346 research outputs found

    Presença De Dente Neo-natal Em Portador Da Síndrome De Ellis Van-creveld: Relato De Caso

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    The aim of this article is go over the literature and describe a case of Ellis Van Creveld syndrome, in which the patient presented a neo-natal tooth Just-born and premature patient, female, came to the hospital of Limeira, São Paulo, and was attended by the Surgery and Maxillofacial Traumatology group from the Dentistry College of Piracicaba (FOPUNICAMP). Her main complaint was the neo-natal tooth. During the extraoral examination it was detected a tower shaped cranium, shortened limbs, lank and sparse hair, bilateral postaxial polydactyly, hypoplasic nails and heart changes. In the intraoral examination the signs included multiple labial frenum associated with gingival changes, and presence of a conic neo natal teeth. The diagnosis of Ellis van Creveld syndrome was based on the clinical and radiographic findings that included characteristics from Chondroectodermaldysplasia. It was not found any history of this syndrome in her family. Being thus, the Ellis Van Creveld Syndrome although rare, presents many characteristics that demand a multidiscipline treatment. Odontologic manifestations are there, that's why surgeons, especially dentists, must be aware of it so that their behavior can contribute for the patient well-being.5115760Polymeropoulos, M.H., Ide, S.E., Wright, M., Goodship, J., Weissenbach, J., Pyeritz, R.E., Silva, D.E.O., Francomano, C.A., The gene for the Ellis-van Creveld syndrome is located on chromosome 4p16 (1996), 35, pp. 1-5. , GenomicsEllis, R.W., Crefeld, V.S., A syndrome characterized by ectodermaldysplasia, polydactyly, chondrodysplasia and congenital morbuscardia (1940) Arch Dis Child, 15, p. 65Goor, D., Rotem, Y., Friedman, A., Neufeld, H.N., Ellis-van Creveld syndrome in identical twins (1965) Br Heart J, 27, pp. 797-804Keizer, D.P.R., Schilder, J.H., Ectodermal dysplasia, achondrodysplasia and congenital morbus cordis (1951) Am J Dis Child, 82, pp. 341-344Mitchell, F.N., Waddell, W.W.Jr., Ellis-van Creveld syndrome: report of 2 cases in siblings (1958) Acta Paediatr, 47, pp. 142-151Digilio, M., Marino, B., Ammirati, A., Borgaza, U., Giannotti, A., Dallapiccola, B., Cardiac malformations in patients with oral-facialskeletal syndromes: clinical similarities with heterotaxia (1999) Am J Med Genet, 84, pp. 350-356Baujat, G., Merrer, L.M., Ellis-Van Creveld syndrome (2007) Orphanet Journal of Rare Diseases, 2, p. 27Kurian, K., Shanmugan, S., Vardat, H.T., Siddharth, G., Chondroecthodermal dysplasia (Ellis van Creveld syndrome): a report of three cases with review of literature (2007) Indian J Dent Res, 1, p. 18Tachdjan, M., Pediatric Orthopaedics, 2a ed (1995) Manole:São PauloSilva, D.E.O., Janovitz, D., Albuquerque, D.S.C., Ellis-van Creveld syndrome: report of 15 cases in an inbred kindred (1980) J Med Genet, 17, pp. 349-56Alvarez-borja, A., Ellis-Van Creveld syndrome. Report of two cases (1960) Pediatrics, 26, pp. 301-9Pinto Jr., S.C., Lammel, C., Kim, J.H., Borges, J.L.P., Displasia condroectodérmica (síndrome de Ellis-van Creveld): relato de dois casos (2003) Rev Bras Ortop, 38, pp. 357-61Winter, L., Pediatric Orthopaedics, 4th ed (1996) Lippincott-Raven: Philadelphi

    Measurement of the ratio of prompt χ c to J / ψ production in pp collisions at √s = 7 TeV

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    The prompt production of charmonium χ c and J / ψ states is studied in proton-proton collisions at a centre-of-mass energy of √s = 7 TeV at the Large Hadron Collider. The χ c and J / ψ mesons are identified through their decays χ c → J / ψ γ and J / ψ → μ + μ - using 36 pb - 1 of data collected by the LHCb detector in 2010. The ratio of the prompt production cross-sections for χ c and J / ψ, σ (χ c → J / ψ γ) / σ (J / ψ), is determined as a function of the J / ψ transverse momentum in the range 2 < p T J / ψ < 15 GeV / c. The results are in excellent agreement with next-to-leading order non-relativistic expectations and show a significant discrepancy compared with the colour singlet model prediction at leading order, especially in the low p T J / ψ region

