Reye’s Syndrome

Reye’s syndrome also known as Reye-Johnson syndrome, although it is a rare but a serious condition which is more common in children with less then of 10 year age. It’s symptoms may include uncontrolled vomiting, confusion, convulsions, altered consciousness (even loss of consciousness in serious cases), fatty liver, fatigue, etc.

This conditions occurs in children who have been gone through viral infection like chicken pox, etc. and the guardian’s / doctor gives aspirin to the child , this combination of viral infection and aspirin may lead to damage of mitochondria of liver which further causes-

• Disturbed liver metabolism – The toxin formed by virus along with aspirin causes damage to mitochondria of liver due to which ATP formation by liver becomes disturbed and the liver cells fails to protect themselves from the chemical toxins which causes liver injury.

• Fatty Liver – Normally liver uses fatty acids and makes ATP for its use but due to liver injury fatty acids are not being used up by the liver which leads to deposition of fat in the liver cells.

• Ballooning of liver – Basically ballooning of liver refers to special form of liver cell degeneration which is associated with cell swelling and enlargement.

• Increase in conjugated bilirubin – Due to injury in liver cells, conjugated bilirubin couldn’t passed / excreted out by liver so there is a minor increase in the level of conjugated bilirubin.

• Disturbed urea cycle – Normally in liver ammonia is converted to urea with the use of ATP, but due to liver injury in reye’s syndrome ammonia can’t be completely converted into urea which causes increase in amount of ammonia in blood.

• Neural injury – This increased ammonia level in blood causes neural injury and edema in brain, due to which child may suffers from intractable vomiting, seizures, confusion, altered consciousness, coma and may even lead to death.

The main cause behind occurance of reye’s syndrome is not known, also it has a 40% of mortality rate but it’s frequency is less than one in a million of children per year

Investigations and Findings –

• Serum Cb – minor increase in levels.

• High blood ammonia level

• Low blood sugar level

• On USG hepatomegaly is seen

• On performing liver biopsy and microscopic examination, fatty liver, ballooning of liver, necrosis in liver could be seen.

•MRI brain gives the evidence of brain edema

Signs and symptoms –

Abnormal behaviour,confusion, seizures, change in personality, fatigue, altered consciousness, uncontrolled vomiting and other signs related to liver and brain conditions.

How to prevent ??

The best way to prevent Reye’s syndrome is to avoid use of Aspirin in children with viral infections, because the use of Aspirin is the key cause of reye’s syndrome.

Complications –

Sometime reye’s syndrome may lead to complications like , poor memory retention, difficulty in speech and language, dysphagia i.e difficulty in swallowing, confused or forgotten daily routine activites, difficulty with normal posture and movements.

Treatment –

Use mannitol to treat cerebral edema and give supportive treatment for liver injury.

Prognosis –

Generally in adults there is a complete recovery seen with no permanent damage or injury to liver or brain.But in children , especially in infants there may be permanent damage to liver or brain functions. Also 30- 40 % may die due to reye’s syndrome.

None of the drug listed above should be taken without proper consultation of the doctor.This post is just for educational purpose and not for any clinical purpose.