A quality improvement initiative standardizing premedication for blood product transfusions.

For children with cancer, blood product transfusions are crucial, but can be complicated by transfusion reactions. To prevent these complications, premedication is often given, although not always evidence-based. Herein, we describe a significant decrease in the use of premedication (72%-28%) at our institution after the implementation of standardized guidelines, without an increase in transfusion reactions (3.2% prior vs. 1.5% after standardization). Importantly, there were no severe transfusion reactions leading to hospitalization or death. Our results provide evidence in favor of more judicious use of premedication prior to transfusions in patients 21 years and younger being treated for cancer.

Clinical Targeted Next-Generation Panel Sequencing Reveals MYC Amplification Is a Poor Prognostic Factor in Osteosarcoma.

PURPOSE: Osteosarcoma risk stratification, on the basis of the presence of metastatic disease at diagnosis and histologic response to chemotherapy, has remained unchanged for four decades, does not include genomic features, and has not facilitated treatment advances. We report on the genomic features of advanced osteosarcoma and provide evidence that genomic alterations can be used for risk stratification. MATERIALS AND METHODS: In a primary analytic patient cohort, 113 tumor and 69 normal samples from 92 patients with high-grade osteosarcoma were sequenced with OncoPanel, a targeted next-generation sequencing assay. In this primary cohort, we assessed the genomic landscape of advanced disease and evaluated the correlation between recurrent genomic events and outcome. We assessed whether prognostic associations identified in the primary cohort were maintained in a validation cohort of 86 patients with localized osteosarcoma tested with MSK-IMPACT. RESULTS: In the primary cohort, 3-year overall survival (OS) was 65%. Metastatic disease, present in 33% of patients at diagnosis, was associated with poor OS (P = .04). The most frequently altered genes in the primary cohort were TP53, RB1, MYC, CCNE1, CCND3, CDKN2A/B, and ATRX. Mutational signature 3 was present in 28% of samples. MYC amplification was associated with a worse 3-year OS in both the primary cohort (P = .015) and the validation cohort (P = .012). CONCLUSION: The most frequently occurring genomic events in advanced osteosarcoma were similar to those described in prior reports. MYC amplification, detected with clinical targeted next-generation sequencing panel tests, is associated with poorer outcomes in two independent cohorts.

Skeletal muscle metastases in neuroblastoma share common progenitors with primary tumor and biologically resemble stage MS disease.

Introduction: While subcutaneous metastases are often observed with stage MS neuroblastoma, an entity that usually resolves spontaneously, skeletal muscle metastases (SMM) have been rarely described. The purpose of this retrospective study was to investigate the significance of SMM in neuroblastoma. Patients and methods: Seventeen patients with neuroblastoma SMM were diagnosed at a median age of 4.3 (0.1-15.6) months. All had SMM at diagnosis and metastases at other sites. Fifteen (88%) had ≥ 2 SMM in disparate muscle groups. One, 14, and 2 patients had low, intermediate, and high-risk disease respectively. Fifteen tumors had favorable histology without MYCN amplification, and 2 were MYCN-amplified. Most SMM (80%; n=12/15 evaluated) were MIBG-avid. Results: Only 1 patient (with MYCN-non-amplified neuroblastoma) had disease progression. All survive at median follow-up of 47.9 (16.9-318.9) months post-diagnosis. Biological markers (histology, chromosomal and genetic aberrations) were not prognostic. Whole genome sequencing of 3 matched primary and SMM lesions suggested that both primary and metastatic tumors arose from the same progenitor. SMM completely resolved in 10 patients by 12 months post-diagnosis. Of 4 patients managed with watchful observation alone without any cytotoxic therapy, 3 maintain complete remission with SMM resolving by 5, 13, and 21 months post-diagnosis respectively. Conclusions: Children with neuroblastoma SMM have an excellent prognosis, with a clinical course suggestive of stage MS disease. Based on these results, the initial management of infants with non-MYCN-amplified NB with SMM could be watchful observation, which could eliminate or reduce exposure to genotoxic therapy.

Is hyperbilirubinemia a contraindication for neonatal circumcision? A survey of practice patterns of pediatric urologists and a review of the literature.

