The preventive Effectiveness of Rituximab in Pediatric Autoimmune and Inflammatory CNS Diseases Relapse: an Iranian Experience.

Objectives: To determine the effectiveness of Rituximab (RTX) therapy as the first therapeutic choice for the long-term prevention of secondary relapse in children with AIND that had relapse after primary treatment with immunosuppressive agents other than RTX. Materials & Methods: We conducted a single-center retrospective study of 9 consecutive pediatric patients (≤ 18 years old) registered on Autoimmune and Demyelinating Disorders Database (ADDD) of Mofid Children Hospital, from 2012 to 2016 and experienced relapse following therapeutic interventions with immunosuppressive agents other than RTX. Result: A remarkable reduction of 94.13% (p=0.015) occurred in annualized relapse rate (ARR) as a clinical indicator of therapeutic efficacy comparing before and after initiating RTX therapy. Conclusion: Rituximab is an effective drug in relapse prevention of AIND when administrated to patients for whom initial treatment with other immunosuppressive agents fail.POWER OF EVIDENCE: This study represents Class IV evidence that RTX therapy significantly reduces ARR in pediatric AIND including DDCNS.

Calculation of Mitral Valve Area in Mitral Stenosis: Comparison of Continuity Equation and Pressure Half Time With Two-Dimensional Planimetry in Patients With and Without Associated Aortic or Mitral Regurgitation or Atrial Fibrillation.

Accurate measurement of Mitral Valve Area (MVA) is essential to determining the Mitral Stenosis (MS) severity and to achieving the best management strategies for this disease. The goal of the present study is to compare mitral valve area (MVA) measurement by Continuity Equation (CE) and Pressure Half-Time (PHT) methods with that of 2D-Planimetry (PL) in patients with moderate to severe mitral stenosis (MS). This comparison also was performed in subgroups of patients with significant Aortic Insufficiency (AI), Mitral Regurgitation (MR) and Atrial Fibrillation (AF). We studied 70 patients with moderate to severe MS who were referred to echocardiography clinic. MVA was determined by PL, CE and PHT methods. The agreement and correlations between MVA's obtained from various methods were determined by kappa index, Bland-Altman analysis, and linear regression analysis. The mean values for MVA calculated by CE was 0.81 cm (±0.27) and showed good correlation with those calculated by PL (0.95 cm, ±0.26 ) in whole population (r=0.771, P<0.001) and MR subgroup (r=0.763, P<0.001) and normal sinus rhythm and normal valve subgroups (r=0.858, P<0.001 and r=0.867, P<0.001, respectively). But CE methods didn't show any correlation in AF and AI subgroups. MVA measured by PHT had a good correlation with that measured by PL in whole population (r=0.770, P<0.001) and also in NSR (r=0.814, P<0.001) and normal valve subgroup (r=0.781, P<0.001). Subgroup with significant AI and those with significant MR showed moderate correlation (r=0.625, P=0.017 and r=0.595, P=0.041, respectively). Bland Altman Analysis showed that CE would estimate MVA smaller in comparison with PL in the whole population and all subgroups and PHT would estimate MVA larger in comparison with PL in the whole population and all subgroups. The mean bias for CE and PHT are 0.14 cm and -0.06 cm respectively. In patients with moderate to severe mitral stenosis, in the absence of concomitant AF, AI or MR, the accuracy of CE or PHT method in measuring MVA is nearly equal. But in the presence of significant AI or MR, PHT method is obviously superior to CE and in the presence of AF neither have sufficient accuracy.