Experiences Among Patients With Cystic Fibrosis in the MucoExocet Study of Using Connected Devices for the Management of Pulmonary Exacerbations: Grounded Theory Qualitative Research.

BACKGROUND: Early detection of pulmonary exacerbations (PEx) in patients with cystic fibrosis is important to quickly trigger treatment and reduce respiratory damage. An intervention was designed in the frame of the MucoExocet research study providing patients with cystic fibrosis with connected devices and educating them to detect and react to their early signs of PEx. OBJECTIVE: This study aims to identify the contributions and conditions of home monitoring in relation to their care teams from the users' point of view to detect PEx early and treat it. This study focused on the patients' experiences as the first and main users of home monitoring. METHODS: A qualitative study was conducted to explore patients' and professionals' experiences with the intervention. We interviewed patients who completed the 2-year study using semistructured guides and conducted focus groups with the care teams. All the interviews were recorded and transcribed verbatim. Their educational material was collected. A grounded analysis was conducted by 2 researchers. RESULTS: A total of 20 patients completed the study. Three main categories emerged from the patients' verbatim transcripts and were also found in those of the professionals: (1) task technology fit, reflecting reliability, ease of use, accuracy of data, and support of the technology; (2) patient empowerment through technology, grouping patients' learnings, validation of their perception of exacerbation, assessment of treatment efficacy, awareness of healthy behaviors, and ability to react to PEx signs in relation to their care team; (3) use, reflecting a continuous or intermittent use, the perceived usefulness balanced with cumbersome measurements, routinization and personalization of the measurement process, and the way data are shared with the care team. Furthermore, 3 relationships were highlighted between the categories that reflect the necessary conditions for patient empowerment through the use of technology. CONCLUSIONS: We discuss a theorization of the process of patient empowerment through the use of connected devices and call for further research to verify or amend it in the context of other technologies, illnesses, and care organizations. TRIAL REGISTRATION: ClinicalTrials.gov NCT03304028; https://clinicaltrials.gov/ct2/show/results/NCT03304028.

Neutrophils expressing major histocompatibility complex class II molecules circulate in blood and milk during mastitis and show high microbicidal activity.

Bovine mastitis is mainly caused by bacterial infection and is responsible for important economic losses as well as alterations of the health and welfare of animals. The increase in somatic cell count (SCC) in milk during mastitis is mainly due to the influx of neutrophils, which have a crucial role in the elimination of pathogens. For a long time, these first-line defenders have been viewed as microbe killers, with a limited role in the orchestration of the immune response. However, their role is more complex: we recently characterized a bovine neutrophil subset expressing major histocompatibility complex class II (MHC-II) molecules (MHC-IIpos), usually distributed on antigen-presenting cells, as having regulatory capacities in cattle. In this study, our objective was to evaluate the implication of different neutrophils subsets in the mammary gland immunity during clinical and subclinical mastitis. Using flow cytometry, we analyzed the presence of MHC-IIpos neutrophils in blood and in milk during clinical mastitis at different time points of inflammation (n = 10 infected quarters) and during subclinical mastitis, defined as the presence of bacteria and an SCC >150,000 cells/mL (n = 27 infected quarters). Our results show, for the first time, that in blood and milk, neutrophils are a heterogeneous population and encompass at least 2 subsets distinguishable by their expression of MHC-II. In milk without mastitis, we observed higher production of reactive oxygen species and higher phagocytosis capacity of MHC-IIpos neutrophils compared with their MHC-IIneg counterparts, indicating the high bactericidal capacities of MHC-IIpos neutrophils. MHC-IIpos neutrophils are enriched in milk compared with blood during subclinical mastitis but not during clinical mastitis. Moreover, we observed a positive and highly significant correlation between MHC-IIpos neutrophils and T lymphocytes present in milk during subclinical mastitis. Our experiments involved a total of 47 cows (40 Holstein and 7 Normande cows). To conclude, our study opens the way to the discovery of new biomarkers of mastitis inflammation.

Trajectories of nutrients concentrations and ratios in the French coastal ecosystems: 20 years of changes in relation with large-scale and local drivers.

