bluuebunny/crossref_metadata_2025 huggingface dataset:
Different isoform patterns for vascular endothelial growth factor between clear cell and papillary renal cell carcinoma
Authors: JACOBSEN JAN , GRANKVIST KJELL , RASMUSON TORGNY , LJUNGBERG BÖRJE
Published: April 2006

OBJECTIVE

To investigate the protein expression of vascular endothelial growth factor (VEGF) isoforms in relation to the clinical course in patients with different renal cell carcinoma (RCC) types, as angiogenesis is essential for tumour growth and metastasis.

PATIENTS AND METHODS

Western blots were assayed of protein extracts from tumour and concomitant kidney cortex samples from 96 patients. The levels of VEGF189, VEGF 165, and VEGF 121 isoforms were correlated with clinicopathological characteristics and survival.

RESULTS

VEGF189 levels were significantly higher in kidney cortex and chromophobe RCC than in papillary and conventional RCC. In papillary RCCs, VEGF189 levels correlated inversely with tumour stage and tumour size. VEGF165 levels were higher in kidney cortex than in RCC, but there was no difference among the RCC types. VEGF121 expression was associated with less advanced tumour stage in conventional RCC. Using multivariate analysis, VEGF189 remained as an independent prognostic factor for patients with papillary RCC.

CONCLUSIONS

VEGF189 was associated with tumour progression; in papillary RCC, VEGF189 was a significant independent prognostic factor. VEGF protein isoform patterns differed among the specific RCC types. Additional knowledge is essential to design new anti‐angiogenic therapies for all RCC types.
China's Integration into the World Economy: implications for developing countries
Authors: Yang Yongzheng
Published: April 2006

With China's accession to the World Trade Organization in late 2001, many of its trading partners have become concerned that the resulting increased competition in the world's goods and capital markets will adversely affect their own growth prospects. This paper argues that China's closer integration into the world economy will impose adjustment costs on its trading partners in the short to medium term, but that the benefits it generates are likely to dominate in the long run.
The Foreign v. the Domestic after September 11th: The Methodology of Political Analysis Revisited
Authors: Haubrich Dirk
Published: April 2006

The implications of the terrorist attacks on 11 September 2001 are far-reaching and have been discussed and analysed at great length. In this article, it is contended that the methodology of analysing the political, too, has been affected. The policies that liberal democracies have adopted over the past three years to contain the new threat of transnational terrorism call into question the methodological approaches that political researchers conventionally employ to analyse their subject matter. Rather than examining political processes at home separately from those occurring abroad, developments since September 11th demand that we dispense with those boundaries and develop an integrated approach.
Widening Participation, Equalising Opportunity? Higher Education's Mission Impossible
Authors: Hale Sarah
Published: April 2006

Current government policy of increasing participation in higher education is justified on the grounds of individual benefit, the national economic interest and, most significantly, as part of a moral agenda of promoting equality of opportunity. This article examines a range of empirical findings in the light of ideas about equality and, in particular, the concept of ‘equality of opportunity’, and what these entail. It concludes that widening participation in higher education, at least as envisaged in current policy, cannot compensate for social and educational disadvantage, and is not only ineffectual in promoting equality of opportunity, but carries serious disbenefits.
The Public Interest and Public Administration
Authors: Elcock Howard
Published: April 2006

Ever since ancient Greece, philosophers have assumed the existence of a public interest that is more than the sum of the interests of the individuals that make up a polity. However, the utilitarians and more recently the theorists of the ‘New Right’ have argued that the public interest consists simply of individual interests summed together, with the result that the related notion that public servants have a special duty to protect and promote the collective public interest has been subjected to sustained attack. However, the collective public interest remains in the form of four values that public servants are required to promote: accountability, legality, integrity and responsiveness. In consequence, public sector management is essentially different from business management and can only be subsumed within the latter to a limited extent when considering the education pf public servants.
A Weekend in Padania: Regionalist Populism and the Lega Nord
Authors: McDonnell Duncan
Published: April 2006

This article argues that the Lega Nord is best understood, first and foremost, as a regionalist populist party. Following a brief discussion of the importance of the territorial reference in explaining the Lega, the article examines its populist discourse using material from the party's newspaper La Padania over the three days in June 2005 around the annual Lega rally in Pontida.
The Lega dei Ticinesi: The Embodiment of Populism
Authors: Albertazzi Daniele
Published: April 2006

This article views ‘populism’ as an ideology, the key features of which are identified and briefly discussed. Having assessed the extent to which such features match the profile of the Swiss Lega dei Ticinesi, the author concludes that this party should be seen as a paradigmatic embodiment of populism due to: a) the party's uneasiness with representative democracy; b) the crucial role played by the concept of the ‘people’ in its propaganda; c) the power of the leader within the organisation; and d) the party's chameleon-like tendency to borrow keywords and ideas from both sides of the political divide.
Educating American Muslim Leadership (Men and Women) for the Twenty‐First Century
Authors: Al‐Islam Amir
Published: April 2006

