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Mediterranean diet and risk of Sjögren's syndrome.
No full textOBJECTIVES
Non-genetic risk factors for Sjögren's syndrome (SS) are poorly understood. Adherence to a Mediterranean diet has been associated with reduction in other autoimmune diseases. We examined the association of Mediterranean diet with SS.
METHODS
New patients attending a single centre warranting investigation for primary SS (pSS) were recruited into the Optimising Assessment in Sjögren's Syndrome cohort established in Birmingham, UK (2014-2018). Participants were classified into pSS and non-SS sicca, considered as cases and non-cases, respectively, and asked to complete an optional food frequency questionnaire on their diet before onset of symptoms. A semi-quantitative Mediterranean diet score (MDS) was calculated (possible range=0 to 18). Using multivariate logistic regression, corrected for energy intake, body-mass index, sex, age, symptom duration, and smoking status, we examined the association of MDS with SS.
RESULTS
Dietary data were available for 133/243 (55%) eligible patients (n=82 pSS and n=51 sicca). In the adjusted model, a higher total MDS (mean ± SD, 9.41±2.31 points) was associated with lower odds of pSS (OR 0.81, 95% CI 0.66-0.99; p=0.038) per one unit of MDS. Among MDS components, the strongest association was seen with fish with OR 0.44 (95% CI 0.24-0.83; p=0.01) in the comparison between <1 portion/week and 1 to 2.5 portions/week. Higher galactose, vitamin A-retinol-equivalents and vitamin C showed associations with lower odds of pSS in multivariate analysis, where the association of vitamin C was attenuated when adjusted for MDS.
CONCLUSIONS
When adjusted for potential confounders, adherence to the Mediterranean diet was associated with lower likelihood of having pSS
A service evaluation: impact of nurse-led regional familial hypercholesterolaemia service on a hospital adult lipid clinic.
No full textThe authors evaluated the impact of genetic screening for familial hypercholesterolaemia (FH) in a lipid clinic cohort of patients with definite and possible FH as defined by the Simon Broome Register (SBR) criteria.
METHODS
Patients with a lipid clinic diagnosis of definite and possible FH based on the SBR criteria were referred to a nurse-led regional service for FH genetic testing.
FINDINGS
140 patients were referred for genetic testing. Six had SBR-definite FH due to the presence of tendon xanthomata and 134 had SBR-possible FH. A monogenic FH mutation was detected in all six patients (100%) with SBR-definite FH and in 34 (25%) of patients with possible FH.
CONCLUSION
The appropriate use of molecular genetics in a lipid clinic will greatly facilitate the management of hyperlipidaemia and cardiovascular risk since the management of FH patients (National Institute for Health and Care Excellence (NICE) Clinical Guideline 71) is different from non-FH patients (NICE Clinical Guideline 181)
Developing Integrated Clinical Pathways for the Management of Clinically Severe Adult Obesity: a Critique of NHS England Policy.
No full textPURPOSE OF THE REVIEW
Pathways for obesity prevention and treatment are well documented, yet the prevalence of obesity is rising, and access to treatment (including bariatric surgery) is limited. This review seeks to assess the current integrated clinical pathway for obesity management in England and determine the major challenges.
RECENT FINDINGS
Evidence for tier 2 (community-based lifestyle intervention) and tier 3 (specialist weight management services) is limited, and how it facilitates care and improve outcomes in tier 4 remains uncertain. Treatment access, rigidity in pathways, uncertain treatment outcomes and weight stigma seems to be major barriers to improved care. More emphasis must be placed on access to effective treatments, treatment flexibility, addressing stigma and ensuring treatment efficacy including long-term health outcomes. Prevention and treatment should both receive significant focus though should be considered to be largely separate pathways. A simplified system for weight management is needed to allow flexibility and the delivery of personalized care including post-bariatric surgery care for those who need it
Serum Copeptin, NLPR3, and suPAR Levels among Patients with Autosomal-Dominant Polycystic Kidney Disease with and without Impaired Renal Function.
No full textBACKGROUND
The pathophysiology of renal disease progression in autosomal-dominant polycystic kidney disease (ADPKD) involves not only cystogenesis but also endothelial dysfunction, leading to the activation of inflammatory and fibrotic pathways. This study evaluated the levels of biomarkers related to osmoregulation, immune system activation, and tubular injury in ADPKD patients with impaired or preserved renal function.
METHODS
This study included 26 ADPKD patients with modestly impaired renal function (estimated glomerular filtration rate [eGFR] 45-70 mL/min/1.73 m2; Group A), 26 age- and sex-matched ADPKD patients with relatively preserved renal function (eGFR >70 mL/min/1.73 m2; Group B), and 26 age- and sex-matched controls (Group C). Serum levels of copeptin, the inflammasome nucleotide-binding and oligomerization domain-like receptors pyrin domain-containing protein 3 (NLRP3), and soluble urokinase-type plasminogen activator receptor (suPAR) were measured with ELISA techniques.
