Syndrome of Reye

The syndrome of Reye is a Maladie rare and potentially fatal which involves many harmful effects with various bodies, in particular the Cerveau and the Foie.

The syndrome owes its name with the Dr. R. Douglas Reye, which Co-published in 1963 (with Dr. George Johnson) the first study on the syndrome, but the disease was for the first time described as such in 1929.

Causes

The cause of the syndrome of Reye is unknown; however, one observed a bond between the use of aspirine or others Salicylé S at Enfant S and Adolescent S reached of a Viral disease the such Influenza (influenza), the Varicelle or the Rhume. It is mainly because of this increased risk of syndrome of Reye that the use of the acetylsalicylic acid (aspirine) is disadvised in lower part of age the 18 years.

et al. pointed to the uses off aspirin during year Upper respiratory leaflet infection and Chicken pox ace has potential indicators for the syndrome. With decrease off the uses off aspirin in children during the 1980s resulted in has corresponding decrease in the number off boxes off Reye' S syndrome, dramatically in children under 10 (Arrowsmith et al. 1987). However, it is worth noting that has decrease in the number off boxes has also been observed in countries where children' S aspirin is still in uses. Further puts studies cuts revealed 19 viruses in conjuction with Salicylates, Pesticide S and Aflatoxin ace potential factors contributing to the disease. -->

Symptoms

the symptoms of the syndrome of Reye are mainly Vomissement S, with or without signs of Déshydratation, a Encéphalopathie at a patient Apyrétique with or without ictère, and a Hépatomégalie at 50% of the patients.
Lovejoy initially described 5 clinical stages with the disease, of I with V, Hurwitz modified this classification by adding an asymptomatic stage 0 (stage 0-5). Centers for Disease Control and Prevention ( CDC ) uses the classification of Hurwitz by adding stage 6 to it. Here classification used generally:

Stage 0: phase of alarm, history of the disease and evocative results biological of a syndrome of Reye, not of clinical demonstration;
Stage 1: Vomiting or persistent nauseas, somnolence, lethargy. Children of less than 2 years being able to present only a Diarrhea or a Hyperventilation;
Stage 2: Hyperactivity, irritability, combativeness, confusion, delirium, tachycardia, hyperventilation, mydriase, ostéotendineux reflexes (ROT) sharp, Sign of Babinski positive, answer appropriate to the stimulix noxious;
Stage 3: Coma, hulling, inappropriate answer to the noxious stimuli;
Stage 4: Deep coma, nonréative decerebration, mydriase, loss of the vestibular reflexes oculo;
Stage 5: Flask paralysis, abolition of the BELCH, not of pupillary answer, respiratory stop;
Stage 6: Patients not being able to be classified elsewhere because having received thirst producing curare or products conscience.

Stage I

Vomiting and/or nauseas persistent or continuous, except in the children of less than two years which can present one.
Appearance of signs of dysfunction of the brain: carelessness, loss of energy, lethargy, Giddiness S

Stage II Personality disorder

: irritability and aggressive behavior
Confusion: confusion, irrational behavior and aggressive
Be delirious, convulsions and Coma -->

An early diagnosis is vital, otherwise death or other serious cerebral attacks can arise.

Diagnosis

Encephalopathy (with swelling off the brain). Jaundice present is NOT usually. The liver may become slightly enlarged and firm, and there has exchange in the appearance off the kidneys. (Suchy, Frederick, ED: J Liver Disease in Children. Mosby, St Louis.1994. Chapter 36. -->

The diagnoses criteria used by the CDC are the following:

noninflammatory acute Encephalopathy, with a deterioration of the hepatic conscience
Anomaly with a Steatosis microvésiculaire with the hepatic Puncture Biopsy whose mechanism is imperfectly elucidated. The other hepatic attacks are characterized by a high amoniemy and a light hepatic cytolysis with ALAT and ALAT with three times the normal.
No another etiology found to explain the cerebral edema and the anomaly hepatic
Less than 8 elements per cubic millimetre in the rachidian liquid taken with the lumbar puncture
Biopsy cerebral with noninflammatory cerebral edema

Forecast

the mortality of this disease passed from 50% to less than 20%, thanks to an early diagnosis, with the recognition of the atypical cases and an aggressive treatment.

the death is generally due to the cerebral edema or intracranial hypertension. It can be also caused by a dysfunction myocardic, a cardiovascular collapse, a respiratory distress, an acute impaired renal function, a hemorrhage, an evil epileptic, a sepsis.
the patients who survive can obtain a complete cure, even if neurological after-effects are frequent.

Differential diagnosis

Among the similar causes of symptoms, let us quote:

Encéphalite viral;
epidural Hématome and Hématome under-dural, concept of cranial Traumatism to be sought;
overdose;
Intoxication with the mushrooms (amatoxine);
Intoxication with organophosphorés or carbamates;
hepatic Insufficiency and other hepatitises;
Meningitis;
Impaired renal function

In the children:

Gastroentérite;
metabolic Anomalies;
Meningitis or encéphalite;
Sudden death of the infant

Epidemiology

Drug consumption. Factors other than has viral infection and salicydes are suspected for the disease, while over 90% off patient admitted to Hospital S in the United States with Reye' S Syndrome cuts taken aspirin to treat the symptoms off has viral infection, less than 0.1% off children who cuts had to their viral infections treated with aspirin develop the syndrome. The syndrome also seems to cuts has family Factor, with has greatly increased odds off developing this disease when has family member has had it.

The incidence off death in patients admitted with Reye' S syndrome in the United States fell in the 1980 ' S from over 40% to between 20-30%. However, the incidence off death increased again in the 1990s (52% in 1996). The CDC speculates that this may Be due to decreased interest in the disease. -->

10% of the children reach of this syndrome presented chicken pox in the days which preceded, and in 10% of the cases it was an influenza due to the virus influenzae B.

References

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