A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus (EBV) infection developed Reye's syndrome. He also suffered from acute liver failure, life-threatening cerebral edema, severe disseminated intravascular coagulation (DIC), and myocardial involvement. EBV infection aggravated the progress of Reye's syndrome, leading to death despite full supportive and symptomatic therapy. This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.
Epstein-Barr Virus Infections/complications* ; Fatal Outcome ; Herpesvirus 4, Human/physiology* ; Humans ; Infant ; Male ; Multiple Organ Failure/complications* ; Reye Syndrome/virology*

A boy aged 6 years and 3 months developed upper respiratory tract infection and pyrexia 2 months ago and was given oral administration of nimesulide by his parents according to directions. Half an hour later, the boy experienced convulsions and cardiopulmonary arrest, and emergency examination found hypoketotic hypoglycemia, metabolic acidosis, significant increases in serum aminotransferases and creatine kinase, and renal damage. Recovery of consciousness and vital signs was achieved after cardiopulmonary resuscitation, but severe mental and movement regression was observed. The boy had a significant reduction in free carnitine in blood and significant increases in medium- and long-chain fatty acyl carnitine, urinary glutaric acid, 3-hydroxy glutaric acid, isovalerylglycine, and ethylmalonic acid, suggesting the possibility of multiple acyl-CoA dehydrogenase deficiency. After the treatment with vitamin B2, L-carnitine, and bezafibrate, the boy gradually improved, and reexamination after 3 months showed normal biochemical parameters. The boy had compound heterozygous mutations in the ETFDH gene, i.e., a known mutation, c.341G>A (p.R114H), from his mother and a novel mutation, c.1484C>G (p.P495R), from his father. Finally, he was diagnosed with multiple acyl-CoA dehydrogenase deficiency. Reye syndrome and sudden death symptoms were caused by nimesulide-induced acute metabolic crisis. It is concluded that inherited metabolic diseases may be main causes of Reye syndrome and sudden death, and biochemical and genetic analyses are the key to identifying underlying diseases.
Acyl-CoA Dehydrogenase ; Administration, Oral ; Carnitine ; Child ; Death, Sudden ; Humans ; Male ; Respiratory Tract Infections ; Reye Syndrome ; Sulfonamides

Rotavirus is the major cause of gastroenteritis in children under the age of 5. Rotavirus infection may lead to several neurological complications as meningitis, encephalitis, convulsion, encephalopathy, hemorrhagic shock, central pontine myelinolysis, Guillain-Barre syndrome, and Reye's syndrome. Further, some reports have described diffuse cerebral white matter lesions on diffusion-weighted magnetic resonance imaging (MRI) in neonates with rotavirus induced seizures. Here, we report on three neonates with rotavirus induced seizures with cerebral white matter abnormalities on MRI.
Brain Diseases ; Child ; Diffusion Magnetic Resonance Imaging ; Encephalitis ; Gastroenteritis ; Guillain-Barre Syndrome ; Humans ; Infant, Newborn ; Magnetic Resonance Imaging* ; Meningitis ; Myelinolysis, Central Pontine ; Reye Syndrome ; Rotavirus Infections* ; Rotavirus* ; Seizures* ; Shock, Hemorrhagic ; White Matter*

