reye syndrome


Summary: A form of encephalopathy with fatty infiltration of the LIVER, characterized by brain EDEMA and VOMITING that may rapidly progress to SEIZURES; COMA; and DEATH. It is caused by a generalized loss of mitochondrial function leading to disturbances in fatty acid and CARNITINE metabolism.

Top Publications

  1. Dimond B. Legal regulation mechanisms in the control of medicines. Br J Nurs. 2003;12:560-3 pubmed
    ..Were the Secretary of State and CSM liable? ..
  2. Duerksen D, Jewell L, Mason A, Bain V. Co-existence of hepatitis A and adult Reye's syndrome. Gut. 1997;41:121-4 pubmed
    ..The association between hepatitis A and Reye's syndrome has not been reported before. As hepatitis A virus infection is not sought routinely in patients with Reye's syndrome, the frequency of this association is unknown. ..
  3. Belay E, Bresee J, Holman R, Khan A, Shahriari A, Schonberger L. Reye's syndrome in the United States from 1981 through 1997. N Engl J Med. 1999;340:1377-82 pubmed
    ..Because Reye's syndrome is now very rare, any infant or child suspected of having this disorder should undergo extensive investigation to rule out the treatable inborn metabolic disorders that can mimic Reye's syndrome. ..
  4. Oka S, Liu W, Masutani H, Hirata H, Shinkai Y, Yamada S, et al. Impaired fatty acid utilization in thioredoxin binding protein-2 (TBP-2)-deficient mice: a unique animal model of Reye syndrome. FASEB J. 2006;20:121-3 pubmed
    ..Because the fatal impairment of fatty acid utilization is a characteristically metabolic feature of Reye (-like) syndrome, TBP-2-/- mouse may represent a novel model for investigating the pathophysiology of these disorders. ..
  5. Sekiyama H, Sakamoto T, Hanaoka K. [Successful use of mild hypothermia therapy in a patient with severe clinical Reye syndrome]. Masui. 2002;51:509-12 pubmed
    This report describes a case of two-yr-old female with severe clinical Reye syndrome (Stage III and over), who was treated with not only conventional therapies but also mild hypothermia therapy...
  6. Bzduch V, Behulova D, Salingova A, Ponec J, Fabriciova K, Kozak L. Serum free carnitine in medium chain acyl-CoA dehydrogenase deficiency. Bratisl Lek Listy. 2003;104:405-7 pubmed
    ..This is the first observation of this pattern during an acute attack and needs to be confirmed by other patients with MCAD deficiency. (Fig. 2, Ref. 7.). ..
  7. Yang T, Chen H, Ni Y, Hwu W, Chang M. Hereditary fructose intolerance presenting as Reye's-like syndrome: report of one case. Acta Paediatr Taiwan. 2000;41:218-20 pubmed
    ..Appropriate dietary management and precautions were recommended. The patient has been symptom-free and exhibited normal growth and development when followed up to 12 years of age. ..
  8. Lemasters J, Theruvath T, Zhong Z, Nieminen A. Mitochondrial calcium and the permeability transition in cell death. Biochim Biophys Acta. 2009;1787:1395-401 pubmed publisher
    ..Thus, the exact role of Ca(2+) for inducing the MPT and cell death depends on the particular biologic setting. ..
  9. Kurup R, Kurup P. The isoprenoid pathway and the pathogenesis of Reye's syndrome. Pediatr Pathol Mol Med. 2003;22:159-70 pubmed
    ..Reye's syndrome is associated with an upregulated isoprenoid pathway, elevated hypothalamic digoxin secretion, and right hemispheric chemical dominance. ..

More Information


  1. Tihista Jiménez J, Guergué Irazabal J, Manrique Celada M. [Refractory epilepsy status in Reye's syndrome in an adult. A case report]. Rev Neurol. 2002;35:528-30 pubmed
  2. Brassier A, Ottolenghi C, Boutron A, Bertrand A, Valmary Degano S, Cervoni J, et al. Dihydrolipoamide dehydrogenase deficiency: a still overlooked cause of recurrent acute liver failure and Reye-like syndrome. Mol Genet Metab. 2013;109:28-32 pubmed publisher
    ..G229C mutation screening as a valuable test in the Arab patients because of its high frequency. It also highlights the usefulness of genome-wide linkage analysis for decisive diagnosis advance in inherited metabolic disorders. ..
