A qualitative study to elicit user requirements for lower limb wearable exoskeletons for gait rehabilitation in spinal cord injury.

OBJECTIVE: We aim to determine a comprehensive set of requirements, perceptions, and expectations that people with spinal cord injury (SCI) and the clinicians in charge of their rehabilitation have regarding the use of wearable robots (WR) for gait rehabilitation. BACKGROUND: There are concerns due to the limited user acceptance of WR for gait rehabilitation. Developers need to emphasize understanding the needs and constraints of all stakeholders involved, including the real-life dynamics of rehabilitation centers. METHODS: 15 people with SCI, 9 without experience with WR and 6 with experience with these technologies, and 10 clinicians from 3 rehabilitation centers in Spain were interviewed. A directed content analysis approach was used. RESULTS: 78 codes grouped into 9 categories (physical results, usability, psychology-related codes, technical characteristics, activities, acquisition issues, context of use, development of the technologies and clinical rehabilitation context) were expressed by at least 20% of the users interviewed, of whom 16 were not found in the literature. The agreement percentage between each group and subgroup included in the study, calculated as the number of codes that more than 20% of both groups expressed, divided over the total amount of codes any of those two groups agreed on (≥ 20%), showed limited agreement between patients and clinicians (50.00%) and between both types of patients (55.77%). The limited accessibility and availability of lower limb exoskeletons for gait rehabilitation arose in most of the interviews. CONCLUSIONS: The limited agreement percentage between patients and clinicians indicates that including both types of users in the design process of these technologies is important, given that their requirements are complementary. Engaging users with prior technology experience is recommended, as they often exhibit strong internal consensus and articulate well-defined requirements. This study adds up the knowledge available in the literature and the new codes found in our data, which enlighten important aspects that ought to be addressed in the field to develop technologies that respond to users' needs, are usable and feasible to implement in their intended contexts. APPLICATION: The set of criteria summarized in our study will be useful to guide the design, development, and evaluation of WR for gait rehabilitation to meet user's needs and allow them to be implemented in their intended context of use.

Pediatric Acute Lung Injury Epidemiology and Natural History study: Incidence and outcome of the acute respiratory distress syndrome in children.

OBJECTIVES: The incidence and outcome of the acute respiratory distress syndrome in children are not well-known, especially under current ventilatory practices. The goal of this study was to determine the incidence, etiology, and outcome of acute respiratory distress syndrome in the pediatric population in the setting of lung protective ventilation. DESIGN: A 1-yr, prospective, multicenter, observational study in 12 geographical areas of Spain (serving a population of 3.77 million ≤ 15 yrs of age) covered by 21 pediatric intensive care units. SUBJECTS: All consecutive pediatric patients receiving invasive mechanical ventilation and meeting American-European Consensus Criteria for acute respiratory distress syndrome. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Data on ventilatory management, gas exchange, hemodynamics, and organ dysfunction were collected. A total of 146 mechanically ventilated patients fulfilled the acute respiratory distress syndrome definition, representing a incidence of 3.9/100,000 population ≤ 15 yrs of age/yr. Pneumonia and sepsis were the most common causes of acute respiratory distress syndrome. At the time of meeting acute respiratory distress syndrome criteria, mean PaO2/FIO2 was 99 mm Hg ± 41 mm Hg, mean tidal volume was 7.6 mL/kg ± 1.8 mL/kg predicted body weight, mean plateau pressure was 27 cm H2O ± 6 cm H2O, and mean positive end-expiratory pressure was 8.9 cm ± 2.9 cm H2O. Overall pediatric intensive care unit and hospital mortality were 26% (95% confidence interval 19.6-33.7) and 27.4% (95% confidence interval 20.8-35.1), respectively. At 24 hrs, after the assessment of oxygenation under standard ventilatory settings, 118 (80.8%) patients continued to meet acute respiratory distress syndrome criteria (PaO2/FIO2 104 mm Hg ± 36 mm Hg; pediatric intensive care units mortality 30.5%), whereas 28 patients (19.2%) had a PaO2/FIO2 >200 mm Hg (pediatric intensive care units mortality 7.1%) (p = .014). CONCLUSIONS: This is the largest study to estimate prospectively the pediatric population-based acute respiratory distress syndrome incidence and the first incidence study performed during the routine application of lung protective ventilation in children. Our findings support a lower acute respiratory distress syndrome incidence and mortality than those reported for adults. PaO2/FIO2 ratios at acute respiratory distress syndrome onset and at 24 hrs after onset were helpful in defining groups at greater risk of dying (clinical trials registered with http://www.clinicaltrials.gov; NCT 01142544).

