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2. Radiocarbon protocols and first intercomparison results from the chronos carbon-cycle facility, University of New South Wales, Sydney, Australia

Author

Turney, C, Becerra Valdivia, LBV, Sookdeo, A, Thomas, ZA, Palmer, J, Haines, HA, Cadd, H, Wacker, L, Baker, A, Andersen, MS, Jacobsen, G, Meredith, K, Chinu, K, Bollhalder, S, and Marjo, C

Abstract

The Chronos 14Carbon-Cycle Facility is a new radiocarbon laboratory at the University of New South Wales, Australia. Built around an Ionplus 200 kV MIni-CArbon DAting System (MICADAS) Accelerator Mass Spectrometer (AMS) installed in October 2019, the facility was established to address major challenges in the Earth, Environmental and Archaeological sciences. Here we report an overview of the Chronos facility, the pretreatment methods currently employed (bones, carbonates, peat, pollen, charcoal, and wood) and results of radiocarbon and stable isotope measurements undertaken on a wide range of sample types. Measurements on international standards, known-age and blank samples demonstrate the facility is capable of measuring 14C samples from the Anthropocene back to nearly 50,000 years ago. Future work will focus on improving our understanding of the Earth system and managing resources in a future warmer world.

Published
2022

3. Eleven-year solar cycles over the last millennium revealed by radiocarbon in tree rings

Author

Brehm, N. (Nicolas), Bayliss, A. (Alex), Christl, M. (Marcus), Synal, H.-A. (Hans-Arno), Adolphi, F. (Florian), Beer, J. (Jürg), Kromer, B. (Bernd), Muscheler, R. (Raimund), Solanki, S. K. (Sami K.), Usoskin, I. (Ilya), Bleicher, N. (Niels), Bollhalder, S. (Silvia), Tyers, C. (Cathy), Wacker, L. (Lukas), Brehm, N. (Nicolas), Bayliss, A. (Alex), Christl, M. (Marcus), Synal, H.-A. (Hans-Arno), Adolphi, F. (Florian), Beer, J. (Jürg), Kromer, B. (Bernd), Muscheler, R. (Raimund), Solanki, S. K. (Sami K.), Usoskin, I. (Ilya), Bleicher, N. (Niels), Bollhalder, S. (Silvia), Tyers, C. (Cathy), and Wacker, L. (Lukas)

Abstract

The Sun provides the principal energy input into the Earth system and solar variability represents a significant external climate forcing. Although observations of solar activity (sunspots) cover only the last about 400 years, radionuclides produced by cosmic rays and stored in tree rings or ice cores serve as proxies for solar activity extending back thousands of years. However, the presence of weather-induced noise or low temporal resolution of long, precisely dated records hampers cosmogenic nuclide-based studies of short-term solar variability such as the 11-yr Schwabe cycle. Here we present a continuous, annually resolved atmospheric ¹⁴C concentration (fractionation-corrected ratio of ¹⁴CO₂ to CO₂) record reconstructed from absolutely dated tree rings covering nearly all of the last millennium (ad 969–1933). The high-resolution and precision ¹⁴C record reveals the presence of the Schwabe cycle over the entire time range. The record confirms the ad 993 solar energetic particle event and reveals two new candidates (ad 1052 and ad 1279), indicating that strong solar events that might be harmful to modern electronic systems probably occur more frequently than previously thought. In addition to showing decadal-scale solar variability over the last millennium, the high-temporal-resolution record of atmospheric radiocarbon also provides a useful benchmark for making radiocarbon dating more accurate over this interval.

Published
2021

4. Radiocarbon Protocols and First Intercomparison Results From The Chronos¹⁴ Carbon-Cycle Facility, University Of New South Wales, Sydney, Australia

Author

Turney, C, Becerra-Valdivia, L, Sookdeo, A, Thomas, ZA, Palmer, J, Haines, HA, Cadd, H, Wacker, L, Baker, A, Andersen, MS, Jacobsen, G, Meredith, K, Chinu, K, Bollhalder, S, Marjo, C, Turney, C, Becerra-Valdivia, L, Sookdeo, A, Thomas, ZA, Palmer, J, Haines, HA, Cadd, H, Wacker, L, Baker, A, Andersen, MS, Jacobsen, G, Meredith, K, Chinu, K, Bollhalder, S, and Marjo, C

