Spontaneous, simultaneous bilateral osteonecrosis of the femoral heads in a patient with sideroblastic anaemia with B-cell immunodeficiency, periodic fever and developmental delay syndrome.

Sideroblastic anaemia with B-cell immunodeficiency, periodic fever and developmental delay is a recently described, rare syndrome characterised by numerous manifestations underpinned by mutations in transfer RNA nucleotidyltransferase. The pathogenesis arises from mitochondrial dysfunction, with impaired intracellular stress response, deficient metabolism and cellular and systemic inflammation. This yields multiorgan dysfunction and early death in many patients with survivors suffering significant disability and morbidity. New cases, often youths, are still being described, expanding the horizon of recognisable phenotypes. We present a mature patient with spontaneous bilateral hip osteonecrosis that likely arises from the impaired RNA quality control and inflammation caused by this syndrome.

At the Crossroad with Morbidity and Mortality Conferences: Lessons Learned through a Narrative Systematic Review.

Objective. To determine the process and structure of Morbidity and Mortality Conference (MMC) and to provide guidelines for conducting MMC. Methods. Using a narrative systematic review methodology, literature search was performed from January 1, 1950, to October 2, 2012. Original articles in adult population were included. MMC process and structure, as well as baseline study demographics, main results, and conclusions, were collected. Results. 38 articles were included. 10/38 (26%) pertained to medical subspecialties and 25/38 (66%) to surgical subspecialties. 15/38 (40%) were prospective, 14/38 (37%) retrospective, 7/38 (18%) interventional, and 2/38 (5%) cross-sectional. The goals were quality improvement and education. Of the 10 medical articles, MMC were conducted monthly 60% of the time. Cases discussed included complications (60%), deaths (30%), educational values (30%), and system issues (40%). Recommendations for improvements were made frequently (90%). Of the 25 articles in surgery, MMCs were weekly (60% of the time). Cases covered mainly complications (72%) and death (52%), with fewer cases dedicated to education (12%). System issues and recommendations were less commonly reported. Conclusion. Fundamental differences existed in medical versus surgical departments in conducting MMC, although the goals remained similar. We provide a schematic guideline for MMC through a summary of existing literature.

Barriers to goals of care discussions with seriously ill hospitalized patients and their families: a multicenter survey of clinicians.

IMPORTANCE: Seriously ill hospitalized patients have identified communication and decision making about goals of care as high priorities for quality improvement in end-of-life care. Interventions to improve care are more likely to succeed if tailored to existing barriers. OBJECTIVE: To determine, from the perspective of hospital-based clinicians, (1) barriers impeding communication and decision making about goals of care with seriously ill hospitalized patients and their families and (2) their own willingness and the acceptability for other clinicians to engage in this process. DESIGN, SETTING, AND PARTICIPANTS: Multicenter survey of medical teaching units of nurses, internal medicine residents, and staff physicians from participating units at 13 university-based hospitals from 5 Canadian provinces. MAIN OUTCOMES AND MEASURES: Importance of 21 barriers to goals of care discussions rated on a 7-point scale (1 = extremely unimportant; 7 = extremely important). RESULTS: Between September 2012 and March 2013, questionnaires were returned by 1256 of 1617 eligible clinicians, for an overall response rate of 77.7% (512 of 646 nurses [79.3%], 484 of 634 residents [76.3%], 260 of 337 staff physicians [77.2%]). The following family member-related and patient-related factors were consistently identified by all 3 clinician groups as the most important barriers to goals of care discussions: family members' or patients' difficulty accepting a poor prognosis (mean [SD] score, 5.8 [1.2] and 5.6 [1.3], respectively), family members' or patients' difficulty understanding the limitations and complications of life-sustaining treatments (5.8 [1.2] for both groups), disagreement among family members about goals of care (5.8 [1.2]), and patients' incapacity to make goals of care decisions (5.6 [1.2]). Clinicians perceived their own skills and system factors as less important barriers. Participants viewed it as acceptable for all clinician groups to engage in goals of care discussions-including a role for advance practice nurses, nurses, and social workers to initiate goals of care discussions and be a decision coach. CONCLUSIONS AND RELEVANCE: Hospital-based clinicians perceive family member-related and patient-related factors as the most important barriers to goals of care discussions. All health care professionals were viewed as playing important roles in addressing goals of care. These findings can inform the design of future interventions to improve communication and decision making about goals of care.

Antibiotic self-stewardship: trainee-led structured antibiotic time-outs to improve antimicrobial use.

BACKGROUND: Antibiotic use is an important quality improvement target. Nearly 50% of antibiotic use is unnecessary or inappropriate. To combat overuse, the Centers for Disease Control and Prevention (CDC) proposed "time-outs" to reevaluate antibiotics. OBJECTIVE: To optimize antibiotic use through trainee-led time-outs. DESIGN: Before-after study. SETTING: Internal medicine (2 units, 46 beds) at a university hospital. PATIENTS: Inpatients (n = 679). INTERVENTION: From January 2012 until June 2013, while receiving monthly education on antimicrobial stewardship, resident physicians adjusted patients' antibiotic therapy through twice-weekly time-out audits using a structured electronic checklist. MEASUREMENTS: Antibiotic costs were standardized and compared in the year before and after the audits. Use was measured as World Health Organization defined daily doses (DDDs) per 1000 patient-days. Total antibiotic use and the use of moxifloxacin, carbapenems, antipseudomonal penicillins, and vancomycin were compared by using interrupted time series. Rates of nosocomial Clostridium difficile infection were compared by using incidence rate ratios. RESULTS: Total costs in the units decreased from $149,743CAD (January 2011 to January 2012) to $80,319 (January 2012 to January 2013), for a savings of $69,424 (46% reduction). Of the savings, $54,150 (78%) was related to carbapenems and $15,274 (22%) was due to other antibiotic classes. Adherence with the auditing process was 80%. In the time-series analyses, the only reliable and statistically significant change was a reduction in the rate of moxifloxicin use, by -1.9 DDDs per 1000 patient-days per month (95% CI, -3.8 to -0.02; P = 0.048). Rates of C. difficile infection decreased from 24.2 to 19.6 per 10,000 patient-days (incidence rate ratio, 0.8 [CI, 0.5 to 1.3]). LIMITATION: Other temporal factors may confound the findings. CONCLUSIONS: An antibiotic self-stewardship bundle to implement the CDC's suggested time-outs seems to have reduced overall costs and targeted antibiotic use. PRIMARY FUNDING SOURCE: None.