Postoperative Remote Automated Monitoring: Need for and State of the Science

Worldwide, more than 230 million adults have major noncardiac surgery each year. Although surgery can improve quality and duration of life, it can also precipitate major complications. Moreover, a substantial proportion of deaths occur after discharge. Current systems for monitoring patients postoperatively, on surgical wards and after transition to home, are inadequate. On the surgical ward, vital signs evaluation usually occurs only every 4-8 hours. Reduced in-hospital ward monitoring, followed by no vital signs monitoring at home, leads to thousands of cases of undetected/delayed detection of hemodynamic compromise. In this article we review work to date on postoperative remote automated monitoring on surgical wards and strategy for advancing this field. Key considerations for overcoming current barriers to implementing remote automated monitoring in Canada are also presented.     

ACSM Symposium: Former Athletes in Later Life

Wrist pain in an active patient is a physician's cue to look for specific clues in the history, physical exam, and diagnostic tests. Without prompt diagnosis and accurate treatment, the chronic pain can impair sports participation and performance. And in certain instances, a misdiagnosed condition, such as a carpal dislocation or distal radial physeal fracture, can lead to deformity or disability from lack of adequate treatment.     

Biology and targeting of the Jumonji-domain histone demethylase family in childhood neoplasia: a preclinical overview

Introduction: Epigenetic mechanisms of gene regulatory control play fundamental roles in developmental morphogenesis, and, as more recently appreciated, are heavily implicated in the onset and progression of neoplastic disease, including cancer. Many epigenetic mechanisms are therapeutically targetable, providing additional incentive for understanding of their contribution to cancer and other types of neoplasia.

Areas covered: The Jumonji-domain histone demethylase (JHDM) family exemplifies many of the above traits. This review summarizes the current state of knowledge of the functions and pharmacologic targeting of JHDMs in cancer and other neoplastic processes, with an emphasis on diseases affecting the pediatric population.

Expert opinion: To date, the JHDM family has largely been studied in the context of normal development and adult cancers. In contrast, comparatively few studies have addressed JHDM biology in cancer and other neoplastic diseases of childhood, especially solid (non-hematopoietic) neoplasms. Encouragingly, the few available examples support important roles for JHDMs in pediatric neoplasia, as well as potential roles for JHDM pharmacologic inhibition in disease management. Further investigations of JHDMs in cancer and other types of neoplasia of childhood can be expected to both enlighten disease biology and inform new approaches to improve disease outcomes.     

Genotype-phenotype relationships in mucopolysaccharidosis type I (MPS I): Insights from the International MPS I Registry

Mucopolysaccharidosis type I (MPS I) is a rare autosomal recessive disorder resulting from pathogenic variants in the α-L-iduronidase (IDUA) gene. Clinical phenotypes range from severe (Hurler syndrome) to attenuated (Hurler-Scheie and Scheie syndromes) and vary in age of onset, severity, and rate of progression. Defining the phenotype at diagnosis is essential for disease management. To date, no systematic analysis of genotype-phenotype correlation in large MPS I cohorts have been performed. Understanding genotype-phenotype is critical now that newborn screening for MPS I is being implemented. Data from 538 patients from the MPS I Registry (380 severe, 158 attenuated) who had 2 IDUA alleles identified were examined. In the 1076 alleles identified, 148 pathogenic variants were reported; of those, 75 were unique. Of the 538 genotypes, 147 (27%) were unique; 40% of patients with attenuated and 22% of patients with severe MPS I had unique genotypes. About 67.6% of severe patients had genotypes where both variants identified are predicted to severely disrupt protein/gene function and 96.1% of attenuated patients had at least one missense or intronic variant. This dataset illustrates a close genotype/phenotype correlation in MPS I but the presence of unique IDUA missense variants remains a challenge for disease prediction.     

Recurrent oral squamous cell carcinoma‐incorporating advance care planning in education and practice

This reflection describes a life‐limiting case of oral squamous cell carcinoma (SCC) that required thoughtful management facilitated by an advance care plan (ACP). A 70‐year‐old female was diagnosed with a T4aN2bM0 biopsy‐proven invasive, well‐differentiated keratinizing SCC. Surgical wide‐local excision included teeth #11‐16 with left unilateral neck dissection, levels I‐V. She was rehabilitated with maxillary obturator prosthesis and underwent chemoradiation therapy. Her course was complicated by dysphagia and trismus. She experienced multiple recurrences. At a certain point, negative margins could not be achieved without facial disfigurement. The patient, her husband, and providers decided together that further management would be palliative. Before the additional surgical procedures, she communicated a thorough ACP with her husband and providers who were prepared to facilitate difficult care decisions on her behalf. The patient passed away at home with hospice care at the age of 74. This motivated patient with oral SCC and impactful postmanagement complications appreciated the clarity of an ACP. Her values and goals of care were incorporated with ongoing communication and documentation of this plan, which was instrumental in facilitating her person‐centered care. The providers apply lessons learned here in future practice and education of residents and students.