bluuebunny/crossref_metadata_2025 huggingface dataset:
Software Requirements Engineering Methodology
Authors: Alford Mack
Published: February 2015

SREM is an acronym for software requirements engineering methodology and is also a shorthand name for the technology for specifying and designing systems resulting from a series of research efforts that started with SREM. The SREM approach is characterized by the following features:

The use of an executable graphic notation to describe system and software behavior (e.g., RNETs and FNETs).

The use of a user‐extensible element‐relation‐attribute (ERA) language to describe the various system requirements and design characteristics, including traceability and decisions.

The use of automated tools to accept the above information, perform static and dynamic consistency–completeness checking, validate the specifications by executing them, and generate required specification documents.

The definition of a method for using the language and tools described as a sequence of steps, each of which adds a specific subset of the ERA and executable information and then uses the tools to check a subset of the consistency–completeness conditions.

An intent to provide a smooth transition from system requirements and design through software requirements and design and testing.

The SREM technology was developed over a 25‐year period, with alternate periods of research, development, and prototype application to validate the concepts. Its research results were at the edge of the state of the art for more than 15 yr. It is unique in its breadth of coverage, and focuses on providing a smooth transition between the different phases of system design.
Test Automation
Authors: Fewster Mark
Published: February 2015

Test automation
generally means automated execution of test cases including automated verification of the test results. However, this is a rather narrow view. This term is more usefully taken to refer to any type of tool support of a testing activity. Tools that support testing activities are almost all computer programs that are generally referred to as test tools. The different types of test tools are outlined.

Almost any testing activity can be tool‐supported in some way, but this is not to say that the activity can be performed entirely by the tool or automatically. In most cases tool‐supported activities require some manual intervention. The main role of a testing tool is usually to support, not replace, a person's efforts.

This article discusses issues associated with most types of tool support for testing, but much of the later sections deal with issues that are most applicable to tool support for test execution automation, the most popular type of test automation.
Software Tools
Authors: Grundy John , Hosking John
Published: February 2015

The demand for computer software applications steadily increases as numbers of users, problem domains, and application areas grow the need for good tools to support the myriad of activities that occur during software development is thus greater than ever. This article provides an overview of current practice in software tool features and usage. It also surveys research work in the area of software tools, indicating recent and likely near‐future trends in tools development. A section outlines recent directions in software process evolution, product domains and people management. As different software tools are used throughout the software process for different activities, changes in processes, products and project organization have introduced a variety of new demands on tools and tool developers. A categorization of software tools aligned with software process activities is then presented, including examples of tools in the various categories, along with a brief explanation of common terms used to describe software tools. We describe key elements of the structure of software tools, ranging from repository and data management to multiple‐view presentation and editing. We then focus on the key issue of tool integration, outlining current approaches to integration, current research trends, and likely near‐future integration support in software tools. We describe various strategies for supporting collaborative work with different software tools, facilitating improved teamwork with tools. We review and discuss current research, practice, and likely trends in automated support in software tools, alleviating developers of tedious tasks and helping manage ever‐growing software complexity. We conclude with a summary of some important factors in successful tool development and deployment.
Space Systems
Authors: Keller T. W. , Rone Kyle Y.
Published: February 2015

A space system is created to operate in the environment of outerspace as opposed to a system which operates near the earth in the presence of the atmosphere. Compared to most places on earth, the space environment is extremely hostile to humans and to the systems humans build and use. Normal means of system support, such as ambient air‐cooling, are no longer viable and so more difficult, costly methods must be used. To ensure that a system remains in space for a reasonable time, it must either attain orbit or escape velocity. Very expensive launch systems must be developed to achieve the speeds necessary to attain orbit or escape velocity. Thus, a fundamental problem is encountered: the cost of launching each pound of a system into space is very high; therefore, components of space systems have always remained small and lightweight. Power on‐board is either supplied by heavy solar panels or electricity generators. The size and weight of these elements can be reduced by minimizing the power consumption of the space systems they support. Because space systems must operate in a remote environment where modifications are not easily made, space systems must be extremely reliable, to reduce the need for repair, and modular to make unavoidable repairs manageable. Safe operation is also required of these systems to protect humans and expensive hardware. These requirements and constraints combine to offer a daunting challenge to the space systems developer who must skillfully balance them against the needs of the user.

Other articles have thoroughly addressed the general problems facing software engineers and the practices established to solve those problems. The purpose of this article is to survey these unique problems applying to data‐processing systems designed to operate in the space environment. This discussion will also include current engineering solutions to those problems and will provide actual examples. An introductory overview of the history of space data‐processing systems will be followed by a discussion of space‐system design considerations including: hardware constraints, compilers and languages, communications and interfaces, software complexity and functionality, environmental factors, and remote maintenance. A discussion of quality assurance processes to “space qualify” data processing systems will then be followed by sections addressing unique aspects of: testing and simulation, autonomy, and problem diagnosis. The article will be completed with a perspective on future challenges facing the engineers of software systems for space applications.
Specification‐Driven Tools and Techniques
Authors: Cleaveland J. Craig
Published: February 2015

Specification‐driven tools (SDTs) translate specifications to products such as programs, documentation, tables, and test scripts. SDTs represent a large class of techniques that have independently evolved under a variety of names including application generators, program generators, 4GLs (fourth generation languages), automatic programming, lower CASE tools, and application‐oriented languages. SDTs increase productivity and quality and are most successful in programming areas that are well understood and repetitive such as databases, user interfaces, and translators. SDTs are an effective way to reuse software code and design. Unlike code reuse libraries, SDTs generate customized software that is not modified by the user. A typical SDT for building parsers from grammar descriptions is shown.

