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.

Fever,poor response, convulsions, and hepatomegaly / 中华实用儿科临床杂志

Objective To investigate the differential diagnosis of Reye syndrome and the characteristics of primary carnitine deficiency,and to provide diagnostic strategy for similar cases.Methods There was a case presented with fever,poor response,convulsions and hepatomegaly hospitalized in Wuhan Children's Hospital,and the clinical manifestations were described,the physical examination was comprehensively conducted,the auxiliary examination results were recorded,some pediatric specialists from ICU,neurology department,genetic metabolic department,digestive system department were invited to discuss the case.The treatment was adjusted according to the suggested opinions;the treatment effects and the final diagnosis were tracked.Results The primary diagnosis of the case was central nervous system infection or toxic encephalopathy at the time of admission,but Reye syndrome could not be excluded.Although the cerebrospinal fluid test and brain MRI examination detected nothing abnormal,liver function suggested alanine aminotrans ferase ALT increase,blood sugar decrease,the liver volume increase,which was detected by liver ultrasound.Blood amino acids examination revealed the carnitine level decreased,and it was confirmed as primary carnitine deficiency in the end.L-carnitine was used to treat the disease,and its effect was good.Conclusions Great importance should be attached to children with onset age,physical check-up,and multidisciplinary cooperation.Use monism to explain the illness and the auxiliary inspection as far as possible,so that it can get early diagnosis and treatment,and the outcome is good.

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.

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.

Nusing of children patients with Reye syndrome / 中国实用护理杂志

Objective To discuss the nursing intervention of Rey syndrome in children patients.so as to search effective nursing measures.Methods 12 children patients with Rey syndrome received comprehensive nursing treatment with reducing intracranial pressure and were under continuous close observation.Slightest changes were feeded back timely,then effective nursing measures were taken to stabilize their illness,patients also received dietary therapy,medication nursing and rehabilitation training,etc.Results All patients ameliorated after 3 to 7 days,and achieved clinical recovery after 20 to 30 days without sequelae.Conclusions Early diagnosis and correct effective nursing care can improve curative rate and avoid the occurrence of complications and sequelae.

A Case of Lennox-Gastaut Syndrome due to 3-Methylcrotonyl CoA Carboxylase Deficiency / 대한소아신경학회지

3-Methylcrotonyl-CoA carboxylase(MCC) is a biotin-dependent enzyme involved in the 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 a viral gastroenteritis and then suffered from Lennox-Gastaut syndrome. Urinary organic acid analysis revealed increased excretions of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine. This patient was managed with a leucine restriction diet and supplementation of biotin and carnitine, which was not so effective. He suffered from neurologic sequelae such as Lennox-Gastaut syndrome, motor and cognitive impairements.

A Case of Ornithine Transcarbamylase (OTC) Deficiency

OTC deficiency is an X-linked disorder in which the synthesis of urea is impaired. OTC catalyzes the synthesis of citrulline from carbamyl phosphate and ornithine. Complete or partial deficiencies of this enzyme may lead to Reye syndrome like picture such as encephalopathy, hepatic dysfunction, hyperammonemia, etc. We recently had a case that was presented as recurrent Reye syndrome, and was effectively treated with hemodialysis, arginine, sodium benzoate, etc. This report describes an experience in treating this condition with review of available literature.

Changes in GFAP Immunoreactivity of Astrocytes in Rats with Reye's Syndrome Induced by Valproic Acid and the Effects of Carnitine Supplementation

PURPOSE: This study was designed to investigate whether valproic acid(VPA) induces Reye's syndrome by analyzing blood chemistry, carnitine(CARN) concentration, and the morphologic changes of hepatocytes. The changes of GFAP immunoreactivity of astrocytes were examined in rats with Reye's syndrome induced by VPA. And the effects of CARN supplementation on Reye's syndrome were also investigated. METHODS: Ten rats were assigned to each of the following 3 groups : control group(0.5ml of normal saline), VPA group(250mg/kg of VPA), and VPA plus CARN group(250mg/kg of VPA and 100mg/kg of CARN). Each dose was injected intraperitoneally every 12 hours for 7 days. All rats fasted overnight following the final injection. RESULTS: In the VPA group, the levels of glucose and ketones decreased and the levels of ammonia, SGOT and lactates increased as compared with the control group. Lower levels of free CARN and higher acyl/free CARN ratios, indicating secondary CARN deficiency, were observed, and the appearance of transformed mitochondria of hepatocytes provided further evidence of mitochondrial metabolic dysfunction in Reye's syndrome. The GFAP immunoreactivity and the area percent of GFAP-positive astrocytes in the cingulum decreased in VPA group. In the VPA plus CARN group, there were no significant differences with the control group in blood chemistry, acyl/free CARN ratios and morphology of hepatocytes. However, the GFAP immunoreativity and the percent of GFAP-positive astrocytes in the cingulum decreased as much as in the VPA group. CONCLUSION: It is suggested that CARN supplementation may prevent abnormal findings of blood chemistry, CARN deficiency and the morphologic changes of liver mitochondria due to VPA, but CARN may have little effect on GFAP immunoreactivity of astrocytes in rats with Reye's syndrome induced by VPA.

Clinical observation on Reye syndrome according to the onset of age in children

No abstract available.

Two Cases of Postvaricella Reye Syndrome

Authors observed two cases of Reye syndrome in 7 and 8 year old male patients who had begun to vomit after chickenpox. The interval between the onset of varicella rash and encephalopathy of Reye syndrome was 4 days in both these cases. The initial laboratory findings revealed increased serum transaminase and blood ammonia levels which returned to normal on 18 th and 11 th days, respectively, of hospital care. A brief review of literatures was presented.

Two Cases of Postvaricella Reye Syndrome

Authors observed two cases of Reye syndrome in 7 and 8 year old male patients who had begun to vomit after chickenpox. The interval between the onset of varicella rash and encephalopathy of Reye syndrome was 4 days in both these cases. The initial laboratory findings revealed increased serum transaminase and blood ammonia levels which returned to normal on 18 th and 11 th days, respectively, of hospital care. A brief review of literatures was presented.