514 research outputs found

    The Fontan operation in infants less than 2 years of age

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    AbstractYoung age remains a reported risk factor for a successful Fontan operation despite improved survival rates. Since March 1978, the Fontan operation has been performed in 47 patients. To avoid a primary or secondary polliative shunt, an early Fontan procedure (Group 1: mean age 1.5 ± 0.5 years, range 0.6 to 2) has been performed in 17 children with the outcome similar to that of the remaining 30 older patients (Group 2: mean age 7.5 ± 5 years, range 2.4 to 23 years). Preoperatively both groups had acceptable hemodynamic status for a successful Fontan result.Operative variables including cardiopulmonary bypass time, aortic cross-clamp time and core temperature were similar between groups and did not affect mortality. The postoperative mortality rate including early surgical (0% vs. 13%, respectively), late (18% vs. 12%) and total (18% vs. 23%) was similar between Groups 1 and 2 (p > 0.05). Immediate postoperative arrhythmias were more frequent in Group 1 (71% vs. 25%, p < 0.01) with no related mortality, while late arrhythmias occurred with equal frequency (29% vs. 39%, p > 0.05). Group 1 infants required a longer hospital stay (22 ± 9 vs. 14 ± 5 days, p < 0.01).Thus, young age is not a risk factor for successful outcome of the Fontan operation in patients with acceptable preoperative hemodynamic status. An early Fontan operation may also avoid prolonged palliative procedures and their potential deleterious effects

    Down-regulation of OPA1 in patients with primary open angle glaucoma

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    PURPOSE: Heterozygous optic atrophy type1 (OPA1) mutations are responsible for dominant optic atrophy, and the down regulation of OPA1 expression in patients with Leber hereditary optic neuropathy may imply that Opa1 protein levels in mitochondria play a role in other spontaneous optic neuropathies as well. Mitochondrial and metabolic abnormalities may put the optic nerve at risk in primary open angle glaucoma (POAG), and this preliminary study was designed to investigate whether altered OPA1 expression might be present in the progressive optic neuropathy of POAG. METHODS: Patients were eligible for inclusion if they met standard clinical criteria for POAG, including age greater than 40 years, intraocular pressure ≄ 21 mmHg in at least one eye before treatment, normal-appearing anterior chamber angles bilaterally on gonioscopy, and optic nerve injury characteristic of POAG. RNA was extracted from leukocytes and converted to cDNA by reverse transcriptase enzyme, and real time PCR was used to assess expression levels of OPA1 and the ÎČ-globulin (HBB) housekeeping gene. The ratio of OPA1 expression to HBB expression (OPA1/HBB) for POAG patients was compared to that of controls and to clinical characteristics of POAG patients. RESULTS: Forty-three POAG patients and 27 controls were completely phenotyped with a full ophthalmologic examination and static perimetry. Mean age (POAG 67.9 years; controls 61.8 years) and sex (POAG 26 males/17 females; controls 11/16) were similar for the two groups. Mean OPA1/HBB of POAG patients (1.16, SD 0.26) was 18% lower than controls (1.41, SD 0.50), and this difference was statistically significant (p≀0.021). OPA1 expression differed between the groups (p≀0.037), but HBB expression did not differ (p≀0.24). OPA1/HBB was not correlated with any clinical feature of POAG patients. CONCLUSIONS: Transcriptional analysis of peripheral blood leucocytes is a limited model system for studying the consequences of mitochondrial abnormalities in the optic nerve. Nevertheless, OPA1 is known to affect mitochondrial stability and has now been implicated in several spontaneous optic neuropathies. Decreased OPA1 expression in POAG patients is another indication that mitochondrial function, and possibly mitochondrially-induced apoptosis, may play a role in the development of POAG

