bluuebunny/crossref_metadata_2025 huggingface dataset:
Did the invisible hand rock the cradle? An investigation of children's hospitalisations in New Zealand
Authors: Johnston Grant , Lynn Robert
Published: November 2004

Objectives

To test the hypothesis that the overall growth in children's hospitalisations since the instigation of New Zealand's economic and social reforms in 1984 reflects an increase in morbidity caused by socio-economic factors such as poverty, unemployment, household overcrowding and the cost of primary care.

Methods

All publicly funded hospitalisations amongst children aged 1-14 years, for the years 1988/89 to 2002/03, were categorised as either avoidable or unavoidable based on primary discharge diagnosis. Trends in avoidable and unavoidable hospitalisation rates over time and for various demographic and socio-economic groups were analysed.

Results

Growth in avoidable and unavoidable hospitalisations amongst children was similar over the period, with unavoidable hospitalisations growing slightly faster. Growth in avoidable hospitalisations was greatest for children who live in the least deprived areas and lowest for children who live in the most deprived areas. Making primary care substantially free for children aged less than six years did not appear to slow the growth in avoidable hospitalisations.

Conclusions

Trends in avoidable and unavoidable hospitalisations over time are not consistent with the hypothesis being tested. The available evidence does not support a link between morbidity attributable to socio-economic factors and growth in children's hospitalisations. Given the lack of other evidence for deterioration in overall child health, the available evidence does not support a link between economic and social reforms in New Zealand and a decline in children's health status.
Analysis of inflammatory response in human plasma samples by an automated multicapillary electrophoresis system
Authors: Larsson Anders , Hansson Lars-Olof
Published: November 2004

A new automated multicapillary zone electrophoresis instrument with a new high-resolution (HR) buffer (Capillarys™ with HR buffer) for analysis of human plasma proteins was evaluated. Albumin, α
Pre-Soaked Lightweight Aggregates as Additives for Internal Curing of High-Strength Concretes
Authors: Kovler K , Souslikov A , Bentur A
Published: December 2004

Self-desiccation and autogenous shrinkage of high-strength concretes is one of their drawbacks which cannot be readily accommodated by conventional curing. The concept of internal curing using lightweight soaked aggregates, to provide internal reservoirs of water which enable uniform curing of the whole cross-section, has been advanced by several groups. The present study is intended to develop this approach further, by optimizing the porosity and size of the aggregates to enable successful internal curing with only a small amount of aggregates, which could be viewed as additives rather than bulk replacement of conventional aggregates. The approach taken was to increase the porosity of the aggregates and reduce their size, to obtain a system with numerous internal reservoirs of sufficiently small spacing to allow water to be readily discharged from the aggregate and transported over the whole range of the matrix. It was shown that aggregates with porosities of about 50% by volume, a size of a few millimeters, and contents of less than 50 kg/m3 could provide full elimination of autogenous shrinkage in concretes having w/cm as low as 0.25, with only a small affect on strength. The parameters controlling the efficiency of the aggregates were assessed, indicating that their pore structure is the most important one, and that water from within the aggregates can be readily transported into the matrix to a distance of few millimeters.
Modeling Drying Shrinkage Stress Gradients in Concrete
Authors: Grasley ZC , Lange DA
Published: December 2004

The potential for drying shrinkage cracking in concrete is related, in part, to the development of a moisture gradient across the cross-section of the concrete element. A model has been developed that incorporates experimental measurements of internal relative humidity to investigate drying shrinkage stress gradients in concrete specimens. The experimental and analytical procedure for using the model is outlined. Initial research involving application of the model has indicated that there is a relationship between drying stress gradient severity and the time to cracking under full restraint. Further development of the model could involve combination with fracture models to investigate microcrack formation and propagation.
Cement and Superplasticizer Combinations: Compatibility and Robustness
Authors: Nkinamubanzi P-C , Aïtcin P-C
Published: December 2004

This study highlights the notion of robustness of combinations of cements and superplasticizers. Tests done with various cements and different families of superplasticizers showed that although a combination of a cement and a superplasticizer could be compatible, it is not necessarily robust. Sometimes a little variation in the dosage of the admixture could lead to detrimental side effects, such as segregation, excessive set retardation, or excess air content in the concrete. Results showed that the chemical composition of the cement is critical to ensure good compatibility and adequate robustness of various combinations of cements and superplasticizers. The chemical nature of the superplasticizer also plays a role in the behavior of such combinations. The C3A content, the soluble (alkali) sulphate content, and the fineness of the cement, which influence the adsorption rate of the superplasticizers on the cement particles, are among the key factors that control the compatibility and the robustness of cement-superplasticizer combinations, especially for polysulfonated admixtures. Based on the results of this study, a rough prediction of the compatibility and robustness of cements and superplasticizers could be made by analyzing the chemical composition of the cement and the chemical nature of the superplasticizer.
Using Electrical Conductivity to Estimate Properties of Mineral Slurries Used in Drilled Shaft Construction
Authors: Abichou T , Tawfiq K , Abdelrazig Y
Published: December 2004