Abstract.  Educating and training Muslim men and women leaders who are capable of effectively navigating the multi‐ethnic and multi‐religious terrain in America – particularly in the post 9–11 milieu – requires the development of a new critical American Muslim pedagogy. This new pedagogy, centered in Islamic epistemology and ontology, should selectively appropriate the best of traditional Muslim educational paradigms and modalities used over time. However, the traditional Muslim model must not be reified, but rather be subjected to a sharp critique which maintains the richness of its spiritual and intellectual legacy but rejects teachings and interpretations used to create false dichotomies resulting in binary constructs, particularly those which pit Muslims against the west. Finally, the new critical American Muslim pedagogy must embrace all of the best discursive practices (e.g., pedagogies of Freire and others) that engage us in a critical analysis of the way in which power and privilege, even in religious communities, operate to marginalize and suppress women, minorities, and people of color.
Catholic Theological Education in a Religiously Pluralistic Age
Authors: Lefebure Leo D.
Published: April 2006

Abstract.  This article describes the transformation of Catholic theological education over the last fifty years from a highly defensive posture vis‐à‐vis other religions toward dialogical engagement with members of other religions and all persons of good will. Until Vatican II, most Catholic theologians and officials distrusted exploration of other religions as leading to a dilution of Catholic identity. Vatican II condemned anti‐Semitism and called for dialogue among religions in pursuit of common values. Since the Council, there have been developments in interfaith education on three levels: religious studies, comparative theology, and inter‐religious practice.
Vodou: A Sacred Multidimensional, Pluralistic Space
Authors: Désir Dowoti
Published: April 2006

Abstract. This paper uses the language of Vodou doctrine to articulate its key tenets and speak to how the challenge of plurality or diversity in the twenty‐first century has been and continues to be addressed among African and Afro Atlantic spiritual leadership. Following the slave trade and colonialism's aftermath, a pluralistic vision, reflecting the harsh new global order, permitted spiritual sustainability by reconfiguring African ontologisms. Embracing pluralism through annexation of non‐native spiritual practices augmented the power of African rulers, providing them with other epistemes and access to spiritual forces they believed enhanced their position. The issue of preparing for the priesthood in a global or pluralistic society is examined in this essay through the historical and metaphysical framework that shaped the making of our societies.
“To a Land that I Will Show You”: Training Rabbis for the Future
Authors: Greenstein David
Published: April 2006

Abstract.  Rabbis are commonly perceived as bearers of Torah – the sacred traditions and ways of life of Judaism. As such, rabbis certainly have an important role to play in a community seeking guidance and inspiration from and a renewed connection to those traditions. Yet, historically, rabbis arose as a class in a period of crisis and were not merely conservative figures, but were also radical agents for change. The training of rabbis in the contemporary world calls for an assessment of our situation. Is our time a time of crisis? If it is, how should we prepare to meet that crisis? Do rabbis have a role to play in the future? While the texts and traditions of the past are available for study, interpretation, and application, is there a need to prepare rabbis to become effective agents for change? How can we embark upon such an uncharted path?
Training vs. Education in Forming Won Buddhist Kyomus in the USA
Authors: Kim Bokin
Published: April 2006

Abstract.  An historically familiar tension in East Asian Buddhism between meditation and cultivation in broad learning has appeared in discussions and planning for preparing ministerial students in Won Buddhism. This paper reviews the history of preparation in this order, which was founded in 1916. While the alternatives of training based on practice and education based on classroom intellectual experience have occurred in Won Buddhism, the tension has appeared within the recently founded Won Institute of Graduate Studies in the USA in a clear manner. While the pre‐ministerial students coming from Korea have preferred the experiential/practical emphasis, it is recognized that graduate education in the USA normally requires broader learning and critical thinking. The faculty of Won Institute respects both strategies and their respective, almost incompatible, goals, and has tried to create a curriculum embracing both. This effort is described and viewed in the context of Won participation in a culture of pluralism and interreligious relations.
Hindu Leaders in North America?
Authors: Sarma Deepak
Published: April 2006

Abstract. “Are there Hindu leaders in North America”? Can there be leaders of a purportedly invented or imaginary religion that has no shared doctrines or beliefs? This provocative essay offers answers to these and related questions about the nature of Hindu leadership in North America. Three ideal types are examined: Ritualists, Guides, and Administrators. Their roles and responsibilities, though relatively clear in India, have become complicated in their current incarnations in North America. The difficulties are further enhanced when combined with a drive to derive a syncretic form of Hinduism, a pan‐Hinduism that never existed before. This article challenges the leaders of Hinduism in North America to confront and perhaps even jettison their invented identity as a way of becoming better leaders.
An Integrative Educational Strategy for Christian Leaders in a Multifaith World
Authors: Talvacchia Kathleen T.
Published: April 2006

Abstract.  This paper asserts that training Christian leaders for faithful and effective leadership in religious communities, which is responsive to the reality of the diverse religious experiences of this country, requires that they learn the skills of integration, specifically the ability to integrate formation into a community within the context of a multicultural, multifaith world. The process of pastoral theological reflection, a process that seeks to methodically put into conversation the student's experience, social context, and religious tradition, holds promise in a Christian context as a way to accomplish such integration. After discussing the process of pastoral theological reflection, the paper examines a seminary ministerial formation curriculum, based on this integrative process, to discern how it might better engage multifaith realities in its formation of leaders for Christian communities.
Patterns of Consent: Evidence from a General Household Survey
Authors: Jenkins Stephen P. , Cappellari Lorenzo , Lynn Peter , Jäckle Annette , Sala Emanuela
Published: April 2006

