Two studies examined contingent take-home medication doses during treatment of opiate or cocaine dependence. In the first study, methadone maintenance patients were randomly assigned to one of two 8-week baseline take-home (TH) conditions differing in frequency of clinic visits per week. This was followed by a 12-week contingency management (CM) procedure in which frequent THs resulted from drug-free urines. Participants receiving more frequent THs during baseline had lower illicit drug use during the first 6 weeks of CM. In the second study, fluoxetine (0-, 20-, 40-mg) TH doses were similarly contingent in treatment of cocaine dependence. The 40-mg group used less cocaine during contingency than did other groups. The combination of fluoxetine and environmental contingencies may produce benefit where neither alone is sufficient.
Objective: KMT2B-related dystonia is a progressive childhood-onset movement disorder, evolving from lower-limb focal dystonia into generalized dystonia. With increasing age, children frequently show prominent laryngeal or facial dystonia manifesting in dysarthria. Bilateral deep brain stimulation of the globus pallidus internus (GPi-DBS) is reported to be an efficient therapeutic option. Especially improvement of dystonia and regaining of independent mobility is commonly described, but detailed information about the impact of GPi-DBS on dysarthria and speech is scarce.Methods: We report the 16-months outcome after bilateral GPi-DBS in an 8-year-old child with KMT2B-related dystonia caused by a de-novo c.3043C>T (p.Arg1015*) non-sense variant with special emphasis on dysarthria and speech. We compare the outcome of our patient with 59 patients identified through a PubMed literature search.Results: A remarkable improvement of voice, articulation, respiration and prosodic characteristics was seen 16 months after GPi-DBS. The patients' speech intelligibility improved. His speech became much more comprehensible not only for his parents, but also for others. Furthermore, his vocabulary and the possibility to express his feelings and wants expanded considerably.Conclusion: A positive outcome of GPi-DBS on speech and dysarthria is rarely described in the literature. This might be due to disease progression, non-effectiveness of DBS or due to inadvertent spreading of the electrical current to the corticobulbar tract causing stimulation induced dysarthria. This highlights the importance of optimal lead placement, the possibility of horizontal steering of the electrical field by applying directional stimulation with segmented leads as well as the use of the lowest possible effective stimulation intensity.
Background:Immature granulocytes (IG) level in peripheral blood is used as early sign of infection. On the other hand, IG could be elevated in other conditions like inflammatory or cancerous diseases and in pregnancy. Similar to CRP, we have observed a series of cases of peripheral enthesitis associated increased level of IG and correlated with CRP elevation and clinical activity.Objectives:To test the concept of IG as a biomarker in peripheral enthesitis, This observation might be a reflection to the innate immunity role in pathogenesis of enthesitis.Methods:We have identified 13 cases over the last 12 months at the Rheumatology clinic in our centre who have shared two features (clinical enthesitis and elevated IG) those 13 cases are mainly presented with recurrent foot and ankle pain and swelling of enthesitis nature.Results:The cohort is of equal gender distribution 46/54% respectively and age range 31-53 years (mean age 38.5), only 3 patients are known to have Psoriatic arthritis whilst the rest either undiagnosed or diagnosed Rheumatoid arthritis or gout prior to clinic visit. The rheumatologist clinical diagnoses are of enthesitis of foot, ankle, knee and hip area as shown on the table [1] apart from one patient who has spinal symptoms mainly. 4/13 patients have the history of psoriasis and after their visits we find them fit in the CASPAR criteria for psoriatic arthritis. All these cases are associated with increased absolute number of IG as well as differential (IG) number compare to 10/13 of these cases are having high CRP. The 3 cases with normal CRP do have relatively slight increase in (IG) number. The main correlation is of response to therapy and was seen in 7 cases who have followed up at the time of submission and it shows a 100% correlation between CRP, absolute (IG) and differential (IG) values. There are 3 patients who have elevated (IG) but normal CRP and it would possible indicate (IG) test has a better yield than CRP in peripheral enthesitis.Conclusion:Immature granulocytes can be elevated in inflammatory disease and more notably in peripheral enthesitis of the lower limbs of inflammatory rather than mechanical nature and do correlate with CRP elevation as well as response to therapy. Larger studies are needed to assess the usefulness and validity of (IG) level in clinical practice.References[1] Rudwaleit Met al, The Assessment of SpondyloArthritis International Society classification criteria for peripheral spondyloarthritis and for spondyloarthritis in genera, Ann Rheum Dis. 2011;70(1):25[2] Moots Ret al, Low-density granulocytes: functionally distinct, immature neutrophils in rheumatoid arthritis with altered properties and defective TNF signaling, Translational & Clinical Immunology 2016 https://doi.org/10.1189/jlb.5A0116-022R[3] Salvarani c et al, Isolated peripheral enthesitis and/or dactylitis: a subset of psoriatic arthritis. J Rheumatol. 1997 Jun;24(6):1106-10Disclosure of Interests:None declared
Systemic sclerosis is an autoimmune condition characterized by a wide range of clinical presentations. Registries may serve to expand understanding about systemic sclerosis and aid in patient care and follow-up. The objective of this study was to analyze the prevalence of systemic sclerosis in a large cohort from the United Arab Emirates Systemic Sclerosis Registry and find the significant similarities and differences between the different subsets. All scleroderma patients in the United Arab Emirates were included in this multicenter national retrospective analysis. Data on demographics, comorbidities, serological characteristics, clinical aspects, and treatment were collected and analyzed, highlighting the most common traits identified. A total of 167 systemic scleroderma patients from diverse ethnic backgrounds were enrolled. Overall, 54.5% (91/167) of the patients were diagnosed with diffuse cutaneous systemic sclerosis, and 45.5% (76/167) with limited cutaneous systemic sclerosis. The prevalence of systemic sclerosis was 1.66 per 100,000 for the total registry and 7.78 per 100,000 for United Arab Emirates patients. Almost all patients in the diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis groups tested positive for the immunofluorescence antinuclear antibody. Antibodies against Scl-70 were significantly more associated with diffuse cutaneous systemic sclerosis, whereas anticentromere antibodies were significantly more associated with the limited cutaneous systemic sclerosis group ( p < 0.001). Sclerodactyly, shortness of breath, and digital ulcers were more common in diffuse cutaneous systemic sclerosis patients compared with the limited cutaneous systemic sclerosis subtype in terms of clinical symptoms and organ involvement. Telangiectasia was much more common in the limited cutaneous systemic sclerosis group. Furthermore, diffuse cutaneous systemic sclerosis patients had more lung fibrosis (interstitial lung disease) than limited cutaneous systemic sclerosis patients (70.5% vs 45.7%), and pulmonary arterial hypertension was twice as common in limited cutaneous systemic sclerosis patients as it was in diffuse cutaneous systemic sclerosis patients. Local registries are paramount to understanding the clinical/serological characteristics of scleroderma. This study emphasizes the importance of raising disease awareness and distinguishing between the various systemic sclerosis subsets to implement patient-tailored strategies for early detection, better management, and higher quality of care.