An investigation was performed to study the relationships between properties of slurries used in drilled shaft construction. Slurries with different bentonite and attapulgite concentrations, densities, and sand contents were prepared. The Marsh Funnel viscosity, Brookfield viscosity, and electrical conductivity of each prepared sample of slurry were measured. Tests on slurries containing no sand showed that the Marsh Funnel viscosity, the Brookfield viscosity, and the density correlated very well to electrical conductivity because properties of slurries are acquired due to the presence of suspended clay particles, which can be directly correlated to electrical conductivity. The effects of sand content on viscosity and electrical conductivity were investigated. Marsh Funnel viscosity increased slightly with increased sand content. The Brookfield viscosity also showed some variation with sand content. The electrical conductivity increased with increasing clay content. As sand is added to the slurry, the electrical conductivity decreased slowly since the sand particles are inert as compared to the clay particles. The response of electrical conductivity to clay content and sand content was used to develop calibration curves to estimate Marsh Funnel viscosity and sand content as a function of density and electrical conductivity. Few techniques are now available to measure the density of slurry as it varies in the trench. If electrical conductivity is also measured, the viscosity and the sand content at any location of the trench can be estimated. This will improve the QA/QC of slurry construction.
Development and Qualification of an Algorithm for the Determination of the Initial Linear Portion of a Force Versus Displacement Record
Authors: Scibetta M , Schuurmans J
Published: December 2004

In mechanical tests, the force versus displacement record generally contains an initial linear elastic portion. In the analysis, the accurate determination of this linear part is essential for the identification of characteristic values of force and/or plastic displacement. In this paper, an algorithm for the determination of this linear portion is presented. This algorithm is qualified on several experimental curves and on virtual data sets generated by a computer program. Results demonstrate that the algorithm is very robust and is able to determine the linear portion accurately.
Demise of the Sepaade Tradition: Cultural and Biological Explanations
Authors: Roth Eric Abella
Published: November 2004

In an attempt to find common theoretical and methodological ground between anthropological demography and human evolutionary ecology, this article analyzes and interprets one recent event, the termination of the sepaade tradition among Rendille pastoralists of northern Kenya. Long described in the anthropological literature as an example of cultural population regulation, the sepaade tradition ended in 1998. Previously collected qualitative and quantitative data were analyzed to assess the various rationales for the demise of the tradition. Qualitative data link its termination to changing group morals, while quantitative data point to the loss of individual‐level benefits as the underlying rationale. Combining such seemingly disparate interpretations highlights common research interests for anthropological demography and human evolutionary ecology. [anthropological demography, human evolutionary ecology, culture change]
Proto‐Uto‐Aztecan: A Community of Cultivators in Central Mexico?
Authors: Hill Jane H.
Published: November 2004

Authorities on the origin and history of Uto‐Aztecan have held that speakers of the protolanguage were foragers who lived in upland regions of Arizona, New Mexico, and the adjacent areas of the Mexican states of Sonora and Chihuahua about 5,000 years ago. New lexical evidence supports a different view, that speakers of the protolanguage were maize cultivators. The Proto‐Uto‐Aztecan speech community was probably located in Mesoamerica and spread northward into the present range because of demographic pressure associated with cultivation. The chronology for the spread and differentiation of the family should then correspond to the chronology for the northward spread of maize cultivation from Mesoamerica into the U.S. Southwest, between 4500 and 3000 B.P. [Uto‐Aztecan, cultivation, Mesoamerican, historical linguistics, migration]
Ethnic Diversity and Its Environmental Determinants: Effects of Climate, Pathogens, and Habitat Diversity
Authors: Cashdan Elizabeth
Published: November 2004

This article documents, and seeks to explain, the geographical patterning in ethnic group distributions. Some areas, chiefly equatorial regions and areas of high habitat diversity, are crowded with a large number of named groups. Elsewhere, people over a large area consider themselves members of a single group. Using three new codes for the Standard Cross‐Cultural Sample (ethnic diversity, habitat diversity, and rainfall variation), I show that regions with relatively few ethnic groups (low ethnic diversity) have unpredictable and highly variable climates and low pathogen loads. In most areas there was no relationship between ethnic diversity and ecosystem productivity, and there was little or none with the chief determinants of productivity, mean annual rainfall and temperature. Habitat diversity was also associated with ethnic diversity, particularly among nonstratified societies. Habitat diversity is correlated with degree of topographic relief, but the effect of habitat diversity on ethnic diversity is larger than, and independent of, the effect of topography. [ethnic diversity, ecological anthropology, spatial organization, cross‐cultural research]
A Cartography of Cognitive and Non‐Cognitive States of Consciousness
Authors: Fischer Roland
Published: November 2004

A theory of consciousness is proposed which integrates much of what we know about the evolution and functioning of the human brammmd. The organism constructs its world of experience as an adaptation to the problem of moving in the world. The relationship between the observed and unobserved world is discussed. A cartography of state‐bound meaning is described in which the continuum of arousal states is linked to different states of consciousness. The inevitable ambiguity of perception is addressed and the evolutionary and developmental importance of self‐awareness is emphasized. All of this is related to poststructuralist formulations.
The Gateway to Inner Space: Sacred Plants, Mysticism and Psychotherapy
Authors: Baker John R.
Published: November 2004

The Gateway to Inner Space: Sacred Plants, Mysticism and Psychotherapy. Festschrift in Honor of Albert Hofmann. edited by Christian Rätsch, Bridport and Dorset, England: Prism Press. 1989. 258 pp. ISBN 1‐85327‐037‐7. $10.95 (paper).
Work‐Oriented Design of Computer Artifacts
Authors: Hakken David
Published: November 2004

Work‐Oriented Design of Computer Artifacts. Pelle Ehn Stockholm: Almqvist and Wiksell International for Arbetslivcentrum, 1988. 496 pp.
The "New Urban History" Where Is It Now?
Authors: Tilly Louise A.
Published: November 2004