Along with their important diversity, coastal ecosystems receive various amounts of nutrients, principally arising from the continent and from the related human activities (mainly industrial and agricultural activities). During the 20th century, nutrients loads have increased following the increase of both the global population and need of services. Alongside, climate change including temperature increase or atmospheric circulation change has occurred. These processes, Ecosystem state changes are hard to monitor and predict. To study the long-term changes of nutrients concentrations in coastal ecosystems, eleven French coastal ecosystems were studied over 20 years as they encompass large climatic and land pressures, representative of temperate ecosystems, over a rather small geographical area. Both univariate (time series decomposition) and multivariate (relationships between ecosystems and drivers) statistical analyses were used to determine ecosystem trajectories as well as typologies of ecosystem trajectories. It appeared that most of the French coastal ecosystems exhibited trajectories towards a decrease in nutrients concentrations. Differences in trajectories mainly depended on continental and human influences, as well as on climatic regimes. One single ecosystem exhibited very different trajectories, the Arcachon Bay with an increase in nutrients concentrations. Ecosystem trajectories based on ordination techniques were proven to be useful tools to monitor ecosystem changes. This study highlighted the importance of local environments and the need to couple uni- and multi-ecosystem studies. Although the studied ecosystems were influenced by both local and large-scale climate, by anthropogenic activities loads, and that their trajectories were mostly similar based on their continental influence, non-negligible variations resulted from their internal functioning.

Use of Home-Based Connected Devices in Patients With Cystic Fibrosis for the Early Detection and Treatment of Pulmonary Exacerbations: Protocol for a Qualitative Study.

BACKGROUND: Early detection of pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF) is important to quickly trigger treatment and reduce respiratory damage. We hypothesized that using home-based and wearable connected devices (CDs) and educating patients to react in case of abnormal variations in a set of parameters would allow patients to detect and manage their PEx early with their care team. OBJECTIVE: This qualitative study aimed to assess the feasibility and appropriate conditions of a new PEx management process from the users' point of view by analyzing the experience of patients and of CF center teams regarding the education program, the use of CDs, and the relationship between the patient and the care team during PEx management. METHODS: We have been conducting a multicenter pilot study involving 36 patients with CF aged ≥12 years. The intervention was divided into 3 phases. In phase 1 (3 months), patients were equipped with CDs, and their parameters were collected on 3 nonconsecutive days each week. Phase 2 involved the development of a "React to PEx" educational program aimed at providing patients with a personalized action plan. A training session to the educational program was organized for the physicians. Physicians then determined the patients' personalized alert thresholds by reviewing the data collected during phase 1 and their patients' clinical history. In phase 3 (12 months), patients were educated by the physician during a clinic visit, and their action plan for reacting in timely fashion to their PEx signs was defined. Education and action plans were revised during clinic visits. At the end of the project, the patients' experience was collected during semistructured interviews with a researcher as part of the qualitative study. The experience of CF teams was collected during focus groups using a semistructured guide once all their patients had finished the study. The interviews and focus groups were recorded and transcribed verbatim to be analyzed. Data from educational sessions were collected throughout the educational program to be put into perspective with the learnings reported by patients. Analyses are being led by 2 researchers using NVivo (QSR International). RESULTS: The study received the favorable reception of the Committee for the Protection of Persons (CPP NORTH WEST III) on June 10, 2017 (#2017-A00723-50). Out of the 36 patients included in phase 1, 27 were educated and entered phase 3. We completed collection of all data from the patients and care providers. Qualitative analysis will provide a better understanding of users' experience on the conditions of data collection, how useful CDs are for detecting PEx, how useful the PEx action plan is for reacting quickly, what patients learned about PEx management, and the conditions for this PEx management to be sustainable in routine care. CONCLUSIONS: This study will open new perspectives for further research into the implementation of an optimal PEx care process in the organization of care teams in order to support patient self-management. TRIAL REGISTRATION: ClinicalTrials.gov NCT03304028; https://clinicaltrials.gov/ct2/show/results/NCT03304028. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/14552.

Consensus Recommendations for the Use of Simulation in Therapeutic Patient Education.