INTRODUCTION AND OBJECTIVE: 50-80% of term newborns develop jaundice, or hyperbilirubinemia (HB), in their first week. The vast majority have benign etiologies, including physiologic jaundice of the newborn and breast milk/breastfeeding jaundice, which do not affect the synthetic capacity of the liver, thus conferring a low risk of peri-procedural bleeding. Though uncommon, HB in the setting of sepsis, biliary obstruction, or metabolic disease, may increase procedural bleeding risk. Circumcision of neonates with HB has not been well studied. We sought to characterize practice patterns among Society of Pediatric Urology (SPU) members and to explore whether HB confers increased bleeding risk for newborn circumcision. METHODS: An anonymous survey of 14 multiple-choice questions was sent to members of the SPU listserv. Questions regarding circumcision and HB were presented. We performed a literature review regarding whether HB confers increased surgical bleeding risk. RESULTS: 100/234 (43%) SPU members completed the survey. The majority (79/100) perform neonatal circumcision and use the Gomco© clamp (68%). 24/79 (30%) factor total bilirubin (Tbili) level in their decision prior to performing circumcision. Of those who consider HB a factor, 11/24 (46%) had cutoff Tbili levels at which they await improvement prior to proceeding. The most common cutoff level was Tbili level of 10-15 mg/dL (6/11, 55%). DISCUSSION: Existing data suggest a possible increased bleeding risk isolated to cases of HB in the setting of biliary obstruction or other associated relevant findings (ill infant, recent infection, congenital syndromes) or known personal/family history (fulminant liver disease, familial bleeding diatheses). While literature from Jewish Mohels and Talmudic discussion suggest that elevated Tbili may be a contraindication to circumcision, no scientific studies exist directly assessing the impact of HB on bleeding risk with circumcision. A review of the scientific literature suggests that isolated HB in otherwise healthy newborns does not increase bleeding risk. CONCLUSIONS: 30% of pediatric urologists survey respondents consider HB a potential contraindication to neonatal circumcision. Despite varied practices in circumcising jaundiced babies, neonatal jaundice rarely confers increased bleeding risks. While deferring circumcision is appropriate in an ill infant with HB, or in those with a genetic/congenital syndrome or with family history of coagulopathic, review of the scientific literature suggests that in otherwise healthy neonates, elevated Tbili likely represents benign causes and is unlikely to increase bleeding risk.

Management of adult cardiac arrest in the COVID-19 era: consensus statement from the Australasian College for Emergency Medicine.

INTRODUCTION: The global pandemic of coronavirus disease 2019 (COVID-19) has caused significant worldwide disruption. Although Australia and New Zealand have not been affected as much as some other countries, resuscitation may still pose a risk to health care workers and necessitates a change to our traditional approach. This consensus statement for adult cardiac arrest in the setting of COVID-19 has been produced by the Australasian College for Emergency Medicine (ACEM) and aligns with national and international recommendations. MAIN RECOMMENDATIONS: In a setting of low community transmission, most cardiac arrests are not due to COVID-19. Early defibrillation saves lives and is not considered an aerosol generating procedure. Compression-only cardiopulmonary resuscitation is thought to be a low risk procedure and can be safely initiated with the patient's mouth and nose covered. All other resuscitative procedures are considered aerosol generating and require the use of airborne personal protective equipment (PPE). It is important to balance the appropriateness of resuscitation against the risk of infection. Methods to reduce nosocomial transmission of COVID-19 include a physical barrier such as a towel or mask over the patient's mouth and nose, appropriate use of PPE, minimising the staff involved in resuscitation, and use of mechanical chest compression devices when available. If COVID-19 significantly affects hospital resource availability, the ethics of resource allocation must be considered. CHANGES IN MANAGEMENT: The changes outlined in this document require a significant adaptation for many doctors, nurses and paramedics. It is critically important that all health care workers have regular PPE and advanced life support training, are able to access in situ simulation sessions, and receive extensive debriefing after actual resuscitations. This will ensure safe, timely and effective management of the patients with cardiac arrest in the COVID-19 era.

Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.

