bluuebunny/crossref_metadata_2025 huggingface dataset:
Commissioning Software on New Build Drillships: Lessons Learned
Authors: Lanier Greg
Published: August 2013

Commissioning software on a drillship involves many programmers, some located remotely, where all personnel are under time pressure to quickly complete the software tasks. Managing software changes under these conditions is difficult; leading to a deviation of the personnel involved in the project. Deviations from quality procedures in many cases lead to loss of time and negatively impact software quality. As an example, software on three of the drilling control chairs was updated and the fourth drilling control chair was missed, which causes improper integration and potentially leads to safety issues and ultimately downtime.

Maintaining software quality during commissioning of the ship is very challenging and becomes more complicated when control system software interfaces have not been verified before commissioning. Early verification of the software provides a known foundation and opportunity to discover concept errors and software defects in advance. Before commissioning, updates and changes can be evaluated more carefully, helping to provide thoughtful consideration of options and impact of the changes to the system’s software as a whole.

At the Factory Acceptance Test (FAT), the software verification may not occur and sometimes the software in the control system is only to demonstrate mechanical functions of the hardware. This leads to insufficiently tested and thus, unacceptable software being delivered with the equipment initially.

The Human Machine Interface (HMI) is another piece of software that has to be updated to match the changes made to the control system software it is connected to. In some cases, the personnel programming the HMIs are separate from the personnel programming the control system, which leads to failed functions and delays.

Software changes of any form must be controlled throughout the process. Modifications made to one software function can affect previously approved and working functions which may lead to unexpected functioning of the software. To mitigate these errors, regression testing of the functions is essential. Without doing so, defects introduced during commissioning may not be discovered until the operational phase of the ship, which can prove much more costly so late in the process.

Following the management of change program requires commitment and communication because of the extensive details involved with all programs.

Effort must be expended to keep communication between programmers functioning smoothly. They have to maintain a common goal and focus on the software change and management should enforce software quality procedures.
A Numerical Investigation Into Ultimate Strength and Buckling Behavior of Locally Corroded Steel Tubular Members
Authors: Khedmati Mohammad Reza , Nazari Masoud , Khalaj Amir Foad
Published: August 2013

In this paper an investigation is presented based on numerical modeling of locally corroded tubular members. A parametric finite element approach was used in order to simulate behavior of damaged members under axial compressive loads. The results were then examined against an available experimental test. Validated models are used to derive a semi-empirical formula for predicting ultimate strength of locally damaged tubes as a function of corrosion dimensions.

Geometry of corrosion can be defined by its depth, length, width and location of damage along the tube. In this study it is focused on the effect of some parameters that have not been addressed yet by other researchers, e.g. slenderness of the tubes and location of patch corrosion.

It was found that location of corrosion has great effect on reduction of ultimate strength. Effect of corrosion geometry was also studied and it was shown that tubes with different corrosion dimensions show different behaviors under compressive loads.

In cases with severe corrosion damages, the occurrence of local buckling plays an important role on reduction of ultimate strength and deformation of damaged region.

The effect of tubular slenderness on behavior of axially compressed tubes was also studied and formulated.
Nova Project: Lessons Learnt During the Conceptual Phase of the Design of a Floating Support Structure for an Offshore Vertical Axis Wind Turbine
Authors: Collu Maurizio , Brennan Feargal P. , Patel Minoo H.
Published: August 2013

The Energy Technologies Institute (ETI, UK) funded project called NOVA (for Novel Vertical Axis wind turbine) examined the feasibility of a large offshore vertical axis wind turbine in the 5 to 10 MW power range, with a view toward 20 MW. The NOVA feasibility study required a methodology to be developed to select the best configuration, based on the system dynamics. Two options have been analysed: fixed and floating support structure, with the floating option is here analysed.

The design space has been investigated, ranking the possible options using a multi-criteria decision making (MCDM) method called TOPSIS. Based on this, two configurations have been promoted to the next analysis stage: a barge and a semisubmersible.

The evolution of the configuration designs, necessary to optimise and compare these two options, is here presented, taking their dynamics and costs into account. The barge concept evolved to the ‘triple doughnut-Miyagawa’ concept, consisting of an annular cylindrical shape with an inner (to control the damping) and outer (to control added mass) bottom flat plates. The semisubmersible was optimised to obtain the best trade-off between dynamic behaviour and amount of material needed. The main conclusion is that, in general, the requirement driving the design is a good dynamic response to waves. If only basic requirements were taken into account (ability to float and ability to counteract the wind turbine inclining moment), a much smaller, lighter, and cheaper structure would fulfil them.
Influence of Model Geometry and Boundary Conditions on the Ultimate Strength of Stiffened Panels Under Uniaxial Compressive Loading
Authors: Xu Mingcai , Fujikubo Masahiko , Soares C. Guedes
Published: August 2013

The aim of this paper is to find out an appropriate configuration of boundary conditions and geometric model to calculate the ultimate strength of a continuous stiffened panel under uniaxial compressive loading in FE analysis. The 1+1 bays model with periodical symmetric boundary conditions is proposed to be used in FE analysis, whose results are compared with 1/2+1+1/2 bays model with periodical symmetric and symmetric boundary conditions, and 1/2+1+1+1/2 bays model with symmetric boundary conditions. The effects of the continuity of the stiffened panel with different geometric models and boundary conditions on its collapse mode are investigated. A beam tension test has been used to define the true stress-strain relationship.
Study of Landing and Lift-Off Operation for Wind Turbine Components on a Ship Deck
Authors: Graczyk Mateusz , Sandvik Peter Christian
Published: August 2013

Access to offshore wind turbines is much more challenging and costly than for the land-based turbines. One of the factors affecting choice of the vessel to perform the maintenance work is its performance in waves.

