Science.gov

Sample records for atypical ra domain

  1. Harmonic Domains and Synchronization in Typically and Atypically Developing Hebrew-Speaking Children

    ERIC Educational Resources Information Center

    Bat-El, Outi

    2009-01-01

    This paper presents a comparative study of typical and atypical consonant harmony (onset-onset place harmony), with emphasis on (i) the size of the harmonic domain, (ii) the position of the harmonic domain within the prosodic word, and (iii) the maximal size of the prosodic word that exhibits consonant harmony. The data, drawn from typically and…

  2. Phosphotyrosine recognition domains: the typical, the atypical and the versatile

    PubMed Central

    2012-01-01

    SH2 domains are long known prominent players in the field of phosphotyrosine recognition within signaling protein networks. However, over the years they have been joined by an increasing number of other protein domain families that can, at least with some of their members, also recognise pTyr residues in a sequence-specific context. This superfamily of pTyr recognition modules, which includes substantial fractions of the PTB domains, as well as much smaller, or even single member fractions like the HYB domain, the PKCδ and PKCθ C2 domains and RKIP, represents a fascinating, medically relevant and hence intensely studied part of the cellular signaling architecture of metazoans. Protein tyrosine phosphorylation clearly serves a plethora of functions and pTyr recognition domains are used in a similarly wide range of interaction modes, which encompass, for example, partner protein switching, tandem recognition functionalities and the interaction with catalytically active protein domains. If looked upon closely enough, virtually no pTyr recognition and regulation event is an exact mirror image of another one in the same cell. Thus, the more we learn about the biology and ultrastructural details of pTyr recognition domains, the more does it become apparent that nature cleverly combines and varies a few basic principles to generate a sheer endless number of sophisticated and highly effective recognition/regulation events that are, under normal conditions, elegantly orchestrated in time and space. This knowledge is also valuable when exploring pTyr reader domains as diagnostic tools, drug targets or therapeutic reagents to combat human diseases. PMID:23134684

  3. Establishing a Core Domain Set to Measure Rheumatoid Arthritis Flares: Report of the OMERACT 11 RA Flare Workshop

    PubMed Central

    Bykerk, Vivian P.; Lie, Elisabeth; Bartlett, Susan J.; Alten, Rieke; Boonen, Annelies; Christensen, Robin; Furst, Daniel E.; Hewlett, Sarah; Leong, Amye L.; Lyddiatt, Anne; March, Lyn; May, James E.; Montie, Pam; Orbai, Ana-Maria; Pohl, Christoph; Voshaar, Marieke Scholte; Woodworth, Thasia; Bingham, Clifton O.; Choy, Ernest H.

    2015-01-01

    Objective The OMERACT Rheumatoid Arthritis (RA) Flare Group (FG) is developing a data-driven, patient-inclusive, consensus-based RA flare definition for use in clinical trials, longterm observational studies, and clinical practice. At OMERACT 11, we sought endorsement of a proposed core domain set to measure RA flare. Methods Patient and healthcare professional (HCP) qualitative studies, focus groups, and literature review, followed by patient and HCP Delphi exercises including combined Delphi consensus at Outcome Measures in Rheumatology 10 (OMERACT 10), identified potential domains to measure flare. At OMERACT 11, breakout groups discussed key domains and instruments to measure them, and proposed a research agenda. Patients were active research partners in all focus groups and domain identification activities. Processes for domain selection and patient partner involvement were case studies for OMERACT Filter 2.0 methodology. Results A pre-meeting combined Delphi exercise for defining flare identified 9 domains as important (> 70% consensus from patients or HCP). Four new patient-reported domains beyond those included in the RA disease activity core set were proposed for inclusion (fatigue, participation, stiffness, and self-management). The RA FG developed preliminary flare questions (PFQ) to measure domains. In combined plenary voting sessions, OMERACT 11 attendees endorsed the proposed RA core set to measure flare with ≥ 78% consensus and the addition of 3 additional domains to the research agenda for OMERACT 12. Conclusion At OMERACT 11, a core domain set to measure RA flare was ratified and endorsed by attendees. Domain validation aligning with Filter 2.0 is ongoing in new randomized controlled clinical trials and longitudinal observational studies using existing and new instruments including a set of PFQ. PMID:24584927

  4. IQGAP proteins reveal an atypical phosphoinositide (aPI) binding domain with a pseudo C2 domain fold.

    PubMed

    Dixon, Miles J; Gray, Alexander; Schenning, Martijn; Agacan, Mark; Tempel, Wolfram; Tong, Yufeng; Nedyalkova, Lyudmila; Park, Hee-Won; Leslie, Nicholas R; van Aalten, Daan M F; Downes, C Peter; Batty, Ian H

    2012-06-29

    Class I phosphoinositide (PI) 3-kinases act through effector proteins whose 3-PI selectivity is mediated by a limited repertoire of structurally defined, lipid recognition domains. We describe here the lipid preferences and crystal structure of a new class of PI binding modules exemplified by select IQGAPs (IQ motif containing GTPase-activating proteins) known to coordinate cellular signaling events and cytoskeletal dynamics. This module is defined by a C-terminal 105-107 amino acid region of which IQGAP1 and -2, but not IQGAP3, binds preferentially to phosphatidylinositol 3,4,5-trisphosphate (PtdInsP(3)). The binding affinity for PtdInsP(3), together with other, secondary target-recognition characteristics, are comparable with those of the pleckstrin homology domain of cytohesin-3 (general receptor for phosphoinositides 1), an established PtdInsP(3) effector protein. Importantly, the IQGAP1 C-terminal domain and the cytohesin-3 pleckstrin homology domain, each tagged with enhanced green fluorescent protein, were both re-localized from the cytosol to the cell periphery following the activation of PI 3-kinase in Swiss 3T3 fibroblasts, consistent with their common, selective recognition of endogenous 3-PI(s). The crystal structure of the C-terminal IQGAP2 PI binding module reveals unexpected topological similarity to an integral fold of C2 domains, including a putative basic binding pocket. We propose that this module integrates select IQGAP proteins with PI 3-kinase signaling and constitutes a novel, atypical phosphoinositide binding domain that may represent the first of a larger group, each perhaps structurally unique but collectively dissimilar from the known PI recognition modules. PMID:22493426

  5. IQGAP Proteins Reveal an Atypical Phosphoinositide (aPI) Binding Domain with a Pseudo C2 Domain Fold

    SciTech Connect

    Dixon, Miles J.; Gray, Alexander; Schenning, Martijn; Agacan, Mark; Tempel, Wolfram; Tong, Yufeng; Nedyalkova, Lyudmila; Park, Hee-Won; Leslie, Nicholas R.; van Aalten, Daan M.F.; Downes, C. Peter; Batty, Ian H.

    2012-10-16

    Class I phosphoinositide (PI) 3-kinases act through effector proteins whose 3-PI selectivity is mediated by a limited repertoire of structurally defined, lipid recognition domains. We describe here the lipid preferences and crystal structure of a new class of PI binding modules exemplified by select IQGAPs (IQ motif containing GTPase-activating proteins) known to coordinate cellular signaling events and cytoskeletal dynamics. This module is defined by a C-terminal 105-107 amino acid region of which IQGAP1 and -2, but not IQGAP3, binds preferentially to phosphatidylinositol 3,4,5-trisphosphate (PtdInsP3). The binding affinity for PtdInsP3, together with other, secondary target-recognition characteristics, are comparable with those of the pleckstrin homology domain of cytohesin-3 (general receptor for phosphoinositides 1), an established PtdInsP3 effector protein. Importantly, the IQGAP1 C-terminal domain and the cytohesin-3 pleckstrin homology domain, each tagged with enhanced green fluorescent protein, were both re-localized from the cytosol to the cell periphery following the activation of PI 3-kinase in Swiss 3T3 fibroblasts, consistent with their common, selective recognition of endogenous 3-PI(s). The crystal structure of the C-terminal IQGAP2 PI binding module reveals unexpected topological similarity to an integral fold of C2 domains, including a putative basic binding pocket. We propose that this module integrates select IQGAP proteins with PI 3-kinase signaling and constitutes a novel, atypical phosphoinositide binding domain that may represent the first of a larger group, each perhaps structurally unique but collectively dissimilar from the known PI recognition modules.

  6. Structural basis for controlling the dimerization and stability of the WW domains of an atypical subfamily.

    PubMed

    Ohnishi, Satoshi; Tochio, Naoya; Tomizawa, Tadashi; Akasaka, Ryogo; Harada, Takushi; Seki, Eiko; Sato, Manami; Watanabe, Satoru; Fujikura, Yukiko; Koshiba, Seizo; Terada, Takaho; Shirouzu, Mikako; Tanaka, Akiko; Kigawa, Takanori; Yokoyama, Shigeyuki

    2008-09-01

    The second WW domain in mammalian Salvador protein (SAV1 WW2) is quite atypical, as it forms a beta-clam-like homodimer. The second WW domain in human MAGI1 (membrane associated guanylate kinase, WW and PDZ domain containing 1) (MAGI1 WW2) shares high sequence similarity with SAV1 WW2, suggesting comparable dimerization. However, an analytical ultracentrifugation study revealed that MAGI1 WW2 (Leu355-Pro390) chiefly exists as a monomer at low protein concentrations, with an association constant of 1.3 x 10(2) M(-1). We determined its solution structure, and a structural comparison with the dimeric SAV1 WW2 suggested that an Asp residue is crucial for the inhibition of the dimerization. The substitution of this acidic residue with Ser resulted in the dimerization of MAGI1 WW2. The spin-relaxation data suggested that the MAGI1 WW2 undergoes a dynamic process of transient dimerization that is limited by the charge repulsion. Additionally, we characterized a longer construct of this WW domain with a C-terminal extension (Leu355-Glu401), as the formation of an extra alpha-helix was predicted. An NMR structural determination confirmed the formation of an alpha-helix in the extended C-terminal region, which appears to be independent from the dimerization regulation. A thermal denaturation study revealed that the dimerized MAGI1 WW2 with the Asp-to-Ser mutation gained apparent stability in a protein concentration-dependent manner. A structural comparison between the two constructs with different lengths suggested that the formation of the C-terminal alpha-helix stabilized the global fold by facilitating contacts between the N-terminal linker region and the main body of the WW domain. PMID:18562638

  7. The Extracellular Domain of Myelin Oligodendrocyte Glycoprotein Elicits Atypical Experimental Autoimmune Encephalomyelitis in Rat and Macaque Species

    PubMed Central

    Curtis, Alan D.; Taslim, Najla; Reece, Shaun P.; Grebenciucova, Elena; Ray, Richard H.; Rosenbaum, Matthew D.; Wardle, Robert L.; Van Scott, Michael R.; Mannie, Mark D.

    2014-01-01

    Atypical models of experimental autoimmune encephalomyelitis (EAE) are advantageous in that the heterogeneity of clinical signs appears more reflective of those in multiple sclerosis (MS). Conversely, models of classical EAE feature stereotypic progression of an ascending flaccid paralysis that is not a characteristic of MS. The study of atypical EAE however has been limited due to the relative lack of suitable models that feature reliable disease incidence and severity, excepting mice deficient in gamma-interferon signaling pathways. In this study, atypical EAE was induced in Lewis rats, and a related approach was effective for induction of an unusual neurologic syndrome in a cynomolgus macaque. Lewis rats were immunized with the rat immunoglobulin variable (IgV)-related extracellular domain of myelin oligodendrocyte glycoprotein (IgV-MOG) in complete Freund’s adjuvant (CFA) followed by one or more injections of rat IgV-MOG in incomplete Freund’s adjuvant (IFA). The resulting disease was marked by torticollis, unilateral rigid paralysis, forelimb weakness, and high titers of anti-MOG antibody against conformational epitopes of MOG, as well as other signs of atypical EAE. A similar strategy elicited a distinct atypical form of EAE in a cynomolgus macaque. By day 36 in the monkey, titers of IgG against conformational epitopes of extracellular MOG were evident, and on day 201, the macaque had an abrupt onset of an unusual form of EAE that included a pronounced arousal-dependent, transient myotonia. The disease persisted for 6–7 weeks and was marked by a gradual, consistent improvement and an eventual full recovery without recurrence. These data indicate that one or more boosters of IgV-MOG in IFA represent a key variable for induction of atypical or unusual forms of EAE in rat and Macaca species. These studies also reveal a close correlation between humoral immunity against conformational epitopes of MOG, extended confluent demyelinating plaques in spinal cord

  8. ATRX ADD domain links an atypical histone methylation recognition mechanism to human mental-retardation syndrome

    SciTech Connect

    Iwase, Shigeki; Xiang, Bin; Ghosh, Sharmistha; Ren, Ting; Lewis, Peter W.; Cochrane, Jesse C.; Allis, C. David; Picketts, David J.; Patel, Dinshaw J.; Li, Haitao; Shi, Yang

    2011-07-19

    ATR-X (alpha-thalassemia/mental retardation, X-linked) syndrome is a human congenital disorder that causes severe intellectual disabilities. Mutations in the ATRX gene, which encodes an ATP-dependent chromatin-remodeler, are responsible for the syndrome. Approximately 50% of the missense mutations in affected persons are clustered in a cysteine-rich domain termed ADD (ATRX-DNMT3-DNMT3L, ADD{sub ATRX}), whose function has remained elusive. Here we identify ADD{sub ATRX} as a previously unknown histone H3-binding module, whose binding is promoted by lysine 9 trimethylation (H3K9me3) but inhibited by lysine 4 trimethylation (H3K4me3). The cocrystal structure of ADD{sub ATRX} bound to H3{sub 1-15}K9me3 peptide reveals an atypical composite H3K9me3-binding pocket, which is distinct from the conventional trimethyllysine-binding aromatic cage. Notably, H3K9me3-pocket mutants and ATR-X syndrome mutants are defective in both H3K9me3 binding and localization at pericentromeric heterochromatin; thus, we have discovered a unique histone-recognition mechanism underlying the ATR-X etiology.

  9. ATRX ADD Domain Links an Atypical Histone Methylation Recognition Mechanism to Human Mental-Retardation Syndrome

    SciTech Connect

    S Iwase; B Xiang; S Ghosh; T Ren; P Lewis; J Cochrane; C Allis; D Picketts; D Patel; et al.

    2011-12-31

    ATR-X (alpha-thalassemia/mental retardation, X-linked) syndrome is a human congenital disorder that causes severe intellectual disabilities. Mutations in the ATRX gene, which encodes an ATP-dependent chromatin-remodeler, are responsible for the syndrome. Approximately 50% of the missense mutations in affected persons are clustered in a cysteine-rich domain termed ADD (ATRX-DNMT3-DNMT3L, ADD{sub ATRX}), whose function has remained elusive. Here we identify ADD{sub ATRX} as a previously unknown histone H3-binding module, whose binding is promoted by lysine 9 trimethylation (H3K9me3) but inhibited by lysine 4 trimethylation (H3K4me3). The cocrystal structure of ADD{sub ATRX} bound to H3{sub 1-15}K9me3 peptide reveals an atypical composite H3K9me3-binding pocket, which is distinct from the conventional trimethyllysine-binding aromatic cage. Notably, H3K9me3-pocket mutants and ATR-X syndrome mutants are defective in both H3K9me3 binding and localization at pericentromeric heterochromatin; thus, we have discovered a unique histone-recognition mechanism underlying the ATR-X etiology.

  10. Identifying flares in rheumatoid arthritis: reliability and construct validation of the OMERACT RA Flare Core Domain Set

    PubMed Central

    Bykerk, Vivian P; Bingham, Clifton O; Choy, Ernest H; Lin, Daming; Alten, Rieke; Christensen, Robin; Furst, Daniel E; Hewlett, Sarah; Leong, Amye; March, Lyn; Woodworth, Thasia; Boire, Gilles; Haraoui, Boulos; Hitchon, Carol; Jamal, Shahin; Keystone, Edward C; Pope, Janet; Tin, Diane; Thorne, J Carter

    2016-01-01

    Objective To evaluate the reliability of concurrent flare identification using 3 methods (patient, rheumatologist and Disease Activity Score (DAS)28 criteria), and construct validity of candidate items representing the Outcome Measures in Rheumatology Clinical Trials (OMERACT) RA Flare Core Domain Set. Methods Candidate flare questions and legacy measures were administered at consecutive visits to Canadian Early Arthritis Cohort (CATCH) patients between November 2011 and November 2014. The American College of Rheumatology (ACR) core set indicators were recorded. Concordance to identify flares was assessed using the agreement coefficient. Construct validity of flare questions was examined: convergent (Spearman's r); discriminant (mean differences between flaring/non-flaring patients); and consequential (proportions with prior treatment reductions and intended therapeutic change postflare). Results The 849 patients were 75% female, 81% white, 42% were in remission/low disease activity (R/LDA), and 16–32% were flaring at the second visit. Agreement of flare status was low–strong (κ's 0.17–0.88) and inversely related to RA disease activity level. Flare domains correlated highly (r's≥0.70) with each other, patient global (r's≥0.66) and corresponding measures (r's 0.49–0.92); and moderately highly with MD and patient-reported joint counts (r's 0.29–0.62). When MD/patients agreed the patient was flaring, mean flare domain between-group differences were 2.1–3.0; 36% had treatment reductions prior to flare, with escalation planned in 61%. Conclusions Flares are common in rheumatoid arthritis (RA) and are often preceded by treatment reductions. Patient/MD/DAS agreement of flare status is highest in patients worsening from R/LDA. OMERACT RA flare questions can discriminate between patients with/without flare and have strong evidence of construct and consequential validity. Ongoing work will identify optimal scoring and cut points to identify RA flares. PMID

  11. Elimination of fast inactivation in Kv4 A-type potassium channels by an auxiliary subunit domain

    PubMed Central

    Holmqvist, Mats H.; Cao, Jie; Hernandez-Pineda, Ricardo; Jacobson, Michael D.; Carroll, Karen I.; Sung, M. Amy; Betty, Maria; Ge, Pei; Gilbride, Kevin J.; Brown, Melissa E.; Jurman, Mark E.; Lawson, Deborah; Silos-Santiago, Inmaculada; Xie, Yu; Covarrubias, Manuel; Rhodes, Kenneth J.; Distefano, Peter S.; An, W. Frank

    2002-01-01

    The Kv4 A-type potassium currents contribute to controlling the frequency of slow repetitive firing and back-propagation of action potentials in neurons and shape the action potential in heart. Kv4 currents exhibit rapid activation and inactivation and are specifically modulated by K-channel interacting proteins (KChIPs). Here we report the discovery and functional characterization of a modular K-channel inactivation suppressor (KIS) domain located in the first 34 aa of an additional KChIP (KChIP4a). Coexpression of KChIP4a with Kv4 α-subunits abolishes fast inactivation of the Kv4 currents in various cell types, including cerebellar granule neurons. Kinetic analysis shows that the KIS domain delays Kv4.3 opening, but once the channel is open, it disrupts rapid inactivation and slows Kv4.3 closing. Accordingly, KChIP4a increases the open probability of single Kv4.3 channels. The net effects of KChIP4a and KChIP1–3 on Kv4 gating are quite different. When both KChIP4a and KChIP1 are present, the Kv4.3 current shows mixed inactivation profiles dependent on KChIP4a/KChIP1 ratios. The KIS domain effectively converts the A-type Kv4 current to a slowly inactivating delayed rectifier-type potassium current. This conversion is opposite to that mediated by the Kv1-specific “ball” domain of the Kvβ1 subunit. Together, these results demonstrate that specific auxiliary subunits with distinct functions actively modulate gating of potassium channels that govern membrane excitability. PMID:11805342

  12. Coordinated activation of the Rac-GAP β2-chimaerin by an atypical proline-rich domain and diacylglycerol.

    PubMed

    Gutierrez-Uzquiza, Alvaro; Colon-Gonzalez, Francheska; Leonard, Thomas A; Canagarajah, Bertram J; Wang, HongBin; Mayer, Bruce J; Hurley, James H; Kazanietz, Marcelo G

    2013-01-01

    Chimaerins, a family of GTPase activating proteins for the small G-protein Rac, have been implicated in development, neuritogenesis and cancer. These Rac-GTPase activating proteins are regulated by the lipid second messenger diacylglycerol generated by tyrosine kinases such as the epidermal growth factor receptor. Here we identify an atypical proline-rich motif in chimaerins that binds to the adaptor protein Nck1. Unlike most Nck1 partners, chimaerins bind to the third SH3 domain of Nck1. This association is mediated by electrostatic interactions of basic residues within the Pro-rich motif with acidic clusters in the SH3 domain. Epidermal growth factor promotes the binding of β2-chimaerin to Nck1 in the cell periphery in a diacylglycerol-dependent manner. Moreover, β2-chimaerin translocation to the plasma membrane and its peripheral association with Rac1 requires Nck1. Our studies underscore a coordinated mechanism for β2-chimaerin activation that involves lipid interactions via the C1 domain and protein-protein interactions via the N-terminal proline-rich region. PMID:23673634

  13. Structural and functional characterization of an atypical activation domain in erythroid Kruppel-like factor (EKLF).

    PubMed

    Mas, Caroline; Lussier-Price, Mathieu; Soni, Shefali; Morse, Thomas; Arseneault, Geneviève; Di Lello, Paola; Lafrance-Vanasse, Julien; Bieker, James J; Omichinski, James G

    2011-06-28

    Erythroid Krüppel-like factor (EKLF) plays an important role in erythroid development by stimulating β-globin gene expression. We have examined the details by which the minimal transactivation domain (TAD) of EKLF (EKLFTAD) interacts with several transcriptional regulatory factors. We report that EKLFTAD displays homology to the p53TAD and, like the p53TAD, can be divided into two functional subdomains (EKLFTAD1 and EKLFTAD2). Based on sequence analysis, we found that EKLFTAD2 is conserved in KLF2, KLF4, KLF5, and KLF15. In addition, we demonstrate that EKLFTAD2 binds the amino-terminal PH domain of the Tfb1/p62 subunit of TFIIH (Tfb1PH/p62PH) and four domains of CREB-binding protein/p300. The solution structure of the EKLFTAD2/Tfb1PH complex indicates that EKLFTAD2 binds Tfb1PH in an extended conformation, which is in contrast to the α-helical conformation seen for p53TAD2 in complex with Tfb1PH. These studies provide detailed mechanistic information into EKLFTAD functions as well as insights into potential interactions of the TADs of other KLF proteins. In addition, they suggest that not only have acidic TADs evolved so that they bind using different conformations on a common target, but that transitioning from a disordered to a more ordered state is not a requirement for their ability to bind multiple partners. PMID:21670263

  14. NMR Study Reveals the Receiver Domain of Arabidopsis ETHYLENE RESPONSE1 Ethylene Receptor as an Atypical Type Response Regulator.

    PubMed

    Hung, Yi-Lin; Jiang, Ingjye; Lee, Yi-Zong; Wen, Chi-Kuang; Sue, Shih-Che

    2016-01-01

    The gaseous plant hormone ethylene, recognized by plant ethylene receptors, plays a pivotal role in various aspects of plant growth and development. ETHYLENE RESPONSE1 (ETR1) is an ethylene receptor isolated from Arabidopsis and has a structure characteristic of prokaryotic two-component histidine kinase (HK) and receiver domain (RD), where the RD structurally resembles bacteria response regulators (RRs). The ETR1 HK domain has autophosphorylation activity, and little is known if the HK can transfer the phosphoryl group to the RD for receptor signaling. Unveiling the correlation of the receptor structure and phosphorylation status would advance the studies towards the underlying mechanisms of ETR1 receptor signaling. In this study, using the nuclear magnetic resonance technique, our data suggested that the ETR1-RD is monomeric in solution and the rigid structure of the RD prevents the conserved aspartate residue phosphorylation. Comparing the backbone dynamics with other RRs, we propose that backbone flexibility is critical to the RR phosphorylation. Besides the limited flexibility, ETR1-RD has a unique γ loop conformation of opposite orientation, which makes ETR1-RD unfavorable for phosphorylation. These two features explain why ETR1-RD cannot be phosphorylated and is classified as an atypical type RR. As a control, phosphorylation of the ETR1-RD was also impaired when the sequence was swapped to the fragment of the bacterial typical type RR, CheY. Here, we suggest a molecule insight that the ETR1-RD already exists as an active formation and executes its function through binding with the downstream factors without phosphorylation. PMID:27486797

  15. NMR Study Reveals the Receiver Domain of Arabidopsis ETHYLENE RESPONSE1 Ethylene Receptor as an Atypical Type Response Regulator

    PubMed Central

    Lee, Yi-Zong; Wen, Chi-Kuang; Sue, Shih-Che

    2016-01-01

    The gaseous plant hormone ethylene, recognized by plant ethylene receptors, plays a pivotal role in various aspects of plant growth and development. ETHYLENE RESPONSE1 (ETR1) is an ethylene receptor isolated from Arabidopsis and has a structure characteristic of prokaryotic two-component histidine kinase (HK) and receiver domain (RD), where the RD structurally resembles bacteria response regulators (RRs). The ETR1 HK domain has autophosphorylation activity, and little is known if the HK can transfer the phosphoryl group to the RD for receptor signaling. Unveiling the correlation of the receptor structure and phosphorylation status would advance the studies towards the underlying mechanisms of ETR1 receptor signaling. In this study, using the nuclear magnetic resonance technique, our data suggested that the ETR1-RD is monomeric in solution and the rigid structure of the RD prevents the conserved aspartate residue phosphorylation. Comparing the backbone dynamics with other RRs, we propose that backbone flexibility is critical to the RR phosphorylation. Besides the limited flexibility, ETR1-RD has a unique γ loop conformation of opposite orientation, which makes ETR1-RD unfavorable for phosphorylation. These two features explain why ETR1-RD cannot be phosphorylated and is classified as an atypical type RR. As a control, phosphorylation of the ETR1-RD was also impaired when the sequence was swapped to the fragment of the bacterial typical type RR, CheY. Here, we suggest a molecule insight that the ETR1-RD already exists as an active formation and executes its function through binding with the downstream factors without phosphorylation. PMID:27486797

  16. Atypicality of Atypical Antipsychotics

    PubMed Central

    Farah, Andrew

    2005-01-01

    Objective: To review the current definition of atypicality, discuss the unique features of each atypical antipsychotic, and determine whether the available drugs in this class really meet the classical definition of atypicality. Data Sources: A PubMed search was conducted to identify literature on the subject of this review, supported by additional articles based on the author's clinical knowledge and experience. Study Selection and Data Extraction: Relevant references were extracted and summarized in order to meet the objective of the article. Data Synthesis: Atypical antipsychotics are considered a major advance over conventional antipsychotics, primarily because they offer effective treatment alternatives that are relatively free of extrapyramidal symptoms. In fact, the term atypicality was originally used to describe antipsychotic agents with a minimal risk of causing extrapyramidal symptoms. However, over the years the definition has been modified such that there is currently no consensus on a true definition of atypicality for these agents. Each of the atypical antipsychotics (clozapine, risperidone, olanzapine, quetiapine, ziprasidone, and aripiprazole) commercially available in the United States is unique in terms of its pharmacologic profile, differing with respect to receptor-binding affinity, mechanism of action, and adverse events. Of the available atypical antipsychotics, clozapine and quetiapine have shown the lowest propensity to cause extrapyramidal symptoms. Although the risk of extra-pyramidal symptoms is lower with risperidone and olanzapine than with conventional antipsychotics, risk increases with dose escalation. Data for ziprasidone indicate that the risk of extrapyramidal symptoms may be similar to that of risperidone and olanzapine. There is a concern of akathisia with aripiprazole; however, more experience with this agent is needed before definitive conclusions are made. Conclusion: If the definition of “atypical” antipsychotic is

  17. Crystal structure of EML1 reveals the basis for Hsp90 dependence of oncogenic EML4-ALK by disruption of an atypical β-propeller domain

    PubMed Central

    Richards, Mark W.; Law, Edward W. P.; Rennalls, La’Verne P.; Busacca, Sara; O’Regan, Laura; Fry, Andrew M.; Fennell, Dean A.; Bayliss, Richard

    2014-01-01

    Proteins of the echinoderm microtubule-associated protein (EMAP)-like (EML) family contribute to formation of the mitotic spindle and interphase microtubule network. They contain a unique hydrophobic EML protein (HELP) motif and a variable number of WD40 repeats. Recurrent gene rearrangements in nonsmall cell lung cancer fuse EML4 to anaplastic lymphoma kinase (ALK), causing expression of several fusion oncoprotein variants. We have determined a 2.6-Å crystal structure of the representative ∼70-kDa core of EML1, revealing an intimately associated pair of β-propellers, which we term a TAPE (tandem atypical propeller in EMLs) domain. One propeller is highly atypical, having a discontinuous subdomain unrelated to a WD40 motif in place of one of its blades. This unexpected feature shows how a propeller structure can be assembled from subdomains with distinct folds. The HELP motif is not an independent domain but forms part of the hydrophobic core that joins the two β-propellers. The TAPE domain binds α/β-tubulin via its conserved, concave surface, including part of the atypical blade. Mapping the characteristic breakpoints of each EML4-ALK variant onto our structure indicates that the EML4 TAPE domain is truncated in many variants in a manner likely to make the fusion protein structurally unstable. We found that the heat shock protein 90 (Hsp90) inhibitor ganetespib induced degradation of these variants whereas others lacking a partial TAPE domain were resistant in both overexpression models and patient-derived cell lines. The Hsp90-sensitive EML4-ALK variants are exceptions to the rule that oncogenic fusion proteins involve breakpoints in disordered regions of both partners. PMID:24706829

  18. Atypical pneumonia

    MedlinePlus

    ... that cause typical pneumonia. These include Legionella pneumophila , Mycoplasma pneumoniae , and Chlamydophila pneumoniae . Atypical pneumonia also tends to have milder symptoms than typical pneumonia. Causes Mycoplasma pneumonia is a type of atypical pneumonia. It ...

  19. Structural and functional characterization of an atypical activation domain in erythroid Krüppel-like factor (EKLF)

    PubMed Central

    Mas, Caroline; Lussier-Price, Mathieu; Soni, Shefali; Morse, Thomas; Arseneault, Geneviève; Di Lello, Paola; Lafrance-Vanasse, Julien; Bieker, James J.; Omichinski, James G.

    2011-01-01

    Erythroid Krüppel-like factor (EKLF) plays an important role in erythroid development by stimulating β-globin gene expression. We have examined the details by which the minimal transactivation domain (TAD) of EKLF (EKLFTAD) interacts with several transcriptional regulatory factors. We report that EKLFTAD displays homology to the p53TAD and, like the p53TAD, can be divided into two functional subdomains (EKLFTAD1 and EKLFTAD2). Based on sequence analysis, we found that EKLFTAD2 is conserved in KLF2, KLF4, KLF5, and KLF15. In addition, we demonstrate that EKLFTAD2 binds the amino-terminal PH domain of the Tfb1/p62 subunit of TFIIH (Tfb1PH/p62PH) and four domains of CREB-binding protein/p300. The solution structure of the EKLFTAD2/Tfb1PH complex indicates that EKLFTAD2 binds Tfb1PH in an extended conformation, which is in contrast to the α-helical conformation seen for p53TAD2 in complex with Tfb1PH. These studies provide detailed mechanistic information into EKLFTAD functions as well as insights into potential interactions of the TADs of other KLF proteins. In addition, they suggest that not only have acidic TADs evolved so that they bind using different conformations on a common target, but that transitioning from a disordered to a more ordered state is not a requirement for their ability to bind multiple partners. PMID:21670263

  20. An atypical receiver domain controls the dynamic polar localization of the Myxococcus xanthus social motility protein FrzS

    PubMed Central

    Fraser, James S; Merlie, John P; Echols, Nathaniel; Weisfield, Shellie R; Mignot, Tâm; Wemmer, David E; Zusman, David R; Alber, Tom

    2007-01-01

    The Myxococcus xanthus FrzS protein transits from pole-to-pole within the cell, accumulating at the pole that defines the direction of movement in social (S) motility. Here we show using atomic-resolution crystallography and NMR that the FrzS receiver domain (RD) displays the conserved switch Tyr102 in an unusual conformation, lacks the conserved Asp phosphorylation site, and fails to bind Mg2+ or the phosphoryl analogue, Mg2+·BeF3. Mutation of Asp55, closest to the canonical site of RD phosphorylation, showed no motility phenotype in vivo, demonstrating that phosphorylation at this site is not necessary for domain function. In contrast, the Tyr102Ala and His92Phe substitutions on the canonical output face of the FrzS RD abolished S-motility in vivo. Single-cell fluorescence microscopy measurements revealed a striking mislocalization of these mutant FrzS proteins to the trailing cell pole in vivo. The crystal structures of the mutants suggested that the observed conformation of Tyr102 in the wild-type FrzS RD is not sufficient for function. These results support the model that FrzS contains a novel ‘pseudo-receiver domain’ whose function requires recognition of the RD output face but not Asp phosphorylation. PMID:17573816

  1. Atypical antigen recognition mode of a shark immunoglobulin new antigen receptor (IgNAR) variable domain characterized by humanization and structural analysis.

    PubMed

    Kovalenko, Oleg V; Olland, Andrea; Piché-Nicholas, Nicole; Godbole, Adarsh; King, Daniel; Svenson, Kristine; Calabro, Valerie; Müller, Mischa R; Barelle, Caroline J; Somers, William; Gill, Davinder S; Mosyak, Lidia; Tchistiakova, Lioudmila

    2013-06-14

    The immunoglobulin new antigen receptors (IgNARs) are a class of Ig-like molecules of the shark immune system that exist as heavy chain-only homodimers and bind antigens by their single domain variable regions (V-NARs). Following shark immunization and/or in vitro selection, V-NARs can be generated as soluble, stable, and specific high affinity monomeric binding proteins of ∼12 kDa. We have previously isolated a V-NAR from an immunized spiny dogfish shark, named E06, that binds specifically and with high affinity to human, mouse, and rat serum albumins. Humanization of E06 was carried out by converting over 60% of non-complementarity-determining region residues to those of a human germ line Vκ1 sequence, DPK9. The resulting huE06 molecules have largely retained the specificity and affinity of antigen binding of the parental V-NAR. Crystal structures of the shark E06 and its humanized variant (huE06 v1.1) in complex with human serum albumin (HSA) were determined at 3- and 2.3-Å resolution, respectively. The huE06 v1.1 molecule retained all but one amino acid residues involved in the binding site for HSA. Structural analysis of these V-NARs has revealed an unusual variable domain-antigen interaction. E06 interacts with HSA in an atypical mode that utilizes extensive framework contacts in addition to complementarity-determining regions that has not been seen previously in V-NARs. On the basis of the structure, the roles of various elements of the molecule are described with respect to antigen binding and V-NAR stability. This information broadens the general understanding of antigen recognition and provides a framework for further design and humanization of shark IgNARs. PMID:23632026

  2. [Atypical odontalgia].

    PubMed

    Türp, Jens Christoph

    2005-01-01

    In spite of its first description by the English surgeon JOHN HUNTER more than 200 years ago, atypical odontalgia (AO), or phantom tooth pain, is not universally known among dentists. AO is a persistent neuropathic pain which may be initiated after deafferentiation of trigeminal nerve fibers following root canal treatment, apicectomy, or tooth extraction. In the absence of pathological clinical or radiological findings, the diagnosis is made by exclusion. After a thorough patient education about the condition, pharmacological and psychological pain management is required. Invasive and irreversible treatment attempts are contraindicated. PMID:16342640

  3. Atypical Inflammasomes.

    PubMed

    Janowski, Ann M; Sutterwala, Fayyaz S

    2016-01-01

    Pattern recognition receptors, including members of the NLR and PYHIN families, are essential for recognition of both pathogen- and host-derived danger signals. A number of molecules in these families are capable of forming multiprotein complexes termed inflammasomes that result in the activation of caspase-1. In addition to NLRP1, NLRP3, NLRC4, and AIM2, which form well-described inflammasome complexes, IFI16, NLRP6, NLRP7, NLRP12, and NLRC5 have also been proposed to form inflammasomes under specific conditions. The structure and function of these atypical inflammasomes will be highlighted here. PMID:27221480

  4. Genes encoding proteins with peritrophin A-type chitin-binding domains in Tribolium castaneum are grouped into three distinct families based on phylogeny, expression and function

    Technology Transfer Automated Retrieval System (TEKTRAN)

    This study is focused on the characterization and expression of genes in the red flour beetle, Tribolium castaneum, encoding proteins that possess six-cysteine-containing chitin-binding domains (CBDs) related to the peritrophin A domain (ChtBD2). An exhaustive bioinformatics search of the genome of...

  5. ATRX binds to atypical chromatin domains at the 3′ exons of zinc finger genes to preserve H3K9me3 enrichment

    PubMed Central

    Chowdhury, Asif H.; Hasson, Dan; Dyer, Michael A.

    2016-01-01

    ABSTRACT ATRX is a SWI/SNF chromatin remodeler proposed to govern genomic stability through the regulation of repetitive sequences, such as rDNA, retrotransposons, and pericentromeric and telomeric repeats. However, few direct ATRX target genes have been identified and high-throughput genomic approaches are currently lacking for ATRX. Here we present a comprehensive ChIP-sequencing study of ATRX in multiple human cell lines, in which we identify the 3′ exons of zinc finger genes (ZNFs) as a new class of ATRX targets. These 3′ exonic regions encode the zinc finger motifs, which can range from 1–40 copies per ZNF gene and share large stretches of sequence similarity. These regions often contain an atypical chromatin signature: they are transcriptionally active, contain high levels of H3K36me3, and are paradoxically enriched in H3K9me3. We find that these ZNF 3′ exons are co-occupied by SETDB1, TRIM28, and ZNF274, which form a complex with ATRX. CRISPR/Cas9-mediated loss-of-function studies demonstrate (i) a reduction of H3K9me3 at the ZNF 3′ exons in the absence of ATRX and ZNF274 and, (ii) H3K9me3 levels at atypical chromatin regions are particularly sensitive to ATRX loss compared to other H3K9me3-occupied regions. As a consequence of ATRX or ZNF274 depletion, cells with reduced levels of H3K9me3 show increased levels of DNA damage, suggesting that ATRX binds to the 3′ exons of ZNFs to maintain their genomic stability through preservation of H3K9me3. PMID:27029610

  6. ATRX binds to atypical chromatin domains at the 3' exons of zinc finger genes to preserve H3K9me3 enrichment.

    PubMed

    Valle-García, David; Qadeer, Zulekha A; McHugh, Domhnall S; Ghiraldini, Flávia G; Chowdhury, Asif H; Hasson, Dan; Dyer, Michael A; Recillas-Targa, Félix; Bernstein, Emily

    2016-06-01

    ATRX is a SWI/SNF chromatin remodeler proposed to govern genomic stability through the regulation of repetitive sequences, such as rDNA, retrotransposons, and pericentromeric and telomeric repeats. However, few direct ATRX target genes have been identified and high-throughput genomic approaches are currently lacking for ATRX. Here we present a comprehensive ChIP-sequencing study of ATRX in multiple human cell lines, in which we identify the 3' exons of zinc finger genes (ZNFs) as a new class of ATRX targets. These 3' exonic regions encode the zinc finger motifs, which can range from 1-40 copies per ZNF gene and share large stretches of sequence similarity. These regions often contain an atypical chromatin signature: they are transcriptionally active, contain high levels of H3K36me3, and are paradoxically enriched in H3K9me3. We find that these ZNF 3' exons are co-occupied by SETDB1, TRIM28, and ZNF274, which form a complex with ATRX. CRISPR/Cas9-mediated loss-of-function studies demonstrate (i) a reduction of H3K9me3 at the ZNF 3' exons in the absence of ATRX and ZNF274 and, (ii) H3K9me3 levels at atypical chromatin regions are particularly sensitive to ATRX loss compared to other H3K9me3-occupied regions. As a consequence of ATRX or ZNF274 depletion, cells with reduced levels of H3K9me3 show increased levels of DNA damage, suggesting that ATRX binds to the 3' exons of ZNFs to maintain their genomic stability through preservation of H3K9me3. PMID:27029610

  7. Discovery of novel interacting partners of PSMD9, a proteasomal chaperone: Role of an Atypical and versatile PDZ-domain motif interaction and identification of putative functional modules

    PubMed Central

    Sangith, Nikhil; Srinivasaraghavan, Kannan; Sahu, Indrajit; Desai, Ankita; Medipally, Spandana; Somavarappu, Arun Kumar; Verma, Chandra; Venkatraman, Prasanna

    2014-01-01

    PSMD9 (Proteasome Macropain non-ATPase subunit 9), a proteasomal assembly chaperone, harbors an uncharacterized PDZ-like domain. Here we report the identification of five novel interacting partners of PSMD9 and provide the first glimpse at the structure of the PDZ-domain, including the molecular details of the interaction. We based our strategy on two propositions: (a) proteins with conserved C-termini may share common functions and (b) PDZ domains interact with C-terminal residues of proteins. Screening of C-terminal peptides followed by interactions using full-length recombinant proteins, we discovered hnRNPA1 (an RNA binding protein), S14 (a ribosomal protein), CSH1 (a growth hormone), E12 (a transcription factor) and IL6 receptor as novel PSMD9-interacting partners. Through multiple techniques and structural insights, we clearly demonstrate for the first time that human PDZ domain interacts with the predicted Short Linear Sequence Motif (SLIM) at the C-termini of the client proteins. These interactions are also recapitulated in mammalian cells. Together, these results are suggestive of the role of PSMD9 in transcriptional regulation, mRNA processing and editing, hormone and receptor activity and protein translation. Our proof-of-principle experiments endorse a novel and quick method for the identification of putative interacting partners of similar PDZ-domain proteins from the proteome and for discovering novel functions. PMID:25009770

  8. Downregulation of 5-HT7 Serotonin Receptors by the Atypical Antipsychotics Clozapine and Olanzapine. Role of Motifs in the C-Terminal Domain and Interaction with GASP-1.

    PubMed

    Manfra, Ornella; Van Craenenbroeck, Kathleen; Skieterska, Kamila; Frimurer, Thomas; Schwartz, Thue W; Levy, Finn Olav; Andressen, Kjetil Wessel

    2015-07-15

    The human 5-HT7 serotonin receptor, a G-protein-coupled receptor (GPCR), activates adenylyl cyclase constitutively and upon agonist activation. Biased ligands differentially activate 5-HT7 serotonin receptor desensitization, internalization and degradation in addition to G protein activation. We have previously found that the atypical antipsychotics clozapine and olanzapine inhibited G protein activation and, surprisingly, induced both internalization and lysosomal degradation of 5-HT7 receptors. Here, we aimed to determine the mechanism of clozapine- and olanzapine-mediated degradation of 5-HT7 receptors. In the C-terminus of the 5-HT7 receptor, we identified two YXXΦ motifs, LR residues, and a palmitoylated cysteine anchor as potential sites involved in receptor trafficking to lysosomes followed by receptor degradation. Mutating either of these sites inhibited clozapine- and olanzapine-mediated degradation of 5-HT7 receptors and also interfered with G protein activation. In addition, we tested whether receptor degradation was mediated by the GPCR-associated sorting protein-1 (GASP-1). We show that GASP-1 binds the 5-HT7 receptor and regulates the clozapine-mediated degradation. Mutations of the identified motifs and residues, located in or close to Helix-VIII of the 5-HT7 receptor, modified antipsychotic-stimulated binding of proteins (such as GASP-1), possibly by altering the flexibility of Helix-VIII, and also interfered with G protein activation. Taken together, our data demonstrate that binding of clozapine or olanzapine to the 5-HT7 receptor leads to antagonist-mediated lysosomal degradation by exposing key residues in the C-terminal tail that interact with GASP-1. PMID:25706089

  9. Atypical autoerotic deaths

    SciTech Connect

    Gowitt, G.T.; Hanzlick, R.L. )

    1992-06-01

    So-called typical' autoerotic fatalities are the result of asphyxia due to mechanical compression of the neck, chest, or abdomen, whereas atypical' autoeroticism involves sexual self-stimulation by other means. The authors present five atypical autoerotic fatalities that involved the use of dichlorodifluoromethane, nitrous oxide, isobutyl nitrite, cocaine, or compounds containing 1-1-1-trichloroethane. Mechanisms of death are discussed in each case and the pertinent literature is reviewed.

  10. "Redefining RA": The RA Tool Kit

    ERIC Educational Resources Information Center

    Wyatt, Neal

    2008-01-01

    No one likes being two steps behind, and the fastest way to fall off the pace is by not keeping up with major titles and hot authors. Fortunately, there are numerous resources, both prepublication and postpublication, that can help. It is best when readers' advisory (RA) librarians know what is coming out months ahead of time--in order to think…

  11. Atypical presentation of atypical mycobacteria in atypical diabetes

    PubMed Central

    Biswas, Sugata Narayan; Chakraborty, Partha Pratim; Satpathi, Partha Sarathi; Patra, Shinjan

    2016-01-01

    A 45-year-old, non-obese male presented with low-grade, remittent fever and a fluctuant swelling over the posterior aspect of his lower left flank. Laboratory tests revealed leukocytosis, raised ESR, hyperglycemia and raised HbA1C levels. Light microscopy of Ziehl–Neelsen-stained pus sample revealed numerous acid-fast bacilli. After 72 h of incubating aspirated pus in Löwenstein–Jensen media, non-pigmented, cream-colored colonies were observed, suggestive of rapid-growing atypical forms of mycobacteria. Polymerase chain reaction of isolated bacteria identified Mycobacterium chelonae as causative organism. Abdominal skiagram revealed extensive pancreatic intraductal calcifications suggestive of fibrocalculous pancreatic diabetes and lumbar vertebral body destruction with evidence of paravertebral abscess. The patient was prescribed a split-mixed insulin regimen, clarithromycin and ciprofloxacin with complete resolution of the subcutaneous abscess at 6 months. Diabetic patients are prone to infections. Mycobacteria, especially atypical ones, involving the spine and subcutaneous tissues have rarely been reported. PMID:27127641

  12. Martin RA-30 Baltimore

    NASA Technical Reports Server (NTRS)

    1943-01-01

    Martin RA-30 Baltimore: The Martin RA-30 Baltimore was a light bomber ordered by the Royal Air Force. Some examples were retained in the United States as part of a 'Reverse Lend-Lease.' This example was flown by the NACA from June 1943 until March 1944.

  13. Atypical Optic Neuritis.

    PubMed

    Gaier, Eric D; Boudreault, Katherine; Rizzo, Joseph F; Falardeau, Julie; Cestari, Dean M

    2015-12-01

    Classic demyelinative optic neuritis is associated with multiple sclerosis and typically carries a good prognosis for visual recovery. This disorder is well characterized with respect to its presentation and clinical features by baseline data obtained through the optic neuritis treatment trial and numerous other studies. Atypical optic neuritis entails clinical manifestations that deviate from this classic pattern of features. Clinical signs and symptoms that deviate from the typical presentation should prompt consideration of less common etiologies. Atypical features to consider include lack of pain, simultaneous or near-simultaneous onset, lack of response to or relapse upon tapering from corticosteroids, or optic nerve head or peripapillary hemorrhages. The most important alternative etiologies to consider and the steps towards their respective diagnostic evaluations are suggested for these atypical features. PMID:26467052

  14. Atypical combinations and scientific impact.

    PubMed

    Uzzi, Brian; Mukherjee, Satyam; Stringer, Michael; Jones, Ben

    2013-10-25

    Novelty is an essential feature of creative ideas, yet the building blocks of new ideas are often embodied in existing knowledge. From this perspective, balancing atypical knowledge with conventional knowledge may be critical to the link between innovativeness and impact. Our analysis of 17.9 million papers spanning all scientific fields suggests that science follows a nearly universal pattern: The highest-impact science is primarily grounded in exceptionally conventional combinations of prior work yet simultaneously features an intrusion of unusual combinations. Papers of this type were twice as likely to be highly cited works. Novel combinations of prior work are rare, yet teams are 37.7% more likely than solo authors to insert novel combinations into familiar knowledge domains. PMID:24159044

  15. Measuring the radium quartet (228Ra, 226Ra, 224Ra, 223Ra) in seawater samples using gamma spectrometry.

    PubMed

    van Beek, P; Souhaut, M; Reyss, J-L

    2010-07-01

    Radium isotopes are widely used in marine studies (eg. to trace water masses, to quantify mixing processes or to study submarine groundwater discharge). While 228Ra and 226Ra are usually measured using gamma spectrometry, short-lived Ra isotopes (224Ra and 223Ra) are usually measured using a Radium Delayed Coincidence Counter (RaDeCC). Here we show that the four radium isotopes can be analyzed using gamma spectrometry. We report 226Ra, 228Ra, 224Ra, 223Ra activities measured using low-background gamma spectrometry in standard samples, in water samples collected in the vicinity of our laboratory (La Palme and Vaccarès lagoons, France) but also in seawater samples collected in the plume of the Amazon river, off French Guyana (AMANDES project). The 223Ra and 224Ra activities determined in these samples using gamma spectrometry were compared to the activities determined using RaDeCC. Activities determined using the two techniques are in good agreement. Uncertainties associated with the 224Ra activities are similar for the two techniques. RaDeCC is more sensitive for the detection of low 223Ra activities. Gamma spectrometry thus constitutes an alternate method for the determination of short-lived Ra isotopes. PMID:20106569

  16. Endocervical Atypical Polypoid Adenomyoma.

    PubMed

    Protopapas, Athanasios; Sotiropoulou, Maria; Athanasiou, Stavros; Loutradis, Dimitrios

    2016-01-01

    Atypical polypoid adenomyomas (APAMs) are rare uterine tumors that occur predominantly in premenopausal women, with less than 250 cases reported so far, worldwide. They may recur after treatment, and they may coexist with, or precede development of an endometrial adenocarcinoma. For this reason cases managed with conservative surgery or medical therapies require long-term follow-up. We report the case of a 41 years old nulliparous patient who during a diagnostic hysteroscopy was found with an endocervical atypical polypoid adenomyoma (APAM). The patient was desirous of a pregnancy, reported menometrorrhagia, and had a coexistent 5 cm, grade 2, submucous myoma, 3 endometrial polyps, and diffuse adenomyosis. She was treated with hysteroscopic resection of the APAM and polyps, plus laparoscopic myomectomy and wedge resection of adenomyosis. She is on an IVF list and after 4 months she is symptoms-free. PMID:26304721

  17. Atypical odontalgia--an update.

    PubMed

    Patel, Seena B; Boros, Audrey L; Kumar, Satish K S

    2012-09-01

    Atypical odontalgia is a commonly misdiagnosed condition that frequently leads to unnecessary dental treatments such as extraction and endodontic therapy. These treatments often worsen the pain. Despite greater recognition and understanding of this condition, proper diagnosis and treatment remains a challenge. It is believed that atypical odontalgia is a neuropathic condition. This article updates the current understanding of the etiology and pathophysiology of atypical odontalgia, and provides appropriate diagnostic and management approaches for this condition. PMID:23097829

  18. Testing atypical depression definitions.

    PubMed

    Benazzi, Franco

    2005-01-01

    The evidence supporting the DSM-IV definition of atypical depression (AD) is weak. This study aimed to test different definitions of AD. Major depressive disorder (MDD) patients (N = 254) and bipolar-II (BP-II) outpatients (N = 348) were interviewed consecutively, during major depressive episodes, with the Structured Clinical Interview for DSM-IV. DSM-IV criteria for AD were followed. AD validators were female gender, young onset, BP-II, axis I comorbidity, bipolar family history. Frequency of DSM-IV AD was 43.0%. AD, versus non-AD, was significantly associated with all AD validators, apart from comorbidity when controlling for age and sex. Factor analysis of atypical symptoms found factor 1 including oversleeping, overeating and weight gain (leaden paralysis at trend correlation), and factor 2 including interpersonal sensitivity, mood reactivity, and leaden paralysis. Multiple logistic regression of factor 1 versus AD validators found significant associations with several validators (including bipolar family history), whereas factor 2 had no significant associations. Findings may support a new definition of AD based on the state-dependent features oversleeping and overeating (plus perhaps leaden paralysis) versus the current AD definition based on a combination of state and trait features. Pharmacological studies are required to support any new definition of AD, as the current concept of AD is based on different response to TCA antidepressants versus non-AD. PMID:16175877

  19. Atypical histiocytosis in the lung

    PubMed Central

    Cao, Weijun; Zhang, Rongxuan; Zhou, Ying; Xu, Jinfu; Garfield, David H.

    2013-01-01

    Objective To report a rare case of atypical histiocytic tumor of the lung with a review of literature. Methods The clinical materials were noted. Literature related to this condition from the past 50 years was reviewed from the group of histiocytic tumors. Results and conclusions Clinical manifestations were non-specific. The imaging characteristics of our case were infiltrative lesions with multiple cysts in both lungs. Pathology showed nodular proliferation of atypical cells. Immunohistochemistry suggested a histiocytic origin of the infiltrating atypical cells. Because the pathological findings did not fall into any particular category of typical histiocytic tumors, the final diagnosis was atypical histiocytic tumor. The presentation of atypical histiocytic tumor of the lungs, only, with infiltrative lesions and multiple air cysts seems very rare, with pathological examination being “gold standard” for the diagnosis. PMID:23991320

  20. Atypical causes of cholestasis

    PubMed Central

    Nguyen, Ken D; Sundaram, Vinay; Ayoub, Walid S

    2014-01-01

    Cholestatic liver disease consists of a variety of disorders. Primary sclerosing cholangitis and primary biliary cirrhosis are the most commonly recognized cholestatic liver disease in the adult population, while biliary atresia and Alagille syndrome are commonly recognized in the pediatric population. In infants, the causes are usually congenital or inherited. Even though jaundice is a hallmark of cholestasis, it is not always seen in adult patients with chronic liver disease. Patients can have “silent” progressive cholestatic liver disease for years prior to development of symptoms such as jaundice and pruritus. In this review, we will discuss some of the atypical causes of cholestatic liver disease such as benign recurrent intrahepatic cholestasis, progressive familial intrahepatic cholestasis, Alagille Syndrome, biliary atresia, total parenteral nutrition induced cholestasis and cholestasis secondary to drug induced liver injury. PMID:25071336

  1. Dermatofibroma: Atypical Presentations.

    PubMed

    Bandyopadhyay, Mousumi Roy; Besra, Mrinal; Dutta, Somasree; Sarkar, Somnath

    2016-01-01

    Dermatofibroma is a common benign fibrohistiocytic tumor and its diagnosis is easy when it presents classical clinicopathological features. However, a dermatofibroma may show a wide variety of clinicopathological variants and, therefore, the diagnosis may be difficult. The typical dermatofibroma generally occurs as a single or multiple firm reddish-brown nodules. We report here two atypical presentations of dermatofibroma - Atrophic dermatofibroma and keloidal presentation of dermatofibroma. Clinical dermal atrophy is a common phenomenon in dermatofibromas as demonstrated by the dimpling on lateral pressure. However, this feature is exaggerated in the atrophic variant of dermatofibroma. Atrophic dermatofibroma is defined by dermal atrophy of more than 50% of the lesion apart from the usual features of common dermatofibroma. The keloidal variant of dermatofibroma should not be overlooked as a simple keloid. The findings of keloidal change in dermatofibromas may support that trauma is a possible cause of dermatofibroma. PMID:26955137

  2. Atypical odontalgia: a review.

    PubMed

    Koratkar, Harish; Pedersen, Jerome

    2008-01-01

    Since persistent and chronic pain is more common in the head and neck region than in any other part of the body, dentists are more likely to encounter these rather complex cases in their practices. This article is a review and update on atypical odontalgia (AO). AO is a persistent neuropathic pain which may be initiated after deafferentiation of trigeminal nerve fibers following root canal treatment, apicectomy, or tooth extraction, or it may be of idiopathic origin. Details concerning its characteristics, pathophysiology, diagnostic criteria, differential diagnosis, and treatment are made. The aim of this article is to help the clinician with the diagnosis and management of AO. The prognosis for AO is most often only fair, and the administration of tricyclic antidepressants often resolves symptoms. Invasive and irreversible treatment attempts are not recommended. PMID:18363287

  3. Atypical neuroleptic malignant syndrome.

    PubMed

    Collins, Ann; Davies, Drew; Menon, Sharmila

    2016-01-01

    A 57-year-old man was admitted to a psychiatric ward in a confused state. He had a 30-year history of lately stable schizophrenia and antipsychotic medication had recently been reduced. The clinical picture was characterised by confusion, agitation, autonomic instability, muscle rigidity and elevated creatine kinase. Despite no other identifiable cause, physicians were reluctant to accept a diagnosis of neuroleptic malignant syndrome (NMS) due to the absence of fever. Despite acute renal failure, the patient was repeatedly transferred between medical and psychiatric wards; diagnosis and management were delayed, with potentially catastrophic consequences. NMS is a rare, life-threatening neurological disorder that can present atypically and requires emergency medical rather than psychiatric care. Clinicians must proactively distinguish between medical emergencies (including acute confusional states/delirium) and mental illness. Prompt, accurate diagnosis, management on the appropriate ward and effective teamwork between specialties are essential to improve patient outcomes in this potentially fatal condition. PMID:27298291

  4. Dermatofibroma: Atypical Presentations

    PubMed Central

    Bandyopadhyay, Mousumi Roy; Besra, Mrinal; Dutta, Somasree; Sarkar, Somnath

    2016-01-01

    Dermatofibroma is a common benign fibrohistiocytic tumor and its diagnosis is easy when it presents classical clinicopathological features. However, a dermatofibroma may show a wide variety of clinicopathological variants and, therefore, the diagnosis may be difficult. The typical dermatofibroma generally occurs as a single or multiple firm reddish-brown nodules. We report here two atypical presentations of dermatofibroma - Atrophic dermatofibroma and keloidal presentation of dermatofibroma. Clinical dermal atrophy is a common phenomenon in dermatofibromas as demonstrated by the dimpling on lateral pressure. However, this feature is exaggerated in the atrophic variant of dermatofibroma. Atrophic dermatofibroma is defined by dermal atrophy of more than 50% of the lesion apart from the usual features of common dermatofibroma. The keloidal variant of dermatofibroma should not be overlooked as a simple keloid. The findings of keloidal change in dermatofibromas may support that trauma is a possible cause of dermatofibroma. PMID:26955137

  5. [Atypical antipsychotics in the elderly].

    PubMed

    van Melick, E J M

    2004-12-01

    Central criteria for the definition of atypical antipsychotics are antipsychotic efficacy and minimal or none extrapyramidal symptoms (EPS). This last criterium is of importance in the differentiation with the traditional antipsychotics. Of the four atypical antipsychotics which are discussed here, clozapine is the most atypical. The best proof is its good efficacy in the treatment of Parkinson psychosis with minimal adverse effects on motor function. Clozapine is the best choice for this indication. At this moment there is not enough evidence available concerning quetiapine. Risperidon and olanzapine give more Dopamine2-occupancy with higher doses and can evoke EPS, but this is still less compared to the traditional antipsychotics. All four atypical drugs cause less tardive dyskinesia. Atypical antipsychotics are not well studied in the treatment of elderly patients with functional psychosis. However the available information and the literature on the treatment of young adults makes it probable that the atypical antipsychotics are at least as effective in the elderly as the traditional antipsychotics. The median daily doses are lower for elderly than for younger patients. Risperidon has been proven effective in the treatment of agressive behaviour in dementia. Atypical antipsychotics have their 'own' adverse effects. Those which have the most impact in the elderly are discussed. PMID:15704604

  6. [Atypical hemolytic uremic syndrome].

    PubMed

    Blasco Pelicano, Miquel; Rodríguez de Córdoba, Santiago; Campistol Plana, Josep M

    2015-11-20

    The hemolytic uremic syndrome (HUS) is a clinical entity characterized by thrombocytopenia, non-immune hemolytic anemia and renal impairment. Kidney pathology shows thrombotic microangiopathy (TMA) with endothelial cell injury leading to thrombotic occlusion of arterioles and capillaries. Traditionally, HUS was classified in 2 forms: Typical HUS, most frequently occurring in children and caused by Shiga-toxin-producing bacteria, and atypical HUS (aHUS). aHUS is associated with mutations in complement genes in 50-60% of patients and has worse prognosis, with the majority of patients developing end stage renal disease. After kidney transplantation HUS may develop as a recurrence of aHUS or as de novo disease. Over the last years, many studies have demonstrated that complement dysregulation underlies the endothelial damage that triggers the development of TMA in most of these patients. Advances in our understanding of the pathogenic mechanisms of aHUS, together with the availability of novel therapeutic options, will enable better strategies for the early diagnosis and etiological treatment, which are changing the natural history of aHUS. This review summarizes the aHUS clinical entity and describes the role of complement dysregulation in the pathogenesis of aHUS. Finally, we review the differential diagnosis and the therapeutic options available to patients with aHUS. PMID:25433773

  7. KINOMIC ALTERATIONS IN ATYPICAL MENINGIOMA

    PubMed Central

    Anderson, Joshua C.; Taylor, Robert B.; Fiveash, John B.; de Wijn, Rik; Gillespie, G. Yancey; Willey, Christopher D.

    2015-01-01

    Background We sought to profile Atypical Meningioma in a high-throughput manner to better understand the altered signaling within these tumors and specifically the kinases altered in recurrent atypical meningioma. Kinomic Profiles could be used to identify prognostic biomarkers for responders/non-responders to classify future patients that are unlikely to benefit from current therapies. Directly these results could be used to identify drug-actionable kinase targets as well. Methods Peptide-substrate microarray kinase activity analysis was conducted with a PamStation®12 analyzing the tyrosine kinome in each tumor kinetically against ~144 target peptides. These data were then analyzed relative to clinical outcome (e.g., tumor recurrence). Results 3 major clusters of atypical meningiomas were identified with highly variant peptides primarily being targets of EGFR family, ABL, BRK and BMX kinases. Kinomic analysis of recurrent atypical meningiomas indicated patterns of increased phosphorylation of BMX, TYRO3 and FAK substrates as compared to non-recurrent tumors. Conclusion The atypical meningiomas profiled here exhibited molecular sub-clustering that may have phenotypic corollaries predictive of outcome. Recurrent tumors had increases in kinase activity that may predict resistance to current therapies, and may guide selection of directed therapies. Taken together these data further the understanding of kinomic alteration in atypical meningioma, and the processes that may not only mediate recurrence, but additionally may identify kinase targets for intervention. PMID:27158663

  8. Atypical hemolytic uremic syndrome

    PubMed Central

    2011-01-01

    Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5 -10% of HUS in children, but the majority of HUS in adults. The incidence of complement-aHUS is not known precisely. However, more than 1000 aHUS patients investigated for complement abnormalities have been reported. Onset is from the neonatal period to the adult age. Most patients present with hemolytic anemia, thrombocytopenia and renal failure and 20% have extra renal manifestations. Two to 10% die and one third progress to end-stage renal failure at first episode. Half of patients have relapses. Mutations in the genes encoding complement regulatory proteins factor H, membrane cofactor protein (MCP), factor I or thrombomodulin have been demonstrated in 20-30%, 5-15%, 4-10% and 3-5% of patients respectively, and mutations in the genes of C3 convertase proteins, C3 and factor B, in 2-10% and 1-4%. In addition, 6-10% of patients have anti-factor H antibodies. Diagnosis of aHUS relies on 1) No associated disease 2) No criteria for Shigatoxin-HUS (stool culture and PCR for Shiga-toxins; serology for anti-lipopolysaccharides antibodies) 3) No criteria for thrombotic thrombocytopenic purpura (serum ADAMTS 13 activity > 10%). Investigation of the complement system is required (C3, C4, factor H and factor I plasma concentration, MCP expression on leukocytes and anti-factor H antibodies; genetic screening to identify risk factors). The disease is familial in approximately 20% of pedigrees, with an autosomal recessive or dominant mode of transmission. As penetrance of the disease is 50%, genetic counseling is difficult. Plasmatherapy has been first line treatment until presently

  9. Identification of atypical flight patterns

    NASA Technical Reports Server (NTRS)

    Statler, Irving C. (Inventor); Ferryman, Thomas A. (Inventor); Amidan, Brett G. (Inventor); Whitney, Paul D. (Inventor); White, Amanda M. (Inventor); Willse, Alan R. (Inventor); Cooley, Scott K. (Inventor); Jay, Joseph Griffith (Inventor); Lawrence, Robert E. (Inventor); Mosbrucker, Chris (Inventor)

    2005-01-01

    Method and system for analyzing aircraft data, including multiple selected flight parameters for a selected phase of a selected flight, and for determining when the selected phase of the selected flight is atypical, when compared with corresponding data for the same phase for other similar flights. A flight signature is computed using continuous-valued and discrete-valued flight parameters for the selected flight parameters and is optionally compared with a statistical distribution of other observed flight signatures, yielding atypicality scores for the same phase for other similar flights. A cluster analysis is optionally applied to the flight signatures to define an optimal collection of clusters. A level of atypicality for a selected flight is estimated, based upon an index associated with the cluster analysis.

  10. [Atypical antipsychotics and metabolic syndrome].

    PubMed

    Baranyi, Andreas; Yazdani, Renè; Haas-Krammer, Alexandra; Stepan, Alexandra; Kapfhammer, Hans-Peter; Rothenhäusler, Hans-Bernd

    2007-01-01

    The introduction of atypical antipsychotics in psychopharmacology represented a major advance in the treatment of psychotic disorders. However, there have been numerous studies that certain atypical antipsychotics may be associated with a greater risk of metabolic abnormalities than others, including weight gain, hyperlipidemia and new-onset typ 2 diabetes mellitus. A G-Protein beta3 subunit Gen (C825T) polymorphism, an increased carbohydrate metabolism and dyshormonism are discussed as pathogenetic mechanisms. High risk patients (adiposity, hyperlipidaemia, hyperglycaemia, preexisting diabetes) should maintain an antipsychotic agent with a favourable side effect profile. In these cases a periodical diabetes screening and blood lipid controls are required. Clinicans must balance the significant benefits of atypical antipsychotics against the risk of metabolic disturbances. In this article recent findings are reviewed. PMID:17915438

  11. [Pathogenesis of atypical femoral fracture].

    PubMed

    Iwata, Ken; Mashiba, Tasuku

    2016-01-01

    We demonstrated microdamage accumulation in the fracture sites in the patients of subtrochanteric atypical femoral fracture with long term bisphosphonate therapy and of incomplete shaft fracture of lateral femoral bowing without bisphosphonate therapy. Based on these findings, pathogenesis of atypical femoral fracture is revealed stress fracture caused by accumulation of microdamages between distal to the lesser trochanter and proximal to the supracondylar flare in the femur in association with severely suppressed bone turnover and/or abnormal lower limb alignment, that causes stress concentration on the lateral side cortex of the femur. PMID:26728533

  12. Atypical neurological manifestations of malaria

    PubMed Central

    Singla, Neeraj; Gupta, Monica; Singh, Ram; Kumar, Ashwani

    2014-01-01

    Malaria is known as a great mimic. It can manifest subtly or abruptly, typically or atypically. This aspect of the disease can frequently mislead physicians. We present two patients of malaria with atypical neurological manifestations. The first patient of Plasmodium falciparum malaria presented with fever and altered sensorium; MRI of the brain suggested cerebral venous thrombosis. The second patient of Plasmodium vivax presented with fever, double vision and right eye lateral rectus palsy due to unilateral sixth cranial nerve involvement. Both patients were managed with antimalarials and supportive medical management, and had uneventful recoveries. PMID:25150266

  13. The Rab GTPase-Activating Protein TBC1D4/AS160 Contains an Atypical Phosphotyrosine-Binding Domain That Interacts with Plasma Membrane Phospholipids To Facilitate GLUT4 Trafficking in Adipocytes

    PubMed Central

    Tan, Shi-Xiong; Ng, Yvonne; Burchfield, James G.; Ramm, Georg; Lambright, David G.; Stöckli, Jacqueline

    2012-01-01

    The Rab GTPase-activating protein TBC1D4/AS160 regulates GLUT4 trafficking in adipocytes. Nonphosphorylated AS160 binds to GLUT4 vesicles and inhibits GLUT4 translocation, and AS160 phosphorylation overcomes this inhibitory effect. In the present study we detected several new functional features of AS160. The second phosphotyrosine-binding domain in AS160 encodes a phospholipid-binding domain that facilitates plasma membrane (PM) targeting of AS160, and this function is conserved in other related RabGAP/Tre-2/Bub2/Cdc16 (TBC) proteins and an AS160 ortholog in Drosophila. This region also contains a nonoverlapping intracellular GLUT4-containing storage vesicle (GSV) cargo-binding site. The interaction of AS160 with GSVs and not with the PM confers the inhibitory effect of AS160 on insulin-dependent GLUT4 translocation. Constitutive targeting of AS160 to the PM increased the surface GLUT4 levels, and this was attributed to both enhanced AS160 phosphorylation and 14-3-3 binding and inhibition of AS160 GAP activity. We propose a model wherein AS160 acts as a regulatory switch in the docking and/or fusion of GSVs with the PM. PMID:23045393

  14. Atypical cadherin negotiates a turn.

    PubMed

    Shi, Dongbo; Fujimori, Toshihiko; Uemura, Tadashi

    2013-07-15

    Planar cell polarity (PCP) signaling is involved in many polarized cell behaviors. In this issue of Developmental Cell, Tatin et al. (2013) show that the atypical cadherin Celsr1 is transiently localized to cellular protrusions in lymphatic endothelial cells and acts to orient valve-forming cells perpendicular to the vessel axis. PMID:23867224

  15. Atypical Cogan's syndrome mimicking encephalitis.

    PubMed

    Lepur, Dragan; Vranjican, Zoran; Himbele, Josip; Barsić, Bruno; Klinar, Igor

    2004-01-01

    Cogan's syndrome is a rare autoimmune multisystem disease. The main clinical features of typical Cogan's syndrome are vestibuloauditory dysfunction and interstitial keratitis. The authors present a case of atypical Cogan's syndrome with headache, fever, deafness, trigeminal neuralgia and electroencephalographic abnormality which mimicked viral encephalitis. PMID:15307593

  16. Recognition and diagnosis of atypical depression.

    PubMed

    Thase, Michael E

    2007-01-01

    The term atypical depression dates to the first wave of reports describing differential response to monoamine oxidase inhibitors (MAOIs) and tricyclic antidepressants (TCAs). In contrast to more TCA-responsive depressions, patients with so-called atypical symptoms (e.g., hypersomnia, interpersonal sensitivity, leaden paralysis, increased appetite and/or weight, and phobic anxiety) were observed to be more responsive to MAOIs. After several decades of controversy and debate, the phrase "with atypical features" was added as an episode specifier in the DSM-IV in 1994. The 1-year prevalence of the defined atypical depression subtype is approximately 1% to 4%; around 15% to 29% of patients with major depressive disorder have atypical depression. Hardly "atypical" in contemporary contexts, atypical depression also is common in dysthymic bipolar II disorders and is notable for its early age at onset, more chronic course, and high rates of comorbidity with social phobia and panic disorder with agoraphobia. The requirement of preserved mood reactivity is arguably the most controversial of the DSM-IV criteria for atypical depression. When compared with melancholia, the neurobiological profiles of patients with atypical depression are relatively normal. The utility of the atypical depression subtype for differential therapeutics diminished substantially when the TCAs were supplanted as first-line antidepressants by the selective serotonin reuptake inhibitors. Although introduction of safer MAOIs has fostered renewed interest in atypical depression, the validity and importance of the DSM-IV definition of atypical depression for the nosology of affective illness remains an open question. PMID:17640153

  17. A case of atypical progressive supranuclear palsy

    PubMed Central

    Spaccavento, Simona; Del Prete, Marina; Craca, Angela; Loverre, Anna

    2014-01-01

    Background Progressive supranuclear palsy (PSP) is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progressive apraxia of speech (AOS) and aphasia. Aim We aimed to investigate the clinical presentation of PSP, using a comprehensive multidimensional evaluation, and the disease response to various pharmacological treatments. Methods A 72-year-old right-handed male, with 17 years education, who first presented with aphasia, AOS, depression, apathy, and postural instability at 69 years; a complete neuropsychological evaluation, tapping the different cognitive domains, was performed. Results Testing revealed a moderate global cognitive deficit (Mini-Mental State Examination test score =20), low memory test scores (story recall, Rey’s 15-word Immediate and Delayed Recall), and poor phonemic and semantic fluency. The patient’s language was characterized by AOS, with slow speech rate, prolonged intervals between syllables and words, decreased articulatory accuracy, sound distortions, and anomia. Behavioral changes, such as depression, anxiety, apathy, and irritability, were reported. The neurological examination revealed supranuclear vertical gaze palsy, poor face miming, and a mild balance deficit. Magnetic resonance imaging showed only widespread cortical atrophy. Single photon emission computed tomography demonstrated left > right frontotemporal cortical abnormalities. After 6 months, a further neuropsychological assessment showed a progression in cognitive deficits, with additional attention deficits. The patient reported frequent falls, but the neurological deficits remained unchanged. Neuroimaging tests showed the same brain involvement. Conclusion Our case highlights the heterogeneity of the clinical features in this syndrome, demonstrating that atypical PSP can present as AOS and aphasia, without the classical features or involvement of the subcortical gray

  18. Atypical fractures, a biased perspective.

    PubMed

    Aspenberg, Per

    2016-01-01

    When stress fractures started to show up in the femurs of elderly ladies, it was soon evident that bisphosphonate use lay behind, and the absolute risk increase due to bisphosphonate use was reasonably well estimated already in 2008. Thereafter followed a period of confusion: the term atypical fracture was introduced, with a definition so vague that the true stress fractures tended to disappear in a cloud of ambiguity. This cast doubt on the association with bisphosphonates. The association was then re-established by large epidemiological studies based on radiographic adjudication. Atypical fractures are largely caused by bisphosphonates. With a correct indication, bisphosphonates prevent many more fractures than they cause, at least during the first years of use. With an incorrect indication they are likely to cause more harm than good. PMID:26768286

  19. Atypical centrioles during sexual reproduction.

    PubMed

    Avidor-Reiss, Tomer; Khire, Atul; Fishman, Emily L; Jo, Kyoung H

    2015-01-01

    Centrioles are conserved, self-replicating, microtubule-based, 9-fold symmetric subcellular organelles that are essential for proper cell division and function. Most cells have two centrioles and maintaining this number of centrioles is important for animal development and physiology. However, how animals gain their first two centrioles during reproduction is only partially understood. It is well established that in most animals, the centrioles are contributed to the zygote by the sperm. However, in humans and many animals, the sperm centrioles are modified in their structure and protein composition, or they appear to be missing altogether. In these animals, the origin of the first centrioles is not clear. Here, we review various hypotheses on how centrioles are gained during reproduction and describe specialized functions of the zygotic centrioles. In particular, we discuss a new and atypical centriole found in sperm and zygote, called the proximal centriole-like structure (PCL). We also discuss another type of atypical centriole, the "zombie" centriole, which is degenerated but functional. Together, the presence of centrioles, PCL, and zombie centrioles suggests a universal mechanism of centriole inheritance among animals and new causes of infertility. Since the atypical centrioles of sperm and zygote share similar functions with typical centrioles in somatic cells, they can provide unmatched insight into centriole biology. PMID:25883936

  20. Atypical centrioles during sexual reproduction

    PubMed Central

    Avidor-Reiss, Tomer; Khire, Atul; Fishman, Emily L.; Jo, Kyoung H.

