Table 7.

Propionic Acidemia: Home Management of Mild Metabolic Status

ManifestationTreatmentConsideration/Other
Mildly ↑ catabolism 1
  • Carbohydrate supplementation orally or via tube feed 2
  • Reduce natural protein intake3
  • Increasing carnitine supplementation4
  • Trial of outpatient treatment at home for up to 12 hrs
  • Reassessment (every ~2 hrs) for clinical changes 5
  • At-home detection & monitoring of urine ketones may be considered.
Fever Administration of antipyretics (acetaminophen, ibuprofen) if temperature rises >38.5 °C 6
Occasional vomiting Antiemetics 7
1.

Fever; enteral or gastrostomy tube feeding is tolerated without recurrent vomiting or diarrhea; absence of neurologic symptoms (altered consciousness, irritability, hypotonia, dystonia)

2.

Stringent guidelines to quantify carbohydrate/caloric requirements are available to guide nutritional arrangements in the outpatient setting, with some centers recommending frequent provision of carbohydrate-rich, protein-free beverages every two hours, with frequent reassessment.

3.

Some centers advocate additional steps such as reducing natural protein intake to zero or to 50% of the normal prescribed regimen for short periods (<24 hours) in the outpatient setting during intercurrent illness.

4.

Temporarily increasing L-carnitine doses (e.g., to 200 mg/kg/day in infants) may be considered.

5.

Alterations in mentation/alertness, fever, and enteral feeding tolerance, with any new or evolving clinical features discussed with the designated center of expertise for inherited metabolic diseases

6.

More aggressive fever management might be indicated in individuals with history of arrythmias and/or prolonged QT interval.

7.

Some classes of antiemetics can be used safely on an occasional basis to temporarily improve enteral tolerance of food and beverages at home or during transfer to hospital (see Agents/Circumstances to Avoid).

From: Propionic Acidemia

Cover of GeneReviews®
GeneReviews® [Internet].
Adam MP, Feldman J, Mirzaa GM, et al., editors.
Seattle (WA): University of Washington, Seattle; 1993-2025.
Copyright © 1993-2025, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved.

GeneReviews® chapters are owned by the University of Washington. Permission is hereby granted to reproduce, distribute, and translate copies of content materials for noncommercial research purposes only, provided that (i) credit for source (http://www.genereviews.org/) and copyright (© 1993-2025 University of Washington) are included with each copy; (ii) a link to the original material is provided whenever the material is published elsewhere on the Web; and (iii) reproducers, distributors, and/or translators comply with the GeneReviews® Copyright Notice and Usage Disclaimer. No further modifications are allowed. For clarity, excerpts of GeneReviews chapters for use in lab reports and clinic notes are a permitted use.

For more information, see the GeneReviews® Copyright Notice and Usage Disclaimer.

For questions regarding permissions or whether a specified use is allowed, contact: ude.wu@tssamda.

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.