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Wednesday, February 23, 2011

A Simple guide to Reye's Syndrome

A Simple Guide to Reye's Syndrome
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What is Reye's Syndrome?
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Reye's Syndrome is an acute hepatitis and metabolic encephalopathy occurring in children.

1.Liver usually shows microvesicular fatty infiltration

2. The brain shows cerebral edema with herniation

Causes are not completely known but:
1.Aspirin and salicylate has been suspected as an aggravating cause

2.A number of fatty acids has been postulated as toxic agents

What are the Signs and Symptoms of Reye's Syndrome
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Symptoms:
1.mild prodromal illness like influenza
Mild cases occur without progression to coma.

2.acute onset with:
a.vomiting
b.delirium
c.lethargy
d.stupor
e.coma within 24-48 hours

Signs:
1.Liver enzymes markedly elevated with normal alkaline phosphatase

2.Increased serum ammonia

3.Raised prothrombin time

4.Low blood glucose especially in younger children

5.Intracranial pressure markedly increased

6.Cerebrospinal fluid normal except for increased protein.

7.Respiratory alkalosis with metabolic alkalosis may occur

Clinical progression with marked cerebral edema occurs with improving liver function.

Outcome depends on reversibility and control of intracranial hypertension

Worse case scenerio will be rapid progression to coma and death.

What are the complications of Reye's Syndrome?
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The complications are:
1.liver damage

2.brain damage

What is the Treatment of Reye's Syndrome?
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Treatment is urgent:
1.Hospitalization with intensive care management

2.Respiratory support

3.Intravenous fluid and glucose to maintain blood glucose

4.Immediate treatment of intracranial pressure important

5.In coma cases, intracranial pressure monitoring with treatment of pressure over 30mm mercury with mannitol, hyper ventilation and removal of CSF if necessary through spinal tap

6. No protein given by mouth because of liver condition

7.Vitamin K as well as fresh blood platelets transfusion to control coagulation problems

Other treatments include:
1.exchange blood transfusion

2.dialysis

3.plasmapheresis

4.Glucose plus insulin

5.citrulline

Prognosis:
Mortality is 50% if inital ammonia is more than 300micrograms per dl and coma present on admission

Outcome depends on management of intra cranial pressure

Subclinal cases are more common than previously thought

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