This study investigates the elastic properties and the photon and neutron shielding properties of TeO
2
–MgO–Na
2
O–Nd
2
O
3
glasses, as well as the mass stopping power and projected range of alpha ...and proton charged particles using SRIM software. PHITS 3.22, MCNP6.2 N-particle Monte Carlo codes and XCOM software were utilised to compute the mass attenuation coefficient (
μ
/
ρ
). The simulated and theoretical results were found to be very similar. The increase in
μ
/
ρ
values was caused by the addition of neodymium to the glasses. The lowest mean free path, half-value layer and tenth-value layer values were observed at the highest Nd
2
O
3
-doped sample. This showed that adding neodymium to glasses improves their photon radiation reduction ability. The glasses' fast neutron removal cross-sections (Σ
R
) were computed and the effective atomic number (
Z
eff
) and effective electron density (
N
eff
) were calculated using Phy-X
/
PSD software. The results revealed that the 75TeO
2
–15MgO–10Na
2
O glass is capable of stopping (proton and alpha) charged particles. In terms of photon and neutron attenuations, the glass system is recommended as a shielding material. In addition, the elastic properties of the samples were determined using bond compression and Makishima and Mackenzie method. According to both models, as the additive ratio increased, an improvement was observed in the elastic properties.
In this paper; photo-neutron cross-sections have been computed for
27
Al,
28,29
Si,
52
Cr,
54,56
Fe isotopes by using nuclear reaction codes TALYS 1.6 and EMPIRE 3.1 with different level density ...models. Constant Temperature Fermi Gas, Back Shifted Fermi Gas and Generalized Superfluid Models have been employed from TALYS 1.6 whereas Generalized Super Fluid Model was the only model employed from EMPIRE 3.1 reaction codes. Obtained results have been analyzed with experimental values taken from the EXFOR database.
BackgroundApproximately 5 to10% of FMF patients do not respond to colchicine treatment and/or intolerant to colchicine because of side effects. Several case reports and case series have pointed out ...the efficacy of IL-1 blockade in colchicine resistant FMFsubgroup.ObjectivesTo review the patients followed in our center with FMF who received anakinra, an anti IL-1 receptor antagonist, because of insufficient colchicine response.MethodsFMF patients who were treated with anakinra were retrospectively reviewed with regard to indication, effect on disease activity and acute phase response, adverse events. Patient global assessment was recorded before and after anakinra treatment.ResultsThere were 30 FMF patients with FMF who were treated with anakinra for various indications (amyloidosis in 7, colchicine resistant recurrent febrile attacks in 19, colchicine related side effects in 4). The mean age of this group was 36.34±11.64 years. The mean duration of the disease was 23.93±12 years. There were various co-existing pathologies among this study group like multiple sclerosis (1), ankylosing spondylitis (1), SLE (1), Behçet's disease (1), low grade lymphoma (1) and PAN (2). Four patients were pregnant. The mean colchicine dose was 2,08±0,5 mg/d. The mean duration of anakinra treatment was 13,5±12,93 months. Twenty three patients reported no attacks after anakinra treatment whereas 5 patients reported at least 50% decrease in the attack frequency. Mean patient global assessment decreased from 8,60±2,3 to 1,92±2,7 under anakinra treatment (p=0.001).Among the 7 patients with amyloidosis, anakinra was stopped in 2 patients because of increased proteinuria. However, a significant decrease in proteinuria was detected in 3 patients. One patient developed severe injection site reaction and therefore the drug was stopped. Overall, 4 of our patients with amyloidosis are still on Anakinra treatment.Three patients had a severe allergic reaction (severe disseminated rash in 1 patient and severe injection site reaction in 2 patients) and therefore the drug was stopped. Two patients had infections (one had genital warts and urinary tract infection, the other had sinusitis and folliculitis) and the treatment was terminated. One of our patients reported that her psoriatic lesions got worse on anakinra. Twenty two patients reported no adverse events during the treatment.ConclusionsAnakinra was effective in controlling the symptoms in colchicine-resistant FMF cases. It was also effective in FMF related amyloidosis. The major cause of treatment termination was injection site reactions. Anakinra seems to be an effective alternative in patients who have insufficient response to colchicine.Disclosure of InterestNone declared
