143 research outputs found
Major coronary artery anomalies in a pediatric population: incidence and clinical importance
AbstractOBJECTIVESWe sought to prospectively determine the incidence and clinical significance of major coronary artery anomalies in asymptomatic children using transthoracic two-dimensional echocardiography.BACKGROUNDAnomalous origins of the left main coronary artery (ALMCA) from the right sinus of Valsalva or anomalous origins the right coronary artery (ARCA) from the left sinus are rarely diagnosed in children and can cause sudden death, especially in young athletes. Because most patients are asymptomatic, the diagnosis is often made post mortem. No study to date has prospectively identified anomalous coronary arteries in asymptomatic children in the general population.METHODSAfter serendipitously identifying an index case with ALMCA, we examined proximal coronary artery anatomy in children with otherwise anatomically normal hearts who were referred for echocardiography. In those diagnosed with ALMCA or ARCA, we performed further tests.RESULTSWithin a three-year period, echocardiograms were obtained in 2,388 children and adolescents. Four children (0.17%) were identified with anomalous origin of their coronary arteries, and angiograms, exercise perfusion studies and/or stress tests were then performed. One ARCA patient had decreased perfusion in the right coronary artery (RCA) perfusion area and showed ventricular ectopy on electrocardiogram (ECG) at rest that diminished but did not resolve with exercise. A second patient with ALMCA had atrial tachycardia immediately after exercise, with inferior and lateral ischemic changes on ECG and frequent junctional and/or ventricular premature complexes both at rest and recovery.CONCLUSIONSThis study demonstrates that although anomalous origins of coronary arteries are rare in asymptomatic children, the prevalence is greater than that found in other prospective studies. Ischemia can occur with both ALMCA and ARCA even though patients remain asymptomatic. Because of the high risk of sudden cardiac death, aggressive surgical management and close follow-up are necessary
Does malnutrition influence outcome in children undergoing congenital heart surgery in a developing country?
Background Most children undergoing cardiac surgery for
congenital heart disease (CHD) in developing countries are
malnourished. Malnutrition is known as a co-morbidity factor
that might predict and influence outcomes after surgery.
Objectives To evaluate the effect of malnutrition and other
associated risk factors on post-operative outcomes in children
with CHDs underwent cardiac surgery.
Methods We conducted a retrospective cohort study in a single
center tertiary pediatric cardiac intensive care unit (PCICU)
in Indonesia. Our cohort included all children between 5 and
36 months of age undergoing congenital heart surgery with
cardiopulmonary bypass from November 2011 until February
2014. Outcomes measured were the length of intubation and the
length of ICU stay. Variables for potential influence investigated
were the nutritional status, age, gender, type of cardiac anomaly
(acyanotic vs. cyanotic), Aristotle score, cardiopulmonary bypass
time, aortic cross-clamp time, and Pediatric Risk of Mortality
(PRISM) III score.
Results Out of 249 patients included, 147 (59%) showed
malnourishment on admission. Malnourished patients were
significantly younger in age, presented with an acyanotic heart
defects, and had higher PRISM III score. Additionally, they also
had a longer mechanical ventilation time and ICU stay than
those with a normal nutritional status. After adjusting for various
variables using a multiple logistic regression model it could be
demonstrated that a higher Z-score for weight to age was a
significant protective factor for the intubation time of more than
29 hours with an odds ratio of 0.66 (95% CI 0.48 to 0.92, P =
0.012). Non-malnourished patients had a 49% significantly higher
chance for extubation with a hazard ratio of 1.49 (95% CI 1.12
to 1.99, P= 0.007).