Performance of two different ships that may be used for exchange of light components for offshore wind turbines is analyzed.

Vertical motion characteristics (displacement and velocity) and forces/accelerations acting on such components and lifting system during lift-off and disposal operation are investigated.

Based on the analysis performed for a monohull and a catamaran, limiting sea-states are calculated and weather windows allowing for performing exchange operations are identified.
Circadian Gene
Bmal1
Regulates Diurnal Oscillations of Ly6C
hi
Inflammatory Monocytes
Authors: Nguyen Khoa D. , Fentress Sarah J. , Qiu Yifu , Yun Karen , Cox Jeffery S. , Chawla Ajay
Published: August 2013

Diurnal Immunology

Like most organ systems in the body, several components of the immune system appear to be regulated in a diurnal manner. However, the cell populations affected, the underlying molecular mechanisms controlling these processes, and the functional consequences of such regulation are poorly understood. Now,

Nguyen
et al.

(p.
1483
, published online 22 August; see the Perspective by

Druzd and Scheiermann

) have found that trafficking of monocytes to sites of inflammation is regulated in a diurnal manner in mice. Mice harboring a myeloid cell-specific deletion in the clock protein, BMAL1, showed reduced fitness in response to both acute and chronic inflammation.
Draft Genome Sequence of the Fast-Growing Bacterium Vibrio natriegens Strain DSMZ 759
Authors: Maida Isabel , Bosi Emanuele , Perrin Elena , Papaleo Maria Cristiana , Orlandini Valerio , Fondi Marco , Fani Renato , Wiegel Juergen , Bianconi Giovanna , Canganella Francesco
Published: August 2013

Vibrio natriegens
is a Gram-negative bacterium known for its extremely short doubling time. Here we present the annotated draft genome sequence of
Vibrio natriegens
strain DSMZ 759, with the aim of providing insights about its high growth rate.
The cost-effectiveness of diagnostic management strategies for children with minor head injury
Authors: Holmes M W , Goodacre S , Stevenson M D , Pandor A , Pickering A
Published: August 2013

Aim

To estimate the cost-effectiveness of diagnostic management strategies for children with minor head injury and identify an optimal strategy.

Methods

A probabilistic decision analysis model was developed to estimate the costs and quality-adjusted life years (QALYs) accrued by each of six potential management strategies for minor head injury, including a theoretical ‘zero option’ strategy of discharging all patients home without investigation. The model took a lifetime horizon and the perspective of the National Health Service.

Results

The optimal strategy was based on the Children's Head injury Algorithm for the prediction of Important Clinical Events (CHALICE) rule, although the costs and outcomes associated with each strategy were broadly similar.

Conclusions

Liberal use of CT scanning based on a high sensitivity decision rule is not only effective but also cost saving, with the CHALICE rule being the optimal strategy, although there is some uncertainty in the results. Incremental changes in the costs and QALYs are very small when all selective CT strategies are compared. The estimated cost of caring for patients with brain injury worsened by delayed treatment is very high compared with the cost of CT scanning. This analysis suggests that all hospitals receiving children with minor head injury should have unrestricted access to CT scanning for use in conjunction with evidence-based guidelines.
Candida dubliniensisencrustation of an obstructing upper renal tract calculus
Authors: O'Kane Dermot , Kiosoglous Anthony , Jones Kay
Published: August 2013

We present the case of a 53-year-old man, with a history of alcohol abuse, requiring intensive care unit admission, with an obstructing right upper renal calculus andKlebsiella pneumoniaeurosepsis. Ureteroscopic treatment of this obstruction displayed a small calculus within the renal pelvis completely encapsulated within a fungal bezoar. Laboratory analysis of the fungal mass found it to beCandida dubliniensis.
Atypical abdominal pain: post-traumatic transverse colon stricture
Authors: Rotar Raluca , Uwechue Raphael , Sasapu Kishore Kumar
Published: August 2013

A driver presented to the emergency department 1 day after an accident driving his excavator with abdominal pain and vomiting. He was admitted to the surgical ward 2 days later, after reattending. A CT scan revealed wall thickening and oedema in the transverse colon. This was supported by a subsequent CT virtual colonoscopy which raised the suspicion of neoplasia. A follow-up colonoscopy was not carried further than the transverse colon due to an indurated, tight stricture. Biopsies from that area showed ulceration and inflammatory changes non-specific for ischaemia, drug-induced changes or inflammatory bowel disease. As a consequence of the subocclusive symptoms and the possibility of a neoplastic diagnosis, a laparoscopic-assisted transverse colectomy was performed. The histology of the resected segment revealed post-traumatic inflammation and fibrosis with no evidence of neoplasia.
Glenohumeral joint tuberculosis with multiple cold abscesses: an uncommon cause of shoulder pain
Authors: Kizildag Betul , Sener Alper , Komurcu Erkam , Karatag Ozan , Kosar Sule
Published: August 2013

