Σύνδρομο Reye

Το σύνδρομο Reye αποτελεί μια πάθηση που μπορεί να οδηγήσει στο θάνατο. Το σύνδρομο Reye εμφανίζεται αιφνιδίως και μπορεί να προσβάλει οποιοδήποτε παιδί, έφηβο ή ενήλικο άτομο χωρίς προειδοποίηση. Επηρεάζει όλα τα όργανα του ανθρώπινου σώματος, ενώ σοβαρότερα από όλα πλήττονται το ήπαρ και ο εγκέφαλος.

Παρότι τα αίτια και η θεραπεία του συνδρόμου Reye εξακολουθούν να παραμένουν άγνωστα, έρευνες έχουν δείξει ότι η εμφάνιση του συνδρόμου Reye σχετίζεται με τη χρήση ασπιρίνης, άλλης φαρμακευτικής ουσίας, μη-συνταγογραφούμενου φαρμάκου ή προϊόντος τοπικής χρήσης που περιέχει σαλικυλικό.

Το 1974, ιδρύθηκε στην Αμερική το Εθνικό Ίδρυμα για το Σύνδρομο Reye, το οποίο ήταν και η πρώτη ομάδα πολιτών που κατάφερε να συντονίσει και να οργανώσει ένα ολόκληρο κίνημα για την πρόληψη του συνδρόμου Reye.

Τι είναι το σύνδρομο Reye;

Το σύνδρομο Ρέι είναι μια ασθένεια που προσβάλει όλα τα όργανα του σώματος αλλά βλάπτει ιδιαίτερα το ήπαρ και τον εγκέφαλο. Το σύνδρομο  Reye είναι μια ασθένεια που εκδηλώνεται σε δύο στάδια καθώς σχεδόν πάντα σχετίζεται με την ύπαρξη προηγούμενης μόλυνσης, όπως γρίπη, κρύωμα ή ανεμοβλογιά. Αυτό που είναι γνωστό από τα μέχρι τώρα επιστημονικά στοιχεία είναι ότι το σύνδρομο Reye δεν είναι μεταδοτικό και τα αίτια εμφάνισής του παραμένουν άγνωστα. Πολύ συχνά το σύνδρομο Reye παρερμηνεύεται ως εγκεφαλίτιδα, μηνιγγίτιδα, διαβήτης, υπερβολική δόση ναρκωτικών, δηλητηρίαση, Σύνδρομο Αιφνίδιου Θανάτου Βρεφών, ή ψυχιατρική διαταραχή.

Το σύνδρομο Reye και η γρίπη

Το σύνδρομο Reye εμφανίζεται συχνότερα κατά τους μήνες Ιανουάριο, Φεβρουάριο και Μάρτιο όταν είναι πιο συχνή και η εμφάνιση γρίπης. Ωστόσο, περιστατικά αναφέρονται κάθε μήνα του έτους. Η εμφάνιση επιδημίας γρίπης ή ανεμοβλογιάς συνήθως ακολουθείται από αύξηση των περιστατικών συνδρόμου Reye.

Το σύνδρομο Reye συνήθως εκδηλώνεται όταν το άτομο βρίσκεται στο στάδιο ανάρρωσης από κάποια ιογενή νόσο. Τότε αρχίζουν και συγκεντρώνονται υπερβολικές ποσότητες λίπους στο ήπαρ και σε άλλα όργανα του σώματος, ενώ ταυτόχρονα αυξάνει η πίεση στον εγκέφαλο. Εάν δεν διαγνωστεί και αντιμετωπιστεί με επιτυχία, συνήθως οδηγεί στο θάνατο μέσα στα επόμενα εικοσιτετράωρα. Η ζωή του ασθενούς εξαρτάται από την έγκαιρη διάγνωση. Ωστόσο, τα στατιστικά στοιχεία δείχνουν ότι το σύνδρομο Reye έχει εξαιρετικά ποσοστά ίασης εάν διαγνωστεί και αντιμετωπιστεί στα πρώτα στάδια εμφάνισής του. Όσο πιο πολύ καθυστερήσει η διάγνωση και η θεραπεία, τόσο λιγότερες είναι οι πιθανότητες για επιτυχή ανάρρωση και επιβίωση του ασθενούς.

