Aspirin rechallenge in an adult patient previously diagnosed with Reye syndrome.

PURPOSE: Aspirin has been the cornerstone of antiplatelet therapy in patients with acute coronary syndromes and is well accepted and recommended by several major healthcare organizations. A combination of aspirin and a P2Y12 inhibitor, commonly known as dual antiplatelet therapy, is recommended in patients with coronary stent implantation to reduce the risk of stent thrombosis and ischemic events. SUMMARY: We recently cared for an adult male who presented with an acute coronary syndrome who had a history of Reye syndrome during childhood. During this admission, he was rechallenged with low-dose aspirin for the first time since his diagnosis of Reye syndrome as a child after aspirin therapy. There have been various case reports in children and adults who have been rechallenged with aspirin within days to weeks after the initial diagnosis of Reye syndrome. These reports show mixed results in children and adults regarding the return of Reye syndrome upon aspirin rechallenge shortly after initial aspirin exposure. CONCLUSION: This, to our knowledge, appears to be the first report of a low-dose aspirin rechallenge 30 years later in life in an adult patient with a history of Reye syndrome while receiving aspirin therapy during childhood.

A Severe Case of Reye's Syndrome with Multiorgan Dysfunction after Epstein-Barr Virus Infection.

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.

Hyponatremic seizures and Reye syndrome.

The review questions that are featured in each of the issues of the JEN are based upon the Emergency Nursing Core Curriculum and other pertinent resources to emergency nursing practice, pediatric and adult. These questions offer emergency nurses an opportunity to test their knowledge about their practice. These questions appear both in print and online.

Case of Reye's syndrome accompanied by hemolytic anemia and cardiac injury after cytomegalovirus infection.

A 6-month-old girl with active human cytomegalovirus (HCMV) infection developed Reye's syndrome after vaccination. She suffered from uncommon complications of HCMV infection, including Coombs-negative hemolytic anemia and cardiac injury, but recovered after the appropriate treatment with prompt ganciclovir and symptomatic support. However, the patient died later as a result of a viral upper respiratory tract infection, which aggravated the primary disease. This case suggests that HCMV infection might be a causative agent of Reye's syndrome.

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.

Hyperammonemia in children: on the crossroad of different disorders.

BACKGROUND: Symptoms of hyperammonemia occur in patients irrespective of the kind of metabolic diseases. Age, metabolic and nutritional status, and decompensation factors such as infections influence clinical manifestations. Prolonged, untreated hyperammonemia leads to brain injury and intellectual disability. Treatment is directed at lowering plasma ammonia. Brain ammonium concentrations are 1.5 to 3.0 times higher than that in blood. REVIEW SUMMARY: The authors discuss the pathophysiology of the symptoms and consequences of hyperammonemia in children, focusing on the metabolic disorders leading to an increased level of ammonia. CONCLUSIONS: Ammonia toxicity has been investigated for a long time. According to the main hypotheses, the neurological alterations are connected to alterations in glutamatergic neurotransmission.

[The Reye syndrome]. / Syndrome de Reye.

The Reye syndrome is a complex disease that remains little-known despite its severity. It can occur in children of all ages, and is often fatal, while surviving children often display neurological damage. The therapy is symptomatic and supportive. The diagnosis of Reye's syndrome is not straightforward, as the symptoms are very diverse. The causes of the disease are moreover still unclear, and, after many years of discussion and research, it can still not be proved irrefutably whether administration of acetylsalicylic acid to children suffering from viral infections is a factor in the development of Reye's syndrome.

Fatal case of Reye's syndrome associated with H3N2 influenza virus infection and salicylate intake in a 12-year-old patient.

We describe a fatal case of Reye's syndrome in a 12-year-old male patient during an influenza A (H3N2) infection for which he received salicylates. In the current situation of the novel A/H1N1 virus pandemic, we believe that it is of high importance to emphasize the risks associated with salicylate intake to avoid the reappearance of Reye's 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.

Metabolic stroke in three years old boy as the consequence of metabolic derangement. A case report of recidiving Reye's-like syndrome.

