bluuebunny/crossref_metadata_2025 huggingface dataset:
Temporal relations between meander deformation, water discharge and sediment fluxes in the floodplain of the Rio Beni (Bolivian Amazonia)
Authors: Gautier E. , Brunstein D. , Vauchel P. , Roulet M. , Fuertes O. , Guyot J. L. , Darozzes J. , Bourrel L.
Published: July 2006

The Andean Cordillera and piedmont significantly influence river system and dynamics, being the source of many of the important rivers of the Amazon basin. The Beni River, whose upper sub‐catchments drain the Andean and sub‐Andean ranges, is a major tributary of the Madeira River. This study examines the river in the south‐western Amazonian lowlands of Bolivia, where it develops mobile meanders. Channel migration, meander‐bend morphology and ox‐bow lakes are analysed at different temporal and spatial scales. The first part of this study was undertaken with the aim to link the erosion–deposition processes in the active channel with hydrological events. The quantification of annual erosion and deposition areas shows high inter‐annual and spatial variability. In this study, we investigate the conditions of sediment exportation in the river in relation to three hydrological parameters (flood intensity, date of discharge peak and duration of the bank‐full stage level). The second part of this study, focusing on the abandoned meanders, analyses the cutoff processes and the post‐abandonment evolution during 1967–2001. This approach shows the influence of the active channel behaviour on the sediment diffusion and sequestration of the abandoned meanders and allows us to build a first model of the contemporary floodplain evolution. Copyright © 2006 John Wiley & Sons, Ltd.
Temporal aggregation of an ESTAR process: some implications for purchasing power parity adjustment
Authors: Paya Ivan , Peel David A.
Published: July 2006

Nonlinear models of deviations from PPP have recently provided an important, theoretically well motivated, contribution to the PPP puzzle. Most of these studies use temporally aggregated data to empirically estimate the nonlinear models. As noted by Taylor (2001), if the true DGP is nonlinear, the temporally aggregated data could exhibit misleading properties regarding the adjustment speeds. We examine the effects of different levels of temporal aggregation on estimates of ESTAR models of real exchange rates. Copyright © 2006 John Wiley & Sons, Ltd.
Modelling firm‐size distribution using Box–Cox heteroscedastic regression
Authors: Yang Z. L. , Tse Y. K.
Published: July 2006

Using the Box–Cox regression model with heteroscedasticity (BCHR), we re‐examine the size distribution of the Portuguese manufacturing firms studied by Machado and Mata (2000) using the Box–Cox quantile regression (BCQR) method. We show that the BCHR model compares favourably against the BCQR method. In particular, the BCHR model can answer the key questions addressed by the BCQR method, with the advantage that the estimated quantile functions are monotonic. Furthermore, estimation of the BCHR model is straightforward and the confidence intervals of the BCHR regression quantiles are easy to compute. Copyright © 2006 John Wiley & Sons, Ltd.
Use of sodium concentration and anion gap to improve correlation between serum chloride and bicarbonate concentrations
Authors: Feldman Mark , Soni Nilam J. , Dickson Beverly
Published: July 2006

Although most acid‐base disorders cause opposite and equal changes in serum chloride and bicarbonate concentrations, this inverse relationship can be distorted by changes in the anion gap and/or water balance. Therefore, we examined the relationship between chloride and bicarbonate before and after adjusting for anion gap and serum sodium concentration. Patients with abnormal electrolytes were grouped by chloride and bicarbonate concentrations (low, normal, and high). Then, chloride and anion gap‐adjusted bicarbonate were adjusted for water excess (or deficit), manifesting as hyponatremia (or hypernatremia), after which patients were reclassified. Classification by chloride and bicarbonate changed in 82% of the 135 patients after adjustment for anion gap and sodium. Serum chloride and bicarbonate were each low (concordant) in 23 patients, while 18 had discordant chlorides and bicarbonates (9 low/high, 9 high/low). After adjustments, chloride and bicarbonate were discordant in 40 patients (31 low/high, 9 high/low) and concordant in none. The correlation between serum chloride and bicarbonate improved from −0.459 to –0.998 after adjustments for sodium and anion gap. A very close inverse relationship between serum chloride and bicarbonate concentrations is commonly distorted by concomitant water disturbances and anion gap acidoses in internal medicine patients admitted with electrolyte disorders. J. Clin. Lab. Anal. 20:154–159, 2006. © 2006 Wiley‐Liss, Inc.
Evaluation of Parkinson's Disease in entrants on the Nebraska State Parkinson's Disease Registry
Authors: Bertoni John M. , Sprenkle Pamela M. , Strickland Daniel , Noedel Nancy
Published: July 2006

