bluuebunny/crossref_metadata_2025_split huggingface dataset:
Surgical Approach and Patient-Reported Outcomes after Total Hip Replacement
Authors: Smith Alison J. , Wylde Vikki , Berstock James R. , Maclean Angus D. , Blom Ashley W. 
Published: August 2012

Background Previous research has mainly focused on how factors such as surgical approach might affect implant survivorship and the incidence of complications. Given the increasing interest in patient-reported outcomes, the purpose of this study is to explore whether surgical approach is associated with patient-reported pain, function, and satisfaction at 1–3 years after primary total hip replacement (THR). Methods Details of surgical factors were collated from operation notes for all consecutive patients at our centre from 2004–2006. All patients were mailed a questionnaire 1–3 years following surgery that collected WOMAC pain and function scores and the Self-Administered Patient Satisfaction Scale for Primary Hip and Knee Arthroplasty. Of the eligible 1,315 patients, 911 patients returned a completed questionnaire (69% response rate). Multivariable fractional logit models were used to identify whether surgical approach was associated with outcome scores. Results Surgical approach was found to be a significant predictor of patient-reported outcomes at 1–3 years after surgery, even after controlling for patient-specific factors. A posterior approach was associated with better scores on all three outcome measures. On average, predicted outcome scores for a typical patient with a posterior approach were between 3.5 and 7.2 percentage points higher than an equivalent patient with an anterolateral approach. Interpretation These findings suggest that clinical decisions concerning surgical approach may have an observable impact on patient-reported levels of pain, function, and satisfaction following THR.
Combined intravitreal ranibizumab and verteporfin photodynamic therapy versus ranibizumab alone for the treatment of age-related macular degeneration
Authors: Giustolisi Rosalia , Fantozzi Nicoletta , Staltari Mariateresa , Marchiori Jessica , Mastrangelo Olga , Marcucci Roberta , Mirra Federica , Mazzotta Paola , Gabrieli Corrado Balacco , The Luce Study Group  
Published: August 2012

PurposeTo compare same-day combined therapy of photodynamic therapy with verteporfin (PDT-V) and intravitreal ranibizumab versus monotherapy with ranibizumab for the treatment of choroidal neovascularization.
MethodsIn this prospective study, the total number of eyes were randomized into two groups: in the first, treatment consisted of a combined therapy of PDT-V and ranibizumab 0.5 mg on the same day; in the second, ranibizumab 0.5 mg in 3 monthly injections. Best-corrected visual acuity (BCVA) and central macular thickness (CMT) on optical coherence tomography (OCT) were recorded before and 6 months after treatment.
ResultsA total of 47 eyes of 47 subjects were enrolled in the study. In the combined-therapy group (group 1), the mean baseline BCVA ± standard deviation (SD) was 32.65 ± 11.09 letters (Snellen equivalent, 20/59); in the ranibizumab-alone group (group 2), 29.13 ± 9.03 letters (20/70). At 6 months’ follow-up, in group 1 the mean baseline BCVA was 39.06 ± 10.12 letters (20/42); in group 2, 33.87 ± 12.06 letters (20/57). Improvement was significant in both group 1 (P = 0.03) and group 2 (P = 0.002). In group 1, the mean CMT at baseline ± SD was 315 ± 95.49 μm; in group 2, 306.33 ± 71.61 μm. At 6 months’ follow-up, in group 1 it was 202 ± 52.02 μm; in group 2, 226 ± 65.58 μm. Reduction was significant in both group 1 (P = 0.0007) and group 2 (P = 0.00001). After 6-months, the rate of retreated eyes was 29.4% in group 1 and 43.3% in group 2. The need for retreatment did not depend on the treatment protocol (P = 0.34).
ConclusionsFrom a functional and anatomic point of view, the two treatments showed equivalent efficacy, with fewer retreatments in group 1. No serious adverse events, such as retinal detachment, endophthalmitis, or ocular hypertension occurred in either group.
Acute systemic histoplasmosis associated with chorioretinitis in an immunocompetent adolescent
Authors: Fowler Brian , Shen Christopher , Mastellone Joseph , Chaum Edward 
Published: August 2012

Histoplasmosis is an endemic, systemic mycosis caused by the dimorphic fungus Histoplasma capsulatum. A minority of patients develop asymptomatic chorioretinitis known as presumed ocular histoplasmosis syndrome (POHS), which is typically associated with chorioretinal scarring and peripapillary atrophy and occasionally with choroidal neovascularization secondary to maculopathy. We report a case of acute severe bilateral chorioretinitis associated with disseminated H. capsulatum in an immunocompetent adolescent boy living in an endemic area. The chorioretinitis did not respond to systemic antifungal therapy, but both his systemic illness and ocular lesions resolved with the addition of systemic steroids.
Concurrent endophthalmitis and orbital cellulitis in a child with congenital glaucoma and a glaucoma drainage device
Authors: Kassam Faazil , Lee Bonita E. , Damji Karim F. 
Published: August 2012

