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Sample records for atypical ra domain

  1. RA diagnostics applying optical tomography in frequency domain

    NASA Astrophysics Data System (ADS)

    Klose, Alexander D.; Prapavat, Viravuth; Minet, Olaf; Beuthan, Juergen; Mueller, Gerhard J.

    1998-01-01

    Our aim is to reconstruct the optical parameters in a slice of a finger joint phantom for further investigations about rheumatoid arthritis (RA). Therefore, we have developed a flexible NIR scanning system in order to collect amplitude and phase delay of photon density waves in frequency-domain. A cylindrical finger joint phantom was embedded in a container of Intralipid solution due to the application of an inverse method for infinite geometry. The joint phantom was investigated by a laser beam obtaining several projections. The average optical parameters of each projection was calculated. Using different reconstruction techniques, e.g. ART and SIRT with a special projection operator, we reconstructed the optical parameters in a slice. The projection operator can be heuristically described by a photon path density function of a homogeneous media with infinite geometry. Applied to an object with an unknown distribution of optical parameters it calculates the expectation value of the investigated object. The potentials and limits of these fast reconstruction methods will be presented.

  2. 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…

  3. 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.

  4. 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.

  5. 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

  6. 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

  7. The extracellular domain of myelin oligodendrocyte glycoprotein elicits atypical experimental autoimmune encephalomyelitis in rat and Macaque species.

    PubMed

    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 and

  8. 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.

  9. 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.

  10. A point mutation in domain 4-segment 6 of the skeletal muscle sodium channel produces an atypical inactivation state.

    PubMed Central

    O'Reilly, J P; Wang, S Y; Wang, G K

    2000-01-01

    We compared wild-type rat skeletal muscle NaChs (micro1) and a mutant NaCh (Y1586K) that has a single amino acid substitution, lysine (K) for tyrosine (Y), at position 1586 in the S6 transmembrane segment of domain 4. In Y1586K, macroscopic current decay is faster, the V(1/2) of the activation curve is shifted in the depolarized direction, and the fast-inactivation curve is less steep compared with mu1. After an 8-ms depolarization pulse, Y1586K recovers from inactivation much more slowly than mu1. The recovery is double exponential, suggesting recovery from two inactivation states. Varying the depolarization protocols isolates entry into an additional, "atypical" inactivation state in Y1586K that is distinct from typical fast or slow inactivation. Substitution of positively charged arginine (R) at Y1586 produces an inactivation phenotype similar to that of Y1586K. Substitution by negatively charged aspartic acid (D) or uncharged alanine (A) at Y1586 produces an inactivation phenotype similar to mu1. Our results suggest that the positive charge of lysine (K) produces the atypical inactivation state in Y1586K. We propose that a conformational change during depolarization alters the relative position of the 1586K residue in the D4-S6 segment and that atypical inactivation in Y1586K occurs via an electrostatic interaction in or near the inner pore region. PMID:10653790

  11. COORDINATED ACTIVATION OF THE RAC-GAP β2-CHIMAERIN BY AN ATYPICAL PROLINE-RICH DOMAIN AND DIACYLGLYCEROL

    PubMed Central

    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 (GAPs) for the small G-protein Rac, have been implicated in development, neuritogenesis, and cancer. These Rac-GAPs are regulated by the lipid second messenger diacylglycerol (DAG) generated by tyrosine-kinases such as the epidermal growth factor receptor (EGFR). Here we identify an atypical Pro-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. EGF promotes the binding of β2-chimaerin to Nck1 in the cell periphery in a DAG-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 Pro-rich region. PMID:23673634

  12. 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

  13. Atypical Depression

    MedlinePlus

    Diseases and Conditions Atypical depression By Mayo Clinic Staff Any type of depression can make you feel sad and keep you from enjoying life. However, atypical depression — also called depression with atypical features — means that ...

  14. Mapping of receptor binding sites on IL-1 beta by reconstruction of IL-1ra-like domains.

    PubMed

    Boraschi, D; Bossù, P; Ruggiero, P; Tagliabue, A; Bertini, R; Macchia, G; Gasbarro, C; Pellegrini, L; Melillo, G; Ulisse, E; Visconti, U; Bizzarri, C; Del Grosso, E; Mackay, A R; Frascotti, G; Frigerio, F; Grifantini, R; Grandi, G

    1995-11-15

    Upon structure comparison between IL-1 beta and its antagonist IL-1ra, single or multiple residues along the IL-1 beta sequence were replaced with the corresponding amino acids present in the IL-1ra protein, in the attempt to identify sites important for receptor binding and for biologic activity on the two molecules. Ten of fifteen mutant proteins had activity comparable to that of wild-type IL-1 beta in three different biologic assays and in receptor binding, indicating that the introduced changes did not influence the functional structure of the protein. Conversely, three mutants (SMIL-9: 127/263 R/T-->W/Y; SMIL-10: 125/127/263/265 T/R/T/Q-->R/W/Y/E; SMIL-15:222/227 I/E-->S/S) showed an increased binding capacity for IL-1RI, not paralleled by increased agonist activity, indicating that the introduced IL-1ra residues could be involved in the nonagonist IL-1RI binding site. On the other hand, two mutants showed diminished binding capacity with concomitant decrease in biologic activity. Both mutants (SMIL-1, five substitutions in the loop 202-214; and SMIL-3, total replacement of the loop 164-173 with the IL-1ra stretch 52-55) included substitutions of residues allegedly important for agonist binding to IL-1RI. Mutant SMIL-3 showed the most profound reduction in binding capacity for IL-1RI (CDw121a) and a more than 1,000-fold reduced biologic activity both in vitro and in vivo, but it retained full capacity of binding to IL-1RII (CDw121b) and acted as a selective antagonist of IL-1RII. From these results the following conclusions can be drawn. IL-1 beta binds to IL-1RI and to IL-1RII through different sites, and the loop 164-173 appears as one of the areas involved in the selective interaction with IL-1RI. Agonist (IL-1 beta) and nonagonist (IL-1ra) binding to IL-1RI occur through distinct sites, with loops 164-173 and 202-214 of IL-1 beta identified as two of the sites selectively involved in agonist binding to the activating receptor.

  15. Synthesis of the Tetracyclic ABCD Ring Domain of Calyciphylline A-Type Alkaloids via Reductive Radical Cyclizations.

    PubMed

    Coussanes, Guilhem; Bonjoch, Josep

    2017-02-17

    A tetracyclic compound with the ABCD ring framework of calyciphylline A-type alkaloids was synthesized from a cis-3a-methyloctahydroindole triggered by a 5-endo radical cyclization. The synthesis required two additional ring-forming steps: the construction of a seven-membered ring by aldol cyclization and the azabicyclic fragment by a radical ring closure of a trichloroacetamide-tethered enol acetate followed by a diastereoselective α-methylation of the lactam group.

  16. Illusory memories of emotionally charged words in autism spectrum disorder: further evidence for atypical emotion processing outside the social domain.

    PubMed

    Gaigg, Sebastian B; Bowler, Dermot M

    2009-07-01

    Recent evidence suggests that individuals with ASD may not accumulate distinct representations of emotional information throughout development. On the basis of this observation we predicted that such individuals would not be any less likely to falsely remember emotionally significant as compared to neutral words when such illusory memories are induced by asking participants to study lists of words that are orthographically associated to these words. Our findings showed that typical participants are far less likely to experience illusory memories of emotionally charged as compared to neutral words. Individuals with ASD, on the other hand, did not exhibit this emotional modulation of false memories. We discuss this finding in relation to the role of emotional processing atypicalities in ASD.

  17. Atypical pneumonia

    MedlinePlus

    Bacteria that cause atypical pneumonia include: Mycoplasma pneumonia is caused by the bacteria Mycoplasma pneumoniae . It often affects people younger than age 40. Pneumonia due to Chlamydophila pneumoniae bacteria ...

  18. Solution structure of the region 51–160 of human KIN17 reveals an atypical winged helix domain

    PubMed Central

    Carlier, Ludovic; Couprie, Joël; le Maire, Albane; Guilhaudis, Laure; Milazzo-Segalas, Isabelle; Courçon, Marie; Moutiez, Mireille; Gondry, Muriel; Davoust, Daniel; Gilquin, Bernard; Zinn-Justin, Sophie

    2007-01-01

    Human KIN17 is a 45-kDa eukaryotic DNA- and RNA-binding protein that plays an important role in nuclear metabolism and in particular in the general response to genotoxics. Its amino acids sequence contains a zinc finger motif (residues 28–50) within a 30-kDa N-terminal region conserved from yeast to human, and a 15-kDa C-terminal tandem of SH3-like subdomains (residues 268–393) only found in higher eukaryotes. Here we report the solution structure of the region 51–160 of human KIN17. We show that this fragment folds into a three-α-helix bundle packed against a three-stranded β-sheet. It belongs to the winged helix (WH) family. Structural comparison with analogous WH domains reveals that KIN17 WH module presents an additional and highly conserved 310-helix. Moreover, KIN17 WH helix H3 is not positively charged as in classical DNA-binding WH domains. Thus, human KIN17 region 51–160 might rather be involved in protein–protein interaction through its conserved surface centered on the 310-helix. PMID:18029424

  19. Atypical Antigen Recognition Mode of a Shark Immunoglobulin New Antigen Receptor (IgNAR) Variable Domain Characterized by Humanization and Structural Analysis

    PubMed Central

    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

    2013-01-01

    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

  20. 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.

  1. Analysis of chitin-binding proteins from Manduca sexta provides new insights into evolution of peritrophin A-type chitin-binding domains in insects.

    PubMed

    Tetreau, Guillaume; Dittmer, Neal T; Cao, Xiaolong; Agrawal, Sinu; Chen, Yun-Ru; Muthukrishnan, Subbaratnam; Haobo, Jiang; Blissard, Gary W; Kanost, Michael R; Wang, Ping

    2015-07-01

    In insects, chitin is a major structural component of the cuticle and the peritrophic membrane (PM). In nature, chitin is always associated with proteins among which chitin-binding proteins (CBPs) are the most important for forming, maintaining and regulating the functions of these extracellular structures. In this study, a genome-wide search for genes encoding proteins with ChtBD2-type (peritrophin A-type) chitin-binding domains (CBDs) was conducted. A total of 53 genes encoding 56 CBPs were identified, including 15 CPAP1s (cuticular proteins analogous to peritrophins with 1 CBD), 11 CPAP3s (CPAPs with 3 CBDs) and 17 PMPs (PM proteins) with a variable number of CBDs, which are structural components of cuticle or of the PM. CBDs were also identified in enzymes of chitin metabolism including 6 chitinases and 7 chitin deacetylases encoded by 6 and 5 genes, respectively. RNA-seq analysis confirmed that PMP and CPAP genes have differential spatial expression patterns. The expression of PMP genes is midgut-specific, while CPAP genes are widely expressed in different cuticle forming tissues. Phylogenetic analysis of CBDs of proteins in insects belonging to different orders revealed that CPAP1s from different species constitute a separate family with 16 different groups, including 6 new groups identified in this study. The CPAP3s are clustered into a separate family of 7 groups present in all insect orders. Altogether, they reveal that duplication events of CBDs in CPAP1s and CPAP3s occurred prior to the evolutionary radiation of insect species. In contrast to the CPAPs, all CBDs from individual PMPs are generally clustered and distinct from other PMPs in the same species in phylogenetic analyses, indicating that the duplication of CBDs in each of these PMPs occurred after divergence of insect species. Phylogenetic analysis of these three CBP families showed that the CBDs in CPAP1s form a clearly separate family, while those found in PMPs and CPAP3s were clustered

  2. Atypical Cities

    ERIC Educational Resources Information Center

    DiJulio, Betsy

    2011-01-01

    In this creative challenge, Surrealism and one-point perspective combine to produce images that not only go "beyond the real" but also beyond the ubiquitous "imaginary city" assignment often used to teach one-point perspective. Perhaps the difference is that in the "atypical cities challenge," an understanding of one-point perspective is a means…

  3. Atypical presentations of atypical antipsychotics.

    PubMed

    Lind, Cpt Christopher K; Carchedi, Cpt Lisa R; Staudenmeier, Ltc James J; Diebold, Ltc P Carroll J

    2005-06-01

    The atypical antipsychotics have been touted by many as having minimal extrapyramidal symptoms. This case series from the Tripler Army Medical Center Psychiatry Graduate Medical Education Program presents the extrapyramidal symptoms observed with four different atypical antipsychotic medications. Also reviewed are the mechanisms of action that atypical antipsychotics and first-generation antipsychotics use to treat the symptoms of schizophrenia. Cases reviewed include a schizophrenic male patient whose dose of risperidone was doubled from 6mg to 12mg overnight and developed an acute dystonic reaction; a young male patient with a substance-induced psychosis who unintentionally doubled his ziprasidone dose in 24 hours, resulting in an acute dystonic reaction; a young female patient on paroxetine who also recently started olanzapine and had complaints consistent with akathisia that resolved with treatment; and an adolescent female patient on escitalopram for obsessive-compulsive disorder who after starting aripiprazole developed Parkinsonism. All four cases illustrate that even though atypical antipsychotics are less likely to cause extrapyramidal symptoms than their first generation cousins, the physician should be aware that these symptoms may still occur and need to be treated.

  4. Atypical Presentations of Atypical Antipsychotics

    PubMed Central

    Carchedi, CPT. Lisa R.; Staudenmeier, LTC. James J.; Diebold, LTC(P). Carroll J.

    2005-01-01

    The atypical antipsychotics have been touted by many as having minimal extrapyramidal symptoms. This case series from the Tripler Army Medical Center Psychiatry Graduate Medical Education Program presents the extrapyramidal symptoms observed with four different atypical antipsychotic medications. Also reviewed are the mechanisms of action that atypical antipsychotics and first-generation antipsychotics use to treat the symptoms of schizophrenia. Cases reviewed include a schizophrenic male patient whose dose of risperidone was doubled from 6mg to 12mg overnight and developed an acute dystonic reaction; a young male patient with a substance-induced psychosis who unintentionally doubled his ziprasidone dose in 24 hours, resulting in an acute dystonic reaction; a young female patient on paroxetine who also recently started olanzapine and had complaints consistent with akathisia that resolved with treatment; and an adolescent female patient on escitalopram for obsessive-compulsive disorder who after starting aripiprazole developed Parkinsonism. All four cases illustrate that even though atypical antipsychotics are less likely to cause extrapyramidal symptoms than their first generation cousins, the physician should be aware that these symptoms may still occur and need to be treated. PMID:21152153

  5. Atypical GTPases as drug targets.

    PubMed

    Soundararajan, Meera; Eswaran, Jeyanthy

    2012-01-01

    The Ras GTPases are the founding members of large Ras superfamily, which constitutes more than 150 of these important class of enzymes. These GTPases function as GDP-GTP-regulated binary switches that control many fundamental cellular processes. There are a number of GTPases that have been identified recently, which do not confine to this prototype termed as "atypical GTPases" but have proved to play a remarkable role in vital cellular functions. In this review, we provide an overview of the crucial physiological functions mediated by RGK and Centaurin class of multi domain atypical GTPases. Moreover, the recently available atypical GTPase structures of the two families, regulation, physiological functions and their critical roles in various diseases will be discussed. In summary, this review will highlight the emerging atypical GTPase family which allows us to understand novel regulatory mechanisms and thus providing new avenues for drug discovery programs.

  6. 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...

  7. 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

  8. "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…

  9. 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.

  10. 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

  11. 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.

  12. A Novel Heterozygous Mutation in the STAT1 SH2 Domain Causes Chronic Mucocutaneous Candidiasis, Atypically Diverse Infections, Autoimmunity, and Impaired Cytokine Regulation

    PubMed Central

    Meesilpavikkai, Kornvalee; Dik, Willem A.; Schrijver, Benjamin; Nagtzaam, Nicole M. A.; van Rijswijk, Angelique; Driessen, Gertjan J.; van der Spek, Peter J.; van Hagen, P. Martin; Dalm, Virgil A. S. H.

    2017-01-01

    Chronic mucocutaneous candidiasis (CMC) is a primary immunodeficiency characterized by persistent or recurrent skin and mucosal surface infections with Candida species. Different gene mutations leading to CMC have been identified. These include various heterozygous gain-of-function (GOF) mutations in signal transducer and activator of transcription 1 (STAT1) that are not only associated with infections but also with autoimmune manifestations. Recently, two STAT1 GOF mutations involving the Src homology 2 (SH2) domain have been reported, while so far, over 50 mutations have been described mainly in the coiled coil and the DNA-binding domains. Here, we present two members of a Dutch family with a novel STAT1 mutation located in the SH2 domain. T lymphocytes of these patients revealed STAT1 hyperphosphorylation and higher expression of STAT1 target genes. The clinical picture of CMC in our patients could be explained by diminished production of interleukin (IL)-17 and IL-22, cytokines important in the protection against fungal infections. PMID:28348565

  13. 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.

    PubMed

    Jasrapuria, Sinu; Arakane, Yasuyuki; Osman, Gamal; Kramer, Karl J; Beeman, Richard W; Muthukrishnan, Subbaratnam

    2010-03-01

    This study is focused on the characterization and expression of genes in the red flour beetle, Tribolium castaneum, encoding proteins that possess one or more six-cysteine-containing chitin-binding domains related to the peritrophin A domain (ChtBD2). An exhaustive bioinformatics search of the genome of T. castaneum queried with ChtBD2 sequences yielded 13 previously characterized chitin metabolic enzymes and 29 additional proteins with signal peptides as well as one to 14 ChtBD2s. Using phylogenetic analyses, these additional 29 proteins were classified into three large families. The first family includes 11 proteins closely related to the peritrophins, each containing one to 14 ChtBD2s. These are midgut-specific and are expressed only during feeding stages. We propose the name "Peritrophic Matrix Proteins" (PMP) for this family. The second family contains eight proteins encoded by seven genes (one gene codes for 2 splice variants), which are closely related to gasp/obstructor-like proteins that contain 3 ChtBD2s each. The third family has ten proteins that are of diverse sizes and sequences with only one ChtBD2 each. The genes of the second and third families are expressed in non-midgut tissues throughout all stages of development. We propose the names "Cuticular Proteins Analogous to Peritophins 3" (CPAP3) for the second family that has three ChtBD2s and "Cuticular Proteins Analogous to Peritophins 1 (CPAP1) for the third family that has 1 ChtBD2. Even though proteins of both CPAP1 and CPAP3 families have the "peritrophin A" domain, they are expressed only in cuticle-forming tissues. We determined the exon-intron organization of the genes, encoding these 29 proteins as well as the domain organization of the encoded proteins with ChtBD2s. All 29 proteins have predicted cleavable signal peptides and ChtBD2s, suggesting that they interact with chitin in extracellular locations. Comparison of ChtBD2s-containing proteins in different insect species belonging to

  14. 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.

  15. 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.

  16. 226Ra and 228Ra in Iowa drinking water.

    PubMed

    Kriege, L B; Hahne, R M

    1982-10-01

    The University Hygienic Laboratory has been performing radiochemical analyses on drinking water in the state of Iowa for over 20 yr. Approximately one half of the 1250 community water supplies that exist in Iowa have been sampled roughly once every 3 yr for the past decade. Originally, raw and finished waters that showed a gross alpha activity of greater than or equal to 3.0 pCi/L were analyzed for 226Ra, but starting in July 1976, finished waters were analyzed for both 226Ra and 228Ra if the gross alpha activity was greater than or equal to 2.0 pCi/L. As of 10 June 1981, 604 community water supplies had submitted composited samples that have been analyzed for gross alpha, 226Ra, and 228Ra concentrations in compliance with the federal Safe Drinking Water Act (Public Law 93-523). Approximately 10% of these supplies were found to exceed the EPA-established maximum contaminant level (MCL) for 226Ra plus 228Ra of 5 pCi/L. The results revealed, consistent with several other investigators (Mc81; Mi80; Mic80), that some supplies had higher concentrations of 228Ra than of 226Ra. It was also concluded, in agreement with McCurdy and Mellor (Mc81), that some ground water samples cannot be accurately measured for gross alpha activity due to their high dissolved solids content.

  17. [Atypical ubiquitination of proteins].

    PubMed

    Buneeva, O A; Medvedev, A E

    2016-07-01

    Ubiquitination is a type of posttranslational modification of intracellular proteins characterized by covalent attachment of one (monoubiquitination) or several (polyubiquitination) of ubiquitin molecules to target proteins. In the case of polyubiquitination, linear or branched polyubiquitin chains are formed. Their formation involves various lysine residues of monomeric ubiquitin. The best studied is Lys48-polyubiquitination, which targets proteins for proteasomal degradation. In this review we have considered examples of so-called atypical polyubiquitination, which mainly involves other lysine residues (Lys6, Lys11, Lys27, Lys29, Lys33, Lys63) and also N-terminal methionine. The considered examples convincingly demonstrate that polyubiquitination of proteins not necessarily targets proteins for their proteolytic degradation in proteasomes. Atypically polyubiquitinated proteins are involved in regulation of various processes and altered polyubiquitination of certain proteins is crucial for development of serious diseases.

  18. Atypical Cogan's Syndrome

    PubMed Central

    Queirós, João; Maia, Sofia; Seca, Mariana; Friande, António; Araújo, Maria; Meireles, Angelina

    2013-01-01

    Background. Cogan's syndrome is a rare clinical entity whose etiopathology is still unknown, and the treatment strategies are not clearly defined. Case. A 23-year-old male presented with symptoms of headache, peripheral facial palsy, persistent right hearing loss and bilateral papillitis. Workup excluded all infectious, granulomatous, neoplastic, and immune causes. The diagnosis of atypical Cogan's syndrome was established, and the patient was treated with systemic corticosteroids and later on with cyclophosphamide and methotrexate. There were improvement of visual symptoms and stabilisation of left hearing. Conclusion. Cogan's syndrome is a very rare disease with no specific biological tests for the diagnosis. The diagnostic exams are mostly important to exclude other etiologies. The atypical ocular and audiovestibular manifestations make the diagnosis difficult, delaying the institution of appropriate therapy which may result in profound bilateral deafness. PMID:23691387

  19. [Atypical presentation of preeclampsia].

    PubMed

    Ditisheim, A; Boulvain, M; Irion, O; Pechère-Bertschi, A

    2015-09-09

    Preeclampsia is a pregnancy-related syndrome, which still represents one of the major causes of maternal-fetal mortality and morbidity. Diagnosis can be made difficult due to the complexity of the disorder and its wide spectrum of clinical manifestations. In order to provide an efficient diagnostic tool to the clinician, medical societies regularly rethink the definition criteria. However, there are still clinical presentations of preeclampsia that escape the frame of the definition. The present review will address atypical forms of preeclampsia, such as preeclampsia without proteinuria, normotensive preeclampsia, preeclampsia before 20 weeks of gestation and post-partum preeclampsia.

  20. Atypical neuroleptics: compulsive disorders.

    PubMed

    2014-02-01

    Compulsive disorders are known adverse effects of dopamine agonists. Atypical neuroleptics (amisulpride, aripiprazole, olanzapine, paliperidone, quetiapine and risperidone) have also been implicated in cases of pathological gambling, hypersexuality, and compulsive eating and shopping, with sometimes serious social and familial consequences. The compulsive disorders improved or ceased when the neuroleptic was withdrawn or replaced. Patients must be informed of these possible adverse effects and monitored for behavioural changes. If such disorders occur, they can be managed by withdrawing the drug, reducing the dosage, or switching to another neuroleptic.

  1. 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.

  2. 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

  3. Atypical Cutaneous Manifestations in Syphilis.

    PubMed

    Ivars Lleó, M; Clavo Escribano, P; Menéndez Prieto, B

    2016-05-01

    Although the diversity of the clinical manifestations of syphilis is well-known, atypical presentations can also occur. Such atypical presentations are associated with a high risk of transmission as a result of diagnostic confusion and treatment delays owing to the disease's ability to mimic other common skin diseases, deviate from classic clinical presentations, and adopt unique forms. Cases of atypical syphilis have been described most frequently in patients with concomitant human immunodeficiency virus (HIV) infection. Because the incidence of syphilis has been growing over recent years -particularly in patients with HIV co-infection- dermatologists need to be familiar with the less well-known clinical presentations of this venereal disease.

  4. Atypical manifestations of tinea corporis.

    PubMed

    Ziemer, Mirjana; Seyfarth, Florian; Elsner, Peter; Hipler, Uta-Christina

    2007-01-01

    Tinea corporis classically presents as an erythematous annular plaque with a scaly, centrifugally advancing border. However, sometimes vesicles and pustules are observed. Occasionally, even frank bullae appear secondary to severe inflammation. Diagnostic difficulties arise when atypical manifestations mimic other inflammatory skin diseases, including atopic or seborrheic dermatitis, subacute cutaneous lupus erythematosus, or vesicular diseases. We report five cases of atypical tinea corporis, where the initial clinical diagnosis was different from dermatophytosis. The differential diagnoses and the diagnostic difficulties related to atypical manifestations of fungal infections are discussed. Moreover, our cases emphasise the importance of conventional histological examination, which enables a fast, correct diagnosis.

  5. Opposing RA and FGF signals control proximodistal vertebrate limb development through regulation of Meis genes.

    PubMed

    Mercader, N; Leonardo, E; Piedra, M E; Martínez-A, C; Ros, M A; Torres, M

    2000-09-01

    Vertebrate limbs develop in a temporal proximodistal sequence, with proximal regions specified and generated earlier than distal ones. Whereas considerable information is available on the mechanisms promoting limb growth, those involved in determining the proximodistal identity of limb parts remain largely unknown. We show here that retinoic acid (RA) is an upstream activator of the proximal determinant genes Meis1 and Meis2. RA promotes proximalization of limb cells and endogenous RA signaling is required to maintain the proximal Meis domain in the limb. RA synthesis and signaling range, which initially span the entire lateral plate mesoderm, become restricted to proximal limb domains by the apical ectodermal ridge (AER) activity following limb initiation. We identify fibroblast growth factor (FGF) as the main molecule responsible for this AER activity and propose a model integrating the role of FGF in limb cell proliferation, with a specific function in promoting distalization through inhibition of RA production and signaling.

  6. Atypical depression: a valid subtype?

    PubMed

    Parker, Gordon B

    2007-01-01

    The concept of atypical depression has evolved over the past several decades, yet remains inadequately defined. As currently defined by the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR), the main criterion of atypical depression is the presence of mood reactivity in combination with at least 2 of 4 secondary criteria (hypersomnia, hyperphagia and weight gain, leaden paralysis, and oversensitivity to criticism and rejection). The focus on mood reactivity as the primary distinguishing criterion remains questionable among researchers who have been unable to verify the primacy of this symptom in relation to the other diagnostic criteria for atypical depression. A model challenging the DSM-IV-TR definition of atypical depression has been developed, redefining the disorder as a dimensional nonmelancholic syndrome in which individuals with a personality subtype of "interpersonal rejection sensitivity" have a tendency toward the onset of anxiety disorders and depression, thereby exhibiting a variety of dysregulated emotional and self-consolatory responses. This reformulated definition of atypical depression (in arguing for the primacy of a personality style or rejection sensitivity as against mood reactivity) may lead to a better understanding and recognition of the disorder and its symptoms as well as other "spectrum" disorders within the scope of major depression.

  7. IL2RA — EDRN Public Portal

    Cancer.gov

    The interleukin 2 receptor exists in three forms which differ in their ability to bind interleukin 2. The low affinity form of the receptor is a monomer of IL2RA, the alpha subunit. The alpha/beta subunit heterodimer, formed by IL2RA and IL2RB, is an intermediate affinity form. The alpha/beta/gamma heterotrimer formed by IL2RA, IL2RB, and IL2RG is the high affinity form. IL2RA is normally an integral membrane protein, although soluble IL2RA has been isolated. There are known alternately-spliced versions of IL2RA mRNAs, but their functions are unknown. Mutations in the IL2RA gene are associated with diabetes mellitus insulin-dependent type 10 (IDDM10). Complications of IDDM10 can adversely affect the eyes, kidneys, nerves, and blood vessels.

  8. 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.

  9. Atypical presentations of orbital cysticercosis.

    PubMed

    Pushker, Neelam; Chaturvedi, Amrita; Balasubramanya, Ramamurthy; Bajaj, Mandeep S; Kumar, Neena; Sony, Parul

    2005-01-01

    We describe three patients with orbital cysticercosis who presented with atypical clinical or radiologic features previously unreported. All three patients had a cyst with a scolex on imaging studies. After 6 weeks of treatment, all three had almost complete resolution of their features.

  10. Sleep Disorders in Atypical Parkinsonism

    PubMed Central

    Abbott, Sabra M.; Videnovic, Aleksandar

    2014-01-01

    Sleep disorders are commonly seen in atypical parkinsonism, with particular disorders occurring more frequently in specific parkinsonian disorders. Multiple systems atrophy (MSA) is a synucleinopathy often associated with nocturnal stridor which is a serious, but treatable condition highly specific to MSA. In addition, this disorder is strongly associated with rapid eye movement (REM) sleep behavior disorder (RBD), which is also seen in dementia with Lewy bodies (DLB). RBD is far less prevalent in progressive supranuclear palsy (PSP), which is a tauopathy. Insomnia and impaired sleep architecture are the most common sleep abnormalities seen in PSP. Corticobasilar degeneration (CBD) is also a tauopathy, but has far fewer sleep complaints associated with it than PSP. In this manuscript we review the spectrum of sleep dysfunction across the atypical parkinsonian disorders, emphasize the importance of evaluating for sleep disorders in patients with parkinsonian symptoms, and point to sleep characteristics that can provide diagnostic clues to the underlying parkinsonian disorder. PMID:24955381

  11. 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

  12. TLRs, future potential therapeutic targets for RA.

    PubMed

    Elshabrawy, Hatem A; Essani, Abdul E; Szekanecz, Zoltán; Fox, David A; Shahrara, Shiva

    2017-02-01

    Toll like receptors (TLR)s have a central role in regulating innate immunity and in the last decade studies have begun to reveal their significance in potentiating autoimmune diseases such as rheumatoid arthritis (RA). Earlier investigations have highlighted the importance of TLR2 and TLR4 function in RA pathogenesis. In this review, we discuss the newer data that indicate roles for TLR5 and TLR7 in RA and its preclinical models. We evaluate the pathogenicity of TLRs in RA myeloid cells, synovial tissue fibroblasts, T cells, osteoclast progenitor cells and endothelial cells. These observations establish that ligation of TLRs can transform RA myeloid cells into M1 macrophages and that the inflammatory factors secreted from M1 and RA synovial tissue fibroblasts participate in TH-17 cell development. From the investigations conducted in RA preclinical models, we conclude that TLR-mediated inflammation can result in osteoclastic bone erosion by interconnecting the myeloid and TH-17 cell response to joint vascularization. In light of emerging unique aspects of TLR function, we summarize the novel approaches that are being tested to impair TLR activation in RA patients.

  13. 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

  14. 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.

  15. [Atypical wounds: definition and classification].

    PubMed

    Situm, Mirna; Kolić, Maja

    2012-10-01

    Wound represents disruption of the anatomic and physiologic continuity of the skin. Regarding the healing process, wounds can be classified as acute or chronic wounds. A wound is considered chronic if healing does not occur within the expected period according to its etiology and localization. Chronic wounds can be classified as typical and atypical. Typical wounds include ischemic, neurotrophic and hypostatic ulcers and two separate entities: diabetic foot and decubitus ulcers. Eighty percent of chronic wounds localized on the lower leg are the result of chronic venous insufficiency, in 5-10 percent the cause is of arterial etiology, whereas the remainder is mostly neuropathic ulcer. Ninety-five percent of chronic wounds manifest as one of the above mentioned entities. Other forms of chronic wounds represent atypical chronic wounds, which can be caused by autoimmune disorders, infectious diseases, vascular diseases and vasculopathies, metabolic and genetic diseases, neoplasm, external factors, psychiatric disorders, drug related reactions, etc. Numerous systemic diseases can present with atypical wounds. The primary cause of the wound can be either systemic disease itself (Crohn's disease) or aberrant immune response due to systemic disease (pyoderma gangrenosum, paraneoplastic syndrome).

  16. 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.

  17. 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.

  18. 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.

  19. Myometrial dysplasia (atypical myometrial hyperplasia).

    PubMed

    Cramer, Stewart F; Newcomb, Patricia M; Bonfiglio, Thomas A

    2007-04-01

    Although precursor lesions are well known for cervical and endometrial neoplasms, precursor lesions are not currently recognized for the most common tumor of the uterus-leiomyomas. Myometrial hyperplasia has been recently described and evaluated by morphometry, but its relationship to uterine leiomyomas has not been systematically explored. Myometrial dysplasia (atypical myometrial hyperplasia) has not been previously recognized. We herein report a case of myometrial dysplasia with immunostains for proliferation marker MIB-1 (Ki-67) and for p53. The paradoxical rarity of myometrial dysplasia is considered in comparison to the striking frequency of uterine leiomyomas.

  20. 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 ...

  1. 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.

  2. RA Construction KC, LLC Information Sheet

    EPA Pesticide Factsheets

    RA Construction KC, LLC (the Company) is located in Gladstone, Missouri. The settlement involves renovation activities conducted at a property constructed prior to 1978, located in Kansas City, Missouri.

  3. Removal of 226Ra and 228Ra from TENORM sludge waste using surfactants solutions.

    PubMed

    Attallah, M F; Hamed, Mostafa M; El Afifi, E M; Aly, H F

    2015-01-01

    The feasibility of using surfactants as extracting agent for the removal of radium species from TENORM sludge produced from petroleum industry is evaluated. In this investigation cationic and nonionic surfactants were used as extracting agents for the removal of radium radionuclides from the sludge waste. Two surfactants namely cetyltrimethylammonium bromide (CTAB) and Triton X-100 (TX100) were investigated as the extracting agents. Different parameters affecting the removal of both (226)Ra and (228)Ra by the two surfactants as well as their admixture were studied by the batch technique. These parameters include effect of shaking time, surfactants concentration and temperature as well as the effect of surfactants admixture. It was found that, higher solution temperature improves the removal efficiency of radium species. Combined extraction of nonionic and cationic surfactants produces synergistic effect in removal both (226)Ra and (228)Ra, where the removals reached 84% and 80% for (226)Ra and (228)Ra, respectively, were obtained using surfactants admixture.

  4. Estuarine geochemistry of /sup 224/Ra, /sup 226/Ra, and /sup 222/Rn

    SciTech Connect

    Elsinger, R.J.

    1982-01-01

    Desorption from river borne sediments is the most likely source of the excess /sup 226/Ra. Laboratory mixing experiments on Pee Dee River sediments show an increase in /sup 226/Ra desorption with increasing salinities with maximum desorption occurring at or above 20/sup 0//oo salinity. Desorption and diffusion are the sources for /sup 226/Ra in the estuarine systems. In Winyah Bay the /sup 228/Ra//sup 226/Ra activity ratio does not change significantly with salinity, averaging around 1.4, indicating desorption as the major source of /sup 228/Ra. In the Yangtze River the /sup 228/Ra//sup 226/Ra activity ratio is constant (approx.1.90) until increasing linearly above 16/sup 0//oo. A diffusive flux from regeneration by /sup 232/Th decay in shelf sediments is the source of the increase. In Delaware Bay /sup 228/Ra increases faster than /sup 226/Ra in the less than or equal to22/sup 0//oo water, indicating a source in addition to desorption. The increase can be balanced by a 0.33 dpm/cm/sup 2/-year flux over the upper part of the Bay where fine grained sediments predominate. /sup 224/Ra behavior is controlled by its 3.64 day half-life. In Winyah Bay a flux of around 0.4 dpm/cm/sup 2/-day is necessary to support the standing crop of non-desorbed /sup 224/Ra in the water column. In Delaware Bay the nearly constant /sup 224/Ra in concentration over the 2.5/sup 0//oo to 12/sup 0//oo salinity range are maintained by regeneration from /sup 228/Th in the turbidity maximum zones and diffusion from bottom sediments. Water leaving on ebb tide from a salt marsh on Delaware Bay had increases in all three radium isotopes (/sup 224/Ra > /sup 228/Ra > /sup 226/Ra) compared to water coming in on the flood tide. Excess /sup 222/Rn concentrations in a fresh water section of the Pee Dee River show a decreasing downstream gradient. Using these gradients to determine evasion rates, stagnant film thicknesses range from 21..mu.. to 62..mu...

  5. [Atypical Cogan's syndrome. Based on a case].

    PubMed

    Nandu, A; Salu, P; Caspers, L; Gordts, F; Sennesael, J

    2004-01-01

    Cogan's syndrome is a systemic inflammatory disease that associates typical (interstitial keratitis) and atypical (such as anterior uveitis) ocular manifestations to vestibulo-auditory dysfunction. It has also a systemic vascular association of vasculitis type. We report a case of a 64 years old woman who presented an atypical form with anterior uveitis.

  6. 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.

  7. 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.

  8. Atypical immunoglobulin light chain amyloidosis

    PubMed Central

    Wu, Xia; Feng, Jun; Cao, Xinxin; Zhang, Lu; Zhou, Daobin; Li, Jian

    2016-01-01

    Abstract Background: Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. Methods: Herein, we present 3 new cases of AL amyloidosis with spontaneous vertebral compression fracture and review 13 cases retrieved from the literature. Results: Moreover, we observed overrepresentations of liver involvement and bone marrow involvement in AL amyloidosis with spontaneous vertebral compression fracture. Conclusion: We believe that better awareness of the rare clinical presentation as spontaneous vertebral compression fracture of AL amyloidosis can facilitate earlier diagnosis and earlier treatment. PMID:27603350

  9. Techniques for precise mapping of 226Ra and 228Ra in the ocean

    NASA Astrophysics Data System (ADS)

    Moore, Willard S.; Key, Robert M.; Sarmiento, Jorge L.

    1985-01-01

    Improvements in the analyses of 226Ra and 228Ra in seawater made possible by better extraction and processing techniques reduce significantly the errors associated with these measurements. These improvements and the extensive sampling for Ra isotopes conducted on the TTO North Atlantic Study should enable us to use the distribution of 228Ra to study mixing processes on a 3-15 year time scale in both the upper and deep North Atlantic. The 228Ra profiles already analyzed show a closer resemblance to GEOSECS tritium data than to TTO tritium data in the upper ocean. This is because the transient tracer tritium was responding on a 10-year time scale during GEOSECS and a 20-year time scale during TTO. The steady state tracer 228Ra should always respond on a time scale of 8 years. Thus the 228Ra data obtained on TTO should provide a means to extend the features of the GEOSECS tritium field to the regions of the TTO study. The 226Ra data are of high enough quality to identify features associated with different water masses. Changes in the positions of the deep-water masses since the GEOSECS cruise are revealed by the 226Radata.

  10. An alternative method for Ra determinations in water

    SciTech Connect

    Loyd, D.H.; Drake, E.N. 2d.

    1989-07-01

    Concentrations of /sup 226/Ra and /sup 224/Ra in 13 wells distributed throughout McCulloch and Mason counties in the Hickory Aquifer of the Llano Uplift Region of West-Central Texas are reported. Measurable alpha-particle activity is present in all wells, with seven wells having /sup 226/Ra radioactivity concentrations greater than 185 Bq m-3 (5 pCi L-1). An alternative methodology for measuring /sup 226/Ra, /sup 224/Ra and /sup 228/Ra is described. The EPA-approved methodology for estimating total Ra is shown to be invalid for aquifers containing significant levels of /sup 224/Ra. Alpha-particle activity measurements made in the interval of 12 to 300 h after Ra isolation lead to self-consistent solutions for radioactivity concentrations of /sup 226/Ra and /sup 224/Ra, with negligible contributions from /sup 228/Ra. Radioactivity concentrations of /sup 228/Ra can be calculated from grow-in terms for this isotope used with alpha-particle activity measurements at post-isolation times significantly longer than 800 h. Comparison of the /sup 226/Ra radioactivity concentration with that reported previously by the Texas Department of Health for a single well indicates acceptable agreement. However, the radioactivity concentration attributable to /sup 228/Ra for the same well was found to be in significant disagreement with the Texas Department of Health value.

  11. 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

  12. 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.

  13. Atypical CF and CF related diseases.

    PubMed

    Kerem, Eitan

    2006-01-01

    The clinical characteristics of atypical CF are: symptoms that may start in infancy but the disease become clinically significant only after 10 years of age, survival into adulthood, chronic sinopulmonary disease, pancreatic sufficiency, and sweat chloride <60 meq/L. Other patients may present with single organ involvement such as CBAVD, biliary cirrhosis and portal hypertension, chronic or recurrent pancreatitis, giant nasal polyposis or hypochloremic alkalosis. It is recommended to refer such patients for CFTR genotyping, however, absence of known common mutation does not rule out CFTR associated disease, since mutations causing atypical CF are rare and whole genome scan is required for their identification. Nasal PD measurements may be helpful to establish the diagnosis of these patients; however, measurements might be also atypical. Several explanations have been suggested to explain the atypical CF disease.

  14. [Atypical onset cryoglobulinemia: case report].

    PubMed

    Consolo, M; Amoroso, A; D'Amico, G; La Rosa, L; Vinci, M

    2012-01-01

    Cryoglobulinemia is a disease mediated by antibodies with the property to precipitate at temperatures below 37°C. It can be distinguished into a primitive form (also referred to as 'essential mixed cryoglobulinemia'), and a secondary form. In the essential mixed variant a key role is played by HCV infection. The pathogenesis of mixed cryoglobulinemia is mediated by immune complexes that are the most important cause of the vasculitic phenomena, typical of the disease. However, the severity of the clinical manifestations is not always related to the serum levels of cryoglobulins and immune complexes. In our case report, a 46-year old man came to our observation with asymmetric diffuse and invalidating arthralgies, with both substitutive and additive behaviour, located at pelvic girdle, inferior limbs and elbows, associated to skin lesion vascultis-like. The remote pathological anamnesis was characterized by a previous surgically treated non-Hodgkin lymphoma, and HCV infection. Despite several attempts were done, it was not possible to reveal cryoglobulins, nor reumatoid factor in the serum. Cryoglobulins resulted positive only after the third day of hospitalization, along with a new fever attack and a worsening of the vasculitic manifestations. In conclusion, this case demonstrated that cryoglobulinemia can occur with a totally atypical sequence of clinical manifestations which can be present before and in absence of the typical laboratory proofs.

  15. SB-RA-2001 inhibits bacterial proliferation by targeting FtsZ assembly.

    PubMed

    Singh, Dipty; Bhattacharya, Anusri; Rai, Ankit; Dhaked, Hemendra Pal Singh; Awasthi, Divya; Ojima, Iwao; Panda, Dulal

    2014-05-13

    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.

  16. Domain walls in antiferromagnetically coupled multilayer films.

    PubMed

    Hellwig, Olav; Berger, Andreas; Fullerton, Eric E

    2003-11-07

    We report experimentally observed magnetic domain-wall structures in antiferromagnetically coupled multilayer films with perpendicular anisotropy. Our studies reveal a first-order phase transition from domain walls with no net moment to domain walls with ferromagnetic cores. The transition originates from the competition between dipolar and exchange energies, which we tune by means of layer thickness. Although observed in a synthetic antiferromagnetic system, such domain-wall structures may be expected to occur in A-type antiferromagnets with anisotropic exchange coupling.

  17. Behavior of atypical amphiphilic molecules

    NASA Astrophysics Data System (ADS)

    Ko, John

    1997-08-01

    The physical behavior of several atypical amphiphilic molecules was studied in various environments including micelles, model bilayer membranes, and emulsions. The molecules under investigation were nor-chenodeoxycholic acid (nor-CDCA), ursodeoxycholic acid (UDCA), sphingosine (Sp), sphingosine hydrochloride (SpċHCl), and tetrahydrolipstatin (THL). The bile acids, nor-CDCA and UDCA, were studied using 13C-Nuclear Magnetic Resonance ([13C) -NMR) in micelles of taurocholate and in bilayers of phosphatidylcholine. The pK a values of the bile acids in each environment were determined by [13C) -NMR and are as follows: 6.08 ±.03 for nor-CDCA and 6.27 ±.01 for UDCA in micelles, and 7.04 ± 12 for nor-CDCA and 6.89 ±.05 for UDCA in vesicles. Using line shape analysis, the transbilayer movement rate at 36oC for nor-CDCA and UDCA was calculated to be 580 sec--1 and 409 sec-1, respectively. [13C) -NMR titration of Sp gave pK a values of 9.09 ±.02 in micelles and 9.69 ±.21 in bilayers. Differential scanning calorimetry (DSC) and X-ray diffraction were used to establish the Spċwater and SpċHClċwater phase diagrams. Anhydrous and hydrated samples ranging from 5- 90% water were analyzed. The DSC thermograms traced out the transition temperatures of each molecule while the X- ray diffraction patterns revealed their chain and crystalline lattice packing structures. In general, sphingosine exists as a hydrated crystal with β packing phase below 43oC and melts into an Lα phase. Sphingosine hydrochloride, however, exists as a gel phase (L_beta or /beta/sp') below 42oC that swells to 61% hydration. At low water concentrations (0-64%), a lamellar liquid crystal phase (L_alpha) is formed above the chain melting transition of 42oC. At medium concentration (65%), a Hexagonal I phase is present, and at high water concentrations (66-90%), a micellar phase is present. THL, a specific inhibitor of lipases, was analyzed with [ 13C) -NMR to study its behavior in various environments

  18. Atypical Pityriasis Rosea with Unilateral Presentation

    PubMed Central

    Fadaei, Fahameh; Badakhsh, Mahin; Balouchi, Abbas

    2016-01-01

    Pityriasis Rosea (PR) is a common skin disease and characterized by generalized scaly eruptions typically on the trunk and proximal extremities. Atypical presentations of PR are common and can be a diagnostic challenge for clinicians. Here we present a case of a 26-year-old female who presented with a sudden onset of several asymptomatic, erythematous and scaly plaques on her trunk. Plaques sized 0.5-1cm in diameter that were distributed unilaterally (right side) on her chest, back and axilla. Atypical cases of PR are fairly common and less readily recognized. Careful history, clinical evaluation and follow-up are important to avoid misdiagnosis of PR and physicians should be aware of PR variants so that appropriate management and reassurance can be offered. For atypical eruptions without a definite diagnosis, it is safer to consider lesional skin biopsy. PMID:28208986

  19. [Atypical antipsychotic-induced weight gain].

    PubMed

    Godlewska, Beata R; Olajossy-Hilkesberger, Luiza; Marmurowska-Michałowska, Halina; Olajossy, Marcin; Landowski, Jerzy

    2006-01-01

    Introduction of a new group of antipsychotic drugs, called atypical because of the proprieties differing them from classical neuroleptics, gave hope for the beginning of a new era in treatment of psychoses, including schizophrenia. Different mechanisms of action not only resulted in a broader spectrum of action and high efficacy but also in a relative lack of extrapiramidal symptoms. However, atypical neuroleptics are not totally free from adverse effects. Symptoms such as sedation, metabolic changes and weight gain, often very quick and severe - present also in the case of classical drugs, but put to the background by extrapiramidal symptoms--have become prominent. Weight gain is important both from the clinical and subjective point of view--as associated with serious somatic consequences and as a source of enormous mental distress. These problems are addressed in this review, with the focus on weight gain associated with the use of specific atypical neuroleptics.

  20. Do A-type stars flare?

    NASA Astrophysics Data System (ADS)

    Pedersen, M. G.; Antoci, V.; Korhonen, H.; White, T. R.; Jessen-Hansen, J.; Lehtinen, J.; Nikbakhsh, S.; Viuho, J.

