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isease progression and decreasing control to be consequences of their illness. Living with the disease, the emphasis in the additional component, was a process for regaining control and adapting to their situations.

Patients with peripheral arterial disease shape their own understandings of their conditions. These beliefs may influence their management of their disease and adherence to treatment. Therefore, the current study suggests that illness perceptions should be addressed when planning secondary prevention for patients with peripheral arterial disease.

Patients with peripheral arterial disease shape their own understandings of their conditions. These beliefs may influence their management of their disease and adherence to treatment. Therefore, the current study suggests that illness perceptions should be addressed when planning secondary prevention for patients with peripheral arterial disease.

To determine the prevalence of cerebrovascular events (CVE) in giant cell arteritis (GCA) and to alert clinicians to the importance of early detection of CVE in this disease.

Retrospective observational study involving a cohort of GCA patients. Demographic, clinical and laboratory data were collected. All patients fulfilled the American College of Rheumatology (ACR) 1990 GCA classification criteria and had a positive ultrasound test for GCA in agreement with the EULAR recommendations. Demographic and clinical parameters were recorded with special attention paid to ischemic cranial events.

We studied 123 consecutive GCA patients, 74 (60.2%) women with a mean age of 79 years. Twelve patients (9.75%) suffered from neurologic symptoms other than AION, of whom 9 (7.3%) experienced ischemic events related to GCA and 3 (2.44%) likely experienced CVE due to other common causes. Of the 9 patients with CVE caused by GCA, 5 were diagnosed with transient ischemic attacks (TIAs), 2 with ischemic stroke, and 2 were cases involving cranial nerve palsies. High rates of mortality were found in patients with a TIA or stroke, while polymyalgia rheumatica (PMR) appeared to confer some protection against ischemic pathologies in GCA patients.

Stroke and TIA are common presentation patterns associated with GCA and should be suspected in all CVE-related cases with high acute-phase reactants commonly present in the elderly. This ischemic subgroup exhibited a higher mortality rate.

Stroke and TIA are common presentation patterns associated with GCA and should be suspected in all CVE-related cases with high acute-phase reactants commonly present in the elderly. This ischemic subgroup exhibited a higher mortality rate.

Statin therapy has become one of the most important advances in stroke secondary prevention. Nevertheless, statin therapy in patients who present an ischemic stroke following cervical artery dissection (CAD) has not yet been supported by clinical evidence. This study aimed to investigate the effect of statins on neurological outcomes after a stroke due to CAD.

We conducted a prospective cohort study including consecutive patients diagnosed with a stroke due to CAD. Subjects were classified into non-statin, simvastatin 20mg, simvastatin 40mg, and high-potency statin groups. After 2 years, the functional outcome, stroke recurrence, major cardiovascular events, and mortality were assessed.

Among the 54 patients included in our cohort, there were 16 (29.6%) patients without statins, 22 (40.7%) with simvastatin 20mg, 12 (22.2%) with simvastatin 40mg and 4 (7.5%) with high-potency statins. Using simvastatin 40mg was associated with a significantly lower incidence of stroke recurrence. Patients with simvastatin 40mg and high-potency statins presented the best functional recovery throughout the follow-up (p<.01).

The use of statins in patients with CAD-related stroke may improve functional outcomes in specific cases. Statins do not prevent stroke recurrence and major cardiovascular events in this type of stroke.

The use of statins in patients with CAD-related stroke may improve functional outcomes in specific cases. Statins do not prevent stroke recurrence and major cardiovascular events in this type of stroke.

IgA nephropathy (IgAN) is one of the main causes of primary glomerulonephritis worldwide, and it is also the main primary disease leading to chronic kidney disease. The purpose of this study is to evaluate the epidemiology and risk factors for progression in Chinese patients with IgAN.

In this retrospective study, 246 patients with renal biopsy-proven IgAN were enrolled from January 2012 to June 2018. The patients' data were divided into two groups according to eGFR at the end of follow-up a high-eGFR group (eGFR≥60ml/min) and a low-eGFR group (eGFR<60ml/min).

At the end of the study, we identified 49 (19.92%) patients with low-eGFR from 246 IgAN patients. Renal function, represented by serum creatinine, urea nitrogen and cystatin-C, was significantly decreased in the low-eGFR group (P<0.001 for all) at the time of renal biopsy. Compared with the high-eGFR group, the age, mean arterial blood pressure (MAP), proteinuria, cholesterol, triglycerides and serum uric acid were significantly higher (P<0.05 for all). According to the Oxford evaluation, the proportion of S1-2 (59.2%) and T1-2 (65.3%) was significantly increased (P<0.001 for both) and the proportion that had a MEST-C score ≥3 was statistically increased in the low-eGFR group (83.7%, P=0.001).

