Systemic light-chain amyloidosis revealed by progressive nail involvement, diffuse alopecia and sicca syndrome: report of an unusual case with a review of the literature.

Immunoglobulin light-chain (AL) amyloidosis is a form of systemic amyloidosis in which the fibrils are derived from monoclonal light chains. We report a case of a 66-year-old woman presenting with nail changes, parchment-like hand changes, progressive alopecia and sicca syndrome. Histopathological studies of biopsy specimens of the scalp, the nail, minor labial salivary glands and abdominal skin revealed deposits of AL κ-type amyloid. Urine protein electrophoresis exhibited a weak band of κ-type light chains. Based on this striking case, we here review the characteristic nail and hair manifestations associated with systemic amyloidosis. Knowledge of these signs is important for an early diagnosis of systemic amyloidosis, identification of the underlying disease and patient management.

Towards a data-driven analysis of hadronic light-by-light scattering

The hadronic light-by-light contribution to the anomalous magnetic moment of the muon was recently analyzed in the framework of dispersion theory, providing a systematic formalism where all input quantities are expressed in terms of on-shell form factors and scattering amplitudes that are in principle accessible in experiment. We briefly review the main ideas behind this framework and discuss the various experimental ingredients needed for the evaluation of one- and two-pion intermediate states. In particular, we identify processes that in the absence of data for doubly-virtual pion–photon interactions can help constrain parameters in the dispersive reconstruction of the relevant input quantities, the pion transition form factor and the helicity partial waves for γ⁎γ⁎→ππ.

Dispersive approach to hadronic light-by-light scattering

Based on dispersion theory, we present a formalism for a model-independent evaluation of the hadronic light-by-light contribution to the anomalous magnetic moment of the muon. In particular, we comment on the definition of the pion pole in this framework and provide a master formula that relates the effect from ππ intermediate states to the partial waves for the process γ * γ * → ππ. All contributions are expressed in terms of on-shell form factors and scattering amplitudes, and as such amenable to an experimental determination.

Accurate evaluation of hadronic uncertainties in spin-independent WIMP-nucleon scattering: Disentangling two- and three-flavor effects

We show how to avoid unnecessary and uncontrolled assumptions usually made in the literature about soft SU(3) flavor symmetry breaking in determining the two-flavor nucleon matrix elements relevant for direct detection of weakly interacting massive particles (WIMPs). Based on SU(2) chiral perturbation theory, we provide expressions for the proton and neutron scalar couplings fp,nu and fp,nd with the pion-nucleon σ term as the only free parameter, which should be used in the analysis of direct detection experiments. This approach for the first time allows for an accurate assessment of hadronic uncertainties in spin-independent WIMP-nucleon scattering and for a reliable calculation of isospin-violating effects. We find that the traditional determinations of Vfpu−fnu and fpd−fnd are off by a factor of 2.

The multiple-scattering series in few-nucleon systems

We discuss under which circumstances the resummation of the multiple-scattering series is justified from an EFT point of view. The application to πd and K̅d scattering is briefly discussed.

Roy-Steiner equations for piN scattering

In this talk, we present a coupled system of integral equations for the πN → πN (s-channel) and ππ → N̅N (t-channel) lowest partial waves, derived from Roy–Steiner equations for pion–nucleon scattering. After giving a brief overview of this system of equations, we present the solution of the t-channel sub-problem by means of Muskhelishvili–Omnès techniques, and solve the s-channel sub-problem after finding a set of phase shifts and subthreshold parameters which satisfy the Roy–Steiner equations.

Dispersive approach to hadronic light-by-light scattering and the muon ɡ − 2

The largest uncertainties in the Standard Model calculation of the anomalous magnetic moment of the muon (ɡ − 2)μ come from hadronic contributions. In particular, it can be expected that in a few years the subleading hadronic light-by-light (HLbL) contribution will dominate the theory uncertainty. We present a dispersive description of the HLbL tensor. This new, model-independent approach opens up an avenue towards a data-driven determination of the HLbL contribution to the (ɡ − 2)μ.

Virtual photon-photon scattering

Based on analyticity, unitarity, and Lorentz invariance the contribution from hadronic vacuum polarization to the anomalous magnetic moment of the muon is directly related to the cross section of e+e− → hadrons. We review the main difficulties that impede such an approach for light-by-light scattering and identify the required ingredients from experiment. Amongst those, the most critical one is the scattering of two virtual photons into meson pairs. We analyze the analytic structure of the process γ*γ* → ππ and show that the usual Muskhelishvili–Omnès representation can be amended in such a way as to remain valid even in the presence of anomalous thresholds.

Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial

IMPORTANCE High-dose immunosuppressive therapy and autologous hematopoietic stem cell transplantation (HSCT) have shown efficacy in systemic sclerosis in phase 1 and small phase 2 trials. OBJECTIVE To compare efficacy and safety of HSCT vs 12 successive monthly intravenous pulses of cyclophosphamide. DESIGN, SETTING, AND PARTICIPANTS The Autologous Stem Cell Transplantation International Scleroderma (ASTIS) trial, a phase 3, multicenter, randomized (1:1), open-label, parallel-group, clinical trial conducted in 10 countries at 29 centers with access to a European Group for Blood and Marrow Transplantation-registered transplant facility. From March 2001 to October 2009, 156 patients with early diffuse cutaneous systemic sclerosis were recruited and followed up until October 31, 2013. INTERVENTIONS HSCT vs intravenous pulse cyclophosphamide. MAIN OUTCOMES AND MEASURES The primary end point was event-free survival, defined as time from randomization until the occurrence of death or persistent major organ failure. RESULTS A total of 156 patients were randomly assigned to receive HSCT (n = 79) or cyclophosphamide (n = 77). During a median follow-up of 5.8 years, 53 events occurred: 22 in the HSCT group (19 deaths and 3 irreversible organ failures) and 31 in the control group (23 deaths and 8 irreversible organ failures). During the first year, there were more events in the HSCT group (13 events [16.5%], including 8 treatment-related deaths) than in the control group (8 events [10.4%], with no treatment-related deaths). At 2 years, 14 events (17.7%) had occurred cumulatively in the HSCT group vs 14 events (18.2%) in the control group; at 4 years, 15 events (19%) had occurred cumulatively in the HSCT group vs 20 events (26%) in the control group. Time-varying hazard ratios (modeled with treatment × time interaction) for event-free survival were 0.35 (95% CI, 0.16-0.74) at 2 years and 0.34 (95% CI, 0.16-0.74) at 4 years. CONCLUSIONS AND RELEVANCE Among patients with early diffuse cutaneous systemic sclerosis, HSCT was associated with increased treatment-related mortality in the first year after treatment. However, HCST conferred a significant long-term event-free survival benefit. TRIAL REGISTRATION isrctn.org Identifier: ISRCTN54371254.

Mucosal Erosion of the Cricoid Cartilage After the Use of an i-Gel Supraglottic Airway Device in a Patient with Diffuse Idiopathic Skeletal Hyperostosis

After standard hip arthroplasty, an 82-year-old patient with previously undiagnosed diffuse idiopathic skeletal hyperostosis of the cervical spine experienced life-threatening side effects after use of a supraglottic airway device (i-gel). Extensive mucosal erosion and denudation of the cricoid cartilage caused postoperative supraglottic swelling and prolonged respiratory failure requiring tracheostomy. In this case report, we highlight the importance of evaluating risk factors for failure of supraglottic airway devices.

Monitoring of tumor response to Cisplatin using optical spectroscopy

INTRODUCTION Anatomic imaging alone is often inadequate for tuning systemic treatment for individual tumor response. Optically based techniques could potentially contribute to fast and objective response monitoring in personalized cancer therapy. In the present study, we evaluated the feasibility of dual-modality diffuse reflectance spectroscopy-autofluorescence spectroscopy (DRS-AFS) to monitor the effects of systemic treatment in a mouse model for hereditary breast cancer. METHODS Brca1(-/-); p53(-/-) mammary tumors were grown in 36 mice, half of which were treated with a single dose of cisplatin. Changes in the tumor physiology and morphology were measured for a period of 1 week using dual-modality DRS-AFS. Liver and muscle tissues were also measured to distinguish tumor-specific alterations from systemic changes. Model-based analyses were used to derive different optical parameters like the scattering and absorption coefficients, as well as sources of intrinsic fluorescence. Histopathologic analysis was performed for cross-validation with trends in optically based parameters. RESULTS Treated tumors showed a significant decrease in Mie-scattering slope and Mie-to-total scattering fraction and an increase in both fat volume fraction and tissue oxygenation after 2 days of follow-up. Additionally, significant tumor-specific changes in the fluorescence spectra were seen. These longitudinal trends were consistent with changes observed in the histopathologic analysis, such as vital tumor content and formation of fibrosis. CONCLUSIONS This study demonstrates that dual-modality DRS-AFS provides quantitative functional information that corresponds well with the degree of pathologic response. DRS-AFS, in conjunction with other imaging modalities, could be used to optimize systemic cancer treatment on the basis of early individual tumor response.

First πK atom lifetime and πK scattering length measurements

The results of a search for hydrogen-like atoms consisting of π∓K±π∓K± mesons are presented. Evidence for πK atom production by 24 GeV/c protons from CERN PS interacting with a nickel target has been seen in terms of characteristic πK pairs from their breakup in the same target (178±49178±49) as well as in terms of produced πK atoms (653±42653±42). Using these results, the analysis yields a first value for the πK atom lifetime of View the MathML sourceτ=(2.5−1.8+3.0) fs and a first measurement of the S-wave isospin-odd πK scattering length View the MathML source|a0−|=13|a1/2−a3/2|=(0.11−0.04+0.09)Mπ−1 (aIaI for isospin I).

Measurement of the total cross section from elastic scattering in pp collisions at √s=7TeV with the ATLAS detector

A measurement of the total pp cross section at the LHC at √s = 7 TeV is presented. In a special run with high-β* beam optics, an integrated luminosity of 80 μb−1 was accumulated in order to measure the differential elastic cross section as a function of the Mandelstam momentum transfer variable t . The measurement is performed with the ALFA sub-detector of ATLAS. Using a fit to the differential elastic cross section in the |t | range from 0.01 GeV2 to 0.1 GeV2 to extrapolate to |t | →0, the total cross section, σtot(pp→X), is measured via the optical theorem to be: σtot(pp→X) = 95.35± 0.38 (stat.)± 1.25 (exp.)± 0.37 (extr.) mb, where the first error is statistical, the second accounts for all experimental systematic uncertainties and the last is related to uncertainties in the extrapolation to |t | → 0. In addition, the slope of the elastic cross section at small |t | is determined to be B = 19.73 ±0.14 (stat.) ±0.26 (syst.) GeV−2.