BackgroundIt has been reported that anakinra, an anti IL-1 R antagonist, may be a safe alternative treatment during pregnancy in a series of patients with various autoinflammatory syndromes ...(1,2).ObjectivesTo assess the safety of anakinra in pregnant FMF patients and its effect on fetal and maternal outcomes.MethodsFour FMF patients who had to use anakinra during pregnancy because of recurrent febrile attacks despite prophylactic colchicine therapy in 3 and colchicine side effect in 1, were monitored closely for disease activity, side effects, fetal USG and pregnancy outcome.ResultsCase 1: A 33-year old FMF patient with a previous diagnosis of multiple sclerosis, developed severe myalgia and high fever at her 21st gestational week (GW) while receiving colchicine 1.5 mg/d. She didn't respond neither to 30 mg/d prednisolone nor to steroid pulses of 500 mg. Anakinra 100 mg/d was initiated. After the 3rd injection, she was symptom-free. At her 36th GW due to vaginal bleeding, she underwent C-section and gave birth to a healthy baby. After the birth she continued with regular 1,5 mg/d of colchicine and anakinra was stopped. The 1st minute APGAR score of the baby was 8. The baby was breast fed and after 30 month of follow-up the mother and the baby are healthy. This case is among the 5 patients that has been previously reported by Lachmann H in 2013 (1).Case 2: 28 year-old FMF patient on colchicine, experienced frequent febrile attacks of abdominal pain and myalgia after the 9th GW. She did not respond to 30 mg/d prednisolone. Anakinra 100 mg/day was started at the 12th GW. Her symptoms gradually decreased after the 2nd week of anakinra. Anti IL-1 treatment together with colchicine continued till delivery. The 1st minute APGAR score of the baby was 10. After the birth anakinra was stopped and she continued with colchicine (2 mg/d) without recurrence of the febrile attacks. The follow up is 18 months. The baby is breast fed and the development remains normal.Case 3: 31-year old FMF patient has been treated with anakinra since 2012 with the indication of recurrent protracted febrile myalgia unresponsive to colchicine 2 mg/d. She conceived while on anakinra treatment. Because she was symptom-free, anakinra was stopped at 29th GW and continued only with colchicine. However she flared 4 weeks later and anakinra was reintroduced. She is now at 36th GW and the control fetal USGs are normal.Case 4: 24 year-old FMF patient developed thrombocytopenia (39,000/mm3) at her 15th GW which we thought might be secondary to colchicine. Colchicine was stopped and anakinra 100 mg/d and prednisolone 10 mg/d were started at 15th GW. The initial improvement in the platelet count was lost when the steroid dose was tapered. Now she is at 37th GW and thrombocyte levels remain low but she is otherwise healthy.ConclusionsOur observation in 4 FMF patients provides additional data on the safety and outcome of pregnancies exposed to anakinra. IL-1 blockade seems to be a very effective approach in the treatment of protracted febrile myalgia, non-responsive to colchicine and corticosteroid therapy, even in pregnant FMF patients.ReferencesLachman HJ, et al. Anti IL-1 therapies and pregnancy outcome. Pediatric Rheumatology 2013, 11(Suppl 1): A269Chang Z, et al. Anakinra use during pregnancy in patients with cryopyrin-associated periodic syndromes (CAPS). Arthritis Rheumatol. 2014 Sep 15.Disclosure of InterestNone declared
BackgroundTransitional care is a purposeful, planed movement of adolescence and young adults with chronic condition from childhood- to adult-oriented health care systems. Well-organised, systematic ...transitional health care is of high importance for providing the continuous medical treatment and for reaching optimal outcomes. Up to date, there is no unique, consensus-based model for patients’ transition from childhood- to adult oriented health care centres in Turkey.ObjectivesWe aimed to assess the transitional care among patients with juvenile onset rheumatologic disease in referral tertiary centre in Turkey.MethodsThe transitional policlinic is held once per month at the department for paediatric rheumatology, consisting of 2 adult and 4 paediatric rheumatologist. A total of 147 patients have undergone the transitional care in the time period from May 2014 to December 2017. Patient s were called by telephone, by two different investigators (EP, OK). Thirty-three (22%) patients were not reached and 17 (11%) of theme were excluded from the study due to short post-transitional period (<6 months). Consequently, 97 (66%) patients have been reached and included in the study. Data on demographic, clinical and socio-economic features and experience with transitional practice were collected by using a structured questionnaire, which was fulfilled during the phone conversation between investigator and patient.ResultsA total of 147 patients (79 (54%) females) underwent transition process and 97 of them were included in the study. There was no statistically significant difference between different patients groups regarding the age of transition. The education levels of patients were as following: university 60 (61.9%), high-school 21 (21.6%), middle-school 13 (13.4%), primary school 3 (3.1%). Majority of patients was single at the time of study (79 (81.4%) patients) while only 18 (18.6%) patients were married; half of them being child owner. At the time of study, 44 (30%) patients were employed and mean age at employment was 19.06±3.1 years. Most of patients had