Reye Syndrome (Reye's Syndrome)

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.

Also Known As:

Networked: 626 relevant articles (13 outcomes, 50 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Liver Diseases (Liver Disease)
2.	Hypothermia
3.	Acidosis
4.	Infection
5.	Hyperammonemia

Experts

1.	Kurup, Ravi Kumar: 3 articles (09/2003 - 01/2003)
2.	Butterworth, Roger F: 3 articles (09/2003 - 08/2002)
3.	Kurup, Parameswara Achutha: 3 articles (09/2003 - 01/2003)
4.	Saratikov, A S: 3 articles (09/2000 - 03/2000)
5.	Kido, Hiroshi: 2 articles (01/2012 - 06/2004)
6.	Nakamura, Hajime: 2 articles (10/2009 - 01/2006)
7.	Yodoi, Junji: 2 articles (10/2009 - 01/2006)
8.	Masutani, Hiroshi: 2 articles (10/2009 - 01/2006)
9.	Mizuguchi, M: 2 articles (04/2007 - 01/2007)
10.	Shiomi, M: 2 articles (04/2007 - 01/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Reye Syndrome:

1.	Carnitine (L-Carnitine)FDA LinkGeneric 06/01/1999 - "Hepatic CPT I deficiency is characterized by recurrent episodes of Reye-like syndrome, whereas severe muscular and cardiac signs are associated with episodes of fasting hypoglycaemia in defects of carnitine transport and translocase. " 11/01/1994 - "An infant girl presented with recurrent episodes of Reye-like syndrome associated with hypoketosis and plasma carnitine levels in the high-normal range. " 08/01/1990 - "The above mitochondrial swelling and abnormal carnitine status are hallmarks of Reye's syndrome (RS). " 08/01/1990 - "In patients diagnosed as Reye syndrome, tissue carnitine deficiency was not always recognized and no decrease in the free/total carnitine ratio was found in the liver or muscle. " 08/01/1990 - "Carnitine deficiency in inherited organic acid disorders and Reye syndrome."
2.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 05/01/1996 - "The decline in Reye's syndrome after the aspirin warnings cannot be explained entirely, as has been proposed, by improved diagnosis of 'Reye-like' inherited metabolic and other disorders: this would not account for the greater decline of the high scoring subgroup which also contained those cases most likely to resemble 'classical' Reye's syndrome and to have received aspirin. " 08/01/2001 - "At the time of the study no information was available in France about the incidence of Reye's Syndrome (RS) and no warnings about RS and aspirin. " 05/16/1999 - "To study the behavior of Reye's syndrome in a group of patients during the years 1990-1996 in a provincial paediatric hospital and review variables such as age, sex, race, prodromal illness, previous consumption of Aspirin, hospital stay, mortality, diagnosis of dicarboxylic aciduria, etc. We selected patients with Reye's syndrome seen during the period mentioned and considered the above variables. " 05/01/1996 - "This study provides further evidence for the role of aspirin in a subset of cases meeting the standard diagnostic criteria for Reye's syndrome and supports the need to consider this disorder as a heterogeneous group of conditions including Reye-like inherited metabolic disorders." 09/01/1989 - "Studies strongly support a link between the use of aspirin and Reye's syndrome. "
3.	Pentobarbital (Nembutal)FDA Link 01/01/1989 - "Pentobarbital quantitation using immunoassays in Reye's syndrome patient serum." 06/01/1979 - "Careful anesthetic and critical-care management, appropriate, reliable monitoring, and pentobarbital therapy may constitute the most successful therapy to date for patients with Reye's syndrome and increased ICP." 04/01/1982 - "The diminished systemic clearance of pentobarbital may result from decreases in intrinsic enzyme activity that accompany hypothermia, as well as hepatic dysfunction in patients with Reye syndrome. " 04/01/1982 - "Outcome in severe Reye syndrome with early pentobarbital coma and hypothermia." 04/01/1982 - "The pharmacokinetics of pentobarbital were examined in 11 children with Reye syndrome, hypoxic encephalopathy, or acute head injury. "
4.	Monoamine Oxidase (MAO)IBA 06/01/1987 - "The results of this study showed that salicylate (1.0 mM) caused an appreciable inhibition of the platelet MAO activity of patients with Reye's syndrome at the onset of the illness. " 06/01/1987 - "The main objective of this investigation was to study the effect of salicylate on platelet mitochondrial monoamine oxidase (MAO) activity isolated from blood of two patients with Reye's syndrome. " 08/01/1985 - "These studies suggest that reduced platelet MAO activity is a specific abnormality in Reye's syndrome, and it may be representative of generalized impairment of mitochondrial function in these patients. " 06/01/1987 - "These preliminary findings suggest that salicylate-induced mitochondrial injury may affect MAO function in children with Reye's syndrome." 06/01/1987 - "Salicylate had only a minimal MAO-inhibitory effect on platelets isolated from blood of recovered Reye's syndrome patients, healthy controls, and non-Reye's hospitalized children, and no apparent effect on enzyme Vmax values. "
