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In the period from 2012-2015 plant samples with fireblight symptoms were collected from pome fruits and indigenous plant, in main fruitgrowing regions of Montenegro. After succesfull isolation, pathogenicity of the obtained strains was tested by artificial inoculation of immature pear fruits, variety Viljamovka. Hypersensitive reaction was tested on tobacco leaves, variety White Burley. Identification and genetic diversity studies were performed using several molecular techniques on 18 Erwinia amylovora strains originating from quince, pear, apple and hawthorn. Bacterial identity was confirmed by nested PCR in which all studied strains produced the expected amplification fragment of plasmid pEA29. To detect potential genetic variations in E. amylovora population, rep-PCR was conducted. Using REP, ERIC and BOX primers, in all three PCR reactions, differences between studied strains were detected, i.e. pear strains had different genetic profiles from all other studied strains, including reference strain. Genetic variability of selected E. amylovora strains was studied by RAPD-PCR as well. Both of the used random primers, CUGEA-3 and CUGEA-5, showed discriminatory potential by separating genetic profiles of pear strains from all other studied strains, including reference strain. This is the first study of genetic variability of E. amylovora in Montenegro.

In the period from 2012-2015 plant samples with fireblight symptoms were collected from pome fruit and indigenous plant, in main fruitgrowing regions of Montenegro. After succesfull isolation, pathogenicity of the obtained strains was tested by artificial inoculation of immature pear fruits, variety Viljamovka. Hypersensitive reaction was tested on tobacco leaves, variety White Burley. Identification and genetic diversity studies were performed using several molecular techniques on 18 Erwinia amylovora strains originating from quince, pear, apple and hawthorn. Bacterial identity was confirmed by nested PCR in which all studied strains produced the expected amplification fragment of plasmid pEA29. To detect potential genetic variations in E. amylovora population, rep-PCR was conducted. Using REP, ERIC and BOX primers, in all three PCR reactions, differences between studied strains were detected, i.e. pear strains had different genetic profiles from all other studied strains, including reference strain. Genetic variability of selected E. amylovora strains was studied by RAPD-PCR as well. Both of the used random primers, CUGEA-3 and CUGEA-5, showed discriminatory potential by separating genetic profiles of pear strains from all other studied strains, including reference strain. This is the first study of genetic variability of E. amylovora in Montenegro.

The aim of this study was to investigate cardiac abnormalities in adults with osteogenesis imperfecta (OI). The clinical and echocardiographic survey included 99 adults with OI divided into 3 clinical types—I, III, and IV—and 52 controls. Left ventricular end-diastolic dimensions (LVIDds), mass, and 4 aortic diameters were measured by standard echocardiography and indexed for body surface area. Hypertension was registered in 37 individuals (37.4%). The OI group had significantly lower body surface area than the control individuals, 1.7 ± 0.3 versus 1.9 ± 0.2 m2 (P < .05). The LVIDd and LV mass were significantly larger in the OI group when compared with the controls, 2.98 ± .64 versus 2.59 ± .26 cm/m2 (P < .05) and 97.3 ± 30.1 versus 73.3 ± 18.0 g/m2 (P < .05), respectively. Type III OI showed significantly enlarged LVIDd as compared with types I and IV OI, 4.33 ± 1.10 versus 2.83 ± .33 (P < .05) versus 2.85 ± .37 cm/m2 (P < .05), respectively. All aortic diameters were significantly larger in the OI group than in the control group, as they were in type III compared with types I and IV; 10.1% mild aortic regurgitation (AR), 10.1% moderate AR, and 7.1% moderate mitral regurgitation were registered in the OI group. Increased LVIDd, LV mass, mitral regurgitation, and AR were found in adult patients with OI compared with the control group. The changes in LV and dilatation of aorta seemed to be more pronounced in patients with type III compared with types I and IV OI.

