bluuebunny/crossref_metadata_2025 huggingface dataset:
Effects of ice particles shattering on optical cloud particle probes
Authors: Lawson R. P.
Published: February 2011

Abstract. Recently, considerable attention has been focused on the issue of large ice particles shattering on the inlets and tips of cloud particle probes, which produces copious ice particles that can be mistakenly measured as real ice particles. Currently two approaches are being used to mitigate the problem: (1) Based on recent high-speed video in icing tunnels, probe tips have been designed that reduce the number of shattered particles that reach the probe sample volume, and (2) Post processing techniques such as image processing and using the arrival time of each individual particle. This paper focuses on exposing suspected errors in measurements of ice particle size distributions due to shattering, and evaluation of the two techniques used to reduce the errors. Data from 2D-S probes constitute the primary source of our investigation, however, comparisons with 2D-C and CIP measurements are also included. Analysis of 2D-S data shows that a particle arrival time algorithm is more effective than probe tips designed to reduce shattering, although application of both techniques ought to be complementary. This finding contrasts results from a recent investigation that found that modified probe tips were more effective than an arrival time algorithm when applied to 2D-C and CIP measurements. The reason for these differing results may be linked to the improved ability of the 2D-S to image small ice particles. The analysis techniques in this paper can be used to estimate the effects of shattering. For example, the additional spurious concentration of (small) shattered ice particles can be measured as a function of the mass concentration of (large) ice particles. The analysis provides estimates of upper bounds on the concentration of natural ice, and on the remaining concentration of shattered ice particles after application of the post-processing techniques. However, a comprehensive investigation of shattering is required to quantify effects that arise from the multiple degrees of freedom associated with this process, including different cloud environments, probe geometries, airspeed, angle of attack, particle size and type.
Estimating maximum global land surface wind power extractability and associated climatic consequences
Authors: Miller L. M. , Gans F. , Kleidon A.
Published: February 2011

Abstract. The availability of wind power for renewable energy extraction is ultimately limited by how much kinetic energy is generated by natural processes within the Earth system and by fundamental limits of how much of the wind power can be extracted. Here we use these considerations to provide a maximum estimate of wind power availability over land. We use several different methods. First, we outline the processes associated with wind power generation and extraction with a simple power transfer hierarchy based on the assumption that available wind power will not geographically vary with increased extraction for an estimate of 68 TW. Second, we set up a simple momentum balance model to estimate maximum extractability which we then apply to reanalysis climate data, yielding an estimate of 21 TW. Third, we perform general circulation model simulations in which we extract different amounts of momentum from the atmospheric boundary layer to obtain a maximum estimate of how much power can be extracted, yielding 18–34 TW. These three methods consistently yield maximum estimates in the range of 18–68 TW and are notably less than recent estimates that claim abundant wind power availability. Furthermore, we show with the general circulation model simulations that some climatic effects at maximum wind power extraction are similar in magnitude to those associated with a doubling of atmospheric CO2. We conclude that in order to understand fundamental limits to renewable energy resources, as well as the impacts of their utilization, it is imperative to use a "top-down" thermodynamic Earth system perspective, rather than the more common "bottom-up" engineering approach.
On the precision of Ptolemy's geographic coordinates in his Geographike Hyphegesis
Authors: Marx C.
Published: February 2011

Abstract. In his Geographike Hyphegesis (~150 AD) Claudius Ptolemy catalogued the positions of over 6300 ancient locations in the form of geographic longitude and latitude. A determination of the frequencies of the fractions of the degrees indicates that the coordinates are given with different precision/resolution and larger regional differences. The author's assumptions concerning the origin of the detected form of the frequency distribution were examined with statistical hypothesis tests and partly confirmed. Differences between the frequencies of the Ω- and Ξ-recension were investigated in terms of possible transcription errors in the manuscripts. A calculation method is given with which the properties of coordinates of different resolutions can be estimated. It is applied both globally and regionally. Such basic information about the precision of the Ptolemaic coordinates is important for detecting systematic and gross errors in the Ptolemaic coordinates and thereby for the identification of the Ptolemaic locations with their today's place.
Applied Psychophysiology and Cognitive and Behavioral Therapy in the Treatment of Sex Offenders1
Authors: Corson John A
Published: February 2011

This article presents an application of applied psychophysiological and cognitive and behavioral strategies to the treatment of sex offenders. The participants were 21 sex offenders treated as outpatients at the Veterans Administration Medical Center in White River Junction, Vermont. A repeated-case-study format was used. Because hyperventilation destabilizes the autonomic nervous system (disrupting and/or preventing erection and other aspects of male sexual arousal) and dramatically changes brain function (degrading train of thought and shifting neuroendocrine function), clients were taught to hyperventilate in response to thoughts and images of the targets of their illegal behavior. Psychophysiological indices included skin conductance and penile plethysmograph. Participants were treated in six individual sessions, and most have been followed up for more than 2 years. Results show that with motivated clients, this procedure produces convincing, positive results.
Remembering Neal Miller
Authors: Kappes Bruno
Published: February 2011

The author describes two encounters with Neal Miller, including a discussion of the pioneering curare/visceral learning experiments and of Miller's perspective on the biofeedback training paradigm.
Following Neal Miller's Footprints: Integrating Biofeedback With the Psychodynamic, Relational, and Intersubjective Approach
Authors: Rolnick Arnon , Rickles William
Published: February 2011

