Ketonex Medical Food for MSUD

Ketonex is a specialized amino acid–modified medical food developed by Abbott for the dietary management of maple syrup urine disease (MSUD). Because individuals with MSUD cannot properly break down the branched-chain amino acids leucine, isoleucine, and valine, Ketonex provides complete nutrition without these three amino acids—allowing families and clinicians to control BCAA intake while still meeting protein, vitamin, and mineral needs. It should always be used under the guidance of a metabolic physician or dietitian, who will individualize intake based on tolerance and blood monitoring.

This collection carries both stages of the formula so nutrition can follow the patient across life. Ketonex-1 is an unflavored, iron-fortified infant formula powder (14.1 oz can, #67048) designed for newborns and infants during the critical early period of diagnosis and growth. Ketonex-2 (14.1 oz can, #67050) is formulated for older children and adults, delivering 410 calories per can with a nutrient profile suited to the greater energy and protein needs of later childhood and adulthood.

Both are supplied as powders that reconstitute with water, giving caregivers flexibility to prepare feeds at prescribed concentrations. Because MSUD management depends on consistency, many families keep a reliable supply on hand and reorder before running low—metabolic formulas are not always available on short notice. Storage is straightforward: keep unopened cans in a cool, dry place and follow the label for use-by timing once mixed.

Ketonex is one of several inherited metabolic disorder formulas we stock. If you're managing a related condition or comparing options, browse our broader Medical Foods selection or explore additional Nutrition products for supporting supplies. Always confirm the correct formula and amount with your care team before switching between stages or products.