Table 6.

Management of Other Complications in Individuals with Arginase Deficiency

Manifestation/ConcernTreatmentConsideration/Other
Seizures Standard ASM depending on seizure type 1Referral to neurologist
Spasticity Consider a trial of Botox®.
Orthotics, walkers, wheelchairs, & other durable medical equipmentReferral to rehabilitation medicine
Persistent hepatic
synthetic function
abnormalities 2
  • In most cases, only clinical monitoring is necessary.
  • W/more severe coagulopathy, FFP is administered prior to surgical procedures.
Referral to hematologist for severe cases
Hepatic fibrosis &
cirrhosis 		Liver transplantationThis is a rare complication.
Joint contractures
  • Physical therapy
  • Tendon release procedures
Referral to orthopedist if severe

ASM = anti-seizure medication; FFP = fresh-frozen plasma

1.

Valproic acid should be avoided (see Agents/Circumstances to Avoid).

2.

Particularly elevated prothrombin time

From: Arginase Deficiency

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.