We analyse patterns of consent and consent bias in the context of a large general household survey, the ‘Improving survey measurement of income and employment’ survey, also addressing issues that arise when there are multiple consent questions. A multivariate probit regression model for four binary outcomes with two incidental truncations is used. We show that there are biases in consent to data linkage with benefit and tax credit administrative records that are held by the Department for Work and Pensions, and with wage and employment data held by employers. There are also biases in respondents’ willingness and ability to supply their national insurance number. The biases differ according to the question that is considered. We also show that modelling questions on consent independently rather than jointly may lead to misleading inferences about consent bias. A positive correlation between unobservable individual factors affecting consent to Department for Work and Pensions record linkage and consent to employer record linkage is suggestive of a latent individual consent propensity.
Improved Likelihood Inference for Discrete Data
Authors: Davison A. C. , Fraser D. A. S. , Reid N.
Published: April 2006

Discrete data, particularly count and contingency table data, are typically analysed by using methods that are accurate to first order, such as normal approximations for maximum likelihood estimators. By contrast continuous data can quite generally be analysed by using third-order procedures, with major improvements in accuracy and with intrinsic separation of information concerning parameter components. The paper extends these higher order results to discrete data, yielding a methodology that is widely applicable and accurate to second order. The extension can be described in terms of an approximating exponential model that is expressed in terms of a score variable. The development is outlined and the flexibility of the approach is illustrated by examples.
Testing Against a High Dimensional Alternative
Authors: Goeman Jelle J. , Van De Geer Sara A. , Van Houwelingen Hans C.
Published: April 2006

As the dimensionality of the alternative hypothesis increases, the power of classical tests tends to diminish quite rapidly. This is especially true for high dimensional data in which there are more parameters than observations. We discuss a score test on a hyperparameter in an empirical Bayesian model as an alternative to classical tests. It gives a general test statistic which can be used to test a point null hypothesis against a high dimensional alternative, even when the number of parameters exceeds the number of samples. This test will be shown to have optimal power on average in a neighbourhood of the null hypothesis, which makes it a proper generalization of the locally most powerful test to multiple dimensions. To illustrate this new locally most powerful test we investigate the case of testing the global null hypothesis in a linear regression model in more detail. The score test is shown to have significantly more power than the F-test whenever under the alternative the large variance principal components of the design matrix explain substantially more of the variance of the outcome than do the small variance principal components. The score test is also useful for detecting sparse alternatives in truly high dimensional data, where its power is comparable with the test based on the maximum absolute t-statistic.
Subsistence and sales: the peasant economy of Württemberg in the early seventeenth century
Authors: WARDE PAUL
Published: April 2006

This article examines the engagement of peasant cultivators with the grain market in Germany in the seventeenth century. It demonstrates a differentiated propensity to sell in regard to different grains; a preference among cultivators for retaining subsistence foodstuffs; the importance of payment in kind in the labour market; and the lack of a clear‐cut social structural divide between grain sellers and buyers among those who cultivate arable land. It is thus argued that the analytical concept of ‘the peasant’ retains its use in understanding this society, but that attitudes displayed by the peasantry to ‘the market’ must be clearly set in the context of the specific product markets and practices to retain any analytical value.
Strategic decision‐making: Process perspectives
Authors: Elbanna Said
Published: April 2006

This paper reviews the strategic decision‐making process literature with respect to the synoptic formalism/political incrementalism debate. Procedural rationality is chosen as a representative of the synoptic formalism perspective; and both intuitive synthesis and political behaviour are employed as representatives of the political‐incrementalism perspective. In this paper, the author discusses the theoretical underpinnings of these three process dimensions, as well as the key research efforts gathered together under each perspective. In conducting this review, a number of areas have been identified which could profitably be examined further, and a number of implications for managers will be highlighted and discussed.
The effectiveness of poverty reduction and the target efficiency of social security transfers in South Korea, 1999–2003
Authors: Choi Jae‐Sung , Choi Jeong‐Kyun
Published: April 2006

The purpose of this study was to investigate the impact of social security transfers between 1999 and 2003 in South Korea, the period during which the Asian economic crisis of 1997 occurred. The study used a secondary data set, which is part of the Korea Labour Panel Data. Of 5,000 original household samples, the results for the 2,728 households (54.6 per cent) that completed the surveys for all five years studied were analysed. One finding was that the average percentage of social security transfers appeared to be nominal, as was the 1.9 per cent in 1999, which grew to 2.4 per cent in 2003. Another finding was that the average poverty‐reduction effectiveness for the five‐year period was as low as 7.9 per cent, indicating a slightly increasing pattern. This percentage is only one‐seventh to one‐tenth that of Western countries. Target efficiency appeared to be 31.8 per cent. We give the following reasons to explain why the level of the poverty‐reduction effectiveness of Korean social security transfers is comparatively low: the immaturity of the Korean Old Age Pension; the lack of diversity in social transfer programmes; and a cultural factor of stronger dependency on private transfers within the family structure.
Iron mineralization and dolomitization in the Paran Fault zone, Israel: implications for low‐temperature basinal fluid processes near the Dead Sea Transform
Authors: GROSZ S. , MATTHEWS A. , ILANI S. , AYALON A. , GARFUNKEL Z.
Published: April 2006