    2017-04-01

    For flares to be generated, stars have to have a sufficiently deep outer convection zone (F5 and later), strong large-scale magnetic fields (Ap/Bp-type stars) or strong, radiatively driven winds (B5 and earlier). Normal A-type stars possess none of these and therefore should not flare. Nevertheless, flares have previously been detected in the Kepler light curves of 33 A-type stars and interpreted to be intrinsic to the stars. Here, we present new and detailed analyses of these 33 stars, imposing very strict criteria for the flare detection. We confirm the presence of flare-like features in 27 of the 33 A-type stars. A study of the pixel data and the surrounding field of view reveals that 14 of these 27 flaring objects have overlapping neighbouring stars and five stars show clear contamination in the pixel data. We have obtained high-resolution spectra for 2/3 of the entire sample and confirm that our targets are indeed A-type stars. Detailed analyses revealed that 11 out of 19 stars with multiple epochs of observations are spectroscopic binaries. Furthermore, and contrary to previous studies, we find that the flares can originate from a cooler, unresolved companion. We note the presence of Hα emission in eight stars. Whether this emission is circumstellar or magnetic in origin is unknown. In summary, we find possible alternative explanations for the observed flares for at least 19 of the 33 A-type stars, but find no truly convincing target to support the hypothesis of flaring A-type stars.

  1. An atypical monomelic presentation of Mazabraud syndrome

    PubMed Central

    Wan, Jun; He, Hong-Bo; Liao, Qian-De; Zhang, Can

    2014-01-01

    Mazabraud syndrome is a rare condition characterized by a combination of fibrous dysplasia and intramuscular myxomas. In Mazabraud syndrome, the distribution of fibrous dysplasia is mostly polyomelic and frequently located in the femur, with myxomas adjacent to the fibrous dysplasia lesion of bone (mostly in the quadriceps muscle). However, when presented as atypical clinical features, patients of Mazabraud syndrome is either misdiagnosed or difficult to diagnose. We report an atypical monomelic case of Mazabraud syndrome in the right upper arm and discuss the difficulties in making an accurate diagnosis. PMID:25143651

  2. Atypical RNAs in the coelacanth transcriptome.

    PubMed

    Nitsche, Anne; Doose, Gero; Tafer, Hakim; Robinson, Mark; Saha, Nil Ratan; Gerdol, Marco; Canapa, Adriana; Hoffmann, Steve; Amemiya, Chris T; Stadler, Peter F

    2014-09-01

    Circular and apparently trans-spliced RNAs have recently been reported as abundant types of transcripts in mammalian transcriptome data. Both types of non-colinear RNAs are also abundant in RNA-seq of different tissue from both the African and the Indonesian coelacanth. We observe more than 8,000 lincRNAs with normal gene structure and several thousands of circularized and trans-spliced products, showing that such atypical RNAs form a substantial contribution to the transcriptome. Surprisingly, the majority of the circularizing and trans-connecting splice junctions are unique to atypical forms, that is, are not used in normal isoforms.

  3. A rapid and inexpensive method for 226Ra and 228Ra measurements of high TDS groundwaters.

    PubMed

    El-Shrakawy, A; Ebaid, Y Y; Burnett, W C; Aldaihan, Soaad K

    2013-07-01

    A series of laboratory-scale studies was conducted by preconcentrating (226)Ra from spiked water test samples using Purolite ion-exchange resin to evaluate the adsorption efficiency of the resin under varying conditions. After removing the resin from the columns, it was sealed in gas-tight containers and measured via gamma spectrometry. The Purolite resin showed high radium uptake and retention from natural waters in the presence of high iron and total dissolved solids (TDS). This procedure allowed us to process a large number of high TDS samples at a typical rate of 15 samples/day using three germanium detectors. Quality assurance and method validation have been achieved by analyzing selected groundwater samples, with different (226)Ra activities and high TDS values, and comparing the results to those using alpha spectrometry with a (133)Ba yield tracer. There was very good agreement between the obtained (226)Ra activities by both methods.

  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. 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…

  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. [Antibiotherapy in children with atypical bacterial infections].

    PubMed

    Haas, H

    2005-04-01

    Atypical bacteria responsible for infections in children are mainly Mycoplasma pneumoniae, Chlamydia pneumoniae and Legionella pneumophila. Atypical pneumonia is a frequent disease in children. Until recently, the outcome was thought to be rather benign and antibiotherapy to have only a minor impact on the prognosis. Recent studies have demonstrated that M. pneumoniae and C. pneumoniae were involved in a variety of infections, including acute upper airway disease, otitis and pharyngitis under five. Antibiotherapy was proven able to decrease the rate of complications and recurrence, notably episodes of wheezing and exacerbations of asthma. Atypical bacteria infections may be severe in immunocompromised children and children with underlying disease such as sickle cell anaemia. Whenever bacteriological documentation is lacking, one of the critical issues in choosing an antibiotic is to consider its activity against Streptococcus pneumoniae, especially in lower respiratory tract infections. The main available molecules are reviewed and discussed, with a special emphasis on ketolides, a newer family of molecules active on both atypical bacteria and S. pneumoniae.

  8. 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...

  9. Atypical anti-glomerular basement membrane disease

    PubMed Central

    Troxell, Megan L.; Houghton, Donald C.

    2016-01-01

    Background Anti-glomerular basement membrane (anti-GBM) disease classically presents with aggressive necrotizing and crescentic glomerulonephritis, often with pulmonary hemorrhage. The pathologic hallmark is linear staining of GBMs for deposited immunoglobulin G (IgG), usually accompanied by serum autoantibodies to the collagen IV alpha-3 constituents of GBMs. Methods Renal pathology files were searched for cases with linear anti-GBM to identify cases with atypical or indolent course. Histopathology, laboratory studies, treatment and outcome of those cases was reviewed in detail. Results Five anti-GBM cases with atypical clinicopathologic features were identified (accounting for ∼8% of anti-GBM cases in our laboratory). Kidney biopsies showed minimal glomerular changes by light microscopy; one patient had monoclonal IgG deposits in an allograft (likely recurrent). Three patients did not have detectable serum anti-GBM by conventional assays. Three patients had indolent clinical courses after immunosuppressive treatment. One patient, untreated after presenting with brief mild hematuria, re-presented after a short interval with necrotizing and crescentic glomerulonephritis. Conclusions Thorough clinicopathologic characterization and close follow-up of patients with findings of atypical anti-GBM on renal biopsy are needed. Review of the literature reveals only rare well-documented atypical anti-GBM cases to date, only one of which progressed to end-stage kidney disease. PMID:26985371

  10. 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.…

  11. On Atypical Dynamics of Reticulated Systems

    NASA Astrophysics Data System (ADS)

    Boutin, C.; Chesnais, C.; Hans, S.

    2010-09-01

    This paper deals with the dynamics of reticulated beams. Through the homogenization method of periodic discrete media and a systematic use of scaling, the existence of atypical behaviours is established. These latter appear when the elastic moment is balanced by the rotation inertia, and/or when macro dynamics occurs conjointly with inner local dynamics.

  12. Atypical pathogens and respiratory tract infections.

    PubMed

    Blasi, F

    2004-07-01

    The atypical respiratory pathogens Chlamydia pneumoniae, Mycoplasma pneumoniae and Legionella pneumophila are now recognised as a significant cause of acute respiratory-tract infections, implicated in community-acquired pneumonia, acute exacerbations of chronic bronchitis, asthma, and less frequently, upper respiratory-tract infections. Chronic infection with C. pneumoniae is common among patients with chronic obstructive pulmonary disease and may also play a role in the natural history of asthma, including exacerbations. The lack of a gold standard for diagnosis of these pathogens still handicaps the current understanding of their true prevalence and role in the pathogenesis of acute and chronic respiratory infections. While molecular diagnostic techniques, such as polymerase chain reaction, offer improvements in sensitivity, specificity and rapidity over culture and serology, the need remains for a consistent and reproducible diagnostic technique, available to all microbiology laboratories. Current treatment guidelines for community-acquired pneumonia recognise the importance of atypical respiratory pathogens in its aetiology, for which macrolides are considered suitable first-line agents. The value of atypical coverage in antibiotic therapy for acute exacerbations of chronic bronchitis and exacerbations of asthma is less clear, while there is no evidence to suggest that atypical pathogens should be covered in antibiotic treatment of upper respiratory-tract infections.

  13. 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.

  14. 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

  15. 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.

  16. 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…

  17. 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…

  18. Atypical leiomyoma: An unusual variant of cutaneous pilar leiomyoma.

    PubMed

    Nocito, Mabel Jimena; Lustia, María Marcela; Luna, Paula Carolina; Cañadas, Nadia Guadalupe; Castellanos Posse, María Laura; Marchesi, Carolina; Carabajal, Graciela; Mazzini, Miguel Angel

    2009-03-15

    Cutaneous atypical leiomyoma is an unusual benign tumor arising from arrector pili muscle that shares histological features with uterine atypical or symplastic leiomyoma: atypical cellularity with pleomorphic nuclei but minimal or no mitosis. Six other cases have been reported so far and, in spite of its name and of being a smooth muscle proliferation, no recurrences nor metastasis have been reported.

  19. Atypical Patterns of Early Attachment: Theory, Research, and Current Directions.

    ERIC Educational Resources Information Center

    Barnett, Douglas; Vondra, Joan I.

    1999-01-01

    Reviews conceptual background on atypical patterns of early attachments, including basic theoretical terms and constructs of attachment theory; the nature and function of the original classification system; notions of "exceptional cases" or "atypical patterns"; and implications of atypicality for both a classification system of attachment behavior…

  20. A Comparative Study of Drosophila and Human A-Type Lamins

    PubMed Central

    Schulze, Sandra R.; Curio-Penny, Beatrice; Speese, Sean; Dialynas, George; Cryderman, Diane E.; McDonough, Caitrin W.; Nalbant, Demet; Petersen, Melissa; Budnik, Vivian; Geyer, Pamela K.; Wallrath, Lori L.

    2009-01-01

    Nuclear intermediate filament proteins, called lamins, form a meshwork that lines the inner surface of the nuclear envelope. Lamins contain three domains: an N-terminal head, a central rod and a C-terminal tail domain possessing an Ig-fold structural motif. Lamins are classified as either A- or B-type based on structure and expression pattern. The Drosophila genome possesses two genes encoding lamins, Lamin C and lamin Dm0, which have been designated A- and B-type, respectively, based on their expression profile and structural features. In humans, mutations in the gene encoding A-type lamins are associated with a spectrum of predominantly tissue-specific diseases known as laminopathies. Linking the disease phenotypes to cellular functions of lamins has been a major challenge. Drosophila is being used as a model system to identify the roles of lamins in development. Towards this end, we performed a comparative study of Drosophila and human A-type lamins. Analysis of transgenic flies showed that human lamins localize predictably within the Drosophila nucleus. Consistent with this finding, yeast two-hybrid data demonstrated conservation of partner-protein interactions. Drosophila lacking A-type lamin show nuclear envelope defects similar to those observed with human laminopathies. Expression of mutant forms of the A-type Drosophila lamin modeled after human disease-causing amino acid substitutions revealed an essential role for the N-terminal head and the Ig-fold in larval muscle tissue. This tissue-restricted sensitivity suggests a conserved role for lamins in muscle biology. In conclusion, we show that (1) localization of A-type lamins and protein-partner interactions are conserved between Drosophila and humans, (2) loss of the Drosophila A-type lamin causes nuclear defects and (3) muscle tissue is sensitive to the expression of mutant forms of A-type lamin modeled after those causing disease in humans. These studies provide new insights on the role of lamins in

  1. Natural radioactivity of 226Ra and 228Ra in thermal and mineral waters in Croatia.

    PubMed

    Bituh, Tomislav; Marovic, Gordana; Petrinec, Branko; Sencar, Jasminka; Franulovic, Iva

    2009-01-01

    Thermal waters are known as valuable natural resources of a country. They contain certain degree of natural radioactivity attributable to the elements of the uranium and thorium natural decay series. Among these elements, the most radiotoxic and the most important is radium that exists in several isotopic forms (226Ra and 228Ra). The focus of attention was the content of radium in samples of thermal and mineral spring water from several spas in Croatia. These waters are mainly used for medical, bathing and recreational purposes, and some of them are used for drinking. Measured activity concentrations of 226Ra ranged from 87 to 6200 mBq l(-1) which, in some springs, exceed the maximal permissible level of 1 Bq l(-1) for drinking water. Measured activity concentrations of 228Ra ranged from 23 to 3480 mBq l(-1). The study showed that radium content for the investigated thermal and mineral waters is below the levels at which negative consequences would arise due to ingestion.

  2. 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

  3. Drugs or disease: evaluating salivary function in RA patients.

    PubMed

    Torres, Sandra Regina; Pedrazas, Carlos Henrique Silva; Correia, Marcos Paulo Veloso; Azevedo, Mario Newton Leitão de; Zamprogno, Thaís; Silva, Arley; Gonçalves, Lucio Souza; Papi, José Angelo de Souza

    2016-10-10

    Oral complications of RA may include temporomandibular joint disorders, mucosa alterations and symptoms of dry mouth. The aim of this study was to evaluate the salivary gland function of subjects with rheumatoid arthritis (RA) comparing it to healthy controls. Subjects with other systemic conditions known to affect salivary functions were excluded. A questionnaire was applied for the evaluation of xerostomia. Resting and chewing-stimulated salivary flow rates (SFR) were obtained under standard conditions. There were 145 subjects included of the study (104 RA and 38 controls). About 66.7% of the RA subjects and 2.4% in control group presented xerostomia. The median resting SFR were 0.24 ml/min for RA subjects and 0.40 mL/min for controls (p = 0.04). The median stimulated SFR were 1.31 mL/min for RA subjects and 1.52 ml/min for controls (p = 0.33). No significant differences were found between resting and stimulated SFR of RA subjects not using xerogenic medications and controls. There was significantly higher number of subjects presenting hyposalivation in the RA group than among controls, even when subjects using xerogenic medications were eliminated from the analysis. In conclusion, hyposalivation and xerostomia were more frequent among RA subjects not using xerogenic medication than among controls, although there were no significant differences in the median SFR between groups.

  4. Heavy cigarette smoking is strongly associated with rheumatoid arthritis (RA), particularly in patients without a family history of RA

    PubMed Central

    Hutchinson, D; Shepstone, L; Moots, R; Lear, J; Lynch, M

    2001-01-01

    OBJECTIVES—To investigate the potential relation between cumulative exposure to cigarette smoking in patients with or without rheumatoid arthritis (RA) and a positive family history of the disease.
METHODS—239 outpatient based patients with RA were compared with 239 controls matched for age, sex, and social class. A detailed smoking history was recorded and expressed as pack years smoked. Conditional logistic regression was used to calculate the association between RA and pack years smoked. The patients with RA were also interviewed about a family history of disease and recorded as positive if a first or second degree relative had RA. The smoking history at the time of the study of the patients with RA with or without a family history of the disease was compared directly with that of their respective controls. Patients with RA with or without a family history of the disease were also compared retrospectively for current smoking at the time of disease onset.
RESULTS—An increasing association between increased pack years smoked and RA was found. There was a striking association between heavy cigarette smoking and RA. A history for 41-50 pack years smoked was associated with RA (odds ratio (OR) 13.54, 95% confidence interval (95% CI) 2.89 to 63.38; p<0.001). The association between ever having smoked and RA was modest (OR 1.81, CI 1.22 to 2.19; p=0.002). Furthermore, cigarette smoking in the patients with RA without a positive family history of RA was more prevalent than in the patients with a positive family history of RA for ever having smoked (72% v 54%; p=0.006), the number of pack years smoked (median 25.0 v 4.0; p<0.001), and for smoking at the time of disease onset (58% v 39%; p=0.003).
CONCLUSIONS—Heavy cigarette smoking, but not smoking itself, is strongly associated with RA requiring hospital follow up and is markedly more prevalent in patients with RA without a family history of RA.

 PMID:11171682

  5. 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

  6. Body piercing complicated by atypical mycobacterial infections.

    PubMed

    Ferringer, Tammie; Pride, Howard; Tyler, William

    2008-01-01

    Body piercing is a growing trend, especially in young people, but the literature on complications of piercing consists mostly of case reports involving ear piercing. Previous reported complications of piercing include contact dermatitis, keloids, traumatic tearing, viral transmission, and bacterial infections. We report two patients who presented with atypical mycobacterial infections of body piercing sites. It is important to recognize the association of piercing and mycobacterial infections so that tissue can be obtained for histopathologic examination and appropriate culture.

  7. 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

  8. Atypical Histiocytosis in Red Squirrels (Sciurus vulgaris).

    PubMed

    Smith, S H; Stevenson, K; Del-Pozo, J; Moss, S; Meredith, A

    2017-03-17

    Four red squirrels (Sciurus vulgaris) were subjected to necropsy examination over a 3-year period as part of a broader surveillance study. The squirrels presented with cutaneous, subcutaneous and/or internal swellings and nodules that consisted microscopically of sheets of atypical round cells and multinucleated giant cells. There was moderate anisokaryosis with rare mitoses. Nuclei ranged from oval to indented or C-shaped and some were bizarre, twisted or multilobulated. Many giant cells also had a bizarre morphology, with anisokaryosis within individual cells. Giant cell nuclei were often multilobulated, ring-shaped or segmented. Affected internal organs varied depending on the squirrel, but included lymph node, kidney, intestinal tract and lungs. Representative lesions from each of the four squirrels were negative for acid-fast organisms. Formalin-fixed tissues from all four squirrels and ethanol-fixed tissue from one animal were negative for Mycobacterium by polymerase chain reaction. Immunohistochemically, the majority of mononuclear and multinucleated giant cells in all four squirrels strongly expressed vimentin and class II molecules of the major histocompatibility complex. Otherwise, the atypical mononuclear and multinucleated cells were negative for CD3, Pax-5, Mac387, CD18 and E-cadherin. Based on the combination of cellular morphology, arrangement and immunophenotype, a novel form of atypical histiocytosis is considered most likely in these squirrels, although the exact origin and triggering factors remain uncertain.

  9. Immune memory in CD4+ CD45RA+ T cells.

    PubMed Central

    Richards, D; Chapman, M D; Sasama, J; Lee, T H; Kemeny, D M

    1997-01-01

    This study addresses the question of whether human peripheral CD4+ CD45RA+ T cells possess antigen-specific immune memory. CD4+ CD45RA+ T cells were isolated by a combination of positive and negative selection. Putative CD4+ CD45RA+ cells expressed CD45RA (98.9%) and contained < 0.1% CD4+ CD45RO+ and < 0.5% CD4+ CD45RA+ CD45RO+ cells. Putative CD45RO+ cells expressed CD45RO (90%) and contained 9% CD45RA+ CD45RO+ and < 0.1% CD4+ CD45RA+ cells. The responder frequency of Dermatophagoides pteronyssinus-stimulated CD4+ CD45RA+ and CD4+ CD45RO+ T cells was determined in two atopic donors and found to be 1:11,314 and 1:8031 for CD4+ CD45RA+ and 1:1463 and 1:1408 for CD4+ CD45RO+ T cells. The responder frequencies of CD4+ CD45RA+ and CD4+ CD45RO+ T cells from two non-atopic, but exposed, donors were 1:78031 and 1:176,903 for CD4+ CD45RA+ and 1:9136 and 1:13,136 for CD4+ CD45RO+ T cells. T cells specific for D. pteronyssinus were cloned at limiting dilution following 10 days of bulk culture with D. pteronyssinus antigen. Sixty-eight clones were obtained from CD4+ CD45RO+ and 24 from CD4+ CD45RA+ T cells. All clones were CD3+ CD4+ CD45RO+ and proliferated in response to D. pteronyssinus antigens. Of 40 clones tested, none responded to Tubercule bacillus purified protein derivative (PPD). No difference was seen in the pattern of interleukin-4 (IL-4) or interferon-gamma (IFN-gamma) producing clones derived from CD4+ CD45RA+ and CD4+ CD45RO+ precursors, although freshly isolated and polyclonally activated CD4+ CD45RA+ T cells produced 20-30-fold lower levels of IL-4 and IFN-gamma than their CD4+ CD45RO+ counterparts. Sixty per cent of the clones used the same pool of V beta genes. These data support the hypothesis that immune memory resides in CD4+ CD45RA+ as well as CD4+ CD45RO+ T cells during the chronic immune response to inhaled antigen. PMID:9301520

  10. Transpupillary thermotherapy for atypical central serous chorioretinopathy

    PubMed Central

    Kawamura, Ryosuke; Ideta, Hidenao; Hori, Hideyuki; Yuki, Kenya; Uno, Tsuyoshi; Tanabe, Tatsurou; Tsubota, Kazuo; Kawasaki, Tsutomu

    2012-01-01

    Background Central serous chorioretinopathy (CSC) has been traditionally treated with laser photocoagulation. We thought that transpupillary thermotherapy (TTT) utilizing a lower temperature than that of conventional laser photocoagulation might minimize permanent retinal and choroidal damage. Studies suggest that undesirable effects on vision due to TTT are minimal even if it is applied to foveal and/or parafoveal lesions when TTT requires a larger irradiation spot. The aim of this study was to evaluate the efficacy of TTT in the management of atypical CSC. Methods We defined atypical CSC as bullous retinal detachment with diffuse or several leakages, severe leakage with fibrin formation under serous retinal detachment, or leakage within a pigment epithelium detachment. Eight consecutive patients with atypical CSC underwent visual acuity testing, ophthalmic examination, color photography, fluorescein angiography, and optical coherence tomography to evaluate the results of transpupillary thermotherapy. Retreatment of atypical CSC was based on ophthalmic examination, optical coherence tomography, and fluorescein angiography. TTT was performed on the leaking spots shown in fluorescein angiography, with a power of 50–250 mW, spot size of 500–1200 μm, and exposure time of 13–60 seconds to minimize retinal damage. Results In five of eight affected eyes, serous detachments completely resolved within 1 month after the initial TTT. One eye had persistent subretinal fluid and required a second TTT treatment. Two eyes showed no resolution of CSC and were treated by conventional photocoagulation. Initial best-corrected visual acuity (BCVA) ranged from 20/600 to 20/20 (mean, 20/40; median, 20/30). Final BCVA ranged from 20/200 to 20/20 (mean, 20/25; median, 20/20). BCVA improved in all cases. Only two eyes with persistent subretinal fibrin and existing retinal pigment epithelial alternations in macular area showed limited improvement of BCVA despite the absence of

  11. 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

  12. 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

  13. Improved limit on the Ra225 electric dipole moment

    DOE PAGES

    Bishof, Michael; Parker, Richard H.; Bailey, Kevin G.; ...

    2016-08-03

    In this study, 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.

  14. 226Ra determination in phosphogypsum by alpha-particle spectrometry

    NASA Astrophysics Data System (ADS)

    Aguado, J. L.; Bolívar, J. P.; García-Tenorio, R.

    1999-01-01

    A radiochemical method for226Ra determination by alpha-particle spectrometry in environmental samples has been developed in our laboratory. The method has been validated by measurements in samples with known concentrations of this radionuclide and it has been applied in studies related to226Ra behaviour in phosphogypsum (the main by-product of producing phosphoric acid from phosphate rocks).

  15. Genetic, immunological, and clinical features of patients with bacterial and fungal infections due to inherited IL-17RA deficiency.

    PubMed

    Lévy, Romain; Okada, Satoshi; Béziat, Vivien; Moriya, Kunihiko; Liu, Caini; Chai, Louis Yi Ann; Migaud, Mélanie; Hauck, Fabian; Al Ali, Amein; Cyrus, Cyril; Vatte, Chittibabu; Patiroglu, Turkan; Unal, Ekrem; Ferneiny, Marie; Hyakuna, Nobuyuki; Nepesov, Serdar; Oleastro, Matias; Ikinciogullari, Aydan; Dogu, Figen; Asano, Takaki; Ohara, Osamu; Yun, Ling; Della Mina, Erika; Bronnimann, Didier; Itan, Yuval; Gothe, Florian; Bustamante, Jacinta; Boisson-Dupuis, Stéphanie; Tahuil, Natalia; Aytekin, Caner; Salhi, Aicha; Al Muhsen, Saleh; Kobayashi, Masao; Toubiana, Julie; Abel, Laurent; Li, Xiaoxia; Camcioglu, Yildiz; Celmeli, Fatih; Klein, Christoph; AlKhater, Suzan A; Casanova, Jean-Laurent; Puel, Anne

    2016-12-20

    Chronic mucocutaneous candidiasis (CMC) is defined as recurrent or persistent infection of the skin, nails, and/or mucosae with commensal Candida species. The first genetic etiology of isolated CMC-autosomal recessive (AR) IL-17 receptor A (IL-17RA) deficiency-was reported in 2011, in a single patient. We report here 21 patients with complete AR IL-17RA deficiency, including this first patient. Each patient is homozygous for 1 of 12 different IL-17RA alleles, 8 of which create a premature stop codon upstream from the transmembrane domain and have been predicted and/or shown to prevent expression of the receptor on the surface of circulating leukocytes and dermal fibroblasts. Three other mutant alleles create a premature stop codon downstream from the transmembrane domain, one of which encodes a surface-expressed receptor. Finally, the only known missense allele (p.D387N) also encodes a surface-expressed receptor. All of the alleles tested abolish cellular responses to IL-17A and -17F homodimers and heterodimers in fibroblasts and to IL-17E/IL-25 in leukocytes. The patients are currently aged from 2 to 35 y and originate from 12 unrelated kindreds. All had their first CMC episode by 6 mo of age. Fourteen patients presented various forms of staphylococcal skin disease. Eight were also prone to various bacterial infections of the respiratory tract. Human IL-17RA is, thus, essential for mucocutaneous immunity to Candida and Staphylococcus, but otherwise largely redundant. A diagnosis of AR IL-17RA deficiency should be considered in children or adults with CMC, cutaneous staphylococcal disease, or both, even if IL-17RA is detected on the cell surface.

  16. Radium Ra 223 dichloride in castration-resistant prostate cancer.

    PubMed

    Joung, J Y; Ha, Y S; Kim, I Y

    2013-08-01

    Radium Ra 223 dichloride (Xofigo®, formerly Alpharadin) is one of the representative α-particle-emitting isotopes that delivers radiation with a higher biological effect to a more localized area. Preclinical studies in mouse, rat and canine models have demonstrated that radium Ra 223 dichloride has a definite skeletal affinity and antitumor effect with a relatively low toxicity on bone marrow. More recently, in a large randomized phase III trial (ALSYMPCA), patients with bone metastasis and castration-resistant prostate cancer (CRPC) received six cycles of 50 kBq/kg of radium Ra 223 dichloride in 4-week intervals. In these men, radium Ra 223 dichloride improved the median overall survival by 3.6 months when compared to the placebo group. Collectively, these results suggest that radium Ra 223 dichloride is a promising candidate for managing bone metastases in patients with CRPC.

  17. 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

  18. Atypical neoglottis after supracricoid laryngectomy: a morphological and functional analysis.

    PubMed

    Alicandri-Ciufelli, Matteo; Piccinini, Alessia; Bergamini, Giuseppe; Ruberto, Marco; Ghidini, Angelo; Marchioni, Daniele; Presutti, Livio

    2011-07-01

    The objective of this study was to analyze atypical neoglottis after supracricoid subtotal laryngectomy (SSL) from a morphological and functional point-of-view using retrospective case series reviewin a Tertiary university referral center setting. From May 2003 until January 2010, 106 patients underwent SSL (CHEP, CHP, THEP, THP) for laryngeal cancer, in the Otolaryngology Department of the University Hospital of Modena. We performed a retrospective analysis of recorded videos in our database of patients who underwent SSL. Patients with atypical neoglottis were included in the study. Six patients with atypical neoglottis were identified and morphologically evaluated. The functional outcomes were collected and analyzed. Atypical neoglottis may form after SSL, in particular in the case of CHEP. In most cases, these atypical conformations are due to anomalous positioning of the epiglottis, or involvement of the lateral pharyngeal wall in the sphincteric and vibratory function of the neoglottis. Atypical neoglottis formation seems to guarantee adequate functional outcomes in terms of vocal and swallowing performance.

  19. Large-eddy simulation and deduced scaling analysis of Rayleigh Bénard convection up to Ra = 109**

    NASA Astrophysics Data System (ADS)

    Peng, S.-H.; Hanjalic, K.; Davidson, L.

    Large-eddy simulation of turbulent Rayleigh Bénard (RB) convection has been performed for a 6:1:6 open-ended domain for Rayleigh numbers ranging from 6.3 × 105 to 109 at Prandtl number of Pr = 0.71. The scaling analysis based on the LES data shows that the heat transfer follows a single relation of Nu = 0.162 Ra0.286, which is consistent with the scaling law for the hard turbulence regime reported in several earlier experimental and DNS studies. The present LES also supports some earlier experimental and DNS findings that most of characteristic parameters can be scaled reasonably well with Ra number in the considered Ra number range using a single relation. Nonetheless, it is found that the scaling of several quantities shows a sensible offset from a single relation, and could be fitted better with the separate scaling relations for the soft and hard convective turbulence transitioned at about Ra = 4 × 107. It has been argued that the transition, reflected in the scaling relation, may be attributed to the increasing ‘containing effect’ of the plume leaving the horizontal wall on the plume approaching the wall at large Ra numbers in the near-wall region. **This paper is a modified version from the paper presented at the Forth International Symposium of Turbulence and Shear Flow Phenomena (Williamsburg, Virginia, 27 29 June 2005).

  20. Prognosis of pneumocystis pneumonia complicated in patients with rheumatoid arthritis (RA) and non-RA rheumatic diseases.

    PubMed

    Yoshida, Yuji; Takahashi, Yuko; Minemura, Nobuyoshi; Ueda, Yo; Yamashita, Hiroyuki; Kaneko, Hiroshi; Mimori, Akio

    2012-08-01

    Clinical presentation of pneumocystis pneumonia (PCP) during immunosuppressive therapy for rheumatic diseases was compared between patients with rheumatoid arthritis (RA; n = 7) and those without RA (non-RA; n = 12) based on a chart review. Both RA and non-RA patients with PCP were treated with methotrexate (n = 7) combined with steroids (n = 6) and/or biologics (n = 4). RA-PCP patients were found to have a higher mortality rate than non-RA-PCP patients (3/7 vs. 0/12, respectively; p = 0.036) due to a later exacerbation of interstitial pneumonia and a higher presentation rate of diffuse pulmonary lesions (4/7 vs. 1/12, respectively; p = 0.036) despite lower mean levels of serum beta-D: -glucan (314 ± 214 vs. 1139 ± 1114 pg/ml, respectively; p = 0.02) that suggested a lower burden of Pneumocystis jirovecii. In conclusion, PCP in RA patients with existing pulmonary lesions may trigger subsequent progression to lethal interstitial pneumonia.

  1. 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.

  2. [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.

  3. Atypical And Severe Enlargement Of Right Atrium.

    PubMed

    Siniscalchi, Carmine; Rossetti, Pietro; Rocci, Anna; Rubino, Pasquale; Basaglia, Manuela; Gaibazzi, Nicola; Quintavalla, Roberto

    2016-09-13

    A 76 year-old woman was admitted to the Emergency Department for recent-onset dyspnea and cough. The electrocardiogram was considered inconclusive. A thoracic X-ray showed global cardiac profile enlargement. Computed tomography, acutely performed in the clinical suspicion of atypical pneumonia/myocarditis or pericardial effusion, showed cardiac enlargement especially of the right chambers. In order to investigate Ebstein's anomaly, pericardial cysts, tumors or other conditions of the right heart a simple trans-thoracic echocardiogram was performed. Four chambers view showed a giant right atrium aneurysm with moderate tricuspid regurgitation without stenosis or typical Ebstein's echocardiographic pattern.

  4. Massive Hemoptysis Caused by Atypical Behcet's Disease

    PubMed Central

    Lee, Won-Young; Hoon, Choi Se

    2014-01-01

    Pulmonary and/or bronchial aneurysm with systemic thrombophlebitis can be seen in the case of both Behcet's disease and Hughes-Stovin syndrome. These two diseases may be indistinguishable since the clinical, radiological, and histopathological findings are similar in both cases. In particular, from the perspective of pulmonary involvement, hemoptysis is the leading cause of death in both conditions and is followed by aneurysmal rupture. Here, we report a case in which surgical resection was performed for a patient presenting recurrent hemoptysis as an atypical presentation of Behcet's disease. PMID:24782975

  5. Atypical intrauterine parvo b19 infection.

    PubMed

    Drašković, Biljana; Uram-Benka, Anna; Fabri, Izabela; Velisavljev Filipović, Gordana

    2012-08-01

    Human parvovirus B19 is a single-stranded DNA virus. During pregnancy, parvovirus B19 infection can be asymptomatic or cause a variety of signs of fetal damage, fetal anemia, nonimmune hydrops fetalis, spontaneous abortion and can result in fetal death. Recent improvements in diagnosing parvovirus infections and the availability of intrauterine transfusion have reduced the overall rate of fetal loss after maternal exposure. There is an approximately 30% risk of vertical transmission and 1% of hydrops. We report of the first case of vertical parvovirus B19 infection with atypical manifestations in our clinic. The neonate had pleural effusion associated with anaemia.

  6. 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.

  7. Atypical outcome in attention deficit hyperactivity disorder.

    PubMed

    Schmidt, K; Freidson, S

    1990-07-01

    This report describes the course of psychiatric illness in two boys. Both presented with attention deficit hyperactivity disorder (ADHD) in midchildhood; after puberty, one boy developed a schizophrenic illness while the other boy developed a major affective illness. Although the major ADHD outcome studies have found no link between the childhood occurrence of ADHD and psychosis in adulthood, occasionally such a link may exist. The theoretical and practical implications of this finding are discussed. It should be noted, however, that such outcome is highly atypical and very rare.

  8. Chimeric antigen receptor-redirected CD45RA-negative T cells have potent antileukemia and pathogen memory response without graft-versus-host activity.

    PubMed

    Chan, W K; Suwannasaen, D; Throm, R E; Li, Y; Eldridge, P W; Houston, J; Gray, J T; Pui, C-H; Leung, W

    2015-02-01

    Chimeric antigen receptor (CAR)-redirected cellular therapy is an attractive modality for cancer treatment. We hypothesized that allogeneic CAR-engineered CD45RA-negative T cells can control cancer and infection without the risk of graft-versus-host disease (GVHD). We used CD19(+) MLL-rearranged leukemia as prototype because it is an aggressive and generally drug-resistant malignancy. CD45RA(-) cells that were transduced with anti-CD19 CAR containing 4-1BB and CD3ζ signaling domains effectively lysed MLL-rearranged leukemia cell lines and primary blasts in vitro. In a disseminated leukemia mouse model, CAR(+)CD45RA(-) cells significantly reduced leukemia burdens and prolonged overall survival without GVHD. CAR(+) cells were sustainable in blood, and all the treated mice remained leukemia-free even after they were re-challenged with leukemia cells. Despite the transduction process, CD45RA(-) cells retained recall activity both in vitro and in vivo against human pathogens commonly found in cancer patients. In comparison with CD45RA(+) cells, CD45RA(-) cells showed less allogeneic activity in mixed leukocyte reactions and in mouse models. Thus, the use of CAR(+)CD45RA(-) cells can separate GVHD from graft-versus-malignancy effect and infection control. These cells should also be useful in nontransplant settings and may be administered as off-the-shelf third-party cells.

  9. 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.

  10. Extending the application of DSAM to atypical stopping media

    NASA Astrophysics Data System (ADS)

    Das, S.; Samanta, S.; Bhattacharjee, R.; Raut, R.; Ghugre, S. S.; Sinha, A. K.; Garg, U.; Chakrabarti, R.; Mukhopadhyay, S.; Dhal, A.; Raju, M. Kumar; Madhavan, N.; Muralithar, S.; Singh, R. P.; Suryanarayana, K.; Rao, P. V. Madhusudhana; Palit, R.; Saha, S.; Sethi, J.

    2017-01-01

    A methodology that manifolds the possibilities of level lifetime measurements using the Doppler Shift Attenuation Method (DSAM), and extends its application beyond the conventional thin-target-on-thick-elemental-backing setups, is presented. This has been achieved primarily through application of the TRIM code to simulate the stopping of the recoils in the target and the backing media. Using the TRIM code, primarily adopted in the domain of materials research, in the context of lifetime analysis require rendition of the simulation results into a representation that appropriately incorporates the nuances of nuclear reaction along with the associated kinematics, besides the transformation from an energy-coordinate representation to a velocity-direction profile as required for lifetime analysis. The present development makes it possible to practice DSAM in atypical experimental scenarios such as those using molecular or multi-layered target and/or backing as the stopping medium. These aberrant cases, that were beyond representation in the customary Doppler shape analysis can, in the light of the present work, be conveniently used in the DSAM based investigations. The new approach has been validated through re-examination of known lifetimes measured both in the conventional as well as in the deviant setups.

  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. 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.

  13. The Role of Retinoic Acid (RA) in Spermatogonial Differentiation.

    PubMed

    Busada, Jonathan T; Geyer, Christopher B

    2016-01-01

    Retinoic acid (RA) directs the sequential, but distinct, programs of spermatogonial differentiation and meiotic differentiation that are both essential for the generation of functional spermatozoa. These processes are functionally and temporally decoupled, as they occur in distinct cell types that arise over a week apart, both in the neonatal and adult testis. However, our understanding is limited in terms of what cellular and molecular changes occur downstream of RA exposure that prepare differentiating spermatogonia for meiotic initiation. In this review, we describe the process of spermatogonial differentiation and summarize the current state of knowledge regarding RA signaling in spermatogonia.

  14. 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.

  15. Wernicke encephalopathy with atypical magnetic resonance imaging.

    PubMed

    Liou, Kuang-Chung; Kuo, Shu-Fan; Chen, Lu-An

    2012-11-01

    Wernicke encephalopathy (WE) is a medical emergency caused by thiamine (vitamin B1) deficiency. Typical clinical manifestations are mental change, ataxia, and ocular abnormalities. Wernicke encephalopathy is an important differential diagnosis in all patients with acute mental change. However, the disorder is greatly underdiagnosed. Clinical suspicion, detailed history taking, and neurologic evaluations are important for early diagnosis. Magnetic resonance imaging (MRI) is currently considered the diagnostic method of choice. Typical MRI findings of WE are symmetrical involvement of medial thalamus, mammillary body, and periaqueductal gray matter. Prompt thiamine supplement is important in avoiding unfavorable outcomes. Here, we report a case of alcoholic WE with typical clinical presentation but with atypical MRI. Axial fluid-attenuated inversion recovery images showing symmetrical hyperintensity lesions in dentate nuclei of cerebellum, olivary bodies, and dorsal pons. Although atypical MRI findings are more common in nonalcoholic WE, it can also occur in alcoholic WE. This article is aimed to highlight the potential pitfalls in diagnosing acute mental change, the importance of clinical suspicion, and early treatment in WE.

  16. Inverse agonist properties of atypical antipsychotic drugs.

    PubMed

    Akam, Elizabeth; Strange, Philip G

    2004-06-01

    Mechanisms of action of several atypical antipsychotic drugs have been examined at the D(2) dopamine receptor expressed in CHO cells. The drugs tested were found to exhibit inverse agonist activity at the D(2) dopamine receptor based on their effects to potentiate forskolin-stimulated cyclic AMP (cAMP) accumulation. Each of the antipsychotic drugs tested (clozapine, olanzapine, quetiapine and risperidone) increased cAMP accumulation to the same extent. The increase in cAMP was also similar to that seen with typical antipsychotic drugs. Inverse agonism at the D(2) dopamine receptor seems, therefore, to be a property common to all classes of antipsychotic drugs. The effect of sodium ions on the binding of the drugs to the receptor was also assessed. Each of the atypical antipsychotic drugs tested here bound with higher affinity in the absence of sodium ions. Previous studies have shown that some antipsychotic drugs are insensitive to sodium ions and some bind with higher affinity in the presence of sodium ions. Given that all of these antipsychotic drugs are inverse agonists, it may be concluded that this sodium ion sensitivity is unrelated to mechanisms of inverse agonism.

  17. Atypical Patterns of Early Attachment: Discussion and Future Directions.

    ERIC Educational Resources Information Center

    Barnett, Douglas; Butler, Christine M.; Vondra, Joan I.

    1999-01-01

    Discusses the role of a study of atypical patterns of attachment behavior for the understanding of attachment theory, parenting, and child socioemotional development. Notes that research on atypical attachments suggest that the developmental integration of biological, psychological, and behavioral responses is more profoundly dependent on social-…

  18. 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 ...

  19. 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)…

  20. 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.

  1. 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

  2. Rapid determination of 226Ra in emergency urine samples

    DOE PAGES

    Maxwell, Sherrod L.; Culligan, Brian K.; Hutchison, Jay B.; ...

    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

  3. 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.

  4. 76 FR 63701 - Culturally Significant Objects Imported for Exhibition Determinations: “Johan Zoffany RA: Society...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-13

    ... for Exhibition Determinations: ``Johan Zoffany RA: Society Observed'' SUMMARY: Notice is hereby given... objects to be included in the exhibition ``Johan Zoffany RA: Society Observed,'' imported from abroad...

  5. Linguistic recycling in typical and atypical interaction.

    PubMed

    Perkins, Michael R

    2014-01-01

    I present evidence that linguistic "recycling" - i.e., the redeployment of linguistic material from prior utterances during conversation - is a striking and prevalent feature not only of interaction between typical speakers, but also, and notably, of interaction involving the communication impaired. In the latter case, recycling may sometimes be used as a compensatory communicative resource when linguistic ability is compromised. Despite its prevalence, however, recycling has largely been ignored by clinical linguists. In addition to providing illustrations of linguistic recycling across a range of communication disorders, I also examine how it is subserved by phenomena such as priming, short-term memory and alignment. I subsequently argue for a shift in perspective that puts recycling at the heart of our perception of how typical and atypical interaction works, and suggest a number of potential benefits for clinical linguistics, ranging from the way we understand and analyse communication disorders to how we assess and treat them.

  6. 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.

  7. Atypical parakeratosis: a marker of dysplasia?

    PubMed

    Voytek, T M; Kannan, V; Kline, T S

    1996-11-01

    The Bethesda System categorizes atypical parakeratosis (APK) as "ASCUS or SIL depending on the degree of cellular abnormalities." APK, however, is not well-defined. We retrospectively reviewed 68 cervicovaginal specimens with follow-up material to identify specific criteria and clinical significance of APK. APK cells were small cells, 2-3 times the diameter of neutrophil, with dense, orangeophilic cytoplasm, high nuclear cytoplasmic ratio, dense, often uneven chromatin, and irregular nuclear contour. Of 62 cases with APK, 37 had accompanying dysplastic cells. Of 25 cases with APK alone, follow-up revealed 12 with squamous intraepithelial lesion (5 HSIL and 7 LSIL) and 13 with benign changes. A major diagnostic pitfall of APK was inflammation with degeneration. Abundant APK cells, minimal inflammation and degeneration, and previous history of dysplasia frequently were associated with follow-up SIL. The findings of this study identify APK as an important marker for dysplasia that warrants careful evaluation and follow-up.

  8. An overview of atypical enteropathogenic Escherichia coli.

    PubMed

    Hernandes, Rodrigo T; Elias, Waldir P; Vieira, Mônica A M; Gomes, Tânia A T

    2009-08-01

    The enteropathogenic Escherichia coli (EPEC) pathotype is currently divided into two groups, typical EPEC (tEPEC) and atypical EPEC (aEPEC). The property that distinguishes these two groups is the presence of the EPEC adherence factor plasmid, which is only found in tEPEC. aEPEC strains are emerging enteropathogens that have been detected worldwide. Herein, we review the serotypes, virulence properties, genetic relationships, epidemiology, reservoir and diagnosis of aEPEC, including those strains not belonging to the classical EPEC serogroups (nonclassical EPEC serogroups). The large variety of serotypes and genetic virulence properties of aEPEC strains from nonclassical EPEC serogroups makes it difficult to determine which strains are truly pathogenic.

  9. Refractory Rheumatic Disorder: Atypical Postpregnancy Osteoporosis

    PubMed Central

    Mourgues, Cindy; Malochet-Guinamand, Sandrine; Soubrier, Martin

    2015-01-01

    This is a case report on a young patient with severe osteoporosis that was initially revealed when she presented with polyarthralgia during her second pregnancy. Postpartum, the pain increased and her X-ray did not show any abnormalities. A bone scintigraphy was performed. It indicated an inflammatory rheumatic disorder. Six months after partum, an investigation of right coxalgia revealed a spontaneous basicervical fracture. Given the persistent polyarthralgia, the patient underwent a new scintigraphy, which revealed areas of what looked to be old rib and L1 fractures. A subsequent full body magnetic resonance imaging (MRI) scan revealed signal abnormalities that could indicate multiple lower limb bone fractures. Despite exhaustive biological, radiological, and histological testing, no secondary cause for the osteoporosis was found. The patient was started on teriparatide. We finally concluded that, despite the atypical presentation, the patient was suffering from postpregnancy osteoporosis. It is possible that the frequency of occurrence of this still poorly understood disease is underestimated. PMID:25785219

  10. 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

  11. 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.

  12. Clinical management of an atypical dental invagination

    PubMed Central

    Badran, Zahi; Lopez-Cazaux, Serena; Crauste, Eléonore; Bray, Estelle; Soueidan, Assem; Armengol, Valérie

    2016-01-01

    Dental invagination (DI) is a tooth malformation that usually affects permanent teeth. Its precise etiology is still controversial and represents a clinical challenge as it can favor the development of carious lesion or periodontal inflammation. This paper presents a case of a 23-year-old Caucasian male, where an atypical buccal DI could not be completely diagnosed in the dens invaginatus category. Furthermore, other differential diagnoses could not be confirmed. The dental malformation was seen on a permanent maxillary first incisor and was associated with periodontal inflammation and attachment loss. Successful clinical management of this case consisted of surgical restorative treatment and regular follow-up, accompanied by thorough oral hygiene procedures. PMID:28042279

  13. Clinical grand rounds: atypical hemolytic uremic syndrome.

    PubMed

    Hodgkins, Kavita S; Bobrowski, Amy E; Lane, Jerome C; Langman, Craig B

    2012-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare, lifethreatening, chronic, genetic disease of uncontrolled alternative pathway complement activation. The understanding of the pathophysiology and genetics of this disease has expanded over recent decades and promising new developments in the management of aHUS have emerged. Regardless of the cause of aHUS, with or without a demonstrated mutation or autoantibody, blockade of terminal complement activation through C5 is of high interest as a mechanism to ameliorate the disease. Eculizumab, an existing monoclonal antibody directed against C5 with high affinity, prevents the perpetuation of the downstream activation of the complement cascade and the damage caused by generation of the anaphylotoxin C5a and the membrane attack complex C5b-9, by blocking C5 cleavage. We report the successful use of eculizumab in a patient after kidney transplantation and discuss the disease aHUS.

  14. Vestibular Schwannoma Atypically Invading Temporal Bone

    PubMed Central

    Park, Soo Jeong; Yang, Na-Rae

    2015-01-01

    Vestibular schwannoma (VS) usually present the widening of internal auditory canal (IAC), and these bony changes are typically limited to IAC, not extend to temporal bone. Temporal bone invasion by VS is extremely rare. We report 51-year-old man who revealed temporal bone destruction beyond IAC by unilateral VS. The bony destruction extended anteriorly to the carotid canal and inferiorly to the jugular foramen. On histopathologic examination, the tumor showed typical benign schwannoma and did not show any unusual vascularity or malignant feature. Facial nerve was severely compressed and distorted by tumor, which unevenly eroded temporal bone in surgical field. Vestibular schwannoma with atypical invasion of temporal bone can be successfully treated with combined translabyrinthine and lateral suboccipiral approach without facial nerve dysfunction. Early detection and careful dissection of facial nerve with intraoperative monitoring should be considered during operation due to severe adhesion and distortion of facial nerve by tumor and eroded temporal bone. PMID:25932298

  15. Atypical Neurotransmitters and the Neurobiology of Depression.

    PubMed

    Joca, Samia Regiane; Moreira, Fabricio Araujo; Wegener, Gregers

    2015-01-01

    Since the first report that the mechanism of action of antidepressants involves the facilitation of monoaminergic neurotransmission in the brain in the 1960s, the leading hypothesis about the neurobiology of depression has been the so called "monoaminergic hypothesis". However, a growing body of evidence from the last two decades also supports important involvement of non-monoaminergic mechanisms in the neurobiology of depression and antidepressant action. The discovery of nitric oxide (NO) and endocannabinoid signaling in the brain during the 1990s challenged the wellestablished criteria of classical neurotransmission. These transmitters are synthesized and released on demand by the postsynaptic neurons, and may act as a retrograde messenger on the presynaptic terminal, modulating neurotransmitter release. These unconventional signaling mechanisms and the important role as neural messengers have classified NO and endocannabinoids as atypical neurotransmitters. They are able to modulate neural signaling mediated by the main conventional neurotransmitters systems in the brain, including the monoaminergic, glutamatergic and GABAergic signaling systems. This review aims at discussing the fundamental aspects of NO- and endocannabinoid-mediated signaling in the brain, and how they can be related to the neurobiology of depression. Both preclinical and clinical evidence supporting the involvement of these atypical neurotransmitters in the neurobiology of depression, and in the antidepressant effects are presented here. The evidence is discussed on basis of their ability to modulate different neurotransmitter systems in the brain, including monoaminergic and glutamatergic ones. A better comprehension of NO and endocannabinoid signaling mechanisms in the neurobiology depression could provide new avenues for the development of novel non-monoamine based antidepressants.

