A preliminary investigation into the determination of the inaudibility of mechanical plant and music noise in the presence of ambient background noise

Currently there are regulations and guidelines that governing bodies have adopted when dealing with the emission of noise that make reference to or imply the term of inaudibility when setting criteria to be met for mechanical p lant and music noise after restricted hours. However, to date no such criteria has been established that can predict the inaudibility of these sources when combined with ambient backgrounds. As a result, stakeholders are met with uncertainty and designers are left with an inadequate subjective term when attempting to meet location-sp ecific noise criteria. This paper involves an investigation into the possibilities of conducting a psychoacoustic experiment that will test for the inaudibility of mechanical p lant and music noise in the presence of ambient background noise typical of the home environment situated in urban and suburban locations. This paper attempts to provide the framework for future larger scale investigations and provides the relevant findings and a methodology to assist in reducing the subjective nature of the responses observed. Through these future investigations, objective definable criteria from which to establish the inaudibility of mechanical plant and music noise in the presence of ambient background noise may be establishe

Poor Outcome in a Mitochondrial Neurogastrointestinal Encephalomyopathy Patient with a Novel TYMP Mutation: The Need for Early Diagnosis.

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a devastating autosomal recessive disorder due to mutations in TYMP, which cause loss of function of thymidine phosphorylase (TP), nucleoside accumulation in plasma and tissues and mitochondrial dysfunction. The clinical picture includes progressive gastrointestinal dysmotility, cachexia, ptosis and ophthalmoparesis, peripheral neuropathy and diffuse leukoencephalopathy, which usually lead to death in early adulthood. Therapeutic options are currently available in clinical practice (allogeneic hematopoietic stem cell transplantation and carrier erythrocyte entrapped TP therapy) and newer, promising therapies are expected in the near future. However, successful treatment is strictly related to early diagnosis. We report on an incomplete MNGIE phenotype in a young man harboring the novel heterozygote c.199 C>T (Q67X) mutation in exon 2, and the previously reported c.866 A>C (E289A) mutation in exon 7 in TYMP. The correct diagnosis was achieved many years after the onset of symptoms and unfortunately, the patient died soon after diagnosis because of multiorgan failure due to severe malnutrition and cachexia before any therapeutic option could be tried. To date, early diagnosis is essential to ensure that patients have the opportunity to be treated. MNGIE should be suspected in all patients who present with both gastrointestinal and nervous system involvement, even if the classical complete phenotype is lacking

A twistor-like D=10 superparticle action with manifest N=8 world-line supersymmetry

We propose a new formulation of the $D=10$ Brink-Schwarz superparticle which is manifestly invariant under both the target-space super-Poincar\'e group and the world-line local $N=8$ superconformal group. This twistor-like construction naturally involves the sphere $S^8$ as a coset space of the $D=10$ Lorentz group. The action contains only a finite set of auxiliary fields, but they appear in unusual trilinear combinations. The origin of the on-shell $D=10$ fermionic $\kappa$ symmetry of the standard Brink-Schwarz formulation is explained. The coupling to a $D=10$ super-Maxwell background requires a new mechanism, in which the electric charge appears only on shell as an integration constant.Comment: 22pages, standard LATEX fil

Free Differential Algebras and Pure Spinor Action in IIB Superstring Sigma Models

In this paper we extend to the case of IIB superstring sigma models the method proposed in hep-th/10023500 to derive the pure spinor approach for type IIA sigma models. In particular, starting from the (Free) Differential Algebra and superspace parametrization of type IIB supergravity, extended to include the BRST differential and all the ghosts, we derive the BRST transformations of fields and ghosts as well as the standard pure spinor constraints for the ghosts $\lambda$ related to supersymmetry. Moreover, using the method first proposed by us, we derive the pure spinor action for type IIB superstrings in curved supergravity backgrounds (on shell), in full agreement with the action first obtained by Berkovits and Howe.Comment: 24 page