    2015-01-01

    Centrioles are conserved, self-replicating, microtubule-based, 9-fold symmetric subcellular organelles that are essential for proper cell division and function. Most cells have two centrioles and maintaining this number of centrioles is important for animal development and physiology. However, how animals gain their first two centrioles during reproduction is only partially understood. It is well established that in most animals, the centrioles are contributed to the zygote by the sperm. However, in humans and many animals, the sperm centrioles are modified in their structure and protein composition, or they appear to be missing altogether. In these animals, the origin of the first centrioles is not clear. Here, we review various hypotheses on how centrioles are gained during reproduction and describe specialized functions of the zygotic centrioles. In particular, we discuss a new and atypical centriole found in sperm and zygote, called the proximal centriole-like structure (PCL). We also discuss another type of atypical centriole, the “zombie” centriole, which is degenerated but functional. Together, the presence of centrioles, PCL, and zombie centrioles suggests a universal mechanism of centriole inheritance among animals and new causes of infertility. Since the atypical centrioles of sperm and zygote share similar functions with typical centrioles in somatic cells, they can provide unmatched insight into centriole biology. PMID:25883936

  1. Radium content and the 226Ra /228Ra activity ratio in groundwater from bedrock

    NASA Astrophysics Data System (ADS)

    Asikainen, Matti

    1981-08-01

    The relative abundance of 226Ra and 228Ra were determined in the groundwater from 125 drilled wells containing from < 0.1 to 51.3 pCi/l of 226Ra. The determination of 228Ra was carried out with a liquid scintillation counter by measuring only the weakly energetic β particles emitted from 228Ra. Thus the interference from the daughter nuclides of 226Ra was avoided, without specific separation of 228Ac. The direct measurement of 228Ra made the method decisively simpler and faster in terms of the chemistry involved. The concentration of 228Ra was found to be independent of the amount of 226Ra present in the samples. The concentrations of 228Ra were nearly the same over the whole range of 226Ra concentrations and the average sol 226Ra /228Ra ratio sharply increased as the 226Ra content of water increased. The 226Ra /228Ra ratio in the drilled wells varied from 0.3 to 26. Abnormally high 226Ra /228Ra ratios were found in areas with known uranium deposits as well as in several drilled wells at other locations. The abnormally high 226Ra /228Ra ratios present in groundwater suggest that the radioactivity anomaly is caused by uranium deposits and not by common rocks. In samples with a low radioactivity level the average 226Ra /228Ra ratio was slightly below unity, corresponding to the typical U/ Th ratio of granite, the most common kind of rock in the study area. The samples from the rapakivi area proved to be exceptional in that they had a low 226Ra /228Ra ratio independent of the concentration of 226Ra.

  2. Information Display System for Atypical Flight Phase

    NASA Technical Reports Server (NTRS)

    Statler, Irving C. (Inventor); Ferryman, Thomas A. (Inventor); Amidan, Brett G. (Inventor); Whitney, Paul D. (Inventor); White, Amanda M. (Inventor); Willse, Alan R. (Inventor); Cooley, Scott K. (Inventor); Jay, Joseph Griffith (Inventor); Lawrence, Robert E. (Inventor); Mosbrucker, Chris J. (Inventor); Rosenthal, Loren J. (Inventor); Lynch, Robert E. (Inventor); Chidester, Thomas R. (Inventor); Prothero, Gary L. (Inventor); Andrei, Adi (Inventor); Romanowski, Timothy P. (Inventor); Robin, Daniel E. (Inventor); Prothero, Jason W. (Inventor)

    2007-01-01

    Method and system for displaying information on one or more aircraft flights, where at least one flight is determined to have at least one atypical flight phase according to specified criteria. A flight parameter trace for an atypical phase is displayed and compared graphically with a group of traces, for the corresponding flight phase and corresponding flight parameter, for flights that do not manifest atypicality in that phase.

  3. Atypical presentation of infective endocarditis.

    PubMed

    Arunachalam, Karuppiah

    2016-01-01

    The HACEK group of organisms are one of the infrequent causes of infective endocarditis. Infective endocarditis should be recognized and treated promptly to prevent excessive morbidity and mortality associated with the disease. Sometimes the diagnosis is delayed due to vague and subtle presentation. Through this case report, risk factors of Cardiobacterium hominis endocarditis and its atypical presentation is illustrated to increase the recognition of infective endocarditis as one of the differential diagnosis. [Full article available at http://rimed.org/rimedicaljournal-2016-07.asp, free with no login]. PMID:27379355

  4. Atypical parkinsonism: diagnosis and treatment.

    PubMed

    Stamelou, Maria; Bhatia, Kailash P

    2015-02-01

    Atypical parkinsonism comprises typically progressive supranuclear palsy, corticobasal degeneration, and mutilple system atrophy, which are distinct pathologic entities; despite ongoing research, their cause and pathophysiology are still unknown, and there are no biomarkers or effective treatments available. The expanding phenotypic spectrum of these disorders as well as the expanding pathologic spectrum of their classic phenotypes makes the early differential diagnosis challenging for the clinician. Here, clinical features and investigations that may help to diagnose these conditions and the existing limited treatment options are discussed. PMID:25432722

  5. Atypical odontalgia: a case report.

    PubMed

    Koratkar, Harish; Koratkar, Sonal

    2008-01-01

    Diagnosis and treatment of orofacial pain is not uncommon; however, reaching a definitive diagnosis in these cases can be a complex challenge. Dentists are most likely to face this situation, because persistent and chronic pain is more common in the head and neck region than in any other part of the body. However, the complexities and diagnostic challenges mean that misdiagnosing neuropathic pain is common. This article presents a case of atypical odontalgia and illustrates the complexities involved when diagnosing the condition. PMID:19284197

  6. Atypical Teratomas of the Pineal

    PubMed Central

    Lewis, I.; Baxter, D. W.; Stratford, J. G.

    1963-01-01

    Atypical teratomas of the pineal were studied pathologically and clinically, and five illustrative cases are described. The results of three postmortem examinations are available, while two of the patients are living, one leading a normal life. Pathological verification revealed that two had suprasellar “ectopic” pinealomas. One neoplasm was located in the pineal (collicular) region. The histology of the tumours was identical, consisting of small cells resembling lymphocytes and large cells with prominent nucleoli and mitoses. This feature plus the midline location led to adoption of the term “atypical teratoma”. Patients with collicular pinealomas presented with headache, vomiting, papilledema, Parinaud's syndrome and, rarely, nystagmus retractorius. Diabetes insipidus, visual difficulty and hypopituitarism were characteristic features in those with suprasellar neoplasms. Treatment of collicular pinealoma has consisted of the use of a palliative shunt followed by a course of radiation. Chiasmal decompression and radiation have produced favourable results in patients with suprasellar pinealoma. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5Fig. 6Fig. 7Fig. 8Fig. 9Fig. 10Fig. 11Fig. 12 PMID:20327617

  7. Association of a bovine prion gene haplotype with atypical BSE

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Background: Atypical bovine spongiform encephalopathies (BSEs) are recently recognized prion diseases of cattle. Atypical BSEs are rare; approximately 30 cases have been identified worldwide. We tested prion gene (PRNP) haplotypes for an association with atypical BSE. Methodology/Principal Findin...

  8. Compilation of Spectroscopic Data of Radium (Ra I and Ra II)

    NASA Astrophysics Data System (ADS)

    Dammalapati, U.; Jungmann, K.; Willmann, L.

    2016-03-01

    Energy levels, wavelengths, lifetimes, and hyperfine structure constants for the isotopes of the first and second spectra of radium, Ra I and Ra II, have been compiled. Wavelengths and wavenumbers are tabulated for 226Ra and for other Ra isotopes. Isotope shifts and hyperfine structure constants of even and odd-A isotopes of neutral radium atom and singly ionized radium are included. Experimental lifetimes of the states for both neutral and ionic Ra are also added, where available. The information is beneficial for present and future experiments aimed at different physics motivations using neutral Ra and singly ionized Ra.

  9. Course and treatment of atypical depression.

    PubMed

    Nierenberg, A A; Alpert, J E; Pava, J; Rosenbaum, J F; Fava, M

    1998-01-01

    Atypical depression is the most common form of depression in outpatients, but compared with melancholia, little is known about its comorbidity, course, and treatment. Beyond the well-characterized constellation of symptoms that define atypical depression (mood reactivity, hypersomnia, leaden paralysis, hyperphagia, and rejection sensitivity), specific Axis I and II comorbid conditions may differentiate atypical from other depressed patients. Similarly, age at onset, duration of episodes, frequency of relapses and recurrences, and frequency of complete remission in atypical depression may be different. It has not even been established if atypical depression is a stable subtype or if it is just one of several forms of depression that an individual may express during a lifetime of recurrent depressions. Monoamine oxidase inhibitors (MAOIs) are superior to tricyclic antidepressants (TCAs) for the treatment of atypical depression, but few studies have compared MAOIs to the newer generation of antidepressants (SSRIs, bupropion, venlafaxine, nefazodone, and mirtazapine). Because of the favorable benefit/risk ratio, clinicians tend to use these newer antidepressants for all outpatients, including those with atypical depression, even though the literature is limited. A review and critique of the relevant literature on atypical depression will be presented. PMID:9840192

  10. Higher Education and the Black Atypical Student.

    ERIC Educational Resources Information Center

    Hull, W. Frank, IV

    The black atypical student, defined as that black student who would be excluded from most colleges and universities in America by traditional admission policy, is beginning to find entrance into institutions of higher education. There is no indication reported of these institutions admitting large numbers of black atypical students. In the…

  11. Klebsiella Phage vB_KleM-RaK2 — A Giant Singleton Virus of the Family Myoviridae

    PubMed Central

    Šimoliūnas, Eugenijus; Kaliniene, Laura; Truncaitė, Lidija; Zajančkauskaitė, Aurelija; Staniulis, Juozas; Kaupinis, Algirdas; Ger, Marija; Valius, Mindaugas; Meškys, Rolandas

    2013-01-01

    At 346 kbp in size, the genome of a jumbo bacteriophage vB_KleM-RaK2 (RaK2) is the largest Klebsiella infecting myovirus genome sequenced to date. In total, 272 out of 534 RaK2 ORFs lack detectable database homologues. Based on the similarity to biologically defined proteins and/or MS/MS analysis, 117 of RaK2 ORFs were given a functional annotation, including 28 RaK2 ORFs coding for structural proteins that have no reliable homologues to annotated structural proteins in other organisms. The electron micrographs revealed elaborate spike-like structures on the tail fibers of Rak2, suggesting that this phage is an atypical myovirus. While head and tail proteins of RaK2 are mostly myoviridae-related, the bioinformatics analysis indicate that tail fibers/spikes of this phage are formed from podovirus-like peptides predominantly. Overall, these results provide evidence that bacteriophage RaK2 differs profoundly from previously studied viruses of the Myoviridae family. PMID:23593293

  12. An Atypical Case of Hemoptysis.

    PubMed

    Wang'ondu, Ruth W; Long, Theodore

    2016-03-01

    Hemoptysis, a common sign of diffuse alveolar hemorrhage, can be caused by multiple factors, both infectious and noninfectious. A 45-year-old male with hypertension, obstructive sleep apnea, and stage IV pulmonary sarcoidosis with cardiac involvement, presented with a two-month history of cough and acute nonmassive hemoptysis with hypoxia. A chest CT showed ground glass consolidation and interlobular septal thickening, concerning for diffuse alveolar hemorrhage. Flexible bronchoscopy confirmed diffuse alveolar hemorrhage; microbiological analyses of bronchoalveolar washings did not reveal a causative organism. Mycoplasma pneumoniae-specific IgM in serum studies was consistent with mycoplasma pneumonia as the most likely etiology of this patient's diffuse alveolar hemorrhage and resultant hemoptysis. This report points to the need to consider atypical mycoplasma pneumonia as a possible etiology of hemoptysis in patients with underlying sarcoidosis. PMID:27169298

  13. [Atypical trajectory of gunshot injury].

    PubMed

    Aygün, Mert; Tulay, Cumhur Murat

    2014-11-01

    Gunshot injuries are common medical-legal issues. Atypical tract lines resulting from this type of injuries cause difficulties in diagnosis and treatment. In this paper, a gunshot injury on the right anterior thigh extending to the right hemithorax was presented. A 67-year-old Syrian refugee patient was brought to the emergency service due to gunshot injury. Bullet entrance hole was determined on the right anterior thigh region; however, exit side could not be seen. Bullet was determined on the right thorax at tomography and the patient was taken to operation due to diaphragm rupture and lung parenchymal injury. Other body parts must be examined radiologically for the bullet which cannot be determined at gunshot injury side. PMID:25541926

  14. Atypical parasitic ischiopagus conjoined twins.

    PubMed

    Corona-Rivera, J Román; Corona-Rivera, Enrique; Franco-Topete, Ramón; Acosta-León, Jorge; Aguila-Dueñas, Virginia; Corona-Rivera, Alfredo

    2003-02-01

    Occurrence of asymmetrical or parasitic conjoined twins (CT) is rare, and currently they are classified analogically to the common unions of symmetrical CT. The authors report on an infant with a parasitic third limb attached to the left lateral aspect of the autosite trunk, in whom male gonadal tissue was found histologically. Parasite parts included complete left lower limb, hemipelvis, lumbosacral vertebral column, spinal cord, and one kidney with ureter and adrenal gland. Autosite anomalies comprised a small left diaphragmatic defect, omphalocele, exstrophy of cloaca, and lumbar meningomyelocele. The authors considered this case to be a rare atypical parasitic ischiopagus CT. The differential diagnosis of the type of twining and other entities with caudal duplications is analyzed briefly. PMID:12596123

  15. Typical and atypical AIS. Pathogenesis.

    PubMed

    Dudin, M; Pinchuk, D

    2012-01-01

    AIS hypothesis has the right to recognition, if it explains the transition of "healthy" vertebra column into status of "scoliotic" one. AIS is the most investigated disease in the history of orthopedics, but up the present time there is no clear explanation of some its phenomena: vertebra column mono-form deformation along with its poly etiology character, interrelation of its origin and development and child's growth process etc. The key for authors' view at AIS was scoliosis with non-standard (concave side) rotation. On the bases of its' multifunctional instrumental investigation results (Rtg, EMG, EEG, optical topography, hormonal and neuropeptides trials, thermo-vision methods and other) in comparison with typical AIS was worked out the new hypothesis, part of it is suggested for discussion. In the work under observation is the sequence of appearance of typical and atypical scoliosis symptomatology beginning from the preclinical stage. PMID:22744477

  16. Imaging appearances of atypical hydatid cysts

    PubMed Central

    Malik, Amita; Chandra, Ranjan; Prasad, Rajni; Khanna, Geetika; Thukral, Brij B

    2016-01-01

    Hydatid disease continues to be a significant health problem in many parts of the world. It can occur in any part of the body, but liver is the commonest site of involvement. The disease may remain asymptomatic for years. Symptoms occur due to compression of local structures or complications like rupture and infection. The diagnosis is clear when typical radiological appearance is observed at the common sites of involvement. Complications give rise to atypical appearances. These coupled with unusual localizations pose diagnostic difficulty. The aim of this pictorial essay is to demonstrate the atypical manifestations of hydatid cysts – atypical either due to complications or the unusual site. PMID:27081221

  17. Functional Analysis of the Phycomyces carRA Gene Encoding the Enzymes Phytoene Synthase and Lycopene Cyclase

    PubMed Central

    Sanz, Catalina; Velayos, Antonio; Álvarez, María Isabel; Benito, Ernesto P.; Eslava, Arturo P.

    2011-01-01

    Phycomyces carRA gene encodes a protein with two domains. Domain R is characterized by red carR mutants that accumulate lycopene. Domain A is characterized by white carA mutants that do not accumulate significant amounts of carotenoids. The carRA-encoded protein was identified as the lycopene cyclase and phytoene synthase enzyme by sequence homology with other proteins. However, no direct data showing the function of this protein have been reported so far. Different Mucor circinelloides mutants altered at the phytoene synthase, the lycopene cyclase or both activities were transformed with the Phycomyces carRA gene. Fully transcribed carRA mRNA molecules were detected by Northern assays in the transformants and the correct processing of the carRA messenger was verified by RT-PCR. These results showed that Phycomyces carRA gene was correctly expressed in Mucor. Carotenoids analysis in these transformants showed the presence of ß-carotene, absent in the untransformed strains, providing functional evidence that the Phycomyces carRA gene complements the M. circinelloides mutations. Co-transformation of the carRA cDNA in E. coli with different combinations of the carotenoid structural genes from Erwinia uredovora was also performed. Newly formed carotenoids were accumulated showing that the Phycomyces CarRA protein does contain lycopene cyclase and phytoene synthase activities. The heterologous expression of the carRA gene and the functional complementation of the mentioned activities are not very efficient in E. coli. However, the simultaneous presence of both carRA and carB gene products from Phycomyces increases the efficiency of these enzymes, presumably due to an interaction mechanism. PMID:21858003

  18. Preparation of (228)Ra standard solution.

    PubMed

    Havelka, Miroslav

    2016-03-01

    For the preparation of a standard solution of (228)Ra, (228)Ra was isolated from (232)Th salt. Two simple methods were developed for Th-Ra separation. Both are based on a very good solubility of thorium nitrate in organic solvents. The first one used Ra co-precipitation with Pb in the form of Pb(NO3)2 from acetic acid solution. The second method was based on solvent extraction, remaining Th in the organic phase, while Ra was concentrated in the aqueous phase. The activity of (228)Ra (up to 20kBq) in the standard solution was related to the (232)Th standard by means of gamma ray spectrometry measurement. The obtained uncertainty was less than 0.7% (k=1). The standard solution was free of (232)Th and contained the carrier in the usual concentration (1gL(-1) BaCl2, 10gL(-1) HCl). PMID:26651171

  19. Interstellar A-type methanol masers

    NASA Astrophysics Data System (ADS)

    Zeng, Q.; Lou, G. F.

    1990-02-01

    The formation conditions for A-type methanol masers are discussed. The correlation between A-type masers and external radiation fields is determined, with emphasis on the energy levels of A-type methanol and brightness temperature. Radiative transfer equations and statistical equilibrium are solved using a large velocity gradient model and the escape probability model. It is demonstrated that the 9(2)-10(1)A+ emission in W3(OH) and 7(0)-6(1)A in SgrB2 are masers, as discovered previously. The formation of the first type of masers requires pumping from an external radiation field, while the second type might be excited in the absence of an external radiation field. It is also pointed out that according to calculations there are A-type maser series similar to E-type methanol maser series of J2-J1E.

  20. Surgical Options for Atypical Facial Pain Syndromes.

    PubMed

    Rahimpour, Shervin; Lad, Shivanand P

    2016-07-01

    Atypical neuropathic facial pain is a syndrome of intractable and unremitting facial pain that is secondary to nociceptive signaling in the trigeminal system. These syndromes are often recalcitrant to pharmacotherapy and other common interventions, including microvascular decompression and percutaneous procedures. Herein, the authors present two other viable approaches (nucleus caudalis dorsal root entry zone lesioning and motor cortex stimulation), their indications, and finally a possible treatment algorithm to consider when assessing patients with atypical facial pain. PMID:27325003

  1. Atypical antipsychotics: sedation versus efficacy.

    PubMed

    Kane, John M; Sharif, Zafar A

    2008-01-01

    Many patients with schizophrenia or bipolar disorder experience disturbances in their sleep-wake cycle, which may be a result of the disorder itself, of pharmacotherapy, or of a comorbid sleep disorder. These sleep disruptions can seriously impair patients' functioning as well as their quality of life. Therefore, accurate assessment of sleep problems is essential to appropriately treat patients and promote symptomatic remission. Sedating antipsychotics may ameliorate sleep disturbances, as well as agitation or other behavioral emergencies; however, these agents may also sedate patients to the point of dissatisfaction with the medication and/or impaired functioning, which may, in turn, increase treatment noncompliance and nonadherence. Using short-term adjunctive medications, such as benzo-diazepines or hypnotic agents, with a nonsedating antipsychotic to alleviate sleep disturbances is a reasonable treatment option for patients with schizophrenia or bipolar disorder. Overall, the pharma-cokinetics and pharmacodynamics of atypical antipsychotics are important factors to consider in the risk-benefit analysis, as are dosing strategies and individual patient factors, and clinicians must decide which agents are most appropriate for which patients. PMID:18484805

  2. Interventional trials in atypical parkinsonism.

    PubMed

    Eschlböck, S; Krismer, F; Wenning, G K

    2016-01-01

    Atypical parkinson disorders (APD) are rapidly progressive neurodegenerative diseases with a variable clinical presentation that may even mimic Parkinson's disease. Multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are commonly summarized under this umbrella term. Significant developments in research have expanded knowledge and have broadened available symptomatic treatments, particularly for the treatment of neurogenic orthostatic hypotension. Nonetheless, symptomatic support still remains limited in all of these disorders. Currently, there exists no effective treatment to delay disease progression and disease-modifying trials have failed to provide coherent and convincing results. Recent trials of rasagiline (in MSA), rifampicin (in MSA), tideglusib (in PSP) and davunetide (in PSP) reported negative results. Nevertheless, large cohorts of patients were recruited for interventional studies in the last few years which improved our understanding of trial methodology in APDs immensely. In addition, remarkable progress in basic research has been reported recently and will provide a solid foundation for future therapeutic trials. In this review, we will summarize published randomized, placebo-controlled clinical trials (RCTs) in APDs. Additionally, the design of ongoing and unpublished interventions will be presented. PMID:26421389

  3. Biopsychosocial Aspects of Atypical Odontalgia

    PubMed Central

    Ciaramella, A.; Paroli, M.; Lonia, L.; Bosco, M.; Poli, P.

    2013-01-01

    Background. A few studies have found somatosensory abnormalities in atypical odontalgia (AO) patients. The aim of the study is to explore the presence of specific abnormalities in facial pain patients that can be considered as psychophysical factors predisposing to AO. Materials and Methods. The AO subjects (n = 18) have been compared to pain-free (n = 14), trigeminal neuralgia (n = 16), migraine (n = 17), and temporomandibular disorder (n = 14). The neurometer current perception threshold (CPT) was used to investigate somatosensory perception. Structured clinical interviews based on the DSM-IV axis I and DSM III-R axis II criteria for psychiatric disorders and self-assessment questionnaires were used to evaluate psychopathology and aggressive behavior among subjects. Results. Subjects with AO showed a lower Aβ, Aδ, and C trigeminal fiber pain perception threshold when compared to a pain-free control group. Resentment was determined to be inversely related to Aβ (rho: 0.62, P < 0.05), Aδ (rho: 0.53, P < 0.05) and C fibers (rho: 0.54, P < 0.05), and depression was inversely related with C fiber (rho: 0.52, P < 0.05) perception threshold only in AO subjects. Conclusion. High levels of depression and resentment can be considered predictive psychophysical factors for the development of AO after dental extraction. PMID:24959561

  4. Biopsychosocial aspects of atypical odontalgia.

    PubMed

    Ciaramella, A; Paroli, M; Lonia, L; Bosco, M; Poli, P

    2013-01-01

    Background. A few studies have found somatosensory abnormalities in atypical odontalgia (AO) patients. The aim of the study is to explore the presence of specific abnormalities in facial pain patients that can be considered as psychophysical factors predisposing to AO. Materials and Methods. The AO subjects (n = 18) have been compared to pain-free (n = 14), trigeminal neuralgia (n = 16), migraine (n = 17), and temporomandibular disorder (n = 14). The neurometer current perception threshold (CPT) was used to investigate somatosensory perception. Structured clinical interviews based on the DSM-IV axis I and DSM III-R axis II criteria for psychiatric disorders and self-assessment questionnaires were used to evaluate psychopathology and aggressive behavior among subjects. Results. Subjects with AO showed a lower A β , A δ , and C trigeminal fiber pain perception threshold when compared to a pain-free control group. Resentment was determined to be inversely related to A β (rho: 0.62, P < 0.05), A δ (rho: 0.53, P < 0.05) and C fibers (rho: 0.54, P < 0.05), and depression was inversely related with C fiber (rho: 0.52, P < 0.05) perception threshold only in AO subjects. Conclusion. High levels of depression and resentment can be considered predictive psychophysical factors for the development of AO after dental extraction. PMID:24959561

  5. Saccular cyst with atypical presentation

    PubMed Central

    Zamfir-Chiru-Anton, A; Gheorghe, DC

    2016-01-01

    Respiratory obstruction and stridor in infants and children are not uncommon. A rare cause of these sometimes life-threatening symptoms is the congenital saccular cyst. Objectives: We present the case of a 5-year-old girl with a cervical tumor, which appeared after a laryngeal endoscopic surgery of a saccular cyst with two relapses and a particular local evolution of its recurrence through the cricothyroid membrane. Material and method: The patient data has been reviewed over the entire follow-up period and a thorough an analysis of her investigations and surgery was performed. Results: The unusual evolution of this case was marked by an atypical exteriorization – not found in the published literature. The surgical approach was external, by paramedian thyrotomy, with no further long-term recurrence. Conclusions: An accurate diagnosis of saccular cysts can be made with the help of medical history, by an endoscopic visualization of the lesion and by the CT-scan imaging of the cervical region. Sometimes, saccular cysts can extend beyond laryngeal limits, determining fluid-filled tumors in the cervical region. PMID:27453755

  6. Non-destructive determination of 224Ra, 226Ra and 228Ra concentrations in drinking water by gamma spectroscopy.

    PubMed

    Parekh, Pravin; Haines, Douglas; Bari, Abdul; Torres, Miguel

    2003-11-01

    The U.S. Environmental Protection Agency mandates that drinking water showing gross alpha-activity greater than 0.19 Bq L(-1) should be analyzed for radium, a known human carcinogen. The recommended testing methods are intricate and laborious. The method reported in this paper is a direct, non-destructive gamma-spectroscopic method for the determination of 224Ra, 226Ra, and 228Ra, the three radium isotopes of environmental concern in drinking water. Large-volume Marinelli beakers (4.1-L capacity), especially designed for measuring radioactive gases, in conjunction with a low-background, high-efficiency (131%) germanium detector were used in this work. It was first established that radon, the gaseous decay product of radium, and its progeny are quantitatively retained in this Marinelli beaker. The 224Ra, 226Ra, and 228Ra activity concentrations are determined from the equilibrium activities of their progeny: 212Pb, 214Pb (214Bi), and 228Ac; and the gamma-lines used in the analysis are 238.6, 351.9 (and 609.2), and 911.2 keV, respectively. The 224Ra activity is determined from the first 1,000-min measurement performed after expulsion of radon from the sample. The 226Ra activity is determined from the second, 2,400-min measurement, made 3 to 5 d later, and the 228Ra activity is determined from either the first or the second measurement, depending on its concentration level. The method's minimum detectable activities are 0.017 Bq L(-1), 0.020 Bq L(-1), and 0.027 Bq L(-1) for 224Ra, 226Ra, and 228Ra, respectively, when measured under radioactive equilibrium. These limits are well within the National Primary Drinking Water Regulations required limit of 0.037 Bq L(-1) for 226Ra and for 228Ra. The precision and accuracy of the method, evaluated using the U.S. Environmental Protection Agency and the Environmental Resource Associates' quality control samples, were found to be within acceptable limits. PMID:14571995

  7. Isolation and characterization of atypical Riemerella columbina strains from pigeons and their differentiation from Riemerella anatipestifer.

    PubMed

    Rubbenstroth, Dennis; Hotzel, Helmut; Knobloch, Johannes; Teske, Lydia; Rautenschlein, Silke; Ryll, Martin

    2011-01-10

    Riemerella columbina (RC) and Riemerella anatipestifer (RA) belong to the genus Riemerella within the family Flavobacteriaceae. While RA is a well-described pathogen of waterfowl and other avian species, only little is known about RC. Previous work reporting the isolation of RC from internal organs of clinically diseased pigeons suggested a potential pathogenic role in this avian species. In this study we examined pharyngeal swabs collected from pigeons and found RC to be widely distributed also among healthy birds. Further characterization of 81 RC-isolates revealed several atypical strains, which differed from all previously described RC-isolates by the lack of aesculin-hydrolysis activity (17 isolates) or by expression of yellow or orange pigmentation (6 isolates). Sequence analysis of the 16S rRNA and outer membrane protein A (ompA) gene supported the affiliation of these strains to the species RC. Aesculin-hydrolysis negative isolates were found to be biochemically indistinguishable from RA. We demonstrated that bacterial fingerprinting using matrix assisted laser desorption/ionisation-time of flight mass spectrometry (MALDI-TOF MS) analysis is useful for the identification and differentiation of RC and RA. PMID:20615634

  8. Phosphoproteome and Transcriptome of RA-Responsive and RA-Resistant Breast Cancer Cell Lines

    PubMed Central

    Carrier, Marilyn; Joint, Mathilde; Lutzing, Régis; Page, Adeline; Rochette-Egly, Cécile

    2016-01-01

    Retinoic acid (RA), the main active vitamin A metabolite, controls multiple biological processes such as cell proliferation and differentiation through genomic programs and kinase cascades activation. Due to these properties, RA has proven anti-cancer capacity. Several breast cancer cells respond to the antiproliferative effects of RA, while others are RA-resistant. However, the overall signaling and transcriptional pathways that are altered in such cells have not been elucidated. Here, in a large-scale analysis of the phosphoproteins and in a genome-wide analysis of the RA-regulated genes, we compared two human breast cancer cell lines, a RA-responsive one, the MCF7 cell line, and a RA-resistant one, the BT474 cell line, which depicts several alterations of the “kinome”. Using high-resolution nano-LC-LTQ-Orbitrap mass spectrometry associated to phosphopeptide enrichment, we found that several proteins involved in signaling and in transcription, are differentially phosphorylated before and after RA addition. The paradigm of these proteins is the RA receptor α (RARα), which was phosphorylated in MCF7 cells but not in BT474 cells after RA addition. The panel of the RA-regulated genes was also different. Overall our results indicate that RA resistance might correlate with the deregulation of the phosphoproteome with consequences on gene expression. PMID:27362937

  9. Activity in A-type Stars

    NASA Astrophysics Data System (ADS)

    Balona, L. A.

    2013-12-01

    Kepler photometry shows that most A-type stars have low frequency variations which can be understood in terms of rotational modulation. Indeed, the distribution of equatorial velocities derived from the photometric periods agrees with the distribution of equatorial velocities of A-type stars in the general field. The amplitude of the rotational frequency varies by 20-30 percent as might be expected of star spots. From the light amplitudes we estimate that most spots are considerably larger than typical sunspots but generally smaller than the largest sunspots. The rotation peaks in the periodograms of a significant fraction of A-type stars have a peculiar structure which is not understood. Although peaks corresponding to the rotation frequency can be identified in many δ Scuti stars, the low frequency peaks in these stars are too numerous to be caused by rotational modulation. It thus appears that while the variability of non-pulsating A stars can be explained by rotation, the low-frequency variability in A-type δ Sct stars requires a new pulsation mechanism. We also find several γ Dor stars much hotter than the theoretical hot edge of the instability strip. We find 13 new A-type flare stars, which means that about 1.5 percent of A stars flare. Less dramatic flares may be common in all A-type stars. We show that these superflares cannot be attributed to normal flares on a cool companion. We conclude that A-type stars are active and, like cooler stars, have starspots and flares. Surprisingly, there does not seem to be a drop in activity as the granulation boundary is crossed.

  10. Cardiovascular risk factor management in patients with RA compared to matched non-RA patients

    PubMed Central

    Cawston, Helene; Bourhis, Francois; Al, Maiwenn; Rutten-van Mölken, Maureen P. M. H.; Liao, Katherine P.; Solomon, Daniel H.

    2016-01-01

    Objective. RA is associated with a 50–60% increase in risk of cardiovascular (CV) death. This study aimed to compare management of CV risk factors in RA and matched non-RA patients. Methods. A retrospective cohort study was conducted using UK clinical practice data. Patients presenting with an incident RA diagnosis were matched 1:4 to non-RA patients based on a propensity score for RA, entry year, CV risk category and treatment received at index date (date of RA diagnosis). Patients tested and treated for CV risk factors as well as those attaining CV risk factor management goals were evaluated in both groups. Results. Between 1987 and 2010, 24 859 RA patients were identified and matched to 87 304 non-RA patients. At index date, groups had similar baseline characteristics. Annual blood pressure, lipids and diabetes-related testing were similar in both groups, although CRP and ESR were higher in RA patients at diagnosis and decreased over time. RA patients prescribed antihypertensives increased from 38.2% at diagnosis to 45.7% at 5 years, from 14.0 to 20.6% for lipid-lowering treatments and from 5.1 to 6.4% for antidiabetics. Similar treatment percentages were observed in non-RA patients, although slightly lower for antihypertensives. Modest (2%) but significantly lower attainment of lipid and diabetes goals at 1 year was observed in RA patients. Conclusion. There were no differences between groups in the frequency of testing and treatment of CV risk factors. Higher CV risk in RA patients seems unlikely to be driven by differences in traditional CV risk factor management. PMID:26705329

  11. Nuclear structure of {sup 231}Ra

    SciTech Connect

    Boutami, R.; Fraile, L. M.; Borge, M. J. G.; Lopez-Jimenez, M. J.; Teijeiro, A. G.; Aas, A. J.; Hageboe, E.; Fogelberg, B.; Mach, H.; Garcia-Raffi, L. M.; Martinez, T.; Rubio, B.; Tain, J. L.; Grant, I. S.; Gulda, K.; Kurcewicz, W.; Loevhoeiden, G.; Tengblad, O.; Thorsteinsen, T. F.

    1999-11-16

    The study of the upper border of the octupole deformation region near A=225, where the octupole deformation vanishes in the presence of a well developed quadrupole field, is of great relevance in order to understand the interplay of octupole and quadrupole collectivities. Within the IS322 collaboration at CERN we carry out a systematic investigation of the heavy Fr-Th nuclei that presently includes {sup 227}Fr, {sup 227,228,229}Ra, {sup 229}Ac and {sup 229,231}Th. The heaviest Ra isotope we have studied so far and in which the fast timing {beta}{gamma}{gamma}(t) method has been applied is {sup 231}Ra.

  12. Nuclear Structure of {sup 231}Ra

    SciTech Connect

    Boutami, R.; Fraile, L.M.; Borge, M.J.G.; Aas, A.J.; Fogelberg, B.; Garcia-Raffi, L.M.; Grant, I.S.; Gulda, K.; Hagebo, E.; Kurcewicz, W.; Lopez-Jimenez, M.J.; Lovhoiden, G.; Mach, H.; Martinez, T.; Rubio, B.; Tain, J.L.; Teijeiro, A.G.; Tengblad, O.; Thorsteinsen, T.F.

    1999-12-31

    The study of the upper border of the octupole deformation region near A=225, where the octupole deformation vanishes in the presence of a well developed quadrupole field, is of great relevance in order to understand the interplay of octupole and quadrupole collectivities. Within the IS322 collaboration at CERN we carry out a systematic investigation of the heavy Fr - Th nuclei that presently includes {sup 227}Fr, {sup 227,228,229}Ra, {sup 229}Ac and {sup 229,231}Th. The heaviest Ra isotope we have studied so far and in which the fast timing {beta}{gamma}{gamma}(t) method has been applied is {sup 231}Ra.

  13. Measuring 226Ra/228Ra in Oceanic Lavas by MC-ICPMS

    NASA Astrophysics Data System (ADS)

    Standish, J. J.; Sims, K.; Ball, L.; Blusztajn, J.

    2007-12-01

    238U-230Th-226Ra disequilibrium in volcanic rocks provides an important and unique tool to evaluate timescales of recent magmatic processes. Determination of 230Th-226Ra disequilibria requires measurement of U and Th isotopes and concentrations as well as measurement of 226Ra. While measurement of U and Th by ICPMS is now well established, few published studies documenting 226Ra measurement via ICPMS exist. Using 228Ra as an isotope spike we have investigated two ion-counting methods; a 'peak-hopping' routine, where 226Ra and 228Ra are measured in sequence on the central discrete dynode ETP secondary electron multiplier (SEM), and simultaneous measurement of 226Ra and 228Ra on two multiple ion-counter system (MICS) channeltron type detectors mounted on the low end of the collector block. Here we present 226Ra measurement by isotope dilution using the Thermo Fisher NEPTUNE MC-ICPMS. Analysis of external rock standards TML and AThO along with mid-ocean ridge basalt (MORB) and ocean island basalt (OIB) samples show three issues that need to be considered when making precise and accurate Ra measurements: 1) mass bias, 2) background, and 3) relative efficiencies of the detectors when measuring in MICS mode. Due to the absence of an established 226Ra/228Ra standard, we have used U reference material NBL-112A to monitor mass bias. Although Ball et. al., (in press) have shown that U does not serve as an adequate proxy for Th (and thus not likely for Ra either), measurements of rock standards TML and AThO are repeatedly in equilibrium within the uncertainty of the measurements (where total uncertainty includes propagation of the uncertainty in the 226Ra standard used for calibrating the 228Ra spike). For this application, U is an adequate proxy for Ra mass bias at the 1% uncertainly level. The more important issue is the background correction. Because of the extensive chemistry required to separate and purify Ra (typically fg/g level in volcanic rocks), we observe large

  14. Atypical muscarinic allosteric modulation: cooperativity between modulators and their atypical binding topology in muscarinic M2 and M2/M5 chimeric receptors.

    PubMed

    Tränkle, Christian; Dittmann, Andreas; Schulz, Uwe; Weyand, Oliver; Buller, Stefan; Jöhren, Kirstin; Heller, Eberhard; Birdsall, Nigel J M; Holzgrabe, Ulrike; Ellis, John; Höltje, Hans Dieter; Mohr, Klaus

    2005-12-01

    The binding and function of muscarinic acetylcholine receptors can be modulated allosterically. Some allosteric muscarinic ligands are "atypical", having steep concentration-effect curves and not interacting competitively with "typical" allosteric modulators. For atypical agents, a second allosteric site has been proposed. Different approaches have been used to gain further insight into the interaction with M2 receptors of two atypical agents, tacrine and the bispyridinium compound 4,4'-bis-[(2,6-dichloro-benzyloxy-imino)-methyl]-1,1'-propane-1,3-diyl-bispyridinium dibromide (Duo3). Interaction studies, using radioligand binding assays and the allosteric ligands obidoxime, Mg2+, and the new tool hexamethonium to antagonize the allosteric actions of the atypical ligands, showed different modes of interaction for tacrine and Duo3 at M2 receptors. A negatively cooperative interaction was observed between hexamethonium and tacrine (but not Duo3). A tacrine dimer that exhibited increased allosteric potency relative to tacrine but behaved like a typical allosteric modulator was competitively inhibited by hexamethonium. M2/M5-receptor mutants revealed a dependence of tacrine and Duo3 affinity on different receptor epitopes. This was confirmed by docking simulations using a three-dimensional model of the M2 receptor. These showed that the allosteric site could accommodate two molecules of tacrine simultaneously but only one molecule of Duo3, which binds in different mode from typical allosteric agents. Therefore, the atypical actions of tacrine and Duo3 involve different modes of receptor interaction, but their sites of attachment seem to be the "common" allosteric binding domain at the entrance to the orthosteric ligand binding pocket of the M2-receptor. Additional complex behavior may be rationalized by allosteric interactions transmitted within a receptor dimer. PMID:16157694

  15. Atypical arthritis revisited: Acute rheumatic fever

    PubMed Central

    Shankar, Binoy; Bhutia, Euden; Kumar, Dinesh

    2016-01-01

    A 13-year-old boy presented with vague musculoskeletal pain and involvement of multiple small and large joints along with axial skeleton for the last 3 years, poorly responsive to aspirin. However, on account of presence of carditis and fulfilment of Jones criteria, a diagnosis of acute rheumatic fever (ARF) with atypical arthritis was made. We report this case to break the myth and sensitize pediatricians and rheumatologists to keep the possibility of atypical articular presentations, as in our case, in patients with ARF and prevent delayed diagnosis and treatment. PMID:27212853

  16. Association between atypical parathyroid adenoma and neurofibromatosis.

    PubMed

    Favere, Aline Mesquita Ferreira de; Tsukumo, Daniela Miti; Matos, Patrícia Sabino de; Santos, Sérgio Luiz Marques dos; Lalli, Cristina Alba

    2015-10-01

    Primary hyperparathyroidism is a disease characterized by excessive production of parathyroid hormone (PTH), which is due to a parathyroid adenoma in 85% of cases. An atypical parathyroid adenoma, with some histopathological features of parathyroid carcinoma, may be found in some of the cases, although it may not fulfill all the criteria for this diagnosis. Neurofibromatosis type 1 (NF1) is an autosomal dominant systemic disease that may be associated with hyperparathyroidism. We report here the rare combination of a patient with NF1 and clinical manifestations of hyperparathyroidism due to an atypical parathyroid adenoma. PMID:26421674

  17. Understanding Rheumatoid Arthritis (RA): Treatment and Causes

    MedlinePlus

    ... this page please turn JavaScript on. Feature: Understanding Rheumatoid Arthritis (RA) Treatment and Causes Past Issues / Summer 2014 Table of Contents How Is Rheumatoid Arthritis Treated? Doctors have many ways to treat this ...

  18. SoRa first flight. Summer 2009

    NASA Astrophysics Data System (ADS)

    Pirrotta, S.; Flamini, E.

    The SoRa (Sounding Radar) experiment was successfully launched from Longyearbyen (Svalbard, Norway) during the summer 2009 campaign managed by the Italian/Norwegian "Nobile Amundsen / Stratospheric Balloon Centre" (NA/SBC). SoRa is part of the Italian Space Agency (ASI) programs for Long Duration Balloon Flights. Carried by the biggest balloon (800.000 m3) ever launched in polar regions, SoRa main experiment and its three piggyback payloads (DUSTER, ISA and SIDERALE) performed a nominal flight of almost 4 days over the North Sea and Greenland, until the separation, landing and recovery in Baffin Island (Canada). Despite the final destructive event that compromise the scientific main goal of SoRa, the 2009 ASI balloon campaign can be considered an important milestone, because of the obtained scientific and technical results but also for the lesson learned by the science, engineering and managerial teams looking at the future ASI scientific balloon-born activities.

  19. Distribution and flux of /sup 226/Ra and /sup 228/Ra in the Amazon River estuary

    SciTech Connect

    Key, R.M.; Sarmiento, J.L.; Stallard, R.F.; Moore, W.S.

    1985-07-20

    Measurements of /sup 226/Ra and /sup 228/Ra in the Amazon River estuary show that desorption from riverborne suspended particulate matter in the estuary increases the riverine flux of both isotopes to the ocean by a factor of approximately 5 over the flux attributable to radium dissolved in the river water alone. The total Amazon flux supplies approximately 0.20% of the /sup 226/Ra and approximately 2.6% of the /sup 228/Ra standing crops in the near-surface Atlantic (0-200 m). Diffusive flux from estuarine and shelf sediments and desorption from resuspended sediments in the region of the estuary approximately double the estuarine /sup 226/Ra concentration and quadruple the estuarine /sup 228/Ra concentration above that caused by the dissolved and desorbed river components alone.

  20. Determination of 226Ra and 224Ra in drinking waters by liquid scintillation counting.

    PubMed

    Manjón, G; Vioque, I; Moreno, H; García-Tenorio, R; García-León, M

    1997-04-01

    A method for the determination of Ra-isotopes in water samples has been developed. Ra is coprecipitated with Ba as sulphate. The precipitate is then dissolved with EDTA and counted with a liquid scintillation system after mixing with a scintillation cocktail. The study of the temporal evolution of the separated activity gives the isotopic composition of the sample, i.e. the 224Ra and 226Ra contribution to the total activity. The method has been applied to some Spanish drinking waters. PMID:9106993

  1. Atypical Alpha Asymmetry in Adults with ADHD

    ERIC Educational Resources Information Center

    Hale, T. Sigi; Smalley, Susan L.; Hanada, Grant; Macion, James; McCracken, James T.; McGough, James J.; Loo, Sandra K.

    2009-01-01

    Introduction: A growing body of literature suggests atypical cerebral asymmetry and interhemispheric interaction in ADHD. A common means of assessing lateralized brain function in clinical populations has been to examine the relative proportion of EEG alpha activity (8-12 Hz) in each hemisphere (i.e., alpha asymmetry). Increased rightward alpha…

  2. Atypical Pyoderma Gangrenosum Mimicking an Infectious Process

    PubMed Central

    To, Derek; Wong, Aaron; Montessori, Valentina

    2014-01-01

    We present a patient with atypical pyoderma gangrenosum (APG), which involved the patient's arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics. PMID:25024856

  3. Atypical pyoderma gangrenosum mimicking an infectious process.

    PubMed

    To, Derek; Wong, Aaron; Montessori, Valentina

    2014-01-01

    We present a patient with atypical pyoderma gangrenosum (APG), which involved the patient's arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics. PMID:25024856

  4. Infant Perception of Atypical Speech Signals

    ERIC Educational Resources Information Center

    Vouloumanos, Athena; Gelfand, Hanna M.

    2013-01-01

    The ability to decode atypical and degraded speech signals as intelligible is a hallmark of speech perception. Human adults can perceive sounds as speech even when they are generated by a variety of nonhuman sources including computers and parrots. We examined how infants perceive the speech-like vocalizations of a parrot. Further, we examined how…

  5. Identification of atypical scrapie in Canadian sheep

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Scrapie, a transmissible spongiform encephalopathy of sheep and goats, exists in most small ruminant producing countries of the world. An atypical form of this disease, originally termed Nor98, was discovered in large abattoir surveillance of clinically normal, predominantly older sheep and rarely ...

  6. Atypical and Typical Antipsychotics in the Schools

    ERIC Educational Resources Information Center

    Noggle, Chad A.; Dean, Raymond S.

    2009-01-01

    The use of antipsychotic medications within the school-age population is rapidly increasing. Although typical antipsychotics may be used in rare cases, this influx is largely secondary to the availability of the atypical antipsychotics. Reduction of possible adverse effects and increased efficacy represent the primary basis for the atypical…

  7. Human Poisoning Through Atypical Routes of Exposure.

    PubMed

    Behal, Niharika; Wong, Alan; Mantara, Ruzly; Cantrell, F Lee

    2016-02-01

    There are over 2 million human exposure cases reported to United States poison centers annually. Much of the data involves exposure through ingestion, dermal contact, inhalation, ocular, or parenteral routes. There is limited data characterizing exposure via atypical routes. We conducted a retrospective review of the California Poison Control System Database for a 24-month period from January 2012 to December 2013 for poison exposure that occurred through the otic, vaginal, or rectal route. There were a total of 634 cases involving single-route and single-substance atypical poison exposure. There were 287 (45%) cases of otic exposure, 190 (30.0%) cases of vaginal exposure, and 157 (25%) cases of rectal exposure. Five hundred forty (85%) of the cases were unintentional. Gasoline exposure through the otic route occurred in 83 (13.1%) cases, followed by hydrogen peroxide (4.7%), acetaminophen (3.8%), and miconazole (2.7%). Adverse effects occurred in 336 (53%) cases. No deaths were reported. The most common treatment was observation only, occurring in 396 (62.4%) cases. The majority of the cases did not warrant hospital evaluation (73.5%). This is the first retrospective characterization study of atypical routes of poison exposure. These results may provide education to providers and the public regarding risks of exposure to substances through atypical routes. PMID:26250476

  8. Atypical Gifted Learners and Their Characteristics.

    ERIC Educational Resources Information Center

    Diket, Read M., Ed.; Abel, Trudy, Ed.

    This collection of 12 handouts focuses on different categories of atypical gifted learners and their characteristics. The handouts are generally two pages long and present a summary of the literature on the topic, some practical teaching suggestions, and references. The handouts include: (1) "Socioeconomically Disadvantaged Gifted Students" (Pam…

  9. Atypical Neural Self-Representation in Autism

    ERIC Educational Resources Information Center

    Lombardo, Michael V.; Chakrabarti, Bhismadev; Bullmore, Edward T.; Sadek, Susan A.; Pasco, Greg; Wheelwright, Sally J.; Suckling, John; Baron-Cohen, Simon

    2010-01-01

    The "self" is a complex multidimensional construct deeply embedded and in many ways defined by our relations with the social world. Individuals with autism are impaired in both self-referential and other-referential social cognitive processing. Atypical neural representation of the self may be a key to understanding the nature of such impairments.…

  10. Atypical Ligon Lintless-2 Phenotype in Cotton

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The mutant Li2 is reported to be a dominant single gene mutation in cotton, Gossypium hirsutum L. It has normal vegetative phenotypic morphology and the phenotype of the seed cotton is reported to be fuzzy seed with short fibers. The objective of this research was to report on atypical phenotypes ob...

  11. Observing Behavior and Atypically Restricted Stimulus Control

    ERIC Educational Resources Information Center

    Dube, William V.; Dickson, Chata A.; Balsamo, Lyn M.; O'Donnell, Kristin Lombard; Tomanari, Gerson Y.; Farren, Kevin M.; Wheeler, Emily E.; McIlvane, William J.

    2010-01-01

    Restricted stimulus control refers to discrimination learning with atypical limitations in the range of controlling stimuli or stimulus features. In the study reported here, 4 normally capable individuals and 10 individuals with intellectual disabilities (ID) performed two-sample delayed matching to sample. Sample-stimulus observing was recorded…

  12. Cohort study of atypical pressure ulcers development.

    PubMed

    Jaul, Efraim

    2014-12-01

    Atypical pressure ulcers (APU) are distinguished from common pressure ulcers (PU) with both unusual location and different aetiology. The occurrence and attempts to characterise APU remain unrecognised. The purpose of this cohort study was to analyse the occurrence of atypical location and the circumstances of the causation, and draw attention to the prevention and treatment by a multidisciplinary team. The cohort study spanned three and a half years totalling 174 patients. The unit incorporates two weekly combined staff meetings. One concentrates on wound assessment with treatment decisions made by the physician and nurse, and the other, a multidisciplinary team reviewing all patients and coordinating treatment. The main finding of this study identified APU occurrence rate of 21% within acquired PU over a three and a half year period. Severe spasticity constituted the largest group in this study and the most difficult to cure wounds, located in medial aspects of knees, elbows and palms. Medical devices caused the second largest occurrence of atypical wounds, located in the nape of the neck, penis and nostrils. Bony deformities were the third recognisable atypical wound group located in shoulder blades and upper spine. These three categories are definable and time observable. APU are important to be recognisable, and can be healed as well as being prevented. The prominent role of the multidisciplinary team is primary in identification, prevention and treatment. PMID:23374746

  13. Atypical Visuomotor Performance in Children with PDD

    ERIC Educational Resources Information Center

    Schlooz, Wim A. J. M.; Hulstijn, Wouter

    2012-01-01

    Children with autism spectrum disorders (ASD) frequently encounter difficulties in visuomotor tasks, which are possibly caused by atypical visuoperceptual processing. This was tested in children (aged 9-12 years) with pervasive developmental disorder (PDD; including PDD-NOS and Asperger syndrome), and two same-age control groups (Tourette syndrome…

  14. 228Ra and 226Ra Profiles from the Northern South China Sea

    NASA Astrophysics Data System (ADS)

    Lin, H.; Chung, Y.; Lin, C.

    2005-05-01

    We previously reported the distributions of 228Ra and 226Ra in the northern South China Sea (SCS) which showed that both nuclides in surface waters were much higher than those in the open oceans because the SCS was enclosed mostly by landmasses which are known as sources of these nuclides. Large temporal and spectial variations were also observed probably due to the monsoons and intrusion of the Kuroshio Current. During a recent cruise conducted in the northern SCS in February, 2004, three vertical 228Ra profiles were measured by gamma spectrometry on the Ra isotopes which were concentrated first by the MnO2-impregnated acrylic fiber and then acid-washed as sample solution for counting. The two deep water 228Ra profiles are remarkably similar, showing high values in the surface layer and fairly uniform at about 10 to 13 dpm/100L below 200m depth but with a clear increase toward the bottom due to input from the underlying sediments. The shallow water profile on the shelf shows higher 228Ra values due to both vertical and horizontal mixing of the shelf water with additional source from the shore zone. Additional 228Ra profiles measured on samples from earlier cruises show that the deep water values may differ significantly (up to 5 dpm/100L) at the same location in different seasons or cruises. The associated 226Ra profiles are also variable but quite comparable to those in the northwest Pacific in deep water. 226Ra activities in the shallow water (less than 1000m depth) are higher in the SCS than in the open oceans. The 228Ra/226Ra activity ratios vary mostly from about 0.3 to 0.5 in the deep water. These values are much higher than those in the open oceans which are generally less than 0.1.

  15. Relationship between atypical depression and social anxiety disorder.

    PubMed

    Koyuncu, Ahmet; Ertekin, Erhan; Ertekin, Banu Aslantaş; Binbay, Zerrin; Yüksel, Cağrı; Deveci, Erdem; Tükel, Raşit

    2015-01-30

    In this study, we aimed to investigate the effects of atypical and non-atypical depression comorbidity on the clinical characteristics and course of social anxiety disorder (SAD). A total of 247 patients with SAD were enrolled: 145 patients with a current depressive episode (unipolar or bipolar) with atypical features, 43 patients with a current depressive episode with non-atypical features and 25 patients without a lifetime history of depressive episodes were compared regarding sociodemographic and clinical features, comorbidity rates, and severity of SAD, depression and functional impairment. Thirty four patients with a past but not current history of major depressive episodes were excluded from the comparisons. 77.1% of current depressive episodes were associated with atypical features. Age at onset of SAD and age at initial major depressive episode were lower in the group with atypical depression than in the group with non-atypical depression. History of suicide attempts and bipolar disorder comorbidity was more common in the atypical depression group as well. Atypical depression group has higher SAD and depression severity and lower functionality than group with non-atypical depression. Our results indicate that the presence of atypical depression is associated with more severe symptoms and more impairment in functioning in patients with SAD. PMID:25454116

  16. The genome of Chelonid herpesvirus 5 harbors atypical genes

    USGS Publications Warehouse

    Ackermann, Mathias; Koriabine, Maxim; Hartmann-Fritsch, Fabienne; de Jong, Pieter J.; Lewis, Teresa D.; Schetle, Nelli; Work, Thierry M.; Dagenais, Julie; Balazs, George H.; Leong, Jo-Ann C.

    2012-01-01

    The Chelonid fibropapilloma-associated herpesvirus (CFPHV; ChHV5) is believed to be the causative agent of fibropapillomatosis (FP), a neoplastic disease of marine turtles. While clinical signs and pathology of FP are well known, research on ChHV5 has been impeded because no cell culture system for its propagation exists. We have cloned a BAC containing ChHV5 in pTARBAC2.1 and determined its nucleotide sequence. Accordingly, ChHV5 has a type D genome and its predominant gene order is typical for the varicellovirus genus within the alphaherpesvirinae. However, at least four genes that are atypical for an alphaherpesvirus genome were also detected, i.e. two members of the C-type lectin-like domain superfamily (F-lec1, F-lec2), an orthologue to the mouse cytomegalovirus M04 (F-M04) and a viral sialyltransferase (F-sial). Four lines of evidence suggest that these atypical genes are truly part of the ChHV5 genome: (1) the pTARBAC insertion interrupted the UL52 ORF, leaving parts of the gene to either side of the insertion and suggesting that an intact molecule had been cloned. (2) Using FP-associated UL52 (F-UL52) as an anchor and the BAC-derived sequences as a means to generate primers, overlapping PCR was performed with tumor-derived DNA as template, which confirmed the presence of the same stretch of "atypical" DNA in independent FP cases. (3) Pyrosequencing of DNA from independent tumors did not reveal previously undetected viral sequences, suggesting that no apparent loss of viral sequence had happened due to the cloning strategy. (4) The simultaneous presence of previously known ChHV5 sequences and F-sial as well as F-M04 sequences was also confirmed in geographically distinct Australian cases of FP. Finally, transcripts of F-sial and F-M04 but not transcripts of lytic viral genes were detected in tumors from Hawaiian FP-cases. Therefore, we suggest that F-sial and F-M04 may play a role in FP pathogenesis.

  17. The genome of Chelonid herpesvirus 5 harbors atypical genes.

    PubMed

    Ackermann, Mathias; Koriabine, Maxim; Hartmann-Fritsch, Fabienne; de Jong, Pieter J; Lewis, Teresa D; Schetle, Nelli; Work, Thierry M; Dagenais, Julie; Balazs, George H; Leong, Jo-Ann C

    2012-01-01

    The Chelonid fibropapilloma-associated herpesvirus (CFPHV; ChHV5) is believed to be the causative agent of fibropapillomatosis (FP), a neoplastic disease of marine turtles. While clinical signs and pathology of FP are well known, research on ChHV5 has been impeded because no cell culture system for its propagation exists. We have cloned a BAC containing ChHV5 in pTARBAC2.1 and determined its nucleotide sequence. Accordingly, ChHV5 has a type D genome and its predominant gene order is typical for the varicellovirus genus within the alphaherpesvirinae. However, at least four genes that are atypical for an alphaherpesvirus genome were also detected, i.e. two members of the C-type lectin-like domain superfamily (F-lec1, F-lec2), an orthologue to the mouse cytomegalovirus M04 (F-M04) and a viral sialyltransferase (F-sial). Four lines of evidence suggest that these atypical genes are truly part of the ChHV5 genome: (1) the pTARBAC insertion interrupted the UL52 ORF, leaving parts of the gene to either side of the insertion and suggesting that an intact molecule had been cloned. (2) Using FP-associated UL52 (F-UL52) as an anchor and the BAC-derived sequences as a means to generate primers, overlapping PCR was performed with tumor-derived DNA as template, which confirmed the presence of the same stretch of "atypical" DNA in independent FP cases. (3) Pyrosequencing of DNA from independent tumors did not reveal previously undetected viral sequences, suggesting that no apparent loss of viral sequence had happened due to the cloning strategy. (4) The simultaneous presence of previously known ChHV5 sequences and F-sial as well as F-M04 sequences was also confirmed in geographically distinct Australian cases of FP. Finally, transcripts of F-sial and F-M04 but not transcripts of lytic viral genes were detected in tumors from Hawaiian FP-cases. Therefore, we suggest that F-sial and F-M04 may play a role in FP pathogenesis. PMID:23056373

  18. 226Ra in the western Indian Ocean

    NASA Astrophysics Data System (ADS)

    Chung, Y.

    1987-09-01

    226Ra profiles have been measured in the western Indian Ocean as part of the 1977-1978 Indian Ocean GEOSECS program. These profiles show a general increase in deep and bottom water Ra concentration from the Circumpolar region to the Arabian Sea. A deep Ra maximum which originates in the Arabian Sea and in the Somali basin at about 3000 m depth spreads southward into the Mascarene basin and remains discernible in the Madagascar and Crozet basins. In the western Indian Ocean, the cold Antarctic Bottom Water spreads northward under the possibly southward-flowing deep water, forming a clear benthic front along the Crozet basin across the Southwest Indian Ridge into the Madagascar and Mascarene basins. The Antarctic Bottom Water continues to spread farther north to the Somali basin through the Amirante Passage at 10°S as a western boundary current. The benthic front and other characteristic features in the western Indian Ocean are quite similar to those observed in the western Pacific where the benthic front as a distinctive feature was first described by Craig et al. [15]. Across the Mid-Indian Ridge toward the Ceylon abyssal plain near the triple junction, Ra profiles display a layered structure, reflecting the topographic effect of the mid-ocean ridge system on the mixing and circulation of the deep and bottom waters. Both Ra and Si show a deep maximum north of the Madagascar basin. Linear relationships between these two elements are observed in the deep and bottom water with slopes increasing northward. This suggests a preferential input of Ra over Si from the bottom sediments of the Arabian Sea and also from the flank sediments of the Somali basin.

  19. Discovery of radioactive decay of /sup 222/Ra and /sup 224/Ra by /sup 14/C emission

    SciTech Connect

    Price, P.B.; Stevenson, J.D.; Barwick, S.W.; Ravn, H.L.

    1985-01-28

    Using the ISOLDE on-line isotope separator at CERN to produce sources of /sup 221/Fr, /sup 221/Ra, /sup 222/Ra, /sup 223/Ra, and /sup 224/Ra, and using polycarbonate track-recording films sensitive to energetic carbon nuclei but not to alpha particles, we have discovered two new cases of the rare /sup 14/C decay mode: in /sup 222/Ra and /sup 224/Ra. Our results for branching ratios, B, relative to alpha decay are for /sup 221/Fr and /sup 221/Ra, B<4.4 x 10/sup -12/; for /sup 222/Ra, B = (3.7 +- 0.6) x 10/sup -10/; for /sup 223/Ra, B = (6.1 +- 1.0) x 10/sup -10/; for /sup 224/Ra, B = (4.3 +- 1.2) x 10/sup -11/. .AE

  20. Atypical Trigeminal Neuralgia Secondary to Meningioma

    PubMed Central

    Niwant, Premeshwar; Motwani, Mukta; Naik, Sushil

    2015-01-01

    Trigeminal neuralgia is a disorder of the fifth cranial nerve that causes episodes of intense, stabbing, electric shock-like pain that lasts from few seconds to few minutes in the areas of the face where the branches of the nerve are distributed. More than one nerve branch can be affected by the disorder. We report an unusual case of trigeminal neuralgia affecting right side of face presenting atypical features of neuralgia and not responding to the usual course of treatment. The magnetic resonance imaging study of brain revealed a large extra-axial mass involving right cerebellopontine angle region causing moderate pressure effect on trigeminal nerve and brain stem. The aim of this case report is to show a tumor of cerebellopontine angle, presenting clinically as atypical trigeminal neuralgia. PMID:26664753

  1. Atypical Trigeminal Neuralgia Secondary to Meningioma.

    PubMed

    Niwant, Premeshwar; Motwani, Mukta; Naik, Sushil

    2015-01-01

    Trigeminal neuralgia is a disorder of the fifth cranial nerve that causes episodes of intense, stabbing, electric shock-like pain that lasts from few seconds to few minutes in the areas of the face where the branches of the nerve are distributed. More than one nerve branch can be affected by the disorder. We report an unusual case of trigeminal neuralgia affecting right side of face presenting atypical features of neuralgia and not responding to the usual course of treatment. The magnetic resonance imaging study of brain revealed a large extra-axial mass involving right cerebellopontine angle region causing moderate pressure effect on trigeminal nerve and brain stem. The aim of this case report is to show a tumor of cerebellopontine angle, presenting clinically as atypical trigeminal neuralgia. PMID:26664753

  2. Atypical ductal hyperplasia: interobserver and intraobserver variability.

    PubMed

    Jain, Rohit K; Mehta, Rutika; Dimitrov, Rosen; Larsson, Lisbeth G; Musto, Paul M; Hodges, Kurt B; Ulbright, Thomas M; Hattab, Eyas M; Agaram, Narasimhan; Idrees, Muhammad T; Badve, Sunil

    2011-07-01

    Interobserver reproducibility in the diagnosis of benign intraductal proliferative lesions has been poor. The aims of the study were to investigate the inter- and intraobserver variability and the impact of the addition of an immunostain for high- and low-molecular weight keratins on the variability. Nine pathologists reviewed 81 cases of breast proliferative lesions in three stages and assigned each of the lesions to one of the following three diagnoses: usual ductal hyperplasia, atypical ductal hyperplasia and ductal carcinoma in situ. Hematoxylin and eosin slides and corresponding slides stained with ADH-5 cocktail (cytokeratins (CK) 5, 14. 7, 18 and p63) by immunohistochemistry were evaluated. Concordance was evaluated at each stage of the study. The interobserver agreement among the nine pathologists for diagnosing the 81 proliferative breast lesions was fair (κ-value=0.34). The intraobserver κ-value ranged from 0.56 to 0.88 (moderate to strong). Complete agreement among nine pathologists was achieved in only nine (11%) cases, at least eight agreed in 20 (25%) cases and seven or more agreed in 38 (47%) cases. Following immunohistochemical stain, a significant improvement in the interobserver concordance (overall κ-value=0.50) was observed (P=0.015). There was a significant reduction in the total number of atypical ductal hyperplasia diagnosis made by nine pathologists after the use of ADH-5 immunostain. Atypical ductal hyperplasia still remains a diagnostic dilemma with wide variation in both inter- and intraobserver reproducibility among pathologists. The addition of an immunohistochemical stain led to a significant improvement in the concordance rate. More importantly, there was an 8% decrease in the number of lesions classified as atypical ductal hyperplasia in favor of usual hyperplasia; in clinical practice, this could lead to a decrease in the number of surgeries carried out for intraductal proliferative lesions. PMID:21532546

  3. Atypical cases of Dowling-Degos disease.

    PubMed

    Naveen, Kikkeri Narayanshetty; Athaniker, Sharatchandra B; Hegde, Spandana P; Shetty, Rahul; Radha, Hanumanthayya; Parinitha, Sadashivappa Sangam

    2016-01-01

    Dowling-Degos disease (DDD) is a rare autosomal dominant condition characterized by multiple, small, round pigmented macules usually arranged in reticular pattern, chiefly distributed in axillae and groins. Here we are reporting three atypical cases of DDD in a family. They had hypopigmented macules with typical features of DDD indicating generalized DDD. Histopathology confirmed the diagnosis. We present these three cases to stress the existence of generalized DDD phenotype in the Indian population. PMID:27057490

  4. [Persistent idiopathic facial pain and atypical odontalgia].

    PubMed

    Gaul, Charly; Ettlin, Dominik; Pfau, Doreen B

    2013-01-01

    The terms 'persistent idiopathic facial pain' (PIFP) and 'atypical odontalgia' (AO) are currently used as exclusion diagnoses for chronic toothache and chronic facial pain. Knowledge about these pain conditions in medical and dental practices is of crucial importance for the prevention of iatrogenic tissue damage by not-indicated invasive interventions, such as endodontic treatment and tooth extraction. In the present paper, etiology and pathogenesis, differential diagnostic criteria, and diagnostic approaches will be explained and relevant therapeutic principles will be outlined. PMID:23916270

  5. SB-RA-2001 Inhibits Bacterial Proliferation by Targeting FtsZ Assembly

    PubMed Central

    2015-01-01

    FtsZ has been recognized as a promising antimicrobial drug target because of its vital role in bacterial cell division. In this work, we found that a taxane SB-RA-2001 inhibited the proliferation of Bacillus subtilis 168 and Mycobacterium smegmatis cells with minimal inhibitory concentrations of 38 and 60 μM, respectively. Cell lengths of these microorganisms increased remarkably in the presence of SB-RA-2001, indicating that it inhibits bacterial cytokinesis. SB-RA-2001 perturbed the formation of the FtsZ ring in B. subtilis 168 cells and also affected the localization of the late cell division protein, DivIVA, at the midcell position. Flow cytometric analysis of the SB-RA-2001-treated cells indicated that the compound did not affect the duplication of DNA in B. subtilis 168 cells. Further, SB-RA-2001 treatment did not affect the localization of the chromosomal partitioning protein, Spo0J, along the two ends of the nucleoids and also had no discernible effect on the nucleoid segregation in B. subtilis 168 cells. The agent also did not appear to perturb the membrane potential of B. subtilis 168 cells. In vitro, SB-RA-2001 bound to FtsZ with modest affinity, promoted the assembly and bundling of FtsZ protofilaments, and reduced the GTPase activity of FtsZ. GTP did not inhibit the binding of SB-RA-2001 to FtsZ, suggesting that it does not bind to the GTP binding site on FtsZ. A computational analysis indicated that SB-RA-2001 binds to FtsZ in the cleft region between the C-terminal domain and helix H7, and the binding site of SB-RA-2001 on FtsZ resembled that of PC190723, a well-characterized inhibitor of FtsZ. The findings collectively suggested that SB-RA-2001 inhibits bacterial proliferation by targeting the assembly dynamics of FtsZ, and this can be exploited further to develop potent FtsZ-targeted antimicrobials. PMID:24749867

  6. Aggressive papillary adenocarcinoma on atypical localization

    PubMed Central

    Balci, Mecdi Gurhan; Tayfur, Mahir; Deger, Ayse Nur; Cimen, Orhan; Eken, Huseyin

    2016-01-01

    Abstract Introduction: Aggressive digital papillary adenocarcinoma (ADPA) is a rare sweat gland tumor that is found on the fingers, toes, and the digits. To date, <100 cases have been reported in the literature. Apart from 1 case reported in the thigh, all of them were on digital or nondigital acral skin. Case presentation: A 67-year-old Caucasian woman was admitted to the hospital due to a mass on the scalp. This lesion was present for almost a year. It was a semimobile cyctic mass that elevated the scalp. There was no change in the skin color. Its dimensions were 1.5 × 1 × 0.6 cm. The laboratory, clinic, and radiologic findings (head x-ray) of the patient were normal. It was evaluated as a benign lesion such as lipoma or epidermal cyst by a surgeon due to a small semimobile mass and no erosion of the skull. It was excised by a local surgery excision. The result of the pathologic examination was aggressive papillary adenocarcinoma. This diagnosis is synonymous with ADPA. Conclusion: In our case, localization was scalp. This localization is the first for this tumor in the literature. In addition, another atypical localization of this tumor (ADPA) is thigh in the literature. This case was presented due to both the rare and atypical localizations. That is why, in our opinion, revision of “digital” term in ADPA is necessary due to seem in atypical localizations like thigh and scalp. PMID:27428196

  7. Overview and potential unifying themes of the atypical chemokine receptor family.

    PubMed

    Vacchini, Alessandro; Locati, Massimo; Borroni, Elena Monica

    2016-06-01

    Chemokines modulate immune responses through their ability to orchestrate the migration of target cells. Chemokines directly induce cell migration through a distinct set of 7 transmembrane domain G protein-coupled receptors but are also recognized by a small subfamily of atypical chemokine receptors, characterized by their inability to support chemotactic activity. Atypical chemokine receptors are now emerging as crucial regulatory components of chemokine networks in a wide range of physiologic and pathologic contexts. Although a new nomenclature has been approved recently to reflect their functional distinction from their conventional counterparts, a systematic view of this subfamily is still missing. This review discusses their biochemical and immunologic properties to identify potential unifying themes in this emerging family. PMID:26740381

  8. Rapid Method for Ra-226 and Ra-228 in Water Samples

    SciTech Connect

    Maxwell, Sherrod, L. III

    2006-02-10

    The measurement of radium isotopes in natural waters is important for oceanographic studies and for public health reasons. Ra-226 (1620 year half-life) is one of the most toxic of the long-lived alpha emitters present in the environment due to its long life and its tendency to concentrate in bones, which increases the internal radiation dose of individuals. The analysis of radium-226 and radium-228 in natural waters can be tedious and time-consuming. Different sample preparation methods are often required to prepare Ra-226 and Ra-228 for separate analyses. A rapid method has been developed at the Savannah River Environmental Laboratory that effectively separates both Ra-226 and Ra-228 (via Ac-228) for assay. This method uses MnO{sub 2} Resin from Eichrom Technologies (Darien, IL, USA) to preconcentrate Ra-226 and Ra-228 rapidly from water samples, along with Ba-133 tracer. DGA Resin{reg_sign} (Eichrom) and Ln-Resin{reg_sign} (Eichrom) are employed in tandem to prepare Ra-226 for assay by alpha spectrometry and to determine Ra-228 via the measurement of Ac-228 by gas proportional counting. After preconcentration, the manganese dioxide is dissolved from the resin and passed through stacked Ln-Resin-DGA Resin cartridges that remove uranium and thorium interferences and retain Ac-228 on DGA Resin. The eluate that passed through this column is evaporated, redissolved in a lower acidity and passed through Ln-Resin again to further remove interferences before performing a barium sulfate microprecipitation. The Ac-228 is stripped from the resin, collected using cerium fluoride microprecipitation and counted by gas proportional counting. By using vacuum box cartridge technology with rapid flow rates, sample preparation time is minimized.

  9. Experience produces the atypicality bias in object perception.

    PubMed

    Kantne, Justin; Tanaka, James W

    2012-01-01

    When a morph face is produced with equal physical contributions from a typical parent face and an atypical parent face, the morph is judged to be more similar to the atypical parent. This discontinuity between physical and perceptual distance relationships, called the "atypicality bias" (Tanaka et al 1998, Cognition 68 199-220), has also been demonstrated with non-face objects (birds and cars; Tanaka and Corneille 2007 Perception & Psychophysics 69 619-627). We tested whether the atypicality bias can be induced for a novel set of artificial objects. Two categories of "blob" stimuli were generated, each composed of typical and atypical members. Morphs averaged from typical and atypical parent exemplars were used to test the presence of an atypicality bias before and after participants were familiarized with blob items. In experiment 1, participants were trained to discriminate between the two blob categories. An atypicality bias was evident after, but not prior to, category training. In experiment 2, participants rated the pleasantness of the blobs instead of learning to categorize them; an atypicality bias was present only after the ratings task. This finding suggests that relatively passive exposure to exemplars is sufficient to influence perceptions of similarity, and that the atypicality bias is a manifestation of this influence. PMID:23025159

  10. An improved method for the simultaneous determination of /sup 224/Ra, /sup 226/Ra and /sup 228/Ra in water, soils and sediments

    SciTech Connect

    Lucas, H.F.