Hypothesizing a trend from structural to more cultural approaches to the urban past in American and European historical writing, the author discovered to the contrary that social historians have consistently seen the subjects of urban his' wry as a place, a population, and the social‐cultural relations between and among individuals, families, institutions, and place. Urban historians in the 1990s, however, have moved towards subjects not easily treated with a purely structural approach. [Culture, urban ethnography, urban history, anthropological theory, urban migration]
The Health Divide
Authors: Berkman Lisa F.
Published: November 2004

The United States is one of the world's wealthiest nations, yet the health of average Americans lags behind that of citizens in other developed countries. The huge amounts we spend on health care are not buying our population good health. The reason is a widening gap between the health of rich and poor Americans.
Highly Selective All‐Plastic, Disposable, Cu2+‐Selective Electrodes
Authors: Michalska Agata , Ocypa Marcin , Maksymiuk Krzysztof
Published: November 2004

Conducting polymers (CP) remain a promising material to construct stable potential all‐solid‐state ion‐selective potentiometric electrodes. The unique properties of poly(3,4‐ethylenedioxythiophene) doped with poly(4‐styrenesulfonate) ions, PEDOT‐PSS: high CP stability and affinity of doping anions towards Cu2+ ions, make it highly attractive for construction of all‐solid‐state copper(II)‐selective electrodes with outstanding selectivity. The additional benefits can arise from solution processability of commercially available PEDOT‐PSS system. This material was highly promising for a new sensor arrangement, i.e. to obtain disposable, planar and flexible all‐plastic Cu2+‐selective electrodes. These sensors can be obtained by casting a commercially available dispersion of PEDOT‐PSS (Baytron P) on a plastic, non‐conducting support material. The CP being both electrical lead and ion‐to‐electron transducer, was covered with plastic, solvent polymeric Cu2+ selective membrane. This extremely simple arrangement, after conditioning in dilute Cu2+ solution, was characterized with linear Nernstian responses within the activities range from: 0.1 to 10−4 M, followed by super‐Nernstian responses for lower activities. The latter result points to effective elimination of primary ions leakage from the plastic membrane / transducer phase and has resulted in significantly improved selectivities. Obtained log K$\rm{ {_{Cu,J}^{pot}}}$ values were equal to −7.6 for Co2+, −7.4 for Zn2+, −7.2 for Ca2+ and −6.8 for Na+, respectively.
Enzymatic synthesis of tritium‐labelled isotopomers of histamine
Authors: Panufnik E. , Kański R. , Kańska M.
Published: November 2004

Three tritium‐labelled isotopomers of histamine (HA) have been synthesized using combined chemical and enzymatic methods. In the first step the tritium‐labelled isotopomers of histidine have been obtained by catalysed exchange with tritiated water. These intermediates have been converted into [1S‐3H]‐HA, [1R‐3H]‐HA and [2′,4′,1S,‐3H3]‐HA using the enzyme histidine decarboxylase (HDC, EC 4.1.1.22) from Lactobacillus 30a. Copyright © 2004 John Wiley & Sons, Ltd.
Fluorine‐18 labelling of PNAs functionalized at their pseudo‐peptidic backbone for imaging studies with PET
Authors: Kuhnast B. , Hinnen F. , Hamzavi R. , Boisgard R. , Tavitian B. , Nielsen P. E. , Dollé F.
Published: November 2004

Peptide nucleic acids (PNAs) form a unique class of synthetic macromolecules, originally designed as ligands for the recognition of double‐stranded DNA, where the deoxyribose phosphate backbone of original DNA is replaced by a pseudo‐peptide N‐(2‐aminoethyl)glycyl backbone, while retaining the nucleobases of DNA. We have previously developed an original method to label oligonucleotide‐based macromolecules with the short‐lived positron‐emitter fluorine‐18 (t1/2: 109.8 min) using the N‐(4‐[18F]fluorobenzyl)‐2‐bromoacetamide reagent. Using this method, we herein report the fluorine‐18‐labelling of 13 decameric PNAs (OLP_1‐13), of the same sequence (CTCATACTCT), but presenting selected modification of the pseudo‐peptidic backbone at two or three of the thymine residues (positions 2, 5 and 8). Structural characteristics of these backbone modifications include either an amino acid side chain (L‐Lys, L‐Glu, L‐Leu and L‐Arg) or a glycosyl moiety (mannose, galactose, fucose, N‐Ac‐galactosamine and N‐Ac‐glucosamine) attached via an appropriate spacer. N‐(4‐[18F]fluorobenzyl)‐2‐bromoacetamide was synthesized in three radiochemical steps from 4‐cyano‐N,N,N‐trimethylanilinium trifluoromethanesulfonate and HPLC‐purified in 85–90 min (typical production: 3.7–4.8 GBq starting from a batch of 29.6–31.4 GBq of [18F]fluoride). Conjugation of the fluorine‐18‐labelled bromoacetamide reagent with the PNAs was performed in a mixture of acetonitrile and HEPES buffer (0.1 M, pH 7.9) for 10 min at 60°C and gave the corresponding pure labelled conjugated PNAs ([18F]c‐OLP_1‐13) after RP‐HPLC purification. The whole synthetic procedure, including the preparation of the fluorine‐18‐labelled reagent, provides up to 0.9 GBq (25 mCi) of HPLC‐purified [18F]c‐OLP_1‐13 in 160 min with a specific radioactivity of 45–65 GBq/µmol (1.2–1.7 Ci/µmol) at the end of synthesis starting from 29.6 to 31.4 GBq (800–850 mCi) of [18F]fluoride. Copyright © 2004 John Wiley & Sons, Ltd.
Inhibition of mitochondrial respiration by nitric oxide: Its role in glucose metabolism and neuroprotection
Authors: Almeida Angeles , Cidad Pilar , Delgado‐Esteban María , Fernández Emilio , García‐Nogales Paula , Bolaños Juan P.
Published: November 2004

