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Background: New biomarkers are necessary to improve detection of the risk of infection in heart transplantation. We performed a multicenter study to evaluate humoral immunity profiles that could better enable us to identify heart recipients at risk of severe infections., Methods: We prospectively analyzed 170 adult heart recipients at 8 centers in Spain. Study points were before transplantation and 7 and 30 days after transplantation. Immune parameters included IgG, IgM, IgA and complement factors C3 and C4, and titers of specific antibody to pneumococcal polysaccharide antigens (anti-PPS) and to cytomegalovirus (CMV). To evaluate potential immunologic mechanisms leading to IgG hypogammaglobulinemia, before heart transplantation we assessed serum B-cell activating factor (BAFF) levels using enzyme-linked immunoassay. The clinical follow-up period lasted 6 months. Clinical outcome was need for intravenous anti-microbials for therapy of infection., Results: During follow-up, 53 patients (31.2%) developed at least 1 severe infection. We confirmed that IgG hypogammaglobulinemia at Day 7 (defined as IgG <600 mg/dl) is a risk factor for infection in general, bacterial infections in particular, and CMV disease. At Day 7 after transplantation, the combination of IgG <600 mg/dl + C3 <80 mg/dl was more strongly associated with the outcome (adjusted odds ratio 7.40; 95% confidence interval 1.48 to 37.03; p = 0.014). We found that quantification of anti-CMV antibody titers and lower anti-PPS antibody concentrations were independent predictors of CMV disease and bacterial infections, respectively. Higher pre-transplant BAFF levels were a risk factor of acute cellular rejection., Conclusion: Early immunologic monitoring of humoral immunity profiles proved useful for the identification of heart recipients who are at risk of severe infection., (Copyright © 2017 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Complement Factor I (CFI) is a serine protease with an important role in complement alternative pathway regulation. Complete factor I deficiency is strongly associated with severe infections. Approximately 30 families with this deficiency have been described worldwide., Patients and Methods: We have studied five new Spanish families suffering from CFI deficiency. From 19 screened people, 7 homozygous, 10 heterozygous and 2 healthy subjects were identified. Clinical, biochemical and genetic descriptions are included., Results: Molecular studies demonstrated 4 novel mutations in the screened individuals; amongst them, we describe here the first great gene deletion reported in the CFI locus, which includes full exon 2 and part of the large intron 1., Conclusion: CFI deficiency is possibly an underestimated defect and the eventual existence of this deficiency should be tested in those patients exhibiting low C3 and recurrent bacterial infections. We propose a simple diagnostic flowchart to help clinicians in the identification and correct diagnosis of such patients.

Introduction: Patients with autoimmune diseases may suffer from hoarseness and voice disorders because of anatomical and functional alterations., Objectives: To assess the prevalence of dysphonia in rheumatic patients and its impact on their quality of life (QOL). To analyze the association of voice disorders in patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and Sjögren syndrome (SS). To determine if voice disorders during the acute phase of these diseases are correlated with specific biochemical parameters., Methods: Present an observational and transverse analytic study of 140 patients selected from February 2009 to January 2010. The subjects (80) were diagnosed with RA (44), SLE (32), and SS (4), and the control group (60) presented no voice disorders or rheumatic diseases. Patients were evaluated using the Voice Handicap Index (VHI) and a three items outcome scale (TIOS). A biochemical and C-reactive protein study was performed on 40 patients with a disease outbreak, measuring their complement, sedimentation velocity (VSG), and anti-DNA antibodies., Results: The prevalence of dysphonia among the subjects was 32-38% as opposed to 5-8% in the control group. The rheumatic patients presented an odds ratio (OR) for dysphonia of 2.82 (VHI) and 5.04 (TIOS) when compared with healthy individuals (P<0.05). We found statistically significant differences in functional, physical, occupational, and emotional subscales of these tests. No significant differences were found when studying the biochemical parameters. A higher incidence of voice disorders (OR=3.07) was associated with SLE, followed by RA (OR=2.8; 95% CI)., Conclusions: Systemic immunomediated diseases may associate voice disorders. Patients with SLE are those who develop these disorders more frequently. The biochemical parameters most affected during a crisis are VSG and anti-DNA antibodies., (Copyright © 2012 The Voice Foundation. Published by Mosby, Inc. All rights reserved.)

Objectives: To determine serum concentrations of proinflammatory (C reactive protein, complement C3 and C4) and anti-inflammatory (alpha(1) antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite cyclists before and during a three week cycle tour., Methods: Seventeen professional cyclists participating in the Vuelta a Espańa volunteered for the study. Their mean (SD) physical characteristics were: age 28 (1) years; height 1.7 (0.06) m; weight 65 (7) kg; body fat 7.6 (0.8)%; Vo(2)max 75.3 (2.3) ml/kg/min. Venepuncture was performed on each subject 24 hours before the tour began (T0), on day 11 (the first rest day; T1) and day 21 (the second to last stage of the tour; T2). Samples at T1 and T2 were taken about 17 hours after the previous stage. Analysis of variance was used to determine changes over time. Where significance was found, a Tukey post hoc test was performed., Results: C reactive protein concentrations were consistently within the normal range, although there was a 228%, non-significant increase at T1. C3 concentrations fell within the normal range at all times assessed. C4 concentrations before the race were within the normal range and were significantly increased 10 days (T1) into the race. C1-INH concentrations did not change significantly throughout the race. alpha(1) Antitrypsin concentration before the race was at the lower end of the normal range and was only significantly raised at T2., Conclusions: Although not as pronounced as those reported in marathon/ultramarathon runners, elite cyclists participating in a three week cycle tour experienced increases in selected proinflammatory and anti-inflammatory acute phase proteins, indicating an acute phase/inflammatory response. It is tenable that the increase in alpha(1) antitrypsin and C1-INH (anti-inflammatory mediators) at T2 served to attenuate the acute phase/inflammatory response. The lower than normal resting concentrations of the acute phase proteins supports the notion that chronic aerobic exercise induces an anti-inflammatory state.