Reyes Syndrome is a serious condition, and potentially fatal. It’s
mostly taking place among children, affects all body organs (especially
the brain and the liver), and causing various diseases, including
Hypoglycemia (low levels of glucose in the blood).
It’s been related with the consumption of Aspirin, but many patients
suffer from the disease where there is no Aspirin involved. This is why
the exact cause of the Reyes Syndrome is still unknown.
The Fatty Liver connection
Reyes
Syndrome is split into different stages, with different symptoms. At
stage 2 of the disease, one of its common symptoms is a non-alcoholic
fatty liver disease. The is simply because the fact that the liver is
one of the most affected organs from this disease. Fatty liver disease
can be diagnosed only by a liver ultrasound and/or a liver biopsy.
Early diagnosis is crucial, as serious brain injury or even death are possible results of the disease.
Reye's syndrome
An acute childhood illness, Reye’s
syndrome causes fatty infiltration of the liver with concurrent
hyperammonemia, encephalopathy, and increased intracranial pressure
(ICP). In addition, fatty infiltration of the kidneys, brain, and
myocardium may occur.
Reye’s syndrome affects children. It’s most common in patients ages 4 to 12, with a peak incidence at age 6.
The prognosis depends on the severity of central nervous system
depression. Previously, mortality was as high as 90%. Today, ICP
monitoring and, consequently, early treatment of increased ICP, along
with other treatment measures, have cut mortality to about 20%. Death
is usually a result of cerebral edema or respiratory arrest. Comatose
patients who survive may have residual brain damage.
Causes
Incidence of Reye’s syndrome usually rises during influenza
outbreaks and is linked to aspirin use. It almost always follows within 1
to 3 days of an acute viral infection, such as an upper respiratory
tract infection, type B influenza, or varicella (chickenpox).
With Reye’s syndrome, damaged hepatic mitochondria disrupt the urea
cycle, which normally changes ammonia to urea for its excretion from
the body. This results in hyperammonemia, hypoglycemia, and an increase
in serum short-chain fatty acids, leading to encephalopathy.
Simultaneously, fatty infiltration is found in renal tubular cells,
neuronal tissue, and muscle tissue, including the heart.
Signs and symptoms
Reye’s syndrome develops in five stages, but the severity of the
child’s signs and symptoms varies with the degree of encephalopathy and cerebral edema. Infants may have atypical presentation.
After the initial viral infection, a brief recovery period follows
when the child doesn’t seem seriously ill. A few days later, he
develops intractable vomiting, lethargy, rapidly changing mental status
(mild to severe agitation, confusion, irritability, delirium),
hyperactive reflexes, and rising blood pressure, respiratory rate, and
pulse rate.
Reye’s syndrome may progress to coma. As the coma deepens, seizures
develop, followed by decreased tendon reflexes and, commonly,
respiratory failure.
Increased ICP, a serious complication, results from cerebral edema.
Such edema may develop as a result of acidosis, increased cerebral
metabolic rate, or an impaired autoregulatory mechanism.
Diagnosis
Early diagnosis and treatment improves chances of recovery. A
history of a recent viral disorder with typical signs and symptoms
strongly suggests Reye’s syndrome. An increased serum ammonia level,
abnormal clotting studies, and hepatic dysfunction confirm it.
Testing the serum salicylate level rules out aspirin overdose.
Absence of jaundice, despite increased liver transaminase levels, rules
out acute hepatic failure and hepatic encephalopathy.
Abnormal test results may include the following:
-
Liver-function studies show aspartate aminotransferase and alanine aminotransferase levels elevated to twice normal; bilirubin level is usually normal.
-
Liver biopsy reveals fatty droplets uniformly distributed throughout cells.
-
Cerebrospinal fluid (CSF) analysis reveals a white blood cell count of less than 10; with coma, CSF pressure increases.
-
Coagulation studies result in prolonged prothrombin and partial thromboplastin times.
-
Blood values show elevated serum ammonia levels; normal or, in 15% of cases, low serum glucose levels; and increased serum fatty acid and lactate levels.
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