Research Topics
| A M BoschSummaryAffiliation: Academic Medical Center Country: The Netherlands Publications
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Detail Information
Publications
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...
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...
Brown-Vialetto-Van Laere and Fazio Londe syndrome is associated with a riboflavin transporter defect mimicking mild MADD: a new inborn error of metabolism with potential treatmentAnnet M Bosch
Department of Pediatrics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
J Inherit Metab Dis 34:159-64. 2011....
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...
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 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...
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 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...