We examined a sample of individuals in the Nebraska State Parkinson's Disease Registry (NSPDR) to determine what proportion meets standard criteria for Parkinson's disease (PD). The NSPDR was established in 1996 in an effort to understand the high prevalence of PD in Nebraska. Only minimal demographic data are included for each entrant. Subjects enter the NSPDR by means of diagnosing physicians, pharmacists dispensing anti‐PD medications and the patients themselves. A series of 356 registrants diagnosed between 1997 and 2001 were contacted and invited to participate in a case–control study. Medical records were reviewed by a single abstractor in a standard manner. When patients consented, history was filled in by interview. A subset of patients were examined by a movement disorders specialist, who assigned all patients a probability of PD. Where sufficient information was available, 78% of registrants were confirmed to have PD (i.e., percent probability > 50%), including 83% of the patients previously diagnosed by a neurologist. Tremor was an initial symptom in 72% of confirmed versus 39% of excluded cases, and resting tremor was present in 86% of those that were confirmed. The most frequent reasons for exclusion were drug‐induced Parkinsonism, multiple systems atrophy, vascular disease, and essential tremor. Use of the NSPDR for epidemiologic study requires careful review of the data set before assignment of cases. When histories are compiled in a standardized, comprehensive manner, the necessity for actual patient examinations can be minimized. © 2006 Movement Disorder Society
Ropinirole is effective in the long‐term management of restless legs syndrome: A randomized controlled trial
Authors: Montplaisir Jacques , Karrasch Jeff , Haan Jean , Volc Dieter
Published: July 2006

The objective of this study was to investigate the long‐term efficacy of ropinirole in patients with restless legs syndrome (RLS) and to assess the potential for relapse after the discontinuation of active treatment. Patients with primary RLS (n = 202) received single‐blind ropinirole for 24 weeks. Patients meeting treatment continuation criteria were randomized to double‐blind treatment with ropinirole or placebo for a further 12 weeks. The primary efficacy variable was the proportion of patients relapsing during double‐blind treatment. Additional efficacy measures included time to relapse, withdrawals due to lack of efficacy, improvement on the Clinical Global Impression–Improvement (CGI‐I) scale, change in International Restless Legs Scale (IRLS) score during double‐blind treatment, and changes in sleep and quality of life (QoL) parameters. Significantly fewer patients relapsed on ropinirole than on placebo (32.6% vs. 57.8%; P = 0.0156). Time to relapse was longer with ropinirole and more patients withdrew due to lack of efficacy with placebo. Patients showed improvements in IRLS and CGI‐I scores, sleep and QoL parameters with single‐blind ropinirole, which were better maintained when ropinirole was continued during the double‐blind phase, but reduced with placebo. Ropinirole was well tolerated; adverse events were typical for dopamine agonists. Ropinirole was highly effective and well tolerated in the long‐term management of RLS, with pharmacological effect over 36 weeks. © 2006 Movement Disorder Society
Olivopontocerebellar atrophy: Toward a better nosological definition
Authors: Berciano José , Boesch Sylvia , Pérez‐Ramos José M. , Wenning Gregor K.
Published: July 2006

Olivopontocerebellar atrophy (OPCA) is a pathological label implying not only olivopontocerebellar changes, but also cases with more widespread lesions involving the CNS. This polytopic pathological background accounts for clinical complexity, essentially defined as cerebellar‐plus syndrome. The term “OPCA” is applicable to an increasing number of neurodegenerative syndromes, including autosomal dominant ataxia, complicated spastic paraplegia, multiple‐system atrophy (MSA), and many cases of idiopathic late‐onset cerebellar ataxia (ILOCA), some of whom also turn out to have MSA. OPCA may also be part of the pathological hallmark of other disorders, such as prion disorders, mitochondrial encephalomyopathies, and hereditary metabolic diseases. Sporadic OPCA and ILOCA with cerebellar‐plus presentation and neuroimaging evidence of brainstem and cerebellar atrophy may represent interchangeable eponyms. Just a quarter of such cases evolve to MSA within 5 years of the onset of symptoms. Therefore, the assumption that MSA and sporadic OPCA necessarily are one and the same disease is no longer tenable. Our review suggests that the label “OPCA” is useful to designate a clinicopathological syndrome that has a variety of etiologies carrying a poor prognosis, particularly if associated with autonomic failure as occurs in MSA. © 2006 Movement Disorder Society
Atypical phenotypes and clinical variability in a large Italian family with DYT1–primary torsion dystonia
Authors: Gambarin Mattia , Valente Enza Maria , Liberini Paolo , Barrano Giuseppe , Bonizzato Alberto , Padovani Alessandro , Moretto Giuseppe , Fiorio Mirta , Dallapiccola Bruno , Smania Nicola , Fiaschi Antonio , Tinazzi Michele
Published: July 2006

The GAG deletion in the DYT1 gene usually causes a typical form of primary torsion dystonia (PTD) with early onset in a limb, rapid generalization, and sparing of cranial–cervical muscles, but atypical phenotypes have often been reported. Here, we describe a large DYT1 Italian family with phenotypically heterogeneous PTD that recapitulates all the atypical features associated with the DYT1 mutation, including late age at onset, focal or segmental phenotypes, onset or spreading of dystonia to the cranial–cervical muscles. Of 38 healthy family members, 15 also carried the DYT1 mutation, with an estimated penetrance of 21%. A literature review of atypical familial cases of DYT1‐PTD showed that late onset, cervical involvement, and limited progression of dystonia are features frequently seen in DYT1 families. However, nearly all of these atypical patients fall within at least one of the clinical categories that best predict the DYT1 carrier status, namely, early onset, onset in a limb, and family history positive for early‐onset dystonia. © 2006 Movement Disorder Society
Relationship between classic Hodgkin lymphoma and overlapping large cell lymphoma investigated by comparative expressed sequence hybridization expression profiling
Authors: Vanhentenrijk V , Vanden Bempt I , Dierickx D , Verhoef G , Wlodarska I , De Wolf‐Peeters C
Published: July 2006

