The role of the supervisor in self-regulated learning in the clinical environment: BEME Guide No. 89.

Introduction: Self-regulated learning (SRL) in medical education is important for successful learning and safe patient care. However, supervisors may be unaware of behaviours that explicitly facilitate or inhibit their students' or residents' SRL, therefore this BEME review explores the role of the supervisor in SRL in clinical environments.

Methods: A qualitative systematic review using meta-aggregation was performed, seeking to draw on the knowledge of included studies and the participants those studies represent to create context-rich recommendations that are relevant and applicable to practice.

Beyond the bedside: A scoping review of the experiences of non-practising health care professionals in Health Professions Education.

Introduction: The shortage of educators within Health Professions Education (HPE) threatens the optimal training of the future health care workforce. Furthermore, without recruitment of diverse and skilled faculty, targets to expand the workforce will not be possible. Non-practising health care professionals offer extensive knowledge and qualifications within health care, without the competing clinical commitments of their clinical academic colleagues, and therefore are ideally positioned to support education and training initiatives.

Exploring the Cost-Effectiveness of Newborn Screening for Metachromatic Leukodystrophy (MLD) in the UK.

Metachromatic leukodystrophy (MLD) is a fatal inherited lysosomal storage disease that can be detected through newborn bloodspot screening. The feasibility of the screening assay and the clinical rationale for screening for MLD have been previously demonstrated, so the aim of this study is to determine whether the addition of screening for MLD to the routine newborn screening program in the UK is a cost-effective use of National Health Service (NHS) resources. A health economic analysis from the perspective of the NHS and Personal Social Services was developed based on a decision-tree framework for each MLD subtype using long-term outcomes derived from a previously presented partitioned survival and Markov economic model.

Twice weekly dosing with Sebelipase alfa (Kanuma®) rescues severely ill infants with Wolman disease.

Background: Sebelipase alfa (Kanuma®) is approved for patients with Wolman disease (WD) at a dosage of 3-5 mg/kg once weekly. Survival rates in the second of two clinical trials was greater, despite recruiting more severely ill patients, probably related to higher initial and maximal doses. We aimed to evaluate the effective pharmacokinetics and pharmacodynamics of Sebelipase alfa when administered to patients with severe WD at 5 mg/kg twice weekly, an intensive regimen which was not assessed in the trials.