Francjan J van Spronsen Selected Research

Tyrosinemias (Tyrosinemia)

1/2022	Neurocognitive outcome and mental health in children with tyrosinemia type 1 and phenylketonuria: A comparison between two genetic disorders affecting the same metabolic pathway.
6/2020	Aspartame and Phe-Containing Degradation Products in Soft Drinks across Europe.
1/2020	Dried blood spot versus venous blood sampling for phenylalanine and tyrosine.
12/2019	Emotional and behavioral problems, quality of life and metabolic control in NTBC-treated Tyrosinemia type 1 patients.
12/2019	Long-Term Outcomes and Practical Considerations in the Pharmacological Management of Tyrosinemia Type 1.
11/2019	The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients.
10/2019	Blood and Brain Biochemistry and Behaviour in NTBC and Dietary Treated Tyrosinemia Type 1 Mice.
8/2019	Biomarkers of Micronutrients in Regular Follow-Up for Tyrosinemia Type 1 and Phenylketonuria Patients.
1/2018	Daily variation of NTBC and its relation to succinylacetone in tyrosinemia type 1 patients comparing a single dose to two doses a day.
1/2017	What Is the Best Blood Sampling Time for Metabolic Control of Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients?

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Francjan J van Spronsen Research Topics

Disease

55	Phenylketonurias (Phenylketonuria) 01/2022 - 10/2004
14	Tyrosinemias (Tyrosinemia) 01/2022 - 02/2014
5	Metabolic Diseases (Metabolic Disease) 12/2019 - 03/2016
5	Intellectual Disability (Idiocy) 01/2019 - 09/2007
3	Hypoglycemia (Reactive Hypoglycemia) 01/2021 - 01/2016
2	Vitamin B 12 Deficiency (Vitamin B12 Deficiency) 11/2021 - 01/2011
2	Medium chain acyl CoA dehydrogenase deficiency 01/2019 - 01/2007
2	Cardiomyopathies (Cardiomyopathy) 10/2018 - 05/2004
2	Cognitive Dysfunction 01/2017 - 12/2010
2	Guanidinoacetate methyltransferase deficiency 01/2014 - 09/2007
1	Congenital, Hereditary, and Neonatal Diseases and Abnormalities (Congenital Disorders) 01/2021
1	Alkaptonuria 01/2021
1	Atrophy 01/2021
1	Glycogen Storage Disease (Glycogenosis) 01/2020
1	Neutropenia 01/2020
1	Type 2 Diabetes Mellitus (MODY) 01/2020
1	Rare Diseases (Rare Disease) 01/2020
1	Glycogen Storage Disease IB 01/2020
1	Epilepsy (Aura) 01/2019
1	Mitochondrial Diseases (Mitochondrial Disease) 10/2018
1	Dystonia (Limb Dystonia) 08/2017
1	Vomiting 01/2017
1	Liver Neoplasms (Liver Cancer) 01/2017
1	Diarrhea 01/2017
1	Biotinidase Deficiency (Multiple Carboxylase Deficiency, Late Onset) 10/2016
1	Obesity 12/2015
1	Disease Progression 10/2014
1	Hepatorenal form of glycogen storage disease 05/2013
1	Citrullinemia 12/2010
1	Acute Liver Failure (Fulminant Hepatic Failure) 12/2010
1	Brain Diseases (Brain Disorder) 11/2008
1	Cholestasis 11/2008
1	Liver Diseases (Liver Disease) 08/2007
1	Glycogen Storage Disease Type II (Pompe's Disease) 08/2007
1	Ventricular Fibrillation 05/2004

Drug/Important Bio-Agent (IBA)

