Anaesthesia Challenges for a Patient with Phosphoglycerate Kinase Deficiency Undergoing Open Gastrostomy Procedure: A Case Report.

Phosphoglycerate kinase deficiency (PGK1D) is a rare X-linked metabolic disorder with variable phenotype. Mutations of the PGK1 gene result in clinically variable spherocytic hemolytic anemia and variable defects in the central nervous system. Rhabdomyolysis, myopathy, migraine, and retinal involvement have also been reported as clinical consequences. We describe for the first time the anaesthetic management of a patient with the X-linked phosphoglycerate kinase deficiency scheduled for an open gastrostomy procedure to facilitate enteral nutrition due to chronic oral aversion. Given the various organs systems involved, we suggest a number of preoperative investigations and describe our intraoperative management. Given the paucity of literature regarding children with this condition, we believe this case report will be a worthwhile addition to the anaesthetic literature to help guide other anaesthetists managing patients with this condition.

Opioid-related harm in a quaternary pediatric hospital: A 5-year review.

BACKGROUND: Opioid therapy in pediatrics may be particularly prone to error, yet the incidence of opioid-related medication error and harm has not yet been described in the pediatric inpatient setting. METHODS: We reviewed a prospectively compiled medication safety database from November 1, 2012 to October 31, 2017. Reports originated from voluntary reporting, hospital code events, naloxone administrations, and reports of unexpected experiences of patient pain. Time, location, error characteristics, drug, route, prescription, error phase, mechanisms, harm, and outcome were collected for all reports. Error and harm were classified by the National Coordinating Council for Medication Error Reporting and Prevention (NCC MERP) system. RESULTS: Over 697 opioid medication safety reports were included during the study period. Opioids were administered at a rate of 79.26 administrations per 100 patient bed days, with morphine and hydromorphone administered at 62 versus 15 administrations per 100 bed days, respectively. Overall error rate was 0.94 errors per 103 patient days. Although the absolute rate of error reporting was greater for morphine (0.65 errors reported per 103 opioid administrations) than for hydromorphone, the adjusted incidence of harm was 0.211 per 103 hydromorphone administrations compared to 0.086 per 103 morphine administrations. 47 opioid errors resulted in harm, and administration errors (29) were almost twice as common as prescribing errors (15). CONCLUSIONS: We report and aim to establish a comparative reference point for incidence of opioid-related error and harm adjusted for both hospital bed days and total opioid administrations within the pediatric hospital inpatient setting based on the above findings.

A retrospective observational study of acquired subglottic stenosis using low-pressure, high-volume cuffed endotracheal tubes.

INTRODUCTION: The safety of cuffed endotracheal tubes in the neonatal and critically ill pediatric population continues to be questioned due to the theoretical risk of acquired subglottic stenosis. The incidence of acquired subglottic stenosis in the high-risk mixed surgical and medical critically ill pediatric cohort using high-volume, low-pressure cuffed endotracheal tube policy has not yet been described. The aim of our study was to describe and evaluate the use and complication rate of cuffed ETT's in our unit over a 5-year period. METHODS: We defined clinically significant subglottic stenosis as a positive stenotic finding of endotracheal tube-related pathology on a microlaryngoscopy within 6 months of invasive ventilation. All patients admitted through our pediatric critical care unit from January 10, 2012 to January 25, 2017 were matched against our theater management system database for the same period. We reviewed all matching patients' baseline demographics, comorbidities, intubation/endotracheal tube history, and subsequent surgical management. RESULTS: Of 5309 pediatric critical care unit admissions (61% ventilated) and 1251 microlaryngoscopies, 23 children had endoscopic findings of clinically significant endotracheal tube-related pathology, reflecting 0.68% of all intubated patients. Eight patients developed acquired subglottic stenosis. All those requiring major surgical correction were ex-premature neonates initially intubated with uncuffed tubes in an external neonatal intensive care. No patient initially intubated with a cuffed endotracheal tube developed subglottic stenosis requiring surgical correction. CONCLUSION: We report no single case of acquired subglottic stenosis in our cohort that required major surgical correction from a cuffed endotracheal tube during a 5-year period. The introduction of a policy of appropriate placement and maintenance of low-pressure, high-volume cuffed endotracheal tubes in the pediatric critical care unit was not associated with an increased rate of endotracheal tube-related subglottic trauma.

