Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich's syndrome): frequency, clinical implication and prognosis

BACKGROUND: Episodic Angioedema with eosinophilia (EAE, Gleich\u27s syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M. METHODS: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France. RESULTS: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone. CONCLUSION: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare

Efficacy and Safety of Lacosamide in Painful Diabetic Neuropathy

OBJECTIVE: To evaluate efficacy and safety of lacosamide compared with placebo in painful diabetic polyneuropathy. RESEARCH DESIGN AND METHODS: Diabetic patients with at least moderate neuropathic pain were randomized to placebo or lacosamide 400 (in a slow or standard titration) or 600 mg/day over 6-week titration and 12-week maintenance periods. Primary efficacy criterion was intra-individual change in average daily Numeric Pain Rating Scale score from baseline to the last 4 weeks. RESULTS: For the primary end point, pain reduction was numerically but not statistically greater with lacosamide compared with placebo (400 mg/day, P = 0.12; 600 mg/day, P = 0.18). Both doses were significantly more effective compared with placebo over the titration (P = 0.03, P = 0.006), maintenance (P = 0.01, P = 0.005), and entire treatment periods (P = 0.03, P = 0.02). Safety profiles between titration schemes were similar. CONCLUSIONS: Lacosamide reduced neuropathic pain and was well tolerated in diabetic patients, but the primary efficacy criterion was not met, possibly due to an increased placebo response over the last 4 weeks.status: publishe

Trophic ulcers in the carpal tunnel syndrome

A patient with carpal tunnel syndrome (CTS) and trophic ulcers is described. Despite the healing of the ulcers after surgery for CTS, the severe sensory deficit and the electrophysiological tests have not shown any significant improvement. We think these findings argue against the hypothesis of the sensory deficit being responsible for the trophic ulcers. We favor a major role for the sympathetic disturbances as the main cause for those lesions

Assessment of systems codes and their coupling with CFD codes in thermal-hydraulic applications to innovative reactors

International audienceThe THINS project of the 7th Framework EU Program on nuclear fission safety is devoted to the investigation of crosscutting thermal-hydraulic issues for innovative nuclear systems. A significant effort in the project has been dedicated to the qualification and validation of system codes currently employed in thermal-hydraulic transient analysis for nuclear reactors. This assessment is based either on already available experimental data, or on the data provided by test campaigns carried out in the frame of THINS project activities. Data provided by TALL and CIRCE facilities were used in the assessment of system codes for HLM reactors, while the PHENIX ultimate natural circulation test was used as reference for a benchmark exercise among system codes for sodium-cooled reactor applications. In addition, a promising grid-free pool model based on proper orthogonal decomposition is proposed to overcome the limits shown by the thermal-hydraulic system codes in the simulation of pool-type systems. Furthermore, multi-scale system-CFD solutions have been developed and validated for innovative nuclear system applications. For this purpose, data from the PHENIX experiments have been used, and data are provided by the tests conducted with new configuration of the TALL-3D facility, which accommodates a 3D test section within the primary circuit. The TALL-3D measurements are currently used for the validation of the coupling between system and CFD codes. © 2014 Elsevier B.V. All rights reserved