The musculoskeletal system involvement of tuberculosis (TB) other than spinal TB is a rare and less-known entity. Furthermore symptoms of musculoskeletal involvement of TB infection are non-spesific, relatively slow and indolent. The diagnosis of TB is less considered by clinicians among other common causes of shoulder pain including traumatic and degenerative conditions. We reported a 72-year-old patient suffering shoulder pain for the last 2 years who was diagnosed with glenohumeral joint TB arthrithis associated with multiple cold abscesses in adjacent soft tissues; emphasising significant clinical and imaging findings.
Prins cyclization of α-bromoethers under basic conditions
Authors: Arpin Patrice , Hill Bryan , Larouche-Gauthier Robin , Spino Claude
Published: August 2013

α-Bromoethers have been found to undergo Prins-type cyclization under basic conditions and without the need to add a promoter. The products are those derived from a Markovnikov addition on the pendant alkene. However, the stereochemistry and even the structure of the products sometimes differ from those expected with the classical Lewis-acid-catalyzed Prins reaction of acetals.
MULTI-OBJECTIVE EVOLUTIONARY ALGORITHMS FOR FILTER BASED FEATURE SELECTION IN CLASSIFICATION
Authors: XUE BING , CERVANTE LIAM , SHANG LIN , BROWNE WILL N. , ZHANG MENGJIE
Published: August 2013

Feature selection is a multi-objective problem with the two main conflicting objectives of minimising the number of features and maximising the classification performance. However, most existing feature selection algorithms are single objective and do not appropriately reflect the actual need. There are a small number of multi-objective feature selection algorithms, which are wrapper based and accordingly are computationally expensive and less general than filter algorithms. Evolutionary computation techniques are particularly suitable for multi-objective optimisation because they use a population of candidate solutions and are able to find multiple non-dominated solutions in a single run. However, the two well-known evolutionary multi-objective algorithms, non-dominated sorting based multi-objective genetic algorithm II (NSGAII) and strength Pareto evolutionary algorithm 2 (SPEA2) have not been applied to filter based feature selection. In this work, based on NSGAII and SPEA2, we develop two multi-objective, filter based feature selection frameworks. Four multi-objective feature selection methods are then developed by applying mutual information and entropy as two different filter evaluation criteria in each of the two proposed frameworks. The proposed multi-objective algorithms are examined and compared with a single objective method and three traditional methods (two filters and one wrapper) on eight benchmark datasets. A decision tree is employed to test the classification performance. Experimental results show that the proposed multi-objective algorithms can automatically evolve a set of non-dominated solutions that include a smaller number of features and achieve better classification performance than using all features. NSGAII and SPEA2 outperform the single objective algorithm, the two traditional filter algorithms and even the traditional wrapper algorithm in terms of both the number of features and the classification performance in most cases. NSGAII achieves similar performance to SPEA2 for the datasets that consist of a small number of features and slightly better results when the number of features is large. This work represents the first study on NSGAII and SPEA2 for filter feature selection in classification problems with both providing field leading classification performance.
A NEW ENSEMBLE LEARNING ALGORITHM USING REGIONAL CLASSIFIERS
Authors: LEE BYUNGWOO , CHOI SUNGHA , OH BYONGHWA , YANG JIHOON , PARK SUNGYONG
Published: August 2013

We present a new ensemble learning method that employs a set of regional classifiers, each of which learns to handle a subset of the training data. We split the training data and generate classifiers for different regions in the feature space. When classifying an instance, we apply a weighted voting scheme among the classifiers that include the instance in their region. We used 11 datasets to compare the performance of our new ensemble method with that of single classifiers as well as other ensemble methods such as RBE, bagging and Adaboost. As a result, we found that the performance of our method is comparable to that of Adaboost and bagging when the base learner is C4.5. In the remaining cases, our method outperformed other approaches.
A NOVEL FEATURE SELECTION ALGORITHM WITH SUPERVISED MUTUAL INFORMATION FOR CLASSIFICATION
Authors: PALANICHAMY JAGANATHAN , RAMASAMY KUPPUCHAMY
Published: August 2013

Feature selection is essential in data mining and pattern recognition, especially for database classification. During past years, several feature selection algorithms have been proposed to measure the relevance of various features to each class. A suitable feature selection algorithm normally maximizes the relevancy and minimizes the redundancy of the selected features. The mutual information measure can successfully estimate the dependency of features on the entire sampling space, but it cannot exactly represent the redundancies among features. In this paper, a novel feature selection algorithm is proposed based on maximum relevance and minimum redundancy criterion. The mutual information is used to measure the relevancy of each feature with class variable and calculate the redundancy by utilizing the relationship between candidate features, selected features and class variables. The effectiveness is tested with ten benchmarked datasets available in UCI Machine Learning Repository. The experimental results show better performance when compared with some existing algorithms.
LOW-MAGNETIC-FIELD MAGNETARS
Authors: TUROLLA ROBERTO , ESPOSITO PAOLO
Published: August 2013

It is now widely accepted that soft gamma repeaters and anomalous X-ray pulsars are the observational manifestations of magnetars, i.e. sources powered by their own magnetic energy. This view was supported by the fact that these "magnetar candidates" exhibited, without exception, a surface dipole magnetic field (as inferred from the spin-down rate) in excess of the electron critical field (≃ 4.4×1013 G). The recent discovery of fully qualified magnetars, SGR 0418+5729 and Swift J1822.3-1606, with dipole magnetic field well in the range of ordinary radio pulsars posed a challenge to the standard picture, showing that a very strong field is not necessary for the onset of magnetar activity (chiefly bursts and outbursts). Here we summarize the observational status of the low-magnetic-field magnetars and discuss their properties in the context of the mainstream magnetar model and its main alternatives.
China's New Leaders
Authors: BO Zhiyue
Published: August 2013

