bluuebunny/crossref_metadata_2025 huggingface dataset:
VOLUNTARY ANGLICANS Restoration, reformation, and reform, 1660–1828: archbishops of Canterbury and their diocese. By Jeremy Gregory. Oxford: Oxford University Press, 2000. Pp. 355. ISBN 0-19-820830-8. £45.00. The church in an age of danger: parsons and parishioners, 1660–1740. By Donald A. Spaeth. Cambridge: Cambridge University Press, 2000. Pp. 279. ISBN 0-521-35313-0. £40.00. The Quakers in English society, 1655–1725. By Adrian Davies. Oxford: Oxford University Press, 2000. Pp. 262. ISBN 0-19-8280820-0. £40.00. Hawksmoor's London churches: architecture and theology. By Pierre de la Ruffinière du Prey. Chicago: University of Chicago Press, 2000. Pp. 179. ISBN 0-226-17301-1. £26.50 (hb); 2003. ISBN 0-226-17303-8. £17.50 (pb). The national church in local perspective: the Church of England and the regions, 1660–1800. Edited by Jeremy Gregory and Jeffrey S. Chamberlain. Woodbridge, Suffolk: Boydell Press, 2003. Pp. 315. ISBN 0-85115-897-8. £50.00.
Authors: GOLDIE MARK
Published: December 2003

The historiography of the eighteenth-century Church of England remains peculiarly preoccupied with vindicating that institution from the condemnation heaped upon it by Anglo-Catholics and Evangelicals in the nineteenth century. The chapters of Jeremy Gregory's Restoration, reformation, and reform characteristically begin with quotations from Victorians on the somnolence and negligence of the Hanoverian Establishment. The starting point is, as it were, a Hogarth cartoon of a corpulent curate and a snoozing congregation. In part this preoccupation is indicative of how little has been done on the subject since the Victorians. Norman Sykes, writing between the 1930s and 1950s, remains an almost solitary beacon for the church's institutional history, though much of his work was biographical, dwelling on clerical high politics rather than on the social fabric of the church in the parishes. About the Hanoverian parish we know little, and probably care less, because without Reformation or Revolution – or nuns or witches – there is little to move the secular-minded to take an interest. It would not, of course, be true to say that nothing has recently been done. There has been something in the field of intellectual history. One thinks of Brian Young's fine Religion and enlightenment in eighteenth-century Britain (1998), a filling out of John Pocock's sketch of an English ‘clerical Enlightenment’ – though most intellectual history of that era prefers the wilder shores of deism, freethinking, and the radical assault on priestcraft. There have been valuable probings of the early eighteenth-century politics of religion (the Sacheverell affair, the charity school movement, the Societies for the Reformation of Manners) and of the late Hanoverian roots of nineteenth-century high churchmanship.
DIPLOMATIC HISTORY SINCE THE CULTURAL TURN Pomp und Politik: Monarchenbegegnungen in Europa zwischen Ancien Régime und Erstem Weltkrieg. By Johannes Paulmann. Paderborn: Schöningh Verlag, 2000. Pp. 478. ISBN 3-506-77160-4. €44.99. Royalty and diplomacy in Europe, 1890–1914. By Roderick R. McLean. Cambridge: University Press, 2001. Pp. xi+239. ISBN 0-8223-1522-X. £35.00. British envoys to Germany, 1816–1866, I: 1816–1829. Camden Fifth Series, Volume 15. Edited by Sabine Freitag and Peter Wende. For the Royal Historical Society in Association with the German Historical Institute London. Pp. xxi+592, Cambridge: Cambridge University Press, 2000. ISBN 0-5217-90662. £40.00. Does peace lead to war? Peace settlements and conflict in the modern age. By Matthew Hughes and Matthew Seligmann. Phoenix Mill: Sutton Publishing, 2002. Pp. xiii+242. ISBN 0-7509-2514-0. £20.00.
Authors: URBACH KARINA
Published: December 2003

Since the 1960s it has been claimed that diplomatic history is in decline – upstaged over the decades by more fashionable subjects like social, gender, or cultural history. However, Clio's anaemic patient is alive and kicking as the four books to be reviewed here all clearly show. This renaissance is in some ways due to a modernization process which proves the inherent flexibility of the subject. Diplomatic historians have adopted new methods for their work by amalgamating cultural, semiotic, and anthropological ideas as well as by going global through multiarchival research. Admittedly these progressive developments are still at an early stage, yet Johannes Paulmann's thought-provoking Pomp und Politik which analyses the subtext of ceremonies and symbolic behaviour among monarchs, could be used as an excellent guideline for further studies in this field.
BUYING AND BECOMING: NEW WORK ON THE BRITISH MIDDLE CLASSES Gender, civic culture and consumerism: middle-class identity in Britain, 1800–1940. Edited by Alan Kidd and David Nicholls. Manchester: Manchester University Press, 1999. Pp. xi+223. ISBN 0-7190-5675-4. £14.99. The Victorian parlour: a cultural study. By Thad Logan. Cambridge: Cambridge University Press, 2001. Pp. xvii+282. ISBN 0-521-631182-3. £40.00. Shopping for pleasure: women in the making of London's West End. By Erika D. Rappaport. Bognor Regis, UK: J. Wiley for Princeton UP, 2001. Pp. xiii+323. ISBN 0-691-04476-7. £13.95.
Authors: COHEN DEBORAH
Published: December 2003

