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This paper highlights the significance of escalating commitment to strategy formulation, briefly examines behavioral explanations of escalating commitment to a losing course of action, and finally proposes an economic theory of the escalating commitment phenomenon. An example of escalating commitment to an outdated technology is presented. It is shown mathematically that there are economic justifications for escalating commitment to a failing strategy. Strategic implications for the first move advantage and for the U.S. steel, auto and semiconductor industries are discussed. [ABSTRACT FROM AUTHOR]

The objective of the present study was to determine whether golf clubs that have titanium (Ti) alloy surfaces can produce sparks when abraded under normal swing conditions. In the present work, sparks are defined as moving particles that emit radiant energy due to the process of combustion on its surface. Two three-irons and a three-wood containing a Ti alloy in the head as well as two three-irons and a three-wood that only contain stainless steel in the head were included in the study. The impact events during abrasion testing were recorded using a high-speed video camera, and abrasion damage was determined using stereometric analysis of scanning electron microscopy images. The findings reveal that Ti alloy faceplates that extend to the sole of the club can produce a number of Ti alloy particles when abraded under swing conditions. The particles then combust for a sufficient duration to potentially ignite a neighboring fuel source such as dry foliage and grasses. Abraded Ti alloy microparticles up to 500 µm in diameter were observed to burn for nearly 1 s, allowing ample time for fuel ignition. By contrast, no sparks were produced by stainless steel club heads when tested under the same conditions. Copyright © 2014 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

This article presents a microstrip-fed slot-aperture hybrid antenna for wideband circularly polarized (CP) operations. The antenna consists of a U-shaped slot, a rectangular aperture, and a probing strip fed by a microstrip line. In general, the U-shaped slot and the rectangular aperture resonate at two separate frequencies. When properly arranged, the proposed antenna may resonate over a single wideband of CP radiation. The 3-dB axial ratio (AR) bandwidth of the presented antenna in free space is about 27% with the gain level about 34 dBic. When a planar reflector is incorporated for the desirable unidirectional pattern, the presented antenna has an enhanced gain about 7 dBic with a 3-dB AR bandwidth of 25% and a front-to-back ratio of about 14 dB. Good agreement between measured and simulated results was achieved. © 2011 Wiley Periodicals, Inc. Microwave Opt Technol Lett 54:412-415, 2012; View this article online at wileyonlinelibrary.com. DOI 10.1002/mop.26569 [ABSTRACT FROM AUTHOR]

Theoretically and empirically, the linkage between outside directors and firm performance is not conclusive in previous studies. We suspect that the mixed results are due to the failure to meet the requirements of the independence of outside directors. As the Taiwanese government has a rigorous definition of outside director independence, we employ a data set from Taiwan to test the impact of independent outside director assignment on a firm's performance. Our findings suggest that after controlling for a firm's past performance, independent outside director appointments do have a significantly positive impact on a firm's performance, and outperforming firms may have better performance but not significantly when assigning outside directors due to their absorptive capacity. [ABSTRACT FROM AUTHOR]

The aim of this study is to foster an understanding of the clinical characteristics, in terms of general psychopathology, of Tourette syndrome (TS), as it manifests in adolescent patients, and to compare these results with those of previous studies conducted in Western countries. In total, 38 male and 5 female adolescent patients who visited a pediatric neurological specialty clinic for TS between 1 January and 1 March 2003, and who met the World Health Organisation criteria for TS, were studied. These patients were clinically interviewed and assessed with the Symptom-Checklist-90-R, Family APGAR and Tic Symptoms questionnaire, a self-report questionnaire based on DSM-IV TS diagnostic criteria. Among this population, the mean age of onset of motor tics was 9.65 ± 3.7 years, and motor tics of the head and eyes were usually the first symptoms to manifest; coprolalia occurred in 44.1% ( n = 19) of our patients. Many of our findings were similar to those reported in trials conducted in other regions of the world. TS symptoms showed stronger correlation with emotional distress in older patients. [ABSTRACT FROM AUTHOR]

Focuses on the dynamics of strategic maneuvering in the U.S. insurance industry. Assessment of competitive positioning; Determination of optimal maneuvering paths and long-term economic performance; Aspects of hypercompetitive markets.

This article identifies key features of the strategy concept as a basis for reviewing existing theories of strategy. It also provides an agenda for future research. The article argues that there is a decision hierarchy in a firm, and defines strategy as the highest-level decision in the hierarchy. Consequently, strategy has to exhibit what may be called three features of dominance: vertical dominance, horizontal dominance, and dynamic dominance. A vertically dominant strategy is one that determines, directly or indirectly, other decisions of a firm. A horizontally dominant strategy seeks to optimize the value of the firm according to some criteria. Dynamic dominance implies that strategy should affect the subsequent decisions of a firm over a relevant period of time. With few exceptions, existing theories of strategy are issue-oriented and focus largely on horizontal dominance. More research should, therefore, be directed towards vertical and dynamic dominance criteria. If a comprehensive theory of strategy which satisfies the three dominance features cannot be developed, it is suggested that development should be directed towards integrating behavioural and economic approaches. [ABSTRACT FROM AUTHOR]

Investigates the rationale for a multinational corporation to enter into a joint venture to serve a host market. Impact of profit sharing, cost reductions, risk reductions and competition reductions on the profits of international joint ventures; Factors behind the growth in popularity of international joint ventures.

