bluuebunny/crossref_metadata_2025_split huggingface dataset:
Two siblings with 46,XY DSD, congenital adrenal hypoplasia, aniridia, craniofacial, and skeletal abnormalities and intrauterine growth retardation: A new syndrome?
Authors: Coman David J. , White Susan M. , Amor David J. 
Published: August 2007

AbstractWe report on two siblings with an unusual constellation of congenital anomalies comprising 46,XY disorder of sex development (DSD), congenital adrenal hypoplasia, aniridia, dysmorphic facial features, intrauterine growth retardation, and minor skeletal abnormalities. This combination of abnormalities is yet to be recognized in the medical literature. As such, we propose that our patients represent either a new dysmorphic syndrome or a thus far unrecognized variation of a known syndrome, such as IMAGe syndrome. The sibling recurrence suggests autosomal recessive or X‐linked patterns of inheritance. © 2007 Wiley‐Liss, Inc.
Parenting children with Proteus syndrome: Experiences with, and adaptation to, courtesy stigma
Authors: Turner Joyce , Biesecker Barbara , Leib Jennifer , Biesecker Leslie , Peters Kathryn F. 
Published: August 2007

AbstractCourtesy stigma refers to the stigmatization an unaffected person experiences due to his or her relationship with a person who bears a stigma. Parents of children with genetic conditions are particularly vulnerable to courtesy stigma, but little research has been done to explore this phenomenon. The purpose of this study was to investigate the courtesy stigma experiences of parents of children with Proteus syndrome (PS) and related overgrowth conditions. Thematic analysis of transcripts from 31 parents identified three distinct themes: stigma experiences, social‐emotional reactions to stigmatizing encounters, and coping responses. Four types of stigmatizing experiences were identified: intrusive inquires, staring and pointing, devaluing remarks, and social withdrawal. Additionally, we uncovered eight strategies parents used to cope with courtesy stigma: attributing cause, assigning meaning to social exchanges, concealing, withdrawing socially, taking the offensive, employing indifference, instructing and learning from family, and educating others. Parents' choices of strategy type were found to be context dependent and evolved over time. This is the first study to document the adaptive evolution of coping strategies to offset courtesy stigma by parents of children with genetic conditions. These results provide groundwork for genetic counseling interventions aimed at addressing issues of courtesy stigma and further investigation of the phenomenon itself. Published 2007 Wiley‐Liss, Inc.
Use of palm trees as a sleeping site for hamadryas baboons (Papio hamadryas hamadryas) in Ethiopia
Authors: Schreier Amy , Swedell Larissa 
Published: August 2007

AbstractHamadryas baboons sleep on cliffs throughout their range, and this can be attributed to the safety cliffs provide against predators in the absence of tall trees. In this paper, we report the first documented occurrence of hamadryas baboons sleeping in doum palm trees rather than on cliffs. Data derive from a study of hamadryas baboons at the Filoha site in lowland Ethiopia. During all‐day follows, data were collected on travel patterns, band activity, and location. Variation in the baboons' home range was characterized using vegetation transects. We discovered that one band in this population, Band 3, occasionally slept in doum palm trees (Hyphaene thebaica). The palm tree sleeping site differed from other palm fragments in the baboons' home range in that it contained a higher density of palm trees. Possible factors influencing this unique use of palm trees as a sleeping site include access to palm fruit, avoiding contact with Afar nomads, avoiding sharing sleeping cliffs with other bands, protection from predators, and the lack of cliffs in a section of the baboons' home range. Evidence from this study suggests that the palm tree sleeping site is used because it affords better protection from predators than other palm fragments in an area of the band's home range that does not contain cliffs. Am. J. Primatol. 70:107–113, 2008. © 2007 Wiley‐Liss, Inc.
Friedreich's ataxia: clinical pilot trial with recombinant human erythropoietin
Authors: Boesch Sylvia , Sturm Brigitte , Hering Sascha , Goldenberg Hans , Poewe Werner , Scheiber‐Mojdehkar Barbara 
Published: August 2007

