bluuebunny/crossref_metadata_2025 huggingface dataset:
Progress in arid-lands rainfall-runoff modelling
Authors: EI-Hames A.S. , Richards K.S.
Published: November 2007

Hydrological data are lacking in arid regions for a variety of reasons, making emprical methods of flood estimation unsatisfactory. Physically based models which are calibrated in the field using measurements of soil hydrological properties offer a viable alternative modelling strategy, and this article reviews the main elements required in such an approach. These include coupled submodels of 1) infiltration and runoff production; 2) overland flow routing; 3) channel routing to propagate flood waves down wadis beyond runoff source areas; and 4) transmissison losses into wadi beds. Examples are presented of flood simulations using a new physically-based model with such a structure. The role of both coarse-grained (bed material) and fine-grained (slackwater deposit) sediments in wadis in providing additional evidence of flood magnitudes is also discussed, and a methodology is suggested in which physical modelling and sedimentological analyses are combined.
Chronostratigraphy of glaciations and permafrost episodes in the Cordillera of western North America
Authors: Harris Stuart A.
Published: November 2007

Glaciations in the Cordillera of western North America began during the Late Miocene in the St Elias Range and coastal ranges near Anchorage, Alaska. Radiometric dating of the tephra and lava flows intercalated in the succession of older tills, loesses and outwash deposits permits the reconstruction of the probable early glacial sequence along the Cordillera. No one site shows the complete sequence, but the available data suggest synchroneity of the major glacial events throughout the region.

The first evidence for cold conditions at low elevations at midlatitudes is from 3.5 Ma BP. By 2.8 Ma, alpine glaciations may have occurred in the Sierra Nevada and ice wedges had formed in bedrock near Fairbanks, Alaska. Three more major glaciations complete with contemporary periglacial and permafrost landforms had occurred by 1.65 Ma, while at least six more major cold events can be recognized during the Quaternary period. Once again, expansion of permafrost conditions occurred during each event and forms an integral part of the evidence for climatic change.
Arid geomorphology 1994
Authors: Lancaster Nicholas
Published: November 2007

The past year has been a landmark year for arid lands geomorphology with the publication of two major texts on desert geomorphology, as well as two edited volumes of articles on aeolian processes, sediments and landforms (Pye, 1993; Pye and Lancaster, 1993) and a special edition of the Journal of Arid Environments devoted to articles from an international symposium on the evolution of deserts in Ahmedabad, India.
Stratospheric ozone depletion - an overview of the scientific debate
Authors: Drake Frances
Published: November 2007

For almost half a century it was widely believed that the photochemistry of the stratosphere and hence ozone distribution were well understoood. As observations revealed a gap between observed and predicted values it was recognized that a number of substances acted as catalysts thereby increasing the destruction of ozone and that humanity could augment those catalysts and affect the ozone layer.

Initial concern focused on nitrogen oxides from the exhausts of supersonic transport, but attention switched in the mid-1970s to chlorofluorocarbons (CFCs). Although the theory of anthropogenic ozone depletion by CFCs found widespread scientific support the perceived threat was minimized in particular by successive model predictions downgrading the amount of depletion. The appearance of the ozone hole over Antarctica in the mid-1980s reopened the debate as to whether such depletion was anthropogenic or natural in origin. It also highlighted the model's inadequate treatment of the processes occurring in the stratosphere and the importance of dynamics and radiative transfer in stratospheric ozone destruction. Scientific consensus again favours the anthropogenic depletion of the ozone layer. In conclusion it is considered that the degree of consensus outweighs the image of scientific uncertainty that is often portrayed in relation to the issue of stratospheric ozone depletion.
Subglacial erosion, deposition and deformation associated with deformable beds
Authors: Hart Jane K.
Published: November 2007

There is an inter-relationship among subglacial deformation, deposition and erosion associated with a glacier moving over a deformable bed. It is suggested that both subglacial deposition and erosion are produced by a number of processes at the 1) icesheet/subglacial deforming layer interface; 2) within the subglacial deforming layer; and 3) at the base of the deforming layer. These processes may result in two styles of net subglacial deforming bed deposition: 1) constructional deformation where the deforming layer moves upwards through the sequence; or 2) excavational deformation where the deforming layer moves down through the sequence. It is suggested that the best evidence for net subglacial deforming bed erosional conditions are drumlins. Changes in subglacial hydrology are also discussed, and the relationship of eskers and tunnel valleys to deforming bed conditions. Sedimentary techniques to identify homogeneous deforming bed tills in the field are also suggested. Criteria include a wide range of fabric strengths depending on the thickness of the deforming layer, rotated clasts, miniflutes, flow around clasts, predictable basal till units (constructional or excavational) and possibly boulder pavements. Finally the extent and style of deforming bed conditions that existed during the glaciations of Britain and Ireland are discussed.
Greenhouse hydrology
Authors: Wilby R.L.
Published: November 2007

Hydrological processes are an integral component of both global climate change arising from increasing concentrations of greenhouse gases and the assessment of subsequent terrestrial impacts. This article examines the potential sensivity of water resources in the UK to climatic change as exemplified by the 1988-92 drought. The representation of hydrological processes at three distinct model scales is then discussed with reference to global hydrology, regional downscaling and catchment-scale responses. A final section speculates on future directions of research for an emerging greenhouse hydrology.
Sediment slugs: large-scale fluctuations in fluvial sediment transport rates and storage volumes
Authors: Nicholas A.P. , Ashworth P.J. , Kirkby M.J. , Macklin M.G. , Murray T.
Published: November 2007

