Richard M. Cantor

Echocardiographic abnormalities and disease severity in Fabry's disease

Fabrys disease is an X-linked recessive genetic deficiency of the enzyme alpha-galactosidase A, which leads to the pathologic deposition of neutral glycosphingolipids in lysosomes of the vascular endothelium of the heart, brain and kidney. The disease is progressive in hemizygous male patients, with increasing involvement of the major organs leading to death. Because cardiac involvement is a constant feature, echocardiograms were performed on 35 patients with Fabrys disease, 23 hemizygotes (aged 28.6 +/- 14 years) and 12 heterozygotes (aged 31.6 +/- 6 years), to determine whether cardiac involvement could be detected noninvasively. The results demonstrated that hemizygous male patients had a greater aortic root diameter, thicker interventricular septum and greater ventricular mass than did heterozygous female patients. Left ventricular mass per square meter of body surface area correlated well with clinical disease severity (r = 0.68, p less than 0.05), suggesting progressive glycosphingolipid deposition. Older heterozygotes (greater than 25 years old) had more severe evidence of cardiac disease than did younger male patients. Although mitral valve prolapse was identified in 12 (54%) of 23 male hemizygotes and in 7 (58%) of 12 female heterozygotes its presence did not correlate with clinical disease severity or other echocardiographic variables. Therefore, echocardiographic evidence of Fabrys disease appears to correlate with age-related disease severity and may be a useful noninvasive marker to follow disease progression and possible regression when appropriate therapy becomes available.

Point-of-care ultrasonography by pediatric emergency medicine physicians

Emergency physicians have used point-of-care ultrasonography since the 1990s. Pediatric emergency medicine physicians have more recently adopted this technology. Point-of-care ultrasonography is used for various scenarios, particularly the evaluation of soft tissue infections or blunt abdominal trauma and procedural guidance. To date, there are no published statements from national organizations specifically for pediatric emergency physicians describing the incorporation of point-of-care ultrasonography into their practice. This document outlines how pediatric emergency departments may establish a formal point-of-care ultrasonography program. This task includes appointing leaders with expertise in point-of-care ultrasonography, effectively training and credentialing physicians in the department, and providing ongoing quality assurance reviews.

Withholding or Termination of Resuscitation in Pediatric Out-of-Hospital Traumatic Cardiopulmonary Arrest

This multiorganizational literature review was undertaken to provide an evidence base for determining whether recommendations for out-of-hospital termination of resuscitation could be made for children who are victims of traumatic cardiopulmonary arrest. Although there is increasing acceptance of out-of-hospital termination of resuscitation for adult traumatic cardiopulmonary arrest when there is no expectation of a good outcome, children are routinely excluded from state termination-of-resuscitation protocols. The decision to withhold resuscitative efforts in a child under specific circumstances (decapitation or dependent lividity, rigor mortis, etc) is reasonable. If there is any doubt as to the circumstances or timing of the traumatic cardiopulmonary arrest, under the current status of limiting termination of resuscitation in the field to persons older than 18 years in most states, resuscitation should be initiated and continued until arrival to the appropriate facility. If the patient has arrested, resuscitation has already exceeded 30 minutes, and the nearest facility is more than 30 minutes away, involvement of parents and family of these children in the decision-making process with assistance and guidance from medical professionals should be considered as part of an emphasis on family-centered care because the evidence suggests that either death or a poor outcome is inevitable.

Emergency Data Set for Children With Special Needs

Abstract [Sachetti A, Gerardi M, Barkin R, Santamaria J, Cantor R, Weinberg J, Gausche M: Emergency data set for children with special needs. Ann Emerg Med September 1996;28:324-327.]

Point-of-Care Ultrasonography by Pediatric Emergency Physicians

Point-of-care ultrasonography is increasingly being used to facilitate accurate and timely diagnoses and to guide procedures. It is important for pediatric emergency physicians caring for patients in the emergency department to receive adequate and continued point-of-care ultrasonography training for those indications used in their practice setting. Emergency departments should have credentialing and quality assurance programs. Pediatric emergency medicine fellowships should provide appropriate training to physician trainees. Hospitals should provide privileges to physicians who demonstrate competency in point-of-care ultrasonography. Ongoing research will provide the necessary measures to define the optimal training and competency assessment standards. Requirements for credentialing and hospital privileges will vary and will be specific to individual departments and hospitals. As more physicians are trained and more research is completed, there should be one national standard for credentialing and privileging in point-of-care ultrasonography for pediatric emergency physicians.

Pediatric care recommendations for freestanding urgent care facilities

Treatment of children at freestanding urgent care facilities has become common in pediatric health care. Well-managed freestanding urgent care facilities can improve the health of the children in their communities, integrate into the medical community, and provide a safe, effective adjunct to, but not a replacement for, the medical home or emergency department. Recommendations are provided for optimizing freestanding urgent care facilities’ quality, communication, and collaboration in caring for children.

Evaluation and management of children and adolescents with acute mental health or behavioral problems. Part I: Common clinical challenges of patients with mental health and/or behavioral emergencies

This document is copyrighted and is property of the American Academy of Pediatrics and its Board of Directors. All authors have fi led confl ict of interest statements with the American Academy of Pediatrics. Any confl icts have been resolved through a process approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any commercial involvement in the development of the content of this publication. Clinical reports from the American Academy of Pediatrics benefi t from expertise and resources of liaisons and internal (AAP) and external reviewers. However, clinical reports from the American Academy of Pediatrics may not refl ect the views of the liaisons or the organizations or government agencies that they represent. The guidance in this report does not indicate an exclusive course of treatment or serve as a standard of medical care. Variations, taking into account individual circumstances, may be appropriate. All clinical reports from the American Academy of Pediatrics automatically expire 5 years after publication unless reaffi rmed, revised, or retired at or before that time. DOI: 10.1542/peds.2016-1570

Handoffs: Transitions of care for children in the emergency department

Transitions of care (ToCs), also referred to as handoffs or sign-outs, occur when the responsibility for a patient’s care transfers from 1 health care provider to another. Transitions are common in the acute care setting and have been noted to be vulnerable events with opportunities for error. Health care is taking ideas from other high-risk industries, such as aerospace and nuclear power, to create models of structured transition processes. Although little literature currently exists to establish 1 model as superior, multiorganizational consensus groups agree that standardization is warranted and that additional work is needed to establish characteristics of ToCs that are associated with clinical or practice outcomes. The rationale for structuring ToCs, specifically those related to the care of children in the emergency setting, and a description of identified strategies are presented, along with resources for educating health care providers on ToCs. Recommendations for development, education, and implementation of transition models are included.

Death of a Child in the Emergency Department

The American Academy of Pediatrics, American College of Emergency Physicians, and Emergency Nurses Association have collaborated to identify practices and principles to guide the care of children, families, and staff in the challenging and uncommon event of the death of a child in the emergency department in this policy statement and in an accompanying technical report.