C1 inhibitor deficiency: 2014 United Kingdom consensus document

C1 inhibitor deficiency is a rare disorder manifesting with recurrent attacks of disabling and potentially life-threatening angioedema. Here we present an updated 2014 United Kingdom consensus document for the management of C1 inhibitor-deficient patients, representing a joint venture between the United Kingdom Primary Immunodeficiency Network and Hereditary Angioedema UK. To develop the consensus, we assembled a multi-disciplinary steering group of clinicians, nurses and a patient representative. This steering group first met in 2012, developing a total of 48 recommendations across 11 themes. The statements were distributed to relevant clinicians and a representative group of patients to be scored for agreement on a Likert scale. All 48 statements achieved a high degree of consensus, indicating strong alignment of opinion. The recommendations have evolved significantly since the 2005 document, with particularly notable developments including an improved evidence base to guide dosing and indications for acute treatment, greater emphasis on home therapy for acute attacks and a strong focus on service organisation. This article is protected by copyright. All rights reserved

Analyzing Femorotibial Cartilage Thickness Using Anatomically Standardized Maps: Reproducibility and Reference Data.

Alterations in cartilage thickness (CTh) are a hallmark of knee osteoarthritis, which remain difficult to characterize at high resolution, even with modern magnetic resonance imaging (MRI), due to a paucity of standardization tools. This study aimed to assess a computational anatomy method producing standardized two-dimensional femorotibial CTh maps. The method was assessed with twenty knees, processed following three common experimental scenarios. Cartilage thickness maps were obtained for the femorotibial cartilages by reconstructing bone and cartilage mesh models in tree-dimension, calculating three-dimensional CTh maps, and anatomically standardizing the maps. The intra-operator accuracy (median (interquartile range, IQR) of -0.006 (0.045) mm), precision (0.152 (0.070) mm), entropy (7.02 (0.71) and agreement (0.975 (0.020))) results suggested that the method is adequate to capture the spatial variations in CTh and compare knees at varying osteoarthritis stages. The lower inter-operator precision (0.496 (0.132) mm) and agreement (0.808 (0.108)) indicate a possible loss of sensitivity to detect differences in a setting with multiple operators. The results confirmed the promising potential of anatomically standardized maps, with the lower inter-operator reproducibility stressing the need to coordinate operators. This study also provided essential reference data and indications for future research using CTh maps

Simultaneous Evaluation of Bone Cut and Implant Placement Accuracy in Robotic-Assisted Total Knee Arthroplasty.

Background: This study aimed to evaluate the accuracy of bone cuts and implant placements, simultaneously, for total knee arthroplasty (TKA) performed using a system with an active robotic arm. Methods: Two experienced orthopaedic surgeons performed TKA on ten cadaveric legs. Computed tomography scans were performed to compare the bone cuts and implant placements with the preoperative planning. The differences between the planned and actual bone cuts and implant placements were assessed using positional and angular errors in the three anatomical planes. Additionally, the cut-implant deviations were calculated. Statistical analysis was performed to detect systematic errors in the bone cuts and implant placements and to quantify the correlations between these errors. Results: The root-mean-square (RMS) errors of the bone cuts (with respect to the planning) were between 0.7-1.5 mm and 0.6-1.7°. The RMS implant placement errors (with respect to the planning) varied between 0.6-1.6 mm and 0.4-1.5°, except for the femur and tibia in the sagittal plane (2.9°). Systematic errors in the bone cuts and implant placements were observed, respectively, in three and two degrees of freedom. For cut-implant deviations, the RMS values ranged between 0.3-2.0 mm and 0.6-1.9°. The bone cut and implant placement errors were significantly correlated in eight degrees-of-freedom (ρ ≥ 0.67, p < 0.05). Conclusions: With most of the errors below 2 mm or 2°, this study supported the value of active robotic TKA in achieving accurate bone cuts and implant placements. The findings also highlighted the need for both accurate bone cuts and proper implantation technique to achieve accurate implant placements

Secondary antibody deficiency: a complication of anti-CD20 therapy for neuroinflammation

B-cell depleting anti-CD20 monoclonal antibody therapies are being increasingly used as long-term maintenance therapy for neuroinflammatory disease compared to many non-neurological diseases where they are used as remission-inducing agents. While hypogammaglobulinaemia is known to occur in over half of patients treated with medium to long-term B-cell-depleting therapy (in our cohort IgG 38, IgM 56 and IgA 18%), the risk of infections it poses seems to be under-recognised. Here, we report five cases of serious infections associated with hypogammaglobulinaemia occurring in patients receiving rituximab for neuromyelitis optica spectrum disorders. Sixty-four per cent of the whole cohort of patients studied had hypogammaglobulinemia. We discuss the implications of these cases to the wider use of anti-CD20 therapy in neuroinflammatory disease

COMPREHENSION & VISUALIZATION - Teaching Students to Solve Word Problems

ABSTRACT In this feasibility study the authors describe and evaluate a word problem solving instruction, based on the principles underlying instructional programs like Solve it! and schema-based instruction. This instruction is executed during a five-week intervention period in a group of four less successful second grade word problem solvers. The effectiveness of the word problem solving instruction is reported by means of students' performances on combine, change and compare problems before and after the intervention period, as well as by examining whether they executed the solution steps of the instruction correctly. This feasibility study provides important insights with regard to varying ways in which a word problem solving instruction can influence the solution strategies and performances of students who perform poorly on word problems. WORD PROBLEM SOLVING PROCESS Look at the following example of a word problem [Word problem example] "Mary has 9 marbles. She has 4 marbles more than John. How many marbles does John have?" Tim, a seven-year-old boy who is in the second grade of elementary school, has difficulties with solving word problems like the one that is given in the example above. While solving these word problems, Tim often uses an impulsive, superficial solution strategy. Significantly, he only focuses on selecting the presented numbers (9 and 4) and identifying the relational keywords (more than), which subsequently form the basis for his mathematical calculations. Tim's strategy often leads to an incorrect answer to the word problem. In this situation, Tim performed an addition operation where a subtraction operation was required, that is 9 + 4 = 13 instead of 9 -4 = 5. The incorrect answer is not the result of a lack of calculation ability, but a result of a problem with deeply and correctly understanding the word problem text. Mathematical word problem solving plays a prominent role in the curriculum of contemporary approaches to teaching mathematics [1

Selective reaching: evidence for multiple frames of reference

Students participated in 3 experiments investigating the use of environment- and action-centered reference frames in selective reaching. They pointed to a green target appearing either with or without a red distractor. Target–distractor distance was manipulated, and distractor interference (difference between distractor trials and no-distractor trials) was measured in reaction time, movement time, and movement endpoint. Target–distractor distance determined the dominant frame of reference. Small distances evoked an environment-centered framework that encoded targets within an external context. Large distances evoked an action-centered framework that encoded targets relative to the start position of the hand. Results support the hypothesis that the brain represents spatial information in multiple frames of reference, with the dominant frame of reference being dependent on the task demands. A well-known example used to highlight the concept of selec-tive attention is picking a ripe apple from a branch when an unripe apple is nearby on the same branch (e.g., Meegan & Tipper, 1999). This example shows that selective attention mediates goal-directed action through selection of appropriate actions and inhibition of inappropriate actions (i.e., picking the unripe apple). These selec