Abstract
Encephalopathies in children arise from an array of sources. Intoxication from accidental ingestion can produce a profound encephalopathy that clears almost as quickly as it appears. The encephalopathy of septic shock is under-appreciated, yet the brain should be counted among organs shut down, almost always temporarily, in children with multiple organ failure. The child’s brain is fairly resilient in the face of many of these entities, except rarely in the face of meningitis, but even here severe long-term damage seems to result only from the rare misdiagnosed or under-treated case.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Albrecht, J. and M.D. Norenberg, Glutamine: A Trojan horse in ammonia neurotoxicity. Hepatology, 2006. 44(4): pp. 788–794
Als-Nielsen, B., L.L. Gluud, and C. Gluud, Non-absorbable disaccharides for hepatic encephalopathy: Systematic review of randomised trials. Bmj, 2004. 328(7447): p. 1046
Azzopardi, J., et al., Lack of evidence of cerebral oedema in adults treated for diabetic ketoacidosis with fluids of different tonicity. Diabetes Res Clin Pract, 2002. 57(2): pp. 87–92
Bachmann, C.,Mechanisms of hyperammonemia. Clin Chem Lab Med, 2002. 40(7): pp. 653–662
Bhowmick, S.K., K.L. Levens, and K.R. Rettig, Hyperosmolar hyperglycemic crisis: An acute life-threatening event in children and adolescents with type 2 diabetes mellitus. Endocr Pract, 2005. 11(1): pp. 23–29
Biancofiore, G., et al., Combined twice-daily plasma exchange and continuous veno-venous hemodiafiltration for bridging severe acute liver failure. Transplant Proc, 2003. 35(8): pp. 3011–3014
Churchwell, K.B., et al., Intensive blood and plasma exchange for treatment of coagulopathy in meningococcemia. J Clin Apher, 1995. 10(4): pp. 171–177
Davis, J., et al., DKA, CVL and DVT. Increased risk of deep venous thrombosis in children with diabetic ketoacidosis and femoral central venous lines. Ir Med J, 2007. 100(1): p. 344
Demetriou, A.A., Hepatic assist devices. Panminerva Med, 2005. 47(1): pp. 31–37
Di Carlo, J.V. and S.R. Alexander, Hemofiltration for cytokine-driven illnesses: The mediator delivery hypothesis. Int J Artif Organs, 2005. 28(8): pp. 777–786
Dunger, D.B., et al., ESPE/LWPES consensus statement on diabetic ketoacidosis in children and adolescents. Arch Dis Child, 2004. 89(2): pp. 188–194
Edge, J.A., et al., Conscious level in children with diabetic ketoacidosis is related to severity of acidosis and not to blood glucose concentration. Pediatr Diabetes, 2006a. 7(1): pp. 11–15
Edge, J.A., et al., The UK case-control study of cerebral oedema complicating diabetic ketoacidosis in children. Diabetologia, 2006b. 49(9): pp. 2002–2009
Festi, D., et al., Management of hepatic encephalopathy: Focus on antibiotic therapy. Digestion, 2006. 73(Suppl 1): pp. 94–101
Fourtner, S.H., S.A. Weinzimer, and L.E. Levitt Katz, Hyperglycemic hyperosmolar non-ketotic syndrome in children with type 2 diabetes. Pediatr Diabetes, 2005. 6(3): pp. 129–135
Glaser, N., et al., Risk factors for cerebral edema in children with diabetic ketoacidosis. The Pediatric Emergency Medicine Collaborative Research Committee of the American Academy of Pediatrics. N Engl J Med, 2001. 344(4): pp. 264–269
Glaser, N.S., et al., Mechanism of cerebral edema in children with diabetic ketoacidosis. J Pediatr, 2004. 145(2): pp. 164–171
Glaser, N.S., et al., Frequency of sub-clinical cerebral edema in children with diabetic ketoacidosis. Pediatr Diabetes, 2006. 7(2): pp. 75–80
Gordon, N., Ornithine transcarbamylase deficiency: A urea cycle defect. Eur J Paediatr Neurol, 2003. 7(3): pp. 115–121
Halestrap, A.P., Calcium, mitochondria and reperfusion injury: A pore way to die. Biochem Soc Trans, 2006. 34(Pt 2): pp. 232–237
Hammer, G.B., et al., Continuous venovenous hemofiltration with dialysis in combination with total hepatectomy and portocaval shunting. Bridge to liver transplantation. Transplantation, 1996. 62(1): pp. 130–132
Henter, J.I. and I. Nennesmo, Neuropathologic findings and neurologic symptoms in twenty-three children with hemophagocytic lymphohistiocytosis. J Pediatr, 1997. 130(3): pp. 358–365
