Pseudoartrosis de húmero en la enfermedad de Paget: presentación de un caso

Presentamos un paciente diagnosticado de enfermedad de Paget que sufrió una fractura patológica del húmero. El tratamiento ortopédico de la fractura deparó una pseudoartrosis. El resultado del tratamiento quirúrgico de ésta fue bueno. Se discute la incidencia de la afectación del húmero en la enfermedad de Paget y aspectos relativos a la curación de las fracturas del mismo.A patient with Paget's disease who had a pathological fracture of the humerus was presented. The fracture was orthopedically treated and developed non-union. Good result was achieved with surgical treatment. Incidence of Paget's disease on the humerus and healing in these fractures are discussed

Familial association of genetic generalised epilepsy with limb-girdle muscular dystrophy through a mutation in CAPN3

Muscular dystrophies are a heterogeneous group of inherited dis-eases that cause progressive muscle weakness. The association of epi-lepsy with some of these diseases has been previously described andhas most commonly been found for Fukuyama-type muscular dystro-phy due to alterations in cerebral neuronal migration[1]. Among mus-cular dystrophies, limb-girdle muscular dystrophies (LGMDs)represent the fourth most common group, with a prevalence of 1.63per 100, 000 individuals[2]. The diseases in this group share a commonphenotype involving progressive weakness of the scapular and pelvicgirdles that starts after 2 years of age and can be accompanied by differ-ent degrees of elevation in blood creatine kinase (CK) levels and by var-ious anatomic pathologicalfindings. LGMDs are subdivided into LGMD1and LGMD2 depending on whether the inheritance is dominant or re-cessive, respectively. LGMD2A, which is caused by deficiency of thecalpain 3 protein owing to mutations in theCAPN3gene, is the mostcommon form of LGMD in Europe and America[2]. Its associationwith epilepsy has been described in only two isolated cases[1, 3], bothof them on the spectrum of genetic generalised epilepsies (GGEs). Thelatter are the most common group of epilepsies with genetic aetiology, accounting for 15–20% of all epilepsy cases[4]. Nonetheless, none of thegenes usually involved in monogenic epilepsies seem to play a majorrole in GGE, probably indicating a polygenic predisposition to GGE andtherefore a complex inheritance pattern[5]. Here, we describe a family..

Evolution of the use of corticosteroids for the treatment of hospitalised COVID-19 patients in Spain between March and November 2020: SEMI-COVID national registry

Objectives: Since the results of the RECOVERY trial, WHO recommendations about the use of corticosteroids (CTs) in COVID-19 have changed. The aim of the study is to analyse the evolutive use of CTs in Spain during the pandemic to assess the potential influence of new recommendations. Material and methods: A retrospective, descriptive, and observational study was conducted on adults hospitalised due to COVID-19 in Spain who were included in the SEMI-COVID- 19 Registry from March to November 2020. Results: CTs were used in 6053 (36.21%) of the included patients. The patients were older (mean (SD)) (69.6 (14.6) vs. 66.0 (16.8) years; p < 0.001), with hypertension (57.0% vs. 47.7%; p < 0.001), obesity (26.4% vs. 19.3%; p < 0.0001), and multimorbidity prevalence (20.6% vs. 16.1%; p < 0.001). These patients had higher values (mean (95% CI)) of C-reactive protein (CRP) (86 (32.7-160) vs. 49.3 (16-109) mg/dL; p < 0.001), ferritin (791 (393-1534) vs. 470 (236- 996) µg/dL; p < 0.001), D dimer (750 (430-1400) vs. 617 (345-1180) µg/dL; p < 0.001), and lower Sp02/Fi02 (266 (91.1) vs. 301 (101); p < 0.001). Since June 2020, there was an increment in the use of CTs (March vs. September; p < 0.001). Overall, 20% did not receive steroids, and 40% received less than 200 mg accumulated prednisone equivalent dose (APED). Severe patients are treated with higher doses. The mortality benefit was observed in patients with oxygen saturation </=90%. Conclusions: Patients with greater comorbidity, severity, and inflammatory markers were those treated with CTs. In severe patients, there is a trend towards the use of higher doses. The mortality benefit was observed in patients with oxygen saturation </=90%

