Phosphorylase kinase (PhK) deficiency causing glycogen storage disease type IX (GSD IX) results from deficiency of the enzyme phosphorylase b kinase, which has a major regulatory role in the breakdown of glycogen. The two types of PhK deficiency are liver PhK deficiency (characterized by early childhood onset of hepatomegaly and growth restriction, and often, but not always, fasting ketosis and hypoglycemia) and muscle PhK deficiency, which is considerably rarer (characterized by any of the following: exercise intolerance, myalgia, muscle cramps, myoglobinuria, and progressive muscle weakness). While symptoms and biochemical abnormalities of liver PhK deficiency were thought to improve with age, it is becoming evident that patients need to be monitored for long-term complications such as liver fibrosis and cirrhosis.

Summary A 9 year-old boy suffering from cough for half a month, fever for 4 days was hospitalized. CT three-dimensional reconstruction showed that a metallic needle-like foreign body within the right main bronchial with pneumonia and atelectasis. In the next day, the boy took examination using an open-tube rigid bronchoscope under general anesthesia. For the first time, the foreign body was jammed in glottis with SpO₂ and HR declining. For the second time, the operator found the foreign was not a needle but a thumbtack. Finally, the thumbtack was removed from the airway after short-term paralysis using succinylcholine.

Malignant hyperthermia is an uncommon, but potentially lethal condition that may be encountered during the perioperative period. There is wide variability in the manner in which malignant hyperthermia may manifest. For a patient to survive a malignant hyperthermia crisis, prompt recognition and treatment is of paramount importance. Perioperative nurses play a pivotal role in the successful management of malignant hyperthermia. The fictitious case study presented in this paper describes the identification, presentation, pathophysiology, and treatment of a general anesthesia patient with fulminant malignant hyperthermia.

Intracranial and Hemodynamic Changes after Succinylcholine Administration in Cats. By Cottrell JE, Hartung J, Giffin JP, and Shwiry B. Anesthesia & Analgesia 1983; 62:1006-9. Reprinted with permission.Bolus injections of succinylcholine (1.5 mg/kg) significantly increased intracranial pressure (ICP) in cats under normal conditions from control levels of 8 +/- 1 mmHg to 16 +/- 3 mmHg (+/- SEM, P less than 0.01), and in the presence of artificially increased ICP from control levels of 27 +/- 1 mmHg to 47 +/- 4 mmHg (P less than 0.01). These approximately 100% increases in ICP were accompanied by a transitory decrease in mean arterial pressure (approximately 10 s), followed by a 15 to 20% increase (P less than 0.05). Pulmonary arterial pressure increased 20 to 30% (P less than 0.05). These results, when considered in conjunction with results previously obtained in humans, suggest that succinylcholine may be contraindicated in neurosurgical patients.

Masseter muscle rigidity is a known complication of drugs such as succinylcholine and volatile agents. However, muscle rigidity is an uncommon complication of propofol. We report the case of a 56-year-old ASA-PS class I woman refereeing for colonoscopy due to chronic constipation under deep intravenous sedation. She suffered masseter spasm after the injection of propofol. Masseter spasm should not be considered limited to special drug groups. In any case of difficult mouth opening, masseter spasm should be kept in mind and deepening of anesthesia or complete blockade of neuromuscular junction should be considered by the use of non-depolarizing muscle relaxants.

Malignant hyperthermia is a rare condition that occurs in susceptible patients exposed to triggering anesthetic agents. It is associated with a high mortality rate if not recognized immediately and treated appropriately. A 52-year-old man presented to our clinic 2 days after an assault for management of jaw pain. A minimally displaced right parasymphyseal fracture and moderately displaced left body fracture of the mandible were diagnosed. There were no known drug allergies. The patient reported no previous difficulty with anesthesia, as well as no known prior adverse reactions to anesthesia in any relatives. The planned surgical intervention was open reduction-internal fixation of bilateral mandibular fractures. The patient received succinylcholine and desflurane during the procedure. A full 70 minutes elapsed before initial signs of hypermetabolism were noted, namely a rise in end-tidal carbon dioxide level. The patient received dantrolene sodium approximately 120 minutes after induction of anesthesia. Signs of hypermetabolism began to abate within 45 minutes of commencement of the malignant hyperthermia treatment protocol. He was subsequently transferred to the surgical intensive care unit for continued management and had a favorable postoperative course. This case underscores the importance of awareness of malignant hyperthermia and its presentation. This condition carries a potential high risk of complications after exposure to triggering anesthetic agents. Taking a complete and detailed history may help to identify potential cases. In this case, it was subsequently discovered that the patient's biological sister had a nearly fatal reaction to general anesthesia several years before this incident. Intraoperative vigilance in the monitoring of vital signs cannot be overemphasized. An increase in end-tidal carbon dioxide values, in addition to other clinical signs that cannot be easily attributed to other causes, should increase the clinical index of suspicion for a diagnosis of malignant hyperthermia.

There is a growing body of literature documenting the use of deep neuromuscular block (NMB) during surgery. Traditional definitions of depth of NMB rely on train-of-four assessment, which can be less reliable in retrospective studies. The goal of our study was to investigate the real-world practice pattern of dosing of neuromuscular blocking agents (NMBA), utilizing the amount of NMBA used during the course of a case, adjusted for patient weight and case duration, as a surrogate measure of depth of NMB. We also aimed to identify case factors associated with larger NMBA doses. In this retrospective observational analysis of our anesthesia information management system, we analyzed all general endotracheal anesthesia cases from 2012 to 2015 in which an intermediate-acting NMBA was used. Cases using a long-acting NMBA or only succinylcholine were excluded. The expected duration of the case was calculated based on the cumulative dose of NMB used, normalized to the patient's ideal body weight and the ED95 of the drug. If the expected duration of the case was greater than the actual case duration documented in the case record, it was classified as higher dosing (HD). If the expected duration was equal to or less than the actual duration, it was considered predicted dosing (PD). Categorical comparisons between HD and PD groups were made for various patient, procedural, and provider factors. 72,684 cases were included in the final analysis, of which 46,358, or 64% of cases, used HD. Cases with patients who were morbidly obese, younger than 65 years, and who were lower ASA Physical Status classification (I or II) used more HD as opposed to PD. Cases that were non-open, used total intravenous anesthesia, emergent cases, or used non-rapid sequence anesthesia induction had higher rates of HD than their matched counterparts. All results were statistically significant. HD was more common in cases that documented train-of-four and used the reversal agent neostigmine. Approximately two-thirds of general endotracheal anesthesia cases using an intermediate-acting NMBA used HD. Cases with higher rates of HD may be those that are traditionally technically complex or emergent, would benefit from greater paralysis, or do not use adjunctive medications for muscle relaxation. Age greater than 65 years was shown to have lower rates of HD, likely due to provider awareness of age-related changes in pharmacokinetics and pharmacodynamics. Intraoperative monitoring and NMB antagonism with neostigmine were used more frequently with HD.

Malignant hyperthermia (MH) is a life-threatening hypermetabolic state that can occur following induction with depolarizing neuromuscular blockade and volatile anesthesia gases. Because succinylcholine is a common choice for prehospital and emergency department inductions, it is important for staff to be able to recognize and effectively treat an MH crisis. This case study highlights a 20-year-old male trauma patient who presented to a Level I trauma center and was intubated for declining mental status. He developed suspected MH following his anesthetic induction with succinylcholine. The following outlines the case, clinical identification of MH, and treatment.