The history of consumption in nineteenth-century Britain has largely been told as the story of the middle classes. Increasingly, as the three books under review demonstrate, the reverse is also true. The middle classes, they argue, derived their identities in significant measure from their consumerist habits rather than their relation to the means of production or the body politic. That was, as one might imagine, especially the case with women. What they bought, how they shopped, where they lived: these things came to define who a person was. How dramatically the literature on the Victorian middle classes has changed is apparent even from this brief description. Most obviously, the research focus has shifted from structures to identities. The old subjects – the professions, the movements (free trade, the franchise, anti-slavery), evangelical religion – are hardly anywhere to be seen in these volumes. Instead, they take up topics more often associated with French history: urban culture, shopping, interior decoration. With this shift in subjects has also come, either implicitly or explicitly, a sense that the defining characteristics of the British middle classes must be sought in the realm of culture, not politics or production.
Black entrepreneurs, local embeddedness and regional economic development in Northern Namibia
Authors: Knutsen Hege M.
Published: December 2003

The article addresses possibilities and barriers to economic activity and development in the Oshana region of Northern Namibia. The focus is on the role of local embeddedness of economic activities in attaining economic development. A network perspective, based on theories of value chains that are embedded both in social relations and spatially, is selected as the analytical framework. The value chains of local black entrepreneurs in the study area are short. Moreover, the analysis reveals that social obligations may impede economic development, but that such practices are diminishing. The economic dominance and competition from South Africa is the main impediment to economic development in Northern Namibia. Local political embeddedness is shallow and political measures have not significantly reduced the implications of this dominance.
Income inequality, economic growth, and political instability in sub-Saharan Africa
Authors: Nel Philip
Published: December 2003

There is much disagreement about the effect that income distribution has on economic growth. This study uses high-quality household-expenditure-based data to estimate this effect for a sample of sub-Saharan African states. It uses an ordinary least squares (OLS) technique to estimate the effects of income inequality in a standard reduced-form growth regression for a set of cross-section data for the period 1986–97. It finds that higher levels of inequality seem to impinge negatively on growth prospects over the medium term, but that this effect is weak and that the finding of a negative relationship is not robust. It then tests whether this negative consequence of inequality for growth is attributable to inequality's effect on political instability, as the literature suggests. The evidence indicates that high levels of inequality do not affect political instability in any statistically significant manner for the countries in the sample, but that they do negatively affect the risk perceptions of potential investors, and so may contribute to lower growth prospects.
Utility privatisation in Sub-Saharan Africa: a case study of water
Authors: Bayliss Kate
Published: December 2003

Over the past twenty years, the focus of development policy has shifted from the state to the private sector. Privatisation is now central to utility reform in much of SSA. This paper sets out developments in water privatisation and reviews the evidence regarding its impact. Water privatisation has been carried out to some degree in at least fourteen countries in the region, and many other governments are at various stages in the privatisation process. However, in some cases privatisation has been difficult to achieve, and a few countries have successfully provided water under public ownership. Evidence on the impact of privatisation indicates that the performance of privatised utilities has not changed dramatically, but that enterprises have continued to perform well, or not so well, depending both on their state when they were privatised and on the wider economic context. The evidence points to internal improvements in terms of financial management. However, governments face considerable difficulties in attracting investors and regulating private utilities. Furthermore, privatisation fails to address some of the fundamental constraints affecting water utilities in SSA, such as finance, the politicised nature of service delivery, and lack of access for the poor. A preoccupation with ownership may obscure the wider goals of reform.
The tragedy of the cocoa pod: rent-seeking, land and ethnic conflict in Ivory Coast
Authors: Woods Dwayne
Published: December 2003

Many people have been surprised by the eruption of ethnic conflict and civil war in Ivory Coast. The country had gained a reputation as a relatively stable and economically prosperous agricultural republic in a region known for ethnic conflict, economic decline and civil war. The underlying factors that have led to the ethnic violence, the flight of immigrants from neighbouring countries, and the division of the country into a predominantly Muslim north and largely Christian south have been known for some time. The country's property rights regime that encouraged easy access to a forest rent – as long as cheap migrant labour and virgin forested land were available – was a recipe for future conflict. As available land declined and labour costs increased, a cycle of sharpening conflicts over these assets contributed to the current situation of ethno-regional division and civil war.
Conflict, Justice, and the Stranger-King Indigenous Roots of Colonial Rule in Indonesia and
Elsewhere
Authors: Henley David
Published: December 2003

Historians of Indonesia often think of states, and especially colonial states, as predatory institutions encroaching aggressively on the territory and autonomy of freedom-loving stateless peoples. For Barbara and Leonard Andaya, early European expansion in Sumatra and the Moluccas was synonymous with the distortion or destruction of decentralized indigenous political systems based on cooperation, alliance, economic complementarity, and myths of common ancestry (B. W. Andaya 1993; L. Y. Andaya 1993). Anthony Reid (1997: 81) has described tribal societies like those of the Batak and Minangkabau in highland Sumatra as ‘miracles of statelessness’ which ‘defended their autonomy by a mixture of guerilla warfare, diplomatic flexibility, and deliberate exaggeration of myths about their savagery’ until ultimately overwhelmed by Dutch military power. Before colonialism, in this view, most Indonesians relied for security not on the protection of a powerful king, but on a ‘complex web of contractual mutualities’ embodying a ‘robust pluralism’ (Reid 1998: 29, 32). ‘So persistently’, concludes Reid (1997: 80-1), ‘has each step towards stronger states in the archipelago arisen from trading ports, with external aid and inspiration, that one is inclined to seek the indigenous political dynamic in a genius for managing without states’. Henk Schulte Nordholt (2002: 54), for his part, cautions against any tendency to downplay the violent, repressive aspects of colonial and post-colonial government in Indonesia, expressing the hope that ‘a new Indonesian historiography will succeed in liberating itself from the interests, perspective, and conceptual framework of the state’. An even more systematic attempt to demonize the (modern) state in Indonesia and elsewhere can be found in the work of James Scott (1998a, 1998b).
Theoretical Approaches to Sri Lankan History and the Early
Portuguese Period
Authors: Strathern Alan
Published: December 2003

