Journal of clinical anesthesia
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Case Reports
General anesthesia in a patient with citrullinemia using Precedex as an adjunct to prevent delayed emergence.
Citrullinemia type I is a rare autosomal recessive genetic condition that causes reduced activity of the enzyme, argininosuccinate synthase, which is needed for proper urea metabolism. The end result is hyperammonemia which can cause life-threatening neurologic symptoms and global developmental delay. Previous case reports of the anesthetic management of patients with citrullinemia describe delayed recovery possibly related to elevated ammonia levels postoperatively or choice of intraoperative anesthetics which have included narcotics. ⋯ Postprocedure, her ammonia level was less than 9 μmol/L. She appeared to be free of pain, was tolerating gastric tube feeds postprocedure, and was discharged to home on the day of surgery. Effective management of ammonia levels in this patient using preoperative hydration and dexmedetomidine as an adjunct to general anesthesia may have helped to prevent delayed emergence.
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Observational Study
Does laryngoscopic view after intubation predict laryngoscopic view before intubation?
To determine if an endotracheal (ET) tube will distort the laryngeal view obtained with direct laryngoscopy measuring with the modified Cormack-Lehane scale (MCL). ⋯ We performed a prospective observational study to address the predictive value of postintubation laryngoscopy grade in adults. The presence of the ET tube both increased visualization of the glottis and worsened the view in different subjects. The important outcome was that the presence of the ET tube did in fact change the view obtained of the larynx during direct laryngoscopy. In conclusion, postintubation MCL grades may not be reliable to predict laryngeal grade and should be used with caution in the right clinical context.
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We present a catheter related severe hypernatremia in a 2-month-old baby who was admitted to the pediatric intensive care. Imbalance of plasma sodium is commonly seen in pediatric intensive care patients. The water and sodium balance is a complex process. ⋯ Central venous catheterization is frequently used in pediatric intensive care patients. Complications of central venous catheter placement still continue despite the usage of ultrasound guidance. Malposition of central venous catheter in the brain veins should be kept in mind as a rare cause of iatrogenic hypernatremia.
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Case Reports
A rare genetic variant of the ryanodine receptor in a suspected malignant hyperthermia susceptible patient.
Malignant hyperthermia (MH) remains a diagnostic challenge. This case report describes the anesthetic management of a suspected intraoperative MH episode and the subsequent, genetic sequence analysis of 3 genes associated with MH. ⋯ Ala4185Thr), in the RYR1 gene encoding the ryanodine receptor. Although the RYR1 gene has previously been implicated in the pathogenesis of MH, (1) this particular variant has only been reported in one other case of MH; (2) the role for diagnostic genetic testing in the diagnosis of MH will be examined.
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Hemispherectomy is an established surgical procedure to treat medically refractory epilepsy caused by diffuse hemispheric diseases. The most common complication of hemispherectomy is intraoperative bleeding. Perioperative allogeneic blood transfusion increases mortality and morbidity in pediatric patients. ⋯ We present a series of three cases undergoing pediatric hemispherectomy, where a new algorithm was employed to manage coagulation. This new algorithm was mainly based on timely thrombelastogram analyses guided clotting factors supplement and continuous administration of tranexamic acid. In our cases, the amount of blood loss and subsequent allogeneic blood transfusion seemed to be less than literature reported.