health insurance at the time of study (94 (96.9%)). Seventy-one (73.2%) patients continued their regular follow up at adult department while 26 (25.8%) patients discontinued medical treatment. The most common reasons for cessation of follow up were the work/school absence (20 (76.9%)), followed by patients’ personal reasons (2 (7.6%)) and dissatisfaction with adult clinic services (4 (15.5%)). Most of patients reported satisfaction with transition process: 96 (99%).Abstract AB1079 – Table 1n=97 Female, n (%)58 (59.7%)Age at transition, mean±SD21.4±1.4 yearsAge at study mean±SD22.8±1.8 yearsJIA26 (26.8%)FMF60 (61.8%)ConnectiveTissueDisease7 (7%)Vasculitis4 (4%)ConclusionsThis is the first Turkish model of transitional care among patients with chronic rheumatic disease. Well-organised transitional health care is of crucial importance for the continuous and optimal health care of adolescents and young adults with chronic rheumatic disease. Further studies with higher number of patients would reveal the relevance of described transitional care model.Reference1 - Foster HE, Minden K, Clemente D, et al. EULAR/PReS standards and recommendations for the transitional care of young people with juvenile-onset rheumatic diseases. Ann Rheum Dis. 2017;76:639–646.Disclosure of InterestNone declared
BackgroundIn Adult-onset Still’s disease (AOSD), cases refractory to typical DMARDs, Canakinumab (an anti-IL-1ß monoclonal antibody) has been reported to be effective in a limited number of ...refractory cases.1 ObjectivesThe aim of this retrospective study was to represent AOSD patients treated with Canakinumab in 3 centres.MethodsThe follow up data of 10 AOSD patients (8 female, 2 male), who were followed up in outpatient clinics of 3 tertiary centres were reviewed retrospectively. The initial characteristics and follow up findings were reported.ResultsThe mean timespan between the initial diagnosis and Canakinumab treatment 45.2±29 months (mean ±SD). Before the onset of Canakinumab therapy, all patients were exposed to methotrexate, 1 to leflunomide, 8 to Tocilizumab and 8 to Anakinra. As for the biologic agents, 3 patients were also treated beforehand with Infliximab, 2 with Adalimumab, 2 with Etanercept and 2 with Rituximab. Canakinumab therapy was initiated in all patients with the indication of refractory disease under other medications, except for the one in whom neutropenia became evident under anakinra. The mean number of Canakinumab injections was 9.3±8. The mean follow-up period of patients treated with Canakinumab was 43.1±33 months. Seven out of 10 patients are still being treated with Canakinumab of 150 mg/month and one of 150 mg/every 2 months. One patient had a single injection and was fully controlled. The mean ferritin measure of 9 patients was reduced from 1292.3±1530 ng/ml to 354±530.2 ng/ml following the Canakinumab therapy (p=0.035). The mean of patient-reported global visual analogue scale (PG-VAS) scores was reduced from 7.4±2.4 to 2.3±2.2 with Canakinumab (p<0.001). Mean Erythrocyte sedimentation rate (ESR) was reduced from 44.2±35.1 to 22.7±26.5 with the help of Canakinumab therapy (p=0.005). Six patients are still on prednisolone at a maximum dose of 10 mg/day. The indication of therapy termination in the remaining 1 patient was the diagnosis of tuberculosis at 9th month of the treatment despite isoniazid prophylaxis. The patient was also treated with multiple biological agents beforehand, therefore it is not easy to conclude that treatment with Canakinumab induces tuberculosis flares.ConclusionsCanakinumab treatment seems to be effective in refractory AOSD patients who were previously treated with various agents. We state that an IL-1 blocking agent, Canakinumab is a relatively safe and effective alternative in managing refractory AOSD cases. On the other hand, randomised controlled trials are needed to further investigate the role of Canakinumab in these cases as well as its use as the first choice of biologic agents.Reference1 Kontzias A, Efthimiou P. The use of Canakinumab, a novel IL-1β long-acting inhibitor, in refractory adult-onset Still’s disease. Semin Arthritis Rheum2012;42(2):201–5.Disclosure of InterestNone declared
It has been demonstrated that normative data of pinch strengths (PSs) vary among healthy populations. Data from 838 participants (420 women and 418 men) aged 15–96 years were analyzed to develop ...normative data of PSs for a healthy Turkish population and to analyze changes in the means according to the physical demands of participants’ jobs. Lateral, palmar, and tip-to-tip pinch strengths were measured in standard manners. Occupations were classified based on the descriptions in the Dictionary of Occupational Titles. Mean PSs peaked between the ages of 30–50 years in general and increased a small amount in parallel with the increasing strength ratings of the occupations. PS changes followed a curvilinear relationship to age. The differences between our findings and those of similar studies in this field emphasize the importance of using normative data specific to a particular population.