Variations in fluvial sediment transport rates and storage volumes have been described previously as sediment waves or pulses. These features have been identified over a wide range of temporal and spatial scales and have been categorized using existing bedform classifications. Here we describe the factors controlling the generation and propagation of what we term sediment slugs. These can be defined as bodies of clastic material associated with disequilibrium conditions in fluvial systems over time periods above the event scale. Slugs range in magnitude from unit bars (Smith, 1974) up to sedimentary features generated by basin-scale sediment supply disturbances (Trimble, 1981). At lower slug magnitudes, perturbations in sediment transport are generated by local riverbank and/or bed erosion. Larger-scale features result from the occurrence of rare high- magnitude geomorphic events, and the impacts on water and sediment production of tectonics, glaciation, climate change and anthropogenic influences. Simple sediment routing functions are presented which may be used to describe the propagation of sediment slugs in fluvial systems. Attention is drawn to components of the fluvial system where future research is urgently required to improve our quantitative understanding of drainage-basin sediment dynamics.
Tunnel valley genesis
Authors: Cofaigh Colm Ó
Published: November 2007

Tunnel valleys are elongate depressions with overdeepened areas along their floors cut into bedrock or unconsolidated sediment. They are frequently sinuous and form anastomosing networks, although they also exist as independent, straight valleys. Their sedimentary infill is variable but is characteristically dominated by sediment gravity flow facies and thick units of glaciofluvial sands. Till tends to be rare and, where present, occurs along valley sides. Three main theories of tunnel valley formation exist at present. The first ascribes the formation of tunnel valleys cut into unconsolidated sediment to the creep of deformable subglacial sediment into a subglacial conduit from the sides and below, followed by removal of this material through the conduit by meltwater flow. Tunnel valleys are thus created by lowering of the sediment surface on either side of the conduit. The second theory argues that tunnel valleys form during deglaciation, at or close to the ice margin, by subglacial meltwater erosion and that the valleys are time transgressive. The third theory also argues for an origin by subglacial meltwater erosion but claims that the discharges involved took the form of catastrophic channelized floods and that the tunnel valleys within anastomosing networks formed synchronously. Following an outline of tunnel valley geomorphol ogy and sedimentology, each theory is critically reviewed and conclusions are drawn. Emphasis is placed upon the importance of sedimentological and geomorphological field observations as a basis for formulating models of tunnel valley genesis.
Applications of freshwater diatoms to geographical research
Authors: Moser K.A. , MacDonald G.M. , Smol J.P.
Published: November 2007

Diatoms (Bacillariophyceae) are a widely distributed group of microscopic, unicellular algae that are characterized by siliceous cell walls (frustules). The size, shape and sculpturing of diatom frustules are taxonomically diagnostic. Moreover, because of their siliceous composition, diatoms are often well preserved in stratigraphic deposits, where they can be used to infer (quantitatively or qualitatively) past environmental conditions. The sensitivity of diatoms to a wide variety of environmental variables provides geographers with a multitude of research opportunities. This review focuses on palaeoecological reconstructions that are based on freshwater diatoms. Geographical applications including studies of climatology, hydrology, geomorphology, bio geography, and water-quality assessment and monitoring are discussed.
Arid geomorphology
Authors: Lancaster Nicholas
Published: November 2007

Geomorphic research in arid environments continues to attract a wide variety of researchers who employ a range of techniques to understand modem processes and landscape evolution in dry lands. A notable feature of much contemporary research is the collaboration between geomorphologists and those with specialized geochemical and geophysical knowledge, for example in the areas of exposure-age dating. Another significant development is the synergism (at least in the western USA) between environ mental and public policy concerns and fundamental geomorphic research. For example, the need to understand landform development and the history of climate in the region of the proposed high-level nuclear-waste storage facility at Yucca Mountain in southern Nevada has generated important fundamental research on soil-forming processes (Reheis et al., 1995), soil-landscape relations (Peterson et al., 1995), slope stability (Whitney and Harrington, 1993) and evidence for past groundwater discharge conditions (Quade et al., 1995). Similarly, concerns about the response of sensitive semi-arid areas to possible future climate change has stimulated research into the Holocene climatic history and aeolian environments of the Great Plains (Muhs and Maat, 1993; Forman et al., 1995; Loope et al., 1995; Madole, 1995; Muhs and Holliday, 1995; Wolfe et al., 1995) and the Negev Desert (Tsoar, 1995). Of particular importance is the realization that droughts and changes in sediment supply from fluvial systems within the past 150 yr have had effects on dune mobility that are comparable to some inferred Holocene climate changes (Muhs and Holliday, 1995).
Fractals in physical geography
Authors: Gao Jay , Xia Zong-guo
Published: November 2007

