ABSTRACT
El síndrome de hipertermia maligna es una miopatía familiar de naturaleza farmacogenética, que se presenta como un síndrome hipercatabólico del músculo esquelético ligado a la anestesia. La incidencia en pediatría es de 1 cada 10 000 cirugías. Puede ser de instalación rápida, asociado a succinilcolina, o tardía, relacionado con agentes inhalatorios. El cuadro ocurre con taquicardia, hipertermia, hipercapnia, acidosis, rigidez muscular, hiperpotasemia, falla renal y arritmia. La mortalidad sin tratamiento específico es del 80% y desciende al 7% con el uso de dantroleno sódico. Reportamos el caso de un paciente de 8 años que se internó para cirugía de fimosis. Por presentar taquicardia, hipercapnia y rigidez muscular, se inició tratamiento con dantroleno sódico en quirófano, el cual se mantuvo durante 72 h. Evolucionó las primeras 12 h con bajo gasto cardíaco, con máximo valor de creatina fosfoquinasa de 155 147 U/L. Permaneció con asistencia mecánica respiratoria por 48 h. Se otorgó alta al sexto día, sin secuelas.
Malignant hyperthermia syndrome is a family myopathy of pharmacogenetic nature, which appears as a skeletal muscle hypercatabolic syndrome linked to anesthesia. The incidence in pediatrics is 1 event per 10 000 surgeries. The clinical picture may have a rapid onset associated with succinylcholine, or a late onset related to inhalation agents. The clinical picture includes tachycardia, hyperthermia, hypercapnia, acidosis, muscle rigidity, hyperkalemia, renal failure and arrhythmia. Mortality without specific treatment is of 80% and drops to 7% with the use of dantrolene sodium. We report an 8-year-old patient admitted for phimosis surgery; having tachycardia, hypercapnia and muscle rigidity, he started treatment with dantrolene sodium in the operating room, which was maintained for 72 hours. He evolved the first 12 hours with low cardiac output and creatine phosphokinase maximum of 155 147 U/L. He remained with mechanical ventilation for 48 hours. Discharge was given on the sixth day without sequelae.
Subject(s)
Humans , Child , Child , Creatine Kinase , Dantrolene , Malignant HyperthermiaABSTRACT
El síndrome de hipertermia maligna es una miopatía familiar de naturaleza farmacogenética, que se presenta como un síndrome hipercatabólico del músculo esquelético ligado a la anestesia. La incidencia en pediatría es de 1 cada 10 000 cirugías. Puede ser de instalación rápida, asociado a succinilcolina, o tardía, relacionado con agentes inhalatorios. El cuadro ocurre con taquicardia, hipertermia, hipercapnia, acidosis, rigidez muscular, hiperpotasemia, falla renal y arritmia. La mortalidad sin tratamiento específico es del 80% y desciende al 7% con el uso de dantroleno sódico. Reportamos el caso de un paciente de 8 años que se internó para cirugía de fimosis. Por presentar taquicardia, hipercapnia y rigidez muscular, se inició tratamiento con dantroleno sódico en quirófano, el cual se mantuvo durante 72 h. Evolucionó las primeras 12 h con bajo gasto cardíaco, con máximo valor de creatina fosfoquinasa de 155 147 U/L. Permaneció con asistencia mecánica respiratoria por 48 h. Se otorgó alta al sexto día, sin secuelas.(AU)
Malignant hyperthermia syndrome is a family myopathy of pharmacogenetic nature, which appears as a skeletal muscle hypercatabolic syndrome linked to anesthesia. The incidence in pediatrics is 1 event per 10 000 surgeries. The clinical picture may have a rapid onset associated with succinylcholine, or a late onset related to inhalation agents. The clinical picture includes tachycardia, hyperthermia, hypercapnia, acidosis, muscle rigidity, hyperkalemia, renal failure and arrhythmia. Mortality without specific treatment is of 80% and drops to 7% with the use of dantrolene sodium. We report an 8-year-old patient admitted for phimosis surgery; having tachycardia, hypercapnia and muscle rigidity, he started treatment with dantrolene sodium in the operating room, which was maintained for 72 hours. He evolved the first 12 hours with low cardiac output and creatine phosphokinase maximum of 155 147 U/L. He remained with mechanical ventilation for 48 hours. Discharge was given on the sixth day without sequelae.(AU)