There is a diagnostic grey zone between classic Hodgkin lymphoma (cHL) and some non‐Hodgkin lymphoma (NHL), including primary mediastinal B cell lymphoma, diffuse large B cell lymphoma, and anaplastic large cell lymphoma. They all have some morphological and/or phenotypic features in common. To investigate this, we undertook an expression profiling study of these lymphomas using comparative expressed sequence hybridization. This technique detects chromosomal regions that are differentially expressed between a test and a reference tissue in a manner similar to comparative genomic hybridization, and is particularly suitable when the number of informative biopsies is limited. Using this approach, we identified a unique expression profile for all lymphoma types investigated. Unsupervised hierarchical cluster analysis of the acquired data showed that cHL separates from all investigated NHLs, including ALCL‐like HL. Moreover, anaplastic lymphoma kinase (ALK)‐negative ALCL clustered in a separate branch together with ALCL‐like HL. Thus, analysing the neoplastic cells concurrently with their microenvironment, ALK‐negative ALCL and ALCL‐like HL seem to be related to each other, while cHL constitutes a separate lymphoma entity. Copyright © 2006 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Pericardial involvement at diagnosis in pediatric Hodgkin lymphoma patients
Authors: Bashir Hamid , Hudson Melissa M. , Kaste Sue C. , Howard Scott C. , Krasin Matthew , Metzger Monika L.
Published: July 2006

Background

Because most cases are clinically silent, the incidence, clinical course, and outcome of pericardial involvement in Hodgkin lymphoma are unknown.

Methods

Records of all patients with newly diagnosed Hodgkin lymphoma treated at our institution between 1991 and 2004 were reviewed. Pericardial involvement was identified by computerized tomography (CT) as focal thickening or nodularity present at the time of diagnosis, and by echocardiography as pericardial effusion. Outcomes measured were incidence of pericardial involvement, relapse‐free survival, and overall survival.

Results

Thirteen of 273 patients (5%) had pericardial involvement. All patients with pericardial involvement had nodular sclerosing tumors versus 183 of 260 patients without pericardial involvement (P = 0.02); 9 (67%) had a bulky mediastinal mass versus 27% (P = 0.002). Two patients required pericardial drainage to drain very large effusions (n = 2). Both patients were symptomatic with either shortness of breath or superior vena cava syndrome. In the 11 cases that did not undergo surgical drainage, the effusion resolved within days after starting chemotherapy. Two patients experienced distant relapse but underwent successful salvage therapy. All 13 patients remain alive and free of disease at a median follow‐up of 9.7 years (range, 1.7–12.9 years) with normal cardiac function.

Conclusions

Pericardial involvement by lymphoma is usually asymptomatic unless accompanied by substantial pericardial effusion. In most cases, pericardial involvement resolves with treatment of the underlying malignancy, but close observation for hemodynamic complications is required. A symptomatic effusion, once treated, does not affect survival. Pediatr Blood Cancer 2007;49:666–671. © 2006 Wiley‐Liss, Inc.
A variety of poly(m‐benzamide)s with low polydispersities from inductive effect‐assisted chain‐growth polycondensation
Authors: Ohishi Tomoyuki , Sugi Ryuji , Yokoyama Akihiro , Yokozawa Tsutomu
Published: July 2006

Chain‐growth polycondensation of 3‐(alkylamino)benzoic acid alkyl esters 1 was investigated for obtaining poly(m‐benzamide)s with defined molecular weights and low polydispersities. Polymerization conditions were first studied to find that ethyl 3‐(octylamino)benzoate (1b) polymerized in a chain polymerization manner in the presence of lithium 1,1,1,3,3,3‐hexamethyldisilazide (LiHMDS) as a base and phenyl 4‐methylbenzoate (2b) as an initiator in THF at 0 °C. The molecular weight of the polymer was controlled by the feed ratio of monomer to initiator. The polymerization of 1c–i with a variety of N‐alkyl groups was then carried out under the established conditions to yield well‐defined poly(m‐benzamide)s, which showed higher solubility than those of the corresponding poly(p‐benzamide)s. Furthermore, the 4‐octyloxybenzyl group on the amide nitrogen in poly1i was removed by treatment with trifluoroacetic acid (TFA) to give N‐unsubstituted poly(m‐benzamide) (poly1j) with a low polydispersity, which is soluble in DMAc and DMSO, contrary to the para‐substituted counterpart. © 2006 Wiley Periodicals, Inc. J Polym Sci Part A: Polym Chem 44: 4990–5003, 2006
Supramolecular polymers based on cyclodextrins
Authors: Harada Akira
Published: July 2006

