P042 Profile of systemic lupus erythematous patients at presentation 10 years of Single center experience-Tripoli-Libya

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Soad S Hashad ◽  
Iman A Almsellati ◽  
Majda N Etfil ◽  
Hala M Etayari ◽  
Zohairah O Awhaidah ◽  
...  

Abstract Background Juvenile Systemic Lupus Erythematous (JSLE) is a systemic autoimmune disorder with speckled manifestations that can emerge overstretched period of time and can affect any organ system, most frequently the skin, joints, kidneys, and the nervous, hematologic, and cardiovascular systems. The Aim is to examine the clinical features, serologic and laboratory characteristics associated with SLE. To probe and outline Clinical and Immunologic features of Incomplete Lupus Erythematous (ILE) patients who progressed from ILE to SLE. Methods The files of patients diagnosed as SLE in pediatric rheumatology clinic from 2001 to 5/2021 were retrospectively reviewed. Result Thirty SLE cases were included; Females were more prevalent with a female: male ratio of 14:1. Mean age at presentation 11 ± 4 years (range of 5 months-13 years), Disease onset was before sixth birthday in (7%), above twelve years in (40%) of the patients, and 53% of the patients was among 6 –12 years age group (31% & 37% of them fulfilled the SLICC & ACR criteria respectively at diagnosis) .The Mean duration between the onset of symptoms and SLE diagnosis was 6 months ±2 years. The Pre-pubertal age group presented early. At diagnosis, 50% of the patient got SLICC score criteria <4, on other hand 70% of the patients had ACR score criteria less than four Variable The most common presenting feature was arthritis (83%) (polyarticular arthritis) followed by dermatological manifestations (46%) photosensitivity, malar rash, and discoid rash in order of most frequent, fatigability (37%), renal manifestation (23%) most commonly as hematuria, one case diagnosed as lupus nephritis by renal biopsy. ANA was positive in 87%, anti-ds-DNA positive in 40% and Anti sm positive in 17%. The most frequently used medications were steroids and hydroxychlorqiune, the most commonly used steroid sparing medications were azathioprine (43%), Mycophenolate mofetil (40%) and ciclosporin (13%), 26% were on antihypertensive, 3% required rituximab to control their disease and one patient received Eltromboag to treat refractory thrombocytopenia. the mean follow-up duration 46.9 ± 43.6m, 20% lost follow up, 13% died due to disease complications (renal system involvement, thrombocytopenia and neurological system involvements). Conclusion jSLE in Libya is very rare before the sixth birthday and presented early among 6–12 years age group with a delay of less than one year between the first presentation and time of diagnosis. SLICC criteria was sufficient to diagnose the disease in > 50% of patients. High index of suspension should be maintained because in some patient’s years may be passed before fulfilling the diagnostic criteria. The most common cause of death is renal involvement. Glucocorticoids are the backbone of jSLE treatment in the acute phase. Both azathioprine and Mycophenolate mofetil are sufficient to control the disease in most patients. The disease outcome is accepted in our cohort as most patients have mild disease activity with low dose steroid and a steroid-sparing agent.

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 270.2-271
Author(s):  
J. Álvarez Troncoso ◽  
J. C. Santacruz Mancheno ◽  
A. Díez Vidal ◽  
S. Afonso Ramos ◽  
A. Noblejas Mozo ◽  
...  

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 588.2-588
Author(s):  
G. Olivieri ◽  
F. Ceccarelli ◽  
F. Natalucci ◽  
F. R. Spinelli ◽  
C. Alessandri ◽  
...  