A 3-year-old monocular boy with a history of congenital glaucoma and aqueous shunt implantation presented with periorbital swelling, erythema, and purulent discharge in his seeing right eye. Examination revealed an extensive hypopyon. Computed tomography (CT) showed right-sided proptosis, and a displaced aqueous shunt device deep in the orbit. Significant purulent fluid was encountered when the device was removed; vitreous and orbital cultures revealed Streptococcus pneumoniae. The patient received intravitreal, topical, intravenous, and oral antibiotics as well as intraocular tissue plasminogen activator and topical steroid. Orbital and intraocular inflammation decreased significantly but he developed a cataract, further complicated by a retinal detachment.
Breeding behavior and sex ratio variation of Pleuroncodes monodon (Crustacea: Galatheidae) off the Peruvian coast
Authors: Franco-Meléndez MT 
Published: August 2012

The breeding behavior and sex ratio variation of the carrot squat lobster Pleuroncodes monodon were analyzed. Specimens were collected from experimental hauls in three acoustic pelagic resource assessment surveys during 2001 off the Peruvian coast. A total of 8517 individuals were differentiated according to sex. Males showed clear sexual dimorphism, while females were divided into ovigerous or nonovigerous individuals. Size did not differ significantly between sexes (Kolmogorov-Smirnov test, P > 0.05); average sizes were smaller than 20 mm cephalothoraxic length. The sex ratio indicated that in winter males were more predominant than females (χ2 test, P < 0.05). Ovigerous females were observed during all the period of study, with major activity in spring, and population dispersion occurred during the breeding season.
Dopaminergic Differentiation of Human Pluripotent Cells
Authors: Boyer Leah F. , Campbell Benjamin , Larkin Samantha , Mu Yanling , Gage Fred H. 
Published: August 2012

AbstractHere we describe protocols for the dopaminergic differentiation of pluripotent stem cells. We have optimized and compared two distinct protocols, both of which are chemically defined and applicable to both embryonic and induced pluripotent stem cells. First, we present a five‐step method based on rosette formation (Basic Protocol 1); then we describe a monolayer paradigm based on inhibition of alternate developmental pathways (Basic Protocol 2). Directed differentiation of pluripotent cells into specific cell types is a crucial step towards understanding human development and realizing the biomedical relevance of these cells, whether for replacement therapy or disease modeling. Curr. Protoc. Stem Cell Biol. 22:1H.6.1‐1H.6.11. © 2012 by John Wiley & Sons, Inc.
Scalable Production of Transplantable Dopaminergic Neurons from hESCs and iPSCs in Xeno‐Free Defined Conditions
Authors: Swistowski Andrzej , Zeng Xianmin 
Published: August 2012

AbstractHuman embryonic stem cells (hESCs) and induced pluripotent stem cells (iPSCs) are potentially an unlimited cell source for cell replacement therapy and personalized medicine. Before hESC‐ and iPSC‐based therapy can be moved from bench to bedside, however, it is essential to establish protocols for generating therapeutically relevant cells, like dopaminergic neurons in defined conditions that are suitable for scalable good manufacturing practice (GMP)‐compliant protocols. Here, the derivation and differentiation of functional dopaminergic neurons from hESCs and iPSCs under xeno‐free defined conditions are described. These protocols have been validated in multiple hESC and iPSC lines. Curr. Protoc. Stem Cell Biol. 22:2D.12.1‐2D.12.20. © 2012 by John Wiley & Sons, Inc.
Dependence of GFA and Crystallization Behaviors of Al86Ni9La5 Metallic Glass on Its Original Liquid State
Authors: Wang Zhi‐Zhi , Zu Fang‐Qiu , Zhang Zhang , Cui Xiao , Wang Li‐Fang , Sun Guo‐Dong 
Published: August 2012

AbstractDuring the heating process the temperature‐induced liquid–liquid structure transition (LLST) of Al86Ni9La5 melt from 1320 to 1490 K was identified by both electrical resistivity and differential thermal analysis measurement. The effects of liquid structural change on the crystallization behavior and glass forming ability (GFA) of Al86Ni9La5 alloy were investigated. The amorphous samples prepared at different melt temperatures according to the liquid states were obtained. The results showed that the formation of intermetallic compounds during the crystallization process of the samples prepared below and within the LLST temperature range was earlier than that prepared above the LLST temperature range. The sample prepared above the LLST temperature range had the lowest GFA.
Liquid Metal Marbles
Authors: Sivan Vijay , Tang Shi‐Yang , O'Mullane Anthony P. , Petersen Phred , Eshtiaghi Nicky , Kalantar‐zadeh Kourosh , Mitchell Arnan 
Published: August 2012