RESULTS
Patients in Group A had higher levels of copeptin (median [interquartile range]: 50.44 [334.85] pg/mL), NLRP3 (5.86 [3.89] ng/mL), and suPAR (390.05 [476.53] pg/mL) compared to patients in Group B (32.38 [58.33], p = 0.042; 2.42 [1.96], p < 0.001; and 313.78 [178.85], p = 0.035, respectively) and Group C (6.75 [6.43]; 1.09 [0.56]; and 198.30 [28.53], respectively; p < 0.001 for all comparisons). Levels of all studied markers were also significantly higher in Group B patients compared to controls (p < 0.001), despite having similar eGFR. In patients with ADPKD, all studied biomarkers were correlated positively with asymmetric-dimethylarginine (ADMA) and endocan levels, and negatively with eGFR. ADMA and endocan levels were the only parameters independently associated with increased copeptin levels.
CONCLUSIONS
This study showed that ADPKD patients with impaired and preserved renal function had higher copeptin, NLRP3, and suPAR levels than controls. Such findings support that cystogenesis and inflammation are associated with endothelial dysfunction, even in the early stages of ADKPD
Multicenter cohort study of patients with buried bumper syndrome treated endoscopically with a novel, dedicated device.
No full textBACKGROUND AND AIMS
Buried bumper syndrome (BBS) is a rare adverse event of percutaneous endoscopic gastrostomy (PEG) placement in which the internal bumper migrates through the stomal tract to become embedded within the gastric wall. Excessive tension between the internal and external bumpers, causing ischemic necrosis of the gastric wall, is thought to be the main etiological factor. Several techniques for endoscopic management of BBS have been described using off-label devices. The Flamingo-Set is a novel, sphincterotome-like device, specifically designed for BBS management. We aimed to evaluate the effectiveness of the Flamingo device in a large, homogeneous cohort of patients with BBS.
METHODS
A guidewire is inserted through the external access of the PEG tube into the gastric lumen. The Flamingo device is then introduced into the stomach over the guidewire. This dedicated tool can be flexed by 180°, exposing a sphincterotome-like, cutting wire, which is used to incise the overgrown tissue until the PEG bumper is exposed. A retrospective, international multicenter cohort study was conducted on 54 patients between December 2016 and February 2019.
RESULTS
The buried bumper was successfully removed in 53 out of 55 procedures (96.4%). The median time for the endoscopic removal of the buried bumper was 22 minutes (range 5-60). Periprocedural endoscopic adverse events occurred in 7 procedures (12.7 %) and were successfully managed endoscopically. A median follow-up of 150 days (range 33-593) was performed in 29 patients (52.7%), during which no significant adverse events occurred.
CONCLUSION
Through our experience, we find that this dedicated novel device is safe, quick, and effective for minimally invasive, endoscopic management of BBS
Diagnostic approach to the evaluation of myeloid malignancies following CAR T-cell therapy in B-cell acute lymphoblastic leukemia.
No full textImmunotherapeutic strategies targeting B-cell acute lymphoblastic leukemia (B-ALL) effectively induce remission; however, disease recurrence remains a challenge. Due to the potential for antigen loss, antigen diminution, lineage switch or development of a secondary or treatment-related malignancy, the phenotype and manifestation of subsequent leukemia may be elusive. We report on two patients with multiply relapsed/refractory B-ALL who, following chimeric antigen receptor T-cell therapy, developed myeloid malignancies. In the first case, a myeloid sarcoma developed in a patient with a history of myelodysplastic syndrome. In the second case, two distinct events occurred. The first event represented a donor-derived myelodysplastic syndrome with monosomy 7 in a patient with a prior hematopoietic stem cell transplantation. This patient went on to present with lineage switch of her original B-ALL to ambiguous lineage T/myeloid acute leukemia. With the rapidly evolving field of novel immunotherapeutic strategies, evaluation of relapse and/or subsequent neoplasms is becoming increasingly more complex. By virtue of these uniquely complex cases, we provide a framework for the evaluation of relapse or evolution of a subsequent malignancy following antigen-targeted immunotherapy
Management of Newly-diagnosed Metastatic Hormone-sensitive Prostate Cancer: a Survey of UK Uro-oncologists.
No full textAIM
To explore the practice and views of uro-oncologists in the UK regarding their use of chemotherapy and androgen receptor-targeted agents (ARTAs) in patients with newly-diagnosed metastatic hormone-sensitive prostate cancer (mHSPC).
METHODS
An expert-devised paper or online questionnaire was completed by members of the British Uro-oncology Group.
RESULTS
All respondents stated that they would offer patients with newly-diagnosed mHSPC docetaxel and androgen deprivation therapy (ADT) if they were sufficiently fit to receive chemotherapy (this was the only option available at the time of the survey); 64% would strongly recommend docetaxel for those with high-volume metastatic disease and 31% for those with low-volume disease. Hypothetically, if both docetaxel and ARTAs were available in the UK for mHSPC, almost 65% of respondents would recommend an ARTA with ADT to these patients in at least one-half of all cases, with the strongest recommendations to patients with high-risk disease. Imaging for response was conducted according to suspicion of disease progression, regardless of treatment, with the minority of clinicians recommending routine imaging. If a choice of therapy was available, docetaxel would be more likely to be offered to patients with liver or lung metastases, and ARTAs to patients with bone or lymph node only metastases. Almost all respondents would offer local radiotherapy to the primary tumour in patients with low-volume disease.