The productivity and quality of SDTs is achieved by two major factors: narrow domains and understandable specification languages. A “domain” is the area of applicability. General‐purpose programming languages are useful for very large problem domains.

The second major factor for increased productivity and quality of SDTs is the specification language. It must be significantly easier to understand than the generated code. It should be much shorter, should not contain unnecessary implementation details, and it should be understandable to nonprogrammers—ideally, the customer. However, specification languages are not necessarily simple. Example problem domains and tools for building SDTs are described.
Subdomain Testing
Authors: Hamlet Richard
Published: February 2015

In subdomain testing, the input domain of a program is conceptually divided into subdomains, each subdomain intuitively composed of input points that are “the same” in some sense. Systematic testing of the program is then organized around these subdomains. In the simplest scheme, a test set is required to include at least one point from each subdomain. The subdomains may be disjoint and exhaust the input space, in which case they constitute the equivalence classes of a partition in the mathematical sense, hence the name “partition testing.” However, in general, subdomains may overlap and some input points may appear in no subdomain.

In so‐called statistical testing, instead of requiring a test set to have at least one subdomain member, uniform random sampling within each subdomain is required.

Any systematic test method can be thought of as a subdomain method.
Software Technology Transfer, Diffusion, Adoption, and Implementation
Authors: Fowler Priscilla , Scacchi Walt
Published: February 2015

Software technologytransferis the movement of a technology from one owner to another, typically by commercial transaction. This type of movement of technology ignores both the technology creation process and the process of its use after transfer. Thus, for a complete understanding of what happens when technology moves from one context to another, we also need to consider “diffusion of innovations” and “adoption” as well as “implementation” or “deployment”.

Software technology transfer, diffusion, adoption, and implementation (STTDAI) cannot be defined without understanding first the maturity of the particular technology, and second, the point of view taken within these constituent transfer processes. Technologies mature as they move from research prototypes into products and make the transition into use within organizations and communities and by individuals. Thus, STTDAI plays a major role in the maturation process.

There are three major life cycles of technology maturation: research and development (R&D), new‐product development, and technology adoption and implementation. Together, these three life cycles cover technology development, popularization within an intended market, and deployment, from the inception of a technology until its retirement. How software technology transfer, diffusion, adoption, and implementation work is best understood in the context of this composite life cycle.

A simplified model of the software technology maturation life cycle is given. The model is described in terms of each subordinate life cycle, and the interactions among them.
Test Generators
Authors: Poston Robert M.
Published: February 2015

Many people think of automated software testing as executing or running test cases. Actually software testing involves three technical activities: creating, executing, and evaluating test cases. Creating test cases is the most essential activity in software testing, because all other testing activities depend on having test cases to run or evaluate.

Tools called test case generators can create test cases automatically. However, to do so, a test case generator needs a precise definition of a test case.

A test case is a set of information that contains

A unique identifier: a name that distinguishes this test case from all others

A list of actions (functions, processes, services, etc.) that this test case will exercise

A list of names and values for inputs to actions that this test case will exercise

A list of outputs that will result when this test case exercises actions

Item 3 is particularly important. A test case is hard to use unless it includes both names and values for all inputs (also called causes or stimuli). For example, if a test case provides the name of an input such as “valid telephone number” but does not include a value such as 911, the test case is incomplete. The tester must finish the test case by adding the telephone number—a simple enough job for one input. But suppose the input to the software under test is a file containing thousands of telephone numbers. Then the tester is confronted with creating and managing huge numbers of input values.

Test case generators can produce test cases, as just defined, at rates exceeding 300 test cases per minute. Considering that the average tester in the U.S. creates 30 to 300 test cases by hand in one month automated test case generators offer compelling productivity gains.
Test Management and Organization
Authors: Black Rex
Published: February 2015

Organizations choose (whether consciously or otherwise) to invest in quality in general, and testing specifically, because they expect the time, money, and care spent prior to releasing a system to pay dividends after the release. These dividends may come in the reverse form they are an improved reputation for quality with internal and external customers; lower post‐release maintenance costs, smoother, better‐understood, and more‐timely release cycles; increased confidence that mission‐critical operations will be performed properly; protection from lawsuits resulting from faulty systems are; in some cases, whole missions and even lives rescued from total loss

Testing is, therefore, a risk management exercise that helps the organization reduce the likelihood of bearing unanticipated future costs. This resembles insurance, except that the actuarial certainty that comes with pooling risk across large numbers of insured seldom applies when a handful of projects present make‐or‐break risks. With such high stakes involved, testing is an endeavor that deserves careful planning, crisp execution, a talented team, and significant resources.

