Correction of the copper transport defect of Menkes patient fibroblasts by expression of two forms of the sheep Wilson ATPase

The Wilson disease (WD) protein (ATP7B) is a copper-transporting P-type ATPase that is responsible for the efflux of hepatic copper into the bile, a process that is essential for copper homeostasis in mammals. Compared with other mammals, sheep have a variant copper phenotype and do not efficiently excrete copper via the bile, often resulting in excessive copper accumulation in the liver. To investigate the function of sheep ATP7B and its potential role in the copper-accumulation phenotype, cDNAs encoding the two forms of ovine ATP7B were transfected into immortalised fibroblast cell lines derived from a Menkes disease patient and a normal control. Both forms of ATP7B were able to correct the copper-retention phenotype of the Menkes cell line, demonstrating each to be functional copper-transporting molecules and suggesting that the accumulation of copper in the sheep liver is not due to a defect in the copper transport function of either form of sATP7B.

Correction of a mouse model of Menkes disease by the human Menkes gene

The brindled mouse is an accurate model of the fatal human X-linked copper deficiency disorder, Menkes disease. Males carrying the mutant allele of the Menkes gene orthologue Atp7a die in the second week of life. To determine whether the genetic defect in the brindled mice could be corrected by expression of the human Menkes gene, male transgenic mice expressing ATP7A from the chicken β-actin composite promoter (CAG) were mated with female carriers of the brindled mutation (Atp7aMo-br). Mutant males carrying the transgene survived and were fertile but the copper defect was not completely corrected. Unexpectedly males corrected with one transgenic line (T25#5) were mottled and resembled carrier females, this effect appeared to be caused by mosaic expression of the transgene. In contrast, males corrected with another line (T22#2) had agouti coats. Copper concentrations in tissues of the rescued mutants also resembled those of the heterozygous females, with high levels in kidney (84.6 ± 4.9 μg/g in corrected males vs. 137.0 ± 44.3 μg/g in heterozygotes) and small intestine (15.6 ± 2.5 μg/g in corrected males vs. 15.7 ± 2.8 μg/g in heterozygotes). The results show that the Menkes defect in mice is corrected by the human Menkes gene and that adequate correction is obtained even when the transgene expression does not match that of the endogenous gene.

Correction of presbyopia with GP contact lenses

Presbyopia can be safely and effectively corrected with gas permeable (GP) multifocal (bifocal) contact lenses, but statistics indicate that they remain the most underutilized contact lenses on the market. We believe that practitioners have been “turned off of” GP multifocals due to their experiences with older generations of these lenses, which tended to be difficult to fit, with poor visual results.

Fortunately, GP multifocals have come a long way, and practitioners who avoid fitting them are missing out on an important niche market. With this booklet, we hope to show you just how far GP multifocals have come and demonstrate the ease with which they can now be fit and managed.

Comparisons of sidewall correction of bed shear stress in open-channel flows

When investigating sediment transport in laboratory open-channel flows, it is often necessary to remove sidewall effects for computing effective bed shear stress. Previous sidewall correction methods are subject to some assumptions that have not been completely verified, and different values of the bed shear stress may be obtained depending on the approach used in making sidewall corrections. This study provides a quantitative assessment of the existing correction procedures by comparing them to a new sidewall correction model proposed in this study. The latter was derived based on the shear stress function and equivalent roughness size for both rigid and mobile bed conditions, which were obtained directly from experimental measurements. The comparisons show that the Einstein correction formula and the Vanoni and Brooks method generally predict relatively lower and higher bed shear stresses, respectively, while the Williams’ empirical function leads to more scatter. This study also demonstrates that the widely used Vanoni and Brooks approach can be well approximated by a simple formula derived based on the Blasius resistance function. The sidewall effects, when removed in the different ways, would consequently affect the presentation of the bedload function. Experimental results of bedload transport, when plotted as the dimensionless transport rate against the dimensionless shear stress with the latter being corrected using the present model, exhibit less scatter than those associated with the previous procedures.

Does implicit motor imagery ability predict reaching correction efficiency? A test of recent models of human motor control

Neurocomputational models of reaching indicate that efficient purposive correction of movement midflight (e.g., online control) depends on one's ability to generate and monitor an accurate internal (neural) movement representation. In the first study to test this empirically, the authors investigated the relationship between healthy young adults’ implicit motor imagery performance and their capacity to correct their reaching trajectory. As expected, after controlling for general reaching speed, hierarchical regression demonstrated that imagery ability was a significant predictor of hand correction speed; that is, faster and more accurate imagery performance associated with faster corrections to reaching following target displacement at movement onset. They argue that these findings provide preliminary support for the view that a link exists between an individual's ability to represent movement mentally and correct movement online efficiently.

