bluuebunny/crossref_metadata_2025 huggingface dataset:
A Review of Research Relevant to Custody and Access Disputes
Authors: Waters Brent , Dimock John
Published: October 2008

With the high rate of marriage breakdown, increasing numbers of children are becoming the subjects of custody and access disputes. The Courts are looking to the services of psychiatrists in these matters. They value our expert opinion on the least detrimental available alternative for the child. This paper reviews available research data which are relevant to psychiatric testimony on the determination of exclusive custody, joint custody and access. It is concluded that there is emerging an objective body of data which can buttress psychiatric opinion in these matters, but that a number of the principles which have guided the Courts to date, have not been thoroughly researched.
Perioperative Complications of HINTEGRA Total Ankle Replacement: Our Initial 50 Cases
Authors: Lee Keun-Bae , Cho Sang-Gwon , Hur Chang-Ich , Yoon Taek-Rim
Published: October 2008

Background: The purpose of the present study was to report the perioperative complications that occurred among our initial 50 consecutive cases of HINTEGRA total ankle replacement. Materials and Methods: This was a retrospective study of 50 cases composed of 30 men and 18 women of average age 57 years. Perioperative complications were used to compare the first 25 cases (Group A) with the subsequent 25 (Group B). Results: Perioperative complications occurred in 15 cases (60%) in Group A but in only five (20%) in Group B. No major wound complications requiring a soft-tissue coverage procedure were encountered. Minor wound complications occurred in three cases in each group which resolved with skin grafting or topical dressing changes. One deep infection occurred in Group A, which required implant removal and antibiotic impregnated spacer prior to revision TAR. Four patients sustained intraoperative malleolus fractures in Group A, but only one in Group B. Coronal malposition of the tibial component occurred in three cases in Group A and in two in Group B. Increased sagittal slope of the tibial component occurred in two cases in Group B and sagittal malposition of the talar component occurred in two cases in Group A. There were seven instances of anterior translation of the talar component with respect to the tibial component; four in Group A and three in Group B. Conclusion: These results suggest that TAR has a steep learning curve. Moreover, knowledge of the perioperative complications of TAR may reduce the incidence of complications.

Level of Evidence: III, Therapeutic Study
Complications Encountered with Circular Ring Fixation in Persons with Diabetes Mellitus
Authors: Wukich Dane K. , Belczyk Ronald J. , Burns Patrick R. , Frykberg Robert G.
Published: October 2008

Background: The purpose of this study was to identify and report the complications associated with the use of circular ring fixation in diabetic patients, and to compare the frequency of complications in patients without diabetes. We hypothesized that complications with circular ring fixation occurred more frequently in patients with diabetes than patients without diabetes. Materials and Methods: Institutional Review Board approval was obtained and patient charts were retrospectively reviewed from June 2004 and February 2007. Fifty six consecutive patients undergoing midfoot, hindfoot and/or ankle surgery were treated with circular ring fixation which included 33 diabetic patients in the study group and 23 non-diabetic patients in the control group. Patient demographics, the duration of treatment with the external fixator, and complications were recorded. Results: Males had a greater number of complications compared to females ( p = 0.0014). The total number of complications was statistically greater in diabetic patients (study group) versus non-diabetic patients (control group) ( p = 0.003). In multivariate logistic regression, diabetes and male sex were the only significant variables associated with wire complications (OR 7.35, 95% CI 1.93-28.04 and OR 0.22, 95% CI 0.05-8584111, respectively). Conclusion: Women are protected from wire complications with a risk reduction of 78% compared to males. Diabetics have a 7-fold risk for any wire complication compared to patients without diabetes. We found no adverse effects of BMI, obesity, age, smoking, neuropathy, or Charcot neuroarthropathy on a satisfactory recovery.

Level of Evidence: IV, Retrospective Case Study
Sliding Oblique versus Segmental Resection Osteotomies for Lesser Metatarsophalangeal Joint Pathology
Authors: Garg Rishi , Thordarson David B. , Schrumpf Mark , Castaneda Deborah
Published: October 2008

Background: Oblique shortening osteotomy (Weil) can address lesser MP pathology but can have a high rate of complications. The purpose of this study was to review the results of a modification of the Weil osteotomy, the segmental resection metatarsal osteotomy. Materials and Methods: Between 2004 and 2006, 48 patients underwent the segmental resection osteotomy with a mean followup of 13 (range, 6 to 26) months. All the patients were evaluated with the American Orthopaedic Foot and Ankle Society (AOFAS) forefoot score and a questionnaire addressing distances they were able to walk, work limitations, sporting activity, and overall satisfaction. Results: The postoperative AOFAS forefoot score was an average of 87.6 (range, 59 to 100; SD, 10.97) and the overall satisfaction rate was 85.4%. The complication rate was 18.8% for transfer metatarsalgia, 27.1% for floating toes, 35.4% for toe weakness, 14.6% for infection, and 10.4% for wound healing problems. Conclusions: Despite the complications, the patients who underwent segmental osteotomy were satisfied with the outcome for lesser MTP joint pain and deformity. This is a preliminary study with significant refinement of the operative method as detailed in the surgical technique section. Further followup will elucidate whether additional changes are necessary in the surgical technique.

