Aim-, The binding of AGEs to RAGE is involved in diabetic vascular complications. We studied sRAGE levels and RAGE protein expression (P) together with N-carboxymethyl lysine (CML), a major AGE, in 74 patients with type 1 diabetes mellitus (DM1) and 43 healthy (C) children. Methods: sRAGE and CML levels were determined by ELISA and RAGE P was evaluated in mononuclcar cells by Western immunoblotting.Results: Serum sRAGE was higher in DM1 than in C (1430 ± 759 vs 1158 ± 595 pg/ml, ρ = 0.047), inversely correlated to diabetes duration (r = -0.265, ρ = 0.037) and directly correlated to LDL-cholesterol levels (r = 0.224, ρ = 0.039). Diabetes duration correlated independently with sRAGE (p = 0.034). Circulating CML levels were not significantly different between DM1 and C groups (3.51 ± 1.49 vs 3.59 ± 1.83 ng/ml, p >0.05) and RAGE P was lower in DM1 than in C (61 ± 46 vs 102 ± 63%, p = 0.0001). Conclusions: Increased serum sRAGE in children with DM1 may provide temporary protection against cell damage and may be sufficient to eliminate excessive circulating CML.
Introduction Only few studies have presented results from real‐world clinical use of Extended Half‐Life (EHL) products in children with haemophilia (CWH). Aim To retrospectively examine real‐life experience with EHL factor VIII products use in CWH A, comparing with clinical experience from standard half‐life products (SHL). Methods A retrospective review of medical records of CWH A who have been prescribed EHL factor concentrates was conducted. All before/after comparisons were performed with the Wilcoxon matched‐pairs signed‐ranks test. Results Twenty‐three children with severe haemophilia A were enrolled in the study (3–6 years old: n = 4, 7–12 years old: n = 7 and 13–18 years old: n = 12). Median length of time that patients were treated with EHL products was 78 weeks. Median dosing interval was significantly lengthened from 2.3 to 3.5 days after switching from SHL to EHL concentrates. Mean trough FVIII levels were significantly increased from 2.3% to 4.1% after treatment with EHL products. Also, CWH A had a reduction of mean annual bleeding rate (ABR) and mean annual joint bleeding rate (AJBR) from 1 and .8 to .3 and .2, respectively, following treatment with EHL concentrates (ABR: p = .02, AJBR: p = .05). However, after switching to factor EHL, actual FVIII consumption, including bleeds, was significantly increased from 94 IU/kg/week to 118 IU/kg/week in CWH A. There was no inhibitor development. Conclusion This study demonstrates the successful transition of 23 CWH A from SHL to EHL factor concentrates.
The first Team Haemophilia Education (THE) Meeting was held on 7-8 May 2015 in Amsterdam, The Netherlands. It aimed to promote the optimal care of patients with haemophilia through education of the multidisciplinary treatment team. This was achieved by reviewing the latest developments in haemophilia management, considering how these can be implemented in the clinic to improve patient care and providing a platform for networking and debate for all haemophilia treatment team members. The second THE Meeting was held on 19-20 May in Frankfurt, Germany, and participants included doctors, nurses, physiotherapists, patient representatives and data management staff from 20 different countries. Topics covered the role of the multidisciplinary team in delivering the best haemophilia care, challenges in the management of haemophilia across Europe, available clotting factor treatments, future treatments and the use of genetics in advising carriers of haemophilia. This report is a summary of the key developments in haemophilia care presented by various investigators and healthcare professionals at THE Meeting 2016.
The increased esRAGE and FRAP with increased levels of CML and MDA possibly reflects a protective response against the formation of diabetic complications in these young diabetic patients.
The aims of the study were to evaluate the presenting features, treatment, and outcomes of patients with AHA in our local population, to identify the predictors of normalization of aPTT after immunosuppressive therapy and overall survival; and to investigate the patient characteristics that predict normalization of aPTT by steroid alone within 6 weeks. Methods: This was a retrospective multi-center study which recruited 108 patients diagnosed with AHA between 1 st January 2002 and 31 st December 2016. AHA is defined by the presence of a neutralizing FVIII inhibitor ≥ 0.6 Bethesda units (BU)/ml and a FVIII activity <50%. Patients with congenital hemophilia A with inhibitors were excluded. Prognostic factors for normalization of aPTT and overall survival were analyzed using Cox proportional hazards regression analysis. Results: Normalization of aPTT was an independent predictor of overall survival (OR 0.14, 95% CI 0.07-0.27, P < 0.001). Patients with ECOG performance status ≤2 were more likely to attain normal aPTT (HR 2.09, 95% CI 1.28-3.42, P 0.003) while patients with underlying autoimmune diseases were less likely to achieve normal aPTT (HR 0.46, 95% CI 0.23-0.91, P 0.027). Female (OR 3.73, CI 1.15-12.2, P 0.028) and an inhibitor titer ≤20 BU/ ml (OR 4.3,, P 0.013) predicted a higher chance of normalization of aPTT with steroid alone within 6 weeks. Summary/Conclusion: Identification of patients who are more likely to respond to steroid alone within 6 weeks may avoid unnecessary immunosuppressants and thus reduce treatment-related adverse events. This finding may help individualize treatment strategies. Normalization of aPTT was an independent prognostic factor of survival. This finding echoed other studies and reiterated the importance of achieving remission. To date, the optimal immunosuppressive strategy remains elusive. Further high-quality evidence is needed to guide the choice and intensity of treatment.
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