Rye syndrome is a rare but very serious disease. Rye can cause inflammation of multiple organs. The brain and liver are often affected. The disease often starts with vomiting and drowsiness. Rye mainly occurs in children, but can also affect adults. The disease causes an enlarged, inflamed liver and inflamed brain. There is a danger that the brain will swell. The kidneys can also function less well. Jaundice usually does not occur. It is important that the diagnosis is made in time to increase the chance of survival.
Symptoms
Rye’s disease can progress very quickly. The complaints often start very quickly. The main features in the first stage are:
- Yield
- Drowsiness/Drowsiness
- Convulsions
- High fever
- Headache
Stage two :
- Inflammation of the brain
- Hyperventilation
- Enlarged liver
- Hyperactive reflexes
Stage three
Symptoms from stages 1 and 2 persist. In addition, the following may occur:
- Possible coma
- Buildup of fluid in limbs
Stage four
- Deep coma
- Liver only works minimally
Stage five
- Deep coma
- Organ failure
- Flabby muscles
- Ultimately death
Cause
The cause of Rye syndrome is still unknown. There may be a connection with a viral infection such as chickenpox. The disease often occurs after a viral infection. Research has shown that taking aspirin is a risk factor for developing Rye’s syndrome. That is why aspirin is not often prescribed to children and teenagers. Coming into contact with insecticides can also possibly lead to Rye’s syndrome.
Diagnosis
It is important that Rye’s syndrome is detected in time. If the patient has just had a viral infection, one should be extra alert for Rye’s syndrome. If suspected, immediate hospitalization and various examinations follow. Blood tests can be used to examine the functioning of the liver. Brain activity can be examined via an EEG. The sick person is given a kind of swimming cap with which the brain waves can be measured. An MRI or CT scan may be done to rule out whether another cause is at play, such as a brain tumor. During a liver puncture, tissue is removed from the liver to be examined under a microscope.
Therapy
There are no medications to cure Rye syndrome. However, the patient can be supported. The sick person is often put on a ventilator. The patient will have to heal on its own. Patients who do not end up in a coma have the greatest chance of overcoming Rye syndrome. However, medications can be given to prevent the brain from swelling and to promote the drainage of accumulating fluid.
Prognosis
In recent years, the prognosis for patients with Rye syndrome has increased. Of the children with Rye, 80 percent now survive the disease. The sooner the syndrome is discovered, the better the chance of survival. Patients who survive the penultimate or final stage of Rye often are left with permanent brain damage, disabilities and developmental problems such as speech and learning difficulties.