BackgroundA decline in the frequency of AA amyloidosis secondary to rheumatoid arthritis and infectious diseases has been reported. This is probably due to more effective treatment strategies. We had ...previously reported that although amyloidosis occurs in less than 0.5% of BS patients, it is one of the leading causes of death.1–3 We had an impression that the frequency of amyloidosis is decreasing among our patients with BS.ObjectivesWe aimed to determine the change in the frequency of AA amyloidosis over years in BS pts in addition to elaborating on clinical characteristics and outcomes.MethodsWe performed a chart review to identify all pts with amyloidosis in our BS centre since 1976. We noted demographic characteristics, BS manifestations, age at BS and amyloidosis diagnosis, treatment modalities of these pts. Our endpoints were death and end stage renal disease (ESRD) requiring renal replacement therapy. The prevalence of amyloidosis was calculated separately for two periods (pts registered between 1976–2000 and 2000–2017)ResultsAmong our 9410 BS pts, 27 (0.29%) had amyloidosis.We identified 24 pts with amyloidosis among the 3820 pts in the earlier cohort and 3 additional amyloidosis among the 5590 pts in the recent cohort. The frequency of AA amyloidosis had declined from 0.62% to 0.054% in the recent cohort. M/F ratio was 22/5 and mean age at BS diagnosis was 29.5±7.4 years. Twenty-two (82%) of the pts with amyloidosis had major organ involvement (vascular involvement in 15, eye involvement in 13 and neurologic involvement in 2). Five (18%) of 27 pts had only mucocutaneous involvement.AA amyloidosis was diagnosed after a mean duration of 9.8±6.7 years (mean age at amyloidosis: 39.3±9.3 years) and was confirmed with renal biopsy in 14 pts and rectal biopsy in 13. Eight pts had non-nephrotic range proteinuria at amyloidosis diagnosis. After amyloidosis diagnosis, 24 pts continued their previous immunosuppressives and colchicine. Two of these 24 were on anti-TNFs at AA diagnosis. Biologics were initiated in 3 pts who were most recently diagnosed to have amyloidosis, anti-TNFs in 2 and tocilizumab in 1. Fourteen (52%) pts had died after a median follow-up of 3 (IQR:1–8.75) years, 3 were lost to follow-up just after amyloidosis diagnosis and 10 (37%) are still alive after a median follow up of 16 (IQR:10–23) years. The reasons for death were infections in 5, related to ESRD in 5, subarachnoid haemorrhage, gastric adenocarcinoma, liver cirrhosis and iatrogenic bowel perforation in 1 patient each. 10 (71%) of these 14 pts had developed ESRD before their deaths. Overall, 15/27 pts developed ESRD after a median follow-up of 3.5 (IQR:1.25–6.5) years after amyloidosis diagnosis. 5 of them had renal transplantation, all but 1 are still alive after 3, 4, 6, and 12 years.ConclusionsAA amyloidosis appears to be a rare, but fatal complication of BS. Around 50% of patients died after a median follow-up of 3 years after amyloidosis. This study showed a decreasing trend of AA amyloidosis due to BS similar to that observed in other inflammatory and infectious causes. The shorter follow-up duration may be contributing for the lower prevalence of AA amyloidosis in the recent cohort.References1 Yurdakul S, Arthritis Rheum1990.2 Melikoğlu M. Rheumatology(Oxford)2001.3 Kural-Seyahi E. Medicine (Baltimore)2003.Disclosure of InterestNone declared
In this study, the theoretical photo-neutron cross-sections produced by (γ,n) reactions for several structural fusion materials such as
51
V,
55
Mn,
58
Ni,
90,91,92,94
Zr, and
181
Ta have been ...investigated in the incident energy range of 7–40 MeV. Reaction cross-sections as a function of photon energy have been calculated theoretically using the PCROSS and TALYS 1.2 computer codes. TALYS 1.2 default and pre-equilibrium models have been used to calculate the pre-equilibrium photo-neutron cross-sections. For the reaction equilibrium component, PCROSS Weisskopf-Ewing model calculations have been preferred. The calculated results have been compared with each other and against the experimental data in the existing databases EXFOR and TENDL-2011. PCROSS Weisskopf-Ewing model calculations show a similar structure with experimental data but they are higher than the experimental values for all reactions except for
90
Zr(γ,n)
89
Zr reaction. Generally, TALYS 1.2 default and pre-equilibrium model cross-section calculations are the best agreement with the experimental data for all reactions except for
58
Ni(γ,n)
57
Ni reaction along the incident photon energy in this study. The TALYS 1.2 curves fit the TENDL-2011 data the best. If photo-neutron cross-section data is needed for an isotope where there is no experimental data available for comparison, TALYS 1.2 pre-equilibrium option has been recommended.
