von Willebrand disease "Vicenza" with larger-than-normal (supranormal) von Willebrand factor multimers

When normal volunteers or patients with type I von Willebrand disease (VWD) are given desmopressin (DDAVP), a set of larger-than-normal (supranormal) von Willebrand factor (VWF) multimers, similar to those present in VWF-containing cells such as platelets megakaryocytes and endothelial cells, appear transiently in postinfusion plasma. In two kindreds with mild lifelong bleeding symptoms transmitted as an autosomal dominant trait, all ten symptomatic members (but none of the five asymptomatic members) had a supranormal multimeric structure for plasma VWF, apparently identical to that seen for postdesmopressin normal plasma. Plasma factor VIII coagulant activity (VIII:C), VWF antigen (VWF:Ag), ristocetin-induced platelet agglutination, and ristocetin cofactor (RiCof) activity were low. Platelet VWF:Ag and RiCof levels (tested for three patients only) were normal. Bleeding times were normal or slightly prolonged. The patients' platelet multimeric structure was the same as that for normal platelets. After desmopressin infusion the plasma VWF multimeric structure remained supranormal as for preinfusion plasma, with VIII:C VWF:Ag and RiCof increasing markedly over baseline values and disappearing at a normal rate. Examination of the VWF subunit composition from three of these patients indicated that proteolytic processing of their VWF did not differ from normal. This study describes the first variant of VWD with a supranormal multimeric structure.     

Listening to your heart and feeling yourself: effects of exposure to interoceptive signals during the ultimatum game

Successful interaction with the external environment requires a balance between novel or exploratory and routine or exploitative behaviours. This distinction is often expressed in terms of location or orientation of the body relative to surrounding space: functions in which the vestibular system plays an important role. However, the distinction can also be applied to novel versus repetitive production of any behaviour or symbol. Here, we investigated whether vestibular inputs contribute to the balance between novel and routine behaviours, independently of their effects on spatial orienting, by assessing effects of galvanic vestibular stimulation (GVS) on a random number generation task. Right-anodal/left-cathodal GVS, which preferentially activates the left cerebral hemisphere decreased the randomness of the sequence, while left-anodal/right-cathodal GVS, which preferentially activates the right hemisphere increased it. GVS did not induce any spatial biases in locations chosen from the number line. Our results suggest that vestibular stimulation of each hemisphere has a specific effect on the balance between novel and routine actions. We found no evidence for effects of non-specific arousal due to GVS on random number generation, and no evidence for effects on number generation consistent with modulation of spatial attention due to GVS.     

Validation of a Fluorescence In Situ Hybridization Method Using Peptide Nucleic Acid Probes for Detection of Helicobacter pylori Clarithromycin Resistance in Gastric Biopsy Specimens

Here, we evaluated a previously established peptide nucleic acid-fluorescence in situ hybridization (PNA-FISH) method as a new diagnostic test for Helicobacter pylori clarithromycin resistance detection in paraffin-embedded gastric biopsy specimens. Both a retrospective study and a prospective cohort study were conducted to evaluate the specificity and sensitivity of a PNA-FISH method to determine H. pylori clarithromycin resistance. In the retrospective study (n = 30 patients), full agreement between PNA-FISH and PCR-sequencing was observed. Compared to the reference method (culture followed by Etest), the specificity and sensitivity of PNA-FISH were 90.9% (95% confidence interval [CI], 57.1% to 99.5%) and 84.2% (95% CI, 59.5% to 95.8%), respectively. In the prospective cohort (n = 93 patients), 21 cases were positive by culture. For the patients harboring clarithromycin-resistant H. pylori, the method showed sensitivity of 80.0% (95% CI, 29.9% to 98.9%) and specificity of 93.8% (95% CI, 67.7% to 99.7%). These values likely represent underestimations, as some of the discrepant results corresponded to patients infected by more than one strain. PNA-FISH appears to be a simple, quick, and accurate method for detecting H. pylori clarithromycin resistance in paraffin-embedded biopsy specimens. It is also the only one of the methods assessed here that allows direct and specific visualization of this microorganism within the biopsy specimens, a characteristic that allowed the observation that cells of different H. pylori strains can subsist in very close proximity in the stomach.     