At the 18th National Party Congress, the fourth generation leadership under Hu Jintao transferred its power to the fifth generation leadership under Xi Jinping. The Congress elected a central committee of 376 members and a central disciplinary commission of 130 members. Hu Jintao also retired from the Central Military Commission (CMC). In his inaugural speech, Xi Jinping emphasised his commitment to rejuvenating the Chinese nation and made anti-corruption one of his highest priorities.
Mineralogy and spectral reflectance of soils and tailings from historical gold mines, Nova Scotia
Authors: Percival J.B. , White H.P. , Goodwin T.A. , Parsons M.B. , Smith P.K.
Published: August 2013

Gold was mined in 64 districts in southern Nova Scotia between 1861 and the early 1940s, followed by limited, intermittent production up to the present. There is extensive dispersion of arsenic- and mercury-bearing mine tailings in the receiving environment downstream from many of these sites. Elevated mercury concentrations, highest near old stamp mill foundations, occur because of the mercury amalgamation process used to extract gold until the 1940s. Arsenic, on the other hand, occurs naturally in arsenopyrite, which is associated with the gold-bearing quartz veins and host rocks. Tailings are composed of fine sand- to silt-sized quartz, feldspar, illite and chlorite, and represent the primary rock-forming minerals in the metasedimentary host rocks of the Cambro-Ordovician Meguma Supergroup. Carbonate and sulphide minerals occur in minor to trace amounts, along with secondary minerals such as scorodite (FeAsO
4
·2H
2
O). The extent of tailings dispersal can be mapped through hyperspectral remote sensing methods, as these major mineral components provide an identifiable spectral signature through visible, near infrared and short-wave infrared regions. This paper examines the mineralogy of soils, tills and tailings in the Upper and Lower Seal Harbour gold districts of Nova Scotia. Ground-truthing of space-borne hyperspectral data demonstrates the potential for remote mapping of the spatial extent of these historical mine wastes.
U–Pb zircon ages and (isotope) geochemical signatures of the Kamanjab Inlier (NW Namibia): constraints on Palaeoproterozoic crustal evolution along the southern Congo craton
Authors: Kleinhanns I. C. , Fullgraf T. , Wilsky F. , Nolte N. , Fliegel D. , Klemd R. , Hansen B. T.
Published: August 2013

The formation of the Kamanjab Inlier (KI) in NW Namibia is poorly known and constrained to Palaeoproterozoic times. With the Epupa complex (EC) and Grootfontein Inlier (GI), the KI marks the southwestern Congo craton margin. Our new geochemical data for granitoids and orthogneisses indicate formation along an active continental margin. Single zircon ages frame granitoid emplacement to 1.86–1.83 Ga, roughly 75 myr older than ages from the northern EC and approximately 100 myr younger than from the GI. The southern EC is the only known Archaean Namibian basement with εNd
1.85 Ga
of −10.2 to −6.3, in contrast to northern EC (−1.8 to 4.4) and KI (−6.2 to 2.6). Thus, earlier speculation that the southern EC is an exotic terrane, among the Namibian basement complexes, is supported by our data. In contrast, the KI is geochemically comparable to the northern EC and GI. The
c.
2.0 Ga Lufubu metamorphic complex roughly 1000 km further east shows similar geochemistry, and a common evolution in the Kamanjab–Bangeweulu magmatic arc has already been proposed. Therefore, our new data point to a major Palaeoproterozoic crustal growth event at the southwestern margin of the Congo Craton starting in the present east and gradually moving towards the present NW.
Big data is 'buzzword du jour;' CS academics 'have the best job'
Authors: Stonebraker Michael , Robertson Judy
Published: August 2013

The
Communications
Web site, http://cacm.acm.org, features more than a dozen bloggers in the BLOG@CACM community. In each issue of
Communications
, we'll publish selected posts or excerpts.

twitter

Follow us on Twitter at http://twitter.com/blogCACM

http://cacm.acm.org/blogs/blog-cacm

Michael Stonebraker analyzes the different varieties of Big Data, while Judy Robertson considers the rewards of teaching computer science.
An overview of non-uniform memory access
Authors: Lameter Christoph
Published: August 2013

NUMA becomes more common because memory controllers get close to execution units on microprocessors.
Q&A: Big challenge
Authors: Hoffmann Leah
Published: August 2013

Jeff Dean and Sanjay Ghemawat talk about scalability.
State-dependent and reflex drives to the upper airway: basic physiology with clinical implications
Authors: Horner Richard L. , Hughes Stuart W. , Malhotra Atul
Published: August 2013

The root cause of the most common and serious of the sleep disorders is impairment of breathing, and a number of factors predispose a particular individual to hypoventilation during sleep. In turn, obstructive hypopneas and apneas are the most common of the sleep-related respiratory problems and are caused by dysfunction of the upper airway as a conduit for airflow. The overarching principle that underpins the full spectrum of clinical sleep-related breathing disorders is that the sleeping brain modifies respiratory muscle activity and control mechanisms and diminishes the ability to respond to respiratory distress. Depression of upper airway muscle activity and reflex responses, and suppression of arousal (i.e., “waking-up”) responses to respiratory disturbance, can also occur with commonly used sedating agents (e.g., hypnotics and anesthetics). Growing evidence indicates that the sometimes critical problems of sleep and sedation-induced depression of breathing and arousal responses may be working through common brain pathways acting on common cellular mechanisms. To identify these state-dependent pathways and reflex mechanisms, as they affect the upper airway, is the focus of this paper. Major emphasis is on the synthesis of established and recent findings. In particular, we specifically focus on 1) the recently defined mechanism of genioglossus muscle inhibition in rapid-eye-movement sleep; 2) convergence of diverse neurotransmitters and signaling pathways onto one root mechanism that may explain pharyngeal motor suppression in sleep and drug-induced brain sedation; 3) the lateral reticular formation as a key hub of respiratory and reflex drives to the upper airway.
Physiology in Medicine: Peripheral arterial disease
Authors: Muller Matthew D. , Reed Amy B. , Leuenberger Urs A. , Sinoway Lawrence I.
Published: August 2013