. Chronic muscle diseases (MD) are progressive and cause wasting and weakness in muscles and are associated with reduced quality of life (QoL). The ACTMuS trial examined whether Acceptance and Commitment Therapy (ACT) as an adjunct to usual care improved QoL for such patients as compared to usual care alone. This two-arm, randomised, multicentre, parallel design recruited 155 patients with MD (Hospital and Depression Scale ⩾ 8 for depression or ⩾ 8 for anxiety and Montreal Cognitive Assessment ⩾ 21/30). Participants were randomised, using random block sizes, to one of two groups: standard medical care (SMC) ( = 78) or to ACT in addition to SMC ( = 77), and were followed up to 9 weeks. The primary outcome was QoL, assessed by the Individualised Neuromuscular Quality of Life Questionnaire (INQoL), the average of five subscales, at 9-weeks. Trial registration was NCT02810028. 138 people (89.0%) were followed up at 9-weeks. At all three time points, the adjusted group difference favoured the intervention group and was significant with moderate to large effect sizes. Secondary outcomes (mood, functional impairment, aspects of psychological flexibility) also showed significant differences between groups at week 9. ACT in addition to usual care was effective in improving QoL and other psychological and social outcomes in patients with MD. A 6 month follow up will determine the extent to which gains are maintained.

Background:The Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire gauges the impact of ankylosing spondylitis on individuals’ overall well-being, shedding light on the quality of life challenges associated with this condition.Objectives:To examine psychometric properties of the Serbian version of the ASQoL and to evaluate its correlations with AS disease activity and functional status indexes.Methods:After translation with transcultural validation and cognitive debriefing interview in 10 randomly chosen persons was successfully done, 60 patients were recruited consecutively, as they entered the Institute of Rheumatology for a regular check-up and tested in two occasions two weeks apart to evaluate the ASQoL psychometric properties. Construct validity was assessed through convergent validity, using Notthingham Health Profile as a comparator and a known-group validity by comparisons with self-perceived disease activity and overall health. Internal reliability was determined by calculating Cronbach’s alpha coefficient. External validity was assessed by test-retest reliability. Also, correlations of the ASQoL with ASDAS, BASFI, BASDAI, respiratory index, Schober’s test and SPARCC index were determinedResults:There was a significant correlation between the ASQoL and NHP domains of pain (r=0.79), emotional reactions (r=0.78), physical activity (r=0.77) and energy (r=0.75). Cronbach alpha coefficents of 0.95 and 0.91 in Visit 1 and Visit 2, respectively, indicated adequate internal reliability. ASQoL showed minimal levels of random error and good test-retest reliability, with a Spearman correlation coefficient of 0.84. Serbian ASQoL was able to differentiate between patients with varying perceived general health status (p<0.05) and disease severity (p<0.05). Statistically significant correlations between ASQoL and ASDAS, BASDAI and BASFI were also demonstrated (p<0.001), whereas correlations with respiratory index, Schober’s test and SPARCC index were insignificant.Conclusion:The Serbian version of the ASQoL demonstrated excellent psychometric properties, and good corelations with disease activity and functional status indexes, affirming its validity as a reliable tool suitable for both clinical research and routine clinical usage.Figure 1. Table showcasing correlations between Serbian Ankylosing Spondylitis Quality of Life and different Nottingham Health Profile domains, as well as results of internal and external reliabilityMedianIQR% of patients scoring min% of patients scoring maxCorrelation coefficients with ASQoL (r)Cronbach alpha coefficientn= 60 patientsASQoLAdministration 13.51.0 – 10.023.31.7-0.95NHPEnergy level33.30 – 66.748.320.00.75-Pain18.80 – 62.538.38.30.79-Emotional reactions00 – 22.253.300.78-Sleep00 – 40.055.05.00.52-Social isolation00 – 080.000.50-Physical mobility12.50 – 37.533.300.77ASQoLAdministration 23.00 – 8.025.41.7-0.91Test-Retest0.84Note: All correlations are significant at the p< 0.001 level (2-tailed)Figure 2.Graphs showcasing correlation of ASQoL with BASDAI, ASDAS and BASFI.Note: All correlations are significant at the p< 0.001 level (2-tailed)REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Membranous glomerulonephritis (MGN) is a common cause of nephrotic syndrome in adults, mediated by glomerular antibody deposition to an increasing number of newly recognised antigens. Previous case reports have suggested an association between patients with anti-contactin-1 (CNTN1)-mediated neuropathies and MGN. In an observational study we investigated the pathobiology and extent of this potential cause of MGN by examining the association of antibodies against CNTN1 with the clinical features of a cohort of 468 patients with suspected immune-mediated neuropathies, 295 with idiopathic MGN, and 256 controls. Neuronal and glomerular binding of patient IgG, serum CNTN1 antibody and protein levels, as well as immune-complex deposition were determined. We identified 15 patients with immune-mediated neuropathy and concurrent nephrotic syndrome (biopsy proven MGN in 12/12), and 4 patients with isolated MGN from an idiopathic MGN cohort, all seropositive for IgG4 CNTN1 antibodies. CNTN1-containing immune complexes were found in the renal glomeruli of patients with CNTN1 antibodies, but not in control kidneys. CNTN1 peptides were identified in glomeruli by mass spectroscopy. CNTN1 seropositive patients were largely resistant to first-line neuropathy treatments but achieved a good outcome with escalation therapies. Neurological and renal function improved in parallel with suppressed antibody titres. The reason for isolated MGN without clinical neuropathy is unclear. We show that CNTN1, found in peripheral nerves and kidney glomeruli, is a common target for autoantibody-mediated pathology and may account for between 1 and 2% of idiopathic MGN cases. Greater awareness of this cross-system syndrome should facilitate earlier diagnosis and more timely use of effective treatment.

