Isolated major aortopulmonary collateral as the sole pulmonary blood supply to an entire lung segment

Congenital systemic-to-pulmonary collateral arteries or major aortopulmonary collaterals are associated with cyanotic congenital heart disease with decreased pulmonary blood flow. Though it is usually associated with congenital heart diseases, there is an increased incidence of isolated acquired aortopulmonary collaterals in premature infants with chronic lung disease. Interestingly, isolated congenital aortopulmonary collaterals can occur without any lung disease, which may cause congestive heart failure and require closure. We present a neonate with an echocardiogram that showed only left-sided heart dilation. Further workup with a CT angiogram demonstrated an anomalous systemic artery from the descending thoracic aorta supplying the left lower lobe. He eventually developed heart failure symptoms and was taken to the catheterization laboratory for closure of the collateral. However, with the collateral being the only source of blood flow to the entire left lower lobe, he required surgical unifocalization. Isolated aortopulmonary collaterals without any other congenital heart disease or lung disease are rare. Our patient is the first reported case to have an isolated aortopulmonary collateral being the sole pulmonary blood supply to an entire lung segment. Due to its rarity, there is still much to learn about the origin and development of these collaterals that possibly developed prenatally

Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: A longitudinal study

Pulmonary arteriovenous malformations (PAVMs) often occur in children with hereditary hemorrhagic telangiectasia (HHT). A 14-year longitudinal study of PAVMs in children with HHT was undertaken to assess the prevalence, the clinical impact, and progression of these malformations. This was a retrospective, single-center study from May 2002 to December 2016 of 129 children with HHT diagnosed using Curacao criteria and/or confirmed by genetic testing. Transthoracic contrast echocardiography (TTCE) was the primary screening modality in all patients and PAVMs were diagnosed based on Barzilai criteria. Moderately positive TTCE (Barzilai criteria ≥ 2) was confirmed with subsequent contrast chest CT. New PAVMs were diagnosed with a positive TTCE after an initial negative TTCE. Embolization of PAVMs were performed according to HHT consensus guidelines. Of 129 children with HHT, 76 (59%) were found to have PAVMs. Sixty-seven (88%) were positive for PAVMs on initial screening. Of 63 children without PAVMs on initial screening, 31 were followed for \u3e1 year. Nine of the 31 (29%) developed new PAVMs after initial negative study. Thirty-eight (50%) of the total 76 children with PAVMs had or developed lesions large enough to be treated with embolization. Nine patients with PAVMs initially too small to be treated with embolization, developed progression of disease and ultimately were treated with embolization over time. The majority, 60% (23/38), of the children with large PAVMs had no related clinical symptoms. After embolization, 21% (8/38), of patients underwent repeat interventions. Genetic diagnosis, age, and gender were not associated with risk of having PAVM nor with need for repeat interventions. Nearly 60% of children with HHT develop PAVMs. The risk for new PAVMs to develop, small PAVMs to become large, and previously embolized PAVMs to require further intervention remains throughout childhood. Thus, children with HHT require continued follow-up until adulthood

Tyrosine-phosphorylated and nonphosphorylated isoforms of α-dystrobrevin: roles in skeletal muscle and its neuromuscular and myotendinous junctions

α-Dystrobrevin (DB), a cytoplasmic component of the dystrophin–glycoprotein complex, is found throughout the sarcolemma of muscle cells. Mice lacking αDB exhibit muscular dystrophy, defects in maturation of neuromuscular junctions (NMJs) and, as shown here, abnormal myotendinous junctions (MTJs). In normal muscle, alternative splicing produces two main αDB isoforms, αDB1 and αDB2, with common NH2-terminal but distinct COOH-terminal domains. αDB1, whose COOH-terminal extension can be tyrosine phosphorylated, is concentrated at the NMJs and MTJs. αDB2, which is not tyrosine phosphorylated, is the predominant isoform in extrajunctional regions, and is also present at NMJs and MTJs. Transgenic expression of either isoform in αDB−/− mice prevented muscle fiber degeneration; however, only αDB1 completely corrected defects at the NMJs (abnormal acetylcholine receptor patterning, rapid turnover, and low density) and MTJs (shortened junctional folds). Site-directed mutagenesis revealed that the effectiveness of αDB1 in stabilizing the NMJ depends in part on its ability to serve as a tyrosine kinase substrate. Thus, αDB1 phosphorylation may be a key regulatory point for synaptic remodeling. More generally, αDB may play multiple roles in muscle by means of differential distribution of isoforms with distinct signaling or structural properties