Studies the links between theories of spatial competition and cognitive taxonomy to strategic groups in a competitive environment. Description of the concept of strategic groups as a theorerical construct; Cognitive models.

Examines competition patterns and competitive strategies when technology changes continually. Optimal behavior for investment in technology; Implications of fixities for a firm's strategy; Mobility barriers between different strategic groups choosing different strategies.

Holoprosencephaly (HPE), a common developmental forebrain malformation, is characterized by failure of the cerebrum to completely divide into left and right hemispheres. The etiology of HPE is heterogeneous and a number of environmental and genetic factors have been identified. Cytogenetically visible alterations occur in 25% to 45% of HPE patients and cytogenetic techniques have long been used to study copy number variants (CNVs) in this disorder. The karyotype approach initially demonstrated several recurrent chromosomal anomalies, which led to the identification of HPE-specific loci and, eventually, several major HPE genes. More recently, higher-resolution cytogenetic techniques such as subtelomeric multiplex ligation-dependent probe amplification and chromosomal microarray have been used to analyze chromosomal anomalies. By using chromosomal microarray, we sought to identify submicroscopic chromosomal deletions and duplications in patients with HPE. In an analysis of 222 individuals with HPE, a deletion or duplication was detected in 107 individuals. Of these 107 individuals, 23 (21%) had variants that were classified as pathogenic or likely pathogenic by board-certified medical geneticists. We identified multiple patients with deletions in established HPE loci as well as three patients with deletions encompassed by 6q12-q14.3, a CNV previously reported by Bendavid et al. In addition, we identified a new locus, 16p13.2 that warrants further investigation for HPE association. Incidentally, we also found a case of Potocki-Lupski syndrome, a case of Phelan-McDermid syndrome, and multiple cases of 22q11.2 deletion syndrome within our cohort. These data confirm the genetically heterogeneous nature of HPE, and also demonstrate clinical utility of chromosomal microarray in diagnosing patients affected by HPE., (© 2018 Wiley Periodicals, Inc.)

Focal facial dermal dysplasias (FFDD) are characterized by congenital bitemporal or preauricular atrophic skin lesions, and either autosomal dominant or autosomal recessive inheritance. Setleis syndrome (SS), FFDD type III, is a severe form of FFDD with the ectodermal lesions plus other striking facial features. Autosomal recessive nonsense and frameshift mutations in TWIST2 have been found to cause SS in some but not all individuals. Here, we report on four unrelated individuals, one with an unclassified FFDD and the other three with classic SS. Chromosomal microarray analyses revealed unique copy number variants of 1p36 in two individuals with duplications at 1p36.22p36.21 and one with a triplication at 1p36.22p36.21. The fourth patient had normal chromosomes by microarray analysis. All four patients had normal TWIST2 exonic sequences. We propose that a dosage effect of one or more of the 30 genes in the 1.3 Mb 1p36.22p36.21 region of overlap is responsible for FFDD/SS manifestations in some individuals, and this mechanism would be inherited as an autosomal dominant trait. In patients with no duplication/triplication of the 1p36.22p36.21 region and no mutations in TWIST2, there are mutation(s) in one of the 30 genes in this region or mutations in other as yet unidentified genes at different locations that may affect the expressions of genes in this region or act independently to cause this developmental disease phenotype., (© 2015 Wiley Periodicals, Inc.)

Kabuki syndrome is a rare, multiple malformation disorder characterized by a distinctive facial appearance, cardiac anomalies, skeletal abnormalities, and mild to moderate intellectual disability. Simplex cases make up the vast majority of the reported cases with Kabuki syndrome, but parent-to-child transmission in more than a half-dozen instances indicates that it is an autosomal dominant disorder. We recently reported that Kabuki syndrome is caused by mutations in MLL2, a gene that encodes a Trithorax-group histone methyltransferase, a protein important in the epigenetic control of active chromatin states. Here, we report on the screening of 110 families with Kabuki syndrome. MLL2 mutations were found in 81/110 (74%) of families. In simplex cases for which DNA was available from both parents, 25 mutations were confirmed to be de novo, while a transmitted MLL2 mutation was found in two of three familial cases. The majority of variants found to cause Kabuki syndrome were novel nonsense or frameshift mutations that are predicted to result in haploinsufficiency. The clinical characteristics of MLL2 mutation-positive cases did not differ significantly from MLL2 mutation-negative cases with the exception that renal anomalies were more common in MLL2 mutation-positive cases. These results are important for understanding the phenotypic consequences of MLL2 mutations for individuals and their families as well as for providing a basis for the identification of additional genes for Kabuki syndrome., (Copyright © 2011 Wiley-Liss, Inc.)