A test effort is a subproject of a development or maintenance project, providing risk management services to various other teams, including project management, senior management, and executive management. As such, it must fit within the project and the organization logically, and in a way that promotes rather than retards the risk management functions it is set up to perform. The specific tests to be performed arise from the customers’ or business users’ reasonable expectations of quality. Once this is understood, the test manager can implement a test process, a test system, and a test team to perform these tests.
Ultrareliability
Authors: Musa John D.
Published: February 2015

Ultrareliability concerns functions for which failure entails exceptionally heavy costs, frequently including loss of life, resulting in a requirement for very low frequency of such failures. There is no clear dividing line between high reliability and ultrareliability, but the term ultrareliability generally is used to refer to failure intensities of less than

failures/hour (one failure per million hours). In order to give a physical feel to failure intensities, some common failure intensities and risks are noted. Note that the need for ultrareliability really does refer to
functions
rather than systems. An important distinction is to be made in regard to reliability of hardware and software components.

The expense, development time, and difficulties involved in building ultrareliable functions are such that we need to take a very realistic attitude in setting failure intensity objectives. It is also important that we separate functions by criticality and apply ultrahigh reliability objectives only to those functions that require them many have concluded that we therefore cannot certify ultrareliable software through testing. This is overly pessimistic.
Unified Process (
UP
)
Authors: Alhir Sinan Si
Published: February 2015

The Unified Process (UP) is a use‐case‐driven, architecture‐centric, iterative, and incremental software development process framework. The UP is broadly applicable to different types of software systems, including small‐scale and large‐scale projects having various degrees of managerial and technical complexity, across different application areas and organizational cultures. The UP leverages the Object Management Group's (OMG) Unified Modeling Language (UML), but may utilize any modeling language. The UML is an evolutionary general‐purpose, broadly applicable, tool‐supported, and industry‐standardized modeling language for specifying, visualizing, constructing, and documenting the artifacts of a system‐intensive process.
Vienna Development Method
Authors: Bartley Michael
Published: February 2015

VDM (Vienna Development Method) is a Formal Method that can be used for both the specification and development of software. It ismodel based, i.e., system behavior is defined by the construction of a mathematical model with an underlying state (data) and a collection of operations on that state. It is based on discrete mathematics, i.e., set theory and logic.

The original VDM Specification Language (VDM‐SL) grew out of an attempt to define formal denotational semantics for the programming PL/1 at the IBM Laboratory in Vienna. The language and method have developed much since then through both academic research and industrial application.
Work Breakdown Structure
Authors: Servello Mark , Evans Michael W.
Published: February 2015

The
work breakdown structure
(WBS) consists of the defined set of tasks that must be accomplished and milestones that must be met on a project. Each task represents the creation or modification of a part of the product and may include items such as

Hardware and/or software products to be produced

Services and support tasks that must be accomplished

Procurement of equipment, tools, and facilities

Training of project personnel

The WBS defines the basis for planning the project, allocating work, and tasking resources in a coordinated and focused manner. The WBS serves as the mechanism for program scheduling and fiscal planning and provides the means to coordinate and control the diverse activities of a program, and to report on progress and status during the project.

The WBS subdivides projects into manageable units of work to be performed through the definition of various levels of hierarchy described and illustrated. The top of the hierarchy is the scope of the project. Each succeeding level in the hierarchy provides greater detail of the groupings and individual tasks to be performed. An example of a typical breakdown of levels in the hierarchical structure for a WBS is provided.

There are typically three different forms of the WBS that can be applied to define and allocate work in a software project: An
end‐item
or
product
WBS structure. A
functional
WBS and an
organizational
WBS structure. Creating and using WBS are detailed in other article.
Requirement
Authors: Davis Alan Mark
Published: February 2015

A requirement is an externally observable characteristic of a desired system. When trying to determine whether a candidate requirement is really a requirement, two criteria from this definition must be met: (
1
) it must be externally observable, and (
2
) it must be desired.
Externally observable
implies that there exists some means by which an observer (whether this is a human, machine, animal, or another system) can sense that the system satisfies the requirement from a perspective external to the system itself.
Desired
implies that some stakeholder (q.v.) wants to have that characteristic present in the system.

In addition to being externally observable and desired, there are numerous other characteristics that a requirement should exhibit and these are discussed.
Life‐Cycle Compression
Authors: Davis Alan Mark
Published: February 2015

Life‐cycle compression is the process of reducing the elapsed time for development from whatever it normally is to a shorter time. The expression “Internet time” generally refers to compression of the development life cycle from its usual elapsed time to 50% or less.

