Skip to main content

05.06.2018 | Original Article

Influence of implementing a protocol for an intravenously administered ammonia scavenger on the management of acute hyperammonemia in a pediatric intensive care unit

verfasst von: David Brossier, Isabelle Goyer, Lydia Ziani, Christopher Marquis, Grant Mitchell, Bruno Ozanne, Philippe Jouvet

Erschienen in: Journal of Inherited Metabolic Disease

Einloggen, um Zugang zu erhalten

Abstract

The purpose of the study was to evaluate the influence of establishing a protocol for the use of combined sodium benzoate and sodium phenylacetate (SBSP) (Ammonul®) to treat acute hyperammonemia. This was a retrospective, single-center study in a 24-bed medical and surgical pediatric intensive care unit (PICU) in a tertiary care teaching maternal–child hospital in Canada. Inclusion criteria were age <18 years, PICU admission between 1 January 2000 and 30 June 2016, and SBSP treatment. An SBSP delivery protocol was implemented in our hospital on 30 August 2008 in order to improve management of acute hyperammonemia. Patients were assigned to one of the two groups, without or with protocol, depending on date of admission. SBSP was ordered 34 times during the study period, and 23 orders were considered for analysis (14 with and 9 without protocol). Patient characteristics were similar between groups. The median time from diagnosis to prescription was significantly shorter in the protocol group [40 min (21–82) vs 100 min (70–150), p = 0.03)] but the median time from diagnosis to administration of the treatment was equivalent [144 min (90–220) vs 195 (143–274), (p = 0.2)]. Other clinical outcomes did not differ. This study is the first to compare two SBSP delivery strategies in the treatment of acute hyperammonemia in this PICU setting. Implementation of a delivery protocol shortened the time from diagnosis of hyperammonemia to prescription of SBSP and helped us identify other parameters that can be improved to optimize treatment delivery.
Anhänge
Nur mit Berechtigung zugänglich
Literatur
Zurück zum Zitat Alfadhel M, Mutairi FA, Makhseed N et al (2016) Guidelines for acute management of hyperammonemia in the Middle East region. Ther Clin Risk Manag 12:479–487CrossRefPubMedPubMedCentral Alfadhel M, Mutairi FA, Makhseed N et al (2016) Guidelines for acute management of hyperammonemia in the Middle East region. Ther Clin Risk Manag 12:479–487CrossRefPubMedPubMedCentral
Zurück zum Zitat Bachmann C (2003) Outcome and survival of 88 patients with urea cycle disorders: a retrospective evaluation. Eur J Pediatr 162(6):410–416PubMed Bachmann C (2003) Outcome and survival of 88 patients with urea cycle disorders: a retrospective evaluation. Eur J Pediatr 162(6):410–416PubMed
Zurück zum Zitat Barsotti RJ (2001) Measurement of ammonia in blood. J Pediatr 138(1 Suppl):S11–S19 discussion S9-20 CrossRefPubMed Barsotti RJ (2001) Measurement of ammonia in blood. J Pediatr 138(1 Suppl):S11–S19 discussion S9-20 CrossRefPubMed
Zurück zum Zitat Bernal W, Hall C, Karvellas CJ, Auzinger G, Sizer E, Wendon J (2007) Arterial ammonia and clinical risk factors for encephalopathy and intracranial hypertension in acute liver failure. Hepatology 46(6):1844–1852CrossRefPubMed Bernal W, Hall C, Karvellas CJ, Auzinger G, Sizer E, Wendon J (2007) Arterial ammonia and clinical risk factors for encephalopathy and intracranial hypertension in acute liver failure. Hepatology 46(6):1844–1852CrossRefPubMed