Στάδια του Συνδρόμου Reye:

Στάδιο I:
Συνεχιζόμενοι και έντονοι εμετοί
Ενδείξεις εγκεφαλικής δυσλειτουργίας:
Κατάπτωση και αδιαφορία
Απώλεια ζωντάνιας και ενεργητικότητας
Ληθαργικότητα
Στάδιο II:
Αλλαγές στη συμπεριφορά/προσωπικότητα του ατόμου:
Ευερεθιστότητα
Επιθετική συμπεριφορά
Αποπροσανατολισμός:
Σύγχυση
Παράλογη συμπεριφορά
Εριστική συμπεριφορά
Παραλήρημα, σπασμοί, κώμα
Θα πρέπει κανείς να υποπτευθεί την ύπαρξη συνδρόμου Reye εάν κάποιο άτομο νοσεί ή μόλις ανάρρωσε από κάποια ιογενή νόσο και εμφανίσει τα παραπάνω συμπτώματα. Δεν είναι απαραίτητο να εμφανιστούν όλα τα συμπτώματα, ούτε είναι απαραίτητο να εκδηλωθούν με αυτή τη σειρά. Συνήθως δεν υπάρχει πυρετός. Πολλές ασθένειες έχουν κοινά συμπτώματα. Οι γιατροί ή το ιατρικό προσωπικό που εργάζονται στα επείγοντα περιστατικά και δεν έχουν εμπειρία στην αντιμετώπιση του συνδρόμου Reye, μπορεί να μην είναι σε θέση να διαγνώσουν την ασθένεια.

Τα συμπτώματα του συνδρόμου Reye σε βρέφη μπορεί να μην είναι ακριβώς όπως και των μεγαλύτερων σε ηλικία ατόμων. Για παράδειγμα, ο εμετός μπορεί να αντικατασταθεί από διάρροια, ενώ επίσης τα βρέφη μπορεί να εμφανίσουν ακανόνιστη αναπνοή.

Πηγή: www.reyessyndrome.org/

Reye's syndrome
Classification and external resources

Reye's syndrome is a potentially fatal disease that causes numerous detrimental effects to many organs, especially the brain and liver, as well as causing a lower than usual level of blood sugar (hypoglycemia).[1] The classic features are liver damage, aspirin use and a viral infection. The exact cause is unknown, and while it has been associated with aspirin consumption by children with viral illness, it also occurs in the absence of aspirin use.
The disease causes fatty liver with minimal inflammation and severe encephalopathy (with swelling of the brain). The liver may become slightly enlarged and firm, and there is a change in the appearance of the kidneys. Jaundice is not usually present.[2]
Early diagnosis is vital; while most children recover with supportive therapy, severe brain injury or death are potential complications.
Contents  ] 
1 Symptoms and signs
2 Causes
2.1 Aspirin
3 Prognosis
4 Differential diagnosis
5 Epidemiology
6 History
7 References
8 External links
[edit]Symptoms and signs

Reye's syndrome progresses through five stages, explained below:
Stage I
Rash on palms of hands and feet
Persistent, heavy vomiting that is not relieved by not eating
Generalized lethargy
Confusion
Nightmares
High fever
Headaches
Stage II
Stupor caused by encephalitis
Hyperventilation
Fatty liver (found by biopsy)
Hyperactive reflexes
Stage III
Continuation of Stage I and II symptoms
Possible coma
Possible cerebral oedema
Rarely, respiratory arrest
Stage IV
Deepening coma
Dilated pupils with minimal response to light
Minimal but still present hepatic dysfunction
Stage V
Very rapid onset following stage IV
Deep coma
Seizures
Multiple organ failure[3]
Flaccidity
Hyperammonemia (above 300 mg/dL of blood)
Death
]Causes

The precise mechanism by which Reye's syndrome occurs remains unknown. This serious condition is described as a "syndrome" rather than a disease as the clinical features that physicians use to diagnose it are quite broad.
]Aspirin
Some studies have demonstrated an association between aspirin taken for viral illnesses and the development of Reye’s syndrome.[4] One small study presented findings that acetaminophen (paracetamol) is a greater risk,[5] but this claim is disputed.[6]
The serious symptoms of Reye's syndrome appear to result from damage to cellular mitochondria,[7] at least in the liver, and there are a number of ways that aspirin could cause or exacerbate mitochondrial damage. A potential increased risk of developing Reye's syndrome is one of the main reasons that aspirin has not been recommended for use in children and teenagers, the age group for which the risk of lasting serious effects is highest. No research has found a definitive cause of Reye's syndrome, and association with aspirin has only been shown through epidemological studies. The diagnosis of "Reye's Syndrome" greatly decreased in the 1980s, when genetic testing for inborn errors of metabolism was becoming available in developed countries.[8] A retrospective study of 49 survivors of cases diagnosed as "Reye's Syndrome" showed that the majority of the surviving patients had various metabolic disorders.[9]
In some countries, oral mouthcare product Bonjela (not the form specifically designed for teething) has labeling cautioning against its use in children, given its salicylate content. There have been no cases of Reye's secondary to its use, and the measure is a precaution.[10] Other medications containing salicylates are often similarly labeled as a precaution.
The Centers for Disease Control and Prevention (CDC), the U.S. Surgeon General, the American Academy of Pediatrics (AAP) and the Food and Drug Administration (FDA) recommend that aspirin and combination products containing aspirin not be given to children under 19 years of age during episodes of fever-causing illnesses. Hence, in the United States, it is advised that the opinion of a doctor or pharmacist be obtained before anyone under 19 years of age is given any medication containing aspirin (also known on some medicine labels as acetylsalicylate, salicylate, acetylsalicylic acid, ASA or salicylic acid).
Current advice in the United Kingdom by the Committee on Safety of Medicines is that aspirin should not be given to those under the age of 16 years, unless specifically indicated in Kawasaki disease or in the prevention of blood clot formation.[11]
]Prognosis

