Intravenous Immunoglobulins Tapering and Withdrawal in Systemic Capillary Leak Syndrome (Clarkson Disease).

Background: The systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a very rare condition characterized by recurrent life-threatening episodes of vascular hyperpermeability in the presence of a monoclonal gammopathy. Extended intravenous immunoglobulin (IVIG) treatment is associated with fewer recurrences and improved survival, but the optimal treatment dosage and duration remain unknown.

Objective: We aim to evaluate the safety of IVIG tapering and withdrawal in patients with SCLS.

Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome.

Background: Monoclonal gammopathy-associated systemic capillary-leak syndrome, also known as Clarkson disease, is a rare condition characterized by recurrent life-threatening episodes of capillary hyperpermeability in the context of a monoclonal gammopathy. This study was conducted to better describe the clinical characteristics, natural history, and long-term outcome of monoclonal gammopathy-associated systemic capillary-leak syndrome.

Methods: We conducted a cohort analysis of all patients included in the European Clarkson disease (EurêClark) registry between January 1997 and March 2016.

Growth and structure of ultrathin alumina films on the (1 1 0) surface of γ-Al4Cu9 complex metallic alloy.

The first stages of oxidation of the (1 1 0) surface of a γ-Al(4)Cu(9) complex metallic alloy were investigated by combining x-ray photoemission spectroscopy, low energy electron diffraction and scanning tunnel microscopy studies. Oxidation at room temperature in the 2 × 10(-8) to 2 × 10(-7) mbar oxygen pressure range occurs in two steps: a fast regime is followed by a much slower one, leading to the formation of a thin aluminium oxide film showing no long range order. Cu-O bonds are never observed, due to fast oxygen induced aluminium segregation.

Transition from molecule to solid state: reactivity of supported metal clusters.

The evolution of the adsorption energy of carbon monoxide (CO) molecules on palladium (Pd) clusters as function of Pd particle size from the molecular regime (less than ~100 atoms per particle) to the bulk regime has been revealed. This adsorption energy is retrieved from the residence time of CO molecules on the Pd clusters, measured by a pulsed molecular beam technique, versus temperature. Unprecedented accuracy on the determination of the particle size has been achieved here by using a regular array of metal clusters exhibiting a size dispersion down to the ultimate limit of a Poisson distribution.

Structural and compositional characterization of ultrathin titanium oxide films grown on Pt3Ti(111).

We have investigated the growth of ultrathin titanium oxide (TiO(x)) films on a Pt(3)Ti(111) single crystal surface as a function of oxidation temperature (300-1000 K) and oxygen exposure (up to 4500 l) by means of Auger electron spectroscopy, low-energy electron diffraction, ultraviolet photoelectron spectroscopy and high-resolution electron energy loss spectroscopy (HREELS). Both the surface composition and the surface structure of the resulting TiO(x) films exhibit a strong dependence on the preparation conditions. Loss of the chemical order and Ti segregation are observed at the Pt(3)Ti(111) surface upon oxygen exposures of more than 135 l at 1000 K.