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.

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.(AU)

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.

Síndrome de Reye. Descripción de un caso con especial interés en sus crisis epilépticas / Reye's syndrome. Description of a case focused on the patient's epileptic seizures

Introducción. El síndrome de Reye es una enfermedad aguda caracterizada por encefalopatía y degeneración grasa del hígado que ocurre casi exclusivamente en niños. Puede dar lugar al fallecimiento del paciente hasta en un tercio de los casos, generalmente por edema cerebral grave. La etiopatogenia de este cuadro es incierta, suele precederse de una infecciónviral generalmente por el virus de influenza o varicela. Hay estudios que han demostrado una fuerte asociación epidemiológica entre la ingestión de ácido acetilsalicílico (AAS) durante la infección viral y el desarrollo del síndrome de Reye. Caso clínico.Niña de 20 meses que desarrolla un síndrome de Reye en el contexto de cuadro viral e ingesta de AAS. La biopsia hepática es congruente con el síndrome. Presenta un estatus no convulsivo durante la fase aguda y al año desarrolla un síndrome de Lennox-Gastaut. Fallece a los 18 años por neumonía. Conclusiones. En todo paciente con clínica sugerente de síndrome de Reye, habrá que descartar errores congénitos del metabolismo que lo pueden imitar. Aunque la incidencia de este síndrome hadisminuido considerablemente en los últimos años, es importante tenerlo aún en mente, ya que un diagnóstico y tratamiento precoz y agresivo de la hipertensión cerebral disminuirá la mortalidad y las secuelas

Introduction. Reye’s syndrome is an acute disease characterised by encephalopathy and fatty degeneration of the liver that occurs almost exclusively in children. It can cause the death of the patient in up to a third of all cases, generally due to severe cerebral oedema. The aetiopathogenesis of this condition is uncertain and is usually preceded by a viral infection,generally from the influenza or varicella virus. Some studies have shown a strong epidemiological association between the ingestion of acetylsalicylic acid (ASA) during the viral infection and development of Reye’s syndrome. Case report. We describe the case of a 20-month-old female who developed Reye’s syndrome within the context of a viral infection and theingestion of ASA. A hepatic biopsy study is appropriate in this syndrome. The patient presented a non-convulsive status during the acute phase and at one year developed Lennox-Gastaut syndrome. She died from pneumonia at the age of 18 years. Conclusions. In all patients with clinical features that suggest Reye’s syndrome, inborn errors of metabolism that can mimic it must be precluded. Although the incidence of this syndrome has gone down considerably in recent years, it is important to keep it in mind as an early and aggressive diagnosis and treatment of cerebral hypertension will reduce the mortality rate and the sequelae

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.

Determinación de valores normales de acilcarnitinas en una población infantil sana como herramienta diagnóstica de errores hereditarios de la Beta-oxidación mitocondrial de los ácidos grasos / Determination of normal acylcarnitine levels in a healthy pediatric population as a diagnostic tool in inherited errors of mitochondrial fatty acid Beta-oxidation

Introducción La determinación de acilcarnitinas en sangre es una prueba útil en el diagnóstico de los errores hereditarios de la β-oxidación mitocondrial de los ácidos grasos. Sin embargo, existen pocos datos en la literatura médica, relacionados con valores de referencia para acilcarnitinas y si esos valores dependen de la edad o el sexo. Objetivos Llamar la atención acerca de los errores innatos de la β-oxidación mitocondrial de los ácidos grasos y establecer valores de referencia para acilcarnitinas en niños. Pacientes y métodos Fueron tomadas muestras de sangre de 309 niños normales divididos en cuatro grupos de edad (grupo A, < 1 mes; grupo B, 1-12 meses; grupo C, 1-7 años; grupo D, 7-18 años) y fueron analizadas por espectrometría de masas en tándem. Resultados y discusión Se aportan valores de referencia para acilcarnitinas en niños. No se encontraron diferencias estadísticamente significativas relacionadas con la edad o el sexo. Nuestros resultados son diferentes cuando se comparan con los de la literatura médica encontrada. Es importante destacar la ausencia de hidroxiacilcarnitinas y glutarilcarnitina cuando se procesan muestras normales. Revisamos la bibliografía relacionada con los principales hallazgos clínicos y de laboratorio en las deficiencias de la β-oxidación mitocondrial de los ácidos grasos

Introduction Acylcarnitine measurement in blood is a useful test for the diagnosis of inherited errors of mitochondrial fatty acid β-oxidation. However, there are few data in the literature on the reference ranges of the various acylcarnitines and on whether these reference ranges are age- or sex-dependent. Objectives To draw attention to inherited errors of mitochondrial fatty acid β-oxidation and to establish reference acylcarnitine values in children. Patients and methods A total of 309 blood samples from healthy children divided into four age groups (group A: < 1 month; group B: 1-12 months; group C: 1-7 years; group D: 7-18 years) were obtained and analyzed using tandem mass spectrometry. Results and conclusion Reference acylcarnitine values in children are provided. No significant differences were found in relation to age or sex. Our results differ from those reported in the literature reviewed. Importantly, hydroxyacylcarnitines and glutaryl carnitine are absent when normal samples are processed. We review the literature on the main clinical and laboratory findings in mitochondrial fatty acid β-oxidation deficiencies

Síndrome de Reye en paciente pediátrico / Reye's syndrome in a pediatric patient

No disponible

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.