Abstract

The Chronos 14Carbon-Cycle Facility is a new radiocarbon laboratory at the University of New South Wales, Australia. Built around an Ionplus 200 kV MIni-CArbon DAting System (MICADAS) Accelerator Mass Spectrometer (AMS) installed in October 2019, the facility was established to address major challenges in the Earth, Environmental and Archaeological sciences. Here we report an overview of the Chronos facility, the pretreatment methods currently employed (bones, carbonates, peat, pollen, charcoal, and wood) and results of radiocarbon and stable isotope measurements undertaken on a wide range of sample types. Measurements on international standards, known-age and blank samples demonstrate the facility is capable of measuring 14C samples from the Anthropocene back to nearly 50,000 years ago. Future work will focus on improving our understanding of the Earth system and managing resources in a future warmer world.

Published
2021

5. Dietary practices in methylmalonic acidaemia: a European survey

Author

Pinto, A., Evans, S., Daly, A., Almeida, M.F., Assoun, M., Belanger-Quintana, A., Bernabei, S.M., Bollhalder, S., Cassiman, D., Champion, H., Chan, H., Corthouts, K., Dalmau, J., Boer, F. de, Laet, C. de, Meyer, A, Desloovere, A., Dianin, A., Dixon, M., Dokoupil, K., Dubois, S., Eyskens, F., Faria, A., Fasan, I., Favre, E., Feillet, F., Fekete, A., Gallo, G., Gingell, C., Gribben, J., Hansen, K.K., Horst, N.T., Jankowski, C., Janssen-Regelink, R.G., Jones, I., Jouault, C., Kahrs, G.E., Kok, I., Kowalik, A., Laguerre, C., Verge, S.L., Liguori, A., Lilje, R., Maddalon, C., Mayr, D., Meyer, U., Micciche, A., Och, U., Robert, M., Rocha, J.C., Rogozinski, H., Rohde, C., Ross, K., Saruggia, I., Schlune, A., Singleton, K., Sjoqvist, E., Skeath, R., Stolen, L.H., Terry, A., Timmer, C., Tomlinson, L., Tooke, A., Kerckhove, K.V., Dam, E. van, Hurk, D.V.D., Ploeg, L.V., Driessche, M. Van, Rijn, M. van de, Wegberg, A.M. van, Vasconcelos, C., Vestergaard, H., Vitoria, I., Webster, D., White, F., White, L., Zweers, H.E., MacDonald, A., Pinto, A., Evans, S., Daly, A., Almeida, M.F., Assoun, M., Belanger-Quintana, A., Bernabei, S.M., Bollhalder, S., Cassiman, D., Champion, H., Chan, H., Corthouts, K., Dalmau, J., Boer, F. de, Laet, C. de, Meyer, A, Desloovere, A., Dianin, A., Dixon, M., Dokoupil, K., Dubois, S., Eyskens, F., Faria, A., Fasan, I., Favre, E., Feillet, F., Fekete, A., Gallo, G., Gingell, C., Gribben, J., Hansen, K.K., Horst, N.T., Jankowski, C., Janssen-Regelink, R.G., Jones, I., Jouault, C., Kahrs, G.E., Kok, I., Kowalik, A., Laguerre, C., Verge, S.L., Liguori, A., Lilje, R., Maddalon, C., Mayr, D., Meyer, U., Micciche, A., Och, U., Robert, M., Rocha, J.C., Rogozinski, H., Rohde, C., Ross, K., Saruggia, I., Schlune, A., Singleton, K., Sjoqvist, E., Skeath, R., Stolen, L.H., Terry, A., Timmer, C., Tomlinson, L., Tooke, A., Kerckhove, K.V., Dam, E. van, Hurk, D.V.D., Ploeg, L.V., Driessche, M. Van, Rijn, M. van de, Wegberg, A.M. van, Vasconcelos, C., Vestergaard, H., Vitoria, I., Webster, D., White, F., White, L., Zweers, H.E., and MacDonald, A.

