Linear Strain Tensors on Hyperbolic Surfaces and Asymptotic Theories for Thin Shells

We perform a detailed analysis of the solvability of linear strain equations on hyperbolic surfaces. We prove that if the surface is a smooth noncharacteristic region, any first order infinitesimal isometry can be matched to an infinitesimal isometry of an arbitrarily high order. The implications of this result for the elasticity of thin hyperbolic shells are discussed

Primary Immune Regulatory Disorders With an Autoimmune Lymphoproliferative Syndrome-Like Phenotype: Immunologic Evaluation, Early Diagnosis and Management

Primary immune regulatory disorders (PIRD) are associated with autoimmunity, autoinflammation and/or dysregulation of lymphocyte homeostasis. Autoimmune lymphoproliferative syndrome (ALPS) is a PIRD due to an apoptotic defect in Fas-FasL pathway and characterized by benign and chronic lymphoproliferation, autoimmunity and increased risk of lymphoma. Clinical manifestations and typical laboratory biomarkers of ALPS have also been found in patients with a gene defect out of the Fas-FasL pathway (ALPS-like disorders). Following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA), we identified more than 600 patients suffering from 24 distinct genetic defects described in the literature with an autoimmune lymphoproliferative phenotype (ALPS-like syndromes) corresponding to phenocopies of primary immunodeficiency (PID) (NRAS, KRAS), susceptibility to EBV (MAGT1, PRKCD, XIAP, SH2D1A, RASGRP1, TNFRSF9), antibody deficiency (PIK3CD gain of function (GOF), PIK3R1 loss of function (LOF), CARD11 GOF), regulatory T-cells defects (CTLA4, LRBA, STAT3 GOF, IL2RA, IL2RB, DEF6), combined immunodeficiencies (ITK, STK4), defects in intrinsic and innate immunity and predisposition to infection (STAT1 GOF, IL12RB1) and autoimmunity/autoinflammation (ADA2, TNFAIP3,TPP2, TET2). CTLA4 and LRBA patients correspond around to 50% of total ALPS-like cases. However, only 100% of CTLA4, PRKCD, TET2 and NRAS/KRAS reported patients had an ALPS-like presentation, while the autoimmunity and lymphoproliferation combination resulted rare n other genetic defects. Recurrent infections, skin lesions, enteropathy and malignancy are the most common clinical manifestations. Some approaches available for the immunological study and identification of ALPS-like patients through flow cytometry and ALPS biomarkers are provided in this work. Protein expression assays for NKG2D, XIAP, SAP, CTLA4 and LRBA deficiencies and functional studies of AKT, STAT1 and STAT3 phosphorylation, are showed as useful tests. Patients suspected to suffer from one of these disorders require rapid and correct diagnosis allowing initiation of tailored specific therapeutic strategies and monitoring thereby improving the prognosis and their quality of life

Disentangling Spectral Phases of Interfering Autoionizing States from Attosecond Interferometric Measurements

We have determined spectral phases of Ne autoionizing states from extreme ultraviolet and midinfrared attosecond interferometric measurements and ab initio full-electron time-dependent theoretical calculations in an energy interval where several of these states are coherently populated. The retrieved phases exhibit a complex behavior as a function of photon energy, which is the consequence of the interference between paths involving various resonances. In spite of this complexity, we show that phases for individual resonances can still be obtained from experiment by using an extension of the Fano model of atomic resonances. As simultaneous excitation of several resonances is a common scenario in many-electron systems, the present work paves the way to reconstruct electron wave packets coherently generated by attosecond pulses in systems larger than heliumWork supported by the ERC proof-of-concept Grant No. 780284-Imaging-XChem within the seventh framework program of the European Union, the MINECO Project No. FIS2013-42002-R, the EU-H2020- LASERLABEUROPE-654148, the ANR Projects No. ANR-15-CE30-0001-CIMBAAD, No. ANR-11- EQPX0005-ATTOLAB, and No. ANR-10-LABX-0039- PALM, the U.S. Department of Energy, Office of Science, Basic Energy Sciences, under Award no. DEGF02-04ER15614, and the NSF Grant No. PHY-1607588. Calculations were performed at CCC-UAM and Marenostrum Supercomputer Center. F. M. acknowledges support from the “Severo Ochoa” Programme for Centres of Excellence in R&D (MINECO, Grant No. SEV-2016- 0686) and the “María de Maeztu” Programme for Units of Excellence in R&D (Grant No. MDM-2014-0377

Usos de la glutamina en pediatría

ResumenLa glutamina, aminoácido “condicionalmente indispensable” tiene un rol clave en la respuesta del intestino delgado ante diferentes agresiones. Bajo ciertas condiciones de estrés clínico y quirúrgico ocurren cambios y atrofi a en la mucosa intestinal, originando diversos grados de desnutrición. Investigadores han demostrado que el intestino es el órgano de mayor actividad de la glutamina y que su epitelio es considerado como blanco en la captura de la glutamina; asignándole un papel especial en la recuperación del intestino delgado. Se revisaron los trabajos recientes sobre su utilidad en el área pediátrica, con énfasis en el metabolismo intestinal y nutrición. Concluyendo que la glutamina puede ser benefi ciosa y absolutamente necesaria durante las situaciones críticas.[Marante J, Rodríguez R, López KC, González LG, Flores LS, Villalobos DC, et al. Usos de la glutamina en pediatría. MedUNAB 2005; 8 (1 Supl 1):S37-S42]Palabras clave: Glutamina, pediatría, intestino, aminoácido, nutrición enteral