AbstractLiquid metal marbles that are droplets of liquid metal encapsulated by micro‐ or nanoparticles are introduced. Droplets of galinstan liquid metal are coated with insulators (including Teflon and silica) and semiconductors (including WO3, TiO2, MoO3, In2O3 and carbon nanotubes) by rolling over a powder bed and also by submerging in colloidal suspensions. It is shown that these marbles can be split and merged, can be suspended on water, and are even stable when moving under the force of gravity and impacting a flat solid surface. Furthermore, the marble coating can operate as an active electronic junction and the nanomaterial coated liquid metal marble can act as a highly sensitive electrochemical based heavy metal ion sensor. This new element thus represents a significant platform for the advancement of research into soft electronics.
Hyperbranched Polyglycerol‐Grafted Superparamagnetic Iron Oxide Nanoparticles: Synthesis, Characterization, Functionalization, Size Separation, Magnetic Properties, and Biological Applications
Authors: Zhao Li , Chano Tokuhiro , Morikawa Shigehiro , Saito Yukie , Shiino Akihiko , Shimizu Sawako , Maeda Takuro , Irie Takayoshi , Aonuma Shuji , Okabe Hidetoshi , Kimura Takahide , Inubushi Toshiro , Komatsu Naoki 
Published: August 2012

AbstractFor biomedical application of nanoparticles, the surface chemical functionality is very important to impart additional functions, such as solubility and stability in a physiological environment, and targeting specificity as an imaging probe and a drug carrier. Although polyethylene glycol (PEG) has been used extensively, here, it is proposed that hyperbranched polyglycerol (PG) is a good or even better alternative to PEG. Superparamagnetic iron oxide nanoparticles (SPIONs) prepared using a polyol method are directly functionalized with PG through ring‐opening polymerization of glycidol. The resulting SPION‐PG is highly soluble in pure water (>40 mg mL−1) and in a phosphate buffer solution (>25 mg mL−1). Such high solubility enables separation of SPION‐PG according to size using size exclusion chromatography (SEC). The size‐separated SPION‐PG shows a gradual increase in transverse relaxivity (r2) with increasing particle size. For biological application, SPION‐PG is functionalized through multistep organic transformations (–OH → –OTs (tosylate) → –N3 → –RGD) including click chemistry as a key step to impart targeting specificity by immobilization of cyclic RGD peptide (Arg‐Gly‐Asp‐D‐Tyr‐Lys) on the surface. The targeting effect is demonstrated by the cell experiments; SPION‐PG‐RGD is taken up by the cells overexpressing αvβ3‐integrin such as U87MG and A549.
A Highly Emissive Conjugated Polyelectrolyte Vector for Gene Delivery and Transfection
Authors: Feng Xuli , Lv Fengting , Liu Libing , Yang Qiong , Wang Shu , Bazan Guillermo C. 
Published: August 2012

AbstractAn intrinsically fluorescent cationic polyfluorene (CCP) has been designed, synthesized, characterized, and examined as a plasmid DNA (pDNA) delivery vector. This material facilitates nucleic acid binding, encapsulation and efficient cellular uptake. CCP can effectively protect pDNA against nuclease degradation, which is necessary for gene carriers. Green fluorescent protein (GFP) expression experiments reveal that CCP can achieve efficient delivery and transfection of pDNA encoding GFP gene with 92% efficiency, which surpasses that of commercial transfection agents, lipofectamine 2000 (Lipo) and polyethylenimine (PEI). CCP is also highly fluorescent, with 43% quantum yield in water, and exhibits excellent photostability, which allows for real‐time tracking the location of gene delivery and transfection. These features and capabilities represent a major step toward designing and applying conjugated polymers that function in both imaging and therapeutic applications.
Controlling Hierarchy in Solution‐processed Polymer Solar Cells Based on Crosslinked P3HT
Authors: Tao Chen , Aljada Muhsen , Shaw Paul E. , Lee Kwan H. , Cavaye Hamish , Balfour Michael N. , Borthwick Robert J. , James Michael , Burn Paul L. , Gentle Ian R. , Meredith Paul 
Published: August 2012