    Evidence for the decay B0→J/ψω and measurement of the relative branching fractions of meson decays to J/ψη and J/ψη′

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    First evidence of the B 0 → J / ψ ω decay is found and the B s 0 → J / ψ η and B s 0 → J / ψ η ′ decays are studied using a dataset corresponding to an integrated luminosity of 1.0 fb -1 collected by the LHCb experiment in proton-proton collisions at a centre-of-mass energy of sqrt(s) = 7 TeV. The branching fractions of these decays are measured relative to that of the B 0 → J / ψ ρ 0 decay:frac(B (B 0 → J / ψ ω), B (B 0 → J / ψ ρ 0)) = 0.89 ± 0.19 (stat) - 0.13 + 0.07 (syst),frac(B (B s 0 → J / ψ η), B (B 0 → J / ψ ρ 0)) = 14.0 ± 1.2 (stat) - 1.5 + 1.1 (syst) - 1.0 + 1.1 (frac(f d, f s)),frac(B (B s 0 → J / ψ η ′), B (B 0 → J / ψ ρ 0)) = 12.7 ± 1.1 (stat) - 1.3 + 0.5 (syst) - 0.9 + 1.0 (frac(f d, f s)), where the last uncertainty is due to the knowledge of f d / f s, the ratio of b-quark hadronization factors that accounts for the different production rate of B 0 and B s 0 mesons. The ratio of the branching fractions of B s 0 → J / ψ η ′ and B s 0 → J / ψ η decays is measured to befrac(B (B s 0 → J / ψ η ′), B (B s 0 → J / ψ η)) = 0.90 ± 0.09 (stat) - 0.02 + 0.06 (syst)

    Focusing in on use of pharmacokinetic profiles in routine hemophilia care

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    Emergence of population pharmacokinetic models for prediction of individual pharmacokinetic (PK) profiles facilitates individualization of prescribed prophylactic therapy for patients with hemophilia A and B and may have a favorable impact on clinical outcomes and annual factor utilization. How providers approach the integration and application of these data into routine clinical practice is not clear. To explore the potential application of and barriers to incorporating PK profiles into current hemophilia prophylaxis decision making. A facilitated group discussion of hematologists practicing within the federally-supported United States Hemophilia Treatment Center Network was conducted. Separately, a group of parents of patients with severe hemophilia less than 18 years of age participated in a focus group on individualizing prophylactic factor regimens with the use of PK data. Physician participants constructed a conceptual model for factors that determined their selection of hemophilia prophylaxis. These factors clustered in five groupings. When charged with creating a prophylaxis regimen for a specific clinical case including PK data, eight of nine providers generated a unique regimen. Parent focus group supported PK data use as they preferred data driven treatment decisions. Clinician application of PK data for prophylaxis decision making is heterogeneous. Prospective evaluation of the use of PK-tailored prophylaxis in routine care and its impact on patient outcomes is needed. Parents perceived that, while obtaining blood draws could be challenging, images of factor activity decay informed their decisions about physical activity timing and provided an opportunity for partnership and shared decision making with their provider

    Therapeutic designed poly (lactic-co-glycolic acid) cylindrical oseltamivir phosphate-loaded implants impede tumor neovascularization, growth and metastasis in mouse model of human pancreatic carcinoma

