Promoting adaptive behaviors by two girls with Rett syndrome through a microswitch-based program

We assessed a microswitch-based program to improve self-determination to access to preferred stimuli and to foster locomotor behavior by two girls with Rett syndrome and multiple disabilities. To enhance the first behavior (access to preferred stimuli) a wobble microswitch (sensitive touch sensor) was used while for the second behavior (step responses) optic sensors were applied. A second aim of the study was to monitor indices of happiness as consequence of the use of assistive technology. Finally, a third objective of the study was the reduction of hand washing and body rocking related stereotypies. The study was carried out according to a multiple probe design across behaviors for both participants, where the two behaviors were first learned independently, then combined together. Results showed an increasing of performance and of indices of happiness and a decreasing of stereotyped behaviors for both participants during intervention phases. Practical, psychological and clinical implications of the findings are discussed.     

Effectiveness of Periodontal Treatment to Improve Metabolic Control in Patients With Chronic Periodontitis and Type 2 Diabetes: A Meta‐Analysis of Randomized Clinical Trials

Background: It was recently suggested that scaling and root planing (SRP) may help to improve glycemic and metabolic control in patients with chronic periodontitis (CP) and type 2 diabetes mellitus (DM2); however, the effectiveness of SRP in this role remains unclear. This meta‐analysis assesses the effectiveness of SRP in improving glycemic and metabolic control in patients with CP and DM2. Methods: A literature search of electronic databases was performed for articles published through May 16, 2012, followed by a manual search of several dental journals. A meta‐analysis was conducted according to the recommendations of the Cochrane Collaboration and the Preferred Reporting Items for Systematic Reviews and Meta‐Analyses (PRISMA). Weighted mean differences (MDs) and 95% confidence intervals (CIs) were calculated for glycated hemoglobin A1c (HbA1c), fasting plasma glucose (FPG), total cholesterol (TC), triglycerides (TG), and high‐ and low‐density lipoprotein cholesterol (HDL and LDL, respectively). All outcomes were evaluated as changes from baseline to the end of follow‐up. Heterogeneity was assessed with the χ²‐based Cochran Q test and I² statistic. The level of significance was set at P < 0.05. Results: After the study selection process, five randomized clinical trials were included. Results of the meta‐analysis indicated that SRP was effective in the reduction of HbA1c (MD = 0.65; 95% CI 0.43 to 0.88; P <0.05) and FPG (MD = 9.04; 95% CI 2.17 to 15.9; P <0.05), but no significant differences were found in the reduction of TC, TG, HDL, or LDL. No evidence of heterogeneity was detected. Conclusion: The meta‐analysis results seem to support the effectiveness of SRP in the improvement of glycemic control in patients with CP and DM2; however, future studies are needed to confirm these results.     

Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study

The natural history of idiopathic pulmonary fibrosis (IPF) is not well defined and its clinical course is variable. We sought to investigate the survival and incidence of acute exacerbations (AEs) and their significant predictors in newly diagnosed patients. 70 patients newly diagnosed with IPF were prospectively followed for at least 3 yrs. Baseline evaluation included Medical Research Council dyspnoea score (MRCDS), 6-min walk test, pulmonary function tests, all of which were repeated at 6 months, and high-resolution computed tomography. A retrospective cohort of 68 patients was used for confirmation. Mean survival from the time of diagnosis was 30 months, with a 3-yr mortality of 46%. A Risk stratificatiOn ScorE (ROSE) based on MRCDS > 3, 6-min walking distance ≤ 72% predicted and composite physiologic index > 41 predicted 3-yr mortality with high specificity. 6-month progression of ROSE predicted rapid progression. 3-yr incidence of AE was 18.6%, mostly occurring in the first 18 months; risk factors for AE were concomitant emphysema and low diffusing coefficient of the lung for carbon monoxide. Results were confirmed in an independent cohort of patients. In newly diagnosed IPF, advanced disease at presentation, rapid progression and AEs are the determinants of 3-yr survival. The purpose of the multifactorial ROSE is to risk-stratify patients in order to predict survival and detect rapid disease progression.     

Myocardial infarction in two cousins heterozygous for ASN41HIS autosomal dominant variant of Bernard–Soulier syndrome

Bernard?CSoulier Syndrome is characterized by thrombocytopenia with large platelets and defective aggregation to ristocetin. The bleeding tendency is variable but may be severe. The syndrome is due to genetic defects of the GPIb-V-IX complex and it has been maintained to be protective from thrombotic events. Here we present the first two cases of documented M.I. in two cousins, heterozygous for the Arg41His mutation which is responsible for a dominant form of Bernard?CSoulier Syndrome. In one of the two patients an aneurysm of the aorta was also present. The patients had a mild bleeding tendency which was severely aggravated by treatment with antiplatelet drugs. These clinical observations are in contrast with experimental studies which demonstrate that Bernard?CSoulier-like strains of mice show a decreased thrombus generation in several experimental settings.