Two types of supramolecular polymers based on cyclodextrins were prepared. One was a host–guest type, and the other was a polyrotaxane type. When a guest part was covalently attached to cyclodextrin, they formed supramolecular dimers, a cyclic daisy chain, supramolecular oligomers, and polymers. t‐Boc‐cinnamamide‐α‐cyclodextrin was found to form chiral supramolecular polymers in aqueous solutions. Supramolecular poly[2]rotaxane polymers and supramolecular α,β‐cyclodextrin copolymers were obtained. Polyrotaxanes containing β‐cyclodextrin or γ‐cyclodextrin were prepared. © 2006 Wiley Periodicals, Inc. J Polym Sci Part A: Polym Chem 44: 5113–5119, 2006
Telling children and adolescents about their cancer diagnosis: cross‐cultural comparisons between pediatric oncologists in the US and Japan
Authors: Parsons Susan K. , Saiki‐Craighill Shigeko , Mayer Deborah K. , Sullivan Amy M. , Jeruss Stefanie , Terrin Norma , Tighiouart Hocine , Nakagawa Kaoru , Iwata Yoko , Hara Junichi , Grier Holcombe E. , Block Susan
Published: July 2006

Purpose: Over the last 50 years, direct communication about cancer with adults has shifted from an approach of not telling to one of telling. Less is known about communication practices with children. The purpose of this study is to (1) describe patterns of communication at diagnosis between pediatric oncologists and children with cancer and (2) compare cultural differences in these practices in the US and Japan.

Methods: This 2003 survey, developed in English and translated into Japanese was mailed to members of the American Society of Pediatric Hematology/Oncology and the two Japanese Societies of Pediatric Hematology and Oncology; there were 350 US and 362 Japanese respondents. Descriptive statistics and logistic regressions were performed.

Results: US physicians had a consistent pattern of telling children (65% always told the child; less than 1% rarely or never told). Japanese physicians had greater variability in their patterns of telling (with only 9.5% always telling, 34.5% rarely or never telling). Direct communication with the child was influenced by personal attitudes, patient factors, and work culture in both countries. Many more variables emerged as influencing Japanese physicians' communication practices than for US physicians. US physicians were influenced by their own sense of responsibility for telling, while Japanese physicians were more influenced by personal attitudes, patient factors, and work culture.

Conclusions: US and Japanese physicians differed when communicating directly with the child about his or her cancer. The impact of these practices on children and their parents should be explored and the parent and child's perspectives elicited. This information will help facilitate culturally sensitive patient and family centered communication. Copyright © 2006 John Wiley & Sons, Ltd.
Association of information satisfaction, psychological distress and monitoring coping style with post‐decision regret following breast reconstruction
Authors: Sheehan Joanne , Sherman Kerry A. , Lam Thomas , Boyages John
Published: July 2006

Little is known of the psychosocial factors associated with decision regret in the context of breast reconstruction following mastectomy for breast cancer treatment. Moreover, there is a paucity of theoretically‐based research in the area of post‐decision regret. Adopting the theoretical framework of the Monitoring Process Model (Cancer 1995;76(1):167–177), the current study assessed the role of information satisfaction, current psychological distress and the moderating effect of monitoring coping style to the experience of regret over the decision to undergo reconstructive surgery. Women (N=123) diagnosed with breast cancer who had undergone immediate or delayed breast reconstruction following mastectomy participated in the study. The majority of participants (52.8%, n=65) experienced no decision regret, 27.6% experienced mild regret and 19.5% moderate to strong regret. Bivariate analyses indicated that decision regret was associated with low satisfaction with preparatory information, depression, anxiety and stress. Multinominal logistic regression analysis showed, controlling for mood state and time since last reconstructive procedure, that lower satisfaction with information and increased depression were associated with increased likelihood of experiencing regret. Monitoring coping style moderated the association between anxiety and regret (β=−0.10, OR=0.91, p=0.01), whereby low monitors who were highly anxious had a greater likelihood of experiencing regret than highly anxious high monitors. Copyright © 2006 John Wiley & Sons, Ltd.
System development and demonstration of a UAV control architecture for information gathering missions
Authors: Cole David T. , Sukkarieh Salah , Göktoğan Ali Haydar
Published: July 2006

This paper presents a system architecture for unmanned aerial vehicles (UAVs) performing information gathering missions. Particular focus in this paper is on the development of the architecture from algorithms to simulation, hardware‐in‐the‐loop testing and flight demonstrations. The architecture has been developed for a team of Brumby Mk 3 fixed wing UAVs. Results from recent flight demonstrations are presented in which a single UAV validated a path‐following guidance algorithm for feature orbiting, and a utility‐based dynamic path planning algorithm applied to a feature tracking problem. The architecture has also been designed to accommodate the use of multiple UAVs, and future work on a team approach to the problem is presented. © 2006 Wiley Periodicals, Inc.
On the performance of proactive mobile IPv6 for context‐aware all‐IP wireless access networks
Authors: Pagtzis Theo , Crowcroft Jon , Clark Keith
Published: July 2006

A major challenge in building ‘all‐IP’ wireless access networks, besides the use of IP as the unifying layer, relates to the transparency of the IP handoff process as the mobile node (MN) transits across heterogeneous wireless network domains in IPv6 mobility management. Transparency in IP handoffs, however, must be effected in two separate contexts: IP‐addressing and (re‐)connection latency. Excessive delays during an IP handoff degrades the seamlessness of IP transmission between the MN and its peers.

Motivated by experimental results over heterogeneous wireless networks, we discuss why dynamic establishment of IP context‐state can help address these limitations that seem inherent in heterogeneous environments. To this end, we provide an in‐depth evaluation of Proactive Mobile IPv6 by means of simulations.