This article provides a brief review of Neal Miller's translation of psychoanalytic theoretical concepts into operational behavioral research and explores relevant interactions of clinical biofeedback and psychoanalytic practice, both now and in Miller's time. Presently, psychoanalytic psychotherapists are more concerned with both the analyst's and the analysand's contribution to the intersubjective field of the therapeutic endeavor than with modifying biologically based, instinctual urges, as they were in Miller's time. Current psychoanalytic theory translates directly into the biofeedback therapeutic situation via the exploration of interpersonal relationship dynamics, or the intersubjective field, which includes the patient, therapist, and biofeedback instrumentation. All figure significantly in the patient's acquisition of a biofeedback task.
A Tribute to Randy Neblett
Authors: Shaffer Fred , Crawford Judy
Published: February 2011

Randy Neblett guided the Biofeedback Certification International Alliance (BCIA) through almost 3 years of intense change following a visionary strategic plan. This article pays tribute to Randy's leadership and highlights BCIA's major achievements in didactic education, creation of new certifications, development of new options to fulfill the human anatomy/physiology requirement, revision of our certification exams, online testing, recertification, ethics, marketing our organization and certifications, advocacy, and international outreach.
Improvement in S-cone–mediated Visual Fields and Rod Function after Correction of Vitamin a Deficiency
Authors: Hayashi Takaaki , Gekka Tamaki , Nakano Tadashi , Tsuneoka Hiroshi
Published: February 2011

Purpose

To evaluate S-cone–mediated visual fields and full-field electroretinograms (ERGs) in a patient with vitamin A deficiency.

Methods

A 65-year-old woman diagnosed with primary sclerosing cholangitis reported experiencing night blindness. The patient underwent comprehensive ophthalmic examination, including funduscopy ERGs, Humphrey standard automated perimetry (SAP), and short-wavelength automated perimetry (SWAP). Serum vitamin A concentrations were measured.

Results

The patient's best-corrected visual acuity was 1.2 in both eyes. The ERG results showed no rod b-waves, reduced combined rod-plus-cone responses (negative type), and normal cone and 30-Hz flicker responses. Serum vitamin A concentration was 18 lU/dL (normal range 97–316 lU/dL). The SAP mean deviation (MD) values were −1.09 dB (OD) and −0.97 dB (OS), whereas the SWAP MD values were −10.10 dB (OD) and −10.50 dB (OS). The rate of sensitivity decreased with increasing eccentricity in SWAP. Four months after starting oral administration of vitamin A, all ERG values were normalized, and SWAP MD values were greatly improved (OD −3.47 dB, OS −4.10 dB) compared with changes in SAP MD values (OD +0.67 dB, OS +0.41 dB). Rod dysfunction and impaired S-cone–mediated pathways were preferentially observed and found to be reversed after the treatment.

Conclusions

The findings in this patient suggest that rods and S cones are more susceptible to vitamin A deficiency than L and M cones. Vitamin A deficiency visual impairment may therefore be reversible with appropriate therapy.
Lack of Effect of Intraocular Lens Asphericity on Visual Performance with Acrylic Intraocular Lenses
Authors: Muñoz Gonzalo , Albarrán-Diego César , Galotto Ma Ángeles , Pascual Javier , Ferrer-Blasco Teresa
Published: February 2011

Purpose.

To determine whether implantation of acrylic intraocular lens (IOL) with aspheric design (Tecnis Z9003, AMO) results in improved visual acuity or contrast sensitivity compared with conventional spherical acrylic IOL (AR40e, AMO).

Methods.

In an intraindividual randomized prospective study of 60 patients with bilateral cataract, the Tecnis Z9003 IOL was compared with the AR40e IOL. Ocular aberrations for a 4.0-mm pupil and 6.0-mm pupil were measured with a Hartmann-Shack aberrometer. Quality of vision was measured using visual acuity and contrast sensitivity under mesopic and photopic conditions.

Results.

Eyes with the Tecnis Z9003 IOL had significantly less spherical aberration and greater Strehl ratio after surgery, showing a better optical quality in comparison with the standard spherical IOL. However, visual acuity and both mesopic and photopic contrast sensitivity were not significantly different between the groups.

Conclusions.

The significantly better optical quality achieved with the aspheric acrylic IOL design did not result in improved visual acuity or contrast sensitivity in comparison with a conventional spherical acrylic IOL.
A Review of Neuroendoscopy and Potential Applications in Veterinary Medicine
Authors: Higginbotham Michael , Levesque Donald
Published: February 2011

The endoscope was first developed over 200 yr ago. Endoscopy has since been applied to many disciplines of medicine. Its application to the nervous system was initially slow and not widely accepted and mainly involved the biopsy of tumors and the treatment of hydrocephalus. Several reasons for neuroendoscopy's limited use include inadequate endoscope technology, high skill level required, the advent of the surgical microscope, and the development of other treatments such as ventricular shunting. Over the past 50 yr, improvements in optical glass lenses, fiber optics, and electrical circuitry has led to better equipment and a revival of neuroendoscopy. Neuroendoscopy is now used in many diseases in human medicine including hydrocephalus, neoplasia, and intracranial cysts. This review presents the history of neuroendoscopy, the equipment and technology used, and the possible translation of techniques currently used in human medicine to veterinary medicine.
Measurement of Tumor Cytolysis by Macrophages
Authors: Escórcio‐Correia Mónica , Hagemann Thorsten
Published: February 2011