Companies attempt to compress their development life cycles because of competitive pressure. With the introduction of increased competition in every industry that incorporates software, and the wide variety of software development techniques in use today, no matter how long it takes you to build software, some other company will find a way to deliver a similar product faster.
Along‐isopycnal variability of spice in the North Pacific
Authors: Klymak Jody M. , Crawford William , Alford Matthew H. , MacKinnon Jennifer A. , Pinkel Robert
Published: February 2015

Two hydrographic surveys in the Gulf of Alaska and the North Pacific subtropical gyre are presented. Both surveys are roughly perpendicular to lateral temperature gradients, and were collected in the summer when there was a shallow mixed layer and a seasonal thermocline. Isopycnal displacements and horizontal velocities are dominated by internal waves. Spice anomalies along isopycnals are examined to diagnose lateral stirring mechanisms. The spectra of spice anomaly gradients along near‐surface isopycnals roughly follow power laws of
(variance spectra power laws of
), and in most cases, the spectra become redder at depth. The near‐surface spectra are possibly consistent with the predictions of quasi‐geostrophic turbulence theory (when surface buoyancy effects are accounted for), but the spectra at depth are inconsistent with any quasi‐geostrophic theory. Probability distributions of spice gradients exhibit a large peak at low gradients and long tails for large gradients, symptomatic of fronts. Vertical coherence of the spice signal falls off with a decorrelation depth scale that has a maximum of about 80 m at 100 km wavelengths and depends on horizontal wavelength with a power law of approximately
. Lateral decorrelation length scales are 20–40 km, close to the baroclinic Rossby radius. Lateral stirring occurs over large scales, with average lateral displacements of about 200 km in the upper 75 m, decreasing to 100 km at greater depths. The depth variation of the statistics indicates that time history of tracer stirring on each isopycnal is important, or that there are unconsidered depth‐dependent stirring mechanisms.
Modeling the interaction between convection and nonthermal ion outflows
Authors: Varney R. H. , Wiltberger M. , Lotko W.
Published: February 2015

Initial demonstrations of an ionosphere/polar wind model including a phenomenological treatment of transverse heating by wave particle interactions (WPIs) are presented. Tests with fixed WPI parameters in a designated heating region on the dayside with time‐varying convection show that the parameters of the resulting nonthermal ion outflow are strongly coupled to the convection. The hemispheric outflow rate is positively correlated with the convection speed with a time delay related to the travel time to the upper boundary. Increases in convection increase the thermal plasma access to the heating region, both by increasing the upflow associated with frictional heating and by increasing the horizontal transport. The average parallel velocities and energies of the escaping nonthermal ions are anticorrelated with the convection speed due to the finite dwell time in the heating region. The computationally efficient model can be readily coupled into global geospace modeling frameworks in the future.
Terrestrial environmental changes around the Gulf of Aden over the last 210 kyr deduced from the sediment n‐alkane record: Implications for the dispersal of Homo sapiens
Authors: Isaji Yuta , Kawahata Hodaka , Ohkouchi Naohiko , Murayama Masafumi , Tamaki Kensaku
Published: February 2015

We analyzed long‐chain (C25–C36) n‐alkanes and pollen grains in sediments from the Gulf of Aden covering the last 212 kyr to reconstruct the surrounding terrestrial environment, a critical region for the dispersal of Homo sapiens. Substantial increases in the flux of n‐alkanes during 200–185, 120–95, and 70–50 ka were interpreted to indicate enhanced vegetation biomass in the Arabian Peninsula and the northern part of the Horn of Africa or increase in lithogenic material inputs. Periods of enhanced n‐alkane flux occurred during or immediately after pluvial episodes, indicating that the increased precipitation may have induced substantially enhanced vegetation biomass, creating favorable conditions for Homo sapiens. Additionally, vegetation may have increased due to moderate precipitation unrecorded by speleothems or in accordance with the lowering of sea level, indicating that the dispersal might have been possible even after the shift to an arid environment indicated by the speleothems.
White Light‐Emitting Diode From Sb‐Doped p‐ZnO Nanowire Arrays/n‐GaN Film
Authors: Ren Xiaoliang , Zhang Xianghui , Liu Nishuang , Wen Li , Ding Longwei , Ma Zongwei , Su Jun , Li Luying , Han Junbo , Gao Yihua
Published: February 2015

A whole interfacial transition of electrons from conduction bands of n‐type material to the acceptor levels of p‐type material makes the energy band engineering successful. It tunes intrinsic ZnO UV emission to UV‐free and warm white light‐emitting diode (W‐LED) emission with color coordinates around (0.418, 0.429) at the bias of 8–15.5 V. The W‐LED is fabricated based on antimony (Sb) doped p‐ZnO nanowire arrays/Si doped n‐GaN film heterojunction structure through one‐step chemical vapor deposition with quenching process. Element analysis shows that the doping concentration of Sb is ≈1.0%. The I–V test exhibits the formation of p‐type ZnO nanowires, and the temperature‐dependent photoluminescence measurement down to 4.65 K confirms the formation of deep levels and shallow acceptor levels after Sb‐doping. The intrinsic UV emission of ZnO at room temperature is cut off in electroluminescence emission at a bias of 4–15.5 V. The UV‐free and warm W‐LED have great potential application in green lights program, especially in eye‐protected lamp and display since television, computer, smart phone, and mobile digital equipment are widely and heavily used in modern human life, as more than 3000 h per year.
Evaporation of pinned droplets containing polymer – an examination of the important groups controlling final shape
Authors: Eales Adam D. , Routh Alexander F. , Dartnell Nick , Simon Goddard
Published: February 2015