    1987-01-01

    The naturally occurring concentrations of radium (/sup 226/Ra and /sup 228/Ra) in public and private water supplies have been studied for many years. Both general surveys ad local studies have established the geographical regions where well waters exceed 3 pCi/L (1-17). In general, the /sup 226/Ra was determined by the emanation method, while the /sup 228/Ra was determined from the beta activity of the /sup 228/Ac daughter. In a recent review (18) of the methods used ''a number of approved analytic methods can bear improvement, especially the method for 228Ra.'' The purpose of the work described here was to develop an improved method for the simultaneous determination of /sup 226/Ra and /sup 228/Ra. 22 refs., 3 tabs.

  11. Characterization of the atypical lymphocytes in African swine fever

    PubMed Central

    Karalyan, Z. A.; Ter-Pogossyan, Z. R.; Abroyan, L. O.; Hakobyan, L. H.; Avetisyan, A. S.; Karalyan, N. Yu; Karalova, E. M.

    2016-01-01

    Aim: Atypical lymphocytes usually described as lymphocytes with altered shape, increased DNA amount, and larger size. For analysis of cause of genesis and source of atypical lymphocytes during African swine fever virus (ASFV) infection, bone marrow, peripheral blood, and in vitro model were investigated. Materials and Methods: Atypical lymphocytes under the influence of ASFV were studied for morphologic, cytophotometric, and membrane surface marker characteristics and were used in vivo and in vitro models. Results: This study indicated the increased size, high metabolic activity, and the presence of additional DNA amount in atypical lymphocytes caused by ASFV infection. Furthermore, in atypical lymphocytes, nuclear-cytoplasmic ratio usually decreased, compared to normal lymphocytes. In morphology, they looking like lymphocytes transformed into blasts by exposure to mitogens or antigens in vitro. They vary in morphologic detail, but most of them are CD2 positive. Conclusions: Our data suggest that atypical lymphocytes may represent an unusual and specific cellular response to ASFV infection. PMID:27536044

  12. Domain Engineering

    NASA Astrophysics Data System (ADS)

    Bjørner, Dines

    Before software can be designed we must know its requirements. Before requirements can be expressed we must understand the domain. So it follows, from our dogma, that we must first establish precise descriptions of domains; then, from such descriptions, “derive” at least domain and interface requirements; and from those and machine requirements design the software, or, more generally, the computing systems.

  13. Management of Typical and Atypical Hangman's Fractures.

    PubMed

    Al-Mahfoudh, Rafid; Beagrie, Christopher; Woolley, Ele; Zakaria, Rasheed; Radon, Mark; Clark, Simon; Pillay, Robin; Wilby, Martin

    2016-05-01

    Study Design Retrospective study of a prospectively maintained database. Objective Our aim was to retrospectively review management and outcomes of patients with low-grade hangman's fractures, specifically looking at differences in outcomes between collars and halo immobilization. We also studied fracture patterns and their treatment outcomes. Methods Forty-one patients with hangman's fractures were identified from 105 patients with axis fractures between 2007 and 2013. Typical hangman's fractures were defined as traumatic spondylolisthesis of the axis causing a bilateral pars interarticularis fracture. Fractures involving the posterior cortex of C2 on one or both sides or an asymmetrical pattern were defined as atypical. Results There were 41 patients with a mean age of 59 years, with 13 (31.7%) typical and 28 (68.2%) atypical fractures. There were 22 (53.6%) type 1 fractures, 7 (41.4%) type 2 fractures, and 2 (4.9%) type 2a fractures in this series. Cervical collars were used to manage 11 patients (27% of all patients with hangman's fractures) and halo orthosis was used in 27 (65.8%). Three (7.3%) patients underwent surgical fixation of the fracture. Bony union was achieved in all patients on radiologic follow-up. Permanent neurologic deficit occurred in one patient due to associated injuries. Neck pain and stiffness were reported more commonly in the atypical group, but this finding was not statistically significant. Conclusions The majority of hangman type fractures can be treated nonoperatively. We found no difference in outcomes between a rigid collar or halo immobilization for treatment of low-grade fractures. Radiologic follow-up is essential to identify cases of nonunion. PMID:27099816

  14. Management of Typical and Atypical Hangman's Fractures

    PubMed Central

    Al-Mahfoudh, Rafid; Beagrie, Christopher; Woolley, Ele; Zakaria, Rasheed; Radon, Mark; Clark, Simon; Pillay, Robin; Wilby, Martin

    2015-01-01

    Study Design Retrospective study of a prospectively maintained database. Objective Our aim was to retrospectively review management and outcomes of patients with low-grade hangman's fractures, specifically looking at differences in outcomes between collars and halo immobilization. We also studied fracture patterns and their treatment outcomes. Methods Forty-one patients with hangman's fractures were identified from 105 patients with axis fractures between 2007 and 2013. Typical hangman's fractures were defined as traumatic spondylolisthesis of the axis causing a bilateral pars interarticularis fracture. Fractures involving the posterior cortex of C2 on one or both sides or an asymmetrical pattern were defined as atypical. Results There were 41 patients with a mean age of 59 years, with 13 (31.7%) typical and 28 (68.2%) atypical fractures. There were 22 (53.6%) type 1 fractures, 7 (41.4%) type 2 fractures, and 2 (4.9%) type 2a fractures in this series. Cervical collars were used to manage 11 patients (27% of all patients with hangman's fractures) and halo orthosis was used in 27 (65.8%). Three (7.3%) patients underwent surgical fixation of the fracture. Bony union was achieved in all patients on radiologic follow-up. Permanent neurologic deficit occurred in one patient due to associated injuries. Neck pain and stiffness were reported more commonly in the atypical group, but this finding was not statistically significant. Conclusions The majority of hangman type fractures can be treated nonoperatively. We found no difference in outcomes between a rigid collar or halo immobilization for treatment of low-grade fractures. Radiologic follow-up is essential to identify cases of nonunion. PMID:27099816

  15. Atypical neuroimaging in Wilson’s disease

    PubMed Central

    Patell, Rushad; Dosi, Rupal; Joshi, Harshal K; Storz, Dennis

    2014-01-01

    Wilson's disease is a rare metabolic disease involving copper metabolism. Neuroimaging plays an important part in evaluation of patients with a neuropsychiatric presentation. We present a case of a 14-year-old girl with atypical confluent white matter disease and cystic degeneration on MRI, with a rapidly progressive course, who succumbed to complications despite treatment with trientine. Wilson's disease should be considered as a differential for leucoencephalopathy in young patients with progressive neurological disease for its early recognition and optimum outcome. PMID:24907221

  16. Atypical odontalgia--a diagnostic dilemma.

    PubMed

    Thorburn, D N; Polonowita, A D

    2012-06-01

    Atypical odontalgia (AO) is a chronic orofacial pain condition of unclear pathophysiology, often presenting as toothache or pain at an extraction site. Idiopathic, psychogenic, vascular, and neuropathic causes have been proposed. In view of demonstrable somatosensory changes, and responses to management proposed for other forms of neuropathic pain, the best current evidence supports a neuropathic hypothesis. It is proposed that certain individuals with as-yet-undefined genetic vulnerability can develop AO when exposed to certain risk factors, including invasive dental treatment. The diagnosis and treatment of AO can be challenging, but can be aided by a multidisciplinary approach. Two cases of differing complexity are presented in this paper. PMID:22788051

  17. Management of Atypical and Anaplastic Meningiomas.

    PubMed

    Buttrick, Simon; Shah, Ashish H; Komotar, Ricardo J; Ivan, Michael E

    2016-04-01

    Meningiomas are the most prevalent primary tumor of central nervous system origin and, although most neoplasms are benign, a small proportion exemplifies an aggressive profile characterized by high recurrence rates, pleomorphic histology, and overall resistance to standard treatment. Standard initial therapy for malignant meningiomas includes maximal safe surgical resection followed by focal radiation in certain cases. The role for chemotherapy during recurrence of these aggressive meningiomas is less clear. Prognosis is poor and recurrence of malignant meningiomas is high. This article provides an overview of atypical and anaplastic malignant meningiomas, their treatment, and ongoing research for more effective treatments. PMID:27012388

  18. Trisomy 18 with unilateral atypical ectrodactyly

    SciTech Connect

    Rogers, R.C.

    1994-01-01

    Becerra et al. recently reported on an infant with multiple congenital anomalies who had trisomy 18. This preterm infant presented with bilateral ectrodactyly of feet, small cleft palate, esophageal atresia with associated tracheoesophageal fistula, congenital heart disease and other anomalies. The authors referenced article by Castle and Bernstein, in which they reported a male with trisomy 18 and cleft foot as well as a review of the literature which showed 2 other infants with trisomy 18 and ectrodactyly of the feet. An additional case of trisomy 18 associated with multiple congenital anomalies, including unilaterial, atypical ectrodactyly of the left foot.

  19. Atypical Bronchial Carcinoid Masquerading as Bronchial Asthma.

    PubMed

    Rajendran, V; Iqbal; Kumar, Vinod

    2015-11-01

    A case study of 35-year-old woman with persistent breathlessness and wheezing that had been unsuccessfully treated with inhaled beta 2-agonists and steroids for about two years. Patient developed dry cough and haemoptysis, so investigated further. Spirometry demonstrated a restrictive pattern. Chest CT demonstrated well defined hyperdense lesion in right middle lobe. Biopsy taken from the mass during bronchoscopy demonstrated the picture of atypical bronchial carcinoid. In this case, due to the lack of awareness, diagnosis of carcinoid was delayed by two years. PMID:27608788

  20. The estimation and potential radiobiological significance of the intake of 228Ra by early Ra dial workers in Illinois.

    PubMed

    Keane, A T; Lucas, H F; Markun, F; Essling, M A; Holtzman, R B

    1986-09-01

    We made radiochemical determinations of 226Ra and the 228Ra-decay product, 228Th, in samples of bone from former Ra dial workers who belonged to a major cohort of Ra-exposed persons under study for health effects at our institution. Most of the former workers were long-term residents of two communities supplied with drinking water containing elevated natural levels of 228Ra and 226Ra, so determinations also were made of radioactivity in samples of bone from long-term residents not occupationally exposed to Ra. The 228Th activity of the bones of the former workers, after correction for the presence of natural radioactivity, showed that some had significant occupational intakes of 228Ra, contrary to published reports that 228Ra was never used by the Illinois company that had employed the cohort of early workers. For 14 workers hired in the years 1920-23, the calculated ratio of the occupational intake of 228Ra to 226Ra activity averaged 0.15 (coefficient of variation 0.65), whereas for three workers hired in 1924, it was not significantly different from zero (mean 0.05, coefficient of variation 1.5). The risk of radiogenic cancer for the typical worker hired before 1924 may have been nearly twice that incurred in the absence of the 228Ra component of the Ra intakes. PMID:3744831

  1. Relationship between air pollution and positivity of RA-related autoantibodies in individuals without established RA: a report on SERA

    PubMed Central

    Gan, Ryan W; Deane, Kevin D; Zerbe, Gary O; Demoruelle, M Kristen; Weisman, Michael H; Buckner, Jane H; Gregersen, Peter K; Mikuls, Ted R; O’Dell, James R; Keating, Richard M; Holers, V Michae; Norris, Jill M

    2013-01-01

    Introduction Studies suggest that respiratory exposures including smoking, proximity to traffic and air pollution might be associated with development of rheumatoid arthritis (RA). RA-related autoantibodies are predictive of the development of RA. Objective We evaluated the relationship between RA-related autoantibodies and exposure to particulate matter (PM), a measure of air pollution of interest to health, in individuals without RA. Methods The Studies of the Etiology of Rheumatoid Arthritis (SERA) is a multicentre study following first-degree relatives (FDRs) of a proband with RA. FDRs are without the 1987 ACR (American College of Rheumatology) classifiable RA at enrolment and are followed for the development of RA-related autoimmunity. RA-related autoantibody outcomes as well as tender and swollen joint outcomes were assessed. Exposure to PM was assigned using ambient air pollution monitoring data and interpolated with inverse distance weighting spatial analyses using Geographic Information Systems. PM exposures were linked to FDR’s residential zip codes. Results RA-related autoantibodies as well as tender or swollen joints are not associated with ambient PM concentrations. Discussion While other respiratory exposures may be associated with increased risk of RA, our data suggest that ambient PM is not associated with autoantibodies and joint signs among individuals without RA, but at increased risk of developing RA. PMID:23572338

  2. Error-control and processes optimization of (223/224)Ra measurement using Delayed Coincidence Counter (RaDeCC).

    PubMed

    Xiaoqing, Cheng; Lixin, Yi; Lingling, Liu; Guoqiang, Tang; Zhidong, Wang

    2015-11-01

    RaDeCC has proved to be a precise and standard way to measure (224)Ra and (223)Ra in water samples and successfully made radium a tracer of several environmental processes. In this paper, the relative errors of (224)Ra and (223)Ra measurement in water samples via a Radium Delayed Coincidence Count system are analyzed through performing coincidence correction calculations and error propagation. The calculated relative errors range of 2.6% ∼ 10.6% for (224)Ra and 9.6% ∼ 14.2% for (223)Ra. For different radium activities, effects of decay days and counting time on final radium relative errors are evaluated and the results show that these relative errors can decrease by adjusting the two measurement factors. Finally, to minimize propagated errors in Radium activity, a set of optimized RaDeCC measurement parameters are proposed. PMID:26233651

  3. Pontine Infarct Presenting with Atypical Dental Pain: A Case Report

    PubMed Central

    Goel, Rajat; Kumar, Sanjeev; Panwar, Ajay; Singh, Abhishek B

    2015-01-01

    Orofacial pain’ most commonly occurs due to dental causes like caries, gingivitis or periodontitis. Other common causes of ‘orofacial pain’ are sinusitis, temporomandibular joint(TMJ) dysfunction, otitis externa, tension headache and migraine. In some patients, the etiology of ‘orofacial pain’ remains undetected despite optimal evaluation. A few patients in the practice of clinical dentistry presents with dental pain without any identifiable dental etiology. Such patients are classified under the category of ‘atypical odontalgia’. ‘Atypical odontalgia’ is reported to be prevalent in 2.1% of the individuals. ‘Atypical orofacial pain’ and ‘atypical odontalgia’ can result from the neurological diseases like multiple sclerosis, trigeminal neuralgia and herpes infection. Trigeminal neuralgia has been frequently documented as a cause of ‘atypical orofacial pain’ and ‘atypical odontalgia’. There are a few isolated case reports of acute pontine stroke resulting in ‘atypical orofacial pain’ and ‘atypical odontalgia’. However, pontine stroke as a cause of atypical odontalgia is limited to only a few cases, hence prevalence is not established. This case is one, where a patient presented with acute onset atypical dental pain with no identifiable dental etiology, further diagnosed as an acute pontine infarct on neuroimaging. A 40 years old male presented with acute onset, diffuse teeth pain on right side. Dental examination was normal. Magnetic resonance imaging(MRI) of the brain had an acute infarct in right pons near the trigeminal root entry zone(REZ). Pontine infarct presenting with dental pain as a manifestation of trigeminal neuropathy, has rarely been reported previously. This stresses on the importance of neuroradiology in evaluation of atypical cases of dental pain. PMID:26464604

  4. Pontine Infarct Presenting with Atypical Dental Pain: A Case Report.

    PubMed

    Goel, Rajat; Kumar, Sanjeev; Panwar, Ajay; Singh, Abhishek B

    2015-01-01

    Orofacial pain' most commonly occurs due to dental causes like caries, gingivitis or periodontitis. Other common causes of 'orofacial pain' are sinusitis, temporomandibular joint(TMJ) dysfunction, otitis externa, tension headache and migraine. In some patients, the etiology of 'orofacial pain' remains undetected despite optimal evaluation. A few patients in the practice of clinical dentistry presents with dental pain without any identifiable dental etiology. Such patients are classified under the category of 'atypical odontalgia'. 'Atypical odontalgia' is reported to be prevalent in 2.1% of the individuals. 'Atypical orofacial pain' and 'atypical odontalgia' can result from the neurological diseases like multiple sclerosis, trigeminal neuralgia and herpes infection. Trigeminal neuralgia has been frequently documented as a cause of 'atypical orofacial pain' and 'atypical odontalgia'. There are a few isolated case reports of acute pontine stroke resulting in 'atypical orofacial pain' and 'atypical odontalgia'. However, pontine stroke as a cause of atypical odontalgia is limited to only a few cases, hence prevalence is not established. This case is one, where a patient presented with acute onset atypical dental pain with no identifiable dental etiology, further diagnosed as an acute pontine infarct on neuroimaging. A 40 years old male presented with acute onset, diffuse teeth pain on right side. Dental examination was normal. Magnetic resonance imaging(MRI) of the brain had an acute infarct in right pons near the trigeminal root entry zone(REZ). Pontine infarct presenting with dental pain as a manifestation of trigeminal neuropathy, has rarely been reported previously. This stresses on the importance of neuroradiology in evaluation of atypical cases of dental pain. PMID:26464604

  5. Atypical developmental of dorsal and ventral attention networks in autism.

    PubMed

    Farrant, Kristafor; Uddin, Lucina Q

    2016-07-01

    Individuals with autism spectrum disorders (ASD) exhibit early and lifelong impairments in attention across multiple domains. While the disorder is known to affect attention processes, very little is currently known about the brain networks underlying attention in ASD, and even less is known about whether these atypicalities persist across the lifespan. We used functional connectivity analysis applied to resting state functional magnetic resonance imaging (fMRI) data to explore the dorsal (DAN) and ventral (VAN) attention networks in two separate age cohorts of children and adults with and without ASD. We find significant developmental differences in functional connectivity of brain regions that are critical for attention in children and adults with ASD. Specifically, children with ASD show hyper-connectivity of regions-of-interest (ROIs) in both attention networks compared with both typically developing (TD) children and adults with ASD. In contrast, adults with ASD show hypo-connectivity of these networks compared with neurotypical adults. These findings are consistent with the notion that consideration of developmental stage is critical in studies of functional connectivity in ASD. This study further illustrates diverging developmental patterns for top-down and bottom-up attention systems in autism. PMID:26613549

  6. Regulation of chemotactic networks by 'atypical' receptors.

    PubMed

    Comerford, Iain; Litchfield, Wendel; Harata-Lee, Yuka; Nibbs, Robert J B; McColl, Shaun R

    2007-03-01

    Directed cell migration is a fundamental component of numerous biological systems and is critical to the pathology of many diseases. Although the importance of secreted chemoattractant factors in providing navigational cues to migrating cells bearing specific chemoattractant receptors is now well-established, how the function of these factors is regulated is not so well understood and may be of key importance to the design of new therapeutics for numerous human diseases. While regulation of migration clearly takes place on a number of different levels, it is becoming clear that so-called 'atypical' receptors play a role in scavenging, or altering the localisation of, chemoattractant molecules such as chemokines and complement components. These receptors do this through binding and/or internalising their chemoattractant ligands without activating signal transduction cascades leading to cell migration. The atypical chemokine receptor family currently comprises the receptors D6, DARC and CCX-CKR. In this review, we discuss the evidence from in vitro and in vivo studies that these receptors play a role in regulating cell migration, and speculate that other orphan receptors may also belong to this family. Furthermore, with the advent of gene therapy on the horizon, the therapeutic potential of these receptors in human disease is also considered. PMID:17295321

  7. Chemokine sequestration by atypical chemokine receptors.

    PubMed

    Hansell, C A H; Simpson, C V; Nibbs, R J B

    2006-12-01

    Leucocyte migration is essential for robust immune and inflammatory responses, and plays a critical role in many human diseases. Chemokines, a family of small secreted protein chemoattractants, are of fundamental importance in this process, directing leucocyte trafficking by signalling through heptahelical G-protein-coupled receptors expressed by the migrating cells. However, several mammalian chemokine receptors, including D6 and CCX-CKR (ChemoCentryx chemokine receptor), do not fit existing models of chemokine receptor function, and do not even appear to signal in response to chemokine binding. Instead, these 'atypical' chemokine receptors are biochemically specialized for chemokine sequestration, acting to regulate chemokine bioavailability and thereby influence responses through signalling-competent chemokine receptors. This is of critical importance in vivo, as mice lacking D6 show exaggerated cutaneous inflammatory responses and an increased susceptibility to the development of skin cancer. CCX-CKR, on the other hand, is predicted to modulate homoeostatic lymphocyte and dendritic cell trafficking, key migratory events in acquired immune responses that are directed by CCX-CKR-binding chemokines. Thus studies on 'atypical' chemokine receptors are revealing functional and biochemical diversity within the chemokine receptor family and providing insights into novel mechanisms of chemokine regulation. PMID:17073739

  8. Atypical Yersinia enterocolitica: clinical and epidemiological parameters.

    PubMed Central

    Bottone, E J

    1978-01-01

    Infections due to biochemically typical Yersinia enterocolitica usually present as gastroenteritis, mesenteric lymphadenitis, terminal ileitis, and septicemia often with visceral abscesses. In these instances, the isolates have been biochemically typical and of well-established serotypes, namely 0:3 or 0:9 and, in the United States, 0:5 or 0:8. The recovery, recognition, and significance of biochemically and serologically atypical Y. enterocolitica in human infections has proceeded more slowly. From an analysis of the clinical histories of 20 patients infected with 21 such aberrant Y. enterocolitica, it appears that these strains are of restricted pathogenic potential, producing various clinical entities such as localized skin abscesses, conjunctivitis, self-limiting enteritis, and wound and urinary tract infections in hosts with predisposing factors. Epidemiologically, whereas episodic acquisition of atypical strains by hospitalized patients is indicative of nosocomial transmission, in the present series sporadic isolations over a 4-year period, mainly from ambulatory patients, suggest an occult reservoir in the community serviced by The Mount Sinai Hospital. In contrast to typical Y. enterocolitica, which has become well adapted in animal and human hosts, it appears that environmental strains may be in the evolutionary process of becoming adapted to humans. PMID:670380

  9. Neuromyelitis optica: atypical clinical and neuroradiological presentation.

    PubMed

    Splendiani, Alessandra; Mariani, Silvia; Anselmi, Monica; Catalucci, Alessia; Di Cesare, Ernesto; Gallucci, Massimo

    2015-02-01

    The extreme variability of clinical and MRI findings in the suspicion of Devic's disease always requires the detection of specific antibodies (AQP4). MRI scans were performed with a high-field MRI scanner (1.5T General Electric Signa Horizon): the MRI protocol of the brain employed axial DP, T2, T1, FLAIR and DWI weighted images (wi) and coronal T2-wi. After intravenous administration of contrast medium axial and sagittal T1-weighted images of the brain were repeated. The spine protocol employed after contrast medium included sagittal T2-wi, T2-wi with fat suppression and T1-wi. In May 2004, a 64-year-old healthy Caucasian woman began to suffer loss of motor and thermal sensitivity in the left lower limb. MRI showed non-specific areas of abnormal signal intensity on the deep left frontal and right frontoparietal white matter with no pathological enhancement after contrast medium and a small intramedullary area of altered signal at metameric level C2-C4 with a diagnosis of post-viral transverse myelitis. The patient had two similar episodes years later so the neurologist decided to search for circulating IgG AQP4 with the definitive diagnosis of neuromyelitis optica. In this case, compared to a clinical presentation of atypical deficit neurological involvement, the neuroradiological results of a progressive diffuse involvement of the white matter were atypical. PMID:25924171

  10. Atypical eating disorder in a male patient.

    PubMed

    Bailer, U; De Zwaan, M; Kasper, S

    1999-01-01

    In the literature there is evidence that a substantial proportion of patients with bulimia nervosa can be helped by cognitive behavioural self-help manuals. As there are no specific recommendations or strategies for the treatment of males with eating disorders we were therefore especially interested in the way a man might deal with such a self-help manual. In this case the book Getting better bit(e) by bit(e) by Treasure and Schmidt (also available in German translation) was given to a young man with an atypical eating disorder (atypical anorexia nervosa according to ICD-10). The patient was offered a maximum of 16 short visits and was seen by a first-year psychiatry resident. Treatment with this self-help manual was effective and the patient succeeded in changing his former eating behaviour. The case report provides preliminary evidence that a self-help manual may be a useful addition to the range of possible interventions in the treatment of eating disorders in men. Self-help manuals are less intensive and less costly than other forms of treatment and might be the lowest-step intervention in a stepped-care approach to treatment. PMID:24941097

  11. Neuromyelitis Optica: Atypical Clinical and Neuroradiological Presentation

    PubMed Central

    Mariani, Silvia; Anselmi, Monica; Catalucci, Alessia; Di Cesare, Ernesto; Gallucci, Massimo

    2015-01-01

    The extreme variability of clinical and MRI findings in the suspicion of Devic's disease always requires the detection of specific antibodies (AQP4). MRI scans were performed with a high-field MRI scanner (1.5T General Electric Signa Horizon): the MRI protocol of the brain employed axial DP, T2, T1, FLAIR and DWI weighted images (wi) and coronal T2-wi. After intravenous administration of contrast medium axial and sagittal T1-weighted images of the brain were repeated. The spine protocol employed after contrast medium included sagittal T2-wi, T2-wi with fat suppression and T1-wi. In May 2004, a 64-year-old healthy Caucasian woman began to suffer loss of motor and thermal sensitivity in the left lower limb. MRI showed non-specific areas of abnormal signal intensity on the deep left frontal and right frontoparietal white matter with no pathological enhancement after contrast medium and a small intramedullary area of altered signal at metameric level C2-C4 with a diagnosis of post-viral transverse myelitis. The patient had two similar episodes years later so the neurologist decided to search for circulating IgG AQP4 with the definitive diagnosis of neuromyelitis optica. In this case, compared to a clinical presentation of atypical deficit neurological involvement, the neuroradiological results of a progressive diffuse involvement of the white matter were atypical. PMID:25924171

  12. Young Peoples' Representations of "Atypical" Work in English Society

    ERIC Educational Resources Information Center

    Crafter, Sarah; O'Dell, Lindsay; de Abreu, Guida; Cline, Tony

    2009-01-01

    In this paper, we explore young peoples' normative representations of work. In particular, we are interested in the ways young people view work roles which could be considered "atypical" such as young caring or language brokering. Interviewed were 46 young people (15-18 years) some who did, and some who did not engage in the "atypical" work roles…

  13. Atypical chest discomfort in a 36-year-old man.

    PubMed

    Alexander, Lisa Mustone; Robie, Stephen; Baute, Scott

    2016-07-01

    This article focuses on aortic aneurysms and the evaluation of patients with atypical chest pain. A complete and accurate family history is essential. Clinicians should pay close attention to persistence of symptoms and age in patients with atypical chest discomfort who do not have routine confirmatory cardiac findings. PMID:27351640

  14. Association of a bovine prion gene haplotype with atypical BSE

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a class of fatal neurodegenerative disorders that occur in humans, ruminants, cats, and mink. Three distinct TSEs afflict cattle: classical bovine spongiform encephalopathy (BSE), atypical H-type BSE, and atypical ...

  15. Keloidal Atypical Fibroxanthoma: Case and Review of the Literature

    PubMed Central

    Tongdee, Emily; Touloei, Khasha; Shitabata, Paul K.; Shareef, Shahjahan; Maranda, Eric L.

    2016-01-01

    Keloidal atypical fibroxanthoma (KAF) has recently been categorized as a variant of atypical fibroxanthoma. This paper will emphasize the importance of including KAF in both clinical and histological differential diagnosis of benign and malignant lesions which exhibit keloidal collagen and will also review the current literature on epidemiology, pathogenesis, histology, immunochemistry and treatments. PMID:27462224

  16. Typical and Atypical Dementia Family Caregivers: Systematic and Objective Comparisons

    ERIC Educational Resources Information Center

    Nichols, Linda O.; Martindale-Adams, Jennifer; Burns, Robert; Graney, Marshall J.; Zuber, Jeffrey

    2011-01-01

    This systematic, objective comparison of typical (spouse, children) and atypical (in-law, sibling, nephew/niece, grandchild) dementia family caregivers examined demographic, caregiving and clinical variables. Analysis was of 1,476 caregivers, of whom 125 were atypical, from the Resources for Enhancing Alzheimer's Caregivers Health (REACH I and II)…

  17. The Genome of Chelonid Herpesvirus 5 Harbors Atypical Genes

    PubMed Central

    Ackermann, Mathias; Koriabine, Maxim; Hartmann-Fritsch, Fabienne; de Jong, Pieter J.; Lewis, Teresa D.; Schetle, Nelli; Work, Thierry M.; Dagenais, Julie; Balazs, George H.; Leong, Jo-Ann C.

    2012-01-01

    The Chelonid fibropapilloma-associated herpesvirus (CFPHV; ChHV5) is believed to be the causative agent of fibropapillomatosis (FP), a neoplastic disease of marine turtles. While clinical signs and pathology of FP are well known, research on ChHV5 has been impeded because no cell culture system for its propagation exists. We have cloned a BAC containing ChHV5 in pTARBAC2.1 and determined its nucleotide sequence. Accordingly, ChHV5 has a type D genome and its predominant gene order is typical for the varicellovirus genus within the alphaherpesvirinae. However, at least four genes that are atypical for an alphaherpesvirus genome were also detected, i.e. two members of the C-type lectin-like domain superfamily (F-lec1, F-lec2), an orthologue to the mouse cytomegalovirus M04 (F-M04) and a viral sialyltransferase (F-sial). Four lines of evidence suggest that these atypical genes are truly part of the ChHV5 genome: (1) the pTARBAC insertion interrupted the UL52 ORF, leaving parts of the gene to either side of the insertion and suggesting that an intact molecule had been cloned. (2) Using FP-associated UL52 (F-UL52) as an anchor and the BAC-derived sequences as a means to generate primers, overlapping PCR was performed with tumor-derived DNA as template, which confirmed the presence of the same stretch of “atypical” DNA in independent FP cases. (3) Pyrosequencing of DNA from independent tumors did not reveal previously undetected viral sequences, suggesting that no apparent loss of viral sequence had happened due to the cloning strategy. (4) The simultaneous presence of previously known ChHV5 sequences and F-sial as well as F-M04 sequences was also confirmed in geographically distinct Australian cases of FP. Finally, transcripts of F-sial and F-M04 but not transcripts of lytic viral genes were detected in tumors from Hawaiian FP-cases. Therefore, we suggest that F-sial and F-M04 may play a role in FP pathogenesis. PMID:23056373

  18. The genome of Chelonid herpesvirus 5 harbors atypical genes

    USGS Publications Warehouse

    Ackermann, Mathias; Koriabine, Maxim; Hartmann-Fritsch, Fabienne; de Jong, Pieter J.; Lewis, Teresa D.; Schetle, Nelli; Work, Thierry M.; Dagenais, Julie; Balazs, George H.; Leong, Jo-Ann C.

    2012-01-01

    The Chelonid fibropapilloma-associated herpesvirus (CFPHV; ChHV5) is believed to be the causative agent of fibropapillomatosis (FP), a neoplastic disease of marine turtles. While clinical signs and pathology of FP are well known, research on ChHV5 has been impeded because no cell culture system for its propagation exists. We have cloned a BAC containing ChHV5 in pTARBAC2.1 and determined its nucleotide sequence. Accordingly, ChHV5 has a type D genome and its predominant gene order is typical for the varicellovirus genus within thealphaherpesvirinae. However, at least four genes that are atypical for an alphaherpesvirus genome were also detected, i.e. two members of the C-type lectin-like domain superfamily (F-lec1, F-lec2), an orthologue to the mouse cytomegalovirus M04 (F-M04) and a viral sialyltransferase (F-sial). Four lines of evidence suggest that these atypical genes are truly part of the ChHV5 genome: (1) the pTARBAC insertion interrupted the UL52 ORF, leaving parts of the gene to either side of the insertion and suggesting that an intact molecule had been cloned. (2) Using FP-associated UL52 (F-UL52) as an anchor and the BAC-derived sequences as a means to generate primers, overlapping PCR was performed with tumor-derived DNA as template, which confirmed the presence of the same stretch of “atypical” DNA in independent FP cases. (3) Pyrosequencing of DNA from independent tumors did not reveal previously undetected viral sequences, suggesting that no apparent loss of viral sequence had happened due to the cloning strategy. (4) The simultaneous presence of previously known ChHV5 sequences and F-sial as well as F-M04 sequences was also confirmed in geographically distinct Australian cases of FP. Finally, transcripts of F-sial and F-M04 but not transcripts of lytic viral genes were detected in tumors from Hawaiian FP-cases. Therefore, we suggest that F-sial and F-M04 may play a role in FP pathogenesis

  19. [Atypical cerebellar neurocytoma resembling a hemangioblastoma. A case report].

    PubMed

    Lista Martínez, Olalla; Rivas López, Luis Alfredo; Pombo Otero, Jorge Francisco; Amaro Cendón, Santiago; Bravo García, Christian; Villa Fernández, Juan Manuel

    2014-01-01

    Through August 2013, 105 cases of intracranial extraventricular neurocytoma (EVN) had been described; 6% were located in cerebellum and 22% were atypical EVN. A rare morphologic form of neurocytoma, atypical EVN has had only 24 cases reported to date. Its prognosis is poorer than the typical central neurocytoma. This case report describes an atypical cerebellar EVN, a form that has not been reported yet, hence the interest of this article. We emphasise its cystic nature and mural nodule, in an infrequent presentation. EVN are low-incidence tumours that we need to take into consideration when making the differential diagnosis of cystic cerebellar lesions with mural nodule. Given that the prognosis of atypical EVNs depends on the atypical nature and on the grade of resection, medical follow up has to be more constant, due to the greater degree of recurrence. PMID:24837842

  20. [An atypical measles case presenting with severe cervical spasm].

    PubMed

    Oztoprak, Nefise; Celebi, Güven; Temiz, Ekrem

    2006-10-01

    Atypical measles is characterized by high fever, headache, myalgia and atypical rash, in patients who were vaccinated against measles. In this report a 22 years old male patient presenting with severe cervical and shoulder muscles spasms, purpuric and petechial lesions on palm, sole and whole body, has been presented. The patient had not an underlying disease or immunosuppression, and he had experienced single dose of measles vaccine when he was nine months old. Diagnosis of atypical measles was based on the clinical and serological findings (by measles IgM and IgG positivity). The aim of the presentation of this case was to emphasize that atypical measles should be considered for differential diagnosis in the cases complaining of muscle spasms and atypical skin rashes even if vaccinated against measles. PMID:17205700

  1. Atypical Response Regulator ChxR from Chlamydia trachomatis Is Structurally Poised for DNA Binding

    PubMed Central

    Barta, Michael L.; Hickey, John M.; Anbanandam, Asokan; Dyer, Kevin; Hammel, Michal; Hefty, P. Scott

    2014-01-01

    ChxR is an atypical two-component signal transduction response regulator (RR) of the OmpR/PhoB subfamily encoded by the obligate intracellular bacterial pathogen Chlamydia trachomatis. Despite structural homology within both receiver and effector domains to prototypical subfamily members, ChxR does not require phosphorylation for dimer formation, DNA binding or transcriptional activation. Thus, we hypothesized that ChxR is in a conformation optimal for DNA binding with limited interdomain interactions. To address this hypothesis, the NMR solution structure of the ChxR effector domain was determined and used in combination with the previously reported ChxR receiver domain structure to generate a full-length dimer model based upon SAXS analysis. Small-angle scattering of ChxR supported a dimer with minimal interdomain interactions and effector domains in a conformation that appears to require only subtle reorientation for optimal major/minor groove DNA interactions. SAXS modeling also supported that the effector domains were in a head-to-tail conformation, consistent with ChxR recognizing tandem DNA repeats. The effector domain structure was leveraged to identify key residues that were critical for maintaining protein - nucleic acid interactions. In combination with prior analysis of the essential location of specific nucleotides for ChxR recognition of DNA, a model of the full-length ChxR dimer bound to its cognate cis-acting element was generated. PMID:24646934

  2. [Atypical early posttraumatic syndromes (author's transl)].

    PubMed

    Muller, G E

    1974-01-01

    In a consecutive series of 1,925 head injuries, 283 patients (14.7%), could not be classified, neither in the group of simple head injuries without cerebral symptoms, nor in the group of typical concussions characterized by immediate amnesia or observed coma. We have prefered the rather neutral term of atypical early posttraumatic syndromes. In this group, apart from neurovegetative manifestations, partial disturbances of consciousness and perception, we have also classified delayed disturbances of consciousness. Special attention has been given to migraineous phenomena and to a syndrome, characteristic for children, described by Mealey. This is an intermediate group important from a medico-legal point of view because certain transient cerebral manifestations risk to be mistaken for psychological reactions. On the other hand symptoms probably of psychic origin were discussed. PMID:4469864

  3. Treatment of Morbidity with Atypical Chest Pain

    PubMed Central

    Cott, Arthur

    1987-01-01

    The appropriate management of atypical chest pain requires an integration of medical and behavioural treatments. Unnecessary medicalization can increase morbidity. A sensitivity to the behavioural factors contributing to symptoms and disability may reduce both. The purpose of this paper is to provide physicians with a cognitive-behavioural perspective of the nature of morbidity and disability associated with chronic chest discomfort; some strategies for detecting heretofore unsuspected disability associated with chronic chest pain and related discomfort in patients with organic findings (both cardiac and non-cardiac), as well those with no identifiable disease process or organic cause; and some simple behavioural and cognitive-behavioural therapeutic techniques for treating and preventing such problems. PMID:21263912

  4. Atypical inflammatory demyelinating syndromes of the CNS.

    PubMed

    Hardy, Todd A; Reddel, Stephen W; Barnett, Michael H; Palace, Jacqueline; Lucchinetti, Claudia F; Weinshenker, Brian G

    2016-08-01

    Atypical inflammatory demyelinating syndromes are rare disorders that differ from multiple sclerosis owing to unusual clinical or MRI findings or poor response to treatments used for multiple sclerosis. These syndromes include neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, Baló's concentric sclerosis, Schilder's disease, and Marburg's multiple sclerosis. The overlapping features of these syndromes with multiple sclerosis and with each other complicate diagnosis and their categorisation as distinct or related conditions. Recognition of these syndromes is crucial because they differ from multiple sclerosis and other demyelinating and non-demyelinating conditions in their prognosis and treatment. Advances in MRI, pathology, and immunobiology are needed to increase understanding of these syndromes, including the extent to which some of them represent distinct entities, and to assist with improvements in their diagnosis and management. PMID:27478954

  5. Recurrence of atypical fibroxanthoma. Diagnosis and treatment

    PubMed Central

    Mattiola, Leandro Ricardo; Mattiola, Lyzandro; Mattiola, Giovani; Mattiola, Sandra Zucchi de Moraes; Moura, Carlos Eduardo; Kirschnick, Alexandre

    2012-01-01

    Summary Introduction: The soft tissue sarcomas (SPM) accounts for only 1% of malignant tumors of the adult population. The SPM is the most frequent malignant fibrous histiocytoma (MFH) that exhibits behavior characterized by the tendency to invasion of adjacent tissue and metastatic spread early. One of its variants is the atypical fibroxanthoma (FA). Objective: To describe a case of probable recurrence of AF underwent surgical treatment and presentation of a literature review. Case report: Patient female, 63 years, presenting with a mass in the face about four inches and a history of prior resection of the lesion in the same topography. The patient underwent surgical resection with a diagnosis of AF. Conclusion: The FA is a rare tumor histological diagnosis difficult. The correct histological diagnosis and patient follow-up are essential. PMID:25991985

  6. [Treatment of atypical and neurotic depression].

    PubMed

    Leitner, P; Umann, E; Kulawik, H

    1986-10-01

    Hitherto it has not been usual to talk in the German language about the therapy-oriented concept of two forms of the progress of atypical depression (Type A and Type V). The characteristic symptom of Type A is angst, together with phobias, physical complaints, etc. In Type V there are vegetative symptoms, often towards evening (Hypersomnia, difficulty in getting to sleep, increased appetite, increased weight, increased libido), accompanied by hysterical extrovert personality traits, and of intermittent occurrence. These clinical pictures are amenable to psychopharmalogical therapy. In conformity with the assumption of "somatic accommodation" treatment with antidepressives is recommended in the case neurotic depression, too, at least in the initial stages of treatment. PMID:3809300

  7. Atypical presentation of clear cell odontogenic carcinoma.

    PubMed

    Infante-Cossio, Pedro; Torres-Carranza, Eusebio; Gonzalez-Perez, Luis-Miguel; Gonzalez-Cardero, Eduardo; Sanchez-Gallego, Felicia

    2012-09-01

    Clear cell odontogenic carcinoma (CCOC) is a rare malignant neoplasm of odontogenic origin. The usual clinical presentation of CCOC is a mass of progressive growth in the mandible sometimes accompanied with loss of teeth, pain, or bleeding. We describe a rare case of CCOC that showed an atypical presentation not previously described in the literature like a fast-growing painless mass in the retromolar area that reached a considerable size in a few days that caused obstruction of the airway. The presence of airway obstruction required immediate treatment, which consisted of a surgical excision of the tumor via a hemimandibulectomy. This clinical report highlights the possibility of odontogenic tumors presenting like a rapid-growing mass and the importance of clinical differential diagnosis of such presentation. PMID:22976710

  8. Atypical odontalgia - pathophysiology and clinical management.

    PubMed

    Baad-Hansen, L

    2008-01-01

    Atypical odontalgia (AO) is a chronic form of dental pain without signs of pathology. Several hypotheses have been put forward regarding the pathophysiology. AO has been proposed to be psychogenic, vascular, neuropathic or idiopathic. The scientific evidence supporting or rejecting these hypotheses are reviewed in this paper. At this time, the best supported hypothesis is that AO is a neuropathic pain condition. Relevant differential diagnoses, such as odontogenic pain, sinusitis, trigeminal neuralgia among others, are presented and the evidence regarding possible management strategies is reviewed. A treatment algorithm for AO is proposed based on the rather scarce scientific evidence available and inspired by a similar treatment algorithm for peripheral neuropathic pain. The proposed strategy involves an interdisciplinary approach including patient education, psychological counselling, topical and systemic medication and, importantly, avoidance of invasive treatments like surgery and endodontics. Two illustrative cases are presented. PMID:18190356

  9. Mycobacterium chelonae Is an Ubiquitous Atypical Mycobacterium

    PubMed Central

    Pinto-Gouveia, Miguel; Gameiro, Ana; Ramos, Leonor; Cardoso, José Carlos; Brites, Maria Manuel; Tellechea, Óscar; Figueiredo, Américo

    2015-01-01

    The type of cutaneous infection varies mainly according to the patient's immune status, and the disseminated form is mostly found in the context of immunosuppression. We report the case of a 62-year-old male who was under long-term systemic corticosteroid therapy and presented with a 7-month history of multiple painless cutaneous lesions at various stages of development: papules, nodules, pustules and hemorrhagic crusts, as well as small erosions and ulcers distributed over the limbs and scalp. Cutaneous biopsy showed a suppurative granulomatous infiltrate with abscess formation. Fite stain revealed numerous extracellular bacilli, suggesting mycobacterial infection, particularly by atypical mycobacteria. Culture of a skin sample revealed Mycobacterium chelonae. The patient started multidrug therapy and showed clinical improvement despite of resistance to one of the antibiotics. This striking presentation underlines the role of immunosuppression with corticotherapy as a major risk factor for these infections. Multidrug therapy is advised and antibiogram is essential in directing treatment. PMID:26351432

  10. Indications of atypical antipsychotics in the elderly.

    PubMed

    McKean, Andrew; Monasterio, Erik

    2015-01-01

    Atypical antipsychotics (AAP) have become some of the most commonly prescribed medications in primary and specialist care settings. Off-label prescribing accounts for much of the expanded use of AAPs. This has become common in the elderly. Marketing by pharmaceutical companies appears to have contributed to the off-label use of AAPs, in situations where their safety and efficacy is far from established. Although evidence provides varying degrees of support for their use for behavioural and psychological symptoms of dementia, augmentation of antidepressants in depression, anxiety, insomnia and in the management of psychosis in Parkinson's Disease, there are a number of potential problems with their expanded use in the elderly. These include weight gain, type two diabetes mellitus, sudden cardiac death and increased mortality rates in the elderly with dementia. It is recommended that whenever AAPs are used off-label, a review date is identified, informed consent is obtained and treatment and side-effects are closely monitored. PMID:25354148

  11. Statistical Detection of Atypical Aircraft Flights

    NASA Technical Reports Server (NTRS)

    Statler, Irving; Chidester, Thomas; Shafto, Michael; Ferryman, Thomas; Amidan, Brett; Whitney, Paul; White, Amanda; Willse, Alan; Cooley, Scott; Jay, Joseph; Rosenthal, Loren; Swickard, Andrea; Bates, Derrick; Scherrer, Chad; Webb, Bobbie-Jo; Lawrence, Robert; Mosbrucker, Chris; Prothero, Gary; Andrei, Adi; Romanowski, Tim; Robin, Daniel; Prothero, Jason; Lynch, Robert; Lowe, Michael

    2006-01-01

    A computational method and software to implement the method have been developed to sift through vast quantities of digital flight data to alert human analysts to aircraft flights that are statistically atypical in ways that signify that safety may be adversely affected. On a typical day, there are tens of thousands of flights in the United States and several times that number throughout the world. Depending on the specific aircraft design, the volume of data collected by sensors and flight recorders can range from a few dozen to several thousand parameters per second during a flight. Whereas these data have long been utilized in investigating crashes, the present method is oriented toward helping to prevent crashes by enabling routine monitoring of flight operations to identify portions of flights that may be of interest with respect to safety issues.

  12. Atypical regions in large genomic DNA sequences

    SciTech Connect

    Scherer, S. |; McPeek, M.S.; Speed, T.P.

    1994-07-19

    Large genomic DNA sequences contain regions with distinctive patterns of sequence organization. The authors describe a method using logarithms of probabilities based on seventh-order Markov chains to rapidly identify genomic sequences that do not resemble models of genome organization built from compilations of octanucleotide usage. Data bases have been constructed from Escherichia coli and Saccharomyces cerevisiae DNA sequences of >1000 nt and human sequences of >10,000 nt. Atypical genes and clusters of genes have been located in bacteriophage, yeast, and primate DNA sequences. The authors consider criteria for statistical significance of the results, offer possible explanations for the observed variation in genome organization, and give additional applications of these methods in DNA sequence analysis.

  13. Case Report: Atypical Cornelia de Lange Syndrome.

    PubMed

    Leanza, Vito; Rubbino, Gabriella; Leanza, Gianluca

    2014-01-01

    Cornelia de Lange Syndrome (CdLS) (also called Bushy Syndrome or Amsterdam dwarfism), is a genetic disorder that can lead to several alterations. This disease affects both physical and neuropsychiatric development. The various abnormalities include facial dysmorphia (arched eyebrows, synophrys, depressed nasal bridge, long philtrum, down-turned angles of the mouth), upper-extremity malformations, hirsutism, cardiac defects, and gastrointestinal alterations. The prevalence of this syndrome is approximately one per 15,000. Ultrasound is not the perfect means to diagnose CdLS, however, many abnormalities can be detected prenatally by scrupulous image observation. We report an atypical CdLS case characterized by increased nuchal translucency in the first trimester, normal karyotype, saddle nose, micrognathia with receding jaw, low set ears, facies senilis, arthrogryposis of the hands, absence of the Aranzio ductus venous, dilatation of gallbladder and bowel, a unique umbilical artery, increased volume of amniotic fluid, and intrauterine growth retardation ending with the interruption of pregnancy. PMID:26834972

  14. Atypical femoral fracture following zoledronic acid treatment.

    PubMed

    Ataoğlu, Baybars; Kaptan, Ahmet Yiğit; Eren, Toygun Kağan; Yapar, Ali Ekber; Berkay, Ahmet Fırat

    2016-04-01

    A 68-year-old female patient admitted to our clinic with right anterior thigh pain ongoing for six months and which increased in last two months. The patient had no trauma history. The patient had been followed-up for 15 years because of osteoporosis and administrated alendronate and ibandronate treatment for 10 years. Patient had three shots of zoledronate once a year during the last three years. Her pain was increasing when she was walking. Physical examination revealed pain in her right thigh. Radiogram showed thickened lateral cortex of the subtrochanteric area. Magnetic resonance imaging also showed thickening and edema of the same area. These images were correlated with atypical fracture in right femoral subthrochanteric zone. Dual energy X-ray absorptiometry revealed that T score was -3.3 in lumbar region and -2.5 in femoral neck. Zoledronate treatment was ended. Prophylactic surgical fixation was performed with titanium elastic nails. PMID:26874637

  15. Case Report: Atypical Cornelia de Lange Syndrome

    PubMed Central

    Leanza, Vito; Rubbino, Gabriella; Leanza, Gianluca

    2015-01-01

    Cornelia de Lange Syndrome (CdLS) (also called Bushy Syndrome or Amsterdam dwarfism), is a genetic disorder that can lead to several alterations. This disease affects both physical and neuropsychiatric development. The various abnormalities include facial dysmorphia (arched eyebrows, synophrys, depressed nasal bridge, long philtrum, down-turned angles of the mouth), upper-extremity malformations, hirsutism, cardiac defects, and gastrointestinal alterations. The prevalence of this syndrome is approximately one per 15,000. Ultrasound is not the perfect means to diagnose CdLS, however, many abnormalities can be detected prenatally by scrupulous image observation. We report an atypical CdLS case characterized by increased nuchal translucency in the first trimester, normal karyotype, saddle nose, micrognathia with receding jaw, low set ears, facies senilis, arthrogryposis of the hands, absence of the Aranzio ductus venous, dilatation of gallbladder and bowel, a unique umbilical artery, increased volume of amniotic fluid, and intrauterine growth retardation ending with the interruption of pregnancy. PMID:26834972

  16. Thymic carcinoma presenting as atypical chest pain.

    PubMed

    Siddiqui, Sadiq; Connelly, Tara; Keita, Luther; Blazkova, Sylvie; Veerasingam, Dave

    2015-01-01

    A 58-year-old woman with a 2-month history of atypical chest pain was referred to the chest pain clinic by the general practitioner. Exercise stress test was positive and subsequent coronary angiogram revealed significant triple vessel disease with left ventricular impairment requiring a coronary artery bypass graft (CABG). The patient had a chest X-ray as part of the preoperative work up. Chest X-ray revealed a large anterior mediastinal mass. Subsequent thorax CT revealed a 7.2 cm anterior mediastinal mass. CT-guided biopsy of the mass revealed the diagnosis of a poorly differentiated thymic basaloid carcinoma. The patient was successfully treated with concomitant surgery involving complete resection of the mass and a CABG procedure. PMID:26607199

  17. Pedal edema associated with atypical antipsychotics

    PubMed Central

    Munshi, Santanu; Mukherjee, Shatavisa; Saha, Indranil; Sen, Sukanta

    2016-01-01

    This study describes a patient diagnosed as a case of bipolar affective disorder complaining of bothersome incidence of pedal edema 1 month after the initiation of atypical antipsychotic regimen with risperidone and quetiapine. All hematological and biochemical profiles were found to be normal. On discontinuation of risperidone, the condition remained unresolved even after 2 weeks, and the edema progressed reaching her calves. On tapering the dose of quetiapine, she started showing gradual improvement in edematous condition. Quetiapine was slowly discontinued. No further recurrence of edema occurred, and hence, no further medication changes were implemented. Pedal edema was found to be resolved within weeks of dechallenge of the regimen. Naranjo adverse drug reaction probability scale gave a score of 7 which denotes “probable” adverse drug reaction with quetiapine. PMID:26997731

  18. Atypic geniculate neuralgia: atypic anatomic correlation of cranial nerve roots and AICA.

    PubMed

    Ozer, Füsun Demirçivi; Duransoy, Yusuf Kurtuluş; Camlar, Mahmut

    2009-08-01

    Geniculate neuralgia is a rare cause of craniofacial pains. The anterior inferior cerebellar artery is the offending vessel which compress nervus intermedius in the patients with typical geniculate neuralgia. We report a patient whose pain was atypical for either geniculate neuralgia and trigeminal neuralgia. At operation the anterior inferior cerebellar artery was coursing with the nerves and was separated. After the decompression the pain resolved immediately. PMID:19404569

  19. Atypical prion diseases in humans and animals.

    PubMed

    Tranulis, Michael A; Benestad, Sylvie L; Baron, Thierry; Kretzschmar, Hans

    2011-01-01

    Although prion diseases, such as Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the "scrapie form" (PrP(Sc)), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans. PMID:21598097

  20. High-Speed RaPToRS

    NASA Astrophysics Data System (ADS)

    Henchen, Robert; Esham, Benjamin; Becker, William; Pogozelski, Edward; Padalino, Stephen; Sangster, Thomas; Glebov, Vladimir

    2008-11-01

    The High-Speed Rapid Pneumatic Transport of Radioactive Samples (HS-RaPToRS) system, designed to quickly and safely move radioactive materials, was assembled and tested at the Mercury facility of the Naval Research Laboratory (NRL) in Washington D.C. A sample, which is placed inside a four-inch-diameter carrier, is activated before being transported through a PVC tube via airflow. The carrier travels from the reaction chamber to the end station where it pneumatically brakes prior to the gate. A magnetic latch releases the gate when the carrier arrives and comes to rest. The airflow, optical carrier-monitoring devices, and end gate are controlled manually or automatically with LabView software. The installation and testing of the RaPToRS system at NRL was successfully completed with transport times of less than 3 seconds. The speed of the carrier averaged 16 m/s. Prospective facilities for similar systems include the Laboratory for Laser Energetics and the National Ignition Facility.

  1. Atypical chemokine receptors in cancer: friends or foes?

    PubMed

    Massara, Matteo; Bonavita, Ornella; Mantovani, Alberto; Locati, Massimo; Bonecchi, Raffaella

    2016-06-01

    The chemokine system is a fundamental component of cancer-related inflammation involved in all stages of cancer development. It controls not only leukocyte infiltration in primary tumors but also angiogenesis, cancer cell proliferation, and migration to metastatic sites. Atypical chemokine receptors are a new, emerging class of regulators of the chemokine system. They control chemokine bioavailability by scavenging, transporting, or storing chemokines. They can also regulate the activity of canonical chemokine receptors with which they share the ligands by forming heterodimers or by modulating their expression levels or signaling activity. Here, we summarize recent results about the role of these receptors (atypical chemokine receptor 1/Duffy antigen receptor for chemokine, atypical chemokine receptor 2/D6, atypical chemokine receptor 3/CXC-chemokine receptor 7, and atypical chemokine receptor 4/CC-chemokine receptor-like 1) on the tumorigenesis process, indicating that their effects are strictly dependent on the cell type on which they are expressed and on their coexpression with other chemokine receptors. Indeed, atypical chemokine receptors inhibit tumor growth and progression through their activity as negative regulators of chemokine bioavailability, whereas, on the contrary, they can promote tumorigenesis when they regulate the signaling of other chemokine receptors, such as CXC-chemokine receptor 4. Thus, atypical chemokine receptors are key components of the regulatory network of inflammation and immunity in cancer and may have a major effect on anti-inflammatory and immunotherapeutic strategies. PMID:26908826

  2. The use of atypical antipsychotics in Bipolar Spectrum disorders

    PubMed Central

    Grünze, H.; Möller, H.J.

    2003-01-01

    Viewed in the context of ever-expanding conceptual boundaries for the diagnosis of bipolar disorder including the spectrum concept of DSM-IV, or even beyond (Akiskal and Pinto, 1999), it becomes obvious that lithium is the treatment of choice in a minority′ of patients only (Bowden et al, 2000). This article reviews what additional benefit atypical antipsychotics may provide in patients with bipolar disorder. Due both to tradition and to the regulatory requirements in the USA (FDA) and European Union (EMEA), the main target of clinical trials with atypical antipsychotics has been typical manic disorder. More recently, a significant subgroup of atypical patients, e.g., with mixed states, marked psychosis, or rapid cycling, have participated in these studies to allow an estimation of the value of atypical antipsychotics in these conditions. For the purposes of filing applications for registration with the regulatory agencies, the existing evidence is probably weak, however; from a clinical perspective, it is important that most atypical antipsychotics have also been tested in combination treatments. Finally, first data are now available on long-term prophylactic efficacy of atypical antipsychotics. These combined efficacy data definitely support the use of atypical antipsychotics in bipolar disorder, and it is now the time to collect more experience with these substances in severely ill patients in clinical settings. PMID:21206806

  3. Ra-224 and Ra-226: A New Method for Measuring Groundwater Seepage in Lake Michigan

    NASA Astrophysics Data System (ADS)

    Stevens, K. R.; Buyan, A. C.; Waples, J. T.

    2008-12-01

    Radium isotopes have been used to estimate groundwater discharge (GWD) in coastal marine waters for decades, but this technique has never before been used in the Laurentian Great Lakes. In this study, we used a RAD7 radon-in-air monitor to measure naturally-occurring radium isotopes Ra-224 (half-life= 3.64 d) and Ra-226 (half-life = 1600 a) in groundwater and three shallow water sites along Lake Michigan's Wisconsin coastline. Radium-224 activities in groundwater ranged from 1153 dpm m-3 in a deep aquifer (New Berlin well no.7) to 31 dpm m-3 in a shallow aquifer (Pryor well). Nearshore Lake Michigan measurements of Ra-224 were lowest at Red Arrow Beach (0.2 dpm m-3), higher in the Milwaukee harbor (GLWI slip, 1.1 dpm m-3) and highest at Harrington Beach (4.1 dpm m-3) and correspond well with groundwater seepage estimates made by Cherkauer et al. (1990) using alternate methods (i.e., where higher radium activity is indicative of higher GWD). These Ra-224 measurements are the first ever made in Lake Michigan (and presumably any of the Great Lakes) and we conclude that, by sampling offshore radium activity gradients, this RAD7 technique is a viable method for directly measuring GWD in Lake Michigan and other freshwater systems.

  4. Intracranial Tuberculoma Presenting as Atypical Eclampsia: A Case Report

    PubMed Central

    Murugesan, Sharmila; Pradeep, Sunitha; John, Lopamudra; Kolluru, Vasavi

    2016-01-01

    Occurrence of eclampsia before 20 weeks of pregnancy and after 48 hours of delivery in the absence of typical signs of hypertension and or proteinuria is termed as atypical eclampsia. Atypical or non-classic eclampsia will have some symptoms of eclampsia but without the usual proteinuria or hypertension. All patients with atypical onset should undergo neurological evaluation to rule out neurologic causes of seizures. Cerebral tuberculosis is a rare and serious form of disease secondary to haematogenous spread of Mycobacterium tuberculosis. Here we present a case of cerebral tuberculoma with seizures in late pregnancy mimicking eclampsia.

  5. Gene expression analysis in RA: towards personalized medicine

    PubMed Central

    Burska, A N; Roget, K; Blits, M; Soto Gomez, L; van de Loo, F; Hazelwood, L D; Verweij, C L; Rowe, A; Goulielmos, G N; van Baarsen, L G M; Ponchel, F

    2014-01-01

    Gene expression has recently been at the forefront of advance in personalized medicine, notably in the field of cancer and transplantation, providing a rational for a similar approach in rheumatoid arthritis (RA). RA is a prototypic inflammatory autoimmune disease with a poorly understood etiopathogenesis. Inflammation is the main feature of RA; however, many biological processes are involved at different stages of the disease. Gene expression signatures offer management tools to meet the current needs for personalization of RA patient's care. This review analyses currently available information with respect to RA diagnostic, prognostic and prediction of response to therapy with a view to highlight the abundance of data, whose comparison is often inconclusive due to the mixed use of material source, experimental methodologies and analysis tools, reinforcing the need for harmonization if gene expression signatures are to become a useful clinical tool in personalized medicine for RA patients. PMID:24589910

  6. [The modern concept of atypical depression: four definitions].

    PubMed

    Ohmae, Susumu

    2010-01-01

    This report describes and compares four current concepts and definitions of atypical depression. Since its emergence, atypical depression has been considered a depressive state that can be relieved by MAO inhibitors. Davidson classified the symptomatic features of atypical depression into type A, which is predominated by anxiety symptoms, and type V, which is represented by atypical vegetative symptoms, such as hyperphagia, weight gain, oversleeping, and increased sexual drive. Features that are shared by both subtypes include: early onset, female predominance, outpatient predominance, mildness, few suicide attempts, nonbipolarity, nonendogeneity, and few psychomotor changes. Based on these features, bipolar depression can also be defined as atypical depression type V. Herein, we examine and classify four concepts of atypical depression according to the endogenous-nonendogenous (melancholic-nonmelancholic) and unipolar-bipolar dichotomies. The Columbia University group (see Quitkin, Stewart, McGrath, Klein et al.) and the New South Wales University group (see Parker) consider atypical depression to be chronic, mild, nonendogenous (nonmelancholic), unipolar depression. The former group postulates that mood reactivity is necessary, while the latter asserts the structural priority of anxiety symptoms over mood symptoms and the significance of interpersonal rejection sensitivity. For the Columbia group, the significance of mood reactivity reflects the theory that mood nonreactivity is the essential symptom of "endogenomorphic depression", which was proposed by Klein as typical depression. Thus, mood reactivity is not related to overreactivity or hyperactivity, which are often observed in atypical depressives. However, Parker postulates that psychomotor symptoms are the essential features of melancholia, which he recognizes as typical depression; therefore, the New South Wales group does not recognize the significance of mood reactivity. The New South Wales group

  7. Preparing concentrated carrier-free /sup 228/Ra

    SciTech Connect

    Volynskii, L.D.; Garbuzov, V.M.; Tsirlin, V.A.

    1988-05-01

    A scheme has been devised for processing large amounts of old thorium salts to obtain concentrated carrier-free /sup 228/Ra preparations. The process includes simple regeneration of the original thorium salt. The main stages in concentrating the /sup 228/Ra are precipitation and separation of thorium peroxide, isolating the radium with a carrier, ion-exchange separation of the radium from the carrier, and final purification by electrolysis. The /sup 228/Ra recovery is 70%.

  8. [Evaluation of joint inflammation by MRI in patients with RA].

    PubMed

    Sumida, Takayuki; Sugihara, Makoto; Hirota, Tomoya; Horikoshi, Masanobu; Suzuki, Takeshi

    2012-02-01

    We compared the characteristics of MRI imaging and US imaging on joint inflammation in patients with rheumatoid arthritis (RA) . MRI imaging is worth for diagnosis of RA at the early stage, evaluation of drug treatment effects, and a new measure for true remission. US imaging is also useful for estimation of therapy and a criteria for complete remission, and cheep machine. We hope that both imaging technologies will be a standard for diagnosis and therapy evaluation in RA patients. PMID:22298072

  9. Rainbow Trout Sleeping Disease Virus Is an Atypical Alphavirus

    PubMed Central

    Villoing, Stéphane; Béarzotti, Monique; Chilmonczyk, Stefan; Castric, Jeannette; Brémont, Michel

    2000-01-01

    Sleeping disease (SD) is currently a matter of concern for salmonid fish farmers in most parts of the world. A viral etiology of SD has recently been suspected, since virus-like particles have been observed in infected rainbow trout cells. In salmonid-derived cell lines, the maximal rate of virus production was observed at 10°C, while little virus was produced at 14°C. Through biochemical, physicochemical, and morphological studies, SD virus (SDV) was shown to be an enveloped virus of roughly 60 nm in diameter. The genome consists of 12 kb of RNA, with the appearance of a 26S subgenomic RNA during the time course of SDV replication. The screening of a random-primed cDNA library constructed from the genomic RNA of semipurified virions facilitated the identification of a specific SDV cDNA clone having an open reading frame related to the alphavirus E2 glycoproteins. To extend the comparison between SDV structural proteins and the alphavirus protein counterparts, the nucleotide sequence of the total 4.1-kb subgenomic RNA has been determined. The 26S RNA encodes a 1,324-amino-acid polyprotein exhibiting typical alphavirus structural protein organization. SDV structural proteins showed several remarkable features compared to other alphaviruses: (i) unusually large individual proteins, (ii) very low homology (ranging from 30 to 34%) (iii) an unglycosylated E3 protein, and (iv) and E1 fusion domain sharing mutations implicated in the pH threshold. Although phylogenetically related to the Semliki Forest virus group of alphaviruses, SDV should be considered an atypical member, able to naturally replicate in lower vertebrates. PMID:10590104

  10. Recurrent ETNK1 mutations in atypical chronic myeloid leukemia.

    PubMed

    Gambacorti-Passerini, Carlo B; Donadoni, Carla; Parmiani, Andrea; Pirola, Alessandra; Redaelli, Sara; Signore, Giovanni; Piazza, Vincenzo; Malcovati, Luca; Fontana, Diletta; Spinelli, Roberta; Magistroni, Vera; Gaipa, Giuseppe; Peronaci, Marco; Morotti, Alessandro; Panuzzo, Cristina; Saglio, Giuseppe; Usala, Emilio; Kim, Dong-Wook; Rea, Delphine; Zervakis, Konstantinos; Viniou, Nora; Symeonidis, Argiris; Becker, Heiko; Boultwood, Jacqueline; Campiotti, Leonardo; Carrabba, Matteo; Elli, Elena; Bignell, Graham R; Papaemmanuil, Elli; Campbell, Peter J; Cazzola, Mario; Piazza, Rocco

    2015-01-15

    Despite the recent identification of recurrent SETBP1 mutations in atypical chronic myeloid leukemia (aCML), a complete description of the somatic lesions responsible for the onset of this disorder is still lacking. To find additional somatic abnormalities in aCML, we performed whole-exome sequencing on 15 aCML cases. In 2 cases (13.3%), we identified somatic missense mutations in the ETNK1 gene. Targeted resequencing on 515 hematological clonal disorders revealed the presence of ETNK1 variants in 6 (8.8%) of 68 aCML and 2 (2.6%) of 77 chronic myelomonocytic leukemia samples. These mutations clustered in a small region of the kinase domain, encoding for H243Y and N244S (1/8 H243Y; 7/8 N244S). They were all heterozygous and present in the dominant clone. The intracellular phosphoethanolamine/phosphocholine ratio was, on average, 5.2-fold lower in ETNK1-mutated samples (P < .05). Similar results were obtained using myeloid TF1 cells transduced with ETNK1 wild type, ETNK1-N244S, and ETNK1-H243Y, where the intracellular phosphoethanolamine/phosphocholine ratio was significantly lower in ETNK1-N244S (0.76 ± 0.07) and ETNK1-H243Y (0.37 ± 0.02) than in ETNK1-WT (1.37 ± 0.32; P = .01 and P = .0008, respectively), suggesting that ETNK1 mutations may inhibit the catalytic activity of the enzyme. In summary, our study shows for the first time the evidence of recurrent somatic ETNK1 mutations in the context of myeloproliferative/myelodysplastic disorders. PMID:25343957

  11. [Apropos of atypical melancholia with Sustiva (efavirenz)].

    PubMed

    Lang, J P; Halleguen, O; Picard, A; Lang, J M; Danion, J M

    2001-01-01

    The treatment of HIV infection has changed dramatically in recent years as a result of the development of new drugs which allows a variety of multitherapy combinations more adapted to patients' needs and thereby improving compliance. Efavirenz is a non-nucleoside reverse transcriptase inhibitor. In addition to a potent antiretroviral activity, efavirenz is an easy-to-take drug with once-daily dosing and is usually well tolerated. Efavirenz, however, may induce psychic alterations which are variable and atypical in both their clinical presentation and severity. As early as the first days of treatment, efavirenz may provoke surprising phenomena such as nightmares, vivid dreams, hallucinations or illusions, and twilight states. Depersonalization and derealization episodes, personality alterations, stream of thought troubles and unusual thought contents, atypical depression and cognitive disorders have also been observed. These phenomena may occur either early or later on treatment. The prevalence of severe psychic disorders is less than 5%, but they are often responsible for harmful treatment discontinuations. Psychiatric side effects are heterogeneous and probably not related to pre-existing psychologic weakness. We do not have enough data to evaluate these side effects and their etiopathogeny. The drug could act directly on the central nervous system since it crosses the blood-brain barrier, on the serotoninergic and dopaminergic systems. Some authors have compared efavirenz-induced psychic effects to those associated with LSD and found structural similarities between the two molecules. However, the heterogeneity and low prevalence of the psychiatric side effects of efavirenz suggest and individual sensitivity. In order to improve patient care, a better clinical approach, neuropsychological evaluation, and functional brain imagery should be used to progress in the analysis and comprehension of these disorders. We discuss in this paper the case of Mister H. This HIV

  12. Aripiprazole versus other atypical antipsychotics for schizophrenia

    PubMed Central

    Komossa, Katja; Rummel-Kluge, Christine; Schmid, Franziska; Hunger, Heike; Schwarz, Sandra; El-Sayeh, Hany George G; Kissling, Werner; Leucht, Stefan

    2014-01-01

    Background In many countries of the industrialised world second generation (atypical) antipsychotics have become first line drug treatments for people with schizophrenia. The question as to whether, and if so how much, the effects of the various second generation antipsychotics differ is a matter of debate. In this review we examine how the efficacy and tolerability of aripiprazole differs from that of other second generation antipsychotics. Objectives To evaluate the effects of aripiprazole compared with other atypical antipsychotics for people with schizophrenia and schizophrenia-like psychoses. Search methods We searched the Cochrane Schizophrenia Group Trials Register (March 2007) which is based on regular searches of BIOSIS, CENTRAL, CINAHL, EMBASE, MEDLINE and PsycINFO. Selection criteria We included all randomised trials comparing oral aripiprazole with oral forms of amisulpride, clozapine, olanzapine, quetiapine, risperidone, sertindole, ziprasidone or zotepine in people with schizophrenia or schizophrenia-like psychoses. Data collection and analysis We extracted data independently. For dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random-effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. For continuous data, we calculated weighted mean differences (MD) again based on a random-effects model. Main results The review currently includes four trials with 1404 participants on two out of eight possible comparisons - aripiprazole versus olanzapine and aripiprazole versus risperidone. The overall number of participants leaving the studies early was considerable (38.5%), limiting the validity of the findings, but with no significant differences between groups. Aripiprazole was less efficacious than olanzapine in terms of the general mental state (PANSS total score: n=794, 2 RCTs, MD 4.96 CI 1.85 to 8.06), but it was associated with fewer side

  13. RaPToRS Sample Delivery System

    NASA Astrophysics Data System (ADS)

    Henchen, Robert; Shibata, Kye; Krieger, Michael; Pogozelski, Edward; Padalino, Stephen; Glebov, Vladimir; Sangster, Craig

    2010-11-01

    At various labs (NIF, LLE, NRL), activated material samples are used to measure reaction properties. The Rapid Pneumatic Transport of Radioactive Samples (RaPToRS) system quickly and safely moves these radioactive samples through a closed PVC tube via airflow. The carrier travels from the reaction chamber to the control and analysis station, pneumatically braking at the outlet. A reversible multiplexer routes samples from various locations near the shot chamber to the analysis station. Also, the multiplexer allows users to remotely load unactivated samples without manually approaching the reaction chamber. All elements of the system (pneumatic drivers, flow control valves, optical position sensors, multiplexers, Geiger counters, and release gates at the analysis station) can be controlled manually or automatically using a custom LabVIEW interface. A prototype is currently operating at NRL in Washington DC. Prospective facilities for Raptors systems include LLE and NIF.

  14. The structure of the atypical killer cell immunoglobulin-like receptor, KIR2DL4.

    PubMed

    Moradi, Shoeib; Berry, Richard; Pymm, Phillip; Hitchen, Corinne; Beckham, Simone A; Wilce, Matthew C J; Walpole, Nicholas G; Clements, Craig S; Reid, Hugh H; Perugini, Matthew A; Brooks, Andrew G; Rossjohn, Jamie; Vivian, Julian P

    2015-04-17

    The engagement of natural killer cell immunoglobulin-like receptors (KIRs) with their target ligands, human leukocyte antigen (HLA) molecules, is a critical component of innate immunity. Structurally, KIRs typically have either two (D1-D2) or three (D0-D1-D2) extracellular immunoglobulin domains, with the D1 and D2 domain recognizing the α1 and α2 helices of HLA, respectively, whereas the D0 domain of the KIR3DLs binds a loop region flanking the α1 helix of the HLA molecule. KIR2DL4 is distinct from other KIRs (except KIR2DL5) in that it does not contain a D1 domain and instead has a D0-D2 arrangement. Functionally, KIR2DL4 is also atypical in that, unlike all other KIRs, KIR2DL4 has both activating and inhibitory signaling domains. Here, we determined the 2.8 Å crystal structure of the extracellular domains of KIR2DL4. Structurally, KIR2DL4 is reminiscent of other KIR2DL receptors, with the D0 and D2 adopting the C2-type immunoglobulin fold arranged with an acute elbow angle. However, KIR2DL4 self-associated via the D0 domain in a concentration-dependent manner and was observed as a tetramer in the crystal lattice by size exclusion chromatography, dynamic light scattering, analytical ultracentrifugation, and small angle x-ray scattering experiments. The assignment of residues in the D0 domain to forming the KIR2DL4 tetramer precludes an interaction with HLA akin to that observed for KIR3DL1. Accordingly, no interaction was observed to HLA by direct binding studies. Our data suggest that the unique functional properties of KIR2DL4 may be mediated by self-association of the receptor. PMID:25759384

  15. The atypical hyperosmotic stress response of Campylobacter jejuni

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The Atypical Hyperosmotic Stress Response of Campylobacter jejuni Background. Campylobacter species are unusually sensitive to hyperosmotic stress conditions imposed in the laboratory and encode no characterized osmoprotectant systems. Despite these limitations, the Gram-negative Campylobacter jeju...

  16. Treating DSM-IV depression with atypical features.

    PubMed

    Stewart, Jonathan W; Thase, Michael E

    2007-04-01

    Depression with atypical features is characterized by mood reactivity and 2 or more symptoms of vegetative reversal (including overeating, oversleeping, severe fatigue or leaden paralysis, and a history of rejection sensitivity). Another important feature of atypical depression is its preferential response to monoamine oxidase inhibitor (MAOI) treatment, especially phenelzine, relative to tricyclic antidepressants (TCAs). The efficacy of newer agents relative to MAOIs and TCAs is unclear. This presentation reviews currently available treatments for DSM-IV depression with atypical features, focusing specifically on placebo-controlled trials. Although phenelzine shows the most efficacy in this population, treatment with TCAs, selective serotonin reuptake inhibitors, cognitive-behavioral therapy, MAOIs other than phenelzine, and other agents are discussed. Following this presentation is a discussion on the treatment of depression with atypical features by experts in this subject area. PMID:17474800

  17. Depression With Atypical Features: Diagnostic Validity, Prevalence, and Treatment.

    PubMed

    Quitkin, Frederic M.