This unit describes two different protocols for the measurement of tumor cytolysis by macrophages. Traditionally, cytotoxicity assays have relied on the use of radioactive isotopes. In Basic Protocol 1, cytotoxic activity is measured by the release into the culture supernatant of a radioisotope that had been incorporated by the target cell and is released upon cell death. This poses a problem for some cell lines in which spontaneous isotope release occurs in the absence of effector cell cytotoxicity. In Basic Protocol 2, a nonradioactive approach is used to measure cytolysis that relies on the fluorescence staining of tumor cells with cell‐death markers. It also provides the obvious advantage of avoiding the use of hazardous radioactive materials. Curr. Protoc. Immunol. 92:14.18.1‐14.18.11. © 2011 by John Wiley & Sons, Inc.
In Vivo and Ex Vivo Protocols for Measuring the Killing of Extracellular Pathogens by Macrophages
Authors: Medina Eva , Goldmann Oliver
Published: February 2011

This unit describes a series of in vivo/ex vivo combined protocols for investigating the interactions (adhesion, phagocytosis, and killing) of extracellular bacteria with peritoneal murine macrophages. It includes steps needed for in vivo infection of murine peritoneal macrophages after intraperitoneal inoculation with the pathogen of interest, as well as the measurement of bacteria associated with or truly internalized by these phagocytic cells. Several protocols for the ex vivo measurement of the ability of peritoneal macrophages to kill the microorganisms that have been ingested during the in vivo infection assay are included. Curr. Protoc. Immunol. 92:14.19.1‐14.19.17. © 2011 by John Wiley & Sons, Inc.
Measurement of Macrophage Growth and Differentiation
Authors: Chitu Violeta , Yeung Yee‐Guide , Yu Wenfeng , Nandi Sayan , Stanley E. Richard
Published: February 2011

This unit provides protocols for measuring the abundance and growth of macrophage precursors in agar cultures and the proliferation of isolated mature macrophages in vitro, by either direct cell counting or by DNA measurement. Methods for the immunohistochemical identification of macrophages and the determination of their proliferative status in vivo by immunofluorescence are also included. It also describes methods for characterization of macrophage differentiation through the immunofluorescence analysis of cell‐surface expression of CSF‐1 receptor. Curr. Protoc. Immunol. 92:14.20.1‐14.20.26. © 2011 by John Wiley & Sons, Inc.
Carbide‐Derived Carbons – From Porous Networks to Nanotubes and Graphene
Authors: Presser Volker , Heon Min , Gogotsi Yury
Published: February 2011

Carbide‐derived carbons (CDCs) are a large family of carbon materials derived from carbide precursors that are transformed into pure carbon via physical (e.g., thermal decomposition) or chemical (e.g., halogenation) processes. Structurally, CDC ranges from amorphous carbon to graphite, carbon nanotubes or graphene. For halogenated carbides, a high level of control over the resulting amorphous porous carbon structure is possible by changing the synthesis conditions and carbide precursor. The large number of resulting carbon structures and their tunability enables a wide range of applications, from tribological coatings for ceramics, or selective sorbents, to gas and electrical energy storage. In particular, the application of CDC in supercapacitors has recently attracted much attention. This review paper summarizes key aspects of CDC synthesis, properties, and applications. It is shown that the CDC structure and properties are sensitive to changes of the synthesis parameters. Understanding of processing–structure–properties relationships facilitates tuning of the carbon material to the requirements of a certain application.
Highly Chemo‐ and Regioselective Transfer Reduction of Aromatic Nitro Compounds using Ammonium Formate Catalyzed by Supported Gold Nanoparticles
Authors: Lou Xia‐Bing , He Lin , Qian Yue , Liu Yong‐Mei , Cao Yong , Fan Kang‐Nian
Published: February 2011

A highly chemo‐ and regioselective reduction of a wide diversity of aromatic nitro compounds to the corresponding amines has been achieved by a combination of gold nanoparticles supported on titania and ammonium formate (HCOONH4) in ethanol at room temperature. Furthermore, a direct and mild route to formanilides from aromatic nitro compounds bearing different functional groups by reductive N‐formylation using the gold‐mediated transfer reduction protocol is also established.
A Novel Carbene Ruthenium Complex as Reusable and Selective Two‐Electron Catalyst for Alkene Epoxidation
Authors: Dakkach Mohamed , Fontrodona Xavier , Parella Teodor , Atlamsani Ahmed , Romero Isabel , Rodríguez Montserrat
Published: February 2011

A new ruthenium aquo catalyst with the formula trans‐[Ru(II)(CN‐Me)(trpy)OH2](PF6)2 [where trpy=2,2′:6′,2′′‐terpyridine and CN‐Me=3‐methyl‐1‐(pyridin‐2‐yl)‐imidazolylidene] has been prepared and thoroughly characterized by spectroscopic and electrochemical techniques. The complex has been tested in epoxidation catalysis both in dichloromethane and dichloromethane:ionic liquid media, displaying excellent performances and selectivities. Reuse of the catalyst in ionic liquid:solvent media has been explored for the first time in ruthenium‐mediated epoxidation catalysis and its performance is fully maintained for up to ten runs.
Bicyclic Cyclopentenones via the Combination of an Iridium‐ Catalyzed Allylic Substitution with a Diastereoselective Intramolecular Pauson–Khand Reaction
Authors: Farwick Andreas , Engelhart Jens U. , Tverskoy Olena , Welter Carolin , Umlauf (née Stang) Quendolin A. , Rominger Frank , Kerr William J. , Helmchen Günter
Published: February 2011

Enantioselective syntheses of bicyclic cyclopentenones are described. Key steps are an iridium‐catalyzed allylic substitution and an intramolecular diastereoselective Pauson–Khand reaction. The diastereoselectivity of the Pauson–Khand reaction was found to be crucially dependent on the unit connecting the propargylic and the olefinic parts of the precursor. Very high degrees of diastereoselection were obtained with an NBoc unit as connector. This methodology has been illustrated by application within an enantioselective formal total synthesis of (−)‐α‐kainic acid.
Copper(I)‐Mediated Cyanation of Boronic Acids
Authors: Zhang Guoying , Zhang Lingli , Hu Maolin , Cheng Jiang
Published: February 2011