Background The use of interferon gamma-release assays (IGRA) has become a very useful tool in the detection of latent tuberculosis (LTB) among patients on biological disease modifying anti-rheumatic drugs (bDMARDs). The IGRA offers some advantages over the intradermal tuberculin skin test (TST), such as, no requirement to re-schedule the patient for the test reading, the elimination of the inter-reader variability and higher specificity. These features facilitate the decision making when deciding which patients require tuberculosis (TB) chemoprophylaxis before starting bDMARDs, particularly in world regions with high incidence of vaccinated individuals and new TB cases. In our tertiary level hospital, the IGRA QuantiFERON-TB Test® (Cellestis Ltd, Australia), was introduced as routine screening test for LTB in late 2008 together with the usual chest X-ray, initially run concurrently and then substituting the old TST in most of the cases. Objectives In this report, we present the benefits of its use in decreasing the number of active TB cases among rheumatoid arthritis (RA) patients prescribed bDMARDs in our centre. Methods An audit was undertaken through the electronic medical record system of our hospital, which was implemented from January 2011 for all medical documentation. All RA patients aged 18 and over seen in the rheumatology outpatient clinic between Jan 2011 and September 2013 and on bDMARDs at any time during this period were identified. Among the cohort of RA patients on bDMARDs, we searched for patients with diagnosis of active TB or receiving anti-TB triple/quadruple therapy. Results were compared to previous RA cohort from the same hospital from the period 2003-2008, which was facilitated by two of the authors of this present paper who worked in our hospital during this period. Results Ninety-seven individuals diagnosed with RA and treated with any of the bDMARDs available during the study period were found. There were no documented cases of TB or active TB treatment among the 97 individuals on bDMARDs during the 34-month audit period. This contrasts with the three cases of TB reactivation among the 86 patients who received bDMARDs in our centre from 2003 to 2008. Conclusions Our data suggest that the IGRA QuantiFERON-TB Test® is more useful than the TST for the LTB screening in adult population on bDMARDs. This is particularly important in mixed populations with a high percentage of individuals being nationals from endemic countries in TB. Our recommendation is to systematically use IGRA in such settings, repeating this on an annual basis. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5158
Background Diagnostic discordance for osteoporosis is the presence of different T-scores in two skeletal sites in the same subject leading to different WHO diagnostic categories. Discordance is defined as minor when the difference between two sites is no more than one WHO diagnostic class and major when one site is osteoporotic and the other is normal. Objectives To determine the percentage of minor and major diagnostic discordance and identify associated factors in patients from the United Arab Emirates (UAE) diagnosed with osteoporosis. Methods All patients born in North Africa-Middle East region (as per WHO classification) ≥50 years old seen in rheumatology clinics of our tertiary level hospital from 2011 to 2013, diagnosed with osteoporosis were identified through an internal audit. Details of the first Dual-X-Ray-Absorptiometry test (DXA) during the study period were extracted, including weight, height, T score at femoral neck and total hip in the right side (RFN, RTH) and left side (LFN,LTH) and T score at lumbar spine (LS). Differences in T scores and degree of discordance between sites were calculated. Age, weight, height and BMI were analysed as contributing factors. Results One hundred and seventy-one patients with osteoporosis and DXA test were identified. The mean age was 64.4 (±9.4 SD) and 142 (83%) were females. Diagnostic agreement among all skeletal sites was found in only 16% of patients. Of the cohort, 42% showed one major discordance and 65% showed minor discordance. Maximum concordance was found between RTH and LTH (80%) and minimum between LS and LTH (26%). Minor discordance was present in about half of the patients when comparing spine to any hip site, and in around one quarter when comparing ipsilateral TH and FN sites. Major discordance in LS compared to hip locations was present almost in one third of the patients. Of the four possible spine-hip comparisons, between 89 and 91% of the discordant cases exhibited LS scores lower than the respective hip site. No significant correlation with age, weight, height or BMI was found for the degree of discordance. Conclusions Major and minor spine-hip diagnostic discordance is higher in patients from UAE diagnosed with osteoporosis compared to that reported elsewhere1,2. Inter-hip discordance is compatible with previous data3. Multiple-site measurements seem mandatory not to miss patients at high risk for osteoporosis. References Gnudi S et al, J Clin Densitom 2000 Mounach A et al, Seminars Arthritis Rheum 2009 Hamdy R et al, Osteoporos Int 2006 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4325
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