BackgroundIt is well known that menstruation triggers several conditions such as migraine, recurrent aphtous stomatitis and acne vulgaris in otherwise healthy individuals (1). It may exacerbate ...chronic autoimmune diseases and familial Mediterranean fever (FMF) (2–4). There is also one study that briefly mentions menstruation causes activation in Behçet's syndrome (BS) (4).ObjectivesWe investigated the relationship between menstruation and specifically the skin-mucosa lesions of BS. As controls, we studied FMF patients.MethodsPremenopausal women with BS and FMF seen consecutively at the outpatient clinic of Cerrahpasa Medical Faculty at Istanbul, were interviewed. BS patients were asked whether they experienced increased skin-mucosa lesions during the menstrual period. A similar questionnaire assessing this time the frequency of serositis and fever attacks was given to the patients with FMF. As a control the participants were also asked whether they experienced headaches during the same period as well.ResultsA total of 140 BS patients with a mean age of 36±8 and mean disease duration of 9±6 years were studied. While 21 (15%) were off treatment, 103 (74%) were using colchicine and the remaining were using other immunosuppressive agents. As shown in the Table, among BS patients, 78 (56%) associated at least one symptom with menstruation. The most commonly reported symptom related with menstruation was the papulopustular involvement (50%), followed by oral (30%) and genital ulcers (21%) and nodular lesions (21%).We also studied 185 patients with FMF. Their mean age was 32±8 and mean disease duration was 12±8 years. All patients were using colchicine for a mean duration of 8±7 years. A total of 138 patients (75%) reported that their attacks overlapped with menstruation. These attacks included mostly peritonitis in 126 patients (68%), pleuritis in 102 (55%), and fever in 73 (40%).Among both BS and FMF patients, similar number of patients (41% and 41%, respectively) reported that menstruation triggered headaches.YesNodo not rememberBehçet syndrome (n=140) Oral ulcer, n (%)42 (30)45 (32)53 (38) Genital ulcer, n (%)30 (21)94 (67)16 (11) Papulopustular lesions, n (%)70 (50)56 (40)14 (10) Nodular lesions, n (%)30 (21)90 (64)20 (14) At least one BS symptom, n (%)78 (55)–– Headache, n (%)58 (41)45 (33)37 (26)Familial Mediterranean fever (n=185) Peritonitis, n (%)126 (68)51 (28)8 (4) Pleuritis, n (%)102 (55)74 (40)9 (5) Fever, n (%)73 (39)100 (54)12 (6) At least one FMF symptom, n (%)138 (75)–– Headache, n (%)76 (41)77 (42)32 (17)ConclusionsThis survey showed that, in about half of the patients with BS at least one skin mucosa lesion is exacerbated with menstruation. Most commonly reported were the papulopustular lesions. Menstruation had a stronger effect on FMF, triggering at least one symptom in about ¾ patients. The main limitation of the study was the self-reported assessment methodology, rather than a prospective diary assessment. Our findings provide further evidence that papulopustular lesions of BS and acne vulgaris are pathologically related (5).ReferencesKhanna N et al. Indian J Dermatol Venereol Leprol. 1991;Akar S et al. Rheumatol Int. 2006;Colangelo K et al. Rheumatology (Oxford). 2011;Karadag O et al. Rheumatol Int. 2013;Kutlubay Z et al. Clin Exp Rheumatol. 2015Disclosure of InterestNone declared
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