Since the fractal concept was introduced to measuring coastline length over three decades ago, fractal analysis has been prolifically applied to many topographic studies. A number of mathematical algorithms are now available to determine the fractal dimension for both linear and areal features. These determination methods require one or more straight-line segments to fit the Richardson's plot. A close examination of the literature shows that not all topographic features are fractal at all scales studied. While the multifractal nature of some geographical phenomena has been explored in great depth, it is not completely understood why some terrains are better modelled with fractal geometry than others. Fractal analysis has been successfully used to measure and characterize irregular linear features such as coastlines and shorelines, to describe and characterize landforms, and to delineate landform regions statistically. Fractal analysis can also be used to produce terrain simulations with a known dimension against which hypotheses can be tested. These studies fail to link fractal dimensions to the underlying geomorphic processes. The failure stems from the fact that there is no one-to-one relationship between geomorphic processes and the landforms they shape.
Bimodal fluvial bed sediments: origin, spatial extent and processes
Authors: Sambrook Smith Gregory H.
Published: November 2007

Historically fluvial channels have been broadly classified as either gravel or sand bed ded. However, a third type, bimodal, also exists. In this case significant quantities of both gravel and sand are present. Studies into bimodal sediments have concentrated on their origin, spatial extent and, more recently, the processes occurring within them. Given the advances in our knowledge of these complex processes it is appropriate to review the developments of research into bimodal sediments. Based on a synthesis of this research it is shown that bimodality may occur readily and be spatially widespread. The effects of the surface sorting prevalent within bimodal sediments on entrainment and transport processes are also explored. From this it is concluded that bimodal beds may represent a distinct threshold between gravel- and sand-bed states. This manifests itself as an abrupt downstream change from a gravel bed to a sand bed.
Slopes and slope processes
Authors: Allison Robert J.
Published: November 2007

Despite suggestions (see Wolman, 1995, for example) that problem-solving in physical geography relies increasingly on interactions between research and application, there is abundant evidence in hillslope studies of research focused on both real-world problems and aimed at generating new knowledge. A study of mass movement patterns (Flagollet, 1996) illustrates application, by examining hazard associated with spatial and temporal variations in landslide activity. Understanding the principle of dynamic equilibrium is, on the other hand, examined and verified using quantitative DEM data (Chandler and Brunsden, 1995) from sequential air photographs for a landslide complex at Black Ven, Dorset, UK. Some studies crosscut applied and basic research. Hazard assessment is one example, where Crozier (1996) uses past temporal records to address magnitude, fre quency and risk issues through the determination of trigger mechanisms and thresholds, rather than simply ranking terrain unit hazard susceptibility.
Simulating present and future climates of southern Africa using general circulation models
Authors: Joubert A.M. , Hewitson B.C.
Published: November 2007

The current state of regional climate and climate change modelling using GCMs is reviewed for southern Africa, and several approaches to regional climate change prediction which have been applied to southern Africa are assessed. Confidence in projected regional changes is based on the ability of a range of models to simulate present regional climate, and is greatest where intermodel consensus in terms of the nature of projected changes is highest. Both equil ibrium and transient climate change projections for southern Africa are considered. Warming projected over southern Africa is within the range of globally averaged estimates. Uncertainties associated with the parameterization of convection ensure that projected changes in rainfall at GCM grid scales remain unreliable. However, empirical downscaling approaches produce rainfall changes consistent with synoptic-scale circulation. Both downscaling and grid-scale approaches indicate a 10-15% decrease in summer rainfall over the central interior which may have important implications for surface hydrology. Climate change may be manifested as a change in variability, and not in mean climate. Over southern Africa, increases in the variability and intensity of daily rainfall events are indicated.
Atmospheric transport of aerosols and trace gases over southern Africa
Authors: Tyson P.D.
Published: November 2007

Atmospheric transport of airborne material over and out of a region has important consequences for global change. In this article, recent developments concerning the transport of air, aerosols and trace gases in the troposphere over southern Africa will be examined. Regional circulation patterns affecting horizontal advection of aerosols and trace gases and the thermo dynamic controls modifying their vertical transport are considered. From trajectory dispersion fields, preferred transport pathways over and out of southern Africa are determined, as too is the extent of recirculation taking place over the subcontinent. Estimates of the mass fluxes of aerosols are given and the consequences of increasing aerosol loading in the atmosphere are examined.
Gully processes and modelling
Authors: Bull L.J. , Kirkby M.J.
Published: November 2007

This article reviews previous investigations into gully and badland research and discusses processes and definitions in the context of existing research in southeast Spain. The theory of badland development is summarized and definitions are proposed which draw on previous work and continuing studies. The processes influencing gully and channel head morphology are then discussed including overland flow, hillslope processes, pipe initiation and enlargement, mass failures and the magnitude and frequency distribution of storm events. Finally, modelling of badland landscapes is discussed. The article highlights that much detailed research has been carried out on badlands, but long-term rates of gully development are not well understood. There are also gaps in our understanding of pipe network formation and collapse. In the short term theoretical modelling may provide the way forward and a direction for more holistic investigations.
Floodplain biogeomorphology
Authors: Hughes Francine M.R.
Published: November 2007