Pulmonary Artery Acceleration Time Provides a Reliable Estimate of Invasive Pulmonary Hemodynamics in Children

Background Pulmonary artery acceleration time (PAAT) is a non-invasive method to assess pulmonary hemodynamics, but lacks validity in children. This study sought to evaluate the accuracy of Doppler echocardiography (DE) derived PAAT in predicting right heart catheterization (RHC) derived pulmonary arterial pressure (PAP), pulmonary vascular resistance (PVR) and compliance in children. Methods Prospectively acquired and retrospectively measured DE derived PAAT and RHC derived systolic PAP (sPAP), mean PAP (mPAP), index PVR (PVRi) and compliance were compared by regression analysis in a derivation cohort of 75 children (median age, 5.3 years; 1.3–12.6) with wide ranges of pulmonary hemodynamics. To account for heart rate variability, PAAT was adjusted for right ventricle ejection time (RVET) and corrected by the RR interval. Regression equations incorporating PAAT and PAAT:RVET from the derivation cohort were then evaluated for the accuracy of its predictive values for invasive pulmonary hemodynamics in a validation cohort of 50 age- and weight- matched children with elevated PAP and PVR. Results There were significant inverse correlations between PAAT and RHC derived mPAP (r = −0.82) and PVRi (r= −0.78) and direct correlation (r= 0.78) between PAAT and pulmonary compliance in the derivation cohort. For detection of pulmonary hypertension (PRVi > 3 WU x m2 and mPAP > 25 mmHg), PAAT < 90 msec and PAAT:RVET < 0.31 resulted in a sensitivity of 97% and a specificity of 95%. In the derivation cohort, the regression equations relating PAAT with mPAP and PVRi were: mPAP = 48 – 0.28 x PAAT and PVRi = 9 –0.07 x PAAT. These PAAT integrated equations predicted RHC measured pulmonary hemodynamics in the validation cohort with good correlations (r = 0.88, 0.83 respectively), small biases (<10%), and minimal coefficient of variation (<8%). Conclusions PAAT inversely correlates with RHC measured pulmonary hemodynamics and directly correlates with pulmonary arterial compliance in children. The study established PAAT based regression equations in children to accurately predict RHC derived PAP and PVR

Limits on the Optical Brightness of the Epsilon Eridani Dust Ring

The STIS/CCD camera on the {\em Hubble Space Telescope (HST)} was used to take deep optical images near the K2V main-sequence star $\epsilon$ Eridani in an attempt to find an optical counterpart of the dust ring previously imaged by sub-mm observations. Upper limits for the optical brightness of the dust ring are determined and discussed in the context of the scattered starlight expected from plausible dust models. We find that, even if the dust is smoothly distributed in symmetrical rings, the optical surface brightness of the dust, as measured with the {\em HST}/STIS CCD clear aperture at 55 AU from the star, cannot be brighter than about 25 STMAG/"$^2$. This upper limit excludes some solid grain models for the dust ring that can fit the IR and sub-mm data. Magnitudes and positions for $\approx$59 discrete objects between 12.5" to 58" from $\epsilon$ Eri are reported. Most if not all of these objects are likely to be background stars and galaxies.Comment: Revision corrects author lis

Association of weekly suicide rates with temperature anomalies in two different climate types