Level of Evidence: IV, Retrospective Case Study
Surgical Treatment of Calcaneal Fractures with Use of β-Tricalcium Phosphate Ceramic Grafting
Authors: Jiang Sheng-Dan , Jiang Lei-Sheng , Dai Li-Yang
Published: October 2008

Background: The treatment of calcaneal fractures is challenging. Internal fixation and grafting with sintered β-tricalcium phosphate (β-TCP) ceramic is alternative to bone grafting in the treatment of calcaneal fractures. Methods: Seventy-four patients with intraarticular calcaneal fractures were treated with open reduction, internal fixation and grafting with sintered β-TCP ceramic. Followup was performed using the Maryland foot score as well as plain radiography. Results: All patients were followed for 15 (range, 12 to 26) months. Clinical results as assessed by the Maryland foot score were excellent in 40 of 74 cases (54.1%), good in 28 (37.8%), and fair in 6 (8.1%). Immediately after surgery, the mean Böhler angle was improved by 23 degrees, and 1 year after surgery, the mean Böhler angle decreased by 4 degrees. A similar change was also seen in Gissane angles and the height and width of the calcaneus. There was no statistical correlation between the severity of fracture and functional outcome in this series. All fractures healed. Conclusion: These findings suggest that use of β-TCP combined with open reduction and internal fixation is an effective and safe method for the surgical treatment of intra-articular calcaneal fractures.

Level of Evidence: IV, Retrospective Case Study
Ankle Arthrodesis with Bone Graft after Distal Tibia Resection for Bone Tumors
Authors: Campanacci Domenico Andrea , Scoccianti Guido , Beltrami Giovanni , Mugnaini Marco , Capanna Rodolfo
Published: October 2008

Background: Treatment of distal tibial tumors is challenging due to the scarce soft tissue coverage of this area. Ankle arthrodesis has proven to be an effective treatment in primary and post-traumatic joint arthritis, but few papers have addressed the feasibility and techniques of ankle arthrodesis in tumor surgery after long bone resections. Materials and Methods: Resection of the distal tibia and reconstruction by ankle fusion using non-vascularized structural bone grafts was performed in 8 patients affected by malignant (5 patients) or aggressive benign (3 patients) tumors. Resection length of the tibia ranged from 5 to 21 cm. Bone defects were reconstructed with cortical structural autografts (from contralateral tibia) or allografts or both, plus autologous bone chips. Fixation was accomplished by antegrade nailing (6 cases) or plating (2 cases). Results: All the arthrodesis successfully healed. At followup ranging from 23 to 113 months (average 53.5), all patients were alive. One local recurrence was observed with concomitant deep infection (a below-knee amputation was performed). Mean functional MSTS score of the seven available patients was 80.4% (range, 53 to 93). Conclusion: Resection of the distal tibia and arthrodesis of the ankle with non-vascularized structural bone grafts, combined with autologous bone chips, can be an effective procedure in bone tumor surgery with durable and satisfactory functional results. In shorter resections, autologous cortical structural grafts can be used; in longer resections, allograft structural bone grafts are needed.

Level of Evidence: IV, Retrospective Case Study
Acute Posterior Tibial Tendon Tear in an Ice-Hockey Player: A Case Report
Authors: Jacoby Sidney M. , Slauterbeck James R. , Raikin Steven M.
Published: October 2008

Level of Evidence: V, Case Report
Locked Second Metatarsal Head Fracture: A Case Report
Authors: Liddle Alexander D. , Rosenfeld Peter F.
Published: October 2008

Level of Evidence: V, Case Report
A New Treatment for Severe Incurved Toenails: A Case Report
Authors: Kim J-Young , Lee Yoon Jung , Park Jun Sic , Lee Sang Hyuck
Published: October 2008

Level of Evidence: V, Case Report
A Comparative Study of the Radical-scavenging Activity of the Phenolcarboxylic Acids Caffeic Acid, p-Coumaric Acid, Chlorogenic Acid and Ferulic Acid, With or Without 2-Mercaptoethanol, a Thiol, Using the Induction Period Method
Authors: Kadoma Yoshinori , Fujisawa Seiichiro
Published: October 2008

Phenolcarboxylic acid antioxidants do not act in vivo as radical-scavengers in isolation, but rather together with GSH (glutathione), a coantioxidant, they constitute an intricate antioxidant network. Caffeic acid, p-coumaric acid, ferulic acid and chlorogenic acid with or without 2-mercaptoethanol (ME), as a substitute for GSH, was investigated by the induction period (IP) method for polymerization of methyl methacrylate (MMA) initiated by thermal decomposition of 2,2'-azobisisobutyronitrile (AIBN, a source of alkyl radicals, R.) and benzoyl peroxide (BPO, a source of peroxy radicals, PhCOO.) using differential scanning calorimetry (DSC). Upon PhCOO. radical scavenging, the stoichiometric factors (n, number of free radical trapped by one mole of antioxidant) for caffeic acid, ferulic acid, p-coumaric acid and chlorogenic acid were 2.4, 1.8, 1.7 and 0.9, whereas upon R. radical scavenging, the corresponding values were 1.3, 1.2, 1.0 and 0.8, respectively. Antioxidants with n values close to 2 suggest the stepwise formation of semiquinone radicals and quinones. By contrast, those with n values close to 1 suggest the formation of dimers after single-electron oxidation, possibly due to recombination of corresponding aryloxy radicals. The ratio of the rate constant of inhibition to that of propagation (kinh/kp) declined in the order chlorogenic acid > p-coumaric acid > ferulic acid > caffeic acid. The ratio of the observed IP for the phenolcarboxylic acid/2-mercapto-ethanol (ME) mixture (1:1 molar ratio) (A) to the calculated IP (the simple sum of phenol acid antioxidant and ME) (B) was investigated. Upon R. scavenging, the caffeic acid or p-coumaric acid/ME mixture was A/B > 1, particularly the former was 1.2, suggesting a synergic effect. By contrast, upon PhCOO. scavenging, the corresponding mixture was A/B < 1, particularly the latter was 0.7, suggesting an antagonistic effect. Upon both radicals scavenging, the A/B for the ferulic acid or chlorogenic acid/ME mixture was approximately 1. The reported beneficial antioxidant, anti-inflammatory and anticancer effects of caffeic acid and p-coumaric acid may be related to their prooxidant-antioxidant balance in the presence of GSH.
Two New Phenolic Glycosides from Viscum articulatum
Authors: Li Yang , Zhao Yan-Li , Huang Ning , Zheng Yong-Tang , Yang Yong-Ping , Li Xiao-Li
Published: October 2008