In the past twenty years or so the history of Sri Lanka has become a site of vibrant controversy, largely because the current ethnic conflict has loaded any kind of reflection on the historical boundaries of political, ethnic or religious identity with an immediate emotional charge. The intellectual reverberations of post-colonialism and the vigorous contributions of anthropologists have added rich strata of theoretical thinking. However, despite one or two calls to the contrary, the periods of Portuguese (1505-1658) and Dutch influence (1658-1796) in the island have tended to moulder on the periphery of these debates. The purpose of this article is to bring some of this thinking to bear on the evidence from the sixteenth century in order to stimulate fresh perspectives on both the events of that time and the models themselves. With the arrival of the Portuguese and their increasing involvement in the affairs of the island during the long reign of Bhuvanekabahu VII (1521-51), the darkness of the Kotte period is suddenly illuminated by wonderfully detailed flashes of events. The flurry of letters written by contemporary Portuguese settlers, officials and missionaries, and the attentions of Portuguese chroniclers such as João de Barros, Diogo do Couto, Gaspar Correia and Fernão de Queirós bring quite new forms of evidence into the historian's purview.
The seroepidemiology and transmission dynamics of varicella in Australia
Authors: GIDDING H. F. , MACINTYRE C. R. , BURGESS M. A. , GILBERT G. L.
Published: December 2003

To enhance our understanding of the epidemiology and transmission dynamics of varicella in the pre-vaccine era we performed a serosurvey using opportunistically collected sera submitted to diagnostic laboratories across Australia during 1997–1999. A representative sample by state and sex of 2027 sera from persons aged 1–49 years was tested using an enzyme immunoassay method. The average age of infection and age-specific forces of infection (the probability that a susceptible individual acquires infection) were calculated using published methodologies. Seropositivity increased with age, with 83% of sera positive by ages 10–14 years. The highest force of infection was in the 5–9 years age group (0·195 per susceptible year) followed by the 0–4 years age group (0·139 per susceptible year) and the average age of infection was 8·15 years. These results provide valuable baseline information to measure the impact of vaccination and indicate that vaccination should be aimed at children less than 5 years of age, although further modelling using the serosurvey data is warranted.
Human herpesvirus-8 antibodies and DNA in HIV-1 infected patients in South Africa
Authors: ALAGIOZOGLOU L. , MORRIS L. , BREDELL H. , MARTIN D. J. , SITAS F.
Published: December 2003

HIV-infected individuals with high levels of IgG antibodies against human herpesvirus-8 (HHV-8) are at increased risk of developing Kaposi's sarcoma. The aim of this study was to measure the association between HHV-8 viraemia and IgG antibody responses (by immunofluorescence) in a group of 201 HIV-infected individuals attending outpatient clinics, 91 in-patients with AIDS and 87 HIV-infected patients admitted with Kaposi's sarcoma. Compared to HIV-infected outpatients, the adjusted odds ratio in relation to Kaposi's sarcoma was 15·4 (95% CI 4·4–54·2) in those with viraemia, 25·1 (95% CI 6·6–95·6) in those with a positive immunofluorescent signal and ∞ (lower exact CI 33·6) in those with a high immunofluorescent signal (all P trend <0·001). Among those without HHV-8 viraemia, 23% were IgG-positive, but only 5·5% had a high immunofluorescent signal. In those who were viraemic, 89·1% were IgG-positive, and 28·2% had a high immunofluorescent signal, suggesting viraemia is associated with high HHV-8 immunofluorescence IgG signal.
Investigation of an outbreak of Escherichia coli O157 infection caused by environmental exposure at a scout camp
Authors: HOWIE H. , MUKERJEE A. , COWDEN J. , LEITH J. , REID T.
Published: December 2003

In May 2000 a scout camp was held on an agricultural showground in New Deer, Aberdeenshire. There were 337 campers at the event, comprising 233 cubs, scouts, and venture scouts, and 104 adults. The event was abandoned early because of heavy rainfall. Twenty campers who became ill between 28 May and 3 June were confirmed as having E. coli O157 infection. Preliminary investigation did not suggest a food vehicle but did indicate environmental exposure at the camp as a risk factor. Subsequent investigations supported the hypothesis that transmission of E. coli O157 was from the environment to cases by contaminated hands, either directly from hand to mouth, or via food. As a result of the investigation the Aberdeenshire Council and the Scout Association jointly prepared interim guidelines to reduce the risk of E. coli O157 infection at scout camps.
Natural history of chronic viral hepatitis in southern Italy: epidemiological changes since the introduction of the anti-HCV test
Authors: PARRILLI G. , ORSINI L. , STRANGES S. , CIMINO L. , ABAZIA C. , SCALICE E. , MANGUSO F. , FARINARO E. , BUDILLON G.
Published: December 2003

