Effect of endorectal pullthrough on external anal sphincter integrity (in cases of Hirchsprung’s disease) using EMG

Objective The transanal mucosectomy of the aganglionic segment is a critical step in the transanal endorectal pullthrough procedure for the treatment of Hirchsprung’s disease. It exerts considerable traction on the anorectal tissue during dissection. Anal sphincter electromyography (EMG) is an indispensable parameter for the diagnosis of patients with any anorectal dysfunction. The aim of our study was to assess the integrity of the anorectal sphincter after transanal endorectal pullthrough using anal EMG.Methods This prospective study was carried out on 25 infants and children with Hirchsprung’s disease who underwent the endorectal pullthrough (soave) procedure. Needle EMG was used to assess the sphincter preoperatively and postoperatively.Results Preoperative EMG showed positive neuropathic changes in 28% of the patients. Postoperative EMG showed neuropathic changes in 60% of the patients, of whom 28% showed preoperative changes and 32% showed absolute postoperative findings, mostly related to difficult operative dissection.Conclusion The functional results of the endorectal pullthrough procedure were acceptable overall. Significance The reduced sphincter function encountered postoperatively was because of a combination of preoperative and intraoperative influences.Keywords: anal electromyography, Hirschsprung’s disease, transanal endorectal pullthroug

The second lumbrical-interossei latency difference in carpal tunnel syndrome: Is it a mandatory or a dispensable test?

Objective: To assess the value of the 2L-INT latency difference in the electrodiagnosis of the carpal tunnel syndrome (CTS) and evaluate its sensitivity in comparison to other routine median motor and sensory studies. Methods: The study was conducted on 100 hands with symptoms and signs suggestive of CTS and 100 non-CTS hands as the control group. All were subjected to routine median motor nerve conduction study with stimulation at midpalm, wrist and elbow, median-versus-radial sensory comparison study and Second lumbrical-versus-interosseus (2L-INT) motor comparison study. Results: The results showed that the most sensitive tests were the median-radial sensory test and the 2LINT test and that both were correlated suggesting that the motor fibers of the median nerve can be compressed as early as sensory fibers. Conclusion: The 2L-INT test is as sensitive and important as the median-radial sensory test. Significance: We recommend the routine use of the 2L-INT test in clinically suspected cases of CTS especially in cases where routine median motor studies are normal together with the median-radial sensory test even if the sensory studies are normal. KEYWORDS: Carpal tunnel syndrome, Second lumbrical-interossei latency differenc

Verification of an ultrasonographic scoring system in discriminating rheumatoid arthritis from osteoarthritic and normal joints in an Egyptian cohorts

Background The use of musculoskeletal ultrasound in rheumatoid arthritis (RA) has been growing over the last decades mainly to monitor response to treatment and for early detection of erosions. Suggestions to include this technique in the diagnosis of RA have been made, but not yet been implemented (because of the lack of specific sonographic criteria for RA). Objectives To verify the performance of a proposed combined structural and synovial scoring system in differentiating RA from osteoarthritis (OA) and healthy sonographic findings in the small joints of the hand. Patients and methods Twenty RA patients, 20 patients with hand OA, and 10 healthy controls were subjected to musculoskeletal ultrasound of the metacarpophalyngeal and proximal interphalyngeal joints. The novel proposed scoring system was applied characterizing each joint as either RA supported or RA unsupported. Grading of synovitis as mild, moderate, or severe was also performed. In the RA group, disease activity was assessed by Disease Activity Score 28 (DAS28) and anticyclic citrullinated peptide serum levels were measured. Results When one or more RA-supported joints were detected using this scoring system, it had a sensitivity of 100.0% and a specificity of 83.0%, with a diagnostic accuracy of 90.0%, for the diagnosis of RA. If two or more joints were detected, it had a sensitivity of 95.0% and a specificity of 96.7%, with a diagnostic accuracy of 96.0% for the diagnosis of RA. Conclusion The novel suggested combined structural and synovial scoring system showed high performance in differentiating RA from OA and controls

Evaluation of visfatin in patients with systemic lupus erythematosus: Correlation with disease activity and lupus nephritis