Two new phenolic glycosides, 1-O-benzyl-[5-O-benzoyl-β-D-apiofuranosyl (1→2)]-β-D-glucopyranoside (1), and 4`-hydroxy-7,3`-dimethoxyflavan-5-O- β-D-gluco-pyranoside (2), together with nine known flavanones 3 - 11, have been isolated from the dried whole plants of Viscum articulatum.Their structures were identified by extensive spectral analysis, especially 2D NMR techniques. Compound 9 showed weak anti-HIV-1 activity.
Effects of Gold Nanoparticles on the Response of Phenol Biosensor Containing Photocurable Membrane with Tyrosinase
Authors: Sharina Abu Hanifah , Lee Yook Heng , Musa Ahmad
Published: October 2008

The role of incorporation of gold nanoparticles (50-130 nm in diameter) into a series of photocurable methacrylic-acrylic based biosensor membranes containing tyrosinase on the response for phenol detection was investigated. Membranes with different hydrophilicities were prepared from 2-hydroxyethyl methacrylate and n-butyl acrylate via direct photocuring. A range of gold nanoparticles concentrations from 0.01 to 0.5 % (w/w) was incorporated into these membranes during the photocuring process. The addition of gold nanoparticles to the biosensor membrane led to improvement in the response time by a reduction of approximately 5 folds to give response times of 5-10 s. The linear response range of the phenol biosensor was also extended from 24 to 90 mM of phenol. The hydrophilicities of the membrane matrices demonstrated strong influence on the biosensor response and appeared to control the effect of the gold nanoparticles. For less hydrophilic methacrylic-acrylic membranes, the addition of gold nanoparticles led to a poorer sensitivity and detection limit of the biosensor towards phenol. Therefore, for the application of gold nanoparticles in the enhancement of a phenol biosensor response, the nanoparticles should be immobilized in a hydrophilic matrix rather than a hydrophobic material.
Model-based Assessment of Data Availability in Health Information Systems
Authors: Strübing A. , Winter A.
Published: October 2008

Objectives:
To introduce a formal definition of data availability as a contribution to trustworthiness of health information systems and to automatically detect respective weaknesses and propose solutions.

Method:
Specifying an ontology, based on enterprise functions and application systems of (health) information systems and closely linked to the Three-Layer Graph-Based Meta Model (3LGM2). Deriving appropriate measures and algorithms.

Results:
A formal definition for data availability is introduced and elucidated by an example. This concept is used e.g. to disclose missing communication links and to suggest solutions.

Conclusions:
Data availability is a necessary but not sufficient condition for trustworthiness of health information systems. If information management has a thorough description of the information system at its disposal, e.g. by using 3LGM2, the calculation of data availability does not need further efforts.
Central suboptimalH∞filter design for nonlinear polynomial systems
Authors: Basin Michael , Shi Peng , Calderon‐Alvarez Dario
Published: October 2008

This paper presents the central finite‐dimensionalH∞filter for nonlinear polynomial systems, which is suboptimal for a given threshold γ with respect to a modified Bolza–Meyer quadratic criterion including the attenuation control term with the opposite sign. In contrast to the previously obtained results, the paper reduces the originalH∞filtering problem to the corresponding optimalH2filtering problem, using the technique proposed in (IEEE Trans. Automat. Control1989;34:831–847). The paper presents the central suboptimalH∞filter for the general case of nonlinear polynomial systems based on the optimalH2filter given in (Int. J. Robust Nonlinear Control2006;16:287–298). The central suboptimalH∞filter is also derived in a closed finite‐dimensional form for third (and less) degree polynomial system states. Numerical simulations are conducted to verify performance of the designed central suboptimal filter for nonlinear polynomial systems against the central suboptimalH∞filter available for the corresponding linearized system. Copyright © 2008 John Wiley & Sons, Ltd.
Vesicle‐Assisted Assembly of Mesoporous Ce‐Doped Pd Nanospheres with a Hollow Chamber and Enhanced Catalytic Efficiency
Authors: Li Hui , Liu Jun , Xie Songhai , Qiao Minghua , Dai Weilin , Lu Yunfeng , Li Hexing
Published: October 2008