A simple cyanation reaction of boronic acids with cuprous cyanide is achieved, providing nitriles in moderate to good yields. In the presence of copper(I) iodide, trimethylsilyl cyanide is also a good cyanating reagent. The reaction can be conducted on a 10‐mmol scale. Thus, this new approach represents an exceedingly practical method for the synthesis of nitriles.
Predicted costs of iron‐chelators in myelodysplastic syndromes: A 10‐year analysis based on actual prevalence and red cell transfusion rates
Authors: Durairaj Senthilkumar , Chew Stephen , Hyslop Ann , Keenan Norene , Groves Michael J. , Tauro Sudhir
Published: February 2011

Consideration of iron‐chelation (IC) in transfusion‐dependent patients is recommended in most clinical‐practice guidelines on myelodysplastic syndromes (MDS). The financial impact of IC on health‐care systems is predicted through economic modeling, but an analysis based on actual prevalence is lacking. Here, we have investigated the potential drug‐costs and need for IC in a cohort of 189 United Kingdom‐based MDS patients diagnosed from 2000 to 2010. Patients with low or intermediate‐1 IPSS scores were identified as eligible for IC if ≥24 red cell units (RCU) had been transfused over 12 consecutive months or the transfusion‐intensity averaged ≥2 RCU per month. Drug‐costs were calculated from the time patients qualified for IC until death or last follow‐up. In 159 patients with low/intermediate‐1 MDS, survival was superior with a low IPSS score (P = 0.014), age <70 years (P = 0.043), transfusion‐independence at diagnosis (P = 0.0056) and transfusion‐intensity of <2 RCU per month (P = 0.009). Reflecting the time elapsed since diagnosis, longer survival was observed with a cumulative red cell load of ≥75 U (P = 0.046). By logistic‐regression analysis, transfusion‐intensity independently predicted survival (P = 0.0035) in low and intermediate‐1 risk MDS patients. Forty‐one patients fulfilled criteria for consideration of IC. Of these, 6 patients died within 1 month; 35 patients survived for a median of 16 months (range 1–61). Had patients commenced IC, the anticipated drug‐costs alone would have been ∼$526,880–$2,064,800 over 10 years. The lack of association between cumulative transfusion‐load and survival calls for a prospective evaluation of the cost‐utility of IC in patients surviving long‐term, to enable evidence‐based recommendations in MDS management. Am. J. Hematol.86:406–410, 2011. © 2011 Wiley‐Liss, Inc.
Dysbindin‐1 gene contributes differentially to early‐ and adult‐onset forms of functional psychosis
Authors: Fatjó‐Vilas Mar , Papiol Sergi , Estrada Gemma , Bombín Igor , Peralta Victor , Rosa Araceli , Parellada Mara , Miret Salvador , Martín María , Lázaro Luisa , Campanera Sílvia , Muñoz Ma José , Lera‐Miguel Sara , Arias Bárbara , Navarro Ma Eulalia , Castro‐Fornieles Josefina , Cuesta Manuel J. , Arango Celso , Fañanás Lourdes
Published: February 2011

Dysbindin‐1 is a relatively ubiquitous protein in the brain which is involved in the modulation of synaptic homeostasis. The dysbindin‐1 gene (DTNBP1) has been associated with schizophrenia and bipolar disorder diagnoses. However, its contribution to the severity of the clinical and neurocognitive expression of these disorders remains controversial. We aimed to explore the association betweenDTNBP1and the phenotypes which are more directly linked with the underlying biology, such as age at onset and neurocognitive impairment. The present family sample comprised 894 Caucasian individuals: 268 patients affected by functional psychosis [58% with illness onset before 18 years, mean age at onset (SD): 14.71 (2.10)], 483 parents and 143 siblings. TenDTNBP1single nucleotide polymorphisms were genotyped in all individuals and their transmission disequilibrium was tested in relation to: (i) the risk for psychosis; (ii) patients' age at onset; and (iii) familial neurocognitive performance (including IQ estimation and executive functioning). In early‐onset families a 5‐marker haplotype encompassing exons 2–4 and the surrounding introns was significantly over‐transmitted to cases, while in adult‐onset families two haplotypes corresponding to the region between introns 4 and 7 were over‐transmitted to cases. Estimated IQ was associated with the rs760666 marker in the whole sample, whereas a significant association between executive functioning and the rs2619522 marker appeared in early‐onset families. Our findings confirm the role of the dysbindin‐1 gene in the risk for functional psychosis and show a differential haplotypic risk pattern in families with early as opposed to adult onset in the affected offspring. © 2011 Wiley‐Liss, Inc.
Genome‐wide association analysis of age at onset and psychotic symptoms in bipolar disorder
Authors: Belmonte Mahon Pamela , Pirooznia Mehdi , Goes Fernando S. , Seifuddin Fayaz , Steele Jo , Lee Phil Hyoun , Huang Jie , Hamshere Marian L. ,   The Bipolar Genome Study (BiGS) Consortium, The Wellcome Trust Case Control Consortium Bipolar Disorder Group, DePaulo J. Raymond , Kelsoe John R. , Rietschel Marcella , Nöthen Markus , Cichon Sven , Gurling Hugh , Purcell Shaun , Smoller Jordan W. , Craddock Nick , Schulze Thomas G. , McMahon Francis J. , Potash James B. , Zandi Peter P.
Published: February 2011