    2002-06-01

    Depression with atypical features is a treatable and relatively common disorder among depressed outpatients. A growing body of evidence suggests this is a biologically distinct subtype of depression. This assertion is supported by genetic epidemiologic studies and by a preferential response of the subtype to monoamine oxidase inhibitors compared with tricyclic antidepressants. The Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) includes atypical features as a parenthetical modifier for depressive illness. According to DSM-IV diagnostic criteria ("atypical features" specifier), the disorder is primarily characterized by 2 or more of the following symptoms as predominant features in patients with major depression or dysthymic disorder: overeating, oversleeping, "leaden paralysis," and interpersonal rejection sensitivity. Patients also show mood reactivity in response to actual or potential positive events. Despite aspects of the disorder resembling a maladaptive, persistent mode of behavior, patients diagnosed with depression with atypical features demonstrate a good response to antidepressant treatment. PMID:15014736

  18. Teaching strategies for atypical presentation of illness in older adults.

    PubMed

    Gray-Miceli, Deanna; Aselage, Melissa; Mezey, Mathy

    2010-07-01

    Atypical presentation of illness is a phenomenon where "seeing is believing." Expert geriatric nurses and clinicians know all too well the early signs and symptoms of this phenomenon, which frequently masquerades bacterial infections, pain, acute myocardial infarction, heart failure, or other serious medical ailments in older adults. Students, however, as novices to clinical practice, require interactive learning approaches to reflect on the patient's illness presentations, help with developing the necessary skills to analyze and synthesize clinically relevant data, and witness resolution of an atypical presentation when found and treated. Use of a case study as an educational tool can facilitate critical thinking about a clinical problem, such as atypical presentation of illness, for students within a problem-based learning format. Furthermore, we highlight strategies for teaching students atypical presentation of illness with consideration of student learning preferences, which include visual, auditory, reading, and kinesthetic modes of learning. PMID:20608591

  19. Retinoic acid signaling and mouse embryonic stem cell differentiation: Cross talk between genomic and non-genomic effects of RA.

    PubMed

    Rochette-Egly, Cécile

    2015-01-01

    Retinoic acid (RA), the active derivative of vitamin A, a fat-soluble vitamin, plays key roles in cell growth and differentiation by activating nuclear receptors, RARs (α, β and γ), which are ligand dependent regulators of transcription. The past years highlighted several novelties in the field that increased the complexity of RA effects. Indeed, in addition to its classical genomic effects, RA also has extranuclear and non-transcriptional effects. RA induces the rapid and transient activation of kinase cascades, which are integrated in the nucleus via the phosphorylation of RARs at a conserved serine residue located in the N-terminal domain and their coregulators. In order to investigate the relevance of RARs' phosphorylation in cell differentiation, mouse embryonic stem (mES) cells were used as a model. When treated with RA, these pluripotent cells give rise to neuronal cells. Cells invalidated for each RAR were generated as well as stable rescue lines expressing RARs mutated in phosphor acceptor sites. Such a strategy revealed that RA-induced neuronal differentiation involves the RARγ2 subtype and requires RARγ2 phosphorylation. Moreover, in gene expression profiling experiments, the phosphorylated form of RARγ2 was found to regulate a small subset of genes through binding a novel RA response element consisting of two direct repeats with a 7 base pair spacer. These new findings suggest an important role for RAR phosphorylation during cell differentiation, and pave the way for further investigations with other cell types and during embryonic development. This article is part of a Special Issue entitled Linking transcription to physiology in lipodomics. PMID:24768681

  20. Treatment with cimetidine of atypical fasciitis panniculitis syndrome.

    PubMed Central

    Naschitz, J E; Yeshurun, D; Rosner, I; Abrahamson, J E; Misselevitch, I; Boss, J H

    1990-01-01

    Three patients presented with septal fasciitis and panniculitis, associated with clinical and laboratory features which precluded straight-forward classification into eosinophilic fasciitis, localised scleroderma, or lupus erythematosus profundus. Treatment with cimetidine caused the remission of cutaneous manifestations and the extracutaneous abnormalities, such as nailfold capillary disturbances and the presence of antithyroid antibodies, improved. It is concluded that features of eosinophilic fasciitis or localised scleroderma and certain additional atypical elements should be categorised as atypical fasciitis-panniculitis syndrome. Images PMID:2241270

  1. Atypical mitochondrial inheritance patterns in eukaryotes.

    PubMed

    Breton, Sophie; Stewart, Donald T

    2015-10-01

    Mitochondrial DNA (mtDNA) is predominantly maternally inherited in eukaryotes. Diverse molecular mechanisms underlying the phenomenon of strict maternal inheritance (SMI) of mtDNA have been described, but the evolutionary forces responsible for its predominance in eukaryotes remain to be elucidated. Exceptions to SMI have been reported in diverse eukaryotic taxa, leading to the prediction that several distinct molecular mechanisms controlling mtDNA transmission are present among the eukaryotes. We propose that these mechanisms will be better understood by studying the deviations from the predominating pattern of SMI. This minireview summarizes studies on eukaryote species with unusual or rare mitochondrial inheritance patterns, i.e., other than the predominant SMI pattern, such as maternal inheritance of stable heteroplasmy, paternal leakage of mtDNA, biparental and strictly paternal inheritance, and doubly uniparental inheritance of mtDNA. The potential genes and mechanisms involved in controlling mitochondrial inheritance in these organisms are discussed. The linkage between mitochondrial inheritance and sex determination is also discussed, given that the atypical systems of mtDNA inheritance examined in this minireview are frequently found in organisms with uncommon sexual systems such as gynodioecy, monoecy, or andromonoecy. The potential of deviations from SMI for facilitating a better understanding of a number of fundamental questions in biology, such as the evolution of mtDNA inheritance, the coevolution of nuclear and mitochondrial genomes, and, perhaps, the role of mitochondria in sex determination, is considerable. PMID:26501689

  2. Transformation of a meningioma with atypical imaging

    PubMed Central

    Kumar, Ashish; Deopujari, Chandrashekhar; Karmarkar, Vikram

    2016-01-01

    Meningiomas are benign tumors of the central nervous system. They have long term curability if they are excised completely. If not, they can recur after a prolonged period and can lead to increased morbidity during re-surgery. Recurrence is rarely associated with invasiveness. Usually de-differentiation in case of meningiomas is uncommon without any predisposing factors including different genetic mutations or radiation to the involved region. We report a case of a 38-year-old female who was operated for a benign para-sagittal meningioma 8 years back and subsequently developed an invasive recurrence off late. Also this time, the imaging morphology was slightly different for a meningioma and gross as well as microscopic findings were very atypical. Awareness for such cases must be there while dealing with recurrent meningiomas as invasiveness may not always be associated with adverse predisposing factors like radiation. As invasiveness is always a histopathological diagnosis, picking up such features on imaging is a daunting task and if done, can help neurosurgeons prognosticate such invasive recurrences in a better fashion. PMID:27366271

  3. Transformation of a meningioma with atypical imaging.

    PubMed

    Kumar, Ashish; Deopujari, Chandrashekhar; Karmarkar, Vikram

    2016-01-01

    Meningiomas are benign tumors of the central nervous system. They have long term curability if they are excised completely. If not, they can recur after a prolonged period and can lead to increased morbidity during re-surgery. Recurrence is rarely associated with invasiveness. Usually de-differentiation in case of meningiomas is uncommon without any predisposing factors including different genetic mutations or radiation to the involved region. We report a case of a 38-year-old female who was operated for a benign para-sagittal meningioma 8 years back and subsequently developed an invasive recurrence off late. Also this time, the imaging morphology was slightly different for a meningioma and gross as well as microscopic findings were very atypical. Awareness for such cases must be there while dealing with recurrent meningiomas as invasiveness may not always be associated with adverse predisposing factors like radiation. As invasiveness is always a histopathological diagnosis, picking up such features on imaging is a daunting task and if done, can help neurosurgeons prognosticate such invasive recurrences in a better fashion. PMID:27366271

  4. Atypical Bilateral Fuchs Uveitis: Diagnostic Challenges

    PubMed Central

    Couto, Cristóbal; Hurtado, Erika; Faingold, Dana; Demetrio, Carmen; Schlaen, Ariel; Zas, Marcelo; Zarate, Jorge; Rosetti, Silvia; de Lima, Andrea Paes; Croxatto, Juan Oscar; Chiaradía, Pablo; Burnier, Miguel N.

    2015-01-01

    Bilateral Fuchs uveitis associated with vitreous infiltration and posterior segment involvement requires a thorough diagnostic evaluation. The lack of well-defined diagnostic criteria makes identification of this entity difficult. The aim of this case report was to present the characteristics of a patient with atypical Fuchs uveitis and the procedures needed to rule out the differential diagnosis with specific attention to the utility of in vivo confocal microscopy (IVCM). Case Report One case of chronic bilateral uveitis with severe vitreous opacities is presented. After extensive systemic workup, including vitrectomy, the case had no identifiable systemic etiology. IVCM of the cornea revealed the presence of dendritiform keratic precipitates. Conclusion The diagnosis of Fuchs uveitis is based on clinical findings as no confirmatory laboratory tests are available. A high index of suspicion is key to an early diagnosis, especially in the cases with vitreous opacities and posterior segment manifestations. Auxiliary tests such as IVCM may aid the clinician in the diagnosis of Fuchs uveitis. PMID:26483668

  5. Clinical pharmacology of atypical antipsychotics: an update

    PubMed Central

    Mauri, M.C.; Paletta, S.; Maffini, M.; Colasanti, A.; Dragogna, F.; Di Pace, C.; Altamura, A.C.

    2014-01-01

    This review will concentrate on the clinical pharmacology, in particular pharmacodynamic data, related to atypical antipsychotics, clozapine, risperidone, paliperidone, olanzapine, que¬tiapine, amisulpride, ziprasidone, aripiprazole, asenapine, iloperidone, lurasidone and cariprazine. A summary of their acute pharmacokinetics properties are also reported. Four new second-generation antipsychotics are available: iloperidone, asenapine, lurasidone and in the next future cariprazine. Similar to ziprasidone and aripiprazole, these new agents are advisable for the lower propensity to give weight gain and metabolic abnormalities in comparison with older second-generation antipsychotics such as olanzapine or clozapine. Actually lurasidone seems to be best in terms of minimizing unwanted alterations in body weight and metabolic variables. Therapeutic drug monitoring is not strictly necessary for all of the new antipsychotic drugs because there are no unequivocal data supporting a relationship between plasma drug levels and clinical outcomes or side effects. The exception can be represented by clozapine for which plasma levels of 350-420 ng/ml are reported to be associated with an increased probability of a good clinical response. Also for olanzapine an established therapeutic range (20-50 ng/ml) is proposed to yield an optimal response and minimize side effects. PMID:26417330

  6. Atypical presentation of mucopolysaccharidosis type IVA.

    PubMed

    Rush, Eric T

    2016-09-01

    A 14 year old patient with short stature, type I diabetes, and cataracts was referred for evaluation of avascular necrosis of the femoral head. Radiography was suggestive of spondyloepiphyseal dysplasia with decreased bone mineral density for age. Targeted molecular and biochemical testing were normal in this patient. Whole exome sequencing was performed and showed compound heterozygosity for previously reported pathogenic GALNS variants which were diagnostic of mucopolysaccharidosis, type IVA (Morquio A). While this case describes neither a novel condition nor a new mutation, it does illustrate three important points in the diagnosis of patients with atypical forms of MPS IVA. First, that in many instances urine glycosaminoglycan analysis is not sufficient to rule out MPS IVA as a potential diagnosis. Patients in whom biochemical screening is advised should have measurement of leukocyte enzymatic activity. Second, that in patients with radiographic evidence of spondyloepiphyseal dysplasia with additional features or with normal targeted testing, MPS IVA should remain in the differential diagnosis. Third, that whole exome sequencing represents a viable diagnostic platform for evaluation of patients with unknown skeletal or metabolic disease. PMID:27331011

  7. Wnt pathway in atypical teratoid rhabdoid tumors

    PubMed Central

    Chakravadhanula, Madhavi; Hampton, Chris N.; Chodavadia, Parth; Ozols, Victor; Zhou, Li; Catchpoole, Daniel; Xu, Jingying; Erdreich-Epstein, Anat; Bhardwaj, Ratan D.

    2015-01-01

    Background Atypical teratoid rhabdoid tumor (ATRT) is an aggressive pediatric brain tumor with limited therapeutic options. The hypothesis for this study was that the Wnt pathway triggered by the Wnt5B ligand plays an important role in ATRT biology. To address this hypothesis, the role of WNT5B and other Wnt pathway genes was analyzed in ATRT tissues and ATRT primary cell lines. Methods Transcriptome-sequencing analyses were performed using nanoString platforms, immunohistochemistry, Western blotting, quantitative reverse transcriptase PCR, immunoprecipitation, short interference RNA studies, cell viability studies, and drug dose response (DDR) assays. Results Our transcriptome-sequencing results of Wnt pathway genes from ATRT tissues and cell lines indicated that the WNT5B gene is significantly upregulated in ATRT samples compared with nontumor brain samples. These results also indicated a differential expression of both canonical and noncanonical Wnt genes. Imunoprecipitation studies indicated that Wnt5B binds to Frizzled1 and Ryk receptors. Inhibition of WNT5B by short interference RNA decreased the expression of FRIZZLED1 and RYK. Cell viability studies a indicated significant decrease in cell viability by inhibiting Frizzled1 receptor. DDR assays showed promising results with some inhibitors. Conclusions These promising therapeutic options will be studied further before starting a translational clinical trial. The success of these options will improve care for these patients. PMID:25246426

  8. Atypical and ischemic features of embolized meningiomas.

    PubMed

    Matsuda, Ken; Takeuchi, Hiroaki; Arai, Yoshikazu; Kitai, Ryuhei; Hosoda, Tetsuya; Tsunetoshi, Kenzo; Arishima, Hidetaka; Sato, Kazufumi; Kikuta, Ken-Ichiro

    2012-01-01

    Preoperative embolization (POE) of meningiomas is widely used to facilitate surgical removal and to reduce intraoperative blood loss. The resulting necrosis and enhanced proliferation have been reported to affect subsequent histologic grading. However, there was little concern about ischemic features, for example small cells resembling atypical meningiomas, cytoplasmic vacuoles resembling clear cell meningioma, intercellular discohesion resembling rhabdoid meningioma, and perivascular cuffs resembling papillary meningioma. Therefore, the extent of these ischemic features was scored and Ki-67 staining indices were investigated in a POE group composed of 29 specimens of meningiomas treated with POE and compared with equivalent results for a non-POE group composed of 29 meningiomas that were not treated with POE. Small cells with high N/C ratios, cytoplasmic vacuoles, intercellular discohesion, and perivascular cuffs were significantly increased in the POE group (versus the non-POE group, p < 0.05). There were no significant differences of the Ki-67 index between the POE group (2.2%) and the non-POE group (1.9%) (p = 0.49). Our results suggest that small cell change resulting in necrosis may be followed by POE, and that clear cell-like, rhabdoid cell-like, or pseudopapillary pattern identified in meningiomas may also be induced by POE. Therefore, histological findings and determination of grading should be evaluated cautiously in cases of embolized meningiomas. PMID:21789536

  9. Laser trapping of 225Ra and 226Ra with repumping by room-temperature blackbody radiation.

    PubMed

    Guest, J R; Scielzo, N D; Ahmad, I; Bailey, K; Greene, J P; Holt, R J; Lu, Z-T; O'Connor, T P; Potterveld, D H

    2007-03-01

    We have demonstrated Zeeman slowing and capture of neutral 225Ra and 226Ra atoms in a magneto-optical trap. The intercombination transition 1S0-->3P1 is the only quasicycling transition in radium and was used for laser-cooling and trapping. Repumping along the 3D1-->1P1 transition extended the lifetime of the trap from milliseconds to seconds. Room-temperature blackbody radiation was demonstrated to provide repumping from the metastable 3P0 level. We measured the isotope shift and hyperfine splittings on the 3D1-->1P1 transition with the laser-cooled atoms, and set a limit on the lifetime of the 3D1 level based on the measured blackbody repumping rate. Laser-cooled and trapped radium is an attractive system for studying fundamental symmetries. PMID:17359153

  10. Determination of gross alpha, 224Ra, 226Ra, and 228Ra activities in drinking water using a single sample preparation procedure.

    PubMed

    Parsa, Bahman; Obed, Reynaldo N; Nemeth, William K; Suozzo, Gail P

    2005-12-01

    The current federal and New Jersey State regulations have greatly increased the number of gross alpha and radium tests for public and private drinking water supplies. The determination of radium isotopes in water generally involves lengthy and complicated processes. In this study, a new approach is presented for the determination of gross alpha, 224Ra, 226Ra, and 228Ra activities in water samples. The method includes a single sample preparation procedure followed by alpha counting and gamma-ray spectroscopy. The sample preparation technique incorporates an EPA-approved co-precipitation methodology for gross alpha determination with a few alterations and improvements. Using 3-L aliquots of sample, spiked with 133Ba tracer, the alpha-emitting radionuclides are isolated by a BaSO4 and Fe(OH)3 co-precipitation scheme. First the gross alpha-particle activity of the sample is measured with a low-background gas-flow proportional counter, followed by radium isotopes assay by gamma-ray spectroscopy, using the same prepared sample. Gamma-ray determination of 133Ba tracer is used to assess the radium chemical recovery. The 224Ra, 226Ra, and 228Ra activities in the sample are measured through their gamma-ray-emitting decay products, 212Pb, 214Pb/214Bi, and 228Ac, respectively. In cases where 224Ra determination is required, the gamma-ray counting should be performed within 2-4 d from sample collection. To measure 226Ra activity in the sample, the gamma-ray spectroscopy can be repeated 21 d after sample preparation to ensure that 226Ra and its progeny have reached the equilibrium state. At this point, the 228Ac equilibration with parent 228Ra is already established. Analysis of aliquots of de-ionized water spiked with NIST-traceable 230Th, 224Ra, 226Ra, and 228Ra standards demonstrated the accuracy and precision of this method. Various performance evaluation samples were also assayed for gross alpha as well as radium isotope activity determination using this procedure and the

  11. Paraprofessional Staff in Transition: The Sophomore RA Experience

    ERIC Educational Resources Information Center

    Brandt Brecheisen, Shannon M.

    2015-01-01

    The sophomore resident assistant (RA) experience exists at the point where the sophomore experience and the RA position intersect. Sophomore students contend with specific transition struggles and challenges unique to their class standing as they make decisions about their sense of purpose and future career, and those who choose to become RAs face…

  12. First Japanese case of atypical progeroid syndrome/atypical Werner syndrome with heterozygous LMNA mutation.

    PubMed

    Motegi, Sei-ichiro; Yokoyama, Yoko; Uchiyama, Akihiko; Ogino, Sachiko; Takeuchi, Yuko; Yamada, Kazuya; Hattori, Tomoyasu; Hashizume, Hiroaki; Ishikawa, Yuichi; Goto, Makoto; Ishikawa, Osamu

    2014-12-01

    Atypical progeroid syndrome (APS), including atypical Werner syndrome (AWS), is a progeroid syndrome involving heterozygous mutations in the LMNA gene encoding the nuclear protein lamin A/C. We report the first Japanese case of APS/AWS with a LMNA mutation (p.D300N). A 53-year-old Japanese man had a history of recurrent severe cardiovascular diseases as well as brain infarction and hemorrhages. Although our APS/AWS patient had overlapping features with Werner syndrome (WS), such as high-pitched voice, scleroderma, lipoatrophy and atherosclerosis, several cardinal features of WS, including short stature, premature graying/alopecia, cataract, bird-like face, flat feet, hyperkeratosis on the soles and diabetes mellitus, were absent. In immunofluorescence staining and electron microscopic analyses of the patient's cultured fibroblasts, abnormal nuclear morphology, an increase in small aggregation of heterochromatin and a decrease in interchromatin granules in nuclei of fibroblasts were observed, suggesting that abnormal nuclear morphology and chromatin disorganization may be associated with the pathogenesis of APS/AWS. PMID:25327215

  13. The use of atypical antipsychotics in the management of schizophrenia

    PubMed Central

    Campbell, M; Young, P I; Bateman, D N; Smith, J M; Thomas, S H L

    1999-01-01

    Long-term drug treatment of schizophrenia with conventional antipsychotics has limitations: an estimated quarter to one third of patients are treatment-resistant; conventional antipsychotics have only a modest impact upon negative symptoms (poverty of thought, social withdrawal and loss of affect); and adverse effects, particularly extrapyramidal symptoms (EPS). Newer, so-called atypical, antipsychotics such as olanzapine, risperidone, sertindole and clozapine (an old drug which was re-introduced in 1990) are claimed to address these limitations. Atypical agents are, at a minimum, at least as effective as conventional drugs such as haloperidol. They also cause substantially fewer extrapyramidal symptoms. However, some other adverse effects are more common than with conventional drugs. For example, clozapine carries a significant risk of serious blood disorders, for which special monitoring is mandatory; it also causes troublesome drowsiness and increased salivation more often than conventional agents. Some atypical agents cause more weight gain or QT prolongation than older agents. The choice of therapy is, therefore, not straightforward. At present, atypical agents represent an advance for patients with severe or intolerable EPS. Most published evidence exists to support the use of clozapine, which has also been shown to be effective in schizophrenia refractory to conventional agents. However, the need for compliance with blood count monitoring and its sedative properties make careful patient selection important. The extent of any additional direct benefit offered by atypical agents on negative symptoms is not yet clear. The lack of a depot formulation for atypical drugs may pose a significant practical problem. To date, only two double-blind studies in which atypical agents were compared directly have been published. Neither provides compelling evidence for the choice of one agent over another. Atypical agents are many times more expensive than conventional drugs

  14. Amisulpride versus other atypical antipsychotics for schizophrenia

    PubMed Central

    Komossa, Katja; Rummel-Kluge, Christine; Hunger, Heike; Schmid, Franziska; Schwarz, Sandra; da Mota Neto, Joaquim I Silveira; Kissling, Werner; Leucht, Stefan

    2014-01-01

    Background In many countries of the industrialised world second generation (atypical) antipsychotics have become first line drug treatments for people with schizophrenia. The question as to whether, and if so how much, the effects of the various second generation antipsychotics differ is a matter of debate. In this review we examine how the efficacy and tolerability of amisulpride differs from that of other second generation antipsychotics. Objectives To evaluate the effects of amisulpride compared with other atypical antipsychotics for people with schizophrenia and schizophrenia-like psychoses. Search methods We searched the Cochrane Schizophrenia Group Trials Register (April 2007) which is based on regular searches of BIOSIS, CINAHL, EMBASE, MEDLINE and PsycINFO. We updated this search in July 2012 and added 47 new trials to the awaiting classification section. Selection criteria We included randomised, at least single-blind, trials comparing oral amisulpride with oral forms of aripiprazole, clozapine, olanzapine, quetiapine, risperidone, sertindole, ziprasidone or zotepine in people with schizophrenia or schizophrenia-like psychoses. Data collection and analysis We extracted data independently. For continuous data we calculated weighted mean differences (MD), for dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. Main results The review currently includes ten short to medium term trials with 1549 participants on three comparisons: amisulpride versus olanzapine, risperidone and ziprasidone. The overall attrition rate was considerable (34.7%) with no significant difference between groups. Amisulpride was similarly effective as olanzapine and risperidone and more effective than ziprasidone (leaving the study early due to inefficacy: n=123, 1 RCT, RR 0.21 CI 0.05 to 0.94, NNT 8 CI 5 to 50

  15. Current treatment of atypical hemolytic uremic syndrome

    PubMed Central

    Kaplan, Bernard S.; Ruebner, Rebecca L.; Spinale, Joann M.; Copelovitch, Lawrence

    2014-01-01

    Summary Tremendous advances have been made in understanding the pathogenesis of atypical Hemolytic Uremic Syndrome (aHUS), an extremely rare disease. Insights into the molecular biology of aHUS resulted in rapid advances in treatment with eculizumab (Soliris®, Alexion Pharmaceuticals Inc.). Historically, aHUS was associated with very high rates of mortality and morbidity. Prior therapies included plasma therapy and/or liver transplantation. Although often life saving, these were imperfect and had many complications. We review the conditions included under the rubric of aHUS: S. pneumoniae HUS (SpHUS), inborn errors of metabolism, and disorders of complement regulation, emphasizing their differences and similarities. We focus on the clinical features, diagnosis, and pathogenesis, and treatment of aHUS that results from mutations in genes encoding alternative complement regulators, SpHUS and HUS associated with inborn errors of metabolism. Mutations in complement genes, or antibodies to their protein products, result in unregulated activity of the alternate complement pathway, endothelial injury, and thrombotic microangiopathy (TMA). Eculizumab is a humanized monoclonal antibody that inhibits the production of the terminal complement components C5a and the membrane attack complex (C5b-9) by binding to complement protein C5a. This blocks the proinflammatory and cytolytic effects of terminal complement activation. Eculizumab use has been reported in many case reports, and retrospective and prospective clinical trials in aHUS. There have been few serious side effects and no reports of tachphylaxis or drug resistance. The results are very encouraging and eculizumab is now recognized as the treatment of choice for aHUS. PMID:25343125

  16. Clozapine versus other atypical antipsychotics for schizophrenia

    PubMed Central

    Asenjo Lobos, Claudia; Komossa, Katja; Rummel-Kluge, Christine; Hunger, Heike; Schmid, Franziska; Schwarz, Sandra; Leucht, Stefan

    2014-01-01

    Background Clozapine is an atypical antipsychotic demonstrated to be superior in the treatment of refractory schizophrenia which causes fewer movement disorders. Clozapine, however, entails a significant risk of serious blood disorders such as agranulocytosis which could be potentially fatal. Currently there are a number of newer antipsychotics which have been developed with the purpose to find both a better tolerability profile and a superior effectiveness. Objectives To compare the clinical effects of clozapine with other atypical antipsychotics (such as amisulpride, aripiprazole, olanzapine, quetiapine, risperidone, sertindole, ziprasidone and zotepine) in the treatment of schizophrenia and schizophrenia-like psychoses. Search methods We searched the Cochrane Schizophrenia Groups Register (June 2007) and reference lists of all included randomised controlled trials. We also manually searched appropriate journals and conference proceedings relating to clozapine combination strategies and contacted relevant pharmaceutical companies. Selection criteria All relevant randomised, at least single-blind trials, comparing clozapine with other atypical antipsychotics, any dose and oral formulations, for people with schizophrenia or related disorders. Data collection and analysis We selected trials and extracted data independently. For dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) based on a random-effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. For continuous data, we calculated mean differences (MD) again based on a random-effects model. Main results The review currently includes 27 blinded randomised controlled trials, which involved 3099 participants. Twelve randomised control trials compared clozapine with olanzapine, five with quetiapine, nine with risperidone, one with ziprasidone and two with zotepine. Attrition from these studies was high (overall 30.1%), leaving the interpretation

  17. The Atypical Response Regulator Protein ChxR Has Structural Characteristics and Dimer Interface Interactions That Are Unique within the OmpR/PhoB Subfamily

    SciTech Connect

    Hickey, John M.; Lovell, Scott; Battaile, Kevin P.; Hu, Lei; Middaugh, C. Russell; Hefty, P. Scott

    2013-05-29

    Typically as a result of phosphorylation, OmpR/PhoB response regulators form homodimers through a receiver domain as an integral step in transcriptional activation. Phosphorylation stabilizes the ionic and hydrophobic interactions between monomers. Recent studies have shown that some response regulators retain functional activity in the absence of phosphorylation and are termed atypical response regulators. The two currently available receiver domain structures of atypical response regulators are very similar to their phospho-accepting homologs, and their propensity to form homodimers is generally retained. An atypical response regulator, ChxR, from Chlamydia trachomatis, was previously reported to form homodimers; however, the residues critical to this interaction have not been elucidated. We hypothesize that the intra- and intermolecular interactions involved in forming a transcriptionally competent ChxR are distinct from the canonical phosphorylation (activation) paradigm in the OmpR/PhoB response regulator subfamily. To test this hypothesis, structural and functional studies were performed on the receiver domain of ChxR. Two crystal structures of the receiver domain were solved with the recently developed method using triiodo compound I3C. These structures revealed many characteristics unique to OmpR/PhoB subfamily members: typical or atypical. Included was the absence of two {alpha}-helices present in all other OmpR/PhoB response regulators. Functional studies on various dimer interface residues demonstrated that ChxR forms relatively stable homodimers through hydrophobic interactions, and disruption of these can be accomplished with the introduction of a charged residue within the dimer interface. A gel shift study with monomeric ChxR supports that dimerization through the receiver domain is critical for interaction with DNA.

  18. Atypical Hemolytic-Uremic Syndrome: A Clinical Review.

    PubMed

    Nayer, Ali; Asif, Arif

    2016-01-01

    Atypical hemolytic-uremic syndrome (HUS) is a rare life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and ischemic injury to organs, especially the kidneys. Microvascular injury and thrombosis are the dominant histologic findings. Complement activation through the alternative pathway plays a critical role in the pathogenesis of atypical HUS. Genetic abnormalities involving complement regulatory proteins and complement components form the molecular basis for complement activation. Endothelial cell dysfunction, probably because of the effects of complement activation, is an intermediate stage in the pathophysiologic cascade. Atypical HUS has a grave prognosis. Although mortality approaches 25% during the acute phase, end-stage renal disease develops in nearly half of patients within a year. Atypical HUS has a high recurrence rate after renal transplantation, and recurrent disease often leads to graft loss. Plasma therapy in the form of plasma exchange or infusion has remained the standard treatment for atypical HUS. However, many patients do not respond to plasma therapy and some require prolonged treatment. Approved by the Food and Drug Administration in the treatment of atypical HUS, eculizumab is a humanized monoclonal antibody that blocks cleavage of complement C5 into biologically active mediators of inflammation and cytolysis. Although case reports have shown the efficacy of eculizumab, randomized clinical trials are lacking. Therapeutic strategies targeting endothelial cells have demonstrated promising results in experimental settings. Therefore, inhibitors of angiotensin-converting enzyme, HMG-CoA reductase, and xanthine oxidase as well as antioxidants, such as ascorbic acid, may have salutary effects in patients with atypical HUS. PMID:24681522

  19. Childhood Learning Disabilities and Atypical Dementia: A Retrospective Chart Review

    PubMed Central

    Seifan, Alon

    2015-01-01

    Objective To further our understanding of the association between self-reported childhood learning disabilities (LDs) and atypical dementia phenotypes (Atypical Dementia), including logopenic primary progressive aphasia (L-PPA), Posterior Cortical Atrophy (PCA), and Dysexecutive-type Alzheimer’s Disease (AD). Methods This retrospective case series analysis of 678 comprehensive neuropsychological assessments compared rates of self-reported LD between dementia patients diagnosed with Typical AD and those diagnosed with Atypical Dementia. 105 cases with neuroimaging or CSF data available and at least one neurology follow-up were identified as having been diagnosed by the neuropsychologist with any form of neurodegenerative dementia. These cases were subject to a consensus diagnostic process among three dementia experts using validated clinical criteria for AD and PPA. LD was considered Probable if two or more statements consistent with prior LD were documented within the Social & Developmental History of the initial neuropsychological evaluation. Results 85 subjects (Typical AD n=68, Atypical AD n=17) were included in the final analysis. In logistic regression models adjusted for age, gender, handedness, education and symptom duration, patients with Probable LD, compared to patients without Probable LD, were significantly more likely to be diagnosed with Atypical Dementia vs. Typical AD (OR 13.1, 95% CI 1.3-128.4). All three of the L-PPA cases reporting a childhood LD endorsed childhood difficulty with language. By contrast, both PCA cases reporting Probable childhood LD endorsed difficulty with attention and/or math. Conclusions In people who develop dementia, childhood LD may predispose to atypical phenotypes. Future studies are required to confirm whether atypical neurodevelopment predisposes to regional-specific neuropathology in AD and other dementias. PMID:26106899

  20. Measurement of 224Ra and 226Ra activities in natural waters using a radon-in-air monitor

    USGS Publications Warehouse

    Kim, G.; Burnett, W.C.; Dulaiova, H.; Swarzenski, P.W.; Moore, W.S.

    2001-01-01

    We report a simple new technique for measuring low-level radium isotopes (224Ra and 226Ra) in natural waters. The radium present in natural waters is first preconcentrated onto MnO2-coated acrylic fiber (Mn fiber) in a column mode. The radon produced from the adsorbed radium is then circulated through a closed air-loop connected to a commercial radon-in-air monitor. The monitor counts alpha decays of radon daughters (polonium isotopes) which are electrostatically collected onto a silicon semiconductor detector. Count data are collected in energy-specific windows, which eliminate interference and maintain very low backgrounds. Radium-224 is measured immediately after sampling via 220Rn (216Po), and 226Ra is measured via 222Rn (218Po) after a few days of ingrowth of 222Rn. This technique is rapid, simple, and accurate for measurements of low-level 224Ra and 226Ra activities without requiring any wet chemistry. Rapid measurements of short-lived 222Rn and 224Ra, along with long-lived 226Ra, may thus be made in natural waters using a single portable system for environmental monitoring of radioactivity as well as tracing of various geochemical and geophysical processes. The technique could be especially useful for the on-site rapid determination of 224Ra which has recently been found to occur at elevated activities in some groundwater wells.

  1. Rainfall Manipulation Plot Study (RaMPS)

    DOE Data Explorer

    Blair, John [Kansas State University; Fay, Phillip [USDA-ARS; Knapp, Alan [Colorado State University; Collins, Scott [University of New Mexico; Smith, Melinda [Yale University

    Rainfall Manipulation Plots facility (RaMPs) is a unique experimental infrastructure that allows us to manipulate precipitation events and temperature, and assess population community, and ecosystem responses in native grassland. This facility allows us to manipulate the amount and timing of individual precipitation events in replicated field plots at the Konza Prairie Long-Term Ecological Research (LTER) site. Questions we are addressing include: • What is the relative importance of more extreme precipitation patterns (increased climatic variability) vs. increased temperatures (increased climatic mean) with regard to their impact on grassland ecosystem structure and function? Both projected climate change factors are predicted to decrease soil water availability, but the mechanisms by which this resource depletion occurs differ. • Will altered precipitation patterns, increased temperatures and their interaction increase opportunities for invasion by exotic species? • Will long-term (6-10 yr) trajectories of community and ecosystem change in response to more extreme precipitation patterns continue at the same rate as initial responses from years 1-6? Or will non-linear change occur as potential ecological thresholds are crossed? And will increased temperatures accelerate these responses? Data sets are available as ASCII files, in Excel spreadsheets, and in SAS format. (Taken from http://www.konza.ksu.edu/ramps/backgrnd.html

  2. A CC′ Loop Decoy Peptide Blocks the Interaction Between Act1 and IL-17RA to Attenuate IL-17- and IL-25-Induced Inflammation

    PubMed Central

    Liu, Caini; Swaidani, Shadi; Qian, Wen; Kang, Zizhen; Sun, Paige; Han, Yue; Wang, Chenhui; Gulen, Muhammet Fatih; Yin, Weiguo; Zhang, Chunjiang; Fox, Paul L; Aronica, Mark; Hamilton, Thomas A; Misra, Saurav; Deng, Junpeng; Li, Xiaoxia

    2012-01-01

    Interleukin-17 (IL-17) and IL-25 signaling induce the expression of genes that encode inflammatory factors and they are implicated in the pathology of various inflammatory diseases. Nuclear factor κB (NF-κB) activator 1 (Act1) is an adaptor protein and E3 ubiquitin ligase that is critical for IL-17 and IL-25 signaling, and it is recruited to their receptors through heterotypic interactions between their SEFIR [SEF (similar expression to fibroblast growth factor genes)/IL-17R] domains. Modeling of SEFIR domains has shown their structural similarity with the Toll-IL-1 receptor (TIR) domains of Toll-like receptors (TLRs) and the IL-1R. Whereas the BB′ loop of TIR is required for TIR-TIR interactions, we found that deletion of the BB′ loop from Act1 or IL-17RA (a common subunit of IL-17R and IL-25R) did not affect Act1–IL-17RA interactions. Instead, deletion of the CC′ loop from Act1 or IL-17RA abolished the interaction between Act1 and IL-17RA, suggesting that SEFIR and TIR domains interact in different manners. Surface plasmon resonance measurements showed that a peptide corresponding to the CC′ loop bound directly to IL-17RA. A cell-permeable decoy peptide based on the CC′ loop sequence inhibited IL-17- and IL-25-mediated signaling, and it inhibited IL-17- and IL-25-induced responses in vitro and pulmonary inflammation in vivo. Together, these findings provide the molecular basis for the specificity of SEFIR versus TIR domain interactions and consequent signaling. Moreover, we suggest that the CC′ loop motif of SEFIR domains is a promising target for therapeutic strategies against IL-17- and IL-25-asssociated inflammatory diseases. PMID:22045852

  3. Coelimination and Survival in Gene Network Evolution: Dismantling the RA-Signaling in a Chordate.

    PubMed

    Martí-Solans, Josep; Belyaeva, Olga V; Torres-Aguila, Nuria P; Kedishvili, Natalia Y; Albalat, Ricard; Cañestro, Cristian

    2016-09-01

    The bloom of genomics is revealing gene loss as a pervasive evolutionary force generating genetic diversity that shapes the evolution of species. Outside bacteria and yeast, however, the understanding of the process of gene loss remains elusive, especially in the evolution of animal species. Here, using the dismantling of the retinoic acid metabolic gene network (RA-MGN) in the chordate Oikopleura dioica as a case study, we combine approaches of comparative genomics, phylogenetics, biochemistry, and developmental biology to investigate the mutational robustness associated to biased patterns of gene loss. We demonstrate the absence of alternative pathways for RA-synthesis in O. dioica, which suggests that gene losses of RA-MGN were not compensated by mutational robustness, but occurred in a scenario of regressive evolution. In addition, the lack of drastic phenotypic changes associated to the loss of RA-signaling provides an example of the inverse paradox of Evo-Devo. This work illustrates how the identification of patterns of gene coelimination-in our case five losses (Rdh10, Rdh16, Bco1, Aldh1a, and Cyp26)-is a useful strategy to recognize gene network modules associated to distinct functions. Our work also illustrates how the identification of survival genes helps to recognize neofunctionalization events and ancestral functions. Thus, the survival and extensive duplication of Cco and RdhE2 in O. dioica correlated with the acquisition of complex compartmentalization of expression domains in the digestive system and a process of enzymatic neofunctionalization of the Cco, while the surviving Aldh8 could be related to its ancestral housekeeping role against toxic aldehydes. PMID:27406791

  4. Ubiquitin plays an atypical role in GPCR-induced p38 MAP kinase activation on endosomes

    PubMed Central

    Grimsey, Neil J.; Aguilar, Berenice; Smith, Thomas H.; Le, Phillip; Soohoo, Amanda L.; Puthenveedu, Manojkumar A.; Nizet, Victor

    2015-01-01

    Protease-activated receptor 1 (PAR1) is a G protein–coupled receptor (GPCR) for thrombin and promotes inflammatory responses through multiple pathways including p38 mitogen-activated protein kinase signaling. The mechanisms that govern PAR1-induced p38 activation remain unclear. Here, we define an atypical ubiquitin-dependent pathway for p38 activation used by PAR1 that regulates endothelial barrier permeability. Activated PAR1 K63-linked ubiquitination is mediated by the NEDD4-2 E3 ubiquitin ligase and initiated recruitment of transforming growth factor-β–activated protein kinase-1 binding protein-2 (TAB2). The ubiquitin-binding domain of TAB2 was essential for recruitment to PAR1-containing endosomes. TAB2 associated with TAB1, which induced p38 activation independent of MKK3 and MKK6. The P2Y1 purinergic GPCR also stimulated p38 activation via NEDD4-2–mediated ubiquitination and TAB1–TAB2. TAB1–TAB2-dependent p38 activation was critical for PAR1-promoted endothelial barrier permeability in vitro, and p38 signaling was required for PAR1-induced vascular leakage in vivo. These studies define an atypical ubiquitin-mediated signaling pathway used by a subset of GPCRs that regulates endosomal p38 signaling and endothelial barrier disruption. PMID:26391660

  5. Atypical Rho GTPases of the RhoBTB Subfamily: Roles in Vesicle Trafficking and Tumorigenesis

    PubMed Central

    Ji, Wei; Rivero, Francisco

    2016-01-01

    RhoBTB proteins constitute a subfamily of atypical Rho GTPases represented in mammals by RhoBTB1, RhoBTB2, and RhoBTB3. Their characteristic feature is a carboxyl terminal extension that harbors two BTB domains capable of assembling cullin 3-dependent ubiquitin ligase complexes. The expression of all three RHOBTB genes has been found reduced or abolished in a variety of tumors. They are considered tumor suppressor genes and recent studies have strengthened their implication in tumorigenesis through regulation of the cell cycle and apoptosis. RhoBTB3 is also involved in retrograde transport from endosomes to the Golgi apparatus. One aspect that makes RhoBTB proteins atypical among the Rho GTPases is their proposed mechanism of activation. No specific guanine nucleotide exchange factors or GTPase activating proteins are known. Instead, RhoBTB might be activated through interaction with other proteins that relieve their auto-inhibited conformation and inactivated through auto-ubiquitination and destruction in the proteasome. In this review we discuss our current knowledge on the molecular mechanisms of action of RhoBTB proteins and the implications for tumorigenesis and other pathologic conditions. PMID:27314390

  6. Symptomatic atypical femoral fractures are related to underlying hip geometry.

    PubMed

    Taormina, David P; Marcano, Alejandro I; Karia, Raj; Egol, Kenneth A; Tejwani, Nirmal C

    2014-06-01

    The benefits of bisphosphonates are well documented, but prolonged use has been associated with atypical femur fractures. Radiographic markers for fracture predisposition could potentially aid in safer medication use. In this case-control designed study, we compared hip radiographic parameters and the demographic characteristics of chronic bisphosphonate users who sustained an atypical femoral fracture with a group of chronic bisphosphonate users who did not sustain an atypical femur fracture and also a group who sustained an intertrochanteric hip fracture. Radiographic parameters included were neck-shaft angle (NSA), hip-axis length (HAL) and center-edge angle (CE). Multivariate regression was used to evaluate the relationship between radiographic measures and femur fracture. Receiver-operating characteristic analysis determined cut-off points for neck-shaft angle and risk of atypical femur fracture. Ultimately, pre-fracture radiographs of 53 bisphosphonate users who developed atypical fracture were compared with 43 asymptomatic chronic bisphosphonate users and 64 intertrochanteric fracture patients. Duration of bisphosphonate use did not statistically differ between users sustaining atypical fracture and those without fracture (7.9 [±3.5] vs. 7.7 [±3.3] years, p=0.7). Bisphosphonate users who fractured had acute/varus pre-fracture neck-shaft angles (p<0.001), shorter hip-axis length (p<0.01), and narrower center-edge angles (p<0.01). Regression analysis revealed associations between neck-shaft angle (OR=0.89 [95% CI=0.81-0.97; p=0.01), center edge angle (OR=0.89 [95% CI=0.80-0.99]; p=0.03), and BMI (OR=1.15 [95% CI=1.02-1.31; p=0.03) with fracture development. ROC curve analysis (AUC=0.67 [95% CI=0.56-0.79]) determined that a cut-off point for neck-shaft angle <128.3° yielded 69% sensitivity and 63% specificity for development of atypical femoral fracture. Ultimately, an acute/varus angle of the femoral neck, high BMI, and narrow center-edge angle were

  7. Imaging of atypical hemangiomas of the liver with pathologic correlation.

    PubMed

    Vilgrain, V; Boulos, L; Vullierme, M P; Denys, A; Terris, B; Menu, Y

    2000-01-01

    Compared with the imaging features of typical hepatic hemangiomas, the imaging features of atypical hepatic hemangiomas have not been well studied or well described. Knowledge of the entire spectrum of atypical hepatic hemangiomas is important and can help one avoid most diagnostic errors. A frequent type of atypical hepatic hemangioma is a lesion with an echoic border at ultrasonography. Less frequent types are large, heterogeneous hemangiomas; rapidly filling hemangiomas; calcified hemangiomas; hyalinized hemangiomas; cystic or multilocular hemangiomas; hemangiomas with fluid-fluid levels; and pedunculated hemangiomas. Adjacent abnormalities consist of arterial-portal venous shunt, capsular retraction, and surrounding nodular hyperplasia; hemangiomas can also develop in cases of fatty liver infiltration. Associated lesions include multiple hemangiomas, hemangiomatosis, focal nodular hyperplasia, and angiosarcoma. Types of atypical evolution are hemangiomas enlarging over time and hemangiomas appearing during pregnancy. Complications consist of inflammation, Kasabach-Merritt syndrome, intratumoral hemorrhage, hemoperitoneum, volvulus, and compression of adjacent structures. In some cases, such as large heterogeneous hemangiomas, calcified hemangiomas, pedunculated hemangiomas, or hemangiomas developing in diffuse fatty liver, a specific diagnosis can be established with imaging, especially magnetic resonance imaging. However, in other atypical cases, the diagnosis will remain uncertain at imaging, and these cases will require histopathologic examination. PMID:10715338

  8. Association of a Bovine Prion Gene Haplotype with Atypical BSE

    PubMed Central

    Clawson, Michael L.; Richt, Juergen A.; Baron, Thierry; Biacabe, Anne-Gaëlle; Czub, Stefanie; Heaton, Michael P.; Smith, Timothy P. L.; Laegreid, William W.

    2008-01-01

    Background Atypical bovine spongiform encephalopathies (BSEs) are recently recognized prion diseases of cattle. Atypical BSEs are rare; approximately 30 cases have been identified worldwide. We tested prion gene (PRNP) haplotypes for an association with atypical BSE. Methodology/Principle Findings Haplotype tagging polymorphisms that characterize PRNP haplotypes from the promoter region through the three prime untranslated region of exon 3 (25.2 kb) were used to determine PRNP haplotypes of six available atypical BSE cases from Canada, France and the United States. One or two copies of a distinct PRNP haplotype were identified in five of the six cases (p = 1.3×10−4, two-tailed Fisher's exact test; CI95% 0.263–0.901, difference between proportions). The haplotype spans a portion of PRNP that includes part of intron 2, the entire coding region of exon 3 and part of the three prime untranslated region of exon 3 (13 kb). Conclusions/Significance This result suggests that a genetic determinant in or near PRNP may influence susceptibility of cattle to atypical BSE. PMID:18350166

  9. Atypical growth of Renibacterium salmoninarum in subclinical infections.

    PubMed

    Hirvelä-Koski, V; Pohjanvirta, T; Koski, P; Sukura, A

    2006-01-01

    Two growth types of Renibacterium salmoninarum were isolated from subclinically infected rainbow trout, one producing the smooth colonies typical of R. salmoninarum and the other forming a thin film on the surface of the agar with no separate colonies. The atypical growth was present on kidney disease medium agar in primary cultures of the kidney but not on selective kidney disease medium (SKDM). Fluorescent antibody staining of the fresh isolate and polymerase chain reaction amplification were the most reliable techniques to identify the atypical growth of R. salmoninarum. The condition was reversible, with growth reverting from atypical to the smooth colony form in experimentally infected rainbow trout and under laboratory conditions. There was no mortality, or any clinical signs of bacterial kidney disease (BKD) in the fish challenged with the atypical growth, although small numbers of smooth colonies of R. salmoninarum were isolated from 8% of these fish. The atypical growth reported here may explain some of the failures of culture, when SKDM agar alone is used for the detection of BKD in subclinically infected fish. PMID:16351695

  10. Sertindole versus other atypical antipsychotics for schizophrenia

    PubMed Central

    Komossa, Katja; Rummel-Kluge, Christine; Hunger, Heike; Schwarz, Sandra; Schmid, Franziska; Lewis, Ruth; Kissling, Werner; Leucht, Stefan

    2014-01-01

    Background In many countries of the industrialised world second generation (atypical) antipsychotics have become the first line drug treatment for people with schizophrenia. The question as to whether and, if so, how much the effects of the various second generation antipsychotics differ is a matter of debate. Objectives To evaluate the effects of sertindole compared with other second generation antipsychotics for people with schizophrenia and schizophrenia-like psychosis. Search methods We searched the Cochrane Schizophrenia Group Trials Register (April 2007) and ClinicalTrials.gov (February 2009). Selection criteria We included all randomised trials comparing oral sertindole with oral forms of amisulpride, aripiprazole, clozapine, olanzapine, quetiapine, risperidone, ziprasidone or zotepine for people with schizophrenia or schizophrenia-like psychosis. Data collection and analysis We extracted data independently. For dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random-effects model. For continuous data, we calculated weighted mean differences (WMD) again based on a random-effects model. Main results The review currently includes two short-term low-quality randomised trials (total n=508) both comparing sertindole with risperidone. One third of participants left the studies early (2 RCTs, n=504, RR 1.23 CI 0.94 to 1.60). There was no difference in efficacy (2 RCTs, n=493, WMD PANSS total change from baseline 1.98 CI −8.24 to 12.20). Compared with relatively high doses of risperidone (between 4 and 12 mg/day), sertindole produced significantly less akathisia and parkinsonism (1 RCT, n=321, RR 0.24 CI 0.09 to 0.69, NNT 14, CI 8 to 100). Sertindole produced more cardiac effects (2 RCTs, n=508, RR QTc prolongation 4.86 CI 1.94 to 12.18), weight change (2 RCTs, n=328, WMD 0.99 CI 0.12 to 1.86) and male sexual dysfunction (2 RCTs, n=437, RR 2.90 CI 1.32 to 6.35, NNH 13 CI 8 to 33

  11. Caspase processing activates atypical protein kinase C zeta by relieving autoinhibition and destabilizes the protein.

    PubMed Central

    Smith, Lucinda; Wang, Zhi; Smith, Jeffrey B

    2003-01-01

    Treatment of HeLa cells with tumour necrosis factor alpha (TNFalpha) induced caspase processing of ectopic PKC (protein kinase C) zeta, which converted most of the holoenzyme into the freed kinase domain and increased immune-complex kinase activity. The goal of the present study was to determine the basis for the increased kinase activity that is associated with caspase processing of PKC zeta. Atypical PKC iota is largely identical with PKC zeta, except for a 60-amino-acid segment that lacks the caspase-processing sites of the zeta isoform. Replacement of this segment of PKC zeta with the corresponding segment of PKC iota prevented caspase processing and activation of the kinase function. Processing of purified recombinant PKC zeta by caspase 3 in vitro markedly increased its kinase activity. Caspase processing activated PKC zeta in vitro or intracellularly without increasing the phosphorylation of Thr410 of PKC zeta, which is required for catalytic competency. The freed kinase domain of PKC zeta had a much shorter half-life than the holoenzyme in transfected HeLa cells and in non-transfected kidney epithelial cells. Treatment with TNF-alpha shortened the half-life of the kinase domain protein, and proteasome blockade stabilized the protein. Studies of kinase-domain mutants indicate that a lack of negative charge at Thr410 can shorten the half-life of the freed kinase domain. The present findings indicate that the freed kinase domain has substantially higher kinase activity and a much shorter half-life than the holoenzyme because of accelerated degradation by the ubiquitin-proteasome system. PMID:12887331

  12. A survey for pulsations in A-type stars using SuperWASP

    NASA Astrophysics Data System (ADS)

    Holdsworth, Daniel L.

    2015-12-01

    "It is sound judgement to hope that in the not too distant future we shall be competent to understand so simple a thing as a star." - Sir Arthur Stanley Eddington, The Internal Constitution of Stars, 1926 A survey of A-type stars is conducted with the SuperWASP archive in the search for pulsationally variable stars. Over 1.5 million stars are selected based on their (J-H) colour. Periodograms are calculated for light curves which have been extracted from the archive and cleaned of spurious points. Peaks which have amplitudes greater than 0.5 millimagnitude are identified in the periodograms. In total, 202 656 stars are identified to show variability in the range 5-300 c/d. Spectroscopic follow-up was obtained for 38 stars which showed high-frequency pulsations between 60 and 235 c/d, and a further object with variability at 636 c/d. In this sample, 13 were identified to be normal A-type δ Sct stars, 14 to be pulsating metallic-lined Am stars, 11 to be rapidly oscillating Ap (roAp) stars, and one to be a subdwarf B variable star. The spectra were used not only to classify the stars, but to determine an effective temperature through Balmer line fitting. Hybrid stars have been identified in this study, which show pulsations in both the high- and low-overtone domains; an observation not predicted by theory. These stars are prime targets to perform follow-up observations, as a confirmed detection of this phenomenon will have significant impact on the theory of pulsations in A-type stars. The detected number of roAp stars has expanded the known number of this pulsator class by 22 per cent. Within these results both the hottest and coolest roAp star have been identified. Further to this, one object, KIC 7582608, was observed by the Kepler telescope for 4 yr, enabling a detailed frequency analysis. This analysis has identified significant frequency variations in this star, leading to the hypothesis that this is the first close binary star of its type. The observational

  13. Leaching of 226Ra from components of uranium mill tailings

    USGS Publications Warehouse

    Landa, E.R.

    1991-01-01

    A sequential extraction procedure was used to characterize the geochemical forms of 226Ra retained by mixtures of quartz sand and a variety of fine-grained rock and mineral species. These mixtures had previously been exposed to the sulfuric acid milling liquor of a simulated acid-leach uranium milling circuit. For most test cases, the major fraction of the 226Ra was extracted with 1 mol/1 NH4Cl and was deemed to be exchangeable. However, 226Ra retained by the barite-containing mixture was resistant to both 1 mol/1 NH4Cl and 1 mol/HCHCl extraction. ?? 1991.

  14. Progress toward an EDM measurement in {sup225}Ra.

    SciTech Connect

    Holt, R. J.; Ahmad, I.; Bailey, K.; Graner, B.; Greene, J. P.; Korsch, W.; Lu, Z.-T.; Mueller, P.; O'Connor, T. P.; Sulai, I. A.; Trimble, W. L.

    2010-11-01

    Permanent electric dipole moments (EDMs) in atoms or molecules are a signature of time-reversal and parity violation and represent an important window onto physics beyond the Standard Model. We are developing a next generation EDM search based on laser-cooled and trapped {sup 225}Ra atoms. Due to octupole deformation of the nucleus, {sup 225}Ra is predicted to be two to three orders of magnitude more sensitive to T-violating interactions than {sup 199}Hg, which currently sets the most stringent limits in the nuclear sector. We will discuss progress toward realizing a first EDM measurement for {sup 225}Ra.

  15. Concurrent determination of 224Ra, 226Ra, 228Ra, and unsupported 212Pb in a single analysis for drinking water and wastewater: dissolved and suspended fractions.

    PubMed

    Parsa, Bahman; Obed, Reynaldo N; Nemeth, William K; Suozzo, Gail

    2004-02-01

    A technique has been developed for the measurement of 224Ra, 226Ra, 228Ra, and unsupported 2t2Pb concurrently in a single analysis. The procedure can be applied to both drinking water and wastewater, including the dissolved and suspended fractions of a sample. For drinking water samples, using 3-L aliquots, the radium isotopes are isolated by a fast PbSO4 co-precipitation and then quantified by gamma-ray spectroscopy. The radium isotopes 224Ra, 226Ra, and 228Ra are measured through their gamma-ray-emitting decay products, 212Pb, 214Pb (and/or 214Bi), and 228Ac, respectively. Because of the short half-life of 224Ra (T1/2 = 3.66 d), the precipitate should be counted within 4 d of the sample collection date. In case the measurement of unsupported 212Pb (T1/2 = 10.64 h) is required, the gamma-ray analysis should be initiated as soon as possible, preferably on the same day of collection. The counting is repeated after about 21 d to ensure the 226Ra progeny are in equilibrium with their parent. At this point, the 228Ac equilibration with its 228Ra parent is already established. In the case of samples containing suspended materials, an aliquot of sample is filtered and then the filtrate is treated as described above for drinking water samples. The suspended fraction of sample, collected on the filter, is directly analyzed by gamma-ray spectroscopy with no further chemical separation. Aliquots of de-ionized water spiked with various radium standards were analyzed to check the accuracy and precision of the method. In addition, analysis results of actual samples using this method were compared with the ones performed using U.S. Environmental Protection Agency-approved procedures, and the measured values were in close agreement. This method simplifies the analytical procedures and reduces the labor while achieving the precision, accuracy, and minimum detection concentration requirements of EPA's Regulations. PMID:14744047

  16. Olanzapine versus other atypical antipsychotics for schizophrenia

    PubMed Central

    Komossa, Katja; Rummel-Kluge, Christine; Hunger, Heike; Schmid, Franziska; Schwarz, Sandra; Duggan, Lorna; Kissling, Werner; Leucht, Stefan

    2014-01-01

    Background In many countries of the industrialised world second generation (“atypical”) antipsychotics have become the first line drug treatment for people with schizophrenia. The question as to whether, and if so how much, the effects of the various second generation antipsychotics differ is a matter of debate. In this review we examined how the efficacy and tolerability of olanzapine differs from that of other second generation antipsychotics. Objectives To evaluate the effects of olanzapine compared to other atypical antipsychotics for people with schizophrenia and schizophrenia-like psychosis. Search methods 1. Electronic searching We searched the Cochrane Schizophrenia Group Trials Register (April 2007) which is based on regular searches of BIOSIS, CENTRAL, CINAHL, EMBASE, MEDLINE and PsycINFO. 2. Reference searching We inspected the reference of all identified studies for more trials. 3. Personal contact We contacted the first author of each included study for missing information. 4. Drug companies We contacted the manufacturers of all atypical antipsychotics included for additional data. Selection criteria We included all randomised trials that used at least single-blind (rater-blind) design, comparing oral olanzapine with oral forms of amisulpride, aripiprazole, clozapine, quetiapine, risperidone, sertindole, ziprasidone or zotepine in people with schizophrenia or schizophrenia-like psychosis. Data collection and analysis We extracted data independently. For dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. For continuous data, we calculated weighted mean differences (WMD) again based on a random effects model. Main results The review currently includes 50 studies and 9476 participants which provided data for six comparisons (olanzapine compared to amisulpride, aripiprazole

  17. Risperidone versus other atypical antipsychotics for schizophrenia

    PubMed Central

    Komossa, Katja; Rummel-Kluge, Christine; Schwarz, Sandra; Schmid, Franziska; Hunger, Heike; Kissling, Werner; Leucht, Stefan

    2014-01-01

    Background In many countries of the industrialised world second-generation (“atypical”) antipsychotics (SGAs) have become the first line drug treatment for people with schizophrenia. The question as to whether and if so how much the effects of the various SGAs differ is a matter of debate. In this review we examined how the efficacy and tolerability of risperidone differs from that of other SGAs. Objectives To evaluate the effects of risperidone compared with other atypical antipsychotics for people with schizophrenia and schizophrenia-like psychosis. Search methods 1. Electronic searching We searched the Cochrane Schizophrenia Group Trials Register (April 2007) which is based on regular searches of BIOSIS, CENTRAL, CINAHL, EMBASE, MEDLINE and PsycINFO. 2. Reference searching We inspected the references of all identified studies for more trials. 3. Personal contact We contacted the first author of each included study for missing information. 4. Drug companies We contacted the manufacturers of all atypical antipsychotics included for additional data. Selection criteria We included all randomised, blinded trials comparing oral risperidone with oral forms of amisulpride, aripiprazole, clozapine, olanzapine, quetiapine, sertindole, ziprasidone or zotepine in people with schizophrenia or schizophrenia-like psychosis. Data collection and analysis We extracted data independently. For dichotomous data we calculated risk ratio (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random-effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. For continuous data, we calculated mean differences (MD), again based on a random-effects model. Main results The review currently includes 45 blinded RCTs with 7760 participants. The number of RCTs available for each comparison varied: four studies compared risperidone with amisulpride, two with aripiprazole, 11 with clozapine, 23 with olanzapine, eleven with

  18. Genetics Underlying Atypical Parkinsonism and Related Neurodegenerative Disorders

    PubMed Central

    Scholz, Sonja W.; Bras, Jose

    2015-01-01

    Atypical parkinsonism syndromes, such as dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy and corticobasal degeneration, are neurodegenerative diseases with complex clinical and pathological features. Heterogeneity in clinical presentations, possible secondary determinants as well as mimic syndromes pose a major challenge to accurately diagnose patients suffering from these devastating conditions. Over the last two decades, significant advancements in genomic technologies have provided us with increasing insights into the molecular pathogenesis of atypical parkinsonism and their intriguing relationships to related neurodegenerative diseases, fueling new hopes to incorporate molecular knowledge into our diagnostic, prognostic and therapeutic approaches towards managing these conditions. In this review article, we summarize the current understanding of genetic mechanisms implicated in atypical parkinsonism syndromes. We further highlight mimic syndromes relevant to differential considerations and possible future directions. PMID:26501269

  19. Genetics Underlying Atypical Parkinsonism and Related Neurodegenerative Disorders.

    PubMed

    Scholz, Sonja W; Bras, Jose

    2015-01-01

    Atypical parkinsonism syndromes, such as dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy and corticobasal degeneration, are neurodegenerative diseases with complex clinical and pathological features. Heterogeneity in clinical presentations, possible secondary determinants as well as mimic syndromes pose a major challenge to accurately diagnose patients suffering from these devastating conditions. Over the last two decades, significant advancements in genomic technologies have provided us with increasing insights into the molecular pathogenesis of atypical parkinsonism and their intriguing relationships to related neurodegenerative diseases, fueling new hopes to incorporate molecular knowledge into our diagnostic, prognostic and therapeutic approaches towards managing these conditions. In this review article, we summarize the current understanding of genetic mechanisms implicated in atypical parkinsonism syndromes. We further highlight mimic syndromes relevant to differential considerations and possible future directions. PMID:26501269

  20. Gender-Atypical Mental Illness as Male Gender Threat.

    PubMed

    Michniewicz, Kenneth S; Bosson, Jennifer K; Lenes, Joshua G; Chen, Jason I

    2016-07-01

    The present study examined whether men view gender-atypical (i.e., feminine) psychological disorders as threats to their gender status. Men and women (N = 355) rated their expectations of gender status loss, feelings of distress, and help-seeking intentions in response to 10 different stereotypically masculine and feminine psychological disorders. Men as compared to women expected greater gender status loss for, and reported more distress to, gender-atypical versus gender-typical disorders. Expectations of gender status loss partially mediated the link between participant gender and distress at the thought of gender-atypical disorders. These findings suggest that feminine disorders pose more powerful gender status threats for men than masculine disorders do and that men's expectations of gender status loss for feminine disorders drive their negative reactions to these mental illnesses. The discussion emphasizes the importance of considering the gender-typicality of disorders, and the implications of these findings for clinical interventions. PMID:25595020

  1. Atypical trigeminal neuralgia: a consequence of central sensitization?

    PubMed

    Hu, Wen-han; Zhang, Kai; Zhang, Jian-guo

    2010-07-01

    Trigeminal neuralgia (TN) is characterized by sudden, recurrent, usually unilateral, severe brief stabbing pains in the distribution of trigeminal nerve. Although it is widely accepted that blood vessel or tumor compression contributes to paroxysms of TN, the pathogenesis of persistent background pain in atypical TN patient is unclear. Central sensitization is pain hypersensitivity caused by central neural plasticity. It is responsible for many temporal and symptomatic features of acute and chronic pain. We hypothesize that central sensitization might account for some symptoms of atypical TN. Based on this hypothesis, we postulate that early medical intervention predicts good outcomes in TN and medicines which are effective on central sensitization may be potential agents for the treatment of atypical TN. PMID:20172658

  2. Atypical language representation in children with intractable temporal lobe epilepsy.

    PubMed

    Maulisova, Alice; Korman, Brandon; Rey, Gustavo; Bernal, Byron; Duchowny, Michael; Niederlova, Marketa; Krsek, Pavel; Novak, Vilem

    2016-05-01

    This study evaluated language organization in children with intractable epilepsy caused by temporal lobe focal cortical dysplasia (FCD) alone or dual pathology (temporal lobe FCD and hippocampal sclerosis, HS). We analyzed clinical, neurological, fMRI, neuropsychological, and histopathologic data in 46 pediatric patients with temporal lobe lesions who underwent excisional epilepsy surgery. The frequency of atypical language representation was similar in both groups, but children with dual pathology were more likely to be left-handed. Atypical receptive language cortex correlated with lower intellectual capacity, verbal abstract conceptualization, receptive language abilities, verbal working memory, and a history of status epilepticus but did not correlate with higher seizure frequency or early seizure onset. Histopathologic substrate had only a minor influence on neuropsychological status. Greater verbal comprehension deficits were noted in children with atypical receptive language representation, a risk factor for cognitive morbidity. PMID:27064828

  3. Microbial release of 226Ra2+ from (Ba,Ra)SO4 sludges from uranium mine wastes.

    PubMed Central

    Fedorak, P M; Westlake, D W; Anders, C; Kratochvil, B; Motkosky, N; Anderson, W B; Huck, P M

    1986-01-01

    226Ra2+ is removed from uranium mine effluents by coprecipitation with BaSO4. (Ba,Ra)SO4 sludge samples from two Canadian mine sites were found to contain active heterotrophic populations of aerobic, anaerobic, denitrifying, and sulfate-reducing bacteria. Under laboratory conditions, sulfate reduction occurred in batch cultures when carbon sources such as acetate, glucose, glycollate, lactate, or pyruvate were added to samples of (Ba,Ra)SO4 sludge. No external sources of nitrogen or phosphate were required for this activity. Further studies with lactate supplementation showed that once the soluble SO4(2-) in the overlying water was depleted, Ba2+ and 226Ra2+ were dissolved from the (Ba,Ra)SO4 sludge, with the concurrent production of S2-. Levels of dissolved 226Ra2+ reached approximately 400 Bq/liter after 10 weeks of incubation. Results suggest that the ultimate disposal of these sludges must maintain conditions to minimize the activity of the indigenous sulfate-reducing bacteria to ensure that unacceptably high levels of 226Ra2+ are not released to the environment. PMID:3752993

  4. Novel antipsychotics: issues and controversies. Typicality of atypical antipsychotics.

    PubMed Central

    Stip, E

    2000-01-01

    The typicality of atypical antipsychotic drugs remains debatable. Preclinical studies and findings from randomized, controlled and open trials of clozapine, olanzapine, risperidone, quetiapine, sertindole, ziprasidone and a substituted benzamide were examined. A MEDLINE search was conducted using key words, including "extrapyramidal side effects," "cognition," "schizophrenia" and the generic drug names. Over 140 articles from peer-reviewed journals were reviewed, some of which were based on a meta-analysis. New-generation neuroleptic agents were found to have greater efficacy on the negative symptoms of schizophrenia and to cause fewer unwanted extrapyramidal side effects (EPS) than the traditional antipsychotic drugs. On one hand, atypical neuroleptic agents could be strictly defined as any neuroleptic agent with antipsychotic effects at a dosage that does not cause extrapyramidal side effects. Thus, clozapine is regarded as the "standard" atypical antipsychotic drug. On the other hand, typicality is about dimension rather than category, and we suggest the use of the term "spectrum of atypicality." For example, an emphasis is placed on quetiapine to illustrate where a new compound fits in this spectrum. Although dose-related, atypicality may be more a question of prescription attitude than of a specific characteristic of a compound. The degree to which a new compound is clinically superior to another atypical antipsychotic drug, in terms of improving positive, negative or affective symptoms, cognitive function and long-term outcome, will require further a priori hypotheses based on conceptual frameworks that are clinically meaningful. In addition, the results from industry-sponsored trials should be more comparable to those obtained from investigator-leading trials. Finally, the patient characteristics that define a patient's response to a specific antipsychotic drug are unknown. PMID:10740987

  5. Atypical Intracranial Epidermoid Cysts: Rare Anomalies with Unique Radiological Features

    PubMed Central

    Law, Eric K. C.; Lee, Ryan K. L.; Ng, Alex W. H.; Siu, Deyond Y. W.; Ng, Ho-Keung

    2015-01-01

    Epidermoid cysts are benign slow growing extra-axial tumours that insinuate between brain structures, while their occurrences in intra-axial or intradiploic locations are exceptionally rare. We present the clinical, imaging, and pathological findings in two patients with atypical epidermoid cysts. CT and MRI findings for the first case revealed an intraparenchymal epidermoid cyst that demonstrated no restricted diffusion. The second case demonstrated an aggressive epidermoid cyst that invaded into the intradiploic spaces, transverse sinus, and the calvarium. The timing of ectodermal tissue sequestration during fetal development may account for the occurrence of atypical epidermoid cysts. PMID:25667778

  6. Uremic parkinsonism with atypical phenotypes and radiologic features.

    PubMed

    Yoon, Jee-Eun; Kim, Ji Sun; Park, Jeong-Ho; Lee, Kyung-Bok; Roh, Hakjae; Park, Sung Tae; Cho, Jin Whan; Ahn, Moo-Young

    2016-04-01

    Uremic encephalopathy with bilateral basal ganglia lesions has been reported as an acute neurometabolic disease which shows reversible clinical course and brain imaging features. The exact nature and pathophysiology have not been well established. We encountered two patients who showed a relapsing and aggravating course and an atypical phenotype including parkinsonism with paroxysmal dystonic head tremor and acute onset monoparesis of the lower extremity. They also showed unusual radiological findings which revealed combined lesions in the basal ganglia and cortex, persistent hemorrhagic transformation, and focal ischemic lesion in the internal capsule. Herein, we present the unusual phenomenology with atypical radiologic findings and suggest the possible multifactorial pathogenesis of uremic encephalopathy. PMID:26631408

  7. Positron Emission Tomography Findings in Atypical Polypoid Adenomyoma

    PubMed Central

    Fukami, Tatsuya; Yoshikai, Tomonori; Tsujioka, Hiroshi; Tohyama, Atsushi; Sorano, Sumire; Matsuoka, Sakiko; Yamamoto, Hiroko; Nakamura, Sumie; Goto, Maki; Matsuoka, Ryoei; Oya, Masafumi; Torii, Yoshikuni; Eguchi, Fuyuki

    2016-01-01

    Atypical polypoid adenomyoma (APAM) is a rare polypoid tumor of the uterus composed of atypical endometrial glands surrounded by smooth muscle. A 29-year-old nulligravida, was clinically diagnosed with endocervical myoma and underwent trans-uterine cervical resection with hysteroscope. The histopathological diagnosis of specimens was APAM. Eight months later, she diagnosed recurrent uterine tumor. The positron emission tomography (PET-CT) imaging showed an increased fluorodeoxyglucose uptake. She has performed hysterectomy and was diagnosed APAM. Therapy for APAM depends on multiple factors such as age at presentation and desire for childbearing among others. This is the first report of PET-CT findings in APAM. PMID:27134711

  8. Seasonal changes in submarine groundwater discharge to coastal salt ponds estimated using 226Ra and 228Ra as tracers

    USGS Publications Warehouse

    Hougham, A.L.; Moran, S.B.; Masterson, J.P.; Kelly, R.P.

    2008-01-01

    Submarine groundwater discharge (SGD) to coastal southern Rhode Island was estimated from measurements of the naturally-occurring radioisotopes 226Ra (t1/2 = 1600??y) and 228Ra (t1/2 = 5.75??y). Surface water and porewater samples were collected quarterly in Winnapaug, Quonochontaug, Ninigret, Green Hill, and Pt. Judith-Potter Ponds, as well as nearly monthly in the surface water of Rhode Island Sound, from January 2002 to August 2003; additional porewater samples were collected in August 2005. Surface water activities ranged from 12-83??dpm 100??L- 1 (60??dpm = 1??Bq) and 21-256??dpm 100??L- 1 for 226Ra and 228Ra, respectively. Porewater 226Ra activities ranged from 16-736??dpm 100??L- 1 (2002-2003) and 95-815??dpm 100??L- 1 (2005), while porewater 228Ra activities ranged from 23-1265??dpm 100??L- 1. Combining these data with a simple box model provided average 226Ra-based submarine groundwater fluxes ranging from 11-159??L m- 2 d- 1 and average 228Ra-derived fluxes of 15-259??L m- 2 d- 1. Seasonal changes in Ra-derived SGD were apparent in all ponds as well as between ponds, with SGD values of 30-472??L m- 2 d- 1 (Winnapaug Pond), 6-20??L m- 2 d- 1 (Quonochontaug Pond), 36-273??L m- 2 d- 1 (Ninigret Pond), 29-76??L m- 2 d- 1 (Green Hill Pond), and 19-83??L m- 2 d- 1 (Pt. Judith-Potter Pond). These Ra-derived fluxes are up to two orders of magnitude higher than results predicted by a numerical model of groundwater flow, estimates of aquifer recharge for the study period, and values published in previous Ra-based SGD studies in Rhode Island. This disparity may result from differences in the type of flow (recirculated seawater versus fresh groundwater) determined using each technique, as well as variability in porewater Ra activity. ?? 2007 Elsevier B.V. All rights reserved.

  9. A putative RA-like region in the brain of the scale-backed antbird, Willisornis poecilinotus (Furnariides, Suboscines, Passeriformes, Thamnophilidae)

    PubMed Central

    de Lima, Jamily L.R.; Soares, Fabricio A.; Remedios, Ana C.S.; Thom, Gregory; Wirthlin, Morgan; Aleixo, Alexandre; Schneider, Maria Paula C.; Mello, Claudio V.; Schneider, Patricia N.

