Systemic-onset juvenile idiopathic arthritis complicated by early onset amyloidosis in a patient carrying a mutation in the <Emphasis Type="Italic">MEFV</Emphasis> gene

Systemic juvenile idiopathic arthritis (SJIA) is a disorder characterized by arthritis in children starting before 16 years of age associated with daily high fever, persisting for more than 2 weeks, and at least one of the following clinical features: evanescent cutaneous rash, lymphadenopathy, serositis or hepatosplenomegaly. SJIA patients carry a significantly higher frequency of MEFV mutations, the gene responsible for familial Mediterranean fever, and may be characterized by a more aggressive disease. In this line, we describe a 9-year-old girl affected with SJIA who carried a heterozygous G196W mutation in MEFV. Our patient was characterized by an aggressive disease course, resistance to conventional immunosuppressive agents and developed renal amyloidosis just 2 years after the disease onset.     

Stress amplifies lung tissue mechanics, inflammation and oxidative stress induced by chronic inflammation

ABSTRACT Background: Mechanisms linking behavioral stress and inflammation are poorly understood, mainly in distal lung tissue. Objective: We have investigated whether the forced swim stress (FS) could modulate lung tissue mechanics, iNOS, cytokines, oxidative stress activation, eosinophilic recruitment, and remodeling in guinea pigs (GP) with chronic pulmonary inflammation. Methods: The GP were exposed to ovalbumin or saline aerosols (2×/wk/4wks, OVA, and SAL). Twenty-four hours after the 4th inhalation, the GP were submitted to the FS protocol (5×/wk/2wks, SAL-S, and OVA-S). Seventy-two hours after the 7th inhalation, lung strips were cut and tissue resistance (Rt) and elastance (Et) were obtained (at baseline and after OVA and Ach challenge). Strips were submitted to histopathological evaluation. Results: The adrenals' weight, the serum cortisol, and the catecholamines were measured. There was an increase in IL-2, IL-5, IL-13, IFN-γ, iNOS, 8-iso-PGF2α, and in %Rt and %Et after Ach challenge in the SAL-S group compared to the SAL one. The OVA-S group has had an increase in %Rt and %Et after the OVA challenge, in %Et after the Ach and in IL-4, 8-iso-PGF2α, and actin compared to the OVA. Adrenal weight and cortisol serum were increased in stressed animals compared to nonstressed ones, and the catecholamines were unaltered. Conclusion & clinical relevance: Repeated stress has increased distal lung constriction, which was associated with an increase of actin, IL-4, and 8-iso-PGF2α levels. Stress has also induced an activation of iNOS, cytokines, and oxidative stress pathways.     

Motor imagery as a complementary technique for functional recovery after stroke: a systematic review

Objective: To evaluate factors associated with 1-year mortality after discharge for acute stroke.

Methods: In this retrospective cohort study, we studied 305 patients with ischemic stroke or intracerebral hemorrhage discharged in 2010/2011. We linked Get With The Guidelines®-Stroke clinical data with New York State administrative data and used multivariate regression models to examine variables related to 1-year all-cause mortality poststroke.

Results: The mean age was 68.6 ± 14.8 years and 51.1% were women. A total of 146 (47.9%) were discharged directly home, 96 (31.5%) to inpatient rehabilitation facilities (IRFs), and 63 (20.7%) to skilled nursing facilities (SNFs). Overall, 24 (7.9%) patients died within 1-year post-discharge. Older age (adjusted odds ratio [OR] 1.05, 95% confidence interval [CI] 1.00–1.10), higher National Institutes of Health Stroke Scale (NIHSS) on admission (OR 1.10, 95% CI 1.03–1.17), and discharge destination (IRF vs. home, OR 0.10, 95% CI 0.01–0.94; and SNF vs. home, OR 2.22, 95% CI 0.71–6.95) were factors associated with 1-year all-cause mortality. When ambulation status at discharge was added to the model, ambulation with assistance and non-ambulation were significantly associated with mortality (ambulatory with assistance vs. ambulatory, OR 9.42, 95% CI 1.87–47.61; nonambulatory vs. ambulatory, OR 12.65, 95% CI 1.89–84.89).

Conclusions: While age and NIHSS on admission are important predictors of long-term outcomes, factors at discharge – ambulation status at discharge and discharge destination – are associated with 1-year mortality post-discharge for acute stroke and therefore could represent therapeutic targets to improve long-term survival in future studies.     

Mechanical and morphological aspects of the calcaneal tendon of mdx mice at 21 days of age

A relationship between compromised muscles and other tissues has been demonstrated in mdx mouse, an animal model studied for understanding of Duchenne muscular dystrophy. The hypothesis is that changes in the calcaneal tendon of mdx mice occur previous to the onset of rigorous and most marked episodes of muscle degeneration, which start suddenly after 21 days of life. Thus, this study aimed to identify possible alterations in the calcaneal tendon of mdx mouse at 21 days of age. Control and mdx tendons were submitted to mechanical tensile testing, quantification of hydroxyproline, and staining with toluidine blue and picrosirius red. Hydroxyproline content was similar between mdx and control groups. The control tendon presented higher mechanical strength (load, stress, and elastic modulus) and its morphological analysis showed a larger number of round fibroblasts, nuclei with well‐decondensed chromatin, and slightly metachromatic well‐stained cytoplasmic material, different from that observed in mdx tendons. The results suggest that the absence of dystrophin in mdx mouse can provoke directly or indirectly alterations in the mechanical properties and morphology of the calcaneal tendon. Anat Rec, 296:1546–1551, 2013. © 2013 Wiley Periodicals, Inc.     

Biofilm formation on denture liners in a randomised controlled in situ trial

ObjectivesThis randomised clinical trial assessed how biofilm development and composition is affected by time and denture material type in denture wearers with and without denture stomatitis.MethodsSpecimens of acrylic resin (control) and denture liners (silicone-based or acrylic resin based, depending on the experimental phase) were inserted into the surface intaglio of 30 denture wearers. Biofilm was formed in two phases of 21 days, and counts of viable micro-organisms in the accumulating biofilm were determined after 7, 14 and 21days of biofilm formation. Data were analysed by three-way ANOVA followed by Tukey test to assess differences among health condition (healthy or with denture stomatitis), materials and time point.ResultsNon-albicans Candida species counts were higher in diseased patients with silicone-based denture liners (p = 0.01). Denture stomatitis patients showed higher mutans streptococci counts after 7 days (p = 0.0041).ConclusionsLonger biofilm formation time periods did not result in differences on biofilm composition. The denture liners evaluated in this study accumulate greater amount of biofilm, and therefore their use should be carefully planned.Clinical significanceThe silicone-based denture liner tested should be used cautiously in patients with denture stomatitis as it showed increased non-albicans species counts, known to be difficult to treat.