Jones, M., Energy metabolism in the developing brain. Seminars in Perinatology, 1979. 3: pp. 121–129
Jones, E.A., Ammonia, the GABA neurotransmitter system, and hepatic encephalopathy. Metab Brain Dis, 2002. 17(4): pp. 275–281
Kawata, H., et al., The use of continuous hemodiafiltration in a patient with diabetic ketoacidosis. J Anesth, 2006. 20(2): pp. 129–131
Legras, A., et al., Late diagnosis of ornithine transcarbamylase defect in three related female patients: Polymorphic presentations. Crit Care Med, 2002. 30(1): pp. 241–244
Magee, M.F. and B.A. Bhatt, Management of decompensated diabetes. Diabetic ketoacidosis and hyperglycemic hyperosmolar syndrome. Crit Care Clin, 2001. 17(1): pp. 75–106
Mattarozzi, K., et al., Distinguishing between clinical and minimal hepatic encephalopathy on the basis of specific cognitive impairment. Metab Brain Dis, 2005. 20(3): pp. 243–249
Morales, A.E. and A.L. Rosenbloom, Death caused by hyperglycemic hyperosmolar state at the onset of type 2 diabetes. J Pediatr, 2004. 144(2): pp. 270–273
Muir, A., et al., Cerebral edema in childhood diabetic ketoacidosis: Natural history, radiographic findings and early identification. 2001
Mustafa, A. and J.T. Clarke, Ornithine transcarbamoylase deficiency presenting with acute liver failure. J Inherit Metab Dis, 2006. 29(4): p. 586
Ogier de Baulny, H., Management and emergency treatments of neonates with a suspicion of inborn errors of metabolism. Semin Neonatol, 2002. 7(1): pp. 17–26
Ogier de Baulny, H. and J.M. Saudubray, Branched-chain organic acidurias. Semin Neonatol, 2002. 7(1): pp. 65–74
Ong, J.P., et al., Correlation between ammonia levels and the severity of hepatic encephalopathy. Am J Med, 2003. 114(3): pp. 188–193
Puliyel, J.M. and V. Bhambhani, Ketoacid levels may alter osmotonicity in diabetic ketoacidosis and precipitate cerebral edema. Arch Dis Child, 2003. 88(4): p. 366
Roberts, J.S., et al., Cerebral hyperemia and impaired cerebral autoregulation associated with diabetic ketoacidosis in critically ill children. Crit Care Med, 2006. 34(8): pp. 2217–2223
Ryan, C.J., et al., Multisorbent plasma perfusion in fulminant hepatic failure: effects of duration and frequency of treatment in rats with grade III hepatic coma. Artif Organs, 2001. 25(2): pp. 109–118
Segura-Bruna, N., et al., Valproate-induced hyperammonemic encephalopathy. Acta Neurol Scand, 2006. 114(1): pp. 1–7
Smith, D.B. and J. Gulinson, Fatal cerebral edema complicating toxic shock syndrome. Neurosurgery, 1988. 22(3): pp. 598–599
Stange, J., et al., The molecular adsorbents recycling system as a liver support system based on albumin dialysis: A summary of preclinical investigations, prospective, randomized, controlled clinical trial, and clinical experience from 19 centers. Artif Organs, 2002. 26(2): pp. 103–110
Summar, M.L., et al., Unmasked adult-onset urea cycle disorders in the critical care setting. Crit Care Clin, 2005. 21(Suppl 4): pp. S1–S8
Thakur, V., et al., Fatal cerebral edema from late-onset ornithine transcarbamylase deficiency in a juvenile male patient receiving valproic acid. Pediatr Crit Care Med, 2006. 7(3): pp. 273–276
Verrotti, A., et al., Valproate-induced hyperammonemic encephalopathy. Metab Brain Dis, 2002. 17(4): pp. 367–373
Wooton-Gorges, S., et al., Detection of cerebral beta-hydroxybutyrate, acetoacetate and lactate on proton MR spectroscopy in children with diabetic ketoacidosis. Am J Neuroradiol, 2005. 26: pp. 1286–1291
Yorgin, P.D., et al., Concurrent centrifugation plasmapheresis and continuous venovenous hemodiafiltration. Pediatr Nephrol, 2000. 14(1): pp. 18–21
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2009 Springer Science+Business Media, LLC
About this chapter
Cite this chapter
DiCarlo, J. (2009). Metabolic Encephalopathies in Children. In: McCandless, D. (eds) Metabolic Encephalopathy. Springer, New York, NY. https://doi.org/10.1007/978-0-387-79112-8_8
Download citation
DOI: https://doi.org/10.1007/978-0-387-79112-8_8
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-0-387-79109-8
Online ISBN: 978-0-387-79112-8
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)