Genome‐wide association studies (GWAS) have identified several susceptibility loci for bipolar disorder (BP), most notably ANK3. However, most of the inherited risk for BP remains unexplained. One reason for the limited success may be the genetic heterogeneity of BP. Clinical sub‐phenotypes of BP may identify more etiologically homogeneous subsets of patients, which can be studied with increased power to detect genetic variation. Here, we report on a mega‐analysis of two widely studied sub‐phenotypes of BP, age at onset and psychotic symptoms, which are familial and clinically significant. We combined data from three GWAS: NIMH Bipolar Disorder Genetic Association Information Network (GAIN‐BP), NIMH Bipolar Disorder Genome Study (BiGS), and a German sample. The combined sample consisted of 2,836 BP cases with information on sub‐phenotypes and 2,744 controls. Imputation was performed, resulting in 2.3 million SNPs available for analysis. No SNP reached genome‐wide significance for either sub‐phenotype. In addition, no SNP reached genome‐wide significance in a meta‐analysis with an independent replication sample. We had 80% power to detect associations with a common SNP at an OR of 1.6 for psychotic symptoms and a mean difference of 1.8 years in age at onset. Age at onset and psychotic symptoms in BP may be influenced by many genes of smaller effect sizes or other variants not measured well by SNP arrays, such as rare alleles. © 2011 Wiley‐Liss, Inc.
Age at onset of psychotic disorder: Cannabis, BDNF Val66Met, and sex‐specific models of gene–environment interaction
Authors: Decoster Jeroen , van Os Jim , Kenis Gunter , Henquet Cecile , Peuskens Joseph , De Hert Marc , van Winkel Ruud
Published: February 2011

Discovering modifiable predictors for age at onset may help to identify predictors of transition to psychotic disorder in the “at‐risk mental state.” Inconsistent effects of sex, BDNF Val66Met (rs6265), and cannabis use on age of onset were previously reported. BDNF Val66Met and cannabis use before illness onset were retrospectively assessed in a sample of 585 patients with schizophrenia and their association with age at onset was evaluated. Cannabis use was significantly associated with earlier age at onset of psychotic disorder (AOP; average difference 2.7 years,P < 0.001), showing dose–response effects with higher frequency and earlier age at first use. There was a weak association between BDNF Val66Met genotype and AOP (difference 1.2 years;P = 0.050). No evidence was found for BDNF × cannabis interaction (interactionχ2(1) = 0.65,P = 0.420). However, a significant BDNF × cannabis × sex interaction was found (interactionχ2(1) = 4.99,P = 0.026). In female patients, cannabis use was associated with earlier AOP in BDNF Met‐carriers (difference 7 years), but not in Val/Val‐genotypes. In male patients, cannabis use was associated with earlier AOP irrespective of BDNF Val66Met genotype (difference 1.3 years). BDNF Val66Met genotype in the absence of cannabis use did not influence AOP, neither in female or male patients with psychotic disorder. Complex interactions between cannabis and BDNF may shape age at onset in female individuals at risk of psychotic disorder. No compelling evidence was found that BDNF genotype is associated with age at onset of psychotic disorder in the absence of cannabis use. © 2011 Wiley‐Liss, Inc.
Lysinuric protein intolerance: Reviewing concepts on a multisystem disease
Authors: Sebastio Gianfranco , Sperandeo Maria P. , Andria Generoso
Published: February 2011

Lysinuric protein intolerance (LPI) is an inherited aminoaciduria caused by defective cationic amino acid transport at the basolateral membrane of epithelial cells in intestine and kidney. LPI is caused by mutations in the SLC7A7 gene, which encodes the y+LAT‐1 protein, the catalytic light chain subunit of a complex belonging to the heterodimeric amino acid transporter family. LPI was initially described in Finland, but has worldwide distribution. Typically, symptoms begin after weaning with refusal of feeding, vomiting, and consequent failure to thrive. Hepatosplenomegaly, hematological anomalies, neurological involvement, including hyperammonemic coma are recurrent clinical features. Two major complications, pulmonary alveolar proteinosis and renal disease are increasingly observed in LPI patients. There is extreme variability in the clinical presentation even within individual families, frequently leading to misdiagnosis or delayed diagnosis. This condition is diagnosed by urine amino acids, showing markedly elevated excretion of lysine and other dibasic amino acids despite low plasma levels of lysine, ornithine, and arginine. The biochemical diagnosis can be uncertain, requiring confirmation by DNA testing. So far, approximately 50 different mutations have been identified in the SLC7A7 gene in a group of 142 patients from 110 independent families. No genotype–phenotype correlation could be established. Therapy requires a low protein diet, low‐dose citrulline supplementation, nitrogen‐scavenging compounds to prevent hyperammonemia, lysine, and carnitine supplements. Supportive therapy is available for most complications with bronchoalveolar lavage being necessary for alveolar proteinosis. © 2011 Wiley‐Liss, Inc.
Disorders of creatine transport and metabolism
Authors: Longo Nicola , Ardon Orly , Vanzo Rena , Schwartz Elizabeth , Pasquali Marzia
Published: February 2011

