Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome : a consensus statement from the FD/MAS international consortium

Fibrous Dysplasia / McCune Albright syndrome (FD/MAS) represents a wide spectrum of diseases due to somatic gain-of-function mutations of the GNAS gene. The mutation leads to overactivity in the target tissues and to a wide phenotype of clinical features that vary in severity and age of onset. The rarity of the disease and its variable presentation to multiple specialities often leads to misdiagnosis and inappropriate variability in investigations and treatments. To address this, our international consortium of clinicians, researchers, and patients’ advocates has developed pragmatic clinical guidelines for best clinical practice for the definition, diagnosis, staging, treatment and monitoring for FD/MAS to empower patients and support clinical teams in both general and specialised healthcare settings. With the lack of strong evidence to inform care, the guidelines were developed based on review of published literature, long-standing extensive experience of authors, input from other healthcare professionals involved in the care of FD/MAS patients and feedback from patients and patient groups across the globe. This has led to the formulation of a set of statements to inform healthcare professionals, patients, their families, carers and patient groups of the best practice of care. It is anticipated the implementation of these recommendations will lead to improvement in the care of patients with FD/MAS internationally

Novel observations underlying the fast growth of suspension-feeding shellfish in turbid environments: Mytilus edulis

For the tirst time for any benthic filter-feeder, this study documents each component process of nutrient acquisition through natural tidal variations of food availabillty. The organic content of seston available during both neap and spring tides in the bay of Marennes-Oléron. France, decreased from 28 to 8 % with mcreasing seston concentration from 10 to about 90 mg total particulate mass 1(1). Throughout this tidal variation, the blue mussel Mylilus edulis L. cleared more water of particles as seston availability increased. Rejection of fillered material as pseudofaeces prior to ingestion remained a constant fraction of about 0 .93 x the mass of filtered material, so that ingestion rate showed no signs of stabilising at even the highest food availabillties. We confirm that M. edulis may preferentially reject inorganic matter with pseudofaeces. More significant was the novel observation that the net selection efficiency with which filtered organics were selectively retained for ingestion increased rapidly with the rate at which seston was filtered, this increase being faster for seston of higher organic content. The result was that the organic content of ingested matter was enriched by up to 5 times the organic content of filtered particles. Further, net absorption efficiency for ingested organics varied in strong positive relation with the organic content of ingested material. Therefore, rates of organic absorption increased with seston filtration rate, and net energy balance increased despite the decreasing organic content of particles available at higher concentrations. These collective find ings demonstrate continuous interrelated changes in feeding physiology that help to maintain rates of nutrient acquisition independent of short-term fluctuations in seston composition

Du bassin versant au territoire maritime : la ressource hydrique objet d'une recherche intégrée et finalisée. L'exemple du bassin versant de la Charente et de la Mer des Pertuis

This document first describes the content and results of the Charente-Marennes-Oleron scientific program, and of the associated work. Then the goals, content and first products of the Interface transfer project are presented. / Ce document décrit dans une première partie le contenu et les résultats du programme scientifique Charente-Marennes-Oléron et des travaux associés, pour se poursuivre par une présentation des objectifs, du contenu et des premiers produits du projet de transfert Interface

Manipulation of dietary conditions for maximal growth in mussels Mytilus edulis from the Marennes-Oleron Bay France

