• Pediatric Infection and Vaccine
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• v.19 no.2
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• pp.79-83
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• 2012

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.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.273-277
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• 2006

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.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.4 no.1
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• pp.46-53
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• 2004

3-Methylcrotonyl-CoA carboxylase (MCC) is a biotin-dependent enzyme involved in leucine metabolism. We describe a patient with MCC deficiency who manifested with Reye syndrome-like illness with status epilepticus, metabolic acidosis, hypoglycemia, hyperammonemia, elevated liver enzymes and neurologic impairments after the viral gastroenteritis and then, has suffered from Lennox-Gastaut syndrome. Urinary organic acid analysis revealed increased excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine. This patient was managed with leucine restriction diet and supplementation of biotin and carnitine but was not so effective. He has suffered from neurologic sequelae such as Lennox-Gastaut syndrome, motor and cognitive impairement.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.13 no.2
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• pp.75-80
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• 2013

Specific genetic conditions may lead to sudden unexpected deaths in infancy, such as inborn errors of fatty acid oxidation and genetic disorders of cardiac ion channels. The disease may present dramatically with severe hypoketotic hypoglycemia, Reye syndrome or sudden death, typically with a peak of frequency around 3-6 month, whilst neonatal sudden death is quite rare. When undetected, approximately 20-25% of infants will die or suffer permanent neurologic impairment as a consequence of the first acute metabolic decompensation. Meanwhile, the advent of newborn screening for metabolic diseases has revealed populations of patients with disorders of fatty acid oxidation (FAO), the most frequent of which is medium chain acyl-CoA dehydrogenase (MCAD) deficiency. Without this screening, affected individuals would likely succumb to sudden infant death syndrome (SIDS). Here we describe an overview of sudden infant death syndrome and inherited metabolic disorder.

• Neonatal Medicine
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• v.25 no.2
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• pp.85-89
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• 2018

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.

• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.930-937
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• 2009

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${\pm}$2.1 days in the aspirin group, and $6.9{\pm}1.9$ 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.

• Journal of Yeungnam Medical Science
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• v.3 no.1
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• pp.375-382
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• 1986

We studied the effects of the acute intermittent peritoneal dialysis in severe acute renal failure of 1 newborn infant and 2 young infants during 18 months period from February 1985 to April 1986. The predisposing illnesses were severe acute gastroenteritis with dehydration. Reye's syndrome, and bilateral nephrolithiasis with hyperuricemia. The concomittent illnesses were severe hypernatremia, hyponatremia, hyperkalemia, hypocalcemia, hypoglycemia, DIC(disseminated intravascular coagulopathy), paralytic ileus, metabolic acidosis and gastrointestinal bleeding. As a dialvsate, Imperinol $solution^R$, 1.5% was used in all cases. The cycles of dialysis were 8, 16, and 41 times in each cases. Observed complications during dialysis were leakage, and abdominal wall and scrotol swelling in 2 cases, hyperglycemia in 1 case, and peritonitis in 1 case. Acinetobacter calcoaceticus was cultured in peritoneal fluid of peritonitis. These complications were treated by stopping dialysis in leakage and abdiminal wall swelling, insulin therapy in hyperglycemia, and intraperitoneal and systemic antibiotics therapy in peritonitis. We experienced improvements of severe acute renal failure with variable concomittant illnesses by acute intermittent peritoneal dialysis despite of the treatable complications of dialysis in all cases.

• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.1024-1028
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• 2003

Influenza-associated encephalopathy is regarded as one of the major neurologic disease entities along with those of Reye syndrome, acute necrotizing encephalopathy, and myelitis which are known to be related to influenza virus, mostly type A. And it is being actively researched in Japan as it has caused a tremendous increase in the number of deaths from 1997 to 2002, but it has not been yet reported in the Korean pediatric medical community. It attacks those previously healthy children, who have not been vaccinated. Patients start with such symptoms as fever and common respiratory problems, but within 24 to 48 hours they suffer from seizures with acute mental deterioration, become worse, and suffer multiple organ failures including marked elevated transaminase levels as well as coagulopathy. It induces deaths in a couple of days after the symptoms appear or remains a serious neurologic sequelae. Confirmative diagnosis is used to demonstrate influenza viral infection. We report here a 37 month aged boy who was admitted to our hospital during the last influenza season under the diagnosis of influenza associated encephalopathy on the basis of serologic testing by hemagglutinin inhibition(HI). This is the first report confirmed by increased antibody titer of the influenza A virus in Korea.