Creatine is a nitrogen containing compound that serves as an energy shuttle between the mitochondrial sites of ATP production and the cytosol where ATP is utilized. There are two known disorders of creatine synthesis (both transmitted as autosomal recessive traits: arginine: glycine amidinotransferase (AGAT) deficiency; OMIM 602360; and guanidinoacetate methyltransferase (GAMT) deficiency (OMIM 601240)) and one disorder of creatine transport (X‐linked recessive SLC6A8 creatine transporter deficiency (OMIM 300036)). All these disorders are characterized by brain creatine deficiency, detectable by magnetic resonance spectroscopy. Affected patients can have mental retardation, hypotonia, autism or behavioral problems and seizures. The diagnosis of these conditions relies on the measurement of plasma and urine creatine and guanidinoacetate. Creatine levels in plasma are reduced in both creatine synthesis defects and guanidinoacetate is increased in GAMT deficiency. The urine creatine/creatinine ratio is elevated in creatine transporter deficiency with normal plasma levels of creatine and guanidinoacetate. The diagnosis is confirmed in all cases by DNA testing or functional studies. Defects of creatine biosynthesis are treated with creatine supplements and, in GAMT deficiency, with ornithine and dietary restriction of arginine through limitation of protein intake. No causal therapy is yet available for creatine transporter deficiency and supplementation with the guanidinoacetate precursors arginine and glycine is being explored. The excellent response to therapy of early identified patients with GAMT or AGAT deficiency candidates these condition for inclusion in newborn screening programs. © 2011 Wiley‐Liss, Inc.
Endoscopic sinus surgery reduces antibiotic utilization in rhinosinusitis
Authors: Bhandarkar Naveen D. , Mace Jess C. , Smith Timothy L.
Published: February 2011

Background

Antibiotics are a mainstay of treatment for chronic rhinosinusitis (CRS) and recurrent acute rhinosinusitis (RARS). Although quality‐of‐life outcomes following endoscopic sinus surgery (ESS) have been studied, the change in antibiotic utilization following ESS is less well known.

Objective

We aimed to determine the effect of ESS on antibiotic utilization in CRS and RARS.

Methods

A multi‐institutional, prospective cohort of patients with CRS and RARS was enrolled between January 2001 and January 2009. Patients completed the medication subscale of the Chronic Sinusitis Survey (CSS), and the Wilcoxon signed‐rank test was used to compare differences in the overall reported time of antibiotic between preoperative and postoperative time points.

Results

A total of 503 patients were followed for an average 17.3 months. Overall, patients reported a 57.2% reduction in time on antibiotics following ESS. The majority of patients (60.4%) reported significantly less antibiotic utilization after ESS (p < 0.001) consisting of an 83.7% reduction in the time on antibiotics. Subgroup analysis also revealed a significant reduction in antibiotic utilization for patients with and without nasal polyposis (59.0% and 58.2%; both p < 0.001) as well as RARS (61.2%; p = 0.001).

Conclusion

ESS significantly reduces antibiotic utilization for CRS and RARS. This finding demonstrates potential for lower health care expenditures related to antibiotics, as well as reduced risk of both antibiotic related morbidity and development of bacterial resistance. © 2011 ARS‐AAOA, LLC.
Impact of chronic rhinosinusitis on work productivity through one‐year follow‐up after balloon dilation of the ethmoid infundibulum
Authors: Stankiewicz James , Tami Thomas , Truitt Theodore , Atkins James , Winegar Bradford , Cink Paul , Schaeffer B. Todd , Raviv Joseph , Henderson Diana , Duncavage James , Hagaman David
Published: February 2011

Background

Although multiple clinical trials have demonstrated that balloon dilation of sinus ostia in patients diagnosed with chronic rhinosinusitis (CRS) results in sustained symptomatic improvement, less data are available to measure the effects of sinusitis on worker productivity. The objective of our research was to analyze work and activity impairment before and after transantral, endoscopically‐guided balloon dilation of the maxillary sinus ostia and ethmoid infundibulum.

Methods

Subjects diagnosed with CRS and computed tomography (CT) evidence of disease in the maxillary sinuses alone, or maxillary and anterior ethmoid sinuses, completed the Work Productivity and Activity Impairment (WPAI) questionnaire and the Work Limitation Questionnaire (WLQ) before treatment and at 3, 6, and 12 months postprocedure.

Results

A total of 56 subjects were enrolled and 53 completed the 1‐year follow‐up. The lost productivity composite score computed from the WLQ improved by 73% (9.0 to 2.4; p < 0.0001) at 1‐year follow‐up whereas lost productivity at work as measured by the WPAI improved by approximately 76% (38.3 to 9.2; p < 0.0001) 12 months after treatment.

Conclusion

These results indicate that sinus‐related health problems impose a substantial burden on work productivity and physical/mental activity levels. Treatment of CRS by dilating the maxillary sinus ostium and ethmoid infundibulum can significantly improve quality of life (QOL) and work productivity. © 2011 ARS‐AAOA, LLC.
Effects of various submucous resection techniques of septal cartilage on nasal tip projection
Authors: Zoumalan Richard A. , Morris Luc G.T. , Zeitler Daniel M. , Shah Anil R.
Published: February 2011

Background

There is little research which determines whether septoplasty affects nasal projection.

Objectives

To assess the effect of various septoplasty (submucous resection, SMR) techniques on nasal tip projection in a fresh cadaver model.

Methods

The nasal tip projection was measured on 6 fresh cadaver heads and compared postoperatively after a sequence of submucous septoplasty maneuvers. Five different septoplasty techniques were performed in the same sequence on each cadaver. After each technique, measurements were performed.

Results

Removal of a central square piece of quadrangular cartilage resulted in a loss of projection in 3 in 6 (50%) heads, with average loss of 7.76%. Removal of additional cartilage along the bony cartilaginous junction resulted in no loss of projection. Removing more septum along the floor resulted caused a change loss in nasal projection in 1 in 6 (17%) cadavers. Removing the remaining septum, except for the L‐strut resulted in a loss of projection in 2 in 6 (33%) heads with an average percent change in tip projection of 9.08%. Swinging door technique resulted in a loss of projection in 1 cadaver (17%), with loss of 6.25%. All 6 cadavers experienced loss of nasal projection. When all maneuvers were taken in total, there was a statically significant average decrease in projection of 8.93% (range, 5.00–13.04%, p = 0.008).