Ventricular assist device as a bridge to transplant, and extracorporeal membrane oxygenation for primary graft failure in a child with hemophilia A.

OBJECTIVE: To report the management of hemophilia in a patient with dilated cardiomyopathy during application of the Berlin-Heart biventricular assist. DESIGN: Case report. INTERVENTIONS AND RESULTS: The Berlin-Heart biventricular assist was successfully used as a bridge to heart transplantation (178 days) in a child with hemophilia A; post-transplant extracorporeal membrane oxygenation was implanted until the patient recovered from primary graft failure. Anticoagulant therapy varied as a function of patient status: 1) postoperative bleeding was treated by partial replacement of deficient factors (VII and VIII) and hemoderivatives in order to maintain factor VIII at 50% of normal levels; 2) once the bleeding had stopped, the effect of the hemophilia itself maintained the degree of anticoagulation required by the ventricular assist device; and 3) transplant surgery was followed by complete replacement of factor VIII and intravenous heparinization (a simple way of preventing clot formation in the device and to ensure proper scarring of surgical wounds). CONCLUSIONS: Selection of anticoagulant therapy as a function of patient status in terms of bleeding and surgical-wound scarring progress is vital for the proper functioning of support techniques (Berlin-Heart biventricular assist and extracorporeal membrane oxygenation) in hemophiliac patients. Collagen dressings placed on surgical wounds achieved good functional and aesthetic results, as well as mechanically isolating the scars from the exterior.

Estimating the burden of disease for autism spectrum disorders in Spain in 2003.

Autism Spectrum Disorders (ASD) are lifelong neurodevelopmental disabilities. Burden of Disease is an indicator that provides important information on health status and outcomes such as premature mortality and disability. In order to estimate the burden of disease of ASD in the Spanish population during 2003, we followed the procedures used in the WHO Global Burden of Disease Study. ASD generated 43,928 Disability Adjusted Life Years (DALY) in Spain in 2003, from which 33,797 were attributable to Autistic Disorder and 10,131 were caused by Asperger's Disorder and Pervasive Developmental Disorder-Not Otherwise Specified. DALY could be a useful tool for health policy makers for setting health service priorities, allocating available resources effectively and providing a comparable measure of output for early intervention.

[Disease registries in the epidemiological researching of rare diseases in Spain]. / Los registros de enfermedades en la investigacion epidemiológica de las enfermedades raras en España.

BACKGROUND: The use of tools such as disease registries poses a problem in the case of rare diseases. This study is aimed at describing the current situation concerning rare disease registries in Spain. METHODS: The information provided by two Spanish health registries directories prepared by the Spanish Health Assessment Technologies Agencies are employed, a descriptive cross-sectional study being conducted. The registries identified in these directories has been classified as: (1) "Specific rare diseases" (2) "Unspecific but with information on rare disease and (3) "Non-informative regarding rare diseases". RESULTS: The 2000 directory listed 82 registries, 15.8% of which were classified under Group 1, whilst a total of 107 registries, 16.8% in Group 1, were identified in the 2005 review. The main health registries in Group 2, by topic, were: cancer, mortality, psychiatry and nephrology. No general rare disease registries were found in the directories. CONCLUSIONS: Although few in number, health registries do exist in Spain including information on rare diseases. Areas have been identified by topic lacking registries and also information systems or registries unidentified in the sources used. Continuing efforts must be made to improve the information available on rare diseases.

Los registros de enfermedades en la investigación epidemiológica de las enfermedades raras en España / Disease registries in the epidemiological researching of rare diseases in Spain

Fundamento: En el caso de las enfermedades raras existen dificultadespara utilizar herramientas como los registros de enfermedades.El objetivo de este trabajo es describir la situación de los registrossobre enfermedades raras en España.Métodos: Se utiliza la información proporcionada por dos directoriosde registros sanitarios españoles elaborados por la AgenciaEspañola de Evaluación de Tecnologías Sanitarias, realizando unestudio descriptivo transversal. Los registros identificados en estosdirectorios se han clasificado como: 1) «Específicos de enfermedadesraras», 2) «No específicos pero con información de enfermedadesraras», y 3) «No informativos sobre enfermedades raras».Resultados: En el directorio del año 2000 aparecían 82 registros,de los que el 15,8% se clasificaron en el grupo 1, mientras que en larevisión de 2005 se identificaron 107 registros, 16,8% en el grupo 1.Las principales áreas temáticas de los registros sanitarios del grupo 2fueron el cáncer, la mortalidad, la psiquiatría y la nefrología. En losdirectorios no se ha hallado ningún registro genérico de enfermedadesraras. Conclusiones: Aunque escasos, existen registros sanitarios enEspaña con información sobre enfermedades raras. Se han identificadoáreas temáticas con carencias de registros y también registros osistemas de información no identificados en las fuentes utilizadas. Esnecesario continuar los esfuerzos por mejorar la información disponiblesobre las enfermedades raras

