• Johannesen Holder opublikował 1 rok, 3 miesiące temu

    BACKGROUND A consensus of early treatment with disease-modifying therapies (DMT) in multiple sclerosis (MS) has been reached based on several observational and experimental studies in adults. However, paediatric onset (PO)MS appears phenotypically different from adult onset MS, characterized by increased relapse rate and pronounced radiological activity on MRI. The objective of this study was to investigate the long-term consequences of delayed treatment start in POMS on disability in a real-world, population-based setting. METHODS Based on prospectively collected data from The Danish Multiple Sclerosis Registry, we defined a cohort of MS patients with onset before the age of 18 years, who were born in 1980 or later, and started treatment with a DMT between 1998 and 2018. The POMS cohort was stratified according to treatment start within 2 years of onset (N = 140) or later (N = 151). Annualised relapse rate in each study group was compared using a negative binomial regression; and Cox proportional hazard moden a DMT later was associated with 61% decreased chance of confirmed EDSS improvement compared to those starting earlier (HR=0.39, 95% CI=0.26-0.59). For every year increment from onset to starting DMT, the risk of confirmed EDSS improvement decreased by 10% (HR=0.90, 95% CI=0.84-0.96). CONCLUSIONS Delayed treatment start in this POMS cohort was associated with shorter time to reach sustained EDSS 4 and confirmed EDSS worsening, and decreased chance of reaching confirmed EDSS improvement, and thus support early treatment start in POMS patients. BACKGROUND Outcome measures typically used to evaluate disease modifying therapies (DMTs) provide important information regarding their effects on disease activity, but they do not capture the full impact of living with multiple sclerosis (MS). Patient reported outcome measures (PROs) are increasingly being used to capture an individual’s subjective experience of disease. We compared DMTs across a wide range of PRO outcomes in individuals with MS. METHODS Subjects enrolled in SysteMS completed the computer adaptive testing version of the Neuro-QoL within four weeks of a clinical neurological exam. Neuro-QoL measures included the following 11 health-related quality of life (HRQOL) domains Ability to participate in Social Roles and Activities, Anxiety, Cognitive Function, Depression, Emotional and Behavioral Dyscontrol, Fatigue, Lower Extremity Function (mobility), Positive Affect and Wellbeing, Satisfaction with Social Roles and Activities, Stigma, and Upper Extremity Function (fine motor). Treatments were groment, the group differences were attenuated and no longer statistically significant. CONCLUSION We examined differences between MS treatment groups across a wide range of HRQOL outcomes. The results suggest that overall there are few differences between treatments on the physical, cognitive and emotional dimensions of well-being. BACKGROUND CONTEXT An important step in improving spinal care is understanding how current health-care resources and associated cost are being utilized and distributed across a health-care system. PURPOSE Our objective was to examine the magnitude and distribution of direct health care costs for spinal conditions across physician type and hospital setting. DESIGN/SETTING Cross-sectional analysis of administrative health data for the fiscal year 2013-2014 from the province of Ontario, Canada. PATIENT SAMPLE Adult population aged 18+ years (N=10,841,302). OUTCOME MEASURES Person visit rates and total number of people and visits by specific care settings were calculated for all spinal conditions as well as stratified by nontrauma and trauma-related conditions. Variation in rates by age and sex was examined. The proportion of patients seeing physicians of different specialties was calculated for each condition grouping. Direct medical costs were estimated and their percentage distribution by care setting calculatED visits cost $28 million for 130,000 visits ($215 per visit). For $32 million spent in primary care, 890,000 visits were made ($36 per visit). Spine imaging costs were $66.5 million, yielding a combined total of $330 million in health care spending for spinal conditions. CONCLUSIONS Spinal conditions place a large and costly burden on the health-care system. The disproportionate annual cost associated with ED visits represents a potential opportunity to redirect costs to fund more clinically and cost-effective models of care for nontraumatic spinal conditions. 18F-fluoro deoxy glucose PET scanner (18F-FDG-PET-CT) has shown its interest in the diagnosis of polymyalgia rheumatica (PMR) and makes possible to evaluate the metabolic activity of the entire musculoskeletal system and in particular muscular structures. The purpose of this study was to evaluate muscle involvement using 18F-FDG-PET-CT in the case of PMR, compared to a non PMR population. METHODS This is a monocentric retrospective study of patients with PMR (ACR/EULAR 2012 criteria) who had an 18F-FDG-PET-CT examination. A control group composed of subjects without rheumatological manifestations who had such an examination as part of neoplastic research or follow-up of neoplastic diseases was also evaluated. The PET assessment included 17 sites suggesting a PMR, as previously reported. Areas of muscle hypermetabolism were classified in the same way according to the same semi quantitative classification. Muscle activity sites were identified. A comparison of patients with PMR with and without muscle damage was performed using the exact Mann-Whitney or Fisher test. RESULTS Two hundred and one cases were examined, involving 101 PMRs (mean age 68.6 years) and 100 controls (mean age 67.7 years). Overall, PET muscle damage was observed in 34 cases (34%) in