Ventilatory function was abnormal (forced vital capacity (FVC) 60% predicted) and there was slight oxygen desaturation in sleep. Since diagnosis she has been closely followed at the Royal London Hospital. The past 4 years have seen landmark clinical genetic studies of BVVLS. 3 In 2010 Green et al found mutations in the SLC52A3 gene in a consanguineous family with multiple affected individuals, a gene with a known role as an intestinal riboflavin transporter, thus suggesting that riboflavin deficiency might be important in BVVLS. 3 In 2012, the notion that defective riboflavin transport plays a role in BVVLS was further supported by the discovery of a mutation in a second member of the riboflavin transporter gene family, SLC52A2. 3 Furthermore, Ho et al found hemizygous deletions in the third riboflavin transporter gene (SLC52A1) in the mother of a newborn presenting with multiple acetyl coenzyme A dehydrogenase deficiency resulting from maternal riboflavin deficiency. 3 These findings have provided a rational basis for treatment of BVVLS with riboflavin supplements, with promising results. 5 The heterozygous mutation that we found in exon 2 of the SLC52A3 gene was not present in the mother.

IntroductionIn adults, muscle disease (MD) is often a chronic long-term condition with no definitive cure. It causes wasting and weakness of the muscles resulting in a progressive decline in mobility, alongside other symptoms, and is typically associated with reduced quality of life (QoL). Previous research suggests that a psychological intervention, and in particular Acceptance and Commitment Therapy (ACT), may help improve QoL in MD. ACT is a newer type of cognitive behavioural treatment that aims to improve QoL by virtue of improvement in a process called psychological flexibility. The primary aim of this randomised controlled trial (RCT) is to evaluate the efficacy of a guided self-help ACT programme for improving QoL in people with MD. Main secondary outcomes are mood, symptom impact, work and social adjustment and function at 9-week follow-up.Methods and analysisAcceptance and Commitment Therapy for Muscle Disease is an assessor-blind, multicentre, two-armed, parallel-group RCT to assess the efficacy of ACT plus standard medical care (SMC) compared with SMC alone. Individuals with a diagnosis of one of four specific MDs, with a duration of at least 6 months and with mild to moderate anxiety or depression (Hospital Anxiety and Depression Scale score ≥8) will be recruited from UK-based MD clinics and MD patient support organisations. Participants will be randomised to either ACT plus SMC or SMC alone by an independent randomisation service. Participants will be followed up at 3, 6 and 9 weeks. Analysis will be intention to treat, conducted by the trial statistician who will be blinded to treatment allocation.Ethics and disseminationThe study has received full ethical approval. Study results will be disseminated via peer-reviewed publications, conference presentations and journal articles. Data obtained from the trial will enable clinicians and health service providers to make informed decisions regarding the efficacy of ACT for improving QoL for patients with MD.Trial registration number NCT02810028.Protocol versionV.11 (4 April 2017).

