RE
MCID: RYS001
MIFTS: 51

Reye Syndrome (RE)

Categories: Bone diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Reye Syndrome

MalaCards integrated aliases for Reye Syndrome:

Name: Reye Syndrome 12 52 58 54 42 43 15 17 71 32
Reye's Syndrome 12 74 52 53
Rasmussen Encephalitis 52 36 54
Fatty Liver with Encephalopathy 52
Rasmussen's Encephalitis 53
Rs 52
Re 52

Classifications:

Orphanet: 58  
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:14525
KEGG 36 H01812
ICD9CM 34 331.81
MeSH 43 D012202
NCIt 49 C34983
SNOMED-CT 67 74351001
ICD10 32 G93.7
MESH via Orphanet 44 D012202
ICD10 via Orphanet 33 G93.7
UMLS via Orphanet 72 C0035400
Orphanet 58 ORPHA3096
UMLS 71 C0035400

Summaries for Reye Syndrome

MedlinePlus : 42 Reye syndrome is a rare illness that can affect the blood, liver, and brain of someone who has recently had a viral infection. It always follows another illness. Although it mostly affects children and teens, anyone can get it. It can develop quickly and without warning. It is most common during flu season. Symptoms include Nausea and vomiting Listlessness Personality change - such as irritability, combativeness or confusion Delirium Convulsions Loss of consciousness If these symptoms occur soon after a viral illness, seek medical attention immediately. Reye syndrome can lead to a coma and brain death, so quick diagnosis and treatment are critical. Treatment focuses on preventing brain damage. There is no cure. The cause of Reye syndrome is unknown. Studies have shown that taking aspirin increases the risk of getting it. Because of that, health care professionals now recommend other pain relievers for young patients. NIH: National Institute of Neurological Disorders and Stroke

MalaCards based summary : Reye Syndrome, also known as reye's syndrome, is related to long-chain 3-hydroxyacyl-coa dehydrogenase deficiency and 3-hydroxyacyl-coa dehydrogenase deficiency, and has symptoms including seizures, tremor and back pain. An important gene associated with Reye Syndrome is ACADM (Acyl-CoA Dehydrogenase Medium Chain), and among its related pathways/superpathways are T cell receptor signaling pathway and Metabolism. The drugs Tacrolimus and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include liver, brain and t cells, and related phenotypes are cardiovascular system and growth/size/body region

Disease Ontology : 12 A syndrome characterized by acute brain damage and liver function problems. It has been associated with aspirin consumption by children with viral illness, although it also occurs in the absence of aspirin use.

NIH Rare Diseases : 52 Rasmussen encephalitis is a chronic inflammatory neurological disease that usually affects only one hemisphere of the brain. It most often occurs in children under the age of 10, although adolescents and adults may also be affected. Rasmussen encephalitis is characterized by frequent and severe seizures , loss of motor skills and speech, paralysis on one side of the body (hemiparesis), inflammation of the brain (encephalitis), and mental deterioration. While the cause of Rasmussen encephalitis is unknown, there is evidence that in many patients it is an autoimmune disorder. Immune therapy and surgery may be used for treatment.

NINDS : 53 Rasmussen’s encephalitis (RE) is a very rare, chronic inflammatory neurological disease that usually affects only one hemisphere (half) of the brain.  It most often occurs in children under the age of 10 but can also affect adolescents and adults.  It has features of an autoimmune disease in which immune system cells enter the brain and cause damage.  RE is characterized by frequent and severe seizures, progressive loss of neurological functions including motor skills, speech, and eventual paralysis on one side of the body (hemiparesis), inflammation of the brain (encephalitis), and mental deterioration.  Children with RE frequently enter a phase of permanent, but stable, neurological deficits after 8 to 12 months; the disease in adults and adolescents may continue to progress slowly.

KEGG : 36 Rasmussen encephalitis is a rare chronic neurological disorder, characterised by unilateral inflammation of the cerebral cortex, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. It predominantly affects children, with the onset in adults having a milder course. Infection occurring around two weeks prior to onset is observed in 38% of the patients. There is evidence of autoimmune involvement in the pathogenesis. The immunopathology of this disease is attributed mainly to activated cytotoxic T cells. It has been reported that genetic variations of immunoregulatory genes associated with Rasmussen syndrome.

Wikipedia : 74 Reye syndrome is a rapidly progressive encephalopathy. Symptoms may include vomiting, personality... more...

