RESUMEN
OBJECTIVE: To determine whether valproic acid (VPA)-associated acute liver failure (ALF; VPA-ALF) explains the poor outcomes after liver transplantation (LT) in children. STUDY DESIGN: Organ Procurement and Transplantation Network data of pediatric patients who underwent LT for VPA-ALF and ALF caused by other drugs (non-VPA-drug-induced acute liver failure [DIALF]) were analyzed. Pre- and post-transplant variables and post-LT survival were compared between VPA-ALF and non-VPA-DIALF. RESULTS: Seventeen children were transplanted for VPA-ALF. Of the 17 children, 82% died within 1 year of LT. Pre- and post-transplant parameters of VPA versus non-VPA-DIALF were comparable with two exceptions. The median alanine aminotransferase level at transplant was remarkably lower in VPA-ALF compared with non-VPA-DIALF (45 versus 1179 IU/L, P = .004). One-year survival probability was worse in VPA-ALF than non-VPA-DIALF (20% versus 69%, P < .0001). Median post-LT survival time for VPA-ALF was 2.8 months. CONCLUSION: Children who underwent LT for VPA-ALF had a significantly lower survival probability than children with non-VPA-DIALF. Current data suggest that VPA-ALF in children represents an "unmasking" of mitochondrial disease. VPA-ALF should be a contraindication for LT, even in the absence of a documented mitochondrial disease.
Asunto(s)
Fallo Hepático Agudo/inducido químicamente , Trasplante de Hígado , Ácido Valproico/envenenamiento , Adolescente , Anticonvulsivantes/envenenamiento , Anticonvulsivantes/uso terapéutico , Niño , Preescolar , Epilepsia/tratamiento farmacológico , Femenino , Estudios de Seguimiento , Humanos , Lactante , Fallo Hepático Agudo/cirugía , Masculino , Estados Unidos , Ácido Valproico/uso terapéuticoRESUMEN
El Síndrome de Stevens Johnson (SSJ), el Síndrome de Superposición (SSJ/ NET) y la Necrólisis Epidérmica Tóxica (NET) son formas clínicas cutáneo-mucosas graves, desencadenadas por fármacos y muy frecuentemente por anticonvulsivantes. Comunicamos el caso de una paciente medicada por su epilepsia, quién presentó un cuadro incipiente de SSJ empeorando rápidamente hasta derivar en la NET. A pesar de la gravedad, la paciente respondió satisfactoriamente a altas dosis de corticoides sistémicos desde el comienzo y por las medidas de soporte del equipo médico multidisciplinario del hospital.
The Stevens Johnson Syndrome, the Overlape Syndrome (SSJ / NET), and the Toxic Epidermal Necrolysis are clinical cutaneous mucouse forms due to drugs administration and most frequently due caused by antiepileptic. We communicate the case of a patient treated with lamotrigina for epilepsy, who presented an incipient case of Stevens Johnson Syndrome, worsened rapidly causing a Toxic Epidermal Necrolysis. Nevertheless, the patient gained a satisfactory answer due to high doses of corticoids, which were given from the beginning as well as the measures and the support of the whole medical team of the hospital.
Asunto(s)
Humanos , Femenino , Adulto , Anticonvulsivantes/envenenamiento , Anticonvulsivantes/efectos adversos , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/complicaciones , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/tratamiento farmacológicoRESUMEN
El síndrome de hipersensibilidad a drogas o síndrome DRESS es una reacción rara y potencialmente fatal; se caracteriza por presentar la tríada de fiebre, rash y compromiso de órganos internos. Fenitoína, fenobarbital y carbamazepina son los anticonvulsivantes aromáticos que con más frecuencia causan esta reacción. Dicho síndrome ocurre entre la 1ra y 8va semanas después de la exposición al fármaco. La eliminación inmediata de la droga es esencial para el manejo de estos pacientes. Presentamos una paciente de sexo femenino, de 62 años, medicada con carbamazepina durante un mes, que consulta por fiebre, mal estado general y rash máculopapuloso que se generalizó hasta abarcar el 90% de la superficie cutánea.
Anticonvulsivant hypersensitivity syndrome (AHS) is a rare and potentially fatal reaction characterized by the appearance of fever, skin rash and internal organ involvement. Phenytoin, phenobarbital and carbamazepine are the most frequent aromatic anticonvulsivant causing the reaction. This syndrome occurs 1-8 weeks after the initial drug exposure.