Annual suicide deaths outnumber the total deaths from homicide and war combined. Suicide is a complex behavioral endpoint, and a simple cause-and-effect model seems highly unlikely, but relationships with weather could yield important insight into the biopsychosocial mechanisms involved in suicide deaths. This study has been designed to test for a relationship between air temperature and suicide frequency that is consistent enough to offer some predictive abilities. Weekly suicide death totals and anomalies from Toronto, Ontario, Canada (1986-2009) and Jackson, Mississippi, USA (1980-2006) are analyzed for relationships by using temperature anomaly data and a distributed lag nonlinear model. For both analysis methods, anomalously cool weeks show low probabilities of experiencing high-end suicide totals while warmer weeks are more likely to experience high-end suicide totals. This result is consistent for Toronto and Jackson. Weekly suicide totals demonstrate a sufficient association with temperature anomalies to allow some prediction of weeks with or without increased suicide frequency. While this finding alone is unlikely to have immediate clinical implications, these results are an important step toward clarifying the biopsychosocial mechanisms of suicidal behavior through a more nuanced understanding of the relationship between temperature and suicide

Confronting Standard Models of Proto--Planetary Disks With New Mid--Infrared Sizes from the Keck Interferometer

We present near and mid-infrared interferometric observations made with the Keck Interferometer Nuller and near-contemporaneous spectro-photometry from the IRTF of 11 well known young stellar objects, several observed for the first time in these spectral and spatial resolution regimes. With AU-level spatial resolution, we first establish characteristic sizes of the infrared emission using a simple geometrical model consisting of a hot inner rim and mid-infrared disk emission. We find a high degree of correlation between the stellar luminosity and the mid-infrared disk sizes after using near-infrared data to remove the contribution from the inner rim. We then use a semi-analytical physical model to also find that the very widely used "star + inner dust rim + flared disk" class of models strongly fails to reproduce the SED and spatially-resolved mid-infrared data simultaneously; specifically a more compact source of mid-infrared emission is required than results from the standard flared disk model. We explore the viability of a modification to the model whereby a second dust rim containing smaller dust grains is added, and find that the two-rim model leads to significantly improved fits in most cases. This complexity is largely missed when carrying out SED modelling alone, although detailed silicate feature fitting by McClure et al. 2013 recently came to a similar conclusion. As has been suggested recently by Menu et al. 2015, the difficulty in predicting mid-infrared sizes from the SED alone might hint at "transition disk"-like gaps in the inner AU; however, the relatively high correlation found in our mid-infrared disk size vs. stellar luminosity relation favors layered disk morphologies and points to missing disk model ingredients instead

The HD 163296 Circumstellar Disk in Scattered Light: Evidence of Time-Variable Self-Shadowing

We present the first multicolor view of the scattered light disk of the Herbig Ae star HD 163296, based on coronagraphic observations from the Hubble Space Telescope Advanced Camera for Surveys (HST ACS). Radial profile fits of the surface brightness along the disk's semimajor axis indicate that the disk is not continuously flared, and extends to ~540 AU. The disk's color (V − I) = 1.1 at a radial distance of 3.5'' is redder than the observed stellar color (V − I) = 0.15. This red disk color might be indicative of either an evolution in the grain size distribution (i.e., grain growth) and/or composition, both of which would be consistent with the observed nonflared geometry of the outer disk. We also identify a single ansa morphological structure in our F435W ACS data, which is absent from earlier epoch F606W and F814W ACS data, but corresponds to one of the two ansae observed in archival HST Space Telescope Imaging Spectrograph (STIS) coronagraphic data. Following transformation to similar bandpasses, we find that the scattered light disk of HD 163296 is 1 mag arcsec^(−2) fainter at 3.5'' in the STIS data than in the ACS data. Moreover, variations are seen in (1) the visibility of the ansa(e) structures, (2) the relative surface brightness of the ansa(e) structures, and (3) the (known) intrinsic polarization of the system. These results indicate that the scattered light from the HD 163296 disk is variable. We speculate that the inner disk wall, which Sitko et al. suggests has a variable scale height as diagnosed by near-IR SED variability, induces variable self-shadowing of the outer disk. We further speculate that the observed surface brightness variability of the ansa(e) structures may indicate that the inner disk wall is azimuthally asymmetric