Citations

Background Propionic acidemia (PA) is a rare inborn error of metabolism that typically presents in infancy; however, late-onset variants may manifest with atypical features, including cardiac involvement. Dilated cardiomyopathy (DCM) is an uncommon but serious complication. Case summary A 15-year-old with no prior medical history but a significant maternal family history of cardiomyopathy of unknown etiology from an Amish background presented with altered mental status, increased work of breathing, and progressive edema in the context of preceding viral illness. Evaluation revealed severe biventricular dysfunction, chamber dilation, and findings consistent with chronic DCM. Despite supportive management, her course was complicated by ventricular arrhythmias and clinical deterioration. Metabolic workup demonstrated elevated propionylcarnitine and increased urinary 2-methylcitric acid, confirming late-onset variant PA. She was treated with intravenous carnitine and a specialized metabolic formula; however, cardiac function remained severely impaired. In accordance with family wishes, she was discharged home on hospice care. Conclusion Late-onset PA can present as isolated cardiomyopathy in adolescence. Early consideration of metabolic etiologies is essential in unexplained DCM, particularly in high-risk populations.

AbstractThe proper use of intravenous fluids has likely been responsible for saving more lives than any other group of substances. Proper use includes prescribing an appropriate electrolyte and carbohydrate solution, at a calculated rate or volume, for the right child, at the right time. Forming intravenous fluid plans for hospitalized children requires an understanding of water and electrolyte physiology in healthy children and how different pathology deviates from the norm. This review highlights fluid management in several disease types, including liver disease, diabetic ketoacidosis, syndrome of inappropriate antidiuretic hormone, diabetes insipidus, kidney disease, and intestinal failure as well as in those with nonphysiologic fluid losses. For each disease, the review discusses specific considerations, evaluations, and management strategies to consider when customizing intravenous fluid plans. Ultimately, all hospitalized children should receive an individualized fluid plan with recurrent evaluations and fluid modifications to provide optimal care.

Objective: Delirium is a common problem in the Pediatric Intensive Care Unit (PICU) and is associated with increased length of stay, cost and mortality. This study evaluated the relationship between noise pollution and delirium risk. Design: This is a Quality Improvement (QI) initiative at an academic PICU. Sound levels were monitored and patients were screened for delirium using the Cornell Assessment of Pediatric Delirium (CAPD). Setting PICU Patients: All PICU patients Interventions: None Measurements and Main Results: Over the 83-week study period (2015-2017), the median [IQR] CAPD score was 8 [3 to 14]. Nursing compliance with the CAPD was 72.2%. The proportion of patients screening positive for delirium (CAPD ≥ 9) was 45.9%. A total of 329 711 hly decibel (dB) measurements were collected and reported. Occupied rooms were louder than unoccupied rooms (51.8 [51.6-51.9] dB vs. 49.8 [49.7-49.9] dB, respectively, p < 0.001). Days (10 AM to 4 PM) were louder than nights (11 PM to 5 AM) (52.8 [52.7-53.0] dB vs. 50.7 [49.9-51.5] dB, respectively p < 0.001) in occupied rooms. Winter (Nov-Feb) months were louder than summer (May-Aug) months (52.0 [51.8-52.3] dB vs. 51.5 [51.3-51.7] dB, respectively, p < 0.002) in occupied rooms. Median weekly nighttime noise levels and CAPD scores demonstrated a correlation coefficient of 0.6 ( p < 0.001). Median weekly risk of mortality (ROM) and CAPD scores demonstrated a correlation coefficient of 0.15 ( p < 0.01). Conclusions: Significant noise pollution exists in the PICU with a moderate correlation between nighttime noise levels and CAPD scores. This could potentially implicate noise pollution as a risk factor for the development of delirium.

Objectives: Noise pollution in pediatric intensive care units (PICU) contributes to poor sleep and may increase risk of developing delirium. The Environmental Protection Agency (EPA) recommends <45 decibels (dB) in hospital environments. The objectives are to assess the degree of PICU noise pollution, to develop a delirium bundle targeted at reducing noise, and to assess the effect of the bundle on nocturnal noise pollution. Methods: This is a QI initiative at an academic PICU. Thirty-five sound sensors were installed in patient bed spaces, hallways, and common areas. The pediatric delirium bundle was implemented in 8 pilot patients (40 patient ICU days) while 108 non-pilot patients received usual care over a 28-day period. Results: A total of 20,609 hourly dB readings were collected. Hourly minimum, average, and maximum dB of all occupied bed spaces demonstrated medians [interquartile range] of 48.0 [39.0-53.0], 52.8 [48.1-56.2] and 67.0 [63.5-70.5] dB, respectively. Bed spaces were louder during the day (10AM to 4PM) than at night (11PM to 5AM) (53.5 [49.0-56.8] vs. 51.3 [46.0-55.3] dB, P < 0.01). Pilot patient rooms were significantly quieter than non-pilot patient rooms at night (n=210, 45.3 [39.7-55.9]) vs. n=1841, 51.2 [46.9-54.8] dB, P < 0.01). The pilot rooms compliant with the bundle had the lowest hourly nighttime average dB (44.1 [38.5-55.5]). Conclusions: Substantial noise pollution exists in our PICU, and utilizing the pediatric delirium bundle led to a significant noise reduction that can be perceived as half the loudness with hourly nighttime average dB meeting the EPA standards when compliant with the bundle.