5.	Acyl-CoA Dehydrogenase (Medium-Chain Acyl-Coenzyme A Dehydrogenase)IBA 12/01/1990 - "This case report of a patient with medium-chain acyl CoA dehydrogenase (MCAD) deficiency illustrates that electron microscopy may help to differentiate this disorder from Reye syndrome even if a liver biopsy is performed in a patient who recovered from an acute metabolic decompensation. " 09/01/2008 - "The most commonly diagnosed metabolic disorder in association with Reye syndrome has been medium-chain acyl coenzyme A dehydrogenase deficiency. " 01/01/2001 - "The most frequent metabolic cause of Reye-like syndrome is medium chain acyl-CoA dehydrogenase (MCAD) deficiency. " 06/01/1988 - "[Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in 2 patients with symptoms of Reye syndrome]." 03/01/1987 - "Familial Reye-like syndrome: a presentation of medium-chain acyl-coenzyme A dehydrogenase deficiency."
6.	4-pentenoic acidIBA 10/01/1991 - "Histological and biochemical approaches were used to examine the effect of this prostaglandin on hepatocellular damage in an experimental Reye's syndrome model produced in rats by 4-pentenoic acid. " 03/01/1975 - "Production of the features of Reye's syndrome in rats with 4-pentenoic acid." 09/01/1990 - "Microvesicular fatty liver in rats with resembling Reye's syndrome induced by 4-pentenoic acid." 04/15/1984 - "Rats treated with six to eight doses (80 mg/kg, i.p.) of 4-pentenoic acid, an inhibitor of mitochondrial fatty acid oxidation in vitro, during a 48-hr starvation period developed microvesicular fatty infiltration of the liver similar to that observed in Reye's Syndrome. " 09/01/2014 - "This study was designed to investigate whether resveratrol could provide protection against Reye's syndrome induced by 4-pentenoic acid in Wistar albino rats. "
7.	neem oilIBA 08/01/1990 - "These caveats were overcome by evaluation of these four treatment modalities in an established margosa-oil-induced animal model of Reye's syndrome. " 11/01/1989 - "Margosa oil (MO), a long-chain fatty acid compound, has been shown to cause a Reye-like syndrome with death from hepatoencephalopathy, in children in Malaysia and India. " 11/01/1989 - "Animal model of margosa oil ingestion with Reye-like syndrome. " 08/01/1987 - "Margosa oil (MO), a fatty acid-rich extract of the seeds of the neem tree and a reported cause of Reye's syndrome, has been used in the induction of an experimental model of Reye's syndrome in rats. " 08/01/1987 - "Inhibition of mitochondrial functions by margosa oil: possible implications in the pathogenesis of Reye's syndrome."
8.	Glucose (Dextrose)FDA LinkGeneric 11/15/1994 - "Glucose levels should be maintained and electrolyte imbalances and coagulopathies should be corrected in patients with early Reye's syndrome. " 12/13/1984 - "Our findings suggest that the prognosis is excellent for survival without sequelae in Grade I Reye's syndrome (98.8 per cent) when management includes hospital surveillance and intravenous glucose and electrolyte infusion." 03/01/1978 - "Metabolic response to hypertonic glucose administration in Reye syndrome." 05/01/2002 - "Glucose fluid therapy is recommended for Reye syndrome. " 10/01/2009 - "Deficiency of TBP-2 in mice causes hyperlipidemia, hepatic steatosis, hypoglycemia, and bleeding tendency, resembling Reye syndrome in a fasting/glucose-deficient state. "
9.	AntiemeticsIBA 05/01/1991 - "Reye syndrome or side-effects of anti-emetics?" 05/01/1991 - "By analysing two patients initially diagnosed as Reye syndrome evidence is given that in some patients considered as having Reye syndrome, the syndrome is an escalation of symptoms due to viral disease and to unrecognized drug-induced encephalopathy, mainly by anti-emetics. " 09/01/2000 - "Moreover, not only the use of acetylsalicylic acid but also of antiemetics is statistically significant in Reye syndrome cases. " 09/01/2000 - "Moreover, a detailed analysis of the epidemiological surveys of the Centers for Disease Control, the Yale study and of the British risk factor study provides evidence that not only the use of acetylsalicylic acid but also that of phenothiazines and other anti-emetics is significantly greater in Reye syndrome cases than in controls. " 07/01/1984 - "Calcium may reverse the hypotensive effects of prochlorperazine maleate and may help reverse hypotension in patients with Reye's syndrome who have been pretreated with phenothiazine antiemetics."
10.	PhenothiazinesIBA 09/01/2000 - "Moreover, a detailed analysis of the epidemiological surveys of the Centers for Disease Control, the Yale study and of the British risk factor study provides evidence that not only the use of acetylsalicylic acid but also that of phenothiazines and other anti-emetics is significantly greater in Reye syndrome cases than in controls. "