Results are presented from laboratory-based experiments in which we investigated short-term responses of Mytilus edulis L. to experimental changes in the amount and composition of suspended seston. Working with large quantities of cultured algae, we have studied feeding behaviour over ranges of food availability and quality that ex tend weil beyond earlier limits. Findings confirm the ability of mussels to selectively reject inorganic particles as pseudofaeces prior to ingestion, thereby enriching the organic content of ingested matter by 30% more than the organic content of natural filtered seston. Our findings also establish acclimation both of selective and absorptive processes, indicating that the extent to which growth of M. edulis can be stimulated in the short-term will depend upon prior nutritional history. Present maximal growth of 14.8% dry soft tissue d-I in a standard M. edulis of 1 g dry soft tissue was much higher than has previously been documented for any adult musse!. Maximal growth was achieved when natural ses ton that had been enriched to more than about 60% organic content with a mixture of algal monocultures was available al concentrations above about 11.5 mg total particulates 1-1 • Neither ingestion rate nor net energy balance were improved with further increases in food availability, associated with regulatory reductions in the rate of water filtration (clearance rate) that maintained organic ingestion rate independent of increases in bath the amount and organic content of available seston. These findings suggest that digestive processes had become saturated when organic ingestion reached about 6.5 mg organics g-I dry soft tissue h-I in a standard M. edulis of 1 g dry soft tissue, representing as much as 20.6% of all soft tissue organic mass mussel-1 d-1. Such saturation of organic ingestion is consistent with previous conclusions based on comparative allometries showing that limitations to growth in M. edulis and other bivalves are associated with the rate of food processing.Des expérimentations ont été réalisées en laboratoire pour déterminer les réponses à court terme de la moule Mytilus edulis soumise à des changements dans la quantité et la qualité de la nourriture. L'apport de fortes quantités d'algues phytoplanctoniques a permis d'étudier le comportement de la moule pour des conditions supérieures à celles décrites antérieurement. Les résultats confirment que la moule rejette préférentiellement les particules minérales dans les pseudofèces, induisant un enrichissement de 30 % de la matière organique ingérée par rapport à la matière organique filtrée. Ainsi, l'acclimatation aux conditions nutritionnelles agit sur les phénomènes de sélection et d'absorption. La croissance de Mytilus edulis peut être stimulée à court terme en fonction de son passé nutritionnel. La croissance maximale obtenue, pour une charge sestonique de 11,5 mg.l- 1 composée de 60 % de matière organique, est de 14,8 % de chair sèche; calculée pour un animal standard de 1 g de chair sèche et par jour, cette croissance est nettement supérieure à celle décrite précédemment pour des moules adultes. Une augmentation de la concentration de la nourriture disponible n'engendre pas d'amélioration, ni de la quantité de matière organique ingérée, ni du bilan énergétique. En effet, ces fonctions physiologiques sont associées à des réductions compensatoires de la filtration de l'animal qui maintiennent l'ingestion de la matière organique indépendante d'une part, de la charge et d'autre part, du taux de matière organique du seston disponible. En conséquence, les processus digestifs atteindraient un seuil de saturation lorsque l'ingestion organique correspondrait à 6,5 mg de matière organique par heure, pour une moule standard de 1 g de chair sèche; ce qui représente près de 20,6 % du poids de matière organique de la chair, par moule et par jour. Une telle saturation de l'ingestion organique est cohérente avec les conclusions antérieures basées sur des aIlométries comparatives, qui montrent que les limites de croissance chez M. edulis et d'autres bivalves sont associées aux taux d'utilisation de la nourriture

Feeding-Behavior Of The Mussel Mytilus-Edulis - Responses To Variations In Quantity And Organic Content Of The Seston

Mussels were fed four concentrations of seston (between 0•99 and 10•3mgtotalseston P), comprising three levels of organic content (71•9, 63•6 and 40•8%), made up from natural silt and two species of cultured phytoplankton. Two of the seston concentrations were below, and two above, the threshold at which pseudofaeces were produced. Measurements of physiological traits (filtration rates, pseudofaeces production, selection efficiency, absorption efficiency, absorption rates and rates of oxygen consumption) were made after 2 days and, for two of the seston concentrations, also after 12 days. When fed at a high concentration of seston of low organic content, the mussels increased their filtration rate, rejected a higher proportion of filtered material as pseudofaeces, and increased the efficiency with which filtered matter of higher organic content was selected for ingestion; this resulted in a constancy of the relationship between ingestion rate and the concentration of particulate organic matter, regardless of differences in seston organic content. Between 2 and 12 d, the mussels increased absorption rates for organics, primarily by increasing absorption efficiency, both for total organics and for the carbohydrate component of the diet. We suggest that these responses to changes in the food environment comprise physiological adjustments which result in higher net rates of absorption than would be predicted from considerations only of the organic/inorganic ratio of the suspended particles and assumptions of a non-compensating feeding behaviour