Who, when and where? Identification of patients at risk of an in-hospital adverse event: Implications for nursing practice

Patients who suffer an adverse event (AE) are more likely to die or suffer permanent disability. Many AEs are preventable. Nurses have long played a pivotal role in the prevention of AEs. Much of the literature to date pertains to the role of nurses in the prevention of AEs such as falls, pressure areas and deep vein thrombosis. Prominent risk factors for AEs are the presence of physiological abnormality, failure to recognize or correct physiological abnormality, advanced patient age and location of patient room. Ongoing physiological assessment of patients is a nursing responsibility and the assessment findings of nurses underpin many patient care decisions. The early recognition and correction of physiological abnormality can improve patient outcomes by reducing the incidence of AEs, making nurses' ability to identify, interpret and act on physiological abnormality a fundamental factor in AE prediction and prevention. This paper will examine the role of nurses in AE prevention, using cardiac arrest as an example, from the perspective of physiological safety; that is, accurate physiological assessment and the early correction of physiological abnormality.

The role of nurses in preventing adverse events related to respiratory dysfunction: literature review

Aims. This paper reports a literature review examining the relationship between specific clinical indicators of respiratory dysfunction and adverse events, and exploring the role of nurses in preventing adverse events related to respiratory dysfunction.

Background. Adverse events in hospital are associated with poor patient outcomes such as increased mortality and permanent disability. Many of these adverse events are preventable and are preceded by a period during which the patient exhibits clearly abnormal physiological signs. The role of nurses in preserving physiological safety by early recognition and correction of physiological abnormality is a key factor in preventing adverse events.

Methods. A search of the Medline and CINAHL databases was conducted using the following terms: predictors of poor outcome, adverse events, mortality, cardiac arrest, emergency, oxygen, supplemental oxygen, oxygen therapy, oxygen saturation, oxygen delivery, assessment, patient assessment, physical assessment, dyspnoea, hypoxia, hypoxaemia, respiratory assessment, respiratory dysfunction, shortness of breath and pulse oximetry. The papers reviewed were research papers that demonstrated a relationship between adverse events and various clinical indicators of respiratory dysfunction.

Results. Respiratory dysfunction is a known clinical antecedent of adverse events such as cardiac arrest, need for medical emergency team activation and unplanned intensive care unit admission. The presence of respiratory dysfunction prior to an adverse event is associated with increased mortality. The specific clinical indicators involved are alterations in respiratory rate, and the presence of dyspnoea, hypoxaemia and acidosis.

Conclusions. The way in which nurses assess, document and use clinical indicators of respiratory dysfunction is influential in identifying patients at risk of an adverse event and preventing adverse events related to respiratory dysfunction. If such adverse events are to be prevented, nurses must not only be able to recognise and interpret signs of respiratory dysfunction, but must also take responsibility for initiating and evaluating interventions aimed at correcting respiratory dysfunction.

The effects of treating male hypogonadism on couples' sexual desire and function

Introduction: Hypogonadism is a common endocrine condition characterized by low levels of testosterone (T) and marked by numerous symptoms, one of which is low sexual desire. Studies comparing T delivery systems have suggested that hypogonadal men’s partners may be at risk from exposure to T gels. Little other mention is found of the impact of hypogonadism and its treatment on a man’s partner and the couple’s sexual function.

Aim: To assess sexual desire and sexual function in hypogonadal men and their woman partners before and after treatment with T replacement therapy.

Methods: Twenty-one hypogonadal men and 18 partners were recruited from a   tertiary endocrine clinic, and were compared with a control group of 20 eugonadal age-matched men and their partners. All men had baseline blood tests to confirm their status as hypogonadal or eugonadal, and hypogonadal men repeated tests at 3-month intervals. All participants completed the Sexual Desire Inventory (SDI) and sexual function questionnaires at baseline and at 3-month intervals until the hypogonadal men attained normal T levels.

Main Outcome Measures: Pre- and post-treatment SDI and sexual function questionnaires were compared once T normalization was achieved. Between- and within-group comparisons were carried out.

Results: Pretreatment hypogonadal men recorded lower levels of sexual desire and function than controls, but significantly improved once hypogonadism was corrected. Eugonadal controls recorded no significant changes in either sexual desire or function during the study. Partners of the hypogonadal men reported no changes on the SDI, but significant improvements in sexual function as their partners recovered.

Conclusion: SDI and sexual function measures reflect sexual changes that  accompany rising serum T levels during correction of male hypogonadism. Women partners reported more satisfaction, less pain, and improved sexual function following the men’s treatment. Treatments affecting one partner potentially have important effects on the other.

An international survey of contact lens prescribing for presbyopia

Purpose:  The aim was to determine world-wide patterns of fitting contact lenses for the correction of presbyopia.

Methods:  Up to 1,000 survey forms were sent to contact lens fitters in each of 38 countries between January and March every year over five consecutive years (2005 to 2009). Practitioners were asked to record data relating to the first 10 contact lens fittings or refittings performed after receiving the survey form.