Conclusion

Primary septoplasty carries a risk of nasal tip projection, with certain maneuvers carrying higher risk. © 2011 ARS‐AAOA, LLC.
Utility of novel 3‐dimensional stereoscopic vision system for endoscopic sinonasal and skull‐base surgery
Authors: Manes R. Peter , Barnett Sam , Batra Pete S.
Published: February 2011

Background

The objective of this pilot study was to evaluate the utility of novel 3‐dimensional (3D) endoscopy during endoscopic sinonasal and skull base surgery.

Methods

Eight surgeries were performed in 7 patients between August 2009 and March 2010 at a tertiary care academic medical center. A high‐definition 2‐dimensional (2D) endoscopy system was employed in all cases. The Visionsense stereoscopic system (Orangeburg, NY) was incorporated during key portions of the procedures. Two independent surgeons assessed utility of the technology for the following 2 variables: (1) ability to facilitate orientation and depth perception; and (2) impact on completeness of surgery and potential complications.

Results

The mean age was 50.4 years and the male:female ratio was 6:1. Indications included anterior skull base (ASB) tumor resection (5), directed skull base biopsies (2), and ethmoid dissection adjacent to dehiscent skull base/optic nerve in allergic fungal rhinosinusitis (1). Endoscopic orientation and depth perception was aided using the 3D endoscope in all cases. Additional interventions were performed in 3 cases (37.5%), including tumor resection (1) and removal of remnant ethmoid partitions (2). Limitations posed included inability to visualize a type III frontal cell (1) and loss of orientation during ASB reconstruction due to overmagnification (1). No complications were observed in this patient series.

Conclusion

This preliminary study demonstrated the effectiveness of binocular 3D endoscopy during sinonasal and skull‐base surgery. The technology facilitated depth perception and completeness of surgery without increase in complications. Additional experience is warranted to define its role in the endoscopic surgical paradigm. © 2011 ARS‐AAOA, LLC.
Middle turbinate medialization for improved access during endoscopic sinus surgery
Authors: Lee Michael R. , Marple Bradley F.
Published: February 2011

Introduction

Proper management of the middle turbinate in endoscopic sinus surgery is often a topic of debate. Most authors, however, agree in the need to prevent lateralization. We describe our preferred technique and stress the timing of the medialization procedure.

Methods

The middle turbinates are sutured to the nasal septum at the beginning of the operation. This allows optimal access to the nasal cavity and paranasal sinuses during the entirety of the operation.

Discussion

Multiple techniques have been described to prevent lateralization of the middle turbinate. It is our opinion that implementing the proposed technique at the start of the surgery allows for optimal exposure during the operation. © 2011 ARS‐AAOA, LLC.
Assessing risk/benefit of lumbar drain use for endoscopic skull‐base surgery
Authors: Ransom Evan R. , Palmer James N. , Kennedy David W. , Chiu Alexander G.
Published: February 2011

Background

Lumbar drains (LD) are frequently employed with the goal of postoperative cerebrospinal fluid (CSF) leak prevention. LD placement is not without risk, however, and complications can significantly increase patient discomfort and resource utilization. The objective of this work was to characterize the risks, benefits, and resource utilization associated with LD use in endoscopic anterior skull‐base surgery.

Methods

A retrospective review of endoscopic anterior skull‐base surgeries performed by the senior authors over the past 5 years was done. Cases with prospective LD were selected using anesthesia and billing records. Analysis was done of indications, LD duration, complications, revisions, and additional care required.

Results

A total of 65 patients had LD placed prospectively at the time of surgery. LD were in place for an average of 63 ± 38 hours. Four cases (6.2%) required revision surgery for postoperative CSF leak. Leak rates did not differ between patients with neoplasm (7.7%) and without neoplasm (5.8%; p = 0.80). One readmission was attributable to a recurrent leak. Nine LD complications occurred in 8 patients (12.3%). Overall, 6 blood patches, 3 head computed tomography (CT) scans, 1 open removal of retained catheter fragments, 1 spine CT, and an infectious disease workup were required. Three readmissions and 10 additional hospital days were attributable to LD complications. No relationship was found between patient demographics or comorbidities and LD complications. Regression analysis showed no significant effect of body mass index (odds ratio [OR], 1.01; 95% confidence interval [CI], 0.89–1.14; p = 0.87) or duration of LD (OR, 1.00; 95% CI, 0.98–1.02; p = 0.85) on complication risk. Diagnosis of neoplasm was associated with a significant increase in likelihood of complication (OR, 5.33; 95% CI, 1.11–25.64; p < 0.04).

Conclusion

Complications of LD may be more frequent than postoperative CSF leaks, adding significantly to health care resource utilization. It is difficult to predict which patients will suffer LD complications. Reduction of prospective LD use may avoid unnecessary morbidity and resource utilization. © 2011 ARS‐AAOA, LLC.
Upregulation of RANTES in nasal polyps from patients with cystic fibrosis
Authors: Scapa Victor I. , Ramakrishnan Vijay R. , Mudd Pamela A. , Kingdom Todd T.
Published: February 2011

Background

Nasal polyps in patients with cystic fibrosis (CF) are believed to be phenotypically different than polyps affecting non‐CF patients. The objective of this study was to characterize the expression of inflammatory cytokines within nasal polyps from CF and non‐CF, aspirin‐tolerant patients. “Regulated on activation, normal T‐cell expressed and secreted” (RANTES) is a chemotactic cytokine involved in the recruitment and activation of eosinophils. Multiple molecular studies of non‐CF polyps have established that RANTES may play an important role in nasal polyposis. Our study suggests RANTES may be upregulated in CF polyps relative to non‐CF polyps.

