Localized pigmented villonodular synovitis of the anterior cruciate ligament of the knee: an exceptional presentation of a rare disease with neoplastic and inflammatory features.

Pigmented villonodular synovitis (PVNS) is a rare condition, most commonly involving the knee joint. PVNS is locally aggressive and can invade and destroy surrounding soft tissue and bone, leading to anatomical and functional deterioration of the affected joint. Localized PVNS is an unusual presentation of the disease, generally consisting of a nodular lesion protruding into the articular cavity. Localized PVNS of the knee can mimic other joint disorders which may pose a challenge for a correct diagnosis. Given the locally aggressive behavior of PVNS, prompt identification and excision of the lesion are instrumental to avoid complications. Here, we report a rare case of localized cystic PVNS involving the anterior cruciate ligament of the knee in a 32-year-old woman with persistent knee pain, in whom magnetic resonance imaging was inconclusive. The diagnosis was achieved via arthroscopy and histology. We also present a concise review of the literature on this pathological entity as well as a discussion on the differential diagnosis between localized PVNS and other intra-articular cystic lesions

Acute inactivation of the medial forebrain bundle imposes oscillations in the SNr: a challenge for the 6-OHDA model?

It has been recently shown that the substantia nigra pars reticulata (SNr) of 6-hydroxydopamine (6-OHDA)-lesioned rats, under urethane anaesthesia, manifests a prominent low frequency oscillation (LFO) of around 1Hz, synchronized with cortical slow wave activity (SWA). Nevertheless, it is poorly understood whether these electrophysiological alterations are correlated only with severe dopamine depletion or may also play a relevant pathogenetic role in the early stages of the dopamine denervation. Hence, here we recorded SNr single units and electrocorticogram (ECoG) in two models of dopamine denervation: (i) acute dopamine denervated rats, obtained by injection of tetrodotoxin (TTX), (ii) chronic dopamine depleted rats, 2 weeks after 6-OHDA lesioning. Both TTX and 6-OHDA were infused into the medial forebrain bundle (MFB). The acute TTX-mediated dopamine depletion caused a fast developing occurrence of a SNr/ECoG coherence, peaking between 0.48 and 1.22 Hz, parallel with a consistent decrease of firing rate (from 22.61 \ub1 7.04 to 15.35 \ub1 9.04 Hz) homolateraly to the infusion. Strikingly, this abnormal 1 Hz synchronization, TTX-mediated was qualitatively similar to the ECoG/SNr synchronization detectable in the 6-OHDA lesioned hemisphere (LH). In addition, TTX infusion in the un-lesioned hemispheres (UH) of 6-OHDA treated rats, produced ECoG/SNr synchronization qualitatively similar to that recordable in the LH. Hence, our data support the proposition that LFO, is tightly correlated to cortex, and represent a critical hallmark of a basal ganglia (BG) failure from the early stages of dopamine denervation

Disease activity and damage in juvenile idiopathic arthritis: Methotrexate era versus biologic era

Objective: To compare the long-term disease state, in terms of activity and damage, of children with juvenile idiopathic arthritis (JIA) who had their disease onset in methotrexate (MTX) or biologic eras. Methods: Patients were included in MTX or biologic era cohort depending on whether their disease presentation occurred before or after January 2000. All patients had disease duration 65 5 years and underwent a prospective cross-sectional assessment, which included measurement of disease activity and damage. Inactive disease (ID) and low disease activity (LDA) states were defined according to Wallace, JADAS10, and cJADAS10 criteria. Articular and extraarticular damage was assessed with the Juvenile Arthritis Damage Index (JADI). Results: MTX and biologic era cohorts included 239 and 269 patients, respectively. Patients were divided in the "functional phenotypes" of oligoarthritis and polyarthritis. At cross-sectional visit, patients in the biologic era cohort with either oligoarthritis or polyarthritis had consistently higher frequencies of ID and LDA by all criteria. The measurement of disease damage at cross-sectional visit revealed that the frequency of impairment of > 1 JADI-Articular items was higher in MTX than in biologic era cohort (17.6% versus 11% in oligoarthritis and 52.6% versus 21.8% in polyarthritis). Likewise, frequency of involvement of > 1 JADI-Extraarticular items was higher in the MTX than in the biologic era cohort (26.5% versus 16.2% in oligoarthritis and 31.4% versus 13.5% in polyarthritis). Conclusion: Our study provides evidence of the remarkable outcome improvement obtained with the recent therapeutic advance in JIA

Effect of a 40-weeks multicomponent exercise program and branched chain amino acids supplementation on functional fitness and mental health in frail older persons

