Familial hypercholesterolaemia: what’s new?

Autosomal Dominant Familial Hypercholesterolaemia (FH) is the commonest inherited disorder of lipoprotein metabolism. Untreated monogenic FH caused by mutations in the LDLR, APOB or PCSK9 genes result in early onset cardiovascular death (below the age of 60 years). In the UK the prevalence of heterozygous FH is 1 in 270 and homozygous FH is 160,000 approximately.The introduction of statins nearly three decades ago has altered the natural history of FH, with a significant reduction of cardiovascular related morbidity and mortality. There is increasing evidence that early childhood interventions such as lifestyle choices, healthy eating and commencing statins by the age of 10 years would potentially prevent early onset cardiovascular disease and mortality in monogenic FH. The medium term safety of statins in children has been demonstrated. The UK paediatric FH register data has shown that children with FH are less obese than the normal population and the register aims to monitor the longer-term safety of statins in children with FH. Child-parent screening would potentially benefit the child and enables identifying a parent with FH, before the onset of a life threatening cardiovascular event. In addition, genetic cascade testing of relatives of an affected individual has been shown to be highly cost effective.We review the current literature with brief updates on genetics, the UK paediatric FH register data, published recommendations for the management of homozygous and heterozygous FH, lipid lowering therapies in children and screening for FH in childhood.     

Safe and Appropriate Use of Methadone in Hospice and Palliative Care: Expert Consensus White Paper

Methadone has several unique characteristics that make it an attractive option for pain relief in serious illness, but the safety of methadone has been called into question after reports of a disproportionate increase in opioid-induced deaths in recent years. The American Pain Society, College on Problems of Drug Dependence, and the Heart Rhythm Society collaborated to issue guidelines on best practices to maximize methadone safety and efficacy, but guidelines for the end-of-life scenario have not yet been developed. A panel of 15 interprofessional hospice and palliative care experts from the U.S. and Canada convened in February 2015 to evaluate the American Pain Society methadone recommendations for applicability in the hospice and palliative care setting. The goal was to develop guidelines for safe and effective management of methadone therapy in hospice and palliative care. This article represents the consensus opinion of the hospice and palliative care experts for methadone use at end of life, including guidance on appropriate candidates for methadone, detail in dosing, titration, and monitoring of patients' response to methadone therapy.     

护理安全管理在门诊抽血室中应用的效果分析及预防交叉感染的意义

目的:探讨护理安全管理应用于门诊抽血室中的效果及预防交叉感染的意义。方法:选取某院门诊抽血室2017年3月~2018年7月接诊500例患者,随机分为对照组和研究组各250例。对照组实施常规护理管理,研究组患者实施护理安全管理,对比两组防范措施掌握情况与安全防范认知度,比较两组护理不良事件、护患纠纷及医源性感染发生率,同时对比两组护理满意度。结果:研究组防范措施掌握情况、安全防范认知度均高于对照组(P<0.05);研究组护理不良事件、护患纠纷及医源性感染发生率均低于对照组(P<0.05);研究组、对照组护理满意度分别为98.80%、90.40%(P<0.05)。结论:护理安全管理的实施具有重要临床意义,可有效减少护理不良事件的发生,增强患者风险防范意识,预防交叉感染,减少护患纠纷,提高护理满意度,值得临床推广应用。