Documented cases of Reye’s syndrome in adults are rare. The recovery of adults with the syndrome is generally complete, with liver and brain function returning to normal within two weeks of the illness. In children, however, mild to severe permanent brain damage is possible, especially in infants. Over thirty percent of the cases reported in the United States from 1981 through 1997 resulted in fatality.
[edit]Differential diagnosis

Causes for similar symptoms include
Various inborn metabolic disorders
Viral encephalitis
Drug overdose or poisoning
Head trauma
Hepatic failure due to other causes
Meningitis
Renal failure
]Epidemiology

Reye’s syndrome occurs almost exclusively in children. While a few adult cases have been reported over the years, these cases do not typically show permanent neural or liver damage. Unlike in the UK, the surveillance for Reye’s syndrome in the US is focused on patients under 18 years of age.
In 1980, after CDC began cautioning physicians and parents about the association between Reye’s syndrome and the use of salicylates in children with chickenpox or viruslike illnesses, the incidence of Reye's syndrome in the United States began to decline. In the United States between 1980 and 1997, the number of reported cases of Reye’s syndrome decreased from 555 cases in 1980 to about 2 cases per year since 1994. During this time period 93% of reported cases for which racial data were available occurred in whites and the median age was six years. In 93% of cases a viral illness had occurred in the preceding three week period. For the period 1991-1994, the annual rate of hospitalizations due to Reye’s syndrome in the US was estimated to be between 0.2 and 1.1 per million population less than 18 years of age.
During the 1980s, a case-control study carried out in the United Kingdom also demonstrated an association between Reye’s syndrome and aspirin exposure.[12] In June 1986, the United Kingdom Committee on Safety of Medicines issued warnings against the use of aspirin in children under 12 years of age and warning labels on aspirin-containing medications were introduced. UK surveillance for Reye’s syndrome documented a decline in the incidence of the illness after 1986. The reported incidence rate of Reye’s syndrome decreased from a high of 0.63 per 100,000 population less than 12 years of age in 1983/84 to 0.11 in 1990/91.
From November 1995 to November 1996 in France, a national survey of pediatric departments for children under 15 years of age with unexplained encephalopathy and a threefold (or greater) increase in serum aminotransferase and/or ammonia led to the identification of nine definite cases of Reye’s syndrome (0.79 cases per million children). Eight of the nine children with Reye’s syndrome were found to have been exposed to aspirin. In part because of this survey result, the French Medicines Agency reinforced the international attention to the relationship between aspirin and Reye’s syndrome by issuing its own public and professional warnings about this relationship.[13]
]History

The syndrome is named after Dr. R. Douglas Reye, who, along with fellow physicians Dr. Graeme Morgan and Dr. Jim Baral, published the first study of the syndrome in 1963 in The Lancet.[14] In retrospect, the occurrence of the syndrome may have first been reported in 1929. Also in 1964, Dr. George Johnson and colleagues published an investigation of an outbreak of influenza B that described 16 children who developed neurological problems, four of whom had a profile remarkably similar to Reye’s syndrome. Some investigators refer to this disorder as Reye-Johnson syndrome, although it is more commonly called Reye's syndrome. In 1979, Dr. Karen Starko and colleagues conducted a case-control study in Phoenix, Arizona and found the first statistically significant link between aspirin use and Reye's syndrome.[15] Studies in Ohio and Michigan soon confirmed her findings [16] pointing to the use of aspirin during an upper respiratory tract or chickenpox infection as a possible trigger of the syndrome. Beginning in 1980, the CDC cautioned physicians and parents about the association between Reye’s syndrome and the use of salicylates in children and teenagers with chickenpox or viruslike illnesses. In 1982 the U.S. Surgeon General issued an advisory, and in 1986 the Food and Drug Administration required a Reye’s syndrome-related warning label for all aspirin-containing medications.

Wikipedia.org