Mesoporous Ce‐doped Pd nanospheres with a hollow chamber are synthesized by chemical reduction of PdCl2 with KBH4 in an aqueous solution containing Ce(NO3)3 and Bu4PBr. The later acts as a template for the hollow chamber via forming organic vesicles. During the liquid‐phase phenol hydrogenation to cyclohexanone, the as‐prepared catalyst exhibits a much‐higher activity than the corresponding solid nanoparticle catalyst prepared in the absence of Bu4PBr. Meanwhile, the Ce dopants greatly enhance the activity and selectivity to cyclohexanone. The hollow chamber is quite stable against heating in solution and the catalyst could be used repetitively many times. Such a catalyst shows a good potential in industrial applications.
Simulated moving bed multiobjective optimization using standing wave design and genetic algorithm
Authors: Lee Ki Bong , Kasat Rahul B. , Cox Geoffrey B. , Wang Nien‐Hwa Linda
Published: October 2008

Multiobjective optimization of simulated moving bed systems for chiral separations is studied by incorporating standing wave design into the nondominated sorting genetic algorithm with jumping genes. It allows simultaneous optimization of seven system and five operating parameters to show the trade‐off between productivity, desorbent requirement (DR), and yield. If pressure limit, product purity, and yield are fixed, higher productivity can be obtained at a cost of higher DR. If yield is not fixed, it can be sacrificed to achieve higher productivity or vice versa. Short zones and high feed concentration favor high productivity, whereas long zones favor high yield and low DR. At fixed product purity and yield, increasing the pressure limit allows the use of smaller particles to increase productivity and to decrease DR. The performance of low‐pressure simulated moving bed can be improved significantly by using shorter columns and smaller particles than those in conventional systems. © 2008 American Institute of Chemical Engineers AIChE J, 54: 2852–2871, 2008
Human T‐cell leukemia virus (HTLV‐I) p24 antibody in New York City blood product recipients
Authors: Jason Janine M. , McDougal J. Steven , Cabradilla Ciril , Kalyanaraman V. S. , Evatt Bruce L.
Published: October 2008

Human T‐cell leukemia virus (HTLV‐I) is known to be associated with certain hematologic malignancies, and a related virus, HTLV‐III/LAV, might be the cause of AIDS. Some persons with AIDS have had evidence of HTLV‐I infection. Unrelated to these findings, it has been suggested that HTLV‐I is transmitted via blood products. We therefore evaluated the serologic status to the HTLV‐I core antigen p24 of 48 persons with hemophilia (Hem A) receiving factor concentrate therapy (a group at risk for AIDS), 49 persons with β‐thalassemia major (Thal) receiving frozen packed red blood cells therapy (FPRC), 26 patients with sickle cell anemia (SCA) receiving FPRC, and 18 persons not receiving any blood products. All participants were clinically well; only one had a risk factor other than hemophilia for AIDS, and all were from New York City, an area with a high incidence of AIDS. No Hem A or nontransfused persons had serum antibody to HTLV core p24 antigen; three with Thal and one with SCA were antibody‐positive. These results were confirmed by both radioimmunoprecipitation and Western blot techniques. Positive serology did not correlate with any immune findings or quantity of blood products used. These data support that HTLV‐I is preferentially transmitted through cellular blood products and that it is an infection for which cellular blood product recipients in at least some areas of the United States are at risk. Concentrate products appear free of transmission risk relative to cellular blood products, but we cannot be certain that this safety is absolute. The public health implications of blood product transmission of HTLV‐I merit active, long‐term investigation.
Poikiloderma with neutropenia, Clericuzio type, in a family from Morocco
Authors: Mostefai Rahima , Morice‐Picard Fanny , Boralevi Franck , Sautarel Michel , Lacombe Didier , Stasia Marie José , McGrath John , Taïeb Alain
Published: October 2008

Three siblings from Morocco consanguineous family presented with cutaneous poikiloderma following postnatal ichthyosiform lesions, associated with papillomatous lesions, palmoplantar keratoderma, pachyonychia of toenails, fragile carious teeth, and lachrymal duct obstruction. Photosensitivity and blistering improved with age. Atrophic scars were prominent on the limbs. Neutropenia developed in the first year secondary to dysmyelopoiesis affecting the granulocyte lineage, associated with a polyclonal hypergammaglobulinemia. Several broncho‐pulmonary infectious episodes complicated the evolution, and cystic fibrosis was first considered on the basis of repeated abnormal sweat chloride tests but not confirmed by molecular analyses. This autosomal recessive disorder matches that described originally as poikiloderma with neutropenia‐Clericuzio type in Navajo Indians (OMIM 604173). It is discussed within the group of the major hereditary poikiloderma disorders, that is, Rothmund–Thomson syndrome, dyskeratosis congenita, and Kindler syndrome. © 2008 Wiley‐Liss, Inc.
Neuroimaging aspects of Aicardi syndrome
Authors: Hopkins Bobbi , Sutton V. Reid , Lewis Richard Alan , Van den Veyver Ignatia , Clark Gary
Published: October 2008