INTRODUCTION: Simulation is rarely used to help individuals with chronic diseases develop skills. The aim of the study was to provide recommendations for the use of simulation in therapeutic patient education (S-TPE). METHODS: Expert consensus was achieved with the participation of the following 3 groups of experts: (a) expert patients and caregivers; (b) health professionals specialized in therapeutic patient education (TPE); and (c) simulation experts. Each expert received a list of questions by e-mail in 3 iterations. The synthesis of the 2 first questionnaires resulted in 34 first recommendations voted during the consensus conference meeting. Each recommendation was subject to an extensive literature review. The quality of the evidence and the strength of the recommendations were assessed through the evaluation, development, and evaluation criteria categories (GRADE criteria). The third questionnaire selected and illustrated recommendations more specific to the use of S-TPE. RESULTS: At the end of the process, the experts identified 26 recommendations specific to the use of S-TPE. They proposed examples of skills in different diseases and stressed the importance of adapting the conditions of use (location, equipment, time of the care) to the circumstances of the patient learner and skills to be developed. Experts should exercise great caution as this technique presents ethical considerations related to patient care. CONCLUSIONS: These recommendations underline the fact that simulation could bring added value to TPE. They provide a framework and examples for the experimental use of simulation in TPE. Research into feasibility and acceptability is needed.

Ecological succession and resilience of plankton recovering from an acute disturbance in freshwater marshes.

The increase in extreme events such as storms is one of the major threats that coastal ecosystems will have to face in the near future. In such a context, both maturation and ecological successions processes remain at the core of ecology to better anticipate the changes to ecosystem biodiversity and functions facing environmental stressors. However, these concepts are mainly approached through closed experimental studies that oversimplify the mechanisms. A survey was carried out on a 'natural' and open ecosystem subjected to an acute disturbance, i.e. a marine submersion of freshwater drained marshes, occurring after a storm. Plankton biomass, production and taxonomic/functional phytoplankton diversity were followed weekly at four stations over 2 months. Most of the stations were disrupted by this acute disturbance and displayed gradual growth and development, as described in the classical maturation process. The main differences between stations were attributed to the heterogeneity of the communities before the storm, the intensity of the disturbance and the different human actions performed to recover the freshwater environment. The concept of 'ecological resilience' was thus better suited than 'engineering resilience' for such open systems facing constant fluctuations in environmental drivers. With regard to ecological succession, the more impacted stations were marked by a significant change in taxonomic beta-diversity, with numerous stochastic processes, due to taxa dispersion. They first exhibited a convergence in functional traits due to the increase in nutrient availability drained from the catchment basin and then an increase in divergence when nutrients became limited.

Impact of human activities on the spatio-seasonal dynamics of plankton diversity in drained marshes and consequences on eutrophication.

The functional diversity of two planktonic functional compartments, the nano-microphytoplankton and the mesozooplankton was used in order to better understand i) the drained marshes functioning and their related ecological functions, ii) the impacts of human control (replenishment) and human activities on the catchment basin (urbanization and catchment basin size). It was based on a monthly seasonal survey on 7 freshwater drained marshes. Both nano-microphyto- and mesozooplankton displayed high seasonal variations linked to the environmental fluctuations and human control on sea lock gates. Winter presented the lower biomasses of both compartments. Winter that is characterized by low water temperature, low light availability and high flood is actually related to the dominance of tychopelagic phytoplankton and K-strategists zooplankton. Spring and summer were characterized by i) the succession of pelagic large cells, small cells and then taxa with alternatives food strategies due to nitrogen limitation and phosphorous desorption from the sediment leading to eutrophication processes and ii) the dominance of r-strategists for mesozooplankton. The artificial summer replenishment acts positively on water quality by decreasing the eutrophication processes since the nitrogen inputs limit the proliferation of phytoplankton mixotrophs and diazotrophs and increase the ecological efficiency during the warm period. Both small and large catchment basins may lead to summer eutrophication processes in drained marshes since the largest ones imply higher hydrodynamic features at the root of large inputs of nitrogen nutrient favoring the phytoplankton development while the smallest ones exhibit hypoxia problems due to high proliferation of macrophytes. Urbanized marshes are less subjected to eutrophication during summer than non urbanized marshes due to more recurrent nutrient inputs from urban waste. However they exhibited a lower ecological efficiency. The results suggest that a better management of the hydrodynamics of such anthropogenic systems can avoid eutrophication risks on coastal areas.

A quality improvement program for adolescents with cystic fibrosis: focus on psychosocial skills.