Male, MAP, haematuria, Scr, cholesterol, hemoglobin, Lee classification more than 3 and C1-2 are independent risk factors for low-eGFR in Chinese IgAN patients.

Male, MAP, haematuria, Scr, cholesterol, hemoglobin, Lee classification more than 3 and C1-2 are independent risk factors for low-eGFR in Chinese IgAN patients.

Dilated cardiomyopathy (DCM) is the most frequent cause of heart transplantation. The prevalence of familial disease can reach 50%. Our objective was to describe the genetic basis of DCM in a cohort with a high proportion of transplanted patients.

We included patients with DCM and genetic testing performed using next-generation sequencing (NGS) that included at least 80 genes. Clinical data, family history and genetic results were retrospectively analysed. When possible, assessment of first-degree relatives was carried out.

Eighty-seven DCM patients and 308 relatives from 70 families were evaluated. Clinical prevalence of familial disease was 37% (32 patients). Forty-four percent of patients (38 patients) had required heart transplantation. A relevant variant was found in 43 patients (49%), 25 patients (29%) carried variants of unknown significance and in 19 patients (22%) the study was negative. Most genetic variants were found in sarcomeric genes and the yield of genetic testing was higher in patients with familial DCM.

The yield of genetic testing in our DCM cohort was high, reaching 69% in familial cases. Mutational spectrum was heterogeneous and the identification of the specific aetiology of the disease often provided prognostic information.

The yield of genetic testing in our DCM cohort was high, reaching 69% in familial cases. Mutational spectrum was heterogeneous and the identification of the specific aetiology of the disease often provided prognostic information.

Historically, germline testing of patients with pancreatic cancer was performed selectively in patients with a strong family history of cancer. Current guidelines recommend universal testing because some patients may have actionable germline pathogenic variants without family history.

We conducted a cost-effectiveness analysis using a decision-tree model to compare universal versus selective testing strategies for patients with pancreatic cancer. Costs, probabilities, and overall survival were estimated from the published literature and institutional data. One-way and probabilistic sensitivity analyses explored model uncertainty.

Universal germline genetic testing had an incremental cost of $310 with an increase of 0.003 life-years. The incremental cost-effectiveness ratio was $121,924/life-years. Parameters which were most impactful (sensitivity analysis) included the median overall survival of patients with advanced disease treated with personalized therapy, cost of personalized therapy for advanced disease, and the probability of receiving personalized therapy in advanced disease. A strategy of selective testing was more cost-effective in 59% of iterations when the willingness-to-pay threshold was set to $100,000/life-years.

Our model suggested that selective germline testing of patients with newly diagnosed pancreatic cancer is more cost-effective than universal testing. Additional research is needed to explore the impact of cascade testing of relatives on cost-effectiveness.

Our model suggested that selective germline testing of patients with newly diagnosed pancreatic cancer is more cost-effective than universal testing. Additional research is needed to explore the impact of cascade testing of relatives on cost-effectiveness.Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack. Unlike multiple sclerosis, a progressive clinical course is very unusual and the accrual of disability is related to relapses. Aggressive treatment of attacks and highly efficient maintenance therapies to prevent attacks are therefore crucial to prevent residual disability. In this article, we review how high dose steroids and most importantly apheresis and modern therapies implicating B cell depletion, inhibition of complement and IL-6 reception are effective to change its natural history. We will emphasize the results of three recent double blind randomized controlled studies using monoclonal antibodies allowing strong hope to modify natural history of NMOSD.Syringomyelia is a rare disorder in which a fluid-filled cyst forms within the spinal cord, resulting in myelopathy. Meanwhile, the abnormal dilatation of the central canal is referred to as hydromyelia or slit-like syrinx. The most prevailing classification is based on anatomical features and pathogeny rather than pathophysiological mechanisms. It is usual to distinguish foraminal syringomyelia related mainly to abnormalities at the craniocervical junction, non-foraminal syringomyelia dealing with any cause of arachnoiditis (infection, inflammation, trauma…) and more rarely syringomyelia associated with intramedullary tumors. Although many pathophysiological theories have been argued over time, the prevailing one is that disturbances in cerebrospinal fluid (CSF) flow in the sub-arachnoid spaces disrupt flow velocity leading to the syrinx. Symptoms of paralysis, sensory loss and chronic pain commonly develop during the third/fourth decades of life. The natural history of syringomyelia is typically one of grad intramedullary tumors resolves spontaneously after tumor resection. Syringomyelia is a rare disease, which requires a dedicated multidisciplinary approach, emphasizing the need for a nationwide scientific organization so as to offer optimal care to the patient.