Peripheral arterial disease (PAD) is an atherosclerotic condition that can provoke symptoms of leg pain (“intermittent claudication”) during exercise. Because PAD is often observed with comorbid conditions such hypertension, dyslipidemia, diabetes, cigarette smoking, and/or physical inactivity, the pathophysiology of PAD is certainly complex and involves multiple organ systems. Patients with PAD are at high risk for myocardial infarction, stroke, and all-cause mortality. For this reason, a better physiological understanding of the pathogenesis and treatment options for PAD patients is necessary and forms the basis of this Physiology in Medicine review.
Does the length dependency of airway smooth muscle force contribute to airway hyperresponsiveness?
Authors: Lee-Gosselin Audrey , Pascoe Chris D. , Couture Christian , Paré Peter D. , Bossé Ynuk
Published: August 2013

Airway wall remodeling and lung hyperinflation are two typical features of asthma that may alter the contractility of airway smooth muscle (ASM) by affecting its operating length. The aims of this study were as follows: 1) to describe in detail the “length dependency of ASM force” in response to different spasmogens; and 2) to predict, based on morphological data and a computational model, the consequence of this length dependency of ASM force on airway responsiveness in asthmatic subjects who have both remodeled airway walls and hyperinflated lungs. Ovine tracheal ASM strips and human bronchial rings were isolated and stimulated to contract in response to increasing concentrations of spasmogens at three different lengths. Ovine tracheal strips were more sensitive and generated greater force at longer lengths in response to acetylcholine (ACh) and K+. Equipotent concentrations of ACh were approximately a log less for ASM stretched by 30% and approximately a log more for ASM shortened by 30%. Similar results were observed in human bronchi in response to methacholine. Morphometric and computational analyses predicted that the ASM of asthmatic subjects may be elongated by 6.6–10.4% (depending on airway generation) due to remodeling and/or hyperinflation, which could increase ACh-induced force by 1.8–117.8% (depending on ASM length and ACh concentration) and enhance the increased resistance to airflow by 0.4–4,432.8%. In conclusion, elongation of ASM imposed by airway wall remodeling and/or hyperinflation may allow ASM to operate at a longer length and to consequently generate more force and respond to lower concentration of spasmogens. This phenomenon could contribute to airway hyperresponsiveness.
Linear Atrophoderma of Moulin: Report of 4 Cases and 20th Anniversary Case Review
Authors: Villani Axel Patrice , Amini-Adlé Mona , Wagschal Daniel , Balme Brigitte , Thomas Luc
Published: August 2013

Background: Linear atrophoderma of Moulin (LAM) is a rare clinical entity which was first described by Moulin et al. in 1992. The diagnosis is clinical, characterized by acquired unilateral hyperpigmented, depressed band-like skin lesions following Blaschko's lines. The disease typically affects children or adolescents and has a good prognosis without evidence of long-term progression. To date, the pathophysiology is unclear. Different authors hypothesize that the disease is secondary to a mosaic manifestation as a result of a post-zygotic mutational event. Observations: Four patients (2 men, 2 women) had a history of unilateral band-like skin lesions located on the lower legs (50%) or the trunk (50%). Physical examination showed atrophic and hyperpigmented skin lesions along Blaschko's lines, which appeared during childhood in 3 cases and at the age of 20 in the last case. Lesions had progressed rapidly but seemed to have stabilized so far except for 1 case who presented spontaneous improvement. Histopathological examination revealed a normal epidermis with a hyperpigmented basal layer and a perivascular lymphocytic infiltrate in the dermis. Conclusion: LAM is a rare disease with 32 reported cases and remains an exclusion diagnosis. Since the problem is mainly esthetic, treatments should not be too aggressive.
Potential Use of Optical Coherence Tomography and High-Frequency Ultrasound for the Assessment of Nail Disease in Psoriasis and Psoriatic Arthritis
Authors: Aydin Sibel Z. , Castillo-Gallego Concepción , Ash Zoe R. , Abignano Giuseppina , Marzo-Ortega Helena , Wittmann Miriam , Del Galdo Francesco , McGonagle Dennis
Published: August 2013

Background: Psoriatic nail disease is increasingly recognised to be of major clinical and research relevance. Clinical assessment remains the current gold standard for its evaluation. Objective: We compared optical coherence tomography (OCT) and ultrasound (US) for nail disease assessment in psoriatic disease. Methods: 18 patients with at least one involved nail and 12 healthy controls were scanned using OCT; psoriatic patients also had an US scan (using a linear probe at 9-14 MHz). Nail and contour abnormalities were documented. Clinical onychopathy was scored independently using the modified Nail Psoriasis Severity Index. Results: Among 180 nails, 67.8% had clinical findings whereas 33.9% were abnormal by US and 44.4% had abnormalities on OCT. A positive OCT had a sensitivity and specificity of 44.4 and 95.8%, respectively, with a positive likelihood ratio of 10.7 for nail disease. OCT demonstrated 76.3% absolute agreement compared with clinical assessment and 65% with US. OCT detected subtle abnormalities in 12 clinically normal nails and in 41 nails with normal US findings. Conclusion: These findings show that OCT has a potential for the systematic characterisation of psoriatic nail changes and could be useful in diagnosis and more objective assessment of treatment response.
Inverted Follicular Keratosis: Dermoscopic and Reflectance Confocal Microscopic Features
Authors: Armengot-Carbo M. , Abrego A. , Gonzalez T. , Alarcon I. , Alos L. , Carrera C. , Malvehy J. , Puig S.
Published: August 2013