Controlling the final shape resulting from evaporation of pinned droplets containing polymer, is important in the fabrication of P‐OLED displays by inkjet printing. Typically, a coffee ‐ ring shape arises, due to the pinning and associated outward capillary flow. For operational reasons, this is undesirable – a flat topography is required. The aim of this work is to understand the important groups governing the shape, to provide a practical guide to ink selection. The theory presented is based on a thin‐film lubrication model. The governing equations are solved numerically and continuously track the lateral progression of a liquid/gel front. A large capillary number or large ratio of initial to maximal polymer volume fraction can suppress the coffee‐ring. White light interferometry is used to confirm these findings experimentally. © 2015 The Authors AIChE Journal published by Wiley Periodicals, Inc. on behalf of American Institute of Chemical Engineers AIChE J, 61: 1759–1767, 2015
Anatomy of a rapid pressure swing adsorption process performance
Authors: Vemula Rama Rao , Kothare Mayuresh V. , Sircar Shivaji
Published: February 2015

A detailed numerical study of the individual and cumulative effects of various mass, heat, and momentum transfer resistances, which are generally present inside a practical adiabatic adsorber, on the overall separation performance of a rapid pressure swing adsorption (RPSA) process is performed for production of nearly pure helium gas from an equimolar binary (N2 +He) gas mixture using 5 A zeolite. Column bed size factor (BSF) and helium recovery (R) from the feed gas are used to characterize the separation performances. All practical impediments like column pressure drop, finite gas‐solid mass and heat transfer resistances, mass and heat axial dispersions in the gas phase, and heats of ad(de)sorption causing nonisothermal operation have detrimental impacts on the overall process performance, which are significantly accentuated when the total cycle time of a RPSA process is small and the product gas helium purity is high. These impediments also prohibit indefinite lowering of BSF (desired performance) by decreasing process cycle time alone. © 2015 American Institute of Chemical Engineers AIChE J, 61: 2008–2015, 2015
Modeling segregation of bidisperse granular materials using physical control parameters in the quasi‐2D bounded heap
Authors: Schlick Conor P. , Fan Yi , Isner Austin B. , Umbanhowar Paul B. , Ottino Julio M. , Lueptow Richard M.
Published: February 2015

Quantitatively predicting segregation of size‐disperse granular materials is of potential value in many industrial applications. We consider granular segregation of size‐bidisperse particles in quasi‐2D bounded heaps, a canonical granular flow, using an advection‐diffusion transport equation with an additional term to account for particle segregation. The equation is characterized by two dimensionless parameters that are functions of control parameters (flow rate, system size, and particle sizes) and kinematic parameters (flowing layer depth, diffusion coefficient, and percolation length scale). As the kinematic parameters are usually difficult to measure in practice, their dependence on the control parameters is determined directly from discrete element method simulations. Using these relationships, it is possible to determine which values of the control parameters result in a mixed or segregated heap. The approach used here is broadly applicable to a wide range of other flow geometries and particle systems. © 2015 American Institute of Chemical Engineers AIChE J, 61: 1524–1534, 2015
A family of distal arthrogryposis type 5 due to a novel PIEZO2 mutation
Authors: Okubo Mariko , Fujita Atsushi , Saito Yoshiaki , Komaki Hirofumi , Ishiyama Akihiko , Takeshita Eri , Kojima Emiko , Koichihara Reiko , Saito Takashi , Nakagawa Eiji , Sugai Kenji , Yamazaki Hiroko , Kusaka Kei , Tanaka Hiroshi , Miyake Noriko , Matsumoto Naomichi , Sasaki Masayuki
Published: February 2015

Distal arthrogryposis (DA) encompasses a heterogeneous group of hereditary disorders with multiple congenital contractures predominant in the distal extremities. A total of 10 subtypes are proposed based on the pattern of contractures and association with extraarticular symptoms. DA5 is defined as a subtype with ptosis/oculomotor limitation. However, affected individuals have a variety of non‐ocular features as well. We report on a two‐generation family, including four affected individuals who all had congenital contractures of the distal joints, ptosis, restricted ocular movements, distinct facial appearance with deep‐set eyes, and shortening of the 1st and 5th toes. The proband and her affected mother had restrictive lung disease, a recently recognized syndromic component of DA5, while younger patients did not. The proband had metacarpal and metatarsal synostosis, and the mother showed excavation of the optic disk. Whole‐exome sequencing revealed a novel heterozygous mutation c.4456G>C (p.A1486P) of PIEZO2. PIEZO2 encodes a mechanosensitive ion channel, malfunction of which provides pleiotropic effects on joints, ocular muscles, lung function, and bone development. © 2015 Wiley Periodicals, Inc.
A case with neonatal hyperinsulinemic hypoglycemia: It is a characteristic complication of sotos syndrome
Authors: Nakamura Yoshie , Takagi Masaki , Yoshihashi Hiroshi , Miura Masaru , Narumi Satoshi , Hasegawa Tomonobu , Miyake Yoshishige , Hasegawa Yukihiro
Published: February 2015