AbstractTo determine the role of recombinant human erythropoietin as a possible treatment option in Friedreich's ataxia, we performed an open‐label clinical pilot study. Primary outcome measure was the change of frataxin levels at week 8 versus baseline. Twelve Friedreich's ataxia patients received 5,000 units recombinant human erythropoietin three times weekly subcutaneously. Frataxin levels were measured in isolated lymphocytes by enzyme‐linked immunosorbent assay. In addition, urinary 8‐hydroxydeoxyguanosine and serum peroxides, were measured. Treatment with recombinant human erythropoietin showed a persistent and significant increase in frataxin levels after 8 weeks (p < 0.01). All patients showed a reduction of oxidative stress markers. Ann Neurol 2007
Riluzole in Huntington's disease: a 3‐year, randomized controlled study
Authors: Landwehrmeyer G. Bernhard , Dubois Bruno , de Yébenes Justo Garcia , Kremer Berry , Gaus Wilhelm , Kraus Peter H. , Przuntek Horst , Dib Michel , Doble Adam , Fischer Wilhelm , Ludolph Albert C. 
Published: August 2007

AbstractObjectiveWe conducted a randomized double‐blind trial of riluzole in Huntington's disease to investigate the efficacy of this antiexcitotoxic drug in slowing disease progression.MethodsThe study included 537 adult patients with a clinical diagnosis of Huntington's disease confirmed by genotyping. Patients were randomized (2:1) to treatment with riluzole (50mg twice daily) or placebo for 3 years. Concomitant use of antichoreic medication was forbidden, and introduction of such medication was a predefined end point. The primary outcome measure was change in a combined score derived from the motor and total functional capacity subscores of the Unified Huntington's Disease Rating Scale. Safety was also evaluated.ResultsA total of 379 patients completed the study (mean age, 47 [standard deviation, 9.5] years; 50% female patients). The principal reason for discontinuation was introduction of antichoreic medication. The median change from baseline in the combined score (primary outcome) for the “per protocol” population was 13.7 (95% confidence interval, 11.1–17.2) in the placebo group and 14.3 (95% confidence interval, 11.7–16.6) in the riluzole group. No intergroup difference in outcome could thus be demonstrated (p = 0.93, Mann–Whitney U test). No differences in secondary efficacy outcome variables were observed except for more frequent recourse to antichoreic medication in the placebo group. No unexpected adverse events were reported, and tolerability was acceptable.InterpretationNo neuroprotective or beneficial symptomatic effects of riluzole in Huntington's disease were demonstrated. Ann Neurol 2007
Hierarchical Nanomanufacturing: From Shaped Zeolite Nanoparticles to High‐Performance Separation Membranes
Authors: Snyder Mark A. , Tsapatsis Michael 
Published: August 2007

AbstractDespite more than a decade of intense research on the high‐resolution selectivity of thin zeolite films as alternatives to energy‐intensive industrial separations, membranes consisting of intergrown, oriented zeolite crystals have fallen short of gaining wide commercial application. Factors including poor performance, high cost, and difficulties in scale up have contributed to this, and have also stunted their application in other niche markets. Until recently, rational design of these materials was limited because of the elusive mechanism of zeolite growth, and forced more empirical approaches. New understanding of zeolite growth along with recent advances in the molecular engineering of crystal microstructure and morphology, assembly of crystal monolayers, and synthesis of ordered films constitute a strong foundation for meeting stringent industrial demands in the future. Together with new processing capabilities, such a foundation should make it possible to synthesize commercially viable zeolite membranes through hierarchical approaches. Such advances open exciting prospects beyond the realm of separations for assembly of novel and complex functional materials including molecular sensors, mechanically stable dielectrics, and novel reaction‐diffusion devices.
Spin coating cellulose derivatives on quartz crystal microbalance plates to obtain hydrogel‐based fast sensors and actuators
Authors: Sannino A. , Pappadà S. , Giotta L. , Valli L. , Maffezzoli A. 
Published: August 2007