Related Diseases for Reye Syndrome

Diseases related to Reye Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 551)
# Related Disease Score Top Affiliating Genes
1 long-chain 3-hydroxyacyl-coa dehydrogenase deficiency 31.7 HADHA ACADVL ACADM
2 3-hydroxyacyl-coa dehydrogenase deficiency 31.7 HADHA ACADVL ACADM
3 3-methylcrotonyl-coa carboxylase deficiency 31.5 HADHA CPT2 ACADVL ACADM
4 carnitine palmitoyltransferase i deficiency 31.0 HADHA CPT2 ACADVL ACADM
5 carbonic anhydrase va deficiency, hyperammonemia due to 30.8 OTC ASS1
6 acyl-coa dehydrogenase, medium-chain, deficiency of 30.6 SLC22A5 HADHA ETFDH CPT2 ACADVL ACADM
7 acyl-coa dehydrogenase, short-chain, deficiency of 30.5 HADHA ETFDH CPT2 ACADVL ACADM
8 acyl-coa dehydrogenase, very long-chain, deficiency of 30.5 HADHA ETFDH CPT2 ACADVL ACADM
9 carbamoyl phosphate synthetase i deficiency, hyperammonemia due to 30.2 OTC ASS1
10 fatty liver disease, nonalcoholic 1 30.1 HADHA GPT
11 hepatic encephalopathy 29.9 GPT GLUL ASS1
12 carnitine deficiency, systemic primary 29.8 SLC22A5 PC HADHA ETFDH CPT2 ACADVL
13 hypoglycemia 28.7 SLC22A5 PC HMGCL HADHA CPT2 ACADVL
14 propionic acidemia 28.6 PC OTC HMGCL HADHA ASS1 ACADVL
15 acyl-coa dehydrogenase deficiency 28.4 SLC22A5 PC OTC HADHA ETFDH ASS1
16 respiratory failure 28.2 HADHA GPT CPT2 ACADVL
17 multiple acyl-coa dehydrogenase deficiency 27.9 SLC22A5 HADHA ETFDH CPT2 ACADVL ACADM
18 severe combined immunodeficiency with sensitivity to ionizing radiation 12.0
19 lymphoma, hodgkin, classic 11.6
20 3-hydroxy-3-methylglutaryl-coa lyase deficiency 11.6
21 adenocarcinoma 11.6
22 retinoschisis 1, x-linked, juvenile 11.6
23 chickenpox 11.5
24 myelodysplastic syndrome 11.5
25 richter's syndrome 11.5
26 raynaud disease 11.5
27 robinow syndrome 11.5
28 bilirubin metabolic disorder 11.4
29 mitochondrial trifunctional protein deficiency 11.3
30 roberts syndrome 11.2
31 lymphoma, hodgkin, x-linked pseudoautosomal 11.2
32 encephalopathy 10.6
33 focal epilepsy 10.5
34 ocular motor apraxia 10.5
35 aphasia 10.4
36 postpartum psychosis 10.4 OTC ASS1
37 low compliance bladder 10.4
38 external auditory canal, bilateral atresia of, with congenital vertical talus 10.4
39 hemifacial atrophy, progressive 10.4
40 facial hemiatrophy 10.4
41 rasmussen johnsen thomsen syndrome 10.4
42 rasmussen subacute encephalitis 10.4
43 dystonia 10.3
44 argininemia 10.2 OTC HADHA ASS1
45 pyrimidine metabolic disorder 10.2 OTC ASS1
46 medium-chain acyl-coenzyme a dehydrogenase deficiency 10.2
47 bronchiolitis 10.2
48 hemimegalencephaly 10.2
49 migraine with or without aura 1 10.2
50 epilepsy 10.2

Graphical network of the top 20 diseases related to Reye Syndrome:



Diseases related to Reye Syndrome

Symptoms & Phenotypes for Reye Syndrome

UMLS symptoms related to Reye Syndrome:


seizures, tremor, back pain, pain, headache, syncope, chronic pain, sciatica, vertigo/dizziness, sleeplessness

MGI Mouse Phenotypes related to Reye Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.02 ACADM ACADVL CES1 CPT2 ETFDH GLUL
2 growth/size/body region MP:0005378 10 ACADVL ASS1 GLUL GRIA3 HADHA HMGCL
3 homeostasis/metabolism MP:0005376 9.97 ACADM ACADVL ASS1 CES1 CPT2 GLUL
4 liver/biliary system MP:0005370 9.65 ACADM ACADVL CES1 GLUL HADHA HMGCL
5 mortality/aging MP:0010768 9.47 ACADM ACADVL ASS1 CPT2 ETFDH GLUL

