A Severe Case of Reye's Syndrome with Multiorgan Dysfunction after Epstein-Barr Virus Infection / 中国医学科学杂志(英文版)

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.

Reye syndrome and sudden death symptoms after oral administration of nimesulide due to upper respiratory tract infection in a boy / 中国当代儿科杂志

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.

Comparative Study between High Dose Aspirin and Ibuprofen in the Treatment of Kawasaki Disease in the Acute Stage

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.

Neonatal Seizures with Diffuse Cerebral White Matter Lesions on Magnetic Resonance Imaging Associated with Rotavirus Infection: A Report of Three Cases

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.

Síndrome de Reye congénito asociado a varicela materna / Congenital Reye syndrome associated to varicella infection in pregnancy

Introducción: el síndrome de Reye es una encefalopatía aguda asociada a una degeneración grasa del hígado que usualmente es precedida de una infección respiratoria o varicela y tiene una alta prevalencia en niños menores de seis años. Objetivo: reportar un caso clínico de síndrome de Reye congénito asociado a la infección por varicela adquirida de la madre. Presentación de caso: se describen los hallazgos de la autopsia, la respectiva correlación clinicopatológica de un recién nacido de sexo masculino de 37 semanas de gestación, hijo de madre con varicela activa desde cuatro días antes del parto, quien presentó súbitamente palidez generalizada, bradicardia y apnea. Resultados: el examen histopatológico encontró en el citoplasma de los hepatocitos y túbulos renales un compromiso vacuolar que correspondía a grasa. En el cerebro se evidenció severo edema, sin inflamación perivascular o meníngea. Conclusión: corresponde a un caso de síndrome de Reye congénito asociado a varicela materna, que terminó manifestándose clínicamente como muerte súbita. Podría ser la primera publicación de un caso de síndrome de Reye congénito asociado a varicela materna. MÉD.UIS. 2014;27(3):113-121.

Introduction: Reye's syndrome is an acute encephalopathy associated with fatty degeneration of the liver that usually is preceded by a respiratory infection or chickenpox and is highly prevalent in children under 6 years old. Objective: to report a clinical case of congenital Reye's syndrome associated with varicella infection acquired from the mother. Case report: we describe the autopsy findings with the respective clinicopathological correlation of a male newborn of 37 weeks of gestation, son of mother with active varicella from 4 days before birth, who presented sudden paleness, bradycardia and apnea. Results: histopathologic examination found in the cytoplasm of hepatocytes and renal tubules a vacuolar commitment that corresponds to fat. The brain showed severe edema without perivascular or meningeal inflammation. Discussion and conclusion: it corresponds a case of congenital Reye's syndrome associated with varicella infection in pregnancy, who finished clinically as sudden death. This could be the first published case of congenital Reye's syndrome associated with varicella infection in pregnancy. MÉD.UIS. 2014;27(3):113-121.

Hypersensitivity reaction to aspirin accompanied by severe eosinophilia in a child with history of Kawasaki disease

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.

Aspirina: síndrome de Reye / Aspirin; Reye`s syndromne

Se presenta el síndrome de Reye y su relación con la administración de salicilatos en niños de 2 a 16 años de edad.

A Case of Reye Syndrome Following Treatment of Kawasaki Disease with Aspirin / 소아감염

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.

Adult Reye-like syndrome associated with serologic evidence of acute parvovirus B19 infection

Reye's syndrome is an infrequently diagnosed medical condition affecting mainly children. The etiology, epidemiology and natural history of Reye's syndrome have been cloudily written in footnotes of medical books and exotic papers since the initial description in early 1950s. We report here a case of adult Reye's syndrome associated with serologic evidence of parvovirus B19 infection.

A Case of Bilateral Sensorineural Hearing Loss after Salicylate Intoxication / 대한이비인후과학회지

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.

Comparison and analysis of the effectiveness to high dose of aspirin and ibuprofen in acute phase of Kawasaki disease / 소아과

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.

Reye syndrome: a case report with review of literature.

Here we describe a case of Reye syndrome diagnosed at postmortem liver biopsy of a three-year old girl who presented with vomiting, low grade fever for three days and loss of consciousness for 18 hours. Clinically, the differential diagnoses were meningitis, encephalitis and septicemia. No history of past illness or any drug ingestion including aspirin were present. Laboratory investigations indicated a diagnosis of Reye syndrome. The child was given supportive treatment but died after two days of admission and postmortem needle-biopsy of the liver showed microvesicular steatosis consistent with Reye syndrome.

Griscelli syndrome: a case report of Reye's syndrome and atopic dermatitis history.

Griscelli syndrome (GS) is a rare autosomal recessive disorder that results in pigmentary dilution of the skin and the hair (silver hair), with the presence of large clumps of pigment in hair shafts, and an accumulation of melanosomes in melanocytes. Sixty cases of GS have been reported in the literature, but we could find no description of its oro-dental symptoms. Reye's syndrome (RS) is characterized by acute noninflammatory encephalopathy and renal and hepatic failure, while atopic dermatitis (AD) is a skin disorder with an immunologic basis. The aim of this paper is to describe the oro-dental and physical findings in a girl who had been diagnosed with GS at 3.5 years of age; she also had AD as well as a history of RS at infancy. We discuss the possible relationship between the three syndromes.

Reye syndrome after acute enteritis during the neonatal period / 소아과

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.

Intoxicación por hierbas y su posible relación con encefalopatía hepática: sindrome de Reye / Herbal poisining with possible association whit Reye syndrome

Lactante, masculino de 7 meses de edad que ingresó al Hospital del Niño de La Paz, por presentar un cuadro de deshidratación, acidosis metabólica, antecedente de diarrea aguda y a quien le administraron una infusión casera de múltiples hierbas con propósitos curativos. Evolucionó con datos clínicos de encafalopatía, hepatopatía y crisis convulsivas. Las aminotransferasas estuvieron elevadas al triple y el amonio sérico incrementado al doble de los valores de referencia. La tomografía de cráneo mostró edema cerebral y la biopsia hépática reporto lesiones microvacuolas en hepatocitos compatibles con Síndrome de Reyé. La evolución del cuadro fue lentamente favorable con uso de terapia antiedema cerebral, soluciones parenterales y apoyo nutricio para paciente insuficiente hepático. El diagnóstico final fue: Síndrome de Reyé posiblemente asociado a intoxicación por hierbas.