Results:  Data were received relating to 16,680 presbyopic (age 45 years or older) and 84,202 pre-presbyopic (15 to 44 years) contact lens wearers. Females are over-represented in presbyopic versus pre-presbyopic groups, possibly reflecting a stronger desire for the cosmetic benefits of contact lenses among older women. The extent to which multifocal and monovision lenses are prescribed for presbyopes varies considerably among nations, ranging from 79 per cent of all soft lenses in Portugal to zero in Singapore. There appears to be significant under-prescribing of contact lenses for the correction of presbyopia, although for those who do receive such corrections, three times more multifocal lenses are fitted compared with monovision fittings. Presbyopic corrections are most frequently prescribed for full-time wear and monthly replacement.

Conclusions:  Despite apparent improvements in multifocal design and an increase in available multifocal options in recent years, practitioners are still under-prescribing with respect to the provision of appropriate contact lenses for the correction of presbyopia. Training of contact lens practitioners in presbyopic contact lens fitting should be accelerated and clinical and laboratory research in this field should be intensified to enhance the prospects of meeting the needs of presbyopic contact lens wearers more fully.

Investigation of the performance of the menifocal Z gas-permeable bifocal contact lens during continuous wear

Purpose. The Menifocal Z is an alternating vision, concentric, bifocal gas-permeable (GP) contact lens; center distance is connected to near periphery by a smooth transition zone. The lens is produced using tisilfocon A (Menicon Z material), which is approved for up to 30 days of continuous wear (CW). The aim of this study was to evaluate the clinical performance of the Menifocal Z when worn for up to 30 days of CW for 6 months.

Methods. Thirty-five existing GP lens wearers were enrolled in the study. Subjects were fitted with Menifocal Z lenses and follow-up visits were conducted after 2 weeks of daily wear and 1 day, 1 week, 6 weeks, 3 and 6 months of CW. A range of objective and subjective clinical performance measures were assessed, including distance and near visual acuity, the physiological response to CW, and subjective evaluation of vision and comfort.

Results. Twenty-seven subjects (77%) completed the study and eight (23%) discontinued: five (14%) as a result of lens-related problems (four vision, one comfort) and three (9%) as a result of non-lens related reasons. Average CW time achieved by the subjects was 22 ± 2 days. Mean binocular logarithm of the minimum angle of resolution (logMAR) acuities at 6 months were: high contrast distance 0.03 (20/20-), low contrast distance 0.63 (20/80-), and high contrast near 0.26 (20/25, N4). Adverse responses and lens binding were minimal, and there were no significant increases in corneal staining, corneal vascularization, or superior palpebral conjunctival papillae over time (p > 0.05). Problems with night vision (distance and near) with the lenses were the most common difficulties reported by the subjects.

Conclusions. The Menifocal Z appears to be a promising option for presbyopic vision correction, providing successful correction of distance and near vision in a group of experienced GP lens wearers. The hyper Dk tisilfocon A (Menicon Z) material allowed for safe wear of the lenses on a CW basis.

Comparison of a self-processed EM3000 multibeam echosounder dataset with a QTC view habitat mapping and a sidescan sonar imagery, Tamaki Strait, New Zealand

A methodology for automatically processing the data files from an EM3000 multibeam echosounder (Kongsberg Maritime, 300 kHz) is presented. Written in MatLab, it includes data extraction, bathymetry processing, computation of seafloor local slope, and a simple correction of the backscatter along-track banding effect. The success of the latter is dependent on operational restrictions, which are also detailed. This processing is applied to a dataset acquired in 2007 in the Tamaki Strait, New Zealand. The resulting maps are compared with a habitat classification obtained with the acoustic ground-discrimination software QTC View linked to a 200-kHz single-beam echosounder and to the imagery from a 100-kHz sidescan sonar survey, both performed in 2002. The multibeam backscatter map was found to be very similar to the sidescan imagery, quite correlated to the QTC View map on one site but mainly uncorrelated on another site. Hypotheses to explain these results are formulated and discussed. The maps and the comparison to prior surveys are used to draw conclusions on the quality of the code for further research on multibeam benthic habitat mapping.

Police officers' perceptions of interviewing offenders on sex offender registries

Police interviews with offenders form an important component of the sex offender registry monitoring process. This study provides an analysis of police professionals’ perceptions of the benefits and process of interviewing offenders on sex offender registries. Participants were a heterogeneous sample of 24 Australian police personnel whose roles were primarily associated with the operation of sex offender registries across three different jurisdictions. Participants’ perceptions of sex offender registry interviews were elicited using non-directive prompts in focus groups of between two and five officers. Transcripts of these focus groups were analysed andkey themes identified. Reported benefits related to the identification and correction of misunderstandings about registry requirements and elicitation of information to assist ongoing management and investigation of new offences. Further, establishing a sound relationship enabled proactive support of the offender in his/her attempt not to re-offend. Interview procedures emphasised the importance of genuine engagement and a relationship based on trust. The findings highlighted several challenges to interviewing and directions for further training, support and research. Participants’ responses underscore the significant role that interviews play in the effective implementation of sex offender registries and emphasise the need for successful engagement of offenders during these interviews to improve the utility of registry schemes. This was the first study to examine the strengths and challenges of interviewing offenders on the registry from a policing perspective.