OBJECTIVE The purpose of this study is to present an illustrative case of pediatric superior semicircular canal dehiscence (SSCD) and to systematically review the current published literature in the pediatric population. METHODS An electronic search of the Scopus, Web of Science, PsycINFO, Cochrane, and Embase databases was performed by 2 independent authors through January 2017. Search term combinations included "pediatrics," "children," "canal," and "dehiscence." Inclusion criteria were as follows: English, full-text clinical studies, case reports, and case series describing pediatric patient(s) (younger than 18 years) with CT evidence of SSCD. Baseline patient demographic characteristics, clinical presentations, dehiscence characteristics, management strategies, and outcome data were extracted. RESULTS A total of 14 studies involving 122 patients were included in the quantitative synthesis. The patients' mean age was 7.22 years. Male predominance was observed (approximate male-to-female ratio of 1.65:1). Neurodevelopmental disorders were common (n = 14, 11.5%). Auditory signs and symptoms were more common than vestibular signs and symptoms. Hearing loss (n = 62, 50.8%) was the most common auditory symptom and an indicator for imaging evaluation. Vertigo was the most common vestibular symptom (n = 8, 6.6%). Hearing aids were recommended in 8 cases (6.6%), and surgical repair was performed in 1 case (0.8%). Symptom outcomes and follow-up durations were infrequently reported. CONCLUSIONS The authors' data suggest that in pediatric SSCD, males are more commonly affected than females. This is different than the adult population in which females are predominantly affected. A history of otologic and/or neurodevelopmental abnormalities was common. There was a preponderance of auditory symptoms in this age group. Conservative management was favored in the majority.

Systematic Analysis of Outcomes for Surgical Resection and Radiotherapy in Patients with Papillary Meningioma.

Introduction Papillary meningiomas (PMs) are characterized by their aggressive nature and high rate of recurrence. Due to their rarity, studies examining the relationship between treatment and clinical outcomes for this disease are limited. Gross total resection (GTR) with or without radiotherapy (RT) is considered the standard treatment; however, when GTR is not feasible, subtotal resection (STR) followed by RT may be an effective alternative. In this study, we analyzed the clinical outcomes in patients who either underwent GTR alone, GTR followed by RT, STR alone, or STR followed by RT. Methods A systematic analysis was performed to identify PM patients with sufficient follow-up and outcome data, as measured by recurrence. Patient data lacking extent of resection, follow-up, or recurrence information were excluded. Results A total of 29 patients with PM were treated with resections (23 GTRs and 6 STRs).The mean age and mean follow-up of patients in this study were 32.3 years and 42.1 months, respectively. Of these patients, 58.6% experienced recurrence. Overall, 47.8% of patients who underwent GTR experienced recurrence. These patients also demonstrated improved survival compared with STR. Among patients whose tumors were only partially excised, a recurrence rate of 83% was observed. Conclusion Our results confirm that GTR results in fewer recurrences compared with STR, supporting GTR as the treatment of choice for PM. Furthermore, GTR in conjunction with RT resulted in improved survival compared with GTR alone. When GTR was not feasible, STR with RT was associated with improved survival compared with STR alone. Future studies with more outcome data are needed to elucidate the optimal treatment for this rare disease.

Passive immunotherapeutic strategies for the treatment of malignant gliomas.

This review provides historical and recent perspectives related to passive immunotherapy for high-grade gliomas. The authors discuss approaches that use lymphokine-activated killer cells, cytotoxic T lymphocytes, and monoclonal antibodies.

Complications associated with the treatment for spinal ependymomas.

Spinal cord ependymomas are rare neoplasms, comprising approximately 5% of all CNS tumors and 15% of all spinal cord tumors. Although surgery was once reserved for diagnosis alone, the evolution of surgical practices has elevated resection to the treatment of choice for these lesions. While technological advances continue to improve the capacity for gross-total resections and thus decrease the risk of recurrence, ependymoma spinal surgery still contains a variety of potential complications. The presence of neurological deficits and deterioration are not uncommonly associated with spinal cord ependymoma surgery, including sensory loss, dorsal column dysfunction, dysesthetic syndrome, and bowel and bladder dysfunction, particularly in the immediate postoperative period. Surgical treatment may also lead to wound complications and CSF leaks, with increased risk when radiotherapy has been involved. Radiation therapy may also predispose patients to radiation myelopathy and ultimately result in neurological damage. Additionally, resections of spinal ependymomas have been associated with postoperative spinal instability and deformities, particularly in the pediatric population. Despite the advances in microsurgical techniques and intraoperative cord monitoring modalities, there remain a number of serious complications related to the treatment of spinal ependymoma tumors. Identification and acknowledgment of these potential problems may assist in their prevention, early detection, and increased quality of life for patients afflicted with this disease.