Petrography, Eh‐pH calculations and the stable isotope composition of oxygen are used to interpret geochemical processes that occurred during iron oxide mineralization and dolomitization along the Menuha Ridge segment of the Paran Fault, southern Israel, adjacent to the Dead Sea Transform (DST). Iron mineralization is strongly localized in the fault zone as ferruginous lenses, whereas Fe dolomitization spreads laterally into the Cenomanian‐Turonian carbonate host rock as stratabound beds. The average oxygen isotope fractionation between syngenetic quartz and iron oxides in the ferruginous lenses gives a temperature of 50 ± 10°C and δ18O SMOW water = −3.5‰; consistent with an origin from metalliferous groundwater flow in the sedimentary basin. Ferroan dolomite initially formed under strongly reducing conditions, but this was followed by oxidation and pseudomorphic replacement of the dolomite by a mesh of fine‐grained iron oxides (simple zoned dolomites). This cycle of ferroan dolomite formation and replacement by iron oxides was repeated in complex zoned dolomites. Dolomite oxygen isotope compositions fall into two groups: a high δ18O group corresponding to the simple zoned dolomites and non‐ferroan dolomites and a low δ18O group corresponding to the complex zoned dolomites. Water‐rock calculations suggest that the epignetic dolomites formed under fluid‐buffered conditions: the high δ18O group are indicated to have formed at temperatures of ca. 25°C for waters with δ18O = −4 to 0‰; the low δ18O complex zoned dolomites at 50–75°C for waters with the same isotopic composition. A kinetic calculation for a complex zoned dolomite‐bearing bed indicates that dolomitization must have occurred at high values of the dolomite saturation index. This requirement for high Mg supersaturation and the indication that epigenetic dolomitization is more protracted in stratigraphically deeper formations located closer to the DST is consistent with models proposing that Mg‐rich solutions originated in the Dead Sea Rift.
IS IT ETHICAL TO STUDY WHAT OUGHT NOT TO HAPPEN?1
Authors: RENNIE STUART
Published: April 2006

In the Democratic Republic of Congo, only an estimated 2% of all AIDS patients have access to treatment. As AIDS treatment access is scaled‐up in the coming years, difficult rationing decisions will have to be made concerning who will come to gain access to this scarce medical resource. This article focuses on the position, expressed by representatives of Médecins sans Frontières (MSF), that the practice of AIDS treatment access rationing is fundamentally unethical because it conflicts with the ideal of universal treatment access and the human right to health. The conclusion is that MSF's position lacks coherence, has negative practical implications, and is unfair to governments struggling to increase patient's access to AIDS treatment in unfavorable circumstances.
DO AID AGENCIES HAVE AN ETHICAL DUTY TO COMPLY WITH RESEARCHERS? A RESPONSE TO RENNIE1
Authors: ZACHARIAH RONY , JANSSENS VINCENT , FORD NATHAN
Published: April 2006

Medical AID organisations such as Médecins Sans Frontières receive several requests from individuals and international academic institutions to conduct research at their implementation sites in Africa.

Do AID agencies have an ethical duty to comply with research requests? In this paper we respond to the views and constructed theories (albeit unfounded) of one such researcher, whose request to conduct research at one of our sites in the Democratic Republic of Congo was turned down.
ETHICS OF CARE AND HIV: A CASE FOR RURAL WOMEN IN INDIA
Authors: CHAKRABORTI CHHANDA
Published: April 2006

Recent literature shows that ethics of care can be used as a theoretical basis to add a new, important dimension to social issues. This paper argues for a similar extension of the theoretical support from ethics of care to an area in bioethics. Specifically, it contends that a justification based ethics of care can be constructed to argue for a moral obligation to give some priority in the HIV‐related initiatives to one of most vulnerable groups; namely, the rural women in India. In an epidemic situation this care‐based approach has certain advantages as a moral justification over the usual traditional approaches.
The analysis of Scottish Parliament committee influence: Beyond capacity and structure in comparing West European legislatures
Authors: CAIRNEY PAUL
Published: April 2006

Abstract.  The Scottish Parliament was set up in the hope that strong committees would foster consensus, with an emphasis on reducing partisanship and adopting a pragmatic approach to the detailed study of draft legislation. However, few empirical studies exist that assess the value of the committee process. This flaw is common within the West European literature. The comparative literature on legislative influence is lacking in detailed empirical studies (in part because of the dominant assumption within the literature that parliaments are peripheral to the policy process). Most studies provide impressionistic discussions of the capacities of committees and the constraints to their effectiveness. They do not follow this through with an analysis of committee ‘outputs’. This study of the amendments process in the Scottish Parliament addresses the gap. It uses data from a four‐year study of legislative amendments to develop indicators of parliamentary outputs. While the results confirm that the committee system operates at the heart of the ‘new politics’ in Scotland, further such individual country studies are necessary to supplement much broader comparative analyses.
May the weak force be with you: The power of the mass media in modern politics
Authors: NEWTON KENNETH
Published: April 2006