BACKGROUND: The two pediatric cystic fibrosis centers (CFCs) in Paris (Robert Debré) and Nantes, France, have been developing therapeutic patient education (TPE) programs since 2006 and have been engaged in the pilot phase of the quality improvement program (QIP) named the Hospital Program to Improve Outcomes and Expertise in Cystic Fibrosis (PHARE-M) since 2011. The objective was to improve the FEV1 of the cohort of adolescents to prepare them for their optimal transition to an adult CFC. METHODS: The two CFCs formed a multidisciplinary quality team and used the analysis of causes of insufficient respiratory function taking into account the adolescents' psychosocial factors. At the Nantes CFC, the approach was centered on adolescents' body image and their motivation to take care of themselves by assigning specific aspects of patient follow-up to each professional in the team. At R. Debré, an individual cause-and-effect diagram identified for each patient the medical and psychosocial factors that could account for insufficient respiratory function. Personalized actions were offered to each patient. RESULTS: In 2014, the median FEV1 (Forced Expiratory Volume in 1 Second) of the adolescent cohort exceeds 90% at the 2 CFCs (Nantes and R. Debré). Between 2011 and 2014 both centers improved their ranking for FEV1% in adolescents in the Registry histograms. At R. Debré, the personalized process allowed to reinforce equality of care, offering to all the opportunity to benefit from TPE sessions and coaching with an adapted physical activity teacher. The psychologist developed a specific tool to support the patient-centered process. CONCLUSION: The link between TPE and QIP was strong at our two centers enhancing patient centered care and targeting an optimal transition to an adult program.

Carbon and nitrogen elemental and isotopic ratios of filter-feeding bivalves along the French coasts: An assessment of specific, geographic, seasonal and multi-decadal variations.

Primary consumers play a key role in coastal ecosystems by transferring organic matter from primary producers to predators. Among them, suspension-feeders, like bivalve molluscs are widely used in trophic web studies. The main goal of this study was to investigate variations of C and N elemental and isotopic ratios in common bivalves (M. edulis, M. galloprovincialis, and C. gigas) at large spatial (i.e. among three coastal regions) and different temporal (i.e. from seasonal to multi-decadal) scales in France, in order to identify potential general or specific patterns and speculate on their drivers. The observed spatial variability was related to the trophic status of the coastal regions (oligotrophic Mediterranean Sea versus meso- to eutrophic English Channel and Atlantic ocean), but not to ecosystem typology (estuaries, versus lagoons versus bays versus littoral systems). Furthermore, it highlighted local specificities in terms of the origin of the POM assimilated by bivalves (e.g., mainly continental POM vs. marine phytoplankton vs. microphytobenthic algae). Likewise, seasonal variability was related both to the reproduction cycle for C/N ratios of Mytilus spp. and to changes in trophic resources for δ13C of species located close to river mouth. Multi-decadal evolution exhibited shifts and trends for part of the 30-year series with decreases in δ13C and δ15N. Specifically, shifts appeared in the early 2000's, likely linking bivalve isotopic ratios to a cascade of processes affected by local drivers.

Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2years of treatment with ivacaftor in a real-world setting.

BACKGROUND: Ivacaftor has been shown to improve lung function and body weight in patients with CF and a gating mutation. Real-world evaluation is warranted to examine its safety and effectiveness over the long term. METHODS: A retrospective observational multicentre study collected clinical data in the year before and the 2years after ivacaftor initiation in patients with CF and a Gly551Asp-CFTR mutation. RESULTS: Fifty-seven patients were included. Mean absolute change in FEV1% predicted improved from baseline to Year 1 (8.4%; p<0.001) and Year 2 (7.2%; p=0.006). Statistically significant benefits were observed with increased body mass index, fewer Pseudomonas aeruginosa and Staphylococcus aureus positive cultures, and decreased IV antibiotics and maintenance treatment prescriptions (including azithromycin, Dornase alpha and nutritional supplements). No significant adverse events were reported. CONCLUSION: The clinical benefits of ivacaftor reported in previous clinical trials were confirmed in a real-world setting two years post-initiation, also reducing treatment burden.

Identifying the educational needs of lung transplant recipients with cystic fibrosis.