Aicardi syndrome is a rare neurodevelopmental disorder characterized by congenital chorioretinal lacunae, corpus callosum dysgenesis, seizures, polymicrogyria, cerebral heterotopias, intracranial cysts, and costovertebral defects. Cerebellar abnormalities have been described occasionally. Aicardi syndrome is sporadic and has been observed only in females and 47,XXY males. Therefore, it is thought to result from a mutation in an X‐linked gene. Improved definition of the clinical phenotype should focus the selection of functional candidate genes for mutation analysis. Because central nervous system abnormalities are the most prominent component of the phenotype, we performed a detailed characterization of abnormalities identified on magnetic resonance neuroimaging studies from 23 girls with Aicardi syndrome, the largest cohort to undergo such review by a single group of investigators. All patients had polymicrogyria that was predominantly frontal and perisylvian and often associated with underopercularization. Periventricular nodular heterotopias, present in all patients, were more frequent than previously reported; 10 had single and 11 had multiple intracranial cysts. Posterior fossa abnormalities were also more frequent than previously described. Cerebellar abnormalities were noted in 95% of studies where they could be evaluated. As a novel finding, we noted tectal enlargement in 10 patients. Since mildly affected girls with variable callosal dysgenesis have now been reported, the constellation of frontal‐dominant and perisylvian polymicrogyria, periventricular nodular heterotopias, intracranial cysts, and posterior fossa abnormalities, including tectal enlargement, should prompt consideration of the diagnosis of Aicardi syndrome. We further propose that improved characterization of the neurological phenotype will benefit the selection of candidate genes for mutation analysis. © 2008 Wiley‐Liss, Inc.
Responsible patient advocacy: Perspectives from the Alpha‐1 Foundation
Authors: Sharp Richard R. , Yarborough Mark , Walsh John W. ,   the Ethical, Legal, Social Issues Working Group of the Alpha‐1 Foundation
Published: October 2008

Patient advocacy groups play an increasingly prominent role in patient care and clinical research. To help manage some of the unique challenges associated with the involvement of patient‐centered organizations in the delivery of medical services and conduct of clinical research, patient advocacy groups may wish to establish an ethics advisory board. Although hospitals and government agencies often adopt this approach, ethics advisory boards have not been widely used by patient advocacy groups. We report on the experiences of an ethics advisory board for a patient advocacy group actively involved in genetic testing and clinical research. Drawing on the experiences of this organization, we examine the potential advantages and limitations of this approach to navigating the complex moral terrain of contemporary medicine and biomedical research. © 2008 Wiley‐Liss, Inc.
Mouse models of Wolf–Hirschhorn syndrome
Authors: Simon Ruth , Bergemann Andrew D.
Published: October 2008

Subtelomeric deletion syndromes represent a significant cause of mental retardation and craniofacial disease. However, for most of these syndromes the pathogenic genes have yet to be identified. Currently there is every indication that identification of these genes will be a slow process if we continue to rely strictly upon clinical data. An alternative approach is the use of mouse models to complement the patient studies. Wolf–Hirschhorn syndrome (WHS), caused by deletions in 4p16.3, is the first recognized subtelomeric deletion syndrome. As with other syndromes of this class, WHS has not yet been subjected to an intensive, systematic analysis using mouse models. Nonetheless, a significant number of targeted mutations have been introduced into mouse genomic region, 5B1, which is orthologous to 4p16.3. Included among these mutations are a series of deletions approximating the deletions in some patients. The mouse lines carrying these deletions display a remarkable concordance of phenotypes with the human patient's characteristics, strongly indicating that the mouse models can be used to phenocopy WHS. In this review, we will catalog the currently existing targeted mutations in mice in the regions orthologous to the WHS critical regions. For each mutation we will discuss the resulting phenotype and its potential relevance to the pathogenesis of the syndrome. Further, we will describe how the phenotypes of some of the mutations suggest new directions for the clinical studies. Finally we will outline approaches for the efficient creation of new mouse models of WHS going forward. © 2008 Wiley‐Liss, Inc.
Update on the clinical features and natural history of Wolf–Hirschhorn (4p‐) syndrome: Experience with 87 patients and recommendations for routine health supervision
Authors: Battaglia Agatino , Filippi Tiziana , Carey John C.
Published: October 2008

Wolf–Hirschhorn syndrome (WHS) is a well‐known multiple congenital anomalies/mental retardation syndrome, firstly described in 1961 by Cooper and Hirschhorn. Its frequency is estimated as 1/50,000–1/20,000 births, with a female predilection of 2:1. The disorder is caused by partial loss of material from the distal portion of the short arm of chromosome 4 (4p16.3), and is considered a contiguous gene syndrome. No single gene deletions or intragenic mutations have been shown to confer the full WHS phenotype. Since the disorder was brought to the attention of geneticists, many additional cases have been published. Only in 1999, however, were the first data on the natural history brought to the attention of the medical community. The purpose of the present study is to help delineate in more detail and over a longer period of time, the natural history of WHS, in order to establish appropriate health supervision and anticipatory guidance for individuals with this disorder. We have collected information on 87 patients diagnosed with WHS (54 females and 33 males) both in USA and Italy. Age at first observation ranged between newborn and 17 years. Twenty patients have been followed from 4 months to 23 years. The deletion proximal breakpoint varied from 4p15.32 to 4p16.3, and, by FISH, was terminal and included both WHSCR. Deletion was detected by standard cytogenetics in 44/87 (50.5%) patients, whereas FISH was necessary in the other 43 (49.5%). Array‐CGH analysis at 1 Mb resolution was performed in 34/87 patients, and, in 15/34 (44%), showed an unbalanced translocation leading to both a 4p monosomy and a partial trisomy for another chromosome arm. Six more patients had been previously shown to have an unbalanced translocation by karyotype analysis or FISH with a WHS‐specific probe. Sixty‐five of 87 patients had an apparent pure, de novo, terminal deletion; and 1/87 a tandem duplication of 4p16.1p16.3 associated with 4p16.3pter deletion. Age at diagnosis varied between 7 months gestation and 16 years. Ninety‐three percent had a seizure disorder with a good outcome; 80% had prenatal onset growth deficiency followed by short stature and slow weight gain; 60% had skeletal anomalies; 50% had heart lesions; 50% had abnormal tooth development; and 40% had hearing loss. Distinctive EEG findings were seen in 90%. Structural CNS anomalies were detected in 80%. Global developmental delay of varying degrees was present in all patients. Almost 50% was able to walk either alone or with support. Hypotonia was present in virtually all patients. A global improvement was observed in all individuals, over time. Our survey has also shown how the characteristic facial phenotype tends to be less pronounced in those patients with a smaller deletion, and microcephaly is not observed in the patients with certain cryptic unbalanced translocations. © 2008 Wiley‐Liss, Inc.
Solvent‐free synthesis of α‐aminonitriles from aldehydes, amines and trimethylsilyl cyanide catalyzed by thallium(III) chloride tetrahydrate
Authors: Majhi Anjoy , Kim Sung Soo , Kadam Santosh T.
Published: October 2008