AbstractCellulose based superabsorbent hydrogels were spin coated and chemically crosslinked on Quartz Crystal Microbalance plates to obtain a thin superabsorbent layer. The hydrogel network was obtained by crosslinking hydroxiethylcellulose (HEC) and Carboxymethylcellulose (CMC) by the difunctional Divinylsulfone (DVS). The presence of the polyelectrolyte CMC was found to be responsible for the material high sorption sensitivity to changes in ionic strength and pH of the external solution, due to the Donnan type equilibrium established between the internal of the gel and the external solution. Since the hydrogel was synthesized in the form of a thin layer, there is negligible barrier to water diffusion through the material, which results in a fast swelling response to changes in the external solution properties. The device was immersed in a flux of water solution, where the ionic strength was continuously changed. The fast change in the equilibrium sorption capacity of the spin coated hydrogel were converted to an electrical signal by the apparatus. The time lag of the response was comparable to the inverse of the quartz oscillation frequency (approximately 10−7s), and the accuracy in the weight change measurement was of the order of the nanogram. FTIR microscope analysis was used to determine the presence of hydrogel and its distribution on the QCM plate. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci, 2007
Novel polyion complex films from chitosan and quarternized poly(4‐vinyl‐N‐carboxymethylpyridine) containing zwitterion structure units
Authors: Liu Changhua , Xiao Chaobo , Huang Yuming 
Published: August 2007

AbstractNovel polyion complexes films of chitosan and quarternized poly(4‐vinyl‐N‐carboxymethylpyridine) containing zwitterion structure units were prepared by casting method. The aim of this work was to produce a hydrophilic film with the potential use as a hydrophilic membranes of the types used in membranes distillation and osmotic distillation for separating azeotropic, close‐boiling, and aqueous organic mixtures. Their structure and properties were studied by infrared, wide‐angle X‐ray diffraction, scanning electron microscopy, thermogravimetric analysis, tensile tester, and swelling measurements. The results indicated that polyion complexes occurred between chitosan and quarternized poly (4‐vinyl‐N‐carboxymethylpyridine) containing zwitterion structure units. The thermostability of these blends decreased with the increase of quarternized poly(4‐vinyl‐N‐carboxymethylpyridine) content. Initially, appreciable improvement in tensile strength and breaking elongation were achieved with increase of quarternized poly(4‐vinyl‐N‐carboxymethyl‐pyridine) content to 30%, the maximum value of 46.65 MPa tensile strength and 25.67% breaking elongation were achieved, respectively. The maximum degree of swelling was achieved when the weight ratio of chitosan versus poly(4‐vinyl‐N‐carboxymethylpyridine) was 50 : 50. Meanwhile, the relationship between their structure and properties was also discussed. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci, 2007
Numerical simulation of DSC and TMDSC curves as well as reversing and nonreversing curve separation
Authors: Cao Jinan 
Published: August 2007

AbstractThe basic physical meaning of temperature modulation for DSC is an arguable research topic, and its interpretation affects the development of thermal analysis and polymer science. This article studies the basic physical meaning of TMDSC by numerical simulation. DSC and TMDSC output curves are computed for a sample with step changes in its specific heat and for a sample with crystallites melting over the temperature range. The TMDSC curves are further analyzed to obtain the reversing and nonreversing components. It is shown that separation of the reversing and nonreversing components from the underlying heat flow cannot be justified. Some common misconceptions regarding TMDSC are discussed as well. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci, 2007
Enantioselective synthesis of (2R, 3S)‐ and (2S, 3R)‐4,4,4‐trifluoro‐N‐Fmoc‐O‐tert‐butyl‐threonine and their racemization‐free incorporation into oligopeptides via solid‐phase synthesis
Authors: Xiao Nu , Jiang Zhong‐Xing , Yu Y. Bruce 
Published: August 2007

