Research Topics
Genomes and GenesSpecies | A M BoschSummaryAffiliation: Academic Medical Center Country: The Netherlands Publications
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Detail Information
Publications
Clinical pathways for inborn errors of metabolism: warranted and feasibleSerwet Demirdas
Department of Pediatrics, Emma Children s Hospital, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Orphanet J Rare Dis 8:37. 2013..In this letter to the editor we describe why it is warranted to develop clinical pathways for IEMs and shortly discuss the process of development for these pathways in the Netherlands...
The Brown-Vialetto-Van Laere and Fazio Londe syndrome revisited: natural history, genetics, treatment and future perspectivesAnnet M Bosch
Department of Pediatrics, University of Amsterdam, Amsterdam, The Netherlands
Orphanet J Rare Dis 7:83. 2012..Clinical improvement may be rapid or gradual over a period of more than 12 months...
High phenylalanine levels directly affect mood and sustained attention in adults with phenylketonuria: a randomised, double-blind, placebo-controlled, crossover trialAmber E Ten Hoedt
Department of Pediatrics, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
J Inherit Metab Dis 34:165-71. 2011..017 and p = 0.040, respectively). High plasma Phe levels have a direct negative effect on both sustained attention and on mood in adult patients with PKU. A Phe-restricted "diet for life" might be an advisable option for many...
Parenting a child with phenylketonuria or galactosemia: implications for health-related quality of lifeAmber E Ten Hoedt
Department of Pediatrics H7 270, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
J Inherit Metab Dis 34:391-8. 2011..The insight that emotional support and loss of friendship influence the HRQoL of the parents enables treating physicians to provide better support for these parents...
The course of life and quality of life of early and continuously treated Dutch patients with phenylketonuriaA M Bosch
Department of Pediatrics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
J Inherit Metab Dis 30:29-34. 2007..The results of this study demonstrate that although PKU is a chronic disease with the burden of strict dietary control, early and continuously treated patients with PKU can have a normal health-related quality of life and course of life...
Classical galactosaemia revisitedAnnet M Bosch
Department of Pediatrics, Division of Metabolic Disorders, Academic Medical Centre G8 205, University Hospital of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
J Inherit Metab Dis 29:516-25. 2006..In the meantime, the follow-up protocol for patients with GALT deficiency should focus on early detection, evaluation and, if possible, early intervention in problems of motor, speech and cognitive development...
"MY PKU": increasing self-management in patients with phenylketonuria. A randomized controlled trialAmber E Ten Hoedt
Department of Pediatrics, Academic Medical Center AMC, University of Amsterdam, AZ Amsterdam, The Netherlands
Orphanet J Rare Dis 6:48. 2011..We aimed to assess whether increased self-management of PKU patients and/or their parents is feasible and safe, by providing direct online access to blood Phe values without immediate professional guidance...
Phenylalanine tolerance can already reliably be assessed at the age of 2 years in patients with PKUF J van Spronsen
Department of Pediatrics, Beatrix Children s Hospital, and Center for Liver, Digestive and Metabolic Diseases, University Medical Center of Groningen, University of Groningen, Groningen, The Netherlands
J Inherit Metab Dis 32:27-31. 2009..So far, little is known about the course of Phe tolerance or the ability of both pre-treatment Phe and Phe tolerance at early age to predict Phe tolerance at later age...
High tolerance for oral galactose in classical galactosaemia: dietary implicationsA M Bosch
Academic Medical Centre, University of Amsterdam, Amsterdam, Netherlands
Arch Dis Child 89:1034-6. 2004..CONCLUSIONS: These findings provide further evidence that attempts to exclude trace amounts of galactose from the diet are not justified. Once the diet is made more liberal, a long term follow up study will be necessary...
Remarkable differences: the course of life of young adults with galactosaemia and PKUA M Bosch
Department of Pediatrics, Academic Medical Center, University of Amsterdam, G8205, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
J Inherit Metab Dis 32:706-12. 2009..We conclude that it is essential that parents and clinicians encourage children with galactosaemia to participate in peer-related activities in order to stimulate social performance, which may result in a more normal CoL...
High incidence of hypermethioninaemia in a single neonatal intensive care unit detected by a newly introduced neonatal screening programmeA E ten Hoedt
Department of Pediatrics, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
J Inherit Metab Dis 30:978. 2007..To prevent hypermethioninaemia, the use of high-methionine containing solutions for TPN should be reconsidered...
Clinical features of galactokinase deficiency: a review of the literatureA M Bosch
Emma Children's Hospital, Amsterdam, The Netherlands
J Inherit Metab Dis 25:629-34. 2002..Although damage may well occur in utero, available evidence suggests that damage will continue after birth. Inhibition of galactokinase may then be a promising approach for controlling damage in GALT-deficient patients...
Beta-alanine and beta-aminoisobutyric acid levels in two siblings with dihydropyrimidinase deficiencyA B P van Kuilenburg
Academic Medical Center, Department of Clinical Chemistry, University of Amsterdam, Amsterdam, The Netherlands
Nucleosides Nucleotides Nucleic Acids 27:825-9. 2008..Thus, the relevance of the shortage of beta-aminoisobutyric acid for the onset of a clinical phenotype in patients with DHP deficiency remains to be established...
Clinical, biochemical and genetic findings in two siblings with a dihydropyrimidinase deficiencyAndre B P van Kuilenburg
Academic Medical Center, University of Amsterdam, Emma Children s Hospital, P O Box 22700, 1100 DE Amsterdam, The Netherlands
Mol Genet Metab 91:157-64. 2007....
Living with classical galactosemia: health-related quality of life consequencesAnnet M Bosch
Department of Pediatrics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Pediatrics 113:e423-8. 2004..Early and regular evaluation and support of possible cognitive problems should be a major part of the protocol for the follow-up of patients with classical galactosemia...
Identification of novel mutations in classical galactosemiaAnnet M Bosch
Academic Medical Centre, University of Amsterdam, Emma s Children s Hospital, Amsterdam, The Netherlands
Hum Mutat 25:502. 2005..508-29delT), and a large deletion encompassing at least exons 1-11. Six of these novel mutations were found in patients of Dutch descent: p.R51Q, p.S135W, p.K229N, p.Q252H, p.X380C, and c.410dupT...
Short-term exogenous galactose supplementation does not influence rate of appearance of galactose in patients with classical galactosemiaHidde H Huidekoper
Department of Paediatrics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Mol Genet Metab 84:265-72. 2005..We conclude that the rate of appearance of galactose is not influenced by exogenous galactose, at least under short-term conditions, in patients with classical galactosemia and in control subjects...
Heterozygosity for the classical galactosemia mutation does not affect ovarian reserve and menopausal ageErik A H Knauff
Department of Reproduction and Gynecology, University Medical Center, Utrecht, The Netherlands
Reprod Sci 14:780-5. 2007..The authors conclude that there is no evidence that GALT mutation carriership affects ovarian reserve or menopausal age...