  16. Atypical Neonatal Marfan Syndrome with p.Glu1073Lys Mutation of FBN1: the First Case in Korea.

    PubMed

    Heo, Ju Sun; Song, Joo Young; Choi, Eun Young; Kim, Eun Hee; Kim, Ji Hee; Park, So Eun; Jeon, Ji Hyun

    2017-01-01

    Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe mitral and/or tricuspid valve insufficiency, ectopia lentis, arachnodactyly, joint contractures, crumpled ear, loose skin, and pulmonary emphysema.We describe a newborn male diagnosed with nMFS. He presented several atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Molecular analysis revealed a missense mutation at nucleotide 3217 (c.3217G>A) in exon 26 of the fibrillin-1 (FBN1) gene, resulting in the substitution of a glutamate for a lysine at codon 1073 (E1073K) in the 12th calcium binding epidermal growth factor-like domain of the FBN1 protein. Here we report a rare case of Nmfs with several combined atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Our report is the first atypical nMFS case with p.Glu1073Lys mutation of FBN1 in Korea and may help clinicians with the diagnosis and follow-up of atypical nMFS.

  17. Atypical Neonatal Marfan Syndrome with p.Glu1073Lys Mutation of FBN1: the First Case in Korea

    PubMed Central

    2017-01-01

    Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe mitral and/or tricuspid valve insufficiency, ectopia lentis, arachnodactyly, joint contractures, crumpled ear, loose skin, and pulmonary emphysema.We describe a newborn male diagnosed with nMFS. He presented several atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Molecular analysis revealed a missense mutation at nucleotide 3217 (c.3217G>A) in exon 26 of the fibrillin-1 (FBN1) gene, resulting in the substitution of a glutamate for a lysine at codon 1073 (E1073K) in the 12th calcium binding epidermal growth factor-like domain of the FBN1 protein. Here we report a rare case of Nmfs with several combined atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Our report is the first atypical nMFS case with p.Glu1073Lys mutation of FBN1 in Korea and may help clinicians with the diagnosis and follow-up of atypical nMFS. PMID:27914124

  18. Radium isotopes ((226)Ra and (228)Ra) in Na-Cl type groundwaters from Tohoku District (Aomori, Akita and Yamagata Prefectures) in Japan.

    PubMed

    Tomita, Jumpei; Zhang, Jing; Yamamoto, Masayoshi

    2014-11-01

    A total of 28 Na-Cl type groundwater samples were collected from Aomori, Akita and Yamagata Prefectures, in the Tohoku District of Japan, and their radium isotope ((226)Ra and (228)Ra) concentrations were measured along with their chemical components and stable isotope ratios (δ(2)H and δ(18)Os). The (226)Ra concentrations in groundwater samples varied widely, ranging from 8.8 to 1587 mBq kg(-1). These concentrations showed an increasing tendency with the increase of the total dissolved solid (TDS) contents. The (228)Ra/(226)Ra activity ratios were in the range from 0.3 to 4.2, with most data being around 0.5-2. These ratios were within those of (232)Th/(238)U found in granitic and related rocks and so on in Japan, indicating that Ra isotopes mainly ejected into the groundwater by the alpha-recoil process. The relationship between (226)Ra and other parameters suggested that Ra isotopes in groundwater samples in this study were mainly constrained by adsorption-desorption reactions depending on salinity with wide variation. Clear correlation between (226)Ra-Ca, (226)Ra-Sr, (226)Ra-Ba and (226)Ra-TDS observed in sulfate-free groundwater samples indicated that Ra isotopes of them were constrained by adsorption-desorption reactions depending on salinity under reducing condition. In contrast, relationship of (226)Ra-Ca, (226)Ra-Sr, (226)Ra-Ba and (226)Ra-TDS in sulfate-containing groundwater samples varied widely, and then, removal or enhanced mobility of Ra isotopes of them were observed.

  19. Regulation of atypical MAP kinases ERK3 and ERK4 by the phosphatase DUSP2

    PubMed Central

    Perander, Maria; Al-Mahdi, Rania; Jensen, Thomas C.; Nunn, Jennifer A. L.; Kildalsen, Hanne; Johansen, Bjarne; Gabrielsen, Mads; Keyse, Stephen M.; Seternes, Ole-Morten

    2017-01-01

    The atypical MAP kinases ERK3 and ERK4 are activated by phosphorylation of a serine residue lying within the activation loop signature sequence S-E-G. However, the regulation of ERK3 and ERK4 phosphorylation and activity is poorly understood. Here we report that the inducible nuclear dual-specificity MAP kinase phosphatase (MKP) DUSP2, a known regulator of the ERK and p38 MAPKs, is unique amongst the MKP family in being able to bind to both ERK3 and ERK4. This interaction is mediated by a conserved common docking (CD) domain within the carboxyl-terminal domains of ERK3 and ERK4 and the conserved kinase interaction motif (KIM) located within the non-catalytic amino terminus of DUSP2. This interaction is direct and results in the dephosphorylation of ERK3 and ERK4 and the stabilization of DUSP2. In the case of ERK4 its ability to stabilize DUSP2 requires its kinase activity. Finally, we demonstrate that expression of DUSP2 inhibits ERK3 and ERK4-mediated activation of its downstream substrate MK5. We conclude that the activity of DUSP2 is not restricted to the classical MAPK pathways and that DUSP2 can also regulate the atypical ERK3/4-MK5 signalling pathway in mammalian cells. PMID:28252035

  20. Atypical antipsychotics to treat the neuropsychiatric symptoms of dementia

    PubMed Central

    Lee, Philip E; Gill, Sudeep S; Rochon, Paula

    2006-01-01

    Neuropsychiatric symptoms are common in older adults with dementia and can be associated with a rapid decline in cognitive and functional status. This article reviews the current literature supporting the use of atypical antipsychotic medications in this population. Among the currently available atypical antipsychotics, risperidone and olanzapine have been the most widely studied in double-blind, randomized, placebo-controlled clinical trials. Despite the common use of other atypical antipsychotic medications, their efficacy and safety in older adults with dementia has not been as extensively studied. Some controversy surrounds the use of atypical antipsychotic agents in older adults with the suggestion that they may increase the incidence of stroke or even death. Despite the potential for increased risk of harm from the use of these medications, atypical antipsychotics are often effective in treating troublesome neuropsychiatric symptoms refractory to other treatments. Whenever possible, these atypical antipsychotic drug treatments should be combined with non-pharmacological treatments to limit the need and dose of antipsychotic drugs and constant monitoring for potential harms should be maintained. The choice of which atypical antipsychotic agent can be guided by the nature and severity of the target symptom and the medication least likely to cause harm to the patient. PMID:19412500

  1. Integrated genomic analyses of de novo pathways underlying atypical meningiomas

    PubMed Central

    Harmancı, Akdes Serin; Youngblood, Mark W.; Clark, Victoria E.; Coşkun, Süleyman; Henegariu, Octavian; Duran, Daniel; Erson-Omay, E. Zeynep; Kaulen, Leon D.; Lee, Tong Ihn; Abraham, Brian J.; Simon, Matthias; Krischek, Boris; Timmer, Marco; Goldbrunner, Roland; Omay, S. Bülent; Baranoski, Jacob; Baran, Burçin; Carrión-Grant, Geneive; Bai, Hanwen; Mishra-Gorur, Ketu; Schramm, Johannes; Moliterno, Jennifer; Vortmeyer, Alexander O.; Bilgüvar, Kaya; Yasuno, Katsuhito; Young, Richard A.; Günel, Murat

    2017-01-01

    Meningiomas are mostly benign brain tumours, with a potential for becoming atypical or malignant. On the basis of comprehensive genomic, transcriptomic and epigenomic analyses, we compared benign meningiomas to atypical ones. Here, we show that the majority of primary (de novo) atypical meningiomas display loss of NF2, which co-occurs either with genomic instability or recurrent SMARCB1 mutations. These tumours harbour increased H3K27me3 signal and a hypermethylated phenotype, mainly occupying the polycomb repressive complex 2 (PRC2) binding sites in human embryonic stem cells, thereby phenocopying a more primitive cellular state. Consistent with this observation, atypical meningiomas exhibit upregulation of EZH2, the catalytic subunit of the PRC2 complex, as well as the E2F2 and FOXM1 transcriptional networks. Importantly, these primary atypical meningiomas do not harbour TERT promoter mutations, which have been reported in atypical tumours that progressed from benign ones. Our results establish the genomic landscape of primary atypical meningiomas and potential therapeutic targets. PMID:28195122

  2. Preparation and characterization of human ADCK3, a putative atypical kinase.

    PubMed

    Wheeler, Brody; Jia, Zongchao

    2015-04-01

    AarF domain containing kinase 3 (ADCK3) is a mitochondrial protein known to have a role in the electron transport chain. Despite being required for the biosynthesis of coenzyme Q10, a lipid-soluble electron transporter found to be essential for aerobic cellular respiration, the precise biological function of ADCK3 remains unknown. Patients with mutations in ADCK3 experience an onset of neurological disorders from childhood, including cerebellar ataxia and exercise intolerance. After extensive screening for soluble recombinant protein expression, an N-terminal fusion of maltose-binding protein was found to facilitate the overexpression of the human ADCK3 kinase domain in Escherichia coli as a soluble and biologically active entity. For the first time our work reveals Mg(2+)-dependent ATPase activity of ADCK3, providing strong support for the theoretical prediction of this protein being a functional atypical kinase.

  3. Mutation in the transcriptional regulator PhoP contributes to avirulence of Mycobacterium tuberculosis H37Ra strain.

    PubMed

    Lee, Jong Seok; Krause, Roland; Schreiber, Jörg; Mollenkopf, Hans-Joachim; Kowall, Jane; Stein, Robert; Jeon, Bo-Young; Kwak, Jeong-Yeon; Song, Min-Kyong; Patron, Juan Pablo; Jorg, Sabine; Roh, Kyoungmin; Cho, Sang-Nae; Kaufmann, Stefan H E

    2008-02-14

    Attenuated strains of mycobacteria can be exploited to determine genes essential for their pathogenesis and persistence. To this goal, we sequenced the genome of H37Ra, an attenuated variant of Mycobacterium tuberculosis H37Rv strain. Comparison with H37Rv revealed three unique coding region polymorphisms. One polymorphism was located in the DNA-binding domain of the transcriptional regulator PhoP, causing the protein's diminished DNA-binding capacity. Temporal gene expression profiles showed that several genes with reduced expression in H37Ra were also repressed in an H37Rv phoP knockout strain. At later time points, genes of the dormancy regulon, typically expressed in a state of nonreplicating persistence, were upregulated in H37Ra. Complementation of H37Ra with H37Rv phoP partially restored its persistence in a murine macrophage infection model. Our approach demonstrates the feasibility of identifying minute but distinct differences between isogenic strains and illustrates the consequences of single point mutations on the survival stratagem of M. tuberculosis.

  4. 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

  5. Mitochondrial function is altered in horse atypical myopathy.

    PubMed

    Lemieux, Hélène; Boemer, François; van Galen, Gaby; Serteyn, Didier; Amory, Hélène; Baise, Etienne; Cassart, Dominique; van Loon, Gunther; Marcillaud-Pitel, Christel; Votion, Dominique-M

    2016-09-01

    Equine atypical myopathy in Europe is a fatal rhabdomyolysis syndrome that results from the ingestion of hypoglycin A contained in seeds and seedlings of Acer pseudoplatanus (sycamore maple). Acylcarnitine concentrations in serum and muscle OXPHOS capacity were determined in 15 atypical myopathy cases. All but one acylcarnitine were out of reference range and mitochondrial respiratory capacity was severely decreased up to 49% as compared to 10 healthy controls. The hallmark of atypical myopathy thus consists of a severe alteration in the energy metabolism including a severe impairment in muscle mitochondrial respiration that could contribute to its high death rate.

  6. 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.…

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

    DOE PAGES

    Rojas, J. V.; Woodward, J. D.; Chen, N.; ...

    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

  8. [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

  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. 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.

  11. Ponticulin is an atypical membrane protein

    PubMed Central

    1994-01-01

    We have cloned and sequenced ponticulin, a 17,000-dalton integral membrane glycoprotein that binds F-actin and nucleates actin assembly. A single copy gene encodes a developmentally regulated message that is high during growth and early development, but drops precipitously during cell streaming at approximately 8 h of development. The deduced amino acid sequence predicts a protein with a cleaved NH2-terminal signal sequence and a COOH-terminal glycosyl anchor. These predictions are supported by amino acid sequencing of mature ponticulin and metabolic labeling with glycosyl anchor components. Although no alpha- helical membrane-spanning domains are apparent, several hydrophobic and/or sided beta-strands, each long enough to traverse the membrane, are predicted. Although its location on the primary sequence is unclear, an intracellular domain is indicated by the existence of a discontinuous epitope that is accessible to antibody in plasma membranes and permeabilized cells, but not in intact cells. Such a cytoplasmically oriented domain also is required for the demonstrated role of ponticulin in binding actin to the plasma membrane in vivo and in vitro (Hitt, A. L., J. H. Hartwig, and E. J. Luna. 1994. Ponticulin is the major high affinity link between the plasma membrane and the cortical actin network in Dictyostelium. J. Cell Biol. 126:1433-1444). Thus, ponticulin apparently represents a new category of integral membrane proteins that consists of proteins with both a glycosyl anchor and membrane-spanning peptide domain(s). PMID:8089175

  12. 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.

  13. 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.

  14. Invasive atypical thymic carcinoid: three case reports and literature review

    PubMed Central

    Zhu, Shan; Wang, Zhong-Tang; Liu, Wen-Zhi; Zong, Shi-Xiang; Li, Bao-Sheng

    2016-01-01

    Atypical thymic carcinoid is an extremely rare thymic neuroendocrine tumor derived from the neuroendocrine system. The aims of this paper were to investigate the clinical features of atypical thymic carcinoid and collate information and experience to improve the diagnosis and treatment of this disease. We describe three cases of atypical carcinoid of the thymus; clinical features, pathological data, treatment modalities, and short-term patient outcomes were summarized and analyzed. The initial clinical symptoms and signs of all three patients were nonspecific and an anterior mediastinal mass was found in each patient on chest computed tomography scan. All three patients underwent surgical resection (total thymectomy and complete excision of the tumor), followed by postoperative radiotherapy, with or without chemotherapy. The diagnoses of three patients were confirmed by pathological and immunohistochemical evaluation. We also present a review of the literature to collate as much information as possible and provide a reference for proper diagnosis and treatment of atypical thyroid carcinoid. PMID:27785065

  15. The Use of Electroconvulsive Therapy in Atypical Psychotic Presentations

    PubMed Central

    Vasu, Devi

    2007-01-01

    Convulsive therapy and its progeny, electroconvulsive therapy (ECT), were originally used for the treatment of catatonic schizophrenia, and there is little doubt that ECT remains an effective intervention for the treatment of schizophrenia. However, current practice tends to favor the use of ECT in severe or treatment refractory affective disorders, and its use in schizophrenia and other nonaffective (atypical) psychotic disorders has become controversial. Case reports have suggested a role for ECT in two specific atypical psychotic disorders: Cotard's syndrome and cycloid psychosis. In this article, we review the atypical psychotic disorders and report a series of five case examples that signify the role of ECT in atypical psychotic presentations, particularly when the symptoms resemble those found in Cotard's syndrome and cycloid psychosis. PMID:20428309

  16. 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

  17. Atypical Takotsubo syndrome during anagrelide therapy.

    PubMed

    Proietti, Riccardo; Rognoni, Andrea; Ardizzone, Fabio; Maccio, Sergio; Santagostino, Alberto; Rognoni, Giorgio

    2009-07-01

    Anagrelide is a phosphodiesterase III inhibitor utilized in the treatment of essential thrombocythemia. Anagrelide can be responsible for positive inotropic and chonotropic activity of the cardiovascular system. Moreover, it can induce vasospam directly on the epicardial coronary arteries. In the literature, it is well reported that this inhibitor can determine serious cardiovascular side effects, including congestive heart failure, arrhythmia and acute coronary syndrome. We describe the case of a 75-year-old woman who developed a mid-ventricular Takotsubo syndrome while on anagrelide therapy. Takotsubo cardiomyopathy, also known as left ventricular ballooning syndrome, is characterized by a reversible ventricular contractile dysfunction with akinesis and expansion of apical segments and hyperkinesis of the basal segments. Recently, atypical cases with akinesia and dilation of mid-ventricular segment and hypercontraction of the apical segments, also called mid-ventricular and inverted Takotsubo syndrome, have been described. Even though the pathogenesis of Takotsubo syndrome is poorly understood, several mechanisms have been proposed, including catecholamine-induced myocardial stunning, and ischemia-mediated stunning due to multivessel epicardial or microvascular spasm. We think that in our case, the adverse response of anagrelide therapy was determined, by accumulated dosage of the drug, through an intensive inotropic stimulation and a sympathetic hyperactivation in a vulnerable myocardium. To our knowledge, this is one of the first reports of an association between anagrelide therapy and Takotsubo cardiomyopathy.

  18. Echovirus 22 is an atypical enterovirus.

    PubMed Central

    Coller, B A; Chapman, N M; Beck, M A; Pallansch, M A; Gauntt, C J; Tracy, S M

    1990-01-01

    Although echovirus 22 (EV22) is classified as an enterovirus in the family Picornaviridae, it is atypical of the enterovirus paradigm, typified by the polioviruses and the coxsackie B viruses. cDNA reverse transcribed from coxsackievirus B3 (CVB3) RNA does not hybridize to genomic RNA of EV22, and conversely, cDNA made to EV22 does not hybridize to CVB3 genomic RNA or to molecular clones of CVB3 or poliovirus type 1. EV22 cDNA does not hybridize to viral RNA of encephalomyocarditis virus or to a molecular clone of Theiler's murine encephalomyelitis virus, members of the cardiovirus genus. The genomic RNA of EV22 cannot be detected by the polymerase chain reaction using generic enteroviral primers. EV22 does not shut off host cell protein synthesis, and the RNA of EV22 is efficiently translated in vitro in rabbit reticulocyte lysates. Murine enterovirus-immune T cells recognize and proliferate against EV22 as an antigen in vitro, demonstrating that EV22 shares an epitope(s) common to enteroviruses but not found among other picornaviruses. Images PMID:2159539

  19. 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

  20. Nocturnal manifestations of atypical parkinsonian disorders.

    PubMed

    Bhidayasiri, Roongroj; Jitkritsadakul, Onanong; Colosimo, Carlo

    2014-01-01

    Although nocturnal disturbances are increasingly recognized as an integral part of the continuum of daytime manifestations of Parkinson's disease (PD), there is still little evidence in the medical literature to support the occurrence of these complex phenomena in patients with atypical parkinsonian disorders (APDs). Based on the anatomical substrates in APDs, which are considered to be more extensive outside the basal ganglia than in PD, we might expect that patients with APDs encounter the whole range of nocturnal disturbances, including motor, sleep disorders, autonomic dysfunctions, and neuropsychiatric manifestations at a similar, or even greater, frequency than in PD. This article is a review of the current literature on the problems at nighttime of patients with progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and dementia with Lewy bodies. MEDLINE, life science journals and online books were searched by querying appropriate key words. Reports were included if the studies were related to nocturnal manifestations in APDs. Forty articles fulfilled the selection criteria. Differences between these symptoms in APDs and PD are highlighted, given the evidence available about each manifestation. This analysis of nocturnal manifestations of APDs suggests the need for future studies to address these issues to improve the quality of life not only of patients with APDs but the caregivers who encounter the challenges of supporting these patients on a daily basis.

  1. 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.

  2. 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

  3. Atypical Miyoshi distal myopathy: A case report

    PubMed Central

    Wang, Meiling; Guo, Yujie; Fu, Yong; Jia, Rui; Chen, Gang

    2016-01-01

    Five distinct predominant distal myopathies have been identified with discrete clinical and genetic patterns. Miyoshi myopathy (MM; early adult-onset, type 2) is a subtype of dysferlinopathy. Furthermore, MM is the most common form of autosomal recessive distal myopathy. MM is typically characterized by muscular weakness, initially affecting the gastrocnemius or soleus muscle from the late teens or early adulthood. The present study reports a case of MM that was confirmed by pathological and immunohistochemical methods, in addition to a review of the relevant literature. A 37-year-old male patient presented with muscular weakness in the left foot. This clinical manifestation was not typical of MM, and the patient was initially diagnosed with inflammatory myopathy. He was treated with dexamethasone at a dose of 10 mg for 5 days followed by gradual tapering, following which the symptoms were alleviated; however, the pathology, immunohistochemistry and electromyography eventually confirmed the diagnosis of MM. The treatment was then terminated and the patient was discharged. The present study further supports the underlying heterogeneity in atypical MM-like phenotypes. Dysferlin protein deficiency can be identified by pathological examination. The pathology of dysferlinopathy is characterized by changes of muscular dystrophy. Inflammatory cellular infiltration is a relatively common finding in the muscle biopsies from numerous patients with dysferlinopathy. Therefore, the detection of dysferlin deficiency or marked reduction on the sarcolemma using immunohistochemical staining is important for the diagnosis of dysferlinopathy. PMID:27882118

  4. Atypical clinical response patterns to ipilimumab.

    PubMed

    Ledezma, Blanca; Binder, Sandra; Hamid, Omid

    2011-08-01

    Patients with advanced melanoma have few treatment options, and survival is poor. However, improved understanding of how the immune system interacts with cancer has led to the development of novel therapies. Ipilimumab is a monoclonal antibody that inhibits cytotoxic T-lymphocyte antigen-4 (CTLA-4), a key negative regulator of host T-cell responses. This article presents cases of patients receiving ipilimumab in clinical trials along with a discussion of their significance and relevance to nursing practice. The patients showed different response patterns to ipilimumab and also had various typical immune-related adverse events (irAEs), which were managed successfully. The atypical response patterns produced by ipilimumab likely reflect its mechanism of action, which requires time for the immune system to mount an effective antitumor response. Meanwhile, lesions may appear to enlarge as a consequence of enhanced T-cell infiltration, although this may not necessarily be true disease progression. Patients receiving ipilimumab may respond very differently compared to how they might react to chemotherapy. Responses can take weeks or months to develop; therefore, clinicians should not terminate treatment prematurely, providing the patient's condition allows for continuation. Early recognition of irAEs combined with prompt management will ensure that events are more likely to resolve without serious consequences.

  5. Observing Behavior and Atypically Restricted Stimulus Control

    PubMed Central

    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 with an eye-tracking apparatus. High accuracy scores indicated stimulus control by both sample stimuli for the 4 nondisabled participants and 4 participants with ID, and eye tracking data showed reliable observing of all stimuli. Intermediate accuracy scores indicated restricted stimulus control for the remaining 6 participants. Their eye-tracking data showed that errors were related to failures to observe sample stimuli and relatively brief observing durations. Five of these participants were then given interventions designed to improve observing behavior. For 4 participants, the interventions resulted initially in elimination of observing failures, increased observing durations, and increased accuracy. For 2 of these participants, contingencies sufficient to maintain adequate observing were not always sufficient to maintain high accuracy; subsequent procedure modifications restored it, however. For the 5th participant, initial improvements in observing were not accompanied by improved accuracy, an apparent instance of observing without attending; accuracy improved only after an additional intervention that imposed contingencies on observing behavior. Thus, interventions that control observing behavior seem necessary but may not always be sufficient for the remediation of restricted stimulus control. PMID:21541173

  6. 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.

  7. 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

  8. Atypical presentation of macrophagic myofasciitis 10 years post vaccination.

    PubMed

    Ryan, Aisling M; Bermingham, Niamh; Harrington, Hugh J; Keohane, Catherine

    2006-12-01

    Macrophagic myofasciitis (MMF) is an uncommon inflammatory disorder of muscle believed to be due to persistence of vaccine-derived aluminium hydroxide at the site of injection. The condition is characterised by diffuse myalgias, arthralgia and fatigue. We describe a patient with histologically confirmed MMF whose presentation was atypical with left chest and upper limb pain beginning more than 10 years post vaccination. Treatment with steroids led to symptomatic improvement. Although rare, clinicians should consider MMF in cases of atypical myalgia.

  9. Atypical orofacial pain disorders: a study of diagnostic subtypes.

    PubMed

    Fricton, J R

    2000-01-01

    Atypical facial pain is a persistent pain in the face or intraoral region that does not fit into the diagnostic criteria associated with specific orofacial pain disorders. This article presents the results of a study, which showed that in most cases of presumed atypical facial pain, there is a specific physical diagnosis or multiple diagnoses that causes the persistent pain. These difficult to diagnose disorders are reviewed with discussion of future research that is needed in this area.

  10. Atypical mycobacterial tenosynovitis and bursitis of the wrist.

    PubMed

    Sanal, Hatice Tuba; Zor, Fatih; Kocaoğlu, Murat; Bulakbaşi, Nail

    2009-12-01

    Atypical mycobacterial tenosynovitis of the wrist can easily be misdiagnosed as synovial chondromatosis. Both sonography and magnetic resonance imaging plays an important role in depicting "rice bodies" within the distended tendon sheaths and bursae of atypical mycobacterial infection. An endemic place for Mycobacterium species and the occupation of the patient should raise the suspicion for the disease. Polymerase chain reaction of the distended tendon fluid is a sensitive, specific and rapid method in identification of the mycobacteria.

  11. Atypical presentation of pheochromocytoma: Central nervous system pseudovasculitis

    PubMed Central

    Rupala, Ketankumar; Mittal, Varun; Gupta, Rajiv; Yadav, Rajiv

    2017-01-01

    Pheochromocytoma has atypical presentation in 9%–10% of patients. Atypical presentations include myocardial infarction, renal failure, and rarely cerebrovascular events. Various etiologies for central nervous system (CNS) involvement in pheochromocytoma have been described in the literature. A rare association of CNS vasculitis-like features has been described with pheochromocytoma. We report a rare case of pheochromocytoma detected on evaluation for CNS vasculitis-like symptoms. PMID:28197038

  12. Generic penetration in the retail atypical antipsychotic market.

    PubMed

    Lenderts, Susan; Kalali, Amir H; Buckley, Peter

    2010-03-01

    In this article, we explore the penetration of generic atypical antipsychotics in the United States market before and after the availability of generic risperidone in July 2008. Analysis suggests that, overall, generic penetration into the atypical antipsychotic market has grown from approximately three percent in January 2008 to more than 25 percent in December 2009. Similar trends are uncovered when branded and generic prescriptions are analyzed by specialty.

  13. Raman-assisted vector Brillouin optical time domain analysis

    NASA Astrophysics Data System (ADS)

    Angulo-Vinuesa, X.; Bacquet, D.; Martin-Lopez, S.; Corredera, P.; Szriftgiser, P.; Gonzalez-Herraez, M.

    2013-05-01

    Raman-assistance (RA) has become a promising technique to enhance the sensing range of standard Brillouin Optical Time Domain Analysis (BOTDA) fiber sensors due to its ability to amplify in a distributed way all the interacting signals within the fiber. Unfortunately, Raman amplification introduces a great amount of Relative Intensity Noise (RIN) to the detected low-frequency probe wave. This RIN transfer problem has been widely identified as a major limitation in RABOTDA. In Vector Brillouin Optical Time Domain Analysis (VBOTDA) the detected signal is transferred to a highfrequency carrier where the Raman RIN transfer turns out to be much less harmful. In this work we demonstrate, for the first time to our knowledge, Raman-assistance in a VBOTDA. Our results show significant reduction of the RIN transfer effect in RA-VBOTDA compared to standard RA-BOTDA, making this type of scheme particularly interesting for long range distributed sensing.

  14. "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…

  15. 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.

  16. 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

  17. 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

  18. 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.

  19. Evaluating North Sea carbon sources using radiogenic (224Ra and 228Ra) and stable carbon isotope (DI13C) tracers

    NASA Astrophysics Data System (ADS)

    Burt, William; Thomas, Helmuth; Hagens, Mathilde; Brenner, Heiko; Paetsch, Johannes; Clargo, Nikki

    2015-04-01

    In the North Sea, much uncertainty still exists regarding the role of boundary fluxes (e.g. benthic input from sediments or lateral inputs from the coastline) in the overall biogeochemical cycling of the system. The stable carbon isotope signature of dissolved inorganic carbon (δ13C-DIC) is a common tool for following transformations of carbon in the water column and identifying carbon sources and sinks. Here, analyses of the first basin-wide observations of δ13C-DIC reveal that a balance between biological production and respiration, as well as a freshwater input near the European continental coast, predominantly control surface distributions in the North Sea. A strong relationship between the biological component of DIC (DICbio) and δ13C-DIC is then used to quantify the metabolic DIC flux associated with changes in the carbon isotopic signature. Correlations are also found between δ13C-DIC and naturally-occurring Radium isotopes (224Ra and 228Ra), which have well-identified sources from the seafloor and coastal boundaries. The relationship between δ13C-DIC and the longer-lived 228Ra isotope (half-life = 5.8 years) is used to derive a metabolic DIC flux from the European continental coastline. 228Ra is also shown to be a highly effective tracer of North Sea total alkalinity (TA) compared to the more conventional use of salinity as a tracer. Coastal alkalinity inputs are calculated using relationships with 228Ra, and ratios of DIC and TA suggest denitrification as the main metabolic pathway for the formation of these coastal inputs. Finally, coastal TA inputs are translated into inputs of protons to quantify their impact on the buffering capacity of the Southern North Sea.

  20. Protein splicing of inteins with atypical glutamine and aspartate C-terminal residues.

    PubMed

    Amitai, Gil; Dassa, Bareket; Pietrokovski, Shmuel

    2004-01-30

    Inteins are protein-splicing domains present in many proteins. They self-catalyze their excision from the host protein, ligating their former flanks by a peptide bond. The C-terminal residue of inteins is typically an asparagine (Asn). Cyclization of this residue to succinimide causes the final detachment of inteins from their hosts. We studied protein-splicing activity of two inteins with atypical C-terminal residues. One having a C-terminal glutamine (Gln), isolated from Chilo iridescent virus (CIV), and another unique intein, first reported here, with a C-terminal aspartate, isolated from Carboxydothermus hydrogenoformans (Chy). Protein-splicing activity was examined in the wild-type inteins and in several mutants with N- and C-terminal amino acid substitutions. We demonstrate that both wild-type inteins can protein splice, probably by new variations of the typical protein-splicing mechanism. Substituting the atypical C-terminal residue to the typical Asn retained protein-splicing only in the CIV intein. All diverse C-terminal substitutions in the Chy intein (Asp(345) to Asn, Gln, Glu, and Ala) abolished protein-splicing and generated N- and C-terminal cleavage. The observed C-terminal cleavage in the Chy intein ending with Ala cannot be explained by cyclization of this residue. We present and discuss several new models for reactions in the protein-splicing pathway.

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

    PubMed

    Jeong, Jee-Yeong; Kim, Jiwook; Kim, Bokyoum; Kim, Joowon; Shin, Yusom; Kim, Judeok; Ryu, Siejeong; Yang, Yu-Mi; Song, Kyoung Seob

    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 Ca(2+) 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.

  2. 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

  3. Acanthomatous ameloblastoma with atypical foci in five dogs.

    PubMed

    Malmberg, Jennifer L; Howerth, Elizabeth W; Powers, Barb E; Schaffer, Paula A

    2017-03-01

    Acanthomatous ameloblastoma is a common, locally invasive, nonmetastasizing tumor of the canine oral cavity. The long-term prognosis for canine acanthomatous ameloblastoma is good if complete excision can be achieved, usually by maxillectomy or mandibulectomy. A variant of acanthomatous ameloblastoma with atypical foci was noted in 5 dogs. There was no age, breed, or sex predisposition. Atypical cells were pleomorphic with a high mitotic rate. They were immunohistochemically negative for cytokeratin, vimentin, melan A, PNL2, CD3, Pax5, CD18, chromogranin A, and synaptophysin. Ultrastructurally, the atypical cells had modest amounts of electron-lucent cytoplasm, abundant rough endoplasmic reticulum, zonula adherens junctions, cleaved or irregular nuclei, and occasional cytoplasmic structures consistent with secretory granules or lysosomes. Complete excision was achieved by maxillectomy or mandibulectomy in 3 dogs; the lesion was incompletely excised in 2 dogs. No ancillary therapy was elected in any patient. No local recurrence or distant metastasis was reported in any case. One patient died of heart failure 20 mo following complete excision; all other patients were alive at last follow-up (average follow-up: 18.8 mo, range: 6-30 mo). The histogenesis of the atypical foci is unclear, but atypical foci within acanthomatous ameloblastoma do not appear to be associated with metastasis or with a poor prognosis relative to acanthomatous ameloblastoma with typical histologic morphology.

  4. Atypical periprosthetic acetabular fracture in long-term alendronate therapy

    PubMed Central

    Marongiu, Giuseppe; Capone, Antonio

    2016-01-01

    Summary Bisphosphonates have been commonly used in the treatment of osteoporosis, demonstrating its efficacy in fracture risk reduction. However, even if are generally safe and well tolerated, concerns have emerged about atypical fractures related to its prolonged use. Although atypical femoral fracture are more common, case reports demonstrated that even other skeletal areas can be involved by unusual pattern of fracture. We report a atypical acetabular periprosthetic fracture in a 83-year-old female patient after prolonged alendronate treatment for osteoporosis and isolated acetabular revision surgery. The patient underwent to clinical, bioumoral and radiological evaluation and all the history cases were fully reported. We believe this periprosthetic fracture, according to the available data, may have similar underlying pathology to atypical femoral fractures. Awareness of symptoms, in addition to a regular radiographic survey may facilitate early diagnosis and possible prevention of spontaneous periprosthetic fractures, in patients receiving bisphosphonate therapy beyond 5 years. The treatment of this atypical periprosthetic fracture should include both surgical than pharmacological therapy to obtained bone healing. PMID:28228784

  5. Clinically significant drug interactions with atypical antipsychotics.

    PubMed

    Kennedy, William Klugh; Jann, Michael W; Kutscher, Eric C

    2013-12-01

    Atypical antipsychotics [also known as second-generation antipsychotics (SGAs)] have become a mainstay therapeutic treatment intervention for patients with schizophrenia, bipolar disorders and other psychotic conditions. These agents are commonly used with other medications--most notably, antidepressants and antiepileptic drugs. Drug interactions can take place by various pharmacokinetic, pharmacodynamic and pharmaceutical mechanisms. The pharmacokinetic profile of each SGA, especially with phase I and phase II metabolism, can allow for potentially significant drug interactions. Pharmacodynamic interactions arise when agents have comparable receptor site activity, which can lead to additive or competitive effects without alterations in measured plasma drug concentrations. Additionally, the role of drug transporters in drug interactions continues to evolve and may effect both pharmacokinetic and pharmacodynamic interactions. Pharmaceutical interactions occur when physical incompatibilities take place between agents prior to drug absorption. Approximate therapeutic plasma concentration ranges have been suggested for a number of SGAs. Drug interactions that markedly increase or decrease the concentrations of these agents beyond their ranges can lead to adverse events or diminished clinical efficacy. Most clinically significant drug interactions with SGAs occur via the cytochrome P450 (CYP) system. Many but not all drug interactions with SGAs are identified during drug discovery and pre-clinical development by employing a series of standardized in vitro and in vivo studies with known CYP inducers and inhibitors. Later therapeutic drug monitoring programmes, clinical studies and case reports offer methods to identify additional clinically significant drug interactions. Some commonly co-administered drugs with a significant potential for drug-drug interactions with selected SGAs include some SSRIs. Antiepileptic mood stabilizers such as carbamazepine and valproate, as

  6. 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

  7. 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

  8. The biological effects of {sup 224}Ra injected into dogs

    SciTech Connect

    Muggenburg, B.A.; Lloyd, R.D.; Hahn, F.F.; Griffith, W.C.; Boecker, B.B.

    1994-11-01

    The purpose of this study was to investigate the toxicity of injected {sup 224}Ra in the dog. Radium-224 is a short-lived isotope of radium with a half-life of 3.62 d. When administered parenterally, it deposits on bone surfaces; because of its short half-life, most of its energy is deposited on bone surfaces, in a manner similar to plutonium. The experimental design included a comparison to the exposed human population. Instead of using a single injection of {sup 224}Ra, groups were included in which dogs were injected once, 10 times, or 50 times. This design provided groups that could be compared to the multiple injections often used in people for the treatment of ankylosing spondylitis.

  9. 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.

  10. Glucagon-like peptide-1 receptors agonists (GLP1 RA).

    PubMed

    Kalra, Sanjay

    2013-10-01

    The glucagon-like peptide-1 receptors agonists (GLP1RA) are a relatively new class of drugs, used for management of type 2 diabetes. This review studies the characteristics of these drugs, focusing upon their mechanism of action, intra-class differences, and utility in clinical practice. It compares them with other incretin based therapies, the dipeptidyl peptidase-IV inhibitors, and predicts future developments in the use of these molecules, while highlighting the robust indications for the use of these drugs.

  11. Characterization of the CpxRA regulon in Haemophilus ducreyi.

    PubMed

    Labandeira-Rey, Maria; Brautigam, Chad A; Hansen, Eric J

    2010-11-01

    The Haemophilus ducreyi 35000HP genome encodes a homolog of the CpxRA two-component cell envelope stress response system originally characterized in Escherichia coli. CpxR, the cytoplasmic response regulator, was shown previously to be involved in repression of the expression of the lspB-lspA2 operon (M. Labandeira-Rey, J. R. Mock, and E. J. Hansen, Infect. Immun. 77:3402-3411, 2009). In the present study, the H. ducreyi CpxR and CpxA proteins were shown to closely resemble those of other well-studied bacterial species. A cpxA deletion mutant and a CpxR-overexpressing strain were used to explore the extent of the CpxRA regulon. DNA microarray and real-time reverse transcriptase (RT) PCR analyses indicated several potential regulatory targets for the H. ducreyi CpxRA two-component regulatory system. Electrophoretic mobility shift assays (EMSAs) were used to prove that H. ducreyi CpxR interacted with the promoter regions of genes encoding both known and putative virulence factors of H. ducreyi, including the lspB-lspA2 operon, the flp operon, and dsrA. Interestingly, the use of EMSAs also indicated that H. ducreyi CpxR did not bind to the promoter regions of several genes predicted to encode factors involved in the cell envelope stress response. Taken together, these data suggest that the CpxRA system in H. ducreyi, in contrast to that in E. coli, may be involved primarily in controlling expression of genes not involved in the cell envelope stress response.

  12. Atypical cellular blue nevus or malignant blue nevus?*

    PubMed Central

    Daltro, Luise Ribeiro; Yaegashi, Lygia Bertalha; Freitas, Rodrigo Abdalah; Fantini, Bruno de Carvalho; Souza, Cacilda da Silva

    2017-01-01

    Blue nevus is a benign melanocytic lesion whose most frequent variants are dendritic (common) blue nevus and cellular blue nevus. Atypical cellular blue nevus presents an intermediate histopathology between the typical and a rare variant of malignant blue nevus/melanoma arising in a cellular blue nevus. An 8-year-old child presented a pigmented lesion in the buttock since birth, but with progressive growth in the last two years. After surgical excision, histopathological examination revealed atypical cellular blue nevus. Presence of mitoses, ulceration, infiltration, cytological atypia or necrosis may occur in atypical cellular blue nevus, making it difficult to differentiate it from melanoma. The growth of blue nevus is unusual and considered of high-risk for malignancy, being an indicator for complete resection and periodic follow-up of these patients. PMID:28225968

  13. 223Ra levels fed in the 223Fr β decay

    NASA Astrophysics Data System (ADS)

    Abdul-Hadi, A.; Barci, V.; Weiss, B.; Maria, H.; Ardisson, G.; Hussonnois, M.; Constantinescu, O.

    1993-01-01

    The 223Fr β decay was reinvestigated using high-resolution single γ spectrometry as well as γ-γ coincidence techniques. For single γ-spectra measurements, radiochemically pure 223Fr sources were obtained by chromatographic separation from a 75 MBq activity 227Ac parent source and continuously purified of 223Ra and daughters. The analysis of the γ spectra of 30 sources showed the existence of 131 γ lines, of which 87 are reported for the first time in the 223Fr β decay although many of them are observed following the 227Th α decay. The 223Fr half-life was remeasured and found to be T1/2=22.00+/-0.07 min. γ-γ-t coincidence measurements were also carried out with 223Fr purified sources. The 223Ra level scheme was built on the basis of our γ data, as well as 227Th α-decay data. Among the 32 excited 223Ra levels, of which 22 were also known from 227Th α decay, 13 are newly reported from 223Fr β decay. Low energy levels (E<400 keV) may be classified as parity doublet bands according to the predictions of the reflection asymmetric rotor model. Above a 700 keV gap, a coexistence of symmetric and asymmetric shapes including both static and dynamic octupole correlations is suggested.

  14. 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

  15. Crystallization of the head and galectin-like domains of porcine adenovirus isolate NADC-1 fibre

    PubMed Central

    Guardado-Calvo, Pablo; Llamas-Saiz, Antonio L.; Fox, Gavin C.; Glasgow, Joel N.; van Raaij, Mark J.

    2009-01-01

    The porcine adenovirus NADC-1 isolate, a strain of porcine adenovirus type 4, has a fibre with an atypical architecture. In addition to a classical virus-attachment region, shaft and head domains, it contains an additional galectin-like domain C-­terminal to the head domain and connected to the head domain by a long RGD-containing loop. The galectin-like domain contains two putative carbohydrate-recognition domains. The head and galectin-like domains have been independently crystallized. Diffraction data have been obtained to 3.2 Å resolution from crystals of the head domain and to 1.9 Å resolution from galectin-like domain crystals. PMID:19923738

  16. Atypical Neuroleptic Malignant Syndrome Associated with Use of Clozapine

    PubMed Central

    Juliana, Granada-Romero; Fernando, Camargo-Arenas Juan

    2017-01-01

    The Neuroleptic Malignant Syndrome (NMS) is a medical emergency of infrequent presentation in the emergency department, which is associated with the use of psychiatric drugs, such as typical and atypical antipsychotics. Our case addresses a 55-year-old patient diagnosed with undifferentiated schizophrenia for 10 years, who had been receiving clozapine and clonazepam as part of their treatment. This patient presents the symptoms of Neuroleptic Malignant Syndrome without fever, which improves with treatment especially with the withdrawal of clozapine. In the absence of fever and clinical improvement, the patient is considered to have an atypical presentation of this disease. PMID:28303200

  17. Atypical forms of lupus vulgaris - a case series.

    PubMed

    Saritha, Mohanan; Parveen, Basheer Ahamed; Anandan, Venkatesan; Priyavathani, Malathy R; Tharini, Karuvelan G

    2009-02-01

    Atypical presentations of cutaneous tuberculosis are not so uncommon and are frequently overlooked in clinical practice, leading to late diagnosis and increased morbidity. We report three cases of lupus vulgaris with atypical presentations. The cases mimicked other chronic skin lesions like actinomycosis, mycetoma, and so on. Strong clinical suspicion, histopathology, and response to antituberculous treatment led to the diagnosis and all three had excellent response to treatment. Today, when tuberculosis threatens to burst into pandemics again, early diagnosis and treatment are more important than ever for control and prevention of morbidity.

  18. Atypical Neuroleptic Malignant Syndrome Associated with Use of Clozapine.

    PubMed

    Leonardo, Quevedo-Florez; Juliana, Granada-Romero; Fernando, Camargo-Arenas Juan

    2017-01-01

    The Neuroleptic Malignant Syndrome (NMS) is a medical emergency of infrequent presentation in the emergency department, which is associated with the use of psychiatric drugs, such as typical and atypical antipsychotics. Our case addresses a 55-year-old patient diagnosed with undifferentiated schizophrenia for 10 years, who had been receiving clozapine and clonazepam as part of their treatment. This patient presents the symptoms of Neuroleptic Malignant Syndrome without fever, which improves with treatment especially with the withdrawal of clozapine. In the absence of fever and clinical improvement, the patient is considered to have an atypical presentation of this disease.

  19. Critical appraisal of eculizumab for atypical hemolytic uremic syndrome.

    PubMed

    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.

  20. Decay and In-Beam Studies of Neutron-Deficient Po and Ra Isotopes at JYFL

    NASA Astrophysics Data System (ADS)

    Leino, M.; Allatt, R. G.; Andreyev, A. N.; Cocks, J. F. C.; Dorvaux, O.; Enqvist, T.; Eskola, K.; Helariutta, K.; Huyse, M.; Jones, P. M.; Julin, R.; Juutinen, S.; Kankaanpaeae, H.; Keenan, A.; Kettunen, H.; Kuusiniemi, P.; Muikku, M.; Rahkila, P.; Savelius, A.; Trzaska, W. H.; Uusitalo, J.; van Duppen, P.

    1999-05-01

    An extensive program to study the production, decay properties, and nuclear structure of very neutron-deficient polonium and radium nuclei is underway at the Department of Physics, University of Jyvaeskylae, Finland (JYFL). The main tools used in these studies are the gas-filled recoil separator RITU and various germanium gamma-ray arrays. In the course of these studies, among others the following new isotopes have been produced: 204Ra, 203Ra, and 202Ra. Isomeric alpha decaying states have been discovered in 203Ra and 191Po. Fine structure in the decay of 192Po to the oblate and prolate band heads in 188Pb has been observed. In-beam gamma-ray spectra have been, for the first time, measured for 192Po, 206Ra, 208Ra, and 210Ra. Development of collectivity in nuclei in the Po-Ra region and the systematics of reduced alpha widths will be discussed.

  1. Atypical protein kinase Clambda binds and regulates p70 S6 kinase.

    PubMed Central

    Akimoto, K; Nakaya, M; Yamanaka, T; Tanaka, J; Matsuda, S; Weng, Q P; Avruch, J; Ohno, S

    1998-01-01

    p70 S6 kinase (p70 S6K) has been implicated in the regulation of cell cycle progression. However, the mechanism of its activation is not fully understood. In the present work, evidence is provided that an atypical protein kinase C (PKC) isotype, PKClambda, is indispensable, but not sufficient, for the activation of p70 S6K. Both the regulatory and kinase domains of PKClambda associate directly with p70 S6K. Overexpression of the kinase domain without kinase activity or the regulatory domain of PKClambda results in the suppression of the serum-induced activation of p70 S6K. In addition, two types of dominant-negative mutants of PKClambda, as well as a kinase-deficient mutant of p70 S6K, suppress serum-induced DNA synthesis and E2F activation. The overexpresion of the active form of PKClambda, however, fails to activate p70 S6K. These results suggest that PKClambda is a mediator in the regulation of p70 S6K activity and plays an important role in cell cycle progression. PMID:9761742

  2. An evaluation of ²²⁶Ra and ²²⁸Ra in drinking water in several counties in Texas, USA.

    PubMed

    Landsberger, S G; George, G

    2013-11-01

    Naturally Occurring Radioactive Material (NORM) or Technology Enhanced Naturally Occurring Radioactive Material (TENORM) can be a potential health risk. It is now well known that the underlying geology in many parts of Texas has given rise to levels of (226)Ra and (228)Ra that often exceed the limits set by the US Environmental Protection Agency. A detailed literature search was undertaken to assess the levels of (226)Ra and (228)Ra in all of the Texas counties. Several statistical evaluations of the data were performed. The Hickory aquifer in the Llano Uplift region of Texas has consistently had the highest number of (226)Ra and (228)Ra concentrations above the legal limit. As well many of the affected rural communities may not have the financial resources to rectify the problem.