Drugs & Therapeutics for Reye Syndrome

Drugs for Reye Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 16)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tacrolimus Approved, Investigational Phase 2, Phase 3 104987-11-3 445643 439492 6473866
2 Antibodies Phase 2, Phase 3
3 Immunoglobulins Phase 2, Phase 3
4 Immunoglobulins, Intravenous Phase 2, Phase 3
5 Rho(D) Immune Globulin Phase 2, Phase 3
6 Immunosuppressive Agents Phase 2, Phase 3
7 gamma-Globulins Phase 2, Phase 3
8 Calcineurin Inhibitors Phase 2, Phase 3
9
rituximab Approved Phase 1 174722-31-7 10201696
10 PK 11195 Phase 1 85532-75-8
11 Antirheumatic Agents Phase 1
12 Immunologic Factors Phase 1
13 Antineoplastic Agents, Immunological Phase 1
14
Adalimumab Approved 331731-18-1 16219006
15 Vaccines
16 Anti-Inflammatory Agents

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Efficacy of Tacrolimus and i.v.-Immunoglobulins in Rasmussen Encephalitis With Start of Treatment in the Acute Disease Stage. Prospective, Randomised, Open Parallel Group Study Unknown status NCT00545493 Phase 2, Phase 3 Tacrolimus;i.v. immunoglobulins
2 The Distribution and Quantification of Specific Binding of the Positron Emission Tomography Tracer R)-[11C]PK11195 in Normal Human Subjects Unknown status NCT00205595 Phase 1
3 A Pilot Study of the Use of Rituximab in the Treatment of Chronic Focal Encephalitis Completed NCT00259805 Phase 1 Rituximab
4 Intracranial Hypertension Detection Using Optic Nerve Sheath Diameter Measurement in Children Completed NCT01796015
5 Randomized Evaluation of Live Attenuated vs.Trivalent Inactivated Influenza Vaccines in Schools (RELATIVES)Pilot Study Completed NCT01995851
6 Observational Study on Patients With Rasmussen Encephalitis Treated With Adalimumab: Efficacy and Tolerance in the Short and Long Term Not yet recruiting NCT04003922

Search NIH Clinical Center for Reye Syndrome

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Perphenazine

Cochrane evidence based reviews: reye syndrome

Genetic Tests for Reye Syndrome

Anatomical Context for Reye Syndrome

MalaCards organs/tissues related to Reye Syndrome:

40
Liver, Brain, T Cells, Heart, Cortex, Kidney, Prostate

Publications for Reye Syndrome

Articles related to Reye Syndrome:

