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Summary
REYE SYNDROME
unknown
  • antibiotics
  • anticonvulsants
  • fluid restriction
  • fresh frozen plasma
  • glucose infusion (acute)
  • low-protein diet
  • supportive/symptomatic therapy
  • vitamin K
Reye syndrome (RS) is an acute metabolic encephalopathy and hepatopathy affecting children and adolescents,
a specific underlying defect should be excluded by careful clinical and biochemical investigation risk factors: acetylsalicylic acid, phenothiazines, anti-emetics
symptoms
coma
defect of deep tendon reflexes
encephalopathy
hyperammonemia
hypoglycemia
lethargy, drowsiness, malaise or sleep disorder
liver involvement or dysfunction
pancreatitis
seizures
tachypnea, hyperpnea, dyspnea, respiratory distress
laboratory finding
Ammonia200.002000.0025.0080.00-Ámol/lblood
Glucose0.503.002.805.00mmol/lserum
pH7.007.407.357.45no unitblood
Transaminases30.002000.0010.0030.00U/lserum
EEG abnormalities -
Creatine kinase increasedserum
Lactate dehydrogenase (LDH) increasedserum
Coagulopathy/Coagulation factors decreasedplasma
Literature
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Saudubray JMSpecola N, Middleton B, Lombes A, Bonnefont JP, Jacobs C, Vassault A, Charpentier CHyperketotic states due to inherited defects of ketolysis4th International congress of inborn errors of metabolism001986
Christopher RRajivnath V, Shetty KTArginase deficiencyIndian J Pediatr642266-2691997
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Lehnert WRuitenbeek WEthylmalonic aciduria associated with progressive neurological disease and partial cytochrome c oxidase deficiencyJ Inh Met Dis160557-5591993
Fenton WARosenberg LEInherited disorders of cobalamin transport and metabolismThe metabolic and molecular bases of inherited disease, 7/e Editors: C.R.Scriver, A.L.Beaudet, W.S.Sly, D.Valle McGraw-Hill Inc.203129-32491995
Brautigam Cet al.Biochemical hallmarks of tyrosine hydroxylase deficiencyClin Chem4491897-19041998
Stacpoole PWet al.The importance of cerebrospinal fluid lactate in the evaluation of congenital lactic acidosisJ Pediatr134099-1021999
Jakobs CSchweitzer S, Dorland BGalactitol in galactosemiaEur J Pediatr Suppl 21540S50-S521995
Nomura YSegawa diseaseRinsho Shinkeigaku37121137-11381997
Santos Silva Eet al.Liver transplantation in a case of argininaemiaJ Inherit Metab Dis248885-8872001
Guneral FBachmann CAge-Related Reference Values for Urinary Organic Acids in a Healthy Turkish Pediatric PopulationClin Chem406862-8681994
Kayani R,Botros S, Moore PBeta-ketothiolase deficiency and pregnancyInt J Obstet Anesth223107-1152013
Stacpoole PWet al.The importance of cerebrospinal fluid lactate in the evaluation of congenital lactic acidosisJ Pediatr134099-1021999
Bremer HJDuram M, Kamerling JP, Przyrembel H, Wadman SKDisturbences of amino acid metabolism: clinical chemistry and diagnosisUrban und Schwarzenberg, Munich001981
Mayatepek EFlock BLeukotriene C4-synthesis deficiency: a new inborn error of metabolism linked to a fatal developmental syndromeLancet35201514-15171998
Stacpoole PWet al.The importance of cerebrospinal fluid lactate in the evaluation of congenital lactic acidosisJ Pediatr134099-1021999