Derks TGJ - Search Results

1

What Is the Best Blood Sampling Time for Metabolic Control of Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients?

van Dam E, Daly A, Venema-Liefaard G, van Rijn M, Derks TGJ, McKiernan PJ, Rebecca Heiner-Fokkema M, MacDonald A, van Spronsen FJ. van Dam E, et al. Among authors: derks tgj. JIMD Rep. 2017;36:49-57. doi: 10.1007/8904_2016_37. Epub 2017 Jan 25. JIMD Rep. 2017. PMID: 28120161 Free PMC article.

2

Safe and unsafe duration of fasting for children with MCAD deficiency.

Derks TG, van Spronsen FJ, Rake JP, van der Hilst CS, Span MM, Smit GP. Derks TG, et al. Eur J Pediatr. 2007 Jan;166(1):5-11. doi: 10.1007/s00431-006-0186-0. Epub 2006 Jun 21. Eur J Pediatr. 2007. PMID: 16788829

3

Dietary treatment of glycogen storage disease type Ia: uncooked cornstarch and/or continuous nocturnal gastric drip-feeding?

Derks TG, Martens DH, Sentner CP, van Rijn M, de Boer F, Smit GP, van Spronsen FJ. Derks TG, et al. Mol Genet Metab. 2013 May;109(1):1-2. doi: 10.1016/j.ymgme.2013.02.005. Epub 2013 Feb 15. Mol Genet Metab. 2013. PMID: 23480859 No abstract available.

4

Single amino acid supplementation in aminoacidopathies: a systematic review.

van Vliet D, Derks TG, van Rijn M, de Groot MJ, MacDonald A, Heiner-Fokkema MR, van Spronsen FJ. van Vliet D, et al. Orphanet J Rare Dis. 2014 Jan 13;9:7. doi: 10.1186/1750-1172-9-7. Orphanet J Rare Dis. 2014. PMID: 24422943 Free PMC article. Review.

5

Recombinant phenylalanine ammonia lyase in phenylketonuria.

van Spronsen FJ, Derks TG. van Spronsen FJ, et al. Lancet. 2014 Jul 5;384(9937):6-8. doi: 10.1016/S0140-6736(13)62075-9. Epub 2014 Apr 14. Lancet. 2014. PMID: 24742999 No abstract available.

6

Favorable outcome after physiologic dose of sodium-D,L-3-hydroxybutyrate in severe MADD.

Van Rijt WJ, Heiner-Fokkema MR, du Marchie Sarvaas GJ, Waterham HR, Blokpoel RG, van Spronsen FJ, Derks TG. Van Rijt WJ, et al. Pediatrics. 2014 Oct;134(4):e1224-8. doi: 10.1542/peds.2013-4254. Epub 2014 Sep 22. Pediatrics. 2014. PMID: 25246622

7

Infants with Tyrosinemia Type 1: Should phenylalanine be supplemented?

van Vliet D, van Dam E, van Rijn M, Derks TG, Venema-Liefaard G, Hitzert MM, Lunsing RJ, Heiner-Fokkema MR, van Spronsen FJ. van Vliet D, et al. JIMD Rep. 2015;18:117-24. doi: 10.1007/8904_2014_358. Epub 2014 Sep 26. JIMD Rep. 2015. PMID: 25256450 Free PMC article.

8

BH4 treatment in BH4-responsive PKU patients: preliminary data on blood prolactin concentrations suggest increased cerebral dopamine concentrations.

van Vliet D, Anjema K, Jahja R, de Groot MJ, Liemburg GB, Heiner-Fokkema MR, van der Zee EA, Derks TG, Kema IP, van Spronsen FJ. van Vliet D, et al. Mol Genet Metab. 2015 Jan;114(1):29-33. doi: 10.1016/j.ymgme.2014.11.009. Epub 2014 Nov 15. Mol Genet Metab. 2015. PMID: 25466353

9

Lipids in hepatic glycogen storage diseases: pathophysiology, monitoring of dietary management and future directions.

Derks TG, van Rijn M. Derks TG, et al. J Inherit Metab Dis. 2015 May;38(3):537-43. doi: 10.1007/s10545-015-9811-2. Epub 2015 Jan 30. J Inherit Metab Dis. 2015. PMID: 25633903 Free PMC article. Review.

10

Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: a prospective cohort study.

Schwahn BC, Van Spronsen FJ, Belaidi AA, Bowhay S, Christodoulou J, Derks TG, Hennermann JB, Jameson E, König K, McGregor TL, Font-Montgomery E, Santamaria-Araujo JA, Santra S, Vaidya M, Vierzig A, Wassmer E, Weis I, Wong FY, Veldman A, Schwarz G. Schwahn BC, et al. Lancet. 2015 Nov 14;386(10007):1955-1963. doi: 10.1016/S0140-6736(15)00124-5. Epub 2015 Sep 3. Lancet. 2015. PMID: 26343839

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