AbstractUnderstanding and controlling the morphology of donor/acceptor blends is critical for the development of solution processable organic solar cells. By crosslinking a poly(3‐n‐hexylthiophene‐2,5‐diyl) (P3HT) film we have been able to spin‐coat [6,6]‐phenyl‐C61‐butyric acid methyl ester (PCBM) onto the film to form a structure that is close to a bilayer, thus creating an ideal platform for investigating interdiffusion in this model system. Neutron reflectometry (NR) demonstrates that without any thermal treatment a smaller amount of PCBM percolates throughout the crosslinked P3HT when compared to a non‐crosslinked P3HT film. Using time‐resolved NR we also show thermal annealing increases the rate of diffusion, resulting in a near‐uniform distribution of PCBM throughout the polymer film. XPS measurements confirm the presence of both P3HT and PCBM at the annealed film's surface indicating that the two components are intermixed. Photovoltaic devices fabricated using this bilayer approach and suitable annealing conditions yielded comparable power conversion efficiencies to bulk heterojunction devices made from the same materials. The crosslinking procedure has also enabled the formation of patterned P3HT films by photolithography. Pillars with feature sizes down to 2 μm were produced and after subsequent deposition of PCBM and thermal annealing devices with efficiencies of up to 1.4% were produced.
Arthrogryposis (multiple congenital contractures) associated with failed termination of pregnancy
Authors: Hall Judith G. 
Published: August 2012

AbstractThe purpose of this study was to establish whether an increase in failed termination of pregnancy is observed among individuals with arthrogryposis (multiple congenital contractures). A total of 2,500 unselected patients with arthrogryposis collected by the author over a 35‐year period were reviewed. The cases included referrals, consultations, personal examinations, and correspondence. They were reviewed for clinical features, pregnancy complications, family history, and a history of attempted terminations of the pregnancy. Eleven cases of failed terminations of pregnancy in which the individuals were subsequently born with arthrogryposis were identified. We conclude that infants who survive attempted termination of pregnancy (both surgical and medical) may be at an increased risk to be born with multiple congenital contractures. Careful examination of the tissue and a post‐procedure ultrasound will help to assure that the termination has been successful. Those infants surviving attempted termination of pregnancy who have arthrogryposis are also at >50% risk for intellectual disability. © 2012 Wiley Periodicals, Inc.
SMARCAL1 deficiency predisposes to non‐Hodgkin lymphoma and hypersensitivity to genotoxic agents in vivo
Authors: Baradaran‐Heravi Alireza , Raams Anja , Lubieniecka Joanna , Cho Kyoung Sang , DeHaai Kristi A. , Basiratnia Mitra , Mari Pierre‐Olivier , Xue Yutong , Rauth Michael , Olney Ann Haskins , Shago Mary , Choi Kunho , Weksberg Rosanna A. , Nowaczyk Malgorzata J.M. , Wang Weidong , Jaspers Nicolaas G.J. , Boerkoel Cornelius F. 
Published: August 2012

AbstractSchimke immuno‐osseous dysplasia (SIOD) is a multisystemic disorder with prominent skeletal, renal, immunological, and ectodermal abnormalities. It is caused by mutations of SMARCAL1 (SWI/SNF‐related, matrix‐associated, actin‐dependent regulator of chromatin, subfamily a‐like 1), which encodes a DNA stress response protein. To determine the relationship of this function to the SIOD phenotype, we profiled the cancer prevalence in SIOD and assessed if defects of nucleotide excision repair (NER) and nonhomologous end joining (NHEJ), respectively, explained the ectodermal and immunological features of SIOD. Finally, we determined if Smarcal1del/del mice had hypersensitivity to irinotecan (CPT‐11), etoposide, and hydroxyurea (HU) and whether exposure to these agents induced features of SIOD. Among 71 SIOD patients, three had non‐Hodgkin lymphoma (NHL) and one had osteosarcoma. We did not find evidence of defective NER or NHEJ; however, Smarcal1‐deficient mice were hypersensitive to several genotoxic agents. Also, CPT‐11, etoposide, and HU caused decreased growth and loss of growth plate chondrocytes. These data, which identify an increased prevalence of NHL in SIOD and confirm hypersensitivity to DNA damaging agents in vivo, provide guidance for the management of SIOD patients. © 2012 Wiley Periodicals, Inc.
Atypical amyoplasia congenita in an infant with Leigh syndrome: A mitochondrial cause of severe contractures?
Authors: Wilnai Yael , Seaver Laurie H. , Enns Gregory M. 
Published: August 2012