Implementation of a guideline for computed tomography head imaging in head injury: a prospective study.

OBJECTIVE: To improve appropriate ordering of head computed tomography (CT) in patients presenting with a head injury by applying an evidence-based head injury guideline. METHODS: This was a comparison observational study of CT head ordering in the setting of head trauma between two groups of patients. There was a pre-guideline implementation group and a post-guideline implementation group. Our Southernhealth Head Injury Guideline was largely based on the Canadian CT Head Rule by Steill et al. 2001.We also applied the Canadian CT Head Rule to our post-guideline implementation group. RESULTS: CT ordering rate in the pre-guideline group was 31.6% compared with 59% in the post-guideline group with a relative risk of 1.88 (95% confidence interval [CI]: 1.56-2.27). Abnormal head CT were reported in 6.8% in the pre-guideline group and 5% in the post-guideline group (relative risk 0.88, 95% CI 0.44-1.51). When we applied the Canadian CT Head Rule to the prospective group, four patients with clinically significant abnormal head CT would not have been scanned. The sensitivity of the guideline was 100% (95% CI 79-100%), with a specificity of 43.22% (95% CI 37-48%) in diagnosing a significant head injury on CT. CONCLUSION: The Southernhealth Head Injury Guideline is safe and easy to apply to minor and major head injuries.

Overcoming the self-promotion dilemma: interpersonal attraction and extra help as a consequence of who sings one's praises.

Prior research has shown that positive information presented by a third party shields people from the negative consequences of being perceived as self-promoting. But in many contexts, those third parties are intermediaries with a financial interest in the person being promoted rather than neutral parties. In three experimental studies, the authors demonstrate that even when intermediaries are not neutral, they can be helpful for overcoming the self-promotion dilemma--the need to assert one's competence but not be harmed by the fact that people who self-promote are viewed negatively. The authors find that hiring an agent to sing one's praises results in more favorable perceptions of the client, which contributes, in turn, to a greater willingness to offer that person assistance. It is also shown that even when the intermediary is physically present and seen to be complicit with the client, the positive effects of having someone else speak on one's behalf persist.

A paediatric trauma study of scooter injuries.

OBJECTIVE: To investigate the incidence and describe the nature of non-motorized scooter related injuries in children presenting to the ED. SETTING: Paediatric ED of a metropolitan tertiary referral hospital. METHODS: A prospective observational study of patients aged under 19 years presenting with injuries sustained while using a non-motorized scooter. Clinicians recorded the data in the patient record. MAIN OUTCOME MEASURES: type of injury sustained; period of experience on the scooter; the use of protective gear; the presence of adult supervision; the place of accident; and the patient outcome. RESULTS: Sixty-two eligible patients were recruited over an 18 month period. The incidence of scooter- related injuries was 1.3% of all paediatric trauma presentations. There was a fall in scooter injury presentations over the study period; however, this was not statistically significant. The most common injury sustained using a scooter was an upper limb fracture (41.9%). Closed head injury comprised 8.1% of all scooter related injuries. The majority of patients were not wearing protective gear and were unsupervised at the time of their accident. Most patients (79%) were managed in the ED and discharged. CONCLUSIONS: There has been no significant change in scooter injury presentations over the two summer periods of 2000 and 2001. Children presenting to the ED with a scooter related injury tend to be primary school aged, which may have implications on scooter design, age recommendations and safety guidelines.

Development of an evidence-based guideline for imaging in cervical spine trauma.

Cervical spine trauma is a common reason for presentation to an emergency department. However, less than 5% of patients who have suffered possible neck injury actually have an injury requiring medical treatment. Nevertheless, the consequences, both for the patient and the doctor, of a missed injury are well recognized by emergency department medical staff. This results in the vast majority of these patients receiving some form of diagnostic imaging. We describe the development of an evidence-based imaging guideline for use in the patient who has suffered cervical spine trauma. The guideline aims to help clinicians determine, at the bedside, when it is appropriate to use imaging and which imaging modality should be used first. Correct utilization of the guideline should lead to a reduction in the number of imaging tests required to reach a diagnosis without adverse patient outcomes.