Clinical Classification of Variants in the Valosin-Containing Protein Gene Associated With Multisystem Proteinopathy.

Background And Objectives: Pathogenic variants in the valosin-containing protein () gene cause a phenotypically heterogeneous disorder that includes myopathy, motor neuron disease, Paget disease of the bone, frontotemporal dementia, and parkinsonism termed multisystem proteinopathy. This hallmark pleiotropy makes the classification of novel variants challenging. This retrospective study describes and assesses the effect of 19 novel or nonpreviously clinically characterized variants identified in 28 patients (26 unrelated families) in the retrospective VCP International Multicenter Study.

Crude and acid oils from olive pomace as alternative fat sources in growing-finishing pigs.

The inclusion of crude and acid oils from olive pomace can lead to more unsaturated meat products and, especially in the case of olive pomace acid oil, achieve a more economically and environmentally sustainable swine production. The objective of this trial was to study the effect of dietary supplementation with crude and acid oils from olive pomace, which are rich in monounsaturated fatty acids (FAs) and have differing free FA content, on growth performance, digestibility, carcass parameters and FA profile of Longissimus muscle (LM) and backfat in growing-finishing pigs compared to the conventional crude palm oil. A total of 224 male and female pigs [(Landrace × Large White) × Duroc] were randomly distributed into 48 pens according to initial BW (58.

Declining Use of Corticosteroids for Crohn's Disease Has Implications for Study Recruitment: Results of a Pilot Randomized Controlled Trial.

Background: Corticosteroids (CS) have been used extensively to induce remission in Crohn's disease (CD); however, they are associated with severe side effects. We hypothesized that the administration of an exclusive enteral nutrition (EEN) formula to CS would lead to increased CD remission rates and to decreased CS-related adverse events. We proposed to undertake a pilot study comparing EEN and CS therapy to CS alone to assess decrease symptoms and inflammatory markers over 6 weeks.

Inertization of pyrite cinders and co-inertization with electric arc furnace flue dusts by pyroconsolidation at solid state.

The viability of a pyroconsolidation process to render pyrite cinders inert and to co-inert pyrite cinders with a hazardous polymetallic residue such as electric arc furnace flue dusts (EAF) containing Pb, Cu, Zn, As, Cr, Ni and Mo were investigated. The effects of pyroconsolidation temperature (800-1200 degrees C), milling pyrite cinders and additions of both CaO and EAF on the resulting microstructure of the pellets were determined. The microstructural changes were then compared with the results of the standard leaching tests.