Abstract

Contains fulltext : 220058.pdf (Publisher’s version ) (Open Access), Background The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim To describe the dietary management of patients with MMA across Europe. Methods A cross-sectional questionnaire was sent to European colleagues managing inherited metabolic disorders (IMDs) (n=53) with 27 questions about the nutritional management of organic acidaemias. Data were analysed by different age ranges (0-6 months; 7-12 months; 1-10 years; 11-16 years; >16 years). Results Questionnaires were returned from 53 centres. Twenty-five centres cared for 80 patients with MMA vitamin B12 responsive (MMAB12r) and 43 centres managed 215 patients with MMA vitamin B12 non-responsive (MMAB12nr). For MMAB12r patients, 44% of centres (n=11/25) prescribed natural protein below the World Health Organization/Food and Agriculture Organization/United Nations University (WHO/FAO/UNU) 2007 safe levels of protein intake in at least one age range. Precursor-free amino acids (PFAA) were prescribed by 40% of centres (10/25) caring for 36% (29/80) of all the patients. For MMAB12nr patients, 72% of centres (n=31/43) prescribed natural protein below the safe levels of protein intake (WHO/FAO/UNU 2007) in at least one age range. PFAA were prescribed by 77% of centres (n=33/43) managing 81% (n=174/215) of patients. In MMAB12nr patients, 90 (42%) required tube feeding: 25 via a nasogastric tube and 65 via a gastrostomy. Conclusions A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake. However, there was inconsistent practices across Europe. Long-term efficacy studies are needed to study patient outcome when using PFAA with different severities of natural protein restrictions in patients with MMA to

Published
2020

6. Findings from an in-Depth Annual Tree-Ring Radiocarbon Intercomparison

Author

Wacker, L, primary, Scott, E M, additional, Bayliss, A, additional, Brown, D, additional, Bard, E, additional, Bollhalder, S, additional, Friedrich, M, additional, Capano, M, additional, Cherkinsky, A, additional, Chivall, D, additional, Culleton, B J, additional, Dee, M W, additional, Friedrich, R, additional, Hodgins, G W L, additional, Hogg, A, additional, Kennett, D J, additional, Knowles, T D J, additional, Kuitems, M, additional, Lange, T E, additional, Miyake, F, additional, Nadeau, M-J, additional, Nakamura, T, additional, Naysmith, J P, additional, Olsen, J, additional, Omori, T, additional, Petchey, F, additional, Philippsen, B, additional, Bronk Ramsey, C, additional, Prasad, G V Ravi, additional, Seiler, M, additional, Southon, J, additional, Staff, R, additional, and Tuna, T, additional

Published
2020
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7. Dietary practices in propionic acidemia: A European survey

Author

Daly, A., primary, Pinto, A., additional, Evans, S., additional, Almeida, M.F., additional, Assoun, M., additional, Belanger-Quintana, A., additional, Bernabei, S.M., additional, Bollhalder, S., additional, Cassiman, D., additional, Champion, H., additional, Chan, H., additional, Dalmau, J., additional, de Boer, F., additional, de Laet, C., additional, de Meyer, A., additional, Desloovere, A., additional, Dianin, A., additional, Dixon, M., additional, Dokoupil, K., additional, Dubois, S., additional, Eyskens, F., additional, Faria, A., additional, Fasan, I., additional, Favre, E., additional, Feillet, F., additional, Fekete, A., additional, Gallo, G., additional, Gingell, C., additional, Gribben, J., additional, Kaalund Hansen, K., additional, Ter Horst, N.M., additional, Jankowski, C., additional, Janssen-Regelink, R., additional, Jones, I., additional, Jouault, C., additional, Kahrs, G.E., additional, Kok, I.L., additional, Kowalik, A., additional, Laguerre, C., additional, Le Verge, S., additional, Lilje, R., additional, Maddalon, C., additional, Mayr, D., additional, Meyer, U., additional, Micciche, A., additional, Och, U., additional, Robert, M., additional, Rocha, J.C., additional, Rogozinski, H., additional, Rohde, C., additional, Ross, K., additional, Saruggia, I., additional, Schlune, A., additional, Singleton, K., additional, Sjoqvist, E., additional, Skeath, R., additional, Stolen, L.H., additional, Terry, A., additional, Timmer, C., additional, Tomlinson, L., additional, Tooke, A., additional, Vande Kerckhove, K., additional, van Dam, E., additional, van den Hurk, T., additional, van der Ploeg, L., additional, van Driessche, M., additional, van Rijn, M., additional, van Wegberg, A., additional, Vasconcelos, C., additional, Vestergaard, H., additional, Vitoria, I., additional, Webster, D., additional, White, F.J., additional, White, L., additional, Zweers, H., additional, and MacDonald, A., additional