Floodplains are unique ecosystems because of their linear form, the sometimes extreme dynamism of their geomorphology and because they process large fluxes of energy and materials from upstream areas. This article focuses on the importance of hydrological inputs to floodplains through 1) their influence on the arrangement of landforms and vegetation communities and 2) the connections between flooding regimes and the regeneration and turnover time of floodplain vegetation. Many researchers have demonstrated close links between the arrangement of vegeta tion communities and sedimentary landform types, elevation, soil characteristics, tolerance to flooding and availability of soil moisture. It is suggested that plants on floodplains are found along a combined gradient of available moisture and oxygen which can be viewed simultaneously as a flooding frequency gradient and a complex soil moisture gradient. Discussion of experi mental work on floodplains demonstrates the importance of these gradients to a range of flood plain species in different environments. The relationships between these environmental gradients and the apparent high level of overlap between planform patterns of landforms and vegetation communities on floodplains are related to lag times in different parts of vegetation communities. Flood regimes greatly influence the availability of areas suitable for vegetation regeneration from year to year and the age structure of floodplain communities over decadal time frames. Biotic factors also influence biogeomorphological relationships on floodplains and range from sediment- trapping by vegetation to the impacts of beaver and grazing animals on floodplain hydrology and vegetation. Restoration of floodplains is high on the agenda in many countries and it is argued that, for sustainable results, restoration of hydrological pathways is essential. Planned flood releases below dams in several African countries have had varied success rates but the develop ment of models for managing flows to achieve different restoration targets is the start of an integrated approach to restoring complex floodplain ecosystems.
Downscaling general circulation model output: a review of methods and limitations
Authors: Wilby R.L. , Wigley T.M.L.
Published: November 2007

General circulation models (GCMs) suggest that rising concentrations of greenhouse gases may have significant consequences for the global climate. What is less clear is the extent to which local (subgrid) scale meteorological processes will be affected. So-called 'downscaling' techniques have subsequently emerged as a means of bridging the gap between what climate modellers are currently able to provide and what impact assessors require. This article reviews the present generation of downscaling tools under four main headings: regression methods; weather pattern (circulation)-based approaches; stochastic weather generators; and limited-area climate models. The penultimate section summarizes the results of an international experiment to intercompare several precipitation models used for downscaling. It shows that circulation-based downscaling methods perform well in simulating present observed and model-generated daily precipitation characteristics, but are able to capture only part of the daily precipitation variability changes associated with model-derived changes in climate. The final section examines a number of ongoing challenges to the future development of climate downscaling.
Remote sensing of soil-vegetation- atmosphere transfer processes
Authors: Burke E.J. , Banks A.C. , Gurney R.J.
Published: November 2007

This article assesses the potentially powerful combination of remotely sensed data and soil-vegetation-atmosphere transfer (SVAT) schemes. SVATS represent the hydrological processes occurring at the land surface and can be used to estimate, among other processes, the exchange of water and energy between the soil, vegetation and the atmosphere. They require information about the interaction of radiation with the soil and vegetation. Bidirectional reflectance models are now starting to be used to determine the partitioning of radiation. An example of the combination of a simple geometric optics model of bidirectional reflectance and computer graphics is given, where simulations of remotely sensed scenes are produced. The next step is to couple these models to a SVAT scheme. The output of a SVAT scheme can also be used to drive radiative transfer models at different wavebands. This approach is illustrated by the estimation of soil hydraulic properties using a time series of passive microwave emission observations.
Sensorineural dysacusis in patients with systemic lupus erythematosus
Authors: Gomides A.P.M. , do Rosário E.J. , Borges H.M. , Gomides H.H.M. , de Pádua P.M. , Sampaio-Barros P.D.
Published: November 2007

The objective of this study was to establish the frequency of involvement of the auditory apparatus in 45 female patients with systemic lupus erythematosus (SLE) submitted to general clinical and laboratory assessments, and tone and vocal audiometry accompanied by a questionnaire evaluating auditory symptoms. The control group consisted of 45 healthy women, matched by age. Auditory symptoms were present in 25 (55.5%) patients, with a diagnosis of sensorineural hearing loss in seven (15.6%) patients. A significant correlation with hypoacusis ( P < 0.001), ear fullness ( P = 0.012) and tinnitus ( P = 0.017) was observed in patients with hearing loss. None of the clinical or laboratory parameters showed an association with sensorineural hearing loss. In the control group, three women (6.7%) presented audiometric alterations, including two with altered tympanometry results and one with mild sensorineural hearing loss. In conclusion, an adequate investigation of auditory symptoms is important during the follow-up of patients with SLE, since manifestations of the auditory apparatus and sensorineural hearing loss can affect a significant proportion of patients. Lupus (2007) 16, 987—990.
Neurometabolic changes in normal white matter may predict appearance of hyperintense lesions in systemic lupus erythematosus
Authors: Appenzeller S. , Li L.M. , Costallat L.T.L. , Cendes F.
Published: November 2007