Motor neuron disease is a progressive, fatal neurodegenerative disease for which there is no cure. Acceptance and Commitment Therapy (ACT) is a psychological therapy incorporating acceptance, mindfulness, and behaviour change techniques. We aimed to evaluate the effectiveness of ACT plus usual care, compared with usual care alone, for improving quality of life in people with motor neuron disease. We conducted a parallel, multicentre, two-arm randomised controlled trial in 16 UK motor neuron disease care centres or clinics. Eligible participants were aged 18 years or older with a diagnosis of definite or laboratory-supported probable, clinically probable, or possible familial or sporadic amyotrophic lateral sclerosis; progressive muscular atrophy; or primary lateral sclerosis; which met the World Federation of Neurology's El Escorial diagnostic criteria. Participants were randomly assigned (1:1) to receive up to eight sessions of ACT adapted for people with motor neuron disease plus usual care or usual care alone by a web-based system, stratified by site. Participants were followed up at 6 months and 9 months post-randomisation. Outcome assessors and trial statisticians were masked to treatment allocation. The primary outcome was quality of life using the McGill Quality of Life Questionnaire-Revised (MQOL-R) at 6 months post-randomisation. Primary analyses were multi-level modelling and modified intention to treat among participants with available data. This trial was pre-registered with the ISRCTN Registry (ISRCTN12655391). Between Sept 18, 2019, and Aug 31, 2022, 435 people with motor neuron disease were approached for the study, of whom 206 (47%) were assessed for eligibility, and 191 were recruited. 97 (51%) participants were randomly assigned to ACT plus usual care and 94 (49%) were assigned to usual care alone. 80 (42%) of 191 participants were female and 111 (58%) were male, and the mean age was 63·1 years (SD 11·0). 155 (81%) participants had primary outcome data at 6 months post-randomisation. After controlling for baseline scores, age, sex, and therapist clustering, ACT plus usual care was superior to usual care alone for quality of life at 6 months (adjusted mean difference on the MQOL-R of 0·66 [95% CI 0·22–1·10]; d=0·46 [0·16–0·77]; p=0·0031). Moderate effect sizes were clinically meaningful. 75 adverse events were reported, 38 of which were serious, but no adverse events were deemed to be associated with the intervention. ACT plus usual care is clinically effective for maintaining or improving quality of life in people with motor neuron disease. As further evidence emerges confirming these findings, health-care providers should consider how access to ACT, adapted for the specific needs of people with motor neuron disease, could be provided within motor neuron disease clinical services. National Institute for Health and Care Research Health Technology Assessment and Motor Neurone Disease Association.

BackgroundThe aim of this study was to assess possible relationship between the altered blood perfusion at hands analysed by 99mTc-pertechnetate hand perfusion scintigraphy (99mTcPHPS) and, morphological microvascular abnormalities detected by nailfold capillaroscopy (NC) in SSc patients.MethodsThe study group consisted of 25 patients with SSc (14 with diffuse SSc, and 11 with limited SSc) and 28 control subjects (18 patients with primary RP, and10 healthy individuals). NC and 99mTcPHPS was performed in all the groups examined. The capillaroscopic pattern was classsified as normal or scleroderma (”early”, ”active”, or ”late”) pattern. Gamma-camera dynamic first-pass study during the first 60 s and a static scintigraphy after 5 min were recorded following a bolus injection of 99m Tc-pertechnetate via a cubital vein. Regions of interest were drawn on the summed images around the fingers and the palmar region. The fingers-to-palm ratios were then calculated.ResultsSSc patients showed a significantly lower blood flow (BF) and blood pool (BP), (0.43±0.21 vs 0.36±0.07, respectively), than PRP patients (0.45±0.18 vs 0.42±0.06, respectively) and healthy subjects (0.58±0.19 vs 0.44±0.06, respectively), (p-value 0.039 vs 0.004, respectively). A gradual decrease of BF and BP was found in SSc patients with progressive severity of NVC patterns of microangiopathy [”early” (0.49±0.03 vs 0.39±0.04, respectively), ”active” (0.43±0.11 vs 0.38±0.06, respectively) or ”late” (0.40±0.28 vs 0.36±0.08, respectively), (p- value 0.462 vs 0.728 respectively], but these differences were not statistically significant. Patients with diffuse SSc showed lower BF, and higher BP (0.42±0.26 vs 0.37±0.07, respectively) than those with limited SSc, (0.44±0.14 vs 0.35±0.064, respectively), but this differences is not statistical significantly (p=0.76 vs p=0.53, respectively). There was no significant correlation between BF and BP values and type of SSc (limited or diffuse) (r=−0.06, p=0.77; r=0.13, p=0.54, respectively) as well as three microangiopathy patterns (r=−0.253, p=0.22; r=– 0.13, p=0.54, respectively).ConclusionsNC represents the best method to analyse microvascular damage in rheumatic diseases, especially SSc. 99m TcPHPS improves the evaluation of vascular damage in SSc patients. There is no direct relationship between these two methods, but one method complements another in the study of vascular damage in SSc patients.Disclosure of InterestNone declared