Germline TP53 mutational spectrum in French Canadians with breast cancer

Abstract Background Specific germline mutations in the hereditary breast-ovarian cancer susceptibility (HBC/HBOC) genes, BRCA1, BRCA2 and PALB2, have been shown to recur in French Canadians of Quebec, Canada, and this has been attributed to common ancestors. Germline TP53 mutation carriers are known to segregate in Li-Fraumeni syndrome families, which feature young age of onset breast cancer. We have reported rare TP53 mutation carriers in French Canadian HBC families, though none recurred possibly due to the limited number of cancer families investigated. Here we describe TP53 germline mutations found in French Canadian cancer families provided from hereditary cancer clinics; investigate 37 new BRCA1 and BRCA2 mutation-negative HBC/HBOC families for the TP53 mutations; and assess the frequency of TP53 mutations in a 1235 French Canadian breast cancer cases not selected for family history of cancer. Methods TP53 mutation-positive pedigrees from French Canadian cancer families were provided from local hereditary cancer clinics. Bidirectional Sanger sequencing of all protein encoding exons of TP53 was performed using peripheral blood lymphocyte DNA from breast/ovarian cancer probands from 37 HBC/HBOC families of French Canadian descent. Targeted bidirectional Sanger sequencing assay of regions containing the identified TP53 mutations was performed on 1235 French Canadian breast cancer cases not selected for family history cancer. Results Five new TP53 mutations were identified in six pedigrees from hereditary cancer clinics. No deleterious mutations were identified in cancer probands from 37 HBC/HBOC families. A targeted mutation screen of the 1235 breast cancer cases identified a c.844C>T [p.Arg282Trp] mutation carrier. This mutation was also found among the six mutation-positive cancer families provided by the local hereditary cancer clinics. The targeted screen also uncovered a new TP53 mutation, c.685T>C [p.Cys229Arg] that was found in two breast cancer cases. All TP53 mutation carriers were among the 656 women with breast cancer diagnosed less than 50 years of age. Conclusions In all six new TP53 mutations were identified in French Canadians, where two each occurred in independently ascertained cases/families. Although all newly identified breast cancer mutation carriers reported a family history of cancer, none were consistent with features of Li-Fraumeni syndrome families

On Lorentz Invariant Actions for Chiral P-Forms

We demonstrate how a Lorentz covariant formulation of the chiral p-form model in D=2(p+1) containing infinitely many auxiliary fields is related to a Lorentz covariant formulation with only one auxiliary scalar field entering a chiral p-form action in a nonpolynomial way. The latter can be regarded as a consistent Lorentz-covariant truncation of the former. We make the Hamiltonian analysis of the model based on the nonpolynomial action and show that the Dirac constraints have a simple form and are all of the first class. In contrast to the Siegel model the constraints are not the square of second-class constraints. The canonical Hamiltonian is quadratic and determines energy of a single chiral p-form. In the case of d=2 chiral scalars the constraint can be improved by use of `twisting' procedure (without the loss of the property to be of the first class) in such a way that the central charge of the quantum constraint algebra is zero. This points to possible absence of anomaly in an appropriate quantum version of the model.Comment: RevTeX file, 7 page

Subcritical Superstrings

We introduce the Liouville mode into the Green-Schwarz superstring. Like massive supersymmetry without central charges, there is no kappa symmetry. However, the second-class constraints (and corresponding Wess-Zumino term) remain, and can be solved by (twisted) chiral superspace in dimensions D=4 and 6. The matter conformal anomaly is c = 4-D < 1. It thus can be canceled for physical dimensions by the usual Liouville methods, unlike the bosonic string (for which the consistency condition is c = D <= 1).Comment: 9 pg., compressed postscript file (.ps.Z), other formats (.dvi, .ps, .ps.Z, 8-bit .tex) available at http://insti.physics.sunysb.edu/~siegel/preprints/ or at ftp://max.physics.sunysb.edu/preprints/siege