Background:The updated EULAR recommendations for the management of systemic lupus erythematosus (SLE) underline the use of Mycophenolate Mofetil (MMF) in the treatment of different disease related manifestations (1). Several randomized controlled trials have demonstrated the efficacy of MMF in lupus nephritis (LN) patients but only case series and open-labelled trials have analyzed the use of this drug in other than LN features. Moreover, no data are available about the MMF retention rate in a real-life setting.Objectives:The present study aims at evaluating the 5-years drug retention rate (DRR) of MMF in a large monocentric SLE cohort. Secondly, we investigated the influence of MMF in disease activity changes and chronic damage progression.Methods:We performed a longitudinal study including all the SLE patients (ACR 1997 criteria) starting MMF treatment in our Lupus Clinic. Data about indications, mean dosage, duration of treatment and reasons for drug withdrawal were registered. The DRR was estimated using the Kaplan–Meier method. Disease activity and chronic damage were assessed by SLE Disease Activity Index 2000 (SLEDAI-2K) and SLICC Damage Index (SDI), respectively.Results:The present analysis included 162 SLE patients (M/F 22/140, median age at the disease diagnosis 25.5 years, IQR 13). At the beginning of MMF treatment, we registered a median age of 34 months (IQR 21) and a median disease duration of 72 months (IQR 123). The most frequent indications for prescribing MMF were LN (101 patients, 62.3%) and musculoskeletal manifestations (39, 24.1%), followed by neuropsychiatric involvement (10, 6.2%), and others disease related manifestations (12, 7.4%; in particular skin involvement, hematological features, myositis, vasculitis). MMF was administered at a mean daily dosage of 2.1±0.6 grams; no differences in dosage were found between the different indications (p=ns).At the longitudinal analysis, we registered a median treatment duration of 30 months (IQR 55). Figure 1 reported data about DRR: in particular, at 60 months follow-up we observed a DRR of 61.1% for LN patients, which was similar to that registered for patients without renal involvement (NLN) (60.5%; p=ns). Interestingly, the DRR at 60 months was higher in the subgroup of patients treated for joint involvement (75.4%), even without reaching a statistically significant difference. During the observation period, 92 patients (59.2%) discontinued MMF (median treatment duration at discontinuation 25 months, IQR 35). Interestingly, the main cause of withdrawal was the achievement of persistent remission, observed in 20 patients (21.7%), followed by loss of efficacy (19 patients, 20.5%), drug intolerance and pregnancy planning (17 patients for both reasons, 18,4%). Furthermore, our analysis confirmed MMF efficacy, as demonstrated by the significant reduction in SLEDAI-2k values after 4, 12 and 24 months of treatment (p< 0.0001 for all the time-points in comparison with baseline). In addition, MMF resulted able to control chronic damage progression, as demonstrated by the lack of significant increase in SDI values (baseline: 0.6, IQR 1; last observation: 0.93, IQR 1; p=ns).Conclusion:The evaluation of a large SLE cohort demonstrated a good retention rate for MMF. In particular, our results demonstrated that MMF is also a safe and effective drug for SLE manifestation other than LN, in particular for joint involvement. Moreover, it is able to control disease activity and to prevent the progression of chronic damage.References:[1]Fanouriakis A et al. Ann Rheum Dis. 2019 Jun;78(6):736-745.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1048.2-1048
Author(s):  
S. Herrera ◽  
J. C. Diaz-Coronado ◽  
D. Rojas-Gualdrón ◽  
L. Betancur-Vasquez ◽  
D. Gonzalez-Hurtado ◽  
...  