We investigated whether there are differences between the natural history of B and C chronic hepatitis in a southern Italian population, and whether the chronic viral hepatitis population was modified by the introduction of the anti-HCV test in 1989. We examined clinical charts of 1120 patients consecutively admitted to our division from January 1979 to December 1998 with the histological diagnosis of chronic viral hepatitis (304 from 1979 to 1988; 816 from 1989 to 1998). We found significant differences only in age at diagnosis (higher in the second decade, P=0·001), and in aetiology (HBV decreased in the second decade, P<0·0001). We were able to follow up 449 patients for 2–20 years (311 with HCV and 138 with HBV infection), and found that chronic HCV evolved to cirrhosis more frequently than did chronic HBV; but in both types time to development of cirrhosis and the incidence of death were similar. Our data confirm that a higher onset age of HBV and of HCV is frequently observed in those subjects who have a faster disease progression.
Antibiotic resistance of Campylobacter in raw retail chickens and imported chicken portions
Authors: WILSON I. G.
Published: December 2003

Campylobacter isolates from raw retail chickens (n=434) sampled between 1998 and 2000 were tested for resistance to 12 antibiotics. Among 208 campylobacters tested, more than 90% of isolates were susceptible to 4 out of 9 antibiotics (nalidixic acid, erythromycin, chloramphenicol and gentamicin). Most campylobacters were resistant to 3 antibiotics and multiple resistance was found in 4%. Ciprofloxacin resistance was 11%. Campylobacter contamination (28%) in imported chickens (n=150) was almost half that found in local whole chickens (50%), but the resistance of imported isolates (n=42) was similar to that of local campylobacters. Resistance in isolates from imported chicken breasts was generally more common, but to only 4 antibiotics. Resistance patterns of chicken isolates were compared to human clinical isolates (n=494), and a greater similarity was found between the clinical and local isolates than with imported campylobacters. Lower chloramphenicol resistance was found in clinical Campylobacter isolates than in those from chicken sources.
Tuberculosis is still a major cause of cervical lymphadenopathies in adults from developing countries
Authors: HERNÁNDEZ-SOLÍS A. , CICERO-SABIDO R. , OLIVERA H. , RIVERO V. , RAMÍREZ E. , ESCOBAR-GUTIÉRREZ A.
Published: December 2003

To establish the frequency of infectious aetiology in Mexican adult patients with cervical lymphadenopathies (CLAs), 87 consecutive patients with enlarged cervical lymphatic nodes, HIV negative and without anti-tuberculous treatment, were selected from a tertiary-level speciality concentration hospital. Histopathological studies, investigation of acid-fast bacilli, cultures in Löwenstein–Jensen and Mycobacterium growth indicator tube (MGIT) media, and in-house polymerase chain reaction (PCR) with IS6110-based primers for Mycobacterium tuberculosis complex were performed in resected lymphatic nodes. Non-infectious aetiology corresponded to 45 cases (52%). Tuberculosis was suspected in 42 cases (48%) by histology and confirmed positive results were obtained by staining in 8 (19%), by culture in 23 (55%), and by PCR in 34 (81%) patients. All were confirmed after therapeutic success. In addition to the epidemiological transition process occurring in Mexico, tuberculosis remains an important cause of CLA. Histopathology with confirmatory studies including PCR can detect tuberculous aetiology.
Serological responses to Cryptosporidium antigens among users of surface- vs. ground-water sources
Authors: FROST F. J. , KUNDE T. R. , MULLER T. B. , CRAUN G. F. , KATZ L. M. , HIBBARD A. J. , CALDERON R. L.
Published: December 2003

Cryptosporidium oocysts are commonly detected in surface-derived drinking water. However, the public health significance of these findings is unclear. This study compared serological responses to two Cryptosporidium antigen groups for blood donors and college students using chlorinated and filtered river water vs. ground-water sources. The surface water received agricultural and domestic sewage discharges upstream. Participants from the surface-water city had a higher relative prevalence (RP) of a serological response to the 15/17-kDa antigen group (72·3 vs. 52·4%, RP=1·36, P<0·001) and to the 27-kDa antigen group (82·6 vs. 72·5%, RP=1·14, P<0·02). Multivariate logistic regression analysis found that the people with a shorter duration of residence or drinking bottled water also had a lower seropositivity for each marker. Use of private wells was associated with a higher prevalence of response to the 15/17-kDa markers. Seroconversion to the 15/17-kDa antigen group was more common in the residents of the city using surface water. These findings are consistent with an increased risk of Cryptosporidium infection for users of surface-derived drinking water compared with users of municipal ground-water-derived drinking water. Users of private well water may also have an increased risk of infections.
The relationship between HIV seroconversion illness, HIV test interval and time to AIDS in a seroconverter cohort
Authors: TYRER F. , WALKER A. S. , GILLETT J. , PORTER K.
Published: December 2003