There is an increasing body of evidence demonstrating that inhibition of cytochrome c oxidase by nitric oxide (NO) may be one more step in a signaling cascade involved in the physiologic regulation of cell functions. For example, in both astrocytes and neurons the inhibition of mitochondrial respiration by endogenously produced NO induces transient and modest decreases in cellular ATP concentrations. This mitochondrial impairment may serve as a cellular sensor of energy charges, hence modulating metabolic pathways, such as glycolysis, through AMP‐activated protein kinase (AMPK) in astrocytes. In neurons, the NO derivative peroxynitrite anion triggers signaling pathways leading to glucose oxidation through the pentose‐phosphate pathway to form reducing equivalents in the form of NADPH. The modulation of these metabolic pathways by nitric oxide or its derivatives may be important for understanding the mechanisms by which this free radical affects neuronal death or survival. © 2004 Wiley‐Liss, Inc.
Study of Grafting Reactions of Polar Groups onto Polystyrene (PS) by Reactive Mixing
Authors: Passaglia Elisa , Coiai Serena , Ricci Lucia , Ciardelli Francesco
Published: November 2004

Two different bulk modifications have been carried out aimed to the introduction of carboxyl groups into atactic polystyrene (PS) based on the reaction at 180°C with diethylmaleate (DEM) or 4‐carboxybenzensulfonyl azide (CBSA) respectively. The former system proceeds with a free radical mechanism giving functionalization around 0.5% by mol but with reduction of molecular weight and Tg increase. On the other side CBSA which grafts by addition to the C‐H bond gives appreciable functionalization with rather limited degradation and moderate Tg increase. Detailed analysis of the molecular structure of the functionalized PS was performed to propose possible reaction mechanisms.
Macromolecular Internal Viscosity. The Role of Stereoregularity
Authors: Allegra Giuseppe
Published: November 2004

Macromolecular dynamics at the scale of a few chain bonds is largely controlled by the “internal viscosity” effect if the energy barriers hindering the skeletal rotations are sufficiently large. In an extensive spin‐echo neutron scattering analysis, Richter and co‐workers (A. Arbe, M. Monkenbusch, J. Stellbrink, D. Richter, B. Farago, K. Almdal and R. Faust, Macromolecules, 2001, 34, 1281) investigated the dynamic properties of polyisobutylene (PIB) and polydimethylsulfoxide (PDMS) in toluene solution, the latter polymer being currently assumed to have very small rotational barriers. Analysis of the data according to a theory proposed by this author enabled them to obtain realistic values both of the rotational barrier around C‐C bonds (∼3kcal/mol) and of the natural frequency of the rotational jumps. ‐ A problem related to chain internal viscosity concerns the iso‐ and syndio‐tactic forms of polystyrene (respectively i‐PS and s‐PS): After a careful conformational analysis it is shown that i‐PS has very large effective energy barriers due to interactions between phenyl rings (S. Br&�uml;ckner, G. Allegra and P. Corradini, Macromolecules, 2002, 35, 3928). This effect is compounded with that of the intrinsic rotational barrier and helps explaining the kinetic difficulty to crystallise of i‐PS as compared with s‐PS.
The Role of the trans‐Planar Mesophase in the Polymorphic Behavior of Syndiotactic Polypropylene
Authors: Guadagno Liberata , Naddeo Carlo , D'Aniello Concetta , Vittoria Vittoria , Meille Stefano Valdo
Published: November 2004

The transformations of the trans‐planar mesophase of syndiotactic polypropylene (sPP) subjected to thermal, mechanical and solvent treatments, were investigated. The unoriented trans‐planar mesophase, obtained by quenching the melt at 0°C, was annealed at 80°C and the thermal transformation was investigated by X‐rays, infrared and dynamic‐mechanical analysis. The presence of the helical form II was recognized in the annealed sample. The oriented trans‐planar mesophase, obtained by drawing at room temperature and releasing the tension, was immersed in liquid dichloromethane for 24 hours. After drying the sample showed the presence of the oriented form II, although it was not possible to exclude a partial transition into form IV. On the basis of the present and literature results we suggested a scheme of the polymorphic transitions of sPP, in which the central role of the trans‐planar mesophase is enlightened.
Influence of Molecular Characteristics on Dielectric Relaxation of Propylene Glycol Oligomers
Authors: Prevosto Daniele , Capaccioli Simone , Lucchesi Mauro , Rolla Pierangelo
Published: November 2004

The relaxation dynamics of polypropylene glycol oligomers having different end groups was studied by broadband dielectric spectroscopy (10 mHz to 10 GHz) in the temperature interval 110‐350 K, above and below the glass transition.?The effects produced on structural and secondary?relaxations by the different end groups are discussed.
Tension‐free vaginal tape: Do patients who fail to follow‐up have the same results as those who do?
Authors: Minassian Vatche A. , Al‐Badr Ahmed , Pascali Dante U. , Lovatsis Danny , Drutz Harold P.
Published: November 2004

Aims

To compare success and complication rates of the Tension‐free vaginal tape (TVT) between patients with good versus poor follow‐up.