AbstractAmyoplasia congenita is a distinct form of arthrogryposis with characteristic features including internally rotated and adducted shoulders, extended elbows, flexion, and ulnar deviation of the wrists, and adducted thumbs. Fetal hypokinesia, secondary to a variety of genetic conditions, neuromuscular disorders, and environmental agents, is associated with contractures. In order to increase our understanding of the phenotypic spectrum associated with SURF 1 deficiency, a common cause of mitochondrial respiratory chain complex IV deficiency and Leigh syndrome, we describe a now 6‐year‐old boy who presented in the neonatal period with amyoplasia congenita. His development was normal until age 10.5 months, at which time he developed severe hypotonia and choreoathetosis following an episode of viral gastroenteritis. Following the onset of neurological symptoms, he gradually developed severe kyphosis and lower limb contractures. Blood and cerebrospinal fluid lactate levels were elevated and head imaging showed characteristic features of Leigh syndrome. He was found to harbor two pathogenic heterozygous mutations in the SURF 1 gene. In this case, mitochondrial dysfunction and the resultant energy deficiency may have played a role in causing abnormal neuronal development during embryogenesis, causing arthrogryposis. A variety of mitochondrial respiratory chain complex deficiencies have been associated with contractures of varying severity. Therefore, mitochondrial disorders should be considered in the differential diagnosis of neonatal arthrogryposis, especially if other characteristic findings such as lactic acidemia or basal ganglia abnormalities are present. © 2012 Wiley Periodicals, Inc.
Normative growth charts for individuals with Costello syndrome
Authors: Sammon Mary R. , Doyle Dan , Hopkins Elizabeth , Sol‐Church Katia , Stabley Deborah L. , McGready John , Schulze Kerry , Alade Yewande , Hoover‐Fong Julie , Gripp Karen W. 
Published: August 2012

AbstractCostello syndrome is a rare condition due to heterozygous germline mutations in the proto‐oncogene HRAS. It affects multiple organ systems and includes severe failure‐to‐thrive, short stature, and macrocephaly. The goal of this study was to develop Costello syndrome‐specific growth curves. We collected height, weight, and head circumference (OFC) measurements from 94 individuals (45 males and 49 females). Their HRAS mutation spectrum reflects previously published cohorts, with p.G12S in 77.7%. Participants received medical care, therefore our data does not reflect natural history per se, but rather growth with nutritional support. Due to limited cohort size, we analyzed data from males and females together. Weight‐for‐age data included 417 separate measurements from 80 individuals age 0–36 months, and 585 measurements from 82 individuals for age 0–10 years. Height‐for‐age data were derived from 391 measurements from 77 individuals age 0–36 months, and 591 measurements from 90 individuals age 0–10 years. Measurements obtained after growth hormone exposure in 15 individuals were excluded in this analysis. The OFC curve was derived from 221 measurements from 55 individuals age 0–36 months. Centiles (5th, 50th, and 95th) were estimated across the age continuum for each growth parameter, and compared to gender‐specific curves for average stature individuals. The resulting curves demonstrate very slow weight gain in the first 2 years. Short stature is seen in many, but after age 4 years the 95th centile for height falls within the low normal range for average stature children. Head circumference curves largely overlap those for average stature, reflecting relative macrocephaly. © 2012 Wiley Periodicals, Inc.
SNP array and phenotype correlation shows that FLI1 deletion per se is not responsible for thrombocytopenia development in Jacobsen syndrome
Authors: Trkova Marie , Becvarova Vera , Hynek Martin , Hnykova Lenka , Hlavova Eva , Kreckova Gabriela , Kulovany Eduard , Cutka David , Zatloukalova Jitka , Markova Kristyna , Sukova Martina , Horacek Jiri , Stejskal David 
Published: August 2012

AbstractJacobsen syndrome (JBS) is a rare chromosomal disorder caused by terminal deletion of the long arm of chromosome 11. We report on four prenatally diagnosed patients with JBS with variable prenatal and postnatal phenotypes and 11q deletions of varying sizes. Precise characterization of the deleted region in three patients was performed by SNP arrays. The severity of both the prenatal and postnatal phenotypes did not correlate with the size of the haploinsufficient region. Despite the large difference in the deletion size (nearly 6 Mb), both of the live‐born patients had similar phenotypes corresponding to JBS. However, one of the most prominent features of JBS, thrombocytopenia, was only present in the live‐born boy. The girl, who had a significantly longer deletion spanning all four genes suspected of being causative of JBS‐related thrombocytopenia (FLI1, ETS1, NFRKB, and JAM3), did not manifest a platelet phenotype. Therefore, our findings do not support the traditional view of deletion size correlation in JBS or the causative role of FLI1, ETS1, NFRKB, and JAM3 deletion per se for the development of disease‐related thrombocytopenia. © 2012 Wiley Periodicals, Inc.