Seroconversion illness is known to be associated with more rapid HIV disease progression. However, symptoms are often subjective and prone to recall bias. We describe symptoms reported as seroconversion illness and examine the relationship between illness, HIV test interval (time between antibody-negative and anibody-positive test dates) and the effect of both on time to AIDS from seroconversion. We used a Cox model, adjusting for age, sex, exposure group and year of estimated seroconversion. Of 1820 individuals, information on seroconversion illness was available for 1244 of whom 423 (34%) reported symptomatic seroconversion. Persons with a short test interval ([les ]2 months) were significantly more likely to report an illness than people with a longer interval (OR 6·76, 95% CI 4·75–9·62). Time to AIDS was significantly faster (P=0·01) in those with a short test interval. The HIV test interval is a useful replacement for information on seroconversion illness in studies of HIV disease progression.
Diagrammatic specifications
Authors: DUVAL DOMINIQUE
Published: December 2003

This paper presents a simple and powerful diagrammatic framework for dealing with specifications in computer science. Following a classical line, we define diagrammatic specifications as a kind of generalised sketch. In addition, the specifications themselves are defined as the realisations of projective sketches. This meta level provides adjunction properties: this is due to a well-known result of Ehresmann. Moreover, we prove in this paper that this meta level also provides an efficient definition of deduction. This work results from a collaboration with Christian Lair.
Hearing loss in Fabry disease: The effect of agalsidase alfa replacement therapy
Authors: Hajioff D. , Enever Y. , Quiney R. , Zuckerman J. , Mackermot K. , Mehta A.
Published: December 2003

Summary: The aim of this study was to describe the nature and prevalence of hearing loss in Fabry disease (McKusick 301500), a rare X‐linked lysosomal storage disorder, and its response to enzyme replacement therapy with agalsidase alfa. Fifteen hemizygous male Fabry patients (aged 25–49 years) were randomized to receive placebo or enzyme replacement therapy for 6 months; all have received open‐label enzyme replacement therapy for an additional 24 months thus far. Pure‐tone audiometry, impedance audiometry and otoacoustic emission testing were performed at 0 (baseline), 6, 18 and 30 months. Four patients (27%) had bilateral and 7 (47%) had unilateral high‐frequency sensorineural hearing loss (SNHL). Two (13%) had unilateral middle ear effusions with conductive losses persisting beyond 6 months. Only 3 (20%) had normal hearing. High‐frequency SNHL deteriorated over the first 6 months in both placebo and active treatment groups by a median 4.3 dB (p=0.002, Wilcoxon matched pairs). This hearing loss subsequently improved above baseline by 2.1 dB at 18 months (p=0.02) and by 4.9 dB at 30 months (p=0.004). In conclusion, significant hearing loss, usually high‐frequency SNHL, is a common manifestation of Fabry disease in adults. α‐Galactosidase A replacement therapy with agalsidase alfa appears to reverse the hearing deterioration in these patients. This improvement is gradual, however, suggesting the need for long‐term enzyme replacement therapy.
Markedly elevated serum biotinidase activity may indicate glycogen storage disease type Ia
Authors: Wolf B. , Freehauf C. L. , Thomas J. A. , Gordon P. L. , Greene C. L. , Ward J. C.
Published: December 2003

Summary: We report two children who presented with symptoms suggestive of biotinidase deficiency. Rather than deficiency, markedly elevated serum biotinidase activities were found. Based upon literature reports of elevated biotinidase activities in children with glycogen storage disease (GSD) type Ia, we considered the latter in our differential diagnosis and subsequently confirmed GSD type Ia in both patients by enzymatic testing. GSD type Ia should be considered in children with markedly elevated serum biotinidase activity.
Niemann–Pick disease: Sixteen‐year follow‐up of allogeneic bone marrow transplantation in a type B variant
Authors: Victor S. , Coulter J. B. S. , Besley G. T. N. , Ellis I. , Desnick R. J. , Schuchman E. H. , Vellodi A.
Published: December 2003

Summary: Allogenic bone marrow transplantation (BMT) was carried out on a 3‐year‐old white caucasian girl with Niemann–Pick disease (NPD) type B. The donor was her unaffected brother. The patient was neurologically normal at the time of transplantation. Engraftment was based on cytogenetic studies and increased leukocyte acid sphingomyelinase (ASM) activity. However, liver biopsies taken up to 33 months post transplantation showed only a moderate reduction in stored sphingomyelin and no significant increase in ASM activity. The post‐transplantation period was complicated by severe graft‐versus‐host disease and a respiratory arrest. By 6 years of age, neurological involvement was observed, including bilateral cherry red spots. The proband is now severely mentally and physically disabled. Liver cirrhosis has continued to progress despite the BMT, and haematemesis due to portal hypertension occurred at 17 years of age. However, pulmonary infiltration regressed after BMT and there has been no clinical evidence of pulmonary insufficiency.
Analysis of organic acids after incubation with (16‐2H3)palmitic acid in fibroblasts from patients with mitochondrial β‐oxidation defects
Authors: Osorio J. H. , Lluch M. , Ribes A.
Published: December 2003

Summary: The analysis of acylcarnitines as products of incubation of intact fibroblasts with isotope‐labelled precursors, usually (16‐2H3)hexadecanoic acid, is an advanced in vitro method for the study of mitochondrial β‐oxidation defects. We propose a technique for the measurement of the organic acid intermediates after hydrolysis of the acylcarnitines using electron‐impact gas chromatography–mass spectrometry. For some mitochondrial β‐oxidation deficiencies, the characteristic profile enables us to approach the diagnosis with clear differentiation.
Glycine N‐methyltransferase deficiency: A new patient with a novel mutation
Authors: Augoustides‐Savvopoulou P. , Luka Z. , Karyda S. , Stabler S. P. , Allen R. H. , Patsiaoura K. , Wagner C. , Mudd S. H.
Published: December 2003

