A web-based collection of genotype-phenotype associations in hereditary recurrent fevers from the Eurofever registry

PubMed ID: 29047407

Low-lying level structure of Cu56 and its implications for the rp process

The low-lying energy levels of proton-rich Cu56 have been extracted using in-beam γ-ray spectroscopy with the state-of-the-art γ-ray tracking array GRETINA in conjunction with the S800 spectrograph at the National Superconducting Cyclotron Laboratory at Michigan State University. Excited states in Cu56 serve as resonances in the Ni55(p,γ)Cu56 reaction, which is a part of the rp process in type-I x-ray bursts. To resolve existing ambiguities in the reaction Q value, a more localized isobaric multiplet mass equation (IMME) fit is used, resulting in Q=639±82 keV. We derive the first experimentally constrained thermonuclear reaction rate for Ni55(p,γ)Cu56. We find that, with this new rate, the …

Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part one

Canakinumab for the Treatment of Autoinflammatory Recurrent Fever Syndromes.

BACKGROUND: Familial Mediterranean fever, mevalonate kinase deficiency (also known as the hyperimmunoglobulinemia D syndrome), and the tumor necrosis factor receptor-associated periodic syndrome (TRAPS) are monogenic autoinflammatory diseases characterized by recurrent fever flares. METHODS: We randomly assigned patients with genetically confirmed colchicine-resistant familial Mediterranean fever, mevalonate kinase deficiency, or TRAPS at the time of a flare to receive 150 mg of canakinumab subcutaneously or placebo every 4 weeks. Patients who did not have a resolution of their flare received an add-on injection of 150 mg of canakinumab. The primary outcome was complete response (resolution…

Determining therp-Process Flow throughNi56: Resonances inCu57(p,γ)Zn58Identified with GRETINA

An approach is presented to experimentally constrain previously unreachable (p, γ) reaction rates on nuclei far from stability in the astrophysical rp process. Energies of all critical resonances in the (57)Cu(p,γ)(58)Zn reaction are deduced by populating states in (58)Zn with a (d, n) reaction in inverse kinematics at 75 MeV/u, and detecting γ-ray-recoil coincidences with the state-of-the-art γ-ray tracking array GRETINA and the S800 spectrograph at the National Superconducting Cyclotron Laboratory. The results reduce the uncertainty in the (57)Cu(p,γ) reaction rate by several orders of magnitude. The effective lifetime of (56)Ni, an important waiting point in the rp process in x-ray burst…