    Mid-infrared spectra of PAH emission in Herbig AeBe stars

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    We present spectra of four Herbig AeBe stars obtained with the Infrared Spectrograph (IRS). on the Spitzer Space Telescope. All four of the sources show strong emission from polycyclic aromatic hydrocarbons (PAHs), with the 6.2 um emission feature shifted to 6.3 um and the strongest C-C skeletal-mode feature occuring at 7.9 um instead of at 7.7 um as is often seen. Remarkably, none of the four stars have silicate emission. The strength of the 7.9 um feature varies with respect to the 11.3 um feature among the sources, indicating that we have observed PAHs with a range of ionization fractions. The ionization fraction is higher for systems with hotter and brighter central stars. Two sources, HD 34282 and HD 169142, show emission features from aliphatic hydrocarbons at 6.85 and 7.25 um. The spectrum of HD 141569 shows a previously undetected emission feature at 12.4 um which may be related to the 12.7 um PAH feature. The spectrum of HD 135344, the coolest star in our sample, shows an unusual profile in the 7-9 um region, with the peak emission to the red of 8.0 um and no 8.6 um PAH feature.Comment: Accepted by ApJ 23 June, 2005, 8 pages (emulateapj), 5 figures (3 in color

    Sex differences in physiological progression of idiopathic pulmonary fibrosis

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    In idiopathic pulmonary fibrosis, incidence is higher in males, and females may have better survival. The aim of the present study was to determine whether the rate of increase in desaturation during serial 6-min walk testing would be greater, and survival worse, for males versus females. Serial changes in the percentage of maximum desaturation area (DA) over 1 yr were estimated using mixed models in 215 patients. DA was defined as the total area above the curve created using desaturation percentage values observed during each minute of the 6-min walk test. Multivariate Cox regression assessed survival differences. Adjusting for baseline DA, 6-min walk distance, change in 6-min walk distance over time and smoking history, the percentage of maximum DA increased by an average of 2.83 and 1.37% per month for males and females, respectively. Females demonstrated better survival overall, which was more pronounced in patients who did not desaturate below 88% on ambulation at baseline and after additionally adjusting for 6-month relative changes in DA and forced vital capacity. These data suggest that differences in disease progression contribute to, but do not completely explain, better survival of females with idiopathic pulmonary fibrosis.This work was supported by National Institutes of Health grants 5P50HL56402, U10HL080371, 2K24HL04212, K12RR024987 and K23HL68713, and the Alberta Heritage Medical Foundation.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/91966/1/2008 ERJ - Sex Differences in Physiologic Progression of Idiopathic Pulmonary Fibrosis.pd

    Pulmonary function measures predict mortality differently in IPF versus combined pulmonary fibrosis and emphysema

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    The composite physiologic index (CPI) was derived to represent the extent of fibrosis on high-resolution computed tomography (HRCT), adjusting for emphysema in patients with idiopathic pulmonary fibrosis (IPF). We hypothesised that longitudinal change in CPI would better predict mortality than forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC) or diffusing capacity of the lung for carbon monoxide (DLCO) in all patients with IPF, and especially in those with combined pulmonary fibrosis and emphysema (CPFE). Cox proportional hazard models were performed on pulmonary function data from IPF patients at baseline (n=321), 6 months (n=211) and 12 months (n=144). Presence of CPFE was determined by HRCT. A five-point increase in CPI over 12 months predicted subsequent mortality (HR 2.1, p=0.004). At 12 months, a 10% relative decline in FVC, a 15% relative decline in DLCO or an absolute increase in CPI of five points all discriminated median survival by 2.1 to 2.2 yrs versus patients with lesser change. Half our cohort had CPFE. In patients with moderate/severe emphysema, only a 10% decline in FEV1 predicted mortality (HR 3.7, p=0.046). In IPF, a five-point increase in CPI over 12 months predicts mortality similarly to relative declines of 10% in FVC or 15% in DLCO. For CPFE patients, change in FEV1 was the best predictor of mortality.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/91949/1/2011 ERJ - Pulmonary function measures predict mortality differently in IPF versus combined pulmonary fibrosis and emphysema.pd

