Reye’s syndrome is a rare form of acute encephalopathy and fatty infiltration of the liver. Sometimes it is a result of a viral infection, especially if salicylates were taken. The diagnosis is made clinically. Treatment is supportive.
The cause of Reye’s syndrome is unknown. but many cases appear to occur after an influenza A or B or a Varizelleninfekt. The use of salicylates (usually aspirin) in these diseases increases the risk by 35%. The findings have led to a significantly lower consumption of salicylates in the United States since the mid-1980s (unless they are specifically indicated as in idiopathic arthritis or Kawasaki disease). In the result, the Reye’s syndrome could be lowered by several 100 cases a year to approx. 2 The syndrome occurs <18 years exclusively in children. Most cases occur in late autumn and winter.
Reye’s syndrome is a rare form of acute encephalopathy and fatty infiltration of the liver. Sometimes it is a result of a viral infection, especially if salicylates were taken. The diagnosis is made clinically. Treatment is supportive. The cause of Reye’s syndrome is unknown. but many cases appear to occur after an influenza A or B or a Varizelleninfekt. The use of salicylates (usually aspirin) in these diseases increases the risk by 35%. The findings have led to a significantly lower consumption of salicylates in the United States since the mid-1980s (unless they are specifically indicated as in idiopathic arthritis or Kawasaki disease). In the result, the Reye’s syndrome could be lowered by several 100 cases a year to approx. 2 The syndrome occurs <18 years exclusively in children. Most cases occur in late autumn and winter. The disease affects the mitochondrial function with a disorder of fatty acid and carnitine metabolism. Pathophysiology and clinical manifestations are similar to a number of inherited metabolic disorders of fatty acid transport and mitochondrial oxidation (Inborn errors of metabolism). Symptoms and signs The severity of the disease varies greatly, however, is characterized by a disease process in two phases. At the start, there is a viral infection (infection of the upper respiratory tract or chickenpox), after 5-7 days following the vigorous nausea and vomiting, with a sudden change of the state of mind. The changes of the mental state can range from weak amnesia, weakness and mildly impaired eyesight and hearing with lethargy on episodes of disorientation and restlessness to a worsening with intensifying comatose states. These are characterized by progressive apathy Dekortikations- and Dezerebrationshaltungen seizures laxity Permanent pupil dilation apnea usually are no focal neurological symptoms present. Hepatomegaly can be observed in 40% of cases, but jaundice is missing. Complications Complications include electrolyte and fluid disorders Increased intracranial pressure diabetes insipidus syndrome of inappropriate antidiuretic hormone release hypotension arrhythmias bleeding (especially the gastrointestinal tract) pancreatitis Respiratory failure hyperammonemia aspiration pneumonia Poor temperature regulation Unkale herniation and death diagnosis Clinical findings related to laboratory tests liver biopsy Reye's syndrome should be suspected in any child, the sudden encephalopathy (without a known Schwermetallintoxikation or poisoning) and violent Erbrech s developed with liver dysfunction. Liver biopsy provides the definitive diagnosis with microvesicular fatty changes and is <2 years particularly useful in sporadic cases and children. Diagnosis can also be made if typical clinical findings and history are associated with the following laboratory values: greatly increased transaminases (GOT, GPT> 3 times the normal value), normal bilirubin, elevated ammonia levels, prolonged prothrombin time. A computed tomography or magnetic resonance imaging is performed in any child with encephalopathy. If the computed tomography or magnetic resonance imaging is normal, a lumbar puncture (lumbar puncture (lumbar puncture)) can be carried out. The CSF usually shows an increased pressure, <8-10 leukocytes / ul, a normal amount of protein and a possible increase in the Glutaminspiegels. They should be tested for metabolic disorders. Hypoglycemia and Hypoglykorrhagie (a very low concentration of CSF glucose) was observed in 15% of cases, especially in children <4 years. The classification is according to the severity of the disease in five stages. To the characters of an extended metabolic disorder include increased serum levels of the amino acids, disorders of acid-base balance (usually a hyperventilation, a mixed respiratory alkalosis / metabolic acidosis), Hyper - and hypoosmolarity, hypernatremia, hypokalemia, and hypophosphatemia. Differential Diagnosis In the differential diagnosis must consider other causes of coma and impaired hepatic function, such as sepsis or hyperthermia (especially in infants), treatable congenital abnormality of the urea synthesis (eg. B. ornithine transcarbamylase deficiency) or fatty acid oxidation (eg. B . systemic carnitine deficiency, medium-chain acyl-CoA dehydrogenase deficiency) intoxication with phosphorus or carbon tetrachloride, a (by salicylates or other drugs z. B. valproate) caused acute encephalopathy, intoxications, viral meningitis or encephalitis and acute hepatitis. Diseases such as idiopathic steatosis of pregnancy and tetracycline-related liver disorder may show similar light microscopic changes. Prognosis The outcome depends on the duration of cerebral disorders, the severity of the coma, and the speed at which it develops, from the increase in the intracranial pressure and the degree of blood ammonia increased. is the transition from stage I in a higher stage probably within an initial blood ammonia levels> 100 ug / dl (> 60 .mu.mol / l) and compared to the control examination at least 3 s prolonged prothrombin time. In fatal cases, the average time from hospitalization to death four days. The mortality rate is on average 21% in patients with stage I it is <2% in patients with stage IV or V to> 80%. The prognosis for survivors is usually good, and relapses are rare. The frequency of neurological sequelae (mental retardation, seizures, cranial nerve paralysis, motor dysfunctions) is 30% in those patients that have been shown during their hospitalization seizures or Dezerebrierungszeichen. Treatment Support Measures Treatment is supportive, with special attention to the intracranial pressure and blood sugar, as a glycogen depletion is common. Increased intracranial pressure is treated with intubation, hyperventilation, fluid restriction to 1500 ml / m2 // day, increasing the head end and osmotic diuretics. An infusion of 10 or 15% dextrose is normal to ensure euglycemia. Coagulopathy may need to be treated with FFP or vitamin K. Other treatments (eg. As exchange transfusion, hemodialysis, inducing a deep coma with barbiturates) have proven to be ineffective, but are sometimes used. Conclusion The Reye’s syndrome with acute encephalopathy and liver dysfunction, usually due to a viral infection (particularly with Salicylatanwendung) has decreased in children since the routine use of aspirin. The diagnosis is made by exclusion of similarly manifested infectious, toxic and metabolic disorders. A liver biopsy can help confirm the diagnosis. Treatment is supportive, with particular measures to reduce intracranial pressure.