Materials and Methods

A prospective cohort study of 108 women undergoing a TVT procedure was conducted. Patients were seen postoperatively at 6 weeks, 3, 6, 12 months, and yearly thereafter. Patients were categorized as poor follow‐up if this schedule was not adhered to. Those who were lost to follow‐up at or after their 6‐week visit were considered as having failed the procedure.

Results

Seventy‐nine (73%) patients had good follow‐up. Of the remaining 29 patients with poor follow‐up, 12 (11%) could not be reached and 17 (16%) were contacted by phone. Reasons given for poor follow‐up were: busy or live far from hospital (11), health problems (4), and dissatisfied from surgery (2). Perioperative complication rates were similar between the two groups (P = 0.16). When patients with complete loss to follow‐up were analyzed as failures, subjective and objective cure rates were significantly higher in patients with good as opposed to poor follow‐up: 92 and 95% versus 72 and 69%, respectively, (P = 0.006).

Conclusions

Patients with poor follow‐up probably have lower cure rates after TVT. It is important to follow postoperative patients closely. When reporting success rates, one has to account for all cases to produce realistic results. © 2004 Wiley‐Liss, Inc.
“Unblinding” in randomized controlled drug trials for urinary incontinence: Implications for assessing outcomes when adverse effects are evident
Authors: DuBeau Catherine E. , Khullar Vik , Versi Eboo
Published: November 2004

Aims

To determine whether women with urinary incontinence (UI) can identify their allocation in a randomized controlled trial (RCT) of tolterodine (TOL), and whether correct identification is associated with outcomes and adverse effects (AEs).

Methods

Exploratory analysis of a randomized, double‐blind, placebo (PLC)‐controlled trial of TOL 4 mg daily for 8 weeks in 743 women with urge‐predominant mixed UI. Patient perception of their randomization was assessed at trial end. Main outcome measures were 7‐day bladder diaries, patient perception of improvement, and UI‐specific quality of life (QoL).

Results

TOL produced a significant decrease in urge UI episodes compared to PLC (78% vs. 51%, P = 0.0001). Fifty‐one percent of women correctly identified their randomization (58% on TOL vs. 37% on PLC, P < 0.001). Women who assumed they took TOL had better bladder diary outcomes than those who assumed they took PLC. Within each assumption group, patient perception outcomes were similar, regardless of actual randomization. QoL improved in all domains except general health for women on TOL. In women who assumed they took TOL, significant drug benefit was evident in three domains. Moderate‐severe dry mouth was higher in those who assumed they took TOL (7.3% vs. 0%, P < 0.0001).

Conclusions

Greater than fifty percent of women in this RCT of antimuscarinic treatment were “unblinded” to their randomization. Patient assumption of randomization was associated with bladder diary and perception outcomes, specific QoL domains, and dry mouth. Efficacy of urge incontinence drugs should be considered in the context of patient assumptions, expectations, and “unblinding” by easily evident side effects. © 2004 Wiley‐Liss, Inc.
Evaluation of urinary and general symptoms and correlation with other clinical parameters in interstitial cystitis patients
Authors: Porru Daniele , Tinelli Carmine , Gerardini Matteo , Giliberto Giovanni Luca , Stancati Salvatore , Rovereto Bruno
Published: November 2004

Aims

Interstitial cystitis (IC) has been deemed by some authors as a local manifestation of a systemic disease, particularly one of the autoimmune disorders. In order to provide an answer to the question whether IC patients do or do not indiscriminately report high scores for various somatic symptoms, we compared University of Wisconsin scores (U‐W scores), including both urinary and non‐urinary symptoms, for 30 IC female patients and 30 female controls.

Materials and Methods

A total of 30 female patients with IC and 30 healthy female controls were recruited for the study. All patients had to meet the symptom criteria established by the National Institute for Diabetes and Digestive and Kidney Diseases. Each patient completed a University of Wisconsin symptom scale, after translation (WICS).

Results

In the IC group median score was zero for all, but two of the reference symptoms: abdominal cramps and dizziness. All urinary symptoms were significantly increased in IC patients compared to controls. In the control group, the median value of urinary symptoms was zero. The duration of the disease was then related to IC symptoms in the patient group. Among the bladder‐related symptoms, a good correlation was found for several specific symptoms and urinary symptoms as a whole. An association could be found between a positive potassium sensitivity test (PST) and burning bladder sensation, as well as pelvic discomfort.

Conclusions

IC patients had significantly higher scores for the seven urinary symptoms compared to controls. The duration of disease was found to be correlated with the group of seven urinary symptoms. © 2004 Wiley‐Liss, Inc.
Preparation and proton conductivity of poly(N‐methylbenzimidazole) doped with acid
Authors: Pu Hongting , Liu Gaihua
Published: November 2004

To increase the solubility and film forming ability of polybenzimidazole (PBI), poly(N‐methylbenzimidazole) (PNMBI) with different degrees of methylation was synthesized. Chemical structure, degree of substitution, and solubility of PNMBI was studied. PNMBI is easier to be doped with acid than PBI. The basicity of PNMBI was improved with the introduction of methyl groups on the imidazole moiety. Effects of methylation degree, H3PO4 content and temperature on proton conductivity of PNMBI doped H3PO4 was studied. Proton conductivity of H3PO4 doped PNMBI‐1.2 membranes increases with increasing doping level. Temperature dependence of proton conductivity of H3PO4 doped PNMBI‐1.2 membranes follows the Arrhenius law. With an increase in the degree of substitution, proton conductivity of H3PO4 doped PNMBI decreases dramatically. The proton transport mechanism was also discussed. The proton conductivity of PNMBI/H3PO4 is mainly contributed by proton hopping or Grotthuss mechanism. Copyright © 2004 John Wiley & Sons, Ltd.
Determination of percolation threshold electroactivity and phase behavior study on conducting blends of thermotropic polyesters and polyaniline
Authors: Bagheri Massoumeh , Didehban Khadije , Razmi Habib , Entezami Ali Akbar
Published: November 2004

