended that low-phenylalanine diet should be commenced at the time of diagnosis. There is uncertainty about the precise level of phenylalanine restriction and when, if ever, the diet should be relaxed. This should be addressed by randomised controlled studies.
- Published
- 2010
- Full Text
- View/download PDF
49. Quadrupole-time-of-flight mass spectrometer modified for higher-energy dissociation reduces protein assemblies to peptide fragments.
- Author
-
Benesch JL, Ruotolo BT, Sobott F, Wildgoose J, Gilbert A, Bateman R, and Robinson CV
- Subjects
- Archaeal Proteins chemistry, Heat-Shock Proteins chemistry, Tandem Mass Spectrometry methods, Mass Spectrometry methods, Peptide Fragments chemistry, Proteins chemistry
- Abstract
The gas-phase dissociation of protein assemblies is becoming crucial for the application of mass spectrometry to structural biology. However certain aspects of the dissociation mechanism remain elusive. Moreover, many protein complexes resist dissociation at the energies accessible with current instrumentation. Here we report new insights into the collision-induced dissociation mechanism of protein assemblies. By holding activation energy constant and varying the charge state of the precursor ion, we show that the total charge of the precursor ion dramatically influences the internal energy required to dissociate monomers from the protein assembly. Furthermore, we have developed a modified quadrupole-time-of-flight instrument capable of accessing activation energies higher than previously possible. Under these conditions, protein assemblies eject subunits with excess internal energy that subsequently fragment into peptides. Together, these data indicate that the non-covalent dissociation is limited by the amount of charge available and not merely the activation energy, and they project the exciting possibility of extracting sequence information directly from intact protein complexes in the gas phase.
- Published
- 2009
- Full Text
- View/download PDF
50. Hospital life pictured en route.
- Author
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Wildgoose J
- Subjects
- London, Exhibitions as Topic, Hospital Design and Construction methods, Photography
- Published
- 2002
- Full Text
- View/download PDF
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