Background: The use of tools such as disease registries poses aproblem in the case of rare diseases. This study is aimed at describingthe current situation concerning rare disease registries inSpain.Methods: The information provided by two Spanish healthregistries directories prepared by the Spanish Health AssessmentTechnologies Agencies are employed, a descriptive cross-sectionalstudy being conducted. The registries identified in these directorieshas been classified as: 1) «Specific rare diseases» 2) «Unspecific butwith information on rare disease» and 3) «Non-informative regardingrare diseases».Results: The 2000 directory listed 82 registries, 15.8% of whichwere classified under Group 1, whilst a total of 107 registries, 16.8%in Group 1, were identified in the 2005 review. The main healthregistries in Group 2, by topic, were: cancer, mortality, psychiatryand nephrology. No general rare disease registries were found in thedirectories. Conclusions: Although few in number, health registries do existin Spain including information on rare diseases. Areas have beenidentified by topic lacking registries and also information systems orregistries unidentified in the sources used. Continuing efforts mustbe made to improve the information available on rare diseases

Characteristics of disability and handicap among Toxic Oil Syndrome (TOS) cohort patients: a cross-sectional study, 17 years after the original food intoxication.

PURPOSE: In 1981, a progressive multi-systemic disease called Toxic Oil Syndrome (TOS) appeared in Spain as an epidemic that affected 20,000 people. The International Classification of Impairments, Disabilities and Handicaps (ICIDH) was chosen to characterize the health status of patients more severely affected by TOS. METHODS: A random sample of 292 with permanent disability was selected. Disability was assessed with a questionnaire based on ICIDH and the Stanford Health Assessment Questionnaire. Handicap was measured using London Handicap Scale. Distributions of the proportions and 95% confidence intervals for disabilities, handicaps were calculated and stratified by dimensions, age and sex. The chi2 test was used for inter-group comparisons. RESULTS: Two hundred and fourteen patients were interviewed. Mobility-related and behaviour disabilities were most prevalent. Disability rose with age and was higher among women, except for behaviour disabilities which were more frequent in young men. Mean handicap score was 78.0 +/- 12.7. Handicap dimensions most affected were physical independence and economic self-sufficiency. CONCLUSIONS: The health profile of the population hardest hit by TOS is characterized by the presence of important functional and psychosocial disabilities that limit performance of daily living activities and social role, and are in accord with the handicap that such persons suffer.

Neurologic outcomes of toxic oil syndrome patients 18 years after the epidemic.

Toxic oil syndrome (TOS) resulted from consumption of rapeseed oil denatured with 2% aniline and affected more than 20,000 persons. Eighteen years after the epidemic, many patients continue to report neurologic symptoms that are difficult to evaluate using conventional techniques. We conducted an epidemiologic study to determine whether an exposure to toxic oil 18 years ago was associated with current adverse neurobehavioral effects. We studied a case group of 80 adults exposed to toxic oil 18 years ago and a referent group of 79 adult age- and sex-frequency-matched unexposed subjects. We interviewed subjects for demographics, health status, exposures to neurotoxicants, and responses to the Kaufman Brief Intelligence Test (K-BIT), Programa Integrado de Exploracion Neuropsicologica (PIEN), and Goldberg depression questionnaires and administered quantitative neurobehavioral and neurophysiologic tests by computer or trained nurses. The groups did not differ with respect to educational background or other critical variables. We examined associations between case and referent groups and the neurobehavioral and neurophysiologic outcomes of interest. Decreased distal strength of the dominant and nondominant hands and increased vibrotactile thresholds of the fingers and toes were significantly associated with exposure to toxic oil. Finger tapping, simple reaction time latency, sequence B latency, symbol digit latency, and auditory digit span were also significantly associated with exposure. Case subjects also had statistically significantly more neuropsychologic symptoms compared with referents. Using quantitative neurologic tests, we found significant adverse central and peripheral neurologic effects in a group of TOS patients 18 years after exposure to toxic oil when compared with a nonexposed referent group. These effects were not documented by standard clinical examination and were found more frequently in women.