204 research outputs found

    Early childhood lung function is a stronger predictor of adolescent lung function in cystic fibrosis than early Pseudomonas aeruginosa infection

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    Pseudomonas aeruginosa has been suggested as a major determinant of poor pulmonary outcomes in cystic fibrosis (CF), although other factors play a role. Our objective was to investigate the association of early childhood Pseudomonas infection on differences in lung function in adolescence with CF

    Deriving a multivariate CO-to-H2_2 conversion function using the [CII]/CO(1-0) ratio and its application to molecular gas scaling relations

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    We present Herschel PACS observations of the [CII] 158 micron emission line in a sample of 24 intermediate mass (9<logM_\ast/M_\odot<10) and low metallicity (0.4< Z/Z_\odot<1.0) galaxies from the xCOLD GASS survey. Combining them with IRAM CO(1-0) measurements, we establish scaling relations between integrated and molecular region [CII]/CO(1-0) luminosity ratios as a function of integrated galaxy properties. A Bayesian analysis reveals that only two parameters, metallicity and offset from the star formation main sequence, Δ\DeltaMS, are needed to quantify variations in the luminosity ratio; metallicity describes the total dust content available to shield CO from UV radiation, while Δ\DeltaMS describes the strength of this radiation field. We connect the [CII]/CO luminosity ratio to the CO-to-H2_2 conversion factor and find a multivariate conversion function αCO\alpha_{CO}, which can be used up to z~2.5. This function depends primarily on metallicity, with a second order dependence on Δ\DeltaMS. We apply this to the full xCOLD GASS and PHIBSS1 surveys and investigate molecular gas scaling relations. We find a flattening of the relation between gas mass fraction and stellar mass at logM_\ast/M_\odot<10. While the molecular gas depletion time varies with sSFR, it is mostly independent of mass, indicating that the low LCO_{CO}/SFR ratios long observed in low mass galaxies are entirely due to photodissociation of CO, and not to an enhanced star formation efficiency.Comment: Submitted to MNRAS, this version after referee comments. 21 page

    Treatment of anorexia and weight loss with megestrol acetate in patients with cystic fibrosis

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    Four patients with severe cystic fibrosis lung disease, anorexia and weight loss, received Megestrol Acetate (MA), as an appetite stimulant. The initial dose was 400–800 mg daily and was continued for 6–15 months. Appetite was improved, with significant weight gain in all patients and an increase in their weight for age percentile from <5% at the start of the study to approximately the 25 th percentile after 6 months of use and improvement in quality of life. One patient discontinued MA after 6 months, and subsequently appetite and weight were depressed. Pediatr Pulmonol. 1999; 28:380–382. © 1999 Wiley-Liss, Inc.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/35316/1/11_ftp.pd

    Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report

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    Newborn screening for cystic fibrosis offers the opportunity for early intervention and improved outcomes. This summary, resulting from a workshop sponsored by the Cystic Fibrosis Foundation to facilitate implementation of widespread high quality cystic fibrosis newborn screening, outlines the steps necessary for success based on the experience of existing programs. Planning should begin with a workgroup composed of those who will be responsible for the success of the local program, typically including the state newborn screening program director and cystic fibrosis care center directors. The workgroup must develop a screening algorithm based on program resources and goals including mechanisms available for sample collection, regional demographics, the spectrum of cystic fibrosis disease to be detected, and acceptable failure rates of the screen. The workgroup must also ensure that all necessary guidelines and resources for screening, diagnosis, and care be in place prior to cystic fibrosis newborn screening implementation. These include educational materials for parents and primary care providers; systems for screening and for providing diagnostic testing and counseling for screen-positive infants and their families; and protocols for care of this unique population. This summary explores the benefits and risks of various screening algorithms, including complex situations that can occur involving unclear diagnostic results, and provides guidelines and sample materials for state newborn screening programs to develop and implement high quality screening for cystic fibrosis
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