  3. 24 CFR 599.505 - Coordinating responsible authority (CoRA).

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... (CoRA). 599.505 Section 599.505 Housing and Urban Development Regulations Relating to Housing and... Requirements § 599.505 Coordinating responsible authority (CoRA). Within 30 days of the Renewal Community... identifying the coordinating responsible authority (CoRA), which is the entity, organization or persons...

  4. 24 CFR 599.505 - Coordinating responsible authority (CoRA).

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... (CoRA). 599.505 Section 599.505 Housing and Urban Development Regulations Relating to Housing and... Requirements § 599.505 Coordinating responsible authority (CoRA). Within 30 days of the Renewal Community... identifying the coordinating responsible authority (CoRA), which is the entity, organization or persons...

  5. 24 CFR 599.505 - Coordinating responsible authority (CoRA).

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... (CoRA). 599.505 Section 599.505 Housing and Urban Development Regulations Relating to Housing and... Requirements § 599.505 Coordinating responsible authority (CoRA). Within 30 days of the Renewal Community... identifying the coordinating responsible authority (CoRA), which is the entity, organization or persons...

  6. 24 CFR 599.505 - Coordinating responsible authority (CoRA).

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... (CoRA). 599.505 Section 599.505 Housing and Urban Development Regulations Relating to Housing and... Requirements § 599.505 Coordinating responsible authority (CoRA). Within 30 days of the Renewal Community... identifying the coordinating responsible authority (CoRA), which is the entity, organization or persons...

  7. 24 CFR 599.505 - Coordinating responsible authority (CoRA).

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... (CoRA). 599.505 Section 599.505 Housing and Urban Development Regulations Relating to Housing and... Requirements § 599.505 Coordinating responsible authority (CoRA). Within 30 days of the Renewal Community... identifying the coordinating responsible authority (CoRA), which is the entity, organization or persons...

  8. Opening of the closed water area and consequent changes of ²²⁸Ra/²²⁶Ra activity ratios in coastal lagoon Nakaumi, southwest Japan.

    PubMed

    Nomura, Ritsuo; Nakamura, Kosaku; Seto, Koji; Inoue, Mutsuo; Kofuji, Hisaki

    2013-11-01

    In Lake Nakaumi, the second largest coastal lagoon in Japan, artificially closed (Honjyo) area, which was left untouched for 28 years, was partly opened in May, 2009. (228)Ra/(226)Ra ratio of waters in Honjyo area and Lake Nakaumi showed a well-tuned seasonal variation exhibiting high value in summer. After the opening event, however, the (228)Ra/(226)Ra ratios in the Honjyo water showed an unclear seasonal variation in both surface and deep water. This opening event caused the change of active movement of lake and marine water.

  9. A naturally occurring cowpox virus with an ectromelia virus A-type inclusion protein gene displays atypical A-type inclusions.

    PubMed

    Okeke, Malachy Ifeanyi; Hansen, Hilde; Traavik, Terje

    2012-01-01

    Human orthopoxvirus (OPV) infections in Europe are usually caused by cowpox virus (CPXV). The genetic heterogeneity of CPXVs may in part be due to recombination with other OPV species. We describe the characterization of an atypical CPXV (CPXV-No-H2) isolated from a human patient in Norway. CPXV-No-H2 was characterized on the basis of A-type inclusion (ATI) phenotype as well as the DNA region containing the p4c and atip open reading frames. CPXV-No-H2 produced atypical V(+/) ATI, in which virions are on the surface of ATI but not within the ATI matrix. Phylogenetic analysis showed that the atip gene of CPXV-No-H2 clustered closely with that of ectromelia virus (ECTV) with a bootstrap support of 100% whereas its p4c gene is diverged compared to homologues in other OPV species. By recombination analysis we identified a putative crossover event at nucleotide 147, downstream the start of the atip gene. Our results suggest that CPXV-No-H2 originated from a recombination between CPXV and ECTV. Our findings are relevant to the evolution of OPVs.

  10. 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…

  11. 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

  12. Atypical extraventricular neurocytoma:A report of two cases.

    PubMed

    Kamboj, Meenakshi; Gandhi, Jatin S; Mehta, Anurag; Sharma, Anila

    2015-01-01

    Central neurocytomas are tumors with neuronal differentiation, generally arising in the lateral ventricles in the region of foramen of Monro. Whenever these tumors arise in the brain parenchyma they are called "extraventricular neurocytomas". We present two unusual cases of extraventricular atypical neurocytomas at uncommon locations with a very high Ki-67 index. The WHO grading of this tumor is yet to be answered.

  13. Genetics Home Reference: atypical hemolytic-uremic syndrome

    MedlinePlus

    ... abnormalities lead to kidney damage and, in many cases, kidney failure and ESRD. Although gene mutations increase the risk of atypical hemolytic-uremic syndrome , studies suggest that they are often not sufficient to cause the disease. In people with certain genetic changes, the signs ...

  14. Subitizing and Counting in Typical and Atypical Development

    ERIC Educational Resources Information Center

    Schleifer, Patrick; Landerl, Karin

    2011-01-01

    Enumeration performance in standard dot counting paradigms was investigated for different age groups with typical and atypically poor development of arithmetic skills. Experiment 1 showed a high correspondence between response times and saccadic frequencies for four age groups with typical development. Age differences were more marked for the…

  15. Nonconvulsive Status Epilepticus Resembling Clinical Absence with Atypical EEG Pattern

    PubMed Central

    Mysore, Channaiah Srikanth; Zabad, Rana; Bertoni, John

    2017-01-01

    Objective. We are reporting two cases: a patient with steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) and another patient with secondary progressive multiple sclerosis (SPMS), both presenting with altered mental status (AMS) and later diagnosed with nonconvulsive atypical absence status epilepticus (AS), with atypical EEG changes. Methods. A report of two cases. Results. A patient with history of SREAT and the other with SPMS had multiple admissions due to AMS. For both, EEG revealed the presence of a high voltage generalized sharply contoured theta activity. A diagnosis of NCSE with clinical features of AS was made based on both clinical and EEG features. There was significant clinical and electrographic improvement with administration of levetiracetam for both patients in addition to sodium valproate and Solumedrol for the SREAT patient. Both patients continued to be seizure free on follow-up few months later. Conclusions. This is a report of two cases of atypical AS, with atypical EEG, in patients with different neurological conditions. Prompt clinical and EEG recovery occurred following appropriate medical treatment. We think that this condition might be underreported and could significantly benefit from prompt treatment when appropriately diagnosed. PMID:28203468

  16. Transposition with atypical coronary pattern: the Aubert technique.

    PubMed

    Pita-Fernández, Ana; González-López, María T; Gil-Jaurena, Juan M

    2017-03-06

    The arterial switch operation is currently the gold standard technique for repair of transposition of the great arteries. Some atypical coronary patterns such as intramural, interarterial, and a unique posterior button are associated with more complexity and surgical risk. We report a successful Aubert operation for transposition of the great arteries associated with a single and interarterial coronary artery arising from a posterior sinus.

  17. Late atypical atrial flutter after ablation of atrial fibrillation.

    PubMed

    Ferreira, Raquel; Primo, João; Adão, Luís; Gonzaga, Anabela; Gonçalves, Helena; Santos, Rui; Fonseca, Paulo; Santos, José; Gama, Vasco

    2016-10-01

    Cardiac surgery for structural heart disease (often involving the left atrium) and radiofrequency catheter ablation of atrial fibrillation have led to an increased incidence of regular atrial tachycardias, often presenting as atypical flutters. This type of flutter is particularly common after pulmonary vein isolation, especially after extensive atrial ablation including linear lesions and/or defragmentation. The authors describe the case of a 51-year-old man, with no relevant medical history, referred for a cardiology consultation in 2009 for paroxysmal atrial fibrillation. After failure of antiarrhythmic therapy, he underwent catheter ablation, with criteria of acute success. Three years later he again suffered palpitations and atypical atrial flutter was documented. The electrophysiology study confirmed the diagnosis of atypical left flutter and reappearance of electrical activity in the right inferior pulmonary vein. This vein was again ablated successfully and there has been no arrhythmia recurrence to date. In an era of frequent catheter ablation it is essential to understand the mechanism of this arrhythmia and to recognize such atypical flutters.

  18. Atypical presentations of melioidosis as emerging threat: a case report.

    PubMed

    Mukhopadhyay, Chiranjoy; Dey, Arindam; Bairy, Indira

    2007-10-01

    We report two atypical presentations of melioidosis as mediastinal lymphadenitis and prostatic abscess with Burkholderia pseudomallei, the emerging category 2 organism which led to diagnostic and therapeutic dilemma and thereby, delay in appropriate management. Any similar presentation should always be supported by microbiological opinion without any delay, which can help in instituting proper antibiotics with successful outcome.

  19. Middle aortic syndrome: an atypical case--a case report.

    PubMed

    Adovasio, R; Canci, U

    2000-06-01

    This case concerns a 61-year-old woman presenting with middle aortic syndrome treated by an aortoaortic thoracoabdominal polytetrafluoroethylene bypass and a right renal Dacron bypass. The case was atypical because of the patient's advanced age at the time of clinical presentation.

  20. Atypical diaper dermatitis: contact allergy to mercapto compounds.

    PubMed

    Onken, Anna Theresa; Baumstark, Julia; Belloni, Benedetta; Ring, Johannes; Schnopp, Christina

    2011-01-01

    We present a case of allergic contact dermatitis in an 18-month-old boy caused by type-IV allergy to mercapto mix and mercaptobenzothiazole as components of the elastic border of diapers. Allergic contact dermatitis should be included in the differential diagnosis of diaper dermatitis, especially in difficult-to-treat cases or atypical clinical presentation.

  1. Atypical pneumonia associated with a Mycoplasma isolate in a kitten.

    PubMed

    Bongrand, Yannick; Blais, Marie-Claude; Alexander, Kate

    2012-10-01

    An atypical case of Mycoplasma pneumonia with an unusual radiographic and computed tomographic pattern was diagnosed in a Siamese kitten. The cat showed no response to broad-spectrum antibiotic therapy including enrofloxacin. The administration of doxycycline led to a dramatic clinical and radiographic improvement.

  2. 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…

  3. Upgrade Rate and Imaging Features of Atypical Apocrine Lesions.

    PubMed

    Chang Sen, Lauren Q; Berg, Wendie A; Carter, Gloria J

    2017-03-23

    The purpose of our work was to identify imaging features of atypical apocrine lesions and determine the rate of upgrade to ductal carcinoma in situ (DCIS) or invasive carcinoma at excision after such a diagnosis on percutaneous breast biopsy. From January 1, 2006, through October 8, 2013, a total of 33,157 breast core biopsies were performed at University of Pittsburgh Medical Center. Of those, 58 (0.2%) showed atypical apocrine lesions. For 24, atypical apocrine adenosis (AAA) or atypical apocrine metaplasia (AAM) was the only risk lesion, with no known ipsilateral malignancy, and the results of excision were available. The median patient age was 58 years (range 43-88). Among 24 atypical apocrine lesions (20 AAA and 4 AAM), four (16.7%; 95% confidence interval: 4.7, 37.4) were upgraded at excision: one invasive ductal carcinoma (grade 2, 0.2 cm, estrogen receptor positive, progesterone receptor positive, HER2/Neu negative) and three DCIS (two grade 3, one grade 2). All four upgraded lesions were AAA (20%; 4/20). Twelve AAA were seen as an irregular (n = 9) or circumscribed (n = 3) mass on ultrasound; three masses had calcifications. Six of 20 (30%) AAA were seen on biopsy of calcifications only and calcifications were within two AAA lesions at histopathology. One AAA (1/20, 5%) was asymmetry only, and one (1/20, 5%) a persistently enhancing MR focus. All four malignancies were masses on ultrasound (three irregular, one circumscribed), and three malignancies had calcifications (two coarse heterogeneous, one amorphous). While concordant with an irregular or circumscribed mass on imaging, with or without amorphous or coarse heterogeneous calcifications, AAA merits excision with a 20% upgrade rate to malignancy. Further study of AAM is warranted.

  4. 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

  5. Predictors of aggressive clinical phenotype among immunohistochemically confirmed atypical adenomas.

    PubMed

    Zaidi, Hasan A; Cote, David J; Dunn, Ian F; Laws, Edward R

    2016-12-01

    Despite formal pathological criteria, not all atypical pituitary adenomas display clinically aggressive behavior. We set out to determine which factors predict a clinically aggressive phenotype among a cohort of atypical pituitary adenomas. Medical records were retrospectively reviewed from April 2008 to July 2015. Of 569 pituitary adenomas, 47 (8.3%) patients were surgically treated for atypical adenomas as defined by the WHO criteria. Clinically aggressive adenomas were defined as occurring in those patients who necessitated additional therapeutic intervention after the index (first) surgery, including additional surgery, medical therapy, or radiosurgery. Forty-seven patients with histopathological and immunohistochemical confirmation of atypical adenomas were identified and of these, 23 were noted to have a clinically aggressive course. Among the remaining 24 patients, the disease remained quiescent after the index surgery. On univariate analysis, clinically aggressive lesions were more likely to have a larger axial diameter on MRI (2.9±1.9cm vs. 1.9±0.7cm, p=0.02), greater incidence of cavernous sinus invasion (65.2% vs. 20.8%, p<0.01), and greater incidence of clival extension (60.9% vs. 0, p<0.01) on preoperative imaging. The two groups were equivalent with regard to immunohistochemical staining for ACTH, HGH, LH, FSH, PRL, and TSH. Clinically aggressive lesions, however, trended towards a greater average MIB-1 proliferative index (7.5%±4.9 vs. 6.0%±3.6, p=0.03). On multivariate analysis, the MIB-1 proliferative index trended towards statistical significance (p=0.06) as an independent predictor of clinical aggressiveness. Atypical pituitary adenomas are defined by a rigid set of immunohistochemical markers, but not all necessarily demonstrate an aggressive clinical phenotype.

  6. Genome-wide association study of atypical psychosis.

    PubMed

    Kanazawa, Tetsufumi; Ikeda, Masashi; Glatt, Stephen J; Tsutsumi, Atsushi; Kikuyama, Hiroki; Kawamura, Yoshiya; Nishida, Nao; Miyagawa, Taku; Hashimoto, Ryota; Takeda, Masatoshi; Sasaki, Tsukasa; Tokunaga, Katsushi; Koh, Jun; Iwata, Nakao; Yoneda, Hiroshi

    2013-10-01

    Atypical psychosis with a periodic course of exacerbation and features of major psychiatric disorders [schizophrenia (SZ) and bipolar disorder (BD)] has a long history in clinical psychiatry in Japan. Based upon the new criteria of atypical psychosis, a Genome-Wide Association Study (GWAS) was conducted to identify the risk gene or variants. The relationships between atypical psychosis, SZ and BD were then assessed using independent GWAS data. Forty-seven patients with solid criteria of atypical psychosis and 882 normal controls (NCs) were scanned using an Affymetrics 6.0 chip. GWAS SZ data (560 SZ cases and 548 NCs) and GWAS BD (107 cases with BD type 1 and 107 NCs) were compared using gene-based analysis. The most significant SNPs were detected around the CHN2/CPVL genes (rs245914, P = 1.6 × 10(-7)) , COL21A1 gene (rs12196860, P = 2.45 × 10(-7) ), and PYGL/TRIM9 genes (rs1959536, P = 7.73 × 10(-7) ), although none of the single-nucleotide polymorphisms exhibited genome-wide significance (P = 5 × 10(-8) ). One of the highest peaks was detected on the major histocompatibility complex region, where large SZ GWASs have previously disclosed an association. The gene-based analysis suggested significant enrichment between SZ and atypical psychosis (P = 0.01), but not BD. This study provides clues about the types of patient whose diagnosis lies between SZ and BD. Studies with larger samples are required to determine the causal variant.

  7. High Resolution Study of the 222Ra Exotic Decay

    NASA Astrophysics Data System (ADS)

    Hussonnois, M.; Le Du, J. F.; Brillard, L.; Dalmasso, J.; Ardisson, G.

    1991-10-01

    The 14C-decay of 222Ra has been reinvestigated using a strong 230U source and the magnetic superconducting spectrometer SOLENO in view of looking for a possible feeding of the first 208Pb excited state. 210 14C-events were recorded in a single peak of (30.930±0.090) MeV energy; the 14C branching ratio values b=(2.31±0.31)×10-10 and <1×10-12 to the respective 208Pb ground and Iπ=3- octupole state were deduced.

  8. 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.

  9. Clinical decision support for atypical orders: detection and warning of atypical medication orders submitted to a computerized provider order entry system.

    PubMed

    Woods, Allie D; Mulherin, David P; Flynn, Allen J; Stevenson, James G; Zimmerman, Christopher R; Chaffee, Bruce W

    2014-01-01

    The specificity of medication-related alerts must be improved to overcome the pernicious effects of alert fatigue. A systematic comparison of new drug orders to historical orders could improve alert specificity and relevance. Using historical order data from a computerized provider order entry system, we alerted physicians to atypical orders during the prescribing of five medications: calcium, clopidogrel, heparin, magnesium, and potassium. The percentage of atypical orders placed for these five medications decreased during the 92 days the alerts were active when compared to the same period in the previous year (from 0.81% to 0.53%; p=0.015). Some atypical orders were appropriate. Fifty of the 68 atypical order alerts were over-ridden (74%). However, the over-ride rate is misleading because 28 of the atypical medication orders (41%) were changed. Atypical order alerts were relatively few, identified problems with frequencies as well as doses, and had a higher specificity than dose check alerts.

  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. Ra5G, a homologue of Ra5 in giant ragweed pollen: isolation, HLA-DR-associated activity and amino acid sequence.

    PubMed

    Goodfriend, L; Choudhury, A M; Klapper, D G; Coulter, K M; Dorval, G; Del Carpio, J; Osterland, C K

    1985-08-01

    Recent studies [Marsh et al. (1982) J. exp. Med. 155, 1439-1451; Coulter (1983) M.Sc. thesis, McGill University, Montreal, Canada; Coulter et al. (1983) in Genetic and Environmental Factors in Clinical Allergy (Edited by Marsh D.G., Blumenthal M.N. and Santilli J., Jr), University of Minnesota Press, Minneapolis, MN] have shown a highly significant association between HLA-Dw2/DR2 and host sensitivity to the 5000-D, 4-disulfide bonded protein Ra5S of short ragweed pollen. To extend these findings, we isolated Ra5G, an Ra5S-like protein, from giant ragweed pollen by gel and ion-exchange chromatography. The protein was homogeneous by polyacrylamide gel electrophoresis (pH 4.3), reverse-phase high-performance liquid chromatography, and antigenic assays. Its mol. wt and amino acid composition (including 8 half-cystine residues) were closely similar to Ra5S, but the two proteins had little or no antigenic or allergenic cross-reactivity. In a study of 200 ragweed-sensitive individuals, host sensitivity simultaneously to Ra5G and Ra5S was significantly associated with the DR2 allele. The amino acid sequence of Ra5G was determined and showed close homology with Ra5S. The potential function of a highly homologous decapeptidyl sequence stretch is discussed in relation to Ir gene control of immune response to the 2 proteins.

  12. Characterization of the RaD-X Mission Instruments

    NASA Technical Reports Server (NTRS)

    Gronoff, Guillaume; Mertens, Christopher J.; Norman, Ryan B.; Straume, Tore; Lusby, Terry C.

    2016-01-01

    The NASA Radiation Dosimetry Experiment (RaD-X) stratospheric balloon flight mission, launched on 25 September 2015, provided dosimetric measurements above the Pfotzer maximum. The goal of taking these measurements is to improve aviation radiation models by providing a characterization of cosmic ray primaries, which are the source of radiation exposure at aviation altitudes. The RaD-X science payload consists of four instruments. The main science instrument is a tissue-equivalent proportional counter (TEPC). The other instruments consisted of three solid state silicon dosimeters: Liulin, Teledyne total ionizing dose (TID) and RaySure detectors. To properly interpret the measurements, it is necessary to evaluate how the payload affects the radiation environment of the detectors. In addition, it is necessary to evaluate how the detectors react to the different particles impacting them. We present the results of the Geant-4 simulations of the interaction of the different radiations with the payload and the instruments. We show how it affect the measurements, and which instruments are better suited for future missions

  13. 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.

  14. Adult-onset Still's disease with atypical cutaneous manifestations

    PubMed Central

    Narváez Garcia, Francisco Javier; Pascual, María; López de Recalde, Mercè; Juarez, Pablo; Morales-Ivorra, Isabel; Notario, Jaime; Jucglà, Anna; Nolla, Joan M.

    2017-01-01

    Abstract The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition. In this study, we present 3 new cases of AOSD with atypical cutaneous manifestations diagnosed during a 30-year period in our department and review 78 additional cases previously reported (PubMed 1990–2016). These 81 patients form the basis of the present analysis. The overall prevalence of atypical cutaneous manifestations in our AOSD population was 14%. These manifestations may appear at any time over the course of the disease, and usually occur in patients who have persistent and severe disease, with a considerable frequency of clinical complications (23%), including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome. The most representative and frequent lesion among the nonclassical skin rashes is the development of persistent pruritic papules and/or plaques. Interestingly, these lesions show a distinctive histological pattern. Other, less frequently observed lesions include urticaria and urticaria-like eruptions, generalized or widespread non-pruritic persistent erythema, vesiculopustular eruptions, a widespread peau d’orange appearance of the skin, and edema of the eyelids mimicking dermatomyositis without any accompanying skin lesion. The great majority of these patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy

  15. Impaired IL-7 signaling may explain a case of atypical JAK3-SCID.

    PubMed

    Li, Jun; Nara, Hidetoshi; Rahman, Mizanur; Juliana, Farha Matin; Araki, Akemi; Asao, Hironobu

    2010-02-01

    Janus kinase 3-severe combined immunodeficiency (JAK3-SCID) is an autosomal recessive immunodeficiency disease caused by various mutations in the JAK3 gene. Typical JAK3-SCID is characterized by a phenotype in which B cells are present but T and NK cells are not, the T(-)B(+)NK(-) phenotype, and by impaired signaling through cytokine receptors that use the common gamma chain (gammac) subunit. An atypical JAK3-SCID case carrying a single glutamate to glycine substitution mutation (E481G) in the JH3 domain of one JAK3 allele, and a deletion mutation (del482-596) in the JH3 and JH2 domains of the other allele was reported previously. Although this patient had CD4(+) T cells and NK cells unlike typical cases, the CD4(+) T cells were functionally impaired. We report here that the JAK3-E481G mutant transduced IL-2-, IL-4-, IL-15-, and IL-21-induced signals as efficiently as wild-type JAK3. However, this mutant failed to respond to IL-7 by phosphorylating JAK1, JAK3, or STAT5. The other mutant JAK3, JAK3-del482-596, was non-functional. Thus, an impaired IL-7 signal may cause SCID and compromise T-cell differentiation, even if the IL-15 signal is preserved and supports NK-cell development, as in this patient.

  16. A-type Lamins Form Distinct Filamentous Networks with Differential Nuclear Pore Complex Associations.

    PubMed

    Xie, Wei; Chojnowski, Alexandre; Boudier, Thomas; Lim, John S Y; Ahmed, Sohail; Ser, Zheng; Stewart, Colin; Burke, Brian

    2016-10-10

    The nuclear lamina is a universal feature of metazoan nuclear envelopes (NEs) [1]. In mammalian cells, it appears as a 10-30 nm filamentous layer at the nuclear face of the inner nuclear membrane (INM) and is composed primarily of A- and B-type lamins, members of the intermediate filament family [2]. While providing structural integrity to the NE, the lamina also represents an important signaling and regulatory platform [3]. Two A-type lamin isoforms, lamins A and C (LaA and LaC), are expressed in most adult human cells. Encoded by a single gene, these proteins are largely identical, diverging only in their C-terminal tail domains. By contrast with that of LaC, the unique LaA tail undergoes extensive processing, including farnesylation and endo-proteolysis [4, 5]. However, functional differences between LaA and LaC are still unclear. Compounding this uncertainty, the structure of the lamina remains ill defined. In this study, we used BioID, an in vivo proximity-labeling method to identify differential interactors of A-type lamins [6]. One of these, Tpr, a nuclear pore complex (NPC) protein, is highlighted by its selective association with LaC. By employing superresolution microscopy, we demonstrate that this Tpr association is mirrored in enhanced interaction of LaC with NPCs. Further superresolution studies visualizing both endogenous A- and B-type lamins have allowed us to construct a nanometer-scale model of the mammalian nuclear lamina. Our data indicate that different A- and B-type lamin species assemble into separate filament networks that together form an extended composite structure at the nuclear periphery providing attachment sites for NPCs, thereby regulating their distribution.

  17. Phelan-McDermid syndrome in two adult brothers: atypical bipolar disorder as its psychopathological phenotype?

    PubMed Central

    Verhoeven, Willem MA; Egger, Jos IM; Willemsen, Marjolein H; de Leijer, Gert JM; Kleefstra, Tjitske

    2012-01-01

    The 22q13.3 deletion, or Phelan-McDermid syndrome, is characterized by global intellectual disability, generalized hypotonia, severely delayed or absent speech associated with features of autism spectrum disorder, and minor dysmorphisms. Its behavioral phenotype comprises sleep disturbances, communication deficits, and motor perseverations. Data on psychological dysfunctions are so far not available. Previous studies have suggested that the loss of one copy of the gene SH3 and multiple ankyrin repeat domains 3 (SHANK3) is related to the neurobehavioral phenotype. Additional genes proximal to SHANK3 are also likely to play a role in the phenotype of patients with larger deletions. The present paper describes two adult brothers with an identical 2.15 Mb 22qter (22q13.32q13.33) deletion, of whom the youngest was referred for evaluation of recurrent mood changes. In both patients, magnetic resonance imaging of the brain showed hypoplasia of the vermis cerebelli. Extensive clinical examinations led to a final diagnosis of atypical bipolar disorder, of which symptoms fully remitted during treatment with a mood stabilizer. In the older brother, a similar psychopathological picture appeared to be present, although less severe and with a later onset. It is concluded that the behavioral phenotype of the 22q13.3 deletion syndrome comprises absent or delayed speech and perseverations with associated autistic-like features, whereas its psychopathological phenotype comprises an atypical bipolar disorder. The latter may have implications for the treatment regime of the syndrome-related behavioral disturbances. PMID:22570549

  18. 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.

  19. 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.

  20. 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

  1. A modified drought index for WMO RA VI

    NASA Astrophysics Data System (ADS)

    Pietzsch, S.; Bissolli, P.

    2011-10-01

    Drought is a phenomenon which can cause large economical impact even in Europe. To assess the magnitude and the spatial extension of drought events, it is important to have a standardized drought index which is applicable for a large climatically heterogeneous region like Europe or the WMO RA VI Region (Europe and the Middle East). Such an index should describe the drought phenomenon adequately, but it should also be derivable from meteorological quantities which are easily and timely available in whole Europe. In a first investigation, some candidates for drought indices were chosen, compared and assessed for applicability in whole Europe. The most appropriate one seems to be the widely known Standardized Precipitation Index (SPI) which is a standardized and handy measurement of drought for any location and requires nothing but precipitation data. However, it has turned out that for some places in the RA VI Region, notably in arid regions in summer, the SPI does not always provide reasonable or easily interpretable results. For that reason, some modifications of the SPI have been tried out and tested statistically. It seems that the gamma distribution of precipitation which is used for computation of the SPI is in fact the most appropriate one and other distributions have not improved the results substantially. On the other hand a so called zero correction, which sets very small precipitation totals to dry values, only dependent on the precipitation distribution, but independent on the individual location delivers more reasonable results. Maps of the new modified drought index and its anomalies from the climate normal are produced quasi-operationally and distributed via the Internet each month. The drought monitoring is part of the monitoring programme of the WMO RA VI Pilot Regional Climate Centre on Climate Monitoring (RCC-CM) hosted by the German Meteorological Service (Deutscher Wetterdienst, DWD), and the maps can be found on its present RCC-CM platform (

  2. 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.

  3. Monoclonal gammopathy of renal significance triggering atypical haemolytic uraemic syndrome.

    PubMed

    Mahmood, Usman; Isbel, Nicole; Mollee, Peter; Mallett, Andrew; Govindarajulu, Sridevi; Francis, Ross

    2017-02-01

    Haemolytic uraemic syndrome is a rare condition with an overall incidence of one to two cases in a population of 100 000 and approximately 10% of these cases are classified as atypical. Atypical haemolytic uraemic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and acute kidney injury. aHUS can be genetic, acquired or idiopathic (negative genetic screening and no environmental triggers). We describe a case of aHUS triggered by monoclonal gammopathy of renal significance (MGRS) successfully treated with plasmapheresis and a bortezomib-based chemotherapy regimen, resulting in marked improvement in renal function and other markers of haemolysis. This patient has been in remission for more than 2 years currently.

  4. Hyperglycemia secondary to consumption of cocaine and atypical antipsychotic drugs.

    PubMed

    Argente Villaplana, Carlos R; Civera Andrés, Miguel; Real Collado, José T; Martínez-Hervás, Sergio; Ascaso Gimilio, Juan F; Carmena Rodríguez, Rafael

    2008-10-01

    Drugs such as cocaine and atypical antipsychotic agents, such as olanzapine, are sometimes related to hyperglycemia. Whereas cocaine raises plasma glucose through catecholamine release, atypical antipsychotic agents mainly increase appetite and induce weight gain and the development of metabolic syndrome. Moreover, the latter group of drugs also act independently from weight gain or adiposity, due to inhibition of beta pancreatic cells and reduction of peripheral insulin action. We present the case of a 29-year-old non-diabetic woman with severe acute hyperglycemia in the context of a suicide attempt through intake of olanzapine and cocaine. After discontinuation of olanzapine and cocaine consumption, glycemia was immediately normalized without subsequent diagnosis of diabetes.

  5. Nightmare-induced atypical midventricular tako-tsubo cardiomyopathy.

    PubMed

    Fibbi, Veronica; Ballo, Piercarlo; Nannini, Marco; Consoli, Lorenzo; Chechi, Tania; Bribani, Andrea; Fiorentino, Francesca; Chiodi, Leandro; Zuppiroli, Alfredo

    2015-01-01

    Tako-Tsubo cardiomyopathy (TTC) is a reversible cardiomyopathy characterized by acute left ventricular segmental dysfunction, whose clinical presentation resembles that of acute myocardial infarction. The syndrome often follows a psychophysical stressful event and is characterized by echocardiographic evidence of akinesia of the left ventricular mid-apical segments. Atypical echocardiographic patterns of TTC have recently been described, often triggered by emotional stressors, rather than physical. In this report, we describe a case of atypical TTC triggered by an unusual stressor (recurrent nightmare) in a 45-year-old woman, with peculiar clinical presentation and evolution characterized by persistent loss of consciousness, neurological deterioration, absence of typical symptoms of TTC, and features suggestive of a hysterical crisis.

  6. Nightmare-Induced Atypical Midventricular Tako-Tsubo Cardiomyopathy

    PubMed Central

    Fibbi, Veronica; Ballo, Piercarlo; Nannini, Marco; Consoli, Lorenzo; Chechi, Tania; Bribani, Andrea; Fiorentino, Francesca; Chiodi, Leandro; Zuppiroli, Alfredo

    2015-01-01

    Tako-Tsubo cardiomyopathy (TTC) is a reversible cardiomyopathy characterized by acute left ventricular segmental dysfunction, whose clinical presentation resembles that of acute myocardial infarction. The syndrome often follows a psychophysical stressful event and is characterized by echocardiographic evidence of akinesia of the left ventricular mid-apical segments. Atypical echocardiographic patterns of TTC have recently been described, often triggered by emotional stressors, rather than physical. In this report, we describe a case of atypical TTC triggered by an unusual stressor (recurrent nightmare) in a 45-year-old woman, with peculiar clinical presentation and evolution characterized by persistent loss of consciousness, neurological deterioration, absence of typical symptoms of TTC, and features suggestive of a hysterical crisis. PMID:25788945

  7. 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.

  8. 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.

  9. Atypical presentations of acute disseminated encephalomyelitis (ADEM) in HIV infection.

    PubMed

    Naidoo, Ansuya; Paruk, Hoosain; Bhagwan, Bhupendra; Moodley, Anand

    2017-02-01

    Acute disseminated encephalomyelitis is a monophasic demyelinating disorder of the central nervous system associated with various viral infections including HIV infection. We present the findings of seven HIV-infected patients with mild to moderate immunosuppression presenting with atypical features. Four patients had a multiphasic course; three patients had tumefactive lesions, and two patients had corpus callosum lesions. Two patients with the multiphasic course also had tumefactive lesions. Their clinical and radiological findings are presented. Despite the few cases, we propose that the dysimmune process lying between marked immunosuppression (CD4 < 200 cells/μL) and normal CD4 counts (CD4 > 500 cells/μL) might be responsible for these atypical presentations.

  10. 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

  11. Identification of the first case of atypical scrapie in Japan

    PubMed Central

    IMAMURA, Morikazu; MIYAZAWA, Kohtaro; IWAMARU, Yoshifumi; MATSUURA, Yuichi; YOKOYAMA, Takashi; OKADA, Hiroyuki

    2016-01-01

    A Corriedale ewe was confirmed as the first atypical scrapie case during an active surveillance program for transmissible spongiform encephalopathies in small ruminants in Japan. The animal was homozygous for the AF141RQ haplotype of PRNP. The animal showed clinical neurological signs possibly due to listeriosis before culling. Western blot analysis showed an unusual multiple banded pattern with a low-molecular fragment at ~7 kDa. Histopathology revealed suppurative meningoencephalitis caused by listeriosis in the brainstem. Fine granular to globular immunostaining of disease-associated prion proteins was mainly detected in the neuropil of the spinal tract of the trigeminal nerve and in the white matter of the spinocerebellar tract. Based on these results, this case was conclusively diagnosed as atypical scrapie with encephalitic listeriosis. PMID:27616556

  12. Transmission of atypical scrapie to homozygous ARQ sheep

    PubMed Central

    OKADA, Hiroyuki; MIYAZAWA, Kohtaro; IMAMURA, Morikazu; IWAMARU, Yoshifumi; MASUJIN, Kentaro; MATSUURA, Yuichi; YOKOYAMA, Takashi

    2016-01-01

    Two Cheviot ewes homozygous for the A136L141R154Q171 (AL141RQ) prion protein (PrP) genotype were exposed intracerebrally to brain pools prepared using four field cases of atypical scrapie from the United Kingdom. Animals were clinically normal until the end of the experiment, when they were culled 7 years post-inoculation. Limited accumulation of disease-associated PrP (PrPSc) was observed in the cerebellar molecular layer by immunohistochemistry, but not by western blot or enzyme-linked immunosorbent assay. In addition, PrPSc was partially localized in astrocytes and microglia, suggesting that these cells have a role in PrPSc processing, degradation or both. Our results indicate that atypical scrapie is transmissible to AL141RQ sheep, but these animals act as clinically silent carriers with long incubation times. PMID:27320968

  13. [Atypical presentation of cutaneous sporotrichosis in an alcoholic patient].

    PubMed

    Nassif, Priscila Wolf; Granado, Inandiara Rafaela Marco de Oliveira; Ferraz, Juliana Soares; Souza, Robson; Nassif, Aissar Eduardo

    2012-06-15

    Sporotrichosis is a subcutaneous mycosis with a high prevalence in Brasil. It is caused by the dimorphic fungus Sporothrix schenckii, and may lead to different clinical presentations. The disseminated cutaneous form is uncommon and corresponds to 4 percent of the total number of cases. We report a case of atypical disseminated sporotrichosis in an alcoholic patient, whose culture for fungi revealed the presence of Sporothrix schenckii. The patient was treated with itraconazole 200 mg/day for 6 months with clinical clearing.

  14. Monocular nasal hemianopia from atypical sphenoid wing meningioma.

    PubMed

    Stacy, Rebecca C; Jakobiec, Frederick A; Lessell, Simmons; Cestari, Dean M

    2010-06-01

    Neurogenic monocular nasal field defects respecting the vertical midline are quite uncommon. We report a case of a unilateral nasal hemianopia that was caused by compression of the left optic nerve by a sphenoid wing meningioma. Histological examination revealed that the pathology of the meningioma was consistent with that of an atypical meningioma, which carries a guarded prognosis with increased chance of recurrence. The tumor was debulked surgically, and the patient's visual field defect improved.

  15. Atypical presentation of antiphospholipid syndrome: a case report.

    PubMed

    Mariotti, Cesare; Giovannini, Alfonso; Reibaldi, Michele; Saitta, Andrea; Viti, Francesca; Nicolai, Michele

    2014-09-01

    We report an atypical presentation of Antiphospholipid syndrome (APS) with concomitant subhyaloid hemorrhage, engorged and tortuous retinal veins, intraretinal hemorrhages, and cotton wool spots in a 38-year-old female. Medical treatment was preferred to any invasive treatment. The subhyaloid hemorrhage resolved spontaneously and the patient recovered a visual acuity of 20/20 in her right eye 3 months after the initial episode. A prompt diagnosis of this condition is fundamental to consider a systemic treatment to avoid any further thrombosis.

  16. 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

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

    PubMed Central

    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. Pharmacological management of atypical antipsychotic-induced weight gain.

    PubMed

    Baptista, Trino; ElFakih, Yamily; Uzcátegui, Euderruh; Sandia, Ignacio; Tálamo, Eduardo; Araujo de Baptista, Enma; Beaulieu, Serge

    2008-01-01

    Excessive bodyweight gain was reported during the 1950s as an adverse effect of typical antipsychotic drug treatment, but the magnitude of bodyweight gain was found to be higher with the atypical antipsychotic drugs that were introduced after 1990. Clozapine and olanzapine produce the greatest bodyweight gain, ziprasidone and aripiprazole have a neutral influence, and quetiapine and risperidone cause an intermediate effect. In the CATIE study, the percentage of patients with bodyweight gain of >7% compared with baseline differed significantly between the antipsychotic drugs, i.e. 30%, 16%, 14%, 12% and 7% for olanzapine, quetiapine, risperidone, perphenazine (a typical antipsychotic) and ziprasidone, respectively (p<0.001). Appetite stimulation is probably a key cause of bodyweight gain, but genetic polymorphisms modify the bodyweight response during treatment with atypical antipsychotics. In addition to nutritional advice, programmed physical activity, cognitive-behavioural training and atypical antipsychotic switching, pharmacological adjunctive treatments have been assessed to counteract excessive bodyweight gain. In some clinical trials, nizatidine, amantadine, reboxetine, topiramate, sibutramine and metformin proved effective in preventing or reversing atypical antipsychotic-induced bodyweight gain; however, the results are inconclusive since few randomized, placebo-controlled clinical trials have been conducted. Indeed, most studies were short-term trials without adequate statistical power and, in the case of metformin, nizatidine and sibutramine, the results are contradictory. The tolerability profile of these agents is adequate. More studies are needed before formal recommendations on the use of these drugs can be made. Meanwhile, clinicians are advised to use any of these adjunctive treatments according to their individual pharmacological and tolerability profiles, and the patient's personal and family history of bodyweight gain and metabolic dysfunction.

  19. Atypical presentation of Boerhaave's syndrome as Enterococcal bacterial pericardial effusion.

    PubMed

    Saha, Arin; Jarvis, Martin; Thorpe, James A C; O'Regan, David J

    2007-02-01

    Boerhaave's perforation is a serious condition describing spontaneous transmural perforation of the oesophagus. The classical presentation of this condition is vomiting, lower thoracic pain and subcutaneous emphysema. However, the condition often presents atypically and it is important to reach the correct diagnosis quickly. We present the case of a 54-year-old woman with a Boerhaave's perforation that presented as Enterococcal bacterial pericardial effusion.

  20. Clues to the diagnosis of atypical melanocytic lesions.

    PubMed

    McKee, Phillip H

    2010-01-01

    This review, based on a lecture given at the 2009 Update in Dermatopathology meeting held at the Institute of Dermatology (London, UK), discusses the problem of misdiagnosing atypical benign melanocytic lesions as melanoma and the alternative problem of interpreting naevoid melanoma as a banal naevus. Consequences are considered. Brief consideration of a range of other melanoma variants that may be a source of diagnostic difficulty is also included.

  1. 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.

  2. Neurologic involvement in patients with atypical Chediak-Higashi disease

    PubMed Central

    Westbroek, Wendy; Cullinane, Andrew R.; Groden, Catherine A.; Bhambhani, Vikas; Golas, Gretchen A.; Baker, Eva H.; Lehky, Tanya J.; Snow, Joseph; Ziegler, Shira G.; Adams, David R.; Dorward, Heidi M.; Hess, Richard A.; Huizing, Marjan; Gahl, William A.; Toro, Camilo

    2016-01-01

    Objective: To delineate the developmental and progressive neurodegenerative features in 9 young adults with the atypical form of Chediak-Higashi disease (CHD) enrolled in a natural history study. Methods: Patients with atypical clinical features, but diagnostically confirmed CHD by standard evaluation of blood smears and molecular genotyping, underwent complete neurologic evaluation, MRI of the brain, electrophysiologic examination, and neuropsychological testing. Fibroblasts were collected to investigate the cellular phenotype and correlation with the clinical presentation. Results: In 9 mildly affected patients with CHD, we documented learning and behavioral difficulties along with developmental structural abnormalities of the cerebellum and posterior fossa, which are apparent early in childhood. A range of progressive neurologic problems emerge in early adulthood, including cerebellar deficits, polyneuropathies, spasticity, cognitive decline, and parkinsonism. Conclusions: Patients with undiagnosed atypical CHD manifesting some of these wide-ranging yet nonspecific neurologic complaints may reside in general and specialty neurology clinics. The absence of the typical bleeding or infectious diathesis in mildly affected patients with CHD renders them difficult to diagnose. Identification of these individuals is important not only for close surveillance of potential CHD-related systemic complications but also for a full understanding of the natural history of CHD and the potential role of the disease-causing protein, LYST, to the pathophysiology of other neurodevelopmental and neurodegenerative disorders. PMID:26944273

  3. An inherited LMNA gene mutation in atypical Progeria syndrome.

    PubMed

    Doubaj, Yassamine; De Sandre-Giovannoli, Annachiara; Vera, Esteves-Vieira; Navarro, Claire Laure; Elalaoui, Siham Chafai; Tajir, Mariam; Lévy, Nicolas; Sefiani, Abdelaziz

    2012-11-01

    Hutchinson-Gilford Progeria syndrome (HGPS) is a rare genetic disorder, characterized by several clinical features that begin in early childhood, recalling an accelerated aging process. The diagnosis of HGPS is based on the recognition of common clinical features and detection of the recurrent heterozygous c.1824C>T (p.Gly608Gly) mutation within exon 11 in the Lamin A/C encoding gene (LMNA). Besides "typical HGPS," several "atypical progeria" syndromes (APS) have been described, in a clinical spectrum ranging from mandibuloacral dysplasia to atypical Werner syndrome. These patients's clinical features include progeroid manifestations, such as short stature, prominent nose, premature graying of hair, partial alopecia, skin atrophy, lipodystrophy, skeletal anomalies, such as mandibular hypoplasia and acroosteolyses, and in some cases severe atherosclerosis with metabolic complications. APS are due in several cases to de novo heterozygous LMNA mutations other than the p.Gly608Gly, or due to homozygous BAFN1 mutations in Nestor-Guillermo Progeria syndrome (NGPS). We report here and discuss the observation of a non-consanguineous Moroccan patient presenting with atypical progeria. The molecular studies showed the heterozygous mutation c.412G>A (p.Glu138Lys) of the LMNA gene. This mutation, previously reported as a de novo mutation, was inherited from the apparently healthy father who showed a somatic cell mosaicism.

  4. Atypical (symplastic) leiomyoma of the uterus--a case report.

    PubMed

    Siti-Aishah, M A; Noriah, O; Malini, M N; Zainul-Rashid, M R; Das, S

    2011-01-01

    A 30-year-old, nulliparous woman presented with a history of subfertility. On examination she was found to have uterine fibroid of 28 weeks size of gravid uterus and subsequently laporatomy myomectomy was performed. Multilobulated masses, with diameters ranging from 22 mm to 160 mm were found. Cut sections of the lobulated masses showed whitish whorled cut surface. One of the multilobulated masses had a cystic cavity, measuring 60x50x35 mm(3). Light microscopic findings of the mass with the cystic cavity showed a well-circumscribed cellular tumour composed of cells exhibiting moderate nuclear atypia which were enlarged, nuclei with prominent chromatin clumping and were distributed in areas. Some tumour cells showed large nuclear pseudoinclusions, multinucleated or multilobated tumour giant cells, smudging and few enlarged nucleoli. Mitotic activity was 4 MFs per 10 HPFs. Occasional cells with intracytoplasmic inclusions resembling rhabdoid - like features were seen. There were no atypical mitoses or tumour necroses were noted. Diagnosis of atypical leiomyoma or symplastic leiomyoma was made. Atypical or symplastic leiomyomas are rare in the region of Malaysia and the present case discusses its incidence in younger age, its morphological features along with diagnosis and clinical outcome.

  5. 230Th, 226Ra and 222Rn in abyssal sediments

    NASA Astrophysics Data System (ADS)

    Kadko, David

    1980-09-01

    A model that predicts the flux of 222Rn out of deep-sea sediment is presented. The radon is ultimately generated by 230Th which is stripped from the overlying water into the sediment. Data from many authors are compared with the model predictions. It is shown that the continental contribution of ionium is not significant, and that at low sedimentation rates, biological mixing and erosional processes strongly affect the surface concentration of the ionium. Two cores from areas of slow sediment accumulation, one from a manganese nodule region of the central Pacific and one from the Rio Grande Rise in the Atlantic were analyzed at closely spaced intervals for 230Th, 226Ra, and 210Pb. The Pacific core displayed evidence of biological mixing down to 12 cm and had a sedimentation rate of only 0.04 cm/kyr. The Atlantic core seemed to be mixed to 8 cm and had a sedimentation rate of 0.07 cm/kyr. Both cores had less total excess 230Th than predicted. Radium sediment profiles are generated from the 230Th model. Adsorbed, dissolved, and solid-phase radium is considered. According to the model, diffusional losses of radium are especially important at low sedimentation rates. Any particulate, or excess radium input is ignored in this model. The model fits the two analyzed cores if the fraction of total radium available for adsorption-desorption is about 0.5-0.7, and if K, the distribution coefficient, is about 1000. Finally, the flux of radon out of the sediments is derived from the model-generated radium profiles. It is shown that the resulting standing crop of 222Rn in the overlying water may be considered as an added constraint in budgeting 230Th and 226Ra in deep-sea sediments.

  6. Functionalized NaA nanozeolites labeled with 224,225Ra for targeted alpha therapy

    NASA Astrophysics Data System (ADS)

    Piotrowska, Agata; Leszczuk, Edyta; Bruchertseifer, Frank; Morgenstern, Alfred; Bilewicz, Aleksander

    2013-11-01

    The 223Ra, 224Ra, and 225Ra radioisotopes exhibit very attractive nuclear properties for application in radionuclide therapy. Unfortunately the lack of appropriate bifunctional ligand for radium is the reason why these radionuclides have not found application in receptor-targeted therapy. In the present work, the potential usefulness of the NaA nanozeolite as a carrier for radium radionuclides has been studied. 224Ra and 225Ra, α-particle emitting radionuclides, have been absorbed in the nanometer-sized NaA zeolite (30-70 nm) through simple ion exchange. 224,225Ra-nanozeolites exhibited very high stability in solutions containing physiological salt, EDTA, amino acids, and human serum. To make NaA nanozeolite particles dispersed in water their surface was modified with a silane coupling agent containing poly(ethylene glycol) molecules. This functionalization approach let us covalently attach a biomolecule to the NaA nanozeolite surface.

