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Internal Medicine

Reye syndrome

Acute self-limiting metabolic insult leading to a rapidly progressive encephalopathy with hepatic dysfunction.

Acute self-limiting metabolic insult leading to a rapidly progressive encephalopathy with hepatic dysfunction.

  • 1956: Najib Khan first described the fever in Jamshedpur, hence the disease is also called ‘Jamshedpur Fever’
  • 1964: Reye & colleagues described the disease in Australia

Etiology

  • Drugs: Salicylates (eg .Aspirin) (M/C cause)
  • Toxins: Aflatoxins
  • Viral infections: Influenza B, chickenpox, varicella
  • Inborn errors of metabolism: Single enzyme defects of β-oxidation

Pathophysiology

Biochemical pathway:

Generalised mitochondrial dysfunction due to the inhibition of fatty acid β-oxidation

Damage to hepatocyte mitochondria

Liver dysfunction

↑ N2-containing toxins

↑ NH3 in blood

Cross BBB

Target astrocytes
(swelling + oxidative damage)

Encephalopathy
(swelling, inflammation, oedema)

Histopathology:

  • Marked microvesicular steatosis
Histopathology of autopsy liver in Reye's syndrome
Histopathology of autopsy liver from child who died of Reye’s syndrome. Hepatocytes are pale-staining due to intracellular fat droplets | By CDC/ Dr. Edwin P. Ewing, Jr. – This media comes from the Centers for Disease Control and Prevention’s Public Health Image Library (PHIL), with identification number #67.Note: Not all PHIL images are public domain; be sure to check copyright status and credit authors and content providers.English | Slovenščina | +/−, Public Domain, https://commons.wikimedia.org/w/index.php?curid=3523776

Presentation

  • Persistent vomiting
  • Anorexia
  • Personality changes
  • Irregular breathing
  • Confusion
  • Drowsiness
  • Seizures
  • Loss of consciousness
  • Decerebration
  • Coma
  • Hepatomegaly
  • No jaundice
Reye's syndrome clinical staging system
Reye’s syndrome clinical staging system | Lisa A. Degnan, N. (2012). Reye’s Syndrome: A Rare But Serious Pediatric Condition. [online] Uspharmacist.com. Available at: https://www.uspharmacist.com/article/reyes-syndrome-a-rare-but-serious-pediatric-condition [Accessed 7 Jun. 2017].

Diagnosis

Diagnostic criteria:

CDC Diagnostic Criteria of Reye's syndrome
CDC Diagnostic Criteria of Reye’s syndrome | Lisa A. Degnan, N. (2012). Reye’s Syndrome: A Rare But Serious Pediatric Condition. [online] Uspharmacist.com. Available at: https://www.uspharmacist.com/article/reyes-syndrome-a-rare-but-serious-pediatric-condition [Accessed 7 Jun. 2017].
usp1203-reye-t1
Lisa A. Degnan, N. (2012). Reye’s Syndrome: A Rare But Serious Pediatric Condition. [online] Uspharmacist.com. Available at: https://www.uspharmacist.com/article/reyes-syndrome-a-rare-but-serious-pediatric-condition [Accessed 7 Jun. 2017].

Blood panel:

  • ↑ blood NH3
  • ↓ blood sugar
  • ↑ prothrombin time
  • LFT

Differential diagnosis:

 Reye’s SyndromeHepatic Encephalopathy of Fulminant Hepatitis
CLINICAL PRESENTATION
OnsetSuddenGradual
ProdromeURTI or chicken pox infection, biphasic courseFever, anorexia, jaundice
Community EpidemiologyRespiratory disease or chickenpoxFrequently hepatitis or rural water supply or suburban septic tanks
JaundiceNoYes
Liver sizeNormal or considerably enlargedMaybe palpable
Fetor hepaticusAbsentPresent
NeurologicCombative

 

Intermittently agitated

Screaming

Marked decerebrate posturing

No flap

Somnolent

 

Sometimes delirious

Liver flap may be present

Signs of increased intracranial pressurePresent earlyAbsent early; may develop
CourseEncephalopathy runs course in 3-7 daysEncephalopathy persists for days to weeks
Liver biopsyYellow or white; very fattyGreen; not fatty
BIOCHEMICAL ABNORMALITIES
Bilirubin< 3.5> 5
Blood sugarMaybe hypoglycemic; readily correctedMaybe hypoglycaemic; hypoglycaemia may be profound and difficult to correct
SGOT/SGPTElevated, variableElevated, variable
NH3Elevated before treatment;
normalises rapidly with treatment
Levels unpredictable
Blood-clotting studiesProthrombin time prolonged;
easily correctable
Prothrombin time prolonged;
difficult to correct
Disseminated intravascular coagulopathy
(thrombocytopenia)
AbsentMaybe present

Management

  • Manage swelling + oedema: Mannitol + glycerol
  • Insulin: Increase glucose metabolism
  • Corticosteroids: Reduce cerebral edema
  • Diuretics: Reduce fluid and ICP
  • Vitamin K, plasma, platelets: Reduce bleeding
  • Cooling measures: Maintain body temperature

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