The College of Anaesthetists of Ireland Simulation Training programme: a descriptive report and analysis of course participants' feedback.

BACKGROUND: Simulation-based education is a modern training modality that allows healthcare professionals to develop knowledge and practice skills in a safe learning environment. The College of Anaesthetists of Ireland (CAI) was the first Irish postgraduate medical training body to introduce mandatory simulation training into its curriculum. Extensive quality assurance and improvement data has been collected on all simulation courses to date. AIMS: Describe The College of Anaesthetists of Ireland Simulation Training (CAST) programme and report the analysis of course participants' feedback. METHODS: A retrospective review of feedback forms from four simulation courses from March 2010 to August 2016 took place. Qualitative and quantitative data from 1069 participants who attended 112 courses was analysed. RESULTS: Feedback was overall very positive. Course content and delivery were deemed to be appropriate. Participants agreed that course participation would influence their future practice. A statistically significant difference (P < 0.001) between self-reported pre- and post-course confidence scores was observed in 19 out of 25 scenarios. The learning environment, learning method and debrief were highlighted as aspects of the courses that participants liked most. CONCLUSIONS: The mandatory integration of CAST has been welcomed with widespread enthusiasm among specialist anaesthesia trainees. Intuitively, course participation instils confidence in trainees and better equips them to manage anaesthesia emergencies in the clinical setting. It remains to be seen if translational outcomes result from this increase in confidence. Nevertheless, the findings of this extensive review have cemented the place of mandatory simulation training in specialist anaesthesia training in Ireland.

Suitability of the forearm for non-invasive blood pressure measurement in children.

BACKGROUND: Measurement of forearm blood pressure (BP) in pediatric patients during general anesthesia is periodically employed despite a lack of evidence for this practice. Upper arm BP measurement may be impossible to perform for either patient or surgical reasons, and the forearm has theoretical benefits over the lower leg when an alternate site is required. We hypothesize that forearm BP measurement provides an accurate and reliable alternative to the upper arm. Published adult data do not support this hypothesis, and the little pediatric data published contain methodological shortcomings. METHODS: A dedicated, externally calibrated noninvasive oscillometer was used to compare BP measurements in the upper arm and ipsilateral forearm of pediatric patients undergoing general anesthesia prior to application of a surgical stimulus. Both upper arm BP and ipsilateral forearm BP were sequentially measured 20 seconds apart on 3 separate occasions with an appropriately sized cuff. The systolic, diastolic, and mean blood pressures were recorded under steady-state conditions. RESULTS: Thirty-five elective surgical patients aged 1 to 10 years were studied. The bias (±limits of agreement) for forearm minus upper arm blood pressures were as follows: mean BP -1.3 mm Hg (±7.2), diastolic BP -3.3 (±5.3), and systolic BP +3.2 mm Hg (±8.3). Differences greater than ±5 mm Hg occurred in 59% (systolic BP), 42% (diastolic BP), and 46% (mean BP) of all observations and greater than ±10 mm Hg in 17% (systolic BP), 8.6% (diastolic BP), and 15% (mean BP). CONCLUSION: The differences within mean ±1.96 standard deviations reside considerably outside the clinically accepted tolerance of ±5 mm Hg. Thus, the forearm may not be used interchangeably with upper limb BP readings in anesthetized healthy children. Future use of the forearm for BP measurement requires a validated anthropomorphically appropriate forearm cuff.

Life-Threatening Lactation or "Bovine" Ketoacidosis: A Case Report.

Lactation or "bovine" ketoacidosis is a rare cause of raised anion gap metabolic acidosis whereby a perfect storm of negative calorie balance (starvation/glucose preferentially used for milk production) and insulin resistance (counter regulatory stress hormone release/infection) leads to a dysregulated ketogenic state. We present a case of life-threatening lactation-related ketoacidosis in a patient 9 weeks postpartum, who presented to the emergency department with an arterial pH of 6.88, HCO3 of 5.8 mmol/L and blood ketone level of 5.8 mmol/L. Treatment consists of aggressive glucose loading, triggering supraphysiologic endogenous insulin release, and subsequent inhibition of ketone body formation.