AbstractIntroductionRenal involvement affects about 50% of SLE patients accounting for significant morbidity and mortality in these patients. The adipokine “visfatin” acting as a growth factor for B-lymphocyte-precursors, exerts several proinflammatory functions. It was demonstrated as a marker of endothelial dysfunction (ED) in chronic kidney disease (CKD) thus could be a factor linking inflammation in SLE and kidney disease.Aim of the workTo assess serum visfatin level in SLE patients and its correlation to disease activity and lupus nephritis (LN) in these patients.Patients and methodsSerum level of visfatin using enzyme-linked immunosorbent assay (ELISA), chemical and immunological markers of SLE and LN were measured in 40 SLE patients and 40 age and sex matched healthy controls. Disease activity and renal involvement were assessed using SLE Disease Activity Index (SLEDAI) and Renal SLEDAI respectively further dividing patients into active versus inactive and LN versus non-LN respectively. Renal biopsies were taken from LN subgroup and were classified according to the modified WHO classification.ResultsA significantly higher serum visfatin level was found on comparing SLE patients (mean 109±180ng/ml, median18) with controls (mean 9.4±11ng/ml, median2.5) with statistically highly significant difference (z=5.2, P<0.001). Also there was a statistically significant difference as regards serum visfatin level between active SLE patients (mean 173±111ng/ml, median 14) and inactive patients (mean 139±88ng/ml, median 5) (z=2.1, P<0.05) as well as between patients with LN (mean 226±180ng/ml, median18) and patients with no LN (mean 101±140ng/ml, median 8(2-229)) (z=2.1, P<0.05). Visfatin had a highly significant positive correlation with disease duration (r=0.48, P<0.001), SLEDAI (r=0.62, P<0.001) as well as ESR, CRP and, renal score (r=0.45, 0.35, and 0.65, respectively) while inverse correlation with estimated GFR (r=−0.614) and C3 and C4 titre (r=−0.26, r=−0.35, respectively) was recorded. Visfatin showed high sensitivity in detecting active SLE and LN 83% and 85%, respectively.ConclusionSerum visfatin is strongly associated with LN in SLE patients and is a promising biomarker for prediction of renal involvement in these patients. It reflects SLE activity specially LN activity namely renal score and GFR decline. Further prospective studies are required to confirm visfatin as a destructive mediator of predictive and prognostic value in active lupus nephritis

Role of tumor necrosis factor-like weak inducer of apoptosis/fibroblast growth factor-inducible molecule 14 pathway in lupus nephritis

Objectives To evaluate urinary tumor necrosis factor-like weak inducer of apoptosis (uTWEAK) levels as well as renal fibroblast growth factor-inducible molecule 14 (Fn14) expression by immunohistochemistry in patients with systemic lupus erythematosus (SLE) to assess the possible role of TWEAK level as an indicator of lupus nephritis (LN) and its relation to disease activity as well as pathological LN classification. Patients and methods Urinary levels of TWEAK using enzyme-linked immunosorbent assay and chemical and immunological markers of SLE were measured in 30 patients with SLE and 15 age-matched and sex-matched apparently healthy controls. Patients with SLE were divided into two subgroups: 15 patients with LN and 15 without LN. Disease activity was assessed using systemic lupus erythematosus disease activity index SLE disease activity index (SLEDAI). Fn14 was examined in renal biopsies from LN group by immunohistochemistry. Results A significantly higher uTWEAK level was found in patients having SLE with LN compared with those without LN and controls (F=149.2, P<0.001). uTWEAK had a highly significant positive correlation with proteinuria (r=0.755, P<0.001) and a significant positive correlation with tSLEDAI and rSLEDAI (r=0.217, P<0.037 and r=0.476, P<0.024, respectively). uTWEAK had a significant negative correlation with anti-dsDNA titer, C3, and C4 (r=−0.579, P<0.008; r=−0.456, P<0.011; and r=−0.552, P<0.002, respectively). Fn14 expression was detected in mesangial and tubular cells, mainly proximal tubular cells, in patients with LN. Conclusion uTWEAK is a specific and sensitive biomarker for detection of active LN

Serum Calprotectin in Rheumatoid Arthritis: A Promising Diagnostic Marker, How Far Is It Related to Activity and Sonographic Findings?

Background: In the past 2 decades, there has been increasing interest in calprotectin. It is released and detected in serum and body fluids as a potentially useful clinical inflammatory marker. The protein has been described in synovial tissue in rheumatoid arthritis (RA) patients, specifically in the lining layer adjacent to the cartilage–pannus junction, which is the primary site of cartilage destruction and bone erosion. Assessment of inflammatory activity in RA is of pivotal importance for the optimal treatment. Our aim in this study is to measure the serum calprotectin levels in RA patients and to assess its association—if there is any—with disease activity score and radiological findings using the musculoskeletal ultrasound. Patients and methods: In our case control study, we included 44 RA patients (Group I) and 20 age- and sex-matched healthy volunteers who served as the control group (Group II). Both groups were subjected to full history taking and thorough clinical examination. Assessment of RA disease activity state was done for all RA patients using the Disease Activity Score 28. Laboratory investigations included the measurement of complete blood cell count, erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, anticitrullinated peptide antibodies, kidney, liver functions; serum calprotectin levels were determined using enzyme-linked immunosorbent assay and radiological joint assessment was done using musculoskeletal ultrasound score. Results: There was a statistically significant elevation of serum calprotectin levels among RA patients when compared with healthy controls. Statistically significant correlations were also found between serum calprotectin and the ultrasound grading score, Disease Activity Score 28, and erythrocyte sedimentation rate, which reflect the degree of inflammatory activity in the affected joints in RA patients. Moreover, the study yielded a significant correlation between serum calprotectin levels and rheumatoid autoantibodies (rheumatoid factor and anticitrullinated peptide antibodies), which are strong predictors of the aggressiveness of the disease. Serum calprotectin at a cutoff level of 93.9 μg/dL had 88.6% sensitivity and 100% specificity for diagnosis of RA. Conclusion: Calprotectin was found to have high association with laboratory and ultrasonography markers of inflammation in RA patients, so it is recommended for use as a marker of inflammatory activity in RA patients especially for the follow-up of patients on biological therapy to assess its efficacy