Inverted follicular keratosis (IFK) is a rare benign tumor which usually appears as a firm papule on the face. The diagnosis is generally made by histopathology because the clinical appearance is difficult to differentiate from other lesions. Dermoscopic features of IFK have not been established to date. Herein we describe the dermoscopic findings of 4 cases of IFK. Radial peripheral hairpin vessels surrounded by a whitish halo arranged around a central white-yellowish amorphous area were observed in 3 cases, and glomerular vessels were present in the central area of one of them. The fourth case also presented a central white amorphous area but showed arborizing vessels. Reflectance confocal microscopy (available in 1 case) revealed a broadened honeycomb pattern, epidermal projections and hairpin and glomerular vessels. To our knowledge this is the first case series describing the dermoscopic features of inverted follicular keratosis and the first confocal microscopy description of this entity.
The RENAL Nephrometry Score and the PADUA Classification for the Prediction of Perioperative Outcomes in Patients Receiving Nephron-Sparing Surgery: Feasible Tools to Predict Intraoperative Conversion to Nephrectomy
Authors: Kobayashi Kazuhiro , Saito Toshihiro , Kitamura Yasuo , Nobushita Tomohiro , Kawasaki Takashi , Hara Noboru , Takahashi Kota
Published: August 2013

Objective: To evaluate the potential of the RENAL nephrometry score and the PADUA classification in the prediction of perioperative outcomes represented by intraoperative conversion to nephrectomy in patients with renal tumors for which nephron-sparing surgery (NSS) was attempted. Methods: Recent 100 open NSSs attempted for cT1 renal tumors at a single institution were studied retrospectively. Results: With the RENAL, the operation time and ischemia time were longer in the high complexity group (p = 0.01 and p = 0.03, respectively), and blood loss was seemingly greater in this group (394 vs. 220 and 167 ml, p = 0.09). Conversion to nephrectomy was more frequent in the high complexity (4 procedures, 33.3%) than in the low (0%) and moderate (1 procedures, 1.5%) groups (p < 0.01). Regarding the PADUA, the operation time, blood loss and ischemia time increased according to the complexity (p = 0.04, p = 0.02, and p = 0.02, respectively). Conversion to nephrectomy was more frequent in the high complexity (4 procedures, 22.2%) than in the low (0%) and moderate (1 procedure, 1.8%) groups (p < 0.01). In patients with achieved NSS, postoperative estimated glomerular filtration rate was more impaired in the high complexity group in the PADUA (p = 0.02), although not significant in the RENAL (p = 0.11). Conclusions: Both the RENAL and PADUA are useful in the prediction of conversion to nephrectomy in addition to NSS-associated perioperative outcomes.
Mortality and Morbidity after Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Carcinomatosis
Authors: Saxena Akshat , Morris David L.
Published: August 2013

Background: Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is associated with improved survival in selected patients with peritoneal carcinomatosis. This treatment, however, has been associated with a relatively high rate of perioperative mortality and morbidity. Method: A review of clinical studies before March 2013 was performed to examine the impact of CRS-HIPEC on early mortality and morbidity. Factors associated with a poor perioperative outcome were identified. The importance of the learning curve was examined. Clinical outcomes were synthesized through a narrative review. Results: In-hospital mortality in high-level centres (>100 patients) ranged from 0.9 to 5.8%. The morbidity rate ranged from 36 to 84%; the overall rate of major or grade III/IV morbidity ranged from 0 to 52%. Factors that were consistently associated with a poor perioperative outcome included extensive peritoneal disease and markers of increased operative effort such as operation length, blood loss, and number of peritonectomy procedures performed. Several studies showed that morbidity decreases as operating volume increases. Moving forward, there is a need to evaluate strategies to reduce perioperative mortality whilst not compromising oncological outcomes. Conclusion: CRS-HIPEC is associated with an acceptable rate of perioperative mortality and morbidity. Morbidity decreases with increased experience and is strongly associated with the extent of disease.
Activation of Extracellular Transglutaminase 2 by Mechanical Force in the Arterial Wall
Authors: Huelsz-Prince Guizela , Belkin Alexey M. , vanBavel Ed , Bakker Erik N.T.P.
Published: August 2013

Inward remodeling of small arteries occurs after prolonged vasoconstriction, low blood flow, and in several models of hypertension. The cross-linking enzyme, transglutaminases 2 (TG2), is able to induce inward remodeling and stiffening of arteries. The activity of TG2 is dependent on its conformation, which can be open or closed, and on its redox state. Several factors have been shown to be involved in modulating TG2 activity, including Ca2+ and GTP/GDP concentrations, as well as the redox state of the environment. This review introduces the hypothesis that mechanical force could be involved in regulating the activity of TG2 during inward remodeling by promoting its open and reduced active state. Several aspects of TG2, such as its structure and localization, are assessed in order to provide arguments that support the hypothesis. We conclude that a direct activation of TG2 by mechanical force exerted by smooth muscle cells may explain the link between smooth muscle activation and inward remodeling, as observed in several physiological and pathological conditions.
Indikationen und Resultate der Peritonektomie bei nichtkolorektaler Peritonealkarzinose
Authors: Rau Beate , Kilian Maik , Köhler Christhardt , Mangler Mandy , Winterfeld Moritz von , Rudl Marc , Raue Wieland
Published: August 2013