Abstract. The idea that the modern mass media have a strong and malign effect on many aspects of social and political life is widely and strongly held. Television is often said to undermine democratic government popular support for leaders and institutions. In spite of all that has been written about media malaise, however, both theory and evidence suggests that the media are a comparatively weak force whose effects can be deflected, diluted and diffused by stronger forces. These include bedrocks political values associated with class, religion, age, gender and education, as well as social networks and discussions, distrust of the mass media, and personal knowledge and experience. Equally, the variables that mediate the media may also magnify its effects so that what appears to be a large media effect is, in fact, the result of an interaction between the media and other forces. This article lays out the argument of the media malaise literature that covers government and politics, then outlines the social forces that mediate the media, and finally provides some evidence to illustrate the argument that the media are generally a weak force in society.
Impairment of Skin Barrier Function is not Inherent in Atopic Dermatitis Patients: A Prospective Study Conducted in Newborns
Authors: Kikuchi Katsuko , Kobayashi Hiromi , O’goshi Ken‐ichiro , Tagami Hachiro
Published: April 2006

Abstract:  We conducted a cohort study to determine whether the barrier dysfunction of the stratum corneum that facilitates the penetration of various exacerbating agents from the environment is inherent in atopic dermatitis patients as suggested by some dermatologists. Clinical observation and biophysical measurements of the skin were performed on the cheek and on the flexor forearm of 24 newborn infants once between 2 and 14 days postnatally and 1, 3, and 6 months later. Nineteen had atopic family histories. Most of the infants had physiologic neonatal xerosis that was observed as a reduced high‐frequency conductance without any impairment in the stratum corneum barrier function assessed by transepidermal water loss. Four of the 24 neonates developed atopic dermatitis around 2 to 3 months after birth. In all of them, barrier impairment noted as increased transepidermal water loss was observed only after the development of skin lesions. During their neonatal period, their transepidermal water loss and skin surface hydration state were indistinguishable from those of the neonates whose skin remained lesion‐free during the observation period. Therefore, we concluded that the barrier impairment found in atopic dermatitis is not inherent but represents a phenomenon secondary to dermatitic skin changes.
Profile of Childhood Vitiligo in China: An Analysis of 541 Patients
Authors: Hu Zhi , Liu Jiang‐Bo , Ma Sui‐Sui , Yang Sen , Zhang Xue‐Jun
Published: April 2006

Abstract:  From July to December 2002, we collected data from 2247 vitiligo patients in order to establish the clinical and epidemiologic profile of vitiligo in China. Of these patients, 541 (24.1%) were children aged equal to or less than 12 years. Of the 541 children, 274 (50.6%) were boys and 267 (49.4%) were girls, with a mean age of 8.87 years and a mean onset age of 7.28 years. Similar to adult patients, boys and girls were affected by vitiligo with equal frequency. The most frequent age of onset was between 4 and 8 years (42.5%). The mean duration of vitiligo was 19.71 months (range: 0–132 months). The most common type of vitiligo was vitiligo vulgaris, the frequency of which was 38.1%, followed by focal vitiligo (34.6%), segmental vitiligo (19.4%), acrofacial vitiligo (7.6%), and universal vitiligo (0.4%). Segmental vitiligo had an earlier the other types. Of the 541 children with vitiligo, 60 (11.1%) had a family history, and 3 (0.6%) had more than one family member who was affected. Forty‐one (7.6%) children had an associated autoimmune disease: halo nevi and alopecia areata, which were observed in 39 (7.2%) and 2 (0.4%) children, respectively.
Patch Testing of Singapore Children and Adolescents: Our Experience over 18 Years
Authors: Goon Anthony Teik‐Jin , Goh Chee‐Leok
Published: April 2006

Abstract:  This is a retrospective epidemiologic study of allergic contact dermatitis in children and adolescents in Singapore who had undergone patch testing from January 1, 1986 to December 31, 2003. A total of 2340 patients below the age of 21 years had undergone patch testing. Of these, 1063 (583 girls, 480 boys) were positive to one or more allergens. The most common allergens were: nickel (40%), thimerosal (15%), colophony (9%), lanolin (8%), cobalt (8%), fragrance mix (5%), and neomycin (4%). Each of these allergens will be discussed individually. This is the first study with over 1000 patients on this subject from Asia.
Congenital Syphilis
Authors: Lugo Alexander , Sanchez Samuel , Sanchez Jorge L.
Published: April 2006

Abstract:  Congenital syphilis is a rare and serious disease that, although preventable, continues to be a major healthcare problem. Its clinical spectrum ranges from an asymptomatic infection to fulminating sepsis or death. A diagnosis of congenital syphilis was made in an 8‐week‐old infant whose mother had adequate prenatal care. We present the clinical and histopathologic findings of this uncommon congenital disease.
Cutaneous Infection with Mycobacterium abscessus in a Child
Authors: Brantley Julie S. , Readinger Allison L. , Morris Elizabeth S.
Published: April 2006

