Urea cycle disorders: triggers for crises

Evidence-based neurology checklist on urea cycle disorders: triggers for crises: Physiological High protein intake Weight loss Post-partum period Physical exertion Fasting Medical Drugs

Physiological

  • High protein intake
  • Weight loss
  • Post-partum period
  • Physical exertion
  • Fasting

Medical

Drugs

References

  1. Häberle J, Boddaert N, Burlina A, et al. Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet J Rare Dis 2012; 7:32. 
  2. Blair NF, Cremer PD, Tchan MC. Urea cycle disorders: a life-threatening yet treatable cause of metabolic encephalopathy in adults. Pract Neurol 2015; 15:45-48.
  3. Seminara J, Tuchman M, Krivitzky L, et al. Establishing a consortium for the study of rare diseases: The Urea Cycle Disorders Consortium. Mol Genet Metab 2010; 100(Suppl 1):S97-S105.
  4. Oechsner M, Steen C, Stürenburg HJ, Kohlschütter A. Hyperammonaemic encephalopathy after initiation of valproate therapy in unrecognised ornithine transcarbamylase deficiency. JNNP 1998; 64:680-682.

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