A straightforward and efficient method has been developed for the synthesis of α‐aminonitriles by combining aldehydes, amines and trimethylsilyl cyanide in the presence of a catalytic amount of thallium(III) chloride tetrahydrate (1 mol%) under solvent‐free conditions at room temperature. Copyright © 2008 John Wiley & Sons, Ltd.
Carbonization of oriented polyacrylonitrile and multiwalled carbon nanotube composite films
Authors: Koganemaru Ai , Bin Yuezhen , Tohora Hidekazu , Okino Fujio , Komiyama Shingo , Zhu John , Matsuo Masaru
Published: October 2008

Composite films of polyacrylonitrile (PAN)/multiwalled carbon nanotubes (MWNTs) were fabricated by gelation/crystallization from solution. The composite films were elongated twofold and heat‐treated at 2800 °C to produce oriented carbon films. The orientation and morphology of the resultant carbon films were investigated by scanning electron microscopy, wide‐angle X‐ray diffraction and Raman spectrometers. The results indicate that the degree of graphitization of PAN was enhanced by the addition of MWNTs, at the same time, the elongation induced the orientation of MWNTs along elongation direction and the co‐orientation of MWNTs and the molecular chains of the matrix PAN greatly promoted the carbonization of composite precursor. It is suggested that carbon nanotubes acted as nucleation side of graphitic crystal during the carbonization of PAN‐MWNTs composites. Copyright © 2008 Curtin University of Technology and John Wiley & Sons, Ltd.
Numerical model for polymer electrolyte membrane fuel cells with experimental application and validation
Authors: Mora Javier Alonso , Husar Attila P., , Serra Maria , Riera Jordi
Published: October 2008

The aim of this paper is to present a simple 3D computational model of a polymer electrolyte membrane fuel cell (PEMFC) that simulates over time the heat distribution, energy, and mass balance of the reactant gas flows in the fuel cell including pressure drop, humidity, and liquid water. Although this theoretical model can be adapted to any type of PEMFC, for verification of the model and to present different analysis it has been adapted to a single cell test fixture. The model parameters were adjusted through a series of experimental tests and the model was experimentally validated for a well‐defined range of operating conditions: H2/air O2 as reactants, flow rates of 0.5–1.5 SLPM, dew points and cell temperatures of 30–80 °C, currents 0–5 A and with/without water condensation. The model is especially suited for the analysis of liquid water condensation in the reactant channels. A key finding is that the critical current at which liquid water is formed is determined at different flows, temperatures, and humidity. Copyright © 2008 Curtin University of Technology and John Wiley & Sons, Ltd.
Biosorption of heavy metal using brown seaweed in a regenerable continuous column
Authors: Rajamohan N. , Sivaprakash B.
Published: October 2008

This paper deals with the experimental investigation on removal of cadmium [Cd(II)] ions from an aqueous solution using a marine alga, Sargassum tenerrimum, in a fixed‐bed column. The effects of the inlet flow rate and the sorbent bed height on the biosorption of Cd(II) ions were studied. The dynamics of column biosorption was modeled by the bed depth service time (BDST) model and the Thomas model. The BDST model was used to study the dynamic sorption behavior at different bed heights, whereas the Thomas model was used to fit the column biosorption data at different flow rates. The uptake capacity and the breakthrough time increase with an increase in the bed height. The sorption capacities of the bed per unit volume and the rate constant Ka were found to be 3819.42 mg/l and 0.0353 mg/h respectively. In flow rate experiments, the results confirmed that the metal uptake capacity and the metal removal efficiency of S. tenerrimum decreased with increasing flow rate. The Thomas model was used to fit the column biosorption data at different flow rates and model constants were evaluated. After five sorption–desorption cycles, the selected marine alga exhibited a high cadmium uptake of 63.43 mg/g. Copyright © 2008 Curtin University of Technology and John Wiley & Sons, Ltd.
Integrated control method for a fuel cell hybrid system
Authors: He Hongwen , Yan Song , Xiao Zhenjun
Published: October 2008

To apply a fuel cell engine (FCE) on a hybrid electric vehicle, an integrated control method is put forward according to the driving characteristics of the vehicle. The FCE's output power is self‐regulated to minimize hydrogen consumption and at the same time to meet the driving power requirement of the vehicle. For the difference of the power output dynamics, the actual power difference between the FCE and the vehicle load is compensated passively by a secondary power battery pack. Vehicle experiments show that the fuel cell hybrid system works well without any adverse influence on the vehicle power performance. Copyright © 2008 Curtin University of Technology and John Wiley & Sons, Ltd.
Liquid phase mineralization of gel‐type anion exchange resin by a hybrid process of Fenton dissolution followed by sonication and wet air oxidation
Authors: Gunale T. L. , Mahajani V.V. , Wattal P. K. , Srinivas C.
Published: October 2008