To determine if neurometabolic changes in the white matter (WM) of systemic lupus erythematosus (SLE) patients may predict the appearance of small hyperintense lesions on T2-weighted magnetic resonance imaging (MRI) inside the magnetic resonance spectroscopy (MRS) region of interest (ROI). We included 30 SLE patients and 23 controls. We performed single voxel proton MRS over the superior-posterior region of the corpus callosum. We measured signals from N-acetyl-compounds (NAA), choline (Cho) and creatine-phosphocreatin (Cr) and determined NAA/Cr and Cho/Cr ratios. After a minimum of 12 months, MRI and MRS were repeated in all patients and nine volunteers. Twenty patients had normal MRI and 10 patients had MRI hyperintense lesions in the MRS ROI at baseline. All patients had hyperintense lesions in the MRS ROI in follow-up MRIs. All SLE patients had increased Cho/Cr values at both MRS when compared with normal controls ( P = 0.001). In addition, there was an increase in Cho/Cr values when patients' baseline and follow-up MRS were compared ( P = 0.001). We observed a correlation between Cho/Cr ratios and number of WM lesions ( r = 0.69; P = 0.001). Increased Cho/Cr in normal appearing WM may be indicative of future appearance of hyperintense T2-weighted MRI lesions in SLE patients. Lupus (2007) 16, 963—971.
The Clinical Genetics of Lupus
Authors: Lewkonia R.M.
Published: November 2007

Genetic aspects of lupus are reviewed, including recognition of genetic and clinical heterogeneity, genetic factors in the aetiology and heritability of disease, reproductive implications and genetic counselling of patients. Despite a large literature on the genetic epidemiology and immunogenetics of lupus, it remains difficult to apply the results of laboratory findings to the circumstances of individual patients. Generally, lupus is not transmitted as a simple Mendelian trait, and genetic counselling is based on the multifactorial model of disease aetiology with interaction of multiple genetic and environmental factors. Further studies are needed to clarify the heritability of lupus and improve the data for recurrence risk prediction in lupus families.
Canine Systemic Lupus Erythematosus. I: A Study of 75 Cases
Authors: Fournel C. , Chabanne L. , Caux C. , Faure J.-R. , Rigal D. , Magnol J.P. , Monier J.C.
Published: November 2007

We studied 75 cases of canine systemic lupus erythematosus (SLE) presenting with at least four criteria of the American Rheumatism Association (ARA), including antinuclear antibodies (ANAb). This disease mainly affects male German shepherds of an average age of 5 years. The most common clinical signs are polyarthritis (91 % of cases), and renal (65 %) and cutaneo mucous disorders (60%). Hemolytic anemia is rare (13%). ANAb are present, often at high levels (> 256 up to 106 by indirect immunofluorescence on mouse blood smears). The titers are correlated with the severity and the stage of the disease. As double-stranded DNA Ab are rare and as antihistone Ab are frequent, the former could be replaced by the latter in the ARA criteria applied to the SLE dog. Another category of ANAb, named anti-type 1, also seems useful in diagnosing canine SLE. As for therapy, long-term remissions (up to 9 years without treatment) were obtained in 55.6% of 27 SLE dogs treated by levamisole. At first, levamisole was associated with induction corticotherapy, then administered alone and finally discon tinued. Side effects were uncommon and transient.
The Sequelae of Chronic Cutaneous Lupus Erythematosus
Authors: De Berker David , Dissaneyeka Manel , Burge Susan
Published: November 2007

Eighty-six patients with chronic cutaneous lupus erythematosus were examined. Twelve of these also suffered from systemic lupus erythematosus. The mean duration of the disease was 15.1 years. Fifty-seven percent of patients (49/86) had scarring of some kind producing destruction and deformity; 47% (41/86) had scarring of glabrous surfaces and 35% (30/86) had scarring alopecia; 35% (30/86) were also suffering from pigmentary disturbance. The details and treatment of these and other non-scarring sequelae are discussed.
Antinuclear Antibody Profile in Italian Patients with Connective Tissue Diseases
Authors: Neri R. , Tavoni A. , Cristofani R. , Levanti C. , Sodini G. , D'Ascanio A. , Vitali C. , Ferri C. , Bombardieri S.
Published: November 2007

In the present work we report data on the specificity of antinuclear antibodies (ANA) in a large series of Italian patients suffering from a broad spectrum of connective tissue diseases (CTD), by using a series of homogeneous and validated techniques.

The present study confirms, on the one hand, generally accepted concepts, i.e. that certain autoantibodies are strictly associated to certain disease states (such as anti-PCNA and anti-Sm in systemic lupus erythematosus, Jo1 in polymyositis, and ACA and Scl-70 in scleroderma); the presence of 'marker' antibodies is, however, restricted to a relative minority of CTD patients. The application of a new methodological approach that considers the entire profile of ANA can greatly augment their diagnostic relevance and may provide useful indications for their interpretation, allowing us to establish for the first time the diagnostic usefulness not only of marker autoantibodies but also of certain associations between non-marker autoantibodies. Finally, the application of a more appropriate and powerful statistical tool (multiple correspondence analysis) has further emphasized the clear relationship existing between antibody specificities and certain disease states.
Pneumocystis carinii Pneumonia in Patients with Systemic Lupus Erythematosus
Authors: Liam Chong-Kin , Wang Florence
Published: November 2007