    Identifying the optimal exercise prescription for patients with coronary artery disease undergoing cardiac rehabilitation: protocol for a systematic review and network meta-analysis of randomized control trials

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    Coronary artery disease (CAD) is one of the leading causes of mortality and morbidity. Exercise-based cardiac rehabilitation (EBCR) has been shown to improve clinical outcomes in these patients, and yet clinicians are often challenged to prescribe the most effective type of exercise training. Therefore, this systematic review and network meta-analysis (NMA) aims to formally quantify the optimal dose of exercise training interventions to improve exercise capacity and quality of life by undertaking direct and indirect pooled comparisons of randomized controlled trials. A detailed search will be conducted on PubMed/MEDLINE, Cumulative Index to Nursing and Allied Health (CINAHL), EMBASE and Web of Science. Two reviewers will screen the existing literature and assess the quality of the studies. Disagreements will be resolved through consensus. We anticipate that the analysis will include pairwise and Bayesian network meta-analyses. Most of the trials have studied the impact of exercise training comparing one or two modalities. As a result, little evidence exists to support which interventions will be most effective. The current NMA will address this gap in the literature and assist clinicians and cardiac rehabilitation specialists in making an informed decision. Results will be disseminated through peer-reviewed journals. Ethical approval is not applicable, as no research participants will be involved. PROSPERO Registration number: CRD42022262644

    The Second-Generation Guide Star Catalog: Description and Properties

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    The GSC-II is an all-sky database of objects derived from the uncompressed DSS that the STScI has created from the Palomar and UK Schmidt survey plates and made available to the community. Like its predecessor (GSC-I), the GSC-II was primarily created to provide guide star information and observation planning support for HST. This version, however, is already employed at some of the ground-based new-technology telescopes such as GEMINI, VLT, and TNG, and will also be used to provide support for the JWST and Gaia space missions as well as LAMOST, one of the major ongoing scientific projects in China. Two catalogs have already been extracted from the GSC-II database and released to the astronomical community. A magnitude-limited (R=18.0) version, GSC2.2, was distributed soon after its production in 2001, while the GSC2.3 release has been available for general access since 2007. The GSC2.3 catalog described in this paper contains astrometry, photometry, and classification for 945,592,683 objects down to the magnitude limit of the plates. Positions are tied to the ICRS; for stellar sources, the all-sky average absolute error per coordinate ranges from 0.2" to 0.28" depending on magnitude. When dealing with extended objects, astrometric errors are 20% worse in the case of galaxies and approximately a factor of 2 worse for blended images. Stellar photometry is determined to 0.13-0.22 mag as a function of magnitude and photographic passbands (B,R,I). Outside of the galactic plane, stellar classification is reliable to at least 90% confidence for magnitudes brighter than R=19.5, and the catalog is complete to R=20.Comment: 52 pages, 33 figures, to be published in AJ August 200

    The state of protoplanetary material 10 Myr after stellar formation: circumstellar disks in the TW Hydrae association

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    We have used the Spitzer Infrared Spectrograph to observe seven members of the TW Hya association, the nearest stellar association whose age (∌\sim 10 Myr) is similar to the timescales thought to apply to planet formation and disk dissipation. Only two of the seven targets display infrared excess emission, indicating that substantial amounts of dust still exist closer to the stars than is characteristic of debris disks; however, in both objects we confirm an abrupt short-wavelength edge to the excess, as is seen in disks with cleared-out central regions. The mid-infrared excesses in the spectra of Hen 3-600 and TW Hya include crystalline silicate emission features, indicating that the grains have undergone significant thermal processing. We offer a detailed comparison between the spectra of TW Hya and Hen 3-600, and a model that corroborates the spectral shape and our previous understanding of the radial structure of these protoplanetary disks.Comment: To appear in the Spitzer special issue of ApJS; 12 pages, 2 figure
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