Reye’s syndrome

Definition:
Reye’s syndrome is a potentially fatal disease that causes numerous detrimental effects to many organs, especially the brain and liver, as well as causing a lower than usual level of blood sugar (hypoglycemia).. Reye’s syndrome most often affects children and teenagers recovering from a viral infection and who may also have a metabolic disorder. The exact cause is unknown, and while it has been associated with aspirin consumption by children with viral illness, it also occurs in the absence of aspirin use.
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The disease causes fatty liver with minimal inflammation and severe encephalopathy (with swelling of the brain). The liver may become slightly enlarged and firm, and there is a change in the appearance of the kidneys. Jaundice is not usually present.

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Early diagnosis is vital; while most children recover with supportive therapy, severe brain injury or death are potential complications.

Boys and girls can both be affected, but the condition is very rare – there were only three reported cases in the UK and Ireland in 2000.

The syndrome is named after Dr. R. Douglas Reye, who, along with fellow physicians Dr. Graeme Morgan and Dr. Jim Baral, published the first study of the syndrome in 1963 in The Lancet. In retrospect, the occurrence of the syndrome may have first been reported in 1929.

Symptoms :
Reye’s syndrome progresses through five stages, explained below:

*Stage I
…#Rash on palms of hands and feet
…#Persistent, heavy vomiting that is not relieved by not eating
…#Generalized lethargy
…#Confusion
…#Nightmares
…#High fever
…#Headaches

*Stage II
…#Stupor caused by encephalitis
…#Hyperventilation
…#Fatty liver (found by biopsy)
…#Hyperactive reflexes

*Stage III
…#Continuation of Stage I and II symptoms
…#Possible coma
…#Possible cerebral oedema
…#Rarely, respiratory arrest

*Stage IV
…#Deepening coma
…#Dilated pupils with minimal response to light
…#Minimal but still present hepatic dysfunction

*Stage V
…#Very rapid onset following stage IV
…#Deep coma
…#Seizures
…#Multiple organ failure
…#Flaccidity
…#Hyperammonemia (above 300 mg/dL of blood)
…#Death

Causes:
The cause of Reye’s syndrome isn’t fully understood. Reye’s syndrome seems to be triggered by using aspirin to treat a viral illness or infection — particularly flu (influenza) and chickenpox — in children and teenagers who have an underlying fatty acid oxidation disorder. Fatty acid oxidation disorders are a group of inherited metabolic disorders in which the body is unable to breakdown fatty acids because an enzyme is missing or not working properly. A screening test is needed to determine if your child has a fatty acid oxidation disorder.

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In some cases, Reye’s syndrome may be an underlying metabolic condition that’s unmasked by a viral illness. Exposure to certain toxins — such as insecticides, herbicides and paint thinner — also may contribute to Reye’s syndrome.

Risk Factors:
The following factors — usually when they occur together — may increase your child’s risk of developing Reye’s syndrome:

Using aspirin to treat a viral infection, such as flu, chickenpox or an upper respiratory infection
Having an underlying fatty acid oxidation disorder

Complications:
Most children and teenagers who have Reye’s syndrome survive, although varying degrees of permanent brain damage are possible. Without proper diagnosis and treatment, Reye’s syndrome can be fatal within a few days.

Diagnosis:
Exams and TestsThe following tests may be used to diagnose Reye syndrome:

•Blood chemistry tests
•Head CT or head MRI scan
•Liver biopsy
•Liver function tests
•Serum ammonia test
•Spinal tap

Treatment:
Immediate emergency treatment is needed for Reye’s syndrome, usually in an intensive care unit.

There is no specific treatment for this condition. The health care provider will monitor the pressure in the brain, blood gases, and blood acid-base balance (pH).

Treatments may include:

•Breathing support (a breathing machine may be needed during a deep coma)
•Fluids by IV to provide electrolytes and glucose
•Steroids to reduce swelling in the brain

Prognosis :
Documented cases of Reye’s syndrome in adults are rare. The recovery of adults with the syndrome is generally complete, with liver and brain function returning to normal within two weeks of the illness. In children, however, mild to severe permanent brain damage is possible, especially in infants. Over thirty percent of the cases reported in the United States from 1981 through 1997 resulted in fatality.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/reyes1.shtml
http://en.wikipedia.org/wiki/Reye’s_syndrome
http://www.mayoclinic.com/health/reyes-syndrome/DS00142
http://www.nlm.nih.gov/medlineplus/ency/article/001565.htm
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