Published
2017
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8. Dietary practices in isovaleric acidemia: A European survey

Author

Pinto, A., Daly, A., Evans, S., Almeida, M.F., Assoun, M., Belanger-Quintana, A., Bernabei, S., Bollhalder, S., Cassiman, D., Champion, H., Chan, H., Dalmau, J., Boer, F. de, Laet, C. de, Meyer, A, Desloovere, A., Dianin, A., Dixon, M., Dokoupil, K., Dubois, S., Eyskens, F., Faria, A., Fasan, I., Favre, E., Feillet, F., Fekete, A., Gallo, G., Gingell, C., Gribben, J., Kaalund-Hansen, K., Horst, N., Jankowski, C., Janssen-Regelink, R.G., Jones, I., Jouault, C., Kahrs, G.E., Kok, I.L., Kowalik, A., Laguerre, C., Verge, S. Le, Lilje, R., Maddalon, C., Mayr, D., Meyer, U., Micciche, A., Robert, M., Rocha, J.C., Rogozinski, H., Rohde, C., Ross, K., Saruggia, I., Schlune, A., Singleton, K., Sjoqvist, E., Stolen, L.H., Terry, A., Timmer, C., Tomlinson, L., Tooke, A., Kerckhove, K. Vande, Dam, E. van, Hurk, T. van den, Ploeg, L. van der, Driessche, M. Van, Rijn, M. van de, Teeffelen-Heithoff, A. van, Wegberg, A.M. van, Vasconcelos, C., Vestergaard, H., Vitoria, I., Webster, D., White, F.J., White, L., Zweers, H.E., Macdonald, A., Pinto, A., Daly, A., Evans, S., Almeida, M.F., Assoun, M., Belanger-Quintana, A., Bernabei, S., Bollhalder, S., Cassiman, D., Champion, H., Chan, H., Dalmau, J., Boer, F. de, Laet, C. de, Meyer, A, Desloovere, A., Dianin, A., Dixon, M., Dokoupil, K., Dubois, S., Eyskens, F., Faria, A., Fasan, I., Favre, E., Feillet, F., Fekete, A., Gallo, G., Gingell, C., Gribben, J., Kaalund-Hansen, K., Horst, N., Jankowski, C., Janssen-Regelink, R.G., Jones, I., Jouault, C., Kahrs, G.E., Kok, I.L., Kowalik, A., Laguerre, C., Verge, S. Le, Lilje, R., Maddalon, C., Mayr, D., Meyer, U., Micciche, A., Robert, M., Rocha, J.C., Rogozinski, H., Rohde, C., Ross, K., Saruggia, I., Schlune, A., Singleton, K., Sjoqvist, E., Stolen, L.H., Terry, A., Timmer, C., Tomlinson, L., Tooke, A., Kerckhove, K. Vande, Dam, E. van, Hurk, T. van den, Ploeg, L. van der, Driessche, M. Van, Rijn, M. van de, Teeffelen-Heithoff, A. van, Wegberg, A.M. van, Vasconcelos, C., Vestergaard, H., Vitoria, I., Webster, D., White, F.J., White, L., Zweers, H.E., and Macdonald, A.

Abstract

Contains fulltext : 169955.pdf (publisher's version ) (Open Access), BACKGROUND: In Europe, dietary management of isovaleric acidemia (IVA) may vary widely. There is limited collective information about dietetic management. AIM: To describe European practice regarding the dietary management of IVA, prior to the availability of the E-IMD IVA guidelines (E-IMD 2014). METHODS: A cross-sectional questionnaire was sent to all European dietitians who were either members of the Society for the Study of Inborn Errors of Metabolism Dietitians Group (SSIEM-DG) or whom had responded to previous questionnaires on dietetic practice (n = 53). The questionnaire comprised 27 questions about the dietary management of IVA. RESULTS: Information on 140 patients with IVA from 39 centres was reported. 133 patients (38 centres) were given a protein restricted diet. Leucine-free amino acid supplements (LFAA) were routinely used to supplement protein intake in 58% of centres. The median total protein intake prescribed achieved the WHO/FAO/UNU [2007] safe levels of protein intake in all age groups. Centres that prescribed LFAA had lower natural protein intakes in most age groups except 1 to 10 y. In contrast, when centres were not using LFAA, the median natural protein intake met WHO/FAO/UNU [2007] safe levels of protein intake in all age groups. Enteral tube feeding was rarely prescribed. CONCLUSIONS: This survey demonstrates wide differences in dietary practice in the management of IVA across European centres. It provides unique dietary data collectively representing European practices in IVA which can be used as a foundation to compare dietary management changes as a consequence of the first E-IMD IVA guidelines availability.

