Introduction: Inborn errors of metabolism (IEM) can present as acute metabolic emergencies resulting in significant morbidity or death. Good intensive care supportive management and specific metabolic crisis treatment by tertiary pediatric intensive care unit (PICU) play a crucial role in optimizing the outcomes. Objectives: We aimed to study the clinical profile and outcome of children with IEM presenting as an acute metabolic crisis in a tertiary PICU. Methods: This retrospective descriptive study was conducted in a tertiary care center in south India between June 2016 and December 2018. We included children admitted in PICU as medical emergency and diagnosed to have IEM either earlier or at the time of PICU presentation by biochemical testing (basic testing and tandem mass spectrometry [TMS], gas chromatography-mass spectrometry [GCMS] and high-performance liquid chromatography [HPLC], and/or specialized testing) and/or molecular analysis. Clinical profile, details of diagnostic workup, and outcome were collected and analyzed. Results: Out of 2815 children admitted in PICU, 15 had IEM (0.9%). Median (interquartile range) age of presentation was 15 months (10-30 months). Consanguinity was found in 80%. The most common disorder was protein metabolic disorder. Seven patients were diagnosed in the newborn period, five during evaluation for developmental delay before PICU admission, and three were newly diagnosed during PICU admission. Supportive therapy of invasive/non-invasive ventilation and peritoneal dialysis was initiated in 10 and 4, respectively. Special formula was started in nine patients. Five (33%) died in spite of intensive care management. TMS, GCMS, and HPLC yielded definitive diagnosis in 12 (80%) patients. Molecular analysis was done in 12 patients. Conclusion: The most common cause for metabolic crisis in PICU is due to protein metabolic disorder. Aggressive intensive care and IEM directed therapy can be lifesaving, but still, the mortality is high.

Objective: Rapid advancements in medicine and changing standards in medical education require new, efficient educational strategies. We investigated whether an online intervention could increase residents’ knowledge and improve knowledge retention in mechanical ventilation when compared with a clinical rotation and whether the timing of intervention had an impact on overall knowledge gains. Design: A prospective, interventional crossover study conducted from October 2015 to December 2017. Setting: Multicenter study conducted in 33 PICUs across eight countries. Subjects: Pediatric categorical residents rotating through the PICU for the first time. We allocated 483 residents into two arms based on rotation date to use an online intervention either before or after the clinical rotation. Interventions: Residents completed an online virtual mechanical ventilation simulator either before or after a 1-month clinical rotation with a 2-month period between interventions. Measurements and Main Results: Performance on case-based, multiple-choice question tests before and after each intervention was used to quantify knowledge gains and knowledge retention. Initial knowledge gains in residents who completed the online intervention (average knowledge gain, 6.9%; sd, 18.2) were noninferior compared with those who completed 1 month of a clinical rotation (average knowledge gain, 6.1%; sd, 18.9; difference, 0.8%; 95% CI, –5.05 to 6.47; p = 0.81). Knowledge retention was greater following completion of the online intervention when compared with the clinical rotation when controlling for time (difference, 7.6%; 95% CI, 0.7–14.5; p = 0.03). When the online intervention was sequenced before (average knowledge gain, 14.6%; sd, 15.4) rather than after (average knowledge gain, 7.0%; sd, 19.1) the clinical rotation, residents had superior overall knowledge acquisition (difference, 7.6%; 95% CI, 2.01–12.97;p = 0.008). Conclusions: Incorporating an interactive online educational intervention prior to a clinical rotation may offer a strategy to prime learners for the upcoming rotation, augmenting clinical learning in graduate medical education.

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