Search results for "TALIS"

H3PW12O40 supportato per l'idratazione di propene in regime gas-solido: attività catalitica e catalitica fotoassistita

2011

Structure Sensitivity of 2‐Methyl--butyn-2-ol Hydrogenation on Pd: Computational and Experimental Modeling

2014

Touristification, Sharing Economies and the New Geography of Urban Conflicts

2018

The aim of this study was to address the highly controversial problem of the increasing touristification of urban centers, analyzing the case of Valencia. The paper begins with a theoretical reflection to disambiguate the term &ldquo

Una revisión del concepto de «acumulación por desposesión» de D. Harvey

2019

espanolEl objetivo del presente articulo sera realizar una revision critica de la interpretacion realizada por D. Harvey del concepto de «acumulacion originaria» expuesto por K. Marx en El Capital. A traves del analisis de los escritos donde Marx aborda esta cuestion, trataremos de demostrar que la aportacion principal de Harvey -reinterpretar la acumulacion originaria, no como proceso fundacional de las condiciones necesarias para la produccion capitalista, sino como proceso continuo y permanente utilizado para restablecer las condiciones optimas de la acumulacion de capital mediante la conquista de nuevos espacios- se sustenta en una interpretacion parcial de los textos de critica de la e…

743 Lysosomal Storage Disorders in Non-Immunological Hydrops Fetalis - More Common than Assumed?

2012

Background Although non immunological hydrops fetalis (NIHF) is a very rare disorder, the disturbance accounts for a disproportionate share (3%) of overall mortality in the perinatal period. Lysosomal storage disorders (LSD) are only exceptionally considered to be the cause of NIHF. The reported incidence is about 1%. On the other hand, in about 18% of all cases, NIHF is classified as idiopathic. Patients and methods We report four cases of transient NIHF due to LSD and reviewed the literature for LSD associated with NIHF. Results At present, 12 different LSD are described to be associated with NIHF. The majority of reported patients already had a family history of NIHF, which had not been …

Greenberg dysplasia: first reported case with additional non-skeletal malformations and without consanguinity

2001

In 1988 Greenberg et al. reported an association of fetal hydrops with irregular calcification and moth-eaten skeletal dysplasia. Here, we report on the first case of this disorder accompanied by additional malformations (omphalocele, intestinal malrotation, disturbed fingernails and toes, hypolobated lungs) in a German couple without consanguinity (karyotype 46,XY). Sonograpically, the fetus was characterised by tetraphokomelia, severe generalised hydrops, pulmonal hypoplasia and hepato-splenomegaly. Greenberg dysplasia should be considered in differential diagnosis in cases with severe fetal hydrops and phokomelia on antenatal sonography.

Acid ceramidase deficiency: Farber lipogranulomatosis, spinal muscular atrophy associated with progressive myoclonic epilepsy and peripheral osteolys…

2020

Abstract An inherited deficiency of the lysosomal enzyme ceramidase leads to accumulation of the sphingolipid ceramide in several organs such as skin, liver, brain and other tissues, resulting in a broad spectrum of clinical manifestations. The most common form, called Farber lipogranulomatosis, is characterized by subcutaneous skin nodules and a progressive hoarseness, in many cases also the central nervous system is affected. A lethal hydrops fetalis represents the most severe form. A ceramidase deficiency was also found in a few patients in whom neurological symptoms such as spinal muscular atrophy and myoclonus epilepsy dominated the clinical picture, in other adult patients peripheral …

The multifaceted spectrum of liver cirrhosis in older hospitalised patients: Analysis of the REPOSI registry

2021

Abstract Background Knowledge on the main clinical and prognostic characteristics of older multimorbid subjects with liver cirrhosis (LC) admitted to acute medical wards is scarce. Objectives To estimate the prevalence of LC among older patients admitted to acute medical wards and to assess the main clinical characteristics of LC along with its association with major clinical outcomes and to explore the possibility that well-distinguished phenotypic profiles of LC have classificatory and prognostic properties. Methods A cohort of 6,193 older subjects hospitalised between 2010 and 2018 and included in the REPOSI registry was analysed. Results LC was diagnosed in 315 patients (5%). LC was ass…

Bienes comunes de la humanidad

2003

Idiopathic Acquired Leukonychia in a 34-Year-Old Patient

2009

We present a rare case of a 34-year-old patient with persistent, progressive, acquired leukonychia totalis and partialis. Idiopathic acquired leukonychia is a rare chromatic disorder of the nail not associated with other abnormalities and discernible etiology. Our case report did not link the inheritance of leukonychia with diverse clinical syndromes. To our knowledge, only five cases of idiopathic, acquired, true total leukonychia were found in literature. This case was the sixth patient with asymptomatic idiopathic, white fingernails, and toenails without a hereditary cause.