Zurück zum Zitat Braissant O (2010) Current concepts in the pathogenesis of urea cycle disorders. Mol Genet Metab 100(Suppl 1):S3–s12CrossRefPubMed Braissant O (2010) Current concepts in the pathogenesis of urea cycle disorders. Mol Genet Metab 100(Suppl 1):S3–s12CrossRefPubMed
Zurück zum Zitat Braissant O, McLin VA, Cudalbu C (2013) Ammonia toxicity to the brain. J Inherit Metab Dis 36(4):595–612CrossRefPubMed Braissant O, McLin VA, Cudalbu C (2013) Ammonia toxicity to the brain. J Inherit Metab Dis 36(4):595–612CrossRefPubMed
Zurück zum Zitat Butterworth RF (2002) Pathophysiology of hepatic encephalopathy: a new look at ammonia. Metab Brain Dis 17(4):221–227CrossRefPubMed Butterworth RF (2002) Pathophysiology of hepatic encephalopathy: a new look at ammonia. Metab Brain Dis 17(4):221–227CrossRefPubMed
Zurück zum Zitat Butterworth RF (2014) Pathophysiology of brain dysfunction in hyperammonemic syndromes: the many faces of glutamine. Mol Genet Metab 113(1–2):113–117CrossRefPubMed Butterworth RF (2014) Pathophysiology of brain dysfunction in hyperammonemic syndromes: the many faces of glutamine. Mol Genet Metab 113(1–2):113–117CrossRefPubMed
Zurück zum Zitat Cagnon L, Braissant O (2007) Hyperammonemia-induced toxicity for the developing central nervous system. Brain Res Rev 56(1):183–197CrossRefPubMed Cagnon L, Braissant O (2007) Hyperammonemia-induced toxicity for the developing central nervous system. Brain Res Rev 56(1):183–197CrossRefPubMed
Zurück zum Zitat Clemmesen JO, Larsen FS, Kondrup J, Hansen BA, Ott P (1999) Cerebral herniation in patients with acute liver failure is correlated with arterial ammonia concentration. Hepatology 29(3):648–653CrossRefPubMed Clemmesen JO, Larsen FS, Kondrup J, Hansen BA, Ott P (1999) Cerebral herniation in patients with acute liver failure is correlated with arterial ammonia concentration. Hepatology 29(3):648–653CrossRefPubMed
Zurück zum Zitat Cooper AJ, Plum F (1987) Biochemistry and physiology of brain ammonia. Physiol Rev 67(2):440–519CrossRefPubMed Cooper AJ, Plum F (1987) Biochemistry and physiology of brain ammonia. Physiol Rev 67(2):440–519CrossRefPubMed
Zurück zum Zitat Dasarathy S, Mookerjee RP, Rackayova V et al (2017) Ammonia toxicity: from head to toe? Metab Brain Dis 32(2):529–538CrossRefPubMed Dasarathy S, Mookerjee RP, Rackayova V et al (2017) Ammonia toxicity: from head to toe? Metab Brain Dis 32(2):529–538CrossRefPubMed
Zurück zum Zitat Davern TJ (2007) Predicting prognosis in acute liver failure: Ammonia and the risk of cerebral edema. Hepatology 46(6):1679–1681CrossRefPubMed Davern TJ (2007) Predicting prognosis in acute liver failure: Ammonia and the risk of cerebral edema. Hepatology 46(6):1679–1681CrossRefPubMed
Zurück zum Zitat Ducharme-Crevier L, Dupont-Thibodeau G, Lortie A, Maranda B, Tasker RC, Jouvet P (2015) Metabolic encephalopathies in children. In: Shaffner DH, Nichols DG (eds) Rogers’ textbook of pediatric intensive care, pp 1053–1065 Ducharme-Crevier L, Dupont-Thibodeau G, Lortie A, Maranda B, Tasker RC, Jouvet P (2015) Metabolic encephalopathies in children. In: Shaffner DH, Nichols DG (eds) Rogers’ textbook of pediatric intensive care, pp 1053–1065