    2015-01-01

    The memorization and production of song in songbirds share important parallels with the process of speech acquisition in humans. In songbirds, these processes are dependent on a group of specialized telencephalic nuclei known as the song system: HVC (used as a proper name), RA (robust nucleus of arcopallium), LMAN (lateral magnocellular nucleus of the nidopallium) and striatal Area X. A recent study suggested that the arcopallium of the Sayornis phoebe, a non vocal learner suboscine species, contains a nucleus with some properties similar to those of songbird RA, suggesting that the song system may have been present in the last common ancestor of these groups. Here we report morphological and gene expression evidence that a region with some properties similar to RA is present in another suboscine, the Amazonian endemic Willisornis poecilinotus. Specifically, a discrete domain with a distinct Nissl staining pattern and that expresses the RA marker RGS4 was found in the arcopallium where the oscine RA is localized. Our findings, combined with the previous report on the S. phoebe, suggest that an arcopallial region with some RA-like properties was present in the ancestor of both Suboscines infraorders Tyranni and Furnarii, and is possibly an ancestral feature of Passeriformes. PMID:26500428

  10. Zotepine versus other atypical antipsychotics for schizophrenia

    PubMed Central

    Subramanian, Selvizhi; Rummel-Kluge, Christine; Hunger, Heike; Schmid, Franziska; Schwarz, Sandra; Kissling, Werner; Leucht, Stefan; Komossa, Katja

    2014-01-01

    Background In many parts of the world, particularly in industrialised countries, second generation (atypical) antipsychotic drugs have become first line treatment for people suffering from schizophrenia. The question as to whether the effects of various second generation antipsychotic drugs differ is a matter of debate. Objectives To evaluate the effects of zotepine compared with other second generation antipsychotic drugs for people suffering from schizophrenia and schizophrenia-like psychoses. Search methods We searched the Cochrane Schizophrenia Group Trials Register (November 2009), inspected references of all identified studies for further trials and contacted authors of trials for additional information. Selection criteria We included only randomised clinical controlled trials that compared zotepine with any forms of amisulpride, aripiprazole, clozapine, olanzapine, risperidone, sertindole or ziprasidone in people suffering from only schizophrenia or schizophrenia-like psychoses. Data collection and analysis SS and KK extracted data independently. For dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random-effects model. For continuous data, we calculated weighted mean differences (MD) again based on a random-effects model. Main results We included three studies (total n=289; 2 RCTs zotepine vs clozapine; 1 RCT zotepine vs clozapine vs risperidone (at 4 mg, 8 mg doses) vs remoxipride. All studies were of limited methodological quality. When zotepine was compared with clozapine, it was clozapine that was found to be more effective in terms of global state (n=59, 1 RCT, RR No clinically significant response 8.23 CI 1.14 to 59.17). Mental state scores also favoured clozapine (n=59, 1 RCT, MD average score (BPRS total, high = poor) 6.00 CI 2.17 to 9.83) and there was less use of antiparkinson medication in the clozapine group (n=116, 2 RCTs, RR 20.96 CI 2.89 to 151.90). In the

  11. Ra: The Sun for Science and Humanity

    NASA Technical Reports Server (NTRS)

    1996-01-01

    To guide the development of the Ra Strategic Framework, we defined scientific and applications objectives. For our primary areas of scientific interest, we choose the corona, the solar wind, the Sun's effect on the Earth, and solar theory and model development. For secondary areas of scientific interest, we selected sunspots, the solar constant, the Sun's gravitational field, helioseismology and the galactic cosmic rays. We stress the importance of stereoscopic imaging, observations at high spatial, spectral, and temporal resolutions, as well as of long duration measurements. Further exploration of the Sun's polar regions is also important, as shown already by the Ulysses mission. From an applications perspective, we adopted three broad objectives that would derive complementary inputs for the Strategic Framework. These were to identify and investigate: possible application spin-offs from science missions, possible solar-terrestrial missions dedicated to a particular application, and possible future applications that require technology development. The Sun can be viewed as both a source of resources and of threats. Our principal applications focus was that of threat mitigation, by examining ways to improve solar threat monitoring and early warning systems. We compared these objectives to the mission objectives of past, current, and planned international solar missions. Past missions (1962-1980) seem to have been focused on improvement of scientific knowledge, using multiple instrument spacecraft. A ten year gap followed this period, during which the results from previous missions were analyzed and solar study programmes were prepared in international organizations. Current missions (1990-1996) focus on particular topics such as the corona, solar flares, and coronal mass ejections. In planned missions, Sun/Earth interactions and environmental effects of solar activity are becoming more important. The corona is the centre of interest of almost all planned missions

  12. Rapid determination of 226Ra in emergency urine samples

    DOE PAGESBeta

    Maxwell, Sherrod L.; Culligan, Brian K.; Hutchison, Jay B.; Utsey, Robin C.; McAlister, Daniel R.

    2014-02-27

    A new method has been developed at the Savannah River National Laboratory (SRNL) that can be used for the rapid determination of 226Ra in emergency urine samples following a radiological incident. If a radiological dispersive device event or a nuclear accident occurs, there will be an urgent need for rapid analyses of radionuclides in urine samples to ensure the safety of the public. Large numbers of urine samples will have to be analyzed very quickly. This new SRNL method was applied to 100 mL urine aliquots, however this method can be applied to smaller or larger sample aliquots as needed.more » The method was optimized for rapid turnaround times; urine samples may be prepared for counting in <3 h. A rapid calcium phosphate precipitation method was used to pre-concentrate 226Ra from the urine sample matrix, followed by removal of calcium by cation exchange separation. A stacked elution method using DGA Resin was used to purify the 226Ra during the cation exchange elution step. This approach combines the cation resin elution step with the simultaneous purification of 226Ra with DGA Resin, saving time. 133Ba was used instead of 225Ra as tracer to allow immediate counting; however, 225Ra can still be used as an option. The rapid purification of 226Ra to remove interferences using DGA Resin was compared with a slightly longer Ln Resin approach. A final barium sulfate micro-precipitation step was used with isopropanol present to reduce solubility; producing alpha spectrometry sources with peaks typically <40 keV FWHM (full width half max). This new rapid method is fast, has very high tracer yield (>90 %), and removes interferences effectively. The sample preparation method can also be adapted to ICP-MS measurement of 226Ra, with rapid removal of isobaric interferences.« less

  13. Age Associated Increase of Low Avidity CMV-Specific CD8+ T Cells That Re-Express CD45RA

    PubMed Central

    Griffiths, Stephen J.; Riddell, Natalie E.; Masters, Joanne; Libri, Valentina; Henson, Sian M.; Wertheimer, Anne; Wallace, Diana; Sims, Stuart; Rivino, Laura; Larbi, Anis; Kemeny, David M.; Nikolich-Zugich, Janko; Kern, Florian; Klenerman, Paul; Emery, Vince C.; Akbar, Arne N.

    2013-01-01

    The mechanisms regulating memory CD8+ T cell function and homeostasis during ageing are unclear. CD8+ effector memory T cells that re-express CD45RA (EMRA T cells) increase considerably in older humans and both ageing and persistent cytomegalovirus (CMV) infection are independent factors in this process. We used MHC class I tetrameric complexes that were mutated in the CD8 binding domain to identify CMV-specific CD8+ T cells with high antigen binding avidity. In individuals who were HLA-A*0201, CD8+ T cells that expressed CD45RA and were specific for the pp65 protein (NLV epitope) had lower avidity than those that expressed CD45RO and demonstrated decreased cytokine secretion and cytolytic potential after specific activation. Furthermore, low avidity NLV-specific CD8+ T cells were significantly increased in older individuals. The stimulation of blood leukocytes with CMV lysate induced high levels of IFNα that in turn induced IL-15 production. Moreover, the addition of IL-15 to CD45RA−CD45RO+ CMV-specific CD8+ T cells induced CD45RA expression while antigen activated cells remained CD45RO+. This raises the possibility that non-specific cytokine driven accumulation of CMV-specific CD8+ CD45RA+ T cells with lower antigen binding avidity may exacerbate the effects of viral re-activation on skewing the T cell repertoire in CMV infected individuals during ageing. PMID:23636061

  14. Concentration of {sup 226}Ra in human teeth

    SciTech Connect

    Yamamoto, Masayoishi; Ueno, Kaoru; Hinoide, Moriyo; Ohkubo, Yoshiteru

    1994-11-01

    {sup 226}Ra concentrations in human teeth from several cities, mainly Tokyo, Japan, were determined with emphasis on the measurement of low-level {sup 226}Ra by alpha-ray spectrometry following chemical separation. No appreciable differences in {sup 226}Ra concentration were found among various permanent teeth samples of different age groups in Tokyo. The mean {sup 226}Ra concentration for Tokyo was 0.51 {+-} 0.06 mBq (g CA){sup -1}. {sup 226}Ra concentration [mean: 0.67 {+-} 0.11 mBq (g Ca){sup -1}] in teeth in western regions of the country was statistically higher than that [mean: 0.48 {+-} 0.09 mBq (g Ca){sup -1}] in eastern ones. The mean {sup 226}Ra concentration [0.51 mBq (g CA){sup -1}] in teeth from Tokyo was less than the concentration [1.11 mBq (g CA){sup -1}] reported for vertebral bone samples of this city. 27 refs., 1 fig., 5 tabs.

  15. Determining groundwater Ra end-member values for the estimation of the magnitude of submarine groundwater discharge using Ra isotope tracers

    NASA Astrophysics Data System (ADS)

    Cho, Hyung-Mi; Kim, Guebuem

    2016-04-01

    Radium isotopes (228Ra and 226Ra) are excellent tracers of submarine groundwater discharge (SGD). To estimate SGD magnitudes, information on the end-member values of Ra concentrations in groundwater is critical; however, the distribution characteristics of Ra in coastal aquifers are poorly understood. In this study, we show that Ra concentrations in coastal groundwater are primarily dependent on salinity based on the data (n > 500) obtained from global coastal aquifers, although previous end-member calculations averaged all Ra concentrations without considering salinity. If we assume that SGD is composed mainly of seawater infiltrating the aquifer, previous estimates of SGD for the Atlantic Ocean and the global ocean were overestimated twofold to threefold. This may be similar for other applications using different Ra isotopes. Our study highlights that the end-members of Ra isotopes in groundwater should be carefully considered when estimating SGD using Ra isotope mass balances in the ocean.

  16. HLA-D region genes and rheumatoid arthritis (RA): importance of DR and DQ genes in conferring susceptibility to RA.

    PubMed Central

    Singal, D P; Green, D; Reid, B; Gladman, D D; Buchanan, W W

    1992-01-01

    The distribution of HLA-D region antigens was studied in three groups (I, IIa, and IIb) of patients with rheumatoid arthritis (RA): group I comprised 43 patients with mild, non-progressive RA, controlled by non-steroidal anti-inflammatory drugs without progression or erosions; group II comprised 94 patients with severe disease, who had earlier been treated with non-steroidal anti-inflammatory drugs and all had incomplete response requiring treatment with gold (sodium aurothiomalate). Of these, 46 patients (group IIa) responded to gold and the disease was well controlled, and the remaining 48 patients (group IIb) did not respond to gold and developed gold induced toxic reactions, including thrombocytopenia or proteinuria, or both. HLA-D region antigens were defined by serological and molecular (Southern blot analysis and oligonucleotide typing) techniques. The results show that DR4 was significantly increased in all three groups of patients. The prevalence of DR1, or DR1 in DR4 negative patients, and DR3 and DR4 associated DQw7 specificities, however, showed differences in these three groups of patients. The prevalence of DR1 and of DR1 in DR4 negative patients was increased only in patients with mild (group I) RA, but not in patients with severe (groups IIa and IIb) disease. On the other hand, the prevalence of DR4 associated DQw7 was significantly increased in patients with severe disease, but not in patients with mild RA. In addition, DR3 was significantly increased only in patients with severe disease who developed gold induced toxic reactions (group IIb). These data suggest that the HLA-D region genes which cause susceptibility to mild RA may be different from those causing susceptibility to severe RA. The results suggest that both DR and DQ (A, B) genes may be important in conferring susceptibility to RA: DR in mild disease and DQ in severe RA. Images PMID:1371662

  17. [Cutaneous atypical mycobacteriosis due to Mycobacterium massiliense in a cat].

    PubMed

    Albini, S; Mueller, S; Bornand, V; Gutzwiller, M E Ricklin; Burnand, C; Hüssy, D; Abril, C; Reitt, K; Korczak, B M; Miserez, R

    2007-12-01

    Fast growing mycobacteria are saprophytic bacteria that prevail in water and soil. They are opportunistic pathogens and may cause various infections if gaining entry into the body through a trauma. We herein describe the clinical presentation, pathology and diagnosis of the first case of cutaneous atypical mycobacteriosis due to Mycobacterium massiliense in a cat. PMID:18225411

  18. Clinical features of Friedreich's ataxia: classical and atypical phenotypes.

    PubMed

    Parkinson, Michael H; Boesch, Sylvia; Nachbauer, Wolfgang; Mariotti, Caterina; Giunti, Paola

    2013-08-01

    One hundred and fifty years since Nikolaus Friedreich's first description of the degenerative ataxic syndrome which bears his name, his description remains at the core of the classical clinical phenotype of gait and limb ataxia, poor balance and coordination, leg weakness, sensory loss, areflexia, impaired walking, dysarthria, dysphagia, eye movement abnormalities, scoliosis, foot deformities, cardiomyopathy and diabetes. Onset is typically around puberty with slow progression and shortened life-span often related to cardiac complications. Inheritance is autosomal recessive with the vast majority of cases showing an unstable intronic GAA expansion in both alleles of the frataxin gene on chromosome 9q13. A small number of cases are caused by a compound heterozygous expansion with a point mutation or deletion. Understanding of the underlying molecular biology has enabled identification of atypical phenotypes with late onset, or atypical features such as retained reflexes. Late-onset cases tend to have slower progression and are associated with smaller GAA expansions. Early-onset cases tend to have more rapid progression and a higher frequency of non-neurological features such as diabetes, cardiomyopathy, scoliosis and pes cavus. Compound heterozygotes, including those with large deletions, often have atypical features. In this paper, we review the classical and atypical clinical phenotypes of Friedreich's ataxia. PMID:23859346

  19. Einstein A-values of A-type methanol

    NASA Astrophysics Data System (ADS)

    Pei, C. C.; Gou, Q. Q.; Zeng, Q.

    1988-11-01

    This paper uses the model of a slightly asymmtric top with hindered internal rotation to calculate the total energies, the transition frequencies and Einstein A-values of the A-type CH3OH-molecule. The levels are lower than 352 cm-1 above the ground state. It means that there are 340 levels in total.

  20. Early Freezing of Gait: Atypical versus Typical Parkinson Disorders.

    PubMed

    Lieberman, Abraham; Deep, Aman; Dhall, Rohit; Tran, An; Liu, Ming-Jai

    2015-01-01

    In 18 months, 850 patients were referred to Muhammad Ali Parkinson Center (MAPC). Among them, 810 patients had typical Parkinson disease (PD) and 212 had PD for ≤5 years. Among the 212 patients with early PD, 27 (12.7%) had freezing of gait (FOG). Forty of the 850 had atypical parkinsonism. Among these 40 patients, all of whom had symptoms for ≤5 years, 12 (30.0%) had FOG. FOG improved with levodopa in 21/27 patients with typical PD but did not improve in the 12 patients with atypical parkinsonism. FOG was associated with falls in both groups of patients. We believe that FOG unresponsive to levodopa in typical PD resembles FOG in atypical parkinsonism. We thus compared the 6 typical PD patients with FOG unresponsive to levodopa plus the 12 patients with atypical parkinsonism with the 21 patients with typical PD responsive to levodopa. We compared them by tests of locomotion and postural stability. Among the patients with FOG unresponsive to levodopa, postural stability was more impaired than locomotion. This finding leads us to believe that, in these patients, postural stability, not locomotion, is the principal problem underlying FOG. PMID:25785228

  1. Regulation of breast cancer metastasis by atypical chemokine receptors.

    PubMed

    Cheng, Xiaoyun; Hung, Mien-Chie

    2009-05-01

    The interaction between chemokines and their G-protein-coupled receptors plays an important role in promoting metastasis of different kinds of human cancers. However, the expression of an atypical chemokine receptor, CCX-CKR, which serves as a decoy receptor to attract chemokines, inhibits the growth and metastasis of breast cancer by sequestration of chemokines. PMID:19383808

  2. [The changes of bone architecture in atypical femoral fracture].

    PubMed

    Yamamoto, Noriaki; Shimakura, Taketoshi; Takahash, Hideaki

    2013-07-01

    The feature of atypical femoral fracture is stress induced cortical bone reaction. It was considered to be the accumulation of microdamage which come from increasing of mechanical stress by femoral lateral bowing, and the decreased of ability of microdamage repair system. PMID:23811584

  3. Atypical sporadic bovine leukosis in a beef feedlot heifer

    PubMed Central

    Hendrick, Steven H.

    2002-01-01

    This case is considered atypical because the clinical signs are exemplary of both the systemic and localized forms of the disease. Although diseases are commonly described and differentiated as either multisystemic or localized, as demonstrated here, disease expression can be a continuum between 2 distinct phenotypes. PMID:12170839

  4. Exploring Atypical Verb+Noun Combinations in Learner Technical Writing

    ERIC Educational Resources Information Center

    Luzon Marco, Maria Jose

    2011-01-01

    Professional and academic discourse is characterised by a specific phraseology, which usually poses problems for students. This paper investigates atypical verb+noun collocations in a corpus of English technical writing of Spanish students. I focus on the type of verbs that most frequently occurred in these awkward or questionable combinations and…

  5. Evidence for Reduced Domain-Specificity in Auditory Processing in Autism

    ERIC Educational Resources Information Center

    Jarvinen-Pasley, Anna; Heaton, Pamela

    2007-01-01

    Neurological and behavioral findings indicate that atypical auditory processing characterizes autism. The present study tested the hypothesis that auditory processing is less domain-specific in autism than in typical development. Participants with autism and controls completed a pitch sequence discrimination task in which same/different judgments…

  6. Ziprasidone versus other atypical antipsychotics for schizophrenia

    PubMed Central

    Komossa, Katja; Rummel-Kluge, Christine; Hunger, Heike; Schwarz, Sandra; Bhoopathi, Paranthaman Sethupathi; Kissling, Werner; Leucht, Stefan

    2014-01-01

    Background In many countries of the industrialised world second generation (‘atypical’) antipsychotics have become the first line drug treatment for people with schizophrenia. The question as to whether, and if so how much, the effects of the various new generation antipsychotics differ is a matter of debate. In this review we examined how the efficacy and tolerability of ziprasidone differs from that of other second generation antipsychotics. Objectives To evaluate the effects of ziprasidone compared with other atypical antipsychotics for people with schizophrenia and schizophrenia-like psychoses. Search methods We searched the Cochrane Schizophrenia Group Specialised Register (April 2007) and references of all identified studies for further trial citations. We contacted pharmaceutical companies and authors of trials for additional information. This search was updated July 2012, 254 citations added to awaiting classification section. Selection criteria We included all randomised, at least single-blind, controlled trials comparing oral ziprasidone with oral forms of amisulpride, aripiprazole, clozapine, olanzapine, quetiapine, risperidone or zotepine in people with schizophrenia or schizophrenia-like psychoses. Data collection and analysis We extracted data independently. For continuous data, we calculated weighted mean differences (MD) for dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random-effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. Main results The review currently includes nine randomised controlled trials (RCTs) with 3361 participants. The overall rate of premature study discontinuation was very high (59.1%). Data for the comparisons of ziprasidone with amisulpride, clozapine, olanzapine, quetiapine and risperidone were available. Ziprasidone was a less acceptable treatment than olanzapine (leaving the studies early for any

  7. Cutaneous and Subcutaneous Metastases From Atypical Laryngeal Carcinoids

    PubMed Central

    Wang, Kui-Rong; Jia, Yuan-Jing; Zhou, Shui-Hong; Wang, Qin-Ying; Bao, Yang-Yang; Feng, Zhi-Ying; Yao, Hong-Tian; Fan, Jun

    2016-01-01

    Abstract The incidence of cutaneous and subcutaneous metastases from atypical laryngeal carcinoids is approximately 20%. However, the pathogenesis and natural history of, and prognostic factors for, the condition remain poorly understood. We reported a 54-year-old female presented with cutaneous and subcutaneous metastases from atypical laryngeal carcinoid. Laryngoscopy revealed a 0.5 × 1.5-cm reddish mass on the laryngeal surface of the epiglottis. Under general anesthesia, a biopsy sample was obtained via suspension laryngoscopy. Routine pathology revealed atypical laryngeal carcinoid. Immunohistochemical staining of the sections of primary tumor was positive for cytokeratin, chromogranin A, synaptophysin, hypoxia-inducible factor-1α, P53, and CD56. GLUT-1, p-Akt, and PI3K were negative. The Ki-67 index was 15%. Supraglottic laryngectomy and selective right-neck dissection were performed. After 6 months, the patient complained of pain in the right wall of the chest; multiple cutaneous and subcutaneous nodules were evident at that site and in the abdomen. An abdominal nodule was biopsied and pathology revealed that the atypical metastatic carcinoid had metastasized to both cutaneous and subcutaneous areas of the abdomen. Chemotherapy was then prescribed. Currently, the intrathecal drug delivery system remains in place. No local recurrence has been detected. Furthermore, we systematically reviewed clinical manifestations of the disease, pathogenesis, prognostic factors, and treatment. The metastasis rate (cutaneous and subcutaneous) was approximately 12.2%. Thirty patients (62.5%) with cutaneous and subcutaneous metastases exhibited contemporaneous lymph node invasion. The 3-, 5-, and 10-year survival rates were 44.0%, 22.0%, and 13.0%, respectively. The prognosis of patients with atypical laryngeal carcinoids was poor. Relevant prognostic factors included the level of p53, human papilloma virus status, certain hypoxic markers, and distant metastasis. No

  8. Risk of atypical femoral fracture during and after bisphosphonate use

    PubMed Central

    Schilcher, Jörg; Koeppen, Veronika; Aspenberg, Per; Michaëlsson, Karl

    2015-01-01

    Background and purpose Use of bisphosphonates in women is associated with higher risk of atypical femoral fractures. The risk in terms of timing of use and type of bisphosphonate, and in men, remains unclear. Patients and methods We reviewed radiographs of 5,342 Swedish women and men aged 55 years or more who had had a fracture of the femoral shaft in the 3-year period 2008–2010 (97% of those eligible), and found 172 patients with atypical fractures (93% of them women). We obtained data on medication and comorbidity. The risk of atypical fracture associated with bisphosphonate use was estimated in a nationwide cohort analysis. In addition, we performed a case-control analysis with comparison to 952 patients with ordinary shaft fractures. A short report of the findings has recently been presented (Schilcher et al. 2014a). Here we provide full details. Results The age-adjusted relative risk (RR) of atypical fracture associated with bisphosphonate use was 55 (95% CI: 39–79) in women and 54 (CI: 15–192) in men. In bisphosphonate users, women had a 3-fold higher risk than men (RR = 3.1, CI: 1.1–8.4). Alendronate users had higher risk than risedronate users (RR = 1.9, CI: 1.1–3.3). The RR after 4 years or more of use reached 126 (CI: 55–288), with a corresponding absolute risk of 11 (CI: 7–14) fractures per 10,000 person-years of use. The risk decreased by 70% per year since last use. Interpretation Women have a higher risk of atypical femoral fracture than men. The type of bisphosphonate used may affect risk estimates and the risk decreases rapidly after cessation. PMID:25582459

  9. Use of Atypical Antipsychotics in Nursing Homes and Pharmaceutical Marketing

    PubMed Central

    Pimentel, Camilla B.; Donovan, Jennifer L.; Field, Terry S.; Gurwitz, Jerry H.; Harrold, Leslie R.; Kanaan, Abir O.; Lemay, Celeste A.; Mazor, Kathleen M.; Tjia, Jennifer; Briesacher, Becky A.

    2014-01-01

    BACKGROUND Many nursing home (NH) residents are prescribed atypical antipsychotics despite US Food and Drug Administration warnings of increased risk of death in older adults with dementia. Aggressive pharmaceutical marketing has been cited as a potential cause, although data are scarce. The objectives of this study were to describe the current extent and type of pharmaceutical marketing in NHs in one state, and to provide preliminary evidence for the potential influence of pharmaceutical marketing on the use of atypical antipsychotics in NHs. DESIGN Nested mixed-methods, cross-sectional study of NHs in a cluster randomized trial. SETTING 41 NHs in Connecticut. PARTICIPANTS NH administrators, directors of nursing and medical directors (n = 93, response rate 75.6%). MEASUREMENTS Quantitative data, including prescription drug dispensing data (September 2009–August 2010) linked with Nursing Home Compare data (April 2011), were used to determine facility-level prevalence of atypical antipsychotic use, facility-level characteristics, NH staffing and NH quality. Qualitative data, including semi-structured interviews and surveys of NH leaders conducted in the first quarter of 2011, were used to determine encounters with pharmaceutical marketing. RESULTS Leadership at 46.3% of NHs (19/41) reported pharmaceutical marketing encounters, consisting of educational training, written/Internet-based materials and/or sponsored training. No association was detected between the level of atypical antipsychotic prescribing and reports of any pharmaceutical marketing by at least one NH leader. CONCLUSION NH leaders frequently encounter pharmaceutical marketing through a variety of ways, although the impact on atypical antipsychotic prescribing is unclear. PMID:25688605

  10. 3C 57 as an atypical radio-loud quasar: implications for the radio-loud/radio-quiet dichotomy

    NASA Astrophysics Data System (ADS)

    Sulentic, J. W.; Martínez-Carballo, M. A.; Marziani, P.; del Olmo, A.; Stirpe, G. M.; Zamfir, S.; Plauchu-Frayn, I.

    2015-06-01

    Lobe-dominated radio-loud (LD RL) quasars occupy a restricted domain in the 4D Eigenvector 1 (4DE1) parameter space which implies restricted geometry/physics/kinematics for this subclass compared to the radio-quiet (RQ) majority of quasars. We discuss how this restricted domain for the LD RL parent population supports the notion for a RQ-RL dichotomy among type 1 sources. 3C 57 is an atypical RL quasar that shows both uncertain radio morphology and falls in a region of 4DE1 space where RL quasars are rare. We present new radio flux and optical spectroscopic measures designed to verify its atypical optical/UV spectroscopic behaviour and clarify its radio structure. The former data confirms that 3C 57 falls off the 4DE1 quasar `main sequence' with both extreme optical Fe II emission (R_{Fe II} ˜ 1) and a large C IV λ1549 profile blueshift (˜-1500 km s-1). These parameter values are typical of extreme Population A sources which are almost always RQ. New radio measures show no evidence for flux change over a 50+ year time-scale consistent with compact steep-spectrum (or young LD) over core-dominated morphology. In the 4DE1 context where LD RL are usually low L/LEdd quasars, we suggest that 3C 57 is an evolved RL quasar (i.e. large blackhole mass) undergoing a major accretion event leading to a rejuvenation reflected by strong Fe II emission, perhaps indicating significant heavy metal enrichment, high bolometric luminosity for a low-redshift source and resultant unusually high Eddington ratio giving rise to the atypical C IV λ1549.

  11. The "RA" Expeditions: The Archaeological and Anthropological Background. The "RA" Expeditions: The Coriolis Effect. The "RA" Expeditions: The Papyrus Reed. Learning Experiences for Coastal and Oceanic Awareness Studies, Nos. 211, 212, 213. [Project COAST].

    ERIC Educational Resources Information Center

    Delaware Univ., Newark. Coll. of Education.

    Included are three units related to coastal and oceanic awareness. These are: (1) The "RA" Expeditions: The Archaeological and Anthropological Background; (2) The "RA" Expeditions: The Coriolis Effect; and (3) The "RA" Expeditions: The Papyrus Reed. Each of the three units are designed for students in grades 6-12. Each unit contains teacher…

  12. Resolution dependence in simulating the African hydroclimate with the HadGEM3-RA regional climate model

    NASA Astrophysics Data System (ADS)

    Moufouma-Okia, W.; Jones, R.

    2015-02-01

    This study documents the effect of horizontal resolution on the ability of the Met Office third-generation Global Atmosphere Regional Climate Model (HadGEM3-RA), a regional atmospheric configuration of the HadGEM3 model, to simulate rainfall variability over Africa. It is based on six 20-year long RCM simulations driven by ERA-Interim reanalysis and performed at 12, 25, 50, 70, 90, and 150 km over the CORDEX-Africa domain. To gain further insight into model errors, we also compared the HadGEM3-RA's performance to that of the parent General Circulation Model using three different spatial resolutions (70, 100, and 150 km), and to HadRM3P—the current Met Office regional climate model. It is found that the 50 km resolution RCM reproduces reasonably the spatial and temporal features of rainfall variability across regions. These include the seasonal progression of the tropical rainbelt, its extent and location, the annual cycle and interannual variability. Although model biases vary across seasons and locations, a prominent feature is the over-prediction of rainfall totals over Central Africa, and underestimation of rainfall in coastal areas of the Guinea Gulf during boreal spring and autumn. HadGEM3-RA improves with increased horizontal resolution, but some model errors persist. Comparison with the parent global model simulations demonstrates generally a realistic and consistent behaviour over large scales—suggesting that the physical formulation is able to capture the key driving processes, but also confirms the benefit of increasing the model horizontal resolution. Despite the model errors, HadGEM3-RA rainfall shows superiority over that from HadRM3P, ERA-Interim and MERRA datasets—indicating that the associated dynamical features of HadGEM3-RA can complement the physical understanding gained from reanalyses. This article also highlights the challenges for evaluating climate models in data sparse regions where satellite derived rainfall and gridded observational

  13. Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA.

    PubMed

    Suzuki, Takuji; Sakagami, Takuro; Rubin, Bruce K; Nogee, Lawrence M; Wood, Robert E; Zimmerman, Sarah L; Smolarek, Teresa; Dishop, Megan K; Wert, Susan E; Whitsett, Jeffrey A; Grabowski, Gregory; Carey, Brenna C; Stevens, Carrie; van der Loo, Johannes C M; Trapnell, Bruce C

    2008-11-24

    Primary pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by accumulation of surfactant in the lungs that is presumed to be mediated by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling based on studies in genetically modified mice. The effects of GM-CSF are mediated by heterologous receptors composed of GM-CSF binding (GM-CSF-Ralpha) and nonbinding affinity-enhancing (GM-CSF-Rbeta) subunits. We describe PAP, failure to thrive, and increased GM-CSF levels in two sisters aged 6 and 8 yr with abnormalities of both GM-CSF-Ralpha-encoding alleles (CSF2RA). One was a 1.6-Mb deletion in the pseudoautosomal region of one maternal X chromosome encompassing CSF2RA. The other, a point mutation in the paternal X chromosome allele encoding a G174R substitution, altered an N-linked glycosylation site within the cytokine binding domain and glycosylation of GM-CSF-Ralpha, severely reducing GM-CSF binding, receptor signaling, and GM-CSF-dependent functions in primary myeloid cells. Transfection of cloned cDNAs faithfully reproduced the signaling defect at physiological GM-CSF concentrations. Interestingly, at high GM-CSF concentrations similar to those observed in the index patient, signaling was partially rescued, thereby providing a molecular explanation for the slow progression of disease in these children. These results establish that GM-CSF signaling is critical for surfactant homeostasis in humans and demonstrate that mutations in CSF2RA cause familial PAP. PMID:18955570

  14. Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA

    PubMed Central

    Suzuki, Takuji; Sakagami, Takuro; Rubin, Bruce K.; Nogee, Lawrence M.; Wood, Robert E.; Zimmerman, Sarah L.; Smolarek, Teresa; Dishop, Megan K.; Wert, Susan E.; Whitsett, Jeffrey A.; Grabowski, Gregory; Carey, Brenna C.; Stevens, Carrie; van der Loo, Johannes C.M.; Trapnell, Bruce C.

    2008-01-01

    Primary pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by accumulation of surfactant in the lungs that is presumed to be mediated by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling based on studies in genetically modified mice. The effects of GM-CSF are mediated by heterologous receptors composed of GM-CSF binding (GM-CSF-Rα) and nonbinding affinity-enhancing (GM-CSF-Rβ) subunits. We describe PAP, failure to thrive, and increased GM-CSF levels in two sisters aged 6 and 8 yr with abnormalities of both GM-CSF-Rα–encoding alleles (CSF2RA). One was a 1.6-Mb deletion in the pseudoautosomal region of one maternal X chromosome encompassing CSF2RA. The other, a point mutation in the paternal X chromosome allele encoding a G174R substitution, altered an N-linked glycosylation site within the cytokine binding domain and glycosylation of GM-CSF-Rα, severely reducing GM-CSF binding, receptor signaling, and GM-CSF–dependent functions in primary myeloid cells. Transfection of cloned cDNAs faithfully reproduced the signaling defect at physiological GM-CSF concentrations. Interestingly, at high GM-CSF concentrations similar to those observed in the index patient, signaling was partially rescued, thereby providing a molecular explanation for the slow progression of disease in these children. These results establish that GM-CSF signaling is critical for surfactant homeostasis in humans and demonstrate that mutations in CSF2RA cause familial PAP. PMID:18955570

  15. Synthesis and characterization of lanthanum phosphate nanoparticles as carriers for 223Ra and 225Ra for targeted alpha therapy

    DOE PAGESBeta

    Rojas, J. V.; Woodward, J. D.; Chen, N.; Rondinone, A. J.; Castano, C. H.; Mirzadeh, S.

    2015-03-19

    Targeted alpha therapy (TAT) has the potential for killing specific tumor cells with minimum collateral damage to surrounding healthy tissue. Radionuclides such as 223Ra, 225Ra, and 225Ac are of special interest for radiotherapeutic applications as they emit multiple -particles during their decay. Utilizing appropriate carriers capable of retaining both the parent radioisotope as well as daughter products is important for the effective delivery of the radioisotope to the tumor site while mitigating global in vivo radiotoxicity. Methods. In this work, core and core+2 shells (NPs with 2 additional layers of cold LaPO4 deposited on the core surfaces) LaPO4 nanoparticles (NPs)more » were synthesized containing either 223Ra or 225Ra/225Ac and the retention of the parents and daughters within the NPs in vitro was investigated. Results. The NPs crystallized in rhabdophane phase with mean diameters of 3.4 and 6.3 nm for core and core+2 shells, respectively. The core LaPO 4 NPs retained up to 88% of 223Ra over 35 days. However, in the core+2 shell NPs, the retention of 223Ra and its daughter, 211Pb, was improved to > 99.9% over 27 days. Additionally, the retention of 225Ra/225Ac parents was > 99.98% and ~80% for the 221Fr and 213Bi daughters over 35 days for the core+2 shell NPs. Conclusions. These results suggest that LaPO4 NPs are potentially effective carriers of radium isotopes.« less

  16. An experimental analysis of the contribution of 224Ra and 226Ra and progeny to the gross alpha-particle activity of water samples.

    PubMed

    Arndt, Michael F; West, Lynn E

    2008-05-01

    The gross alpha-particle activity of water samples analyzed by EPA Method 900.0 is investigated as a function of residue mass and geometry, time between sample collection and analysis, and time between sample preparation and analysis for samples containing 224Ra, 212Pb, and 226Ra. It is shown that the gross alpha-particle activity due to 224Ra and its progeny can be up to 10 times the 224Ra activity at collection time and that due to 212Pb progeny can be up to 3 times the 212Pb activity at collection time. In samples with roughly equal activities of 224Ra and 226Ra analyzed soon after collection, it is shown that the gross alpha-particle activity is approximately constant with time because the decay of 224Ra and its progeny is offset by the ingrowth of 226Ra progeny. PMID:18403967

  17. Incidence of typical and atypical hanging among 66 hanging cases.

    PubMed

    Talukder, M A; Mansur, M A; Kadir, M M

    2008-07-01

    Present Study conducted prospectively to find out the position of knot in the neck in all hanging cases coming for post mortem examination at Mymensingh Medical College Mortuary in the year 2005. Hanging is a process in which the body is suspended with a ligature around the neck, which causes constriction of air passage preventing exchange of air between atmosphere & alveoli of lungs, leading to asphyxia & death. The constriction force is either the weight of the whole body or the weight of the head alone. A weight of 2Kg is sufficient for death in hanging. According to position of knot hanging is of two types-Typical hanging and Atypical hanging. In typical hanging the knot of the ligature should be at the nape of the neck and the knot of the ligature at any site other than the nape of the neck is Atypical hanging. A total of 557 postmortems were conducted in the year 2005 out of which 66 cases were of hanging. Among these, 66 cases (100%) position of knot on neck were on center of occiput (Typical hanging) in 14 cases (21.21%), on right side in 17 cases (25.75%), on left side in 21 cases (31.81%) and on front of neck in 14 cases (21.21%). Total of 52 cases are of Atypical hanging. This study suggests that Atypical hanging is more common (78.78%) than Typical hanging (21.21%) and more on left side of the neck (31.81%). Study at S.P. Medical College and A.G Hospitals, Bikaner in 2004 shows that out of 390 cases 35 (8.97%) were of hanging. Amongst those 35 cases in 33 cases position of knot on nape of the neck in 5 cases (15.15%), right side of neck 15 cases (45.45%), left side of neck in 11 cases (33.33%) and front of the neck 2 cases (6%). In their study, Typical hanging was found in 15.15% and Atypical hanging was in 84.85%; and Maximum on right side of the neck i.e. 45.45%. In both the studies Atypical hanging was more common but Maximum was on right side in their study and We found maximum on left side. PMID:18626449

  18. Determination of 226Ra in urine samples by alpha spectrometry.

    PubMed

    Kehagia, K; Potiriadis, C; Bratakos, S; Koukouliou, V; Drikos, G

    2007-01-01

    A radiation protection system to assess the internal contamination of workers during decontamination activities in an abounded fertilizer industry in the region of Attika, Greece, has been implemented. This system concerns, among other radionuclides, 226Ra. Because of the low 226Ra activities in urine, alpha spectrometry was used as the determination method after radiochemical separation. Radium was co precipitated with lead sulphate and purified using anion and cation exchange techniques. The source for the alpha spectrometric measurement was prepared by the electrodeposition of radium, from an aqueous/ethanol solution, onto stainless steel. The tracer used was 229Th. The chemical yield and the activity concentration were calculated via its daughter radionuclide 217At. Using the time-evolution formulas to calculate the 217At growth from its parent radionuclide 225Ra, a computer software was developed. This software was incorporated in a database, which automatically calculates and stores the results. PMID:17827131

  19. Sequence analysis and expression regulation of rbp4 by 9-cis-RA in Megalobrama amblycephala.

    PubMed

    Xu, Mengxia; Huang, Chunxiao; Chen, Nan; Wu, Xinjie; Zhu, Kecheng; Wang, Weimin; Wang, Huanling

    2015-04-01

    Retinol-binding protein 4 (rbp4) is mainly synthesized in the liver, where it binds retinol and then enters the bloodstream, delivering retinol to cells. The full-length cDNA coding rbp4 was cloned from Megalobrama amblycephala. The amino acid sequence showed strong homology with the homologues of other vertebrates, and all structural and functional domains were highly conserved. The mRNA levels in different tissues and development stages detected by quantitative real-time PCR revealed that M. amblycephala rbp4 was highly expressed in liver (P < 0.001), but the lower levels were also detected in eyes, kidney, intestine, and spleen. During the different development stages, the rbp4 mRNA appeared until 28 hours post-fertilization (hpf), underwent a slight drop, and then gradually increased after 50 hpf. In addition, the promoter sequence of M. amblycephala rbp4 was obtained using thermal asymmetric interlaced PCR. Two single nucleotide polymorphism sites (-385A>G and -329C>T) were found in the promoter. Transfection with recombinant plasmids of two different haplotypes (GT, AC) showed that 9-cis-retinoic acid (RA) increased the promoter activity, but the AC haplotype was more sensitive to RA. PMID:25274419

  20. The new WMO RA VI Regional Climate Centre on Climate Monitoring

    NASA Astrophysics Data System (ADS)

    Rapp, J.; Nitsche, H.

    2010-09-01

    Regional Climate Centres (RCCs) are institutions with the capacity and mandate by WMO to develop high quality regional-scale products using global products and incorporating regional information. Recently a pilot network of three RCC consortia was established for the WMO region RA VI (Europe and Middle East): • RCC node on climate data, • RCC node on climate monitoring, • RCC node on long-range forecasting. DWD/Germany has taken the responsibility of the RCC node on climate monitoring (RRC-CM). Further consortium members are Armstatehydromet/Armenia, Météo-France/France, KNMI/The Netherlands, RHMS/Serbia, and TSMS/Turkey. RCCs provide online access to their products and services to national meteorological and hydrological services and to other regional users. Vice versa, RCCs receive data, products, know-how and feedbacks from the meteorological services as a main source for regional information. By the same time, they provide regional data, products and feedbacks to Global Production Centres and Lead Centres for respective verification and product optimisation of the global-scale information. The RCC-CM will perform basic functions covering the domain of climate monitoring: • Annual and monthly climate diagnostic bulletins, • Monthly monitoring maps: global, RAVI, Eastern Mediterranean, South Caucasus, • Reference climatologies and trend maps, • RA VI climate monitoring WebPortal, • Climate watches, • Training; Research and Development (R&D). The poster shows the current stage of development of the RCC-CM by means of example products.

  1. Rapid determination of 226Ra in environmental samples

    SciTech Connect

    Maxwell, Sherrod L.; Culligan, Brian K.

    2012-02-04

    A new rapid method for the determination of {sup 228}Ra in natural water samples has been developed at the SRNL/EBL (Savannah River National Lab/ Environmental Bioassay Laboratory) that can be used for emergency response or routine samples. While gamma spectrometry can be employed with sufficient detection limits to determine {sup 228}Ra in solid samples (via {sup 228}Ac) , radiochemical methods that employ gas flow proportional counting techniques typically provide lower MDA (Minimal Detectable Activity) levels for the determination of {sup 228}Ra in water samples. Most radiochemical methods for {sup 228}Ra collect and purify {sup 228}Ra and allow for {sup 228}Ac daughter ingrowth for ~36 hours. In this new SRNL/EBL approach, {sup 228}Ac is collected and purified from the water sample without waiting to eliminate this delay. The sample preparation requires only about 4 hours so that {sup 228}Ra assay results on water samples can be achieved in < 6 hours. The method uses a rapid calcium carbonate precipitation enhanced with a small amount of phosphate added to enhance chemical yields (typically >90%), followed by rapid cation exchange removal of calcium. Lead, bismuth, uranium, thorium and protactinium isotopes are also removed by the cation exchange separation. {sup 228}Ac is eluted from the cation resin directly onto a DGA Resin cartridge attached to the bottom of the cation column to purify {sup 228}Ac. DGA Resin also removes lead and bismuth isotopes, along with Sr isotopes and {sup 90}Y. La is used to determine {sup 228}Ac chemical yield via ICP-MS, but {sup 133}Ba can also be used instead if ICP-MS assay is not available. Unlike some older methods, no lead or strontium holdback carriers or continual readjustment of sample pH is required.

  2. Mutation of the PAX6 gene in a sporadic patient with atypical aniridia

    SciTech Connect

    Zhu, D.; Li, Y.; Traboulsi, E.I.

    1994-09-01

    A 28 year-old man presented with poor vision since childhood and gradual further decline of several years duration. His visual acuity measures 20/200 OD with -11.50 + 0.50 x 150 and 20/100 OS with -12.25 + 0.25 x 35. He had a fine nystagmus. His visual fields were full. There was a circumferential pannus with areas of corneal stromal opacification. The iris was hypoplastic with atypical colobomatous defects. The lenses had scattered cortical opacities. The intraocular pressures were normal. The optic nerves had cup disk ratios of 0.6 OU. The family history was negative for similar defects. A diagnosis of aniridia was made and blood was drawn for analysis of the PAX6 gene. PCR amplification of exon 5 showed heterozygous fragments with one allele being larger than normal. Direct DNA sequencing of the individual heterozygous allele showed a 41 base pair insertion at nucleotide 483 in exon 5 of the paired domain. This frameshift mutation changed codon 71 to a stop codon. The diagnosis of aniridia was confirmed in this atypical patient, who will need to be monitored for his high risk of glaucoma. The risk of developing Wilms` tumor in patients with mutations within the aniridia gene is presumably negligible since the neighboring Wilms` tumor gene is unaffected. The identification of intragenic mutations of the PAX6 gene in patients with sporadic aniridia modifies the management of such patients because of recognition of the increased risk of glaucoma and by reducing the necessity for frequent monitoring for the presence of Wilms` tumor.

  3. RAPID DETERMINATION OF RA-226 IN ENVIRONMENTAL SAMPLES

    SciTech Connect

    Maxwell, S.

    2012-01-03

    A new rapid method for the determination of {sup 226}Ra in environmental samples has been developed at the Savannah River Site Environmental Lab (Aiken, SC, USA) that can be used for emergency response or routine sample analyses. The need for rapid analyses in the event of a Radiological Dispersive Device or Improvised Nuclear Device event is well-known. In addition, the recent accident at Fukushima Nuclear Power Plant in March, 2011 reinforces the need to have rapid analyses for radionuclides in environmental samples in the event of a nuclear accident. {sup 226}Ra (T1/2 = 1,620 years) is one of the most toxic of the long-lived alpha-emitters present in the environment due to its long life and its tendency to concentrate in bones, which increases the internal radiation dose of individuals. The new method to determine {sup 226}Ra in environmental samples utilizes a rapid sodium hydroxide fusion method for solid samples, calcium carbonate precipitation to preconcentrate Ra, and rapid column separation steps to remove interferences. The column separation process uses cation exchange resin to remove large amounts of calcium, Sr Resin to remove barium and Ln Resin as a final purification step to remove {sup 225}Ac and potential interferences. The purified {sup 226}Ra sample test sources are prepared using barium sulfate microprecipitation in the presence of isopropanol for counting by alpha spectrometry. The method showed good chemical recoveries and effective removal of interferences. The determination of {sup 226}Ra in environmental samples can be performed in less than 16 h for vegetation, concrete, brick, soil, and air filter samples with excellent quality for emergency or routine analyses. The sample preparation work takes less than 6 h. {sup 225}Ra (T1/2 = 14.9 day) tracer is used and the {sup 225}Ra progeny {sup 217}At is used to determine chemical yield via alpha spectrometry. The rapid fusion technique is a rugged sample digestion method that ensures that any

  4. Process waste assessment: Color print processing (RA-4)

    SciTech Connect

    Catlett, P.

    1994-05-01

    The Kodak RA-4 process is used to develop prints and overhead transparencies from photographic negatives. The assessment was based on usage, effluent discharge, and final disposition of waste generated by the process. Two options explored were bleach-fix regeneration and the conversion to a digital image processing system. The RA-4 process is process is environmentally sound and generates a relatively small amount of waste. The bleach-fix option would provide only a small effluent reduction. The digital imaging conversion option, if fully implemented, could greatly reduce waste generated in the photo lab.

  5. A CC' loop decoy peptide blocks the interaction between Act1 and IL-17RA to attenuate IL-17- and IL-25-induced inflammation.

    PubMed

    Liu, Caini; Swaidani, Shadi; Qian, Wen; Kang, Zizhen; Sun, Paige; Han, Yue; Wang, Chenhui; Gulen, Muhammet Fatih; Yin, Weiguo; Zhang, Chunjiang; Fox, Paul L; Aronica, Mark; Hamilton, Thomas A; Misra, Saurav; Deng, Junpeng; Li, Xiaoxia

    2011-01-01

    Interleukin-17 (IL-17) and IL-25 signaling induce the expression of genes encoding inflammatory factors and are implicated in the pathology of various inflammatory diseases. Nuclear factor κB (NF-κB) activator 1 (Act1) is an adaptor protein and E3 ubiquitin ligase that is critical for signaling by either IL-17 or IL-25, and it is recruited to their receptors (IL-17R and IL-25R) through heterotypic interactions between the SEFIR [SEF (similar expression to fibroblast growth factor genes) and IL-17R] domain of Act1 and that of the receptor. SEFIR domains have structural similarity with the Toll-IL-1 receptor (TIR) domains of Toll-like receptors and IL-1R. Whereas the BB' loop of TIR is required for TIR-TIR interactions, we found that deletion of the BB' loop from Act1 or IL-17RA (a common subunit of both IL-17R and IL-25R) did not affect Act1-IL-17RA interactions; rather, deletion of the CC' loop from Act1 or IL-17RA abolished the interaction between both proteins. Surface plasmon resonance measurements showed that a peptide corresponding to the CC' loop of Act1 bound directly to IL-17RA. A cell-permeable decoy peptide based on the CC' loop sequence inhibited IL-17- or IL-25-mediated signaling in vitro, as well as IL-17- and IL-25-induced pulmonary inflammation in mice. Together, these findings provide the molecular basis for the specificity of SEFIR-SEFIR versus TIR-TIR domain interactions and consequent signaling. Moreover, we suggest that the CC' loop motif of SEFIR domains is a promising target for therapeutic strategies against inflammatory diseases associated with IL-17 or IL-25 signaling. PMID:22045852

  6. Atypical localizations of calcific deposits in the shoulder

    PubMed Central

    Vinanti, G.B.; Pavan, D.; Rossato, A.; Biz, Carlo

    2015-01-01

    Introduction Calcific tendinopathies of the shoulder are due to inflammation around deposits of calcium within periarticular tendineal structures. Presentation of cases We present three cases of atypical localization of calcium deposits in the shoulder. All of the cases have been treated with arthroscopic excision, followed by post-operative rehabilitation, regaining excellent results. Patients were evaluated 6 months after surgery using the Visual Analogue Scale (VAS), the Simple Shoulder Test (SST) and the UCLA modified shoulder rating. Discussion Calcific tendinopathy is a self-limiting condition or is successfully treated with conservative therapy especially during the early phases of the pathology. If conservative measures fail, removal of calcium deposits is recommended. Arthroscopic management showed good results in our three cases. Conclusion We suggest that arthroscopic treatment of calcific tendonitis guarantees good results even when calcium deposits are in atypical locations. PMID:25884610

  7. Learning, plasticity, and atypical generalization in children with autism.

    PubMed

    Church, Barbara A; Rice, Courtney L; Dovgopoly, Alexander; Lopata, Christopher J; Thomeer, Marcus L; Nelson, Andrew; Mercado, Eduardo

    2015-10-01

    Individuals with autism spectrum disorder (ASD) show accelerated learning in some tasks, degraded learning in others, and distinct deficits when generalizing to novel situations. Recent simulations with connectionist models suggest that deficits in cortical plasticity mechanisms can account for atypical patterns of generalization shown by some children with ASD. We tested the surprising theoretical prediction, from past simulations, that the children with ASD who show atypical generalization in perceptual categorization tasks will benefit more from training with a single prototypical member of the category than from training with multiple examples, but children with ASD who generalize normally will be comparatively harmed. The experimental results confirmed this prediction, suggesting that plasticity deficits may well underlie the difficulties that some children with ASD have generalizing skills, and these deficits are not specific to the acquisition of social skills, but rather reflect a more general perceptual learning deficit that may impact many abilities. PMID:25561418

  8. Atypical presentation of thoracic spondylodiscitis caused by Streptococcus mitis

    PubMed Central

    Cariati, Vincent P; Deng, Wu

    2014-01-01

    Spondylodiscitis, which is most commonly caused by Staphylococcus aureus, is an uncommon infection in adults. The diagnosis of spondylodiscitis is often delayed by its vague and non-specific presentations. As part of the normal flora in human mouth and sinuses, Streptococcus mitis is a very rare cause of spondylodiscitis. We report a case of thoracic spondylodiscitis caused by S. mitis in a patient with chronic sinusitis. The patient atypically presented with a sharp chest pain that radiated to the back and the imaging studies were initially negative. He failed outpatient pain management and the diagnosis of spondylodiscitis was confirmed by bone biopsy 6 weeks later. Treatment with antibiotics completely alleviated the pain. Increased awareness and a high index of suspicion are essential for early diagnosis of spondylodiscitis with an atypical presentation. PMID:24842345

  9. [Mammary hamartoma with atypical stromal cells: a potential diagnostic dilemma].

    PubMed

    Agabiti, S; Gurrera, A; Amico, P; Vasquez, E; Magro, G

    2007-12-01

    Hamartoma of the breast is a pseudotumoural lesion that does not usually pose diagnostic problems for the pathologist. Although atypical stromal cell (ASCs) can be encountered in several benign and malignant breast lesions, their occurrence in hamartoma has not been reported to date. The authors report a case of breast hamartoma containing numerous atypical mono- or multinucleated stromal cells within the fibro-fatty component. This unusual feature raised differential diagnostic problems with pleomorphic lipoma, well-differentiated liposarcoma and malignant phylloid tumour with a lipomatous heterologous component. Immunohistochemistry, showing positivity to vimentin and CD34, revealed that ASCs are fibroblastic in nature, and thus are likely to represent a morphological variant of the fibroblasts of the native mammary stroma. PMID:18416336

  10. Sensitivity of atypical lateral fire spread to wind and slope

    NASA Astrophysics Data System (ADS)

    Simpson, Colin. C.; Sharples, Jason J.; Evans, Jason P.

    2016-02-01

    This study presents new knowledge of the environmental sensitivity of a dynamic mode of atypical wildland fire spread on steep lee-facing slopes. This is achieved through a series of idealized numerical simulations performed with the Weather Research and Forecasting (WRF) and WRF-Fire coupled atmosphere-fire models. The sensitivity of the atypical lateral fire spread across lee slopes is tested for a varying background wind speed, wind direction relative to the terrain aspect, and lee slope steepness. The results indicate that the lateral spread characteristics are highly sensitive to each of these environmental conditions, and there is a broad agreement with the empirical thresholds calculated for lateral spread events observed in the 2003 Canberra bushfires. A theory to explain these environmental thresholds and their apparent interdependency is presented. The results are expected to have important implications for the management of wildland fires in rugged terrain.

  11. [The atypical course of syphilis in HIV infection].

    PubMed

    Mahrle, G; Rasokat, H; Kurz, K; Steigleder, G K

    1989-05-15

    We report on 3 HIV patients showing atypical courses of syphilis. Both the history and serology of the first patient proved a recent re-infection with T. pallidum, whereas the histopathological findings corresponded to an advanced stage of the disease (S II-III). The second patient showed the clinical picture of syphilis maligna with slowly converting and slightly positive serological reactions. The third patient had a refractory syphilis and an early relapse. Our observations suggest that syphilis might take an unusual course in HIV patients. Considering our total HIV clientèle (800 patients greater than or equal to WR 2) the frequency of these atypical cases must be rated very low (0.38%). PMID:2741530

  12. Atypical Behavior Identification in Large Scale Network Traffic

    SciTech Connect

    Best, Daniel M.; Hafen, Ryan P.; Olsen, Bryan K.; Pike, William A.

    2011-10-23

    Cyber analysts are faced with the daunting challenge of identifying exploits and threats within potentially billions of daily records of network traffic. Enterprise-wide cyber traffic involves hundreds of millions of distinct IP addresses and results in data sets ranging from terabytes to petabytes of raw data. Creating behavioral models and identifying trends based on those models requires data intensive architectures and techniques that can scale as data volume increases. Analysts need scalable visualization methods that foster interactive exploration of data and enable identification of behavioral anomalies. Developers must carefully consider application design, storage, processing, and display to provide usability and interactivity with large-scale data. We present an application that highlights atypical behavior in enterprise network flow records. This is accomplished by utilizing data intensive architectures to store the data, aggregation techniques to optimize data access, statistical techniques to characterize behavior, and a visual analytic environment to render the behavioral trends, highlight atypical activity, and allow for exploration.

  13. Diagnosis and management of typical and atypical lung carcinoids.

    PubMed

    Pusceddu, Sara; Lo Russo, Giuseppe; Macerelli, Marianna; Proto, Claudia; Vitali, Milena; Signorelli, Diego; Ganzinelli, Monica; Scanagatta, Paolo; Duranti, Leonardo; Trama, Annalisa; Buzzoni, Roberto; Pelosi, Giuseppe; Pastorino, Ugo; de Braud, Filippo; Garassino, Marina Chiara

    2016-04-01

    An estimated 20% to 30% of all neuroendocrine tumours originate in the bronchial tree and lungs. According to the 2015 World Health Organization categorization, these tumours are separated into four subtypes characterized by increasing biological aggressiveness: typical carcinoid, atypical carcinoid, large-cell neuroendocrine carcinoma and small-cell carcinoma. Although typical and atypical lung carcinoids account for less than 1-5% of all pulmonary malignancies, the incidence of these neoplasms has risen significantly in recent decades. Surgery is the treatment of choice for loco-regional disease but for advanced lung carcinoids there is no recognized standard of care and successful management requires a multidisciplinary approach. The aim of this review is to provide a useful guide for the clinical management of lung carcinoids. PMID:26917456

  14. Critical appraisal of eculizumab for atypical hemolytic uremic syndrome

    PubMed Central

    Palma, Lilian M Pereira; Langman, Craig B

    2016-01-01

    The biology of atypical hemolytic uremic syndrome has been shown to involve inability to limit activation of the alternative complement pathway, with subsequent damage to systemic endothelial beds and the vasculature, resulting in the prototypic findings of a thrombotic microangiopathy. Central to this process is the formation of the terminal membrane attack complex C5b-9. Recently, application of a monoclonal antibody that specifically binds to C5, eculizumab, became available to treat patients with atypical hemolytic uremic syndrome, replacing plasma exchange or infusion as primary therapy. This review focuses on the evidence, based on published clinical trials, case series, and case reports, on the efficacy and safety of this approach. PMID:27110144

  15. IL-1ra Secreted by ATP-Induced P2Y2 Negatively Regulates MUC5AC Overproduction via PLCβ3 during Airway Inflammation

    PubMed Central

    Kim, Jiwook; Kim, Bokyoum; Kim, Joowon; Shin, Yusom; Kim, Judeok; Ryu, Siejeong; Yang, Yu-Mi

    2016-01-01

    Mucus secretion is often uncontrolled in many airway inflammatory diseases of humans. Identifying the regulatory pathway(s) of mucus gene expression, mucus overproduction, and hypersecretion is important to alleviate airway inflammation in these diseases. However, the regulatory signaling pathway controlling mucus overproduction has not been fully identified yet. In this study, we report that the ATP/P2Y2 complex secretes many cytokines and chemokines to regulate airway inflammation, among which IL-1 receptor antagonist (IL-1ra) downregulates MUC5AC gene expression via the inhibition of Gαq-induced Ca2+ signaling. IL-1ra inhibited IL-1α protein expression and secretion, and vice versa. Interestingly, ATP/P2Y2-induced IL-1ra and IL-1α secretion were both mediated by PLCβ3. A dominant-negative mutation in the PDZ-binding domain of PLCβ3 inhibited ATP/P2Y2-induced IL-1ra and IL-1α secretion. IL-1α in the presence of the ATP/P2Y2 complex activated the ERK1/2 pathway in a greater degree and for a longer duration than the ATP/P2Y2 complex itself, which was dramatically inhibited by IL-1ra. These findings suggest that secreted IL-1ra exhibits a regulatory effect on ATP/P2Y2-induced MUC5AC gene expression, through inhibition of IL-1α secretion, to maintain the mucus homeostasis in the airway. Therefore, IL-1ra could be an excellent modality for regulating inflamed airway microenvironments in respiratory diseases. PMID:27034593

  16. Buspirone hydrochloride in the treatment of an atypical paraphilia.

    PubMed

    Fedoroff, J P

    1992-08-01

    A case report involving buspirone hydrochloride in the successful treatment of a patient with an atypical paraphilia and transvestic fetishism is presented. Treatment outcome was assessed by the patient's self-report as well as by retrospective examination of detailed notes about paraphilia fantasies which unknown to the therapists, had been kept by the patient. Preliminary evidence indicates that buspirone appears to effectively treat some paraphilias. PMID:1497477

  17. Atypical spatiotemporal signatures of working memory brain processes in autism.

    PubMed

    Urbain, C M; Pang, E W; Taylor, M J

    2015-01-01

    Working memory (WM) impairments may contribute to the profound behavioural manifestations in children with autism spectrum disorder (ASD). However, previous behavioural results are discrepant as are the few functional magnetic resonance imaging (fMRI) results collected in adults and adolescents with ASD. Here we investigate the precise temporal dynamics of WM-related brain activity using magnetoencephalography (MEG) in 20 children with ASD and matched controls during an n-back WM task across different load levels (1-back vs 2-back). Although behavioural results were similar between ASD and typically developing (TD) children, the between-group comparison performed on functional brain activity showed atypical WM-related brain processes in children with ASD compared with TD children. These atypical responses were observed in the ASD group from 200 to 600 ms post stimulus in both the low- (1-back) and high- (2-back) memory load conditions. During the 1-back condition, children with ASD showed reduced WM-related activations in the right hippocampus and the cingulate gyrus compared with TD children who showed more activation in the left dorso-lateral prefrontal cortex and the insulae. In the 2-back condition, children with ASD showed less activity in the left insula and midcingulate gyrus and more activity in the left precuneus than TD children. In addition, reduced activity in the anterior cingulate cortex was correlated with symptom severity in children with ASD. Thus, this MEG study identified the precise timing and sources of atypical WM-related activity in frontal, temporal and parietal regions in children with ASD. The potential impacts of such atypicalities on social deficits of autism are discussed. PMID:26261885

  18. Epithelioid Schwannomas: An Analysis of 58 Cases Including Atypical Variants.

    PubMed

    Hart, Jesse; Gardner, Jerad M; Edgar, Mark; Weiss, Sharon W

    2016-05-01

    The histologic features and outcome of 58 cases of epithelioid schwannoma were studied to determine the significance of atypical histologic features. Cases were retrieved from personal consultation files from 1999 to 2013. Patients (31 male and 26 female patients) ranged in age from 14 to 80 years (median, 38 y). Two patients had schwannomatosis 1. Tumors developed in the dermis/subcutis (n=56) or muscle (n=2) of the upper extremity (34.5%), lower extremity (34.5%), thorax/abdomen/back (18%), and less common anatomic locations including the scalp, neck, lip, and breast. They ranged in size from 0.25 to 4.5 cm (median, 2.0 cm). Typically circumscribed and surrounded by a perineurium, they comprised single or small groups of epithelioid schwann cells with a moderate amphophilic cytoplasm and occasional nuclear pseudoinclusions. Stroma varied from myxoid to hyalinized, often with thick-walled vessels (55 cases). Mitotic rate ranged from 0 to 9 mitoses/10 high-power field (HPF) (2.37 mm) in the most active areas (mean, 2 to 3 mitoses/10 HPFs). Thirteen cases (22%) were "atypical," defined by a high mitotic rate (≥3 mitoses per 10 HPFs) and nuclear size variation (≥3:1). All (56/56) expressed S100 protein; type IV collagen invested groups or individual cells (16/17). Melanoma markers were negative, except for melan A (1 case). Follow-up in 39 patients (median, 78 mo; range, 6 to 174 mo) indicated that 31 (79%) were alive without disease (including 9/13 atypical cases; median, 78 mo), 7 (18%) were alive with unknown status, and 1 patient had died of unrelated causes. One tumor recurred, but none metastasized. Epithelioid schwannomas, even those with atypical features, are benign and do not constitute a histologic continuum with epithelioid malignant peripheral nerve sheath tumors, which typically occur in deep soft tissues and have more anaplastic features. PMID:26752543

  19. A Case of Atypical Cleft Hand - Reported with Ontogenetic Review

    PubMed Central

    Kundu, Sujit Kumar; Datta, Abhijit

    2014-01-01

    An asymptomatic atypical U shaped cleft hand has been found in a 21-year-old lady attending OPD. On digital skiagram it was found that central digits were absent with remnants of bases of the metacarpals, which have fused with the carpal bones. Moreover, the scaphoid and trapezium had fused to form a single mass. There was no other anomaly in other limbs, so far searched for. An endeavor has been made to explain the anomaly with ontogenetic review. PMID:25653934

  20. Early adjuvant radiotherapy in the treatment of atypical meningioma.

    PubMed

    Jenkinson, Michael D; Waqar, Mueez; Farah, Jibril Osman; Farrell, Michael; Barbagallo, Giuseppe M V; McManus, Robin; Looby, Seamus; Hussey, Deirdre; Fitzpatrick, David; Certo, Francesco; Javadpour, Mohsen

    2016-06-01

    Atypical meningiomas have a greater propensity to recur than benign meningiomas and the benefits of early adjuvant radiotherapy are unclear. Existing studies report conflicting results. This retrospective cohort study evaluated the role of early adjuvant radiotherapy following surgical resection of atypical meningioma. A triple center case-note review of adults with newly-diagnosed atypical meningiomas between 2001 and 2010 was performed. Pathology diagnosis was made according to the World Health Organization classification in use at the time of surgery. Patients with multiple meningiomas, neurofibromatosis type 2 and radiation-induced meningiomas were excluded. Extent of resection was defined as gross total resection (GTR; Simpson Grade I-III) or subtotal resection (STR; Simpson Grade IV-V). Survival analysis was performed using the Kaplan-Meier method. One hundred thirty-three patients were identified with a median age of 62years (range 22-86years) and median follow-up of 57.4months (range 0.1-152.2months). Tumors were mostly located in the convexity (50.4%) or falcine/parasagittal regions (27.1%). GTR (achieved in 85%) was associated with longer progression free survival (PFS) (5year PFS 81.2% versus 40.08%, log-rank=11.117, p=0.001) but not overall survival (OS) (5year OS 76.6% versus 39.7%, log-rank=3.652, p=0.056). Following GTR, early adjuvant radiotherapy was administered to 28.3% of patients and did not influence OS (5year OS 77.0% versus 75.7%, log-rank=0.075, p=0.784) or PFS (5year PFS 82.0% versus 79.3%, log-rank=0.059, p=0.808). Although extent of resection emerged as an important prognostic variable, early adjuvant radiotherapy did not influence outcome following GTR of atypical meningiomas. Prospective randomized controlled trials are planned. PMID:26775147

  1. A case of atypical cleft hand - reported with ontogenetic review.

    PubMed

    Kundu, Sujit Kumar; Roy, Hironmoy; Datta, Abhijit

    2014-12-01

    An asymptomatic atypical U shaped cleft hand has been found in a 21-year-old lady attending OPD. On digital skiagram it was found that central digits were absent with remnants of bases of the metacarpals, which have fused with the carpal bones. Moreover, the scaphoid and trapezium had fused to form a single mass. There was no other anomaly in other limbs, so far searched for. An endeavor has been made to explain the anomaly with ontogenetic review. PMID:25653934

  2. Pathogenesis, Management and Prevention of Atypical Femoral Fractures

    PubMed Central

    Jeong, Seung-Hyo

    2015-01-01

    Much attention has been paid to the relationship between atypical femoral fractures (AFF) and use of bisphosphonates (BPs). While a significant cause-effect relationship was not established in earlier studies, more recent data shows a growing relationship between AFF and BPs use. The definition of an 'AFF' has also undergone significant changes. This review briefly summarizes the definition, pathogenesis, and management of AFF. PMID:25774358

  3. A hoarse voice: atypical mycobacterial infection of the larynx.

    PubMed

    McEwan, J A; Mohsen, A H; Schmid, M L; McKendrick, M W

    2001-11-01

    Myobacterium malmoense is a non-tuberculous mycobacterium that most commonly causes pulmonary infection, particularly in patients with underlying pulmonary disease or immunodeficiency. We describe a case of Mycobacterium malmoense infection of the larynx in a previously well middle-aged woman, which has previously not been reported. The case highlights the importance of considering atypical mycobacterial infection in the differential diagnosis of laryngeal lesions. PMID:11779312

  4. Psychological and Behavioral Dynamics in Chronic Atypical Facial Pain

    PubMed Central

    Baile, Walter F.; Myers, Daniel

    1986-01-01

    The authors discuss the relationship between atypical facial pain and psychiatric disturbance. They present contemporary viewpoints and describe four cases that illustrate underlying psychodynamic mechanisms associated with pain in patients who had undergone various dental procedures and other treatments without success. They identify factors which might lead to the early detection of underlying psychological problems and discuss the role of learning, the family system and other factors in producing a chronic pain syndrome. PMID:3465263

  5. An atypical clay shoveler's fracture: a case report

    PubMed Central

    Feldman, Victor B; Astri, Frank

    2001-01-01

    A case of an atypical clay shoveler's fracture with involvement of the spinolaminar line is described. Causative mechanisms of injury, radiographic appearances, differential diagnosis, treatment and prognosis are reviewed. Classic clay shoveler's fractures are considered stable fractures. However, when the spinolaminar line is disrupted, spinal cord involvement must be ruled out. ImagesFigure 1Figure 2Figure 3Figure 4aFigure 4bFigure 5Figure 6

  6. Paranoid Personality Masking an Atypical Case of Frontotemporal Dementia

    PubMed Central

    Iroka, Nneka; Jehangir, Waqas; II, Jay Littlefield; Pattan, Vishwanath; Yousif, Abdalla; Mishra, Arunesh K

    2015-01-01

    Frontotemporal dementia (FTD) is a debilitating disease that is well described in the “Diagnostic and statistical manual of mental disorders, fifth edition (DSM-5)”, and typically presents with memory impairment, progressive decline in cortical functioning, and behavioral changes. Age of onset is generally in the late fifties, and usually the first presentation involves a change in behavior and emotional blunting. Treatment of FTD involves management of any neurobehavioral symptoms while trials of atypical antipsychotics are ongoing but suggest some efficacy. We present a case of a patient who first presented with severe paranoid personality traits and frank persecutory delusions. This atypical presentation of our patient first led to her incorrect diagnosis of a psychotic disorder and paranoid personality disorder. As a result of this diagnosis, she was treated unsuccessfully. A subsequent magnetic resonance imaging (MRI) then showed atrophy of frontal and temporal lobes bilaterally (left more prominent than right) which confirmed the diagnosis of FTD. The importance of this case involves the atypical presentation of paranoia and delusions, and our patient’s incorrect diagnosis based on her clinical presentation led to a trial of unsuccessful treatment. Only after performing an MRI, which showed atrophy, was the patient appropriately treated and deemed medically stable. This case report illustrates the importance of considering a rare presentation of frontotemporal lobe dementia with patients who are in the typical age range and present with paranoia and delusions. PMID:25780487

  7. Paranoid personality masking an atypical case of frontotemporal dementia.

    PubMed

    Iroka, Nneka; Jehangir, Waqas; Ii, Jay Littlefield; Pattan, Vishwanath; Yousif, Abdalla; Mishra, Arunesh K

    2015-05-01

    Frontotemporal dementia (FTD) is a debilitating disease that is well described in the "Diagnostic and statistical manual of mental disorders, fifth edition (DSM-5)", and typically presents with memory impairment, progressive decline in cortical functioning, and behavioral changes. Age of onset is generally in the late fifties, and usually the first presentation involves a change in behavior and emotional blunting. Treatment of FTD involves management of any neurobehavioral symptoms while trials of atypical antipsychotics are ongoing but suggest some efficacy. We present a case of a patient who first presented with severe paranoid personality traits and frank persecutory delusions. This atypical presentation of our patient first led to her incorrect diagnosis of a psychotic disorder and paranoid personality disorder. As a result of this diagnosis, she was treated unsuccessfully. A subsequent magnetic resonance imaging (MRI) then showed atrophy of frontal and temporal lobes bilaterally (left more prominent than right) which confirmed the diagnosis of FTD. The importance of this case involves the atypical presentation of paranoia and delusions, and our patient's incorrect diagnosis based on her clinical presentation led to a trial of unsuccessful treatment. Only after performing an MRI, which showed atrophy, was the patient appropriately treated and deemed medically stable. This case report illustrates the importance of considering a rare presentation of frontotemporal lobe dementia with patients who are in the typical age range and present with paranoia and delusions. PMID:25780487

  8. Atypical Findings of Guillain-Barré Syndrome in Children

    PubMed Central

    KARIMZADEH, Parvaneh; BAKHSHANDEH BALI, Mohammad Kazem; NASEHI, Mohammad Mehdi; TAHERI OTAGHSARA, Seyyed Mohaddeseh; Ghofrani, Mohammad

    2012-01-01

    Objective Guillain-Barre syndrome (GBS) is an immune-mediated polyneuropathy that occurs mostly after prior infection. The diagnosis of this syndrome is dependent heavily on the history and examination, although cerebrospinal fluid analysis and electrodiagnostic testing usually confirm the diagnosis. This is a retrospective study which was performed to investigate the atypical features of GBS. Materials & Methods Thirty three patients (21/63.6% males and 12/36.4% females) with GBS were retrospectively studied and prospectively evaluated at the Child Neurology institute of Mofid Children Hospital of Shahid Beheshti University of Medical Sciences between May 2011 and September 2012. Results The mean age was 5.4 years (range, 1.5-10.5).Twenty one patients (87.9%) had previous history of infections. Eight patients (24.2%) admitted with atypical symptoms like upper limb weakness (3%), ptosis (3%), neck stiffness (3%), inability to stand (proximal weakness) (9.1%), headache (3%) and dysphagia (3%).According to disease process, weakness was ascending in 26 (78.8%), descending in 5 (15.2%) and static in 2 (6.1%) patients. Cranial nerve involvement was found in 8(24.3%) children, most commonly as facial palsy in 3 (9.1%). Conclusion In this study, 24.3% of our patients presented with atypical symptoms of GBS as upper limb weakness, ptosis, neck stiffness, inability to stand (proximal weakness), headache and dysphagia. PMID:24665275

  9. Atypical resource allocation may contribute to many aspects of autism

    PubMed Central

    Goldknopf, Emily J.

