Reye’s syndrome is a very rare pediatric disease that causes encephalopathy and liver dysfunction. Causes, symptoms, diagnosis, treatment… We take stock.
Definition: what is Reye’s syndrome?
Reye’s syndrome refers to a extremely rare and life-threatening pediatric disease, which affects several organs, in particular the brain (in the form of encephalitis) and the liver (in the form of steatosis or fatty infiltration). Its origin is not known but a link with taking aspirin is mentioned, therefore aspirin is contraindicated in children in many countries, eg the United States and Great Britain.
What is the difference between Reye’s syndrome and chickenpox?
Reye’s syndrome associates a dliver failure with hyperammonemia and non-infectious encephalopathy. Its origin is not known but it occurs classically after a cured or recovering viral illness. Chickenpox is a very common viral infectious disease and extremely contagious due to the varicella virus, which manifests itself by skin rash with pimples and severe itching. It can very rarely be complicated by varicella meningoencephalitis, most often during reactivation of the virus or reinfection.
What is the cause of Reye’s syndrome?
The etiology of Reye’s syndrome remains unknown. The syndrome usually begins within 2-5 days of recovering from a viral illness (flu, chickenpox) in which aspirin was used. The hypothesis is that it would be a rimpaired immune reaction in response to a viral disease, with a possible genetic predisposition of the host and a favoring role of aspirin. Whether or not aspirin plays a role in the development of this pathology is still the subject of much debate. A causal relationship between taking aspirin and the development of Reye’s syndrome has never been established, but is strongly suggested. by several epidemiological studies. “The vast majority of children who developed this disease had taken aspirin in the previous days, and this syndrome has almost disappeared since the recommendations to no longer prescribe aspirin in children. Some refute the role of aspirin by arguing the poor quality of the studies, the frequent absence of detectable aspirin in the blood of children who have presented this syndrome. In my opinion, the causal relationship can probably never be established in the future due to the rarity of the disease“, develops Dr. Frédérique Rodieux.
Is it possible in adults?
Reye syndrome is a disease overwhelmingly pediatric which mainly affects children between 1 and 14 years old. It is non-existent beyond the age of 18, even if a few cases are described.
What are the symptoms of Reye’s syndrome?
Reye’s syndrome is manifested by a encephalopathy associated with steatosis and liver dysfunction which can lead to general organ dysfunction. It most often appears within three to five days of a viral illness such as the flu or chickenpox. “Reye’s syndrome initially manifests as nausea, vomiting and impaired consciousness such as drowsiness and confusion and may progress to seizures, coma and potentially death“, explains the head of clinic at the Department of Clinical Pharmacology & Toxicology of the University Hospitals of Geneva.
Reye’s syndrome is difficult to diagnose because its symptoms are not very specific. “It is essentially based on clinical signs such as severe vomiting and changes in the child’s mental status suggestive of brain dysfunction (confusion, drowsiness, delirium, stupor), blood tests (including a search for hyperammonemia) and cerebrospinal fluid by lumbar puncture as well as imaging exams. A liver biopsy is sometimes necessary“, continues the specialist. It is important to exclude infectious causes of liver damage and encephalitis as well as metabolic diseases. Reye’s syndrome is thus often a diagnosis of exclusion.
Aspirin should not be given to children
What is the treatment for Reye’s syndrome?
There is no cure for Reye’s syndrome. Treatment is symptomatic and not specific. It is based on so-called supportive care, i.e. sugar infusion for hypoglycaemia, correction of hyperammonemia and possible coagulation disorders with vitamin K and/or blood products, artificial ventilation in case of severe respiratory disorders, control and treatment of possible intracranial hypertension. Reye’s syndrome most often requires a hospitalization of the child in an intensive care unit.
“Prevention is essential. With very few exceptions, aspirin should not be given to children. There are safer alternatives for the treatment of fever and pain in children. Note that many drugs that can be obtained without a prescription contain aspirin or derivatives (salicylates). He is essential to consult the doctor or pharmacist before administering any medicationwhoever it is, to a child“recalls the specialist.
Thank you to Dr Frédérique Rodieux, head physician at the Clinical Pharmacology & Toxicology Department of the University Hospitals of Geneva.