PURPOSE: To elucidate the education needs of patients who have undergone lung transplant for cystic fibrosis while participating in the development of therapeutic education programs in French transplant centers. METHODS: From January 2009 to March 2012, in-depth educational diagnosis interviews were conducted with 42 adult transplant recipients with cystic fibrosis who were being followed up at 7 French transplant centers. Several areas were explored: health status, social and occupational outcomes, knowledge about the disease and treatments, and experience of the disease. The interviews combined open- and closed-ended questions for more systematic exploration of knowledge about the disease and treatments. RESULTS: After receiving the transplant, the patients' health had improved and their lives were returning to normal. They had acquired much usable knowledge about managing their disease. Educational needs regarding transplant-related complications and management of immunosuppressive drugs were evident. The analysis also demonstrated the need to better inform patients about pregnancy and new social rights and to offer them psychological support in adjusting to their new health status. CONCLUSION: Therapeutic education of patients should continue well after transplant to maintain safety knowledge and meet patients' new needs. A comprehensive therapeutic education program for transplant candidates that includes the preparatory, immediate posttransplant, and late posttransplant phases is needed.

Climatic facilitation of the colonization of an estuary by Acartia tonsa.

Global change has become a major driving force of both terrestrial and marine systems. Located at the interface between these two realms, estuarine ecosystems are probably the place where both direct and indirect effects of human activities conspire together to affect biodiversity from phytoplankton to top predators. Among European estuarine systems, the Gironde is the largest estuary of Western Europe and many studies have provided evidence that it has been affected by a variety of anthropogenic stressors such as thermal and chemical pollution, physical alterations and exploitation, especially for maritime traffic. In such a context, species introduction is also a current major issue with the establishment of strong competitive species that could lead to ecosystem reorganization with potential decrease or even disappearance of native species. In the Gironde estuary, this hypothesis was proposed for the invasive shrimp species Palaemon macrodactylus as a decrease in the native species abundance was observed at the same time. Although species introduction often takes place via ballast water, the influence of climate-driven changes on the establishment of new species remains a key issue. The calanoid copepod Acartia tonsa, observed in the Gironde estuary for the first time in 1983, have since colonized most part of the estuary, reaching a level of abundance comparable to the dominant native species Eurytemora affinis. In this study, using both the concept of the ecological niche sensu Hutchinson (fundamental and realized niches) and statistical models, we reveal that the dynamics of the colonization of A. tonsa was facilitated by environmental conditions that have become closer to its environmental optimum with respect to temperature and salinity.

Objectives and applications of phenotyping network set-up for livestock.

Providing phenotypic information, which is accurate, reliable, repeatable and comparable across countries or laboratories, is critical to gain a better understanding of the relationship between genes and phenotypes. So far, it is indeed extremely difficult to combine different sources of phenotypic data from multiple origins, partly because of the variability in the methods of phenotyping. The phenotyping program of livestock involves the definition of complex phenotypes obtained from data integration at different levels (from molecules to herds), the implementation of the latest technologies to accurately characterize at high speed and low cost, the greatest number of animals in a better characterized environment, and the development and sharing of large databases for data analysis and modeling. Such a program also involves the construction of a coordinated network of research and professional facilities and a common language with shared definition of unambiguous animal traits and of methods to assess them. To this end, it will build on the 'Animal Trait Ontology of Livestock' (ATOL) project with the objective of defining precisely the phenotypes of interest for farm animals. Then, it will be necessary to combine an environmental information system related to animal husbandry and associated methods to capture the phenotypic differences between animals.

Assessment of vaccination by a phase I Coxiella burnetii-inactivated vaccine in goat herds in clinical Q fever situation.

A study was carried out to assess the efficacy of vaccination, using a phase I Coxiella burnetii-inactivated vaccine (Coxevac®; CEVA), within three goat herds experiencing Q fever abortions waves. The stratification of the population (n = 905) was based on parity and on infection status related to both serological and qPCR vaginal shedding results. Control (n = 443) and vaccinated (n = 462) groups were established in each farm. Vaccination was administered to does before mating and to kids after active immunity acquisition (at least 3­4 months old). The vaccine effectiveness was analyzed at subsequent farrowing on both clinical incidence and vaginal shedding at the delivery day. Among the 231 animals considered as susceptible, that is, seronegative nonshedders, about 90% were infected whatever the group, showing that vaccination did not prevent infection under high infection exposure. Fortunately, vaccination induced an overall decrease in shedding levels. A significant average difference between groups was estimated to 1.16 log(10) bacteria per swab for primiparous and even higher (1.81 log(10)) for initially susceptible ones. Thus, in a clinical context, vaccination should be implemented first in renewal animals. Indeed, young animals are those which best respond to vaccination by significantly reducing C. burnetii burden and, conversely, which excrete bacteria most massively if not vaccinated.

