Lysosomal acid lipase deficiency (LAL-D) is a rare disorder of cholesterol metabolism with an autosomal recessive mode of inheritance. The absence or deficiency of the LAL enzyme gives rise to pathological accumulation of cholesterol esters in various tissues. A severe LAL-D phenotype manifesting in infancy is associated with adrenal calcification and liver and gastrointestinal involvement with characteristic early mortality. LAL-D presenting in childhood and adulthood is associated with hepatomegaly, liver fibrosis, cirrhosis, and premature atherosclerosis. There are currently no curative pharmacological treatments for this life-threatening condition. Supportive management with lipid-modifying agents does not ameliorate disease progression. Hematopoietic stem cell transplantation as a curative measure in infantile disease has mixed success and is associated with inherent risks and complications. Sebelipase alfa (Kanuma) is a recombinant human LAL protein and the first enzyme replacement therapy for the treatment of LAL-D. Clinical trials have been undertaken in infants with rapidly progressive LAL-D and in children and adults with later-onset LAL-D. Initial data have shown significant survival benefits in the infant group and improvements in biochemical parameters in the latter. Sebelipase alfa has received marketing authorization in the United States and Europe as long-term therapy for all affected individuals. The availability of enzyme replacement therapy for this rare and progressive disorder warrants greater recognition and awareness by physicians.

Introduction of an NST increased both the total PN use and the percentage of referrals where enteral nutrition could replace PN. Medical specialty influenced the referral pattern and the likelihood that a referral resulted in PN being initiated. Safety of PN catheters improved significantly following NST introduction.

Malnutrition is both a common and concerning problem with almost 60 % of elderly care in-patients being at risk (1) . It is well established that malnourished patients will have poorer clinical outcomes (2) and that malnutrition is a key factor in prolonging length of stay in elderly patients (1) . The Malnutrition Universal Screening Tool (MUST) has been developed as a method of identifying these at-risk patients and has been advocated across the NHS since 2003 (3) . The nutrition team at SRFT were keen to understand local current practice and then take action to improve the situation. In order to achieve this the team designed a quality improvement project based on the Model for Improvement (4) . 3 elderly care wards were invited to participate in the first phase of this project as these wards have many patients at high risk of malnutrition. Each developed a multidisciplinary improvement team of ward staff to work on improving MUST screening.The nutrition team designed a driver diagram which articulated the aim of the project and the key workstreams that would be necessary to achieve it. This was used as a clear visual method of communicating the project to the ward teams. Each month the timeliness (within 6 hours of admission) and accuracy (compared to a dietician assessment) of MUST scores on the three wards was reviewed. Ward teams received monthly feedback of their results to create ownership, maintain enthusiasm and generate momentum for improvement. Baseline data identified that a MUST screen was performed in < 60 % of patients and in those with an assessment only 65 % were accurate.Plan-Do-Study-Act (PDSA) cycles were used to rapidly test changes in the ward areas. Tests included a nutrition study day, one-to-one ward based nutrition training, focus on the use of alternative anthropometric measurements, development of a training pack and identification of the challenges to undertaking accurate and timely assessments.During the project all the wards achieved an improvement in the proportion of patients screened with one ward achieving 100%. It was identified that strong ward leadership was a key factor in this ward's success. There was also some evidence that the drive to screen within 6 hours resulted in less accurate assessments and that assessments within 24 hours might be more accurate. We also identified that only 60-70% patients screened had a nutrition care plan and thus that screening does not necessarily result in action to improve nutritional state.The nutrition team is now working with wards to evaluate the link between timeliness and accuracy of assessment in more detail. We are also looking at the role of Health Care Assistants in MUST screening. The team continues to work on improving nutritional screening but is now focussed on developing reliable processes, using quality improvement methodologies and the application of reliability science, to ensure the delivery of appropriate interventions to patients identified as high risk.

Careful use of antimicrobial drugs is important in reducing the number of cases of C difficile infection. A collaborative learning model can enable teams to test and implement changes that can accelerate, amplify, and sustain control of C difficile.

The results showed a positive relationship between the change package and a reduction of 41% in cardiac arrests outside of critical care areas from the baseline period (April 2007-March 2008) to December 2012. The BTS model has the potential to reduce cardiac arrests without the need for initial large-scale financial investment.