Abstract:  Nontuberculous mycobacteria can cause cutaneous infections in both children and adults. Localized nontuberculous mycobacterial infections of the skin have been reported in children following injections or surgical procedures. We report a child who developed a cutaneous nontuberculous mycobacterial infection after exposure of a skin wound to pond water. Cutaneous infection with the rapidly growing mycobacteria Mycobacterium abscessus was demonstrated by clinical and histologic evaluation on the lower leg of an otherwise healthy 12‐year‐old Caucasian boy. We describe this as an instance of an unusual acquisition of cutaneous M. abscessus infection in a child.
Endemic Pemphigus Foliaceus in Venezuela: Report of Two Children
Authors: González Francisco , Sáenz Ana Maria , Cirocco Antonietta , Tacaronte Inés Maria , Fajardo Javier Enrique , Calebotta Adriana
Published: April 2006

Abstract:  Two native Yanomami children from the Venezuelan Amazonia with erythroderma were hospitalized on our service. Clinical, histologic, and immunofluorescence studies diagnosed endemic pemphigus foliaceous. Human leukocyte antigen class II showed DRB1*04 subtype *0411, which has not been previously associated with this disease. However, it shares a common epitope with all the human leukocyte antigen DRB1 alleles that have been involved in this disease among Brazilian populations. Although this condition is endemic in Brazil, our patients are the first two reported in Venezuela.
Holocarboxylase Synthetase Deficiency Presenting as Ichthyosis
Authors: Arbuckle H. Alan , Morelli Joseph
Published: April 2006

Abstract:  Holocarboxylase synthetase deficiency is a rare autosomal recessive disorder of biotin metabolism. Clinical manifestations usually present within the first few days of life and include severe acidosis, feeding difficulties, breathing abnormalities, vomiting, seizures, progressive loss of consciousness, coma, and death. Skin findings, when present, usually develop within the first weeks of life and are described as an erythroderma‐like dermatitis involving the eyebrows, eyelashes, and scalp. We were asked to consult on a newborn with a collodion membrane and severe metabolic acidosis who was eventually diagnosed with holocarboxylase synthetase deficiency and ichthyosis. The diagnosis of holocarboxylase synthetase deficiency might be considered in a newborn with collodion membrane, ichthyosis, and acidosis.
POEMS in Childhood
Authors: Marina Sonya , Broshtilova Valentina
Published: April 2006

Abstract:  A 17‐year‐old boy had a 3‐year history of diabetes mellitus, malabsorption syndrome, and skin changes consisting of induration, hyperpigmentation, and hypertrichosis on the anterior aspect of both thighs, lower abdomen, and scrotum. Physical examination found hypogonadism, hepatomegaly, gynecomastia, growth retardation, and ankle edema. There was no neuropathy or plasma cell dyscrasia. However, the characteristic skin changes and the combination of symptoms suggest polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes syndrome. This is a rare multisystemic disorder of obscure pathogenesis and no conspicuous heredity. Overproduction of vascular endothelial growth factor is thought to cause microangiopathy, neovascularization, and accelerated vasopermeability causing the multiorgan deterioration. Cyclophosphamid cytostatic therapy seems beneficial.
Localized Epidermolysis Bullosa Simplex with Generalized Enamel Hypoplasia in a Child
Authors: Çagirankaya Leyla Berna , Hatipoglu Müjgan Güngör , Hatipoglu Hasan
Published: April 2006

Abstract:  Epidermolysis bullosa is an uncommon disease characterized by the formation of blisters following minor trauma. The three major types are simplex, junctional, and dystrophic. Generalized enamel hypoplasia is accepted as a feature of the junctional type. Teeth with enamel hypoplasia are liable to destruction in the form of caries and/or gingivitis. In this report, a patient with epidermolysis bullosa simplex and severe enamel hypoplasia is described and the importance of early dental consultation for all children with this disease is stressed.
Growth, Bone Mineral Accretion, and Adrenal Function in Glucocorticoid‐Treated Infants with Hemangiomas – A Retrospective Study
Authors: Lomenick Jefferson P. , Backeljauw Philippe F. , Lucky Anne W.
Published: April 2006

Abstract:  Hemangiomas, common proliferative vascular tumors, can grow rapidly in the first months of life. Although therapy with high‐dose oral glucocorticoids is standard for lesions that threaten vital functions or are disfiguring, little is known about the endocrine consequences of this treatment. Using retrospective data, we examined growth velocity, changes in bone mineral density, and adrenal function in infants with hemangiomas treated with systemic glucocorticoids. Treatment consisted of oral prednisolone 2 to 4 mg/kg/day or dexamethasone 1 mg/kg/day. Mean growth velocity Z score on glucocorticoid therapy was −1.41 standard deviations in 13 patients. In four infants with adequate follow‐up, growth velocity increased to +1.90 standard deviations after glucocorticoid treatment (Δ growth velocity +3.31 standard deviations). Mean lumbar spine bone mineral density Z score was −2.46 standard deviations before glucocorticoid treatment and −1.08 standard deviations at the end of treatment in six infants. Adrenal function after glucocorticoid therapy was assessed by low‐dose adrenocorticotropic hormone stimulation test in 10 infants. Eight had a normal cortisol response, and one had a borderline response. One infant, who had been treated with dexamethasone, had an abnormal test result. In conclusion, systemic glucocorticoid treatment in infants with hemangiomas slowed linear growth, but “catch‐up” growth was observed after treatment ceased. Glucocorticoids did not affect bone mineralization adversely. Only 1 of 10 glucocorticoid‐treated infants had clear evidence of adrenal insufficiency after therapy was stopped.
Vitiligo‐Like Lesions and Diffuse Lightening of the Skin in a Pediatric Patient Treated with Imatinib Mesylate: A Noninvasive Colorimetric Assessment
Authors: Brazzelli Valeria , Roveda Elena , Prestinari Francesca , Barbagallo Tania , Bellani Eleonora , Trevisan Valentina , Rona Claudia , Locatelli Franco , Zecca Marco , Borroni Giovanni
Published: April 2006