AbstractAn efficient method for the enantioselective synthesis of (2R, 3S)‐ and (2S, 3R)‐4,4,4‐trifluoro‐N‐Fmoc‐O‐tert‐butyl‐threonine on multigram scales was developed. Absolute configurations of the two stereoisomers were ascertained by X‐ray crystallography. Racemization‐free coupling conditions for the incorporation of tfT into oligopeptides were then explored. For solution‐phase synthesis, tfT racemization was not an issue under conventional coupling conditions. For solid‐phase synthesis, the following conditions were identified to achieve racemization‐free synthesis: if tfT (3.0 equiv) was not the first amino acid to be linked to the resin (1.0 equiv), the condition is 2.7 equiv DIC/3.0 equiv HOBt as the coupling reagent at 0°C for 20 h; if tfT (3.0 equiv) was the first amino acid to be linked to the resin (1.0 equiv), then 1.0 equiv of CuCl2 needs to be added to the coupling reagent. © 2007 Wiley Periodicals, Inc. Biopolymers (Pept Sci) 88: 781–796, 2007.This article was originally published online as an accepted preprint. The “Published Online” date corresponds to the preprint version. You can request a copy of the preprint by emailing the Biopolymers editorial office at biopolymers@wiley.com
Predictive value of common symptom combinations in diagnosing colorectal cancer
Authors: Thompson M R , Perera R , Senapati A , Dodds S 
Published: August 2007

Abstract

Background
This study compared the diagnostic values of age and single symptoms of colorectal cancer with those of age and symptom combinations.

Methods
Consecutive patients with lower gastrointestinal symptoms referred to a surgical clinic over a 12-year period were studied prospectively. The diagnostic value of age and common symptoms of bowel cancer, individually and in combination, was determined by measuring positive predictive value, sensitivity and specificity.

Results
In total, 467 (5·5 per cent) of 8529 patients had colorectal cancer. Symptom combination analyses showed that patients presenting with rectal bleeding and change in bowel habit without anal symptoms had the highest risk of cancer. Those with rectal bleeding and perianal symptoms without change in bowel habit were at the lowest risk of having cancer. Symptom subgroups defined by age had positive predictive values for cancer that varied from less than 1 to 35 per cent.

Conclusion
Symptom combinations defined by age have greater diagnostic value than single symptoms alone.
Laparoscopic subtotal cholecystectomy without cystic duct ligation
Authors: Sinha I , Smith M Lawson , Safranek P , Dehn T , Booth M 
Published: August 2007

Abstract

Background
Cholecystectomy is made hazardous by distortion of the anatomy of Calot's triangle by acute or chronic inflammation. Laparoscopic subtotal cholecystectomy (LSTC) without cystic duct ligation is an alternative to conversion to open surgery in difficult cases.

Methods
This prospective study included all cholecystectomies performed in a district general hospital upper gastrointestinal unit between 2003 and 2005, after the introduction of LSTC.

Results
Of 889 laparoscopic cholecystectomies, 28 LSTCs without cystic duct ligation were performed in 18 men and ten women of median age 68 years. Median operating time was 90 min and median duration of hospital stay was 3 days. Two temporary bile leaks resolved spontaneously on days 14 and 19. Three patients required endoscopic retrograde cholangiopancreatography, extraction of bile duct stones and stent insertion for persistent leaks. All five bile leaks were expected from peroperative findings. One patient had a myocardial infarction and one developed a subphrenic abscess. There were no deaths. Open conversion rates were reduced from 5·0 per cent in 1997–2002 to 0·3 per cent in 2005 (P &lt; 0·001).

Conclusion
LSTC without cystic duct ligation is an alternative to open conversion when dissection of Calot's triangle is hazardous. Bile leaks are predictable and readily managed.
Autoimmune pancreatitis
Authors: Toomey D P , Swan N , Torreggiani W , Conlon K C 
Published: August 2007

Abstract

Background
Approximately 2 per cent of pancreatic masses resected for suspected malignancy are found instead to be a form of chronic pancreatitis defined by a characteristic lymphoplasmacytic infiltrate. This condition is now commonly classified as ‘autoimmune pancreatitis’.

Methods
A literature review of autoimmune pancreatitis was performed using Medline and PubMed. The reference lists of identified articles were searched for further relevant publications.

Results
Patients are predominantly 55-65 years old and present with obstructive jaundice, abdominal pain and weight loss. Imaging may show a mass of malignant appearance or pancreatobiliary tree strictures precipitating surgical exploration. Raised serum levels of IgG4 and specific autoantibodies, when combined with particular radiological features and a biopsy negative for malignancy, enable a preoperative diagnosis and successful treatment with steroids.