Background:Systemic lupus erythematosus (SLE) clinical manifestations, and their severity, vary according to age, ethnicity and socioeconomic status. Both Hispanic and Afro-Americans have a higher incidence and more sever presentation when compared to Caucasian patients with SLEObjectives:To analyze clinical and immunological characteristics associated with time to severe renal involvement in patients with Systemic Lupus Erythematous in a Colombian cohort followed for one year, between January 2015 and December 2018Methods:Retrospective follow-up study based in clinical records. Patients with SLE diagnosis that fulfilled either 1987 American College of Rheumatology Classification Criteria for SLE or 2011 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE. We included patients with diagnosis of lupus nephritis according to Wallace and Dubois criteria. Patients who did not have at least two follow-up measurements or had a cause of nephritis other than lupus were excluded. The main outcome was defined as time from diagnosis to sever renal involvement defined as creatinine clearance ≤50 ml/min, 24-hour proteinuria ≥3.5 grams o end stage renal disease.We analyzed clinical and immunological characteristics. Descriptive statistical analyses of participant data during the first evaluation are reported as frequencies and percentages for categorical variables, and as medians and interquartile ranges (IQR) for quantitative variables. Age and sex adjusted survival functions and Hazard ratios (HR) with 95% confidence intervals and p-values were estimated using parametric Weibull models por interval-censored data. P values < 0.05 were considered statistically significantResults:548 patients were analyzed: 67 were left-censored as they presented renal involvement at entry, 6 were interval censored as outcome occurred between study visits, and 475 were right-censored as involvement was not registered during follow-up. 529 (96.5%) patients were female, median age at entry was 46 (IQR = 23) and median age to diagnosis was 29.5 (IQR = 20.6). 67% were mestizo, 13% Caucasian and 0.3% Afro-Colombian. Age and sex adjusted variables associated with time to severe lupus nephritis were high blood pressure HR = 3.5 (95%CI 2.2-5.6; p-value <0.001) and Anti-RO (per unit increase) HR = 1.002 (95%CI 1.001-1.004; p-value = 0.04). Figure 1 shows age and sex adjusted survival function.Conclusion:In our cohort the appearance of severe lupus nephritis occurs in less than 15% of patients at 10 years. Both high blood pressure and elevated anti-Ro titers were associated with a higher rate of onset in the presentation of severe lupus nephritis, as seen in some polymorphs of anti Ro.References:Disclosure of Interests:Sebastian Herrera Speakers bureau: academic conference, Juan camilo Diaz-Coronado: None declared, Diego Rojas-Gualdrón: None declared, Laura Betancur-Vasquez: None declared, Daniel Gonzalez-Hurtado: None declared, Juanita Gonzalez-Arango: None declared, laura Uribe-Arango: None declared, Maria Fernanda Saavedra Chacón: None declared, Jorge Lacouture-Fierro: None declared, Santiago Monsalve: None declared, Sebastian Guerra-Zarama: None declared, Juan david Lopez: None declared, Juan david Serna: None declared, Julian Barbosa: None declared, Ana Sierra: None declared, Deicy Hernandez-Parra: None declared, Ricardo Pineda.Tamayo: None declared


2012 ◽  
Vol 2 (1) ◽  
pp. 12 ◽  
Author(s):  
Tomoaki Kimura ◽  
Kiyoshi Suzuki ◽  
Seiya Uchida ◽  
Hiroshi Katamura

Shorter and easier methods of conducting community health surveys would be useful. We conducted a study to demonstrate the responsiveness of the 10-item Mokichi Okada Association quality of life questionnaire (MQL-10) in a follow-up survey and to determine the minimally important difference (MID) for this measure. In 2007, Japanese adults participated in a survey on health prac- tices. We analyzed the MQL-10 scores (n=6365) together with the following factors: gender, age group, disease, reason for participation, and complementary health practices, such as food and eating. The mean baseline MQL-10 score was 26.4±5.83 [standard deviation (SD)] and the mean follow-up score was 27.6±5.45 SD with a mean change of 1.20±4.41 SD. The effect size for change was 0.21 and the standardized response mean was 0.27. The MQL-10 scores in the baseline condition were associated with gender, age group, disease, reason for participation and complementary health practices. Furthermore, the changes in the MQL-10 during the 12 weeks of study were associated with age group, disease, reason for participa- tion and complementary health practices. The increase in frequency of health practices was significantly associated with improvements in the participants’ quality of life (QOL). These results suggest that the MQL-10 is use- ful for assessing the effects of complementary health practices on QOL. The estimate of 3 points for the range of this measure (0-40) was higher than half of the SD of scores; therefore, it was considered reasonable for the MID.


2007 ◽  
Vol 67 (4) ◽  
pp. 500-504 ◽  
Author(s):  
A M Bertoli ◽  
L M Vilá ◽  
J D Reveille ◽  
G S Alarcón ◽  