  3. Prandota J. Important role of prodromal viral infections responsible for inhibition of xenobiotic metabolizing enzymes in the pathomechanism of idiopathic Reye's syndrome, Stevens-Johnson syndrome, autoimmune hepatitis, and hepatotoxicity of the therapeutic dose. Am J Ther. 2002;9:149-56 pubmed
  4. Donald K, Doherty S, Shun A. Duodenal perforation: an interesting case report. Emerg Med Australas. 2005;17:46-8 pubmed
    ..We describe the difficulties in making the diagnosis of duodenal perforation in children and some of the factors that led to the delay in diagnosis in this case...
  5. Ee L. Rotavirus, hepatitis and Reye syndrome. J Paediatr Child Health. 2002;38:533 pubmed
  6. Meekin S, Glasgow J, McCusker C, Rooney N. A long-term follow-up of cognitive, emotional, and behavioural sequelae to Reye syndrome. Dev Med Child Neurol. 1999;41:549-53 pubmed
    Eighteen adolescents who had survived Reye syndrome (RS) in early childhood were assessed on cognitive, emotional, and behavioural variables in a second follow-up study tracking this group. Siblings were used as controls...
  7. Mizuguchi M. [Influenza encephalopathy and Reye's syndrome]. Nihon Naika Gakkai Zasshi. 2006;95:1263-7 pubmed
  8. Khater F, Moorman J. Complications of influenza. South Med J. 2003;96:740-3 pubmed
  9. Duong M, Dinoulos J, Gupta A, Bryk T, Saps M, Di Lorenzo C, et al. Index of suspicion. Pediatr Rev. 2005;26:23-33 pubmed
  10. Paprocka J, Jamroz E. Hyperammonemia in children: on the crossroad of different disorders. Neurologist. 2012;18:261-5 pubmed publisher
    ..Ammonia toxicity has been investigated for a long time. According to the main hypotheses, the neurological alterations are connected to alterations in glutamatergic neurotransmission. ..
  11. Goldman R. Bismuth salicylate for diarrhea in children. Can Fam Physician. 2013;59:843-4 pubmed
    ..Despite these findings, its limited effectiveness and concerns about it potentially causing Reye syndrome, compliance, and cost are the key reasons it is not routinely recommended for children.
  12. Brannelly N, Hamilton Shield J, Killard A. The Measurement of Ammonia in Human Breath and its Potential in Clinical Diagnostics. Crit Rev Anal Chem. 2016;46:490-501 pubmed publisher
    ..This review explores the diagnostic applications of ammonia measurement and the impact that the move from blood to breath analysis could have on how these processes and diseases are studied and managed. ..
  13. Eiris J, Ribes A, Fernández Prieto R, Rodriguez Garcia J, Rodriguez Segade S, Castro Gago M. [3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome]. Rev Neurol. 1998;26:911-4 pubmed
    ..Diagnostic of Reye syndrome was suggested by hystopathologic finding of hepatic steatosis and clinical and biochemical data...
  14. John T. Encephalopathy without rash, caused by measles virus? More evidence is needed. Indian Pediatr. 2003;40:589-93 pubmed
  15. Wei C, Chen H, Lee P, Chen C, Ma C, Hwu W. Reye's syndrome developing in an infant on treatment of Kawasaki syndrome. J Paediatr Child Health. 2005;41:303-4 pubmed
    ..His histopathological and ultrastructural findings from the liver biopsy were compatible with Reye's syndrome. He recovered completely, and there was no recurrence. ..
  16. Hou J. Primary systemic carnitine deficiency presenting as recurrent Reye-like syndrome and dilated cardiomyopathy. Chang Gung Med J. 2002;25:832-7 pubmed
    ..If SCD is suggested as a cause of Reye-like syndrome or dilated cardiomyopathy, L-carnitine therapy should be initiated as a diagnostic test immediately, until the definite diagnosis is confirmed. ..
  17. Jimenez Caballero P, Montes Gonzalo M, Velázquez Pérez J. [Reye's syndrome. Description of a case focused on the patient's epileptic seizures]. Rev Neurol. 2008;47:571-4 pubmed
  18. Pecar A. [Acetylsalicylic acid in the 4-year old child with middle ear inflammation]. Med Monatsschr Pharm. 2006;29:305 pubmed
  19. John T. Outbreak of killer brain disease in children: mystery or missed diagnosis?. Indian Pediatr. 2003;40:863-9 pubmed
  20. John T. Chandipura virus, encephalitis, and epidemic brain attack in India. Lancet. 2004;364:2175; author reply 2175-6 pubmed
  21. Dyer C. Government sued for 11 week delay in warning about aspirin. BMJ. 2002;324:134 pubmed
  22. Yoshida I. [Influenza encephalopathy, related disorders and differential diagnosis]. Nihon Rinsho. 2003;61:1959-62 pubmed
  23. Wittenstein B, Rogers V, Novelli V, Clayton P, Kenny M, Peters M. "African medicine" and Reye's syndrome. Lancet. 2004;363:860 pubmed
  24. Koizumi T, Nikaido H, Hayakawa J, Nonomura A, Yoneda T. Infantile disease with microvesicular fatty infiltration of viscera spontaneously occurring in the C3H-H-2(0) strain of mouse with similarities to Reye's syndrome. Lab Anim. 1988;22:83-7 pubmed
    ..The clinical, biochemical and histopathological findings of an infantile disease occurring in the C3H-H-2 degree strain of mice, which has similarities with Reye's syndrome in children, is described. ..