  7. Functionalized NaA nanozeolites labeled with (224,225)Ra for targeted alpha therapy.

    PubMed

    Piotrowska, Agata; Leszczuk, Edyta; Bruchertseifer, Frank; Morgenstern, Alfred; Bilewicz, Aleksander

    2013-01-01

    The (223)Ra, (224)Ra, and (225)Ra radioisotopes exhibit very attractive nuclear properties for application in radionuclide therapy. Unfortunately the lack of appropriate bifunctional ligand for radium is the reason why these radionuclides have not found application in receptor-targeted therapy. In the present work, the potential usefulness of the NaA nanozeolite as a carrier for radium radionuclides has been studied. (224)Ra and (225)Ra, α-particle emitting radionuclides, have been absorbed in the nanometer-sized NaA zeolite (30-70 nm) through simple ion exchange. (224,225)Ra-nanozeolites exhibited very high stability in solutions containing physiological salt, EDTA, amino acids, and human serum. To make NaA nanozeolite particles dispersed in water their surface was modified with a silane coupling agent containing poly(ethylene glycol) molecules. This functionalization approach let us covalently attach a biomolecule to the NaA nanozeolite surface.

  8. Weeding atypical glandular cell look-alikes from the true atypical lesions in liquid-based Pap tests: a review.

    PubMed

    Wood, Moira D; Horst, Julie A; Bibbo, Marluce

    2007-01-01

    The purpose of this review is to identify features that separate atypical glandular cells (AGC) associated with glandular neoplasia from its mimickers, both benign and neoplastic. We reviewed cases of AGC diagnosed on liquid-based Pap tests (LBP) for which corresponding histological follow-up was available. A review of the literature for similar studies in LBP tests was also conducted. We find that certain benign mimics can be reliably separated from AGC, but recommend caution in attempting to increase specificity at the risk of losing sensitivity. Although accounting for only a small percentage of diagnoses AGC require a thorough clinical evaluation, including colposcopy. Most cases are ultimately found to be benign. When evaluating smears suspicious for AGC, it is important to examine the subtle features which make truly atypical cells discernible from their numerous benign mimickers.

  9. Iloperidone: a new benzisoxazole atypical antipsychotic drug. Is it novel enough to impact the crowded atypical antipsychotic market?

    PubMed

    Albers, Lawrence James; Musenga, Alessandro; Raggi, Maria Augusta

    2008-01-01

    Iloperidone is a new-generation atypical antipsychotic agent, acting as a serotonin/dopamine (5-HT(2A)/D(2)) antagonist, under development by Vanda Pharmaceuticals for the treatment of schizophrenia, bipolar disorder and other psychiatric conditions. Chemically, iloperidone is a benzisoxazole, like risperidone, and shows a multiple receptor binding profile, sharing this feature with the other atypical antipsychotic agents. Administered orally, the drug is highly bound to plasma proteins and extensively metabolised. Several clinical trials have been carried out, to check efficacy, safety and side effects. In order to introduce iloperidone as an agent for the treatment of schizophrenia, a short overview of the disease and of the most important antipsychotic drugs available or under development will be reported. Iloperidone pharmacokinetics and pharmacodynamics are presented herein, together with an evaluation of clinical safety and efficacy results.

  10. 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

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

    PubMed

    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.

  12. 13cRA regulates the differentiation of antler chondrocytes through targeting Runx3.

    PubMed

    Zhang, Hong-Liang; Cao, Hang; Yang, Zhan-Qing; Geng, Shuang; Wang, Kai; Yu, Hai-Fan; Guo, Bin; Yue, Zhan-Peng

    2017-03-01

    Although 13cRA is involved in the regulation of cellular proliferation and differentiation, its physiological roles in chondrocyte proliferation and differentiation still remain unknown. Here, we showed that 13cRA could induce the proliferation of sika deer antler chondrocytes and expression of Ccnd3 and Cdk6. Administration of 13cRA to antler chondrocytes resulted in an obvious increase in the expression of chondrocyte marker Col II and hypertrophic chondrocyte marker Col X. Silencing of Crabp2 expression by specific siRNA could prevent the 13cRA-induced up-regulation of Col X, whereas overexpression of Crabp2 showed the opposite effects. Further study found that Crabp2 mediated the regulation of 13cRA on the expression of Runx3 which was highly expressed in the antler cartilage and inhibited the differentiation of antler chondrocytes. Moreover, attenuation of Runx3 expression greatly raised 13cRA-induced chondrocyte differentiation. Simultaneously, 13cRA could stimulate the expression of Cyp26a1 and Cyp26b1 in the antler chondrocytes. Inhibition of Cyp26a1 and/or Cyp26b1 reinforced the effects of 13cRA on the expression of Col X and Runx3, while overexpression of Cyp26b1 rendered the antler chondrocytes hyposensitive to 13cRA. Collectively, 13cRA may play an important role in the differentiation of antler chondrocytes through targeting Runx3. Crabp2 enhances the effects of 13cRA on chondrocyte differentiation, while Cyp26a1 and Cyp26b1 weaken the sensitivity of antler chondrocytes to 13cRA.

  13. 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.

  14. Rapid method for the determination of 226Ra in hydraulic fracturing wastewater samples

    DOE PAGES

    Maxwell, Sherrod L.; Culligan, Brian K.; Warren, Richard A.; ...

    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.

  15. First Measurement of the Atomic Electric Dipole Moment of (225)Ra.

    PubMed

    Parker, R H; Dietrich, M R; Kalita, M R; Lemke, N D; Bailey, K G; Bishof, M; Greene, J P; Holt, R J; Korsch, W; Lu, Z-T; Mueller, P; O'Connor, T P; Singh, J T

    2015-06-12

    The radioactive radium-225 ((225)Ra) atom is a favorable case to search for a permanent electric dipole moment. Because of its strong nuclear octupole deformation and large atomic mass, (225)Ra is particularly sensitive to interactions in the nuclear medium that violate both time-reversal symmetry and parity. We have developed a cold-atom technique to study the spin precession of (225)Ra atoms held in an optical dipole trap, and demonstrated the principle of this method by completing the first measurement of its atomic electric dipole moment, reaching an upper limit of |d((225)Ra)|<5.0×10(-22)  e cm (95% confidence).

  16. 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

  17. Genomic and functional characterization of the modular broad-host-range RA3 plasmid, the archetype of the IncU group.

    PubMed

    Kulinska, Anna; Czeredys, Magdalena; Hayes, Finbarr; Jagura-Burdzy, Grazyna

    2008-07-01

    IncU plasmids are a distinctive group of mobile elements with highly conserved backbone functions and variable antibiotic resistance gene cassettes. The IncU archetype is conjugative plasmid RA3, whose sequence (45,909 bp) shows it to be a mosaic, modular replicon with a class I integron different from that of other IncU replicons. Functional analysis demonstrated that RA3 possesses a broad host range and can efficiently self-transfer, replicate, and be maintained stably in alpha-, beta-, and gammaproteobacteria. RA3 contains 50 open reading frames clustered in distinct functional modules. The replication module encompasses the repA and repB genes embedded in long repetitive sequences. RepA, which is homologous to antitoxin proteins from alpha- and gammaproteobacteria, contains a Cro/cI-type DNA-binding domain present in the XRE family of transcriptional regulators. The repA promoter is repressed by RepA and RepB. The minireplicon encompasses repB and the downstream repetitive sequence r1/r2. RepB shows up to 80% similarity to putative replication initiation proteins from environmental plasmids of beta- and gammaproteobacteria, as well as similarity to replication proteins from alphaproteobacteria and Firmicutes. Stable maintenance functions of RA3 are most like those of IncP-1 broad-host-range plasmids and comprise the active partitioning apparatus formed by IncC (ParA) and KorB (ParB), the antirestriction protein KlcA, and accessory stability components KfrA and KfrC. The RA3 origin of transfer was localized experimentally between the maintenance and conjugative-transfer operons. The putative conjugative-transfer module is highly similar in organization and in its products to transfer regions of certain broad-host-range environmental plasmids.

  18. 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

  19. Migration of the FDNPP-derived (134)Cs and (137)Cs along with (226)Ra and (228)Ra concentrations across the northwestern North Pacific Ocean.

    PubMed

    Inoue, M; Shirotani, Y; Nagao, S; Kofuji, H; Volkov, Y N; Nishioka, J

    2016-10-01

    We examined lateral distributions of (134)Cs, (137)Cs, (226)Ra, and (228)Ra in the surface seawaters around the Kuril Islands and the Kamchatka Peninsula in the northwestern North Pacific Ocean during June 2014. The sampling area included three water current areas, the Oyashio Current, the current from the Okhotsk Sea, and the coastal current along the east Kamchatka Peninsula. (226)Ra and (228)Ra distributions differed along the three currents. Low levels of (134)Cs were detected in the surface waters of the Oyashio Current (0.09-0.35 mBq/L), but it was <∼0.1 mBq/L at the surface along the other two currents. This indicates that the distribution of Fukushima Dai-ichi Nuclear Power Plant (FDNPP)-derived radiocesium in surface waters off the Kamchatka and along the Kuril Islands is predominantly governed by the Oyashio current system.

  20. Understanding the Public Domain.

    ERIC Educational Resources Information Center

    Russell, Carrie

    2003-01-01

    This overview of the public domain covers: defining the public domain; figuring out if a work is protected by copyright; being sure a work is in the public domain; asserting the copyright protection and term; the Creative Commons initiative; building the Information Commons; when permission is needed for using a public domain work; and special…

  1. All-trans-retinoic acid induces integrin-independent B-cell adhesion to ADAM disintegrin domains.

    PubMed

    Bridges, Lance C; Lingo, Joshuah D; Grandon, Rachel A; Kelley, Melissa D

    2008-04-15

    Cell adhesion is an integral aspect of immunity facilitating extravasation of immune cells during homing and activation. All -trans-Retinoic acid ( t-RA) regulates leukocyte differentiation, proliferation, and transmigration. However, the role of t-RA in immune cell adhesion is poorly defined. In this study, we evaluated the impact of t-RA and its metabolism on B and T cell adhesion. Specifically, we address the impact of t-RA on the adhesive properties of the human mature B and T cell lines RPMI 8866, Daudi and Jurkats. The effect of t-RA exposure on cell adhesion to vascular cell adhesion molecule-1 (VCAM-1), a well-established integrin counter receptor involved in immunity, and to nonconventional ADAM integrin ligands was assessed. We show for the first time that t-RA potently induces B cell adhesion in an integrin-independent manner to both VCAM-1 and select ADAM disintegrin domains. Using retinoid extraction and reverse-phase HPLC analysis, we identify the retinoid that is functionally responsible for this augmented adhesion. We also provide evidence that this novel t-RA adhesive response is not prototypical of lymphocytes since both Daudi and Jurkats do not alter their adhesive properties upon t-RA treatment. Further, the t-RA metabolic profiles between these lineages is distinct with 9- cis-retinoic acid being exclusively detected in Jurkat media. This study is the first to demonstrate that t-RA directly induces B cell adhesion in an integrin-independent manner and is not contingent upon t-RA metabolism.

  2. Role of Divalent Metal Ions in Atypical Nonlinear Plasma Protein Binding Behavior of Tigecycline.

    PubMed

    Singh, Ravi Shankar Prasad; Mukker, Jatinder Kaur; Deitchman, Amelia N; Drescher, Stephanie K; Derendorf, Hartmut

    2016-11-01

    In typical nonlinear plasma protein binding (PPB) behavior, the free fraction increases with increasing total concentrations. In contrast, when a drug exhibits atypical nonlinear PPB behavior, the free fraction decreases with increasing total concentrations. Tigecycline, a novel glycylcycline, exhibits atypical nonlinear PPB behavior, but the mechanism of such behavior is currently unknown. Because tigecycline can form complexes with metal ions, an interaction between metal ion, tigecycline, and plasma proteins was hypothesized but not further investigated. The current work explores the role of metal ions in the atypical nonlinear PPB behavior of tigecycline and proposes a plausible mechanism of atypical nonlinear PPB behavior. The addition of ethylenediaminetetraacetic acid resulted in 10- to 30-fold higher unbound fractions, and the atypical behavior was nullified. The saturation of ethylenediaminetetraacetic acid chelation, by addition of excessive divalent metal ions, such as calcium and magnesium, led to the return of the atypical nonlinear PPB behavior. Different possible mechanisms were evaluated by simulation, and a plausible mechanism was proposed.

  3. A Prospective Approach to Investigating the Natural History of Pre-Clinical Rheumatoid Arthritis (RA) Using First-Degree Relatives of Probands with RA

    PubMed Central

    Kolfenbach, Jason R.; Deane, Kevin D.; Derber, Lezlie A.; O’Donnell, Colin; Weisman, Michael H.; Buckner, Jane H.; Gersuk, Vivian H.; Wei, Shan; Mikuls, Ted R.; O’Dell, James; Gregersen, Peter K.; Keating, Richard M.; Norris, Jill M.; Holers, V. Michael

    2009-01-01

    Objective To describe a large, multi-center prospective cohort study of first-degree relatives (FDRs) of probands with rheumatoid arthritis (RA), and outline the utility of such a study in investigating the natural history of RA development. Methods 1058 FDRs, none of whom met the American College of Rheumatology (ACR) criteria for RA, have been enrolled into a prospective study investigating genetic and environmental influences on the development of RA-related autoimmunity. Demographic, epidemiologic, genetic, autoantibody, and physical examination data from the initial study enrollment visit is described for these FDRs, and the relationship is examined between genetic factors, autoantibodies, inflammation, and joint disease. Results Fifty-five percent of FDRs have ≥1 copy of the shared epitope (SE); 20% have ≥1 copy of PTPN22 polymorphism; ~16% are positive for rheumatoid factor (RF, including isotypes), and/or anti-cyclic citrullinated peptide (anti-CCP) antibody. RF-IgM positivity is associated with ≥1 tender joint/s on examination (OR 2.50, 95% CI 1.27 to 4.89, p<0.01), and elevated levels of CRP (OR 5.31, 95% CI 1.45 to 19.52, p = 0.01). Conclusion FDRs without RA demonstrate high prevalence of genetic risk factors and RA-related autoantibodies. Additionally, RF association with tender joints and elevated CRP suggests autoantibodies are a valid intermediate marker of RA-related autoimmunity in this cohort. This prospective FDR cohort will be a valuable resource for evaluating the relationship between genetic, epidemiologic factors and the development of RA-related autoimmunity. PMID:19950324

  4. 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.

  5. Association of atypical protein kinase C isotypes with the docker protein FRS2 in fibroblast growth factor signaling.

    PubMed

    Lim, Y P; Low, B C; Lim, J; Wong, E S; Guy, G R

    1999-07-02

    FRS2 is a docker protein that recruits signaling proteins to the plasma membrane in fibroblast growth factor signal transduction. We report here that FRS2 was associated with PKC lambda when Swiss 3T3 cells were stimulated with basic fibroblast growth factor. PKC zeta, the other member of the atypical PKC subfamily, could also bind FRS2. The association between FRS2 and PKC lambda is likely to be direct as shown by yeast two-hybrid analysis. The C-terminal fragments of FRS2 (amino acid residues 300-508) and SNT2 (amino acids 281-492), an isoform bearing 50% identity to FRS2, interacted with PKC lambda at a region (amino acids 240-562) that encompasses the catalytic domain. In vitro kinase assays revealed neither FRS2 nor SNT2 was a substrate of PKC lambda or zeta. Mutation of the alanine residue (Ala-120) to glutamate in the pseudo-substrate region of PKC lambda results in a constitutively active kinase that exhibited more than 2-fold greater binding to FRS2 in vitro than its "closed" wild-type counterpart. Tyrosine phosphorylation of FRS2 did not affect its binding to the constitutively active PKC lambda mutant, suggesting that the activation of PKC lambda is necessary and sufficient for its association with FRS2. It is likely that FRS2 serves as an anchoring protein for targeting activated atypical PKCs to the cell plasma membrane in signaling pathways.

  6. 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.

  7. Search for possible solar influences in Ra-226 decays

    NASA Astrophysics Data System (ADS)

    Stancil, Daniel D.; Balci Yegen, Sümeyra; Dickey, David A.; Gould, Chris R.

    Measurements of Ra-226 activity from eight HPGe gamma ray detectors at the NC State University PULSTAR Reactor were analyzed for evidence of periodic variations, with particular attention to annual variations. All measurements were made using the same reference source, and data sets were of varying length taken over the time period from September 1996 through August 2014. Clear evidence of annual variations was observed in data from four of the detectors. Short time periodograms from the data sets suggest temporal variability of both the amplitude and frequency of these variations. The annual variations in two of the data sets show peak values near the first of February, while surprisingly, the annual variations in the other two are roughly out of phase with the first two. Three of the four detectors exhibited annual variations over approximately the same time period. A joint statistic constructed by combining spectra from these three shows peaks approximating the frequencies of solar r-mode oscillations with νR = 11.74 cpy, m = 1, and l = 3, 5, 6. The fact that similar variations were not present in all detectors covering similar time periods rules out variations in activity as the cause, and points to differing sensitivities to unspecified environmental parameters instead. In addition to seasonal variations, the modulation of environmental parameters by solar processes remains a possible explanation of periodogram features, but without requiring new physics.

  8. Renal cell carcinoma: Atypical metastasis to inguinal lymph nodes

    PubMed Central

    Chaudhry, Qamar Saeed; Bhatty, Tanweer Ahmed Naveed; Khan, Ziauddin; Osman, Elsawi Medani

    2017-01-01

    Renal cell carcinoma (RCC) is a common tumor of the urinary tract. It is known to have variable presentations due to the extremely vascular nature of the organ. RCC are known to metastasize to lungs, bone, and brain commonly but atypical metastasis to various sites are reported in literature but as very rare pathology. We report a case of a 60-year-old female who presented with multiple inguinal and axillary lymph node enlargements which on excision biopsy showed metastatic RCC. RCC can present with synchronous metastatic deposits in the various organs. RCC can metastasize to some atypical sites as well such as thyroid, orbit, and neck as mentioned earlier in literature. The patient presenting with extra-regional lymph nodes like inguinal and axillary is extremely rare, and so far only one clinical case could be found from India in 2008. A 61-year-old female presented in the emergency department with left flank pain and hematuria. Imaging showed left swollen kidney but multiple lymph nodes in retroperitoneum, left inguinal and axillary region. Excisional biopsy confirmed metastatic renal clear cell carcinoma. The case was referred to an oncologist after left radical nephrectomy for further treatment. Renal cancer is quite common aggressive disease. Due to its vascular nature, it may present quite atypically as evident from literature. Although treatment of metastatic carcinoma is still controversial surgery is the mainstay of treatment and guidelines consider metastasectomy and cytoreductive nephrectomy as valid option followed by targeted systemic therapies. RCC has quite a high potential to metastasize in the versatile pattern, in our case, it is evident that valid management is still surgery but needs support from the multidisciplinary team. PMID:28216937

  9. “Atypical” atypical femur fractures and use of bisphosphonates

    PubMed Central

    van de Laarschot, Denise M.; Somford, Matthijs P.; Jager, Agnes; Oei, Edwin H.; Bos, P. Koen; Zillikens, M. Carola

    2016-01-01

    Summary Background Atypical femur fractures (AFFs) present a rare but serious condition associated with use of bisphosphonates. Underlying mechanisms and clinical risk factors remain unclear. According to the diagnostic criteria formulated by the ASBMR, a lateral localization of an AFF is required. Case history We present a patient who developed bilateral leg pain while using an oral bisphosphonate and aromatase inhibitor in the course of adjuvant treatment for breast cancer. Initially she was diagnosed with bone metastases and received radiotherapy on the right femur. However, the bilateral periosteal reactions of the subtrochanteric femur are highly suggestive of AFFs. Our case meets all criteria for AFF except that she presented with lesions at the medial side of the femur. Therefore they could be best described as “atypical” atypical femur fractures. Discussion Since the pathogenesis of AFFs is not fully understood, we cannot rule out that AFFs also occur in the medial femur or in other weight-baring bones. Hence we propose that medial stress reactions belong to a spectrum of atypical fractures associated with use of antiresorptive drugs. The localization may depend on yet unknown biomechanical factors. Conclusion We propose that these periosteal reactions of the subtrochanteric femur are in fact AFFs with uncommon medial localization and could hence be considered “atypical” AFFs. We recommend being alert of AFFs in patients with bone pain and medial subtrochanteric lesions. More epidemiological studies are needed to investigate the occurrence of both medial and lateral AFFs and to gain more insight into its frequency and pathogenesis. PMID:28228783

  10. [Haemophilus influenzae type B meningitis: typical and atypical presentation].

    PubMed

    Sánchez, J M; Zurro, F J; Ferreiro, D; Llana, R; Uría, D F

    1998-02-01

    We present 2 cases of Haemophilus influenzae meningitis. The first is a patient with atypical simptomatology: abdominal pain, fever and two days later pain in the back of his legs. Abdominal pathology was not found. The cerebrospinal fluid (CSF) showed polymorphonuclear cells, hyperproteinorachia and lowered glucose. CSF culture revealed Haemophilus influenzae, blood culture was sterile. The second had suffered surgery at maxilar and ethmoid sinuses four years before, and unknown germ meningitis 6 months before. Haemophilus influenzae was isolated from CSF cultures and CSF rhinorrhea was detected by isotopic cisternography.

  11. Atypical aHUS: State of the art.

    PubMed

    Nester, Carla M; Barbour, Thomas; de Cordoba, Santiago Rodriquez; Dragon-Durey, Marie Agnes; Fremeaux-Bacchi, Veronique; Goodship, Tim H J; Kavanagh, David; Noris, Marina; Pickering, Matthew; Sanchez-Corral, Pilar; Skerka, Christine; Zipfel, Peter; Smith, Richard J H

    2015-09-01

    Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs) have revealed distinct disease mechanisms within this heterogeneous group of diseases. As a direct result of this knowledge, both children and adults with complement-mediated TMA now enjoy higher expectations for long-term health. In this update on atypical hemolytic uremic syndrome, we review the clinical characteristics; the genetic and acquired drivers of disease; the broad spectrum of environmental triggers; and current diagnosis and treatment options. Many questions remain to be addressed if additional improvements in patient care and outcome are to be achieved in the coming decade.

  12. Posterior cortical atrophy: an atypical variant of Alzheimer disease.

    PubMed

    Suárez-González, Aida; Henley, Susie M; Walton, Jill; Crutch, Sebastian J

    2015-06-01

    Posterior cortical atrophy (PCA) is a neurodegenerative syndrome characterized by striking progressive visual impairment and a pattern of atrophy mainly involving posterior cortices. PCA is the most frequent atypical presentation of Alzheimer disease. The purpose of this article is to provide a summary of PCA's neuropsychiatric manifestations. Emotional and psychotic symptoms are discussed in the context of signal characteristic features of the PCA syndrome (the early onset, focal loss of visual perception, focal posterior brain atrophy) and the underlying cause of the disease. The authors' experience with psychotherapeutic intervention and PCA support groups is shared in detail.

  13. An atypical case of necrotizing fasciitis of the breast.

    PubMed

    Mufty, H; Smeets, A; Christiaens, M R

    2014-01-01

    Necrotizing fasciitis is a rare and aggressive soft tissue infection involving the fascia and subcutaneous tissues. It carries a high mortality and morbidity rate. In literature, the few case reports on necrotizing fasciitis of the breast, describe the need for a mastectomy in 90% of the cases. We report on a case of a 72-year old Caucasian women with an atypical presentation of necrotizing fasciitis of the breast in combination with an acute abdomen, successfully treated with breast-conserving debridement and secondary wound closure.

  14. Lyme Disease: A Case Report with Typical and Atypical Lesions

    PubMed Central

    Sharma, Anuj; Guleria, Sandesh; Sharma, Reena; Sharma, Anita

    2017-01-01

    Lyme disease is a multisystem infectious disease caused by the spirochete “Borrelia burgdorferi,” which is transmitted by “Ixodes” tick, with skin being the most common and earliest organ to be affected. Diagnosis of erythema chronicum migrans (ECM), which is the characteristic lesion of early disease, may help in early treatment and prevention of complications. Here, we are reporting a case of Lyme disease in a 10-year-old young boy from a non-endemic zone of Himachal Pradesh, who presented with typical as well as atypical ECM lesions. The clinical diagnosis was confirmed serologically, and the child was treated successfully with doxycycline.

  15. Multiphasic acute disseminated encephalomyelitis associated with atypical rubella virus infection.

    PubMed

    Shinoda, Koji; Asahara, Hideaki; Uehara, Taira; Miyoshi, Katsue; Suzuki, Satoshi O; Iwaki, Toru; Kira, Jun-ichi

    2015-02-01

    We report the first case of an occurrence of multiphasic acute disseminated encephalomyelitis (ADEM) associated with atypical rubella virus infection with no rash and long-term increased titers of serum anti-rubella IgM in a 17-year-old male who had no history of rubella vaccination. He suffered from at least six clinical exacerbations with disseminated hyperintense lesions on FLAIR MR images during the course of 18 months. Repeated methylprednisolone pulse therapy and intravenous immunoglobulin therapy resolved the exacerbations. In patients with multiphasic ADEM of unknown etiology, clinicians should also consider the possibility of preceding infection with rubella virus.

  16. Unusual imaging presentation of infantile atypical Kawasaki disease.

    PubMed

    Kumar, Nishith; Mittal, Mahesh Kumar; Sinha, Mukul; Gupta, Arpita; Thukral, Brij Bhushan

    2016-01-01

    Kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age. There are no specific diagnostic tests, and thus, the diagnosis of the disease is primarily made on the basis of clinical criteria. Unusual presentations of Kawasaki disease have been variably reported from different parts of the world. However, presentation of the disease in the form of peripheral thromboembolism and florid non-coronary aneurysms has rarely been described This report describes the imaging findings in infantile atypical Kawasaki disease with aneurysms of multiple medium-sized arteries, including coronary arteries, emphasizing the detection of clinically silent aneurysms in the disease.

  17. 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.

  18. Bacillary Angiomatosis in Immunocompetent Patient with Atypical Manifestations

    PubMed Central

    Iraji, Fariba; Pourazizi, Mohsen; Abtahi-Naeini, Bahareh; Meidani, Mohsen; Rajabi, Parvin

    2015-01-01

    Bacillary angiomatosis is an infectious disease caused by two Gram-negative bacilli; this disease usually affects immunosuppressed hosts with a history of cat scratch. We report a rare case of bacillary angiomatosis in an immunocompetent 26-year-old woman with no history of exposure to cats, and with atypical clinical features (very pruritic vascular papules and nodules with ulceration and hemorrhage on the right arm and fingers). She was successfully treated with clarithromycin for 3 months. Bacillary angiomatosis must be kept in mind in the differential diagnosis of any papules and nodules in cases of unknown etiology and also in immunocompetent patients and HIV-negative individual. PMID:26538736

  19. 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.

  20. Leachable 226Ra in Philippine phosphogypsum and its implication in groundwater contamination in Isabel, Leyte, Philippines.

    PubMed

    Cañete, Socrates Jose P; Palad, Lorna Jean H; Enriquez, Eliza B; Garcia, Teofilo Y; Yulo-Nazarea, Teresa

    2008-07-01

    Phosphogypsum (PG), the major waste material in phosphate fertilizer processing, has been known to contain enhanced levels of naturally-occurring radionuclides especially (226)Ra. The lack of radioactivity data regarding Philippine phosphogypsum and its environmental behavior in the Philippine setting has brought concern on possible contamination of groundwater beneath the phosphogypsum ponds in Isabel, Leyte, Philippines. The radioactivity of Philippine phosphogypsum was determined and the leaching of (226)Ra from phosphogypsum and through local soil was quantified. Level of (226)Ra in groundwater samples in Isabel, Leyte, Philippines was also quantified to address the primary concern. It was found that the (226)Ra activity in Philippine phosphogypsum is distributed in a wide range from 91.5 to 935 Bq/kg. As much as 5% of (226)Ra can be leached from Philippine PG with deionized water. In vitro soil leach experiments suggest that the soil in the phosphate fertilizer plant area would be able to deter the intrusion of (226)Ra into the water table. Compared to reported values of natural groundwater levels of (226)Ra, the concentration of this radionuclide in Isabel, Leyte groundwater suggest that there is no (226)Ra intrusion brought about by the presence of phosphogypsum ponds in the area.

  1. The CpxRA two-component system is essential for Citrobacter rodentium virulence.

    PubMed

    Thomassin, Jenny-Lee; Giannakopoulou, Natalia; Zhu, Lei; Gross, Jeremy; Salmon, Kristiana; Leclerc, Jean-Mathieu; Daigle, France; Le Moual, Hervé; Gruenheid, Samantha

    2015-05-01

    Citrobacter rodentium is a murine intestinal pathogen used as a model for the foodborne human pathogens enterohemorrhagic Escherichia coli and enteropathogenic E. coli. During infection, these pathogens use two-component signal transduction systems to detect and adapt to changing environmental conditions. In E. coli, the CpxRA two-component signal transduction system responds to envelope stress by modulating the expression of a myriad of genes. Quantitative real-time PCR showed that cpxRA was expressed in the colon of C57BL/6J mice infected with C. rodentium. To determine whether CpxRA plays a role during C. rodentium infection, a cpxRA deletion strain was generated and found to have a colonization defect during infection. This defect was independent of an altered growth rate or a defective type III secretion system, and single-copy chromosomal complementation of cpxRA restored virulence. The C. rodentium strains were then tested in C3H/HeJ mice, a lethal intestinal infection model. Mice infected with the ΔcpxRA strain survived infection, whereas mice infected with the wild-type or complemented strains succumbed to infection. Furthermore, we found that the cpxRA expression level was higher during early infection than at a later time point. Taken together, these data demonstrate that the CpxRA two-component signal transduction system is essential for the in vivo virulence of C. rodentium. In addition, these data suggest that fine-tuned cpxRA expression is important for infection. This is the first study that identifies a C. rodentium two-component transduction system required for pathogenesis. This study further indicates that CpxRA is an interesting target for therapeutics against enteric pathogens.

  2. Use of Ra isotopes to deduce rapid transfer of sediment-derived inputs off Kerguelen

    NASA Astrophysics Data System (ADS)

    Sanial, V.; van Beek, P.; Lansard, B.; Souhaut, M.; Kestenare, E.; d'Ovidio, F.; Zhou, M.; Blain, S.

    2015-03-01

    The Southern Ocean is known to be the largest high-nutrient, low-chlorophyll (HNLC) region of the global ocean due to iron limitation. However, a large phytoplankton bloom develops annually downstream of the Kerguelen Islands, a bloom which is sustained partly by iron released from the sediments deposited onto the shelves. In the framework of the KEOPS-2 project, we used radium isotopes (224Ra, T1/2 = 3.66 d; 223Ra, T1/2 = 11.4 d; 228Ra, T1/2 = 5.75 yr) to provide information on the origin of iron fertilization and on the timescales of the transfer of sediment-derived inputs (including iron and other micronutrients) towards offshore waters. Significant 224Ra and 223Ra activities were found in the near vicinity of the Kerguelen Islands, in agreement with the short half-lives of these isotopes. Significant 224Ra and 223Ra activities were also detected up to 200 km downstream of the islands and more unexpectedly in offshore waters south of the polar front. These observations thus clearly indicate (i) that the sediment-derived inputs are rapidly transferred towards offshore waters (on timescales on the order of several days up to several weeks) and (ii) that the polar front is not a physical barrier for the chemical elements released from the sediments of the Kerguelen Plateau. The Ra data set suggests that iron and other micronutrients released by the shelves of the Kerguelen Islands may contribute to fueling the phytoplankton bloom downstream of the islands, despite the presence of the polar front. However, the heterogeneous distribution of the 224Ra and 223Ra activities in surface waters suggests that this supply across the front is not a continuous process but rather a process that is highly variable in space and time.

  3. Use of Ra isotopes to deduce rapid transfer of sediment-derived inputs off Kerguelen

    NASA Astrophysics Data System (ADS)

    Sanial, V.; van Beek, P.; Lansard, B.; Souhaut, M.; Kestenare, E.; d'Ovidio, F.; Zhou, M.; Blain, S.

    2014-09-01

    The Southern Ocean is known as the largest High-Nutrient, Low-Chlorophyll (HNLC) region of the global ocean due to iron limitation. However, a large phytoplankton bloom develops annually downstream of the Kerguelen Islands, which is sustained partly by iron released from the sediments deposited onto the margins. In the framework of the KEOPS-2 project, we used radium isotopes (224Ra, T1/2 = 3.66 d; 223Ra, T1/2 = 11.4 d; 228Ra, T1/2 = 5.75 yr) to provide information on the origin of iron fertilization and on the timescales of the transfer of sediment-derived inputs (including iron and other micronutrients) towards offshore waters. Significant 223Ra and 223Ra activities were found in the near vicinity of the Kerguelen Islands, in agreement with the short half-lives of these isotopes. Significant 224Ra and 223Ra activities were also detected up to 200 km downstream of the islands and more unexpectedly in offshore waters south of the Polar Front. These observations thus clearly indicate (i) that the sediment-derived inputs are rapidly transferred towards offshore waters (on timescales in the order of several days up to several weeks) and (ii) that the Polar Front is not a physical barrier for the chemical elements released from the sediments of Kerguelen Plateau. The Ra dataset suggests that iron and other micronutrients released by the shallow sediments of the Kerguelen margins may contribute to fuel the phytoplankton bloom downstream of the islands, despite the presence of the Polar Front. However, the heterogeneous distribution of the 224Ra and 223Ra activities in surface waters suggests that this supply across the front is not a continuous process, but rather a process that is highly variable in space and time.

  4. The CpxRA Two-Component System Is Essential for Citrobacter rodentium Virulence

    PubMed Central

    Thomassin, Jenny-Lee; Giannakopoulou, Natalia; Zhu, Lei; Gross, Jeremy; Salmon, Kristiana; Leclerc, Jean-Mathieu; Daigle, France; Le Moual, Hervé

    2015-01-01

    Citrobacter rodentium is a murine intestinal pathogen used as a model for the foodborne human pathogens enterohemorrhagic Escherichia coli and enteropathogenic E. coli. During infection, these pathogens use two-component signal transduction systems to detect and adapt to changing environmental conditions. In E. coli, the CpxRA two-component signal transduction system responds to envelope stress by modulating the expression of a myriad of genes. Quantitative real-time PCR showed that cpxRA was expressed in the colon of C57BL/6J mice infected with C. rodentium. To determine whether CpxRA plays a role during C. rodentium infection, a cpxRA deletion strain was generated and found to have a colonization defect during infection. This defect was independent of an altered growth rate or a defective type III secretion system, and single-copy chromosomal complementation of cpxRA restored virulence. The C. rodentium strains were then tested in C3H/HeJ mice, a lethal intestinal infection model. Mice infected with the ΔcpxRA strain survived infection, whereas mice infected with the wild-type or complemented strains succumbed to infection. Furthermore, we found that the cpxRA expression level was higher during early infection than at a later time point. Taken together, these data demonstrate that the CpxRA two-component signal transduction system is essential for the in vivo virulence of C. rodentium. In addition, these data suggest that fine-tuned cpxRA expression is important for infection. This is the first study that identifies a C. rodentium two-component transduction system required for pathogenesis. This study further indicates that CpxRA is an interesting target for therapeutics against enteric pathogens. PMID:25712925

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

    SciTech Connect

    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) 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.

  6. 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

  7. The atypical cadherin Flamingo is required for sensory axon advance beyond intermediate target cells.

    PubMed

    Steinel, Martin C; Whitington, Paul M

    2009-03-15

    The Drosophila atypical cadherin Flamingo plays key roles in a number of developmental processes. We have used the sensory nervous system of the Drosophila embryo to shed light on the mechanism by which Flamingo regulates axon growth. flamingo loss of function mutants display a highly penetrant sensory axon stall phenotype. The location of these axon stalls is stereotypic and corresponds to the position of intermediate target cells, with which sensory axons associate during normal development. This suggests that Flamingo mediates an interaction between the sensory neuron growth cones and these intermediate targets, which is required for continued axon advance. Mutant rescue experiments show that Flamingo expression is required only in sensory neurons for normal axon growth. The flamingo mutant phenotype can be partially rescued by expressing a Flamingo construct lacking most of the extracellular domain, suggesting that regulation of sensory axon advance by Flamingo does not absolutely depend upon a homophilic Flamingo-Flamingo interaction or its ability to mediate cell-cell adhesion. Loss of function mutants for a number of key genes that act together with Flamingo in the planar cell polarity pathway do not display the highly penetrant stalling phenotype seen in flamingo mutants.

  8. Structure of human Sp140 PHD finger: an atypical fold interacting with Pin1.

    PubMed

    Zucchelli, Chiara; Tamburri, Simone; Quilici, Giacomo; Palagano, Eleonora; Berardi, Andrea; Saare, Mario; Peterson, Pärt; Bachi, Angela; Musco, Giovanna

    2014-01-01

    Sp140 is a nuclear leukocyte-specific protein involved in primary biliary cirrhosis and a risk factor in chronic lymphocytic leukemia. The presence of several chromatin related modules such as plant homeodomain (PHD), bromodomain and SAND domain suggests a role in chromatin-mediated regulation of gene expression; however, its real function is still elusive. Herein we present the solution structure of Sp140-PHD finger and investigate its role as epigenetic reader in vitro. Sp140-PHD presents an atypical PHD finger fold which does not bind to histone H3 tails but is recognized by peptidylprolyl isomerase Pin1. Pin1 specifically binds to a phosphopeptide corresponding to the L3 loop of Sp140-PHD and catalyzes cis-trans isomerization of a pThr-Pro bond. Moreover co-immunoprecipitation experiments demonstrate FLAG-Sp140 interaction with endogenous Pin1 in vivo. Overall these data include Sp140 in the list of the increasing number of Pin1 binders and expand the regulatory potential of PHD fingers as versatile structural platforms for diversified interactions.

  9. Interactions between Melanin Enzymes and Their Atypical Recruitment to the Secretory Pathway by Palmitoylation

    PubMed Central

    Upadhyay, Srijana; Xu, Xinping

    2016-01-01

    ABSTRACT Melanins are biopolymers that confer coloration and protection to the host organism against biotic or abiotic insults. The level of protection offered by melanin depends on its biosynthesis and its subcellular localization. Previously, we discovered that Aspergillus fumigatus compartmentalizes melanization in endosomes by recruiting all melanin enzymes to the secretory pathway. Surprisingly, although two laccases involved in the late steps of melanization are conventional secretory proteins, the four enzymes involved in the early steps of melanization lack a signal peptide or a transmembrane domain and are thus considered “atypical” secretory proteins. In this work, we found interactions among melanin enzymes and all melanin enzymes formed protein complexes. Surprisingly, the formation of protein complexes by melanin enzymes was not critical for their trafficking to the endosomal system. By palmitoylation profiling and biochemical analyses, we discovered that all four early melanin enzymes were strongly palmitoylated during conidiation. However, only the polyketide synthase (PKS) Alb1 was strongly palmitoylated during both vegetative hyphal growth and conidiation when constitutively expressed alone. This posttranslational lipid modification correlates the endosomal localization of all early melanin enzymes. Intriguingly, bioinformatic analyses predict that palmitoylation is a common mechanism for potential membrane association of polyketide synthases (PKSs) and nonribosomal peptide synthetases (NRPSs) in A. fumigatus. Our findings indicate that protein-protein interactions facilitate melanization by metabolic channeling, while posttranslational lipid modifications help recruit the atypical enzymes to the secretory pathway, which is critical for compartmentalization of secondary metabolism. PMID:27879337

  10. Differential effects of typical and atypical antipsychotic drugs on striosome and matrix compartments of the striatum.

    PubMed

    Bubser, Michael; Deutch, Ariel Y

    2002-02-01

    Administration of typical antipsychotic drugs (APDs) is often accompanied by extrapyramidal side-effects (EPS). Treatment with atypical APDs has a lower incidence of motor side-effects and atypical APDs are superior to typical APDs in treating the negative symptoms of schizophrenia. Although typical APDs strongly induce the immediate-early gene c-fos in the striatum while atypical APDs do so only weakly, it is possible that the effects of atypical APDs are more pronounced within certain regions of the striatum. The striatum contains two histochemically defined compartments, the striosome (patch) and the matrix. These compartments have been well characterized anatomically but their functional attributes are unclear. We therefore examined the effects of typical and atypical APDs on Fos expression in the striosome and matrix of the rat. Typical and atypical APDs were distinguished by the pattern of striatal compartmental activation they induced: the striosome : matrix ratio of Fos-li neurons was greater in rats treated with atypical APDs. Pretreating animals with selective antagonists of receptors that atypical APDs target with high affinity did not increase the striosome : matrix Fos ratio of typical APD-treated rats and thus did not mimic the ratio seen in response to atypical APDs. However, pretreatment with the atypical APD clozapine did recapitulate the characteristic compartmental Fos pattern seen in response to typical APDs. These data suggest that some characteristics of atypical APDs, such as the lower EPS liability and greater reduction of negative symptoms, may be linked to the coordinate regulation of the striatal striosome and matrix.

  11. Molecular pathology of atypical polypoid adenomyoma of the uterus.

    PubMed

    Ota, Shunichiro; Catasus, Lluis; Matias-Guiu, Xavier; Bussaglia, Elena; Lagarda, Helena; Pons, Cristina; Muñoz, Josefina; Kamura, Toshiharu; Prat, Jaime

    2003-08-01

    Atypical polypoid adenomyoma (APA) is an uncommon and benign tumor of the uterus. In some patients, however, APA has been found to coexist with or to precede the development of an endometrioid adenocarcinoma similarly to complex endometrial hyperplasia. The molecular changes underlying the progression from APA to adenocarcinoma are unknown. DNA from paraffin-embedded tissue of 6 APAs was evaluated for microsatellite instability (MI), MLH-1 promoter hypermethylation, and CTNNB-1 mutations. Tissue sections were also subjected to MLH-1, MSH-2, and beta-catenin immunostaining. MI was not detected in any case. Two tumors exhibited MLH-1 promoter hypermethylation and showed focal negative MHL-1 immunostaining; 1 of these showed marked architectural complexity and cellular pleomorphism. Five cases presented beta-catenin nuclear immunoreactivity, but none of them had CTNNB-1 mutations. The results of this study suggest that APA and complex endometrial hyperplasia may share some molecular alterations. Some APAs exhibit MLH-1 promoter hypermethylation with focal lack of MLH-1 immunostaining, a molecular abnormality involved in the transition from complex atypical hyperplasia to endometrioid adenocarcinoma.

  12. Neuropsychological changes in melancholic and atypical depression: A systematic review.

    PubMed

    Bosaipo, Nayanne Beckmann; Foss, Maria Paula; Young, Allan H; Juruena, Mario Francisco

    2017-02-01

    There is not a consensus as to whether neuropsychological profiling can distinguish depressive subtypes. We aimed to systematically review and critically analyse the literature on cognitive function in patients with melancholic and atypical depression. We searched in databases PubMed, SCOPUS, Web of Knowledge and PsycInfo for papers comparing the neuropsychological performance of melancholic patients (MEL) to non-melancholic depressive patients (NMEL), including atypical depressives, and healthy controls (HC). All studies were scrutinised to determine the main methodological characteristics and particularly possible sources of bias influencing the results reported, using the STROBE statement checklist. We also provide effect size of the results reported for contrasts between MEL; patients and NMEL patients. Seventeen studies were included; most of them demonstrated higher neuropsychological impairments of MEL patients compared to both NMEL patients and HC on tasks requiring memory, executive function, attention and reaction time. Detailed analysis of the methodologies used in the studies revealed significant variability especially regarding the participants' sociodemographic characteristics, clinical characteristics of patients and differences in neuropsychological assessment. These findings suggest that MEL may have a distinct and impaired cognitive performance compared to NMEL depressive patients on tasks involving verbal and visual memory, executive function, sustained attention and span, as well as psychomotor speed, this last especially when cognitive load is increased. Additional studies with adequate control of potentially confounding variables will help to clarify further differences in the neuropsychological functioning of depressive subtypes.

  13. 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

  14. 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.

  15. Clinical and molecular review of atypical congenital adrenal hyperplasia

    PubMed Central

    2015-01-01

    Congenital adrenal hyperplasia (CAH) is one of the most common inherited metabolic disorders. It comprises a group of autosomal recessive disorders caused by the mutations in the genes encoding for steroidogenic enzymes that involved cortisol synthesis. More than 90% of cases are caused by a defect in the enzyme 21-hydroxylase. Four other enzyme deficiencies (cholesterol side-chain cleavage, 17α-hydroxylase [P450c17], 11β-hydroxylase [P450c11β], 3β-hydroxysteroid dehydrogenase) in the steroid biosynthesis pathway, along with one cholesterol transport protein defect (steroidogenic acute regulatory protein), and one electrontransfer protein (P450 oxidoreductase) account for the remaining cases. The clinical symptoms of the different forms of CAH result from the particular hormones that are deficient and those that are produced in excess. A characteristic feature of CAH is genital ambiguity or disordered sex development, and most variants are associated with glucocorticoid deficiency. However, in the rare forms of CAH other than 21-hydroxylase deficiency so-called "atypical CAH", the clinical and hormonal phenotypes can be more complicated, and are not well recognized. This review will focus on the atypical forms of CAH, including the genetic analyses, and phenotypic correlates. PMID:25883920

  16. Structural basis of ligand interaction with atypical chemokine receptor 3

    NASA Astrophysics Data System (ADS)

    Gustavsson, Martin; Wang, Liwen; van Gils, Noortje; Stephens, Bryan S.; Zhang, Penglie; Schall, Thomas J.; Yang, Sichun; Abagyan, Ruben; Chance, Mark R.; Kufareva, Irina; Handel, Tracy M.

    2017-01-01

    Chemokines drive cell migration through their interactions with seven-transmembrane (7TM) chemokine receptors on cell surfaces. The atypical chemokine receptor 3 (ACKR3) binds chemokines CXCL11 and CXCL12 and signals exclusively through β-arrestin-mediated pathways, without activating canonical G-protein signalling. This receptor is upregulated in numerous cancers making it a potential drug target. Here we collected over 100 distinct structural probes from radiolytic footprinting, disulfide trapping, and mutagenesis to map the structures of ACKR3:CXCL12 and ACKR3:small-molecule complexes, including dynamic regions that proved unresolvable by X-ray crystallography in homologous receptors. The data are integrated with molecular modelling to produce complete and cohesive experimentally driven models that confirm and expand on the existing knowledge of the architecture of receptor:chemokine and receptor:small-molecule complexes. Additionally, we detected and characterized ligand-induced conformational changes in the transmembrane and intracellular regions of ACKR3 that elucidate fundamental structural elements of agonism in this atypical receptor.

  17. 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.

  18. Atypical neural specialization for social percepts in autism spectrum disorder.

    PubMed

    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 nonsocial 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 nonsocial information. Findings concord with the notion of specific dysfunction in social brain systems rather than nonspecific information-processing difficulties in autism.

  19. Socialization and nonverbal communication in atypically developing infants and toddlers.

    PubMed

    Konst, Matthew J; Matson, Johnny L; Goldin, Rachel L; Williams, Lindsey W

    2014-12-01

    Emphasis on early identification of atypical development has increased as evidence supporting the efficacy of intervention has grown. These increases have also directly affected the availability of funding and providers of early intervention services. A majority of research has focused on interventions specific to an individual's primary diagnoses. For example, interventions for those with cerebral palsy (CP) have traditionally focused on physiological symptoms, while intervention for individuals with Autism Spectrum Disorder (ASD) focus on socialization, communication, and restricted interests and repetitive behaviors. However deficits in areas other than those related to their primary diagnoses (e.g., communication, adaptive behaviors, and social skills) are prevalent in atypically developing populations and are significant predictors of quality of life. Therefore, the purpose of the current study was to examine impairments in socialization and nonverbal communication in individuals with Down's syndrome (DS), CP, and those with CP and comorbid ASD. Individuals with comorbid CP and ASD exhibited significantly greater impairments than any diagnostic group alone. However, individuals with CP also exhibited significantly greater impairments than those with DS. The implications of these results are discussed.

  20. 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.