Summary: We report studies of a Greek boy of gypsy origin that show that he has severe deficiency of glycine N‐methyltransferase (GNMT) activity due to apparent homozygosity for a novel mutation in the gene encoding this enzyme that changes asparagine‐140 to serine. At age 2 years he was found to have mildly elevated serum liver transaminases that have persisted to his present age of 5 years. At age 4 years, hypermethioninaemia was discovered. Plasma methionine concentrations have ranged from 508 to 1049 µmol/L. Several known causes of hypermethioninaemia were ruled out by studies of plasma metabolites: tyrosinaemia type I by a normal plasma tyrosine and urine succinylacetone; cystathionine β‐synthase deficiency by total homocysteine of 9.4–12.1 µmol/L; methionine adenosyltransferase I/III deficiency by S‐adenosylmethionine (AdoMet) levels elevated to 1643–2222 nmol/L; and S‐adenosylhomocysteine (AdoHcy) hydrolase deficiency by normal AdoHcy levels. A normal plasma N‐methylglycine concentration in spite of elevated AdoMet strongly suggested GNMT deficiency. Molecular genetic studies identified a missense mutation in the coding region of the boy's GNMT gene, which, upon expression, retained only barely detectable catalytic activity. The mild hepatitis‐like manifestations in this boy are similar to those in the only two previously reported children with GNMT deficiency, strengthening the likelihood of a causative association. Although his deficiency of GNMT activity may well be more extreme, his metabolic abnormalities are not strikingly greater. Also discussed is the metabolic role of GNMT; several additional metabolite abnormalities found in these patients; and remaining questions about human GNMT deficiency, such as the long‐term prognosis, whether other individuals with this defect are currently going undetected, and means to search for such persons.
Identification of a common mutation (Gly194Cys) in both Arab Moslem and Ashkenazi Jewish patients with dihydrolipoamide dehydrogenase (E3) deficiency: Possible beneficial effect of vitamin therapy
Authors: Hong Y. S. , Korman S. H. , Lee J. , Ghoshal P. , Wu Q. , Barash V. , Kang S. , Oh S. , Kwon M. , Gutman A. , Rachmel A. , Patel M. S.
Published: December 2003

Summary: Dihydrolipoamide dehydrogenase (E3) deficiency with a clinical phenotype and genotype (Gly194Cys homozygous), previously identified only in Ashkenazi Jewish patients, was diagnosed in two Palestinian Arab siblings and two unrelated Ashkenazi Jewish patients. While three of the four patients died in childhood without specific treatment, the surviving patient at age 18 years may have benefited from long‐term daily supplementation with a cocktail of riboflavin, biotin, coenzyme Q and carnitine.
Gravity wave packet effects on chemical exothermic heating in the mesopause region
Authors: Hickey Michael P. , Huang Tai‐Yin , Walterscheid Richard
Published: December 2003

Numerical simulations of the interaction of transient, dissipating gravity wave packets with the minor species involved in the OH airglow chemistry in the mesopause region reveal large wave‐driven downward fluxes of the minor species. In addition to the minor species fluctuating in response to the wave, a strong secular variation of minor species densities occurs. At 90 km altitude this secular variation becomes significant ∼80 min after the wave packet onset, and increases approximately linearly with increasing time for the following 2 hours. At later times the secular variation diminishes. Heating rates due to exothermic chemical reactions consequently exhibit a fluctuating component as well as a secular variation. When averaged over time, the net effect of each of the two waves considered is to cause large (∼22% and 37%) increases in average heating rates. This secular variation in heating rates could be more significant than the present results suggest because larger amplitude gravity waves can exist in the mesopause region.
Newly imaged shape of the deep seismic zone within the subducting Pacific plate beneath the Hokkaido corner, Japan‐Kurile arc‐arc junction
Authors: Katsumata Kei , Wada Naoto , Kasahara Minoru
Published: December 2003

The geometry of the deep seismic zone associated with the subducting Pacific plate beneath the North American plate in the Hokkaido corner, Japan‐Kurile arc‐arc junction has been investigated based on the hypocenters accurately relocated by a dense local seismographic network with three‐dimensional P and S wave velocity structures. The model suggests that the lateral changes in the dip of the subduction zone are more rapid than previously believed. We found that the deep seismic zone in the Pacific plate dips at 20° to 30° on the side of the Japan arc, the dip angle increased in a narrow region around 143°–144°E by 10° to 20°, and the dip was 40° to 50° on the side of the Kurile arc. In the transitional zone around 143°–144°E an unusual distribution of microearthquakes has been discovered within the Pacific plate, which was located on a near‐vertical plane with an area of 50 × 100 km2 with strike that was perpendicular to the Kurile Trench axis. This feature was named the Tokachi‐Oki slab‐cracking zone. We suggest that this is a slab‐cracking zone torn by the extensional stress due to the rapid lateral changes in the dip of the subduction zone. The slab‐cracking zone may possibly grow into the large disruption or tear of the slab in the future.
Modeling long‐term ground deformation due to the cooling of a magma chamber: Case of Basiluzzo island, Aeolian Islands, Italy
Authors: Tallarico A. , Dragoni M. , Anzidei M. , Esposito A.
Published: December 2003