Published
2017

9. Dietary practices in isovaleric acidemia: A European survey

Author

Pinto, A., primary, Daly, A., additional, Evans, S., additional, Almeida, M.F., additional, Assoun, M., additional, Belanger-Quintana, A., additional, Bernabei, S., additional, Bollhalder, S., additional, Cassiman, D., additional, Champion, H., additional, Chan, H., additional, Dalmau, J., additional, de Boer, F., additional, de Laet, C., additional, de Meyer, A., additional, Desloovere, A., additional, Dianin, A., additional, Dixon, M., additional, Dokoupil, K., additional, Dubois, S., additional, Eyskens, F., additional, Faria, A., additional, Fasan, I., additional, Favre, E., additional, Feillet, F., additional, Fekete, A., additional, Gallo, G., additional, Gingell, C., additional, Gribben, J., additional, Kaalund-Hansen, K., additional, Horst, N., additional, Jankowski, C., additional, Janssen-Regelink, R., additional, Jones, I., additional, Jouault, C., additional, Kahrs, G.E., additional, Kok, I.L., additional, Kowalik, A., additional, Laguerre, C., additional, Le Verge, S., additional, Lilje, R., additional, Maddalon, C., additional, Mayr, D., additional, Meyer, U., additional, Micciche, A., additional, Robert, M., additional, Rocha, J.C., additional, Rogozinski, H., additional, Rohde, C., additional, Ross, K., additional, Saruggia, I., additional, Schlune, A., additional, Singleton, K., additional, Sjoqvist, E., additional, Stolen, L.H., additional, Terry, A., additional, Timmer, C., additional, Tomlinson, L., additional, Tooke, A., additional, Vande Kerckhove, K., additional, van Dam, E., additional, van den Hurk, T., additional, van der Ploeg, L., additional, van Driessche, M., additional, van Rijn, M., additional, van Teeffelen-Heithoff, A., additional, van Wegberg, A., additional, Vasconcelos, C., additional, Vestergaard, H., additional, Vitoria, I., additional, Webster, D., additional, White, F.J., additional, White, L., additional, Zweers, H., additional, and MacDonald, A., additional

Published
2017
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11. How strict is galactose restriction in adults with galactosaemia? International practice

Author

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.

Published
2015

12. How strict is galactose restriction in adults with galactosaemia? International practice

Author

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.

Published
2015

13. How strict is galactose restriction in adults with galactosaemia? International practice

Author

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.

Published
2015

14. How strict is galactose restriction in adults with galactosaemia? International practice

Author

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

Published
2015
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18. Dietary practices in methylmalonic acidaemia: a European survey.

Author

Pinto A, Evans S, Daly A, Almeida MF, Assoun M, Belanger-Quintana A, Bernabei SM, Bollhalder S, Cassiman D, Champion H, Chan H, Corthouts K, Dalmau J, Boer F, Laet C, Meyer A, Desloovere A, Dianin A, Dixon M, Dokoupil K, Dubois S, Eyskens F, Faria A, Fasan I, Favre E, Feillet F, Fekete A, Gallo G, Gingell C, Gribben J, Hansen KK, Horst NT, Jankowski C, Janssen-Regelink R, Jones I, Jouault C, Kahrs GE, Kok I, Kowalik A, Laguerre C, Verge SL, Liguori A, Lilje R, Maddalon C, Mayr D, Meyer U, Micciche A, Och U, Robert M, Rocha JC, Rogozinski H, Rohde C, Ross K, Saruggia I, Schlune A, Singleton K, Sjoqvist E, Skeath R, Stolen LH, Terry A, Timmer C, Tomlinson L, Tooke A, Kerckhove KV, van Dam E, Hurk DVD, Ploeg LV, van Driessche M, van Rijn M, Wegberg AV, Vasconcelos C, Vestergaard H, Vitoria I, Webster D, White F, White L, Zweers H, and MacDonald A