Our study contrasts the efficiency of proactive context‐state establishment, between candidate points of attachment (PoAs), against reactive MIPv6 standard practices over handoff delay, jitter and associated packet loss. Departing from reactive MIPv6 standards, this study brings new insights towards seamless handoffs by investigating the influential aspects of non‐determinism in the mobility pattern of the MN. In addition, it demonstrates quantitatively the performance benefit attained by maximising MN's service utility through PoA selectivity embedded in MN IPv6 handoff decision. Copyright © 2006 John Wiley & Sons, Ltd.
A simulation study of IP‐based vertical handoff in wireless convergent networks
Authors: Song Jung Kee , Wang Wenye
Published: July 2006

The advances in wireless networks and IP technologies has brought ubiquitous access to all‐IP information into reality. For wireless IP services, IP‐based handoff is a critical issue to the performance of application‐level services. Although mobile IP (MIP) and its extensions, as network layer solutions, have been proposed asde factostandard, transmission throughput degradation due to packet loss, registration delay, and transport layer blocking are unavoidable because of MIP handoff mechanisms. In this paper, we evaluate the performance of a transport layer handoff approach, mobile stream control transmission protocol (mSCTP), and compare it with that of a network layer solution, MIP. mSCTP is based on stream control transmission protocol (SCTP), which is the third general purpose transport layer protocol from IETF. We investigate the use of mSCTP forseamless vertical handoffwithout any change in IP protocol stack by its multi‐homing feature and dynamic address reconfiguration (DAR) extension. We evaluate the performance of mSCTP and MIP by introducing handoff delay, end‐to‐end transmission throughput, and packet loss, and verify our observations by a simulation study of the two protocols in UMTS/802.11b integrated networks using NS‐2 network simulator. Copyright © 2006 John Wiley & Sons, Ltd.
F‐PCM: a fragmentation‐based power control MAC protocol for IEEE 802.11 mobile ad hoc networks
Authors: Kim Dongkyun , Toh C. K.
Published: July 2006

A great deal of research has been directed toward reducing energy consumption at the 802.11‐based MAC layer for mobile ad hoc networks (MANETs). There exists an approach that uses maximum power during the RTS‐CTS exchange and computes the required amount of power in order for DATA and ACK messages to reach the receiver and sender. It, however, does not consider the existence of an interference range (IR), or often called a carrier sensing zone. In order to address the problem, an existing approach forces nodes, located within a sender's carrier sensing zone, to defer their transmission trials in order to avoid collisions at the sender. It, however, does not consider possible collisions at the receiver, which can result in frequent retransmissions and hence greater energy consumption. Therefore, we propose an efficient protocol called Fragmentation‐based Power Control MAC (F‐PCM), which utilizes the fragmentation mechanism of the IEEE 802.11 MAC protocol so as to avoid collisions at senders as well as those at receivers. Through extensive simulations, our F‐PCM has performed better in terms of providing a higher throughput and incurring lower energy consumption. This is particularly advantageous in a dense mobile network environment where collisions are more severe. Copyright © 2006 John Wiley & Sons, Ltd.
Blood‐brain barrier transport of amino acids in healthy controls and in patients with phenylketonuria
Authors: Knudsen G. M. , Hasselbalch S. , Toft P. B. , Christensen E. , Paulson O. B. , Lou H.
Published: July 2006

Blood‐brain barrier permeability to phenylalanine and leucine in four patients with phenylketonuria and in four volunteers was measured five times by the double‐indicator method at increasing plasma concentrations of phenylalanine. Based on the permeability‐surface area product (PS) from blood to brain (PS1) and on plasma phenylalanine levels, Vmax and the apparentKm for phenylalanine were determined.

Statistically significant relationships between plasma phenylalanine and PS1 were established in three out of four volunteers, the averageVmax value being 46.7 nmol/g per min and the apparentKm 0.328 mmol/L. Owing to saturation of the carrier, such a relationship could not be established in the patients.

In phenylketonuria, PS1 for phenylalanine and leucine decreased significantly by 55% and 46%, respectively. Transport from brain back to blood, PS2, decreased significantly and cerebral large neutral amino acid net uptake was generally decreased in patients with phenylketonuria.

In conclusion, the transport ofl‐phenylalanine across the human blood‐brain barrier follows Michaelis‐Menten kinetics. In phenylketonuria, brain permeability to large neutral amino acids is reduced by about 50% and net uptake appears decreased.
Non‐ketotic hyperglycinaemia presenting as pachygyria
Authors: Fletcher J. M. , Bye A. M. E. , Nayanar V. , Wilcken B.
Published: July 2006

A 2‐day‐old infant with lethargy and hypoventilation had pachygyria and agenesis of the corpus callosum on CT scand. Increased concentrations of glycine in plasma and CSF, together with an increased CSF/plasma ratio, confirmed a clinical diagnosis of non‐ketotic hyperglycinaemia. This is the first report of pachygyria in this disorder, although agenesis of the corpus callosum is well recognized, and non‐specific gyral malformations have been described previously. The specific diagnosis of an inborn error of metabolism in infants with structural brain malformations is of critical importance for accurate genetic counselling.
Identification ofN‐acetyl‐S‐(3‐oxo‐3‐carboxy‐n‐propyl)cysteine in the urine of a patient with cystathioninuria using LC/APCI‐MS
Authors: Zhang J. , Masuoka N. , Ubuka T. , Sugahara K. , Kodama H.
Published: July 2006

