Role of project manager during the construction process of the project Rekonstrukce silnice II/115 Černošice

Diplomová práce se věnuje podrobnému popisu činností, které vykonává manažer stavby při realizaci liniové stavby II/115 Černošice, rekonstrukce silnice. Práce popisuje nejen funkce manažera stavby, ale také společnost, která jako generální zhotovitel stavbu vykonává, a nechybí ani popis konkrétního projektu včetně postupu při výběrovém řízení. Druhá část je zaměřena na popis změn během výstavby a přesný postup toho, jak jsou procesovány tyto nepředvídatelné skutečnosti dle zákona o veřejných zakázkách. Vše je reflektováno na konkrétních příkladech.This thesis describes in detail all working operations and actions, that the manager has to execute while supervising the line construction II/115 - the road restoration in Černošice. It not only calls out all the duties of the manager, but also takes a deeper look at the general construction company, which manages the project. A more specific description of the particular project and a tender (selection process) timeline is also included in this thesis. The second part of this document focuses on changes that had to be made during the construction and the way these unpredictable circumstances are usually being handled according to the Public Procurement Act, shown on specific case studies

Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children

International audienceMultisystem inflammatory syndrome in children (MIS-C) is a rare and severe condition that follows benign COVID-19. We report autosomal recessive deficiencies of OAS1 , OAS2 , or RNASEL in five unrelated children with MIS-C. The cytosolic double-stranded RNA (dsRNA)–sensing OAS1 and OAS2 generate 2′-5′-linked oligoadenylates (2-5A) that activate the single-stranded RNA–degrading ribonuclease L (RNase L). Monocytic cell lines and primary myeloid cells with OAS1, OAS2, or RNase L deficiencies produce excessive amounts of inflammatory cytokines upon dsRNA or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) stimulation. Exogenous 2-5A suppresses cytokine production in OAS1-deficient but not RNase L–deficient cells. Cytokine production in RNase L–deficient cells is impaired by MDA5 or RIG-I deficiency and abolished by mitochondrial antiviral-signaling protein (MAVS) deficiency. Recessive OAS–RNase L deficiencies in these patients unleash the production of SARS-CoV-2–triggered, MAVS-mediated inflammatory cytokines by mononuclear phagocytes, thereby underlying MIS-C