
Metabolics
The Nutricia Learning Center features tools and educational resources for Metabolic Healthcare Professionals.
Healthcare Professional Tools
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Summary of the Science Behind Complex MSD Formulas for MSUD
A simplified explanation of the published data on Complex MSD medical foods for maple syrup urine disease (MSUD).
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Monogen (Fall 2020 Update)
Updated Formulation, Same MCT % (Fall 2020 Update)
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Guides to Nutricia’s Metabolic Product Portfolio
Acquaint yourself with Nutricia’s products for inherited metabolic disorders. The poster provides a visual overview, the quick reference booklet gives a bit more detail about each product, and the full Nutricia product reference guide provides detailed nutrition and ingredient information.
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Nutricia Navigator
A free service to help your patients navigate the complexities of formula coverage and product access. Find 3 easy steps to get started.
- About Nutricia Navigator
- Prescriber Information Form
- Patient Information Form
- For More Information Email NutriciaNavigator@Nutricia.com
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Maternal PKU - A Quick Guide and Practical Tips for Healthcare Professionals
A quick guide and practical tips on how to manage maternal PKU.
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Monogen Dilution Chart
This resource has been developed for use by Healthcare Professionals. It includes recipes for mixing Monogen at multiple dilutions (from 20 to 30 calories per fluid ounce), as well as household measures.
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Household Measurements
Approximate US household measurements available for the following products:
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Periflex®/Anamix® Early Years Transition Tools
These interactive spreadsheets allow you to calculate DRIs from formula and create simple step-wise transitions to switch your infants to Early Years or transition your toddlers from Early Years to their next stage formula.
- PKU Periflex Early Years Transition Guide & DRI Charts
- GA-1 Anamix Early Years Transition Guide & DRI Charts
- HCU Anamix Early Years Transition Guide & DRI Charts
- IVA Anamix Early Years Transition Guide & DRI Charts
- MMA/PA Anamix Early Years Transition Guide & DRI Charts
- MSUD Anamix Early Years Transition Guide & DRI Charts
- TYR Anamix Early Years Transition Guide & DRI Charts
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Intro to Metabolic Disorders - Slide Decks for Healthcare Providers
These slide decks provide a simple, scientific overview of rare metabolic disorders and their management. These decks can be used to educate new metabolic healthcare providers, students/interns, or families with high medical literacy.
- Phenylketonuria (PKU)
- Glutaric Acidemia Type 1 (GA-1)
- Homocystinuria (HCU)
- Isovaleric Acidemia (IVA)
- Maple Syrup Urine Disease (MSUD)
- Methylmalonic Acidemia (MMA)
- Propionic Acidemia (PA)
- Tyrosinemia Type 1 (TYR)
- Medium-chain acyl-CoA dehydrogenase (MCAD)
- Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD)
- Very-long-chain acyl-CoA dehydrogenase (VLCAD)
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Vademecum Metabolicum—Diagnosis and Treatment of Inborn Errors of Metabolism (J. Zschocke, GF Hoffmann; Schattauer)
Free web-based e-book and Apple/Android app of the 3rd edition of this publication, which provides a systematic and practical approach to the diagnosis of and therapies for metabolic diseases.
- To view the e-book, please visit vademetab.org
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Sample Request Links
Request samples for your patients or clinic.
- Send a sample to a patient https://shop.medicalfood.com/patientsamples.aspx
- Request samples for clinic https://shop.medicalfood.com/clinicsamples.aspx
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Metabolic Product DRI Calculators
These are interactive tools for Healthcare Professionals to help calculate the amount of Nutricia metabolic product needed to achieve a desired amount of protein equivalent for individual patients. The tool can then be used to compare macro and micro nutrients to the DRIs (as of 2010). (Microsoft® Excel files - note, please right click on the link and “Save Target As...” to save this to your desktop and then open through Excel).
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Eat Right-Stay Bright
An anticipatory guidance tool, developed by The Children's Hospital of Colorado, Denver, to aid healthcare professionals in the treatment of patients with PKU. Materials and topics include: Clinic Encounter Check Lists, Experience and Thoughts, Teaching Aids and Handouts and other resources. All chapters have been developed as an anticipatory guidance tool with patient education and improved patient compliance as its main goal. We welcome you to print, individualize, and add to any and all of the sections. Whatever your approach, we hope this educational tool assists you in your clinic setting.