Sotos syndrome (SoS, OMIM #117550) is an overgrowth syndrome. Deletions or intragenic mutations of the NSD1 , which is located at chromosome 5q35, are responsible for more than 75% of SoS. Conventionally, neonatal hypoglycemia was reported briefly as one of the infrequent symptoms of SoS. However, Matsuo et al. published a report describing five patients with SoS who presented with transient hyperinsulinemic hypoglycemia (HIH) in the neonatal period. We report on an additional patient of SoS, who presented transient HIH in the neonatal period. All of this patient and previous patients have microdeletions at the 5q35 chromosome. Therefore, we examined the following three in considering the possibility that other factor than NSD1 caused HIH. 1) This patient had no mutation of four currently known HIH related genes, ABCC8, KCNJ11, GLUD1, and GCK. 2) He had no further deletion than commonly observed region encompassing NSD1 by comparative genomic hybridization to DNA microarrays. 3) He had no mutation in the 5q35 region in the non‐deleted chromosome using exsome sequence analysis. In conclusion, our patient supported that HIH could be one of the characteristic symptoms of SoS in the neonatal period, and could be useful for early diagnosis. © 2015 Wiley Periodicals, Inc.
Comparing apples and oranges—the influence of food mechanical properties on ingestive bite sizes in lemurs
Authors: Hartstone‐Rose Adam , Parkinson Jennifer A. , Criste Taylor , Perry Jonathan M.G.
Published: February 2015

Previously we found that Maximum Ingested Bite Size (Vb)—the largest piece of food that an animal will ingest whole without biting first—scales isometrically with body size in 17 species of strepsirrhines at the Duke Lemur Center (DLC). However, because this earlier study focused on only three food types (two with similar mechanical properties), it did not yield results that were easily applied to describing the broad diets of these taxa. Expressing Vb in terms of food mechanical properties allows us to compare data across food types, including foods of wild lemurs, to better understand dietary adaptations in lemurs. To this end, we quantified Vb in five species of lemurs at the DLC representing large and small frugivores and folivores using ten types of food that vary widely in stiffness and toughness to determine how these properties relate to bite sizes. We found that although most species take smaller bites of stiffer foods, this negative relationship was not statistically significant across the whole sample. However, there is a significant relationship between bite size and toughness. All three of the more frugivorous taxa in our sample take significantly smaller bites of tougher foods. However, the two more folivorous lemurs do not. They take small bites for all foods. This suggests that the species most adapted to the consumption of tough foods do not modulate their ingestive sizes to accommodate larger pieces of weak foods. Am J Phys Anthropol 157:513–518, 2015. © 2015 Wiley Periodicals, Inc.
Skeletal evidence of tuberculosis in a modern identified human skeletal collection (Certosa cemetery, Bologna, Italy)
Authors: Mariotti Valentina , Zuppello Micol , Pedrosi Maria Elena , Bettuzzi Matteo , Brancaccio Rosa , Peccenini Eva , Morigi Maria Pia , Belcastro Maria Giovanna
Published: February 2015

The diagnosis of tuberculosis (TB) in osteoarcheological series relies on the identification of osseous lesions caused by the disease. The study of identified skeletal collections provides the opportunity to investigate the distribution of skeletal lesions in relation to this disease. The aim of this study was to examine the skeletal evidence for TB in late adolescent and adult individuals from the identified human collection of the Certosa cemetery of Bologna (Italy, 19th–20th c.). The sample group consists of 244 individuals (138 males, 106 females) ranging from 17 to 88 years of age. The sample was divided into three groups on the basis of the recorded cause of death: TB (N = 64), pulmonary non‐TB (N = 29), and other diseases (N = 151). Skeletal lesions reported to be related to TB were analyzed. The vertebral lesions were classified into three types: enlarged foramina (EnF, vascular foramina with diameter of 3–5 mm), erosions (ER), and other foramina (OtF, cavities of various shapes > 3 mm). A CT scan analysis was also performed on vertebral bodies. Some lesions were seldom present in our sample (e.g., tuberculous arthritis). OtF (23.7%) and subperiosteal new bone formation on ribs (54.2%) are significantly more frequent in the TB group with respect to the other groups. The CT scan analysis showed that the vertebrae of individuals who have died of TB may have internal cavities in the absence of external lesions. These traits represent useful elements in the paleopathological diagnosis of TB. Am J Phys Anthropol 157:389–401, 2015. © 2015 Wiley Periodicals, Inc.
Accentuated lines in the enamel of primary incisors from skeletal remains: A contribution to the explanation of early childhood mortality in a medieval population from Poland
Authors: Żądzińska Elżbieta , Lorkiewicz Wiesław , Kurek Marta , Borowska‐Strugińska Beata
Published: February 2015