OBJECTIVE: Reye's syndrome can be caused by high dose of aspirin which is for treatment of acute phase of Kawasaki disease. We evaluated the effectiveness of treatment and coronary complications of replacing high dose of aspirin with ibuprofen for children in acute phase of Kawasaki disease. METHODS: Children with Kawasaki disease (n=235) were admitted in the pediatric department from January 1, 2015 to December 31, 2017. Echocardiography and laboratory tests were performed during admission, and the children were followed-up at 6–8 weeks after the onset. We retrospectively analyzed their characteristics and clinical outcomes. RESULTS: The children were assigned to receive either high dose of aspirin with intravenous immunoglobulin (IVIG) (aspirin group) or ibuprofen with IVIG (ibuprofen group). A total of 119 and 116 children were included in the aspirin and ibuprofen groups. Total fever duration was 6.5±1.6 days in the aspirin group, and 6.5±1.7 days in the ibuprofen group (P=0.674). The number of resistance to initial treatments was 11 in the aspirin group and 11 in the ibuprofen group (P=0.571). There were 13 initial coronary complications in the aspirin group, and 10 in the ibuprofen group (P=0.552) and children who showed coronary artery abnormalities improvement at 6- to 8-week follow-up was seven and five in the aspirin and ibuprofen groups, respectively (P=0.769). CONCLUSION: We may consider using ibuprofen in acute phase of Kawasaki disease to prevent the severe complications of aspirin use, such as Reye's syndrome in the case concerning influenza or varicella.
Aspirin* ; Chickenpox ; Child ; Coronary Vessels ; Echocardiography ; Fever ; Follow-Up Studies ; Humans ; Ibuprofen* ; Immunoglobulins ; Immunoglobulins, Intravenous ; Influenza, Human ; Mucocutaneous Lymph Node Syndrome* ; Retrospective Studies ; Reye Syndrome

Drug hypersensitivity is one of drug adverse reactions that develop in susceptible patients following exposure to certain drugs and cannot be predicted from the known pharmacology of a drug. Severe hypersensitivity is associated with high morbidity and mortality. Although the issue of hypersensitivity to nonsteroidal anti-inflammatory drugs (NSAIDs) has been largely investigated in adults, data related to NSAIDs hypersensitivity is insufficient in childhood. And in spite of the recommendation to avoid use of aspirin due to Reye syndrome in children, aspirin is one of major treatment along with intravenous immunoglobulin in Kawasaki disease. We report a case of a 10-month-old boy who underwent intravenous immunoglobulin and aspirin treatment for Kawasaki disease, and subsequently revealed severe leukocytosis and eosinophilia. To our knowledge, there have been no previous reports of aspirin-induced eosinophilia in Korea.
Adult ; Anti-Inflammatory Agents, Non-Steroidal ; Aspirin* ; Child* ; Drug Hypersensitivity ; Eosinophilia* ; Humans ; Hypersensitivity* ; Immunoglobulins ; Infant ; Korea ; Leukocytosis ; Male ; Mortality ; Mucocutaneous Lymph Node Syndrome* ; Pharmacology ; Reye Syndrome

Reye syndrome is a rapidly progressive encephalopathy with hepatic dysfunction, which often begins several days after apparent recovery from a viral illness, especially varicella or influenza A or B. Salicylate use was identified as a major precipitating factor for the development of Reye syndrome. With the recommendation to avoid use of salicylates in children, Reye syndrome has virtually disappeared in recent years. We report a case of Reye syndrome in a 5-month-old infant who had been treated with intravenous immunoglobulin and aspirin under the diagnosis of Kawasaki disease, and showed symptoms of sudden onset of irritability, rigidity, decreased activity, vomiting, poor appetite, lethargy, liver dysfunction without jaundice, coagulopathy, and hyperammonemia.
Appetite ; Aspirin ; Chickenpox ; Child ; Humans ; Hyperammonemia ; Immunoglobulins ; Infant ; Influenza, Human ; Jaundice ; Lethargy ; Liver Diseases ; Mucocutaneous Lymph Node Syndrome ; Precipitating Factors ; Reye Syndrome ; Salicylates ; Vomiting

Salicylates, such as aspirin, are considered the most commonly used medicine in Korea for its anti-inflammatory, anti-pyretic, and analgesic properties. In spite of its wide range of benefits, aspirin produces unwanted adverse effects such as mucosal bleeding in the gastrointestinal tract, renal and hepatic dysfunction, Reye's syndrome in children, and hypersensitivity reactions, etc. Aspirin can also induce ototoxicity, such as reversible hearing loss and tinnitus. The pattern of hearing loss is typically mild to moderate and bilaterally flat in the absence of preexisting hearing loss. Hearing usually recovers in 72 hours after medication. However, it's rare that salicylate-induced ototoxicity are encountered. So we present this case of bilateral hearing loss that occurred after salicylate intoxication with a review of relevant literature.
Aspirin ; Child ; Ear, Inner ; Gastrointestinal Tract ; Hearing ; Hearing Loss ; Hearing Loss, Bilateral ; Hearing Loss, Sensorineural ; Hemorrhage ; Humans ; Hypersensitivity ; Korea ; Reye Syndrome ; Salicylates ; Sodium Salicylate ; Tinnitus