A hybrid process of Fenton dissolution followed by sonication and wet air oxidation (WAO), has been demonstrated to mineralize strongly basic anion exchange resin (gel type). The solid anion exchange resin beads could be dissolved in water by Fenton process wherein the copper‐catalyzed hydrogen peroxide (H2O2) reaction makes the resin hydrophilic by the disintegration of polymer matrix. Sonication of the dissolved resin thus obtained made the waste stream more amenable to WAO. Parameters for Fenton dissolution and sonication were studied to aid effective mineralization by WAO. The kinetic studies of WAO were performed using copper sulfate (CuSO4) as the homogeneous catalyst, in the temperature range of 483–523 K. It is thus possible to address the disposal of solid ion exchange resin by the hybrid process, described here. Copyright © 2008 Curtin University of Technology and John Wiley & Sons, Ltd.
CFD modeling of subcooling process for beer fermentation liquid
Authors: Yuan Yuejin , Xu Yingying , Liu Xiangdong
Published: October 2008

A model of subcooling process for beer fermentation liquid was established at the base of a computational fluid dynamics (CFD) technique which assumed that all the biochemical reactions were suppressed to a lower extent by the cryogenic condition and their influences on heat and momentum transfer of liquid could be ignored. The subcooling process of fermentation liquid in a cooling tank was simulated, where the temperature was from 10 to − 1 °C. The transient temperature and velocity distributions of the fermentation liquid were obtained by the simulation. The results indicated that the temperature delamination was distinct in the direction of the tank axis, while the temperature gradient was inconspicuous along the radial direction. The fermentation liquid showed a complicated movement including numerous local small circumfluences, which was different from our conventional knowledge. Copyright © 2008 Curtin University of Technology and John Wiley & Sons, Ltd.
Effect of nanoclay on the microcellular structure and morphology of high internal phase emulsion (HIPE) foams
Authors: Ahmed M. Serry , Lee Y. H. , Park C. B. , Atalla N.
Published: October 2008

High internal phase emulsion (HIPE) polymerization foaming process is controlled for the production of novel foams with various microcellular structures and morphologies. In this study, two types of organoclay and one type of natural clay, sodium montmorillonite (NaMMT) were introduced at different concentrations to control the microcellular morphologies, cell sizes and open cell contents. A scanning electron microscopy was used to observe the microcellular morphologies and open cell contents; volume expansion ratio (VER) and cell sizes were evaluated. It was believed that the open cell contents increased as organoclay content increased, due to the lowering of the viscosity ratio of dispersed to continuous phases, as a result of adding nanoclay into the oil phase of emulsion. A correlation between the open cell content and VER that depended on clay content was attempted and the result was quite satisfactory. Copyright © 2008 Curtin University of Technology and John Wiley & Sons, Ltd.
Habitat selection of freshwater‐dependent cetaceans and the potential effects of declining freshwater flows and sea‐level rise in waterways of the Sundarbans mangrove forest, Bangladesh
Authors: Smith B.D. , Braulik G. , Strindberg S. , Mansur R. , Diyan M.A.A. , Ahmed B.
Published: October 2008

Generalized additive models of sighting data for cetaceans collected during two surveys of waterways in the Sundarbans mangrove forest of Bangladesh indicated that Ganges River dolphin Platanista gangetica gangetica distribution was conditionally dependent (P<0.05) on low salinity, high turbidity, and moderate depth during both low and high freshwater flow; and Irrawaddy dolphin Orcaella brevirostris distribution was conditionally dependent (P<0.05) on low salinity during high freshwater flow, high and moderate depths during low and high freshwater flow, respectively; low and high‐low extremes of turbidity during low and high freshwater flow, respectively; and high temperature and increasing numbers of large–small channel confluences during low freshwater flow.

According to sighting data collected over a 3‐year period by the captains of three nature tourism vessels, there were significant differences between the actual and expected frequencies of Ganges River dolphin sightings and individuals according to various channel types (chi‐square=64.22, P<0.0001 and chi‐square=134.14, P<0.0001, respectively, df=6) and of Irrawaddy dolphin sightings and individuals (chi‐square=15.28, P=0.0182, and chi‐square=29.42, P<0.0001, respectively, df=6), with shared preferences for wide sinuous channels with at least two small confluences or one large confluence.

The dependency exhibited by both species for environmental characteristics associated with abundant freshwater flow, including low salinity and the availability of confluences, make them particularly vulnerable to habitat loss due to upstream water abstraction and sea‐level rise.

Although the results of this study may not affect plans for construction in India of large‐scale, inter‐basin water transfer projects that will result in further declines in freshwater flows, or decisions within the international community about CO2 emissions affecting global sea levels, they can be used to prioritize locations where protective measures could be employed to benefit the long‐term conservation of both species.

