 |
| Downloads |
|
This page is intended to provide you with general information about Reye's syndrome and
Reye-like illnesses and a contact number where further information can be obtained.
Click
links below to jump to a section:-
What is Reye’s Syndrome?
Reye’s syndrome is an acute disorder which affects patients apparently recovering from a viral illness like ‘flu’, chicken pox or gastroenteritis. Unless diagnosed and treated successfully, death or severe disability may result.The patient has a change in his/her personality or becomes drowsy or unconscious and develops frequent or persistent effortless vomiting.There is disordered liver function with raised serum hepatic transaminases and hyperammonaemia without hyperbilirubinaemia. There is often hypoglycaemia and disordered clotting.Abnormal accumulations of fat develop in the liver and some other organs of the body. There is also cerebral oedema which may cause permanent brain injury or death if treated inadequately. The disease affects children from infancy to teenage. It also occurs in adults, but it is rarely recognised after 19 years of age. Children of both sexes and all races can be affected.
*What causes Reye's Syndrome?
The cause is unknown; but a number of studies suggest a link between the development of Reye's syndrome in children and the use of aspirin to treat the viral illness which precedes it. These studies do not prove that aspirin causes Reye's syndrome but may aggravate it. Other chemical substances including insecticides and emulsifiers have also been implicated but the evidence that these are involved is controversial. Risk factors for adult RS have not been studied. Because there are alternatives, there is no need to use aspirin for feverish illnesses in childhood.
Since 1986 aspirin preparations have been labeled with differing warnings. The latest advice from the Medicines and Healthcare products Regulatory Agency which came into force on 1st October 2003 requires all aspirin products to carry a warning that children under 16 should not take aspirin unless on the advice of a doctor. The warning to appear on the Carton/Label and Patient Information Leaflet is as follows:-
Carton/Label
DO NOT GIVE TO CHILDREN UNDER 16 YEARS, UNLESS ON THE ADVICE OF A DOCTOR |
Patient Information Leaflet
THERE IS A POSSIBLE ASSOCIATION BETWEEN ASPIRIN AND REYE'S
SYNDROME WHEN GIVEN TO CHILDREN. REYE'S SYNDROME IS A VERY RARE DISEASE WHICH CAN BE FATAL. FOR THIS REASON ASPIRIN SHOULD NOT BE GIVEN TO CHILDREN AGED UNDER 16 YEARS UNLESS ON THE ADVICE OF A DOCTOR |
* What are Reye-Like Illnesses?
Many rare inherited metabolic disorders, for example fatty acid oxidation defects and urea cycle disorders, are now known to cause an illness exactly like Reye's syndrome. The early treatment of these "Reye-like illnesses" is similar to that of Reye's syndrome but some require special drugs or diets for treatment and to prevent recurrence of illness. Special laboratory tests are required to make certain that such disorders are excluded in all children in whom a diagnosis of Reye's syndrome is considered. Furthermore other children in the family may also have the genetic disorder in a latent form. These disorders may also rarely manifest for the first time in teenagers or older adults (Reye Syndrome-like Disorders which can simulate RS).
What are the early stages of Reye's Syndrome?
Persistent or continuous vomiting and/or:Signs of Cerebral Oedema:
- stlessness
- Loss of energy
- Drowsiness
- Irritability
- Aggressive behaviour
- Disorientation
- Confusion
- Irrational behaviour
- Delirium, Convulsions
Reye's syndrome should be suspected if a patient displays all or part of this pattern of symptoms a few days after the onset of viral illness such as 'flu' or ‘chickenpox’. Fever is not usually present. Many diseases have similar features. Medical staff who do not have experience of Reye's syndrome may initially suspect encephalitis, meningitis, poisoning, mental illness or even drug abuse. In infants the symptoms of Reye's syndrome may not follow a typical pattern; for example vomiting is less common. However it should be emphasized that it is in infants that the "Reye-like illnesses” are most likely to occur.
*What should be done if symptoms of Reye's Syndrome develop?
Advice to general practitioners
A child's life may depend on obtaining emergency treatment in the nearest children's unit.
Advice to pediatricians, physicians and A&E doctors Statistics indicate a better chance of survival when Reye's syndrome is diagnosed and treated in its earliest stages. The later the diagnosis the more advanced the coma and the chances of survival and full recovery are greatly reduced. It is also essential to investigate all patients with unexplained encephalopathy and hyper- ammonaemia for inherited metabolic disorders.When brain dysfunction is so severe that the patient is drowsy and irritable, he or she will require the services of an intensive care unit for the specialist management required. This will be directed at correcting metabolic abnormalities and protecting the brain against irreversible damage caused by cerebral oedema. For further information about Reye's syndrome and Reye-Iike illnesses:- the 'doctor on call', Dept. Chemical Pathology, Sheffield Children's Hospital is willing to advise on further investigations and can provide contact names for further advice on clinical management. Tel: 0114 271 7000.Before treatment is started (which should be as soon as possible) blood should be taken immediately for liver function tests, prothrombin time, blood ammonia, blood sugar and for investigation of Reye-like inherited metabolic disorders. Any urine passed should also be saved for investigation of these disorders (Clinical Chemistry should be consulted).
Glucose infusions may stop some of the harmful effects of Reye's syndrome if they are given early enough. This should start immediately, even before the initial results are available. Reye's syndrome can successfully be managed with a good outcome if diagnosed early and meticulously treated.
* Are there lasting effects'?
Survival is related to the severity of the brain swelling. Some patients recover completely. Others may sustain slight to severe permanent brain damage. Patients surviving Reye's syndrome require follow up, with developmental and neurological assessment. (After Reye’s Syndrome)
*Is Reye's Syndrome a common disease'?
Reye's syndrome was first described in 1963 by an Australian pathologist, R. Douglas Reye, M.D. Since then it has been recognised in many parts of the world. It used to occur in minor epidemics, which in some countries (most notably the USA) were associated with influenza epidemics. Although the exact incidence of the disease in the UK and Ireland is not known, reported cases showed a dramatic fall from nearly a hundred in 1984 to only one in 2002.
It is likely that there is some under-reporting and under-diagnosis, but this downward trend has also been seen in the USA and is attributed to reduction in the use of aspirin in children since the public warnings in 1986. Nevertheless, there may be a resurgence, especially if there is a large 'flu' epidemic or the aspirin warning is ignored as time goes by. It is thus essential that clinicians retain high diagnostic awareness of Reye's syndrome and Reye-like illnesses.
Remember
- Reye’s syndrome can appear soon after a viral infection, especially a flu-like illness or chickenpox.
- Early signs of Reye's syndrome are continuous vomiting, listlessness, drowsiness, irritability, confusion, irrational behaviour or a convulsion.
- In patients with the signs and symptoms described above, abnormal liver function tests, including raised blood ammonia strongly suggest a diagnosis of Reye's syndrome or a “Reye-Iike” inherited metabolic disorder.
- Inherited metabolic disorders can present with a "Reye-like" illness especially in children under 4 years. It is essential to diagnose them because of implications for genetic counseling, specific treatment and investigation of other children in the family.
- Medicines can mask symptoms. Therefore a patient developing any of the symptoms of Reye's syndrome, should not be given anti-nausea or fever reducing medicine.
- Children and teenagers under 16 should not take or be given aspirin unless clinically indicated, for example as specific anti- inflammatory or anti-platelet therapy.
- Reye's syndrome can rarely occur in adults.
- Early diagnosis and appropriate treatment is vital.
|