Zurück zum Zitat Enns GM (2008) Neurologic damage and neurocognitive dysfunction in urea cycle disorders. Semin Pediatr Neurol 15(3):132–139CrossRefPubMed Enns GM (2008) Neurologic damage and neurocognitive dysfunction in urea cycle disorders. Semin Pediatr Neurol 15(3):132–139CrossRefPubMed
Zurück zum Zitat Enns GM, Berry SA, Berry GT, Rhead WJ, Brusilow SW, Hamosh A (2007) Survival after treatment with phenylacetate and benzoate for urea-cycle disorders. N Engl J Med 356(22):2282–2292CrossRefPubMed Enns GM, Berry SA, Berry GT, Rhead WJ, Brusilow SW, Hamosh A (2007) Survival after treatment with phenylacetate and benzoate for urea-cycle disorders. N Engl J Med 356(22):2282–2292CrossRefPubMed
Zurück zum Zitat Feillet F, Leonard JV (1998) Alternative pathway therapy for urea cycle disorders. J Inherit Metab Dis 21(Suppl 1):101–111CrossRefPubMed Feillet F, Leonard JV (1998) Alternative pathway therapy for urea cycle disorders. J Inherit Metab Dis 21(Suppl 1):101–111CrossRefPubMed
Zurück zum Zitat Gunduz M, Unal S, Okur I, Ayranci Sucakli I, Guzel F, Koc N (2015) Neonates with inborn errors of metabolism: spectrum and short-term outcomes at a tertiary care hospital. Turk J Pediatr 57(1):45–52PubMed Gunduz M, Unal S, Okur I, Ayranci Sucakli I, Guzel F, Koc N (2015) Neonates with inborn errors of metabolism: spectrum and short-term outcomes at a tertiary care hospital. Turk J Pediatr 57(1):45–52PubMed
Zurück zum Zitat Haberle J, Boddaert N, Burlina A et al (2012) Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet J Rare Dis 7:32CrossRefPubMedPubMedCentral Haberle J, Boddaert N, Burlina A et al (2012) Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet J Rare Dis 7:32CrossRefPubMedPubMedCentral
Zurück zum Zitat Husson MC, Schiff M, Fouilhoux A et al (2016) Efficacy and safety of i.V. Sodium benzoate in urea cycle disorders: a multicentre retrospective study. Orphanet J Rare Dis 11(1):127CrossRefPubMedPubMedCentral Husson MC, Schiff M, Fouilhoux A et al (2016) Efficacy and safety of i.V. Sodium benzoate in urea cycle disorders: a multicentre retrospective study. Orphanet J Rare Dis 11(1):127CrossRefPubMedPubMedCentral
Zurück zum Zitat Jacques J, Carrier P, Debette-Gratien M, Sobesky R, Loustaud-Ratti V (2016) Encéphalopathie hépatique. Presse Med 45(1):46–59CrossRefPubMed Jacques J, Carrier P, Debette-Gratien M, Sobesky R, Loustaud-Ratti V (2016) Encéphalopathie hépatique. Presse Med 45(1):46–59CrossRefPubMed
Zurück zum Zitat Kamate M, Chetal V, Kulgod V, Patil V, Christopher R (2010) Profile of inborn errors of metabolism in a tertiary care Centre PICU. Indian J Pediatr 77(1):57–60CrossRefPubMed Kamate M, Chetal V, Kulgod V, Patil V, Christopher R (2010) Profile of inborn errors of metabolism in a tertiary care Centre PICU. Indian J Pediatr 77(1):57–60CrossRefPubMed
Zurück zum Zitat Leteurtre S, Martinot A, Duhamel A et al (2003) Validation of the paediatric logistic organ dysfunction (PELOD) score: prospective, observational, multicentre study. Lancet 362(9379):192–197CrossRefPubMed Leteurtre S, Martinot A, Duhamel A et al (2003) Validation of the paediatric logistic organ dysfunction (PELOD) score: prospective, observational, multicentre study. Lancet 362(9379):192–197CrossRefPubMed
Zurück zum Zitat Maranda B, Cousineau J, Allard P, Lambert M (2007) False positives in plasma ammonia measurement and their clinical impact in a pediatric population. Clin Biochem 40(8):531–535CrossRefPubMed Maranda B, Cousineau J, Allard P, Lambert M (2007) False positives in plasma ammonia measurement and their clinical impact in a pediatric population. Clin Biochem 40(8):531–535CrossRefPubMed