At the University Hospital, Kuala Lumpur, Malaysia, nine patients with systemic lupus erythematosus (SLE) were treated for Pneumocystis carinii pneumonia (PCP) between January 1987 and December 1988. When they developed PCP all the patients' SLE disease course was active and eight of them were on prednisolone. Four of these eight patients were also receiving cyclophosphamide. Patients who were on more intensive immunosuppressive therapy were found to develop more severe PCP. All the patients except one were treated with high-dose cotrimoxazole. Four patients responded to antipneumocystis treatment and survived, while PCP was responsible for the death of the five non-survivors.
Expression and Relationships of Seven Public Idiotypes of DNA-Binding Autoantibodies on Monoclonal Antibodies and Serum Immunoglobulins
Authors: Staines N.A. , Ravirajan C.T. , Morgan A. , Belcher A.J. , Henry A.J. , Lake R.A. , Smith D.A. , Hamblin A.S. , Hara M. , Adu D. , Morland C. , Isenberg D.A.
Published: November 2007

Many studies have shown that DNA-reactive autoantibodies share cross-reactive public idiotypes that are defined, usually, by single anti-idiotype reagents. Because anti-idiotype antibodies or antisera will be limited in their ability to detect all the idiotopes of a particular antibody, their use will tend to underestimate the full extent of idiotype sharing between different antibodies. In order to define more comprehensively the extent of idiotype sharing in DNA autoantibodies, a panel of DNA-binding monoclonal autoantibodies from lupus mice was examined with a range of anti-idiotype antisera prepared in rabbits (five sera), guinea pigs (four sera) and a sheep. Each idiotype was detected on more antibodies than its original reference monoclonal antibody, and idiotopes of each were also present on serum immunoglobulins from lupus mice. Of 23 monoclonal antibodies 65% reacted with one or more of the anti-idiotype reagents. On these criteria, all the idiotypes were public; none was private in its expression.

In about half the cases the idiotypes were located in or near the antigen-binding sites of the antibodies, but a direct relationship to specificity was not obvious except in the case of Id.228 present on antibodies with a relatively high affinity for single-stranded DNA. In other cases there was no obvious relationship between idiotype and specificity. Antibodies from the same mouse did not each express the same array of idiotopes. None of the idiotypes was restricted to either (NZB x NZW)F1 or MRL/Mp-lpr/lpr mice, but there were differences between the strains. In the former, the expression of idiotype Id.88 notably decreased with age, whereas it rose with age in mice of the latter strain. The extent of sharing of the idiotypes of these DNA antibodies indicates that the great majority of the antibodies are related to each other idiotypically. From these studies, it is clear that the definition of idiotype sharing can be achieved comprehensively only if antibodies against many idiotopes are used.
Brain Synaptosomal Antibodies in Systemic Lupus Erythematosus
Authors: Hanly John G. , Hong Connie , White Thomas D.
Published: November 2007

Nervous system involvement in systemic lupus erythematosus (SLE) has been linked to the production of autoantibodies that may bind to surface antigens on neuronal cells and cause cellular dysfunction. At present, little is known of the target antigens recognized by these antibodies. The aim of the present study was to examine reactivity to rat brain synaptosomes (RBS) in sera from patients with SLE. Sera from 73 unselected SLE patients and controls were studied. Crude RBS were prepared by differential centrifugation and enriched fractions of synaptosomes (SY-E), myelin (MY-E) and mitochondria (MI-E) were obtained by sucrose density centrifugation. Rat liver (RL) was used for control antigens. Wheat-germ lectin affinity chromatography yielded membrane-enriched fractions of RBS, RL and whole rat brain (WRB). Antibody binding was examined by Western blotting. IgM reactivity was detected in 12/73 sera (16%) and was directed to proteins of 62K, 48K and 37K molecular weight. IgG reactivity was present in 5/73 sera (7%) to proteins of 52K, 48K, 37K and 29K molecular weight. Except for binding to the 52K and 37K proteins these autoantibodies were not detected in control sera. Reactivity was usually absent, or present in reduced amounts, in WRB and RL. Additional experiments revealed that binding of IgM to 62K was found predominantly in SY-E and MY-E fractions, 48K in SY-E and MI-E fractions and 37K in the SY-E fraction. Binding of IgG to 48K and 29K was detected in the SY-E and MI-E, but reactivity to 52K and 37K was restricted to the SY-E fraction. Thus, sera from SLE patients contain antibodies to synaptosomal antigens that may contribute to the neuropsychiatric manifestations of the disease.
Calprotectin in Patients with Systemic Lupus Erythematosus: Relation to Clinical and Laboratory Parameters of Disease Activity
Authors: Haga Hans-Jacob , Brun Johan G. , Berner Berntzen Hilde , Cervera Ricard , Khamashta Munther , Hughes Graham R.V.
Published: November 2007

Calprotectin (L1) is a granulocyte and monocyte cytosolic protein released during activation of these cells. The plasma level of L1 has been shown to be a good marker of disease activity in rheumatoid arthritis. In this cross-sectional study of 100 patients with systemic lupus erythematosus (SLE), the serum level of L1 was found to be higher in patients than in matched controls (3661 μg/l versus 1051 μg/l; P < 0.001). The serum level of L1 was the only laboratory parameter with significant association to the disease activity index SLEDAI (r = 0.28; P < 0.01). Furthermore, the serum level of L1 was significantly higher in SLE patients with anti-DNA antibodies compared to patients without anti-DNA antibodies (4501 μg/l versus 3279 μg/l; P = 0.01). SLE patients with arthritis had higher serum levels of L1 than patients without arthritis (7652 μg/l versus 2811 μg/l; P < 0.01), indicating that the serum level of L1 also reflects arthritis activity in SLE.
Analysis of Clinical and Laboratory Profile in Indian Childhood Systemic Lupus Erythematosus and its Comparison with SLE in Adults
Authors: Pande Ira , Sekharan N.G. , Kailash S. , Uppal S.S. , Singh R.R. , Kumar A. , Malaviya A.N.
Published: November 2007