Vitamin D Deficiency in Egyptian Systemic Lupus Erythematosus Patients: How Prevalent and Does it Impact Disease Activity?

Background The emerging role of vitamin D in immunology and autoimmune disorders has been a worldwide interest in the last decade. Systemic lupus erythematosus (SLE) patients are particularly at a delicate position predisposing them to suffer from vitamin D deficiency due to the multiple risk factors accompanying the disease. Whether vitamin D deficiency is also involved as a risk factor for developing SLE and affecting its course is a considerable concern. Objectives The objective of this study was to estimate the prevalence of vitamin D deficiency in SLE patients and its relation to disease. MATERIALS AND METHODS: In our observational cross-sectional study, serum levels of vitamin D [25(OH)D] in 60 SLE patients and 30 age- and sex-matched healthy controls were assessed and estimated for deficiency and insufficiency at 10 and 30 ng/mL, respectively. Disease activity was evaluated by SLE disease activity index (SLEDAI), irreversible organ damage by Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI), and severity by Severity of Disease Index. Fatigue was measured by visual analog scale. Results Significantly lower levels of 25(OH)D were found in SLE patients (17.6 ± 6.9 ng/mL) in comparison to controls (79.0 ± 28.7 ng/mL), with a statistically high significant difference ( t = -11.2, P < 0.001). High prevalence of vitamin D insufficiency and deficiency was detected as 73.3% and 23.3%, respectively. Vitamin D had a highly significant negative correlation with SLEDAI ( r = -0.495, P < 0.001), SLICC ( r = -0.431, P < 0.05), and fatigue ( r = -0.436, P < 0.05). Conclusion Vitamin D deficiency and insufficiency were found to be prevalent in SLE patients in our study and related to disease activity and fatigue. If needed, routine screening and consequent repletion of vitamin D are recommended in SLE patients. Restoring adequate vitamin D levels in SLE patients should be more explored as a potential yet simple measure to their usual management to improve their condition

Consensus on the definitions and descriptions of the domains of the OMERACT Core Outcome Set for shared decision making interventions in rheumatology trials

Objective To gain consensus on the definitions and descriptions of the domains of the Outcome Measures in Rheumatology (OMERACT) core domain set for rheumatology trials evaluating shared decision making (SDM) interventions. Methods Following the OMERACT Handbook methods, our Working Group (WG), comprised of 90 members, including 17 patient research partners (PRPs) and 73 clinicians and researchers, had six virtual meetings in addition to email exchanges to develop draft definitions and descriptions. The WG then conducted an international survey of its members to gain consensus on the definitions and descriptions. Finally, the WG members had virtual meetings and e-mail exchanges to review survey results and finalize names, definitions and descriptions of the domains. Results WG members contributed to developing the definitions. Fifty-two members representing four continents and 13 countries completed the survey, including 15 PRPs, 33 clinicians and 37 researchers. PRPs and clinicians/researchers agreed with all definitions and descriptions with agreements ranging from 87% to 100%. Respondents suggested wording changes to the names, definitions and descriptions to better reflect the domains. Discussions led to further simplification and clarification to address common questions/concerns about the domains. Conclusion Our WG reached consensus on the definitions and descriptions of the domains of the core domain set for rheumatology trials of SDM interventions. This step is crucial to understand each domain and provides the foundation to identify instruments to measure each domain for inclusion in the Core Outcome Measurement Set. Clinical significance The current study provides consensus-based definitions and descriptions for the domains of the OMERACT core domain set for shared decision making interventions from patients/caregivers, clinicians and researchers. This is a crucial step to understand each domain and provides the foundation to identify instruments to measure each domain for inclusion in the Core Outcome Measurement Set for trials of SDM interventions