Physiological disruptions resulting from an impoverished environment during the first years of life are of key importance for the health and biological status of individuals and populations. Studies of growth processes in archaeological populations point to the fact that the main causes of childhood mortality in the past are to be sought among extrinsic factors. Based on this assumption, one would expect random mortality of children, with the deceased individuals representing the entire subadult population. The purpose of this study is to explore whether differences in early childhood survival are reflected in differences in deciduous tooth enamel, which can provide an insight into the development of an individual during prenatal and perinatal ontogeny. Deciduous incisors were taken from 83 individuals aged 2.0–6.5 years from a medieval inhumation cemetery dated AD 1300–1600. Prenatal and postnatal enamel formation time, neonatal line width, and the number of accentuated lines were measured using an optical microscope. The significantly wider neonatal line and the higher frequency of accentuated lines in the enamel of the incisors of children who died at the age of 2–3 years suggest the occurrence of stronger or more frequent stress events in this group. These results indicate that in skeletal populations mortality was not exclusively determined by random external factors. Individuals predisposed by an unfavorable course of prenatal and perinatal growth were more likely to die in early childhood. Am J Phys Anthropol 157:402–410, 2015. © 2015 Wiley Periodicals, Inc.
Selbstassoziation organischer Kationen über NCH⋅⋅⋅O‐Wasserstoffbrückenbindungen
Authors: Gamrad Waltraud , Dreier Angelika , Goddard Richard , Pörschke Klaus‐Richard
Published: February 2015

Die Ionenpaarung gegensätzlich geladener Ionen ist ein Grundprinzip der Chemie. Dagegen ist die Assoziation gleich geladener Ionen, z. B. organischer Kationen zu Organopolykationen, experimentell kaum belegt. Bei Strukturuntersuchungen an 4‐Oxopiperidiniumsalzen [OC5H8NH2]X mit Anionen X− abnehmender Basizität wurde eine allmähliche Selbstassoziation der Kationen bis zur diskreten Paarbildung beobachtet. In kristallinem 4‐Oxopiperidinium[bis(trifluormethylsulfonyl)amid] sind die Kationen über NH⋅⋅⋅OC‐Wasserstoffbrücken zu Ketten verbunden, die von den Anionen flankiert werden. Dagegen liegen im Tetra(perfluor‐tert‐butoxy)aluminat Anionen und Kationen vollständig separiert vor. Die Kationen sind hier über NCH⋅⋅⋅OC‐Wasserstoffbrücken zu zentrosymmetrischen Paaren assoziiert. Die Verbindungen belegen erstmals strukturanalytisch die Selbstassoziation einfacher organischer Kationen basierend auf Wasserstoffbrücken und haben somit Modellcharakter für diesen Verbindungstyp.
Reversible photochemische Modifikationen an Metallacyclen aus Dicarbenen mit Cumarinsubstituenten
Authors: Han Ying‐Feng , Jin Guo‐Xin , Daniliuc Constantin G. , Hahn F. Ekkehardt
Published: February 2015

Molekulare Rechtecke werden aus zwei Bis(NHC)‐Liganden, die jeweils zwei endständige Cumaringruppen tragen, sowie zwei Ag+‐, Au+‐ oder Cu+‐Ionen erhalten. Bei UV‐Bestrahlung (λ=365 nm) erfolgt eine photochemische Modifikation der zweikernigen Komplexe durch eine [2+2]‐Cycloaddition von zwei gegenüberliegenden Cumaringruppen unter Bildung eines makrocyclischen Tetra(NHC)‐Liganden. Die Photodimerisierung der Cumarinsubstituenten verläuft stereoselektiv und ergibt ausschließlich das syn‐Kopf‐Kopf‐Isomer. Eine nachfolgende Bestrahlung bei λ=254 nm initiiert die Photospaltung unter Rückbildung des zweikernigen Ausgangskomplexes mit vier Cumarinsubstituenten.
Cation–Cation Pairing by NCH⋅⋅⋅O Hydrogen Bonds
Authors: Gamrad Waltraud , Dreier Angelika , Goddard Richard , Pörschke Klaus‐Richard
Published: February 2015