Copyright © 2008 John Wiley & Sons, Ltd.
Multiple Testing with Minimal Assumptions
Authors: Westfall Peter H. , Troendle James F.
Published: October 2008

Resampling‐based multiple testing methods that control the Familywise Error Rate in the strong sense are presented. It is shown that no assumptions whatsoever on the data‐generating process are required to obtain a reasonably powerful and flexible class of multiple testing procedures. Improvements are obtained with mild assumptions. The methods are applicable to gene expression data in particular, but more generally to any multivariate, multiple group data that may be character or numeric. The role of the disputed “subset pivotality” condition is clarified. (© 2008 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
General Multistage Gatekeeping Procedures
Authors: Dmitrienko Alex , Tamhane Ajit C. , Wiens Brian L.
Published: October 2008

A general multistage (stepwise) procedure is proposed for dealing with arbitrary gatekeeping problems including parallel and serial gatekeeping. The procedure is very simple to implement since it does not require the application of the closed testing principle and the consequent need to test all nonempty intersections of hypotheses. It is based on the idea of carrying forward the Type I error rate for any rejected hypotheses to test hypotheses in the next ordered family. This requires the use of a so‐called separable multiple test procedure (MTP) in the earlier family. The Bonferroni MTP is separable, but other standard MTPs such as Holm, Hochberg, Fallback and Dunnett are not. Their truncated versions are proposed which are separable and more powerful than the Bonferroni MTP. The proposed procedure is illustrated by a clinical trial example. (© 2008 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Resampling‐Based Empirical Bayes Multiple Testing Procedures for Controlling Generalized Tail Probability and Expected Value Error Rates: Focus on the False Discovery Rate and Simulation Study
Authors: Dudoit Sandrine , Gilbert1 Houston N. , van der Laan Mark J.
Published: October 2008

This article proposes resampling‐based empirical Bayes multiple testing procedures for controlling a broad class of Type I error rates, defined as generalized tail probability (gTP) error rates, gTP (q,g) = Pr(g (Vn,Sn) > q), and generalized expected value (gEV) error rates, gEV (g) = E [g (Vn,Sn)], for arbitrary functions g (Vn,Sn) of the numbers of false positives Vn and true positives Sn. Of particular interest are error rates based on the proportion g (Vn,Sn) = Vn /(Vn + Sn) of Type I errors among the rejected hypotheses, such as the false discovery rate (FDR), FDR = E [Vn /(Vn + Sn)]. The proposed procedures offer several advantages over existing methods. They provide Type I error control for general data generating distributions, with arbitrary dependence structures among variables. Gains in power are achieved by deriving rejection regions based on guessed sets of true null hypotheses and null test statistics randomly sampled from joint distributions that account for the dependence structure of the data. The Type I error and power properties of an FDR‐controlling version of the resampling‐based empirical Bayes approach are investigated and compared to those of widely‐used FDR‐controlling linear step‐up procedures in a simulation study. The Type I error and power trade‐off achieved by the empirical Bayes procedures under a variety of testing scenarios allows this approach to be competitive with or outperform the Storey and Tibshirani (2003) linear step‐up procedure, as an alternative to the classical Benjamini and Hochberg (1995) procedure. (© 2008 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Antioxidant Activity of the Anti‐Inflammatory Compound Ebselen: A Reversible Cyclization Pathway via Selenenic and Seleninic Acid Intermediates
Authors: Sarma Bani Kanta , Mugesh Govindasamy
Published: October 2008

A revised mechanism that accounts for the glutathione peroxidase (GPx)‐like catalytic activity of the organoselenium compound ebselen is described. It is shown that the reaction of ebselen with H2O2yields seleninic acid as the only oxidized product. The X‐ray crystal structure of the seleninic acid shows that the selenium atom is involved in a noncovalent interaction with the carbonyl oxygen atom. In the presence of excess thiol, the SeN bond in ebselen is readily cleaved by the thiol to produce the corresponding selenenyl sulfide. The selenenyl sulfide thus produced undergoes a disproportionation in the presence of H2O2to produce the diselenide, which upon reaction with H2O2, produces a mixture of selenenic and seleninic acids. The addition of thiol to the mixture containing selenenic and seleninic acids leads to the formation of the selenenyl sulfide. When the concentration of the thiol is relatively low in the reaction mixture, the selenenic acid undergoes a rapid cyclization to produce ebselen. The seleninic acid, on the other hand, reacts with the diselenide to produce ebselen as the final product. DFT calculations show that the cyclization of selenenic acids to the corresponding selenenyl amides is more favored than that of sulfenic acids to the corresponding sulfenyl amides. This indicates that the regeneration of ebselen under a variety of conditions protects the selenium moiety from irreversible inactivation, which may be responsible for the biological activities of ebselen.
ChemInform Abstract: Beyond the Icosahedron: A Density Functional Theory Study of 14‐Atom Germanium Clusters.
Authors: King R. Bruce , Silaghi‐Dumitrescu Ioan , Uta Matei M.
Published: October 2008

ChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
ChemInform Abstract: Isomorphism of Anhydrous Tetrahedral Halides and Silicon Chalcogenides: Energy Landscape of Crystalline BeF2, BeCl2, SiO2, and SiS2
Authors: Zwijnenburg Martijn A. , Cora Furio , Bell Robert G.
Published: October 2008

ChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
ChemInform Abstract: Ternary Rare‐Earth Titanium Antimonides RE2Ti11‐xSb14+x (RE: Sm, Gd, Tb, Yb).
Authors: Bie Haiying , Mar Arthur
Published: October 2008

ChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
ChemInform Abstract: The Crystal Structure of LaPtInH0.53.
Authors: Jensen I. J. T. , Fjellvaag H. , Hauback B. C.
Published: October 2008

ChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.