  1. A macroprolactinoma becoming resistant to cabergoline and developing atypical pathology.

    PubMed

    Sbardella, Emilia; Farah, George; Fathelrahman, Ahmed; Cudlip, Simon; Ansorge, Olaf; Karavitaki, Niki; Grossman, Ashley B

    2016-01-01

    Pituitary adenomas are a common intracranial neoplasm, usually demonstrating a benign phenotype. They can be classified according to pathological, radiological or clinical behaviour as typical, atypical or carcinomas, invasive or noninvasive, and aggressive or nonaggressive. Prolactinomas account for 40-60% of all pituitary adenomas, with dopamine agonists representing the first-line treatment and surgery/radiotherapy reserved for drug intolerance/resistance or in neuro-ophthalmological emergencies. We present the case of a 62-year-old man with an apparently indolent prolactin-secreting macroadenoma managed with partial resection and initially showing a biochemical response to cabergoline. Five years later, the tumour became resistant to cabergoline, despite a substantial increase in dosage, showing rapid growth and causing worsening of vision. The patient then underwent two further transsphenoidal operations and continued on high-dose cabergoline; despite these interventions, the tumour continued enlarging and prolactin increased to 107 269 U/L. Histology of the third surgical specimen demonstrated features of aggressive behaviour (atypical adenoma with a high cell proliferation index) not present in the tumour removed at the first operation. Subsequently, he was referred for radiotherapy aiming to control tumour growth.

  2. 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.

  3. Structural basis of ligand interaction with atypical chemokine receptor 3

    PubMed Central

    Gustavsson, Martin; Wang, Liwen; van Gils, Noortje; Stephens, Bryan S.; Zhang, Penglie; Schall, Thomas J.; Yang, Sichun; Abagyan, Ruben; Chance, Mark R.; Kufareva, Irina; Handel, Tracy M.

    2017-01-01

    Chemokines drive cell migration through their interactions with seven-transmembrane (7TM) chemokine receptors on cell surfaces. The atypical chemokine receptor 3 (ACKR3) binds chemokines CXCL11 and CXCL12 and signals exclusively through β-arrestin-mediated pathways, without activating canonical G-protein signalling. This receptor is upregulated in numerous cancers making it a potential drug target. Here we collected over 100 distinct structural probes from radiolytic footprinting, disulfide trapping, and mutagenesis to map the structures of ACKR3:CXCL12 and ACKR3:small-molecule complexes, including dynamic regions that proved unresolvable by X-ray crystallography in homologous receptors. The data are integrated with molecular modelling to produce complete and cohesive experimentally driven models that confirm and expand on the existing knowledge of the architecture of receptor:chemokine and receptor:small-molecule complexes. Additionally, we detected and characterized ligand-induced conformational changes in the transmembrane and intracellular regions of ACKR3 that elucidate fundamental structural elements of agonism in this atypical receptor. PMID:28098154

  4. [Neuropathic pain due to herpes zoster infection with atypical localization].

    PubMed

    Sağır, Özlem; Özaslan, Sabri; Meriç, Yücel; Arslan, İsmail; Köroğlu, Ahmet

    2013-01-01

    Acute herpes zoster infection appears in the situation of depression of immune system and reactivation of varicella zoster virus which causes small pox. Pain and maculopapular lesion accompany clinical symptoms. Various pharmacological and invasive methods can be used for treatment. Efficient therapy is important for prevention of postherpetic neuralgia and cure of acute pain and dermatological lesions. A 55 years old, 160 cm height and 65 kg weight female patient with complaints of severe pain, sensation of burning, tingling at the right hand and forearm was admitted to our pain department. The patient who was diagnosed as cervical hernia at an other medical center had a normal physical servical spine examination. Patient history and physical examination findings with acute herpes zoster infection was considered. Right stellate ganglion blockade for diagnosis and treatment was performed because of regressed and atypically located lesions and a visual analog scale score of 10. VAS score decreased 50% at 9th min after block, VAS score at 2nd hour was 2. Antiviral, gabapentin, and tricyclic antidepressant treatment was started after stellat ganglion blockade and patient was discharged. After 3 months complaints dissapeared and drug doses were discreased and stopped. In conclusion we think that stellate ganglion blockade can be useful in diagnosis, acute pain control, improving patient comfort and compatibility to drug therapy in atypically located herpes zoster.

  5. 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

  6. 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

  7. Isotonic similarities in isotope shifts from Hg to Ra.

    NASA Astrophysics Data System (ADS)

    Stroke, H. H.

    2003-04-01

    Isotope shifts (IS) in atomic spectra of heavy elements reflect largely the variation in of the nuclear charge distribution. Our early systematic measurements of IS for an extended range of stable and radioactive isotopes and nuclear isomers in Tl and Hg^1 showed that by displaying the relative IS, normalized to a chosen pair of isotopes, there was a striking similarity for the IS of isotones. This essentially divides out the electronic factor in the IS and allows the comparison of Δ for neighboring Z as N is varied. Following our further studies on Pb and Bi^2 and those on Fr at ISOLDE by the Orsay spectroscopy group^3, we found that the isotonic similarity extended to these elements. Since then, many additional measurements were made, principally at ISOLDE^4, extending to Ra the elements studied. The isotonic shift similarities persist from Z=80 to 88. We noted that the relative isotope and isomer shifts can be used to investigate the polarization of the nucleus by the added neutrons, a model used in a calculation by Barrett.^5 . The new data may serve further in this direction. ^1W,J.Tomlinson, H.H. Stroke, Nucl.Phys. 60, 614 (1964). ^2M. Barboza-Flores et al., Z.Phys. A 321, 85 (1985), ^3S. Liberman et al., Phys .Rev. A 22, 2732 (1980). ^4E,g. M.R. Pearson et al., J.Phys. G 26, 1829 (2000). ^5R.C. Barrett, Nucl. Phys. 88, 128 (1966).

  8. Gene-rich chromosomal regions are preferentially localized in the lamin B deficient nuclear blebs of atypical progeria cells.

    PubMed

    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.

  9. 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

  10. Metrological Determination of Natural Radioactive Isotopes {sup 226}Ra, {sup 228}Ra and {sup 210}Pb by Means of Ge Detector

    SciTech Connect

    Almeida, Maria Candida M. de; Delgado, Jose U.; Poledna, Roberto; Oliveira, Estela Maria de; Silva, Ronaldo L. da

    2008-08-07

    A metrological method to determine the activity per mass unity (activity concentration) of {sup 226}Ra and {sup 210}Pb ({sup 238}U decay series) and {sup 228}Ra ({sup 232}Th series) by gamma-ray spectrometers based on hyper-pure coaxial germanium detector was developed. In the soil the {sup 22}Ra (half-life = 1600 years) exhibits the same level of radioactivity as {sup 238}U (half-life 4.5x10{sup 9} years) because of a natural phenomenon called secular equilibrium. {sup 226}Ra decays into {sup 222}Rn (half-life = 3.8 days), a radioactive inert gas. After several days, the {sup 222}Rn naturally decays to {sup 218}Po (half-life = 3 minutes), where finally {sup 210}Pb (half-life = 22 years) is produced. The metrological capability of high-resolution gamma-ray spectrometry for naturally occurring radionuclides at environmental levels is showed, with emphasis on the use of 2 mL standard sources volume in a glass ampoule. Source preparation and calibration procedures are described. Radionuclide standards in an activity range of 10 to 250 Bq/g were produced which can be applied in a variety of environmental sample analysis (water, plant material, sediment, etc.). Uncertainties for {sup 226}Ra and {sup 210}Pb around 3% (k = 1) were obtained.

  11. Determination of (210)Pb and (226)Ra/(228)Ra in continental water using HIDEX 300SL LS-spectrometer with TDCR efficiency tracing and optimized α/β-discrimination.

    PubMed

    Eikenberg, J; Beer, H; Jäggi, M

    2014-11-01

    An analytical method for determination of (210)Pb, (226)Ra and (228)Ra is presented based on adsorption on 3M Empore RadDiscs, and measurement applying liquid scintillation spectrometry (LSC) after elution. The LSC measurement was performed with optimized α/β-discrimination and isotope standardization using the triple to double coincidence ratio (TDCR). The consistency of measurement results between radioactive parent-daughter pairs (210)Pb/(210)Bi, (226)Ra/(222)Rn and (228)Ra/(228)Ac was checked in long-term counting experiments and the influence ofinterference of in-growing daughters from (226)Ra into the β-spectrum of (228)Ra+(228)Ac was studied as well. Recommendations for optimized LSC (228)Ra measurement besides presence of (226)Ra are given.

  12. cDNA cloning and expression of the human A-type platelet-derived growth factor (PDGF) receptor establishes structural similarity to the B-type PDGF receptor

    SciTech Connect

    Claesson-Welsh, L.; Eriksson, A.; Westermark, B.; Heldin, C.H. )

    1989-07-01

    The primary structure of the human A-type receptor for platelet-derived growth factor (PDGF) has been determined. A 6.5-kilobase (kb) transcript was identified through low-stringency hybridization with a probe derived from the B-type PDGF receptor cDNA. The sequence of a cDNA clone corresponding to the 6.5-kb transcript contains an open reading frame that predicts a 1,089-amino acid growth factor receptor-like molecule, which displays 44% overall amino acid similarity with the PDGF B-type receptor. The two receptors have a similar domain organization, with five immunoglobulin-like domains extracellularly and an intracellular split protein tyrosine kinase domain. Transfection of the new cDNA into COS cells led to the expression of a protein specifically recognized by an antiserum previously shown to react with the PDGF A-type receptor. The expressed protein was shown to display high-affinity binding of all three {sup 125}I-labeled dimeric forms of PdGF A and B chains in a manner that is characteristic for the PDGF A-type receptor.

  13. Preparation of A-type proanthocyanidin dimers from peanut skins and persimmon pulp and comparison of the antioxidant activity of A-type and B-type dimers.

    PubMed

    Dong, Xiao-qian; Zou, Bo; Zhang, Ying; Ge, Zhen-zhen; Du, Jing; Li, Chun-mei

    2013-12-01

    We have established a simple method for preparing large quantities of A-type dimers from peanut skin and persimmon for further structure-activity relationship study. Peanut skins were defatted with hexane and oligomeric proanthocyanidins were extracted from it with 20% of methanol, and the extract was fractionated with ethyl acetate. Persimmon tannin was extracted from persimmon with methanol acidified with 1% hydrochloric acid, after removing the sugar and small phenols, the high molecular weight persimmon tannin was partially cleaved with 6.25% hydrochloric acid in methanol. The ethyl acetate fraction from peanut skins and persimmon tannin cleaved products was chromatographed on AB-8 macroporous resin followed by Toyopearl HW-50F resin to yield about 378.3mg of A-type (epi)catechin (EC) dimer from 1 kg dry peanut skins and 34.3mg of A-type (epi)catechin-3-O-gallate (ECG) dimer and 37.7 mg of A-type (epi)gallocatechin-3-O-gallate (EGCG) dimer from 1 kg fresh persimmon fruit. The antioxidant properties of the A-type and B-type dimers were compared in five different assays, namely, 1,1-diphenyl-2-picrylhydrazyl (DPPH) radical, 2,2-azino-bis (3-ethylbenzthiazoline-6-sulfonic acid) (ABTS) radical, hydroxyl radical, lipid peroxidation in mice liver homogenate and erythrocyte hemolysis in rat blood. Our results showed that both A-type and B-type dimers showed high antioxidant potency in a dose-dependent manner. In general, B-type dimers showed higher radical scavenging potency than A-type ones with the same subunits in aqueous systems. But in tissue or lipid systems, A-type dimers showed similar or even higher antioxidant potency than B-type ones.

  14. Embracing the Unusual: Feeling Tired and Happy Is Associated with Greater Acceptance of Atypical Ideas

    ERIC Educational Resources Information Center

    Middlewood, Brianna L.; Gallegos, Jonathan; Gasper, Karen

    2016-01-01

    Three studies examined the hypothesis that feeling tired along with feeling happy might be linked to the acceptance of atypical ideas. Consistent with this hypothesis, across 3 studies and using 2 different measures of accepting atypical ideas, feelings of happiness and tiredness interacted. When people were high in tiredness, as happiness…

  15. 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…

  16. Atypical Social Judgment and Sensitivity to Perceptual Cues in Autism Spectrum Disorders

    ERIC Educational Resources Information Center

    Forgeot d'Arc, Baudouin; Ramus, Franck; Lefebvre, Aline; Brottier, Delphine; Zalla, Tiziana; Moukawane, Sanaa; Amsellem, Frédérique; Letellier, Laurence; Peyre, Hugo; Mouren, Marie-Christine; Leboyer, Marion; Delorme, Richard

    2016-01-01

    Evaluation of faces is an important dimension of social relationships. A degraded sensitivity to facial perceptual cues might contribute to atypical social interactions in autism spectrum disorder (ASD). The current study investigated whether face based social judgment is atypical in ASD and if so, whether it could be related to a degraded…

  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. 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…

  19. 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…

  20. 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.

  1. Olanzapine treatment in patients with typical and atypical neuroleptic-associated agranulocytosis.

    PubMed

    Finkel, B; Lerner, A; Oyffe, I; Rudinski, D; Sigal, M; Weizman, A

    1998-05-01

    Two cases are reported of patients who developed agranulocytosis after treatment with typical and atypical antipsychotics. The patients were successfully treated with olanzapine. We suggest the consideration of olanzapine as a safer treatment in patients who require immediate continuation of antipsychotic medication and have a prior history of classic and atypical neuroleptic-induced agranulocytosis.

  2. 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…

  3. Diagnostic utility of Fli-1 and D2-40 in distinguishing atypical fibroxanthoma from angiosarcoma.

    PubMed

    Cuda, Jonathan; Mirzamani, Neda; Kantipudi, Ramya; Robbins, Jason; Welsch, Micheal Jude; Sundram, Uma N

    2013-05-01

    Although in most cases one can easily distinguish between atypical fibroxanthomas and angiosarcomas, hemorrhagic atypical fibroxanthomas can pose a diagnostic problem. In rare cases, the large atypical cells of atypical fibroxanthoma can stain with CD31, leading to the erroneous diagnosis of angiosarcoma. We elected to further study this conundrum with 2 additional markers of lymphatic and vascular elements, namely D2-40 (podoplanin) and Fli-1, respectively. We studied 26 cases of atypical fibroxanthoma and 20 cases of angiosarcoma with Fli-1 and D2-40. We found that both Fli-1 and D2-40 stained a majority of cases of angiosarcoma (16/20 and 12/20, respectively), although only staining a minority of cases of atypical fibroxanthoma (8/26 for both). In addition, D2-40 staining of atypical fibroxanthoma was usually weak when positive, whereas Fli-1 staining of angiosarcomas was mostly strong and nuclear. Thus, both D2-40 and Fli-1 seem to be useful in distinguishing between atypical fibroxanthomas and angiosarcomas.

  4. Masculine girls and feminine boys: genetic and environmental contributions to atypical gender development in early childhood.

    PubMed

    Knafo, Ariel; Iervolino, Alessandra C; Plomin, Robert

    2005-02-01

    In this genetic study of atypical gender role development, parents of 5,799 twin pairs, ages 3 and 4, rated their twin children's masculinity and femininity. Boys were selected as gender atypical if they were highly feminine (top 5%, 10%, or 15%) relative to other boys, and girls were selected if they were highly masculine relative to other girls. Gender-atypical boys and girls were each divided into 2 groups: fully gender atypical (e.g., feminine boys also low on masculinity) and partially gender atypical (e.g., feminine boys who are not low on masculinity). DeFries-Fulker (DF; J. C. DeFries & D. W. Fulker, 1985, 1988) extremes analysis yielded moderate group heritability and substantial shared environment effects for atypical gender role behavior. However, for fully gender-atypical girls, group heritability accounted for most of the variance, and shared environment had no effect. The results are discussed in light of past studies and potential implications for atypical gender development.

  5. Atypical Speech and Language Development: A Consensus Study on Clinical Signs in the Netherlands

    ERIC Educational Resources Information Center

    Visser-Bochane, Margot I.; Gerrits, Ellen; van der Schans, Cees P.; Reijneveld, Sijmen A.; Luinge, Margreet R.

    2017-01-01

    Background: Atypical speech and language development is one of the most common developmental difficulties in young children. However, which clinical signs characterize atypical speech-language development at what age is not clear. Aim: To achieve a national and valid consensus on clinical signs and red flags (i.e. most urgent clinical signs) for…

  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. 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

  8. The role of histaminergic H1 and H3 receptors in food intake: a mechanism for atypical antipsychotic-induced weight gain?

    PubMed

    Deng, Chao; Weston-Green, Katrina; Huang, Xu-Feng

    2010-02-01

    Atypical antipsychotics such as olanzapine and clozapine are effective at treating the multiple domains of schizophrenia, with a low risk of extra-pyramidal side-effects. However a major downfall to their use is metabolic side-effects particularly weight gain/obesity, which occurs by unknown mechanisms. The present paper explores the potential candidature of histaminergic neurotransmission in the mechanisms of atypical antipsychotic-induced weight gain, with a focus on the histaminergic H1 and H3 receptors. Olanzapine and clozapine have a high affinity for the H1 receptor, and meta-analyses show a strong correlation between risk of weight gain and H1 receptor affinity. In addition, olanzapine treatment decreases H1 receptor binding and mRNA expression in the rat hypothalamus. Furthermore, a complex role is emerging for the histamine H3 receptor in the control of hunger. The H3 receptor is a pre-synaptic autoreceptor that inhibits the synthesis and release of histamine, and a heteroreceptor that inhibits other neurotransmitters such as serotonin (5-HT), noradrenaline (NA) and acetylcholine (ACh), which are also implicated in the regulation of food intake. Thus, the H3 receptor is in a prime position to regulate food intake, both through its control of histamine and its influence on other feeding pathways. We proposed that a mechanism for atypical antipsychotic-induced weight gain may be partly through the H3 receptor, as a drug-induced decrease in H1 receptor activity may decrease histamine tone through the H3 autoreceptors, compounding the weight gain problem. In addition, atypical antipsychotics may affect food intake by influencing 5-HT, NA and ACh release via interactions with the H3 heteroreceptor.

  9. Sanshool on The Fingertip Interferes with Vibration Detection in a Rapidly-Adapting (RA) Tactile Channel

    PubMed Central

    Kuroki, Scinob; Hagura, Nobuhiro; Nishida, Shin’ya; Haggard, Patrick; Watanabe, Junji

    2016-01-01

    An Asian spice, Szechuan pepper (sanshool), is well known for the tingling sensation it induces on the mouth and on the lips. Electrophysiological studies have revealed that its active ingredient can induce firing of mechanoreceptor fibres that typically respond to mechanical vibration. Moreover, a human behavioral study has reported that the perceived frequency of sanshool-induced tingling matches with the preferred frequency range of the tactile rapidly adapting (RA) channel, suggesting the contribution of sanshool-induced RA channel firing to its unique perceptual experience. However, since the RA channel may not be the only channel activated by sanshool, there could be a possibility that the sanshool tingling percept may be caused in whole or in part by other sensory channels. Here, by using a perceptual interference paradigm, we show that the sanshool-induced RA input indeed contributes to the human tactile processing. The absolute detection thresholds for vibrotactile input were measured with and without sanshool application on the fingertip. Sanshool significantly impaired detection of vibrations at 30 Hz (RA channel dominant frequency), but did not impair detection of higher frequency vibrations at 240 Hz (Pacinian-corpuscle (PC) channel dominant frequency) or lower frequency vibrations at 1 Hz (slowly adapting 1 (SA1) channel dominant frequency). These results show that the sanshool induces a peripheral RA channel activation that is relevant for tactile perception. This anomalous activation of RA channels may contribute to the unique tingling experience of sanshool. PMID:27935970

  10. Radium Ra 223 dichloride injection: U.S. Food and Drug Administration drug approval summary.

    PubMed

    Kluetz, Paul G; Pierce, William; Maher, V Ellen; Zhang, Hui; Tang, Shenghui; Song, Pengfei; Liu, Qi; Haber, Martin T; Leutzinger, Eldon E; Al-Hakim, Ali; Chen, Wei; Palmby, Todd; Alebachew, Elleni; Sridhara, Rajeshwari; Ibrahim, Amna; Justice, Robert; Pazdur, Richard

    2014-01-01

    On May 15, 2013, the U.S. Food and Drug Administration (FDA) approved radium Ra 223 dichloride (Ra-223; Xofigo injection; Bayer HealthCare Pharmaceuticals Inc.) for the treatment of patients with castration-resistant prostate cancer (CRPC), symptomatic bone metastases, and no known visceral metastatic disease. The FDA review was based on clinical trial BC1-06, which randomly allocated patients (2:1) to either Ra-223 plus best standard of care (BSoC) or placebo plus BSoC. The primary endpoint was overall survival (OS) with a key secondary endpoint of time to first symptomatic skeletal event (SSE). A statistically significant improvement in OS was demonstrated [HR, 0.70; 95% confidence interval, 0.55-0.88, P = 0.0019]. At the prespecified interim analysis, the median OS durations were 14.0 and 11.2 months in the Ra-223 and placebo arms, respectively. The improvement in OS was supported by a delay in time to first SSE favoring the Ra-223 arm. The most common (>10%) adverse reactions in patients receiving Ra-223 were nausea, diarrhea, vomiting, and peripheral edema. The most common (>10%) hematologic laboratory abnormalities were anemia, lymphocytopenia, leukopenia, thrombocytopenia, and neutropenia. Ra-223 is the first α-emitting radiotherapeutic and the first radiopharmaceutical to demonstrate an OS advantage in metastatic prostate cancer.

  11. Carbachol-Induced Reduction in the Activity of Adult Male Zebra Finch RA Projection Neurons

    PubMed Central

    Meng, Wei; Wang, Song-Hua; Li, Dong-Feng

    2016-01-01

    Cholinergic mechanism is involved in motor behavior. In songbirds, the robust nucleus of the arcopallium (RA) is a song premotor nucleus in the pallium and receives cholinergic inputs from the basal forebrain. The activity of projection neurons in RA determines song motor behavior. Although many evidences suggest that cholinergic system is implicated in song production, the cholinergic modulation of RA is not clear until now. In the present study, the electrophysiological effects of carbachol, a nonselective cholinergic receptor agonist, were investigated on the RA projection neurons of adult male zebra finches through whole-cell patch-clamp techniques in vitro. Our results show that carbachol produced a significant decrease in the spontaneous and evoked action potential (AP) firing frequency of RA projection neurons, accompanying a hyperpolarization of the membrane potential, an increase in the evoked AP latency, afterhyperpolarization (AHP) peak amplitude, and AHP time to peak, and a decrease in the membrane input resistance, membrane time constant, and membrane capacitance. These results indicate that carbachol reduces the activity of RA projection neurons by hyperpolarizing the resting membrane potential and increasing the AHP and the membrane conductance, suggesting that the cholinergic modulation of RA may play an important role in song production. PMID:26904300

  12. 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.

  13. Age-dependent dose assessment of 226Ra from bottled water intake.

    PubMed

    Bronzovic, Maja; Marovic, Gordana

    2005-05-01

    Water may present a source of prolonged exposure to naturally occurring radionuclides. One of the most frequently occurring radionuclides in natural mineral and spring waters is 226Ra and its decay products. The contribution of drinking water to the total exposure is very small, at about 5% of the average effective dose attributable annually to natural background radiation, but that exposure contributes to the risk of adverse health consequences. In this study the mean values of 226Ra concentration determined in natural mineral and spring bottled waters range from 6 to 412 mBq L(-1), which is in accord with Croatian legislation. 226Ra effective doses per year from spring water consumption range up to 86 microSv, while 226Ra effective doses per year from mineral water consumption show much higher values. The highest 226Ra effective doses per year from mineral waters consumption, which are up to seven times higher than the dose recommended by WHO (100 microSv), were found in infants and teens. Based on this study, drinking of certain brands of bottled mineral water is not recommended for these age groups because assessed 226Ra effective doses per year exceed the recommended limits. From other research it is known that testosterone appears in elevated concentration during these life periods and affects bone calcification. Therefore, testosterone could affect the retention of 226Ra into the bone. To make more precise conclusions further research is necessary. Adults and especially elderly people are much less susceptible to the presence of 226Ra. According to the results obtained in this study, 226Ra effective doses per year assessed for these age groups were considerably lower (i.e., 10 microSv).

  14. Guiding atypical facial growth back to normal. Part 1: Understanding facial growth.

    PubMed

    Galella, Steve; Chow, Daniel; Jones, Earl; Enlow, Donald; Masters, Ari

    2011-01-01

    Many practitioners find the complexity of facial growth overwhelming and thus merely observe and accept the clinical features of atypical growth and do not comprehend the long-term consequences. Facial growth and development is a strictly controlled biological process. Normal growth involves ongoing bone remodeling and positional displacement. Atypical growth begins when this biological balance is disturbed With the understanding of these processes, clinicians can adequately assess patients and determine the causes of these atypical facial growth patterns and design effective treatment plans. This is the first of a series of articles which addresses normal facial growth, atypical facial growth, patient assessment, causes of atypical facial growth, and guiding facial growth back to normal.

  15. Redox modulation of A-type K+ currents in pain-sensing dorsal root ganglion neurons.

    PubMed

    Hsieh, Chi-Pan

    2008-06-06

    Redox modulation of fast inactivation has been described in certain cloned A-type voltage-gated K(+) (Kv) channels in expressing systems, but the effects remain to be demonstrated in native neurons. In this study, we examined the effects of cysteine-specific redox agents on the A-type K(+) currents in acutely dissociated small diameter dorsal root ganglion (DRG) neurons from rats. The fast inactivation of most A-type currents was markedly removed or slowed by the oxidizing agents 2,2'-dithio-bis(5-nitropyridine) (DTBNP) and chloramine-T. Dithiothreitol, a reducing agent for the disulfide bond, restored the inactivation. These results demonstrated that native A-type K(+) channels, probably Kv1.4, could switch the roles between inactivating and non-inactivating K(+) channels via redox regulation in pain-sensing DRG neurons. The A-type channels may play a role in adjusting pain sensitivity in response to peripheral redox conditions.

  16. Frequency-domain optical tomographic imaging of arthritic finger joints.

    PubMed

    Hielscher, Andreas H; Kim, Hyun Keol; Montejo, Ludguier D; Blaschke, Sabine; Netz, Uwe J; Zwaka, Paul A; Illing, Gerd; Muller, Gerhard A; Beuthan, Jürgen

    2011-10-01

    We are presenting data from the largest clinical trial on optical tomographic imaging of finger joints to date. Overall we evaluated 99 fingers of patients affected by rheumatoid arthritis (RA) and 120 fingers from healthy volunteers. Using frequency-domain imaging techniques we show that sensitivities and specificities of 0.85 and higher can be achieved in detecting RA. This is accomplished by deriving multiple optical parameters from the optical tomographic images and combining them for the statistical analysis. Parameters derived from the scattering coefficient perform slightly better than absorption derived parameters. Furthermore we found that data obtained at 600 MHz leads to better classification results than data obtained at 0 or 300 MHz.

  17. Complements Spurned: Our Experience with Atypical Hemolytic Uremic Syndrome

    PubMed Central

    Nagar, Vidya S.; Chaterjee, Rudrarpan; Sood, Ankita; Sajjan, Basavaraj; Kaushik, Aniruddha; Vyahalkar, Sameer V.

    2017-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare disorder resulting from a dysregulated activation of the alternative pathway of the complement system. It results in significant morbidity and mortality if not diagnosed and treated promptly. It lends itself to myriad renal and extrarenal manifestations, all potentially disabling. Eculizumab, a monoclonal antibody to complement C5 is now the widely accepted norm for treatment. However, in resource-limited settings, plasma exchange if instituted early may be as beneficial. We report a case of aHUS treated with extended plasma exchange with excellent results. Critical care monitoring is essential for the management of the disease in view of a tendency to develop multiple complications. Long-term immunosuppression may be successful in maintaining remission. PMID:28250608

  18. Incomplete Stevens-Johnson syndrome secondary to atypical pneumonia.

    PubMed

    Ramasamy, Anantharaman; Patel, Chiraush; Conlon, Christopher

    2011-10-04

    Steven-Johnson syndrome is a common condition characterised by erythematous target lesions on the skin and involvement of the oral mucosa, genitals and conjunctivae. It has been documented as one of the extra-pulmonary manifestations of Mycoplasma pneumoniae infection. Recently, there has been several documentation of an incomplete presentation of this syndrome - without the typical rash but with mucosal, conjunctival and genital involvement. Our case illustrates that the incomplete Steven-Johnson syndrome may present with oral mucosal and conjunctival involvement alone without skin or genital involvement. This important clinical diagnosis should not be missed due to its atypical presentation. Treatment of Steven-Johnson syndrome remains supportive along with treating the underlying infection if recognised.

  19. 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

  20. ACKR2: An Atypical Chemokine Receptor Regulating Lymphatic Biology

    PubMed Central

    Bonavita, Ornella; Mollica Poeta, Valeria; Setten, Elisa; Massara, Matteo; Bonecchi, Raffaella

    2017-01-01

    The lymphatic system plays an important role in the induction of the immune response by transporting antigens, inflammatory mediators, and leukocytes from peripheral tissues to draining lymph nodes. It is emerging that lymphatic endothelial cells (LECs) are playing an active role in this context via the expression of chemokines, inflammatory mediators promoting cell migration, and chemokine receptors. Particularly, LECs express atypical chemokine receptors (ACKRs), which are unable to promote conventional signaling and cell migration while they are involved in the regulation of chemokine availability. Here, we provide a summary of the data on the role of ACKR2 expressed by lymphatics, indicating an essential role for this ACKRs in the regulation of the inflammation and the immune response in different pathological conditions, including infection, allergy, and cancer. PMID:28123388

  1. 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.

  2. Carcinoma cuniculatum: an atypical presentation in the foot.

    PubMed

    Thomas, Eric J; Graves, Nathan C; Meritt, Stephen M

    2014-01-01

    Carcinoma cuniculatum is a rarely occurring, low-grade variant of squamous cell carcinoma most commonly found in the forefoot. It should be suspected in the differential diagnosis for chronic nonhealing verrucous wounds. The initial diagnosis of carcinoma cuniculatum is difficult and often delayed and can require repeated histopathologic evaluation by an experienced pathologist. In many cases, the final diagnosis will be made from the microscopic findings correlated with the clinical examination findings. Metastasis from carcinoma cuniculatum is rare, although it can invade deep into the soft tissues and can extend to the bone. Wide local excision with a 5-mm tumor-free margin has been the recommended treatment. If the tumor extends into the bone, amputation might be warranted. The present report describes the case of an atypical carcinoma cuniculatum found in the hindfoot of an adult female and represents the surgical follow-up to the histopathologic description of the same patient's lesion as previously described in a published report.

  3. Post-Surgical Atypical FDG-PET Uptake

    PubMed Central

    Dubroff, Jacob G.; Alavi, Abass; Friedberg, Joseph S.; Cengel, Keith A.

    2011-01-01

    False positive recognition is crucial for proper interpretation of FDG-PET studies. The authors present a case of a woman who underwent surgery over a month prior to PET/CT imaging which revealed significant tracer uptake within muscles and soft tissue in several sites contralateral to the location of surgery. The FDG-PET images of this case illustrate the importance of communication between physicians ordering and physicians reading FDG-PET/CT scans as well as atypical FDG-PET findings that could be interpreted as concerning but are, in fact, innocuous. This study also demonstrates the unusual glucose metabolic patterns which may arise following treatment be it surgical, chemotherapeutic or radiation. PMID:19851183

  4. 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

  5. [Venous thrombosis of atypical location in patients with cancer].

    PubMed

    Campos Balea, Begoña; Sáenz de Miera Rodríguez, Andrea; Antolín Novoa, Silvia; Quindós Varela, María; Barón Duarte, Francisco; López López, Rafael

    2015-01-01

    Venous thromboembolism (VTE) is a complication that frequently occurs in patients with neoplastic diseases. Several models have therefore been developed to identify patient subgroups diagnosed with cancer who are at increased risk of developing VTE. The most common forms of thromboembolic episodes are deep vein thrombosis in the lower limbs and pulmonary thromboembolism. However, venous thrombosis is also diagnosed in atypical locations. There are few revisions of unusual cases of venous thrombosis. In most cases, VTE occurs in the upper limbs and in the presence of central venous catheters, pacemakers and defibrillators. We present the case of a patient diagnosed with breast cancer and treated with surgery, chemotherapy and radiation therapy who developed a thrombosis in the upper limbs (brachial and axillary).

  6. 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

  7. Atypical Pupillary Light Reflex in Individuals With Autism

    DTIC Science & Technology

    2012-07-01

    Brain connectivity and high functioning  autism : A promising path of research that needs refined  models , methodological  convergence, and stronger...propose to further evaluate the atypical dynamic pupillary light reflex (PLR)  observed in children  autism . PLR refers to the involuntary response whereby...human subjects including 100 children with  autism  (“ASD”  group), 65 typically developing children (“TC” group), 35 children with early brain

  8. 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).

  9. Surgical Management and Scheimpflug Analysis of an Atypical Lens Coloboma

    PubMed Central

    Hernadez-Camarena, Julio C.; Ayup-Arguijo, Eduardo; Chavez-Mondragon, Eduardo; Ramirez-Miranda, Arturo

    2012-01-01

    A lens coloboma is not a true coloboma; it is just a zonular absence that causes a defect in the lens equator and hence a more spherical lens. It can be isolated or in association with iris, choroid or retinal colobomas. Typically the defect is present at the site of the embryonic fissure, but it can be present elsewhere. This congenital defect can cause amblyopia, especially when it is associated with cataract. We describe a case of a 39-year-old male with an atypical coloboma that was managed successfully with phacoemulsification, capsular tension ring and intraocular lens implantation. Scheimpflug image analysis and full ophthalmological assessment were performed. Scheimpflug images demonstrated a notching of the lens equator and an absence of zonulae. During the surgical procedure an ophthalmic viscosurgical device was used to tamponade the vitreous, and after continuous curvilinear capsulorhexis the capsular tension ring was inserted to allow the surgeon to perform a safe phacoemulsification. PMID:23139675

  10. Cutaneous atypical mycobacteriosis in a clouded leopard (Neofelis nebulosa).

    PubMed

    Cerveny, Shannon N S; Thompson, Michelle E; Corner, Sarah M; Swinford, Amy K; Coke, Rob L

    2013-09-01

    A 16-yr-old male clouded leopard (Neofelis nebulosa) was presented for lethargy and anorexia. A cutaneous abdominal mass extending from the pubis to just caudal to the xiphoid process was present. A biopsy revealed histologic lesions consistent with an atypical mycobacterial infection consisting of diffuse, severe, pyogranulomatous dermatitis and panniculitis, with clear vacuoles and 3-5 microm, intravacuolar, faintly eosinophilic, filamentous bacilli that stained positively with FiteFaraco modified acid-fast stain. The clouded leopard had biochemical findings suggestive of chronic renal failure and euthanasia was elected. Histological evaluation of tissues collected at postmortem examination revealed multicentric B-cell lymphoma involving the oral cavity, liver, spleen, and multiple lymph nodes, bilateral testicular seminomas, thyroid follicular cell adenoma, thyroid C cell adenoma, and biliary cystadenomas. Bacterial culture and molecular sequencing identified the causative agent of the cutaneous abdominal mass as belonging to the Mycobacterium fortuitum group.

  11. The Impact of Statin Use on Lipid Levels in Statin-Naive Patients with Rheumatoid Arthritis (RA) vs. non-RA Subjects: Results from a Population-Based Study

    PubMed Central

    Myasoedova, Elena; Gabriel, Sherine E.; Green, Abigail B.; Matteson, Eric L.; Crowson, Cynthia S.

    2013-01-01

    Objectives To examine lipid profiles among statin-naive patients with rheumatoid arthritis (RA) and those without RA before and after the initiation of statins. Methods Information regarding lipid measures and statin use was gathered in a population-based incident cohort of patients with RA (1987 ACR criteria first met between 1/1/1988 and 1/1/2008) and in a cohort of non-RA subjects from the same underlying population. Only patients with no prior history of statin use were included. Results The study included 161 patients with RA (mean age 56.3 years, 57% female) and 221 non-RA subjects (mean age 56.0 years, 66% female). Prior to the start of statins, the levels of total cholesterol (TC) and low-density lipoprotein cholesterol (LDL) were lower in RA vs non-RA cohort (p<0.001 and p=0.003, respectively). The absolute and percent change in LDL after at least 90 days of statin use tended to be smaller in RA vs non-RA cohort (p=0.03 and p=0.09). After at least 90 days of statin use patients with RA were less likely to achieve therapeutic goals for LDL than the non-RA subjects (p=0.046). Increased erythrocyte sedimentation rate (ESR) at baseline (OR 0.47; 95% CI 0.26, 0.85) was associated with lower likelihood of achieving therapeutic LDL goals. Conclusion Patients with RA had lower TC and LDL levels before statin initiation and lower likelihood of achieving therapeutic LDL goals following statin use than the non-RA subjects. Some RA disease characteristics, in particular ESR at baseline, may have an adverse impact on achieving therapeutic LDL goals. PMID:23592565

  12. Atypical mycobacterial cutaneous infections in Egyptians: a clinicopathological study.

    PubMed

    El-Khalawany, Mohamed A

    2014-04-01

    Atypical mycobacteria comprise an uncommon heterogenous non-tuberculous group of acid-fast bacteria that rarely involve skin. The pattern of atypical mycobacterial cutaneous infections (AMCI) has not been previously studied in Egypt. The aim of this study was to describe the clinical characteristics, pathological features and species profile of AMCI among Egyptian patients. A retrospective study included 46 cases, diagnosed with AMCI during the period 2002 to 2012. The study included 34 males (73.9%) and 12 females (26.9%). The average age of patients was 39 years while the average duration of lesions was 15 months. The lesions were mostly located on the extremities (91.3%) and there was predominance of single (65.2%) and nodular (41.4%) lesions. History of trauma was confirmed in 91.3%. Histologically, the granulomas were mostly superficial (67.4%) with predominance of nodular suppurative pattern (84.8%). Other significant histological findings included epidermal hypertrophy (100%), presence of large-sized multinucleated giant cells (87%) and intrafollicular neutrophilic abscesses (84.8%). The diagnosis was proved by direct smear in 6.5%, skin biopsy in 10.9%, tissue culture in 47.8% and polymerase chain reaction (PCR) in 34.8%. Isolated species included Mycobacterium marinum (84.8%), Mycobacterium fortuitum (10.9%) and Mycobacterium kansasii (4.3%). Although the results of this study recommend that the diagnosis of AMCI is based mainly on culture and PCR, other clinicopathological features such as history of trauma, acral location of the lesion and suppurative granulomatous reaction with intrafollicular abscesses could be helpful clues in suspecting AMCI.

  13. Magnetic Properties of Hydrothermalized A-type Red Granites

    NASA Astrophysics Data System (ADS)

    Trindade, R. I. F.; Nédélec, A.; Peschler, A.; Archanjo, C. J.; Poitrasson, F.; Bouchez, J. L.

    Hydrothermalized A-type granites are commonly identified by their pink to red-brick colour attributed to tiny flakes of hematite in the alkali feldspars. These inclusions can be of interest in magnetic studies, but their timing and process of formation are still unclear. Formation of chlorite after biotite is the commonest effect of hydrother- malization and may occur quite early after crystallization due to late-magmatic or externally-derived fluids. The reddish colour appears at a later stage. Five cases of A-type granites were investigated for their magnetic mineralogy and properties. The selected cases range from nearly unmodified granites (Panafrican stratoid granites of Madagascar) to strongly hydrothermalized ones (Meruoca, Brazil; Tana, Corsica); in- termediate cases are : Mount Scott (Oklahoma), Bushveld (granitic core kindly pro- vided by R.G. Cawthorn) and. Hydrothermal alteration is often associated to a de- crease of the magnetic susceptibility magnitude (K) and of the anisotropy degree (P). It also strongly affects the rockt's bulk coercivity parameters, since alteration changes the relative amounts of coarse-grained primary magnetite, fine-grained PSD to SD sec- ondary magnetite, and hematite. Correspondingly, most samples plot away from the magnetite trend in the Dayt's diagram, but the different groups identified after coer- civity parameters do not directly correlate with whole-rock colour. In addition, IRM- acquisition curves and thermal demagnetization of tri-axial IRM show that hematite occurs in almost all analysed samples despite their colour. Various hematite coercivity ranges are also evidenced. In fact, hematite can be formed either in feldspar crys- tals or after magnetite. Tiny hematite within feldspars can appear either by exsolu- tion process or, more likely, by precipitation from a fluid phase. For these reasons, hematite inclusions may carry a remanence acquired shortly after granite crystalliza- tion or, conversely, a recent

  14. A macroprolactinoma becoming resistant to cabergoline and developing atypical pathology

    PubMed Central

    Farah, George; Fathelrahman, Ahmed; Cudlip, Simon; Ansorge, Olaf; Karavitaki, Niki; Grossman, Ashley B

    2016-01-01

    Summary Pituitary adenomas are a common intracranial neoplasm, usually demonstrating a benign phenotype. They can be classified according to pathological, radiological or clinical behaviour as typical, atypical or carcinomas, invasive or noninvasive, and aggressive or nonaggressive. Prolactinomas account for 40–60% of all pituitary adenomas, with dopamine agonists representing the first-line treatment and surgery/radiotherapy reserved for drug intolerance/resistance or in neuro-ophthalmological emergencies. We present the case of a 62-year-old man with an apparently indolent prolactin-secreting macroadenoma managed with partial resection and initially showing a biochemical response to cabergoline. Five years later, the tumour became resistant to cabergoline, despite a substantial increase in dosage, showing rapid growth and causing worsening of vision. The patient then underwent two further transsphenoidal operations and continued on high-dose cabergoline; despite these interventions, the tumour continued enlarging and prolactin increased to 107 269 U/L. Histology of the third surgical specimen demonstrated features of aggressive behaviour (atypical adenoma with a high cell proliferation index) not present in the tumour removed at the first operation. Subsequently, he was referred for radiotherapy aiming to control tumour growth. Learning points: The development of secondary resistance to dopamine agonists (DAs) is a serious sign as it may be associated with de-differentiation of the prolactinoma and thus of aggressive or malignant transformation. Significant de-differentiation of the adenoma documented on consecutive histologies suggests a possible transition to malignancy. A combination of histological ‘alarm’ features associated with persistent growth and escape from DAs treatment in recurrent adenomas should alert clinicians and demands close follow-up. A multidisciplinary approach by pathologists, endocrinologists and neurosurgeons is essential. PMID

  15. 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

  16. Release of 226Ra from uranium mill tailings by microbial Fe(III) reduction

    USGS Publications Warehouse

    Landa, E.R.; Phillips, E.J.P.; Lovley, D.R.

    1991-01-01

    Uranium mill tailings were anaerobically incubated in the presence of H2 with Alteromonas putrefaciens, a bacterium known to couple the oxidation of H2 and organic compounds to the reduction of Fe(III) oxides. There was a direct correlation between the extent of Fe(III) reduction and the accumulation of dissolved 226Ra. In sterile tailings in which Fe(III) was not reduced, there was negligible leaching of 226Ra. The behavior of Ba was similar to that of Ra in inoculated and sterile systems. These results demonstrate that under anaerobic conditions, microbial reduction of Fe(III) may result in the release of dissolved 226Ra from uranium mill tailings. ?? 1991.

  17. 78. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    78. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 REAR PLATE, LEAN-TO SHOWING NOTCHING for ATTIC FLOOR BEAM - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  18. 76. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    76. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ELEVATION of INTERMEDIATE RAFTER in LEAN-TO - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  19. 83. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    83. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 SPLICING of PURLINS over INTERMEDIATE RAFTER in LEAN-TO LOOKING SOUTH EAST - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  20. 115. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    115. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 SIDE of DRESSER in PANTRY LEAN-TO - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  1. 71. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    71. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 RAFTER on REAR PLATE (INTERMEDIATE RAFTER LEAN-TO) - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  2. 79. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    79. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ATTIC FLOOR TIMBER in LEAN-TO INVERTED - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  3. 70. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    70. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ELEVATION FRAMING of NORTH WEST CORNER LEAN-TO LOOKING SOUTH WEST - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  4. 69. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    69. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 CORNER POST & RAFTER LOOOKING SOUTH WEST (NORTH WEST CORNER LEAN-TO) - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  5. Rapid method for the determination of 226Ra in hydraulic fracturing wastewater samples

    SciTech Connect

    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.

  6. Laser-cooled RaF as a promising candidate to measure molecular parity violation

    SciTech Connect

    Isaev, T. A.; Berger, R.; Hoekstra, S.

    2010-11-15

    The parameter W{sub a}, which characterizes nuclear-spin-dependent parity violation (PV) in the molecular spin-rotational Hamiltonian, was computed with a quasirelativistic Hartree-Fock approach for radium fluoride (RaF) and found to be one of the largest absolute values predicted so far. The peculiar electronic structure of RaF leads to highly diagonal Franck-Condon matrices between the energetically lowest two electronic states, which qualifies RaF for direct laser cooling. A subset of diatomic molecules with a wide range of internal structures suitable for this cooling technique is also indicated. As trapped cold molecules offer superior coherence times, RaF can be considered promising for high-precision experiments aimed at molecular PV.

  7. Guidance: Strategies to Achieve Timely Settlement and Implementation of RD/RA at Superfund Sites

    EPA Pesticide Factsheets

    Memorandum recommends strategies to encourage PRPs to enter into a settlement using the model RD/RA Consent Decree; discusses the current model UAO; and suggests practical alternatives to expedite Superfund settlements and the cleanup process.

  8. 117. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    117. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 OLD WALL PAPER from CUPBOARD in 1st. FLOOR WEST ROOM of MAIN HOUSE - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  9. 73. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    73. Historic American Buildings Survey R.A. Waugh, Photographer October 1936 SOUTH END of WEST PLATE (INVERTED) - Colonel Paul Wentworth House, Dover Street (moved to MA, Dover), Dover, Strafford County, NH

  10. Assessment of (226)Ra age-dependent dose from water intake.

    PubMed

    Porntepkasemsan, Boonsom; Srisuksawad, Kanitha

    2008-11-01

    The radioactivity in canal and ground waters collected in a 2-year long observation from the vicinity of the Rare Earth Research and Development Center (RRDC), Phathumthani Province, Thailand, was measured in order to determine the concentration of (226)Ra and to estimate the age-dependent effective dose to humans due to consumption. (226)Ra activities in both canal and ground waters were well below the WHO guidance level for drinking water quality of 1 Bq L(-1). The highest (226)Ra effective doses per year were found for infants and teens. However, the observed levels of calculated (226)Ra effective doses for all age groups in both canal and ground waters show satisfactory low values (less than 15 microSv yr(-1)). These values are acceptable in accordance with the WHO recommended reference dose level of 100 microSv yr(-1) from water intake of 2 Lday(-1).

  11. 226Ra bioavailability to plants at the Urgeiriça uranium mill tailings site.

    PubMed

    Madruga, M J; Brogueira, A; Alberto, G; Cardoso, F

    2001-01-01

    Large amounts of solid wastes (tailings) resulting from the exploitation and treatment of uranium ore at the Urgeiriça mine (north of Portugal) have been accumulated in dams (tailing ponds). To reduce the dispersion of natural radionuclides into the environment, some dams were revegetated with eucalyptus (Eucalyptus globolus) and pines (Pinus pinea). Besides these plants, some shrubs (Cytisus spp.) are growing in some of the dams. The objective of this study is to determine the 226Ra bioavailability from uranium mill tailings by quantifying the total and available fraction of radium in the tailings and to estimate its transfer to plants growing on the tailing piles. Plant and tailing samples were randomly collected and the activity concentration of 226Ra in plants (aerial part and roots) and tailings was measured by gamma-spectrometry. The exchangeable fraction of radium in tailings was quantified using one single step extraction with 1 mol dm-3 ammonium acetate (pH = 7) or 1 mol dm-3 calcium chloride solutions. The results obtained for 226Ra uptake by plants show that 226Ra concentration ratios for eucalyptus and pines decrease at low 226Ra concentrations in the tailings and appear relatively constant at higher radium concentrations. For shrubs, the concentration ratios increase at higher 226Ra solid waste concentrations approaching a saturation value. Percentage values of 16.0 +/- 8.3 and 12.9 +/- 8.9, for the fraction of radium extracted from the tailings, using 1 mol dm-3 ammonium acetate or calcium chloride solutions, respectively, were obtained. The 226Ra concentration ratios determined on the basis of exchangeable radium are one order of magnitude higher than those based on total radium. It can be concluded that, at a 95% confidence level, more consistent 226Ra concentration ratios were obtained when calculated on the basis of available radium than when total radium was considered, for all the dams.

