Clinical and surgical data of affected members of a classic CFEOM 1 family

BACKGROUND: Congenital fibiosis of the extraocular muscles (CFEOM1) refers to a group of congenital eye movement disorders that are characterized by non-progressive restrictive ophthalmoplegia. We present clinical and surgical data on affected members of a classic CFEOM1 family. METHODS: Ten members of a fifteen-member, three-generation Italian family affected by classic CFEOM participated in this study. Each affected family member underwent ophthalmologic (corrected visual acuity, pupillary function, anterior segment and fundus examination), orthoptic (cover test, cover-uncover test, prism alternate cover test), and preoperative examinations. Eight of the ten affected members had surgery and underwent postoperative examinations. Surgical procedures are listed. RESULTS: All affected members were born with varying degrees of bilateral ptosis and ophthalmoplegia with both eyes fixed in a hypotropic position (classic CFEOM). The affected members clinical data prior to surgery, surgery procedures and postoperative outcomes are presented. On 14 operated eyes to correct ptosis there was an improvement in 12 eyes. In addition, the head position improved in all patients. CONCLUSIONS: Surgery is effective at improving ptosis in the majority of patients with classic CFEOM. However, the surgical approach should be individualized to each patient, as inherited CFEOM exhibits variable expressivity and the clinical features may differ markedly between affected individuals, even within the same family

Comparison of a new transcutaneous bilirubinometer (Bilimed®) with serum bilirubin measurements in preterm and full-term infants

<p>Abstract</p> <p>Background</p> <p>The gold standard to assess hyperbilirubinemia in neonates remains the serum bilirubin measurement. Unfortunately, this is invasive, painful, and costly. Bilimed^®, a new transcutaneous bilirubinometer, suggests more accuracy compared to the existing non-invasive bilirubinometers because of its new technology. It furthermore takes into account different skin colours. No contact with the skin is needed during measurement, no additional material costs occur. Our aim was to assess the agreement between the Bilimed^®and serum bilirubin in preterm and term infants of different skin colours.</p> <p>Methods</p> <p>The transcutaneous bilirubin measurements were performed on the infant's sternum and serum bilirubin was determined simultaneously. The agreement between both methods was assessed by Pearson's correlation and by Bland-Altman analysis.</p> <p>Results</p> <p>A total of 117 measurement cycles were performed in 99 term infants (group1), further 47 measurements in 38 preterm infants born between 34 - 36 6/7 gestational weeks (group 2), and finally 21 measurements in 13 preterm infants born between 28 - 33 6/7 gestational weeks (group 3). The mean deviation and variability (+/- 2SD) of the transcutaneous from serum bilirubin were: -14 (+/- 144) μmol/l; -0.82 (+/- 8.4) mg/dl in group 1, +16 (+/- 91) μmol/l;+0.93(+/- 5.3) mg/dl in group 2 and -8 (+/- 76) μmol/l; -0.47 (+/- 4.4) mg/dl in group 3. These limits of agreement are too wide to be acceptable in a clinical setting. Moreover, there was to be a trend towards less good agreement with increasing bilirubin values.</p> <p>Conclusion</p> <p>Despite its new technology the Bilimed^®has no advantages, and more specifically no better agreement not only in term and near-term Caucasian infants, but also in non-Caucasian and more premature infants.</p

Computer-aided DSM-IV-diagnostics – acceptance, use and perceived usefulness in relation to users' learning styles

BACKGROUND: CDSS (computerized decision support system) for medical diagnostics have been studied for long. This study was undertaken to investigate how different preferences of Learning Styles (LS) of psychiatrists might affect acceptance, use and perceived usefulness of a CDSS for diagnostics in psychiatry. METHODS: 49 psychiatrists (specialists and non-specialists) from 3 different clinics volunteered to participate in this study and to use the CDSS to diagnose a paper-based case (based on a real patient). LS, attitudes to CDSS and complementary data were obtained via questionnaires and interviews. To facilitate the study, a special version of the CDSS was created, which automatically could log interaction details. RESULTS: The LS preferences (according to Kolb) of the 49 physicians turned out as follows: 37% were Assimilating, 31% Converging, 27% Accommodating and 6% Diverging. The CDSS under study seemed to favor psychiatrists with abstract conceptualization information perceiving mode (Assimilating and Converging learning styles). A correlation between learning styles preferences and computer skill was found. Positive attitude to computer-aided diagnostics and learning styles preferences was also found to correlate. Using the CDSS, the specialists produced only 1 correct diagnosis and the non-specialists 2 correct diagnoses (median values) as compared to the three predetermined correct diagnoses of the actual case. Only 10% had all three diagnoses correct, 41 % two correct, 47 % one correct and 2 % had no correct diagnose at all. CONCLUSION: Our results indicate that the use of CDSS does not guarantee correct diagnosis and that LS might influence the results. Future research should focus on the possibility to create systems open to individuals with different LS preferences and possibility to create CDSS adapted to the level of expertise of the user