Coxiella burnetii vaginal shedding and antibody responses in dairy goat herds in a context of clinical Q fever outbreaks.

This study, carried out in three goat herds, was aimed at describing individual responses to Q fever infection in an abortive context, focusing on both antibody and shedding levels. Seroprevalence of Coxiella burnetii (Cb) infection and vaginal shedding of 1083 goats were investigated using ELISA and realtime qPCR assays, respectively. At the end of the outbreaks, a seroprevalence of 45.0% was found, and vaginal shedding appeared massive with levels above 10(4) Cb per swab in 42.3% of the whole population and above 10(6) Cb per swab for 90.9% of aborted goats. Susceptible animals (i.e. seronegative nonshedders) were unfrequent (31.2%), most of them being kids (94.7%). Seronegative females were predominant among nonshedders and conversely seropositive ones, predominant among high shedders (above 10(6) Cb per swab). Nevertheless, at least 43.3% of seronegative goats shed bacteria confirming the need of interpreting serology on a herd scale. The subsequent farrowing period was characterized by a significant reduction in the number of clinical cases. Females that had already aborted were more often involved than others. Shedding quantities remained high, particularly for primiparous does, mainly when facing infection for the first time. Thus, Q fever control must be based on both preventive measures directed to the preherd and environmental precautions.

Variability of fresh- and salt-water marshes characteristics on the west coast of France: a spatio-temporal assessment.

The degradation of water quality and the multiple conflicts of interest between users make marsh restoration very important. A Water Quality Evaluation System (WQES) was developed for river systems by the European Water Framework Directive (WFD). Some form of biologically-based, habitat-specific reference standard seems absolutely essential for wise management and stewardship of marsh ecosystems. The goal of this study was to develop a statistical method to define and to characterize a water body typology for drained marshes of the Charente-Maritime wetlands on the French Atlantic coast, placing particular emphasis on environmental factors as hydraulic functioning, human activities and pedological substratum. The Charente-Maritime marshes represent a good field study because of his high diversity of types of marshes and of anthropogenic activities in a restrictive area thus erasing spatial climatic effect (latitude effect). The statistical method developed here had permitted to define and characterize 12 different water bodies, 7 in freshwater (F1 to F7) and 5 in salt water marshes for the Charente-Maritime area. This typology demonstrated an important link between the size catchment area, nitrate concentrations, and leaching of precipitation from cultured soils. Even though the Charente-Maritime marshes are strongly impacted by humans, they may still retain the ability to remove nitrate. The increasing gradient of water renewal in the freshwater marshes from F1 to F7 explained the decreasing gradient of eutrophication. A better management of the hydrodynamic of the marshes can avoid eutrophication risk on the coastal sea area. Reliance on the WFD parameter set necessarily placed limits on the kinds of interpretations that could be made and on the study's potential contribution to the basic science of marshes. Ecologically-based insights regarding both external flows (links between ecosystems, meta-ecosystem theory) and internal flows (structure of the planktonic food web) seem an essential prerequisite for further advances in the study of marsh ecosystems.

Psychological effects of false-positive results in cystic fibrosis newborn screening: a two-year follow-up.

OBJECTIVE: To evaluate parental stress after a false-positive result at the time of the cystic fibrosis (CF) newborn screening (NBS), attributable to heterozygotism or persistent hypertrypsinemia. STUDY DESIGN: A prospective study was conducted in 86 French families at 3, 12, and 24 months after NBS. A psychologist conducted interviews with a questionnaire, the Perceived Stress Scale, and the Vulnerable Child Scale. RESULTS: Overall, 96.5% of parents said they had been anxious at the time of the sweat test. However, 86% felt entirely reassured 3 months after the test. The mean Perceived Stress Scale score did not differ from that observed in the French population. Mean Vulnerable Child Scale scores were high, associated with a low Parental Perception of Child Vulnerability. These results did not differ significantly at 1 and 2 years. In total, 86% to 100% of families no longer worried about CF. All parents stated that they would have the test performed again for another child. CONCLUSIONS: CF NBS can lead to false-positive results, causing parental anxiety, which quickly decreases after a sweat test performed soon after the phone call.