ABSTRACT
Malignant hyperthermia syndrome is a family myopathy of pharmacogenetic nature, which appears as a skeletal muscle hypercatabolic syndrome linked to anesthesia. The incidence in pediatrics is 1 event per 10 000 surgeries. The clinical picture may have a rapid onset associated with succinylcholine, or a late onset related to inhalation agents. The clinical picture includes tachycardia, hyperthermia, hypercapnia, acidosis, muscle rigidity, hyperkalemia, renal failure and arrhythmia. Mortality without specific treatment is of 80% and drops to 7% with the use of dantrolene sodium. We report an 8-year-old patient admitted for phimosis surgery; having tachycardia, hypercapnia and muscle rigidity, he started treatment with dantrolene sodium in the operating room, which was maintained for 72 hours. He evolved the first 12 hours with low cardiac output and creatine phosphokinase maximum of 155,147 U/L. He remained with mechanical ventilation for 48 hours. Discharge was given on the sixth day without sequelae.
Subject(s)
Malignant Hyperthermia/diagnosis , Child , Dantrolene , Humans , MaleABSTRACT
Malignant hyperthermia syndrome is a family myopathy of pharmacogenetic nature, which appears as a skeletal muscle hypercatabolic syndrome linked to anesthesia. The incidence in pediatrics is 1 event per 10 000 surgeries. The clinical picture may have a rapid onset associated with succinylcholine, or a late onset related to inhalation agents. The clinical picture includes tachycardia, hyperthermia, hypercapnia, acidosis, muscle rigidity, hyperkalemia, renal failure and arrhythmia. Mortality without specific treatment is of 80
and drops to 7
with the use of dantrolene sodium. We report an 8-year-old patient admitted for phimosis surgery; having tachycardia, hypercapnia and muscle rigidity, he started treatment with dantrolene sodium in the operating room, which was maintained for 72 hours. He evolved the first 12 hours with low cardiac output and creatine phosphokinase maximum of 155 147 U/L. He remained with mechanical ventilation for 48 hours. Discharge was given on the sixth day without sequelae.
ABSTRACT
Las anormalidades congénitas del desarrollo de los vasos linfáticos son infrecuentes, subdiagnosticadas y de difícil manejo clínico. Se presenta un caso clínico sobre Síndrome de Displasia Linfática que incluye linfedema (idiopático), quilotórax, derrames idiopáticos (pericárdico, pleural, peritoneal y linfedema sin causa identificable como trauma o cáncer) y linfangiectasia intestinal con síndrome de mala absorción. La alteración linfática se debe tener en cuenta al existir mala evolución frente a un síndrome ascítico edematoso. La combinación de tratamiento médico, quirúrgico y fisioterapéutico resulta de mayor beneficio para aquellos con marcado linfedema
Subject(s)
Humans , Child, Preschool , Ascitic Fluid , Hyperaldosteronism , Lymphangiectasis, Intestinal , Lymphedema , Chylothorax/diagnosis , Chylothorax/therapy , Lymphatic System/abnormalitiesABSTRACT
Las anormalidades congénitas del desarrollo de los vasos linfáticos son infrecuentes, subdiagnosticadas y de difícil manejo clínico. Se presenta un caso clínico sobre Síndrome de Displasia Linfática que incluye linfedema (idiopático), quilotórax, derrames idiopáticos (pericárdico, pleural, peritoneal y linfedema sin causa identificable como trauma o cáncer) y linfangiectasia intestinal con síndrome de mala absorción. La alteración linfática se debe tener en cuenta al existir mala evolución frente a un síndrome ascítico edematoso. La combinación de tratamiento médico, quirúrgico y fisioterapéutico resulta de mayor beneficio para aquellos con marcado linfedema