A new cystathionine metabolite has been identified in the urine of a patient with cystathioninuria using liquid chromatography‐mass spectrometry with an atmospheric pressure chemical ionization interface system (LC/APCI‐MS). By this method a very intense quasi‐molecular ion was observed as a base peak of syntheticN‐acetyl‐S‐(3‐oxo‐3‐carboxy‐n‐propyl)cysteine (NAc‐OCPC). The quasimolecular ion [M+H]+ of NAc‐OCPC observed in the urine of a patient with cystathioninuria was the same as that of the authentic compound (m/z 264). The retention time andRf value on paper chromatography of the synthetic compound were the same as those of the urinary compound from the patient with cystathioninuria. From these results, this new cystathionine metabolite was identified asN‐acetyl‐S‐(3‐oxo‐3‐carboxy‐n‐propyl)cysteine.
Clinical, biochemical, and molecular analysis of a maternally inherited case of Leight syndrome (MILS) associated with the mtDNA T8993G point mutation
Authors: Degoul F. , Diry M. , Rodriguez D. , Robain O. , François D. , Ponsot G. , Marsac C. , Desguerre I.
Published: July 2006

We report a new case of Leigh disease (subacute necrotizing encephalomyelopathy) in a girl with mitochondrial DNA (mtDNA) mutation in the ATPase6 gene at nucleotide position 8993. Sequence analysis of mtDNA revealed a T‐to‐G transversion at nucleotide position 8993 in the ATPase6 gene. Southern blot restriction analysis of patient muscle mtDNA showed only a mutant pattern for the mutation 8993. Molecular analysis of seven subjects from the family showed that except for the father they all carried the 8993 mtDNA mutation in all studied tissues, with high percentages in the two symptomatic children and even in one asymptomatic boy.
Elevated plasma chitotriosidase activity in various lysosomal storage disorders
Authors: Guo Yufeng , He Wang , Boer A. M. , Wevers R. A. , de Bruijn A. M. , Groener J. E. M. , Hollak C. E. M. , Aerts J. M. F. G. , Galjaard H. , van Diggelen O. P.
Published: July 2006

Recently a striking elevation of the activity of chitotriosidase, an endo β‐glucosaminidase distinct from lysozyme, was found in plasma from patients with Gaucher type I disease (McKusick 230800). Plasma chitotriosidase originates from activated macrophages and this elevation is secondary to the basic defect in Gaucher disease. To investigate the specificity of this phenomenon, we have investigated 24 different lysosomal storage diseases. In 11 different diseases increased chitotriosidase activity in plasma was found (in 28% of the patients). None of these diseases showed elevations as high as in Gaucher disease. Chitotriosidase was not significantly elevated in plasma from 20 different non‐lysosomal enzymopathies or in plasma from patients with infectious diseases associated with hepatomegaly. The results show that marked elevation of chitotriosidase activity in plasma appears to be specific for Gaucher disease. The data further suggest that elevated levels of chitotriosidase activity in plasma from patients with unexplained diseases may be indicative for a lysosomal disorder.
Allosteric activation of antithrombin is independent of charge neutralization or reversal in the heparin binding site
Authors: Langdown Jonathan , Carter Wendy J. , Baglin Trevor P. , Huntington James A.
Published: July 2006

We investigate the hypothesis that heparin activates antithrombin (AT) by relieving electrostatic strain within helix D. Mutation of residues K125 and R129 to either Ala or Glu abrogated heparin binding, but did not activate AT towards inhibition of factors IXa or Xa. However, substitution of residues C‐terminal to helix D (R132 and K133) to Ala had minimal effect on heparin affinity but resulted in appreciable activation. We conclude that charge neutralization or reversal in the heparin binding site does not drive the activating conformational change of AT, and that the role of helix D elongation is to stabilize the activated state.
Scaphotrapeziotrapezoid Joint Arthritis: A Pilot Study of Treatment with the Scaphoid Trapezium Pyrocarbon Implant
Authors: PEGOLI L. , ZORLI I. P. , PIVATO G. , BERTO G. , PAJARDI G.
Published: July 2006

Isolated scaphotrapeziotrapezoid osteoarthritis is rare but can cause weakness and wrist pain. We present the results of a pilot study of interposition arthoplasty with a scaphoid trapezium pyrocarbon implant (STPI) by an open and arthroscopic approach for this problem. We performed this procedure in 10 hands in eight patients, all women, with a mean age of 60.5 (range 51–70) years. The average follow-up was 19 (range 2–24) months. Pre-operative X-rays in all cases identified an isolated degenerative arthritis of the scaphotrapeziotrapezoid joint. Average DASH evaluation pre-operatively of 49 was reduced to 39. All of the patients had functional improvement and returned to daily activities after 3 months. The results of this pilot study suggest that the STPI may prove a useful compromise treatment for this problem.
A Comparison of Two Limited Open Techniques for Carpal Tunnel Release
Authors: ZYLUK A. , STRYCHAR J.
Published: July 2006

