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Reye syndrome

MedGen UID:
19772
Concept ID:
C0035400
Disease or Syndrome
Synonyms: Fatty Liver with Encephalopathy; Reye's Syndrome
SNOMED CT: Reye syndrome (74351001); Reye's syndrome (74351001); Reye's encephalopathy (74351001)
 
Monarch Initiative: MONDO:0005942
Orphanet: ORPHA3096

Definition

A rare, systemic disease characterized by persistent vomiting with confusion, lethargy, disorientation, hyperreflexia, hyperventilation, and tachycardia, with rapid progression to seizures, non-inflammatory encephalopathy, coma and death. It typically develops between 12 hours and 3 weeks after recovery from a viral illness, such as upper respiratory tract infection or gastroenteritis. Hepatomegaly, acute hepatic steatosis, fatty liver degeneration and multiple laboratory abnormalities are associated. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVReye syndrome
Follow this link to review classifications for Reye syndrome in Orphanet.

Professional guidelines

PubMed

Saudubray JM, Martin D, de Lonlay P, Touati G, Poggi-Travert F, Bonnet D, Jouvet P, Boutron M, Slama A, Vianey-Saban C, Bonnefont JP, Rabier D, Kamoun P, Brivet M
J Inherit Metab Dis 1999 Jun;22(4):488-502. doi: 10.1023/a:1005556207210. PMID: 10407781
Adam D, Stankov G
Eur J Pediatr 1994 Jun;153(6):394-402. doi: 10.1007/BF01983400. PMID: 8088292
Trauner DA
Ann Neurol 1980 Jan;7(1):2-4. doi: 10.1002/ana.410070103. PMID: 6987947

Recent clinical studies

Etiology

Paneth N
Dev Med Child Neurol 2023 Dec;65(12):1551-1556. Epub 2023 May 7 doi: 10.1111/dmcn.15633. PMID: 37149891
Esposito S, Rahamat-Langendoen J, Ascolese B, Senatore L, Castellazzi L, Niesters HG
J Clin Virol 2014 Jun;60(2):84-9. Epub 2014 Mar 13 doi: 10.1016/j.jcv.2014.03.003. PMID: 24690382
Schrör K
Paediatr Drugs 2007;9(3):195-204. doi: 10.2165/00148581-200709030-00008. PMID: 17523700
Cox NJ, Subbarao K
Lancet 1999 Oct 9;354(9186):1277-82. doi: 10.1016/S0140-6736(99)01241-6. PMID: 10520648
Hurwitz ES
Epidemiol Rev 1989;11:249-53. doi: 10.1093/oxfordjournals.epirev.a036043. PMID: 2680560

Diagnosis

Paneth N
Dev Med Child Neurol 2023 Dec;65(12):1551-1556. Epub 2023 May 7 doi: 10.1111/dmcn.15633. PMID: 37149891
Esposito S, Rahamat-Langendoen J, Ascolese B, Senatore L, Castellazzi L, Niesters HG
J Clin Virol 2014 Jun;60(2):84-9. Epub 2014 Mar 13 doi: 10.1016/j.jcv.2014.03.003. PMID: 24690382
Pugliese A, Beltramo T, Torre D
Cell Biochem Funct 2008 Oct;26(7):741-6. doi: 10.1002/cbf.1465. PMID: 18711704
Cox NJ, Subbarao K
Lancet 1999 Oct 9;354(9186):1277-82. doi: 10.1016/S0140-6736(99)01241-6. PMID: 10520648
De Vivo DC
Neurol Clin 1985 Feb;3(1):95-115. doi: 10.1016/S0733-8619(18)31058-2. PMID: 3887130Free PMC Article

Therapy

Pugliese A, Beltramo T, Torre D
Cell Biochem Funct 2008 Oct;26(7):741-6. doi: 10.1002/cbf.1465. PMID: 18711704
Schrör K
Paediatr Drugs 2007;9(3):195-204. doi: 10.2165/00148581-200709030-00008. PMID: 17523700
Cox NJ, Subbarao K
Lancet 1999 Oct 9;354(9186):1277-82. doi: 10.1016/S0140-6736(99)01241-6. PMID: 10520648
Hurwitz ES
Epidemiol Rev 1989;11:249-53. doi: 10.1093/oxfordjournals.epirev.a036043. PMID: 2680560
De Vivo DC
Neurol Clin 1985 Feb;3(1):95-115. doi: 10.1016/S0733-8619(18)31058-2. PMID: 3887130Free PMC Article

Prognosis

Glasgow JF, Middleton B
Arch Dis Child 2001 Nov;85(5):351-3. doi: 10.1136/adc.85.5.351. PMID: 11668090Free PMC Article
Maheady DC
J Pediatr Health Care 1989 Sep-Oct;3(5):246-50. doi: 10.1016/0891-5245(89)90004-7. PMID: 2674393
Rockoff MA, Pascucci RC
Emerg Med Clin North Am 1983 Apr;1(1):87-100. PMID: 6440775
Crocker JF
Semin Liver Dis 1982 Nov;2(4):340-52. doi: 10.1055/s-2008-1040720. PMID: 6763346
Knight V
Dis Mon 1976 Aug;22(11):1-48. doi: 10.1016/s0011-5029(76)80009-0. PMID: 1048031

Clinical prediction guides

Butterworth RF
Neurochem Int 2002 Aug-Sep;41(2-3):81-5. doi: 10.1016/s0197-0186(02)00027-x. PMID: 12020607
Adam D, Stankov G
Eur J Pediatr 1994 Jun;153(6):394-402. doi: 10.1007/BF01983400. PMID: 8088292
Hurwitz ES
Epidemiol Rev 1989;11:249-53. doi: 10.1093/oxfordjournals.epirev.a036043. PMID: 2680560
Heubi JE, Partin JC, Partin JS, Schubert WK
Hepatology 1987 Jan-Feb;7(1):155-64. doi: 10.1002/hep.1840070130. PMID: 3542776
Knight V
Dis Mon 1976 Aug;22(11):1-48. doi: 10.1016/s0011-5029(76)80009-0. PMID: 1048031

Recent systematic reviews

van Rijt WJ, Koolhaas GD, Bekhof J, Heiner Fokkema MR, de Koning TJ, Visser G, Schielen PC, van Spronsen FJ, Derks TG
Neonatology 2016;109(4):297-302. Epub 2016 Feb 24 doi: 10.1159/000443874. PMID: 26907928

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