bluuebunny/crossref_metadata_2025_split huggingface dataset:
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

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

AbstractAimsTo 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).MethodsExploratory 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).ResultsTOL 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).ConclusionsGreater 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

AbstractAimsInterstitial 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 MethodsA 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).ResultsIn 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.ConclusionsIC 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

AbstractTo 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

AbstractThe 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

AbstractGeneralized 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

AbstractThe 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

AbstractAtmospheric 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

SummaryOur 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

SummaryOrthotopic 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

SummaryRecent 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

SummaryOrnithine 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

SummaryA 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

SummaryWe 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

SummaryX‐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

SummarySerum 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

SummaryWe 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

SummaryMaternal 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.