Methods

Nasal polyps were prospectively obtained from CF and non‐CF, aspirin‐tolerant patients. The Quantibody™ Human Cytokine Array I from RayBiotech, Inc., was used to identify differences in cytokine expression between protein extracts of two polyp groups. Four CF polyp extracts and 4 non‐CF, aspirin‐tolerant polyp extracts were each incubated on identical antibody subarrays, each containing 20 human cytokines in quadruplicate. Western blot analysis confirmed expression of RANTES.

Results

The protein microarray suggests a greater than 4‐fold upregulation of RANTES in CF polyps relative to non‐CF polyps. Western blot analysis confirmed expression of RANTES in CF polyps.

Conclusion

Chemokines such as RANTES are responsible for the activation of inflammatory cells within the lamina propria of nasal polyps. We have demonstrated that RANTES may be an important cytokine associated with CF polyps. © 2011 ARS‐AAOA, LLC.
Fate Map of Serotonin Transporter‐Expressing Cells in Developing Mouse Thyroid
Authors: Spina Anna , Rea Silviana , De Pasquale Valeria , Mastellone Vincenzo , Avallone Luigi , Pavone Luigi Michele
Published: February 2011

A Cre/loxP‐based fate mapping approach was used to follow the regions of the mouse thyroid labeled by the serotonin transporter SERT. Reporter gene expression (lacZ) is activated by Cre expression from the SERT locus in SERTCre/+;ROSA26R compound mouse embryos. Cell labeling, first detected in the thyroid primordium at the E10.5 prenatal stage, was followed until the postnatal day P30. The co‐localization of lacZ staining in the same cells that express the transcription factors Nkx2.1 and Pax8 at the E12.5 stage confirms their identity as thyroid cell precursors. SERT immunohistochemistry on thyroid sections of E18.5 embryos showed SERT expression in thyroid follicular cells. Western blotting analysis confirmed the expression of the protein in adult thyroid tissue and cultured FRTL‐5 cells. These results describe the fate of SERT‐expressing cells during thyroid development, suggesting an active role of SERT in the development and functions of mammalian thyroid. They also highlight the possibility to use the SERT‐Cre mouse line as a good Cre driver in early thyroid development. Anat Rec, 2011. © 2011 Wiley‐Liss, Inc.
Characterization of mosquito CYP6P7 and CYP6AA3: differences in substrate preference and kinetic properties
Authors: Duangkaew Panida , Pethuan Sirikun , Kaewpa Dolnapa , Boonsuepsakul Soamrutai , Sarapusit Songklod , Rongnoparut Pornpimol
Published: February 2011

Cytochrome P450 monooxygenases are involved in insecticide resistance in insects. We previously observed an increase in CYP6P7 and CYP6AA3 mRNA expression in Anopheles minimus mosquitoes during the selection for deltamethrin resistance in the laboratory. CYP6AA3 has been shown to metabolize deltamethrin, while no information is known for CYP6P7. In this study, CYP6P7 was heterologously expressed in the Spodoptera frugiperda (Sf9) insect cells via baculovirus‐mediated expression system. The expressed CYP6P7 protein was used for exploitation of its enzymatic activity against insecticides after reconstitution with the An. minimus NADPH‐cytochrome P450 reductase enzyme in vitro. The ability of CYP6P7 to metabolize pyrethroids and insecticides in the organophosphate and carbamate groups was compared with CYP6AA3. The results revealed that both CYP6P7 and CYP6AA3 proteins could metabolize permethrin, cypermethrin, and deltamethrin pyrethroid insecticides, but showed the absence of activity against bioallethrin (pyrethroid), chlorpyrifos (organophosphate), and propoxur (carbamate). CYP6P7 had limited capacity in metabolizing λ‐cyhalothrin (pyrethroid), while CYP6AA3 displayed activity toward λ‐cyhalothrin. Kinetic properties suggested that CYP6AA3 had higher efficiency in metabolizing type I than type II pyrethroids, while catalytic efficiency of CYP6P7 toward both types was not significantly different. Their kinetic parameters in insecticide metabolism and preliminary inhibition studies by test compounds in the flavonoid, furanocoumarin, and methylenedioxyphenyl groups elucidated that CYP6P7 had different enzyme properties compared with CYP6AA3. © 2011 Wiley Periodicals, Inc.
Semantic search log analysis: A method and a study on professional image search
Authors: Hollink Vera , Tsikrika Theodora , de Vries Arjen P.
Published: February 2011

Existing methods for automatically analyzing search logs describe search behavior on the basis of syntactic differences (overlapping terms) between queries. Although these analyses provide valuable insights into the complexity and successfulness of search interactions, they offer a limited interpretation of the observed searching behavior, as they do not consider the semantics of users' queries. In this article we propose a method to exploit semantic information in the form of linked data to enrich search queries so as to determine the semantic types of the queries and the relations between queries that are consecutively entered in a search session. This work provides also an in‐depth analysis of the search logs of professional users searching a commercial picture portal. Compared to previous image search log analyses, in particular those of professional users, we consider a much larger dataset. We analyze the logs both in a syntactic way and using the proposed semantic approach and compare the results. Our findings show the benefits of using semantics for search log analysis: the identified types of query modifications cannot be appropriately analyzed by only considering term overlap, since queries related in the most frequent ways do not usually share terms.