(show top 50) (show all 1684)
# Title Authors PMID Year
1
Co-ingestion of aspirin and acetaminophen promoting fulminant liver failure: A critical review of Reye syndrome in the current perspective at the dawn of the 21st century. 61 42
28945927 2018
2
REYE (RAY'S) SYNDROME: A PROBLEM EVERYONE SHOULD REMEMBER. 61 42
29227269 2017
3
Carnitine palmitoyltransferase 1A deficiency: abnormal muscle biopsy findings in a child presenting with Reye's syndrome. 61 42
28466427 2017
4
Evidence that antioxidants prevent the inhibition of Na+,K(+)-ATPase activity induced by octanoic acid in rat cerebral cortex in vitro. 54 61
12892042 2003
5
Successful use of alternate waste nitrogen agents and hemodialysis in a patient with hyperammonemic coma after heart-lung transplantation. 54 61
10199339 1999
6
Frequency of 985A-to-G mutation in medium-chain acyl-CoA dehydrogenase gene among patients with sudden infant death syndrome, Reye syndrome, severe motor and intellectual disabilities and healthy newborns in Japan. 54 61
8840534 1996
7
[Hydroxy-methyl-glutaryl-coenzyme A lyase deficiency manifesting as Reye's syndrome in a 3-year-old girl]. 54 61
7777393 1993
8
Displacement of hepatic ornithine carbamoyltransferase from mitochondria to cytosol in Reye's syndrome. 54 61
1782015 1991
9
Prevalence of K329E mutation in medium-chain acyl-CoA dehydrogenase gene determined from Guthrie cards. 54 61
1678810 1991
10
[Hyperlysinemia and hyperammonemia]. 54 61
1904697 1991
11
Hepatic polyamine metabolism in children with Reye's syndrome. 54 61
1823865 1991
12
Medium-chain acyl CoA dehydrogenase deficiency: electron microscopic differentiation from Reye syndrome. 54 61
2279505 1990
13
[Medium-chain acyl-CoA dehydrogenase defect. Acute cerebral episodes and nonketotic hypoglycemia in children]. 54 61
2387217 1990
14
A case of ornithine transcarbamylase deficiency with acute and late onset simulating Reye's syndrome in an adult male. 54 61
2255172 1990
15
A rare case of Reye's syndrome induced by influenza A virus with use of ibuprofen in an adult. 61
31988769 2020
16
Aspirin rechallenge in an adult patient previously diagnosed with Reye syndrome. 61
31788685 2020
17
Fructose-1,6-Bisphosphatase Deficiency 61
31804789 2019
18
United States Pharmacopeia Safety Review of Willow Bark. 61
31604354 2019
19
First Case Report of Primary Carnitine Deficiency Manifested as Intellectual Disability and Autism Spectrum Disorder. 61
31200524 2019
20
Presence of unreported carcinogens, Aflatoxins and their hydroxylated metabolites, in industrialized Oaxaca cheese from Mexico City. 61
30468840 2019
21
Detection and monitoring of in vitro formation of salicylic acid from aspirin using fluorescence spectroscopic technique and DFT calculations. 61
30445361 2018
22
[Reye syndrome and sudden death symptoms after oral administration of nimesulide due to upper respiratory tract infection in a boy]. 61
30477628 2018
23
Lipid Storage Myopathy with Ketonuria: A Case of Fatty Acid Oxidation-Related Myopathy and Encephalopathy due to Multiple Acyl-CoA Dehydrogenase Deficiency. 61
30271477 2018
24
Hemorrhagic Stroke and Cerebral Venous Thrombosis: Rare Neurological Sequelae of Chickenpox Infection. 61
30258269 2018
25
Acute Kidney Failure as a Single Complication of Varicella Virus Infection in an Adult Patient. 61
30197901 2018
26
A Case of Reye Syndrome Caused by Influenza A Virus. 61
30559633 2018
27
Presumed acute fatty liver of pregnancy following influenza A hepatitis. 61
29225680 2017
28
[ACTION OF L-CARNITINE, CORVITIN AND THEIR COMBINATION ON FUNCTIONAL STATE OF LIVER IN EXPERIMENTAL MODEL OF REYE SYNDROME IN RATS]. 61
28452736 2017
29
Aspirin increases mitochondrial fatty acid oxidation. 61
27856258 2017
30
Management and long-term evolution of a patient with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. 61
28257639 2017
31
The Measurement of Ammonia in Human Breath and its Potential in Clinical Diagnostics. 61
26907707 2016
32
[Side Effects of Acetaminophen and their Management]. 61
30358301 2016
33
Clinical aspects of urea cycle dysfunction and altered brain energy metabolism on modulation of glutamate receptors and transporters in acute and chronic hyperammonemia. 61
27261594 2016
34
The Use of p-Aminobenzoic Acid as a Probe Substance for the Targeted Profiling of Glycine Conjugation. 61
26484797 2016
35
Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules. 61
26826406 2016
36
Acetylsalicylic acid as a potential pediatric health hazard: legislative aspects concerning accidental intoxications in the European Union. 61
27418941 2016
37
Inborn Errors of Metabolism That Cause Sudden Infant Death: A Systematic Review with Implications for Population Neonatal Screening Programmes. 61
26907928 2016
38
Impact of fatty acid oxidation disorders in child neurology: from Reye syndrome to Pandora's box. 61
25761966 2015
39
Aspirin-Induced Acute Liver Injury. 61
26157904 2014
40
Resveratrol protects against experimental induced Reye's syndrome by prohibition of oxidative stress and restoration of complex I activity. 61
25162205 2014
41
Update on pediatric neurocritical care. 61
24698020 2014
42
Cerebellar mutism caused by primary varicella infection in an immunocompetent child. 61
23446802 2014
43
Pediatric parechovirus infections. 61
24690382 2014
44
The history of external ventricular drainage. 61
23889138 2014
45
Nonsteroidal anti-inflammatory drugs exposure and the central nervous system. 61
24365321 2014
46
[Aspirin and its danger: Reye syndrome in young adult]. 61
24161294 2013
47
Clinical and biological features at diagnosis in mitochondrial fatty acid beta-oxidation defects: a French pediatric study of 187 patients. 61
23053472 2013
48
Growth and development of a new subspecialty: pediatric hepatology. 61
23788321 2013
49
Bismuth salicylate for diarrhea in children. 61
23946025 2013
50
Hyponatremic seizures and Reye syndrome. 61
23829993 2013

Variations for Reye Syndrome

Expression for Reye Syndrome

Search GEO for disease gene expression data for Reye Syndrome.

