Antagonisme et solidarité: les relations entre Peuls et Dogons du Mali central

ASC – Publicaties niet-programma gebonde

De betrekkingen tussen Peul en Dogon in centraal-Mali

De Peul en de Dogon leven, ruim voor het tijdperk van grote Peulstaten als het Macinarijk, reeds lang naast elkaar in centraal Mali. De Peul weidden er hun kudden, pleegden er overvallen en gebruikten het gebied als slavenreservoir. Ook de Dogon organiseerden van tijd tot tijd overvallen. Door de geschiedenis heen hebben de betrekkingen tussen de Dogon en de Peul afwisselende uitingsvormen gekend afhankelijk van de diverse woongebieden in de Seno-Gondo- en Seno-Mangovlakten. Rond de Falaise van Bandiagara blijkt uit diverse rituelen van de Dogon een diepe rancune jegens de Peul. In het spraakgebruik van de Dogon staat de Peul symbool voor het beeld van 'de ander', de bewoner van de wildernis. Maar als mens van de wildernis vertegenwoordigt de Peul ook andere waarden: omdat de wildernis wijs en sterk is, maar ook gevaarlijk en grillig, is de Peul dat ook. De betrekkingen tussen de Houmbebe, een Dogon subgroep van landbouwers in de Hayre, en de Peul, veehouders en halfnomaden, hebben een werkbare vorm gekregen door de opkomst van de 'njaatigi' (gastheer). In elk dorp heeft de Peul een 'njaatigi' op wie hij een beroep kan doen. Door de aanhoudende droogte in het gebied verandert de 'njaatigi'-verhouding echter steeds meer in een afhankelijkheidsrelatie. Noten. [Samenvatting ASC Leiden]ASC – Publicaties niet-programma gebonde

Long-term follow-up of 17 patients with childhood Pompe disease treated with enzyme replacement therapy

Objectives: Pompe disease is a progressive metabolic myopathy for which enzyme replacement therapy (ERT) was approved in 2006. While various publications have examined the effects of ERT in classic-infantile patients and in adults, little has been published on ERT in children with non-classic presentations. Study design: This prospective study was conducted from June 1999 to May 2015. Seventeen patients from various countries participated. Outcome measures comprised muscle function (6-minute walk test, quick motor-function test (QMFT)), muscle strength (hand-held dynamometry; manual muscle testing), and lung function (FVC sitting and supine). For each outcome measure, we used linear mixed-effects models to calculate the difference at group level between the start of therapy and 7 years of ERT. Patients’ individual responses over time were also evaluated. Results: Eleven males and six females started ERT at ages between 1.1 and 16.4 years (median 11.9 years); 82% of them carried the common c.-32-13T > G GAA gene variant on one allele. At group level, distance walked increased by 7.4 percentage points (p < 0.001) and QMFT scores increased by 9.2 percentage points (p = 0.006). Muscle strength scores seemed to remain stable. Results on lung function were more variable. Patients’ individual data show that the proportion of patients who stabilized or improved during treatment ranged between 56 and 69% for lung function outcomes and between 71 and 93% for muscle strength and muscle function outcomes. Conclusions: We report a positive effect of ERT in patients with childhood Pompe disease at group level. For some patients

Learning from failure

We study decentralized learning in organizations. Decentralization is captured through a symmetry constraint on agents’ strategies. Among such attainable strategies, we solve for optimal and equilibrium strategies. We model the organization as a repeated game with imperfectly observable actions. A fixed but unknown subset of action profiles are successes and all other action profiles are failures. The game is played until either there is a success or the time horizon is reached. For any time horizon, including infinity, we demonstrate existence of optimal attainable strategies and show that they are Nash equilibria. For some time horizons, we can solve explicitly for the optimal attainable strategies and show uniqueness. The solution connects the learning behavior of agents to the fundamentals that characterize the organization: Agents in the organization respond more slowly to failure as the future becomes more important, the size of the organization increases and the probability of success decreases.Game theory

Positive association between physical outcomes and patient-reported outcomes in late-onset Pompe disease: a cross sectional study

BACKGROUND: Pompe disease is a rare, progressive metabolic myopathy. The aim of this study is to investigate the associations of physical outcomes with patient-reported outcome measures (PROMs) in late-onset Pompe disease. METHODS: We included 121 Dutch adult patients with Pompe disease. Physical outcomes comprised muscle strength (manual muscle testing using Medical Research Council [MRC] grading, hand-held dynamometry [HHD]), walking ability (6-min walk test [6MWT]), and pulmonary function (forced vital capacity [FVC] in upright and supine positions). PROMs comprised quality of life (Short Form 36 health survey [SF-36]), participation (Rotterdam Handicap Scale [RHS]) and daily-life activities (Rasch-Built Pompe-Specific Activity [R-PAct] Scale). Analyses were cross-sectional: the time-point before, and closest to, start of Enzyme Replacement Therapy was chosen. Associations between PROMs and physical outcomes were investigated using linear regression models. RESULTS: RHS and R-PAct scores were better in patients with higher FVC supine and upright, HHD, MRC and 6MWT scores, accounting for the effect of sex, disease duration, use of wheelchair and ventilator support. While the SF-36 Physical Component Summary (PCS) was correlated positively with FVC upright, HHD, MRC and 6MWT scores, there was no significant relationship between the SF-36 Mental Component Summary (MCS) and any of the physical outcomes. CONCLUSIONS: Participation, daily-life activities, and the physical component of quality of life of adult Pompe patients are positively correlated to physical outcomes. This work serves as a first step towards assessing how changes over time in physical outcomes are related to changes in PROMs, and to define the minimal change in physical outcomes required to make an important difference for the patient