BCS1L mutations cause disease phenotypes ranging from highly restricted pili torti and sensorineural hearing loss (the Björnstad syndrome) to profound multisystem organ failure (complex III deficiency and the GRACILE syndrome). All BCS1L mutations disrupted the assembly of mitochondrial respirasomes (the basic unit for respiration in human mitochondria), but the clinical expression of the mutations was correlated with the production of reactive oxygen species. Mutations that cause the Björnstad syndrome illustrate the exquisite sensitivity of ear and hair tissues to mitochondrial function, particularly to the production of reactive oxygen species.

The healthcare industry has seen an increase in the adoption of team training, such as crew resource management (CRM), to improve teamwork and coordination within acute care medical teams. A meta-analysis was carried out in order to quantify the effects of CRM training on reactions, learning, behaviour and clinical care outcomes. Biases in the research evidence are identified and recommendations for training development and evaluation are presented. PUBMED, EMBASE and PsychInfo were systematically searched for all relevant papers. Peer reviewed papers published in English between January 1985 and September 2013, which present empirically based studies focusing on interventions to improve team effectiveness in acute health care domains, were included. A total of 20 CRM-type team training evaluation studies were found to fulfil the a priori criteria for inclusion in the meta-analysis. Overall, CRM trained participants responded positively to CRM (mean score 4.25 out of a maximum of 5), the training had large effects on participants' knowledge (d=1.05), a small effect on attitudes (d=0.22) and a large effect on behaviours (d=1.25). There was insufficient evidence to support an effect on clinical care outcomes or long term impacts. The findings support the premise that CRM training can positively impact teamwork in healthcare and provide estimates of the expected effects of training. However, there is a need for greater precision in outcome assessment, improved standardisation of methods and measures, and more robust research design. Stronger evidence of effectiveness will require multi-level, multicentre, multispecialty and longitudinal studies.

BackgroundSurgical checklists has been shown to improve patient safety and teamwork in the operating theatre. However, despite the known benefits of the use of checklists in surgery, in some cases the practical implementation has been found to be less than universal. A questionnaire methodology was used to quantitatively evaluate the attitudes of theatre staff towards a modified version of the World Health Organisation (WHO) surgical checklist with relation to: beliefs about levels of compliance and support, impact on patient safety and teamwork, and barriers to the use of the checklist.MethodsUsing the theory of planned behaviour as a framework, 14 semi-structured interviews were conducted with theatre personnel regarding their attitudes towards, and levels of compliance with, a checklist. Based upon the interviews, a 27-item questionnaire was developed and distribute to all theatre personnel in an Irish hospital.ResultsResponses were obtained from 107 theatre staff (42.6% response rate). Particularly for nurses, the overall attitudes towards the effect of the checklist on safety and teamworking were positive. However, there was a lack of rigour with which the checklist was being applied. Nurses were significantly more sensitive to the barriers to the use of the checklist than anaesthetists or surgeons. Moreover, anaesthetists were not as positively disposed to the surgical checklist as surgeons and nurse. This finding was attributed to the tendency for the checklist to be completed during a period of high workload for the anaesthetists, resulting in a lack of engagement with the process.ConclusionIn order to improve the rigour with which the surgical checklist is applied, there is a need for: the involvement of all members of the theatre team in the checklist process, demonstrated support for the checklist from senior personnel, on-going education and training, and barriers to the implementation of the checklist to be addressed.

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