Abstract:  Imatinib mesylate is a drug that has been recently approved for the treatment for chronic myeloid leukemia. It acts as a potent and selective inhibitor of BCR‐ABL tyrosine kinase. It also inhibits both c‐kit and platelet‐derived growth factor receptor tyrosine kinases. Hypopigmentation of the skin in patients receiving this drug has been recently reported. We report a 17‐year‐old Caucasian patient affected by chronic myeloid leukemia in therapy with imatinib mesylate who developed hypopigmented vitiligo‐like patches and generalized lightening of the skin. In order to evaluate the lightening observed clinically, we measured the progressive skin color hypopigmentation by using a colorimeter over several months. The colorimetric evaluation confirmed the generalized and gradual lightening of patient's skin over treatment with imatinib mesylate. We believe that this is the first reported instance of vitiligo‐like lesions in a pediatric patient treated with imatinib mesylate, and the second in a Caucasian patient.
Albendazole: Single or Combination Therapy with Permethrin against Pediculosis Capitis
Authors: Akisu Ciler , Delibas Songul Bayram , Aksoy Umit
Published: April 2006

Abstract:  Pediculosis capitis is a worldwide problem and a growing concern because of resistance to pediculicides. In the present study, we investigated whether albendazole could be used in the treatment of pediculosis capitis in combination with 1% permethrin or alone. A total of 150 children were randomly divided to five groups of 30 each. Group 1 got albendazole in a single dose (400 mg), group 2 got albendazole at 400 mg for 3 days, group 3 was given 1% permethrin, group 4 took 1% permethrin and albendazole in a single dose (400 mg), and group 5 got 1% permethrin and albendazole in a dose of 400 mg for 3 days. Groups given albendazole were also given another 400 mg dose of albendazole after 1 week. The success rate of treatment at the 2‐week follow‐up for all groups was 61.5%, 66.6%, 80.0%, 84.6%, and 82.1%, respectively. No statistically significant difference was found between the groups. The results of this study suggest that albendazole is effective against pediculosis capitis and there is no synergistic effect between albendazole and 1% permethrin.
Inhibition of the Multidrug Transporter P‐Glycoprotein Improves Seizure Control in Phenytoin‐treated Chronic Epileptic Rats
Authors: Van Vliet Erwin A. , Van Schaik Rosalinde , Edelbroek Peter M. , Redeker Sandra , Aronica Eleonora , Wadman Wytse J. , Marchi Nicola , Vezzani Annamaria , Gorter Jan A.
Published: April 2006

Summary:  Purpose: Overexpression of multidrug transporters such as P‐glycoprotein (P‐gp) may play a significant role in pharmacoresistance, by preventing antiepileptic drugs (AEDs) from reaching their targets in the brain. Until now, many studies have described increased P‐gp expression in epileptic tissue or have shown that several AEDs act as substrates for P‐gp. However, definitive proof showing the functional involvement of P‐gp in pharmacoresistance is still lacking. Here we tested whether P‐gp contributes to pharmacoresistance to phenytoin (PHT) by using a specific P‐gp inhibitor in a model of spontaneous seizures in rats.

Methods: The effects of PHT on spontaneous seizure activity were investigated in the electrical post–status epilepticus rat model for temporal lobe epilepsy, before and after administration of tariquidar (TQD), a selective inhibitor of P‐gp.

Results: A 7‐day treatment with therapeutic doses of PHT suppressed spontaneous seizure activity in rats, but only partially. However, an almost complete control of seizures by PHT (93 ± 7%) was obtained in all rats when PHT was coadministered with TQD. This specific P‐gp inhibitor was effective in improving the anticonvulsive action of PHT during the first 3–4 days of the treatment. Western blot analysis confirmed P‐gp upregulation in epileptic brains (140–200% of control levels), along with ∼20% reduced PHT brain levels. Inhibition of P‐gp by TQD significantly increased PHT brain levels in chronic epileptic rats.

Conclusions: These findings show that TQD significantly improves the anticonvulsive action of PHT, thus establishing a proof‐of‐concept that the administration of AEDs in combination with P‐gp inhibitors may be a promising therapeutic strategy in pharmacoresistant patients.
Imaging the Neural Substrates Involved in the Genesis of Pentylenetetrazol‐induced Seizures
Authors: Brevard Mathew E. , Kulkarni Praveen , King Jean A. , Ferris Craig F.
Published: April 2006

Summary:  Purpose: Functional imaging of animal models makes it possible to map the functional neuroanatomy contributing to the genesis of seizures. Pentylenetetrazol (PTZ)‐induced seizure in rats, a relevant model of human absence and of generalized tonic–clonic epilepsy, was used to stimulate seizure activity within 30 s of administration while collecting continuous, high‐resolution, multislice images at subsecond intervals.