Three years old boy with developmental renal dysplasia was hit as newborn child by attack of cerebral edema with metabolic disturbances (hypoglycemia, hypophosphatemia, ketoacidosis and with hypocoagulation state) and was classified as child at risk in the pediatric evidence. In the third year of the age he went through nephrectomy and after the operation, the similar metabolic disturbances occurred (hypoglycemia, ketoacidosis, derangement of the metabolic situation). Cerebral edema and the metabolic stroke developed. Reye's-like syndrome was considered and serious functional disturbances of basal ganglia and brain-stem structure were observed.

Catastrophic presentation of mitochondrial disease due to a mutation in the tRNA(His) gene.

The authors describe a patient who presented with headache, seizures, and severe cerebral edema in whom they identified a novel mutation in the mitochondrial (mt-) tRNA(His) gene. This G12147A transition is heteroplasmic, predicted to disrupt a highly conserved base pair, and segregates with the cytochrome c oxidase deficiency in single muscle fibers.

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.

[Microvesicular steatosis]. / Steatoza hepatica microveziculara.

BACKGROUND: "Bright" liver at ultrasonography predicts fatty liver. AIM: To assess prevalence and predictors of a bright liver. METHODS: Prevalence arm--Prospective collection of records of unselected patients undergoing liver ultrasound. Protocol arm--A sample of patients with bright liver underwent routine laboratory investigations, liver tests, HBsAg and anti-HCV. RESULTS: No difference was found in mean values of fasting plasma glucose, LDL cholesterol, total bilirubin, GOT, GPT, AP, GGT, and serum bile acids or in the prevalence of serum HBsAg and anti-HCV in bright liver vs control groups. Univariate analysis showed body mass index, age, total cholesterol triglycerides, albumin, HDL cholesterol to be significantly. CONCLUSIONS: Roughly 20% of patients undergoing US for routine clinical practice will have a bright liver and levels are the independent predictors of a bright liver.

Neonatal presentation of ventricular tachycardia and a Reye-like syndrome episode associated with disturbed mitochondrial energy metabolism.

BACKGROUND: Hyperammonemia, hypoglycemia, hepatopathy, and ventricular tachycardia are common presenting features of carnitine-acylcarnitine translocase deficiency (Mendelian Inheritance in Man database: *212138), a mitochondrial fatty acid oxidation disorder with a lethal prognosis. These features have not been identified as the presenting features of mitochondrial cytopathy in the neonatal period. CASE PRESENTATION: We describe an atypical presentation of mitochondrial cytopathy in a 2 day-old neonate. She presented with a Reye-like syndrome episode, premature ventricular contractions and ventricular tachycardia. Initial laboratory evaluation exhibited a large amount of 3-methylglutaconic acid on urine organic acid analysis, mild orotic aciduria and a nonspecific abnormal acylcarnitine profile. The evaluation for carnitine-acylcarnitine translocase deficiency and other fatty acid oxidation disorders was negative. The patient later developed a hypertrophic cardiomyopathy and continued to be affected by recurrent Reye-like syndrome episodes triggered by infections. A muscle biopsy exhibited signs of a mitochondrial cytopathy. During the course of her disease, her Reye-like syndrome episodes have subsided; however, cardiomyopathy has persisted along with fatigue and exercise intolerance. CONCLUSIONS: This case illustrates that, in the neonatal period, hyperammonemia and ventricular tachycardia may be the presenting features of a lethal carnitine-acylcarnitine translocase deficiency or of a mitochondrial cytopathy, associated with a milder clinical course. This association broadens the spectrum of presenting phenotypes observed in patients with disturbed mitochondrial energy metabolism. Also, the presence of 3-methylglutaconic aciduria suggests mitochondrial dysfunction and mild orotic aciduria could potentially be used as a marker of mitochondrial disease.

Uso de antiinflamatorios nao-hormonais em crianças com doenças virais versus risco potencial para sindrome de Reye e doencas invasivas graves por estreptococos do grupo A / Nonsteroidal anti-inflammatory drugs use in children with viral diseases versus potential risks for Reyes's syndrome and invasive group A streptococal infections

Desde o inicio da decada de 1980, o uso de aspirina tem sido relacionado a sindrome de Reye e, na ultima decada, apos ter sido detectado um aumento na frequencia das doencas invasivas graves causadas por estreptococo beta-hemolitico do grupo A, tem sido publicados diversos artigos sobre a possivel relacao entre o uso de antiinflamatorios nao-hormonais em criancas com varicela e a ocorrencia de fasciite necrosante e sindrome do choque toxico. Neste artigo, a autora relata os...