Zurück zum Zitat Matoori S, Leroux JC (2015) Recent advances in the treatment of hyperammonemia. Adv Drug Deliv Rev 90:55–68CrossRefPubMed Matoori S, Leroux JC (2015) Recent advances in the treatment of hyperammonemia. Adv Drug Deliv Rev 90:55–68CrossRefPubMed
Zurück zum Zitat McBryde KD, Kershaw DB, Bunchman TE et al (2006) Renal replacement therapy in the treatment of confirmed or suspected inborn errors of metabolism. J Pediatr 148(6):770–778CrossRefPubMed McBryde KD, Kershaw DB, Bunchman TE et al (2006) Renal replacement therapy in the treatment of confirmed or suspected inborn errors of metabolism. J Pediatr 148(6):770–778CrossRefPubMed
Zurück zum Zitat Ozanne B, Nelson J, Cousineau J et al (2012) Threshold for toxicity from hyperammonemia in critically ill children. J Hepatol 56(1):123–128CrossRefPubMed Ozanne B, Nelson J, Cousineau J et al (2012) Threshold for toxicity from hyperammonemia in critically ill children. J Hepatol 56(1):123–128CrossRefPubMed
Zurück zum Zitat Slater A, Shann F, Pearson G (2003) PIM2: a revised version of the Paediatric index of mortality. Intensive Care Med 29(2):278–285CrossRefPubMed Slater A, Shann F, Pearson G (2003) PIM2: a revised version of the Paediatric index of mortality. Intensive Care Med 29(2):278–285CrossRefPubMed
Zurück zum Zitat Smith W, Kishnani PS, Lee B et al (2005) Urea cycle disorders: clinical presentation outside the newborn period. Crit Care Clin 21(4 Suppl):S9–17PubMed Smith W, Kishnani PS, Lee B et al (2005) Urea cycle disorders: clinical presentation outside the newborn period. Crit Care Clin 21(4 Suppl):S9–17PubMed
Zurück zum Zitat Sushma S (1992) Sodium benzoate in the treatment of acute hepatic encephalopathy: a double-blind randomized trial. Hepatology 16:138–144CrossRefPubMed Sushma S (1992) Sodium benzoate in the treatment of acute hepatic encephalopathy: a double-blind randomized trial. Hepatology 16:138–144CrossRefPubMed
Zurück zum Zitat Uchino T, Endo F, Matsuda I (1998) Neurodevelopmental outcome of long-term therapy of urea cycle disorders in Japan. J Inherit Metab Dis 21(Suppl 1):151–159CrossRefPubMed Uchino T, Endo F, Matsuda I (1998) Neurodevelopmental outcome of long-term therapy of urea cycle disorders in Japan. J Inherit Metab Dis 21(Suppl 1):151–159CrossRefPubMed
Zurück zum Zitat Unsinn C, Das A, Valayannopoulos V et al (2016) Clinical course of 63 patients with neonatal onset urea cycle disorders in the years 2001-2013. Orphanet J Rare Dis 11(1):116CrossRefPubMedPubMedCentral Unsinn C, Das A, Valayannopoulos V et al (2016) Clinical course of 63 patients with neonatal onset urea cycle disorders in the years 2001-2013. Orphanet J Rare Dis 11(1):116CrossRefPubMedPubMedCentral
Metadaten
Titel
Influence of implementing a protocol for an intravenously administered ammonia scavenger on the management of acute hyperammonemia in a pediatric intensive care unit
verfasst von
David Brossier
Isabelle Goyer
Lydia Ziani
Christopher Marquis
Grant Mitchell
Bruno Ozanne
Philippe Jouvet
Publikationsdatum
05.06.2018
Verlag
Springer Netherlands
Erschienen in
Journal of Inherited Metabolic Disease
Print ISSN: 0141-8955
Elektronische ISSN: 1573-2665
DOI
https://doi.org/10.1007/s10545-018-0206-z

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.