    2013-01-01

    Based on a review of the literature and on reports by people with autism, this paper suggests that atypical resource allocation is a factor that contributes to many aspects of autism spectrum conditions, including difficulties with language and social cognition, atypical sensory and attentional experiences, executive and motor challenges, and perceptual and conceptual strengths and weaknesses. Drawing upon resource theoretical approaches that suggest that perception, cognition, and action draw upon multiple pools of resources, the approach hypothesizes that compared with resources in typical cognition, resources in autism are narrowed or reduced, especially in people with strong sensory symptoms. In narrowed attention, resources are restricted to smaller areas and to fewer modalities, stages of processing, and cognitive processes than in typical cognition; narrowed resources may be more intense than in typical cognition. In reduced attentional capacity, overall resources are reduced; resources may be restricted to fewer modalities, stages of processing, and cognitive processes than in typical cognition, or the amount of resources allocated to each area or process may be reduced. Possible neural bases of the hypothesized atypical resource allocation, relations to other approaches, limitations, and tests of the hypotheses are discussed. PMID:24421760

  10. Face processing in Williams syndrome is already atypical in infancy.

    PubMed

    D'Souza, Dean; Cole, Victoria; Farran, Emily K; Brown, Janice H; Humphreys, Kate; Howard, John; Rodic, Maja; Dekker, Tessa M; D'Souza, Hana; Karmiloff-Smith, Annette

    2015-01-01

    Face processing is a crucial socio-cognitive ability. Is it acquired progressively or does it constitute an innately-specified, face-processing module? The latter would be supported if some individuals with seriously impaired intelligence nonetheless showed intact face-processing abilities. Some theorists claim that Williams syndrome (WS) provides such evidence since, despite IQs in the 50s, adolescents/adults with WS score in the normal range on standardized face-processing tests. Others argue that atypical neural and cognitive processes underlie WS face-processing proficiencies. But what about infants with WS? Do they start with typical face-processing abilities, with atypicality developing later, or are atypicalities already evident in infancy? We used an infant familiarization/novelty design and compared infants with WS to typically developing controls as well as to a group of infants with Down syndrome matched on both mental and chronological age. Participants were familiarized with a schematic face, after which they saw a novel face in which either the features (eye shape) were changed or just the configuration of the original features. Configural changes were processed successfully by controls, but not by infants with WS who were only sensitive to featural changes and who showed syndrome-specific profiles different from infants with the other neurodevelopmental disorder. Our findings indicate that theorists can no longer use the case of WS to support claims that evolution has endowed the human brain with an independent face-processing module. PMID:26124729

  11. Elevated rates of atypical handedness in paedophilia: Theory and implications

    PubMed Central

    Fazio, Rachel L.; Lykins, Amy D.; Cantor, James M.

    2014-01-01

    Multiple factors determine handedness including genetics, prenatal stress and post-natal environmental conditions. Atypical handedness, whether manifest as increased sinistrality or decreased strength of lateral preference, has been noted in a wide variety of populations with neuropathology. Those with atypical sexual preferences, specifically paedophilia, also manifest reduced rates of right-handedness. This paper uses the largest sample of phallometrically assessed men to date to establish the pattern of atypical handedness in paedophilia. Specifically, whereas prior research has largely characterized participants dichotomously as right-handed or non-right-handed and/or used self-report of writing hand, this paper expands upon such reports by using the Edinburgh Handedness Inventory's laterality quotient. Participants' handedness and phallometrically assessed sexual preference were analyzed both as continuous and categorical variables, and the responses of those scoring in the range of ambiguous-handedness were evaluated to ascertain whether they were ambiguously handed or more accurately described as mixed-handed. Results indicated those producing scores in the range of ambiguous-handedness demonstrated response patterns consistent with ambiguous-handedness, rather than mixed-handedness. Paedophiles demonstrated high rates of non-right-handedness primarily manifested as sinistrality, whereas those who had a sexual preference for pubescent children evidenced increased ambiguous-handedness. Results support a view of ambiguous-handedness as less pathological than previously hypothesized, and of a neurodevelopmental origin of paraphilic sexual preferences. PMID:24666135

  12. Pregnancy-Associated Atypical Hemolytic-Uremic Syndrome

    PubMed Central

    Saad, Antonio F.; Roman, Jorge; Wyble, Aaron; Pacheco, Luis D.

    2016-01-01

    Précis Introduction Early diagnosis of atypical uremic–hemolytic syndrome may be challenging during the puerperium period. Correct diagnosis and timely management are crucial to improve outcomes. Background Pregnancy-associated atypical hemolytic-uremic syndrome (p-aHUS) is a rare condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Triggered by pregnancy, genetically predisposed women develop the syndrome, leading to a disastrous hemolytic disease characterized by diffuse endothelial damage and platelet consumption. This disease is a life-threatening condition that requires prompt diagnosis and therapy. Case A 19-year-old G1P1 Caucasian female with suspicion of HELLP syndrome was treated at our facility for severe thrombocytopenia and acute kidney injury. A diagnosis of atypical uremic–hemolytic syndrome was later confirmed. The patient's condition improved with normalization of platelets and improvement in kidney function after 14 days of plasmapheresis. She was subsequently treated with eculizumab, a monoclonal antibody against C5. The patient tolerated well the therapy and is currently in remission. Conclusion Diagnosis of p-aHUS is challenging, as it can mimic various diseases found during pregnancy and the postpartum. Plasma exchange should be promptly initiated within 24 hours of diagnosis. Eculizumab has risen to become an important tool to improve long-term comorbidities and mortality in this group population. PMID:26989566

  13. Elevated rates of atypical handedness in paedophilia: theory and implications.

    PubMed

    Fazio, Rachel L; Lykins, Amy D; Cantor, James M

    2014-01-01

    Multiple factors determine handedness including genetics, prenatal stress and post-natal environmental conditions. Atypical handedness, whether manifest as increased sinistrality or decreased strength of lateral preference, has been noted in a wide variety of populations with neuropathology. Those with atypical sexual preferences, specifically paedophilia, also manifest reduced rates of right-handedness. This paper uses the largest sample of phallometrically assessed men to date to establish the pattern of atypical handedness in paedophilia. Specifically, whereas prior research has largely characterized participants dichotomously as right-handed or non-right-handed and/or used self-report of writing hand, this paper expands upon such reports by using the Edinburgh Handedness Inventory's laterality quotient. Participants' handedness and phallometrically assessed sexual preference were analyzed both as continuous and categorical variables, and the responses of those scoring in the range of ambiguous-handedness were evaluated to ascertain whether they were ambiguously handed or more accurately described as mixed-handed. Results indicated those producing scores in the range of ambiguous-handedness demonstrated response patterns consistent with ambiguous-handedness, rather than mixed-handedness. Paedophiles demonstrated high rates of non-right-handedness primarily manifested as sinistrality, whereas those who had a sexual preference for pubescent children evidenced increased ambiguous-handedness. Results support a view of ambiguous-handedness as less pathological than previously hypothesized, and of a neurodevelopmental origin of paraphilic sexual preferences. PMID:24666135

  14. Pregnancy-Associated Atypical Hemolytic-Uremic Syndrome.

    PubMed

    Saad, Antonio F; Roman, Jorge; Wyble, Aaron; Pacheco, Luis D

    2016-03-01

    Introduction Early diagnosis of atypical uremic-hemolytic syndrome may be challenging during the puerperium period. Correct diagnosis and timely management are crucial to improve outcomes. Background Pregnancy-associated atypical hemolytic-uremic syndrome (p-aHUS) is a rare condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Triggered by pregnancy, genetically predisposed women develop the syndrome, leading to a disastrous hemolytic disease characterized by diffuse endothelial damage and platelet consumption. This disease is a life-threatening condition that requires prompt diagnosis and therapy. Case A 19-year-old G1P1 Caucasian female with suspicion of HELLP syndrome was treated at our facility for severe thrombocytopenia and acute kidney injury. A diagnosis of atypical uremic-hemolytic syndrome was later confirmed. The patient's condition improved with normalization of platelets and improvement in kidney function after 14 days of plasmapheresis. She was subsequently treated with eculizumab, a monoclonal antibody against C5. The patient tolerated well the therapy and is currently in remission. Conclusion Diagnosis of p-aHUS is challenging, as it can mimic various diseases found during pregnancy and the postpartum. Plasma exchange should be promptly initiated within 24 hours of diagnosis. Eculizumab has risen to become an important tool to improve long-term comorbidities and mortality in this group population. PMID:26989566

  15. Evidence for Broadening Criteria for Atypical Depression Which May Define a Reactive Depressive Disorder

    PubMed Central

    Silverstein, Brett; Angst, Jules

    2015-01-01

    Objective. Arguing that additional symptoms should be added to the criteria for atypical depression. Method. Published research articles on atypical depression are reviewed. Results. (1) The original studies upon which the criteria for atypical depression were based cited fatigue, insomnia, pain, and loss of weight as characteristic symptoms. (2) Several studies of DSM depressive criteria found patients with atypical depression to exhibit high levels of insomnia, fatigue, and loss of appetite/weight. (3) Several studies have found atypical depression to be comorbid with headaches, bulimia, and body image issues. (4) Most probands who report atypical depression meet criteria for “somatic depression,” defined as depression associated with several of disordered eating, poor body image, headaches, fatigue, and insomnia. The gender difference in prevalence of atypical depression results from its overlap with somatic depression. Somatic depression is associated with psychosocial measures related to gender, linking it with the descriptions of atypical depression as “reactive” appearing in the studies upon which the original criteria for atypical depression were based. Conclusion. Insomnia, disordered eating, poor body image, and aches/pains should be added as criteria for atypical depression matching criteria for somatic depression defining a reactive depressive disorder possibly distinct from endogenous melancholic depression. PMID:26258131

  16. "LJ" Series "Redefining RA": Keeping up with Genres

    ERIC Educational Resources Information Center

    Wyatt, Neal

    2008-01-01

    Genre is a hot issue in readers' advisory (RA) circles right now. Many people are looking at how genre functions and morphs. Studying a genre is not a new idea. Over two decades ago, and even longer in less formal ways, librarians began gathering to study genres--asking such questions as in what genre does this book fit and why, what are its…

  17. Observation of the negative ions: Ra[sup [minus

    SciTech Connect

    Zhao, X.; Nadeau, M.; Garwan, M.A.; Kilius, L.R.; Litherland, A.E. )

    1993-11-01

    The negative ions of the isotopes [sup 226]Ra, [sup 231]Pa, and [sup 244]Pu have been observed by means of accelerator mass spectrometry and their properties compared with the negative ions of Th and U. The electron affinities of all these elements have been estimated to be similar and greater than 50 meV.

  18. Improved experimental limit on the EDM of 225Ra

    NASA Astrophysics Data System (ADS)

    Bishof, Michael; Bailey, Kevin; Dietrich, Matthew R.; Greene, John P.; Holt, Roy J.; Kalita, Mukut R.; Korsch, Wolfgang; Lemke, Nathan D.; Lu, Zheng-Tian; Mueller, Peter; O'Connor, Tom P.; Parker, Richard H.; Rabga, Tenzin; Singh, Jaideep T.

    2015-10-01

    Searches for permanent electric dipole moments (EDMs) in fundamental and composite particles are sensitive probes of beyond-standard-model symmetry violation that could explain the dominance of matter over anti-matter. The 225Ra (t1/2 = 15d, I = 1/2) atom is a particularly attractive system to use for an EDM measurement because its large nuclear octupole deformation, closely spaced ground-state parity doublet, and large atomic mass make 225Ra uniquely sensitive to symmetry-violating interactions in the nuclear medium. We have developed an experiment to measure the EDM of 225Ra and demonstrated the first ``proof-of-principle'' measurement, giving a 95% confidence upper limit of 5E-22 e-cm. After implementing a vacuum upgrade, we have observed nuclear spin coherence after 20 s of free evolution - a factor of ten improvement over our earlier results - and have lowered the 225Ra EDM limit by over an order of magnitude. Upcoming experimental upgrades have the potential to further improve our EDM sensitivity by many orders of magnitude, allowing us to test symmetry violation at an unprecedented level. This work is supported by U.S. DOE, Office of Science, Office of Nuclear Physics, under Contract DE-AC02-06CH11357.

  19. Genome Sequence of Avirulent Riemerella anatipestifer Strain RA-JLLY

    PubMed Central

    Zhang, Rongrong; Luo, Qingping; Wen, Guoyuan; Ai, Diyun; Wang, Honglin; Luo, Ling; Wang, Hongcai

    2015-01-01

    Riemerella anatipestifer is an important bacterial pathogen associated with epizootic infections in waterfowl and various other birds. Riemerella anatipestifer strain RA-JLLY is an avirulent strain, isolated from the brain of an old duck in Hubei province, China. Here, we report the genome sequence of this species. PMID:26404587

  20. Improved limit on the 225Ra electric dipole moment

    NASA Astrophysics Data System (ADS)

    Bishof, Michael; Parker, Richard H.; Bailey, Kevin G.; Greene, John P.; Holt, Roy J.; Kalita, Mukut R.; Korsch, Wolfgang; Lemke, Nathan D.; Lu, Zheng-Tian; Mueller, Peter; O'Connor, Thomas P.; Singh, Jaideep T.; Dietrich, Matthew R.

    2016-08-01

    Background: Octupole-deformed nuclei, such as that of 225Ra, are expected to amplify observable atomic electric dipole moments (EDMs) that arise from time-reversal and parity-violating interactions in the nuclear medium. In 2015 we reported the first "proof-of-principle" measurement of the 225Ra atomic EDM. Purpose: This work reports on the first of several experimental upgrades to improve the statistical sensitivity of our 225Ra EDM measurements by orders of magnitude and evaluates systematic effects that contribute to current and future levels of experimental sensitivity. Method: Laser-cooled and trapped 225Ra atoms are held between two high-voltage electrodes in an ultrahigh-vacuum chamber at the center of a magnetically-shielded environment. We observe Larmor precession in a uniform magnetic field using nuclear-spin-dependent laser light scattering and look for a phase shift proportional to the applied electric field, which indicates the existence of an EDM. The main improvement to our measurement technique is an order-of-magnitude increase in spin-precession time, which is enabled by an improved vacuum system and a reduction in trap-induced heating. Results: We have measured the 225Ra atomic EDM to be less than 1.4 ×10-23e cm (95 % confidence upper limit), which is a factor of 36 improvement over our previous result. Conclusions: Our evaluation of systematic effects shows that this measurement is completely limited by statistical uncertainty. Combining this measurement technique with planned experimental upgrades, we project a statistical sensitivity at the 1 ×10-28e cm level and a total systematic uncertainty at the 4 ×10-29e cm level.

  1. EFFECTS OF FOSSIL MAGNETIC FIELDS ON CONVECTIVE CORE DYNAMOS IN A-TYPE STARS

    SciTech Connect

    Featherstone, Nicholas A.; Toomre, Juri; Browning, Matthew K.; Brun, Allan Sacha

    2009-11-01

    The vigorous magnetic dynamo action achieved within the convective cores of A-type stars may be influenced by fossil magnetic fields within their radiative envelopes. We study such effects through three-dimensional simulations that model the inner 30% by radius of a 2 M {sub sun} A-type star, capturing the convective core and a portion of the overlying radiative envelope within our computational domain. We employ the three-dimensional anelastic spherical harmonic code to model turbulent dynamics within a deep rotating spherical shell. The interaction between a fossil field and the core dynamo is examined by introducing a large-scale magnetic field into the radiative envelope of a mature A star dynamo simulation. We find that the inclusion of a twisted toroidal fossil field can lead to a remarkable transition in the core dynamo behavior. Namely, a super-equipartition state can be realized in which the magnetic energy built by dynamo action is 10-fold greater than the kinetic energy of the convection itself. Such strong-field states may suggest that the resulting Lorentz forces should seek to quench the flows, yet we have achieved super-equipartition dynamo action that persists for multiple diffusion times. This is achieved by the relative co-alignment of the flows and magnetic fields in much of the domain, along with some lateral displacements of the fastest flows from the strongest fields. Convection in the presence of such strong magnetic fields typically manifests as 4-6 cylindrical rolls aligned with the rotation axis, each possessing central axial flows that imbue the rolls with a helical nature. The roll system also possesses core-crossing flows that couple distant regions of the core. We find that the magnetic fields exhibit a comparable global topology with broad, continuous swathes of magnetic field linking opposite sides of the convective core. We have explored several poloidal and toroidal fossil field geometries, finding that a poloidal component is

  2. Enhanced excitability and suppression of A-type K+ currents in joint sensory neurons in a murine model of antigen-induced arthritis

    PubMed Central

    Qu, Lintao; Caterina, Michael J.

    2016-01-01

    Pain is a dominant symptom of rheumatoid arthritis (RA) and its adequate treatment represents a major unmet need. However, the cellular mechanisms that drive arthritis pain are largely unexplored. Here, we examined the changes in the activity of joint sensory neurons and the associated ionic mechanisms using an animal model of antigen-induced arthritis (AIA). Methylated-bovine serum albumin (mBSA), but not vehicle challenge, in the ankle of previously immunized mice produced time-dependent symptoms of arthritis, including joint inflammation, primary mechanical hyperalgesia in the ipsilateral ankle, and secondary mechanical and heat hyperalgesia in the ipsilateral hindpaw. In vivo electrophysiological recordings revealed that Dil-labeled joint sensory neurons in AIA mice exhibited a greater incidence of spontaneous activity, mechanically evoked after-discharges, and/or increased responses to mechanical stimulation of their receptive fields, compared to control animals. Whole-cell recordings in vitro showed that AIA enhanced the excitability of joint sensory neurons. These signs of neuronal hyperexcitability were associated with a significant reduction in the density of A-type K+ currents. Thus, our data suggest that neuronal hyperexcitability, brought about in part by reduced A-type K+ currents, may contribute to pain-related behaviors that accompany antigen-induced arthritis and/or other antigen-mediated diseases. PMID:27363579

  3. Enhanced excitability and suppression of A-type K(+) currents in joint sensory neurons in a murine model of antigen-induced arthritis.

    PubMed

    Qu, Lintao; Caterina, Michael J

    2016-01-01

    Pain is a dominant symptom of rheumatoid arthritis (RA) and its adequate treatment represents a major unmet need. However, the cellular mechanisms that drive arthritis pain are largely unexplored. Here, we examined the changes in the activity of joint sensory neurons and the associated ionic mechanisms using an animal model of antigen-induced arthritis (AIA). Methylated-bovine serum albumin (mBSA), but not vehicle challenge, in the ankle of previously immunized mice produced time-dependent symptoms of arthritis, including joint inflammation, primary mechanical hyperalgesia in the ipsilateral ankle, and secondary mechanical and heat hyperalgesia in the ipsilateral hindpaw. In vivo electrophysiological recordings revealed that Dil-labeled joint sensory neurons in AIA mice exhibited a greater incidence of spontaneous activity, mechanically evoked after-discharges, and/or increased responses to mechanical stimulation of their receptive fields, compared to control animals. Whole-cell recordings in vitro showed that AIA enhanced the excitability of joint sensory neurons. These signs of neuronal hyperexcitability were associated with a significant reduction in the density of A-type K(+) currents. Thus, our data suggest that neuronal hyperexcitability, brought about in part by reduced A-type K(+) currents, may contribute to pain-related behaviors that accompany antigen-induced arthritis and/or other antigen-mediated diseases. PMID:27363579

  4. Newborn brain event-related potentials revealing atypical processing of sound frequency and the subsequent association with later literacy skills in children with familial dyslexia.

    PubMed

    Leppänen, Paavo H T; Hämäläinen, Jarmo A; Salminen, Hanne K; Eklund, Kenneth M; Guttorm, Tomi K; Lohvansuu, Kaisa; Puolakanaho, Anne; Lyytinen, Heikki

    2010-01-01

    The role played by an auditory-processing deficit in dyslexia has been debated for several decades. In a longitudinal study using brain event-related potentials (ERPs) we investigated 1) whether dyslexic children with familial risk background would show atypical pitch processing from birth and 2) how these newborn ERPs later relate to these same children's pre-reading cognitive skills and literacy outcomes. Auditory ERPs were measured at birth for tones varying in pitch and presented in an oddball paradigm (1100 Hz, 12%, and 1000 Hz, 88%). The brain responses of the typically reading control group children (TRC group, N=25) showed clear differentiation between the frequencies, while those of the group of reading disability with familial risk (RDFR, 8 children) and the group of typical readers with familial risk (TRFR, 14 children) did not differentiate between the tones. The ERPs of the latter two groups differed from those of the TRC group. However, the two risk groups also showed a differential hemispheric ERP pattern. Furthermore, newborn ERPs reflecting passive change detection were associated with phonological skills and letter knowledge prior to school age and with phoneme duration perception, reading speed (RS) and spelling accuracy in the 2nd grade of school. The early obligatory response was associated with more general pre-school language skills, as well as with RS and reading accuracy (RA). Results suggest that a proportion of dyslexic readers with familial risk background are affected by atypical auditory processing. This is already present at birth and also relates to pre-reading phonological processing and speech perception. These early differences in auditory processing could later affect phonological representations and reading development. However, atypical auditory processing is unlikely to suffice as a sole explanation for dyslexia but rather as one risk factor, dependent on the genetic profile of the child. PMID:20656284

  5. Solitary Atypical Adenomatous Hyperplasia in a 12-Year-Old Girl

    PubMed Central

    Jin, Moran; Lee, Yang-Haeng; Kim, Bomi; Yoon, Young Chul; Wi, Jin Hong

    2016-01-01

    Atypical adenomatous hyperplasia is a premalignant lesion reflecting a focal proliferation of atypical cells. These lesions are usually observed as incidental findings in lungs that have been resected due to other conditions, such as lung cancer. We report the youngest case of atypical adenomatous hyperplasia on record in a 12-year-old girl. In this patient, the lesion was found in association with pneumothorax. PMID:27065090

  6. Atypical hemifacial spasm due to vertebral artery dolichoectasia: rare cause of a rarer clinical entity

    PubMed Central

    Mathur, Tarun; Srivastava, Trilochan; Sardana, Vijay; Jain, Rahul

    2013-01-01

    A 32-year-old man presented with atypical left hemifacial spasm (HFS) with MRI showing compression of left facial nerve at the root exit zone. HFS rarely presents atypically with onset in orbicularis oris and later spreading upward to involve the orbicularis oculi as was seen in our case. Atypical presentation of hemifacial spasm is rare and vertebral artery dolichoectasia as the underlying aetiology, like in our case is even rarer and has not been described in literature so far. PMID:23845680

  7. Atypical hemifacial spasm due to vertebral artery dolichoectasia: rare cause of a rarer clinical entity.

    PubMed

    Mathur, Tarun; Srivastava, Trilochan; Sardana, Vijay; Jain, Rahul

    2013-01-01

    A 32-year-old man presented with atypical left hemifacial spasm (HFS) with MRI showing compression of left facial nerve at the root exit zone. HFS rarely presents atypically with onset in orbicularis oris and later spreading upward to involve the orbicularis oculi as was seen in our case. Atypical presentation of hemifacial spasm is rare and vertebral artery dolichoectasia as the underlying aetiology, like in our case is even rarer and has not been described in literature so far. PMID:23845680

  8. Periprosthetic Atypical Femoral Fracture-like Fracture after Hip Arthroplasty: A Report of Three Cases.

    PubMed

    Lee, Kyung-Jae; Min, Byung-Woo; Jang, Hyung-Kyu; Ye, Hee-Uk; Lim, Kyung-Hwan

    2015-09-01

    Atypical femoral fractures are stress or insufficient fractures induced by low energy trauma or no trauma and have specific X-ray findings. Although the American Society for Bone and Mineral Research has excluded periprosthetic fractures from the definition of an atypical femoral fracture in 2013, this is still a matter of controversy because some authors report periprosthetic fractures showing specific features of atypical fractures around a well-fixed femoral stem. We report 3 cases of periprosthetic femur fractures that had specific radiographic features of atypical femoral fractures in patients with a history of prolonged bisphosphonate use; we also review relevant literature. PMID:27536624

  9. The validity of major depression with atypical features based on a community study.

    PubMed

    Horwath, E; Johnson, J; Weissman, M M; Hornig, C D

    1992-10-01

    This article reports on evidence for the validity of major depression (MDD) with atypical features (defined as overeating and oversleeping) as a distinct subtype based on cross-sectional and 1-year prospective data from the Epidemiologic Catchment Area study. MDD with atypical features, when compared to MDD without atypical features, was associated with a younger age of onset, more psychomotor slowing, and more comorbid panic disorder, drug abuse or dependence, and somatization disorder. These differences could not be explained by differences in demographic characteristics or by symptom severity. This study, based on a community sample, found that major depression with atypical features may constitute a distinct subtype. PMID:1447429

  10. Periprosthetic Atypical Femoral Fracture-like Fracture after Hip Arthroplasty: A Report of Three Cases

    PubMed Central

    Lee, Kyung-Jae; Jang, Hyung-Kyu; Ye, Hee-Uk; Lim, Kyung-Hwan

    2015-01-01

    Atypical femoral fractures are stress or insufficient fractures induced by low energy trauma or no trauma and have specific X-ray findings. Although the American Society for Bone and Mineral Research has excluded periprosthetic fractures from the definition of an atypical femoral fracture in 2013, this is still a matter of controversy because some authors report periprosthetic fractures showing specific features of atypical fractures around a well-fixed femoral stem. We report 3 cases of periprosthetic femur fractures that had specific radiographic features of atypical femoral fractures in patients with a history of prolonged bisphosphonate use; we also review relevant literature.

  11. 76 FR 63701 - Culturally Significant Objects Imported for Exhibition Determinations: “Johan Zoffany RA: Society...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-13

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF STATE Culturally Significant Objects Imported for Exhibition Determinations: ``Johan Zoffany RA: Society Observed... determine that the objects to be included in the exhibition ``Johan Zoffany RA: Society Observed,''...

  12. A Family of Congenital Hepatic Fibrosis and Atypical Retinitis Pigmentosa

    PubMed Central

    Zanwar, Vinay; Mohite, Ashok; Surude, Ravindra; Rathi, Pravin; Balasubramani, Meenakshi

    2015-01-01

    Congenital hepatic fibrosis is a rare cause of portal hypertension and esophageal varices in children. We report cases of siblings with biopsy proven congenital hepatic fibrosis and with atypical retinitis pigmentosa. They presented with repeated episodes of jaundice along with progressive decrease of vision in night. They had hepatosplenomegaly and portal hypertension with esophageal varices. One of the siblings had a large regenerating nodule replacing the entire right lobe of the liver and other one developed repeated hematemesis. This constellation of diagnosis belongs to the ciliopathy group of disorders. The spectrum of ciliopathy disorders has been evolving, and it varies from mild to severe manifestations. PMID:26918098

  13. A Family of Congenital Hepatic Fibrosis and Atypical Retinitis Pigmentosa.

    PubMed

    Pawar, Sunil; Zanwar, Vinay; Mohite, Ashok; Surude, Ravindra; Rathi, Pravin; Balasubramani, Meenakshi

    2015-11-01

    Congenital hepatic fibrosis is a rare cause of portal hypertension and esophageal varices in children. We report cases of siblings with biopsy proven congenital hepatic fibrosis and with atypical retinitis pigmentosa. They presented with repeated episodes of jaundice along with progressive decrease of vision in night. They had hepatosplenomegaly and portal hypertension with esophageal varices. One of the siblings had a large regenerating nodule replacing the entire right lobe of the liver and other one developed repeated hematemesis. This constellation of diagnosis belongs to the ciliopathy group of disorders. The spectrum of ciliopathy disorders has been evolving, and it varies from mild to severe manifestations. PMID:26918098

  14. Atypical facial pain as a defense against psychosis.

    PubMed

    Delaney, J F

    1976-10-01

    The author describes three women who presented psychotic symptoms 24--48 hours before scheduled neurosurgical procedures for atypical facial pain; all had had extensive dental reconstruction and attempted nerve blocks with no relief. Psychiatric hospitalization and administration of major tranquilizers resulted in control of symptoms and relief of pain. Two patients were followed for a year and have had return of psychiatric symptoms or facial pain; both have been maintained on medication and have returned to normal activities. The author suggests that the facial pain may have served as a defense against the emergence of psychosis. PMID:9836

  15. Severe dyspnea as atypical presenting symptom of Madelung's disease

    PubMed Central

    Milisavljevic, D; Zivic, M; Radovanović, Z; Stankovic, P

    2010-01-01

    Madelung's disease (benign symmetrical lipomatosis) is a rare disease of unknown etiology manifesting as symmetric abnormal deposits of adipose tissue in the head, neck and upper trunk. We report a case of a 58-year-old man with a long lasting Madelungs disease in whom progressive fatty tissue accumulation caused a severe inspiratory dyspnea as atypical presenting symptom. The etiopathogenetic, clinico-diagnostic and therapeutic aspects of this rare disease are discussed. Due to its progressive but not so easily predictable enlarging behavior Madelung's disease has not only aesthetic but also functional and sometimes life threatening consequences which need to be treated. PMID:20596272

  16. Dengue Fever Presenting Atypically with Viral Conjunctivitis and Subacute Thyroiditis.

    PubMed

    Sheraz, Faizan; Tahir, Hassan; Saqi, Jannavi; Daruwalla, Vistasp

    2016-06-01

    The majority of dengue viral infections are asymptomatic, though symptoms may range from self-limiting febrile illness to life threatening hemorrhagic manifestations. As the burden of disease is dramatically rising in recent years, more patients with atypical presentations and rare complications are increasingly reported. Dengue virus may rarely involve different organ systems including CNS, liver, and heart. However, involvement of eye and thyroid is extremely rare. We present a case of 32-year old patient who presented with conjunctivitis and subacute thyroiditis and was found to have dengue viral infection as the cause of these conditions. PMID:27376214

  17. Aspergilloma in atypical localisation in severe asthma patient - case report.

    PubMed

    Zieliński, Michał; Mazur-Zielińska, Henryka; Ziora, Dariusz

    2016-01-01

    Pulmonary aspergillosis is a condition caused by the fungi Aspergillus. The form of disease depends on the immunological condition of the host organism and other concomitant illnesses that influence the pulmonary tissue. Asthmatic patients, in particular with the severe form of disease, who require the use of systemic glucocorticoids, are predisposed to develop allergic bronchopulmonary aspergillosis. Development of aspergilloma in the lung is preceded by the formation of pathological cavity in the course of another illness. The study reports a case of a severe asthma patient who developed aspergilloma in atypical localisation, without the presence of predisposing anatomical changes and illnesses. PMID:26966025

  18. Radiation-induced malignant and atypical peripheral nerve sheath tumors

    SciTech Connect

    Foley, K.M.; Woodruff, J.M.; Ellis, F.T.; Posner, J.B.

    1980-04-01

    The reported peripheral nerve complications of therapeutic irradiation in humans include brachial and lumbar plexus fibrosis and cranial and peripheral nerve atrophy. We have encountered 9 patients with malignant (7) and atypical (2) peripheral nerve tumors occurring in an irradiated site suggesting that such tumors represent another delayed effect of radiation treatment on peripheral nerve. In all instances the radio-theray was within an acceptable radiation dosage, yet 3 patients developed local radiation-induced skin and bony abnormalities. The malignant peripheral nerve sheath tumors developed only in the radiation port. Animal studies support the clinical observation that malignant peripheral nerve sheath tumors can occur as a delayed effect of irradiation.

  19. Clinical Trials: past, current and future for atypical parkinsonian syndromes

    PubMed Central

    Tsai, Richard M.; Boxer, Adam L.

    2016-01-01

    There are currently no effective, Food and Drug Administration (FDA) approved treatments for atypical parkinsonian disorders such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), dementia with lewy bodies (DLB) or multiple system atrophy (MSA). Previous treatment trials for these disorders were focused on symptomatic support and did not affect disease progression. Recent breakthroughs in neuropathology and pathophysiology have allowed a new eunderstanding of these disorders and investigation into potentially disease modifying therapies. Randomized, placebo-controlled clinical trials of these disorders will be reviewed here. Suggestions for future therapeutic targets, clinical trial design, with a focus on PSP will also be provided. PMID:24963682

  20. [Atypic toxocariasis: a case report from the Peruvian north coast].

    PubMed

    Terrones-Campos, Cynthia; Andrade, Teresa; Lachira, Arnaldo; Valladolid, Omar; Lanata, Claudio F

    2010-03-01

    We present the case of a 4.5 years old boy with atypic toxocariasis, from La Matanza, Morropon, Piura. The patient had non-specific symptoms during 9 days. Suspicion of Toxocariasis was supported by marked eosinophilia in the cell blood count (15% or 1470 cells/μL). Diagnosis was confirmed by laboratory with ELISA serology demonstrating the presence of IgG and IgM anti-Toxocara antibodies. Symptoms receded before the patient received a five-day treatment with albendazol 15mg/kg/day. PMID:21072459

  1. Deep venous thrombosis and atypical antipsychotics: three cases report

    PubMed Central

    2012-01-01

    Background Deep venous Thrombosis is a serious, possible life threatening event which is often ignored in psychiatric Settings. Purpose In this paper three cases of deep venous Thrombosis (DVT) following the use of olanzapine and risperidone are presented. Methods The data of Three patients was collected from hospital records. Results The patients were in good general physical health and had no personal or familial history of DVT. The patients were not overweight (BMI < 25) but they suffered from DVT after initiating risperidone and olanzapine. Conclusion Risk of DVT exists in patients under treatment with atypical antipsychotics in spite of no pre existing risk factor. PMID:23351722

  2. Atypical Isolated Infections of the Infratemporal Fossa: A Diagnostic Challenge

    PubMed Central

    Tan, Sien Hui; Chong, Aun Wee; Prepageran, Narayanan

    2015-01-01

    Introduction: Atypical infratemporal fossa infections are rare and potentially fatal. Case Report: A case of an aspergillosis localized in the infratemporal fossa and another case of tuberculosis of the infratemporal fossa originating from the maxillary sinus, is described. The first patient was immunocompromised and showed symptoms of facial numbness; whereas the other was an immunocompetent man who complained of trigeminal neuralgia type pain. It was difficult to differentiate between infection and tumour despite the utilization of computed tomography scans and magnetic resonance imaging. Conclusion: These cases illustrate the need for a high index of suspicion; in addition to endoscopic confirmation and histopathology to establish precise diagnosis and early intervention. PMID:26568944

  3. Repeated activation of mania by atypical antipsychotics in a patient

    PubMed Central

    Raghunath, Ashwati

    2012-01-01

    A 50-year-old, white female patient was diagnosed with schizophrenia in her teens. Her illness did not respond adequately to treatment until she was placed on a combination of fluoxetine and conventional antipsychotics. She discontinued the conventional antipsychotics on a number of occasions, which caused her to become psychotic, but not manic. On two separate occasions she was placed on atypical antipsychotics that were associated with the occurrence of manic symptoms. Once the patient was restarted on conventional antipsychotics, she remained stable. PMID:23188864

  4. Atypical presentation of salivary mucocele: diagnosis and management.

    PubMed

    Nilesh, Kumar; Chandra, Jagadish

    2015-01-01

    A mucocele is a common pathological lesion involving the minor salivary glands. It usually presents as an asymptomatic small superficial swelling over the lower labial mucosa. However, uncommon variants of oral mucoceles sometimes occur. Such lesions may be difficult to diagnose due to their unusual size and atypical clinical presentation. This article describes the case of a deeply embedded large mucocele over the buccal mucosa. Ultrasonography was used to visualize the size and position of the lesion, and aspiration was used to help in the eventual diagnosis. An intraoral approach was used in the complete removal of the lesion. PMID:25574732

  5. Clinical guides for atypical hemolytic uremic syndrome in Japan.

    PubMed

    Kato, Hideki; Nangaku, Masaomi; Hataya, Hiroshi; Sawai, Toshihiro; Ashida, Akira; Fujimaru, Rika; Hidaka, Yoshihiko; Kaname, Shinya; Maruyama, Shoichi; Yasuda, Takashi; Yoshida, Yoko; Ito, Shuichi; Hattori, Motoshi; Miyakawa, Yoshitaka; Fujimura, Yoshihiro; Okada, Hirokazu; Kagami, Shoji

    2016-08-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. In 2013, we developed diagnostic criteria to enable early diagnosis and timely initiation of appropriate treatment for aHUS. Recent clinical and molecular findings have resulted in several proposed classifications and definitions of thrombotic microangiopathy and aHUS. Based on recent advances in this field and the emerging international consensus to exclude secondary TMAs from the definition of aHUS, we have redefined aHUS and proposed diagnostic algorithms, differential diagnosis, and therapeutic strategies for aHUS. PMID:27422619

  6. Clinical guides for atypical hemolytic uremic syndrome in Japan.

    PubMed

    Kato, Hideki; Nangaku, Masaomi; Hataya, Hiroshi; Sawai, Toshihiro; Ashida, Akira; Fujimaru, Rika; Hidaka, Yoshihiko; Kaname, Shinya; Maruyama, Shoichi; Yasuda, Takashi; Yoshida, Yoko; Ito, Shuichi; Hattori, Motoshi; Miyakawa, Yoshitaka; Fujimura, Yoshihiro; Okada, Hirokazu; Kagami, Shoji

    2016-07-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. In 2013, we developed diagnostic criteria to enable early diagnosis and timely initiation of appropriate treatment for aHUS. Recent clinical and molecular findings have resulted in several proposed classifications and definitions of thrombotic microangiopathy and aHUS. Based on recent advances in this field and the emerging international consensus to exclude secondary TMAs from the definition of aHUS, we have redefined aHUS and proposed diagnostic algorithms, differential diagnosis, and therapeutic strategies for aHUS. PMID:27460397

  7. Fast electric dipole transitions in Ra-Ac nuclei

    SciTech Connect

    Ahmad, I.

    1985-01-01

    Lifetime of levels in /sup 225/Ra, /sup 225/Ac, and /sup 227/Ac have been measured by delayed coincidence techniques and these have been used to determine the E1 gamma-ray transition probabilities. The reduced E1 transition probabilities. The reduced E1 transition probabilities in /sup 225/Ra and /sup 225/Ac are about two orders of magnitude larger than the values in mid-actinide nuclei. On the other hand, the E1 rate in /sup 227/Ac is similar to those measured in heavier actinides. Previous studies suggest the presence of octupole deformation in all the three nuclei. The present investigation indicates that fast E1 transitions occur for nuclei with octupole deformation. However, the studies also show that there is no one-to-one correspondence between E1 rate and octupole deformation. 13 refs., 4 figs.

  8. An improved limit on the EDM of 225 Ra

    NASA Astrophysics Data System (ADS)

    Bishof, Michael; Bailey, Kevin; Dietrich, Matthew R.; Greene, John P.; Holt, Roy J.; Kalita, Mukut R.; Korsch, Wolfgang; Lemke, Nathan D.; Lu, Zheng-Tian; Mueller, Peter; O'Connor, Thomas P.; Parker, Richard H.; Rabga, Tenzin; Singh, Jaideep T.

    2016-05-01

    Searches for permanent electric dipole moments (EDMs) are sensitive probes of symmetry violation that could explain the dominance of matter over anti-matter. The 225 Ra (t1/2 = 15 days, I = 1/2) atom is a particularly attractive system to use for an EDM measurement because its octupole-deformed nucleus, closely spaced ground-state parity doublet, and large nuclear charge make 225 Ra uniquely sensitive to symmetry-violating interactions in the nuclear medium. In 2015, we reported the first ``proof of principle'' measurement of the 225 Ra EDM, giving a 95% confidence upper limit of 5* 10-22 e-cm; representing the first EDM measurement using laser-trapped atoms as well as the first EDM measurement of an atom with an octupole-deformed nucleus. After implementing upgrades to our apparatus, we now observe nuclear spin coherence after 20 s of free evolution - a factor of ten improvement. A new EDM measurement based on the upgraded system improved the 95% confidence upper limit by a factor of 36. We also report on the progress of current experimental upgrades that have the potential to further improve our EDM sensitivity by many orders of magnitude, allowing us to test symmetry violation at an unprecedented level. This work is supported by U.S. DOE, Office of Science, Office of Nuclear Physics, under Contract DE-AC02-06CH11357.

  9. Search for a Permanent Electric Dipole Moment of 225Ra

    NASA Astrophysics Data System (ADS)

    Kalita, Mukut Ranjan

    The observation of a permanent electric dipole moment (EDM) in a non-degenerate system would indicate the violation of discrete symmetries of Time reversal (T) or combined application of Charge (C) and Parity (P) symmetry violation through the CPT theorem. The diamagnetic 225Ra atom with nuclear spin I=1/2 is a favorable candidate for an EDM search. Experimental sensitivity to its EDM is enhanced due to its high atomic mass and the increased Schiff moment of its octupole deformed nucleus. An experimental setup is developed where laser cooled neutral radium atoms are collected in a magneto-optical trap (MOT). The collected atoms are transported 1 meter with a far off-resonant optical dipole trap (ODT) and then the atoms are transferred to a second standing-wave ODT in an experimental chamber. The atoms are then optically polarized and allowed to Larmor precess in parallel and antiparallel electric and magnetic fields. The difference between the Larmor precession frequency for parallel and antiparallel fields is experimentally determined to measure the EDM. This thesis is about the first measurement of the EDM of the 225Ra atom where an upper limit of |d(225Ra)| < 5.0 x 10-22 e·cm (95% confidence) is reached. Keywords: Permanent EDM, CP violation, laser cooling and trapping, rare isotopes, radium.

  10. EGF or PDGF receptors activate atypical PKClambda through phosphatidylinositol 3-kinase.

    PubMed Central

    Akimoto, K; Takahashi, R; Moriya, S; Nishioka, N; Takayanagi, J; Kimura, K; Fukui, Y; Osada, S i; Mizuno, K; Hirai, S i; Kazlauskas, A; Ohno, S

    1996-01-01

    Overexpression of a TPA-insensitive PKC member, an atypical protein kinase C (aPKClambda), results in an enhancement of the transcriptional activation of TPA response element (TRE) in cells stimulated with epidermal growth factor (EGF) or platelet-derived growth factor (PDGF). EGF or PDGF also caused a transient increase in the in vivo phosphorylation level and a change in the intracellular localization of aPKClambda from the nucleus to the cytosol, indicating the activation of aPKClambda in response to this growth factor stimulation. These immediate signal-dependent changes in aKPClambda were observed for a PDGF receptor add-back mutant (Y40/51) that possesses only two of the five major autophosphorylation sites and binds PI3-kinase, and were inhibited by wortmannin, an inhibitor of PI3-kinase. Furthermore, an N-terminal fragment of the catalytic subunit of PI3-kinase, p110alpha, inhibited aPKClambda-dependent activation of TRE in Y40/51 cells stimulated with PDGF. Overexpression of p110alpha resulted in an enhancement of TRE expression in response to PDGF and the regulatory domain of aPKClambda inhibited this TRE activation in Y40/51 cells. These results provide the first in vivo evidence supporting the presence of a novel signalling pathway from receptor tyrosine kinases to aPKClambda through PI3-kinase. Images PMID:8631300

  11. Hedgehog-regulated atypical PKC promotes phosphorylation and activation of Smoothened and Cubitus interruptus in Drosophila.

    PubMed

    Jiang, Kai; Liu, Yajuan; Fan, Junkai; Epperly, Garretson; Gao, Tianyan; Jiang, Jin; Jia, Jianhang

    2014-11-11

    Smoothened (Smo) is essential for transduction of the Hedgehog (Hh) signal in both insects and vertebrates. Cell surface/cilium accumulation of Smo is thought to play an important role in Hh signaling, but how the localization of Smo is controlled remains poorly understood. In this study, we demonstrate that atypical PKC (aPKC) regulates Smo phosphorylation and basolateral accumulation in Drosophila wings. Inactivation of aPKC by either RNAi or a mutation inhibits Smo basolateral accumulation and attenuates Hh target gene expression. In contrast, expression of constitutively active aPKC elevates basolateral accumulation of Smo and promotes Hh signaling. The aPKC-mediated phosphorylation of Smo at Ser680 promotes Ser683 phosphorylation by casein kinase 1 (CK1), and these phosphorylation events elevate Smo activity in vivo. Moreover, aPKC has an additional positive role in Hh signaling by regulating the activity of Cubitus interruptus (Ci) through phosphorylation of the Zn finger DNA-binding domain. Finally, the expression of aPKC is up-regulated by Hh signaling in a Ci-dependent manner. Our findings indicate a direct involvement of aPKC in Hh signaling beyond its role in cell polarity. PMID:25349414

  12. Hedgehog-regulated atypical PKC promotes phosphorylation and activation of Smoothened and Cubitus interruptus in Drosophila

    PubMed Central

    Jiang, Kai; Liu, Yajuan; Fan, Junkai; Epperly, Garretson; Gao, Tianyan; Jiang, Jin; Jia, Jianhang

    2014-01-01

    Smoothened (Smo) is essential for transduction of the Hedgehog (Hh) signal in both insects and vertebrates. Cell surface/cilium accumulation of Smo is thought to play an important role in Hh signaling, but how the localization of Smo is controlled remains poorly understood. In this study, we demonstrate that atypical PKC (aPKC) regulates Smo phosphorylation and basolateral accumulation in Drosophila wings. Inactivation of aPKC by either RNAi or a mutation inhibits Smo basolateral accumulation and attenuates Hh target gene expression. In contrast, expression of constitutively active aPKC elevates basolateral accumulation of Smo and promotes Hh signaling. The aPKC-mediated phosphorylation of Smo at Ser680 promotes Ser683 phosphorylation by casein kinase 1 (CK1), and these phosphorylation events elevate Smo activity in vivo. Moreover, aPKC has an additional positive role in Hh signaling by regulating the activity of Cubitus interruptus (Ci) through phosphorylation of the Zn finger DNA-binding domain. Finally, the expression of aPKC is up-regulated by Hh signaling in a Ci-dependent manner. Our findings indicate a direct involvement of aPKC in Hh signaling beyond its role in cell polarity. PMID:25349414

  13. Asenapine in the treatment of borderline personality disorder: an atypical antipsychotic alternative.

    PubMed

    Martín-Blanco, Ana; Patrizi, Barbara; Villalta, Laia; Gasol, Xero; Soler, Joaquim; Gasol, Miquel; Pascual, Juan C

    2014-03-01

    Many individuals with borderline personality disorder (BPD) receive medical treatment in clinical practice, although to date, there are no drugs specifically available for BPD. The recent Cochrane guideline suggests a benefit from using second-generation antipsychotics such as olanzapine or aripiprazole; nevertheless, side effects limit their use. Asenapine is a novel FDA-approved atypical antipsychotic for schizophrenia and bipolar disorder. However, it has not yet been tested for BPD. The goal of this observational open-label study was to assess the safety, tolerability and efficacy of asenapine in a series of cases of patients with BPD. Twelve individuals with BPD were recruited and treated with asenapine during an 8-week period. Eight individuals completed the study; a significant improvement was observed in the CGI-BPD (P<0.001) and BSL-23 (P<0.048) scales for BPD symptomatology. Besides, there was a significant improvement in the general psychopathology domains (BPRS, P<0.004), whereas no significant differences were observed in depressive symptoms. No serious adverse effects were reported and a significant weight reduction was observed (P=0.002). Asenapine appears to be a safe and effective agent in the treatment of patients with BPD, especially when other alternatives are not tolerated. These preliminary findings should be replicated in a controlled clinical trial. PMID:23962963

  14. The Effect of Stopping Smoking on Disease Activity in Rheumatoid Arthritis (RA). Data from BARFOT, a Multicenter Study of Early RA

    PubMed Central

    Andersson, Maria LE; Bergman, Stefan; Söderlin, Maria K

    2012-01-01

    Objective: We studied the effect of stopping smoking on disease activity in patients with RA. Methods: Between 1992 and 2005, 2,800 adult patients were included in the BARFOT early RA study in Sweden. Disease Activity Score 28 joints (DAS28), C-reactive protein (CRP), Health Assessment Questionnaire (HAQ), rheumatoid factor (RF), anti-CCP, general health and pain visual analog scales (VAS), EULAR response and treatment were registered at inclusion and at follow-up 2, 5 and 8 years. In 2010, a self-completion postal questionnaire was sent to 2,102 patients, enquiring about lifestyle factors, including cessation of smoking. Results: A total of 1,460 adult RA patients with disease duration ≤2 years were included in this study. Seventeen percent smoked in 2010. In total, 127 patients stopped smoking after inclusion in the study. Smoking cessation after inclusion in the study was negatively associated with EULAR good outcome at 8 years (OR 0.44, 95% CI 0.22–0.86, p=0.02), controlled for age, disease duration, sex, socioeconomic class, smoking status, RF, and DAS28 at inclusion. Conclusion: Seventeen percent of the RA patients smoked in 2010 in this large Swedish RA cohort. Stopping smoking after onset of RA did not change the poor prognosis of smokers with RA, but all RA patients need to stop smoking because of the high risk of cardiovascular mortality and morbidity and the association of smoking with vasculitis and noduli in RA. PMID:23115602

  15. The influence of atypical antipsychotic drugs on sexual function

    PubMed Central

    Just, Marek J

    2015-01-01

    Human sexuality is contingent upon many biological and psychological factors. Such factors include sexual drive (libido), physiological arousal (lubrication/erection), orgasm, and ejaculation, as well as maintaining normal menstrual cycle. The assessment of sexual dysfunction can be difficult due to the intimate nature of the problem and patients’ unwillingness to discuss it. Also, the problem of dysfunction is often overlooked by doctors. Atypical antipsychotic treatment is a key component of mental disorders’ treatment algorithms recommended by the National Institute of Health and Clinical Excellence, the American Psychiatric Association, and the British Society for Psychopharmacology. The relationship between atypical antipsychotic drugs and sexual dysfunction is mediated in part by antipsychotic blockade of pituitary dopamine D2 receptors increasing prolactin secretion, although direct correlations have not been established between raised prolactin levels and clinical symptoms. Variety of mechanisms are likely to contribute to antipsychotic-related sexual dysfunction, including hyperprolactinemia, sedation, and antagonism of a number of neurotransmitter receptors (α-adrenergic, dopaminergic, histaminic, and muscarinic). Maintaining normal sexual function in people treated for mental disorders can affect their quality of life, mood, self-esteem, attitude toward taking medication, and compliance during therapy. PMID:26185449

  16. Time course of lung function changes in atypical pneumonia.

    PubMed Central

    Benusiglio, L N; Stalder, H; Junod, A F

    1980-01-01

    We measured pulmonary function in each of 21 patients suffering from "atypical", non-bacterial pneumonia during the acute illness and during convalescence (two to 18 months) to study the course and the nature of functional impairment at different stages of the disease. In six patients, no aetiological agent was found. An aetiological agent was identified in 15 of the patients: Mycoplasma pneumoniae (seven patients), influenza A (three patients), parainfluenza 3 (one patient), varicella (two patients), Q fever (one patient), coxsackie B3 (one patient). At the time of admission we observed a restrictive pattern in 52%, an obstructive pattern (decreased FEV1/FVC ratio) in 52% abnormalities in distribution of ventilation (abnormal slope of phase 3) in 63%, and abnormalities in gas exchange (increased AaDO2) in 75% of the patients. The frequency of abnormalities in these pulmonary function tests decreased dramatically after two to four weeks and nearly disappeared in most patients during convalescence. The only major residual abnormality was a decreased FEV1/FVC ratio in five subjects, four of whom were smokers. However, when MMEF and V75 were measured at this stage, their average value for all the groups of patients with the exclusion of the Mycoplasma pneumoniae group, was markedly reduced. These data suggest that small airways involvement can be demonstrated during the convalescence of patients recovering from various types of atypical pneumonia other than those caused by Mycoplasma pneumoniae. PMID:7444825

  17. Two Subtypes of Atypical Leiomyoma: Clinical, Histologic, and Molecular Analysis.

    PubMed

    Ubago, Julianne M; Zhang, Qing; Kim, Julie J; Kong, Beihua; Wei, Jian-Jun

    2016-07-01

    Atypical leiomyoma (ALM) is a rare variant of uterine smooth muscle tumors. Several recent studies have suggested that ALM has distinct, but also heterogenous, histologic and molecular features, yet little is known about the biology and histogenesis of ALM. Some have even postulated whether the atypical histologic features represent true atypia or simply degenerative changes. In this study, we analyzed the cytologic features of 60 ALM cases and found that ALM could be further divided into 2 subtypes, type I and type II, based primarily on nuclear features. Type I ALM showed round or oval nuclei, distinct and smooth nuclear membranes, prominent nucleoli with perinucleolar halos, and open coarse chromatin. Type II ALM showed elongated or spindled nuclei, irregular nuclear membranes, pinpoint or no nucleoli, and dark smudgy chromatin. There were also architectural differences between type I and type II ALM. Type I ALM often showed diffuse atypia within the tumor, whereas the atypia in type II ALM was patchy, surrounded by usual-type leiomyoma. The 2 subtypes also differed when we compared the immunohistochemical and molecular patterns. Type II tumors showed significantly higher rates of immunoreactivity for p16, p53, and HMGA2 and showed MED12 mutations more frequently than the type I counterparts. Our findings suggest that the type I and type II subtypes of ALM may arise from 2 different pathways. Type I tumors may be related to fumarate hydratase mutations, whereas type II ALM appear to arise in a existing usual-type leiomyomas. PMID:27015034

  18. Orthostatic Hypotension in Patients with Parkinson's Disease and Atypical Parkinsonism

    PubMed Central

    Lökk, Johan

    2014-01-01

    Orthostatic hypotension (OH) is one of the commonly occurring nonmotor symptoms in patients with idiopathic Parkinson's disease (IPD) and atypical parkinsonism (AP). We aimed to review current evidences on epidemiology, diagnosis, treatment, and prognosis of OH in patients with IPD and AP. Major electronic medical databases were assessed including PubMed/MEDLINE and Embase up to February 2013. English-written original or review articles with keywords such as “Parkinson's disease,” “atypical parkinsonism,” and “orthostatic hypotension” were searched for relevant evidences. We addressed different issues such as OH definition, epidemiologic characteristics, pathophysiology, testing and diagnosis, risk factors for symptomatic OH, OH as an early sign of IPD, prognosis, and treatment options of OH in parkinsonian syndromes. Symptomatic OH is present in up to 30% of IPD, 80% of multiple system atrophy (MSA), and 27% of other AP patients. OH may herald the onset of PD before cardinal motor symptoms and our review emphasises the importance of its timely diagnosis (even as one preclinical marker) and multifactorial treatment, starting with patient education and lifestyle approach. Advancing age, male sex, disease severity, and duration and subtype of motor symptoms are predisposing factors. OH increases the risk of falls, which affects the quality of life in PD patients. PMID:24634790

  19. Hepatic Safety of Atypical Antipsychotics: Current Evidence and Future Directions.

    PubMed

    Slim, Mahmoud; Medina-Caliz, Inmaculada; Gonzalez-Jimenez, Andres; Cabello, M Rosario; Mayoral-Cleries, Fermin; Lucena, M Isabel; Andrade, Raul J

    2016-10-01

    The newer atypical antipsychotic agents (AAPs) represent an attractive therapeutic option for a wide range of psychotic disorders, including schizophrenia and bipolar mania, because of the reduced risk of disabling extrapyramidal symptoms. However, their growing use has raised questions about their tolerability over the endocrine, metabolic, and cardiovascular axes. Indeed, atypical antipsychotic drugs are associated, to differing extents, with mild elevation of aminotransferases related to weight gain, AAP-induced metabolic syndrome, and nonalcoholic fatty liver disease. Although the hepatic safety of new AAPs seems improved over that of chlorpromazine, they can occasionally cause idiosyncratic liver injury with varying phenotypes and, rarely, lead to acute liver failure. However, AAPs are a group of heterogeneous, chemically unrelated compounds with distinct pharmacological and pharmacokinetic properties and substantially different safety profiles, which precludes the notion of a class effect for hepatotoxicity risk and highlights the need for an individualized therapeutic approach. We discuss the current evidence on the hepatotoxicity potential of AAPs, the emerging underlying mechanisms, and the limitations inherent to this group of drugs for both establishing a proper causality assessment and developing strategies for risk management. PMID:27449495

  20. Mass loss in main-sequence A-type stars?

    NASA Astrophysics Data System (ADS)

    Lanz, T.; Catala, C.

    1992-04-01

    We present new observations of the H-alpha line profile for five main-sequence A-type stars, where very high SNRs were achieved. A search for weak asymmetries was carried out to detect a stellar wind, but the many telluric absorption lines in this range prevent us from taking the full benefit of the high SNRs. This situation was improved by modeling the telluric absorptions to remove them from the observed spectra, but the H-alpha profiles were nevertheless found to be quite symmetric. We calculated the profile of the H-alpha line for a grid of model atmospheres of an A-type star including a weak wind, in order to assess an upper limit on the mass loss rate. The asymmetry is found to be sensitive to the velocity law and to the turbulent velocity of the wind, but its first moment depends much less on them. The upper limits deduced on the mass loss rate are between 1 and 2 x 10 exp -10 solar mass/yr. An improvement up to a factor 10 could be anticipated if new observations could be secured from a much drier location, achieving similar SNRs.

  1. Atypical centromeres in plants—what they can tell us

    PubMed Central

    Cuacos, Maria; H. Franklin, F. Chris; Heckmann, Stefan

    2015-01-01

    The centromere, visible as the primary constriction of condensed metaphase chromosomes, is a defined chromosomal locus essential for genome stability. It mediates transient assembly of a multi-protein complex, the kinetochore, which enables interaction with spindle fibers and thus faithful segregation of the genetic information during nuclear divisions. Centromeric DNA varies in extent and sequence composition among organisms, but a common feature of almost all active eukaryotic centromeres is the presence of the centromeric histone H3 variant cenH3 (a.k.a. CENP-A). These typical centromere features apply to most studied species. However, a number of species display “atypical” centromeres, such as holocentromeres (centromere extension along almost the entire chromatid length) or neocentromeres (ectopic centromere activity). In this review, we provide an overview of different atypical centromere types found in plants including holocentromeres, de novo formed centromeres and terminal neocentromeres as well as di-, tri- and metapolycentromeres (more than one centromere per chromosomes). We discuss their specific and common features and compare them to centromere types found in other eukaryotic species. We also highlight new insights into centromere biology gained in plants with atypical centromeres such as distinct mechanisms to define a holocentromere, specific adaptations in species with holocentromeres during meiosis or various scenarios leading to neocentromere formation. PMID:26579160

  2. Atypical gunshot wound: Bullet trajectory analyzed by computed tomography

    PubMed Central

    Ro, Tae; Murray, Richard; Galvan, Dan; Nazim, Muhammad H.

    2015-01-01

    Introduction Gunshot injuries are a result of a bullet or projectile fired from a weapon that penetrates the body. Homicide, suicide, and occasionally, accidental events are a significant cause of firearm-related injuries. In rare cases, the damage from the gunshot injury can be masked due to an atypical bullet trajectory. Presentation of case A 63-year-old male was found with a gunshot wound to the anterior left knee. Computed tomography (CT) scans revealed a bullet track extending from the anterior aspect of the left knee that traveled cephalad subcutaneously and entered into the peritoneal cavity, perforating the distal descending colon. The bullet was found to be at rest adjacent to the spleen and posterior chest wall, with no injury to the lungs, kidneys or the spleen. The patient required a sigmoid colectomy with descending colostomy and was subsequently discharged home without any complications. Discussion Intra-abdominal organ damage from a gunshot wound to the distal limb is a rare occurrence. Atypical gun shot wounds, such as this case, have the potential for multiple issues including: delayed diagnostic tests, inaccurate radiological readings, and inappropriate medical management. Conclusion If an abnormal trajectory is maintained, it is possible for a bullet to traverse half the length of the body without the patient realizing it. Accurate CT analysis and quick decisions in surgical and medical management are critical takeaways to provide quality care to patients with these injuries. PMID:26263447

  3. [Ketosis-prone atypical diabetes mellitus: report of two cases].

    PubMed

    Belhadi, L; Chadli, A; Bennis, L; Ghomari, H; Farouqi, A

    2007-12-01

    An atypical presentation of diabetes mellitus was described in black subjects, initially in adolescents by Winter et al. then, in adult populations. The principal characteristics of "African" diabetes are an acute onset with severe hyperglycemia and ketosis, and a clinical course of type 2 diabetes mellitus. In the subsequent clinical course after initiation of insulin therapy, prolonged remission is often possible with cessation of insulin therapy and maintenance of appropriate metabolic control. In the subsequent clinical course after initiation of insulin therapy, prolonged remission is often possible with cessation of insulin therapy and maintenance of appropriate metabolic control. The molecular mechanisms underlining the insulin secretory dysfunction are still to be understood and may involve glucolipotoxicity processes. The HLA alleles associated with susceptibility to type 1 diabetes were reported of high frequency in some populations with this form of diabetes, in the absence of makers of pancreatic beta cell autoimmunity. The aim of the present review is to discuss two cases of African diabetes and review the specific diagnostic, metabolic, pathogenic and management features of this atypical diabetes. PMID:17692810

  4. Atypical presentation of ectopia lentis in Marfan's syndrome.

    PubMed

    Chaudhry, Mridu; Grover, Samit; Baisakhiya, Shikha; Sharma, Neha; Bajaj, Aakarsh

    2014-02-01

    The purpose of this article is to report an unusual bilateral inferior subluxation of the lens in a patient with Marfan's syndrome. A 14-year-old boy presented with gradual painless diminution of vision in both eyes. His family history showed that his maternal uncle also had similar complaints. Systemic examination of the patient revealed no neurological deficits. Cardiovascular system examination was unremarkable. Hands and fingers were long and slender with hyperflexible joints. The ratio of arm spam to height was 1.06. He was myopic with a best-corrected visual acuity of 6/24 with -11 D spherical/- 2 D cylindrical in both eyes. Anterior chambers were deep with the presence of mild iridodonesis in both eyes. Pupillary reactions were sluggish. On pupillary dilatation, the lens was found to be subluxated inferiorly which is unlike the typical superotemporal subluxation of the lens in Marfan's syndrome. The diagnosis of Marfan's syndrome is usually made on clinical examination only, as there is no specific investigation for this condition; however, it may have atypical presentations. Therefore, it is important to recognize and report such atypical cases. PMID:23479050

  5. Atypical streptococcal infection of gingiva associated with chronic mouth breathing.

    PubMed

    Haytac, M Cenk; Oz, I Attila

    2007-01-01

    Streptococcal infections of oral tissues are mainly seen in young children who experience a variety of upper respiratory tract infections. The disease is characterized by fever, lymphadenopathy, and ulcers on the gingiva, lips, and tonsils. This case report presents an atypical streptococcal infection of the gingiva in an 18-year-old man. The patient was referred to the periodontology department complaining of a 2-month history of gingival enlargement. He had persistent fever (39.5 degrees C) and general malaise for 2 weeks. Intraoral examination revealed extremely inflamed and enlarged gingiva with spontaneous bleeding and suppuration. Based on the otolaryngologic consultation and the hematologic, immunologic, and microbiologic tests, the final diagnosis was an atypical streptococcal gingivitis with chronic adenoid-related mouth breathing and oral hygiene neglect as contributing factors. Treatment consisted of a broad-spectrum antibiotic regimen, supragingival and subgingival debridement, adenoidectomy, and scaling and root planing. A good response to nonsurgical therapy was achieved despite poor patient compliance, and no recurrence of gingival enlargement was observed after 1 year. Streptococcal gingivitis should be included in the differential diagnosis of suppurative gingival enlargements. Furthermore, chronic mouth breathing may initiate and/or contribute to this disease. PMID:18197316

  6. Current status of atypical antipsychotics for the treatment of fibromyalgia.

    PubMed

    Rico-Villademoros, F; Calandre, E P; Slim, M

    2014-06-01

    The treatment of fibromyalgia requires pharmacological and nonpharmacological therapies. The pharmacological treatment of fibromyalgia is limited to a few drugs that have been demonstrated to be moderately effective in some but not all dimensions of the disease. Therefore, the search for new drugs to treat this condition is warranted. Atypical antipsychotics offered an attractive alternative because they had been shown to be active against several key symptoms of fibromyalgia. The results of open-label studies, however, appear to indicate that atypical antipsychotics are poorly tolerated in patients with fibromyalgia, and only quetiapine XR has been studied in randomized controlled trials. Quetiapine XR has demonstrated effectiveness in treating comorbid major depression, anxiety and sleep disturbance. However, in two randomized controlled trials, quetiapine XR was not differentiated from placebo and failed to demonstrate noninferiority to amitriptyline in terms of improving overall symptomatology. The effect of quetiapine XR on pain and its usefulness as part of a combination pharmacological regimen should be further evaluated. Overall, the use of quetiapine (initiated at a low dose and slowly titrated) in fibromyalgia should be limited to patients with comorbid major depression or patients who are currently receiving other treatments and have unresolved and disabling depressive and/or anxiety symptoms. PMID:24983591

  7. Questioning the association between bisphosphonates and atypical femoral fractures

    PubMed Central

    Pazianas, Michael; Kim, Se-min; Yuen, Tony; Sun, Li; Epstein, Sol; Zaidi, Mone

    2014-01-01

    Bisphosphonates are the first line treatment for osteoporosis. Structurally, they are stable analogues of pyrophosphate and therefore exhibit a high affinity for bone mineral. They reduce bone loss by attenuating the ability of the osteoclast to resorb bone, decreasing activation frequency and the rate of remodelling. Large prospective randomized placebo-control trials provide unequivocal evidence for a reduction in the incidence of fractures.1 Impressively, 40 years since their first use in patients, the safety profile of bisphosphonates has been equally re-assuring.2 Questions have arisen lately as to whether bisphosphonates could cause atypical fractures, a rare type of atraumatic or minimal trauma femur fracture occurring below the great trochanter. This question has prompted calls for a broader examination of the long-term effects of bisphosphonate use. An attempt by the Food and Drug Administration (FDA) to garner consensus and provide definitive views was not successful.3 This has led to continued anxiety among treating physicians and patients alike, resulting in an overall reduction in prescriptions for bisphosphonates and for osteoporosis therapies in general. Here, we provide an overview of the current data on atypical fractures and bisphosphonate use. PMID:25294742

  8. Dual mechanism of action of the atypical tetracycline chelocardin.

    PubMed

    Stepanek, Jennifer J; Lukežič, Tadeja; Teichert, Ines; Petković, Hrvoje; Bandow, Julia E

    2016-06-01

    Classical tetracyclines targeting the protein biosynthesis machinery are commonly applied in human and veterinary medicine. The development and spread of resistance seriously compromise the successful treatment of bacterial infections. The atypical tetracycline chelocardin holds promise as it retains activity against tetracycline-resistant strains. It has been suggested that chelocardin targets the bacterial membrane, thus differing in mode of action from that of classical tetracyclines. We investigated the mechanism of action of chelocardin using global proteome analysis. The proteome profiles after sublethal chelocardin stress were compared to a reference compendium containing antibiotic response profiles of Bacillus subtilis. This approach revealed a concentration-dependent dual mechanism of action. At low concentrations, like classical tetracyclines, chelocardin induces the proteomic signature for peptidyl transferase inhibition demonstrating that protein biosynthesis inhibition is the dominant physiological challenge. At higher concentrations B. subtilis mainly responds to membrane stress indicating that at clinically relevant concentrations the membrane is the main antibiotic target of chelocardin. Studying the effects on the membrane in more detail, we found that chelocardin causes membrane depolarization but does not lead to formation of large pores. We conclude that at growth inhibiting doses chelocardin not only targets protein biosynthesis but also corrupts the integrity of the bacterial membrane. This dual mechanism of action might prove beneficial in slowing the development of new resistance mechanisms against this atypical tetracycline. PMID:26969785

  9. Off-label indications for atypical antipsychotics: A systematic review

    PubMed Central

    Fountoulakis, Konstantinos N; Nimatoudis, Ioannis; Iacovides, Apostolos; Kaprinis, George

    2004-01-01

    Introduction With the introduction of newer atypical antipsychotic agents, a question emerged, concerning their use as complementary pharmacotherapy or even as monotherapy in mental disorders other than psychosis. Material and method MEDLINE was searched with the combination of each one of the key words: risperidone, olanzapine and quetiapine with key words that refered to every DSM-IV diagnosis other than schizophrenia and other psychotic disorders, bipolar disorder and dementia and memory disorders. All papers were scored on the basis of the JADAD index. Results The search returned 483 papers. The selection process restricted the sample to 59 papers concerning Risperidone, 37 concerning Olanzapine and 4 concerning Quetiapine (100 in total). Ten papers (7 concerning Risperidone and 3 concerning Olanzapine) had JADAD index above 2. Data suggest that further research would be of value concerning the use of risperidone in the treatment of refractory OCD, Pervasive Developmental disorder, stuttering and Tourette's syndrome, and the use of olanzapine for the treatment of refractory depression and borderline personality disorder. Discussion Data on the off-label usefulness of newer atypical antipsychotics are limited, but positive cues suggest that further research may provide with sufficient hard data to warrant the use of these agents in a broad spectrum of psychiatric disorders, either as monotherapy, or as an augmentation strategy. PMID:14975068

  10. Atypical pathogens and challenges in community-acquired pneumonia.

    PubMed

    Thibodeau, Kristopher P; Viera, Anthony J

    2004-04-01

    Atypical organisms such as Mycoplasma pneumoniae, Chlamydia pneumoniae, and Legionella pneumophila are implicated in up to 40 percent of cases of community-acquired pneumonia. Antibiotic treatment is empiric and includes coverage for both typical and atypical organisms. Doxycycline, a fluoroquinolone with enhanced activity against Streptococcus pneumoniae, or a macrolide is appropriate for outpatient treatment of immunocompetent adult patients. Hospitalized adults should be treated with cefotaxime or ceftriaxone plus a macrolide, or with a fluoroquinolone alone. The same agents can be used in adult patients in intensive care units, although fluoroquinolone monotherapy is not recommended; ampicillin-sulbactam or piperacillin-tazobactam can be used instead of cefotaxime or ceftriaxone. Outpatient treatment of children two months to five years of age consists of high-dose amoxicillin given for seven to 10 days. A single dose of ceftriaxone can be used in infants when the first dose of antibiotic is likely to be delayed or not absorbed. Older children can be treated with a macrolide. Hospitalized children should be treated with a macrolide plus a beta-lactam inhibitor. In a bioterrorist attack, pulmonary illness may result from the organisms that cause anthrax, plague, or tularemia. Sudden acute respiratory syndrome begins with a flu-like illness, followed two to seven days later by cough, dyspnea and, in some instances, acute respiratory distress. PMID:15086042

  11. Atypical neural specialization for social percepts in autism spectrum disorder

    PubMed Central

    McPartland, James C.; Wu, Jia; Bailey, Christopher A.; Mayes, Linda C.; Schultz, Robert T.; Klin, Ami

    2011-01-01

    The social motivation hypothesis posits that aberrant neural response to human faces in autism is attributable to atypical social development and consequently reduced exposure to faces. The specificity of deficits in neural specialization remains unclear, and alternative theories suggest generalized processing difficulties. The current study contrasted neural specialization for social information versus non-social information in 36 individuals with autism and 18 typically developing individuals matched for age, race, sex, handedness, and cognitive ability. Event-related potentials elicited by faces, inverted faces, houses, letters, and pseudoletters were recorded. Groups were compared on an electrophysiological marker of neural specialization (N170), as well as behavioral performance on standardized measures of face recognition and word reading/decoding. Consistent with prior results, individuals with autism displayed slowed face processing and decreased sensitivity to face inversion; however, they showed comparable brain responses to letters, which were associated with behavioral performance in both groups. Results suggest that individuals with autism display atypical neural specialization for social information but intact specialization for non-social information. They concord with the notion of specific dysfunction in social brain systems rather than non-specific information processing difficulties in autism. PMID:21777159

  12. Atypical Face Perception in Autism: A Point of View?

    PubMed

    Morin, Karine; Guy, Jacalyn; Habak, Claudine; Wilson, Hugh R; Pagani, Linda; Mottron, Laurent; Bertone, Armando

    2015-10-01

    Face perception is the most commonly used visual metric of social perception in autism. However, when found to be atypical, the origin of face perception differences in autism is contentious. One hypothesis proposes that a locally oriented visual analysis, characteristic of individuals with autism, ultimately affects performance on face tasks where a global analysis is optimal. The objective of this study was to evaluate this hypothesis by assessing face identity discrimination with synthetic faces presented with and without changes in viewpoint, with the former condition minimizing access to local face attributes used for identity discrimination. Twenty-eight individuals with autism and 30 neurotypical participants performed a face identity discrimination task. Stimuli were synthetic faces extracted from traditional face photographs in both front and 20° side viewpoints, digitized from 37 points to provide a continuous measure of facial geometry. Face identity discrimination thresholds were obtained using a two-alternative, temporal forced choice match-to-sample paradigm. Analyses revealed an interaction between group and condition, with group differences found only for the viewpoint change condition, where performance in the autism group was decreased compared to that of neurotypical participants. The selective decrease in performance for the viewpoint change condition suggests that face identity discrimination in autism is more difficult when access to local cues is minimized, and/or when dependence on integrative analysis is increased. These results lend support to a perceptual contribution of atypical face perception in autism. PMID:25683613

  13. Gene-rich chromosomal regions are preferentially localized in the lamin B deficient nuclear blebs of atypical progeria cells

    PubMed Central

    Bercht Pfleghaar, Katrin; Taimen, Pekka; Butin-Israeli, Veronika; Shimi, Takeshi; Langer-Freitag, Sabine; Markaki, Yolanda; Goldman, Anne E; Wehnert, Manfred; Goldman, Robert D

    2015-01-01

    More than 20 mutations in the gene encoding A-type lamins (LMNA) cause progeria, a rare premature aging disorder. The major pathognomonic hallmarks of progeria cells are seen as nuclear deformations or blebs that are related to the redistribution of A- and B-type lamins within the nuclear lamina. However, the functional significance of these progeria-associated blebs remains unknown. We have carried out an analysis of the structural and functional consequences of progeria-associated nuclear blebs in dermal fibroblasts from a progeria patient carrying a rare point mutation p.S143F (C428T) in lamin A/C. These blebs form microdomains that are devoid of major structural components of the nuclear envelope (NE)/lamina including B-type lamins and nuclear pore complexes (NPCs) and are enriched in A-type lamins. Using laser capture microdissection and comparative genomic hybridization (CGH) analyses, we show that, while these domains are devoid of centromeric heterochromatin and gene-poor regions of chromosomes, they are enriched in gene-rich chromosomal regions. The active form of RNA polymerase II is also greatly enriched in blebs as well as nascent RNA but the nuclear co-activator SKIP is significantly reduced in blebs compared to other transcription factors. Our results suggest that the p.S143F progeria mutation has a severe impact not only on the structure of the lamina but also on the organization of interphase chromatin domains and transcription. These structural defects are likely to contribute to gene expression changes reported in progeria and other types of laminopathies. PMID:25738644

  14. Atypical Activation during the Embedded Figures Task as a Functional Magnetic Resonance Imaging Endophenotype of Autism

    ERIC Educational Resources Information Center

    Spencer, Michael D.; Holt, Rosemary J.; Chura, Lindsay R.; Calder, Andrew J.; Suckling, John; Bullmore, Edward T.; Baron-Cohen, Simon

    2012-01-01

    Atypical activation during the Embedded Figures Task has been demonstrated in autism, but has not been investigated in siblings or related to measures of clinical severity. We identified atypical activation during the Embedded Figures Task in participants with autism and unaffected siblings compared with control subjects in a number of temporal…

  15. TABS Manual for the Temperament and Atypical Behavior Scale: Early Childhood Indicators of Developmental Dysfunction.