BACKGROUND: The ageing process implies several physiological and psychological changes that hence affect the general health, mood states, and quality of life of older persons. Exercise and adequate nutrition are renowned non-pharmacological strategies that significantly delay and alleviate the adverse consequences of the ageing process. This study aimed to evaluate the effects of branched-chain amino acid (BCAA) supplementation and a multicomponent exercise program (ME) on the physical frailty and mood states of older persons. METHODS: 35 participants (women and men; 83 ± 3 years old) from residential care homes were submitted to a 40-week exercise-washout-retraining intervention (16 weeks of the elastic band based exercise and/or supplementation, 8 weeks of washout, and 16 weeks of multicomponent exercise and/or resupplementing), with or without BCAA supplementation. The experimental groups were: (i) ME plus BCAA supplementation (ME+BCAA); (ii) ME; (iii) BCAA supplementation (BCAA), and (iv) control group (CG). Fried's phenotype was used to assess frailty prevalence. Geriatric Depression Scale (GDS), Profile of Mood State (POMS), Mini-Mental State Examination (MMSE), were used to access mental health and cognition. The Short Physical Performance Battery (SPPB) was used to access functional capacity. Salivary testosterone levels (ST) were also determined to access the anabolic effects of the intervention. RESULTS: Exercise was effective in improving functional capacity and prevented the increase in frailty that occurred in the non-exercising CG, where the frailty scores increased over time (p < 0.01). BCAAs supplement alone had no impact on functional fitness, but in a short time (16 weeks) contributed to diminishing frailty and combined with exercise may have the potential to reduce the effect of a detraining period on functional capacity. Salivary testosterone levels correlated with handgrip strength and could be a useful indicator of susceptibility to frailty. No effects were found for mood states, cognition, and depression. CONCLUSION: This study showed that a long-term exercise program, independent of being multicomponent or strength elastic band-based, was effective in improving functional capacity and prevented an increase in frailty in frail and pre-frail older persons living in residential care homes

Visitors' awareness of ICZM and WTP for beach preservation in four European Mediterranean regions.

This paper discusses the results of a multi-country survey about private stakeholders' contribution to coastal preservation. It was conducted in four coastal sites of Greece, Italy and France, in order to collect information about beach visitors' perception of Integrated Coastal Zone Management (ICZM) and their willingness to pay (WTP) for beach preservation, intended here as defence from erosion. In order to find out whether ICZM perception is a determinant of WTP, regression analysis is applied. Results show that in these sites respondents have a low level of information about the nature of ICZM, despite local authorities having implemented some ICZM strategies for preserving the coast. Nevertheless, those who are informed about ICZM have a higher probability of paying for beach preservation. This suggests to policymakers that promoting public awareness about ICZM may increase the probability of paying. Finally, some categories of visitors, such as women and young and middle-aged people, have a higher probability of paying than men and older people, thus suggesting a more sensitive attitude to beach preservation. Therefore, policy-makers should also pay attention to the categories of visitors less likely to pay

Genetic dynamics in untreated CLL patients with either stable or progressive disease: A longitudinal study

Clonal evolution of chronic lymphocytic leukemia (CLL) often follows chemotherapy and is associated with adverse outcome, but also occurs in untreated patients, in which case its predictive role is debated. We investigated whether the selection and expansion of CLL clone(s) precede an aggressive disease shift. We found that clonal evolution occurs in all CLL patients, irrespective of the clinical outcome, but is faster during disease progression. In particular, changes in the frequency of nucleotide variants (NVs) in specific CLL-related genes may represent an indicator of poor clinical outcome

The deindustrialisation/tertiarisation hypothesis reconsidered: a subsystem application to the OECD7

The diffusion of outsourcing, both national and international, and vertical FDIs among manufacturing firms, along with the higher integra- tion of business services in manufacturing, has recently led to question the empirical evidence supporting the Deindustrialisation/Tertiarisation (DT) hypothesis. Rather than a \real" phenomenon, it has been argued, DT would be an \apparent" one, mainly due to the reorganization of production across national and sectoral boundaries. The empirical studies that have dealt with the topic so far have not been able to effectively rule out such possibility, because of two main limitations: the sectoral level of the analysis and/or the national focus. In order to overcome them, the paper carries out an appreciative investigation of the actual extent of the DT occurred in the OECD area over the '80s and the '90s by moving from a sector to a subsystem perspective, thus retaining both direct and indirect relations, and by referring to a \pseudo-World" of 7 OECD countries, thus taking into account the \global" dimension of the phenomenon. The results strongly support the DT hypothesis: although the weight of business sector services in the manufacturing subsystem increased, acting as a counterbalancing tendency to the manufacturing decline, subsystem shares significantly decreased, thus confirming DT as a more fundamental trend of modern economies