Flowcharts/Guidelines
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Guidelines for the Usage of PhenylAde PheBLOC
Practical guidelines on how to incorporate PhenylAde PheBLOC, a medical food containing large neutral amino acids, into the PKU diet.
Reference Library
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PKU Periflex® Early Years (call Periflex® Infant in Europe)
Select Clinical References for PKU Periflex Early Years
- MacDonald A, et al. Specific prebiotics in a formula for infants with phenylketonuria. Mol Genet Metab. 2011;104 Suppl:S55-9. PubMed
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Periflex® Junior Plus
Select Clinical References for Periflex Junior Plus
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XPhe Maxamum®
Select Clinical References for XPhe Maxamum
- Brown MC, et al Economic impact of feeding a phenylalanine-restricted diet to adults with previously untreated phenylketonuria. J Intell Disabil Res. 1999; 43:30-7. PubMed
- Cleary MA, et al. Magnetic resonance imaging in phenylketonuria: reversal of cerebral white matter change. J Pediatr. 1995; 127:251-255. PubMed
- Wardley BL, Taitz LS. Clinical trial of a concentrated amino acid formula for older patients with phenylketouria (Maxamum XP). Eur J Clin Nutr. 1988; 42:81-86. PubMed
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Phlexy-10®
Select Clinical References for Phlexy-10
- MacDonald A, et al. Protein substitutes for PKU: What’s new? J Inherit Metab Dis. 2004; 27:363-371. PubMed
- Moats RA, et al. Brain phenylalanine concentrations in phenylketonuria: Research and treatment of adults. Pediatrics. 2003;112(6): 1575-9. PubMed
- Rohr FJ, et al. Acceptability of a new modular protein substitute for the dietary treatment of phenylketonuria. J Inherit Metab Dis. 2001; 24:623-630. PubMed
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PhenylAde® PheBLOC™ LNAA
Select Clinical References for PhenylAde PheBLOC LNAA
- Yano S, et al. Melatonin and dopamine as biomarkers to optimize treatment in phenylketonuria: effects of tryptophan and tyrosine supplementation. J Pediatr. 2014;165:184-9. PubMed
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GlutarAde™ Junior Drink Mix
Select Clinical References for GlutarAde Junior Drink Mix
- Stauss KA, et al. Safety, efficacy and physiological actions of a lysine-free, arginine-rich formula to treat glutaryl-CoA dehydrogenase deficiency: focus on cerebral amino acid influx. Mol Genet Metab. 2011;104(1-2):93-106. PubMed
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Complex MSD Products
Select Clinical References for Complex MSD Products
- Strauss KA, et al. Classical maple syrup urine disease and brain development: principles of management and formula design. Mol Genet Metab. 2010;99(4):333-45. PubMed
- Strauss KA, et al. Branched-chain α-ketoacid dehydrogenase deficiency (maple syrup urine disease): Treatment, biomarkers, and outcomes. Mol Genet Metab. 2020;129:193-206. PubMed
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Monogen®
Select Clinical References for Monogen
- Cormack, BE et al. Use of Monogen for pediatric postoperative chylothorax. Ann Thorac Surg. 2004; 77(1):301-5. PubMed
- Ficicioglu C, et al. Very long-chain acyl-CoA dehydrogenase deficiency: The effects of accidental fat loading in a a patient detected through newborn screening. J Inherit Metab Dis. 2009;32 Suppl 1:S187-90. PubMed
- Lessen R. Use of Skim Breast Milk for an Infant with Chylothorax. ICAN (2009); 1 (6):303-10. Sage Journals
- Ng PC, et al. Deceptive hyperbilirubinaemia in a newborn with familial lipoprotein lipase deficiency. J Paediatr Child Health. 2001;37:314-6. PubMed
Helpful Links
- Society for Inherited Metabolic Disorders (SIMD)
- USDA Food Composition Database
- CDC Growth Charts
- CDC Newborn Screening Portal
- National Newborn Screening and Global Resource Center (NNSGRC)
- Genetic Metabolic Dietitians International (GMDI)