Pathways for Reye Syndrome

Pathways related to Reye Syndrome according to KEGG:

36
# Name Kegg Source Accession
1 T cell receptor signaling pathway hsa04660

Pathways related to Reye Syndrome according to GeneCards Suite gene sharing:

(show all 19)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.51 SCD PC OTC OAT HMGCR HMGCL
2
Show member pathways
12.72 SCD HMGCR HMGCL HADHA CPT2 ACADVL
3
Show member pathways
12.31 SCD HADHA CPT2 ACADVL ACADM
4
Show member pathways
12.06 PC OTC HADHA GPT GLUL ASS1
5
Show member pathways
11.81 HMGCL HADHA ACADM
6 11.58 SCD CPT2 ACADM
7
Show member pathways
11.51 HADHA ACADVL ACADM
8
Show member pathways
11.35 HADHA CPT2 ACADVL ACADM
9
Show member pathways
11.28 OAT GPT GLUL
10 11.21 GPT GLUL ASS1
11 11.18 PC OTC OAT HMGCL GSR GLUL
12 11 CPT2 ACADM
13
Show member pathways
10.99 OTC OAT ASS1
14 10.92 SCD PC
15
Show member pathways
10.87 OTC GPT GLUL ASS1
16 10.85 SCD HMGCR
17
Show member pathways
10.66 PC GPT ASS1
18
Show member pathways
10.55 OAT GLUL
19
Show member pathways
10.54 HADHA ACADM

GO Terms for Reye Syndrome

Cellular components related to Reye Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 mitochondrial matrix GO:0005759 9.56 PC OTC OAT HMGCL GSR ETFDH
2 mitochondrial inner membrane GO:0005743 9.55 OTC HADHA ETFDH CPT2 ACADVL
3 mitochondrion GO:0005739 9.44 PC OTC OAT HMGCL HADHA GZMB

Biological processes related to Reye Syndrome according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 oxidation-reduction process GO:0055114 9.95 SCD HMGCR HADHA GSR ETFDH ACADVL
2 fatty acid metabolic process GO:0006631 9.72 SCD HADHA CPT2 ACADVL ACADM
3 regulation of lipid metabolic process GO:0019216 9.71 HMGCR CPT2 ACADM
4 liver development GO:0001889 9.7 OTC HMGCL ASS1
5 response to nutrient GO:0007584 9.67 HMGCR HMGCL ASS1
6 lipid metabolic process GO:0006629 9.56 SCD PC HMGCR HMGCL HADHA CPT2
7 response to fatty acid GO:0070542 9.54 SCD HMGCL ASS1
8 urea cycle GO:0000050 9.51 OTC ASS1
9 midgut development GO:0007494 9.48 OTC ASS1
10 medium-chain fatty acid metabolic process GO:0051791 9.46 CES1 ACADM
11 fatty acid beta-oxidation GO:0006635 9.46 HADHA CPT2 ACADVL ACADM
12 arginine biosynthetic process GO:0006526 9.43 OTC ASS1
13 fatty acid beta-oxidation using acyl-CoA dehydrogenase GO:0033539 9.43 ETFDH ACADVL ACADM
14 cellular amino acid biosynthetic process GO:0008652 9.02 OTC OAT GPT GLUL ASS1

Molecular functions related to Reye Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 catalytic activity GO:0003824 9.62 PC HMGCL HADHA GLUL
2 ligase activity GO:0016874 9.61 PC GLUL ASS1
3 oxidoreductase activity, acting on the CH-CH group of donors GO:0016627 9.46 ACADVL ACADM
4 transaminase activity GO:0008483 9.4 OAT GPT
5 amino acid binding GO:0016597 9.37 OTC ASS1
6 fatty-acyl-CoA binding GO:0000062 9.33 HMGCL HADHA ACADVL
7 acyl-CoA dehydrogenase activity GO:0003995 9.26 ACADVL ACADM
8 flavin adenine dinucleotide binding GO:0050660 9.26 GSR ETFDH ACADVL ACADM
9 oxidoreductase activity GO:0016491 9.17 SCD HMGCR HADHA GSR ETFDH ACADVL

Sources for Reye Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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