Objectives To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan. Study design Cross-sectional. The Pediatric Quality of Life ...Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age <19 years) or Short Form Health Survey (age ≥19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined. Results Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL ( P < .001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points ( P = .004) and the emotional functioning score decreased by an average of 0.64 points ( P = .03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age ( P = .02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire ( P < .001) and Short Form Health Survey scores ( P < .001). Conclusions Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood. Trial registration ClinicalTrials.gov : NCT00132782.
At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z -scores for weight and height below the normative population, and body mass index was similar to the normative ...population. Males had the greatest increase in z -scores for body mass index. Trial registration ClinicalTrials.gov: NCT00115934.
We determined the quantum requirements for growth (1/ϕμ) and fatty acid (FA) biosynthesis (1/ϕFA) in the marine diatom, Phaeodactylum tricornutum, grown in nutrient replete conditions with nitrate or ...ammonium as nitrogen sources, and under nitrogen limitation, achieved by transferring cells into nitrogen free medium or by inhibiting nitrate assimilation with tungstate. A treatment in which tungstate was supplemented to cells grown with ammonium was also included. In nutrient replete conditions, cells grew exponentially and possessed virtually identical 1/ϕμ of 40–44 mol photons · mol C−1. In parallel, 1/ϕFA varied between 380 and 409 mol photons · mol C−1 in the presence of nitrate, but declined to 348 mol photons · mol C−1 with ammonium and to 250 mol photons · mol C−1 with ammonium plus tungstate, indicating an increase in the efficiency of FA biosynthesis relative to cells grown on nitrate of 8% and 35%, respectively. While the molecular mechanism for this effect remains poorly understood, the results unambiguously reveal that cells grown on ammonium are able to direct more reductant to lipids. This analysis suggests that when cells are grown with a reduced nitrogen source, fatty acid biosynthesis can effectively become a sink for excess absorbed light, compensating for the absence of energetically demanding nitrate assimilation reactions. Our data further suggest that optimal lipid production efficiency is achieved when cells are in exponential growth, when nitrate assimilation is inhibited, and ammonium is the sole nitrogen source.
Despite hypothesized concerns about deterioration beginning in adolescence, longitudinal data and associated factors regarding standardized assessment of physical functioning are not available for ...Fontan patients. Parents who participated in the Fontan Cross-Sectional Study completed the Child Health Questionnaire at 2 time points for 245 subjects ages 6–18 years. Associations between change in Physical Functioning Summary Score and baseline patient, medical, and laboratory characteristics (mean age 9.5 ± 1.7 years) and follow-up patient and medical characteristics (mean age 16.2 ± 1.6 years) were determined by regression analyses. During a mean of 6.7 ± 0.4 years, a small (not clinically important) but statistically significant decrease in score from 46.2 ± 11.7 to 44.5 ± 12.1 (
p
< 0.03) was noted. Subjects with higher baseline scores had a greater decrease in score (
r
= −0.48;
p
< 0.001). A multivariable model of patient and medical characteristics (
R
2
= 0.11) showed that a greater decrease in score was significantly associated with interim development of asthma (
n
= 13; parameter estimate PE −6.6;
p
< 0.05) or other chronic respiratory, lung, or breathing problems (
n
= 13; PE −12.5;
p
< 0.001) and the presence of protein-losing enteropathy at any time (
n
= 12; PE −9.4;
p
= 0.006). Change in score was not significantly associated with baseline laboratory measures of exercise capacity and ventricular characteristics and function. Therefore, although physical functioning may be stable during adolescence for many Fontan patients, deterioration occurs in some in association with respiratory conditions and protein-losing enteropathy. Further longitudinal study is necessary to better understand the relationship between clinical morbidities and functional health status as these patients transition into adulthood.