The pairing of ions of opposite charge is a fundamental principle in chemistry, and is widely applied in synthesis and catalysis. In contrast, cation–cation association remains an elusive concept, lacking in supporting experimental evidence. While studying the structure and properties of 4‐oxopiperidinium salts [OC5H8NH2]X for a series of anions X− of decreasing basicity, we observed a gradual self‐association of the cations, concluding in the formation of an isolated dicationic pair. In 4‐oxopiperidinium bis(trifluoromethylsulfonyl)amide, the cations are linked by NH⋅⋅⋅OC hydrogen bonds to form chains, flanked by hydrogen bonds to the anions. In the tetra(perfluoro‐tert‐butoxy)aluminate salt, the anions are fully separated from the cations, and the cations associate pairwise by NCH⋅⋅⋅OC hydrogen bonds. The compounds represent the first genuine examples of self‐association of simple organic cations based merely on hydrogen bonding as evidenced by X‐ray structure analysis, and provide a paradigm for an extension of this class of compounds.
Reversible Photochemical Modifications in Dicarbene‐Derived Metallacycles with Coumarin Pendants
Authors: Han Ying‐Feng , Jin Guo‐Xin , Daniliuc Constantin G. , Hahn F. Ekkehardt
Published: February 2015

Molecular rectangles were obtained from two bis(NHC) ligands, each featuring two terminal coumarin groups and two Ag+, Au+, or Cu+ ions. Upon UV irradiation (λ=365 nm), the dinuclear complexes undergo photochemical modification through a [2+2] cycloaddition reaction of two adjacent coumarin moieties to give a macrocyclic tetra(NHC) ligand. The photodimerization of the coumarin pendants proceeds stereoselectively to give the syn‐head‐head isomers in all cases. Subsequent irradiation at λ=254 nm initiates a photocleavage reaction with reconstitution of the initial dinuclear complexes with coumarin pendants.
Replication of Standardized ADOS Domain Scores in the Simons Simplex Collection
Authors: Bal Vanessa Hus , Lord Catherine
Published: February 2015

Raw totals from diagnostic and screening measures for autism spectrum disorder (ASD) are frequently used as dimensional measures of autism symptom severity without appropriate correction for confounding factors, such as developmental level or non‐ASD‐specific behavior problems. Although these associated features are important to consider when diagnosing ASD and developing intervention plans, both researchers and clinicians sometimes need metrics of ASD severity that are not influenced by these factors. The Autism Diagnostic Observation Schedule (ADOS) domain calibrated severity scores (CSS) were created to provide separate estimates of social affect (SA‐CSS) and restricted, repetitive behaviors (RRB‐CSS) that are relatively independent of child characteristics (Hus et al., 2014). Using a sample of 2,509 probands with ASD from the Simons Simplex Collection (SSC), this study provides the first replication of the ADOS domain CSS in an independent sample. Consistent with the original standardization study, when applied to existing SSC data, the ADOS domain CSS were less influenced by age and cognitive ability compared to raw domain totals. Domain CSS were also relatively independent of behavior problems. Use of the ADOS domain CSS to assess relationships between ASD symptoms and genetic risk factors will increase confidence that associations reflect domain‐specific relationships. Scores also offer less developmentally‐influenced estimates of ASD severity for future phenotypic explorations in the SSC. This independent replication provides support for the application of the ADOS domain CSS in other samples, though further replication in population‐based samples will be an important next step. Autism Res 2015, 8: 583–592. © 2015 International Society for Autism Research, Wiley Periodicals, Inc.
Meso-Rex bypass for extrahepatic portal vein obstruction in children
Authors: Guérin F , Bidault V , Gonzales E , Franchi-Abella S , De Lambert G , Branchereau S
Published: February 2015

Background

Meso-Rex bypass (MRB) and portosystemic surgical shunt (PSS) are both used to treat extrahepatic portal vein obstruction (EHPVO) in children. The aim of this study was to analyse the outcome of MRB and PSS to select patients who could benefit from a prophylactic MRB.

Methods

This single-centre retrospective study of children who underwent either MRB or PSS for EHPVO was conducted between 1996 and 2010. Details of patient demographics and preoperative evaluation were collected. Success rates, defined as shunt patency after a minimum of 6 months and clearance of varices or symptoms, were compared. Determinants of outcomes were explored.

Results

Sixty-nine patients underwent a MRB or PSS. Median (interquartile range, i.q.r.) age at surgery was 6·6 (4·0–10·6) years. Twenty-four patients (35 per cent) had had a neonatal umbilical catheter (NUC) placed previously and 47 (68 per cent) had experienced an upper gastrointestinal bleed. Imaging assessment of the intrahepatic left portal vein was considered favourable in 40 patients. Of 43 MRBs attempted, 11 failed during surgery and four patients had persistent thrombosis after a median of 55 (i.q.r. 18–107) months. The success rate of MRB was 60 per cent (26 of 43) compared with 100 per cent (26 of 26) for PSS (P < 0·001). It was lower among patients in whom a NUC had been used (2 of 10 versus 24 of 33; P = 0·004), for procedures undertaken early in the series (6 of 16 versus 20 of 27; P = 0·020) and when the imaging pattern was unfavourable (0 of 5 versus 26 of 38; P = 0·006). On multivariable analysis, only a previous history of NUC predicted failure (P = 0·016).

Conclusion

Prophylactic MRB seems a good treatment option for EHPVO in children, but should be done only by an experienced team in patients with favourable imaging and without a previous history of NUC.

Presented in part to the 43rd Annual Meeting of the American Pediatric Surgical Association, San Antonio, Texas, USA, May 2012