Associated factors to serious infections in a large cohort of juvenile-onset systemic lupus erythematosus from Lupus Registry (RELESSER).

Objective: To assess the incidence of serious infection (SI) and associated factors in a large juvenile-onset systemic lupus erythematosus (jSLE) retrospective cohort. Methods: All patients in the Spanish Rheumatology Society Lupus Registry (RELESSER) who meet =4 ACR-97 SLE criteria and disease onset <18 years old (jSLE), were retrospectively investigated for SI (defined as either the need for hospitalization with antibacterial therapy for a potentially fatal infection or death caused by the infection). Standardized SI rate was calculated per 100 patient years. Patients with and without SI were compared. Bivariate and multivariate logistic and Cox regression models were built to calculate associated factors to SI and relative risks. Results: A total of 353 jSLE patients were included: 88.7% female, 14.3 years (± 2.9) of age at diagnosis, 16.0 years (± 9.3) of disease duration and 31.5 years (±10.5) at end of follow-up. A total of 104 (29.5%) patients suffered 205 SI (1, 55.8%; 2-5, 38.4%; and =6, 5.8%). Incidence rate was 3.7 (95%CI: 3.2–4.2) SI per 100 patient years. Respiratory location and bacterial infections were the most frequent. Higher number of SLE classification criteria, SLICC/ACR DI score and immunosuppressants use were associated to the presence of SI. Associated factors to shorter time to first infection were higher number of SLE criteria, splenectomy and immunosuppressants use. Conclusions: The risk of SI in jSLE patients is significant and higher than aSLE. It is associated to higher number of SLE criteria, damage accrual, some immunosuppressants and splenectomy

The 7 June 2007 mbLg 4.2 escopete earthquake : An event with significant ground motion in a stable zone (Central Iberian Peninsula)

An earthquake occurred on 7 June 2007 at 01h:42m:09.5s (UTC) with geographical coordinates 40°.41N, 2°.98W and magnitude mbLg = 4.2 according to the Seismic national network (RSN) of Madrid's Instituto Geográfico Nacional (IGN). The epicenter was located close to the town of Escopete, where the earthquake was felt with intensity IV, waking up inhabitants and causing widespread alarm. Historically, rare but similar events have been felt in the area, near the towns of Pastrana (1922) and Duron (1982). Nevertheless, this part of Iberia experiences very low seismic activity. The earthquake with the largest magnitude recorded to date by the RSN since the mid-1920s was 4.1. Due to low seismicity values, the hazard map of the Norma de la Construcción Sismorresistente Española (Spanish seismic building code; NCSE-02) establishes a basic acceleration value of less than 0.04 g, which is the threshold value for the application of the earthquake-resistant building code. However, this value was exceeded in strong-motion recordings during the 2007 event. An important consideration is the existence of two nuclear power plants in the Guadalajara administrative province: José Cabrera and Trillo. Strong-motion instruments at the José Cabrera nuclear power plant (JCNPP) recorded a peak ground acceleration (PGA) value of 0.07g. This is the first acceleration recording made from central Iberia and also one of the highest values read from instruments to date for the whole of the Iberian peninsula. This paper presents an overview of the results of our multidisciplinary analysis of the earthquake, which we researched in terms of its regional and local tectonic setting, local seismicity, focal mechanics, strong-motion records, and macroseismic effects.Universidad Complutense de MadridMinisterio de Educación y CienciaDepto. de Geodinámica, Estratigrafía y PaleontologíaFac. de Ciencias GeológicasTRUEpu

Natural History and Risk Stratification in Andersen-Tawil Syndrome Type 1

Background: Andersen-Tawil Syndrome type 1 (ATS1) is a rare arrhythmogenic disorder, caused by loss-of-function mutations in the KCNJ2 gene. We present here the largest cohort of patients with ATS1 with outcome data reported. Objectives: This study sought to define the risk of life-threatening arrhythmic events (LAE), identify predictors of such events, and define the efficacy of antiarrhythmic therapy in patients with ATS1. Methods: Clinical and genetic data from consecutive patients with ATS1 from 23 centers were entered in a database implemented at ICS Maugeri in Pavia, Italy, and pooled for analysis. Results: We enrolled 118 patients with ATS1 from 57 families (age 23 ± 17 years at enrollment). Over a median follow-up of 6.2 years (interquartile range: 2.7 to 16.5 years), 17 patients experienced a first LAE, with a cumulative probability of 7.9% at 5 years. An increased risk of LAE was associated with a history of syncope (hazard ratio [HR]: 4.54; p = 0.02), with the documentation of sustained ventricular tachycardia (HR 9.34; p = 0.001) and with the administration of amiodarone (HR: 268; p &lt; 0.001). The rate of LAE without therapy (1.24 per 100 person-years [py]) was not reduced by beta-blockers alone (1.37 per 100 py; p = 1.00), or in combination with Class Ic antiarrhythmic drugs (1.46 per 100 py, p = 1.00). Conclusions: Our data demonstrate that the clinical course of patients with ATS1 is characterized by a high rate of LAE. A history of unexplained syncope or of documented sustained ventricular tachycardia is associated with a higher risk of LAE. Amiodarone is proarrhythmic and should be avoided in patients with ATS1. © 202