Objective:To determine the features associated with acute onset systemic lupus erythaematosus (SLE).Methods:A total of 631 SLE patients from LUMINA (for “lupus in minority populations: nature vs nurture”), a multiethnic (Hispanics, African–Americans and Caucasians) cohort, were studied. Acute disease onset was defined as the accrual of ⩾4 American College of Rheumatology (ACR) criteria for the classification of SLE in ⩽4 weeks. Socioeconomic demographic features, clinical manifestations, disease activity, damage accrual, mortality, autoantibodies, HLA class II and FCGR alleles, behavioural/psychological variables were compared between patients with acute and insidious disease onset by univariable (χ2 and Student t test) and multivariable (stepwise logistic regression) analyses.Results:A total of 94 (15%) patients had acute disease onset. In the multivariable analysis, patients with acute onset lupus had more renal involvement (odds ratio (OR) = 1.845, 95% CI 1.076–3.162; p = 0.026) and higher disease activity (OR = 1.057, 95% CI 1.005–1.112; p = 0.030). By contrast, age (OR = 0.976, 95% CI 0.956–0.997; p = 0.025), education (OR = 0.901, 95% CI 0.827–0.983, p = 0.019), health insurance (OR = 0.423, 95% CI 0.249–0.718; p = 0.001) and skin involvement (OR = 0.346, 95% CI 0.142–0.843; p = 0.019) were negatively associated with acute onset lupus. No differences were found regarding the serological, genetic and behavioural/psychological features; this was also the case for damage accrual and mortality.Conclusions:Patients with acute onset lupus seem to be younger, have a lower socio-economic status and display more severe disease in terms of clinical manifestations and disease activity. However, intermediate (damage) and long-term (mortality) outcomes appear not to be influenced by the type of disease onset in SLE.


1979 ◽  
Vol 65 (3) ◽  
pp. 389-399 ◽  
Author(s):  
Fabrizio Lombardi ◽  
Marco Gasparini ◽  
Cristina Gianni ◽  
Raffaele Petrillo ◽  
John David Tesoro-Tess ◽  
...  

All the pertinent radiographs of 83 patients with histologically proven Ewing's sarcoma were reviewed. Forty-nine patients were in the pediatric age group, and 34 were adults. The mean age, the symptoms and time from symptoms to diagnosis were evaluated in the 2 groups. The site of primary involvement was in 54 % the long bones, 35 % the flat bones, 8 % the small bones and 3 % extraosseous. For the primary site we considered the diagnostic results of the standard radiographic investigations and in some cases the usefulness of angiography, xeroradiography and telethermography. At presentation we also evaluated the possible diffusion of the disease with standard radiographic surveys (chest and skeletal, including limbs) and with foot lymphography in selected cases. In this way, 57 patients (69 %) were considered to have localized disease. In this group, we also considered the value of the periodic radiographic follow-up, which enabled us to disclose the appearance of metastases (chest 64 %, bone 54 %, lymph nodes 11 %) in 28 cases (49 %). Finally, we made a comparison of the different radiologic and epidemiologic findings between children and adults.


2005 ◽  
Vol 15 (3) ◽  
pp. 166-170 ◽  
Author(s):  
K.H. Lin ◽  
Y.W. Lim ◽  
Y.J. Wu ◽  
K.S. Lam

The aims were to prospectively assess the mortality risk following proximal hip fractures, identify factors predictive of increased mortality and to investigate the time trends in mortality with comparison to previous studies. Prospectively collected data from 68 consecutive patients who had been admitted to a regional hospital from May 2001 to September 2001 were reviewed. The mean age of the patients was 79.3 years old (range, 55–98) and 72.1% females. Patients were followed prospectively to determine the mortality risk associated with hip fracture over a two-year follow-up period. The acute in-hospital mortality rate at six months, one year and two years was 5.9% (4/68), 14.7% (10/68), 20.6% (14/68) and 25% (17/68) respectively. One-year and two-year mortality for those patients who were 80 or older was significantly higher than for other patients and the number of co-morbid illnesses also had significant effect. Cox regression was performed to determine the significant predictors for survival time. It was noted that patients 80 years or older were at higher risk of death compared with those less than 80 years as well as those with higher number of co-morbid illnesses. Our mortality rates have not declined in the past 10 years when compared with previous local studies. We conclude that for this group of patients studied, their mortality at one year and two years could be predicted by their age group and their number of co-morbid illnesses.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1441-1441
Author(s):  
Zaher Naji ◽  
Madhvi Rajpurkar ◽  
Sureyya Savasan ◽  
Roland Chu ◽  
Meera Chitlur ◽  
...  

