Sindrome de hipertermia maligna: a propósito de un caso clínico / Malignant hyperthermia syndrome: case report

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.

Malignant hyperthermia during general anesthesia for a surgery of idiopathic scoliosis: A case report / 대한마취과학회지

Malignant hyperthermia is a potentially fatal hypermetabolic syndrome characterized by skeletal muscle rigidity and hyperpyrexia. We had an 11-year-old female presented for a surgery for idiopathic scoliosis of the thoracic vertebra. Generalized muscle rigidity and tachycardia were revealed 1 hour 35 minutes after isoflurane administration. Then the body temperature was risen to 43degrees C within 10 minutes and the arterial blood gas analysis showed a severe respiratory and metabolic acidosis. Under the suspicion of malignant hyperthermia, the patient was treated by discontinuing all anesthetic agents, hyperventilation with 100% oxygen, surface cooling with ice pack and the administration of dantrolene sodium. But, the patient died of renal failure and disseminated intravascular coagulation after 50 days postoperatively.

The Effect of Intrauterine Irradiation on Cortical Neurons and Glial Cells / 대한소아신경학회지

PURPOSE: Our study was an experimental model for the mechanism of cortical dysplasia. We examined the changes of neuronal cells and glial cells by intrauterine irradiation. This paper will elucidate the effect of these changes on the development of cortical dysplasia. METHODS: The cytotoxic effect of irradiation was examined by viability and numbers in cerebral cortical neurons and glial cells, which were derived from a mouse exposed to 225 cGy of gamma-irradiation on embryonic day 17. In addition, the protective effect of an inhibitor of intracellular calcium release, dantrolene sodium(DS), on irradiation-induced neurotoxicity was examined after DS(10 mg/kg) was administrated via intraperitoneal injection after intrauterine irradiation. RESULTS: 1) Irradiation induced the decrement of the cell number and cell viability of cerebral cortical neurons in the developing stages. 2) The number of glial cells in the mouse treated with intrauterine irradiation was increased in E20-P4 stages compared with the control group, but there was no difference in cell viability. 3) The glial fibrillary acidic protein(GFAP)-positive cells were seen in developing stages (E20-P4). 4) In the protective effect from neuronal cell death by intrauterine irradiation, DS attenuated cell death by an increase of neuronal cells. CONCLUSION: From these results, it is suggested that intrauterine irradiation has the neurotoxic effect as neuronal cell death and induced glial cell proliferation. A selective inhibitor of intracellular calcium release such as DS is effective in protecting neuronal cell death induced by irradiation of the intrauterine period. Cortical dysplasia induced by intrauterine irradiation may be involved in neuronal cell death and the hyperproliferation of glial cells. Intracellular calcium influx may contribute to the pathogenesis of irradiation-induced neuronal cell death.

Malignant Hyperthermia during General Anesthesia and Anesthetic Management for That Same Patient in Day Surgery Center: A case report / 대한마취과학회지

Malignant hyperthermia is a potentially fatal hypermetabolic syndrome characterized by hyperpyrexia and skeletal muscle rigidity. We experienced a case of malignant hyperthermia after general anesthesia with halothane and succinylcholine in day surgery center. 2 years old male patient arrived to get congenital ptosis operation. He was relatively healthy and had no considerable past or family history of hereditary disease. Anesthesia induced with halothane inhalation and succinylcholine. After induction, he abruptly showed masseter muscle rigidity, total body rigidity, elevation of end-tidal CO2 tension, tachycardia and hyperthermia. Under the suspicion of malignant hyperthermia, all anesthetics were discontinued and vigorous emergency treatment was attemped with dantrolene sodium. The patient survived without any sequele and discharged after 11 days. About 10 months later, that same patient comes to our hospital to take the operation. We experienced successful anesthetic management for malignant hyperthermia.

Suspicious Malignant Hyperthermia in Child during General Anesthesia: A case report / 대한마취과학회지

We had a 16-month-old male presented for a surgery for degloving injury of left lower extremity. He had no considerable past or family history. High fever and increased end-tidal CO2 with tachycardia was revealed after induction of general anesthesia with thiopental sodium, vecuronium, isoflurane-N2O and O2. Arterial blood gas analysis showed a severe mixed acidosis. Under the suspicion of malignant hyperthermia, all anesthetics were discontinued. Anesthesia was maintained with fentanyl and midazolam. The patient was managed with surface cooling, gastric lavage with cold saline, sodium bicarbonate and diuretics. Dantrolene sodium 50 mg was administered. The patient survived without any sequelae and discharged after 9 days. The etiologic factors, incidence, clinical features, prevention and treatments of malignant hyperthermia are discussed.

Effects of Dantrolene on the Interaction of Calcium and Erythrocyte Membrane

Dantrolene sodium in vitro inhibited the ATP-dependent efflux of calcium from human Fed cells, the Ca++-ATPase activity of red blood cell membrane fragments (RBCMF) and passive calcium binding to RBCMF. These effects were obtained With concentrations of dantrolene sodium between 2.5 and 20 uM. However the passive influx of Ca++ was measured at 37 degrees C in cells pretreated to abolish Ca++ pumping and was not influenced by dantrolene sodium. From these results, it was concluded that dantrolene sodium inhibits an active Ca++ extrusion across the red cell membrane by inhibiting Ca++-ATPase activity which is intimately involved with the Ca++ transport mechanism in the red cell membrane.