Neurological disease caused by Oropouche virus in northern Brazil: should it be included in the scope of clinical neurological diseases?

Journal of NeuroVirology - We describe two neurological cases of Oropouche virus infection in northern Brazil, where the virus is endemic but neglected as a pathogen. This study reiterates the...     

Peripheral dentinogenic ghost cell tumor—report of two cases and review of the literature

Oral and Maxillofacial Surgery - Peripheral dentinogenic ghost cell tumor (DGCT) is a rare and non-aggressive benign odontogenic tumor. They usually affect the elderly and are predominantly located...     

Support system for decision making in the identification of risk for body dysmorphic disorder: A fuzzy model

PurposeTo develop a fuzzy linguistic model to quantify the level of distress of patients seeking cosmetic surgery. Body dysmorphic disorder (BDD) is a mental condition related to body image relatively common among cosmetic surgery patients; it is difficult to diagnose and is a significant cause of morbidity and mortality. Fuzzy cognitive maps are an efficient tool based on human knowledge and experience that can handle uncertainty in identifying or grading BDD symptoms and the degree of body image dissatisfaction. Individuals who seek cosmetic procedures suffer from some degree of dissatisfaction with appearance.MethodsA fuzzy model was developed to measure distress levels in cosmetic surgery patients based on the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV), diagnostic criterion B for BDD. We studied 288 patients of both sexes seeking abdominoplasty, rhinoplasty, or rhytidoplasty in a university hospital.ResultsPatient distress ranged from “none” to “severe” (range = 7.5–31.6; cutoff point = 18; area under the ROC curve = 0.923). There was a significant agreement between the fuzzy model and DSM-IV criterion B (kappa = 0.805; p < 0.001).ConclusionThe fuzzy model measured distress levels with good accuracy, indicating that it can be used as a screening tool in cosmetic surgery and psychiatric practice.     

Impact of anterior occlusal conditions in the mixed dentition on oral health–related quality-of-life item levels:: A multivariate analysis

ObjectivesTo evaluate the impact of anterior occlusal conditions in the mixed dentition on item-level analysis of oral health–related quality of life (OHRQoL).Materials and MethodsA population-based cross-sectional study of 787 children aged 8 to 10 years was conducted. The Child Perceptions Questionnaire (CPQ_8-10) was used to evaluate OHRQoL, and the analysis of item levels was performed on CPQ_8-10 domains. Anterior occlusal characteristics were diagnosed according to the Dental Aesthetic Index criteria. Individual analyses were performed relating the outcome as independent variables. The variables with P < .20 in the individual analyses were tested in multiple logistic regression models, and those with P < .10 remained in the model. The adjusted odds ratio (OR) was estimated with a 95% confidence interval (CI).ResultsNo associations were found with regard to anterior occlusal characteristics (P < .001) after the variables of the previous determinants were adjusted for multivariate analysis. However, the following variables were significantly associated with negative impact on OHRQoL item levels: sex, in oral symptoms (OR = 1.42; CI, 1.07–1.89) and emotional well-being (OR = 1.34; CI, 1.00–1.79); race, in oral symptoms (OR = 1.48; CI, 1.10–1.98), emotional well-being (OR = 1.54; CI, 1.14–2.06), and social well-being (OR = 1.34; CI, 1.00–1.80); and family income in functional limitation (OR = 1.46; CI, 1.06–2.02), emotional well-being (OR = 1.71; CI, 1.21–2.42), and social well-being (OR = 1.59; CI, 1.14–2.21).ConclusionsAnterior occlusal conditions did not affect the levels of OHRQoL items.