We estimate the long‐term vertical deformation of Basiluzzo island, located in the volcanic arc of the Aeolian Islands, Italy, by inferring the subsidence of a Roman age submerged wharf dated 2000 ± 50 years ago. We model the crustal deformation that produced this displacement as a cooling magma chamber emplaced during the formation of the island. This is the first attempt to model crustal deformation using the subsidence of a submerged archaeological structure. Nowadays the top of the wharf, which can be considered an unconventional leveling benchmark, is located near Punta Levante, at an average depth of 3.20 ± 0.10 m below actual sea level, and it is still in good conservation. Its present location is due to combined effect of sea level rise and volcanic and tectonic activity that occurred since it was built. Taking into account the architectural features of the wharf and that the mean sea level rise of the Mediterranean sea has been estimated as ∼0.45 m during the last 2000 years [Flemming and Webb, 1986], we estimate a total subsidence of 3.75 ± 0.10 m, at a rate of about 1.87 mm yr−1 [Anzidei et al., 2002]. We propose a possible mechanism for the long‐term subsidence, which occurred on a timescale of thousands of years, by considering the Earth's crust as a Maxwell body. We assume that the magmatic source is located directly beneath Basiluzzo dome and underwent progressive solidification and subsequent volume reduction since its emplacement 50,000 years ago.
Carving and adaptive drainage enforcement of grid digital elevation models
Authors: Soille Pierre , Vogt Jürgen , Colombo Roberto
Published: December 2003

An effective and widely used method for removing spurious pits in digital elevation models consists of filling them until they overflow. However, this method sometimes creates large flat regions which in turn pose a problem for the determination of accurate flow directions. In this study, we propose to suppress each pit by creating a descending path from it to the nearest point having a lower elevation value. This is achieved by carving, i.e., lowering, the terrain elevations along the detected path. Carving paths are identified through a flooding simulation starting from the river outlets. The proposed approach allows for adaptive drainage enforcement whereby river networks coming from other data sources are imposed to the digital elevation model only in places where the automatic river network extraction deviates substantially from the known networks. An improvement to methods for routing flow over flat regions is also introduced. Detailed results are presented over test areas of the Danube basin.
Regional variability of ice core dust composition and provenance in Greenland
Authors: Bory A. J.‐M. , Biscaye P. E. , Piotrowski A. M. , Steffensen J. P.
Published: December 2003

Mineralogical and isotopic composition (Sr and Nd) of six dust samples, obtained from six widely spread ice‐coring sites in Greenland, were analyzed in order to investigate the regional geographic variability of dust provenance. We show that long‐range transport from eastern Asian deserts provides mineral dust with essentially the same composition to all elevated interior sites (Dye 3, Site A, GRIP, and NorthGRIP), while most material deposited at sites located closer to the edge and at lower altitude (Hans Tausen and Renland) derives from proximal source regions. No contribution from other sources is apparent at any of the interior sites from the mineralogical and isotopic composition of the dust samples, each of which represents several decades of dust deposition during the 17th–18th century. These results provide additional evidence that African and North American deserts do not play a significant role in the dust deposited over Greenland, which has implications for ice core record interpretation and atmospheric dust transport model validation.
Synchronous bidecadal periodic changes of oxygen, phosphate and temperature between the Japan Sea deep water and the North Pacific intermediate water
Authors: Watanabe Yutaka W. , Wakita Masahide , Maeda Nobuhiro , Ono Tsuneo , Gamo Toshitaka
Published: December 2003

From time series of dissolved oxygen (O2), phosphate (PO4) and water temperature (T) during the last forty years in the Japan Sea Deep Water and the North Pacific Intermediate Water, we found that O2, PO4 and T show a clear bidecadal oscillations of about 18 years superimposed on the linear trends of decrease of 0.47 μmol‐O2 kg−1 yr−1 and increase of 0.003 μmol‐PO4 kg−1 yr−1 and 0.005°C yr−1 in both two oceanic areas. The changes of O2, PO4 and T in the two oceanic areas were synchronized despite the water formation systems of each area being independent. Both the linear trends and the oscillations of O2, PO4 and T in the two oceanic areas also showed a strong correlation with the anomaly of the sea surface level pressure in the North Pacific that possibly affects the change of surface oceanic condition.
The relationship between the summer precipitation in the Yangtze River valley and the boreal spring Southern Hemisphere annular mode
Authors: Nan Sulan , Li Jianping
Published: December 2003

The relationship between the boreal spring (April–May) Southern Hemisphere annular mode (SAM) and the following summer (June–August) precipitation in China for the period of 1951–2001 is examined statistically in this study. There is a significantly positive correlation between the boreal spring SAM index (SAMI) and the following summer rainfall in the middle and lower reaches of the Yangtze River. The summer large‐scale atmospheric circulation anomalies over East Asia are also related to the boreal spring SAMI events. A strong SAM in boreal spring is followed by a weakened East Asian summer monsoon, a strengthened and westward expanded western Pacific subtropical high (WPSH), as well as increased ascending vertical velocity, specific humidity and water vapor flux convergence. These situations provide necessary circulation and water vapor conditions for increasing the summer precipitation in the middle and lower reaches of the Yangtze River valley, and vice versa. The boreal spring SAM variation provides a potential valuable signal for predicting the summertime precipitation in the middle and lower reaches of the Yangtze River valley.
Interannual variations of tropical cyclone size over the western North Pacific
Authors: Chan Johnny C. L. , Yip Cleo K. M.
Published: December 2003