PMR and 10 cases (10%) in controls (P=0.004). Lesions are bi or multi-focal in half of the cases. The affected muscle sites are spinal muscles 19, scapular girdle 14, pelvic girdle 13, and thigh 6. Fasciitis was found in 3 cases. In patients with PMR, PET muscle involvement was not associated with age, CRP or overall PMR PET score. CONCLUSION Muscle damage assessed by 18F-Fluorodeoxyglucose PET-CT is common in PMR (1/3 of cases), located at the usual sites of disease symptoms, without association with age, CRP levels or the overall PET PMR score. The muscle must be carefully evaluated during a PET examination in cases of PMR. Joint complaints, most commonly intermittent arthritis, are the initial manifestation in about three-fourths of Whipple’s disease cases. We herein report on two cases wherein Whipple’s disease manifested itself as chronic bursitis and tenosynovitis at several sites. A 42 year-old man had bilateral olecranon bursitis, a right patellar bursitis and an extensor tenosynovitis on the left wrist and a 54 year-old man had extensor tenosynovitis at both wrists and a bilateral tenosynovitis of the extensors at both ankle. Methotrexate in both patients and etanercept in one of them were not effective. Polymerase chain reaction testing revealed Tropheryma whipplei on feces, bursitis and articular fluid samples. Duodenal biopsy proved to be normal. Doxycycline and hydroxychloroquine were rapidly effective. Chronic bursitis and tenosynovitis must be added to the list of rheumatologic manifestations that may evoke the diagnosis of Whipple disease. Bronchiectasis is defined as irreversibly damaged and dilated bronchi and is one of the most common pulmonary manifestations in patients with rheumatoid arthritis (RA). The model of RA-associated autoimmunity induced in some individuals by chronic bacterial infection in bronchiectasis is becoming increasingly acceptable, although a genetic predisposition to RA-associated bronchiectasis has also been demonstrated. Bronchiectasis should be suspected in RA patients with chronic cough and sputum production or frequent respiratory infections and the diagnosis must be confirmed by thoracic high-resolution computed tomography. Management of patients with RA-associated bronchiectasis includes a multimodal treatment approach. Similar to all patients with non-cystic fibrosis bronchiectasis, patients with RA-associated bronchiectasis benefit from a pulmonary rehabilitation program, including an exercise/muscle strengthening program and an education program with a specific session on airway clearance techniques. Prophylactic antibiotics are recommended for patients with frequent (3 or more infective exacerbations per year) or severe infections requiring hospitalization/intravenous antibiotics and inhaled corticosteroids and long-acting β2-agonists should be used in patients with non-cystic fibrosis bronchiectasis and associated airway hyper-responsiveness. In patients with RA-associated bronchiectasis the use of immunomodulatory drugs has to be carefully considered, as they are essential to control disease activity, despite being associated with an increased infectious risk. Pneumococcal and influenza vaccines are advised to all patients with RA-associated bronchiectasis in order to reduce the risk of infection. Patients with RA-associated bronchiectasis have a poorer prognosis than those with either RA or bronchiectasis alone and require regular follow-up, under the joint care of a rheumatologist and a pulmonologist. INTRODUCTION Familial Mediterranean fever is the most frequent monogenic auto-inflammatory disorder that mostly affects Mediterranean population. Although this auto-inflammatory disease has historically been described as a recessive genetic disorder with homozygous or compound heterozygous mutations in the MEFV gene, an increasing number of cases are described with the detection of new single MEFV gene heterozygous mutations with modern molecular techniques. CASE DESCRIPTION We report the cases of Caucasian French descent father and daughter who exhibited joint and abdominal inflammatory attacks resembling Familial Mediterranean Fever. Genetic studies revealed in both a heterozygous mutation p.T577N in exon 8 of MEFV gene, and in which colchicine was effective for preventing the attacks. CONCLUSION Single heterozygous mutation of MEFV can be responsible for typical Familial Mediterranean Fever clinical pattern and, what is more, in non-Mediterranean ethnic background patients. A growing body of literature found that anxiety about aging is related to health and well-being of older adults. However, very few studies have been conducted on Chinese older adults residing in different countries and examined the role of resilience. Using the Pearlin’s Stress Process Model, this study aims to fill in this gap by examining the relationship between anxiety about aging as the stressor and health status among Chinese older adults living in Honolulu, the United States (N = 292) and Wuhan, China (N = 532). The survey data were collected through June 2017 to September 2018, using snowball and convenience sampling strategy. The moderating role of resilience on the focal relationship is also explored. Results showed that for both samples, the negative relationship between anxiety about aging and self-rated health was significantly moderated by resilience (18 % and 13 %, respectively), implying the stress-buffering role of resilience. Although both mean levels of resilience and anxiety about aging were lower for the Honolulu sample, the moderating effect appeared to be stronger, implying that older adults in the Honolulu sample might rely more on psychological resources such as resilience in coping with stressors, compared with their counterparts in Wuhan.

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