  12. Rapid determination of 226Ra in emergency urine samples

    SciTech Connect

    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. 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.

  13. Half-life and excitation energy of the Iπ=13/2+ isomer in Ra209

    NASA Astrophysics Data System (ADS)

    Hauschild, K.; Lopez-Martens, A.; Yeremin, A. V.; Dorvaux, O.; Belozerov, A. V.; Chelnokov, M. L.; Chepigin, V. I.; Gall, B.; Gorshkov, V. A.; Guttormsen, M.; Jones, P.; Kabachenko, A. P.; Khouaja, A.; Larsen, A. C.; Malyshev, O. N.; Minkova, A.; Nyhus, H. T.; Oganessian, Yu. Ts.; Pantelica, D.; Popeko, A. G.; Rotaru, F.; Saro, S.; Shutov, A. V.; Siem, S.; Svirikhin, A. I.; Syed, N. U. H.

    2008-04-01

    An isomeric state in Ra209 has been observed for the first time, using the GABRIELA setup at the focal plane of VASSILISSA, to decay to the ground state of Ra209 via a cascade of 238-keV (M2) and 644-keV transitions. The half-life of the isomer has been measured to be 117(5)μs and from systematics is assigned as a neutron i13/2-1 excitation.

  14. Ra isotopes in trees: Their application to the estimation of heartwood growth rates and tree ages

    NASA Astrophysics Data System (ADS)

    Hancock, Gary J.; Murray, Andrew S.; Brunskill, Gregg J.; Argent, Robert M.

    2006-12-01

    The difficulty in estimating growth rates and ages of tropical and warm-temperate tree species is well known. However, this information has many important environmental applications, including the proper management of native forests and calculating uptake and release of atmospheric carbon. We report the activities of Ra isotopes in the heartwood, sapwood and leaves of six tree species, and use the radial distribution of the 228Ra/226Ra activity ratio in the stem of the tree to estimate the rate of accretion of heartwood. A model is presented in which dissolved Ra in groundwater is taken up by tree roots, translocated to sapwood in a chemically mobile (ion-exchangeable) form, and rendered immobile as it is transferred to heartwood. Uptake of 232Th and 230Th (the parents of 228Ra and 226Ra) is negligible. The rate of heartwood accretion is determined from the radioactive decay of 228Ra (half-life 5.8 years) relative to long-lived 226Ra (half-life 1600 years), and is relevant to growth periods of up to 50 years. By extrapolating the heartwood accretion rate to the entire tree ring record the method also appears to provide realistic estimates of tree age. Eight trees were studied (three of known age, 72, 66 and 35 years), including three Australian hardwood eucalypt species, two mangrove species, and a softwood pine (P. radiata). The method indicates that the rate of growth ring formation is species and climate dependent, varying from 0.7 rings yr-1 for a river red gum (E. camaldulensis) to around 3 rings yr-1 for a tropical mangrove (X. mekongensis).

  15. Dihydropyridines and atypical MDR: a novel perspective of designing general reversal agents for both typical and atypical MDR.

    PubMed

    Miri, Ramin; Mehdipour, Ahmadreza

    2008-09-15

    Multidrug resistance (MDR) is defined as resistance of tumor cells to the cytotoxic action of multiple structurally dissimilar and functionally divergent drugs commonly used in chemotherapy. 1,4-Dihydropyridines (DHP(s)) are one of the major classes of Ca2+ channel blockers, and it has been shown that these agents are a new class of drug resistance reversers in cancer treatment. Analysis of various investigations on MDR reversing effects of DHPs shows that they can be a potential class for designing compounds simultaneously effective on both typical and atypical MDR. Also, it is possible to include some considerations on essential structural features for MDR reversing and further decreasing of Ca2+ channel blocking activity as an adverse effect.

  16. Leaching of 226Ra from U mill tailings by sulfate-reducing bacteria.

    PubMed

    Landa, E R; Miller, C L; Updegraff, D M

    1986-10-01

    Relatively insoluble sulfate precipitates appear to be a major host for Ra in sulfuric acid-treated, U mill tailings. The dissolution of such precipitates by natural processes, such as metabolism by sulfate-reducing bacteria (SRB), creates the potential for release of Ra to contacting waters. Significant leaching of Ra by SRB was achieved in the laboratory during the anaerobic incubation (1 to 119 days) of U mill tailings with pure cultures of Desulfovibrio desulfuricans and mixed cultures containing SRB isolated from the tailings, all grown on a lactate medium at room temperature. While the maximum 226Ra concentration reached in a sterile media control was 0.44 Bq/L (12 pCi/L), that in the SRB systems was 61 Bq/L (1640 pCi/L) or about 20% of the total Ra inventory in the original tailings sample. The leaching of Ra in SRB systems was accompanied by a decrease in soluble sulfate concentration, an increase in total sulfide concentration, and an increase in the number of SRB. The observed leaching effect does not appear to be due to the action of microbial chelates or to binding to cell walls. Potential implications of these findings to the management of U mill tailings and other radioactive wastes are discussed.

  17. Distribution and retention in bone of /sup 226/Ra and comparison with the ICRP 20 model

    SciTech Connect

    Holtzman, R.B.; Rundo, J.; Sha, J.Y.; Spaletto, M.I.

    1981-01-01

    Analyses are presented of the ratios of /sup 226/Ra to calcium in over 650 samples of compact and cancellous bone from 66 female and 26 male subjects who had died from less than one to 60 years after first exposure to radium. The /sup 226/Ra/Ca ratios were normalized to the terminal /sup 226/Ra skeletal content. The /sup 226/Ra/Ca ratios for vertebrae were essentialy identical to those for other cancellous bone for a given subject. Comparisons of the data with predictions of the ICRP model of alkaline earth metabolism show that for female cancellous bone the normalized /sup 226/Ra/Ca ratios tended to be greater than predicted, while those for female cortical bone (femoral and tibial shaft) tended to be less. The data for males were fitted better by the model. A modification of the model to reduce the amount of radium deposited in soft tissue fitted the data better in some respects. A straight line linear least squares fit to the data appeared to fit as well as, or better than, the models. A radiation effect was suggested in that the normalized /sup 226/Ra/Ca ratio for vertebrae relative to the ratio expected increased with skeletal absorbed dose for vertebra. However, no such effect was apparent for compact bone or for the cancellous bone as a whole.

  18. Sex differences of excitatory synaptic transmission in RA projection neurons of adult zebra finches.

    PubMed

    Wang, Songhua; Meng, Wei; Liu, Shaoyi; Liao, Congshu; Huang, Qingyao; Li, Dongfeng

    2014-10-17

    Zebra finches are ideal animals to investigate sex difference in songbirds. Only males can sing. The brain nuclei controlling song learning and production in males are considerably larger than in females. The robust nucleus of the arcopallium (RA) is a premotor nucleus, playing a key role in controlling singing. RA receives denser synapse inputs in males than in females. Sex differences of excitatory synaptic transmission in the RA projection neurons (PNs) have not been reported. In the present study, using whole-cell voltage-clamp recording, spontaneous EPSCs (sEPSCs) and miniature EPSCs (mEPSCs) of RA PNs in the intact males and females were recorded. The average frequency and amplitude of sEPSCs/mEPSCs in the intact males were higher than females. The half-width and decay time of sEPSCs/mEPSCs in the intact males were longer than females. In order to verify whether these sex differences related to sex steroids, males were castrated. The average frequency of sEPSCs/mEPSCs in castrated males was lower than intact males and was similar to in females; the amplitude was not changed after castrating. These results demonstrate the sexually dimorphic of the excitatory synaptic transmission in the RA PNs, the RA PNs in males receive more excitatory synaptic transmission and these sex differences were partly affected by sex hormones. These findings contribute to further illuminate the neural mechanisms under the sexually dimorphism in song production of adult zebra finches.

  19. Overview of the Radiation Dosimetry Experiment (RaD-X) flight mission

    NASA Astrophysics Data System (ADS)

    Mertens, Christopher J.

    2016-11-01

    The NASA Radiation Dosimetry Experiment (RaD-X) stratospheric balloon flight mission addresses the need to reduce the uncertainty in predicting human exposure to cosmic radiation in the aircraft environment. Measurements were taken that characterize the dosimetric properties of cosmic ray primaries, the ultimate source of aviation radiation exposure, and the cosmic ray secondary radiations that are produced and transported to aviation altitudes. In addition, radiation detectors were flown to assess their potential application to long-term, continuous monitoring of the aircraft radiation environment. RaD-X was successfully launched from Fort Sumner, New Mexico (34.5°N, 104.2°W), on 25 September 2015. Over 18 h of science data were obtained from a total of four different type dosimeters at altitudes above 20 km. The RaD-X flight mission was supported by laboratory radiation exposure testing of the balloon flight dosimeters and also by coordinated radiation measurements taken on ER-2 and commercial aircraft. This paper provides the science background and motivation for the RaD-X flight mission, a brief description of the balloon flight profile and the supporting aircraft flights, and a summary of the articles included in the RaD-X special collection and their contributions to the science goals of the RaD-X mission.

  20. Field study of Ra accumulation in trout with assessment of radiation dose to man

    SciTech Connect

    Ropes, S.K.; Whicker, F.W.

    1985-08-01

    The purpose of this study was to determine the concentrations of /sup 226/Ra in edible fish from surface ponds near an open pit U mine. Because one reclamation plan for the U mine proposed formation of an artificial lake in the open pit, potential radiation dose to man from ingestion of fish needed to be investigated. Trout were collected from four existing ponds which varied in mean /sup 226/Ra concentration from 12-33 pCi/l and in Ca concentration from 30-330 mg Ca/l. Radium and Ca accumulation in trout flesh, skin, fins and bone were measured. Geometric mean concentrations of /sup 226/Ra in trout flesh from four ponds ranged from 6.3-30 pCi/kg wet weight. The distribution of Ra in the trout body was similar to that of Ca. The calculated dose equivalent commitment to human endosteal tissue range from 0.2-2 mrem per fish consumed, depending on the assumed dietary and environmental parameters. Neglecting the consumption of trout skin underestimated the ingestion dose from /sup 226/Ra by a factor of 5-10. Estimated annual dose equivalent rates to human endosteal tissue ranged from 1.0-83 mrem/yr for an individual who consumed one fish per week for a 50-yr period. The dose to man from ingestion of /sup 226/Ra in fish would not likely preclude the establishment of a recreational lake at this site.

  1. Overview of the Radiation Dosimetry Experiment (RaD-X) Flight Mission

    NASA Technical Reports Server (NTRS)

    Mertens, Christopher J.

    2016-01-01

    The NASA Radiation Dosimetry Experiment (RaD-X) stratospheric balloon flight mission addresses the need to reduce the uncertainty in predicting human exposure to cosmic radiation in the aircraft environment. Measurements were taken that characterize the dosimetric properties of cosmic ray primaries, the ultimate source of aviation radiation exposure, and the cosmic ray secondary radiations that are produced and transported to aviation altitudes. In addition, radiation detectors were flown to assess their potential application to long-term, continuous monitoring of the aircraft radiation environment. RaD-X was successfully launched from Fort Sumner, New Mexico (34.5 N, 104.2 W), on 25 September 2015. Over 18 h of science data were obtained from a total of four different type dosimeters at altitudes above 20 km. The RaD-X flight mission was supported by laboratory radiation exposure testing of the balloon flight dosimeters and also by coordinated radiation measurements taken on ER-2 and commercial aircraft. This paper provides the science background and motivation for the RaD-X flight mission, a brief description of the balloon flight profile and the supporting aircraft flights, and a summary of the articles included in the RaD-X special collection and their contributions to the science goals of the RaD-X mission.

  2. Gain of chromosome arm 1q in atypical meningioma correlates with shorter progression-free survival

    PubMed Central

    Jansen, M.; Mohapatra, G.; Betensky, R.A.; Keohane, C.; Louis, D.N.

    2013-01-01

    Aims Atypical (WHO grade II) meningiomas have moderately high recurrence rates; even for completely resected tumours, approximately one-third will recur. Postoperative radiotherapy (RT) may aid local control and improve survival, but carries the risk of side effects. More accurate prediction of recurrence risk is therefore needed for patients with atypical meningioma. Previously, we used high-resolution array CGH to identify genetic variations in 47 primary atypical meningiomas and found that approximately 60% of tumors show gain of 1q at 1q25.1 and 1q25.3 to 1q32.1 and that 1q gain appeared to correlate with shorter progression-free survival. This study aimed to validate and extend these findings in an independent sample. Methods 86 completely resected atypical meningiomas (with 25 recurrences) from two neurosurgical centres in Ireland were identified and clinical follow up was obtained. Utilizing a dual-colour interphase FISH assay, 1q gain was assessed using BAC probes directed against 1q25.1 and 1q32.1. Results The results confirm the high prevalence of 1q gain at these loci in atypical meningiomas. We further show that gain at 1q32.1 and age each correlate with progression-free survival in patients who have undergone complete surgical resection of atypical meningiomas. Conclusions These independent findings suggest that assessment of 1q copy number status can add clinically useful information for the management of patients with atypical meningiomas. PMID:21988727

  3. Atypical Antipsychotic Use in the Treatment of Psychosis in Primary Care

    PubMed Central

    Leo, Raphael J.; Regno, Paula Del

    2000-01-01

    Atypical antipsychotics are a class of novel agents increasingly employed for the treatment of psychotic disorders. The pharmacodynamic properties of the atypicals appear to impact a broader spectrum of psychotic symptoms than had been appreciated with older generation antipsychotics. In addition, the atypical agents appear to have a reduced risk of neurologic side effects compared with conventional antipsychotic use. Both of these features enhance the appeal of the atypical antipsychotics and may be associated with enhanced patient compliance. The atypical antipsychotics appear to be effective for schizophrenia as well as other psychotic disorders, including schizoaffective disorder and mood disorders with psychotic features. Consequently, atypical antipsychotics are now considered to be the first-line treatment for schizophrenia, with the exception of clozapine, which is considered a second-line agent because of risks associated with its use. This review will discuss the literature on atypical antipsychotic efficacy in psychotic disorders. Issues related to antipsychotic use, dosing, adverse effects, and drug interactions are also discussed. PMID:15014629

  4. Atypical scrapie isolates involve a uniform prion species with a complex molecular signature.

    PubMed

    Götte, Dorothea R; Benestad, Sylvie L; Laude, Hubert; Zurbriggen, Andreas; Oevermann, Anna; Seuberlich, Torsten

    2011-01-01

    The pathobiology of atypical scrapie, a prion disease affecting sheep and goats, is still poorly understood. In a previous study, we demonstrated that atypical scrapie affecting small ruminants in Switzerland differs in the neuroanatomical distribution of the pathological prion protein (PrP(d)). To investigate whether these differences depend on host-related vs. pathogen-related factors, we transmitted atypical scrapie to transgenic mice over-expressing the ovine prion protein (tg338). The clinical, neuropathological, and molecular phenotype of tg338 mice is similar between mice carrying the Swiss atypical scrapie isolates and the Nor98, an atypical scrapie isolate from Norway. Together with published data, our results suggest that atypical scrapie is caused by a uniform type of prion, and that the observed phenotypic differences in small ruminants are likely host-dependant. Strikingly, by using a refined SDS-PAGE technique, we established that the prominent proteinase K-resistant prion protein fragment in atypical scrapie consists of two separate, unglycosylated peptides with molecular masses of roughly 5 and 8 kDa. These findings show similarities to those for other prion diseases in animals and humans, and lay the groundwork for future comparative research.

  5. Molecular Basis for Failure of “Atypical” C1 Domain of Vav1 to Bind Diacylglycerol/Phorbol Ester*

    PubMed Central

    Geczy, Tamas; Peach, Megan L.; El Kazzouli, Saïd; Sigano, Dina M.; Kang, Ji-Hye; Valle, Christopher J.; Selezneva, Julia; Woo, Wonhee; Kedei, Noemi; Lewin, Nancy E.; Garfield, Susan H.; Lim, Langston; Mannan, Poonam; Marquez, Victor E.; Blumberg, Peter M.

    2012-01-01

    C1 domains, the recognition motif of the second messenger diacylglycerol and of the phorbol esters, are classified as typical (ligand-responsive) or atypical (not ligand-responsive). The C1 domain of Vav1, a guanine nucleotide exchange factor, plays a critical role in regulation of Vav activity through stabilization of the Dbl homology domain, which is responsible for exchange activity of Vav. Although the C1 domain of Vav1 is classified as atypical, it retains a binding pocket geometry homologous to that of the typical C1 domains of PKCs. This study clarifies the basis for its failure to bind ligands. Substituting Vav1-specific residues into the C1b domain of PKCδ, we identified five crucial residues (Glu9, Glu10, Thr11, Thr24, and Tyr26) along the rim of the binding cleft that weaken binding potency in a cumulative fashion. Reciprocally, replacing these incompatible residues in the Vav1 C1 domain with the corresponding residues from PKCδ C1b (δC1b) conferred high potency for phorbol ester binding. Computer modeling predicts that these unique residues in Vav1 increase the hydrophilicity of the rim of the binding pocket, impairing membrane association and thereby preventing formation of the ternary C1-ligand-membrane binding complex. The initial design of diacylglycerol-lactones to exploit these Vav1 unique residues showed enhanced selectivity for C1 domains incorporating these residues, suggesting a strategy for the development of ligands targeting Vav1. PMID:22351766

  6. Prevention and treatment of atypical haemolytic uremic syndrome after kidney transplantation.

    PubMed

    Okumi, Masayoshi; Tanabe, Kazunari

    2016-07-01

    Atypical haemolytic uraemic syndrome is a rare disorder characterized by an over-activated, dysregulated alternative complement pathway due to genetic mutation and environmental triggers. Atypical haemolytic uraemic syndrome is a serious, life-threatening disease characterized by thrombotic microangiopathy, which causes haemolytic anaemia, thrombocytopaenia, and acute renal failure. Since recurrences of atypical haemolytic uraemic syndrome frequently lead to end-stage kidney disease even in renal allografts, kidney transplantation for patients with end-stage kidney disease secondary to atypical haemolytic uraemic syndrome has long been contraindicated. However, over the past several years, advancements in the management of atypical haemolytic uraemic syndrome have allowed successful kidney transplantation in these patients. The key factor of this success is eculizumab, a humanized anti-C5 monoclonal antibody, which inhibits terminal membrane-attack complex formation and thrombotic microangiopathy progression. In the setting of kidney transplantation, there are different possible triggers of post-transplant atypical haemolytic uraemic syndrome recurrence, such as brain-death related injury, ischaemia-reperfusion injury, infections, the use of immunosuppressive drugs, and rejection. Principal strategies are to prevent endothelial damage that could potentially activate alternative complement pathway activation and subsequently lead to atypical haemolytic uraemic syndrome recurrence in kidney allograft. Published data shows that prophylactic eculizumab therapy is highly effective for the prevention of post-transplant atypical haemolytic uraemic syndrome recurrence, and prompt treatment with eculizumab as soon as recurrence is diagnosed is important to maintain renal allograft function. Further study to determine the optimal dosing and duration of prophylactic therapy and treatment of post-transplant atypical haemolytic uraemic syndrome recurrence is needed.

  7. Role of Atypical Bacteria in Hospitalized Patients With Nursing Home-Acquired Pneumonia.

    PubMed

    Meyer-Junco, Laura

    2016-10-01

    Background: Nursing home-acquired pneumonia (NHAP) has been identified as one of the leading causes of mortality and hospitalization for long-term care residents. However, current and previous pneumonia guidelines differ on the appropriate management of NHAP in hospitalized patients, specifically in regard to the role of atypical bacteria such as Chlamydiae pneumonia, Mycoplasma pneumoniae, and Legionella. Objectives: The purpose of this review is to evaluate clinical trials conducted in hospitalized patients with NHAP to determine the prevalence of atypical bacteria and thus the role for empiric antibiotic coverage of these pathogens in NHAP. Methods: Comprehensive MEDLINE (1966-April 2016) and Embase (1980-April 2016) searches were performed using the terms "atypical bacteria", "atypical pneumonia", "nursing-home acquired pneumonia", "pneumonia", "elderly", "nursing homes", and "long term care". Additional articles were retrieved from the review of references cited in the collected studies. Thirteen published clinical trials were identified. Results: In the majority of studies, atypical bacteria were infrequently identified in patients hospitalized with NHAP. However, when an active community-acquired pneumonia (CAP) cohort was available, the rate of atypical bacteria between NHAP and CAP study arms was similar. Only 3 studies in this review adhered to recommended strategies for investigating atypical bacteria; in 2 of these studies, C. pneumoniae was the most common pathogen identified in NHAP cohorts. Conclusion: Although atypical bacteria were uncommon in most NHAP studies in this review, suboptimal microbial investigations were commonly performed. To accurately describe the role of atypical bacteria in NHAP, more studies using validated diagnostic tests are needed.

  8. Typical and atypical appearance of early-onset Alzheimer's disease: A clinical, neuroimaging and neuropathological study.

    PubMed

    Kawakatsu, Shinobu; Kobayashi, Ryota; Hayashi, Hiroshi

    2017-04-01

    The International Working Group (IWG) has classified Alzheimer's disease (AD) as two different types, the typical form and the atypical form, but clinicopathological studies of atypical AD are limited. Because atypical AD cases usually present with early-onset dementia, we investigated 12 patients with early-onset AD, including two patients with typical AD and 10 patients with atypical AD. Of these patients, six had the posterior variant, three had the frontal variant and one had the logopenic variant mixed with semantic dementia. We reported MRI, single-photon emission CT and neuropathological findings in six representative cases. We also described a "left temporal variant" of AD presenting with transcortical cortical sensory aphasia, which has not been reported previously and is another subtype of the posterior variant of AD. We found a significant correlation between regional cerebral blood flow and counts of NFTs in the cerebral cortices. An atypical presentation with focal neuropsychological symptoms roughly correlated with the density of NFTs in the cerebral cortex and more directly related to spongiform changes in the superficial layers of these areas. In contrast, the distribution of amyloid depositions was diffuse and did not necessarily correlate with focal neuropsychological symptoms. Braak staging or ABC score is not necessarily appropriate to evaluate atypical AD, and instead, spongiform changes in addition to tau pathology in the association cortices better explain the diversity of atypical AD. Interestingly, another patient with a posterior variant of AD had a novel type of atypical plaque, which we referred to as "lucent plaque". They were recognizable with HE staining in the circumference and dystrophic neurites were abundant with Gallyas-Braak staining. These plaques demonstrated intense immunoreactivity to both tau AT-8 and amyloid β (Aβ), suggesting a peculiar coexistence pattern of amyloid and tau in these plaques. Clinicopathological studies

  9. 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

  10. First In Vivo Evaluation of Liposome-encapsulated 223Ra as a Potential Alpha-particle-emitting Cancer Therapeutic Agent

    SciTech Connect

    Jonasdottir, Thora J.; Fisher, Darrell R.; Borrebaek, Jorgen; Bruland, Oyvind S.; Larsen, Roy H.

    2006-09-13

    Liposomes carrying chemotherapeutics have had some success in cancer treatment and may be suitable carriers for therapeutic radionuclides. This study was designed to evaluate the biodistribution of and to estimate the radiation doses from the alpha emitter 223Ra loaded into pegylated liposomes in selected tissues. 223Ra was encapsulated in pegylated liposomal doxorubicin by ionophore-mediated loading. The biodistribution of liposomal 223Ra was compared to free cationic 223Ra in Balb/C mice. We showed that liposomal 223 Ra circulated in the blood with an initial half-time in excess of 24 hours, which agreed well with that reported for liposomal doxorubicin in rodents, while the blood half-time of cationic 223Ra was considerably less than one hour. When liposomal 223 Ra was catabolized, the released 223Ra was either excreted or taken up in the skeleton. This skeletal uptake increased up to 14 days after treatment, but did not reach the level seen with free 223Ra. Pre-treatment with non-radioactive liposomal doxorubicin 4 days in advance lessened the liver uptake of liposomal 223 Ra. Dose estimates showed that the spleen, followed by bone surfaces, received the highest absorbed doses. Liposomal 223 Ra was relatively stable in vivo and may have potential for radionuclide therapy and combination therapy with chemotherapeutic agents.

  11. Atypical Carcinoid Tumor with Anaplastic Lymphoma Kinase (ALK) Rearrangement Successfully Treated by an ALK Inhibitor.

    PubMed

    Nakajima, Masayuki; Uchiyama, Naoki; Shigemasa, Rie; Matsumura, Takeshi; Matsuoka, Ryota; Nomura, Akihiro

    This is the first report in which crizotinib, an anaplastic lymphoma kinase (ALK) inhibitor, reduced an atypical carcinoid tumor with ALK rearrangement. A 70-year-old man developed a tumor in the left lung and multiple metastases to the lung and brain. The pathology of transbronchial biopsied specimens demonstrated an atypical carcinoid pattern. Combined with immunohistochemical findings, we diagnosed the tumor as atypical carcinoid. ALK gene rearrangement was observed by both immunohistochemical (IHC) and fluorescence in situ hybridization. He was treated with chemotherapy as first-line therapy, however, the tumor did not respond to chemotherapy. Thereafter, he was treated with crizotinib, which successfully reduced the tumors.

  12. [A case of highly atypical hyperplasia--difficult to differentiate from carcinoma].

    PubMed

    Katayama, S; Furukawa, T; Omura, G; Kubo, H; Momose, K; Kameda, N

    1984-06-01

    We present a 51-year-old nulliparous woman with highly atypical hyperplasia of the uterus induced by the prolonged use of exogenous estrogen. The patient was given nearly 1,415 mg of estradiol dipropionate intramuscularly for over 23 years after operation for bilateral ovarian cysts. She complained of vaginal bleeding and diagnostic endometrial curettage revealed highly atypical hyperplasia which was difficult to differentiate from well differentiated adenocarcinoma. On hysterectomy, atypical tissues were limited to the upper part of the posterior wall of the uterus with no muscle invasion.

  13. Visualizing domain wall and reverse domain superconductivity.

    PubMed

    Iavarone, M; Moore, S A; Fedor, J; Ciocys, S T; Karapetrov, G; Pearson, J; Novosad, V; Bader, S D

    2014-08-28

    In magnetically coupled, planar ferromagnet-superconductor (F/S) hybrid structures, magnetic domain walls can be used to spatially confine the superconductivity. In contrast to a superconductor in a uniform applied magnetic field, the nucleation of the superconducting order parameter in F/S structures is governed by the inhomogeneous magnetic field distribution. The interplay between the superconductivity localized at the domain walls and far from the walls leads to effects such as re-entrant superconductivity and reverse domain superconductivity with the critical temperature depending upon the location. Here we use scanning tunnelling spectroscopy to directly image the nucleation of superconductivity at the domain wall in F/S structures realized with Co-Pd multilayers and Pb thin films. Our results demonstrate that such F/S structures are attractive model systems that offer the possibility to control the strength and the location of the superconducting nucleus by applying an external magnetic field, potentially useful to guide vortices for computing application.

  14. Low pathogenicity of anti-desmoglein 3 immunoglobulin G autoantibodies contributes to the atypical clinical phenotypes in pemphigus.

    PubMed

    Saleh, Marwah A; Hashimoto, Rena; Kase, Yuko; Amagai, Masayuki; Yamagami, Jun

    2015-07-01

    The clinical phenotypes of pemphigus can be explained by the desmoglein (Dsg) compensation theory. However, some atypical cases such as cutaneous pemphigus vulgaris (cPV), in which patients have anti-Dsg3 antibodies without oral erosions, do not conform to this theory. To explain the discrepancy between clinical phenotypes and anti-Dsg antibody profiles, the pathogenic strength of immunoglobulin (Ig)G autoantibodies against Dsg3 must be taken into consideration. We analyzed the epitopes and blister-inducing pathogenic strength of the sera from three patients having IgG against Dsg3 without oral erosions with domain-swapped recombinant proteins and dissociation assay using cultured normal human epidermal keratinocytes. The results showed that all sera contained IgG directed against the amino terminal EC1 domain of Dsg3, as is found in most PV sera. However, dissociation assays revealed that the pathogenic strength of the anti-Dsg3 antibodies in all three cases was extremely lower than that of typical PV cases with mucosal involvement. In conclusion, when anti-Dsg3 IgG antibodies are not sufficient to inhibit the expression of Dsg3 in the oral mucosa, but can inhibit the expression in the skin, skin blisters can result. Therefore, the pathogenicity of anti-Dsg3 antibodies should be regarded as a key factor contributing to the clinical phenotype in pemphigus patients with conflicting antibody profiles.

  15. 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

  16. A Domain Analysis Bibliography

    DTIC Science & Technology

    1990-06-01

    Bauhaus , a prototype CASE workstation for D-SAPS development. [ARAN88A] Guillermo F. Arango. Domain Engineering for Software Reuse. PhD thesis...34 VITA90B: Domain Analysis within the ISEC Rapid Center 48 CMU/SEI-90-SR-3 Appendix III Alphabetical by Organization/Project BAUHAUS * ALLE87A

  17. 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.

  18. 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…

  19. 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…

  20. Long-term blood pressure variability in patients with rheumatoid arthritis (RA) and its impact on cardiovascular events and all-cause mortality in RA: a population-based comparative cohort study

    PubMed Central

    Myasoedova, Elena; Crowson, Cynthia S.; Green, Abigail B.; Matteson, Eric L.; Gabriel, Sherine E.

    2014-01-01

    Objectives To examine long-term visit-to-visit blood pressure (BP) variability in rheumatoid arthritis (RA) vs non-RA subjects and to assess its impact on cardiovascular events and mortality in RA. Methods Clinic BP measures were collected in a population-based incident cohort of RA patients (1987 ACR criteria met between 1/1/1995 and 1/1/2008) and non-RA subjects. BP variability was defined as within-subject standard deviation (SD) in systolic and diastolic BP. Results Study included 442 RA patients (mean age 55.5 years, 70% females) and 424 non-RA subjects (mean age 55.7 years, 69% females). RA patients had higher visit-to-visit variability in systolic BP (13.8±4.7 mm Hg), than non-RA subjects (13.0±5.2 mm Hg, p=0.004). Systolic BP variability declined after the index date in RA (p<0.001), but not in the non-RA cohort (p=0.73), adjusting for age, sex and calendar year of RA. During the mean follow-up of 7.1 years, 33 cardiovascular events and 57 deaths occurred in RA cohort. Visit-to-visit systolic BP variability was associated with increased risk of cardiovascular events (hazard ratio [HR] per 1 mm Hg increase in BP variability 1.12, 95% confidence interval [CI] 1.01-1.25); diastolic BP variability was associated with all-cause mortality in RA (HR 1.14, 95%CI 1.03-1.27), adjusting for systolic and diastolic BP, body mass index, smoking, diabetes, dyslipidemia, use of antihypertensives. Conclusion Patients with RA had higher visit-to-visit systolic BP variability vs non-RA subjects. There was a significant decline in systolic BP variability after RA incidence. Higher visit-to-visit BP variability was associated with adverse cardiovascular outcomes and all-cause mortality in RA. PMID:24986852

  1. Multi-Wavelength Observations of Asteroid 2100 Ra-Shalom: Visible, Infrared, and Thermal Spectroscopy Results

    NASA Technical Reports Server (NTRS)

    Clark, Beth Ellen; Shepard, M.; Bus, S. J.; Vilas, F.; Rivkin, A. S.; Lim, L.; Lederer, S.; Jarvis, K.; Shah, S.; McConnochie, T.

    2004-01-01

    The August 2003 apparition of asteroid 2100 Ra-Shalom brought together a collaboration of observers with the goal of obtaining rotationally resolved multiwavelength spectra at each of 5 facilities: infrared spectra at the NASA Infrared Telescope Facility (Clark and Shepard), radar images at Arecibo (Shepard and Clark), thermal infrared spectra at Palomar (Lim, McConnochie and Bell), visible spectra at McDonald Observatory (Vilas, Lederer and Jarvis), and visible lightcurves at Ondrojev Observatory (Pravec). The radar data was to be used to develop a high spatial resolution physical model to be used in conjunction with spectral data to investigate compositional and textural properties on the near surface of Ra Shalom as a function of rotation phase. This was the first coordinated multi-wavelength investigation of any Aten asteroid. There are many reasons to study near-Earth asteroid (NEA) 2100 Ra-Shalom: 1) It has a controversial classification (is it a C- or K-type object)? 2) There would be interesting dynamical ramifications if Ra-Shalom is a K-type because most K-types come from the Eos family and there are no known dynamical pathways from Eos to the Aten population. 3) The best available spectra obtained previously may indicate a heterogeneous surface (most asteroids appear to be fairly homogeneous). 4) Ra-Shalom thermal observations obtained previously indicated a lack of regolith, minimizing the worry of space weathering effects in the spectra. 5) Radar observations obtained previously hinted at interesting surface structures. 6) Ra-Shalom is one of the largest Aten objects. And 7) Ra-Shalom is on a short list of proposed NEAs for spacecraft encounters and possible sample returns. Preliminary results from the visible, infrared, and thermal spectroscopy measurements will be presented here.

  2. Mutations in the interleukin receptor IL11RA cause autosomal recessive Crouzon-like craniosynostosis

    PubMed Central

    Keupp, Katharina; Li, Yun; Vargel, Ibrahim; Hoischen, Alexander; Richardson, Rebecca; Neveling, Kornelia; Alanay, Yasemin; Uz, Elif; Elcioğlu, Nursel; Rachwalski, Martin; Kamaci, Soner; Tunçbilek, Gökhan; Akin, Burcu; Grötzinger, Joachim; Konas, Ersoy; Mavili, Emin; Müller-Newen, Gerhard; Collmann, Hartmut; Roscioli, Tony; Buckley, Michael F; Yigit, Gökhan; Gilissen, Christian; Kress, Wolfram; Veltman, Joris; Hammerschmidt, Matthias; Akarsu, Nurten A; Wollnik, Bernd

    2013-01-01

    We have characterized a novel autosomal recessive Crouzon-like craniosynostosis syndrome in a 12-affected member family from Antakya, Turkey, the presenting features of which include: multiple suture synostosis, midface hypoplasia, variable degree of exophthalmos, relative prognathism, a beaked nose, and conductive hearing loss. Homozygosity mapping followed by targeted next-generation sequencing identified a c.479+6T>G mutation in the interleukin 11 receptor alpha gene (IL11RA) on chromosome 9p21. This donor splice-site mutation leads to a high percentage of aberrant IL11RA mRNA transcripts in an affected individual and altered mRNA splicing determined by in vitro exon trapping. An extended IL11RA mutation screen was performed in a cohort of 79 patients with an initial clinical diagnosis of Crouzon syndrome, pansynostosis, or unclassified syndromic craniosynostosis. We identified mutations segregating with the disease in five families: a German patient of Turkish origin and a Turkish family with three affected sibs all of whom were homozygous for the previously identified IL11RA c.479+6T>G mutation; a family with pansynostosis with compound heterozygous missense mutations, p.Pro200Thr and p.Arg237Pro; and two further Turkish families with Crouzon-like syndrome carrying the homozygous nonsense mutations p.Tyr232* and p.Arg292*. Using transient coexpression in HEK293T and COS7 cells, we demonstrated dramatically reduced IL11-mediated STAT3 phosphorylation for all mutations. Immunofluorescence analysis of mouse Il11ra demonstrated specific protein expression in cranial mesenchyme which was localized around the coronal suture tips and in the lambdoidal suture. In situ hybridization analysis of adult zebrafish also detected zfil11ra expression in the coronal suture between the overlapping frontal and parietal plates. This study demonstrates that mutations in the IL11RA gene cause an autosomal recessive Crouzon-like craniosynostosis. PMID:24498618

  3. Sac phosphatase domain proteins.

    PubMed Central

    Hughes, W E; Cooke, F T; Parker, P J

    2000-01-01

    Advances in our understanding of the roles of phosphatidylinositol phosphates in controlling cellular functions such as endocytosis, exocytosis and the actin cytoskeleton have included new insights into the phosphatases that are responsible for the interconversion of these lipids. One of these is an entirely novel class of phosphatase domain found in a number of well characterized proteins. Proteins containing this Sac phosphatase domain include the yeast Saccharomyces cerevisiae proteins Sac1p and Fig4p. The Sac phosphatase domain is also found within the mammalian phosphoinositide 5-phosphatase synaptojanin and the yeast synaptojanin homologues Inp51p, Inp52p and Inp53p. These proteins therefore contain both Sac phosphatase and 5-phosphatase domains. This review describes the Sac phosphatase domain-containing proteins and their actions, with particular reference to the genetic and biochemical insights provided by study of the yeast Saccharomyces cerevisiae. PMID:10947947

  4. Investigations on the activity concentrations of 238U, 226RA, 228RA, 210PB and 40K in Jordan phosphogypsum and fertilizers.

    PubMed

    Al-Jundi, J; Al-Ahmad, N; Shehadeh, H; Afaneh, F; Maghrabi, M; Gerstmann, U; Höllriegl, V; Oeh, U

    2008-01-01

    The activity concentrations of naturally occurring radionuclides ((238)U, (226)Ra, (228)Ra, (210)Pb and (40)K) in Jordanian phosphate ore, fertilizer material and phosphogypsum piles were investigated. The results show the partitioning of radionuclides in fertilizer products and phosphogypsum piles. The outcome of this study will enrich the Jordanian radiological map database, and will be useful for an estimation of the radiological impact of this industrial complex on the immediate environment. The activity concentration of (210)Pb was found to vary from 95 +/- 8 to 129 +/- 8 Bq kg(-1) with a mean value of 111 +/- 14 Bq kg(-1) in fertilizer samples, and from 364 +/- 8 to 428 +/- 10 Bq kg(-1) with a mean value of 391 +/- 30 Bq kg(-1) in phosphogypsum samples; while in phosphate wet rock samples, it was found to vary between 621 +/- 9 and 637 +/- 10 Bq kg(-1), with a mean value of 628 +/- 7 Bq kg(-1). The activity concentration of (226)Ra in fertilizer samples (between 31 +/- 4 and 42 +/- 5 Bq kg(-1) with a mean value of 37 +/- 6 Bq kg(-1)) was found to be much smaller than the activity concentration of (226)Ra in phosphogypsum samples (between 302 +/- 8 and 442 +/- 8 Bq kg(-1) with a mean value of 376 +/- 62 Bq kg(-1)). In contrast, the activity concentration of (238)U in fertilizer samples (between 1011 +/- 13 and 1061 +/- 14 Bq kg(-1) with a mean value of 1033 +/- 22 Bq kg(-1)) was found to be much higher than the activity concentration of (238)U in phosphogypsum samples (between 14 +/- 5 and 37 +/- 7 Bq kg(-1) with a mean value of 22 +/- 11 Bq kg(-1)). This indicates that (210)Pb and (226)Ra show similar behaviour, and are concentrated in phosphogypsum piles. In addition, both isotopes enhanced the activity concentration in phosphogypsum piles, while (238)U enhanced the activity concentration in the fertilizer. Due to the radioactivity released from the phosphate rock processing plants into the environment, the highest collective dose commitment for the lungs

  5. 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.

  6. Traditional and atypical presentations of anxiety in youth with autism spectrum disorder.

    PubMed

    Kerns, Connor Morrow; Kendall, Philip C; Berry, Leandra; Souders, Margaret C; Franklin, Martin E; Schultz, Robert T; Miller, Judith; Herrington, John

    2014-11-01

    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 explored. Fifty-nine participants (7-17 years, M(age) = 10.48 years; IQ > 60) with ASD and parents completed semi-structured interviews, self- and parent-reports. Seventeen percent of youth presented with traditional anxiety, 15 % with atypical anxiety, and 31 % with both. Language ability, anxious cognitions and hypersensitivity predicted traditional anxiety, whereas traditional anxiety and ASD symptoms predicted atypical anxiety. Findings suggest youth with ASD express anxiety in ways similar and dissimilar to DSM definitions. Similarities support the presence of comorbid anxiety disorders in ASD. Whether dissimilarities are unique to ASD requires further examination.

  7. Atypical major depressive episode as initial presentation of intracranial germinoma in a male adolescent

    PubMed Central

    Chen, Yi-Ting; Su, Kuan-Pin; Chang, Jane Pei-Chen

    2017-01-01

    A 17-year-old adolescent boy presented with atypical major depressive episode (MDE) without specific focal neurological signs for 6 months. He had a diagnosis of intra-cranial germinoma, and the atypical MDE symptoms subsided after the operation. However, he had a relapse of atypical MDE 7 months after the first surgery. His mood and binge eating symptoms subsided, but intractable body weight gain only partially improved after treatment. When encountering manifestations of depression with atypical features, especially with binge eating symptoms in male children and adolescents, with early onset age, no family history, and prolonged depressive episodes, clinicians should consider not only mood disorders including bipolar spectrum disorders but also organic brain lesions such as intracranial germinoma. PMID:28053535

  8. 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.

  9. Cross Domain Analogies for Learning Domain Theories

    DTIC Science & Technology

    2007-01-01

    Example Problem and Worked Solution All problems and worked solutions used in this work were taken from the same physics textbook ( Giancoli 1991...domain theory. We close with a discussion of related work and our plans for the future. Representations and Problem Solving Representing physics ...small compared to the 30,000+ concepts and 8,000+ predicates already defined in the KB. Thus, objects, relations, and events that appear in physics

  10. Structural and Functional Studies of the Ras-Associating and Pleckstrin Homology Domains of Grb10 and Grb14

    SciTech Connect

    Depetris, R.; Wu, J; Hubbard, S

    2009-01-01

    Growth factor receptor-binding proteins Grb7, Grb10 and Grb14 are adaptor proteins containing a Ras-associating (RA) domain, a pleckstrin-homology (PH) domain, a family-specific BPS (between PH and SH2) region and a C-terminal Src-homology-2 domain. Previous structural studies showed that the Grb14 BPS region binds as a pseudosubstrate inhibitor in the tyrosine kinase domain of the insulin receptor to suppress insulin signaling. Here we report the crystal structure of the RA and PH domains of Grb10 at 2.6-A resolution. The structure reveals that these two domains, along with the intervening linker, form an integrated, dimeric structural unit. Biochemical studies demonstrated that Grb14 binds to activated Ras, which may serve as a timing mechanism for downregulation of insulin signaling. Our results illuminate the membrane-recruitment mechanisms not only of Grb7, Grb10 and Grb14 but also of MIG-10, Rap1-interacting adaptor molecule, lamellipodin and Pico, proteins involved in actin-cytoskeleton rearrangement that share a structurally related RA-PH tandem unit.

  11. 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

  12. A Photometric Survey of Peculiar A-type

    NASA Astrophysics Data System (ADS)

    Kurtanidze, O. M.; Ogadze, G.

    The rapidly-oscillating Ap stars represent the only main-sequence stars, despite the Sun, which pulsate in high-overtone low degree p-moges with their axis aligned with oblique ones. We have undertook a long-term programme of high-speed photometric observations about two hundred Normal and Peculiar A-type stars with 125cm RC telescope equipped by Two-Star Photometer. It enable us to chop as frequently as need between objects, sky and dark due to effects of sky transparency and background variations are remouved and scintillation noise spectra are obtained. An attempt was made to include representative number of each spectral subtypes. The selected objects lie in the range of 8-10 magnitudes in the Jonhson wide-band B filter. At first stage it is planned to carry out a pilot survey with duration of 6-8 hours divided between two observing sessions. The objects with noticable oscillations will be studied photometrically as well as spectroscopically in detail. During the pleliminary observations one rapidly-oscillating Ap star HD231427 was revealed which should be considered as tentative.

  13. Atypical Time Course of Object Recognition in Autism Spectrum Disorder

    PubMed Central

    Caplette, Laurent; Wicker, Bruno; Gosselin, Frédéric

    2016-01-01

    In neurotypical observers, it is widely believed that the visual system samples the world in a coarse-to-fine fashion. Past studies on Autism Spectrum Disorder (ASD) have identified atypical responses to fine visual information but did not investigate the time course of the sampling of information at different levels of granularity (i.e. Spatial Frequencies, SF). Here, we examined this question during an object recognition task in ASD and neurotypical observers using a novel experimental paradigm. Our results confirm and characterize with unprecedented precision a coarse-to-fine sampling of SF information in neurotypical observers. In ASD observers, we discovered a different pattern of SF sampling across time: in the first 80 ms, high SFs lead ASD observers to a higher accuracy than neurotypical observers, and these SFs are sampled differently across time in the two subject groups. Our results might be related to the absence of a mandatory precedence of global information, and to top-down processing abnormalities in ASD. PMID:27752088

  14. Atypical Network Connectivity for Imitation in Autism Spectrum Disorder

    PubMed Central

    Shih, Patricia; Shen, Mark; Öttl, Birgit; Keehn, Brandon; Gaffrey, Michael S.; Müller, Ralph-Axel

    2010-01-01

    Imitation has been considered as one of the precursors for sociocommunicative development. Impairments of imitation in autism spectrum disorder (ASD) could be indicative of dysfunctional underlying neural processes. Neuroimaging studies have found reduced activation in areas associated with imitation, but a functional connectivity MRI network perspective of these regions in autism is unavailable. Functional and effective connectivity was examined in 14 male participants with ASD and 14 matched typically developing (TD) participants. We analyzed intrinsic, low-frequency blood oxygen level dependent (BOLD) fluctuations of three regions in literature found to be associated with imitation (inferior frontal gyrus [IFG], inferior parietal lobule [IPL], superior temporal sulcus [STS]). Direct group comparisons did not show significantly reduced functional connectivity within the imitation network in ASD. Conversely, we observed greater connectivity with frontal regions, particularly superior frontal and anterior cingulate gyri, in the ASD compared to TD group. Structural equation modeling of effective connectivity revealed a significantly reduced effect of IPL on IFG together with an increased influence of a region in dorsal prefrontal cortex (dPFC) on IFG in the ASD group. Our results suggest atypical connectivity of the imitation network with an enhanced role of dPFC, which may relate to behavioral impairments. PMID:20558187

  15. Novel Atypical Antipsychotics: Metabolism and Therapeutic Drug Monitoring (TDM).

    PubMed

    Mandrioli, Roberto; Protti, Michele; Mercolini, Laura

    2015-01-01

    Medicinal chemistry is continually developing and testing new drugs and drug candidates to satisfactorily address the needs of patients suffering from schizophrenia. In the last few years, some significant additions have been made to the list of widely available atypical antipsychotics. In particular, iloperidone, asenapine and lurasidone have been approved by the USA's Food and Drug Administration in 2009-10. In this paper, the most notable metabolic characteristics of these new drugs are addressed, with particular attention to their potential for pharmacokinetic interactions, and to the respective advantages and disadvantages in this regard. Moreover, current perspectives on the therapeutic drug monitoring (TDM) of the considered drugs are discussed. Since TDM is most valuable when it allows the personalisation and optimisation of therapeutic practices, it is even more interesting in the case of novel drugs, such as those discussed here, whose real impact in terms of side and toxic effects on very large populations is still unknown. Some analytical notes, related to TDM application, are included for each drug.

  16. Rest mutant zebrafish swim erratically and display atypical spatial preferences.

    PubMed

    Moravec, Cara E; Li, Edward; Maaswinkel, Hans; Kritzer, Mary F; Weng, Wei; Sirotkin, Howard I

    2015-05-01

    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.

  17. 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.

  18. Three boys and their stories: atypical eating and primitive relations.

    PubMed

    Escosteguy Carneiro, Maria Inês Neuenschwander

    2008-12-01

    Eating disorders of whatever category, especially the most severe ones such as anorexia or bulimia, are often considered typical of the female sex. While this is correct concerning these two disorders, I suggest that there are other eating disorders that are more frequent among boys, especially the ones concerning atypical relations to food, notwithstanding the evidence that the already very well-known syndromes of bulimia and anorexia are also gaining ground among males. However, similar, unspecified eating disorders do not seem to occur so often among girls. Clinical data from my private practice should be taken for granted considering these matters, since I do not yet have statistical data to back up my thesis. In examples from three clinical cases involving boys and one female vignette, I shall attempt to establish a relation between eating disorders and primitive relations with men and women. This relation goes well beyond the oft-cited 'contemporary' causes of eating disorders. In addition, I shall situate these differences among symptoms of both sexes and relate them to impasses in different stages of emotional development, as well as to the extent to which girls' identification with their mother's sex can influence their acquisition of eating disorders.