Data on the clinical and laboratory profiles of 83 children with SLE have been analysed and compared with data on 187 adults with the disease. The clinical features of childhood SLE are similar to those seen in adults, although clinical and laboratory parameters reflect propensity to a more severe form of the disease in the juvenile age group. However, in comparison to descriptions of childhood lupus from developed countries, in India the female-to-male ratio in this age group parallels that in adults. Renal involvement is noted to be more common, whereas gastro-intestinal and haematological abnormalities are less common. Interestingly, leucopenia, lymphopenia and nephritic type of renal involvement are commonly seen in boys with SLE, but these features are non-existent in men.
A Simple Severity of Disease Index for Systemic Lupus Erythematosus
Authors: Katz James D. , Senegal Jean-Luc , Rivest Charles , Goulet Jean-Richard , Rothfield Naomi
Published: November 2007

Although indices of activity for systemic lupus erythematosus have been developed and validated, a disease staging system requires the measurement of severity as well. We have constructed such a scale, the Lupus Severity of Disease Index (Lupus SDI). Accepted clinical, pathologic and physiologic classification schemes were employed to validate this index at two separate research institutions. The Lupus SDI allows homogenization of patient populations for the purposes of research and, possibly, for case mix adjustment.
Fetal Outcome in Lupus Pregnancy: A Retrospective Case-Control Study of 242 Pregnancies in 112 Patients
Authors: Julkunen Heikki , Jouhikainen Taneli , Kaaja Risto , Leirisalo-Repo Marjatta , Stephansson Eija , Palosuop Timo , Teramo Kari , Friman Claes
Published: November 2007

Fetal outcome in systemic lupus erythematosus (SLE) was retrospectively analysed in 242 pregnancies in 112 unselected patients, and the outcome was compared with that of 417 pregnancies in 192 control women matched for age, parity and socio-economic status. Relative risk for fetal loss after the diagnosis of SLE was 2.5 (95% confidence interval (CI), 1.4-4.5), for prematurity 5.8 (3.2-10.5) and for intra-uterine growth retardation (IUGR) 8.6 (3.0-24.3). Fetal outcome of pregnancy in patients with pre-existing stable lupus nephritis was no worse than in other SLE pregnancies.

Relations of three lupus anticoagulant (LA) assays and three anticardiolipin (aCL) enzyme-linked immunosorbent assays to fetal outcome were studied. Patients positive by any LA assay had a previous fetal loss more often than patients negative by all LA assays (odds ratio 3.4; 95% CI, 1.3-9.0; P = 0.01). Of the 41 patients whose antiphospholipid antibody (aPL) tests were all negative, five (12%) had a history of fetal loss (16% in controls). As a group, aCL was more sensitive for fetal loss than LA (64% vs 50%), but LA was more specific (77% vs 52%). Combinations of one aCL assay with one LA assay had a 41-73% sensitivity and a 64-73% specificity for a history of fetal loss. aPL did not correlate to prematurity or fetal growth retardation.

In conclusion, fetal loss in SLE is 2.5 times more prevalent than in the normal population. The presence of LA indicates a high risk for fetal loss, and the absence of aPL is an indication of a favorable pregnancy outcome. Prematurity and IUGR are common in SLE, but they are not associated with aPL.
Reciprocal Changes in Complement Activity and Immune-Complex Levels during Plasma Infusion in a C2-Deficient SLE Patient
Authors: Erlendsson Kristján , Traustadóttir Kristin , Freysdóttir Jóna , Steinsson Kristján , Jónsdóttir Ingileif , Valdimarsson Helgi
Published: November 2007

Although systemic lupus erythematosus (SLE) is abnormally common in individuals with complement deficiency, conclusive evidence has been lacking for a direct causal relationship between disease manifestations and a missing complement component. A patient with C2 deficiency and SLE has been treated with 56 courses of fresh frozen plasma (FFP) infusions over a period of 8 years. Each infusion, involving a total of 12 units of FFP administered in equal doses over 4 consecutive days, has consistently resulted in a transient restoration of the classical pathway of complement, and a full clinical remission lasting 6-8 weeks.