The new thermotropic polyester/polyaniline (PIn/PAni) blends have been prepared by solution blend of synthesized liquid crystalline poly[4,4′‐bis (ω‐alkoxy) biphenylisophthalate]s having four and six methylene units in spacer (PI4 and PI6) with PAni doped with camphorsolfonic acid (CSA). The percolation threshold electroactivity of prepared blend films has been determined by cyclic voltammetry. The effect of the PAni concentration, solvent nature and polyester structure on the electroactivity of the blends has been investigated. The extremely low percolation threshold of prepared PIn/PAni‐CSA blends from dimethylformamide (DMF) and m‐cresol solution was 3% weight of PAni‐CSA. The amount of conducting polymer necessary to retard the formation of the liquid crystalline (LC) phase is up to 45% by weight. Phase behavior studies by differential scanning calorimetry and polarizing microscopy show that blends with 45% of conducting polymer are both liquid crystal and conductive. The morphology of the blends has been investigated by scanning electron microscopy. Copyright © 2004 John Wiley & Sons, Ltd.
Generalized linear modelling in periglacial studies: terrain parameters and patterned ground
Authors: Luoto Miska , Hjort Jan
Published: November 2004

Generalized linear models (GLM) are mathematical extensions of linear models. GLM models are more flexible and better suited for analysing relationships of spatial data, which can often be poorly represented by classical Gaussian distributions such as least‐square‐regression techniques. This paper demonstrates GLM model‐building procedures step‐by‐step for the distribution and abundance of active patterned ground in northern Finland. The exercise is based on data from an area of 200 km2 (800 modelling squares of 0.25 km2). Both the distribution and abundance models clearly indicate an increasing activity of patterned ground with (1) increasing soil moisture and (2) proportion of concave topography. Activity decreases with increasing altitude. We conclude that GLM techniques combined with a geographic information system can play an important role in analysing and modelling periglacial data sets. Copyright © 2004 John Wiley & Sons, Ltd.
Interpretation of geothermal profiles perturbed by topography: the alpine permafrost boreholes at Stockhorn Plateau, Switzerland
Authors: Gruber Stephan , King Lorenz , Kohl Thomas , Herz Thomas , Haeberli Wilfried , Hoelzle Martin
Published: November 2004

The temperature regime of alpine permafrost is altered by the generally warmer atmospheric temperatures of recent decades. Eight boreholes, with depths between 100 and 130 m, have recently been drilled in European mountain permafrost as part of the Permafrost and Climate in Europe (PACE) project. They have been equipped with temperature sensors in order to better understand and quantify the effect of climate change on permafrost temperatures. Their interpretation with respect to signals of surface temperature histories is complicated by topographic effects. An apparent warming signal is present in all of the PACE boreholes but quantification of this effect in mountainous terrain remains difficult. The influence of topography and spatially‐variable surface temperatures on temperature‐depth profiles is demonstrated with measurements from the Stockhorn borehole and a simple model. A conceptual framework for the interpretation of topographically‐disturbed temperature‐depth profiles and the modelling of temperature histories based on these data is proposed. Copyright © 2004 John Wiley & Sons, Ltd.
Atmospheric pressure chemical ionisation liquid chromatography/multistage mass spectrometry for assignment of sedimentary bacteriochlorophyll derivatives
Authors: Wilson Michael A. , Hodgson Dominic A. , Keely Brendan J.
Published: November 2004

Atmospheric pressure chemical ionisation liquid chromatography/multistage mass spectrometry (APCI‐LC/MSn) provides a rapid, on‐line method for the assignment of individual bacteriophaeophorbide c and d methyl esters (BPMEs) in complex mixtures. The MS2 spectrum for each component is diagnostic of the type of BPME (c or d), and characteristic losses in MS5 and MS6 permit assignment of the alkyl substituents at positions C‐8 and C‐12 of the macrocycle. MS5 mass chromatograms permit the deconvolution of coeluting isobaric BPMEs, revealing the true profiles of the individual components. The distributions are different in lake sediments from la Salada de Chiprana (Spain) and Kirisjes Pond (Antarctica), and a novel BPME c with a neo‐pentyl substituent has been observed in the Kirisjes Pond sediment. Copyright © 2004 John Wiley & Sons, Ltd.
Adrenoleukodystrophy: natural history, treatment and outcome
Authors: Moser H. W.
Published: November 2004

Our laboratory has identified nearly 2000 patients with X‐linked adrenoleukodystrophy (ALD) and conducted therapeutic trials in groups of patients who represent the major phenotypes. We report recent results of dietary therapy with a mixture of glyceryl trioleate and glyceryl trierucate oil, also referred to as Lorenzo's Oil, in the asymptomatic and childhood cerebral phenotypes. Fifty‐three patients started this therapy at a mean age of 7.5 years at a time when they were free of neurological symptoms. Although analysis of data is hampered by the lack of a concurrent control group, follow‐up studies after 39 months of therapy suggest that subsequent neurological involvement was less frequent and less severe than anticipated from historical controls. Retrospective analysis of the effect of the oil in patients with the severe childhood cerebral phenotype indicates that there was a slight but statistically significant slowing of clinical progression and delay of death.
Liver transplantation in two cases of propionic acidaemia
Authors: Schlenzig J. S. , Poggi‐Travert F. , Laurent J. , Rabier D. , Jan D. , Wendel U. , Sewell A. C. , Revillon Y. , Kamoun P. , Saudubray J. M.
Published: November 2004

