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Frits A. Wijburg

Emma Kinderziekenhuis · NL
Area of research
Physiology · Clinical Biochemistry
Research interest
Research interests include Lysosomal Storage Disorders Research, Metabolism and Genetic Disorders, Mitochondrial Function and Pathology, and Glycogen Storage Diseases and Myoclonus.
h-index
73
citations
19,010
works
413
NIH funding
primary concept
email

Recent publications

Navigating the outcome maze: a scoping review of outcomes and instruments in clinical trials in genetic neurodevelopmental disorders and intellectual disability
Therapeutic Advances in Rare Disease 2024cited by 14position: middledoi
Association Between Lysine Reduction Therapies and Cognitive Outcomes in Patients With Pyridoxine-Dependent Epilepsy
Neurology 2022cited by 41position: middledoi
Timing of therapy and neurodevelopmental outcomes in 18 families with pyridoxine-dependent epilepsy
Molecular Genetics and Metabolism 2022cited by 27position: middledoi
Consensus guidelines for the diagnosis and management of pyridoxine‐dependent epilepsy due to α‐aminoadipic semialdehyde dehydrogenase deficiency
Journal of Inherited Metabolic Disease 2020cited by 116position: middledoi
Genotype‐phenotype relationships in mucopolysaccharidosis type I (MPS I): Insights from the International MPS I Registry
Clinical Genetics 2019cited by 84position: middledoi
Impact of newborn screening for very‐long‐chain acyl‐CoA dehydrogenase deficiency on genetic, enzymatic, and clinical outcomes
Journal of Inherited Metabolic Disease 2019cited by 52position: middledoi
Low-dose agalsidase beta treatment in male pediatric patients with Fabry disease: A 5-year randomized controlled trial
Molecular Genetics and Metabolism 2019cited by 32position: lastdoi
<i>EPG5</i> -related Vici syndrome: a paradigm of neurodevelopmental disorders with defective autophagy
Brain 2016cited by 141position: middledoi
Fatty acid oxidation flux predicts the clinical severity of VLCAD deficiency
Genetics in Medicine 2015cited by 62position: middledoi
Characterization of Early Disease Status in Treatment-Naive Male Paediatric Patients with Fabry Disease Enrolled in a Randomized Clinical Trial
PLoS ONE 2015cited by 48position: firstdoi
Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia
Orphanet Journal of Rare Diseases 2014cited by 711position: middledoi
Epidemiology and diagnosis of lysosomal storage disorders; challenges of screening
Best Practice & Research Clinical Endocrinology & Metabolism 2014cited by 146position: lastdoi
Longitudinal analysis of endurance and respiratory function from a natural history study of Morquio A syndrome
Molecular Genetics and Metabolism 2014cited by 41position: middledoi
Disease and patient characteristics in NP-C patients: findings from an international disease registry
Orphanet Journal of Rare Diseases 2013cited by 196position: middledoi
Cross‐sectional observational study of 208 patients with non‐classical urea cycle disorders
Journal of Inherited Metabolic Disease 2013cited by 79position: middledoi
Treatment of hip dysplasia in patients with mucopolysaccharidosis type I after hematopoietic stem cell transplantation: results of an international consensus procedure
Orphanet Journal of Rare Diseases 2013cited by 46position: lastdoi
Correction: Disease and patient characteristics in NP-C patients: findings from an international disease registry
Orphanet Journal of Rare Diseases 2013cited by 9position: middledoi
Recessive mutations in EPG5 cause Vici syndrome, a multisystem disorder with defective autophagy
Nature Genetics 2012cited by 279position: middledoi
Acid sphingomyelinase (Asm) deficiency patients in The Netherlands and Belgium: Disease spectrum and natural course in attenuated patients
Molecular Genetics and Metabolism 2012cited by 102position: middledoi
Capturing phenotypic heterogeneity in MPS I: results of an international consensus procedure
Orphanet Journal of Rare Diseases 2012cited by 33position: lastdoi

Grants

No grants ingested yet.

Frequent collaborators

· 4 papers (2012–2019) · 3 papers (2013–2014) · 3 papers (2012–2019)Simon Jones · New York University3 papers (2013–2019) · 2 papers (2014–2019)Camilla Tøndel · British Heart Foundation2 papers (2015–2019) · 2 papers (2015–2019) · 2 papers (2015–2019)Emma Footitt · Great Ormond Street Hospital2 papers (2022–2022)Mirian C. H. Janssen · Radboud University Nijmegen2 papers (2022–2022)Levinus A. Bok · Radboud University Nijmegen2 papers (2022–2022) · 2 papers (2013–2013)Eugen Mengel · Johannes Gutenberg University Mainz2 papers (2013–2013)Gepke Visser · Utrecht University2 papers (2015–2019) · 2 papers (2013–2013) · 2 papers (2015–2019)Peter M. van Hasselt · Utrecht University2 papers (2013–2019) · 2 papers (2015–2019)Sídney M. Gospe · Duke University2 papers (2022–2022)José E. Abdenur · Children’s Institute2 papers (2022–2022)