Abstract Abstract 1441 Chronic ITP and Evans syndrome are diseases characterized by autoantibody formation with resultant destruction of platelets or both platelets and red blood cells on neutrophils, respectively. Affected patients are at risk of life threatening bleeding complications and/or life threatening anemia. Conventional therapies are often ineffective or transiently effective and have significant toxicity. One of the most commonly used therapeutic strategies employs chronic corticosteroid administration with the attendant weight gain, hypertension, hyperglycemia, bone loss, infection risk, mood changes and protean other undesirable side effects. Mycophenolate Mofetil (MMF) is an immunosuppressive agent with a favorable side effect profile. It is converted to the active metabolite, mycophenolic acid, and interferes with purine metabolism in T-lymphocytes, effectively killing many of these cells and down-regulating autoimmune phenomena. With approval of our local IRB /HIC we retrospectively reviewed the charts of 11 chronic ITP/Evans syndrome patients who had received MMF, all such patients treated at a large urban Children's Hospital. Clinical variables included age, sex, duration of disease, steroid use, IVIG use, Anti-D use, platelet counts, hemoglobin concentrations and reticulocyte percentages. These data were analyzed using paired t-tests, one-sample t-test and descriptive statistics. The 11 patients ranged in age from 9–22 years old, with a mean age of 15 years. The mean time from diagnosis of disease was 41.8 months with a range of 6–95 months. There were 5 female subjects, 6 Evans syndrome patients and 5 with chronic ITP alone. The median platelet count over the 6 months prior to MMF was 70×109/L, (18-223), with a median of 90×109/L with (27-145) during the first 6 months of MMF therapy (p=0.4). In the Evans syndrome group, the mean hemoglobin prior to MMF was 10.9 g/dL, (9.1-14.6), with a mean of 12.1 g/dL, (6.9-16.7) on MMF (p=0.54). Similarly, the mean reticulocyte percentage was 2.7%, (0.5-14) prior to MMF, with a mean of 2.3%, (0.3-8.9) during MMF therapy. The mean total dose of steroids used in the 6 months prior to MMF was 84.2 mg/kg (prednisone equivalent), (11.2-170), compared to 62.2 mg/kg, (0-193.8) on MMF. 9 of 11 patients had reductions in steroid requirement by an average of 49.3% (p=0.0013). During the first 6 months of MMF both IVIG and anti-D usage decreased from total doses for the entire group of 17 and 3 doses to 8 and 1 dose, respectively. None of the patients experienced severe bleeding episodes or side effects more serious than a transient rash while on MMF. These data suggest that MMF may have a role as a steroid sparing therapy in the treatment of chronic ITP and Evans syndrome. Disclosures: Off Label Use: mycophenolate mofetil for ITP Evans syndrome.


2021 ◽  
Vol 8 ◽  
Author(s):  
Ho So ◽  
Evelyn Chow ◽  
Isaac T. Cheng ◽  
Sze-Lok Lau ◽  
Tena K. Li ◽  
...  

Objective: To investigate the factors associated with telemedicine (TM) use for follow-up of Systemic Lupus Erythematous (SLE) patients in the COVID-19 pandemic.Methods: This was a single-centered cross-sectional study conducted in Hong Kong. Consecutive patients followed up at the lupus nephritis clinic were contacted for their preference in changing the coming consultation to TM in the form of videoconferencing. The demographic, socioeconomic, and disease data of the first 140 patients opted for TM and 140 control patients preferred to continue standard in-person follow-up were compared.Results: The mean age of all the participants was 45.6 ± 11.8 years, and the disease duration was 15.0 ± 9.2 years. The majority of them were on prednisolone (90.0%) and immunosuppressants (67.1%). The mean SLEDAI-2k was 3.4 ± 2.4, physician global assessment (PGA) was 0.46 ± 0.62 and Systemic Lupus International Collaborating Clinics (SLICC) damage index was 0.97 ± 1.23. A significant proportion of the patients (72.1%) had 1 or more comorbidities. It was found that patients with higher mean PGA (TM: 0.54 ± 0.63 vs. control: 0.38 ± 0.59, p = 0.025) and family monthly income &gt; USD 3,800 (TM: 36.4% vs. control: 23.6%; p = 0.028) preferred TM, while full-time employees (TM: 40.0% vs. control: 50.7%; p = 0.041) preferred in-person follow-up. These predictors remained significant in the multivariate analysis after adjusting for age and gender. No other clinical factors were found to be associated with the preference of TM follow-up.Conclusion: When choosing the mode of care delivery between TM and physical clinic visit for patients with SLE, the physician-assessed disease activity and patient's socio-economic status appeared to be important.


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