| 1. |
- Egg, David, et al.
(författare)
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Therapeutic options for CTLA-4 insufficiency
- 2022
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Ingår i: Journal of Allergy and Clinical Immunology. - : MOSBY-ELSEVIER. - 0091-6749 .- 1097-6825. ; 149:2, s. 736-746
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Tidskriftsartikel (refereegranskat)abstract
- Background: Heterozygous germline mutations in cytotoxic T lymphocyte-associated antigen-4 (CTLA4) impair the immunomodulatory function of regulatory T cells. Affected individuals are prone to life-threatening autoimmune and lymphoproliferative complications. A number of therapeutic options are currently being used with variable effectiveness. Objective: Our aim was to characterize the responsiveness of patients with CTLA-4 insufficiency to specific therapies and provide recommendations for the diagnostic workup and therapy at an organ-specific level. Methods: Clinical features, laboratory findings, and response to treatment were reviewed retrospectively in an international cohort of 173 carriers of CTLA4 mutation. Patients were followed between 2014 and 2020 for a total of 2624 months from diagnosis. Clinical manifestations were grouped on the basis of organ-specific involvement. Medication use and response were recorded and evaluated. Results: Among the 173 CTLA4 mutation carriers, 123 (71%) had been treated for immune complications. Abatacept, rituximab, sirolimus, and corticosteroids ameliorated disease severity, especially in cases of cytopenias and lymphocytic organ infiltration of the gut, lungs, and central nervous system. Immunoglobulin replacement was effective in prevention of infection. Only 4 of 16 patients (25%) with cytopenia who underwent splenectomy had a sustained clinical response. Cure was achieved with stem cell transplantation in 13 of 18 patients (72%). As a result of the aforementioned methods, organ-specific treatment pathways were developed. Conclusion: Systemic immunosuppressants and abatacept may provide partial control but require ongoing administration. Allogeneic hematopoietic stem cell transplantation offers a possible cure for patients with CTLA-4 insufficiency.
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| 2. |
- Prinoth, Bibiana, et al.
(författare)
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Titanium oxide and chemical inhomogeneity in the atmosphere of the exoplanet WASP-189 b
- 2022
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Ingår i: Nature Astronomy. - : Springer Science and Business Media LLC. - 2397-3366. ; 6:4, s. 449-457
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Tidskriftsartikel (refereegranskat)abstract
- The temperature of an atmosphere decreases with increasing altitude, unless a shortwave absorber that causes a temperature inversion exists. Ozone plays this role in the Earth’s atmosphere. In the atmospheres of highly irradiated exoplanets, the shortwave absorbers are predicted to be titanium oxide (TiO) and vanadium oxide (VO). Detections of TiO and VO have been claimed using both low-, and high- spectral-resolution observations, but subsequent observations have failed to confirm these claims or overturned them. Here we report the unambiguous detection of TiO in the ultra-hot Jupiter WASP-189 b using high-resolution transmission spectroscopy. This detection is based on applying the cross-correlation technique to many spectral lines of TiO from 460 to 690 nm. Moreover, we report detections of metals, including neutral and singly ionized iron and titanium, as well as chromium, magnesium, vanadium and manganese (Fe, Fe+, Ti, Ti+, Cr, Mg, V, Mn). The line positions of the detected species differ, which we interpret as a consequence of spatial gradients in their chemical abundances, such that they exist in different regions or dynamical regimes. This is direct observational evidence for the three-dimensional thermochemical stratification of an exoplanet atmosphere derived from high-resolution ground-based spectroscopy.
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| 3. |
- Temperton, Robert H., et al.
(författare)
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Dip-and-pull ambient pressure photoelectron spectroscopy as a spectroelectrochemistry tool for probing molecular redox processes
- 2022
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Ingår i: Journal of Chemical Physics. - : AIP Publishing. - 0021-9606 .- 1089-7690. ; 157:24
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Tidskriftsartikel (refereegranskat)abstract
- Ambient pressure x-ray photoelectron spectroscopy (APXPS) can provide a compelling platform for studying an analyte's oxidation and reduction reactions in solutions. This paper presents proof-of-principle operando measurements of a model organometallic complex, iron hexacyanide, in an aqueous solution using the dip-and-pull technique. The data demonstrates that the electrochemically active liquid meniscuses on the working electrodes can undergo controlled redox reactions which were observed using APXPS. A detailed discussion of several critical experimental considerations is included as guidance for anyone undertaking comparable experiments.
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