Nature reserves as catalysts for landscape change

Scientists have called repeatedly for a broader conservation agenda that emphasizes not only protected areas but also the landscapes in which those areas are embedded. We describe key advances in the science and practice of engaging private landowners in biodiversity conservation and propose a conceptual model for integrating conservation management on reserves and privately owned lands. The overall goal of our model is to blur the distinction between land management on reserves and the surrounding landscapes in a way that fosters widespread implementation of conservation practices. Reserves assume a new role as natural laboratories where alternative land-use practices, designed to achieve conservation objectives, can be explored. We articulate the details of the model using a case study from the North American tallgrass prairie ecoregion.Peer reviewedNatural Resource Ecology and Managemen

Can gold be used as a hedge against the risks of Sharia-compliant securities? Application for Islamic portfolio management

In this paper, we investigate whether gold hedges Sharia-compliant stocks and Sukuk during the period from September 2005 to October 2017. The inference is taken by using both the DCC-GARCH model and the wavelet coherence analysis. On the whole, our finding suggests that gold is not effective in hedging the fluctuations of Sharia-compliant securities. However, we find that combining gold with stocks (and Sukuk) is useful in diversification and portfolio optimization. These results imply that, while gold is an excellent hedge for plain vanilla securities, it is not for Islamic exposures. This is important in light of the increasing amount of assets that are managed according to Islamic screening

The Vulnverability Cube: A Multi-Dimensional Framework for Assessing Relative Vulnerability

The diversity and abundance of information available for vulnerability assessments can present a challenge to decision-makers. Here we propose a framework to aggregate and present socioeconomic and environmental data in a visual vulnerability assessment that will help prioritize management options for communities vulnerable to environmental change. Socioeconomic and environmental data are aggregated into distinct categorical indices across three dimensions and arranged in a cube, so that individual communities can be plotted in a three-dimensional space to assess the type and relative magnitude of the communities’ vulnerabilities based on their position in the cube. We present an example assessment using a subset of the USEPA National Estuary Program (NEP) estuaries: coastal communities vulnerable to the effects of environmental change on ecosystem health and water quality. Using three categorical indices created from a pool of publicly available data (socioeconomic index, land use index, estuary condition index), the estuaries were ranked based on their normalized averaged scores and then plotted along the three axes to form a vulnerability cube. The position of each community within the three-dimensional space communicates both the types of vulnerability endemic to each estuary and allows for the clustering of estuaries with like-vulnerabilities to be classified into typologies. The typologies highlight specific vulnerability descriptions that may be helpful in creating specific management strategies. The data used to create the categorical indices are flexible depending on the goals of the decision makers, as different data should be chosen based on availability or importance to the system. Therefore, the analysis can be tailored to specific types of communities, allowing a data rich process to inform decision-making

The influence of input and output modality on following instructions in working memory

Following instructions is an important component of learning and has been shown to rely on working memory. This study examined the ability to follow instructions within working memory under varying input and output modalities. In Experiment 1, participants heard, read, or viewed demonstration of short sequences of instructions, and recalled either by oral repetition or physical enactment. There was a significant main effect of encoding, showing superior recall performance when instructions were demonstrated relative to spoken or written presentation. Experiment 2 examined whether recall is further improved when instructions are presented both in spoken and demonstrated form, relative to single modality presentation. The advantage for demonstration over spoken instructions was replicated, and dual input was superior to spoken instructions. However, dual input did not bring extra benefit compared to demonstration of instructions. We also observed a significant enacted-retrieval recall advantage. These findings suggest effects of both input and output modalities on the ability to remember and follow instructions in working memory. Outcomes substantially inform the underexplored but important new area of action-based working memory and its links to embodied cognition, with implications for pedagogic practice