Refractory immune thrombocytopenic purpura: current strategies for investigation and management

There is currently no consensus on how best to manage refractory immune thrombocytopenic purpura (ITP). In part, this reflects the need for individualized treatment due to the wide spectrum of patients' requirements and responsiveness to therapies. The objective of this review is to provide a clinically useful guide to current management strategies. This article suggests investigations to identify factors that may exacerbate thrombocytopenia and underlie poor therapeutic responses, and highlights emerging therapies, including the thrombopoietic agents, which are anticipated to dramatically alter the natural history of "refractory" ITP. Morbidity, mortality and heath-related quality of life are also discussed.     

The immune thrombocytopenic purpura (ITP) bleeding score: assessment of bleeding in patients with ITP

A method for objective quantification of bleeding symptoms in immune thrombocytopenic purpura (ITP) has not been established. The ITP Bleeding Scale (IBLS) is a novel bleeding assessment system comprising 11 site-specific grades. Implementation of the IBLS on 100 patient visits revealed that although platelet count and large platelet count correlated well with bleeding symptoms overall, this relationship disappeared in marked thrombocytopenia. The IBLS is a useful clinical tool for monitoring bleeding and may be used to aid the development of laboratory parameters that correlate with underlying bleeding propensity in thrombocytopenia.     

Experiences and opinions of multi-professional non-medical oncology prescribers on post-qualification training: a qualitative study

Background: Within the UK, a non-medical prescriber is a non-medical healthcare professional who has undertaken post-registration training to gain prescribing rights. Lack of post-qualification NMP training has previously been identified as a barrier to the development of oncology non-medical prescribing practice. Aim: To explore the experiences and opinions of multi-professional non-medical oncology prescribers on post-qualification training. Method: Nine out of 30 oncology non-medical prescribers (three nurses, three pharmacists and three radiographers) from a single cancer centre in Wales, were selected from a study site NMP database using randomisation sampling within Microsoft® Excel. Participants were interviewed using a validated and piloted semi-structured interview design on the topic of post-qualification training for non-medical prescribers. Participants were invited via organisational email. Interviews were audio-recorded and transcribed verbatim. Anonymised data were thematically analysed aided by NVivo® software. Results: Main themes identified: experience related to training, competency, support and training methods. Competency assessment methods discussed were the annual non-medical prescriber appraisal, peer review and a line manager’s overarching appraisal. Support requirements identified included greater consultant input to help non-medical prescribers identify training and peer support opportunities. Organisational support was requested regarding regular study leave and governance around clinical judgement and errors. The need for regular structured in-house training related to non-medical prescriber’s level of experience was identified. Conclusion: Development of organisation-led governance strategies and in-house training programmes will support training equity for all non-medical prescribers within the organisation.

     

Supporting sexual activity in long-term care

Although nurses in almost every long-term care facility face daily challenges involving issues related to residents' sexual lives, guidelines for ethically supporting sexual activity are rare and inadequate. A decision-making framework was developed to guide care providers in responding to the sexual expression of residents in long-term care. The framework recommends that nurses should weigh the documented substantial benefits of having a sexual life against harm to the resident and others, and against offence to others. This article illustrates the use of this ethical decision-making framework by using the example of nurses supporting a resident's expression of his sexuality. It is suggested that nurses use this framework to guide their practice when related ethical issues arise.     

多发性硬化口服药值得期待

复发一缓解型多发性硬化(RRMS)有5种新型的口服治疗药物有望上市.9月份,默克递交了克拉曲滨治疗RRMS 的新药申请.同月,fingolimod(即FTY720)治疗RRMS的Ⅲ期安慰剂对照临床试验结果也出来了.五种新药作用机制不同,见表1.     

The nature of telomere fusion and a definition of the critical telomere length in human cells

The loss of telomere function can result in telomeric fusion events that lead to the types of genomic rearrangements, such as nonreciprocal translocations, that typify early-stage carcinogenesis. By using single-molecule approaches to characterize fusion events, we provide a functional definition of fusogenic telomeres in human cells. We show that approximately half of the fusion events contained no canonical telomere repeats at the fusion point; of those that did, the longest was 12.8 repeats. Furthermore, in addition to end-replication losses, human telomeres are subjected to large-scale deletion events that occur in the presence or absence of telomerase. Here we show that these telomeres are fusogenic, and thus despite the majority of telomeres being maintained at a stable length in normal human cells, a subset of stochastically shortened telomeres can potentially cause chromosomal instability. Telomere fusion was accompanied by the deletion of one or both telomeres extending several kilobases into the telomere-adjacent DNA, and microhomology was observed at the fusion points. This contrasted with telomere fusion that was observed following the experimental disruption of TRF2. The distinct error-prone mutational profile of fusion between critically shortened telomeres in human cells was reminiscent of Ku-independent microhomology-mediated end-joining.