Search results for "orage"

Storage Diseases: Diagnostic Position

2013

Storage diseases are metabolic multiorgan conditions, which may be divided into lysosomal and nonlysosomal diseases. Disorders of the lysosomal type require electron microscopy for morphological diagnosis. It is the metabolic substrate that determines involvement of the cell type or organ in the individual storage disease, allowing extracerebral biopsies, for instance, in the neuronal ceroid-lipofuscinoses (NCL). A hierarchy of tissues biopsied for diagnosis can be based on easy accessibility: blood lymphocytes, skin, conjunctiva, rectum, skeletal muscle. Lysosomal diseases are divided into vacuolar and nonvacuolar ones. NCL display variegated ultrastructural patterns. Drugs may induce lyso…

Severe neonatal onset of glycogenosis type IV: Clinical and laboratory findings leading to diagnosis in two siblings

2005

Glycogenosis type IV is an autosomal recessive disease, exceptionally diagnosed at birth: only very few reports of the fatal perinatal neuromuscular form have been described. We report on two sibling male newborns who died at 10 and 4 weeks of age with clinical signs of a systemic storage disease. Prenatal history included polyhydramnios, reduced fetal movements and fetal hydrops, and Caesarean section was performed at 36 weeks of gestational age because of fetal distress. At birth, both babies showed severe hypotonia, hyporeflexia and no spontaneous breathing activity. They never showed active movements, sucking and swallowing and were respirator-dependent until death. A muscle biopsy reve…

CNS manifestations of Fabry's disease

2006

Summary Background Fabry's disease is a rare hereditary lysosomal storage disease with multiorgan involvement. Deficiency of α-galactosidase A activity leads to accumulation of neutral glycosphingolipids, especially in vascular endothelial and smooth-muscle cells. Along with progressive renal and cardiac dysfunction, stroke is a major and often life-threatening burden of the disease. Cerebral vasculopathy, confirmed by neuropathological, neuroradiological, and functional studies, occurs commonly and leads to ischaemic cerebrovascular events at an early age. Recent developments Fabry's disease is an X-linked disease and women have been regarded as only mildly affected carriers. However, rese…

Accounting meets economics: towards an ‘accounting view’ of money.

2018

Questo studio pone le fondamenta dell’ “Approccio Contabile” (“Accounting View”) alla moneta. Sulla base dei principi di Ragioneria Generale riconosciuti internazionalmente, lo studio argomenta che le monete emesse dallo stato e dalla banca centrale non costituiscono debito e che nei regimi di riserva frazionaria soltanto una quota della moneta emessa dalle banche commerciali pu essere considerata quale debito. Lo studio chiarisce come il signoraggio associato all’emissione di queste monete debba essere contabilizzato nei bilanci delle istituzioni emittenti ed esamina le implicazioni di ci`o per la corretta comprensione della moneta. Il nuovo approccio fa luce su argomenti quali la vera nat…

Long Term Efficiency Analysis of Infiltration Trenches Subjected to Clogging

2018

Abstract: In recent years, limitations linked to traditional urban drainage schemes have been pointed out and new approaches were developed introducing more natural methods for retaining and/or disposing of stormwater. Such practices include infiltration and storage tanks in order to reduce the peak flow and retain part of the polluting components. The impact of such practices on stormwater quantity and quality is not easily assessable because of the complexity of physical and chemical processes involved. In such cases, integrated urban drainage models may play a relevant role providing tools for long term analysis. In this study, the effect of the clogging phenomenon has been assessed by m…

Myrosinase activity and quality parameters on fresh-cut Chinese cabbage stored at two different temperatures

2009

Cover Picture: Bis(2,2′‐biphenoxy)borates for Electrochemical Double‐Layer Capacitor Electrolytes (Chem. Eur. J. 11/2011)

2011

Phase analysis and oxygen strorage capacity of ceria-lanthana-based TWC promoters prepared by sol-gel routes

2002

Ceria–lanthana-based promoters of three-way catalysts are synthesized by two different sol–gel routes, involving nitrate precursors. The oxygen uptake ability of these compounds is measured by O2 chemisorption. The specific surface area is determined by N2 adsorption (BET). X-ray diffraction data are analyzed by Rietveld refinement, demonstrating that lanthanum forms solid solution with CeO2; its total amount in ceria depends on the competitive formation of La–Al mixed oxides and on the synthetic method. The O2 uptake ability is essentially determined by the La content in the ceria–lanthana solid solution, while it is independent on the surface area and on the CeO2 particle size. The O2 upt…

EFFECT OF HOT AIR TREATMENT ON MINIMALLY PROCESSED CAULIFLOWER

2013

The request of minimally processed vegetable is continuously increasing, and there is a growing interest for new fresh cut products. Cauliflower, as other cabbages, is highly appreciated for its nutritional value due to the good content of vitamins, antioxidants and anti-carcinogenic compounds. It is suitable to be used as a minimally processed vegetable, but harvesting and the following processing can cause a severe stress determining the appearance of accelerated senescence symptoms. The aim of the present work was to investigate the effect of hot air treatment and cold storage on minimally processed green cauliflower. Fresh-cut cauliflower florets put in sealed PE bags were treated at 48…

Arylazopyrazoles for Long-Term Thermal Energy Storage and Optically Triggered Heat Release below 0 °C.

2020

Arylazopyrazole derivatives based on four core structures (4pzMe, 3pzH, 4pzH, and 4pzH-F2) and functionalized with a dodecanoate group were demonstrated to store thermal energy in their metastable Z isomer liquid phase and release the energy by optically triggered crystallization at -30 °C for the first time. Three heat storage-release schemes were discovered involving different activation methods (optical, thermal, or combined) for generating liquid-state Z isomers capable of storing thermal energy. Visible light irradiation induced the selective crystallization of the liquid phase via Z-to-E isomerization, and the latent heat stored in the liquid Z isomers was preserved for longer than 2 …