Therapies and Procedures

1.	Critical Care 07/01/1981 - "Reye's syndrome: a challenge not limited to critical care nurses." 06/01/1979 - "Critical care and anesthetic management of Reye's syndrome." 06/01/1979 - "Careful anesthetic and critical-care management, appropriate, reliable monitoring, and pentobarbital therapy may constitute the most successful therapy to date for patients with Reye's syndrome and increased ICP."
2.	Intensive Care (Surgical Intensive Care) 05/01/1986 - "[Reye's syndrome in Spain, 1980-1984 (A cooperative study: Pediatric Intensive Care Section of the Asociación Española de Pediatría)]." 11/01/2010 - "The treatment of Reye syndrome is usually medical with intensive care management. " 12/02/1988 - "During a two-year period, we evaluated four consecutive patients referred for intensive care of Reye's syndrome. " 03/01/1987 - "SEPs were relatively stable during the intensive care observation, with the exception of 6 patients with Reye syndrome. " 07/01/2014 - "Paralytic poliomyelitis, Reye syndrome, Hemophilus Influenzae type B epiglottitis, bacterial meningitis, and meningococcal septic shock are catastrophic illnesses that in the last 60 years have shaped the development of pediatric intensive care. "
3.	Nursing Care 07/07/1977 - "Nursing care study: Reye's syndrome."
4.	Transplants (Transplant) 08/01/1998 - "Microscopic observations disclosed two types of steatotic pattern: (1) A predominantly small-droplet lipid vacuolzation (high-grade microsteatosis), similar to the steatosis associated with Reye syndrome, was seen in 29% of SS sections and 25% of TBS sections--approximately one-fourth of grafts; and (2) a combined pattern of large and small fat drops (high-grade macromicrosteatosis) was seen in 20% of SS sections and 22% of TBS sections. " 12/01/1996 - "The native liver regenerated in three patients: one with Reye's syndrome who died of irreversible neurologic damage, one with FH caused by the hepatitis B virus who is alive 20 months after ABO incompatible graft removal, and one with FH caused by the hepatitis A virus who had her graft removed at 4 months. " 04/01/1979 - "A case is reported of a successful transplant of kidneys from a 15-month-old girl who died of complications of Reye's syndrome. " 01/01/1992 - "Fatal varicella or Reye's syndrome: an unusual cause of death in a renal graft recipient."
5.	Hemoperfusion 11/01/1988 - "[The use of hemosorption in the treatment of Reye's syndrome in a nursing infant]." 08/01/1982 - "[Hemoperfusion through activated charcoal in Reye's syndrome]." 12/01/1978 - "Severe coagulopathy complicating charcoal hemoperfusion in children with Reye syndrome."