A Family Doctor's Tale - REYE'S SYNDROME

DOC I HAVE REYE'S SYNDROME


Reye's Syndrome is an acute hepatitis and metabolic encephalopathy occurring in children.

1.Liver usually shows micro vesicular fatty infiltration

2. The brain shows cerebral edema with herniation

Causes are not completely known but:

1.Reye's syndrome was found to be caused by the usage of aspirin and salicylate during treatment of chickenpox and influenza.

Since the stoppage of aspirin in children below 12 years old. the illness seems to become very rare.


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



Symptoms:

1.acute onset with:
a.vomiting


b.confusion

c.delirium


d.lethargy


e.seizures

f. unusual position of the arms and legs, the arms are  held straight and turned towards the body , legs are straightened and toes turned down

g.stupor


e.coma within 24-48 hours

2.Other symptoms include:


a.hearing loss

b.speech difficultioes

c.double vision

d.weakness of muscles

e.paralysis of arms and legs

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 intra cranial hypertension

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



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

2.brain damage - seizures and coma


Treatment of Reye's Syndrome:

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 intra cranial pressure important - sometimes corticosteroid is used to reduce swelling in the brain


5.In coma cases, intra cranial 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 fresh blood platelets transfusion to control coagulation problems

Other treatments include:
1.exchange blood transfusion with fresh blood can remove toxic metabolic products from the blood


2.dialysis - works by filtering the toxic materials of the blood through a special filter membrane


3.plasmapheresis - the removal of plasma from the patient , detoxified and returned to the patient


4.Glucose plus insulin can reduce the production of fatty cells in the liver


5.citrulline is a amino acid from food which can detoxify and remove ammonia from the body


Prognosis of Reye's Syndrome:


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

Outcome depends on management of intra cranial pressure

Sub clinical cases are more common than previously thought.

Prevention of Reye's Syndrome:

Avoid the use of aspirin in children below 12 years old.

Other medicines which contains aspirin or salicylates such as wintergreen  oil , chinese medicines should be avoided.