Methods: Pilot studies were conducted to establish a quick and effective PTZ model for the imaging experiments. PTZ was then used to stimulate seizure activity in rats while collecting multislice functional MRI (fMRI) images from the entire forebrain at 4.7 Tesla. Ethosuximide (ESM) also was used to block seizure activity.

Results: Within 2–4 s of PTZ administration, a rapid increase in blood oxygen level–dependent (BOLD) signal intensity was noted in the thalamus, especially the anterior thalamic nuclei. Activity in the anterior thalamus peaked ∼15 s before seizure onset and was more than twofold greater than that in all other thalamic areas. The retrosplenial cortex showed a twofold greater increase in activity as compared with other cortical areas, also peaking at ∼15 s. The dentate gyrus was twice as active as other hippocampal areas but peaked just before seizure onset. Treatment with ESM blocked seizures, decreasing PTZ‐induced activation in most forebrain areas. The anterior thalamus and retrosplenial cortex were essentially blocked by pretreatment with ESM.

Conclusions: The anterior thalamus, retrosplenial cortex, and dentate gyrus show the greatest increases in BOLD signal activity before seizure onset. Neurons in these areas may contribute to the neural network controlling the initiation of generalized tonic–clonic seizure.
Nocturnal Hypermotor Seizures, Suggesting Frontal Lobe Epilepsy, Can Originate in the Insula
Authors: Ryvlin Philippe , Minotti Lorella , Demarquay Geneviève , Hirsch Edouard , Arzimanoglou Alexis , Hoffman Dominique , Guénot Marc , Picard Fabienne , Rheims Sylvain , Kahane Philippe
Published: April 2006

Summary:  Purpose: To report three patients with drug‐resistant nocturnal hypermotor seizures (NHSs), no detectable brain lesion, and clinically defined nocturnal frontal lobe epilepsy (NFLE) or autosomal dominant NLFE (ADNFLE), whose intracerebral EEG ictal onset primarily involved the insula, rather than the mesial or orbital frontal cortex.

Methods: Fourteen to 15 intracerebral electrodes were implanted in each patient, primarily sampling the frontal lobes with 80 to 91 recording leads covering the most likely side of seizure onset, and two to six leads placed within the ipsilateral insula. Electrical stimulation was used to test the epileptic threshold of frontal and insular brain regions at the various recording sites.

Results: In all three patients, a low‐voltage fast activity was recorded within the anterosuperior aspect of the insula at ictal onset, either in isolation, or extending to the nearby frontal operculum in the ADNFLE patient. The role of the insula was further supported in all three patients either by the presence of high‐amplitude spikes that clearly predominated over that region (n = 2) or by triggering the patient's typical aura or seizure when applying an electrical stimulation at that site, selectively (n = 2).

Conclusions: The anterosuperior portion of the insula might play a pivotal role in generating nocturnal hypermotor seizures in some patients with nonlesional drug‐resistant epilepsy suggesting NFLE or ADNFLE. Whether these patients are amenable to successful surgery remain an open issue.
Language‐induced Epilepsy, Acquired Stuttering, and Idiopathic Generalized Epilepsy: Phenotypic Study of One Family
Authors: Valenti Maria Paola , Rudolf Gabrielle , Carré Sophie , Vrielynck Pascal , Thibault Anne , Szepetowski Pierre , Hirsch Edouard
Published: April 2006

Summary: Purpose:Language‐induced epilepsy involves seizure precipitation by speaking, reading, and writing. Seizures are similar to those of reading epilepsy (RE). The nosologic position of language‐induced epilepsy is not clear. We performed a clinical and neurophysiological study in a multigenerational family with the association of idiopathic generalized epilepsy (IGE) with ictal stuttering as a manifestation of reflex language‐induced epilepsy.

Methods:Nine members on three generations were studied. All patients underwent video‐polygraphic EEG recordings (awake and during sleep). A standardized protocol was applied to test the effect of language and non–language‐related tasks.

Results:Six patients presented language‐induced jaw jerking that mimicked stuttering and corresponded to focal myoclonus involving facial muscles. This was associated with an IGE phenotype in four of these patients. Focal EEG spikes were found in all six patients by visual analysis and/or back‐averaging techniques. The focal spikes were either asymptomatic (when followed by a slow wave) or symptomatic of facial myoclonia (when isolated). Levetiracetam, used as add‐on or monotherapy in four patients, suppressed ictal stuttering. One additional case only had a phenotype of IGE without focal features.

Conclusions:This family study demonstrates the phenotypic heterogeneity of the association of IGE phenotype with ictal stuttering (language‐related reflex seizure). Our data suggest that this particular form of reflex epilepsy related to language has more similarities with generalized epilepsies than with focal ones. Neurophysiological investigations should be performed more systematically in patients with acquired stuttering, especially if there is family history of IGE.