Search results for "Dosis"
showing 10 items of 369 documents
Attenuated mucopolysaccharidosis: are you missing this debilitating condition?
2011
Metabolic Consequences after Urinary Diversion
2014
Metabolic disturbances are well-known, but sometimes neglected immediate consequences or late sequelae following urinary diversion (UD) using bowel segments. Whereas subclinical disturbances appear to be quite common, clinically relevant metabolic complications, however, are rare. Exclusion of bowel segments for UD results in loss of absorptive surface for its physiological function. Previous studies demonstrated that at least some of the absorbtive and secreting properties of the bowel are preserved when exposed to urine.For each bowel segment typical consequences and complications have been reported. The use of ileal and/or colonic segments may result in hyperchloremic metabolic acidosis …
Validación de los datos de radiación solar UV del Ozone Monitoring Instrument (OMI) a partir de medidas con base en tierra en la costa mediterránea
2016
<p align="justify">The erythemal UV daily dose (EDD) and the local noon UV Index (UVI) obtained from the Ozone Monitoring Instrument (OMI), on board NASA’s Aura satellite, have been validated for the period 2005-2013 using ground based measurements at 5 different sites in the Mediterranean coast: Murcia, Valencia, Palma de Mallorca, Barcelona and Rome (where only measurements of the local noon UVI were available). Ground based measurements were made using YES UVB-1 radiometers in Murcia, Valencia, Palma de Mallorca and Barcelona, and a Brewer MKIV 067 spectrophotometer in Rome. The results of the validation showed good agreement between the satellite instrument and the ground based me…
Safety study of sodium pentosan polysulfate for adult patients with mucopolysaccharidosis type II
2019
Current therapies for the mucopolysaccharidoses (MPS) do not effectively address skeletal and neurological manifestations. Pentosan polysulfate (PPS) is an alternative treatment strategy that has been shown to improve bone architecture, mobility, and neuroinflammation in MPS animals. The aims of this study were to a) primarily establish the safety of weekly PPS injections in attenuated MPS II, b) assess the efficacy of treatment on MPS pathology, and c) define appropriate clinical endpoints and biomarkers for future clinical trials. Subcutaneous injections were administered to three male Japanese patients for 12 weeks. Enzyme replacement therapy was continued in two of the patients while th…
Polymer-doxycycline conjugates as fibril disrupters: an approach towards the treatment of a rare amyloidotic disease.
2014
The term amyloidosis describes neurological diseases where an abnormal protein is misfolded and accumulated as deposits in organs and tissues, known as amyloid, disrupting their normal function. In the most common familial amyloid polyneuropathy (FAP), transthyretin (TTR) displays this role primarily affecting the peripheral nervous system (PNS). Advanced stages of this inherited rare amyloidosis, present as fibril deposits that are responsible for disease progression. In order to stop disease progression, herein we designed an efficient family of nanoconjugates as fibril disrupters. These polymer conjugates are based on doxycycline (doxy), already in phase II trials for Alzheimer's disease…
Fetal presentation of Morquio disease type A.
1992
A fetus with mucopolysaccharidosis type IV A (Morquio type A) is described. The family had one affected child exhibiting symptoms of classical Morquio A disease, and late in the subsequent pregnancy prenatal diagnosis was requested. At 23 weeks' gestation, moderate ascites was detected by detailed ultrasound scan and keratan sulphate was found in the amniotic fluid. The pregnancy was terminated by prostaglandin induction and the diagnosis of mucopolysaccharidosis type IV A was confirmed by demonstration of a deficiency of N-acetylgalactosamine-6-sulphate (GalNac-6-S) sulphatase in cultured amniotic cells and in post-mortem fibroblast cultures. The activities of beta-galactosidase and arylsu…
Scrotal Masses
2009
No abstract available
Probing ensemble polymorphism and single aggregate structural heterogeneity in insulin amyloid self-assembly.
2020
Ensembles of protein aggregates are characterized by a nano- and micro-scale heterogeneity of the species. This diversity translates into a variety of effects that protein aggregates may have in biological systems, both in connection to neurodegenerative diseases and immunogenic risk of protein drug products. Moreover, this naturally occurring variety offers unique opportunities in the field of protein-based biomaterials. In the above-mentioned fields, the isolation and structural analysis of the different amyloid types within the same ensemble remain a priority, still representing a significant experimental challenge. Here we address such complexity in the case of insulin for its relevance…
Der Einfluss aliment�rer Acidosen und Alkalosen auf die Wachstumsgeschwindigkeit und die Eiweissausnutzung der Ratte
1958
Die Erzeugung einer alimentaren Acidose mit 200 mAq NH4Cl/kg Futter bei wachsenden Ratten fuhrt bei Mannchen und Weibchen zu einer schlechteren Protein-Efficiency, aber nur bei den Mannchen zu einer geringeren Wachstumsgeschwindigkeit. Eine mit NaHCO3 in analoger Weise induzierte alimentare Alkalose hat weder bei den Mannchen noch bei den Weibchen einen sicheren Einflus auf die Protein-Efficiency und auf die Wachstumsgeschwindigkeit.
Myocardial contrast echocardiography in biopsy-proven primary cardiac amyloidosis.
2008
Abstract Cardiac vasculature is affected in 88-90% of patients with primary cardiac amyloidosis (CA). Myocardial contrast echocardiography (MCE) relies on the ultrasound detection of microbubble contrast agents that are solely confined to the intravascular space, and are therefore useful in the evaluation of flow in the microvasculature. This is the first case report describing the use of MCE during vasodilator stress to evaluate coronary flow reserve in a patient with biopsy-proven primary CA and angiographically normal coronaries. Qualitative MCE demonstrated delayed replenishment of microbubbles during peak stress; quantitative analysis was consistent with a reduction in total myocardial…