    ERIC Educational Resources Information Center

    Neisworth, John T.; Bagnato, Stephen J.; Salvia, John; Hunt, Frances M.

    This manual describes the rationale, use, and validity of the Temperament and Atypical Behavior Scale (TABS), a norm-referenced measure of dysfunctional behavior appropriately used with infants and young children between the ages of 11 and 71 months. TABS is intended to identify children who are developing atypically or are at risk for atypical…

  16. Insecure Attachment States of Mind and Atypical Caregiving Behavior among Foster Mothers

    ERIC Educational Resources Information Center

    Ballen, Natasha; Bernier, Annie; Moss, Ellen; Tarabulsy, George M.; St-Laurent, Diane

    2010-01-01

    The current study examined the links between attachment state of mind (assessed with the Adult Attachment Interview) and atypical parenting behavior among 39 foster mothers. Insecure states of mind were associated with increased atypical parenting while interacting with the foster child, whereas unexpectedly, an unresolved state of mind was not.…

  17. Cognitive Function and Depression in Symptom Resolution in Schizophrenia Patients Treated with an Atypical Antipsychotic

    ERIC Educational Resources Information Center

    Stip, Emmanuel; Mancini-Marie, Adham

    2004-01-01

    Objective: To investigate which cognitive and affective features contribute most to responder/non-responder group separation during a switching trial with atypical antipsychotic. Design: A prospective open trial with an atypical antipsychotic (olanzapine). Patients: One hundred and thirty-four patients meeting diagnostic criteria for…

  18. Literary Life-Cycle Research as an Atypical Research Modality for Adult Education.

    ERIC Educational Resources Information Center

    McKenzie, Leon

    Literary analysis is approached as an atypical research modality: a modality that falls outside the parameters of experimental research usually described in the literature of educational meta-research. It is maintained that atypical research is appropriate as ancillary and complementary to typical research. Following Guba's explication of…

  19. Effects of Physical Atypicality on Children's Social Identities and Intergroup Attitudes

    ERIC Educational Resources Information Center

    Patterson, Meagan M.; Bigler, Rebecca S.

    2007-01-01

    Individuals vary in the degree to which they are representative, or typical, of their social groups. To investigate the effects of atypicality on intergroup attitudes, elementary-school-age children (N = 97) attending a summer school program were assigned to novel color groups that included typical (blue or green) and atypical (light blue or light…

  20. Traditional and Atypical Presentations of Anxiety in Youth with Autism Spectrum Disorder

    ERIC Educational Resources Information Center

    Kerns, Connor Morrow; Kendall, Philip C.; Berry, Leandra; Souders, Margaret C.; Franklin, Martin E.; Schultz, Robert T.; Miller, Judith; Herrington, John

    2014-01-01

    We assessed anxiety consistent (i.e., "traditional") and inconsistent (i.e., "atypical") with diagnostic and statistical manual (DSM) definitions in autism spectrum disorder (ASD). Differential relationships between traditional anxiety, atypical anxiety, child characteristics, anxiety predictors and ASD-symptomology were…

  1. [New employment opportunities: atypical jobs state of the art and general consideration on legislation].

    PubMed

    Messineo, A; Abetti, P; Bossi, A

    2003-01-01

    The Authors in this contribute, describe new job opportunities, that are to considered a need for those who are looking for a new employment. Otherwise this new kind of work, called atypical job, offers new possibility in lack of regular job. It also described the law juridical profiles of this new emerging atypical workers. PMID:14994921

  2. 9 mm ammunition used in a 40 caliber glock pistol: an atypical gunshot wound.

    PubMed

    Thogmartin, J R; Start, D A

    1998-05-01

    Atypical gunshot wounds due to ricochet and intermediate targets have been well described in the literature. We represent a case of suicide with an atypical entrance wound and bullet without rifling marks due to 9 mm ammunition being loaded and fired from a 40 caliber semiautomatic pistol. PMID:9608712

  3. A rare case of atypical skull base meningioma with perineural spread

    PubMed Central

    Walton, Henry; Morley, Simon; Alegre-Abarrategui, Javier

    2015-01-01

    Atypical meningioma is a rare cause of perineural tumour spread. In this report, we present the case of a 46-year-old female with an atypical meningioma of the skull base demonstrating perineural tumour spread. We describe the imaging features of this condition and its distinguishing features from other tumours exhibiting perineural spread. PMID:27200171

  4. [Angiolymphoid hyperplasia with eosinophilia. The spectrum from Kimura disease to atypical pyogenic granuloma].

    PubMed

    Wustrow, A; Mahrle, G

    1987-04-15

    A patient showed the "atypical pyogenic granuloma" of the head in combination with atopic dermatitis and proceeding pyogenic granuloma of the back. The diagnostic aspects of angiolymphoid hyperplasia with eosinophilia (ALHE) including Kimura's disease, subcutaneous ALHE, and atypical pyogenic granuloma are discussed. PMID:3111112

  5. Self-limiting Atypical Antipsychotics-induced Edema: Clinical Cases and Systematic Review

    PubMed Central

    Umar, Musa Usman; Abdullahi, Aminu Taura

    2016-01-01

    A number of atypical antipsychotics have been associated with peripheral edema. The exact cause is not known. We report two cases of olanzapine-induced edema and a brief review of atypical antipsychotic-induced edema, possible risk factors, etiology, and clinical features. The recommendation is given on different methods of managing this side effect. PMID:27335511

  6. Behavioral Activation for the Treatment of Atypical Depression: A Pilot Open Trial

    ERIC Educational Resources Information Center

    Weinstock, Lauren M.; Munroe, Mary K.; Miller, Ivan W.

    2011-01-01

    Psychosocial interventions for atypical depression (AD) have been relatively ignored in the clinical research literature, despite evidence that the atypical subtype of major depression is marked by earlier age of onset, longer duration of mood episode, greater symptom severity, and poorer response to pharmacologic treatment. Given the symptom…

  7. Gender Atypicality and Anxiety Response to Social Interaction Stress in Homosexual and Heterosexual Men.

    PubMed

    Jacobson, Roi; Cohen, Hagit; Diamond, Gary M

    2016-04-01

    Gender non-conforming behavior and a homosexual sexual orientation have both been linked to higher levels of anxiety. This study examined the independent and interactive effects of gender atypicality and sexual orientation on levels of state anxiety immediately following a stressful social interaction task among a sample of homosexual and heterosexual Israeli men (n = 36). Gender atypicality was measured via both self-report and observer ratings. State anxiety was measured via both self-report immediately subsequent to the stressful social interaction task and pre- to post task changes in salivary cortisol. Results showed that self-reported gender atypicality and heterosexual sexual orientation predicted higher levels of self-reported social interaction anxiety, but not changes in cortisol. There were no sexual orientation by gender behavior interactions and there were no significant effects for observer rated gender atypicality. These findings suggest that gender atypicality, not homosexuality, place individuals at risk for increased anxiety. PMID:25946903

  8. 226Ra or 226Ra/Ba dating of Holocene volcanic rocks: application to Mt. Etna and Merapi volcanoes

    NASA Astrophysics Data System (ADS)

    Condomines, M.; Gauthier, P. J.; Tanguy, J. C.; Gertisser, R.; Thouret, J. C.; Berthommier, P.; Camus, G.

    2005-02-01

    This paper shows how 226Ra- 230Th disequilibria can be used to date Holocene volcanic rocks from some well selected volcanoes. A systematic study of these disequilibria on historical or well-dated volcanic samples is indeed first required to test the applicability of this method. Two examples are described here to illustrate its potential. In the case of Mt. Etna, the good correlation observed between ( 226Ra) 0 activities at the time of eruption and Th contents in lava flows from the last two millennia [M. Condomines, J.C. Tanguy, V. Michaud, Magma dynamics at Mt. Etna: constraints from U-Th-Ra-Pb radioactive disequilibria and Sr isotopes in historical lavas, Earth Planet. Sci. Lett. 132 (1995) 25-41] is used to infer the ages of several newly analysed lava flows. The calculated ages are in good agreement with those deduced from the archaeomagnetic curve describing the variation of the geomagnetic field direction in southern Italy [J.C. Tanguy, I. Bucur, J.F.C. Thompson, Geomagnetic secular variation in Sicily and revised ages of historic lavas from Mt. Etna, Nature 318 (1985) 453-455, J.C. Tanguy, M. Le Goff, V. Chillemi, A. Paiotti, C. Principe, S. La Delfa, G. Patane, Variation séculaire de la direction du champ géomagnétique enregistrée par les laves de l'Etna et du Vésuve pendant les deux derniers millénaires, C. R. Acad. Sci. Paris 329 (1999) 557-564, J.C. Tanguy, M. Le Goff, C. Principe, S. Arrighi, V. Chillemi, A. Paiotti, S. La Delfa, G. Patane, Archaeomagnetic dating of Mediterranean volcanics of the last 2100 years: validity and limits. Earth Planet. Sci. Lett. 211 (2003) 111-124]. We also present a whole set of new U-series data on historical, recent, and older samples from Merapi (Indonesia), and show that the ( 226Ra)/Ba ratio has probably maintained a quasi-steady state value during at least the past four millennia, and can be used to infer the ( 226Ra) 0/Ba ratio of old volcanics at the time of eruption, and thus their ages. Comparison with

  9. Prevalence of the F-type lectin domain.

    PubMed

    Bishnoi, Ritika; Khatri, Indu; Subramanian, Srikrishna; Ramya, T N C

    2015-08-01

    F-type lectins are fucolectins with characteristic fucose and calcium-binding sequence motifs and a unique lectin fold (the "F-type" fold). F-type lectins are phylogenetically widespread with selective distribution. Several eukaryotic F-type lectins have been biochemically and structurally characterized, and the F-type lectin domain (FLD) has also been studied in the bacterial proteins, Streptococcus mitis lectinolysin and Streptococcus pneumoniae SP2159. However, there is little knowledge about the extent of occurrence of FLDs and their domain organization, especially, in bacteria. We have now mined the extensive genomic sequence information available in the public databases with sensitive sequence search techniques in order to exhaustively survey prokaryotic and eukaryotic FLDs. We report 437 FLD sequence clusters (clustered at 80% sequence identity) from eukaryotic, eubacterial and viral proteins. Domain architectures are diverse but mostly conserved in closely related organisms, and domain organizations of bacterial FLD-containing proteins are very different from their eukaryotic counterparts, suggesting unique specialization of FLDs to suit different requirements. Several atypical phylogenetic associations hint at lateral transfer. Among eukaryotes, we observe an expansion of FLDs in terms of occurrence and domain organization diversity in the taxa Mollusca, Hemichordata and Branchiostomi, perhaps coinciding with greater emphasis on innate immune strategies in these organisms. The naturally occurring FLDs with diverse domain organizations that we have identified here will be useful for future studies aimed at creating designer molecular platforms for directing desired biological activities to fucosylated glycoconjugates in target niches. PMID:25943580

  10. (226) RA AND (228) RA ACTIVITIES ASSOCIATED WITH AGRICULTURAL DRAINAGE PONDS AND WETLAND PONDS IN THE KANKAKEE WATERSHED, IL-IN, USA

    EPA Science Inventory

    Background radioactivity is elevated in many agricultural drainage ponds and also constructed wetland ponds in the Kankakee watershed. During 1995-1999, gross-a and -B activities were measured up to 455 and 1650 mBq L-1, respectively. 226Ra and 228Ra averaged 139 and 192 mBq L-01...

  11. Atomic Mass and Nuclear Binding Energy for Ra-186 (Radium)

    NASA Astrophysics Data System (ADS)

    Sukhoruchkin, S. I.; Soroko, Z. N.

    This document is part of the Supplement containing the complete sets of data of Subvolume B `Nuclei with Z = 55 - 100' of Volume 22 `Nuclear Binding Energies and Atomic Masses' of Landolt-Börnstein - Group I `Elementary Particles, Nuclei and Atoms', and additionally including data for nuclei with Z = 101 - 130. It provides atomic mass, mass excess, nuclear binding energy, nucleon separation energies, Q-values, and nucleon residual interaction parameters for atomic nuclei of the isotope Ra-186 (Radium, atomic number Z = 88, mass number A = 186).

  12. Atypical phenotype in two patients with LAMA2 mutations.

    PubMed

    Marques, Joana; Duarte, Sofia T; Costa, Sónia; Jacinto, Sandra; Oliveira, Jorge; Oliveira, Márcia E; Santos, Rosário; Bronze-da-Rocha, Elsa; Silvestre, Ana Rita; Calado, Eulália; Evangelista, Teresinha

    2014-05-01

    Congenital muscular dystrophy type 1A is caused by mutations in the LAMA2 gene, which encodes the α2-chain of laminin. We report two patients with partial laminin-α2 deficiency and atypical phenotypes, one with almost exclusive central nervous system involvement (cognitive impairment and refractory epilepsy) and the second with marked cardiac dysfunction, rigid spine syndrome and limb-girdle weakness. Patients underwent clinical, histopathological, imaging and genetic studies. Both cases have two heterozygous LAMA2 variants sharing a potentially pathogenic missense mutation c.2461A>C (p.Thr821Pro) located in exon 18. Brain MRI was instrumental for the diagnosis, since muscular examination and motor achievements were normal in the first patient and there was a severe cardiac involvement in the second. The clinical phenotype of the patients is markedly different which could in part be explained by the different combination of mutations types (two missense versus a missense and a truncating mutation). PMID:24534542

  13. An Atypical Case of Lymphocytic Panhypophysitis in a Pregnant Woman.

    PubMed

    Davies, Emma C; Jakobiec, Frederick A; Stagner, Anna M; Rizzo, Joseph F

    2016-09-01

    We describe a case of lymphocytic panhypophysitis (LPH) in a 30-year-old woman presenting with throbbing headaches and vision changes during her third trimester. LPH is the rarest subclassification of lymphocytic hypophysitis; it is typically found in males and has not previously been associated with pregnancy. Anterior and posterior pituitary deficits together with headaches should raise a high degree of suspicion regarding the possibility of LPH. The atypical magnetic resonance imaging finding of a heterogeneous pituitary mass additionally raised concern about pituitary apoplexy. Tissue from a transsphenoidal biopsy permitted diagnosis of lymphocytic hypophysitis. There was infiltration of the pituitary gland by small B and T lymphocytes. Resolution of the visual symptoms occurred after the biopsy and treatment with intravenous steroids. PMID:27008424

  14. Atypical β-Catenin Activated Child Hepatocellular Tumor

    PubMed Central

    Unlu, Havva Akmaz; Karakus, Esra; Yazal Erdem, Arzu; Yakut, Zeynep Ilerisoy

    2015-01-01

    Hepatocellular adenomas are a benign, focal, hepatic neoplasm that have been divided into four subtypes according to the genetic and pathological features. The β-catenin activated subtype accounts for 10-15% of all hepatocellular adenomas and specific magnetic resonance imaging features have been defined for different hepatocellular adenomas subtypes. The current study aimed to report the magnetic resonance imaging features of a well differentiated hepatocellular carcinoma that developed on the basis of β-catenin activated hepatocellular adenomas in a child. In this case, atypical diffuse steatosis was determined in the lesion. In the literature, diffuse steatosis, which is defined as a feature of the hepatocyte nuclear factor-1α-inactivated hepatocellular adenomas subtype, has not been previously reported in any β-catenin activated hepatocellular adenomas case. Interlacing magnetic resonance imaging findings between subtypes show that there are still many mysteries about this topic and larger studies are warranted. PMID:26157702

  15. [Atypical biliary stenting in patient with obstructive biliary jaundice].

    PubMed

    Garcarek, Jerzy; Kurcz, Jacek; Guziński, Maciej; Janczak, Dariusz

    2012-01-01

    Obstructive biliary jaundice is a common complication in patients with malignancies which infiltrate biliary ducts. If untreated efficiently the jaundice is fatal a short period of time. We present a case of 60-year-old male patient who had undergone Whipple procedure in the past and presented with local recurrence treated successfully by percutaneous stenting of obstructed biliary duct. When passing through the obstruction we observed a contrast-bile leakage at the level of occluded segment and instability of implanted stent which was a complication that extorted atypical approach. We applied a covered stent in association with oversized nitinol stent which allowed to form a funnel-like construction efficiently decompressing biliary tree. Thanks to this management we also avoided further complications. PMID:23276050

  16. Atypical distal renal tubular acidosis confirmed by mutation analysis.

    PubMed

    Weber, S; Soergel, M; Jeck, N; Konrad, M

    2000-12-01

    In autosomal dominant distal renal tubular acidosis type I (dRTA) impaired hydrogen ion secretion is associated with metabolic acidosis, hyperchloremic hypokalemia, hypercalciuria, nephrocalcinosis, and/or nephrolithiasis. A retardation of growth is commonly observed. In this report we present a family with autosomal dominant dRTA with an atypical and discordant clinical picture. The father presented with severe nephrocalcinosis, nephrolithiasis, and isosthenuria but metabolic acidosis was absent. His 6-year-old daughter, however, suffered from metabolic acidosis, hypokalemia, and hypercalciuria. In addition, sonography revealed multiple bilateral renal cysts but no nephrocalcinosis. Mutation analysis of the AE1 gene coding for the renal Cl-/HCO3(-)-exchanger AE1 displayed a heterozygous Arg589Cys exchange in both patients but not in the healthy family members. This point mutation is frequently associated with autosomal dominant dRTA. Diagnosis of autosomal dominant dRTA is supported in this family by results of AE1 mutation analysis. PMID:11149111

  17. Atypical antipsychotics in the treatment of early-onset schizophrenia

    PubMed Central

    Hrdlicka, Michal; Dudova, Iva

    2015-01-01

    Atypical antipsychotics (AAPs) have been successfully used in early-onset schizophrenia (EOS). This review summarizes the randomized, double-blind, controlled studies of AAPs in EOS, including clozapine, risperidone, olanzapine, aripiprazole, paliperidone, quetiapine, and ziprasidone. No significant differences in efficacy between AAPs were found, with the exception of clozapine and ziprasidone. Clozapine demonstrated superior efficacy in treatment-resistant patients with EOS, whereas ziprasidone failed to demonstrate efficacy in the treatment of EOS. Our review also focuses on the onset of action and weight gain associated with AAPs. The data on onset of action of AAPs in pediatric psychiatry are scanty and inconsistent. Olanzapine appears to cause the most significant weight gain in patients with EOS, while ziprasidone and aripiprazole seem to cause the least. PMID:25897226

  18. Utilizing hardware culture in a case of atypical keratitis.

    PubMed

    Ahmad, Sumayya; Jun, Albert S; Alkharashi, Majed

    2014-05-01

    This case report describes the diagnosis and management of a 22-year-old male soft contact lens wearer, who presented with an atypical corneal ulcer in the right eye. The patient presented with a one-day history of significant eye pain, photophobia, and multiple pseudodendritic lesions on the cornea with stromal infiltrate; the differential remained broad. The management was guided by the culture of hardware of the patient's contact lenses, which grew aspergillus only in the affected contact. In cases where corneal culture is negative, multiple organisms have been isolated from the corneal culture, or in cases where there is no response to medical treatment, culture of hardware may be a useful methodology for isolating the offending microbe in infectious corneal ulcers. PMID:24506413

  19. Atypical β-Catenin Activated Child Hepatocellular Tumor.

    PubMed

    Turan, Aynur; Unlu, Havva Akmaz; Karakus, Esra; Yazal Erdem, Arzu; Yakut, Zeynep Ilerisoy

    2015-06-01

    Hepatocellular adenomas are a benign, focal, hepatic neoplasm that have been divided into four subtypes according to the genetic and pathological features. The β-catenin activated subtype accounts for 10-15% of all hepatocellular adenomas and specific magnetic resonance imaging features have been defined for different hepatocellular adenomas subtypes. The current study aimed to report the magnetic resonance imaging features of a well differentiated hepatocellular carcinoma that developed on the basis of β-catenin activated hepatocellular adenomas in a child. In this case, atypical diffuse steatosis was determined in the lesion. In the literature, diffuse steatosis, which is defined as a feature of the hepatocyte nuclear factor-1α-inactivated hepatocellular adenomas subtype, has not been previously reported in any β-catenin activated hepatocellular adenomas case. Interlacing magnetic resonance imaging findings between subtypes show that there are still many mysteries about this topic and larger studies are warranted. PMID:26157702

  20. Exome Sequencing of an Adult Pituitary Atypical Teratoid Rhabdoid Tumor

    PubMed Central

    Biswas, Swethajit; Wood, Madeleine; Joshi, Abhijit; Bown, Nick; Strain, Lisa; Martinsson, Tommy; Campbell, James; Ashworth, Alan; Swain, Amanda

    2015-01-01

    Atypical teratoid rhabdoid tumors (AT/RTs) are rare pediatric brain tumors characterized by bialleic loss of the SMARCB1 tumor suppressor gene. In contrast to pediatric AT/RT that has a simple genome, very little is known about the adult AT/RT genomic landscape. Using a combination of whole-exome sequencing and high-resolution SNP array in a single adult pituitary AT/RT, we identified a total of 47 non-synonymous mutations, of which 20 were predicted to cause non-conservative amino acid substitutions, in addition to a subclone of cells with trisomy 8. We suggest that adult AT/RT may not be markedly dissimilar to other adult brain tumors where mutations in a range of genes, reflecting the functional specialization of different brain regions, but including SMARCB1 inactivation, may be required for its pathogenesis. PMID:26557502

  1. Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis

    PubMed Central

    Das, Biplab; Goyal, Manoj Kumar; Modi, Manish; Mehta, Sahil; Chakravarthi, Sudheer; Lal, Vivek; Vyas, Sameer

    2016-01-01

    Objectives: Subacute sclerosing panencephalitis (SSPE) is rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection with measles virus. No cure for SSPE exists, but the condition can be managed by medication if treatment is started at an early stage. Methods and Results: Heterogeneity of imaging findings in SSPE is not very uncommon. But pial and gyral enhancements are very rarely noticed. Significant asymmetric onset as well as pial-gyral enhancements is not reported. Herein we present a case of 16 years adolescent of SSPE having remarkable asymmetric pial-gyral enhancements, which were misinterpreted as tubercular infection. Conclusion: Early diagnosis and treatment is encouraging in SSPE, although it is not curable with current therapy. Clinico-radiological and electrophysiological correlation is very important in diagnosis of SSPE, more gravely in patients having atypical image findings as in our index case. PMID:27293348

  2. The biochemistry and biology of the atypical chemokine receptors.

    PubMed

    Graham, G J; Locati, M; Mantovani, A; Rot, A; Thelen, M

    2012-07-30

    A subset of chemokine receptors, initially called "silent" on the basis of their apparent failure to activate conventional signalling events, has recently attracted growing interest due to their ability to internalize, degrade, or transport ligands and thus modify gradients and create functional chemokine patterns in tissues. These receptors recognize distinct and complementary sets of ligands with high affinity, are strategically expressed in different cellular contexts, and lack structural determinants supporting Gα(i) activation, a key signalling event in cell migration. This is in keeping with the hypothesis that they have evolved to fulfil fundamentally different functions to the classical signalling chemokine receptors. Based on these considerations, these receptors (D6, Duffy antigen receptor for chemokines (DARC), CCX-CKR1 and CXCR7) are now collectively considered as an emerging class of 'atypical' chemokine receptors. In this article, we review the biochemistry and biology of this emerging chemokine receptor subfamily. PMID:22698181

  3. Epidermoid cyst of the testis: An atypical sonographic appearance.

    PubMed

    Chen, Shu-Ting; Chiou, Hong-Jen; Pan, Chin-Chen; Shen, Shu-Huei; Chou, Yi-Hong; Tiu, Chui-Mei; Wang, Hsin-Kai; Lai, Yi-Chen; Lin, Yung-Hui; Wang, Jane; Chang, Cheng-Yen

    2016-09-01

    Epidermoid cysts are rare. They represent the most common benign tumor of the testis. The sonographic appearances of testicular epidermoid cysts usually include avascular, mostly lamellated, heterogeneous internal echotexture, with hypoechoic and hyperechoic concentric rings, accounting for the typical onion-ring appearance. On MRI, epidermoid cysts show a low-signal-intensity center, with internal concentric rings of alternating high- and low-signal intensity on T2-weighted images, which correlates with the onion-ring appearance. We report a patient with testicular epidermoid cyst with atypical ultrasound and MRI appearances that led to the erroneous initial diagnosis of "burned-out" tumor. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:448-451, 2016. PMID:27028726

  4. Accidental low velocity atypical missile injury to the head.

    PubMed

    Chattopadhyay, Saurabh

    2008-12-01

    Missile injuries on the head are mostly due to firearms. Atypical missiles may be encountered in case of shrapnel of bomb explosions but rarely because of stones. The present case is a rare case where a stone propelled by the pressure from the rear wheel of a speeding truck on the highway, struck the head of a 7-year-old girl resulting in fatality. Reconstruction of the incident on the basis of history and postmortem findings throws some light on the mechanism. The case is unique as it is the first reported case of an accidental missile injury to the head resulting in fatality without any direct human involvement for propulsion of the projectile. PMID:19259020

  5. Atypical Attentional Networks and the Emergence of Autism

    PubMed Central

    Keehn, Brandon; Müller, Ralph-Axel; Townsend, Jeanne

    2012-01-01

    The sociocommunicative impairments that define autism spectrum disorder (ASD) are not present at birth but emerge gradually over the first two years of life. In typical development, basic attentional processes may provide a critical foundation for sociocommunicative abilities. Therefore early attentional dysfunction in ASD may result in atypical development of social communication. Prior research has demonstrated that persons with ASD exhibit early and lifelong impairments in attention. The primary aim of this paper is to provide a review of the extant research on attention in ASD using a framework of functionally independent attentional networks as conceptualized by Posner and colleagues: the alerting, orienting and executive control networks (Posner and Petersen, 1990; Petersen & Posner, 2012). The neural substrates and typical development of each attentional network is briefly discussed, a review of the ASD attention literature is presented, and a hypothesis is proposed that links aberrant attentional mechanisms, specifically impaired disengagement of attention, with the emergence of core ASD symptoms. PMID:23206665

  6. Atypical post-finasteride syndrome: A pharmacological riddle.

    PubMed

    Gupta, Anita K; Sharma, Neetu; Shukla, Prashant

    2016-01-01

    Finasteride and dutasteride are commonly used 5-alpha reductase inhibitors. While finasteride is a selective inhibitor of 5-alpha reductase Type II, dutasteride inhibits 5- alpha reductase Type I and II. The United States Food and Drug Administration approved the use of finasteride for benign prostatic hypertrophy (BPH) as well as androgenic alopecia (AGA) while dutasteride is approved only for BPH. Off-label use of dutasteride is not uncommon in AGA as well. Although the postfinasteride syndrome (PFS) is a well-established entity, its symptomatology is quite variable. Here, we describe a case of an atypical PFS in a patient treated with dutasteride and finasteride for AGA. The multisystem involvement and irreversible nature of this case warrant its reporting. PMID:27298504

  7. Atypical post-finasteride syndrome: A pharmacological riddle

    PubMed Central

    Gupta, Anita K.; Sharma, Neetu; Shukla, Prashant

    2016-01-01

    Finasteride and dutasteride are commonly used 5-alpha reductase inhibitors. While finasteride is a selective inhibitor of 5-alpha reductase Type II, dutasteride inhibits 5- alpha reductase Type I and II. The United States Food and Drug Administration approved the use of finasteride for benign prostatic hypertrophy (BPH) as well as androgenic alopecia (AGA) while dutasteride is approved only for BPH. Off-label use of dutasteride is not uncommon in AGA as well. Although the postfinasteride syndrome (PFS) is a well-established entity, its symptomatology is quite variable. Here, we describe a case of an atypical PFS in a patient treated with dutasteride and finasteride for AGA. The multisystem involvement and irreversible nature of this case warrant its reporting. PMID:27298504

  8. Atypical patterns of cardiac involvement in Fabry disease.

    PubMed

    Coughlan, J J; Elkholy, K; O'Brien, J; Kiernan, T

    2016-01-01

    A 58-year-old woman was referred to our cardiology service with chest pain, exertional dyspnoea and palpitations on a background of known Fabry disease diagnosed with genetic testing in 1994. ECG showed sinus rhythm, shortened PR interval, widespread t wave inversion, q waves in the lateral leads and left ventricular hypertrophy (LVH). Coronary angiogram showed only mild atheroma. Transthoracic echocardiogram showed anterolateral LVH and reduced left ventricular cavity size in keeping with Fabry cardiomyopathy. Cardiac MRI demonstrated asymmetric hypertrophy with evidence of diffuse myocardial fibrosis in the maximally hypertrophied segments from base to apex with late gadolinium enhancement in the anterior and anteroseptal walls. This was quite an atypical appearance for Fabry cardiomyopathy. This case highlights the heterogeneity of patterns of cardiac involvement that may be associated with this rare X-linked lysosomal disorder. PMID:26989114

  9. Atypical work hours and metabolic syndrome among police officers.

    PubMed

    Violanti, John M; Burchfiel, Cecil M; Hartley, Tara A; Mnatsakanova, Anna; Fekedulegn, Desta; Andrew, Michael E; Charles, Luenda E; Vila, Bryan J

    2009-01-01

    This study examined whether atypical work hours are associated with metabolic syndrome among a random sample of 98 police officers. Shift work and overtime data from daily payroll records and reported sleep duration were obtained. Metabolic syndrome was defined as elevated waist circumference and triglycerides, low HDL cholesterol, hypertension, and glucose intolerance. Multivariate analysis of variance and analysis of covariance models were used for analyses. Officers working midnight shifts were on average younger and had a slightly higher mean number of metabolic syndrome components. Stratification on sleep duration and overtime revealed significant associations between midnight shifts and the mean number of metabolic syndrome components among officers with less sleep (p = .013) and more overtime (p = .007). Results suggest shorter sleep duration and more overtime combined with midnight shift work may be important contributors to the metabolic syndrome. PMID:19864222

  10. [Aortic intramural hematoma fissuration: atypical presentation in an aircraft pilot].

    PubMed

    Fozzato, Francesca; Prioli, Maria Antonia; Santini, Francesco; Menini, Fabio; Pavan, Michela; Guarise, Paola; Vassanelli, Corrado

    2010-02-01

    Aortic intramural hematoma is a life-threatening thoracic aortic pathology. In this report we describe a case of fissuration of an aortic intramural hematoma with atypical clinical presentation, which occurred in an aircraft pilot. The patient was admitted to our emergency room with transient chest pain developed during a flight landing, followed only by persistent abdominal pain. The ECG and cardiac enzymes were normal. A portable two-dimensional transthoracic echocardiogram showed aortic root dilation and pericardial effusion. Transesophageal echocardiography showed aortic intramural hematoma with fissuration into the pericardial space. The angio-computed tomography confirmed the diagnosis. Two hours after admission the patient, with signs of cardiac tamponade, underwent Bentall surgical intervention without complications. PMID:20408481

  11. Atypical trait inferences from facial cues in alexithymia.

    PubMed

    Brewer, Rebecca; Collins, Fredrika; Cook, Richard; Bird, Geoffrey

    2015-10-01

    It is often difficult to distinguish strangers' permanent facial shapes from their transient facial expressions, for example, whether they are scowling or have narrow-set eyes. Overinterpretation of ambiguous cues may contribute to the rapid character judgments we make about others. Someone with narrow eyes might be judged untrustworthy, because of strong associations between facial anger and threat. To test this hypothesis, we investigated the trait judgments made by individuals with severe alexithymia, associated with impaired recognition of facial emotion. Consistent with the hypothesis, alexithymic participants demonstrated reduced interrater consistency when judging the character traits of unfamiliar faces, and the presence of subtle emotions. Nevertheless, where alexithymics perceived, or misperceived, emotion cues, the character traits inferred thereafter were broadly typical. The finding that individuals with developmental deficits of emotion recognition exhibit atypical attribution of character traits, confirms the hypothesis that emotion-recognition mechanisms play a causal role in character judgments. PMID:25867918

  12. Figurative language processing in atypical populations: the ASD perspective.

    PubMed

    Vulchanova, Mila; Saldaña, David; Chahboun, Sobh; Vulchanov, Valentin

    2015-01-01

    This paper is intended to provide a critical overview of experimental and clinical research documenting problems in figurative language processing in atypical populations with a focus on the Autistic Spectrum. Research in the comprehension and processing of figurative language in autism invariably documents problems in this area. The greater paradox is that even at the higher end of the spectrum or in the cases of linguistically talented individuals with Asperger syndrome, where structural language competence is intact, problems with extended language persist. If we assume that figurative and extended uses of language essentially depend on the perception and processing of more concrete core concepts and phenomena, the commonly observed failure in atypical populations to understand figurative language remains a puzzle. Various accounts have been offered to explain this issue, ranging from linking potential failure directly to overall structural language competence (Norbury, 2005; Brock et al., 2008) to right-hemispheric involvement (Gold and Faust, 2010). We argue that the dissociation between structural language and figurative language competence in autism should be sought in more general cognitive mechanisms and traits in the autistic phenotype (e.g., in terms of weak central coherence, Vulchanova et al., 2012b), as well as failure at on-line semantic integration with increased complexity and diversity of the stimuli (Coulson and Van Petten, 2002). This perspective is even more compelling in light of similar problems in a number of conditions, including both acquired (e.g., Aphasia) and developmental disorders (Williams Syndrome). This dissociation argues against a simple continuity view of language interpretation. PMID:25741261

  13. Predictors of Effect of Atypical Antipsychotics on Speech

    PubMed Central

    Sinha, Preeti; Vandana, Valiya Parambath; Lewis, Nikita V; Jayaram, Mannaralukrishnaiah; Enderby, Pamela

    2015-01-01

    Background: Most of the studies have looked into the effect of typical antipsychotics on speech secondary to tardive dyskinesia. Aims: This study was aimed to explore the factors predicting the effect of atypical antipsychotic medications on the production of speech. Materials and Methods: One hundred and forty patients on stable regimen of three or more months on risperidone (92), olanzapine (28), aripiprazole (14), and clozapine (6) were recruited for the study. Speech was assessed by maximum phonation duration task, s/z ratio, diadochokinetic task, acoustic analysis and Frenchay Dysarthria Assessment (FDA). Extrapyramidal symptoms (EPS) were assessed by Simpson Angus scale. Statistical Analysis: Spearman correlation analysis was carried out to find the association between speech parameters and continuous variables. Effect of EPS, duration and dose of antipsychotic treatment on speech parameters was compared using Mann-Whitney test. Results: The risperidone group differ from other antipsychotics groups significantly in s/z ratio (0.07), FDA-total (0.23) and FDA-reflex (0.25). People who took antipsychotic for more than 2 years had lower score of FDA-palate (P = 0.042), and FDA-respiratory (P = 0.04) and higher values in noise-harmonic ratio (P = 0.011) and maximum /fundamental frequency (MFF) for males (P = 0.02). Effect of EPS was seen on MFF for males (spearman correlation coefficient = 0.34) and on almost all sections of FDA (spearman correlation coefficients = -0.2 to -0.33). Conclusion: Both duration of use and propensity of atypical antipsychotics to cause EPS can influence the speech performance of the patients. This information can be useful, particularly in people with the requirement of high quality speech. PMID:26702176

  14. Figurative language processing in atypical populations: the ASD perspective

    PubMed Central

    Vulchanova, Mila; Saldaña, David; Chahboun, Sobh; Vulchanov, Valentin

    2015-01-01

    This paper is intended to provide a critical overview of experimental and clinical research documenting problems in figurative language processing in atypical populations with a focus on the Autistic Spectrum. Research in the comprehension and processing of figurative language in autism invariably documents problems in this area. The greater paradox is that even at the higher end of the spectrum or in the cases of linguistically talented individuals with Asperger syndrome, where structural language competence is intact, problems with extended language persist. If we assume that figurative and extended uses of language essentially depend on the perception and processing of more concrete core concepts and phenomena, the commonly observed failure in atypical populations to understand figurative language remains a puzzle. Various accounts have been offered to explain this issue, ranging from linking potential failure directly to overall structural language competence (Norbury, 2005; Brock et al., 2008) to right-hemispheric involvement (Gold and Faust, 2010). We argue that the dissociation between structural language and figurative language competence in autism should be sought in more general cognitive mechanisms and traits in the autistic phenotype (e.g., in terms of weak central coherence, Vulchanova et al., 2012b), as well as failure at on-line semantic integration with increased complexity and diversity of the stimuli (Coulson and Van Petten, 2002). This perspective is even more compelling in light of similar problems in a number of conditions, including both acquired (e.g., Aphasia) and developmental disorders (Williams Syndrome). This dissociation argues against a simple continuity view of language interpretation. PMID:25741261

  15. Familial Atypical Cold Urticaria: Description of a New Hereditary Disease

    PubMed Central

    Gandhi, Chhavi; Healy, Chris; Wanderer, Alan A.; Hoffman, Hal M.

    2009-01-01

    Background Acquired Cold Urticaria (ACU) is usually a self-limited, sporadic, cutaneous disease diagnosed by history and positive cold stimulation time tests (CSTT). We describe three unrelated families (A,B,C) with lifelong atypical cold urticaria, distinguished from ACU and Familial Cold Autoinflammatory Syndrome (FCAS). Objective To describe a new hereditary disease of cold urticaria and study its pathogenesis. Methods Questionnaires, interviews, physical exams, skin testing and biopsies were obtained. Absolute values, means and prevalence percentages of data are reported. Results 35 subjects are described with Familial Atypical Cold Urticaria (FACU) (A:17, B:8, C:10) displaying an autosomal dominant (AD) pattern of inheritance. All tested subjects had negative CSTT. Completed questionnaires from family A and B (35) revealed that all affected subjects had lifelong symptoms that began in early childhood with pruritis, erythema and urticaria after cold exposure. Angioedema (A:23%; B:42%), syncope and/or near-syncope (A:46%; B:86%) were also present. Triggers included cold atmosphere (100%), aquatic activities (A:92%, B:100%), handling cold objects (A:54%, B:71%) and ingestion of cold food or beverage (A:69%, B:100%). Skin biopsies demonstrated a mast cell infiltrate with the appearance of degranulation after cold challenge. Conclusions FACU is a new cold-induced inherited disease that is different than ACU in its natural history, atmospheric cold elicitation, severity of systemic reactions and CSTT results. FACU differs from FCAS in symptom-timing and the absence of fever, chills and joint pain. The etiology is suspected to be mast cell-related. Treatment of reactions is similar to ACU. Further evaluation of pathogenesis and genetics is warranted. PMID:19910034

  16. Raman spectroscopic analysis of atypical proliferative lesions of the breast

    NASA Astrophysics Data System (ADS)

    Subramanian, K.; Kendall, C.; Stone, N.; Brown, J. C.; McCarthy, K.; Bristol, J.; Chan, Y. H.

    2006-02-01

    Atypical lesions of the breast have potential to turn malignant. The diagnosis of these lesions has increased considerably with screening mammography. A good understanding of their progression to invasive cancer is yet to be proved. Using Raman spectroscopy to study their chemical finger printing at different stages of proliferation a clear picture of whether a progression exists between lesions could be made. At present there is no clear recognition of the biochemical changes that distinguish between the different proliferative lesions of the breast. Our aim is to understand these changes through Raman mapping studies. Raman spectroscopy is a highly sensitive and specific technique for demonstration of biochemical changes in different atypical proliferative lesions of the breast. The technique could be used to classify the different grades and analyse progression of pathology in the proliferative lesions of the breast. Breast pathologists carefully marked 50 ducts and classified the different pathology on H and E sections from biopsy samples. Raman spectra were measured, using a Renishaw Raman Spectrometer, on a 20-micron thick consecutive frozen section. Principal component analysis was undertaken using Matlab. Pseudocolor maps of the principal components scores have been generated. The peaks of the corresponding loads were identified enabling visualisation of the biochemical changes associated with proliferative lesions. Proliferative lesions of the duct were grouped according to the existing standard pathological classification and formed four major groups-HUT, ADH, DCIS and IDC. Spectra of biochemical constituents were fitted to mean spectra from selected regions, taken from maps of each pathology, to identify the relative concentration of the constituents. The study gave an insight into chemical make up of the ducts in each pathology group and showed similar results to earlier studies in progression but no clear-cut demarcation or continuum of the

  17. Atypical Noncontiguous Multiple Spinal Tuberculosis: A Case Report

    PubMed Central

    Kim, Jang-Hoon; Choi, Jong-Il; Lim, Dong-Jun

    2014-01-01

    Objective Spinal tuberculosis-associated symptoms are not so unique as to immediately indicate the proper diagnosis in most cases. Distinguishing spinal tuberculosis (Pott's disease) from pyogenic spondylitis is often difficult, and lesions metastatic from systemic malignancy are the other major entity from which spinal tuberculosis must be distinguished. Clinical Presentation A 27-year-old male patient presented with a history of back pain after a minor trauma 1 month ago. Computed tomography and magnetic resonance imaging of the thoracic spine showed multiple osteolytic bone lesions at the bodies of T9, T10 and T11 vertebrae and the spinous processes of T12 and L1. Other noncontiguous osteolytic lesions were noted at S2 body and right sacro-iliac joint. Intervention To confirm the pathologic diagnosis, the patient underwent an open biopsy for the T12 and L1 spinous process lesions and a percutaneous transpedicular biopsy on T9, T10, T11 lesions. Frozen biopsy was reported as compatible with chronic granulomatous caseating necrosis without malignant cells. The final diagnosis was an atypical presentation of multiple spinal tuberculosis. The patient received an appropriate enteral anti-tuberculosis therapy and recovered without any complications. Follow-up MRI taken after a year of medical treatment revealed marked resolution of the lesions. Conclusion Current research indicates the incidence of multi-level noncontiguous, remote vertebral tuberculosis is 1.1% to 16%. Because tuberculous spondylitis could represent variant and atypical pattern, the disease should be considered in differential diagnosis along with other diseases such as metastatic neoplasm, pyogenic spondylitis, especially when the radiologic studies are revealing multiple spinal lesions. PMID:25110488

  18. Skin biopsies in the evaluation of atypical optic neuropathies.

    PubMed

    Bielory, L; Kupersmith, M; Warren, F; Bystryn, J; Frohman, L

    1993-01-01

    Patients with atypical clinical presentations of common optic neuropathies such as optic neuritis (ON), anterior ischemic optic neuropathy (AION), or optic neuropathy of unknown etiology (UON) are difficult to distinguish from inflammatory autoimmune optic neuropathy (AON) which is typically associated with a poor visual prognosis, unless treated with high doses of corticosteroids and/or immunosuppressive agents. The authors retrospectively evaluated 34 patients [AON (n = 12); AION (n = 5); ON (n = 9); UON (n = 8)] with visual loss which deteriorated over weeks to months or followed an atypical course, for the presence of immunological markers suggestive of AON. These markers included serological testing for antiphospholipid (APA) and antinuclear (ANA) antibodies, and evaluation of histopathologic and immunofluorescent staining of skin biopsies. All patients underwent a skin biopsy. Four of the 12 patients with AON had urticarial cutaneous lesions which revealed leukocytoclastic and/or lymphohistiocytic vasculitis. Seven of the remaining eight AON patients had skin biopsies of non-lesional skin which revealed immunoreactant deposition. Seven of the 21 skin biopsies obtained from the non-AON patients had findings of vacuolization or mild perivascular infiltration of lymphocytes (n = 5) and immunofluorescent deposits (n = 2). Abnormal skin biopsies (92%;p = 0.0009) and circulating APA (82%; p = 0.013) were common in AON patients while ANA was not statistically increased in AON patients (p = 0.06) when compared to the remaining patients as a whole. AON patients typically demonstrate evidence of systemic autoimmune involvement, as manifested by cutaneous abnormalities such as urticarial vasculitis and/or immunoreactant deposition and circulating APA. These may serve as markers for identifying AON patients who may be treated with immunomodulatory agents. PMID:22822778

  19. Atypical Integration of Motion Signals in Autism Spectrum Conditions

    PubMed Central

    Robertson, Caroline E.; Martin, Alex; Baker, Chris I.; Baron-Cohen, Simon

    2012-01-01

    Vision in Autism Spectrum Conditions (ASC) is characterized by enhanced perception of local elements, but impaired perception of global percepts. Deficits in coherent motion perception seem to support this characterization, but the roots and robustness of such deficits remain unclear. We aimed to investigate the dynamics of the perceptual decision-making network known to support coherent motion perception. In a series of forced-choice coherent motion perception tests, we parametrically varied a single stimulus dimension, viewing duration, to test whether the rate at which evidence is accumulated towards a global decision is atypical in ASC. 40 adult participants (20 ASC) performed a classic motion discrimination task, manually indicating the global direction of motion in a random-dot kinematogram across a range of coherence levels (2–75%) and stimulus-viewing durations (200–1500 ms). We report a deficit in global motion perception at short viewing durations in ASC. Critically, however, we found that increasing the amount of time over which motion signals could be integrated reduced the magnitude of the deficit, such that at the longest duration there was no difference between the ASC and control groups. Further, the deficit in motion integration at the shortest duration was significantly associated with the severity of autistic symptoms in our clinical population, and was independent from measures of intelligence. These results point to atypical integration of motion signals during the construction of a global percept in ASC. Based on the neural correlates of decision-making in global motion perception our findings suggest the global motion deficit observed in ASC could reflect a slower or more variable response from the primary motion area of the brain or longer accumulation of evidence towards a decision-bound in parietal areas. PMID:23185249

  20. Modified Ham test for atypical hemolytic uremic syndrome

    PubMed Central

    Gavriilaki, Eleni; Yuan, Xuan; Ye, Zhaohui; Ambinder, Alexander J.; Shanbhag, Satish P.; Streiff, Michael B.; Kickler, Thomas S.; Moliterno, Alison R.; Sperati, C. John

    2015-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by excessive activation of the alternative pathway of complement (APC). Atypical HUS is frequently a diagnosis of exclusion. Differentiating aHUS from other TMAs, especially thrombotic thrombocytopenic purpura (TTP), is difficult due to overlapping clinical manifestations. We sought to develop a novel assay to distinguish aHUS from other TMAs based on the hypothesis that paroxysmal nocturnal hemoglobinuria cells are more sensitive to APC-activated serum due to deficiency of glycosylphosphatidylinositol- anchored complement regulatory proteins (GPI-AP). Here, we demonstrate that phosphatidylinositol-specific phospholipase C–treated EA.hy926 cells and PIGA-mutant TF-1 cells are more susceptible to serum from aHUS patients than parental EA.hy926 and TF-1 cells. We next studied 31 samples from 25 patients with TMAs, including 9 with aHUS and 12 with TTP. Increased C5b-9 deposition was evident by confocal microscopy and flow cytometry on GPI-AP–deficient cells incubated with aHUS serum compared with heat-inactivated control, TTP, and normal serum. Differences in cell viability were observed in biochemically GPI-AP–deficient cells and were further increased in PIGA-deficient cells. Serum from patients with aHUS resulted in a significant increase of nonviable PIGA-deficient TF-1 cells compared with serum from healthy controls (P < .001) and other TMAs (P < .001). The cell viability assay showed high reproducibility, sensitivity, and specificity in detecting aHUS. In conclusion, we developed a simple, rapid, and serum-based assay that helps to differentiate aHUS from other TMAs. PMID:25862562

  1. The structural plasticity of SCA7 domains defines their differential nucleosome-binding properties

    PubMed Central

    Bonnet, Jacques; Wang, Ying-Hui; Spedale, Gianpiero; Atkinson, R Andrew; Romier, Christophe; Hamiche, Ali; Pijnappel, W W M Pim; Timmers, H Th Marc; Tora, László; Devys, Didier; Kieffer, Bruno

    2010-01-01

    SAGA (Spt–Ada–Gcn5 acetyltransferase), a coactivator complex involved in chromatin remodelling, harbours both histone acetylation and deubiquitination activities. ATXN7/Sgf73 and ATXN7L3, two subunits of the SAGA deubiquitination module, contain an SCA7 domain characterized by an atypical zinc-finger. We show that the yeast Sgf73–SCA7 domain is not required to recruit Sgf73 into SAGA. Instead, it binds to nucleosomes, a property that is conserved in the human ATXN7–SCA7 domain but is lost in the ATXN7L3 domain. The solution structures of the SCA7 domain of both ATXN7 and ATXN7L3 reveal a new, common zinc-finger motif at the heart of two distinct folds, providing a molecular basis for the observed functional differences. PMID:20634802

  2. 226Ra/238U disequilibrium in an upland organic soil exhibiting elevated natural radioactivity.

    PubMed

    Dowdall, Mark; O'Dea, John

    2002-01-01

    This paper presents the results of a study into the anomalous 226Ra/238U disequilibrium (226Ra/238U of 0.5-9) exhibited by an upland organic soil in Co. Donegal, Ireland. Radiochemical speciation of 226Ra, 238U and 225Ra indicates that in this organic soil the high 226Ra/238U ratio is due to loss of 235U relative to 226Ra via oxidation and mobilisation of 238U in the upper layers of the soil and subsequent loss in solution. At the lower, more reducing depths of the soil profile, 238U and 226Ra are essentially in equilibrium. Loss of 238U appears to occur primarily from the easily oxidised organic and iron oxide fractions of the soil, samples exhibiting high 226Ra/238U ratios displaying significantly lower 238U levels in these fractions than samples whose ratio is below the average value for the soil of the valley. Selective enrichment of 226Ra by plants or preferential leaching of 226Ra from the underlying rock is not supported by the results of this study. PMID:11848154

  3. Transition to turbulent thermal convection beyond Ra=1010 detected in numerical simulations

    NASA Astrophysics Data System (ADS)

    Vincent, Alain P.; Yuen, David A.

    2000-05-01

    We have conducted high-resolution two-dimensional calculations for a Boussinesq convection model with a Prandtl number of unity in an aspect-ratio 3 box, going from Rayleigh numbers between 108 to 1014. A grid of 1024×3076 grid points consisting of a cosine-sine basis set has been employed for free-slip boundary conditions. We have found evidence for a transition involving the branching of plumes at a Rayleigh number of 1010. Inside the core of these ``superplumes,'' the structure is extremely complex. There may be another transition at Ra of 1012, where a secondary instability may develop in regions of the local Rayleigh number which becomes supercritical inside the core of the complex ``superplumes.'' For Ra of 108 to 1010, Ra follows a 13 power law in the Nusselt-Rayleigh number relationship. From Ra of 1010 to 1012, Ra follows a 12 power law. Above this value the Nusselt number becomes insensitive to the variation in the global Rayleigh number and this is due to the development of small-scale convection cells vertically aligned in the interior of the extremely high Ra number flow. The global Reynolds number scales as Re~Ra1/4 up to Ra of 1014. Scaling relationships based on global properties would not work in extremely high Ra situations beyond Ra of 1012 because of the complex turbulent layered convection in the core of the flow and the severe degradation of the boundary layers.

  4. Smoking and Subclinical ILD in RA versus the Multi-Ethnic Study of Atherosclerosis.

    PubMed

    Johnson, Cheilonda; Giles, Jon T; Bathon, Joan; Lederer, David; Hoffman, Eric A; Barr, R Graham; Danoff, Sonye K

    2016-01-01

    A population-based cohort showed an association between cigarette smoking and subclinical parenchymal lung disease defined as regions of increased computed tomography (CT) lung densitometry. This technique has not been applied to the rheumatoid arthritis (RA) population where associated ILD is highly prevalent. The association between cumulative cigarette smoking and volume of areas of high attenuation (HAA: >-600 and <-250 Hounsfield Units) on full inspiratory CT was compared in 172 RA participants and 3,969 controls in a general population sample. Multivariable regression models were used to adjust for demography, anthropometrics, percent emphysema, and CT parameters. The mean cumulative cigarette smoking exposure was 25 (IQR 10-42) and 15(IQR 5-31) pack-years for the RA and non-RA cohorts, respectively. Mean HAA was 153(±57) cm3 and 129(±50) cm3 in the RA and non-RA cohorts, respectively. Each 10 cigarette pack-year increment was associated with a higher HAA by 0.03% (95% CI, 0.007-0.05%) in RA patients and by 0.008% (95% CI, 0.003-0.01%) in those without RA (interaction p = 0.001). Cigarette smoking was associated with higher lung attenuation; with a magnitude of association more pronounced in those with RA than in the general population. These data suggest that cigarette smoking may be a more potent ILD risk factor for RA patients than in the general population. PMID:27050433

  5. Smoking and Subclinical ILD in RA versus the Multi-Ethnic Study of Atherosclerosis

    PubMed Central

    Johnson, Cheilonda; Giles, Jon T.; Bathon, Joan; Lederer, David; Hoffman, Eric A.; Barr, R. Graham; Danoff, Sonye K.

    2016-01-01

    A population-based cohort showed an association between cigarette smoking and subclinical parenchymal lung disease defined as regions of increased computed tomography (CT) lung densitometry. This technique has not been applied to the rheumatoid arthritis (RA) population where associated ILD is highly prevalent. The association between cumulative cigarette smoking and volume of areas of high attenuation (HAA: >-600 and <-250 Hounsfield Units) on full inspiratory CT was compared in 172 RA participants and 3,969 controls in a general population sample. Multivariable regression models were used to adjust for demography, anthropometrics, percent emphysema, and CT parameters. The mean cumulative cigarette smoking exposure was 25 (IQR 10–42) and 15(IQR 5–31) pack-years for the RA and non-RA cohorts, respectively. Mean HAA was 153(±57) cm3 and 129(±50) cm3 in the RA and non-RA cohorts, respectively. Each 10 cigarette pack-year increment was associated with a higher HAA by 0.03% (95% CI, 0.007–0.05%) in RA patients and by 0.008% (95% CI, 0.003–0.01%) in those without RA (interaction p = 0.001). Cigarette smoking was associated with higher lung attenuation; with a magnitude of association more pronounced in those with RA than in the general population. These data suggest that cigarette smoking may be a more potent ILD risk factor for RA patients than in the general population. PMID:27050433

  6. Dynamic-domain-decomposition parallel molecular dynamics

    NASA Astrophysics Data System (ADS)

    Srinivasan, S. G.; Ashok, I.; Jônsson, Hannes; Kalonji, Gretchen; Zahorjan, John

    1997-05-01

    Parallel molecular dynamics with short-range forces can suffer from load-imbalance problems and attendant performance degradation due to density variations in the simulated system. In this paper, we describe an approach to dynamical load balancing, enabled by the Ādhāra runtime system. The domain assigned to each processor is automatically and dynamically resized so as to evenly distribute the molecular dynamics computations across all the processors. The algorithm was tested on an Intel Paragon parallel computer for two and three-dimensional Lennard-Jones systems containing 99 458 and 256000 atoms, respectively, and using up to 256 processors. In these benchmarks, the overhead for carrying out the load-balancing operations was found to be small and the total computation time was reduced by as much as 50%.

  7. The Bayo Canyon/radioactive lanthanum (RaLa) program

    SciTech Connect

    Dummer, J.E.; Taschner, J.C.; Courtright, C.C.

    1996-04-01

    LANL conducted 254 radioactive lanthanum (RaLa) implosion experiments Sept. 1944-March 1962, in order to test implosion designs for nuclear weapons. High explosives surrounding common metals (surrogates for Pu) and a radioactive source containing up to several thousand curies of La, were involved in each experiment. The resulting cloud was deposited as fallout, often to distances of several miles. This report was prepared to summarize existing records as an aid in evaluating the off-site impact, if any, of this 18-year program. The report provides a historical setting for the program, which was conducted in Technical Area 10, Bayo Canyon about 3 miles east of Los Alamos. A description of the site is followed by a discussion of collateral experiments conducted in 1950 by US Air Force for developing an airborne detector for tracking atmospheric nuclear weapons tests. All known off-site data from the RaLa program are tabulated and discussed. Besides the radiolanthanum, other potential trace radioactive material that may have been present in the fallout is discussed and amounts estimated. Off-site safety considerations are discussed; a preliminary off-site dose assessment is made. Bibliographical data on 33 persons important to the program are presented as footnotes.

  8. Investigations On The EnviSAT RA2 Tropospheric Correction

    NASA Astrophysics Data System (ADS)

    Blarel, F.; Legresy, B.

    2013-12-01

    The Center for the Topography of Oceans and the Hydrosphere (CTOH), the Altimeter Data Service of the LEGOS laboratory pursue an effort to validate and improve ENVISAT RA2 altimetry. We investigate the stability and reliability of different corrections for the altimetric measurements. With the one before last ENVISAT RA2 processing (v1.0), the ICE Validation chain of the CTOH detected some issues with the Dry Tropospheric Correction (DTC) over the Cryosphere. Here we present the validation of the latest reprocessing (v2.1) of ENVISAT data. We observe the limitation of this re-processed DTC, especially the jump observed at cycle 44 over Antarctica which causes significant local trend and suspect changes in the variability of this correction over time. We investigated a solution and re- compute this correction all over the globe with the ERA Interim ECMWF pressure fields and using directly the altimetric range measurement rather than any external digital elevation model (DEM). We present the results of this investigation, the statistics of previous (DTC_v2.1) and new corrections (DTC_ctoh) and in the context of different surfaces. The improvement is significant, and we discuss the aspects and impacts of this improvement, in terms of meteorological-fields, procedure, and location.

  9. Predictors of atypical femoral fractures during long term bisphosphonate therapy: A case series & review of literature

    PubMed Central

    Bhadada, Sanjay Kumar; Sridhar, Subbiah; Muthukrishnan, Jeyaram; Mithal, Ambrish; Sharma, Dinesh C.; Bhansali, Anil; Dhiman, Vandana

    2014-01-01

    Background & objectives: Bisphosphonates (BPs) are the most widely prescribed medicines for the treatment of osteoporosis because of their efficacy and favourable safety profile. There have been, several reports on an increased incidence of atypical femoral fractures after long term treatment with BPs. The objective of this study was to evaluate the clinical presentation including prodromal symptoms, skeletal radiograph findings, type and duration of BPs received and treatment outcome of patients who developed atypical femoral fractures during bisphosphonate therapy. Methods: In this retrospective study, eight patients with atypical femoral fractures were analysed based on clinical features, biochemical and radiological investigations. Results: Of the eight patients, who sustained atypical femoral fractures, six were on alendronate and two were on zoledronate therapy before the fractures. In addition to BPs, two patients were on long term corticosteroid therapy for rheumatoid arthritis and Addison's disease. Three patients had bilateral atypical femoral fractures. Except one, all of them had prodromal symptoms prior to fracture. Skeletal radiograph showed cortical thickening, pointed (beaking of) cortical margin and transverse fracture in meta-diaphyseal location. Serum calcium, phosphate, alkaline phosphatase (ALP) and intact parathyroid hormone (iPTH) concentrations were within the reference range in all patients. Interpretation & conclusions: Long term bisphosphonate therapy may increase the risk of atypical femoral fractures. Presence of prodromal pain, thickened cortex with cortical beaking may be an early clue for predicting the atypical fractures. High risk patients need periodical skeletal survey and a close follow up for early detection of cases. PMID:25222777

  10. Parafibromin and APC as screening markers for malignant potential in atypical parathyroid adenomas.

    PubMed

    Juhlin, C Christofer; Nilsson, Inga-Lena; Johansson, Kenth; Haglund, Felix; Villablanca, Andrea; Höög, Anders; Larsson, Catharina

    2010-09-01

    The identification of parathyroid carcinomas is based upon histopathological criteria in which an invasive growth pattern or distant metastasis is demonstrated. A dilemma arises when tumours present with atypical histopathological features but lack direct evidence of malignancy. Recently, reduced expression or loss of the tumour suppressor proteins parafibromin and adenomatous polyposis coli (APC) has been associated with parathyroid malignancy. We report results from APC and parafibromin expression analyses by immunohistochemistry and Western blot in five cases of atypical adenoma, a single case of carcinoma and 54 adenomas without atypical features. Complete loss of APC immunoreactivity and reduced expression of parafibromin was evident in two of the atypical adenomas and in the parathyroid carcinoma. By contrast, all adenomas displayed APC expression, including two cases with hyperparathyroidism 2 gene (HRPT2) mutations and loss of parafibromin expression. We conclude that loss of APC is a frequent molecular event in atypical adenomas and carcinomas, but not in adenomas. Following verification in an independent material, APC could become a valuable tool when assessing parathyroid tumours in the clinical setting. Furthermore, the molecular resemblance of atypical adenomas with carcinoma concerning parafibromin and APC expression indicates that atypical adenomas should be subjects to watchful follow-up. PMID:20473645

  11. Hospitalization and cost after switching from atypical to typical antipsychotics in schizophrenia patients in Thailand

    PubMed Central

    Boonlue, Tuanthon; Subongkot, Suphat; Dilokthornsakul, Piyameth; Kongsakon, Ronnachai; Pattanaprateep, Oraluck; Suanchang, Orabhorn; Chaiyakunapruk, Nathorn

    2016-01-01

    Background Several clinical practice guidelines suggest using atypical over typical antipsychotics in patients diagnosed with schizophrenia. Nevertheless, cost-containment policy urged restricting usage of atypical antipsychotics and switching from atypical to typical antipsychotics. Objective This study aimed to evaluate clinical and economic impacts of switching from atypical to typical antipsychotics in schizophrenia patients in Thailand. Methods From October 2010 through September 2013, a retrospective cohort study was performed utilizing electronic database of two tertiary hospitals. Schizophrenia patients aged 18 years or older and being treated with atypical antipsychotics were included. Patients were classified as atypical antipsychotic switching group if they switched to typical antipsychotics after 180 days of continual atypical antipsychotics therapy. Outcomes were schizophrenia-related hospitalization and total health care cost. Logistic and Poisson regression were used to evaluate the risk of hospitalization, and generalized linear model with gamma distribution was used to determine the health care cost. All analyses were adjusted by employing propensity score and multivariable analyses. All cost estimates were adjusted according to 2013 consumer price index and converted to US$ at an exchange rate of 32.85 Thai bahts/US$. Results A total of 2,354 patients were included. Of them, 166 (7.1%) patients switched to typical antipsychotics. The adjusted odds ratio for schizophrenia-related hospitalization in atypical antipsychotic switching group was 1.87 (95% confidence interval [CI] 1.23–2.83). The adjusted incidence rate ratio was 2.44 (95% CI 1.57–3.79) for schizophrenia-related hospitalizations. The average total health care cost was lower in patients with antipsychotic switching (−$64; 95% CI −$459 to $332). Conclusion Switching from atypical to typical antipsychotics is associated with an increased risk of schizophrenia-related hospitalization

  12. Simultaneous, bilateral, complete atypical femoral fractures after long-term alendronate use.

    PubMed

    Higgins, Mark; Morgan-John, Sam; Badhe, Sachin

    2016-12-01

    Over the past decade there have been increasing reports of atypical femoral fractures (AFFs) associated with bisphosphonate use. Reported cases of bilateral involvement usually refer to sequential injuries, or a complete fracture with an incomplete injury to the contralateral limb. In this case report we describe simultaneous, bilateral, complete atypical femoral fractures following a simple fall. A history of prodromal pain, previous radiological evidence of cortical thickening and long term alendronate therapy for osteoporosis secondary to corticosteroid treatment paint a classical picture of the presentation of an atypical fracture pattern of which orthopaedic surgeons should be aware. PMID:27570414

  13. Genetic Characterization of Atypical Mansonella (Mansonella) ozzardi Microfilariae in Human Blood Samples from Northeastern Peru

    PubMed Central

    Marcos, Luis A.; Arrospide, Nancy; Recuenco, Sergio; Cabezas, Cesar; Weil, Gary J.; Fischer, Peter U.

    2012-01-01

    DNA sequence comparisons are useful for characterizing proposed new parasite species or strains. Microfilariae with an atypical arrangement of nuclei behind the cephalic space have been recently described in human blood samples from the Amazon region of Peru. Three blood specimens containing atypical microfilariae were genetically characterized using three DNA markers (5S ribosomal DNA, 12S ribosomal DNA, and cytochrome oxidase I). All atypical microfilariae were clustered into the Mansonella group and indistinguishable from M. ozzardi based on these DNA markers. PMID:22826497

  14. Study of soil-plant transfer of 226Ra under greenhouse conditions.

    PubMed

    Soudek, Petr; Petrová, Sárka; Benesová, Dagmar; Kotyza, Jan; Vágner, Martin; Vanková, Radomíra; Vanek, Tomás

    2010-06-01

    A soil-plant transfer study was performed using soil from a former uranium ore processing factory in South Bohemia. We present the results from greenhouse experiments which include estimates of the time required for phytoremediation. The accumulation of (226)Ra by different plant species from a mixture of garden soil and contaminated substrate was extremely variable, ranging from 0.03 to 2.20 Bq (226)Ra/g DW. We found differences in accumulation of (226)Ra between plants from the same genus and between cultivars of the same plant species. The results of (226)Ra accumulation showed a linear relation between concentration of (226)Ra in plants and concentration of (226)Ra in soil mixtures. On the basis of these results we estimated the time required for phytoremediation, but this appears to be too long for practical purposes. PMID:18823682

  15. IL2RA and IL7RA genes confer susceptibility for multiple sclerosis in two independent European populations.

    PubMed

    Weber, F; Fontaine, B; Cournu-Rebeix, I; Kroner, A; Knop, M; Lutz, S; Müller-Sarnowski, F; Uhr, M; Bettecken, T; Kohli, M; Ripke, S; Ising, M; Rieckmann, P; Brassat, D; Semana, G; Babron, M-C; Mrejen, S; Gout, C; Lyon-Caen, O; Yaouanq, J; Edan, G; Clanet, M; Holsboer, F; Clerget-Darpoux, F; Müller-Myhsok, B

    2008-04-01

    Multiple sclerosis (MS) is the most common chronic inflammatory neurologic disorder diagnosed in young adults and, due to its chronic course, is responsible for a substantial economic burden. MS is considered to be a multifactorial disease in which both genetic and environmental factors intervene. The well-established human leukocyte antigen (HLA) association does not completely explain the genetic impact on disease susceptibility. However, identification and validation of non-HLA-genes conferring susceptibility to MS has proven to be difficult probably because of the small individual contribution of each of these genes. Recently, associations with two single nucleotide polymorphisms (SNPs) in the IL2RA gene (rs12722489, rs2104286) and one SNP in the IL7RA gene (rs6897932) have been reported by several groups. These three SNPs were genotyped in a French and a German population of MS patients using the hME assay by the matrix-assisted laser desorption/ionization time of flight technology (Sequenom, San Diego, CA, USA). We show that these SNPs do contribute to the risk of MS in these two unrelated European MS patient populations with odds ratios varying from 1.1 to 1.5. The discovery and validation of new genetic risk factors in independent populations may help toward the understanding of MS pathogenesis by providing valuable information on biological pathways to be investigated. PMID:18354419

  16. Laser-trapping of {sup 225}Ra and {sup 226}Ra with repumping by room-temperature blackbody radiation.

    SciTech Connect

    Guest, J. R.; Scielzo, N. D.; Ahmad, I.; Bailey, K.; Greene, J. P.; Holt, R. J.; Lu, Z.-T.; O'Connor, T. P.; Potterveld, D. H.; Physics; Enrico Fermi Inst.; Univ. of Chicago

    2007-02-27

    We have demonstrated Zeeman slowing and capture of neutral {sup 225}Ra and {sup 226}Ra atoms in a magneto-optical trap. The intercombination transition {sup 1}S{sub 0} {yields} {sup 3}P{sub 1} is the only quasicycling transition in radium and was used for laser-cooling and trapping. Repumping along the {sup 3}D{sub 1} {yields} {sup 1}P{sub 1} transition extended the lifetime of the trap from milliseconds to seconds. Room-temperature blackbody radiation was demonstrated to provide repumping from the metastable {sup 3}P{sub 0} level. We measured the isotope shift and hyperfine splittings on the {sup 3}D{sub 1} {yields} {sup 1}P{sub 1} transition with the laser-cooled atoms, and set a limit on the lifetime of the {sup 3}D{sub 1} level based on the measured blackbody repumping rate. Laser-cooled and trapped radium is an attractive system for studying fundamental symmetries.

  17. Domains and Naive Theories

    PubMed Central

    Gelman, Susan A.; Noles, Nicholaus S.

    2013-01-01

    Human cognition entails domain-specific cognitive processes that influence memory, attention, categorization, problem-solving, reasoning, and knowledge organization. This review examines domain-specific causal theories, which are of particular interest for permitting an examination of how knowledge structures change over time. We first describe the properties of commonsense theories, and how commonsense theories differ from scientific theories, illustrating with children’s classification of biological and non-biological kinds. We next consider the implications of domain-specificity for broader issues regarding cognitive development and conceptual change. We then examine the extent to which domain-specific theories interact, and how people reconcile competing causal frameworks. Future directions for research include examining how different content domains interact, the nature of theory change, the role of context (including culture, language, and social interaction) in inducing different frameworks, and the neural bases for domain-specific reasoning. PMID:24187603

  18. Production of Ra225 precursor for Ac225/Bi213 generators. Final CRADA Report.

    SciTech Connect

    Ehst, D. A.; Nuclear Engineering Division

    2009-10-02

    Russian subcontractors shipped two small deliveries of Ra225 to PNNL for evaluation. The activity was close to the requisite amount in the subcontract. The first labeled properly; the second did not. This showed that the Russians could supply Ra225 to the US; however they proved unable to meet future demands for larger amounts of activity. The US DOE attempts to get Ra225 from Russian should be terminated; the Russians, under Rosatom, are not interested in this business.

  19. Multimeric and differential binding of CIN85/CD2AP with two atypical proline-rich sequences from CD2 and Cbl-b*.

    PubMed

    Ceregido, M Angeles; Garcia-Pino, Abel; Ortega-Roldan, Jose L; Casares, Salvador; López Mayorga, Obdulio; Bravo, Jeronimo; van Nuland, Nico A J; Azuaga, Ana I

    2013-07-01

    The CD2AP (CD2-associated protein) and CIN85 (Cbl-interacting protein of 85 kDa) adaptor proteins each employ three Src homology 3 (SH3) domains to cluster protein partners and ensure efficient signal transduction and down-regulation of tyrosine kinase receptors. Using NMR, isothermal titration calorimetry and small-angle X-ray scattering methods, we have characterized several binding modes of the N-terminal SH3 domain (SH3A) of CD2AP and CIN85 with two natural atypical proline-rich regions in CD2 (cluster of differentiation 2) and Cbl-b (Casitas B-lineage lymphoma), and compared these data with previous studies and published crystal structures. Our experiments show that the CD2AP-SH3A domain forms a type II dimer with CD2 and both type I and type II dimeric complexes with Cbl-b. Like CD2AP, the CIN85-SH3A domain forms a type II complex with CD2, but a trimeric complex with Cbl-b, whereby the type I and II interactions take place at the same time. Together, these results explain how multiple interactions among similar SH3 domains and ligands produce a high degree of diversity in tyrosine kinase, cell adhesion or T-cell signaling pathways. PMID:23663663

  20. Flow rates and reaction rates in the Galapagos Rise spreading center hydrothermal system as inferred from 228Ra/226Ra in vesicomyid clam shells

    PubMed Central

    Turekian, Karl K.; Cochran, J. Kirk

    1986-01-01

    The 228Ra/226Ra ratios in a previously dated vesicomyid clam shell were used to determine that seawater was in contact with mid-oceanic-ridge basalt glass for 22-45 years prior to arrival to the surface at 350°C at the Galapagos Rise Spreading Center. The minimum rate of reaction for the 45-year sojourn time, based on a water/rock ratio of 2.8 derived from 226Ra concentrations, is 8 g of basalt altered per kg of seawater per year. PMID:16593746

  1. Interaction of atypical cadherin Fat1 with SoHo adaptor proteins CAP/ponsin and ArgBP2.

    PubMed

    Braun, Gerald S; Kuszka, Andrzej; Dau, Cécile; Kriz, Wilhelm; Moeller, Marcus J

    2016-03-25

    Mammalian Fat1 is a giant atypical cadherin/tumor suppressor involved in the regulation of cellular orientation, migration, and growth. Fat1 is implicated in the development of the brain, eye, and kidney. Altered expression or mutations of FAT1 are also associated with cancer and facioscapulohumeral muscular dystrophy (FSHD). Yet, the mechanistic functions of this pathway remain incompletely understood. Here, we report the identification of Sorbin-homology (SoHo) proteins as novel interaction partners of Fat1 by virtue of a yeast-two-hybrid screen. SoHo proteins play diverse roles as adaptor proteins in cell signaling, cell adhesion and sarcomere architecture, including altered expression in cancer and FSHD. Specifically, we found SoHo proteins CAP/ponsin-1 and -2 (Sorbs1) and ArgBP2 (Sorbs2) to interact with the cytoplasmic domain of Fat1. We mapped the interaction to a prolin-rich classic type II PXXP motif within Fat1 and to the three Src-homology (SH3) domains within SoHo proteins using mutant expression in yeast, pulldown assays, and cell culture. Functionally, endogenous ponsin-2 expression of NRK-52E cells at cellular leading edges was lost upon knockdown of Fat1. In summary, our data point to an interaction of Fat1 with SoHo proteins that is able to recruit SoHo proteins to sites of Fat1 expression. PMID:26903299

  2. Clinical Utility of Amyloid PET Imaging in the Differential Diagnosis of Atypical Dementias and Its Impact on Caregivers.