Conclusion
Autoimmune pancreatitis is not uncommon and steroid treatment can effect a dramatic improvement. Care is needed to ensure that pancreatic cancer is not misdiagnosed.
Randomized clinical trial of the effect of adding subfascial endoscopic perforator surgery to standard great saphenous vein stripping
Authors: Kianifard B , Holdstock J , Allen C , Smith C , Price B , Whiteley M S 
Published: August 2007

Abstract

Background
This randomized trial was undertaken to investigate the fate of incompetent perforating veins (IPVs) following saphenofemoral ligation and stripping of the great saphenous vein (GSV), with or without subfascial endoscopic perforator surgery (SEPS).

Methods
Patients with venous reflux (greater than 0·5 s) of the GSV and additional IPVs were allocated randomly to standard surgery (saphenofemoral ligation, stripping and phlebectomies alone) or with the addition of SEPS. Patients with ulceration, recurrent veins, deep venous reflux/thrombosis or saphenopopliteal reflux were excluded. Duplex ultrasonography was carried out before operation, and at 1 week, 6 weeks, 6 months and 1 year after surgery. Quality of life questionnaires were completed and visual analogue scale scores collected at the same time points.

Results
Thirty-eight patients were allocated to SEPS and 34 to the no SEPS group. Two patients in the no SEPS group were excluded (one withdrew and the other had the wrong treatment). There were no differences between the two groups with respect to pain, mobility or quality of life scores during follow-up. A significantly higher proportion of patients in the no SEPS group had IPVs on duplex imaging at 1 year (25 of 32 versus 12 of 38; P &lt; 0·001).

Conclusion
IPVs do not remain closed following standard varicose vein surgery. The addition of SEPS was not associated with significant morbidity but did reduce the number of IPVs. Up to 1 year this had no effect on recurrence rates or quality of life, but late results remain to be seen. Registration number: ISRCTN18288048 (http://www.controlled-trials.com).
Randomized clinical trial comparing partial parotidectomy versus superficial or total parotidectomy
Authors: Roh J-L , Kim H S , Park C I 
Published: August 2007

Abstract

Background
In recent decades the treatment of benign parotid tumours has shifted from superficial or total parotidectomy to partial parotidectomy. This study examined whether current surgical techniques improved functional outcomes after surgery for benign parotid tumours.

Methods
One hundred and one patients were assigned randomly to conventional (49 patients) or function-preserving (52) surgery. The latter consisted of modified facelift incision, greater auricular nerve preservation, partial parotidectomy and coverage with parotid fascia.

Results
The mean duration of operation was 0·7 h shorter and the overall complication rate significantly lower in the functional surgery group. In this group, more patients were satisfied with their scars and facial contours, the auricular nerve sensory recovery rate was high, and transient facial paralysis and Frey's syndrome were infrequent (12 and 6 per cent respectively). Stimulated salivary flow on the operated side decreased to 71·9 per cent after function-preserving surgery compared with 20·7 per cent after conventional operation. There was no tumour recurrence in either group during a mean follow-up of 48 months.

Conclusion
Compared with conventional procedures, function-preserving surgery for benign parotid tumours improved cosmetic, sensory and salivary functions, and reduced the duration of surgery and operative morbidity.
The lack of a critical perspective in environmental management research: distortion in the scientific discourse
Authors: Ählström Jenny , Macquet Monica , Richter Ulf 
Published: August 2007

AbstractAlthough a lack of critical perspective in environmental management has been acknowledged, the frequency of this perspective has not been measured. This was noted in the editorial of Business Strategy and the Environment (BSE) 1998, where Richard Welford called for more critical theory in environmental management. BSE can be seen as a forerunner in the field. Therefore, it could be expected that the most critical and reflective research contributing to sustainable development in companies could be published in the journal. In order to analyse the extent to which critical perspective is present in environmental management, we reviewed the last six years of BSE. We found that the studies undertaken generally follow the positivist paradigm, which almost exclusively echoes marginal adjustments. Papers with a critical perspective are in the minority. Using Jürgen Habermas' work on communicative action and discourse, we conclude that there is a distortion in this scientific discourse. Copyright © 2007 John Wiley & Sons, Ltd and ERP Environment.