Kabuki syndrome (KS) is associated with multiple organ system involvement. Characteristic features include long palpebral fissures with everted lower lids, prominent ears, skeletal abnormalities, mental retardation, and short stature. An increased incidence of infection has been reported in KS, and a few patients have been noted to have immune defects. However, the frequency and severity of the immune deficiency has not been clearly defined. Immunologic evaluation of 19 consecutive individuals with KS was performed at The Children's Hospital of Philadelphia. Decreased IgA levels were noted in 15/19 individuals (79%), 2 of whom had undetectable levels. Eight patients (42%) also had low total IgG levels. Specific IgG subclass abnormalities were found in 6 of 13 patients evaluated. IgM levels were less frequently decreased. One patient failed to generate anti-tetanus antibodies despite immunization. This study suggests that hypogammaglobulinemia is a frequent finding in children with KS. The pattern of antibody abnormalities seen in children with KS resembles common variable immune deficiency (CVID). Due to this increased susceptibility to infection, children with KS should have immunologic evaluations at the time of diagnosis in order to reduce preventable morbidity and mortality.

Kabuki syndrome is a multiple congenital anomaly/mental retardation syndrome. This study of Kabuki syndrome had two objectives. The first was to further describe the syndrome features. In order to do so, clinical geneticists were asked to submit cases-providing clinical photographs and completing a phenotype questionnaire for individuals in whom they felt the diagnosis of Kabuki syndrome was secure. All submitted cases were reviewed by four diagnosticians familiar with Kabuki syndrome. The diagnosis was agreed upon in 48 previously unpublished individuals. Our data on these 48 individuals show that Kabuki syndrome variably affects the development and function of many organ systems. The second objective of the study was to explore possible etiological clues found in our data and from review of the literature. We discuss advanced paternal age, cytogenetic abnormalities, and familial cases, and explore syndromes with potentially informative overlapping features. We find support for a genetic etiology, with a probable autosomal dominant mode of inheritance, and speculate that there is involvement of the interferon regulatory factor 6 (IRF6) gene pathway. Very recently, a microduplication of 8p has been described in multiple affected individuals, the proportion of individuals with the duplication is yet to be determined., ((c) 2004 Wiley-Liss, Inc.)

Kabuki (Niikawa-Kuroki) syndrome is associated with a characteristic facial appearance, cleft palate, congenital heart defects, and developmental delay. Structural brain anomalies have only occasionally been described in Kabuki syndrome. Chiari type I malformation, characterized by caudal herniation of the cerebellar tonsils through the foramen magnum, has been described only infrequently in association with defined syndromes and has been reported once in association with Kabuki syndrome. We report three additional children with Kabuki syndrome who have Chiari I malformation. Two children presented with chronic headaches and the third patient presented with gait abnormalities in adolescence. The incidence of Chiari I malformation may be higher than previously reported in Kabuki syndrome since it may not be diagnosed until later in childhood, whereas most reports of Kabuki syndrome are of young children. Further, symptoms of Chiari I anomaly can be somewhat nonspecific. Thus, we suggest that Chiari type I be considered in patients with Kabuki syndrome who present with persistent headache, neck pain, or other symptoms suggestive of Chiari I anomaly., ((c) 2004 Wiley-Liss, Inc.)

Kabuki syndrome is associated with abnormalities in multiple organ systems. While many of the anomalies are congenital malformations, other clinical manifestations may not appear until later in childhood. Among these associated conditions, autoimmune abnormalities have been described in several patients. These include idiopathic thrombocytopenic purpura (ITP), hemolytic anemia, thyroiditis, and vitiligo. In this report, we describe five affected patients with autoimmune manifestations. Four patients had ITP, and two of these patients had concurrent hemolytic anemia. The fifth patient had vitiligo. Two of the patients with ITP had a chronic and relapsing course. Of note, some of these patients also had hypogammaglobulinemia. The autoimmune disorders may be manifestations of abnormal immune regulation. We conclude that Kabuki syndrome is associated with an increased incidence of autoimmune disorders. In addition, the presence of an underlying immune defect may predispose these children to a chronic course of these autoimmune conditions., ((c) 2004 Wiley-Liss, Inc.)

Kabuki (Niikawa-Kuroki) syndrome is associated with growth retardation, developmental delay, congenital heart disease, cleft palate, and characteristic facial features. Although the external appearance of the eyes has been well-described, the type and frequency of structural and functional eye anomalies has not been emphasized. We report three children with Kabuki syndrome who also had a retinal coloboma. A diagnosis of CHARGE association was initially suggested in two of the patients before the typical facial features of Kabuki syndrome emerged. A detailed review of reported cases of Kabuki syndrome shows that a variety of eye anomalies are associated with Kabuki syndrome. The incidence of coloboma is greatly increased in Kabuki syndrome. Thus, ophthalmologic abnormalities are frequently associated with Kabuki syndrome, and an ophthalmologic evaluation should be performed for each patient. Phenotypic overlap, including congenital heart, ear, and renal defects, can lead to the diagnosis of CHARGE association, especially since the typical facial features of Kabuki syndrome may not be apparent in early infancy. Thus, Kabuki syndrome should be considered in patients with coloboma if other features consistent with this condition are present, and follow-up evaluations are indicated for establishing the proper diagnosis., (Copyright 2003 Wiley-Liss, Inc.)