  19. 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

  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. Contraction stimulates muscle glucose uptake independent of atypical PKC.

    PubMed

    Yu, Haiyan; Fujii, Nobuharu L; Toyoda, Taro; An, Ding; Farese, Robert V; Leitges, Michael; Hirshman, Michael F; Mul, Joram D; Goodyear, Laurie J

    2015-11-01

    Exercise increases skeletal muscle glucose uptake, but the underlying mechanisms are only partially understood. The atypical protein kinase C (PKC) isoforms λ and ζ (PKC-λ/ζ) have been shown to be necessary for insulin-, AICAR-, and metformin-stimulated glucose uptake in skeletal muscle, but not for treadmill exercise-stimulated muscle glucose uptake. To investigate if PKC-λ/ζ activity is required for contraction-stimulated muscle glucose uptake, we used mice with tibialis anterior muscle-specific overexpression of an empty vector (WT), wild-type PKC-ζ (PKC-ζ(WT)), or an enzymatically inactive T410A-PKC-ζ mutant (PKC-ζ(T410A)). We also studied skeletal muscle-specific PKC-λ knockout (MλKO) mice. Basal glucose uptake was similar between WT, PKC-ζ(WT), and PKC-ζ(T410A) tibialis anterior muscles. In contrast, in situ contraction-stimulated glucose uptake was increased in PKC-ζ(T410A) tibialis anterior muscles compared to WT or PKC-ζ(WT) tibialis anterior muscles. Furthermore, in vitro contraction-stimulated glucose uptake was greater in soleus muscles of MλKO mice than WT controls. Thus, loss of PKC-λ/ζ activity increases contraction-stimulated muscle glucose uptake. These data clearly demonstrate that PKC-λζ activity is not necessary for contraction-stimulated glucose uptake.

  2. New functional aspects of the atypical protein tyrosine phosphatase VHZ.

    PubMed

    Kuznetsov, Vyacheslav I; Hengge, Alvan C

    2013-11-12

    LDP3 (VHZ) is the smallest classical protein tyrosine phosphatase (PTP) known to date and was originally misclassified as an atypical dual-specificity phosphatase. Kinetic isotope effects with steady-state and pre-steady-state kinetics of VHZ and mutants with p-nitrophenol phosphate 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 of 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.

  3. Atypical neural synchronization to speech envelope modulations in dyslexia.

    PubMed

    De Vos, Astrid; Vanvooren, Sophie; Vanderauwera, Jolijn; Ghesquière, Pol; Wouters, Jan

    2017-01-01

    A fundamental deficit in the synchronization of neural oscillations to temporal information in speech could underlie phonological processing problems in dyslexia. In this study, the hypothesis of a neural synchronization impairment is investigated more specifically as a function of different neural oscillatory bands and temporal information rates in speech. Auditory steady-state responses to 4, 10, 20 and 40Hz modulations were recorded in normal reading and dyslexic adolescents to measure neural synchronization of theta, alpha, beta and low-gamma oscillations to syllabic and phonemic rate information. In comparison to normal readers, dyslexic readers showed reduced non-synchronized theta activity, reduced synchronized alpha activity and enhanced synchronized beta activity. Positive correlations between alpha synchronization and phonological skills were found in normal readers, but were absent in dyslexic readers. In contrast, dyslexic readers exhibited positive correlations between beta synchronization and phonological skills. Together, these results suggest that auditory neural synchronization of alpha and beta oscillations is atypical in dyslexia, indicating deviant neural processing of both syllabic and phonemic rate information. Impaired synchronization of alpha oscillations in particular demonstrated to be the most prominent neural anomaly possibly hampering speech and phonological processing in dyslexic readers.

  4. Atypical network connectivity for imitation in autism spectrum disorder.

    PubMed

    Shih, Patricia; Shen, Mark; Ottl, Birgit; Keehn, Brandon; Gaffrey, Michael S; Müller, Ralph-Axel

    2010-08-01

    Imitation has been considered as one of the precursors for sociocommunicative development. Impairments of imitation in autism spectrum disorder (ASD) could be indicative of dysfunctional underlying neural processes. Neuroimaging studies have found reduced activation in areas associated with imitation, but a functional connectivity MRI network perspective of these regions in autism is unavailable. Functional and effective connectivity was examined in 14 male participants with ASD and 14 matched typically developing (TD) participants. We analyzed intrinsic, low-frequency blood oxygen level dependent (BOLD) fluctuations of three regions in literature found to be associated with imitation (inferior frontal gyrus [IFG], inferior parietal lobule [IPL], superior temporal sulcus [STS]). Direct group comparisons did not show significantly reduced functional connectivity within the imitation network in ASD. Conversely, we observed greater connectivity with frontal regions, particularly superior frontal and anterior cingulate gyri, in the ASD compared to TD group. Structural equation modeling of effective connectivity revealed a significantly reduced effect of IPL on IFG together with an increased influence of a region in dorsal prefrontal cortex (dPFC) on IFG in the ASD group. Our results suggest atypical connectivity of the imitation network with an enhanced role of dPFC, which may relate to behavioral impairments.

  5. RNA-Binding Proteins in Trichomonas vaginalis: Atypical Multifunctional Proteins.

    PubMed

    Figueroa-Angulo, Elisa E; Calla-Choque, Jaeson S; Mancilla-Olea, Maria Inocente; Arroyo, Rossana

    2015-11-26

    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.

  6. MITF accurately highlights epidermal melanocytes in atypical intraepidermal melanocytic proliferations.

    PubMed

    Nybakken, Grant E; Sargen, Michael; Abraham, Ronnie; Zhang, Paul J; Ming, Michael; Xu, Xiaowei

    2013-02-01

    Atypical intraepidermal melanocytic proliferations (AIMP) have random cytologic atypia and other histologic features that are concerning for malignancy and often require immunohistochemistry to differentiate from melanoma in situ. Immunostaining with S100, Melan-A, and microphthalmia-associated transcription factor (MITF) was performed for 49 morphologically well-characterized AIMP lesions. The percentage of cells in the basal layer of the epidermis that were identified as melanocytes by immunohistochemistry was compared with the percentage observed by morphology on hematoxylin and eosin staining, which is the gold standard stain for identifying cytologic atypia within an AIMP. Melan-A estimated the highest percentage of melanocytes and S100 the fewest in 47 of the 49 lesions examined. The estimated percentage of melanocytes was 23.3% (95% confidence interval: 18.6-28.1; P < 0.001) higher for Melan-A compared with hematoxylin and eosin staining. Melanocyte estimates were similar for hematoxylin and eosin and MITF (P = 0.15) although S100 estimated 21.8% (95% confidence interval: -27.2 to -16.4; P < 0.001) fewer melanocytes than hematoxylin and eosin. Melan-A staining produces higher estimates of epidermal melanocytes than S100 and MITF, which may increase the likelihood of diagnosing melanoma in situ. In contrast, melanoma in situ may be underdiagnosed with the use of S100, which results in lower estimates of melanocytes than the other 2 immunostains. Therefore, the best immunohistochemical marker for epidermal melanocytes is MITF.

  7. 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.

  8. 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.

  9. VizieR Online Data Catalog: VAST Survey. A-type stars multiplicity (De Rosa+, 2014)

    NASA Astrophysics Data System (ADS)

    De Rosa, R. J.; Patience, J.; Wilson, P. A.; Schneider, A.; Wiktorowicz, S. J.; Vigan, A.; Marois, C.; Song, I.; Macintosh, B.; Graham, J. R.; Doyon, R.; Bessell, M. S.; Thomas, S.; Lai, O.

    2014-11-01

    To measure the frequency of stellar binary companions, and the distribution of their separations and mass ratios, we have obtained observations of a sample of 435 nearby A-type stars. The sample is composed of two overlapping sets of A-type stars within 75pc: a 363 star sample observed with AO instrumentation and a 228 star sample investigated with astrometry obtained from all-sky photographic surveys, with an overlap of 156 stars. (5 data files).

  10. Computed Tomography (CT) Findings of a Diagnostic Dilemma: Atypically Located Acute Appendicitis

    PubMed Central

    Evrimler, Sehnaz; Okumuser, Irfan; Unal, Nermin

    2016-01-01

    Summary Background Acute appendicitis is an emergent surgically treated disease generally represented by right lower abdominal pain. The most common location of the appendix is descending intraperitoneal. However, it can also show atypical locations such as inguinal canal, femoral canal, subhepatic, retrocecal, intraperitoneal abdominal midline and left side in situs inversus or intestinal malrotation patients. Atypical location can lead to atypical clinical presentations. Ultrasonography is the first choice modality for imaging. However, it can be insufficient for demonstration of the appendix. Therefore, computed tomography (CT) is needed for further examination. We aim to review the CT findings of atypically located acute appendicitis with cases and remind the clinicians and radiologists the importance of the prompt diagnosis. Case Report We presented five atypically-located appendix cases, including four with acute appendicitis that presented to our emergency department with acute abdominal pain. Two of the acute appendicitis cases had normal, the other two had elevated white blood cell count, but all of them had elevated CRP. Ultrasonography imaging was performed as a first-line imaging modality. Because of the inconclusive results of both clinical-laboratory findings and ultrasonography, CT imaging was performed. Abdominal CT demonstrated all of the atypically localised appendices successfully, which were left-sided in a malrotated patient, retrocecal, subhepatic, retrocecal ascending, intraperitoneal abdominal midline localised. Conclusıons Atypically located acute appendicitis can show atypical presentation and result in misdiagnosis. If ultrasonograpgy is inconclusive, we suggest abdominal CT in such confusing, complicated cases, because misdiagnosis or delay in the right diagnosis can result in complications and increased morbidity and mortality rates. PMID:28058072

  11. Dermatological clues to the diagnosis of atypical complete DiGeorge syndrome.

    PubMed

    Seminario-Vidal, Lucia; Kole, Lauren; Knapp, Charles; Fort, Prem; Kankirawatana, Suthida; Atkinson, T Prescott; McKay, Kristopher M; Theos, Amy

    2016-11-15

    Atypical complete DiGeorge syndrome (DGS) is an extremely rare congenital disease characterized by an eczematous dermatitis, lymphadenopathy, and an oligoclonal T-cell proliferation. Because its initial presentation may be confused with other types of eczematous dermatitis, diagnosis and treatment are usually delayed. We describe herein a case of an infant with atypical complete DGS to draw attention to the clinical and histopathological findings that lead us to the diagnosis.

  12. Atypical mole syndrome and dysplastic nevi: identification of populations at risk for developing melanoma - review article

    PubMed Central

    Silva, Juliana Hypólito; de Sá, Bianca Costa Soares; de Ávila, Alexandre Leon Ribeiro; Landman, Gilles; Neto, João Pedreira Duprat

    2011-01-01

    Atypical Mole Syndrome is the most important phenotypic risk factor for developing cutaneous melanoma, a malignancy that accounts for about 80% of deaths from skin cancer. Because the diagnosis of melanoma at an early stage is of great prognostic relevance, the identification of Atypical Mole Syndrome carriers is essential, as well as the creation of recommended preventative measures that must be taken by these patients. PMID:21552679

  13. Transfer coefficient of 226Ra from vegetation to meadow voles, Microtus pennsylvanicus, on U mill tailings

    SciTech Connect

    Cloutier, N.R.; Clulow, F.V.; Lim, T.P.; Dave, N.K.

    1986-06-01

    The 226Ra level in vegetation growing on U mine tailings in Elliot Lake, Ontario, Canada, was 211 + 22 mBq g-1 (dry weight) compared to less than 7 mBq g-1 (dry weight) in material from a control site. Skeletons of meadow voles (Microtus pennsylvanicus) established on the tailings had concentrations of 226Ra of 6083 +/- 673 mBq per animal in winter; 7163 +/- 1077 mBq per animal in spring; 1506 +/- 625 mBq per animal in summer; and 703 +/- 59 mBq per animal in fall, compared to less than 7 mBq per animal in controls. The /sup 226/Ra transfer coefficient from vegetation to voles (defined as total millibecquerels of /sup 226/Ra in adult vole per total millibecquerels of 226Ra consumed by the vole in its lifetime) was calculated as 4.6 +/- 2.9 X 10(-2) in summer and 2.8 +/- 0.6 X 10(-2) in fall.

  14. [The effect of the mutant genes Ta, Ra and Sd on early embryogenesis in mice].

    PubMed

    Sakharova, N Iu; Malashenko, A M; Mezhevikina, L M; Lepikhov, K A; Fialkovskaia, L A

    1996-01-01

    We studied early embryonic mortality of mice from mutant stocks Tabby (Ta, X-chromosome) and RaSd (RaSd/++, chromosome 2) maintained in the heterozygous state in F1 CBA x C57B1/6 hybrid. Tabby and RaSd mice were reciprocally crossed with F1 mice and examined for the morphological status of embryos washed from the oviduct on the third day of pregnancy, when the stage of eight blastomeres is normally attained. Mortality was evaluated from the number of embryos which did not reach the expected stage by this time. The results have shown that 2-4 cell embryos, which have received gene Ta with the X-chromosome of the female parent, differed from embryos with F1 genotype at the same stage of development by their increased mortality rate, whereas among embryos obtained from RaSd, the mortality was mainly observed before cleavage. Death of embryos receiving the mutant gene from hemizygous Ta males or heterozygous RaSd/++ males was not significantly different from the mortality of embryos without these mutations.

  15. Comparative Analysis Of 226Ra Soil-To-Plant Transfer In Cabbage Grown In Various Regions

    NASA Astrophysics Data System (ADS)

    Madruga, M. J.; Carvalho, F. P.; Silva, L.; Gouveia, J.

    2008-08-01

    The transfer of 226Ra from soil to cabbage was compared amongst regions, namely the surroundings of Urgeiriça uranium milling tailings (GE), regions with past uranium mining activities (GN1), and regions with no uranium mining activities and no uranium deposits (GN2). Results show a slight increase of the concentration ratio values at low radium concentration in soils. Statistical analysis of the mean 226Ra activity concentrations in soil and cabbage for the three regions was carried out. The comparison of 226Ra activity concentrations in soils indicated no difference (p>0.05), between GE and GN2 and significant differences (p<0.05) between GE and GN1 and between GN1 and GN2. Similar statistical results were obtained for 226Ra activity concentrations in cabbage from the same regions. It was concluded that radium Concentration Ratio (CR) for cabbage grown in the region of the main uranium milling site (GE) is of the same order of magnitude of CR in cabagge grown in background regions (GN2). However, 226Ra CR was higher in cabagge from the region with past uranium mining activities (GN1).

  16. Transfer coefficient of 226Ra from vegetation to meadow voles, Microtus pennsylvanicus, on U mill tailings.

    PubMed

    Cloutier, N R; Clulow, F V; Lim, T P; Davé, N K

    1986-06-01

    The 226Ra level in vegetation growing on U mine tailings in Elliot Lake, Ontario, Canada, was 211 + 22 mBq g-1 (dry weight) compared to less than 7 mBq g-1 (dry weight) in material from a control site. Skeletons of meadow voles (Microtus pennsylvanicus) established on the tailings had concentrations of 226Ra of 6,083 +/- 673 mBq per animal in winter; 7,163 +/- 1,077 mBq per animal in spring; 1,506 +/- 625 mBq per animal in summer; and 703 +/- 59 mBq per animal in fall, compared to less than 7 mBq per animal in controls. The 226Ra transfer coefficient from vegetation to voles (defined as total millibecquerels of 226Ra in adult vole per total millibecquerels of 226Ra consumed by the vole in its lifetime) was calculated as 4.6 +/- 2.9 X 10(-2) in summer and 2.8 +/- 0.6 X 10(-2) in fall.

  17. (210)Pb-(226)Ra disequilibria in young gas-laden magmas.

    PubMed

    Reagan, Mark; Turner, Simon; Handley, Heather; Turner, Michael; Beier, Christoph; Caulfield, John; Peate, David

    2017-03-24

    We present new (238)U-(230)Th-(226)Ra-(210)Pb and supporting data for young lavas from southwest Pacific island arcs, Eyjafjallajökull, Iceland, and Terceira, Azores. The arc lavas have significant (238)U and (226)Ra excesses, whereas those from the ocean islands have moderate (230)Th and (226)Ra excesses, reflecting mantle melting in the presence of a water-rich fluid in the former and mantle melting by decompression in the latter. Differentiation to erupted compositions in both settings appears to have taken no longer than a few millennia. Variations in the ((210)Pb/(226)Ra)0 values in all settings largely result from degassing processes rather than mineral-melt partitioning. Like most other ocean island basalts, the Terceira basalt has a (210)Pb deficit, which we attribute to ~8.5 years of steady (222)Rn loss to a CO2-rich volatile phase while it traversed the crust. Lavas erupted from water-laden magma systems, including those investigated here, commonly have near equilibrium ((210)Pb/(226)Ra)0 values. Maintaining these equilibrium values requires minimal persistent loss or accumulation of (222)Rn in a gas phase. We infer that degassing during decompression of water-saturated magmas either causes these magmas to crystallize and stall in reservoirs where they reside under conditions of near stasis, or to quickly rise towards the surface and erupt.

  18. 210Pb-226Ra disequilibria in young gas-laden magmas

    NASA Astrophysics Data System (ADS)

    Reagan, Mark; Turner, Simon; Handley, Heather; Turner, Michael; Beier, Christoph; Caulfield, John; Peate, David

    2017-03-01

    We present new 238U-230Th-226Ra-210Pb and supporting data for young lavas from southwest Pacific island arcs, Eyjafjallajökull, Iceland, and Terceira, Azores. The arc lavas have significant 238U and 226Ra excesses, whereas those from the ocean islands have moderate 230Th and 226Ra excesses, reflecting mantle melting in the presence of a water-rich fluid in the former and mantle melting by decompression in the latter. Differentiation to erupted compositions in both settings appears to have taken no longer than a few millennia. Variations in the (210Pb/226Ra)0 values in all settings largely result from degassing processes rather than mineral-melt partitioning. Like most other ocean island basalts, the Terceira basalt has a 210Pb deficit, which we attribute to ~8.5 years of steady 222Rn loss to a CO2-rich volatile phase while it traversed the crust. Lavas erupted from water-laden magma systems, including those investigated here, commonly have near equilibrium (210Pb/226Ra)0 values. Maintaining these equilibrium values requires minimal persistent loss or accumulation of 222Rn in a gas phase. We infer that degassing during decompression of water-saturated magmas either causes these magmas to crystallize and stall in reservoirs where they reside under conditions of near stasis, or to quickly rise towards the surface and erupt.

  19. RA-RAR-β counteracts myelin-dependent inhibition of neurite outgrowth via Lingo-1 repression.

    PubMed

    Puttagunta, Radhika; Schmandke, André; Floriddia, Elisa; Gaub, Perrine; Fomin, Natalie; Ghyselinck, Norbert B; Di Giovanni, Simone

    2011-06-27

    After an acute central nervous system injury, axonal regeneration is limited as the result of a lack of neuronal intrinsic competence and the presence of extrinsic inhibitory signals. The injury fragments the myelin neuronal insulating layer, releasing extrinsic inhibitory molecules to signal through the neuronal membrane-bound Nogo receptor (NgR) complex. In this paper, we show that a neuronal transcriptional pathway can interfere with extrinsic inhibitory myelin-dependent signaling, thereby promoting neurite outgrowth. Specifically, retinoic acid (RA), acting through the RA receptor β (RAR-β), inhibited myelin-activated NgR signaling through the transcriptional repression of the NgR complex member Lingo-1. We show that suppression of Lingo-1 was required for RA-RAR-β to counteract extrinsic inhibition of neurite outgrowth. Furthermore, we confirm in vivo that RA treatment after a dorsal column overhemisection injury inhibited Lingo-1 expression, specifically through RAR-β. Our findings identify a novel link between RA-RAR-β-dependent proaxonal outgrowth and inhibitory NgR complex-dependent signaling, potentially allowing for the development of molecular strategies to enhance axonal regeneration after a central nervous system injury.

  20. SA1 and RA afferent responses to static and vibrating gratings.

    PubMed

    Bensmaïa, S J; Craig, J C; Yoshioka, T; Johnson, K O

    2006-03-01

    SA1 and RA afferent fibers differ both in their ability to convey information about the fine spatial structure of tactile stimuli and in their frequency sensitivity profiles. In the present study, we investigated the extent to which the spatial resolution of the signal conveyed by SA1 and RA fibers depends on the temporal properties of the stimulus. To that end, we recorded the responses evoked in SA1 and RA fibers of macaques by static and vibrating gratings that varied in spatial period, vibratory frequency, and amplitude. Gratings were oriented either parallel to the long axis of the finger (vertical) or perpendicular to it (horizontal). We examined the degree to which afferent responses were dependent on the spatial period, vibratory frequency, amplitude, and orientation of the gratings. We found that the spatial modulation of the afferent responses increased as the spatial period of the gratings increased; the spatial modulation was the same for static and vibrating gratings, despite large differences in evoked spike rates; the spatial modulation in SA1 responses was independent of stimulus amplitude over the range of amplitudes tested, whereas RA modulation decreased slightly as the stimulus amplitude increased; vertical gratings evoked stronger and more highly modulated responses than horizontal gratings; the modulation in SA1 responses was higher than that in RA responses at all frequencies and amplitudes. The behavioral consequences of these neurophysiological findings are examined in a companion paper.

  1. Epithelial IL-22RA1-mediated fucosylation promotes intestinal colonization resistance to an opportunistic pathogen.

    PubMed

    Pham, Tu Anh N; Clare, Simon; Goulding, David; Arasteh, Julia M; Stares, Mark D; Browne, Hilary P; Keane, Jacqueline A; Page, Andrew J; Kumasaka, Natsuhiko; Kane, Leanne; Mottram, Lynda; Harcourt, Katherine; Hale, Christine; Arends, Mark J; Gaffney, Daniel J; Dougan, Gordon; Lawley, Trevor D

    2014-10-08

    Our intestinal microbiota harbors a diverse microbial community, often containing opportunistic bacteria with virulence potential. However, mutualistic host-microbial interactions prevent disease by opportunistic pathogens through poorly understood mechanisms. We show that the epithelial interleukin-22 receptor IL-22RA1 protects against lethal Citrobacter rodentium infection and chemical-induced colitis by promoting colonization resistance against an intestinal opportunistic bacterium, Enterococcus faecalis. Susceptibility of Il22ra1(-/-) mice to C. rodentium was associated with preferential expansion and epithelial translocation of pathogenic E. faecalis during severe microbial dysbiosis and was ameloriated with antibiotics active against E. faecalis. RNA sequencing analyses of primary colonic organoids showed that IL-22RA1 signaling promotes intestinal fucosylation via induction of the fucosyltransferase Fut2. Additionally, administration of fucosylated oligosaccharides to C. rodentium-challenged Il22ra1(-/-) mice attenuated infection and promoted E. faecalis colonization resistance by restoring the diversity of anaerobic commensal symbionts. These results support a model whereby IL-22RA1 enhances host-microbiota mutualism to limit detrimental overcolonization by opportunistic pathogens.

  2. The IL-17A/IL-17RA axis in pulmonary defence and immunopathology.

    PubMed

    Lorè, Nicola Ivan; Bragonzi, Alessandra; Cigana, Cristina

    2016-08-01

    The interleukin (IL)-17A/IL-17 receptor A (IL-17RA) axis is emerging as a key player in host defence. Several studies have demonstrated that IL-17A-mediated responses play a critical role in both acute and chronic inflammation induced by infectious agents, environmental stimuli and genetic diseases in the airways. In this regard, it is becoming evident that IL-17A/IL-17RA signalling may have a protective and beneficial impact on health, but that it can also result in detrimental outcomes. On one hand, the IL-17A/IL-17RA axis can contribute to the elimination of noxious stimuli and to the resolution of acute inflammatory processes; on the other hand, it can exacerbate immunopathological responses, contributing to the development and progression of chronic respiratory illnesses. In addition, cellular and molecular signatures underlying IL-17A/IL-17RA signalling have been increasingly identified, although further studies are needed to clarify such complex responses. Here, we discuss the latest discoveries on the role of the IL-17A/IL-17RA axis in driving host pulmonary defence and immunopathology.

  3. PADI4 haplotypes in association with RA Mexican patients, a new prospect for antigen modulation.

    PubMed

    Zavala-Cerna, Maria Guadalupe; Gonzalez-Montoya, Norma Guadalupe; Nava, Arnulfo; Gamez-Nava, Jorge I; Moran-Moguel, Maria Cristina; Rosales-Gomez, Roberto Carlos; Gutierrez-Rubio, Susan Andrea; Sanchez-Corona, Jose; Gonzalez-Lopez, Laura; Davalos-Rodriguez, Ingrid Patricia; Salazar-Paramo, Mario

    2013-01-01

    Peptidyl arginine deiminase IV (PAD 4) is the responsible enzyme for a posttranslational modification called citrullination, originating the antigenic determinant recognized by anti-cyclic citrullinated peptide antibodies (ACPA). Four SNPs (single nucleotide polymorphisms) have been described in PADI4 gene to form a susceptibility haplotype for rheumatoid arthritis (RA); nevertheless, results in association studies appear contradictory in different populations. The aim of the study was to analyze if the presence of three SNPs in PADI4 gene susceptibility haplotype (GTG) is associated with ACPA positivity in patients with RA. This was a cross-sectional study that included 86 RA patients and 98 healthy controls. Polymorphisms PADI4_89, PADI4_90, and PADI4_92 in the PADI4 gene were genotyped. The susceptibility haplotype (GTG) was more frequent in RA patients; interestingly, we found a new haplotype associated with RA with a higher frequency (GTC). There were no associations between polymorphisms and high scores in Spanish HAQ-DI and DAS-28, but we did find an association between RARBIS index and PADI4_89, PADI4_90 polymorphisms. We could not confirm an association between susceptibility haplotype presence and ACPA positivity. Further evidence about proteomic expression of this gene will determine its participation in antigenic generation and autoimmunity.

  4. PADI4 Haplotypes in Association with RA Mexican Patients, a New Prospect for Antigen Modulation

    PubMed Central

    Gonzalez-Montoya, Norma Guadalupe; Gamez-Nava, Jorge I.; Moran-Moguel, Maria Cristina; Rosales-Gomez, Roberto Carlos; Gutierrez-Rubio, Susan Andrea; Sanchez-Corona, Jose; Davalos-Rodriguez, Ingrid Patricia; Salazar-Paramo, Mario

    2013-01-01

    Peptidyl arginine deiminase IV (PAD 4) is the responsible enzyme for a posttranslational modification called citrullination, originating the antigenic determinant recognized by anti-cyclic citrullinated peptide antibodies (ACPA). Four SNPs (single nucleotide polymorphisms) have been described in PADI4 gene to form a susceptibility haplotype for rheumatoid arthritis (RA); nevertheless, results in association studies appear contradictory in different populations. The aim of the study was to analyze if the presence of three SNPs in PADI4 gene susceptibility haplotype (GTG) is associated with ACPA positivity in patients with RA. This was a cross-sectional study that included 86 RA patients and 98 healthy controls. Polymorphisms PADI4_89, PADI4_90, and PADI4_92 in the PADI4 gene were genotyped. The susceptibility haplotype (GTG) was more frequent in RA patients; interestingly, we found a new haplotype associated with RA with a higher frequency (GTC). There were no associations between polymorphisms and high scores in Spanish HAQ-DI and DAS-28, but we did find an association between RARBIS index and PADI4_89, PADI4_90 polymorphisms. We could not confirm an association between susceptibility haplotype presence and ACPA positivity. Further evidence about proteomic expression of this gene will determine its participation in antigenic generation and autoimmunity. PMID:24454473

  5. 210Pb-226Ra disequilibria in young gas-laden magmas

    PubMed Central

    Reagan, Mark; Turner, Simon; Handley, Heather; Turner, Michael; Beier, Christoph; Caulfield, John; Peate, David

    2017-01-01

    We present new 238U-230Th-226Ra-210Pb and supporting data for young lavas from southwest Pacific island arcs, Eyjafjallajökull, Iceland, and Terceira, Azores. The arc lavas have significant 238U and 226Ra excesses, whereas those from the ocean islands have moderate 230Th and 226Ra excesses, reflecting mantle melting in the presence of a water-rich fluid in the former and mantle melting by decompression in the latter. Differentiation to erupted compositions in both settings appears to have taken no longer than a few millennia. Variations in the (210Pb/226Ra)0 values in all settings largely result from degassing processes rather than mineral-melt partitioning. Like most other ocean island basalts, the Terceira basalt has a 210Pb deficit, which we attribute to ~8.5 years of steady 222Rn loss to a CO2-rich volatile phase while it traversed the crust. Lavas erupted from water-laden magma systems, including those investigated here, commonly have near equilibrium (210Pb/226Ra)0 values. Maintaining these equilibrium values requires minimal persistent loss or accumulation of 222Rn in a gas phase. We infer that degassing during decompression of water-saturated magmas either causes these magmas to crystallize and stall in reservoirs where they reside under conditions of near stasis, or to quickly rise towards the surface and erupt. PMID:28338093

  6. Possible cluster preformation in the 14C decay of 223Ra

    NASA Astrophysics Data System (ADS)

    Hussonnois, M.; Le Du, J. F.; Brillard, L.; Ardisson, G.

    1990-08-01

    A recent experiment on the 14C fine structure has shown that the branching ratio to 209Pb ground state is quite different from the one predicted by the various models of cluster emission. Assuming that the 14C is preformed in the 223Ra nucleus, we have calculated the hindrance factor F14C of the three 14C branches to 209Pb states with a barrier penetrability approximated by a square-well Coulomb potential. The F14C=3.9 and 4.6 values found for the 14C transitions to the two excited states at 779 keV (Iπ=11/2+) and 1423 keV (Iπ=15/2-), respectively, reveal that the cluster formation amplitudes in both transitions are close to those of the 222Ra and 224Ra 14C decays. Such values could be the result of ||nlj> shell-model components common to both the wave functions of the octupole deformed 223Ra nucleus and of the two first excited states of 209Pb spherical nucleus. The high hindrance factor F14C=583 of the 14C branch to the 209Pb ground state (Iπ=9/2+) could reveal the absence of ||1g9/2> shell-model component in the 223Ra wave function.

  7. Epithelial IL-22RA1-Mediated Fucosylation Promotes Intestinal Colonization Resistance to an Opportunistic Pathogen

    PubMed Central

    Pham, Tu Anh N.; Clare, Simon; Goulding, David; Arasteh, Julia M.; Stares, Mark D.; Browne, Hilary P.; Keane, Jacqueline A.; Page, Andrew J.; Kumasaka, Natsuhiko; Kane, Leanne; Mottram, Lynda; Harcourt, Katherine; Hale, Christine; Arends, Mark J.; Gaffney, Daniel J.; Dougan, Gordon; Lawley, Trevor D.

    2014-01-01

    Summary Our intestinal microbiota harbors a diverse microbial community, often containing opportunistic bacteria with virulence potential. However, mutualistic host-microbial interactions prevent disease by opportunistic pathogens through poorly understood mechanisms. We show that the epithelial interleukin-22 receptor IL-22RA1 protects against lethal Citrobacter rodentium infection and chemical-induced colitis by promoting colonization resistance against an intestinal opportunistic bacterium, Enterococcus faecalis. Susceptibility of Il22ra1−/− mice to C. rodentium was associated with preferential expansion and epithelial translocation of pathogenic E. faecalis during severe microbial dysbiosis and was ameloriated with antibiotics active against E. faecalis. RNA sequencing analyses of primary colonic organoids showed that IL-22RA1 signaling promotes intestinal fucosylation via induction of the fucosyltransferase Fut2. Additionally, administration of fucosylated oligosaccharides to C. rodentium-challenged Il22ra1−/− mice attenuated infection and promoted E. faecalis colonization resistance by restoring the diversity of anaerobic commensal symbionts. These results support a model whereby IL-22RA1 enhances host-microbiota mutualism to limit detrimental overcolonization by opportunistic pathogens. PMID:25263220

  8. The question of autism in an atypical infant.

    PubMed

    Zeanah, C H; Davis, S; Silverman, M

    1988-01-01

    The evaluation and brief treatment of a less-than-a-year-old girl whose parents were concerned she was autistic is described. We review what is known about the initial symptoms of autism and consider how early it is feasible to make a diagnosis. We outline the domains of cognitive, language, and social development that ought to be monitored given concern about autism and explain how we evaluated these domains in our home-based intervention. Finally, we consider possible explanations for this infant's recovery from a serious early disturbance in social relatedness.

  9. How Category Structure Influences the Perception of Object Similarity: The Atypicality Bias

    PubMed Central

    Tanaka, James William; Kantner, Justin; Bartlett, Marni

    2011-01-01

    Why do some faces appear more similar than others? Beyond structural factors, we speculate that similarity is governed by the organization of faces located in a multi-dimensional face space. To test this hypothesis, we morphed a typical face with an atypical face. If similarity judgments are guided purely by their physical properties, the morph should be perceived to be equally similar to its typical parent as its atypical parent. However, contrary to the structural prediction, our results showed that the morph face was perceived to be more similar to the atypical face than the typical face. Our empirical studies show that the atypicality bias is not limited to faces, but extends to other object categories (birds) whose members share common shape properties. We also demonstrate atypicality bias is malleable and can change subject to category learning and experience. Collectively, the empirical evidence indicates that perceptions of face and object similarity are affected by the distribution of stimuli in a face or object space. In this framework, atypical stimuli are located in a sparser region of the space where there is less competition for recognition and therefore, these representations capture a broader range of inputs. In contrast, typical stimuli are located in a denser region of category space where there is increased competition for recognition and hence, these representation draw a more restricted range of face inputs. These results suggest that the perceived likeness of an object is influenced by the organization of surrounding exemplars in the category space. PMID:22685441

  10. Can Baropodometric Analysis be a Useful Tool in the Early Diagnosis of Atypical Parkinsonism? Preliminary Findings

    PubMed Central

    Furnari, Anna; Imbesi, Donatella; La Fauci Belponer, Francesca; Militi, David; Gervasi, Giuseppe; Pastura, Concetta; Bramanti, Placido

    2014-01-01

    Objective: The differential diagnosis between atypical parkinsonism and Parkinson’s disease is difficult, especially in the early stage. Severe postural instability, falls, and complex gait impairments are usually confined to the later stage of Parkinson’s disease, while atypical parkinsonism patients may present a severe postural instability with consequent falls in the earlier stages. Methods: We retrospectively studied 20 subjects with parkinsonism using clinical and baropodometric tools to give quantitative and objective data on the postural, balance, and gait disturbances. Results: The statistical analysis between atypical parkinsonism and Parkinson’s disease patients showed a significant difference in the frequency of long lead time parameter, foot area, foot load and speed, and, in particular, atypical parkinsonism patients presented a prevalent long lead time impairment (8/8 patients) when compared with Parkinson’s disease patients. Discussion: Beside significant differences in the clinical features between the Parkinson’s disease and atypical parkinsonism, our study showed that baropodometric investigation may a valuable tool for the definition of postural and motor extrapyramidal abnormalities, permitting an earlier differentiation between atypical parkinsonism and Parkinson’s disease. PMID:24653938

  11. Development of duplex PCR assay for detection and differentiation of typical and atypical Melissococcus plutonius strains.

    PubMed

    Arai, Rie; Miyoshi-Akiyama, Tohru; Okumura, Kayo; Morinaga, Yuiko; Wu, Meihua; Sugimura, Yuya; Yoshiyama, Mikio; Okura, Masatoshi; Kirikae, Teruo; Takamatsu, Daisuke

    2014-04-01

    Melissococcus plutonius is the causative agent of an important honeybee disease, European foulbrood (EFB). In addition to M. plutonius strains with typical characteristics (typical M. plutonius), we recently reported the presence of atypical M. plutonius, which are phenotypically and genetically distinguished from typical M. plutonius. Because typical and atypical M. plutonius may have different pathogenic mechanisms, differentiation of these two types is very important for diagnosis and more effective control of EFB. In this study, therefore, a duplex PCR assay was developed to detect and differentiate typical and atypical M. plutonius rapidly and easily. On the basis of the results of comparative genomic analyses, we selected Na(+)/H(+) antiporter gene and Fur family transcriptional regulator gene as targets for detection of typical and atypical strains, respectively, by PCR. Under optimized conditions, the duplex PCR system using the designed primers successfully detected and differentiated all typical and atypical M. plutonius strain/isolates tested, while no product was generated from any other bacterial strains/isolates used in this study, including those isolated from healthy honeybee larval guts. Detection limits of the PCR were 50 copies of chromosome/reaction for both types, and it could detect typical and atypical M. plutonius directly from diseased honeybee larvae. Moreover, the duplex PCR diagnosed mixed infections with both M. plutonius types more precisely than standard culture methods. These results indicate that the duplex PCR assay developed in this study is extremely useful for precise diagnosis and epidemiological study of EFB.

  12. Development of Duplex PCR Assay for Detection and Differentiation of Typical and Atypical Melissococcus plutonius strains

    PubMed Central

    ARAI, Rie; MIYOSHI-AKIYAMA, Tohru; OKUMURA, Kayo; MORINAGA, Yuiko; WU, Meihua; SUGIMURA, Yuya; YOSHIYAMA, Mikio; OKURA, Masatoshi; KIRIKAE, Teruo; TAKAMATSU, Daisuke

    2013-01-01

    ABSTRACT Melissococcus plutonius is the causative agent of an important honeybee disease, European foulbrood (EFB). In addition to M. plutonius strains with typical characteristics (typical M. plutonius), we recently reported the presence of atypical M. plutonius, which are phenotypically and genetically distinguished from typical M. plutonius. Because typical and atypical M. plutonius may have different pathogenic mechanisms, differentiation of these two types is very important for diagnosis and more effective control of EFB. In this study, therefore, a duplex PCR assay was developed to detect and differentiate typical and atypical M. plutonius rapidly and easily. On the basis of the results of comparative genomic analyses, we selected Na+/H+ antiporter gene and Fur family transcriptional regulator gene as targets for detection of typical and atypical strains, respectively, by PCR. Under optimized conditions, the duplex PCR system using the designed primers successfully detected and differentiated all typical and atypical M. plutonius strain/isolates tested, while no product was generated from any other bacterial strains/isolates used in this study, including those isolated from healthy honeybee larval guts. Detection limits of the PCR were 50 copies of chromosome/reaction for both types, and it could detect typical and atypical M. plutonius directly from diseased honeybee larvae. Moreover, the duplex PCR diagnosed mixed infections with both M. plutonius types more precisely than standard culture methods. These results indicate that the duplex PCR assay developed in this study is extremely useful for precise diagnosis and epidemiological study of EFB. PMID:24334815

  13. Newer atypical antipsychotic medication in comparison to clozapine: a systematic review of randomized trials.

    PubMed

    Tuunainen, Arja; Wahlbeck, Kristian; Gilbody, Simon

    2002-07-01

    The aim of this study was to evaluate the effectiveness of newer atypical antipsychotic drugs in comparison to clozapine for schizophrenia. Publications in all languages were searched from all relevant databases and all randomized controlled trials comparing clozapine with newer atypical drugs were included. The review and meta-analysis includes eight studies, most of them short in duration. Newer atypical drugs were broadly similar to clozapine when improvement was measured using a psychosis symptom rating scale or a global index. There was a trend for clozapine to be more effective than the others for positive symptoms, and less effective for the negative symptoms. The adverse effect profile of clozapine and newer atypical drugs was dissimilar: while clozapine produced more fatigue, hypersalivation, and orthostatic dizziness, new atypical drugs, with the exception of olanzapine, produced more extrapyramidal symptoms. As these results were obtained from few studies and a relatively small amount of patients, the equal effectiveness and tolerability of new atypical drugs in comparison with clozapine is not yet demonstrated. More trials of sufficient power, with longer duration, and measuring clinically important outcomes are urgently needed.

  14. Hox genes define distinct progenitor sub-domains within the second heart field

    PubMed Central

    Bertrand, Nicolas; Roux, Marine; Ryckebüsch, Lucile; Niederreither, Karen; Dollé, Pascal; Moon, Anne; Capecchi, Mario; Zaffran, Stéphane

    2011-01-01

    Much of the heart, including the atria, right ventricle and outflow tract (OFT) is derived from a progenitor cell population termed the second heart field (SHF) that contributes progressively to the embryonic heart during cardiac looping. Several studies have revealed anterior-posterior patterning of the SHF, since the anterior region (anterior heart field) contributes to right ventricular and OFT myocardium whereas the posterior region gives rise to the atria. We have previously shown that Retinoic Acid (RA) signal participates to this patterning. We now show that Hoxb1, Hoxa1, and Hoxa3, as downstream RA targets, are expressed in distinct sub-domains within the SHF. Our genetic lineage tracing analysis revealed that Hoxb1, Hoxa1 and Hoxa3-expressing cardiac progenitor cells contribute to both atria and the inferior wall of the OFT, which subsequently gives rise to myocardium at the base of pulmonary trunk. By contrast to Hoxb1Cre, the contribution of Hoxa1-enhIII-Cre and Hoxa3Cre-labeled cells is restricted to the distal regions of the OFT suggesting that proximo-distal patterning of the OFT is related to SHF sub-domains characterized by combinatorial Hox genes expression. Manipulation of RA signaling pathways showed that RA is required for the correct deployment of Hox-expressing SHF cells. This report provides new insights into the regulatory gene network in SHF cells contributing to the atria and sub-pulmonary myocardium. PMID:21385575

  15. Charge density influences C1 domain ligand affinity and membrane interactions

    PubMed Central

    Lewin, Nancy E.; Kedei, Noemi; Hill, Colin S.; Selezneva, Julia S.; Valle, Christopher J.; Woo, Wonhee; Gorshkova, Inna; Blumberg, Peter M.

    2014-01-01

    The C1 domain, which represents the recognition motif on protein kinase C for the lipophilic second messenger diacylglycerol and its ultrapotent analog the phorbol esters, has emerged as a promising therapeutic target for cancer and other indications. Potential target selectivity is markedly enhanced both because binding reflects ternary complex formation between ligand, the C1 domain, and phospholipid, and because binding drives membrane insertion of the C1 domain, permitting aspects of the C1 domain surface outside the binding site per se to influence binding energetics. Here, focusing on charged residues identified in atypical C1 domains which contribute to their loss of ligand binding activity, we show that increasing charge along the rim of the binding cleft of the protein kinase C δ C1b domain raises the requirement for anionic phospholipids. Correspondingly, it shifts the selectivity of C1 domain translocation to the plasma membrane, which is more negatively charged than internal membranes. This change in localization is most pronounced in the case of more hydrophilic ligands, which provide weaker membrane stabilization than do the more hydrophobic ligands, and thus contributes an element to the structure activity relations for C1 domain ligands. Co-expressing pairs of C1 containing constructs with differing charges each expressing a distinct fluorescent tag provided a powerful tool to demonstrate the effect of increasing charge in the C1 domain. PMID:24777910

  16. Altered expression of retinoic acid (RA) receptor mRNAs in the fetal mouse secondary palate by all-trans and 13-cis RAs: implications for RA-induced teratogenesis.

    PubMed

    Naitoh, H; Mori, C; Nishimura, Y; Shiota, K

    1998-01-01

    Retinoic acid (RA) is mandatory for various biological processes and normal embryonic development but is teratogenic at high concentrations. In rodents, one of the major malformations induced by RA is cleft palate (CP). RA mediates its effects by RA receptors (RARs), but the expression patterns of RARs in the developing palate are still unclear. We investigated the normal expression of RAR alpha, beta, and gamma messenger RNAs (mRNAs) in the fetal mouse secondary palate and the effects of all-trans and 13-cis RAs on the expression of RAR mRNAs by Northern blot analysis. RAR alpha (2.8, 3.8 kb), RAR beta (3.3 kb), and RAR gamma (3.7 kb) mRNAs were detected in the fetal palate on gestational days (GD) 12.5-14.5. The expression of RAR alpha and gamma mRNAs did not show apparent sequential changes, but that of RAR beta mRNA increased at GD 13.5. Treatment of pregnant mice with 100 mg/kg all-trans RA induced CP in 94% of the fetuses and elevated the levels of RAR beta and gamma mRNAs in the fetal palate. The up-regulation of RAR beta mRNA by all-trans RA was more marked than that of RAR gamma mRNA. Treatment with 100 mg/kg 13-cis RA induced CP in only 19% of the fetuses. Although 13-cis RA elevated the RAR beta and gamma mRNA levels in fetal palates, its up-regulation was slower and less marked than that induced by all-trans RA. These findings indicate that the induction of RAR beta mRNA in the fetal palate correlates well with the tissue concentration of all-trans RA after RA treatment, and RAR beta may be one of the most influential candidate molecules for RA-induced teratogenesis.

  17. 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.

  18. Applications of 211At and 223Ra in targeted alpha-particle radiotherapy.

    PubMed

    Vaidyanathan, Ganesan; Zalutsky, Michael R

    2011-10-01

    Targeted radiotherapy using agents tagged with α-emitting radionuclides is gaining traction with several clinical trials already undertaken or ongoing, and others in the advanced planning stage. The most commonly used α-emitting radionuclides are 213Bi, 211At, 223Ra and 225Ac. While each one of these has pros and cons, it can be argued that 211At probably is the most versatile based on its half life, decay scheme and chemistry. On the other hand, for targeting bone metastases, 223Ra is the ideal radionuclide because simple cationic radium can be used for this purpose. In this review, we will discuss the recent developments taken place in the application of 211At-labeled radiopharmaceuticals and give an overview of the current status of 223Ra for targeted α-particle radiotherapy.

  19. Concentrations of (226)Ra, (232)Th and (4)(0)K in industrial kaolinized granite.

    PubMed

    Todorović, Nataša; Hansman, Jan; Mrđa, Dušan; Nikolov, Jovana; Kardos, Richárd; Krmar, Miodrag

    2017-03-01

    Activity concentrations of (226)Ra, (232)Th and (4)(0)K in 120 kaolinized granite samples imported in Serbia from the Motajica mine, Bosnia and Herzegovina, were measured. The (226)Ra concentration ranged from 61 to 319 Bq kg(-1), the (232)Th from 44 to 272 Bq kg(-1), and the (4)(0)K from 590 to 1470 Bq kg(-1). The frequency distribution of (4)(0)K concentrations was near-Gaussian, where those of (226)Ra and (232)Th were right-skewed. In 6 samples, the gamma index, I, was higher than 2, which exceeds the exemption dose criterion (0.3 mSv y(-1)). The absorbed dose rate and annual effective doses for workers in the ceramic industries in Serbia who worked with kaolinized granite were below levels of concern.

  20. Laser spectroscopy of trapped short-lived Ra{sup +} ions

    SciTech Connect

    Versolato, O. O.; Giri, G. S.; Wansbeek, L. W.; Berg, J. E. van den; Hoek, D. J. van der; Jungmann, K.; Kruithof, W. L.; Onderwater, C. J. G.; Sahoo, B. K.; Santra, B.; Shidling, P. D.; Timmermans, R. G. E.; Willmann, L.; Wilschut, H. W.

    2010-07-15

    As an important step toward an atomic parity violation experiment in one single trapped Ra{sup +} ion, laser spectroscopy on short-lived {sup 212,213,214}Ra{sup +} ions was conducted. The isotope shift of the 6 {sup 2}D{sub 3/2}-7 {sup 2}P{sub 1/2} and 6 {sup 2}D{sub 3/2}-7 {sup 2}P{sub 3/2} transitions and the hyperfine structure constants of the 7 {sup 2}P{sub 1/2} and 6 {sup 2}D{sub 3/2} states in {sup 213}Ra{sup +} were measured, which provides a benchmark for the required atomic theory. A lower limit of 232(4) ms for 6 {sup 2}D{sub 5/2} state lifetime was determined.