This paper presents the results of an investigation on the interannual variations of tropical cyclone (TC) size, defined as the average radius of 15 m s−1 surface winds, over the western North Pacific from 1999 to 2002. The wind data are from QuikSCAT. The average TC size is found to increase from 1999 to 2002, with the TC sizes in 1999 and 2000 being significantly smaller than those in both 2001 and 2002. Differences in the environmental flow patterns associated with the tracks and formation positions of the TCs among these years apparently explain such size changes. Since 1999 and 2000 were La Niña years, TCs in these two years formed more westward with fewer recurvers so that they tended to be smaller. On the other hand, with 2002 being an El Niño year, TCs formed further eastward with more recurvers, and thus tended to be larger.
Ocean‐to‐ice heat flux at the North Pole environmental observatory
Authors: McPhee Miles G. , Kikuchi Takashi , Morison James H. , Stanton Timothy P.
Published: December 2003

Data from drifting buoys deployed in April, 2002, as part of the North Pole Environmental Observatory project have been analysed to estimate ocean heat flux in the time period from 1 May 2002 to 11 Mar 2003. Prior to late January, the observatory remained in deep water, but subsequently drifted directly over the Yermak Plateau, a relatively shallow feature north of Svalbard. While over deep water, heat flux was dominated by storage and release of solar energy in the ocean boundary layer during summer. The most likely annual average value for 2002 was 2.6 W m−2, less than previous determinations in the western Arctic. Over Yermak Plateau, heat flux at the interface came from mixing of warmer water into the boundary layer from below. When the observatory was in water with depths less than 1200 m, the average heat flux was around 22 W m−2.
SuperDARN observations of ionospheric heater–induced upper hybrid waves
Authors: Hughes J. M. , Bristow W. A. , Parris R. T. , Lundell E.
Published: December 2003

Observations are reported of an undocumented HF scattering mode detected by the Kodiak SuperDARN radar during an O‐mode heating experiment recently conducted at the HAARP facility near Gakona, AK. The main features of these observations include enhancements to the Kodiak radar spectrum at frequencies equal to the radar plus the heater frequencies (fr + fH) as well as somewhat weaker enhancements at (fr + fH) ± 85 and (fr + fH) ± 108 kHz. Observations were recorded for ∼20 minutes and enhanced spectra from the heated volume above HAARP were consistently observed by the Kodiak radar during times when the HAARP heater was transmitting. We suggest that the spectral enhancements indicate the presence of perpendicularly propagating upper hybrid (UH) waves which are believed to exist near the UH resonance layer.
A fully coupled constitutive model for thermo‐hydro‐mechanical analysis in elastic media with double porosity
Authors: Khalili N. , Selvadurai A. P. S.
Published: December 2003

The partial differential equations governing non‐isothermal flow and deformation in an elastic medium with double porosity are presented. The governing equations satisfy an effective stress concept, Darcy's law, Fourier' law and the equations of Hookean thermoelasticity. The equations are derived using a systematic macroscopic approach that satisfies conservation laws applicable to balance of linear momentum, mass and energy. The Thermo‐Hydro‐Mechanical coupling take into account processes associated with: thermal expansion, thermal convection by moving fluid, fluid flux due to temperature gradients, heat flux due to pressure gradients, fluid and heat exchange between the two pore system, and the heat of phase compression.
On the thermo‐kinetic consequences of slab melting
Authors: Liang Yan
Published: December 2003

REE abundances in melts produced by partial melting of a hornblende‐bearing eclogite were examined using a dynamic disequilibrium‐melting model and following the P‐T path of a young subducting slab. Model calculations show that melts produced by small degrees of slab melting at 700∼900°C are more likely affected by sluggish kinetics, and have lower La/Yb ratios, higher Li2O and Na2O abundances than melts produced by large degrees of melting at higher temperatures. The extent of disequilibrium depends on deformation and melting histories of the slab, and effective grain sizes and transport properties of the minerals. The LREE abundances in the large‐degree melts are broadly similar to those in adakites. If kinetics is important in slab melting, the degrees of melting required to generate adakites may be higher than values predicted by equilibrium partial melting models and REE abundances in the melts.
Residual solar cycle influence on trends in ionospheric F2‐layer peak height
Authors: Clilverd M. A. , Ulich T. , Jarvis M. J.
Published: December 2003

The longest data sets available for estimating thermospheric temperature trends are those from ground‐based ionosondes, which often begin during the International Geophysical Year of 1957, close to a solar activity maximum. It is important to investigate inconsistencies in trend estimates from these data sets so that trends can be clearly determined. Here we use selected ionosonde stations to show that one of the most significant factors affecting the trend estimates is the removal of the solar cycle. The stations show trend behavior that is close to the behavior of a theoretical model of damped harmonic oscillation. The ringing features are consistent with the presence of solar cycle residuals from the analysis with an amplitude of 2.5 km. Some stations do not show trend behavior that is close to either the average behavior of the stations studied here or the theoretical model of oscillation. Four European stations (Poitiers, Lannion, Juliusruh, and Slough), three of which are closely located in western Europe, were analyzed with the expectation that their trend should be similar. Only Poitiers and Juliusruh showed an evolution that was close to the average behavior of other stations, while the other two were significantly different. The primary cause of this appears to be changes in the M(3000)F2 parameter and demonstrates the importance of incorporating consistency checks between neighboring ionosondes into global thermospheric trend estimates.