This report is concerned with changes in the levels of immune complexes, C2 and C3d during an infusion cycle. Four progressively rising peaks in C2 and C3d were observed during the 4 days of the plasma infusion, and these peaks coincided with four reciprocally descending troughs in the levels of immune complexes. Identical fluctuations have been consistent in all the plasma-infusion cycles that have so far been monitored, and their consistent association with clinical remissions indicates a causal relationship between the C2 restoration and clinical remissons in this C2-deficient SLE patient.
The Association of Malignancy with SLE: An Analysis of 150 Patients Long-Term Review
Authors: Menon S. , Snaith M.L. , Isenberg D.A.
Published: November 2007

We describe seven patients who developed malignancy before, after or at the onset of systemic lupus erythematosus (SLE). They comprise three cases of breast adenocarcinoma, two cases of Hodgkin's lymphoma, and one each of cholangiocarcinoma and thymoma. Only one of this group had been treated with cytotoxic agents, and five have subsequently died. They belonged to a group of 150 SLE patients, many of whom had been treated with steroids and cytotoxic agents, under long-term follow-up at a specialized lupus clinic. We discuss the reported association of malignancy in patients with SLE.
Clinical Diversity of Raynaud's in Childhood: Report of Six Cases
Authors: Buoncompagni Antonella , Scribanis Raffaele , Picco Paolo , Di Ricco Maja , Gandullia Paolo , Baldi Lucia , Pistoia Vito
Published: November 2007

Six pediatric patients with Raynaud's phenomenon are reported. Three patients had clinical and immunological evidence for a connective tissue disease; one had primary Raynaud's phenomenon associated with antinuclear antibodies and two had Raynaud's phenomenon secondary to exposure to lead and to a vibrating instrument, respectively. The diagnostic and therapeutic implications of these observations will be discussed.
Allergic Disorders in Systemic Lupus Erythematosus
Authors: Sequeira Joao F. , Cesic Dajana , Keser Gokhan , Bukelica Mirjana , Karanagnostis Sotirios , Khamashta Munther A. , Hughes Graham R.V.
Published: November 2007

Allergic disorders commonly occur in patients with systemic lupus erythematosus (SLE) and allergies to some drugs may occasionally be related to disease flares.

We evaluated the prevalence of several types of allergy in 132 patients with SLE and their families and compared the SLE patients with a control group of 66 patients with non-lupus disorders. Total IgE levels were tested in 117 SLE patients and 61 controls.

Eighty-three of the 132 SLE patients (63 %) had a history of at least one type of allergic disorder. This prevalence was significantly higher (P < 0.0001) than that of the control group (20 of 66 controls). The prevalence of drug allergy (38 % vs. 17 %; P < 0.005), skin allergy (36 % vs. 17 %; P < 0.01) and insect allergy (14 % vs. 2 %; P < 0.02) were significantly higher in SLE patients when compared with controls. Family members of SLE patients had an increased prevalence of at least one type of allergy (55 % vs. 24 %; P < 0.0001), allergic rhinitis (30 % vs. 14 %; P < 0.02), asthma (25 % vs. 9 % P < 0.02), and both rhinitis or asthma (44 % vs. 20 %; P ≤ 0.002). The presence of allergy both in the patient and in the family was more frequent in SLE patients than controls (42 '10 vs. 15 %; P < 0.001). We found no differences in total IgE levels between the two groups.

Drug, skin and insect allergies were particularly frequent in SLE patients. We also found a high prevalence of rhinitis and asthma in their families.
Hepatic Infarction in a Pregnant Patient with the 'Primary' Antiphospholipid Syndrome
Authors: Millán-Mon A. , Porto J.L. , Novo C. , García-Martin C. , Guitián D.
Published: November 2007

We describe a patient with previous venous thrombosis while using oral contraceptives and recurrent pregnancy loss, who presented with massive hepatic infarction in the last trimester of the fourth gestation. Thrombocytopenia, the lupus anticoagulant (LA) and the anticardiolipin antibody (aCL) were detected and a diagnosis of a 'primary' antiphospholipid syndrome (APS) was made. The clinical and histological manifestations and the differential diagnosis, especially with DIC and pre-eclampsia, are discussed.
Prevalence of the Type I Complement C2 Deficiency Gene in Swedish Systemic Lupus Erythematosus Patients
Authors: Truedsson Lennart , Sturfelt Gunnar , Nived Ola
Published: November 2007

The prevalence of type I complement C2 deficiency in Swedish systemic lupus erythematosus (SLE) patients was investigated by DNA analysis. The characteristic 28 base pair deletion was determined by polymerase chain reaction analysis followed by gel electrophoresis. Five of the 86 patients (5.8%) retrieved from a defined population of 160 000 individuals were heterozygous for the C2Q0 gene compared with one heterozygote of 100 local blood donors (1%), the difference in prevalence not being significant. Among 26 other SLE patients, two patients who are siblings were C2Q0 homozygous. No distinctive clinical features among the patients with C2Q0 genes were obvious, although none had renal involvement.
Bullous Systemic Lupus Erythematosus - A Variable Disease
Authors: Yell J.A. , Wojnarowska F. , Allen J. , Burge S.M.
Published: November 2007

We describe a patient with the rare diagnosis of 'Bullous Systemic Lupus Erythematosus'. She is unusual in presenting with classical dermatitis herpetiformis, before the emergence of systemic features. In addition, on indirect immunofluorescence on sodium chloride split skin, there was epidermal binding. Immunoblotting was negative for type VII collagen.
Case Report Antiphospholipid Syndrome in Rheumatoid Arthritis Presenting with Sensorineural Deafness
Authors: Etherington J. , Cooper A. , Buchanan N.
Published: November 2007

This case illustrates the varied presentation of the antiphospholipid syndrome, its rare association with rheumatoid arthritis and the need for rapid and accurate diagnosis of the condition to prevent further thrombotic episodes.