  25. Ninove L, Daniel L, Gallou J, Cougard P, Charpentier A, Viard L, et al. Fatal case of Reye's syndrome associated with H3N2 influenza virus infection and salicylate intake in a 12-year-old patient. Clin Microbiol Infect. 2011;17:95-7 pubmed publisher
    ..In the current situation of the novel A/H1N1 virus pandemic, we believe that it is of high importance to emphasize the risks associated with salicylate intake to avoid the reappearance of Reye's syndrome. ..
  26. Novikov I, Oberman B, Freedman L. Modification of the computational procedure in Parker and Bregman's method of calculating sample size from matched case-control studies with a dichotomous exposure. Biometrics. 2005;61:1123-6; discussion 1126-7 pubmed
    ..However, its recommended implementation involves a computational approximation. We show here that the approximation performs poorly in extreme situations and can be easily replaced with a more exact calculation. ..
  27. Baldellou Vazquez A. [Reye's syndrome. Forty years later]. An Pediatr (Barc). 2003;59:319-22 pubmed
  28. Watanabe K, Oie M, Higuchi M, Nishikawa M, Fujii M. Isolation and characterization of novel human parechovirus from clinical samples. Emerg Infect Dis. 2007;13:889-95 pubmed
    ..Vero cells, we isolated a virus (NII561-2000) from a cerebrospinal fluid specimen of a 1-year-old girl with Reye syndrome. The determined amino acid sequence of the virus indicated that the isolate was a human parechovirus (HPeV), a ..
  29. Bassareo P, Fanos V, Iacovidou N, Mercuro G. Antiplatelet therapy in children: why so different from adults'?. Curr Pharm Des. 2012;18:3019-33 pubmed
    ..In brief, an attempt has been made to ascertain the interesting potential of these drugs which are so often neglected in children. ..
  30. Murphy M, Crocker J, Her H. Abnormalities in hepatic fatty-acid metabolism in a surfactant/influenza B virus mouse model for acute encephalopathy. Biochim Biophys Acta. 1996;1315:208-16 pubmed
    ..Exacerbation of this and related effects by exposing young animals to xenobiotic surfactants could be the basis of surfactant-mediated potentiation of virus-induced mortality. ..
  31. Berry M. The potential of trace amines and their receptors for treating neurological and psychiatric diseases. Rev Recent Clin Trials. 2007;2:3-19 pubmed
    ..In addition, recent evidence linking trace amines to the development of drug dependence will be discussed. ..
  32. Krawitz M, Perry H. Corneal involvement in Reye syndrome. Ann Ophthalmol. 1988;20:113-4 pubmed
    Classic Reye syndrome and its sequelae have been described repeatedly in recent literature without any correlations to ocular pathology...
  33. Xia J, Wang Y. [Oral aspirin caused Reye's syndrome in a child]. Zhonghua Er Ke Za Zhi. 2004;42:243 pubmed
  34. Bajracharya B, Piya A, Manandhar D. Reye's syndrome. Kathmandu Univ Med J (KUMJ). 2003;1:138-40 pubmed
    ..Next day, however he developed the signs of encephalopathy with altered liver function. ..
  35. Ishikawa A. [Brainstem edema in patients with acute encephalopathy and encephalitis]. No To Hattatsu. 1998;30:383-6 pubmed
    The author reported eleven patients with brainstem edema on cranial CT. Nine had acute encephalopathy or Reye syndrome and the other two had acute encephalitis. Seven cases died and four cases survived...
  36. Viñas Machín P, Núñez Díaz B. [Reye syndrome: a case study in a Cuban provincial hospital from 1990 to 1996]. Rev Neurol. 1999;28:959-61 pubmed
    ..One boy had dicarboxylic aciduria. The fatal character of Reye's syndrome has been shown, as has its relation to the use Aspirin. In our environment there seems to be a tendency for Caucasian boys to be affected. ..