    PubMed

    Bensaïdane, Mohamed Reda; Beauregard, Jean-Mathieu; Poulin, Stéphane; Buteau, François-Alexandre; Guimond, Jean; Bergeron, David; Verret, Louis; Fortin, Marie-Pierre; Houde, Michèle; Bouchard, Rémi W; Soucy, Jean-Paul; Laforce, Robert

    2016-04-18

    Recent studies have supported a role for amyloid positron emission tomography (PET) imaging in distinguishing Alzheimer's disease (AD) pathology from other pathological protein accumulations leading to dementia. We investigated the clinical utility of amyloid PET in the differential diagnosis of atypical dementia cases and its impact on caregivers. Using the amyloid tracer 18F-NAV4694, we prospectively scanned 28 patients (mean age 59.3 y, s.d. 5.8; mean MMSE 21.4, s.d. 6.0) with an atypical dementia syndrome. Following a comprehensive diagnostic workup (i.e., history taking, neurological examination, blood tests, neuropsychological evaluation, MRI, and FDG-PET), no certain diagnosis could be arrived at. Amyloid PET was then conducted and classified as positive or negative. Attending physicians were asked to evaluate whether this result led to a change in diagnosis or altered management. They also reported their degree of confidence in the diagnosis. Caregivers were met after disclosure of amyloid PET results and completed a questionnaire/interview to assess the impact of the scan. Our cohort was evenly divided between positive (14/28) and negative (14/28) 18F-NAV4694 cases. Amyloid PET resulted in a diagnostic change in 9/28 cases (32.1%: 17.8% changed from AD to non-AD, 14.3% from non-AD to AD). There was a 44% increase in diagnostic confidence. Altered management occurred in 71.4% (20/28) of cases. Knowledge of amyloid status improved caregivers' outcomes in all domains (anxiety, depression, disease perception, future anticipation, and quality of life). This study suggests a useful additive role for amyloid PET in atypical cases with an unclear diagnosis beyond the extensive workup of a tertiary memory clinic. Amyloid PET increased diagnostic confidence and led to clinically significant alterations in management. The information gained from that test was well received by caregivers and encouraged spending quality time with their loved ones. PMID:27104896

  3. Simulations of Core Convection in Rotating A-Type Stars: Magnetic Dynamo Action

    NASA Astrophysics Data System (ADS)

    Brun, Allan Sacha; Browning, Matthew K.; Toomre, Juri

    2005-08-01

    Core convection and dynamo activity deep within rotating A-type stars of 2 Msolar are studied with three-dimensional nonlinear simulations. Our modeling considers the inner 30% by radius of such stars, thus capturing within a spherical domain the convective core and a modest portion of the surrounding radiative envelope. The magnetohydrodynamic (MHD) equations are solved using the anelastic spherical harmonic (ASH) code to examine turbulent flows and magnetic fields, both of which exhibit intricate time dependence. By introducing small seed magnetic fields into our progenitor hydrodynamic models rotating at 1 and 4 times the solar rate, we assess here how the vigorous convection can amplify those fields and sustain them against ohmic decay. Dynamo action is indeed realized, ultimately yielding magnetic fields that possess energy densities comparable to that of the flows. Such magnetism reduces the differential rotation obtained in the progenitors, partly by Maxwell stresses that transport angular momentum poleward and oppose the Reynolds stresses in the latitudinal balance. In contrast, in the radial direction we find that the Maxwell and Reynolds stresses may act together to transport angular momentum. The central columns of slow rotation established in the progenitors are weakened, with the differential rotation waxing and waning in strength as the simulations evolve. We assess the morphology of the flows and magnetic fields, their complex temporal variations, and the manner in which dynamo action is sustained. Differential rotation and helical convection are both found to play roles in giving rise to the magnetic fields. The magnetism is dominated by strong fluctuating fields throughout the core, with the axisymmetric (mean) fields there relatively weak. The fluctuating magnetic fields decrease rapidly with radius in the region of overshooting, and the mean toroidal fields less so due to stretching by rotational shear.

  4. Exploring the role of auditory analysis in atypical compared to typical language development.

    PubMed

    Grube, Manon; Cooper, Freya E; Kumar, Sukhbinder; Kelly, Tom; Griffiths, Timothy D

    2014-02-01

    The relationship between auditory processing and language skills has been debated for decades. Previous findings have been inconsistent, both in typically developing and impaired subjects, including those with dyslexia or specific language impairment. Whether correlations between auditory and language skills are consistent between different populations has hardly been addressed at all. The present work presents an exploratory approach of testing for patterns of correlations in a range of measures of auditory processing. In a recent study, we reported findings from a large cohort of eleven-year olds on a range of auditory measures and the data supported a specific role for the processing of short sequences in pitch and time in typical language development. Here we tested whether a group of individuals with dyslexic traits (DT group; n = 28) from the same year group would show the same pattern of correlations between auditory and language skills as the typically developing group (TD group; n = 173). Regarding the raw scores, the DT group showed a significantly poorer performance on the language but not the auditory measures, including measures of pitch, time and rhythm, and timbre (modulation). In terms of correlations, there was a tendency to decrease in correlations between short-sequence processing and language skills, contrasted by a significant increase in correlation for basic, single-sound processing, in particular in the domain of modulation. The data support the notion that the fundamental relationship between auditory and language skills might differ in atypical compared to typical language development, with the implication that merging data or drawing inference between populations might be problematic. Further examination of the relationship between both basic sound feature analysis and music-like sound analysis and language skills in impaired populations might allow the development of appropriate training strategies. These might include types of musical

  5. Learning and Domain Adaptation

    NASA Astrophysics Data System (ADS)

    Mansour, Yishay

    Domain adaptation is a fundamental learning problem where one wishes to use labeled data from one or several source domains to learn a hypothesis performing well on a different, yet related, domain for which no labeled data is available. This generalization across domains is a very significant challenge for many machine learning applications and arises in a variety of natural settings, including NLP tasks (document classification, sentiment analysis, etc.), speech recognition (speakers and noise or environment adaptation) and face recognition (different lighting conditions, different population composition).

  6. Classic and Atypical FOP Phenotypes are Caused by Mutations in the BMP Type I Receptor ACVR1

    PubMed Central

    Kaplan, Frederick S.; Xu, Meiqi; Seemann, Petra; Connor, Michael; Glaser, David L.; Carroll, Liam; Delai, Patricia; Fastnacht-Urban, Elisabeth; Forman, Stephen J.; Gillessen-Kaesbach, Gabriele; Hoover-Fong, Julie; Köster, Bernhard; Pauli, Richard M.; Reardon, William; Zaidi, Syed-Adeel; Zasloff, Michael; Morhart, Rolf; Mundlos, Stefan; Groppe, Jay; Shore, Eileen M.

    2010-01-01

    Fibrodysplasia ossificans progressiva (FOP) is an autosomal dominant human disorder of bone formation that causes developmental skeletal defects and extensive debilitating bone formation within soft connective tissues (heterotopic ossification) during childhood. All patients with classic clinical features of FOP (great toe malformations and progressive heterotopic ossification) have previously been found to carry the same heterozygous mutation (c.617G>A; p.R206H) in the GS activation domain of activin A type I receptor/activin-like kinase 2 (ACVR1/ALK2), a bone morphogenetic protein (BMP) type I receptor. Among patients with FOP-like heterotopic ossification and/or toe malformations, we identified patients with clinical features unusual for FOP. These atypical FOP patients form two classes: FOP-plus (classic defining features of FOP plus one or more atypical features) and FOP variants (major variations in one or both of the two classic defining features of FOP). All patients examined have heterozygous ACVR1 missense mutations in conserved amino acids. While the recurrent c.617G>A; p.R206H mutation was found in all cases of classic FOP and most cases of FOP-plus, novel ACVR1 mutations occur in the FOP variants and two cases of FOP-plus. Protein structure homology modeling predicts that each of the amino acid substitutions activates the ACVR1 protein to enhance receptor signaling. We observed genotype-phenotype correlation between some ACVR1 mutations and the age of onset of heterotopic ossification or on embryonic skeletal development. PMID:19085907

  7. Rapid method for the determination of 226Ra in hydraulic fracturing wastewater samples

    DOE PAGESBeta

    Maxwell, Sherrod L.; Culligan, Brian K.; Warren, Richard A.; McAlister, Daniel R.

    2016-03-24

    A new method that rapidly preconcentrates and measures 226Ra from hydraulic fracturing wastewater samples was developed in the Savannah River Environmental Laboratory. The method improves the quality of 226Ra measurements using gamma spectrometry by providing up to 100x preconcentration of 226Ra from this difficult sample matrix, which contains very high levels of calcium, barium, strontium, magnesium and sodium. The high chemical yield, typically 80-90%, facilitates a low detection limit, important for lower level samples, and indicates method ruggedness. Ba-133 tracer is used to determine chemical yield and correct for geometry-related counting issues. The 226Ra sample preparation takes < 2 hours.

  8. Atypical Renal Cysts: A Morphologic, Immunohistochemical, and Molecular Study.

    PubMed

    Matoso, Andres; Chen, Ying-Bei; Rao, Vishal; Wang, Lu; Cheng, Liang; Epstein, Jonathan I

    2016-02-01

    There is a lack of standardized nomenclature for renal cysts lined by multiple cell layers or with short papillary projections but without nests of epithelial cells within the stroma. We retrieved 29 cases (15 nephrectomies, 14 partial nephrectomies) from the surgical pathology files of Johns Hopkins Hospital from 1993 to 2014 and performed immunohistochemistry for CK7, alpha-methylacyl-CoA racemase (AMACR), CAIX, and CD10 and fluorescence in situ hybridization for trisomy 7 and 17 and 3p deletion. The mean age at excision was 58 years (range, 29 to 80 y) with 16 men and 13 women. Mean size was 2.9 cm (range, 0.3 to 10 cm). The cysts were grouped by their morphology into (1) clear cell, (2) eosinophilic stratified, and (3) eosinophilic papillary. By immunohistochemistry, 7/9 (78%) of the clear cell cases were diffusely positive for both CK7 and CAIX resembling the pattern seen in clear cell papillary renal cell carcinoma. The majority of eosinophilic stratified (4/6; 67%) and eosinophilic papillary (12/14; 86%) cases were positive for CK7 and had variable staining for AMACR, CD10, or CAIX, suggesting a differentiation more aligned with papillary renal cell carcinoma. The most common molecular alterations detected were trisomy 17 (n=6) and trisomy 7 (n=4). One case showed deletion of chromosome 3p. Clinical follow-up information was available in 23 patients; 20 were alive with no evidence of disease after a median follow-up of 20 months (range, 3 to 120 mo), 1 patient was dead due to metastatic lung cancer, 1 of sepsis, and 1 of unknown reason. Atypical renal cysts present as complex radiologic lesions, as secondary lesions in patients with a renal mass, or in a background of chronic renal disease. These atypical cysts appear heterogenous, and some follow in their morphology and immunoprofile with well-established renal tumors. The presence of 3p deletion and trisomy 7/17 suggests that in some cases they may be precursors of renal cell carcinoma. Longer follow

  9. ENVISAT RA-2/MWR Cross-Calibration and Validation Results

    NASA Astrophysics Data System (ADS)

    Benveniste, J.; Ra-2/Mwr Cross-Calibration; Validation Team

    2003-04-01

    ESA launched its environmental research satellite, ENVISAT, on 1 March 2002. It carries a suite of 10 instruments offering opportunities for a broad range of scientific research and applications. In particular ENVISAT carries a radar altimeter (RA-2), a microwave radiometer (MWR) and a precise tracking system (DORIS). The evaluation of the quality of the ENVISAT Altimetry geophysical data products, the validation against in-situ data and models and the cross-calibration on ERS-2 and other flying altimeters has been performed during the commissioning phase within a team of scientists drawn up from the pre-launch announcement of opportunity. An overview of the objective and approach will be recalled and the results obtained from this teamwork will be presented.

  10. Massive Resurfacing of the Ionian Volcano Ra Patera

    NASA Technical Reports Server (NTRS)

    1996-01-01

    Four views of the volcano Ra Patera on Jupiter's moon Io showing changes seen on June 27th, 1996 by the Galileo spacecraft as compared to views seen by the Voyager spacecraft during the 1979 flybys. Clockwise from upper left is a Voyager 1 high resolution image, a Voyager 1 color image, a Galileo color image, and a Voyager 2 color image. North is to the top of the picture. Observations obtained by J. Spencer and others with the Hubble Space Telescope had indicated a major change in recent years. The Galileo images reveal the detailed morphology of new deposits. Dark materials, previously confined to a summit caldera, appear to have overflowed the caldera walls to produce a small flow to the south and a larger flow to the southeast. New bright deposits covering an area of about 40,000 square kilometers (the size of New Jersey) surround the dark materials. The morphology of the bright materials suggests emplacement as lava flows rather than pyroclastics. Notice the lobate margins and how the bright materials embay a plateau in the upper left. The Voyager 1 images also reveal relatively bright lava flows emanating from Ra Patera, especially to the northeast. The colors of the flows match those of sulfur plus SO2 frost. Images are 953 km wide. The Jet Propulsion Laboratory, Pasadena, CA manages the mission for NASA's Office of Space Science, Washington, DC. This image and other images and data received from Galileo are posted on the World Wide Web, on the Galileo mission home page at URL http://galileo.jpl.nasa.gov. Background information and educational context for the images can be found at URL http://www.jpl.nasa.gov/galileo/sepo

  11. Atypical Teratoid/Rhabdoid Tumors in Adults: A Case Report and Treatment-Focused Review

    PubMed Central

    Shonka, Nicole A.; Armstrong, Terri S.; Prabhu, Sujit S.; Childress, Amanda; Choi, Shauna; Langford, Lauren A.; Gilbert, Mark R.

    2011-01-01

    Atypical teratoid/rhabdoid tumor is predominantly a childhood tumor and has only been rarely reported in adults; therefore, treatment regimens are often extrapolated from the pediatric experience. Typically, children are treated with craniospinal radiation therapy which is often followed by systemic chemotherapy. Employing pediatric regimens to treat this tumor in adult patients poses a particular risk for myelosuppression, as the prescribed doses in pediatric protocols exceed those tolerated by adults, and conventional craniospinal radiation can be associated with prolonged myelotoxicity and a depletion of the bone marrow reserve in vertebrae of adults. Here we present a case of a woman with a pineal region atypical teratoid/rhabdoid tumor, an unusual adult cancer presenting in an atypical location. This is followed by a review of the disease in adult patients with an emphasis on treatment and suggestions to minimize myelotoxicity. Keywords Atypical rhabdoid tumor; AT/RT; Pineal tumor; Adult PMID:21811535

  12. General Information about Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor

    MedlinePlus

    ... Teratoid/Rhabdoid Tumor Treatment (PDQ®)–Patient Version General Information About Childhood Central Nervous System (CNS) Atypical Teratoid/ ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  13. Cardiogenic shock from atypical Takotsubo cardiomyopathy attributed to acute disseminated encephalomyelitis lesion involving the medulla.

    PubMed

    Venkatraman, A; Bajaj, N S; Khawaja, A; Meador, W

    2016-04-01

    We present here a case of atypical Takotsubo cardiomyopathy arising as a result of a lesion in the medulla oblongata. The patient was diagnosed with acute disseminated encephalomyelitis, and had improvement with intravenous steroids. PMID:26868678

  14. Bilateral Persistent Sciatic Artery Aneurysm Discovered by Atypical Sciatica: A Case Report

    SciTech Connect

    Mazet, Nathalie; Soulier-Guerin, Karine; Ruivard, Marc; Garcier, Jean-Marc; Boyer, Louis

    2006-12-15

    We report a case of a bilateral persistent sciatic artery aneurysm, diagnosed by atypical sciatica on computed tomography and magnetic resonance imaging. The different variants, the revealing features, and possible treatment are discussed.

  15. The atypical entrance wound: differential diagnosis and discussion of an unusual cause.

    PubMed

    Molina, D Kimberley; Rulon, Jennifer J; Wallace, Edward I

    2012-09-01

    The appearance of gunshot entrance wounds on the body depends upon many factors including, the type of firearm, the type of ammunition, the location of the wound on the body, and the circumstances of how a wound was sustained. Atypical gunshot entrance wounds are usually created when the bullet is destabilized prior to entering the body and consequently does not enter the body nose first but sideways or at an angle. The most common causes of an atypical entrance wound are bullet ricochet and interaction with an intermediate target. A case is presented in which the decedent sustained a gunshot wound with an atypical entrance. The cause of the atypical nature of the wound was determined to be increased yaw due to bullet instability caused by the condition of the firearm used, not a ricochet or intermediate target. The case emphasizes the importance of collaborative investigation between the different forensic agencies in gunshot cases. PMID:23066534

  16. Atypical Antipsychotic Augmentation for Treatment-Resistant Depression: A Systematic Review and Network Meta-Analysis

    PubMed Central

    Zhou, Xinyu; Keitner, Gabor I; Qin, Bin; Ravindran, Arun V; Bauer, Michael; Del Giovane, Cinzia; Zhao, Jingping; Liu, Yiyun; Fang, Yiru; Zhang, Yuqing

    2015-01-01

    Background: Previous meta-analyses of atypical antipsychotics for depression were limited by few trials with direct comparisons between two treatments. We performed a network meta-analysis, which integrates direct and indirect evidence from randomized controlled trials (RCTs), to investigate the comparative efficacy and tolerability of adjunctive atypical antipsychotics for treatment-resistant depression (TRD). Methods: Systematic searches resulted in 18 RCTs (total n = 4422) of seven different types and different dosages of atypical antipsychotics and a placebo that were included in the review. Results: All standard-dose atypical antipsychotics were significantly more efficacious than placebo in the efficacy (standardized mean differences [SMDs] ranged from -0.27 to -0.43). There were no significant differences between these drugs. Low-dose atypical antipsychotics were not significantly more efficacious than the placebo. In terms of tolerability, all standard-dose atypical antipsychotics, apart from risperidone, had significantly more side-effect discontinuations than placebo (odds ratios [ORs] ranged from 2.72 to 6.40). In terms of acceptability, only quetiapine (mean 250–350mg daily) had significantly more all-cause discontinuation than placebo (OR = 1.89). In terms of quality of life/functioning, standard-dose risperidone and standard-dose aripiprazole were more beneficial than placebo (SMD = -0.38; SMD = -0.26, respectively), and standard-dose risperidone was superior to quetiapine (mean 250–350mg daily). Conclusions: All standard-dose atypical antipsychotics for the adjunctive treatment of TRD are efficacious in reducing depressive symptoms. Risperidone and aripiprazole also showed benefits in improving the quality of life of patients. Atypical antipsychotics should be prescribed with caution due to abundant evidence of side effects. PMID:26012350

  17. Atypical femoral fractures bilaterally in a patient receiving bisphosphonate: a case report

    PubMed Central

    Moghnie, Alessandro; Scamacca, Veronica; De Fabrizio, Giovanni; Valentini, Roberto

    2016-01-01

    Summary Atypical femoral fractures are often associated with prolonged bisphosphonate use. The American Society for Bone and Mineral Research (ASBMR) has set the diagnosis criteria for atypical subtrochanteric and diaphyseal femoral fractures by classifying them according to their major and minor criteria. Prolonged bisphosphonate use is correlated with AFF, but the pathogenetic mechanism that causes this kind of fracture has not been defined yet. We describe simultaneous bilaterally femoral fractures in a 76-year-old woman. PMID:27252749

  18. Atypical depression: useful concept, but it's time to revise the DSM-IV criteria.

    PubMed

    Thase, Michael E

    2009-12-01

    Stewart et al (2009) have outlined the evidence in support of the validity of the DSM-IV definition of the 'With Atypical Features' episode specifier. Although recognizing the historical significance and clinical utility of the concept of atypical depression, this article takes issue with the DSM-IV criteria. It is concluded that mood reactivity, the A or obligative criterion, is neither significantly associated with the other symptomatic criteria nor useful to diagnose atypical depression, and thus should be eliminated. Problems with operationalization, specification, and reliability of ratings of the diagnostic criteria further limit validity. Despite these limitations in classification, many of the features associated with atypical depression are linked to an early onset of affective illness, including trait-like interpersonal sensitivity, comorbid social anxiety and agoraphobia, a history of childhood physical or sexual trauma, and indicators of the 'soft' side of the bipolar spectrum. Neurophysiologic studies also suggest that chronic, early-onset atypical depressions differ from both melancholia and normality. Re-analyses of the Columbia group's seminal studies suggest that preferential response to phenelzine vs imipramine--arguably the strongest validator of atypical depression--similarly appears to be limited to patients with chronic, early-onset syndromes. The criteria for atypical depression need to be revised in DSM-V, including sharpening the operational definitions for the specific symptoms. The importance of age of onset and comorbid anxiety warrant further study. Research examining the validity of a subform of atypical depression characterized by trait-like interpersonal sensitivity and a chronic, early-onset course may further enhance the clinical utility of the DSM-V classification. PMID:19741592

  19. Gastrointestinal stromal tumours (GISTs) with a thousand faces: atypical manifestations and causes of misdiagnosis on imaging.

    PubMed

    Kim, S W; Kim, H C; Yang, D M; Won, K Y

    2016-02-01

    Gastrointestinal stromal tumours (GISTs) can lead to emergency situations, such as gastrointestinal bleeding, intestinal obstruction, and tumoural rupture with haemoperitoneum or peritonitis. In addition, if a GIST grows exophytically to a large size, it is often misdiagnosed as a tumour arising from adjacent organs. Sometimes, the atypical appearance of GISTs on imaging causes diagnostic confusion. In this article, we illustrate a variety of GISTs with atypical presentations and also discuss the important diagnostic clues for differentiating GISTs from other lesions. PMID:26646370

  20. Abundances in A-type Horizontal Branch Stars

    NASA Astrophysics Data System (ADS)

    Wilhelm, R.; Smith, V. V.

    1998-12-01

    As part of a program to explore correlations between abundance anomilies and physical parameters (e.g. Teff, vsini) in horizontal branch stars, we present preliminary results from high-resolution (R ~ 18,000) spectral observations of a small sample of A-type, horizontal branch stars. The sample was obtained using the 2.1m telescope and Sandiford Echelle at McDonald Observatory. A total of six standard FHB stars were observed including two, HD 130095 and HD 167105, which have been previously shown by Adleman and Philip to posses anomalously low [Ca/Fe] values. We have also obtained observations of eight of the brighter (B = 11.5-12.5) FHB stars from the HK objective-prism survey and two BHB stars from the globular cluster, M4. We will present abundance results that include [Ca/Fe], [Mg/Fe], and vsini values for the sample along with O and Na results for the two M4 stars. Our findings will be compared to previously published results for cluster BHB and field HB stars.

  1. Pilocytic astrocytoma presenting with atypical features on magnetic resonance imaging.

    PubMed

    Nakano, Yoshiteru; Yamamoto, Junkoh; Takahashi, Mayu; Soejima, Yoshiteru; Akiba, Daisuke; Kitagawa, Takehiro; Ueta, Kunihiro; Miyaoka, Ryo; Umemura, Takeru; Nishizawa, Shigeru

    2015-10-01

    Pilocytic astrocytoma, which is classified as a grade I astrocytic tumor by the World Health Organization, is the most common type of glioma in children and young adults. Pilocytic astrocytoma generally appears as a well-circumscribed, contrast-enhancing lesion, frequently with cystic components on magnetic resonance imaging (MRI). However, it has been reported that the MRI appearance of pilocytic astrocytoma may be similar to that of high-grade gliomas in some cases. We here report on 6 cases of pilocytic astrocytoma with atypical MRI findings, including small cyst formation, heterogeneously enhancing tumor nodules, irregularly enhancing tumor nodules, and enhancing tumor nodules with internal hemorrhage. All tumors were successfully resected, and the histological diagnoses were pilocytic astrocytoma. When the tumor is located near a cerebral cistern or ventricle, the risk of leptomeningeal dissemination is increased. Furthermore, partial resection has also been associated with a higher risk of recurrence and leptomeningeal dissemination. To date, all but one patient are alive and recurrence-free. Because the preoperative diagnosis influences the decision on the extent of resection and because of the high risk of leptomeningeal dissemination associated with these tumors, careful and correct diagnosis by MRI is important. PMID:25454397

  2. TRW A-type polyimide resin for filament winding

    NASA Technical Reports Server (NTRS)

    Vaughan, R. W.; Ueda, K. K.

    1974-01-01

    Studies were conducted to select a TRW A-type polyimide resin that would be suitable for fabrication of filament wound reinforced plastics structures. Several different formulations were evaluated after which the P105AC formulation was selected as the most promising. Procedures were developed for preparing P105AC/S-glass roving prepreg and for fabricating filament wound structural composites. Composites were fabricated and then tested in order to obtain tensile and shear strength information. Small, closed-end cylindrical pressure vessels then were fabricated using a stainless steel liner and end-fittings with a P105AC/S-glass polar-wound overwrap. These pressure vessels were cured in an air circulating oven without augmented pressure. It was concluded upon completion of this study that the P105AC resin system is suitable for filament winding; that low void content, high strength composites are obtained by the filament winding process; and that augmented pressure is not required to effect the fabrication of filament wound P105AC composites.

  3. Recurrent SETBP1 mutations in atypical chronic myeloid leukemia

    PubMed Central

    Piazza, Rocco; Valletta, Simona; Winkelmann, Nils; Redaelli, Sara; Spinelli, Roberta; Pirola, Alessandra; Antolini, Laura; Mologni, Luca; Donadoni, Carla; Papaemmanuil, Elli; Schnittger, Susanne; Kim, Dong-Wook; Boultwood, Jacqueline; Rossi, Fabio; Gaipa, Giuseppe; De Martini, Greta P; di Celle, Paola Francia; Jang, Hyun Gyung; Fantin, Valeria; Bignell, Graham R; Magistroni, Vera; Haferlach, Torsten; Pogliani, Enrico Maria; Campbell, Peter J; Chase, Andrew J; Tapper, William J; Cross, Nicholas C P; Gambacorti-Passerini, Carlo

    2013-01-01

    Atypical chronic myeloid leukemia (aCML) shares clinical and laboratory features with CML, but it lacks the BCR-ABL1 fusion. We performed exome sequencing of eight aCMLs and identified somatic alterations of SETBP1 (encoding a p.Gly870Ser alteration) in two cases. Targeted resequencing of 70 aCMLs, 574 diverse hematological malignancies and 344 cancer cell lines identified SETBP1 mutations in 24 cases, including 17 of 70 aCMLs (24.3%; 95% confidence interval (CI) = 16–35%). Most mutations (92%) were located between codons 858 and 871 and were identical to changes seen in individuals with Schinzel-Giedion syndrome. Individuals with mutations had higher white blood cell counts (P = 0.008) and worse prognosis (P = 0.01). The p.Gly870Ser alteration abrogated a site for ubiquitination, and cells exogenously expressing this mutant exhibited higher amounts of SETBP1 and SET protein, lower PP2A activity and higher proliferation rates relative to those expressing the wild-type protein. In summary, mutated SETBP1 represents a newly discovered oncogene present in aCML and closely related diseases. PMID:23222956

  4. Rest Mutant zebrafish swim erratically and display atypical spatial preferences

    PubMed Central

    Moravec, Cara E.; Li, Edward; Maaswinkel, Hans; Kritzer, Mary F.; Weng, Wei; Sirotkin, Howard I.

    2015-01-01

    The Rest/Nrsf transcriptional repressor modulates expression of a large set of neural specific genes. Many of these target genes have well characterized roles in nervous system processes including development, plasticity and synaptogenesis. However, the impact of Rest-mediated transcriptional regulation on behavior has been understudied due in part to the embryonic lethality of the mouse knockout. To investigate the requirement for Rest in behavior, we employed the zebrafish rest mutant to explore a range of behaviors in adults and larva. Adult rest mutants of both sexes showed abnormal behaviors in a novel environment including increased vertical swimming, erratic swimming patterns and a proclivity for the tank walls. Adult males also had diminished reproductive success. At 6 days post fertilization (dpf), rest mutant larva were hypoactive, but displayed normal evoked responses to light and sound stimuli. Overall, these results provide evidence that rest dysfunction produces atypical swimming patterns and preferences in adults, and reduced locomotor activity in larvae. This study provides the first behavioral analysis of rest mutants and reveals specific behaviors that are modulated by Rest. PMID:25712696

  5. New functional aspects of the atypical protein tyrosine phosphatase VHZ

    PubMed Central

    Kuznetsov, Vyacheslav I.; Hengge, Alvan C.

    2013-01-01

    LDP3 (VHZ) is the smallest classical protein tyrosine phosphatase (PTP) known to date, and was originally misclassified as an atypical dual specificity phosphatase (DSP). Kinetic isotope effects with steady state and pre-steady state kinetics of VHZ and mutants with para-nitrophenol phosphate (pNPP) have revealed several unusual properties. VHZ is significantly more active than previously reported, but remains one of the least active PTPs. Highly unusual for a PTP, VHZ possesses two acidic residues (E134 and D65) in the active site. D65 occupies the position corresponding to the typical general acid in the PTP family. However, VHZ primarily utilizes E134 as the general acid, with D65 taking over this role when E134 is mutated. This unusual behavior is facilitated by two coexisting, but unequally populated, substrate binding modes. Unlike most classical PTPs, VHZ exhibits phosphotransferase activity. Despite the presence of the Q-loop that normally prevents alcoholysis of the phosphoenzyme intermediate in other classical PTPs, VHZ readily phosphorylates ethylene glycol. Although mutations to Q-loop residues affect this phosphotransferase activity, mutations on the IPD-loop that contains the general acid exert more control over this process. A single P68V substitution on this loop completely abolishes phosphotransferase activity. The ability of native VHZ to catalyze transphosphorylation may lead to an imbalance of intracellular phosphorylation, which could explain the correlation of its overexpression with several types of cancer. PMID:24073992

  6. Physiological and molecular characterization of atypical isolates of Malassezia furfur.

    PubMed

    González, A; Sierra, R; Cárdenas, M E; Grajales, A; Restrepo, S; Cepero de García, M C; Celis, A

    2009-01-01

    The species constituting the genus Malassezia are considered to be emergent opportunistic yeasts of great importance. Characterized as lipophilic yeasts, they are found in normal human skin flora and sometimes are associated with different dermatological pathologies. We have isolated seven Malassezia species strains that have a different Tween assimilation pattern from the one typically used to differentiate M. furfur, M. sympodialis, and M. slooffiae from other Malassezia species. In order to characterize these isolates of Malassezia spp., we studied their physiological features and conducted morphological and molecular characterization by PCR-restriction fragment length polymorphism and sequencing of the 26S and 5.8S ribosomal DNA-internal transcribed spacer 2 regions in three strains from healthy individuals, four clinical strains, and eight reference strains. The sequence analysis of the ribosomal region was based on the Blastn algorithm and revealed that the sequences of our isolates were homologous to M. furfur sequences. To support these findings, we carried out phylogenetic analyses to establish the relationship of the isolates to M. furfur and other reported species. All of our results confirm that all seven strains are M. furfur; the atypical assimilation of Tween 80 was found to be a new physiological pattern characteristic of some strains isolated in Colombia. PMID:18971363

  7. Quantitative methods for somatosensory evaluation in atypical odontalgia.

    PubMed

    Porporatti, André Luís; Costa, Yuri Martins; Stuginski-Barbosa, Juliana; Bonjardim, Leonardo Rigoldi; Conti, Paulo César Rodrigues; Svensson, Peter

    2015-01-01

    A systematic review was conducted to identify reliable somatosensory evaluation methods for atypical odontalgia (AO) patients. The computerized search included the main databases (MEDLINE, EMBASE, and Cochrane Library). The studies included used the following quantitative sensory testing (QST) methods: mechanical detection threshold (MDT), mechanical pain threshold (MPT) (pinprick), pressure pain threshold (PPT), dynamic mechanical allodynia with a cotton swab (DMA1) or a brush (DMA2), warm detection threshold (WDT), cold detection threshold (CDT), heat pain threshold (HPT), cold pain detection (CPT), and/or wind-up ratio (WUR). The publications meeting the inclusion criteria revealed that only mechanical allodynia tests (DMA1, DMA2, and WUR) were significantly higher and pain threshold tests to heat stimulation (HPT) were significantly lower in the affected side, compared with the contralateral side, in AO patients; however, for MDT, MPT, PPT, CDT, and WDT, the results were not significant. These data support the presence of central sensitization features, such as allodynia and temporal summation. In contrast, considerable inconsistencies between studies were found when AO patients were compared with healthy subjects. In clinical settings, the most reliable evaluation method for AO in patients with persistent idiopathic facial pain would be intraindividual assessments using HPT or mechanical allodynia tests. PMID:25627886

  8. Atypical odontalgia: an up-to-date view.

    PubMed

    Tarce, M; Barbieri, C; Sardella, A

    2013-05-01

    Atypical odontalgia (AO) is a little known chronic pain condition. It usually presents as pain in a site where a tooth was endodontically treated or extracted, in the absence of clinical or radiographic evidence of tooth pathology. It is a rare clinical challenge for most clinicians, which leads to the patients being referred to several specialists and sometimes undergoing unnecessary surgical procedures. The pain mechanisms involved in AO are far from clear, and numerous potential mechanisms have been suggested. Currently, the most accredited hypothesis is that AO is a neuropathic pain condition caused by deafferentation. The differential diagnosis of AO remains difficult, because it shares symptoms with many others pathologies affecting this area. Patients have difficulties accepting the AO diagnosis and treatment. As a result, they frequently change physicians, and may potentially also receive several invasive treatments, usually resulting in an aggravation of the pain. Although some patients do get complete pain relief following treatment, for most patients the goal should be to achieve adequate pain management. Currently, most management is based on expert opinion and case reports. More research and high quality randomized controlled trials are needed in order to develop evidence-based treatments, currently based on expert opinion or carried over from other neuropathic pain conditions in the orofacial region. PMID:23715202

  9. Current evidence on atypical odontalgia: diagnosis and clinical management.

    PubMed

    Abiko, Yoshihiro; Matsuoka, Hirofumi; Chiba, Itsuo; Toyofuku, Akira

    2012-01-01

    Patients with atypical odontalgia (AO) complain of medically unexplained toothache. No evidence-based diagnostic criteria or treatment guidelines are yet available. The present paper addresses seven clinical questions about AO based on current knowledge in the literature and discusses diagnostic criteria and guidelines for treatment and management. The questions are (i) What is the prevalence of AO in the community? (ii) What psychological problems are experienced by patients with AO? (iii) Are there any comorbidities of AO? (iv) Is local anesthesia effective for the relief of pain in AO? (v) Are there any characteristic symptoms of AO other than spontaneous pain? (vi) Are antidepressants effective for treatment of AO? (vii) Are anticonvulsants effective for treatment of AO? Our literature search provided answers for these questions; however, there is insufficient evidence-based data to establish guidelines for the diagnosis and treatment of AO. Overall, some diagnostic criteria for neuropathic pain and persistent dentoalveolar pain disorder may be applied to AO patients. The patient's psychogenic background should always be considered in the treatment and/or management of AO. The clinicians may need to treat AO patients using Patient-Oriented Evidence that Matters approach. PMID:22844283

  10. Age-related penetrance of hereditary atypical hemolytic uremic syndrome.

    PubMed

    Sullivan, Maren; Rybicki, Lisa A; Winter, Aurelia; Hoffmann, Michael M; Reiermann, Stefanie; Linke, Hannah; Arbeiter, Klaus; Patzer, Ludwig; Budde, Klemens; Hoppe, Bernd; Zeier, Martin; Lhotta, Karl; Bock, Andreas; Wiech, Thorsten; Gaspert, Ariana; Fehr, Thomas; Woznowski, Magdalena; Berisha, Gani; Malinoc, Angelica; Goek, Oemer-Necmi; Eng, Charis; Neumann, Hartmut P H

    2011-11-01

    Hereditary atypical hemolytic uremic syndrome (aHUS), a dramatic disease frequently leading to dialysis, is associated with germline mutations of the CFH, CD46, or CFI genes. After identification of the mutation in an affected aHUS patient, single-site gene testing of relatives is the preventive care perspective. However, clinical data for family counselling are scarce. From the German-Speaking-Countries-aHUS-Registry, 33 index patients with mutations were approached for permission to offer relatives screening for their family-specific mutations and to obtain demographic and clinical data. Mutation screening was performed using direct sequencing. Age-adjusted penetrance of aHUS was calculated for each gene in index cases and in mutation-positive relatives. Sixty-one relatives comprising 41 parents and 20 other relatives were enrolled and mutations detected in 31/61. In total, 40 research participants had germline mutations in CFH, 19 in CD46 and in 6 CFI. Penetrance at age 40 was markedly reduced in mutation-positive relatives compared to index patients overall with 10% versus 67% (P < 0.001); 6% vs. 67% (P < 0.001) in CFH mutation carriers and 21% vs. 70% (P= 0.003) in CD46 mutation carriers. Age-adjusted penetrance for hereditary aHUS is important to understand the disease, and if replicated in the future, for genetic counselling. PMID:21906045

  11. Discovering Atypical Flights in Sequences of Discrete Flight Parameters

    NASA Technical Reports Server (NTRS)

    Budalakoti, Suratna; Srivastava, Ashok N.; Akella, Ram

    2006-01-01

    This paper describes the results of a novel research and development effort conducted at the NASA Ames Research Center for discovering anomalies in discrete parameter sequences recorded from flight data. Many of the discrete parameters that are recorded during the flight of a commercial airliner correspond to binary switches inside the cockpit. The inputs to our system are records from thousands of flights for a given class of aircraft and destination. The system delivers a list of potentially anomalous flights as well as reasons why the flight was tagged as anomalous. This output can be analyzed by safety experts to determine whether or not the anomalies are indicative of a problem that could be addressed with a human factors intervention. The final goal of the system is to help safety experts discover significant human factors issues such as pilot mode confusion, i.e., a flight in which a pilot has lost situational awareness as reflected in atypicality of the sequence of switches that he or she throws during descent compared to a population of similar flights. We view this work as an extension of Integrated System Health Management (ISHM) where the goal is to understand and evaluate the combined health of a class of aircraft ar a given destination.

  12. RNA-Binding Proteins in Trichomonas vaginalis: Atypical Multifunctional Proteins.

    PubMed

    Figueroa-Angulo, Elisa E; Calla-Choque, Jaeson S; Mancilla-Olea, Maria Inocente; Arroyo, Rossana

    2015-01-01

    Iron homeostasis is highly regulated in vertebrates through a regulatory system mediated by RNA-protein interactions between the iron regulatory proteins (IRPs) that interact with an iron responsive element (IRE) located in certain mRNAs, dubbed the IRE-IRP regulatory system. Trichomonas vaginalis, the causal agent of trichomoniasis, presents high iron dependency to regulate its growth, metabolism, and virulence properties. Although T. vaginalis lacks IRPs or proteins with aconitase activity, possesses gene expression mechanisms of iron regulation at the transcriptional and posttranscriptional levels. However, only one gene with iron regulation at the transcriptional level has been described. Recently, our research group described an iron posttranscriptional regulatory mechanism in the T. vaginalis tvcp4 and tvcp12 cysteine proteinase mRNAs. The tvcp4 and tvcp12 mRNAs have a stem-loop structure in the 5'-coding region or in the 3'-UTR, respectively that interacts with T. vaginalis multifunctional proteins HSP70, α-Actinin, and Actin under iron starvation condition, causing translation inhibition or mRNA stabilization similar to the previously characterized IRE-IRP system in eukaryotes. Herein, we summarize recent progress and shed some light on atypical RNA-binding proteins that may participate in the iron posttranscriptional regulation in T. vaginalis. PMID:26703754

  13. The atypical homeodomain transcription factor Mohawk controls tendon morphogenesis.

    PubMed

    Liu, Wenjin; Watson, Spencer S; Lan, Yu; Keene, Douglas R; Ovitt, Catherine E; Liu, Han; Schweitzer, Ronen; Jiang, Rulang

    2010-10-01

    The Mohawk homeobox (Mkx) gene encodes a new atypical homeodomain-containing protein with transcriptional repressor activity. Mkx mRNA exhibited dynamic expression patterns during development of the palate, somite, kidney, and testis, suggesting that it may be an important regulator of multiple developmental processes. To investigate the roles of Mkx in organogenesis, we generated mice carrying a null mutation in this gene. Mkx(-/-) mice survive postnatally and exhibit a unique wavy-tail phenotype. Close examination revealed that the mutant mice had smaller tendons than wild-type littermates and that the rapid postnatal growth of collagen fibrils in tendons was disrupted in Mkx(-/-) mice. Defects in tendon development were detected in the mutant mouse embryos as early as embryonic day 16.5 (E16.5). Although collagen fibril assembly initially appeared normal, the tendons of Mkx(-/-) embryos expressed significantly reduced amounts of collagen I, fibromodulin, and tenomodulin in comparison with control littermates. We found that Mkx mRNA was strongly expressed in differentiating tendon cells during embryogenesis and in the tendon sheath cells in postnatal stages. In addition to defects in tendon collagen fibrillogenesis, Mkx(-/-) mutant mice exhibited abnormal tendon sheaths. These results identify Mkx as an important regulator of tendon development. PMID:20696843

  14. Septic arthritis of the pubic symphysis: an atypical abdominal pain.

    PubMed

    Ghislain, L; Heylen, A; Alexis, F; Tintillier, M

    2015-02-01

    Septic arthritis of the pubic symphysis is a rare infection mostly caused by Staphylococcus aureus, and is traditionally associated with risk factors (sports, female incontinence surgery). Typical features of pubic symphysis infection include abdominal, pelvic, or groin pain that increases upon standing and walking, causing limping to occur. Acute onset of fever is often associated. It is important to distinguish septic arthritis of the pubic symphysis from its aseptic homologue, improperly called 'osteitis pubis' in English literature. This general term is mostly used to designate a mechanical pubic pain and has several aetiological meanings (joint stress, postoperative pain, rheumatic diseases). However, some authors consider the infection of the pubic symphysis as a variant of osteitis pubis, placing the two diseases in the continuum of the same entity. This confusion in pubic pathology related to its rarity and its atypical presentation, may in some cases lead to diagnostic and therapeutic delay. In this article, we would like to make practitioners aware of this uncommon and often ignored anatomical site, so that it can recover its place in the differential diagnosis of abdominal pain. PMID:25227947

  15. Atypical fibroxanthoma: a histological and immunohistochemical review of 171 cases.

    PubMed

    Beer, Trevor W; Drury, Paul; Heenan, Peter J

    2010-08-01

    The clinical and histological features of 171 atypical fibroxanthomas (AFX) from a single institution in Western Australia are outlined. This area experiences high levels of solar radiation, and all assessable biopsies showed solar elastosis. Patients were aged between 41 and 97 years (median age 74), with 76% of tumors occurring in men (male to female ratio approximately 3 to 1). Most tumors were small, with a median diameter of 10 mm and a range of 4-35 mm. Only 5% exceeded 20 mm in diameter. Most AFX were well-circumscribed dermal lesions, with limited invasion of subcutis in a minority. Histological variants identified included keloidal (n = 8), clear cell (n = 3), and granular cell (n = 3), plaque like (n = 4), and myxoid (n = 1). Bland cytological appearances (spindle cell nonpleomorphic AFX) were noted in 5 tumors, with osteoclast-like giant cells in 2. Features suggesting regression were present in 22 cases. Two cases recurred locally, none metastasized. No tumors expressed melanocytic or epithelial markers. Seventy-four percent of cases expressed smooth muscle actin, typically strongly and diffusely. No AFX stained with desmin. Only 1 of 50 cases was CD117 positive. In conclusion, AFX may show a wide range of histological appearances, and a panel of immunohistochemical markers is essential to make the correct diagnosis. Histological mimics, such as poorly differentiated squamous cell carcinoma, must be carefully excluded. Specific diagnosis is important because there seems to be a very low risk of recurrence or metastasis despite the frequently alarming histology. PMID:20526171

  16. [Atypical HUS caused by complement-related abnormalities].

    PubMed

    Yoshida, Yoko; Matsumoto, Masanori

    2015-02-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The term aHUS was historically used to distinguish this disorder from Shiga-toxin producing Escherichia coli (STEC)-HUS. Many aHUS cases (approximately 70%) are reportedly caused by uncontrolled complement activation due to genetic mutations in the alternative pathway, including complement factor H (CFH), complement factor I (CFI), membrane cofactor protein (MCP), thrombomodulin (THBD), complement component C3 (C3), and complement factor B (CFB). Mutations in the coagulation pathway, such as diacylglycerol kinase ε (DGKE) and plasminogen, are also reported to be causes of aHUS. In this review, we have focused on aHUS due to complement dysfunction. aHUS is suspected based on plasma ADAMTS13 activity of 10% or more, and being negative for STEC-HUS, in addition to the aforementioned triad. Complement genetic studies provide a more specific diagnosis of aHUS. Plasma therapy is the first-line treatment for patients with aHUS and should be initiated as soon as the diagnosis is suspected. Recently, eculizumab, a humanized monoclonal antibody against C5, was shown to be an effective treatment for aHUS. Therefore, early diagnosis and identification of the underlying pathogenic mechanism is important for improving the outcome of aHUS. PMID:25765799

  17. An Atypical Riboflavin Pathway Is Essential for Brucella abortus Virulence

    PubMed Central

    Klinke, Sebastián; Ugalde, Juan E.; Zylberman, Vanesa; Ugalde, Rodolfo A.; Comerci, Diego J.; Goldbaum, Fernando Alberto

    2010-01-01

    Brucellosis is a worldwide zoonosis that affects livestock and humans and is caused by closely related Brucella spp., which are adapted to intracellular life within cells of a large variety of mammals. Brucella can be considered a furtive pathogen that infects professional and non-professional phagocytes. In these cells Brucella survives in a replicative niche, which is characterized for having a very low oxygen tension and being deprived from nutrients such as amino acids and vitamins. Among these vitamins, we have focused on riboflavin (vitamin B2). Flavin metabolism has been barely implicated in bacterial virulence. We have recently described that Brucella and other Rhizobiales bear an atypical riboflavin metabolic pathway. In the present work we analyze the role of the flavin metabolism on Brucella virulence. Mutants on the two lumazine synthases (LS) isoenzymes RibH1 and RibH2 and a double RibH mutant were generated. These mutants and different complemented strains were tested for viability and virulence in cells and in mice. In this fashion we have established that at least one LS must be present for B. abortus survival and that RibH2 and not RibH1 is essential for intracellular survival due to its LS activity in vivo. In summary, we show that riboflavin biosynthesis is essential for Brucella survival inside cells or in mice. These results highlight the potential use of flavin biosynthetic pathway enzymes as targets for the chemotherapy of brucellosis. PMID:20195542

  18. Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome

    PubMed Central

    Lemaire, Mathieu; Frémeaux-Bacchi, Véronique; Schaefer, Franz; Choi, Murim; Tang, Wai Ho; Le Quintrec, Moglie; Fakhouri, Fadi; Taque, Sophie; Nobili, François; Martinez, Frank; Ji, Weizhen; Overton, John D.; Mane, Shrikant M.; Nürnberg, Gudrun; Altmüller, Janine; Thiele, Holger; Morin, Denis; Deschenes, Georges; Baudouin, Véronique; Llanas, Brigitte; Collard, Laure; Majid, Mohammed A.; Simkova, Eva; Nürnberg, Peter; Rioux-Leclerc, Nathalie; Moeckel, Gilbert W.; Gubler, Marie Claire; Hwa, John; Loirat, Chantal; Lifton, Richard P.

    2013-01-01

    Pathologic thrombosis is a major cause of mortality. Hemolytic-uremic syndrome (HUS) features episodes of small vessel thrombosis resulting in microangiopathic hemolytic anemia, thrombocytopenia and renal failure1. Atypical HUS (aHUS) can result from genetic or autoimmune factors2 that lead to pathologic complement cascade activation3. By exome sequencing we identify recessive mutations in DGKE (diacylglycerol kinase epsilon) that co-segregate with aHUS in 9 unrelated kindreds, defining a distinctive Mendelian disease. Affected patients present with aHUS before age 1, have persistent hypertension, hematuria and proteinuria (sometimes nephrotic range), and develop chronic kidney disease with age. DGKE is found in endothelium, platelets, and podocytes. Arachidonic acid-containing diacylglycerols (DAG) activate protein kinase C, which promotes thrombosis. DGKE normally inactivates DAG signaling. We infer that loss of DGKE function results in a pro-thrombotic state. These findings identify a new mechanism of pathologic thrombosis and kidney failure and have immediate implications for treatment of aHUS patients. PMID:23542698

  19. Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome

    PubMed Central

    Feng, Shuju; Eyler, Stephen J.; Zhang, Yuzhou; Maga, Tara; Nester, Carla M.; Kroll, Michael H.

    2013-01-01

    Complement dysregulation leads to atypical hemolytic uremic syndrome (aHUS), while ADAMTS13 deficiency causes thrombotic thrombocytopenic purpura. We investigated whether genetic variations in the ADAMTS13 gene partially explain the reduced activity known to occur in some patients with aHUS. We measured complement activity and ADAMTS13 function, and completed mutation screening of multiple complement genes and ADAMTS13 in a large cohort of aHUS patients. In over 50% of patients we identified complement gene mutations. Surprisingly, 80% of patients also carried at least 1 nonsynonymous change in ADAMTS13, and in 38% of patients, multiple ADAMTS13 variations were found. Six of the 9 amino acid substitutions in ADAMTS13 were common single nucleotide polymorphisms; however, 3 variants—A747V, V832M, and R1096H— were rare, with minor allele frequencies of 0.0094%, 0.5%, and 0.32%, respectively. Reduced complement and ADAMTS13 activity (<60% of normal activity) were found in over 60% and 50% of patients, respectively. We concluded that partial ADAMTS13 deficiency is a common finding in aHUS patients and that genetic screening and functional tests of ADAMTS13 should be considered in these patients. PMID:23847193

  20. EVOLUTION OF ROTATIONAL VELOCITIES OF A-TYPE STARS

    SciTech Connect

    Yang Wuming; Bi Shaolan; Tian Zhijia; Meng Xiangcun E-mail: yangwuming@bnu.edu.cn

    2013-03-10

    The equatorial velocity of A-type stars undergoes an acceleration in the first third of the main sequence (MS) stage, but the velocity decreases as if the stars were not undergoing any redistribution of angular momentum in the external layers in the last stage of the MS phase. Our calculations show that the acceleration and the decrease of the equatorial velocity can be reproduced by the evolution of the differential rotation zero-age MS model with the angular momentum transport caused by hydrodynamic instabilities during the MS stage. The acceleration results from the fact that the angular momentum stored in the interiors of the stars is transported outward. In the last stage, the core and the radiative envelope are uncoupling, and the rotation of the envelope is a quasi-solid rotation; the uncoupling and the expansion of the envelope indicate that the decrease of the equatorial velocity approximately follows the slope for the change in the equatorial velocity of the model without any redistribution of angular momentum. When the fractional age 0.3 {approx}< t/t{sub MS} {approx}< 0.5, the equatorial velocity remains almost constant for stars whose central density increases with age in the early stage of the MS phase, while the velocity decreases with age for stars whose central density decreases with age in the early stage of the MS phase.

  1. Physella acuta: atypical mitochondrial gene order among panpulmonates (Gastropoda)

    PubMed Central

    Nolan, Journey R.; Bergthorsson, Ulfar; Adema, Coen M.

    2014-01-01

    Mitochondrial (mt) sequences are frequently used for phylogenetic reconstruction and for identification of species of molluscs. This study expands the phylogenetic range of Hygrophila (Panpulmonata) for which such sequence data are available by characterizing the full mt genome of the invasive freshwater snail Physella acuta (Physidae). The mt genome sequences of two P. acuta isolates from Stubblefield Lake, New Mexico, USA, differed in length (14,490 vs 14,314 bp) and showed 11.49% sequence divergence, whereas ITS1 and ITS2 sequences from the nuclear genome differed by 1.75%. The mt gene order of P. acuta (cox1, P, nad6, nad5, nad1, D, F, cox2, Y, W, nad4L, C, Q, atp6, R, E, rrnS, M, T, cox3, I, nad2, K, V, rrnL, L1, A, cytb, G, H, L2, atp8, N, nad2, S1, S2, nad4) differs considerably from the relatively conserved gene order within Panpulmonata. Phylogenetic trees show that the 13 protein-encoding mt gene sequences (equivalent codons) of P. acuta group according to gastropod phylogeny, yet branch lengths and dN/dS ratios for P. acuta indicate elevated amino acid substitutions relative to other gastropods. This study indicates that mt sequences of P. acuta are phylogenetically informative despite a considerable intraspecific divergence and the atypical gene order in its mt genome. PMID:25368439

  2. Causal Learning Across Domains

    ERIC Educational Resources Information Center

    Schulz, Laura E.; Gopnik, Alison

    2004-01-01

    Five studies investigated (a) children's ability to use the dependent and independent probabilities of events to make causal inferences and (b) the interaction between such inferences and domain-specific knowledge. In Experiment 1, preschoolers used patterns of dependence and independence to make accurate causal inferences in the domains of…

  3. Modeling Protein Domain Function

    ERIC Educational Resources Information Center

    Baker, William P.; Jones, Carleton "Buck"; Hull, Elizabeth

    2007-01-01

    This simple but effective laboratory exercise helps students understand the concept of protein domain function. They use foam beads, Styrofoam craft balls, and pipe cleaners to explore how domains within protein active sites interact to form a functional protein. The activity allows students to gain content mastery and an understanding of the…

  4. Domain wall filters

    SciTech Connect

    Baer, Oliver; Narayanan, Rajamani; Neuberger, Herbert; Witzel, Oliver

    2007-03-15

    We propose using the extra dimension separating the domain walls carrying lattice quarks of opposite handedness to gradually filter out the ultraviolet fluctuations of the gauge fields that are felt by the fermionic excitations living in the bulk. This generalization of the homogeneous domain wall construction has some theoretical features that seem nontrivial.

  5. U, Ra and Ba incorporation during precipitation of hydrothermal carbonates: Implications for {sup 226}Ra-Ba dating of impure travertines

    SciTech Connect

    Rihs, S.; Condomines, M.; Sigmarsson, O.

    2000-02-01

    The authors studied U, Ra and Ba incorporation in calcite in a natural CO{sub 2}-rich hydrothermal area from the French Massif Central. Along the western border of the Limagne graben, several springs are exploited for the petrification of various artifacts with calcite. These sites offer the opportunity to sample the water and the calcite layers downflow from the spring, and thus to follow the evolution of their U, Ra and Ba contents as precipitation proceeds. Results show that the apparent partition coefficients of U, Ra and Ba between water and calcite decrease during precipitation for the three elements. The authors found no direct relation between this variation and the main factors able to influence the partition coefficient, such as precipitation rate, which suggests that the incorporation of these trace elements could result from a composite process of adsorption and coprecipitation. Ra and Ba have a similar behavior, with an apparent partition coefficient decreasing from 0.80 to 0.47 for Ra and 0.96 to 0.68 for Ba, resulting in a small ({le}10%) variation of the Ra/Ba ratio. The apparent partition coefficient of U decreases from 0.38 to 0.20. These apparent coefficients are much higher than equilibrium values but might be applicable to natural systems with high precipitation rates. The authors also investigated the possibility of using the decay of the {sup 226}Ra-excess, or the decrease of the ({sup 226}Ra)/Ba ratio to date older deposits. Whereas the {sup 226}Ra initial activity at the time of deposition has not remained constant, and cannot be used for dating, the ({sup 226}Ra)/Ba method gives better results, when appropriate corrections for detrital contamination in Ba are made. Mixing diagrams using Th as an indicator of contamination allow calculation of the ({sup 226}Ra)/Ba ratio of the pure carbonate component. The calculated ages of five travertine layers range from 330 to 800 years, suggesting a mean deposition rate of about 1 cm/yr. The

  6. Are atypical lymphocytes present with viral influenza-like illnesses (ILIs) in hospitalized adults?

    PubMed

    Cunha, B A; Connolly, J J; Irshad, N

    2016-09-01

    The purpose of this investigation was to determine if atypical lymphocytes were of diagnostic value in viral influenza-like illnesses (ILIs) in hospitalized adults during the influenza season. Are atypical lymphocytes present with viral ILIs in hospitalized adults? During the influenza season, hospitals are inundated with influenza and viral ILIs, e.g., human parainfluenza virus-3 (HPIV-3). Without specific testing, clinically, it is difficult to differentiate influenza from ILIs, and surrogate influenza markers have been used for this purpose, e.g., relative lymphopenia. The diagnostic significance of atypical lymphocytes with ILIs is not known. We retrospectively reviewed the charts of 35 adults admitted with pneumonia due to viral ILI. The diagnosis of 14 patients was by respiratory virus polymerase chain reaction (PCR). During the 2015 influenza A season with ILIs, atypical lymphocytes were not present in influenza A (H3N2) patients but atypical lymphocytes were present in some ILIs, particularly HPIV-3. With viral ILIs, atypical lymphocytes should suggest a non-influenza viral diagnosis. PMID:27250631

  7. Distribution of (223)Ra and (224)Ra in the Bo Sea embayment in Tianjin and its implication of submarine groundwater discharge.

    PubMed

    Liu, Lingling; Yi, Lixin; Cheng, Xiaoqing; Tang, Guoqiang

    2015-12-01

    Submarine groundwater discharge (SGD) is now recognized as an important pathway between land and sea. In this study, in order to analyze the distribution of naturally occurring short-lived radium isotopes and the relative SGD effect in Bo Sea embayment, (223)Ra and (224)Ra were measured in three parts of the embayment with the radium-delayed coincidence counting (RaDeCC) system. Subsequently, the mixing process was studied by the calculation of diffusion coefficients (Kx and Kz) and advection velocities (Vx and Vz) based on the 2D advection-diffusion model. Additionally, the apparent residence ages and SGD flux were quantified based on the (224)Ra and (223)Ra activities. The results showed that the Ra activities exponentially decreased with the distance offshore, and both the Kx and Vx took the order of northern part > southern part > middle part. In vertical direction, there was the maximum value of Vz and minimum Kz in middle part and the maximum Kz and minimum Vz in southern part. The average ages for the northern, middle and southern parts were 4.28, 7.38 and 3.73 days, respectively. The final SGD flux yielded by (224)Ra was 0.09, 0.01 and 0.03 m d(-1) in the northern, middle and southern parts, respectively. The SGD flux yielded by (223)Ra was 0.08, 0.01 and 0.03 m d(-1) in northern, middle and southern parts, respectively. The result indicates that there is the fastest exchange rate and the biggest SGD flux in the southern part in Bo Sea embayment. PMID:26318773

  8. Atypical Mole (Atypical Nevus)

    MedlinePlus

    ... 2006-2013 Logical Images, Inc. All rights reserved. Advertising Notice This Site and third parties who place ... would like to obtain more information about these advertising practices and to make choices about online behavioral ...

  9. Tracking suspended particle transport via radium isotopes ((226)Ra and (228)Ra) through the Apalachicola-Chattahoochee-Flint River system.

    PubMed

    Peterson, Richard N; Burnett, William C; Opsahl, Stephen P; Santos, Isaac R; Misra, Sambuddha; Froelich, Philip N

    2013-02-01

    Suspended particles in rivers can carry metals, nutrients, and pollutants downstream which can become bioactive in estuaries and coastal marine waters. In river systems with multiple sources of both suspended particles and contamination sources, it is important to assess the hydrologic conditions under which contaminated particles can be delivered to downstream ecosystems. The Apalachicola-Chattahoochee-Flint (ACF) River system in the southeastern United States represents an ideal system to study these hydrologic impacts on particle transport through a heavily-impacted river (the Chattahoochee River) and one much less impacted by anthropogenic activities (the Flint River). We demonstrate here the utility of natural radioisotopes as tracers of suspended particles through the ACF system, where particles contaminated with arsenic (As) and antimony (Sb) have been shown to be contributed from coal-fired power plants along the Chattahoochee River, and have elevated concentrations in the surficial sediments of the Apalachicola Bay Delta. Radium isotopes ((228)Ra and (226)Ra) on suspended particles should vary throughout the different geologic provinces of this river system, allowing differentiation of the relative contributions of the Chattahoochee and Flint Rivers to the suspended load delivered to Lake Seminole, the Apalachicola River, and ultimately to Apalachicola Bay. We also use various geochemical proxies ((40)K, organic carbon, and calcium) to assess the relative composition of suspended particles (lithogenic, organic, and carbonate fractions, respectively) under a range of hydrologic conditions. During low (base) flow conditions, the Flint River contributed 70% of the suspended particle load to both the Apalachicola River and the bay, whereas the Chattahoochee River became the dominant source during higher discharge, contributing 80% of the suspended load to the Apalachicola River and 62% of the particles entering the estuary. Neither of these hydrologic

  10. Large 224Ra and 228Ra disequilibrium indicates intense groundwater processes in the Cabo Frio coastal system

    NASA Astrophysics Data System (ADS)

    Souza, Giovana; Silva-Filho, Emmanoel; Sanders, Christian; Marques, Eduardo; Caldeira, Pedro; Santos, Luiz Eduardo; Kutter, Vinicius; Smoak, Joseph

    2014-05-01

    Input of subterranean water, referred to as Submarine Groundwater Discharge (SGD), typically is a mixture of infiltrated seawater and terrestrially derived freshwater and includes all advective fluid flow between the land and the continental shelf, regardless of the source or composition of the fluids (Smoak et al., 2013; Burnett et al., 2003). A substantial flow of nutrient and metal input via SGD, which may contribute to coastal eutrophication, has been document in recent studies (Sanders et al., 2012; Santos et al., 2008). Direct measurements (e.g. seepage meters) of SGD are difficult on a regional scale. However, radium isotopes have been shown to be powerful tools to quantify SGD fluxes and indicate the sources (Sanders et al., 2012). The direct parent of each radium isotope is a thorium isotope. Thorium is strongly adsorbed to sediment and provides a source of radium, which is generated on a range of time scales. In freshwater, radium is strongly adsorbed to particles. However, under reducing conditions, low pH and/or increasing salinity, radium can be released into solution. These characteristics make radium an excellent tracer of brackish SGD and a net flux of nutrients and metals to the ocean. The coastal plain in the region of Cabo Frio and its surroundings consist of Quaternary deposits of continental sediments, coastal lagoons and marine sands forming a large coastal aquifer. During the winter of 2012 and summer of 2013 was performed a physicochemical characterization and measurements of radio isotopes in groundwater along a transect perpendicular to the beach. The high radio activities associated with the imbalance exists between the 224Ra and 228Ra can be useful tools in understanding the role of submarine groundwater discharge in this region highly productive and high marine biodiversity.

  11. Measurement of (238)U, (228)Ra, (226)Ra, (40)K and (137)Cs in foodstuffs samples collected from coastal areas of China.

    PubMed

    Tuo, Fei; Zhang, Qing; Zhou, Qiang; Xu, Cuihua; Zhang, Jing; Li, Wenhong; Zhang, Jianfeng; Su, Xu

    2016-05-01

    This study represents a total of 245 samples collected. The activities of (238)U, (228)Ra, (226)Ra, (40)K and (137)Cs were determined in samples of vegetables, tea, cereal (rice, wheat and corn), meat, poultry, freshwater product, seafood and seaweed that collected from the 30km safety zone of the Nuclear Power Plants (NPPs) area. All the samples radionuclide activities were quantified by using High Purity Germanium (HPGe) gamma spectrometry. The geometric mean concentrations (Bqkg(-1) wet weight) for (238)U, (228)Ra, (226)Ra, (40)K, and (137)Cs in all investigated foodstuffs samples, are 0.13, 0.16, 0.11, 68 and 0.02, respectively. The arithmetic mean concentrations (Bqkg(-1) wet weight) for (238)U, (228)Ra, (226)Ra, (40)K, and (137)Cs in all investigated foodstuffs samples, are 0.34, 0.65, 0.32, 111 and 0.09, respectively. Results of this study were compared with others, the measured values are the same with those of a previous investigation. Radiation doses due to the consumption of these foodstuffs to humans are estimated to comprise around 37-46% of the annual dose limit for public. PMID:26926376

  12. A correlation between soil descriptions and {sup 226}Ra concentrations in Florida soils

    SciTech Connect

    Harrison, D.P.

    1992-12-31

    The soil radium content in Florida is highly variable. The range in radium concentrations, where the samples involved in this study are concerned, is from 0.1 pCi/g to 18.5 pCi/g. Low {sup 226}Ra concentrations (0.1 to 5 pCi/g) are evidenced in sands, moderate concentrations (5 to 11 pCi/g) are found in silt and gravel, and high {sup 226}Ra concentrations (>11 pCi/g) are found in soil horizons with shell, clay, and strata with phosphate. Strata containing phosphate yields a high concentration of {sup 226}Ra. The information obtained in this study, soil descriptions with their corresponding {sup 226}Ra concentrations, comes from geological cores drilled by geotechnical consultants with gamma spectrometry analysis performed by high resolution gamma spectroscopy. Concentration; of {sup 226}Ra generally increase with depth. These cores are usually terminated at 20 feet deep, with some cores being shallower than this due to hitting bedrock or encountering the water table. These frequency distributions give the core-logging geologist an approximate concentration of {sup 226}Ra based on the description of the soil. Since the correlation of {sup 226}Ra and soil descriptions can be used as a tool in assigning indoor radon potential, this study is of importance to land managers, contractors, developers, and regulating agencies who are attempting to place standards on tracts of land with {sup 226}Ra concentration used as a criterion.

  13. 238U- 230Th- 226Ra disequilibrium in young Mt. Shasta andesites and dacites

    NASA Astrophysics Data System (ADS)

    Volpe, Alan M.

    1992-11-01

    The paper describes 238U-series nuclides and 230Th/ 232Th ratios measured by mass spectrometry in mineral separates of young Mt. Shasta andesites and dacites. The results constrain the timing of recent calc-alkaline magma fractionation at this volcano. Hotlum, Misery Hill and Black Butte rocks show small, < 13% 230Th- 238U and < 6% 226Ra- 230Th, disequilibria. Plagioclase have 7-26% 226Ra excesses, magnetite and groundmass have 4-5% 226Ra deficits, and pyroxenes have equilibrium ( 226Ra/ 230Th) activity ratios. Internal ( 230Th)-( 238U)and Ba-normalized ( 226Ra)-( 230Th) isotope diagrams for Hotlum and Black Butte dacites suggest that closed-system Th-U and Ra-Th fractionation occurred less than 10,000 years ago. Significant 226Ra- 230Th disequilibria in the Black Butte dacite strongly suggests that this rock erupted more recently than 9400 years ago. Results for Hotlum andesites suggest a longer pre-eruption crystal residence time compared to the dacites. There may also have been recent open Ra-Th system changes in the melt composition. Initial Th/U ratios for the rocks are low (2.43-2.57), similar to those in mid-ocean ridge basalts (MORB), and preclude significant assimilation of crust with markedly different Th-U composition.

  14. Draft Genome of Janthinobacterium sp. RA13 Isolated from Lake Washington Sediment.

    PubMed

    McTaggart, Tami L; Shapiro, Nicole; Woyke, Tanja; Chistoserdova, Ludmila

    2015-01-01

    Sequencing the genome of Janthinobacterium sp. RA13 from Lake Washington sediment is announced. From the genome content, a versatile life-style is predicted, but not bona fide methylotrophy. With the availability of its genomic sequence, Janthinobacterium sp. RA13 presents a prospective model for studying microbial communities in lake sediments. PMID:25676775

  15. Draft Genome of Janthinobacterium sp. RA13 Isolated from Lake Washington Sediment

    PubMed Central

    McTaggart, Tami L.; Shapiro, Nicole; Woyke, Tanja

    2015-01-01

    Sequencing the genome of Janthinobacterium sp. RA13 from Lake Washington sediment is announced. From the genome content, a versatile life-style is predicted, but not bona fide methylotrophy. With the availability of its genomic sequence, Janthinobacterium sp. RA13 presents a prospective model for studying microbial communities in lake sediments. PMID:25676775

  16. IL-1Ra and its delivery strategies: inserting the association in perspective.

    PubMed

    Akash, Muhammad Sajid Hamid; Rehman, Kanwal; Chen, Shuqing

    2013-11-01

    Interleukin-1 receptor antagonist (IL-1Ra) is a naturally occurring anti-inflammatory antagonist of interleukin-1 family of pro-inflammatory cytokines. The broad spectrum anti-inflammatory effects of IL-1Ra have been investigated against various auto-immune diseases such as diabetes mellitus, rheumatoid arthritis. Despite of its outstanding broad spectrum anti-inflammatory effects, IL-1Ra has short biological half-life (4-6 h) and to cope with this problem, up till now, many delivery strategies have been applied either to extend the half-life and/or prolong the steady-state sustained release of IL-1Ra from its target site. Here in our present paper, we have provided an overview of all approaches attempted to prolong the duration of therapeutic effects of IL-1Ra either by fusing IL-1Ra using fusion protein technology to extend the half-life and/or development of new dosage forms using various biodegradable polymers to prolong its steady-state sustained release at the site of administration. These approaches have been characterized by their intended impact on either in vitro release characteristics and/or pharmacokinetic and pharmacodynamic parameters of IL-1Ra. We have also compared these delivery strategies with each other on the basis of bioactivity of IL-1Ra after fusion with fusion protein partner and/or encapsulation with biodegradable polymer. PMID:23794040

  17. [Uptake of radionuclides from soil to plant and the discovery of 226Ra, 232Th hyperaccumulator].

    PubMed

    Zhang, Zhi-Qiang; Chen, Di-Yun; Song, Gang; Yue, Yu-Mei

    2011-04-01

    11 sorts of plant samples and corresponding soil samples were collected in Conghua and Taishan, Pearl River Delta. The specific activity of 238U, 226Ra, 232Th and 40K of samples were investigated by using HPGe-gamma-ray spectra analysis. The results showed that the average specific activity of 238U, 226Ra, 232Th and 40K in soil samples were 151.8, 146.3, 226.6, 665.5 Bq/kg, which were higher than the average values of China and the world. The concentration of 238U in all sort of plants are very low and most of them are lower than detection limit, while the values of 226Ra, 232Th and 40K were high. The contents of 226Ra and 232Th in Dicranopteris dichotoma were the highest, whose average specific activity is 285.9, 986.2 Bq/kg respectively. The average bioconcentration factors (BFs)of 26Ra, 232Th of Dicranopteris dichotoma were 2.20, 4.23, respectively, the other 10 sort of plants have BFs of 2266Ra, 232Th were in the range of 10(-1)-10(-2). The bioconcentration factors and the translocation factors of 226Ra, 232Th of Dicranopteris dichotoma. were all bigger than 1, so Dicranopteris dichotoma can be defined as hyperaccumulator of 226Ra and 232Th. PMID:21717763

  18. O-linked-N-acetylglucosamine modification of mammalian Notch receptors by an atypical O-GlcNAc transferase Eogt1

    SciTech Connect

    Sakaidani, Yuta; Ichiyanagi, Naoki; Saito, Chika; Nomura, Tomoko; Ito, Makiko; Nishio, Yosuke; Nadano, Daita; Matsuda, Tsukasa; Furukawa, Koichi; Okajima, Tetsuya

    2012-03-02

    Highlights: Black-Right-Pointing-Pointer We characterized A130022J15Rik (Eogt1)-a mouse gene homologous to Drosophila Eogt. Black-Right-Pointing-Pointer Eogt1 encodes EGF domain O-GlcNAc transferase. Black-Right-Pointing-Pointer Expression of Eogt1 in Drosophila rescued the cell-adhesion defect in the Eogt mutant. Black-Right-Pointing-Pointer O-GlcNAcylation reaction in the secretory pathway is conserved through evolution. -- Abstract: O-linked-{beta}-N-acetylglucosamine (O-GlcNAc) modification is a unique cytoplasmic and nuclear protein modification that is common in nearly all eukaryotes, including filamentous fungi, plants, and animals. We had recently reported that epidermal growth factor (EGF) repeats of Notch and Dumpy are O-GlcNAcylated by an atypical O-GlcNAc transferase, EOGT, in Drosophila. However, no study has yet shown whether O-GlcNAcylation of extracellular proteins is limited to insects such as Drosophila or whether it occurs in other organisms, including mammals. Here, we report the characterization of A130022J15Rik, a mouse gene homolog of Drosophila Eogt (Eogt 1). Enzymatic analysis revealed that Eogt1 has a substrate specificity similar to that of Drosophila EOGT, wherein the Thr residue located between the fifth and sixth conserved cysteines of the folded EGF-like domains is modified. This observation is supported by the fact that the expression of Eogt1 in Drosophila rescued the cell-adhesion defect caused by Eogt downregulation. In HEK293T cells, Eogt1 expression promoted modification of Notch1 EGF repeats by O-GlcNAc, which was further modified, at least in part, by galactose to generate a novel O-linked-N-acetyllactosamine structure. These results suggest that Eogt1 encodes EGF domain O-GlcNAc transferase and that O-GlcNAcylation reaction in the secretory pathway is a fundamental biochemical process conserved through evolution.

  19. Search for Electric dipole moment (EDM) in laser cooled and trapped 225Ra atoms

    NASA Astrophysics Data System (ADS)

    Kalita, Mukut; Bailey, Kevin; Dietrich, Matthew; Green, John; Holt, Roy; Korsch, Wolfgang; Lu, Zheng-Tian; Lemke, Nathan; Mueller, Peter; O'Connor, Tom; Parker, Richard; Singh, Jaideep; Trimble, Will; Argonne National Laboratory Collaboration; University Of Chicago Collabration; University Of Kentucky Collaboration

    2014-05-01

    We are searching for an EDM of the diamagnetic 225Ra atom. 225Ra has nuclear spin I =1/2. Experimental sensitivity to its EDM is enhanced due to its heavy mass and the increased Schiff moment of its octupole deformed nucleus. Our experiment involves collecting laser cooled Ra atoms in a magneto-optical trap (MOT), transporting them 1 meter with a far off-resonant optical dipole trap (ODT) and then transferring the atoms to a second standing-wave ODT in our experimental chamber. We will report our recent experiences in polarizing and observing Larmor precession of 225Ra atoms in parallel electric and magnetic fields in a magnetically shielded region and progress towards a first measurement of the EDM of 225Ra. This work is supported by DOE, Office of Nuclear Physics, under contract No. DE-AC02-06CH11357 and contract No. DE-FG02-99ER41101.

  20. Optical Frequency Domain Imaging

    NASA Astrophysics Data System (ADS)

    Bouma, Brett E.; Tearney, Guillermo J.; Vakoc, Benjamin; Yun, Seok Hyun

    In this chapter, we discuss a frequency-domain approach, optical frequency-domain imaging (OFDI), which is based on optical frequency-domain reflectometry and uses a wavelength-swept laser and standard single-element photodetectors. The chapter begins with an overview of the fundamental aspects of the technology, including the detected signal, sensitivity, depth range, and resolution, and then goes on to discuss specific component technologies including the light source, interferometer and acquisition electronics, and image processing. The final section of the chapter provides a brief glimpse at some of the biomedical applications that most directly take advantage of the improved speed and sensitivity of OFDI.