Orthotopic liver transplantation (OLT) was performed in two patients with propionic acidaemia, a 7‐year‐old boy and a 9‐year‐old girl, diagnosed with a severe neonatal form with high risk of metabolic decompensation. In both cases the metabolic liver functions recovered within the 12 postoperative hours; no clinical symptoms of propionic acid toxicity, metabolic acidosis, severe hyperammonaemia, hyperglycinaemia or haematological abnormalities were observed. In both cases insulin‐dependent diabetes mellitus occurred early after OLT (persisting in the boy's case). Severe post‐transplantation complications were observed (acute rejection and CMV infection in both patients) which did not trigger metabolic decompensation. The boy developed chronic rejection and vanishing bile duct syndrome due to incomplete hepatic arterial thrombosis. He required permanent in‐patient care with chronic hyperammonaemia and neurological sequelae involving the basal ganglia and died 15 months after OLT. The girl left hospital after 2 months and is presently leading a normal life with almost no dietary protein restriction (40g protein per day). Urinary urea excretion and daily protein intake increased after liver transplantation. Propionyl‐ and tiglylglycine disappeared immediately after OLT. Urinary methylcitrate and 3‐hydroxypropionate remained at concentrations corresponding to those before OLT. However, the total of all characteristic metabolites of organic acid analysis was reduced to 50–60% of the values before OLT in both patients. Propionylcarnitine was still detected at significant concentrations. Plasma odd‐chain fatty acid concentrations decreased continuously after OLT only in the girl's case. Tissue of both transplanted livers showed increased odd‐chain fatty acid concentrations 9 and 15 months after OLT, respectively, in both patients. We consider that at present OLT should only be performed in severe forms of propionic acidaemia.
Gene therapy for cystic fibrosis
Authors: Alton E. W. F. W.
Published: November 2004

Recent studies have identified the underlying molecular defect in cystic fibrosis (CF). Reduced or absent cAMP‐mediated chloride transport in epithelial‐lined organs characterizes this disease. With the identification of the CF gene, gene therapy has become a potential novel form of treatment for this disease. This article reviews the rapid progress in CF research from the understanding of the bioelectric defect to the recently begun human gene therapy trials.
Identification of two new aberrant splicings in the ornithine carbamoyltransferase (OCT) gene in two patients with early and late onset OCT deficiency
Authors: Matsuura T. , Hoshide R. , Komaki S. , Kiwaki K. , Endo F. , Nakamura S. , Jitosho T. , Matsuda I.
Published: November 2004

Ornithine carbamoyltransferase (OCT) is a liver‐specific enzyme located in the mitochondrial matrix. OCT deficiency is an X‐linked disease with a heterogeneous phenotype, even in affected males. We studied two male patients (K.M., K.G.) with early and late onset, respectively. OCT activity was zero in the autopsied liver of patient K.M. and was 6% of control in the biopsied liver of K.G. Sequencing of OCT cDNAs revealed exon 5 skipping in K.M., resulting from a T‐to‐C transition of the initial dinucleotide of the 5′ splicing donor site of intron 5, and a G‐to‐T transversion at position +45 in exon 9 (L304F) in K.G., providing three OCT mRNAs of different lengths: a normally spliced transcript, 23 bp insertion of intron 8 and the first 50bp missing within exon 9. Exon 5 skipping and two other aberrant splicings produced stop codons early downstream in mature OCT mRNAs. Expression study of a missense allele, L304F, transfected to cultured Cos 1 cells revealed a 34.4% value of the control. The difference of OCT activities between the patient liver and transfected cells (6% vs. 34%) can be explained by this splicing abnormality.
Defective molybdopterin biosynthesis: clinical heterogeneity associated with molybdenum cofactor deficiency
Authors: Mize C. , Johnson J. L. , Rajagopalan K. V.
Published: November 2004

A patient with molybdenum cofactor deficiency (producing the biochemical abnormalities associated with deficiencies of sulphite oxidase and xanthine dehydrogenase) clinically expressed Marfan‐like habitus with dislocated lenses, vertebral abnormality, learning disability, moderate hemiplegia, increased medial lentiform MRI signal and intermittent microscopic haematuria.S‐Sulphocysteine was present in plasma and urine, and the oxidized derivative of a molybdopterin precursor (precursor Z), together with xanthine and hypoxanthine, were elevated in urine. Blood uric acid was <1 mg/dl, while urinary urothione was not detected. These data indicate a functionally inadequate terminal enzyme for converting precursor Z to active molybdopterin (complementation group B of general molybdenum cofactor deficiency). Although the biochemical parameters were indicative of a severe deficiency state, the patient has survived into the third decade with a less severe clinical spectrum than has generally been associated with this disease.
Prenatal monitoring of ornithine transcarbamoylase deficiency in two families by DNA analysis
Authors: Matsuura T. , Hoshide R. , Fukushima M. , Sakiyama T. , Owada M. , Matsuda I.
Published: November 2004

We examined the DNA in two families with ornithine transcarbamoylase (OTC) deficiency. Two point mutations of the OTC gene, a C‐to‐T (codon 141) and a G‐to‐A (codon 141), were identified. This allowed prenatal monitoring to be made for two fetuses in each family, using polymerase chain reaction (PCR), followed by allele‐specific oligonucleotide hybridization orTaqI digestion of amplified sequence. The diagnoses showed heterozygotes of a wild type gene and the corresponding mutant gene in these fetuses; each was confirmed postnatally.
Characterization of X‐linked adrenoleukodystrophy in different biological specimens from ten Portuguese families
Authors: Jorge P. , Quelhas D. , Nogueira A.
Published: November 2004

X‐linked adrenoleukodystrophy (ALD) is a severe neurodegenerative disease characterized by progressive demyelination, adrenocortical insufficiency and accumulation in tissues and body fluids of unbranched, saturated very long‐chain fatty acids (VLCFA). The diagnosis of ALD is usually based on clinical history, neurological examination and the determination of levels of VLCFA in plasma and cultured skin fibroblasts.