Pancreatic enzyme replacement therapy for young cystic fibrosis patients.

UNLABELLED: Maldigestion in cystic fibrosis (CF) affects approximately 90% of patients. As soon as pancreatic insufficiency is identified, enzyme supplementation is prescribed even with breast fed infants. A pancreatic enzyme preparation developed particularly for infants, Creon for children (CfC), contains smaller granules to be administered with a dosing spoon (5000 lipase units per scoop). PATIENTS AND METHODS: In a prospective, randomised, multi-centre study, 40 infants and toddlers received both CfC and Creon 10000 (C10) for two weeks each in a cross-over design. Dosing of pancreatic enzymes was continued as applied before the study. The primary endpoint was the parents' treatment preference. Secondary endpoints included coefficient of fat absorption (CFA), clinical symptoms and safety parameters. RESULTS: 20 parents (51%) from the N=39 intent to treat sample preferred CfC, 9 (23%) preferred C10, and 10 (26%) had no preference The applied doses led to a mean CFA with similar results for both treatments (77.8% vs. 78.7%). Gastrointestinal symptoms were reported on a number of study days, and some children had abnormal results for laboratory parameters of malabsorption. Safety and tolerability of the preparations were good and all these parameters were comparable for both treatments. CONCLUSION: Those parents who had a preference favoured CfC over C10. Both enzyme preparations improved malabsorption to a similar degree, although the applied dosages could have been too low in some children reflected in a suboptimal CFA. These data support the use of CfC for young patients with cystic fibrosis improving the daily care of this cohort detected mainly now through neonatal screening programmes.

Third case of paternal isodisomy for chromosome 7 with cystic fibrosis: a new patient presenting with normal growth.

Many patients with maternal uniparental disomy of chromosome 7 (UPD7) have been described, mainly with intrauterine and postnatal growth retardation or with Silver-Russell syndrome. In contrast, only three cases of paternal UPD7 have been reported, all associated with recessive disorders. Here, we report on the clinical and molecular data of the third patient with paternal UPD7 and cystic fibrosis. Pre- and postnatal growth were normal. These findings support the hypothesis that paternal isodisomy for human chromosome 7 may have no phenotypic effect on growth.

Remnant amount and cleanup for 3 adhesives after debracketing.

The cleanup of remnant bonding adhesive from the enamel surface after debonding is an important factor for clinicians. The purposes of this study were to compare the weight, the surface area, and the cleanup times of remnant adhesive for a composite resin, Transbond (TB); a resin-modified glass ionomer, Fuji ORTHO LC bonded to enamel both conditioned (FOC) and nonconditioned (FONC); and a fluoride-releasing composite resin, Advance (ADV), bonded to nonetched enamel. In addition, 2 qualitative methods for scoring remnant adhesive were compared with the quantitative weight and area data. Forty extracted human incisors were weighed, bonded with brackets, debonded, weighed, and photographed. Area was measured from the photographs with a sonic digitizer. Mean adhesive remnant weights differed between groups (analysis of variance [ANOVA], P =.02): The remnants from ADV and FOC were equal and both significantly heavier than the remnants from FONC; the weights of the TB remnants were intermediate between the heavier ADV and FOC remnants and the lighter FONC remnants. Mean remnant areas differed between groups (ANOVA, P =.03): The remnants from ADV were significantly larger than the remnants from TB and FONC, which were equal; the areas of the FOC remnants were intermediate between the larger ADV remnants and the smaller remnants from TB and FONC. Mean cleanup times also differed between groups (ANOVA, P <.001): TB and FOC had equal times that were significantly longer than the times for ADV and FONC, which were equal. Adhesives bonded to acid-etched or conditioned enamel took about 1 and a half times longer to clean up than did those bonded to nonetched enamel. When bonded to conditioned enamel, the resin-modified glass ionomer had mean remnant adhesive weights, areas, and cleanup times statistically equivalent to TB. ADV had the fastest mean cleanup time per amount of remnant (ANOVA, P <.002). The graphs of scores for 2 qualitative methods used for scoring remnant amount did not closely resemble the graphs based on weight and area. The graph of a newly introduced qualitative method better reflected the area data. Weighing may be a useful method for quantifying remnant adhesive.