Subjects
Adolescent, Amino Acid Metabolism, Inborn Errors epidemiology, Child, Child, Preschool, Cross-Sectional Studies, Europe epidemiology, Female, Humans, Infant, Infant, Newborn, Male, Nutritional Support, Amino Acid Metabolism, Inborn Errors diet therapy, Dietary Proteins administration & dosage, Surveys and Questionnaires standards
Abstract

Background The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim To describe the dietary management of patients with MMA across Europe. Methods A cross-sectional questionnaire was sent to European colleagues managing inherited metabolic disorders (IMDs) (n=53) with 27 questions about the nutritional management of organic acidaemias. Data were analysed by different age ranges (0-6 months; 7-12 months; 1-10 years; 11-16 years; >16 years). Results Questionnaires were returned from 53 centres. Twenty-five centres cared for 80 patients with MMA vitamin B12 responsive (MMAB12r) and 43 centres managed 215 patients with MMA vitamin B12 non-responsive (MMAB12nr). For MMAB12r patients, 44% of centres (n=11/25) prescribed natural protein below the World Health Organization/Food and Agriculture Organization/United Nations University (WHO/FAO/UNU) 2007 safe levels of protein intake in at least one age range. Precursor-free amino acids (PFAA) were prescribed by 40% of centres (10/25) caring for 36% (29/80) of all the patients. For MMAB12nr patients, 72% of centres (n=31/43) prescribed natural protein below the safe levels of protein intake (WHO/FAO/UNU 2007) in at least one age range. PFAA were prescribed by 77% of centres (n=33/43) managing 81% (n=174/215) of patients. In MMAB12nr patients, 90 (42%) required tube feeding: 25 via a nasogastric tube and 65 via a gastrostomy. Conclusions A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake. However, there was inconsistent practices across Europe. Long-term efficacy studies are needed to study patient outcome when using PFAA with different severities of natural protein restrictions in patients with MMA to guide future practice.

Published
2020
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19. Effects of Inadequate Amino Acid Mixture Intake on Nutrient Supply of Adult Patients with Phenylketonuria.

Author

Hochuli M, Bollhalder S, Thierer C, Refardt J, Gerber P, and Baumgartner MR

Subjects
Adolescent, Adult, Cross-Sectional Studies, Diet, Dietary Fats administration & dosage, Dietary Proteins administration & dosage, Female, Folic Acid blood, Humans, Male, Middle Aged, Prospective Studies, Selenium blood, Vitamin B 12 blood, Young Adult, Amino Acids administration & dosage, Nutrients analysis, Nutritional Status, Phenylketonurias diet therapy
Abstract

Background: Adult phenylketonuria (PKU) patients often reduce their intake of amino acid mixture (AAM) to less than the prescribed amounts. Effects of reduced AAM intake on nutrient supply were evaluated., Methods: Nutrient intake was calculated in 20 adult PKU patients based on a structured food record and complemented by laboratory assessment of nutritional status. Patients were classified into 2 groups, (A) regular AAM intake, or (B) AAM intake below calculated requirements., Results: Group B consumed a higher proportion of natural protein (60 ± 23 vs. 33 ± 12%, p = 0.002); however, the total protein intake was below the recommended amounts in 60% of patients in group B versus 7% in group A (p = 0.03). Fat intake was higher in group B (39 ± 9% of energy vs. 31 ± 6%, p = 0.03), mainly from saturated fats. Selenium, folate, and vitamin B12 intake was below the recommended intake in group B. However, serum concentrations of these analytes remained within the normal range in both groups, although vitamin B12 levels were lower in group B. Plasma tyrosine correlated with AAM intake, and hydroxyproline correlated with the amount of natural protein consumed., Conclusion: Relaxed AAM intake resulted in insufficient nutrient supply, despite a compensatory increase in consumption of natural protein. Care needs to be taken to ensure adequate nutrition in adults with PKU., (© 2017 S. Karger AG, Basel.)

Published
2017
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