FarmDay: A Gamified Virtual Reality Neurorehabilitation Application for Upper Limb Based on Activities of Daily Living

Patients with upper limb disorders are limited in their activities of daily living and impose an important healthcare burden due to the repetitive rehabilitation they require. A way to reduce this burden is through home-based therapy using virtual reality solutions, since they are readily available, provide immersion, and enable accurate motion tracking, and custom applications can be developed for them. However, there is lack of guidelines for the design of effective VR rehabilitation applications in the literature, particularly for bimanual training. This work introduces a VR telerehabilitation system that uses off-the-shelf hardware, a real-time remote setup, and a bimanual training application that aims to improve upper extremity motor function. It is made of six activities and was evaluated by five physiotherapists specialised in (2) neuromotor disorders and (3) functional rehabilitation and occupational therapy. A descriptive analysis of the results obtained from the System Usability Scale test of the application and a collection of qualitative assessments of each game have been carried out. The application obtained a mean score of 86.25 (±8.96 SD) in the System Usability Scale, and the experts concluded that it accurately reproduces activities of daily living movements except for wrist and finger movements. They also offer a set of design guidelines.The study was funded by the Spanish Ministry of Science, Innovation and University, under the grant European Regional Development Fund (ERDF) with project reference IDI-20191120, and also by the SPANISH GOVERNMENT (FEDER/Ministry of Science and Innovation/AEI), grant number RTI2018-097122-A-I00

Los primeros robots manipuladores aéreos

[Resumen] En este artículo se describen los primeros robots manipuladores aéreos dotados con brazos robóticos de seis grados de libertad y capacidades de percepción y planificación que se han desarrollado en el mundo. Estos robots son el resultado del proyecto ARCAS (Aerial Robotics Cooperative Assembly System) coordinado por el primero de los autores y financiado por el Séptimo Programa Marco de la Unión Europea. Los resultados de este proyecto han comenzado a emplearse, entre otros, en el proyecto H2020 AEROARMS y en el español AEROMAIN. El artículo introduce las diferentes plataformas utilizadas en el proyecto y a continuación resume las principales características de los sistemas de control, percepción y planificación desarrollados. Se resume también la integración y validación del sistema.Este trabajo ha sido financiado parcialmente por el Proyecto Europeo del FP7 ARCAS (ICT-2011-287617) y por el H2020 AEROARMS (ICT-23-2014-644271), así como por el proyecto nacional AEROMAIN DPI2014-59383-C2-1-R del Ministerio de Economía y Competitividadhttps://doi.org/10.17979/spudc.978849749808

COVID-19 severity and mortality in patients with chronic lymphocytic leukemia: a joint study by ERIC, the European Research Initiative on CLL, and CLL Campus

Chronic lymphocytic leukemia (CLL) is a disease of the elderly, characterized by immunodeficiency. Hence, patients with CLL might be considered more susceptible to severe complications from COVID-19. We undertook this retrospective international multicenter study to characterize the course of COVID-19 in patients with CLL and identify potential predictors of outcome. Of 190 patients with CLL and confirmed COVID-19 diagnosed between 28/03/2020 and 22/05/2020, 151 (79%) presented with severe COVID-19 (need of oxygen and/or intensive care admission). Severe COVID-19 was associated with more advanced age (≥65 years) (odds ratio 3.72 [95% CI 1.79–7.71]). Only 60 patients (39.7%) with severe COVID-19 were receiving or had recent (≤12 months) treatment for CLL at the time of COVID-19 versus 30/39 (76.9%) patients with mild disease. Hospitalization rate for severe COVID-19 was lower (p &lt; 0.05) for patients on ibrutinib versus those on other regimens or off treatment. Of 151 patients with severe disease, 55 (36.4%) succumbed versus only 1/38 (2.6%) with mild disease; age and comorbidities did not impact on mortality. In CLL, (1) COVID-19 severity increases with age; (2) antileukemic treatment (particularly BTK inhibitors) appears to exert a protective effect; (3) age and comorbidities did not impact on mortality, alluding to a relevant role of CLL and immunodeficiency. © 2020, The Author(s), under exclusive licence to Springer Nature Limited