In the present paper we report the biochemical findings in plasma, cultured skin fibroblasts and lymphoblastoid cell lines from ALD patients. The results obtained indicate that the increment of the ratios C24:0 to C22:0 and C26:0 to C22:0 and of the concentration C26:0 (µg/ml) in plasma was parallel with that of fibroblasts, but not with that of Epstein‐Barr virus (EBV)‐transformed lymphocytes, suggesting that this cell line is not reliable for diagnosis of ALD by VLCFA analysis.

Subsequent studies carried out on family members revealed heterozygotes other than obligate carriers and hemizygotes who were pre‐symptomatic or had a misdiagnosis of multiple sclerosis or psychosis.
Controlled diet in phenylketonuria may cause serum carnitine deficiency
Authors: Vilaseca M. A. , Briones P. , Ferrer I. , Campistol J. , Riverola A. , Castillo P. , Ramon F.
Published: November 2004

Serum carnitine levels may be reduced in patients with phenylketonuria (PKU) owing to low carnitine intake, deficient carnitine synthesis and acylcarnitine production from phenylalanine metabolites. In order to investigate the possible carnitine deficiency we determined serum carnitine in its different forms and the precursors and cofactors involved in its synthesis in a group of patients with PKU or hyperphenylalaninaemia. Free, total and acylcarnitine values were significatively reduced only in PKU patients with Phe‐restricted diet which had not been supplemented with carnitine. Acylcarnitine/free carnitine ratio and all the other parameters studied were normal in all patients. We conclude that the low serum carnitine levels in PKU patients with a strict diet are a consequence of the low carnitine intake.
Plasma phenylalanine and tyrosine levels revisited in heterozygotes for hyperphenylalaninaemia
Authors: Saraiva J. M. , Seakins J. W. T. , Smith I.
Published: November 2004

We examined the value of the fasting plasma phenylalanine/tyrosine ratio obtained in an ordinary clinical setting for assessing the probability of being a heterozygote for hyperphenylalaninaemia. This biochemical test was found to be of little value in those with a high (66%) prior risk of heterozygosity, because it could not reduce the risk below 12%. However, in a population with a prior risk of only 2%, it discriminates the 3% with a 19% risk from the 97% with a risk of 1.5% or less. This simple method could usefully be applied to such a population, in order to select those at higher risk for further investigation using molecular genetics.
The use of gelatin capsules for ingestion of formula in dietary treatment of maternal phenylketonuria
Authors: Kecskemethy H. H. , Lobbregt D. , Levy H. L.
Published: November 2004

Maternal phenylketonuria (PKU) represents a high risk for birth defects, including mental retardation, in offspring. Thus, it could cancel gains represented by the prevention of PKU‐induced mental retardation in the current generation. Effective dietary treatment of maternal PKU pregnancies could avoid this potentially tragic occurrence. However, dietary compliance is often difficult because a necessary component of the diet, medical nutritional formulas, often have an unpleasant taste and odour. We treated the second pregnancy of a phenylketonuric women who had required extended hospitalization during her first pregnancy because of poor formula tolerance, and who had similar difficulty in the second pregnancy. To alleviate this problem, we developed a system whereby she could pack the formula into gelatin capsules for ingestion. Packing and ingestion of 20 capsules required less than 30 minutes three times a day. With capsules her blood phenylalanine level was almost always within the recommended range of 120–360 µmol/L (2–6 mg/dl) and hospitalization was not required. The phenylalanine content of the capsules was easily accommodated by a small reduction in allowable food. Other amino acid levels, including tyrosine and other essential nutrient levels, were normal. We believe that using gelatin capsules for formula ingestion can be very beneficial in the management of maternal PKU pregnancies and could be extended to the dietary treatment of other inborn errors of metabolism.
Signals on proteins, intracellular targeting and inborn errors of organellar metabolism
Authors: Tager J. M. , Aerts J. M. F. G. , van den Bogert C. , Wanders R. J. A.
Published: November 2004

Newly synthesized polypeptides contain signals that direct them to the appropriate intracellular organelles and the organelles contain receptors that recognize the signals. Protein synthesis occurs either on free ribosomes or on ribosomes bound to the endoplasmic reticulum. The proteins synthesized on bound ribosomes are co‐translationally translocated into the lumen of the endoplasmic reticulum and contain or acquire targeting information for retention in the endoplasmic reticulum or for sorting to lysosomes and other compartments of the secretory and endocytic pathways. Proteins synthesized on free ribosomes remain in the cytosol or contain signals for import into the nucleus, mitochondria or peroxisomes. The nature of the targeting signals and the mechanisms of import are discussed briefly. Examples are given of inborn errors of metabolism caused by incorrect or impaired incorporation of proteins into mitochondria, lysosomes or peroxisomes.