Hintergund: In dieser Übersichtsarbeit soil der Stellenwert der zytoreduktiven chirurgischen Therapie (CRS) in Kombination mit einer hyperthermen intraperitonealen Chemotherapie (HIPEC) der Peritonealkarzinose bei nichtkolorektalen Karzinomen dargestellt werden. Hierzu zählen als primäre Tumoren des Peritoneums das Mesotheliom und das Pseudomyxoma peritonei. Zu den wichtigsten sekundären malignen Erkrankungen des Peritoneums zählen die Peritonealkarzinose des Magenkarzinoms, des Pankreaskarzinoms und des Ovarialkarzinoms sowie die Sarkomatose. Methode: Pathologische Besonderheiten der einzelnen Entitäten und Therapieempfehlungen anhand der aktuellen Literatur werden im Einzelnen beschrieben. Ergebnisse: Prinzipiell ist die chirurgische Behandlung der Peritonealkarzinose im Sinne einer CRS in Kombination mit einer HIPEC ein sinnvolles Therapiekonzept - vorausgesetzt, dass eine nahezu komplette Tumorentfernung erzielt werden kann. Schlussfolgerung: Aufgrund der erhöhten Morbidität sollte ein chirurgischer Eingriff gut indiziert sein. Bei ausgewählten Patienten ist eine CRS der Peritonealkarzinose in Kombination mit einer HIPEC ein sinnvolles und lebensverlängerndes Verfahren.
Nutrient Intake in Infancy and Body Mass Index at Six Years in Two Population-Based Cohorts Recruited before and after Revision of Infant Dietary Recommendations
Authors: Thorisdottir Birna , Gunnarsdottir Ingibjorg , Thorisdottir Asa Vala , Palsson Gestur Ingvi , Halldorsson Thorhallur Ingi , Thorsdottir Inga
Published: August 2013

Background/Aims: The dietary intake in infancy can affect growth and the risk of childhood overweight. The aim of this study was to compare the nutrient intake in infancy and the body mass index (BMI) at age 6 years in two population-based cohorts recruited before and after the revision of infant dietary recommendations. Methods: Two randomly selected population-based cohorts were recruited at birth and studied until 12 months of age and again at 6 years of age. Subjects were 90 and 170 children born in 1995-1996 and 2005, respectively. The dietary intake at 9 and 12 months was assessed via weighed food records. The height and weight from birth to age 12 months and at age 6 years were measured. Results: The contribution of protein to the total energy intake (E%) was significantly lower in the 2005 cohort than in the 1995-1996 cohort [11.9 vs. 14.4 E% (p < 0.001) at 9 months and 14.6 vs. 15.6 E% (p = 0.016) at 12 months]. Relatively fewer children were classified as being overweight (including obese) in the 2005 cohort, i.e. 12 versus 21% (p = 0.045). Conclusion: The emphasis placed on the revised infant dietary recommendations resulted in a lower protein intake in the latter half of the first year. Changes in the infants' diets on the population level might have contributed to the lower overweight prevalence seen in the 2005 cohort.
Antitumor Effect of Everolimus in a Patient with Type 3 Gastric Neuroendocrine Tumor
Authors: Bariani Giovanni M. , Carvalheira José B. , Riechelmann Rachel P.
Published: August 2013

Background: Gastric neuroendocrine tumors (NET) are rare and are classified into 3 types: type 1 and 2 (characterized by hypergastrinemia), and type 3 (characterized by normal gastrin). Surgery is the standard procedure, and systemic treatment is reserved for unresectable disease. Currently, targeted therapies are being evaluated in NET. The activity of everolimus, an mTOR inhibitor, has been shown in pancreatic NET but not reported in type 3 gastric carcinoid tumors. Case Report: Here we report a case of a patient who, after multiple lines of systemic therapy, had a prolonged disease control of nearly 1 year, significant clinical benefit, and minor tumor shrinkage with oral everolimus 10 mg continuously. Conclusion: There is no effective treatment for type 3 gastric carcinoid tumors. The frequency of mTOR expression in these tumors is not known, but the case reported here suggests that inhibition of this pathway may play an important role.
Characterization of Cardiovascular Involvement in Pseudoxanthoma Elasticum Families
Authors: Campens Laurence , Vanakker Olivier M. , Trachet Bram , Segers Patrick , Leroy Bart P. , De Zaeytijd Julie , Voet Dirk , De Paepe Anne , De Backer Tine , De Backer Julie
Published: August 2013

Objective—

Pseudoxanthoma elasticum (PXE) is an autosomal recessive connective tissue disorder with involvement of the skin, the retina, and the cardiovascular system. Cardiovascular involvement is mainly characterized by mineralization and fragmentation of elastic fibers of blood vessels and premature atherosclerosis. We conducted an ultrasound study to investigate the cardiovascular phenotype and to propose recommendations for the management of patients with PXE and heterozygous
ABCC6
mutation carriers.

