ObjectiveTo assess the impact of service improvements implemented because of latent threats (LTs) detected during in situ simulation.DesignRetrospective review from April 2008 to April 2015.SettingPaediatric Intensive Care Unit in a specialist tertiary hospital.InterventionService improvements from LTs detection during in situ simulation. Action plans from patient safety incidents (PSIs).Main outcome measuresThe quantity, category, and subsequent service improvements for LTs. The quantity, category, and subsequent action plans for PSIs. Similarities between PSIs and LTs before and after service improvements.Results201 Simulated inter-professional team training courses with 1,144 inter-professional participants. 44 LTs were identified (1 LT per 4.6 courses). Incident severity varied: 18 (41%) with the potential to cause harm, 20 (46%) that would have caused minimal harm, and 6 (13%) that would have caused significant temporary harm. Category analysis revealed the majority of LTs were resources (36%) and education and training (27%). The remainder consisted of equipment (11%), organizational and strategic (7%), work and environment (7%), medication (7%), and systems and protocols (5%). 43 service improvements were developed: 24 (55%) resources/equipment; 9 (21%) educational; 6 (14%) organizational changes; 2 (5%) staff communications; and 2 (5%) guidelines. Four (9%) service improvements were adopted trust wide. 32 (73%) LTs did not recur after service improvements. 24 (1%) of 1,946 PSIs were similar to LTs: 7 resource incidents, 7 catastrophic blood loss, 4 hyperkalaemia arrests, 3 emergency buzzer failures, and 3 difficulties contacting staff. 34 LTs (77%) were never recorded as PSIs.ConclusionAn in situ simulation program can identify important LTs which traditional reporting systems miss. Subsequent improvements in workplace systems and resources can improve efficiency and remove error traps.

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OBJECTIVES:Greater congenital heart disease (CHD) complexity is associated with lower health-related quality of life (HRQOL). There are no data on the association between surgical and ICU factors and HRQOL in CHD survivors. This study assess the association between surgical and ICU factors and HRQOL in child and adolescent CHD survivors. DESIGN:This was a corollary study of the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study. SETTING:Eight pediatric hospitals participating in the PCQLI Study. PATIENTS:Patients in the study had the Fontan procedure, surgery for tetralogy of Fallot (TOF), and transposition of the great arteries (TGAs). MEASUREMENTS AND MAIN RESULTS:Surgical/ICU explanatory variables were collected by reviewing the medical records. Primary outcome variables (PCQLI Total patient and parent scores) and covariates were obtained from the Data Registry. General linear modeling was used to create the multivariable models. There were 572 patients included: mean ± sd of age 11.7 ± 2.9 years; CHD Fontan 45%, TOF/TGA 55%; number of cardiac surgeries 2 (1-9); and number of ICU admissions 3 (1-9). In multivariable models, lowest body temperature on cardiopulmonary bypass (CPB) was negatively associated with patient total score (p < 0.05). The total number of CPB runs was negatively associated with parentreported PCQLI Total score (p < 0.02). Cumulative days on an inotropic/vasoactive drug in the ICU was negatively associated with all patient-/parent-reported PCQLI scores (p < 0.04). Neurological deficit at discharge was negatively associated with parent-reported PCQLI total score (p < 0.02). The variance explained by these factors ranged from 24% to 29%. CONCLUSIONS: Surgical/ICU factors, demographic, and medical care utilization variables explain a low-to-moderate amount of variation in HRQOL. Research is needed to determine whether modification of these surgical and ICU factors improves HRQOL, and to identify other factors that contribute to unexplained variability.

Background We report a clinical-biochemical findings in a child who presented with acute hyperammonemic encephalopathy. Methods Case notes and electronic record was used to collect data Results A 21 month old male child presented to accident and emergency with history of diarrhoea and vomiting for 2 days, but became progressively lethargic, unresponsive at home with grunty respirations.On arrival to A and E he was noted to be poorly perfused, with reduced consciousness. Needed 30 ml/kg fluid bolus through intra-osseous route. He had metabolic acidosis which was partially corrected with the fluid bolus. After a brief period of being stable on the ward he became suddenly unresponsive, tachyopneic needing intubation and mechanical ventilation and transfer to PICU.At PICU he had had further bloods including ammonia in view of acidosis, the ammonia was significantly elevated at 850 mmol/L along with elevated lactates.He was commenced on emergency regime of sodium benzoate, sodium phenyl butyrate along with arginine and carglumic acid. He also needed haemofiltration to manage hyperammonemia. His ammonia normalized over 18 hours.Plasma amino acid showed high levels of glutamine, tyrosine, lysine and aspartate with low citrulline.Urinary organic acids demonstrated increased lactic acid, large increase in 3 hydroxybutyrate and acetoacetate with appropriate increase in MCFA(medium chain fatty acids)-normal response to fasting or hypoglycemia.However DNA analysis did not confirm a diagnosis of OTC deficiency. He has been discussed with Great Ormond St team who opine that OTC deficiency is very likely, however as per their advice DNA has been sent for hyperammonemia extended metabolic panel.He has an emergency plan in place if becomes unwell and has an emergency kit containing essential drugs. Conclusion Urea cycle disorders should be considered in the differential diagnosis of patients who present with symptoms of acute hyperammonemia. If a urea cycle disorder is being considered, treatment should be started prior to confirmation of the diagnosis. Only by clinical suspicion and careful investigation will inborn errors of metabolism be uncovered and treated in a timely manner.