Related articles: Reye syndrome
Definition
Reye's syndrome is a rare form of acute encephalopathy associated with hepatic dysfunction. It occurs almost exclusively in children and young people under the age of 18.
In many cases, this condition arises after some acute viral infections (eg influenza A or B, chicken pox, etc.), especially when using salicylates.
Based on this evidence, since the 1980s, the marked decrease in the administration of medicines containing acetylsalicylic acid during childhood has led to a corresponding decrease in the incidence of Reye's syndrome.
The disease affects the mitochondrial function, causing alteration of the metabolism of fatty acids and carnitine.
Most common symptoms and signs *
- Arrhythmia
- Asthenia
- Muscular atrophy and paralysis
- Muscular atrophy
- Increased transaminases
- Coma
- Convulsions
- Difficulty concentrating
- Dehydration
- Temporal and spatial disorientation
- Dyspnoea
- Dysentery
- Mood disorders
- Gastrointestinal hemorrhage
- Hepatomegaly
- Intracranial hypertension
- Hyperventilation
- Hearing loss
- Hypoglycemia
- Hypokalemia
- Hypotension
- Jaundice
- Lethargy
- Mydriasis
- Nausea
- Nervousness
- Memory loss
- Polyuria
- Proteinuria
- Thirst
- Drowsiness
- Confusional state
- Blurred vision
- He retched
Further indications
Reye's syndrome varies greatly in terms of severity, but is characteristically biphasic.
The initial signs associated with infectious diseases (flu, cold or chickenpox) are followed, after about 5-7 days, by uncontrollable nausea and vomiting and sudden deterioration of the mental state. The latter may present with mild amnesia, weakness, visual and auditory changes, intermittent episodes of disorientation, agitation and lethargy.
Reye's syndrome can progress rapidly to a state of coma, manifested by progressive lack of response, flaccidity, signs of decortication and decerebration, seizures, fixed mydriasis and respiratory arrest. Usually, no focal neurological signs are present.
Hepatomegaly due to fatty liver infiltration occurs in about 40% of Reye's syndrome cases, but jaundice is usually absent.
Possible complications include hydro-electrolyte abnormalities (high serum amino acid levels, acid-base balance disorders, hyperammonemia, hypernatremia, hypokalemia and hypophosphataemia), increased intracranial pressure, hypotension, arrhythmias and pancreatitis.
In addition, diabetes insipidus, respiratory failure, inhalation pneumonia and bleeding diathesis (especially gastrointestinal) may occur.
Reye's syndrome can lead to the patient's death.
The diagnosis is based on the history and the finding of typical clinical findings (including increased transaminases, normal bilirubinemia, hyperammonemia and extended prothrombin time) and provides for the exclusion of infectious, toxic and metabolic diseases that occur in a similar manner.
Reye's syndrome must be suspected in any child who presents with an acute onset encephalopathy (without a previous exposure to heavy metals or toxins) and uncontrollable vomiting associated with hepatopathy.
Brain CT or MRI and liver biopsy help confirm diagnostic suspicion.
The treatment is supportive and includes, in particular, measures to reduce intracranial pressure and control glycaemia, as glycogen depletion is frequent.
The outcome depends on the duration of brain dysfunction, the severity and speed of coma progression and the severity of the increase in intracranial pressure. In lethal cases, between admission and death, on average, 4 days elapse.
The prognosis for surviving patients is generally good, however neurological sequelae are possible (eg intellectual retardation, convulsions, cranial nerve paralysis and motor disorders).