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    Michael Hrynyk,1 Jordon P Ellis,1 Fiona Haxho,2 Stephanie Allison,1 Joseph AM Steele,1 Samar Abdulkhalek,2 Ronald J Neufeld,1 Myron R Szewczuk21Department of Chemical Engineering, 2Department of Biomedical and Molecular Sciences, Queen&rsquo;s University, Kingston, ON, CanadaAbstract: Poly (lactic-co-glycolic acid) (PLGA) copolymers have been extensively used in cancer research. PLGA can be chemically engineered for conjugation or encapsulation of drugs in a particle formulation. We reported that oseltamivir phosphate (OP) treatment of human pancreatic tumor-bearing mice disrupted the tumor vasculature with daily injections. Here, the controlled release of OP from a biodegradable PLGA cylinder (PLGA-OP) implanted at tumor site was investigated for its role in limiting tumor neovascularization, growth, and metastasis. PLGA-OP cylinders over 30 days in vitro indicated 20%&ndash;25% release profiles within 48 hours followed by a continuous metronomic low dose release of 30%&ndash;50% OP for an additional 16&nbsp;days. All OP was released by day 30. Surgically implanted PLGA-OP containing 20 mg OP and blank PLGA cylinders at the tumor site of heterotopic xenografts of human pancreatic PANC1 tumors in RAGxC&gamma; double mutant mice impeded tumor neovascularization, growth rate, and spread to the liver and lungs compared with the untreated cohort. Xenograft tumors from PLGA and PLGA-OP-treated cohorts expressed significant higher levels of human E-cadherin with concomitant reduced N-cadherin and host CD31+ endothelial cells compared with the untreated cohort. These results clearly indicate that OP delivered from PLGA cylinders surgically implanted at the site of the solid tumor show promise as an effective treatment therapy for cancer.Keywords: pancreatic cancer, oseltamivir phosphate, PLGA, tumor neovascularization, metastasi

    Measurement of the time-dependent CP asymmetry in B0 -> J/ψ KS0 decays

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    This Letter reports a measurement of the CP violation observables SJ/ψK0S and CJ/ψK0S in the decay channel B0→J/ψK0S performed with 1.0 fb−1 of pp collisions at s√=7 TeV collected by the LHCb experiment. The fit to the data yields SJ/ψK0S=0.73±0.07(stat)±0.04(syst) and CJ/ψK0S=0.03±0.09(stat)±0.01(syst). Both values are consistent with the current world averages and within expectations from the Standard Model

    Human CCAAT displacement protein is homologous to the Drosophila homeoprotein, cut.

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    Human CCAAT displacement protein (CDP), a putative repressor of developmentally regulated gene expression, was purified fron HeLa cells by DNA binding-site affinity chromatography. cDNA encoding CDP was obtained by immunoscreening a lambda gt11 library with antibody raised against purified protein. The deduced primary amino acid sequence of CDP reveals remarkable homology to Drosophila cut with respect to the presence of a unique homeodomain and "cut repeats". As cut participates in determination of cell fate in several tissues in Drosophila, the similarity predicts a broad role for CDP in mammalian development

    Measurement of the CP-violating phase \phi s in Bs->J/\psi\pi+\pi- decays

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    Measurement of the mixing-induced CP-violating phase phi_s in Bs decays is of prime importance in probing new physics. Here 7421 +/- 105 signal events from the dominantly CP-odd final state J/\psi pi+ pi- are selected in 1/fb of pp collision data collected at sqrt{s} = 7 TeV with the LHCb detector. A time-dependent fit to the data yields a value of phi_s=-0.019^{+0.173+0.004}_{-0.174-0.003} rad, consistent with the Standard Model expectation. No evidence of direct CP violation is found

    Measurement of the Bs0J/ψKS0B_s^0\to J/\psi K_S^0 branching fraction

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    The B 0 s → J/ψK 0 S branching fraction is measured in a data sample corresponding to 0.41 fb−1 of integrated luminosity collected with the LHCb detector at the LHC. This channel is sensitive to the penguin contributions affecting the sin 2β measurement from B 0 → J/ψK 0 S . The time-integrated branching fraction is measured to be B(B 0 s → J/ψK 0 S ) = (1.83±0.28)×10−5 . This is the most precise measurement to date

    Observation of J/ψ-pair production in pp collisions at √s=7 TeV

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    The production of J/ψ pairs in proton–proton collisions at a centre-of-mass energy of 7 TeV has been observed using an integrated luminosity of 37.5 pb−1 collected with the LHCb detector. The production cross-section for pairs with both J/ψ in the rapidity range 2 < yJ/ψ < 4.5 and transverse momentum pJ/ψ T <10 GeV/c is σJ/ψJ/ψ =5.1±1.0±1.1 nb, where the first uncertainty is statistical and the second systematic
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