Review of the literature shows the effectiveness of limited open carpal tunnel release to be comparable to that of endoscopic carpal tunnel release in respect of recovery of grip strength, time of return to work and complication rate. A randomised, controlled study was designed to compare the effectiveness of a single versus a double limited open technique of carpal tunnel release. Sixty-five patients (73 hands) with a mean age of 48 years were operated on, 40 hands by the single incision and 33 by the double incision method. Grip and pinch strengths, digital sensibility (Filament and 2PD tests) and Levine scores were evaluated throughout 12 months of follow-up. We found that the single incision method offers better results in respect of grip and pinch strengths: less weakness at 1 month after surgery and a faster improvement relative to pre-operative values which is statistically significant. This, however, did not translate directly into Levine functional and symptom scores which, at each assessment, did not differ significantly between the two methods.
Watch This Space: Civil Liberties, Concept Wars and the Future of the Urban Fortress
Authors: BEWLEY-TAYLOR DAVID R.
Published: July 2006

On 20 September 2001 the founder and CEO of a New Jersey-based face-recognition technology company called Visionics testified before a special government committee appointed by the secretary of transportation. Joseph Atick's message to the committee was simple: his company's face recognition equipment could dramatically improve security in US airports and embassies. The science of biometrics, a method of identifying people by scanning unique physical characteristics like facial structures and retinal patterns, could, claimed Atick, be deployed as part of a comprehensive national surveillance plan that he called Operation Noble Shield. According to this plan the Office of Homeland Security might take the lead and liaise with local police forces to install cameras linked to a web-based biometric network throughout American cities. In order to protect America from further terrorist acts, Atick claimed, “We need to create an invisible fence, an invisible shield.” Members of the committee liked his ideas and seemed ready to endorse his recommendations.
Race and the Politics of Memory: Mark Twain and Paul Laurence Dunbar
Authors: FISHKIN SHELLEY FISHER
Published: July 2006

In an 1899 essay Mark Twain wrote,

It would not be possible for a humane and intelligent person to invent a rational excuse for slavery; yet you will remember that in the early days of the emancipation agitation in the North the agitators got but small help or countenance from any one. Argue and plead and pray as they might, they could not break the universal stillness that reigned, from pulpit and press all the way down to the bottom of society – the clammy stillness created and maintained by the lie of silent assertion – the silent assertion … that nothing is going on which fair and intelligent men are aware of and are engaged by their duty to try to stop.
Guantánamo, Eatonville, Accompong: Barbecue and the Diaspora in the Writings of Zora Neale Hurston
Authors: WARNES ANDREW
Published: July 2006

African American writers often express great affection for barbecue, a food many describe as “scrumptious” – to use a term that recurs throughout Bobby Seale's cookbook, Barbeque'n with Bobby (1988) – and invest with a particular capacity to lend shape and coherence to the idea of the African Diaspora. In the writings of Ntozake Shange, Albert Murray, Alice Walker, and others, barbecue seems able to reunite black communities, to gather together people dispersed across the USA or even the world. This literary use of barbecue is epitomized by Alice Walker's The Color Purple (1982), the concluding pages of which make the food central to a rejuvenating Diasporic optimism that stands in sharp contrast to the novel's desolate opening description of Celie's sexual abuse. Having grown up in West Africa, Adam, offspring of Celie's rape, finally meets his mother at a barbecue held in the American South. The food provides a conversation opener, a point of contact that the estranged family badly need:

Everybody make a lot of miration over Tashi. People look at her and Adam's scars like that's they business. Say they never suspect African ladies could look so good. They make a fine couple. Speak a little funny, but us gitting use to it.
A self-perpetuating bamboo disturbance cycle in a neotropical forest
Authors: Griscom Bronson W. , Ashton P. Mark S.
Published: July 2006

We investigate a hypothesis for explaining maintenance of forest canopy dominance: bamboo (Guadua weberbaueri and Guadua sarcocarpa) loads and crushes trees, resulting in a self-perpetuating disturbance cycle. Forest inventory data revealed a peculiar pattern of tree form and size class distribution in bamboo-dominated plots within the Tambopata River watershed, Madre de Dios, Peru. Bamboo disproportionately loaded trees 5–29 cm in diameter, and this size class had over seven times more canopy damage than trees in control plots without bamboo. These differences were accompanied by reduced tree basal area and tree density in the 5–29-cm-diameter size class in the presence of bamboo. Elevated tree canopy damage was not apparent for trees ≥30 cm dbh, which are beyond the reach of bamboo. Additional evidence for the impact of bamboo was revealed by an experiment using artificial metal trees. Artificial trees in bamboo-dominated forest plots had nine times higher frequency of physical damage and nine times more plant mass loading as compared with control plots. Our results support the hypothesis that bamboo loading causes elevated physical damage to trees and suppresses tree recruitment, particularly for trees 5–29 cm in diameter.
Phyllostomid bats of a fragmented landscape in the north-eastern Atlantic forest, Brazil
Authors: Faria Deborah
Published: July 2006

