Asthma Cases Treated With Inhaled Anesthetics or Extracorporeal Membrane Oxygenation: A Virtual Pediatric Systems Database Study of Outcomes.

OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) and/or inhaled anesthetics (IAs) are considered in the management of asthma when refractory to conventional therapy. We aimed to compare the outcomes of these two modalities in asthma PICU care and determine associated survival to hospital discharge among patients in a United States database. DESIGN: Retrospective analysis using the Virtual Pediatric Systems (VPS, LLC) database. SETTING: PICUs participating in the VPS database. PATIENTS: Patients less than 18 years old with diagnosis of asthma treated with IA and/or ECMO from January 2010 to December 2020. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 221 patients were included; 149 (67%) received ECMO, 62 (28%) received IA, and 10 (5%) received both interventions. We failed to identify any difference between the ECMO and IA groups in demographics, Pediatric Index of Mortality 2 percentage, Pediatric Risk of Mortality 3 score, Pediatric Logistic Organ Dysfunction score, or pre-intervention pH and Pa co2 levels. Use of ECMO versus IA was associated with lower pre-intervention Pa o2 (60 torr [7.99 kPa] vs 78 torr [10.39 kPa]; p < 0.001) and higher utilization of high-frequency oscillatory ventilation. We failed to identify an association between type of intervention (IA vs ECMO) and greater odds of survival (57/62 [92%] vs 128/149 [86%]; odds ratio [OR], 1.87; 95% CI, 0.67-5.21; p = 0.23). However, these data do not exclude the possibility that IA use is associated with more than five-fold greater odds of survival. ECMO use was associated with longer duration of intervention (5 vs 1.3 d; p < 0.001) and PICU length of stay (LOS) (13 vs 7 d; p < 0.001). As expected, ECMO versus IA was also associated with greater odds of undergoing bronchoscopy (34% vs 11%; OR, 3.7; 95% CI, 1.5-9.4; p = 0.004). CONCLUSIONS: In the VPS database of asthma management cases, we failed to identify an association between ECMO versus IA use and survival to hospital discharge. However, ECMO was associated with longer duration of intervention and PICU LOS.

Infantile-onset Pompe disease with neutropenia: Treatment decisions in the face of a unique phenotype.

Infantile-onset Pompe disease manifests with early signs of cardiomyopathy during the first few days to weeks of life. We present the case of a newborn born via emergency cesarean section with atrial flutter and moderate biventricular hypertrophy who was diagnosed with Pompe disease on New York State newborn screen. Diagnosis was confirmed with repeat leukocyte acid alpha-glucosidase (GAA) enzyme activity, GAA gene sequencing, urine Hex4, and evaluation of Cross-Reactive Immunological Material (CRIM) status. The patient was also found to be persistently neutropenic which to our knowledge has not been previously reported in the literature in association with Pompe disease. This report highlights the impact that newborn screening had on time to diagnosis and initiation of treatment with enzyme replacement therapy. We also discuss how our patient's concurrent neutropenia impacted decision making related to immune tolerance induction prior to starting enzyme replacement therapy.

Effect of IV Acetaminophen Usage on Opioid Requirements, Outcomes and Costs of Care for Postoperative Children in a Pediatric Intensive Care Unit.

OBJECTIVE: Children admitted to the ICU are commonly treated with opioids for postoperative pain. We hypothesized that administration of IV acetaminophen in the immediate postoperative period is effective in lowering cumulative opioid use leading to other benefits. METHODS: This was a retrospective chart review of patients admitted to the PICU between December 2016 and April 2019. For each patient, data including demographics, cumulative opioid usage per kilogram, oral or rectal acetaminophen, x-ray findings, hospital costs, and surgical procedure were collected. Cumulative opioid usage was determined by converting all opioids to morphine equivalents (MEs) per kg. Standard descriptive and comparative analyses were conducted using SAS 9.4 (SAS Institute, Inc, Cary, NC). RESULTS: A total of 200 patients met inclusion and exclusion criteria (N = 92 in IV acetaminophen group and N = 108 in no IV acetaminophen group). There was no significant difference in ME per kilogram between the groups (0.3 ME/kg in IV acetaminophen group, IQR 0.5 ME/kg versus 0.4 ME/kg in no IV acetaminophen group, IQR 0.5 ME/kg, adjusted p = 0.38). Rate of atelectasis was not significant between the groups (47.8% in IV acetaminophen versus 45.4% in no acetaminophen group, p = 0.28). There was a significant difference in median total hospital costs between the groups ($22,456 in IV acetaminophen group, IQR $18,650 versus $18,552 in no IV acetaminophen group, IQR $13,361, adjusted p = 0.04). CONCLUSIONS: IV acetaminophen in the immediate postoperative period did not lead to a decrease in cumulative opioid usage or rate of atelectasis. IV acetaminophen usage was associated with increase in overall hospital costs per patient.

Arrhythmogenic causes of syncope.

Syncope is one of the common presenting complaints in the pediatric emergency department. The evaluation may begin with consideration of the most common causes. However, it is important to exclude the rare causes, including cardiac arrhythmias that may lead to sudden death in young patients. Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia are some of the rare causes of primary electrical disorders of the heart. High suspicion of these disorders in the evaluation, and appropriate referral to a cardiologist may prevent sudden deaths in these patients. Here, we report 2 children with arrhythmogenic causes of syncope.

Role of MRI in screening, diagnosis and management of breast cancer.

Screening and early diagnosis has an important role in reducing the morbidity and mortality associated with breast cancer. Mammography has an established role and has been approved for routine screening. MRI is an emerging tool and has the highest sensitivity of current breast imaging techniques. Although low specificity and high cost of MRI restricted its use in routine screening, it has been increasingly used in the screening of high-risk individuals, diagnosing occult cases, staging and assessing the response to chemotherapy. MRI-guided techniques, including needle-localization biopsy and vacuum-assisted breast biopsy, have a special role in diagnosis and management. This article focuses on the role of MRI in diagnosis, screening and management of breast cancer, and reviews the current indications for breast MRI.