Approach and Results—

Thirty-two patients, 23 carriers, and 28 healthy volunteers underwent cardiac and vascular ultrasound studies. Cardiac imaging revealed left ventricular diastolic dysfunction in patients with PXE with a significantly prolonged deceleration time and lower septal early diastolic velocities of the mitral annulus compared with controls. Carriers also demonstrated significantly prolonged deceleration time. Carotid-to-femoral pulse wave velocity was significantly increased in patients with PXE when compared with carriers and controls. Vascular imaging revealed a high prevalence of peripheral artery disease in both patients and carriers and a significantly higher carotid intima-media thickness compared with controls.

Conclusions—

The results of this study clearly demonstrate impaired left ventricular diastolic function, impairment of the elastic properties of the aorta, and a high prevalence of peripheral artery disease in patients with PXE. Carriers also seem to exhibit a cardiovascular phenotype with mainly mild diastolic dysfunction and accelerated atherosclerosis. Increased awareness for cardiovascular events in both patients and heterozygous carriers is warranted.
Sodium-Dependent Phosphate Cotransporters and Phosphate-Induced Calcification of Vascular Smooth Muscle Cells
Authors: Crouthamel Matthew H. , Lau Wei Ling , Leaf Elizabeth M. , Chavkin Nicholas W. , Wallingford Mary C. , Peterson Danielle F. , Li Xianwu , Liu Yonggang , Chin Michael T. , Levi Moshe , Giachelli Cecilia M.
Published: August 2013

Objective—

Elevated serum phosphate has emerged as a major risk factor for vascular calcification. The sodium-dependent phosphate cotransporter, PiT-1, was previously shown to be required for phosphate-induced osteogenic differentiation and calcification of cultured human vascular smooth muscle cells (VSMCs), but its importance in vascular calcification in vivo and the potential role of its homologue, PiT-2, have not been determined. We investigated the in vivo requirement for PiT-1 in vascular calcification using a mouse model of chronic kidney disease and the potential compensatory role of PiT-2 using in vitro knockdown and overexpression strategies.

Approach and Results—

Mice with targeted deletion of PiT-1 in VSMCs were generated (PiT-1

Δ
sm

). PiT-1 mRNA levels were undetectable, whereas PiT-2 mRNA levels were increased 2-fold in the vascular aortic media of PiT-1

Δsm

compared with PiT-1

flox/flox

control. When arterial medial calcification was induced in PiT-1

Δsm

and PiT-1

flox/flox

by chronic kidney disease followed by dietary phosphate loading, the degree of aortic calcification was not different between genotypes, suggesting compensation by PiT-2. Consistent with this possibility, VSMCs isolated from PiT-1

Δsm

mice had no PiT-1 mRNA expression, increased PiT-2 mRNA levels, and no difference in sodium-dependent phosphate uptake or phosphate-induced matrix calcification compared with PiT-1

flox/flox

VSMCs. Knockdown of PiT-2 decreased phosphate uptake and phosphate-induced calcification of PiT-1

Δsm

VSMCs. Furthermore, overexpression of PiT-2 restored these parameters in human PiT-1–deficient VSMCs.

Conclusions—

PiT-2 can mediate phosphate uptake and calcification of VSMCs in the absence of PiT-1. Mechanistically, PiT-1 and PiT-2 seem to serve redundant roles in phosphate-induced calcification of VSMCs.
Function and Distribution of Apolipoprotein A1 in the Artery Wall Are Markedly Distinct From Those in Plasma
Authors: DiDonato Joseph A. , Huang Ying , Aulak Kulwant S. , Even-Or Orli , Gerstenecker Gary , Gogonea Valentin , Wu Yuping , Fox Paul L. , Tang W.H. Wilson , Plow Edward F. , Smith Jonathan D. , Fisher Edward A. , Hazen Stanley L.
Published: August 2013

Background—

Prior studies show that apolipoprotein A1 (apoA1) recovered from human atherosclerotic lesions is highly oxidized. Ex vivo oxidation of apoA1 or high-density lipoprotein (HDL) cross-links apoA1 and impairs lipid binding, cholesterol efflux, and lecithin-cholesterol acyltransferase activities of the lipoprotein. Remarkably, no studies to date directly quantify either the function or HDL particle distribution of apoA1 recovered from the human artery wall.

Methods and Results—

A monoclonal antibody (10G1.5) was developed that equally recognizes lipid-free and HDL-associated apoA1 in both native and oxidized forms. Examination of homogenates of atherosclerotic plaque–laden aorta showed >100-fold enrichment of apoA1 compared with normal aorta (
P
<0.001). Surprisingly, buoyant density fractionation revealed that only a minority (<3% of total) of apoA1 recovered from either lesions or normal aorta resides within an HDL-like particle (1.063≤d≤1.21). In contrast, the majority (>90%) of apoA1 within aortic tissue (normal and lesions) was recovered within the lipoprotein-depleted fraction (d>1.21). Moreover, both lesion and normal artery wall apoA1 are highly cross-linked (50% to 70% of total), and functional characterization of apoA1 quantitatively recovered from aorta with the use of monoclonal antibody 10G1.5 showed ≈80% lower cholesterol efflux activity and ≈90% lower lecithin-cholesterol acyltransferase activity relative to circulating apoA1.

Conclusions—

The function and distribution of apoA1 in human aorta are quite distinct from those found in plasma. The lipoprotein is markedly enriched within atherosclerotic plaque, predominantly lipid-poor, not associated with HDL, extensively oxidatively cross-linked, and functionally impaired.