41	Phenylalanine (L-Phenylalanine)FDA Link 01/2022 - 10/2004
12	Tyrosine (L-Tyrosine)FDA Link 01/2022 - 04/2009
12	Amino Acids FDA Link 01/2021 - 09/2010
8	sapropterin (tetrahydrobiopterin)FDA Link 01/2020 - 04/2009
8	Proteins (Proteins, Gene)FDA Link 01/2017 - 10/2008
7	EnzymesIBA 01/2021 - 01/2013
5	Neurotransmitter Agents (Neurotransmitter)IBA 09/2019 - 12/2010
4	Glucose (Dextrose)FDA LinkGeneric 01/2021 - 12/2015
4	nitisinone (Orfadin)FDA Link 01/2021 - 02/2014
4	Phenylalanine Hydroxylase (Phenylalanine 4 Monooxygenase)IBA 01/2021 - 04/2009
4	Neutral Amino AcidsIBA 01/2018 - 09/2013
3	MicronutrientsIBA 08/2019 - 01/2017
2	Vitamin B 12 (Cyanocobalamin)FDA LinkGeneric 11/2021 - 01/2011
2	Biomarkers (Surrogate Marker)IBA 08/2019 - 01/2018
2	Acyl-CoA Dehydrogenase (Medium-Chain Acyl-Coenzyme A Dehydrogenase)IBA 01/2019 - 01/2007
2	Essential Fatty AcidsIBA 01/2018 - 01/2017
2	Guanidinoacetate N-Methyltransferase (Guanidinoacetate Methyltransferase)IBA 01/2014 - 09/2007
1	Amyloid (Amyloid Fibrils)IBA 01/2021
1	Sodium-Glucose Transport ProteinsIBA 01/2020
1	1,5-anhydroglucitol-6-phosphateIBA 01/2020
1	Granulocyte Colony-Stimulating Factor (G-CSF)IBA 01/2020
1	Glucose-6-Phosphatase (Glucose 6 Phosphatase)IBA 01/2020
1	Hypoglycemic Agents (Hypoglycemics)IBA 01/2020
1	empagliflozinIBA 01/2020
1	Sodium-Glucose Transporter 2 InhibitorsIBA 01/2020
1	NitrogenIBA 01/2019
1	Gln Transfer RNAIBA 10/2018
1	succinylacetoneIBA 01/2018
1	Dopamine (Intropin)FDA LinkGeneric 08/2017
1	Serotonin (5 Hydroxytryptamine)IBA 08/2017
1	Tumor Biomarkers (Tumor Markers)IBA 01/2017
1	Fetal Proteins (Fetoprotein)IBA 01/2017
1	fumarylacetoacetase (fumarylacetoacetate hydrolase)IBA 01/2016
1	LipidsIBA 12/2015
1	Ketone BodiesIBA 10/2014
1	Brain Natriuretic Peptide (Natrecor)FDA Link 10/2014
1	Phenylalanine Ammonia-LyaseIBA 07/2014
1	Pharmaceutical PreparationsIBA 06/2013
1	Starch (Cornstarch)IBA 05/2013
1	Essential Amino AcidsIBA 12/2010
1	Prealbumin (Transthyretin)IBA 01/2010
1	AmmoniaIBA 11/2008
1	Alkaline PhosphataseIBA 11/2008
1	Aspartate Aminotransferases (Aspartate Transaminase)IBA 11/2008
1	gamma-Glutamyltransferase (gamma-Glutamyl Transpeptidase)IBA 11/2008
1	Lactic Acid (Lactate)FDA LinkGeneric 11/2008
1	BilirubinIBA 11/2008
1	Alanine Transaminase (SGPT)IBA 11/2008
1	CreatineIBA 09/2007
1	Protons (Proton)IBA 09/2007
1	Transaminases (Aminotransferases)IBA 08/2007
1	CationsIBA 05/2004
1	Organic Cation Transporter 2IBA 05/2004
1	Systemic carnitine deficiencyIBA 05/2004

Therapy/Procedure

2	Therapeutics 01/2020 - 01/2019
2	Diet Therapy (Therapy, Diet) 09/2019 - 08/2019
2	Medical Day Care (Partial Hospitalization) 03/2015 - 06/2010
2	Liver Transplantation 12/2010 - 11/2008
1	Off-Label Use 01/2020
1	High-Protein Diet 11/2016
1	Protein-Restricted Diet (Diet, Protein Restricted) 02/2014
1	Enzyme Replacement Therapy 08/2007