PURPOSE: We evaluated the effectiveness of treatment and cardiac complications of replacing a high dose of aspirin with a high dose of ibuprofen for children in acute phase of Kawasaki disease. We also analyzed the possibility of replacing a high dose of aspirin with a high dose of ibuprofen to prevent complications such as Reye's syndrome caused by aspirin. METHODS: One hundred eight children with Kawasaki disease were admitted in the pediatrics department from January 1, 2004 to December 31, 2008. Echocardiography and laboratory tests were performed during diagnosis, and the children were followed-up at 6-8 weeks after the diagnosis. We retrospectively analyzed their characteristics and clinical results. RESULTS: The children were assigned to receive either a high dose of aspirin with intravenous immunoglobulin (IVIG) (aspirin group) or a high dose of ibuprofen with IVIG (ibuprofen group). A total of 55 and 53 children were included in the aspirin and ibuprofen groups, respectively. The mean defervescence period was 6.5+/-2.1 days in the aspirin group, and 6.9+/-1.9 days in the ibuprofen group (P=0.309). The number of failed treatments, during and after treatment, was 8 in the aspirin group and 10 in the ibuprofen group (P=0.547). There were 11 initial cardiac complications in the aspirin group, and 14 in the ibuprofen group, but children who showed improvement at follow-up was 7 and 13 in the aspirin and ibuprofen groups, respectively (P=0.133). Laboratory findings were also improved in both groups. CONCLUSION: We can be considered whether we will replace high dose of aspirin by high dose of ibuprofen in acute phase of Kawasaki disease. Therefore, we can prevent the severe complications of aspirin use, such as Reye's syndrome.
Aspirin ; Child ; Echocardiography ; Follow-Up Studies ; Humans ; Ibuprofen ; Immunoglobulins ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome ; Pediatrics ; Retrospective Studies ; Reye Syndrome

Autopsy ; Female ; Humans ; Reye Syndrome ; pathology ; physiopathology ; Young Adult

PURPOSE: The prevalence of Reye syndrome has decreased since late 1980's. But we report that recently there were concentrative attacks of Reye syndrome after acute enteritis during the neonatal period. METHODS: Clinical symptoms and laboratory results(quantitative organic acid analysis, routine chemistry, arterial blood gas analysis, serum ammonia) of seven patients admitted at the Samsung Medical Center, Sanggye Paik Hospital, Wonju Christian Hospital and Chuncheon Sacred Heart Hospital, referred from Jan. 2005 to Apr. 2005, were analysed retrospectively. The major clinical symptoms were derived from the patients' clinical records sended with urine samples and quantification of organic acids were done with gas chromatography and mass spectrometry. RESULTS: The mean age of seven cases is 18 days and the major preceding symptoms were gastrointestinal symptoms(vomiting, diarrhea, refusal to feeding). The major clinical symptoms were clouded conciousness, repiratory difficulty, vomiting, seizures, and diarrhea. One patient died; that patient's serum ammonia was twenty times higher than normal. CONCLUSION: The seven patients were neonates. Reye syndrome has been known to be closely related with upper respiratory infections as a preceding disease and to internal use of aspirin, but in our study, the major preceding disease of the seven cases was gastrointestinal infection and none of these used aspirin.
Ammonia ; Aspirin ; Blood Gas Analysis ; Chemistry ; Chromatography, Gas ; Diarrhea ; Disulfiram ; Enteritis* ; Gangwon-do ; Heart ; Humans ; Infant, Newborn ; Mass Spectrometry ; Prevalence ; Respiratory Tract Infections ; Retrospective Studies ; Reye Syndrome* ; Seizures ; Vomiting