This paper addresses the effects of habitat fragmentation on the phyllostomid bats of the Atlantic rain forest in Brazil, by comparing community structure (species richness and capture frequency) and the frequency of three bat species sampled along 36 transects encompassing six habitat categories: interiors and edges of large (>1000 ha) and small fragments (<100 ha), and the surrounding matrix of second-growth forests and areas of shade cocoa plantation. Species composition, richness and total captures were not directly affected by forest size per se, although the frequency of one dominant forest species (Artibeus obscurus) was significantly lower in small fragments compared with larger ones. The high connectivity among forest patches in the study area and the ability of some species to use the surrounding matrix of secondary forests and shade cocoa plantations possibly precludes the insularization effect. Qualitative habitat changes induced by fragmentation, such as edge formation and forest regrowth affected bat community structure; both modified habitats comprised a limited subset of the species assemblage found in the interiors of mature forests. The results presented here provide evidence of impoverished bat assemblages in man-modified habitats linked with deforestation and overall disturbances related with forest fragmentation.
Differential seedling growth response to soil resource availability among nine neotropical tree species
Authors: Baraloto Christopher , Bonal Damien , Goldberg Deborah E.
Published: July 2006

Although the potential contribution to tropical tree species coexistence of niche differentiation along light gradients has received much attention, the degree to which species perform differentially along soil resource gradients remains unclear. To examine differential growth response to soil resources, we grew seedlings of nine tropical tree species at 6.0% of full sun for 12 mo in a factorial design of two soil types (clay and white sand), two phosphate fertilization treatments (control and addition of 100 mg P kg−1) and two watering treatments (field capacity and water limitation to one-third field capacity). Species differed markedly in biomass growth rate, but this hierarchy was almost completely conserved across all eight treatments. All species grew more slowly in sand than clay soils, and no species grew faster with phosphate additions. Only Eperua grandiflora and E. falcata showed significant growth increases in the absence of water limitation. Faster-growing species were characterized by high specific leaf area, high leaf allocation and high net assimilation rate but not lower root allocation. Slower-growing species exhibited greater plasticity in net assimilation rate, suggesting that tolerance of edaphic stress in these species is related more to stomatal control than to whole-plant carbon allocation. Although relative growth rate for biomass was correlated with both its physiological and morphological components, interspecific differences were best explained by differences in net assimilation rate across six of the eight treatments. A suite of traits including high assimilation and high specific leaf area maintains rapid growth rate of faster-growing species across a wide gradient of soil resources, but the lack of plasticity they exhibit may compromise their survival in the poorest soil environments.
Adapting research activity AHRC review of practice-led research
Authors: Till Jeremy , Mottram Judith , Rust Chris
Published: July 2006

In 2005 the Arts and Humanities Research Council initiated a review of practice-led research in art, design and architecture. The purpose of the review was to develop a ‘comprehensive map of recent and current research activity in the area’. What quickly became obvious to the team that won the bid to run the review (led by the three authors) was that to map activity one first had to attempt to define it. The term ‘practice-led research’ means many different things to different people and so immediately raises debate. The positions range from those who believe that the act of making or designing alone constitutes research, to those who believe that research (as analytical activity) is incommensurable with design (as synthetic activity). For the former, the knowledge contained within the artefact is self-evident and beyond the need for additional explication; for the latter, knowledge resides outside the artefact and in the realm of its dissemination and interpretation. The importance of the AHRC review is not that it will settle these arguments, but that it will provide a much firmer context in which to place them.
The cost of sustainability: Witheridge Primary School ‘Classroom of the Future’
Authors: Wigginton Michael
Published: July 2006

Analysis of the detailed design of a primary school classroom suggests that intelligent sustainable design can be both environmentally beneficial and cost effective.
The architecture of the well-serviced environment
Authors: Steiner Hadas A.
Published: July 2006

Following the lead of Buckminster Fuller’s experiments with the prefabricated, mechanical core, Archigram like the Smithsons, extended the service model to the domestic programme.
A gateway to the ‘backwoods’: Aalto and the matter of rooting modernity
Authors: Menin Sarah
Published: July 2006

Mediating between forest and garden, tradition and modernity, a small wooden gate at Alvar Aalto's Villa Mairea is shown to be deeply rooted in Finnish cultural memories.
Unrest at Campi Flegrei: A contribution to the magmatic versus hydrothermal debate from inverse and finite element modeling
Authors: Gottsmann J. , Folch A. , Rymer H.
Published: July 2006

We present results from the modeling of ground deformation and microgravimetric data recorded at Campi Flegrei in order to assess the causative phenomena of caldera unrest between 1981 and 2001. We find that residual gravity changes during ground uplift (1982–1984) are indicative of mass changes in a hybrid of magmatic and hydrothermal sources. During deflation between 1985 and 2001, the inversion of gravity residuals for a single source does not provide convincing results. We then performed the joint inversion of gravity and deformation data for multiple spherical sources and refined source parameters by finite element modeling in order to mitigate against limitations of the analytical solutions. The data recorded during inflation and rapid deflation may be best explained by mass and pressure changes in a deep magmatic source at about 5 km depth and a shallow (2 km deep) hydrothermal source. Both sources contribute equally to the gravity changes observed between 1982 and 1984; the contemporary uplift appears to be mainly caused by the shallow source. The subsequent deflation is dominated by a pressure decrease in the hydrothermal source; the magmatic source contributes chiefly to the observed gravity changes. Pressure and density variations within multiple shallow‐seated hydrothermal sources provide acceptable fits to the deflation and accompanying gravity changes recorded since 1988. These shallow level dynamics also appear to trigger spatially and temporarily random short‐term reversals of the overall mode of ground subsidence since 1985. Our analysis does not support the idea of magmatic contributions to these short‐lived periods of inflation.