Fluorescent pigments in several Indo-Pacific and Caribbean anthozoans have recently been identified as proteins related to the green-fluorescent protein (GFP) of the hydromedusa Aequorea victoria. ...Here we show that GFP is widely distributed in many Caribbean species. The fluorescence excitation and emission spectra for the pigment are similar to those reported elsewhere for coral and noncoral GFP and the fluorescence quantum yield is estimated to be 35%. Spectral and molecular characterization of the isolated protein clearly show it to be GFP, and laboratory and in situ fluorescence measurements and Western blot analysis show that it is widespread. Bathymetric studies of GFP content using Western blots for the ecologically important congeneric corals Montastraea faveolata and Montastraea cavernosa show that there is no significant correlation between depth and GFP concentration. Nucleotide sequence data of GFP from M. faveolata and M. cavernosa show 88.2% sequence homology with each other and 46.4% homology with A. victoria GFP, whereas the percent homology with A. victoria at the amino acid level was 31.1 and 28.4% for M. cavernosa and M. faveolata, respectively, and 82.7% with each other. Measurements of reflectance and of the excitation spectrum for chlorophyll fluorescence in GFP-containing corals indicate that GFP absorption, emission, and reflection have negligible impact on the level of solar radiation reaching the zooxanthellae and therefore play no role in coral photosynthesis by either addition or removal of photons.
At age 6 years, patients with hypoplastic left heart syndrome had mean age-adjusted z-scores for weight (WAZ) and height (HAZ) below and body mass index (BMIZ) similar to the normative population. ...Higher birth weight infants had the greatest decrease in WAZ and HAZ. Males had the greatest increase in BMIZ.
Trial registration ClinicalTrials.gov: NCT00115934
The effects of iron limitation on photosystem II (PSII) composition and photochemical energy conversion efficiency were studied in the unicellular chlorophyte alga Dunaliella tertiolecta. The quantum ...yield of photochemistry in PSII, inferred from changes in variable fluorescence normalized to the maximum fluorescence yield, was markedly lower in iron-limited cells and increased 3-fold within 20 h following the addition of iron. The decrease in the quantum yield of photochemistry was correlated with increased fluorescence emission from the antenna. In iron-limited cells, flash intensity saturation profiles of variable fluorescence closely followed a cumulative one-hit Poisson model, suggesting that PSII reaction centers are energetically isolated, whereas in iron-replete cells, the slope of the profile was steeper and the calculated probability of energy transfer between reaction centers increased to 0.6. Immunoassays revealed that in iron-limited cells the reaction center proteins, D1, CP43, and CP47, were markedly reduced relative to the peripheral light-harvesting Chl-protein complex of PSII, whereas the alpha subunit of cytochrome b559 was about 10-fold higher. Spectroscopic analysis established that the cytochrome b559 peptide did not contain an associated functional heme. We conclude that the photochemical conversion of absorbed excitation energy in iron-limited cells is limited by the number of photochemical traps per unit antenna
The maximal change in the quantum yield of fluorescence $(\triangle \emptyset_sat)$ is a quantitative measure of photosynthetic conversion efficiency of phytoplankton. Using a pump-and-probe ...fluorometer, we measured $\triangle \emptyset_sat$ along the 100-m isobath in the Gulf of Maine in June 1987. The hydrographic regime was characterized by a nutrient-rich, vertically mixed region in the northeast and a nutrient-depleted, stratified region to the southwest. The results reveal that $\triangle \emptyset_sat$ is strongly related to the rate of supply of dissovled inorganic nitrogen and provide strong evidence that photosynthetic energy conversion can be nutrient limited in natural phytoplankton communities.
To explore the perspectives of normal bladder function among women with lower urinary tract symptoms.
This was a secondary analysis of qualitative data from structured interviews with 50 adult women ...with lower urinary tract symptoms. A directed content analysis of the transcripts explored women's perspectives on normal bladder function.
Participants’ descriptions of “normal” took many forms and were based on several aspects of bladder function. A prominent feature of normal was that voiding occurred as a seamless process, beginning with an urge sensation, followed by voiding with ease and to completion, and then “being done.” Descriptions of normal were based largely on concepts of voiding regularity, including voiding frequency, intervals, and patterns during the day and night. Another aspect of normal bladder function was the notion of having control in terms of not leaking urine, as well as the ability to hold urine and defer urination. Views of normal bladder function extended to the absence of symptoms and the impact of being symptom-free on day-to-day life, including not having to think about or worry about the bladder or limit daily activities.
Women's perspectives on normal bladder function are multifaceted, reflecting attributes most salient to each individual and likely informed by their personal experience with symptoms and their influence on daily life. This work has implications for how clinicians might engage women in discussing bladder symptoms and can inform future research and public health messaging about normal bladder function.
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