High protein prescription in methylmalonic and propionic acidemia patients and its negative association with long-term outcome.

[1]  M. Baumgartner,et al.  Organic acidurias: Major gaps, new challenges, and a yet unfulfilled promise , 2020, Journal of inherited metabolic disease.

[2]  F. Eyskens,et al.  Dietary practices in methylmalonic acidaemia: a European survey , 2019, Journal of pediatric endocrinology & metabolism : JPEM.

[3]  M. Langeveld,et al.  Retrospective evaluation of the Dutch pre‐newborn screening cohort for propionic acidemia and isolated methylmalonic acidemia: What to aim, expect, and evaluate from newborn screening? , 2019, Journal of inherited metabolic disease.

[4]  P. Krishan,et al.  Dietary restriction regimens for fighting kidney disease: Insights from rodent studies , 2019, Experimental Gerontology.

[5]  S. Kölker,et al.  Evaluation of dietary treatment and amino acid supplementation in organic acidurias and urea‐cycle disorders: On the basis of information from a European multicenter registry , 2019, Journal of inherited metabolic disease.

[6]  J. Jans,et al.  Pathophysiology of propionic and methylmalonic acidemias. Part 1: Complications , 2019, Journal of inherited metabolic disease.

[7]  De-Pei Liu,et al.  Caloric Restriction Induces MicroRNAs to Improve Mitochondrial Proteostasis , 2019, iScience.

[8]  M. Baumgartner,et al.  Decreased plasma l-arginine levels in organic acidurias (MMA and PA) and decreased plasma branched-chain amino acid levels in urea cycle disorders as a potential cause of growth retardation: Options for treatment. , 2019, Molecular genetics and metabolism.

[9]  William B. Mair,et al.  Dietary Restriction and AMPK Increase Lifespan via Mitochondrial Network and Peroxisome Remodeling. , 2017, Cell metabolism.

[10]  F. Eyskens,et al.  Dietary practices in propionic acidemia: A European survey , 2017, Molecular genetics and metabolism reports.

[11]  H. Truby,et al.  The Relationship between Dietary Intake, Growth, and Body Composition in Inborn Errors of Intermediary Protein Metabolism , 2017, The Journal of pediatrics.

[12]  J. Pennings,et al.  Diet restriction delays accelerated aging and genomic stress in DNA repair deficient mice , 2016, Nature.

[13]  V. Patel,et al.  Our future: a Lancet commission on adolescent health and wellbeing , 2016, The Lancet.

[14]  L. Ferrucci,et al.  Child Stunting is Associated with Low Circulating Essential Amino Acids , 2016, EBioMedicine.

[15]  S. Santra,et al.  The Nutritional Intake of Patients with Organic Acidaemias on Enteral Tube Feeding: Can We Do Better? , 2015, JIMD reports.

[16]  C. Venditti,et al.  A critical reappraisal of dietary practices in methylmalonic acidemia raises concerns about the safety of medical foods. Part 1: Isolated methylmalonic acidemias (MMA) , 2015, Genetics in Medicine.

[17]  K. Strauss,et al.  A critical reappraisal of dietary practices in methylmalonic acidemia raises concerns about the safety of medical foods. Part 2: Cobalamin C deficiency (cblC).¶ , 2015, Genetics in Medicine.

[18]  M. Baumgartner,et al.  The phenotypic spectrum of organic acidurias and urea cycle disorders. Part 2: the evolving clinical phenotype , 2015, Journal of Inherited Metabolic Disease.

[19]  Anita MacDonald,et al.  Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia , 2014, Orphanet Journal of Rare Diseases.

[20]  J. Dopazo,et al.  Two Novel Mutations in the BCKDK (Branched‐Chain Keto‐Acid Dehydrogenase Kinase) Gene Are Responsible for a Neurobehavioral Deficit in Two Pediatric Unrelated Patients , 2014, Human mutation.

[21]  Stephan J Sanders,et al.  Mutations in BCKD-kinase Lead to a Potentially Treatable Form of Autism with Epilepsy , 2012, Science.

[22]  D. Tomé.  Criteria and markers for protein quality assessment – a review , 2012, British Journal of Nutrition.

[23]  M. Baumgartner,et al.  Propionic acidemia: neonatal versus selective metabolic screening , 2012, Journal of Inherited Metabolic Disease.

[24]  T. Cole,et al.  Revised birth centiles for weight, length and head circumference in the UK-WHO growth charts , 2011, Annals of human biology.

[25]  A. Moser,et al.  Classical maple syrup urine disease and brain development: principles of management and formula design. , 2010, Molecular genetics and metabolism.

[26]  M. Baumgartner,et al.  Prediction of outcome in isolated methylmalonic acidurias: combined use of clinical and biochemical parameters , 2009, Journal of Inherited Metabolic Disease.

[27]  R. Ball,et al.  Indicator amino acid oxidation: concept and application. , 2008, The Journal of nutrition.

[28]  M. Baumgartner,et al.  Long-Term Outcome in Methylmalonic Acidurias Is Influenced by the Underlying Defect (mut0, mut−, cblA, cblB) , 2007, Pediatric Research.

[29]  D. Rabier,et al.  Methylmalonic and propionic acidaemias: Management and outcome , 2005, Journal of Inherited Metabolic Disease.

[30]  T J Cole,et al.  British 1990 growth reference centiles for weight, height, body mass index and head circumference fitted by maximum penalized likelihood. , 1998, Statistics in medicine.

[31]  C. Venditti,et al.  Effects of medical food leucine content in the management of methylmalonic and propionic acidemias , 2018, Current opinion in clinical nutrition and metabolic care.

[32]  N. Hauser,et al.  Variable dietary management of methylmalonic acidemia: metabolic and energetic correlations. , 2011, The American journal of clinical nutrition.

[33]  F. Scaglia.  New insights in nutritional management and amino acid supplementation in urea cycle disorders. , 2010, Molecular genetics and metabolism.

[34]  T. Cole.  The LMS method for constructing normalized growth standards. , 1990, European journal of clinical nutrition.

[35]  H D Dakin,et al.  On Amino-acids. , 1918, The Biochemical journal.