  37. Bhutta A, Van Savell H, Schexnayder S. Reye's syndrome: down but not out. South Med J. 2003;96:43-5 pubmed
    ..This report describes a patient with Reye's syndrome and serves as a reminder for health professionals to continue to discourage the use of aspirin for the treatment of viral infections. ..
  38. Schror K. Aspirin and Reye syndrome: a review of the evidence. Paediatr Drugs. 2007;9:195-204 pubmed
    b>Reye syndrome is an extremely rare but severe and often fatal disease. Death occurs in about 30-40% of cases from brainstem dysfunction...
  39. Vashishtha V. Mystery behind mysterious disease: far from unraveled!. Indian Pediatr. 2004;41:204-5 pubmed
  40. Sullivan K, Belay E, Durbin R, Foster D, Nordenberg D. Epidemiology of Reye's syndrome, United States, 1991-1994: comparison of CDC surveillance and hospital admission data. Neuroepidemiology. 2000;19:338-44 pubmed
    ..The true annual incidence of RS during the study years was probably between 0.2 and 1.1 cases per million population <18 years of age. ..
  41. Toovey S. Influenza-associated central nervous system dysfunction: a literature review. Travel Med Infect Dis. 2008;6:114-24 pubmed publisher
    ..Influenza is a viral pathogen that imposes an under-recognized burden of central nervous system (CNS) disease...
  42. Ghosh A, Pradhan S, Swami R, K C S, Talwar O. Reye syndrome: a case report with review of literature. JNMA J Nepal Med Assoc. 2008;47:34-7 pubmed
    Here we describe a case of Reye syndrome diagnosed at postmortem liver biopsy of a three-year old girl who presented with vomiting, low grade fever for three days and loss of consciousness for 18 hours...
  43. Casteels Van Daele M, Wouters C, Van Geet C, McGovern M, Glasgow J, Stewart M. Reye's syndrome revisited. Outdated concept of Reye's syndrome was used. BMJ. 2002;324:546 pubmed
  44. Dyer C. Woman damaged by aspirin loses court claim. BMJ. 2002;324:444 pubmed
  45. Autret Leca E, Jonville Bera A, Llau M, Bavoux F, Saudubray J, Laugier J, et al. Incidence of Reye's syndrome in France: a hospital-based survey. J Clin Epidemiol. 2001;54:857-62 pubmed
  46. Langford N. Aspirin and Reye's syndrome: is the response appropriate?. J Clin Pharm Ther. 2002;27:157-60 pubmed
  47. Pugliese A, Beltramo T, Torre D. Reye's and Reye's-like syndromes. Cell Biochem Funct. 2008;26:741-6 pubmed publisher
    ..Finally a possible diagnostic differentiation from classic Reye's syndrome and Reye's-like syndromes plus therapeutic prospects are briefly examined. ..
  48. Kirzioglu Z, Altun A. Griscelli syndrome: a case report of Reye's syndrome and atopic dermatitis history. J Indian Soc Pedod Prev Dent. 2008;26 Suppl 3:S118-20 pubmed
    ..5 years of age; she also had AD as well as a history of RS at infancy. We discuss the possible relationship between the three syndromes. ..
  49. Orlowski J, Hanhan U, Fiallos M. Is aspirin a cause of Reye's syndrome? A case against. Drug Saf. 2002;25:225-31 pubmed
    ..Reye's syndrome was probably either a viral mutation which spontaneously disappeared, or a conglomeration of metabolic disorders that had not been recognized or described at that time. ..
  50. Okabe N. [Epidemiology--surveillance information]. No To Hattatsu. 2000;32:137-41 pubmed
    ..Further investigation is necessary to find the pathogenesis of these serious complications and clarify whether this phenomenon is due to unclarified factor(s) unique in Japan or it has reminded unrecognized in other countries. ..
  51. Sans Fitó A, Campistol Plana J, Mas Salguero M, Póo Argüelles P, Fernandez Alvarez E. Pontocerebellar hypoplasia type 2 and Reye-like syndrome. J Child Neurol. 2002;17:132-4 pubmed
    ..Differential diagnosis was established with intrauterine injuries, other malformative syndromes, and neurodegenerative or neurometabolic disorders, which can be associated with cerebellar hypoplasia. ..
  52. Vashishtha V. Brief profile of an epidemic of acute encephalopathy in Western Uttar Pradesh. Indian Pediatr. 2003;40:920-2 pubmed
  53. Chen Y, Su Z. Reveal genes functionally associated with ACADS by a network study. Gene. 2015;569:294-302 pubmed publisher
    ..The current study presents a comprehensible layout of ACADS network, its functional strategies and candidate disease approach associated with ACADS network. ..