CONCLUSION
All the UK uro-oncologists those surveyed stated that they would offer docetaxel in combination with ADT to all newly-diagnosed patients with mHSPC if fit enough for chemotherapy. ARTAs would be offered to many patients if available, especially those with high-risk disease or those unfit to receive chemotherapy. Scanning was typically conducted following treatment only at suspicion of disease progression
A Systematic Review of the Use of Physiological Tests Assessing the Acute Response to Treatment During Exacerbations of COPD (with a Focus on Small Airway Function).
No full textExacerbations are prevalent in Chronic Obstructive Pulmonary Disease (COPD) patients and associated with poor clinical outcomes. Currently, there is a lack of sensitive and specific tools that can objectively identify exacerbations and assess their progress or treatment response. FEV is often reported as a study outcome, but it has significant limitations. Studies have suggested that small airways measures might provide physiological biomarkers during exacerbations. Therefore, this study was done to assess which physiological tests of small airways function have been used in the acute setting during exacerbations of COPD and the evidence to support their use. An electronic databases search was conducted in April 2019. A standard systematic review methodology was used. Eligible studies were those of ≥10 participants that compared at least one small airway test with FEV to assess response to treatment with baseline and a follow-up measurement ≤2 months after. Analyses were narrative. Of 1436 screened studies, seven studies were eligible. There was heterogeneity in which tests of small airways were used and three different small airways measures were reported. Studies were small (including 20 to 87 subjects). Six articles reported improvements in small airway measurements during the recovery from exacerbation which correlated with FEV. Included studies varied in their timing and duration of the assessment. There is some evidence to support the use of small airway tests in acute exacerbations of COPD. However, studies have been small with different tests being utilized. Further studies to determine the usefulness of each test may be of interest
Patient and public perspectives on cell and gene therapies: a systematic review.
No full textCell and gene therapies offer opportunities for treating disease with potential to restore function, and cure disease. However, they are not without risk and pose complex logistical, economic, ethical and social challenges for health systems. Here we report our systematic review of the current evidence on patient and public knowledge and perspectives of cell and gene therapies, to inform future research, education and awareness raising activities. We screened 10,735 titles and abstracts, and evaluated the full texts of 151 publications. The final selection was 35 publications. Four themes were generated from the narrative synthesis of the study findings namely: (1) Knowledge and understanding of cell and gene therapies, (2) Acceptance of cell and gene therapies (3) Understanding of risk and benefits of therapy, and (4) Information needs and current sources of information. As potential funders or future recipients, it is important that the public and patients are aware of these therapies, understand the issues involved, and can contribute to the debate. This review highlights the need for appropriate patient and public education on the various aspects of cell and gene therapies. High quality studies exploring patient and public opinions and experiences of cell and gene therapy are required. Patient and public perceptions of these therapies, alongside evidence of clinical and cost-effectiveness, will be central to their uptake and use
BSR guideline on the management of adults with systemic lupus erythematosus (SLE) 2018: baseline multi-centre audit in the UK.
No full textOBJECTIVES
To assess the baseline care provided to patients with SLE attending UK Rheumatology units, audited against standards derived from the recently published BSR guideline for the management of adults with SLE, the NICE technology appraisal for belimumab, and NHS England's clinical commissioning policy for rituximab.
METHODS
SLE cases attending outpatient clinics during any 4-week period between February and June 2018 were retrospectively audited to assess care at the preceding visit. The effect of clinical environment (general vs dedicated CTD/vasculitis clinic and specialized vs non-specialized centre) were tested. Bonferroni's correction was applied to the significance level.
RESULTS
Fifty-one units participated. We audited 1021 episodes of care in 1003 patients (median age 48 years, 74% diagnosed >5 years ago). Despite this disease duration, 286 (28.5%) patients had active disease. Overall in 497 (49%) clinic visits, it was recorded that the patient was receiving prednisolone, including in 28.5% of visits where disease was assessed as inactive. Low documented compliance (<60% clinic visits) was identified for audit standards relating to formal disease-activity assessment, reduction of drug-related toxicity and protection against comorbidities and damage. Compared with general clinics, dedicated clinics had higher compliance with standards for appropriate urine protein quantification (85.1% vs 78.1%, P ≤ 0.001). Specialized centres had higher compliance with BILAG Biologics Register recruitment (89.4% vs 44.4%, P ≤ 0.001) and blood pressure recording (95.3% vs 84.1%).
CONCLUSIONS
This audit highlights significant unmet need for better disease control and reduction in corticosteroid toxicity and is an opportunity to improve compliance with national guidelines. Higher performance with nephritis screening in dedicated clinics supports wider adoption of this service-delivery model