Conclusion Malnourishment is clearly associated in a linear
fashion with longer mechanical ventilation and ICU stay. As one of
significant and potentially treatable co-morbidity factors, prevention
of malnourishment by early diagnosis and optimal timing for surgery
is important
MuRF1 mono-ubiquitinates TRα to inhibit T3-induced cardiac hypertrophy in vivo
Thyroid hormone (TH) is recognized for its role in cellular metabolism and growth and participates in homeostasis of the heart. T3 activates pro-survival pathways including Akt and mTOR. Treatment with T3 after myocardial infarction is cardioprotective and promotes elements of physiological hypertrophic response after cardiac injury. Although T3 is known to benefit the heart, very little about its regulation at the molecular level has been described to date. The ubiquitin proteasome system (UPS) regulates nuclear hormone receptors such as estrogen, progesterone, androgen, and glucocorticoid receptors by both degradatory and non-degradatory mechanisms. However, how the UPS regulates T3-mediated activity is not well understood. In this study, we aim to determine the role of the muscle-specific ubiquitin ligase muscle ring finger-1 (MuRF1) in regulating T3-induced cardiomyocyte growth. An increase in MuRF1 expression inhibits T3-induced physiological cardiac hypertrophy, whereas a decrease in MuRF1 expression enhances T3’s activity both in vitro and in cardiomyocytes in vivo. MuRF1 interacts directly with TRα to inhibit its activity by posttranslational ubiquitination in a non-canonical manner. We then demonstrated that a nuclear localization apparatus that regulates/inhibits nuclear receptors by sequestering them within a subcompartment of the nucleus was necessary for MuRF1 to inhibit T3 activity. This work implicates a novel mechanism that enhances the beneficial T3 activity specifically within the heart, thereby offering a potential target to enhance cardiac T3 activity in an organ-specific manner
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Development and Utility of Quality Metrics for Ambulatory Pediatric Cardiology in Kawasaki Disease.
The Adult Congenital and Pediatric Cardiology (ACPC) Section of the American College of Cardiology sought to develop quality indicators/metrics for ambulatory pediatric cardiology practice. The objective of this study was to report the creation of metrics for patients with Kawasaki disease. Over a period of 5 months, 12 pediatric cardiologists developed 24 quality metrics based on the most relevant statements, guidelines, and research studies pertaining to Kawasaki disease. Of the 24 metrics, the 8 metrics deemed the most important, feasible, and valid were sent on to the ACPC for consideration. Seven of the 8 metrics were approved using the RAND method by an expert panel. All 7 metrics approved by the ACPC council were accepted by ACPC membership after an "open comments" process. They have been disseminated to the pediatric cardiology community for implementation by the ACPC Quality Network
Kawasaki Disease Shock Syndrome vs Classical Kawasaki Disease: A Meta-analysis and Comparison With SARS-CoV-2 Multisystem Inflammatory Syndrome.
BACKGROUND: The emergence of increasing reports worldwide of a severe inflammatory process and shock in pediatric patients resembling Kawasaki disease (KD) and more specifically Kawasaki disease shock syndrome (KDSS), prompted us to explore KDSS in a preamble of a systematic comparison between the two conditions. METHODS: We completed a systematic review of KDSS and performed a meta-analysis comparison between reported KDSS cases and KD controls. RESULTS: A total of ten case-control series were included in the meta-analysis. KDSS patients were older (38.4 ± 30.6 vs. 21.9±19.5 months; P<0.001) compared to standard KD with equal sex distribution and completeness of clinical diagnostic criteria. KDSS present higher CRP (59.4±29.2 mg/dL vs. 20.8±14.8 mg/dL; p<0.001), lower albumin (2.7±0.5 g/dL vs. 3.3±0.5 g/dL; p<0.01), and lower platelets (255±149 109/L vs. 394±132 109/L; p<0.001) but only borderline higher WBC's (p=0.06). Differences in ALT, AST and ESR were non-significant. The odds of IVIG resistance (44.4% vs. 9.6%; (p<0.001) and the hospital length of stay (10.9±5.8 vs. 5.0±3.0 days; p<0.001) were higher in KDSS as were the odds of coronary artery abnormalities (33.9% vs. 8.6%; p<0.001). CONCLUSION: This first meta-analysis on KDSS versus KD represents a basis for future works on KDSS and opens the opportunity for future multicenter studies in the search of causal relationships between presenting elements and the eventual complications of KDSS. The similarities between SARS-CoV-2 multisystem inflammatory syndrome in children (MIS-C) and KDSS open new horizons to the understanding of the etiology and pathophysiology related to KDSS
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<i>De Novo</i> and Rare Variants at Multiple Loci Support the Oligogenic Origins of Atrioventricular Septal Heart Defects
Congenital heart disease (CHD) has a complex genetic etiology, and recent studies suggest that high penetrance de novo mutations may account for only a small fraction of disease. In a multi-institutional cohort surveyed by exome sequencing, combining analysis of 987 individuals (discovery cohort of 59 affected trios and 59 control trios, and a replication cohort of 100 affected singletons and 533 unaffected singletons) we observe variation at novel and known loci related to a specific cardiac malformation the atrioventricular septal defect (AVSD). In a primary analysis, by combining developmental coexpression networks with inheritance modeling, we identify a de novo mutation in the DNA binding domain of NR1D2 (p.R175W). We show that p.R175W changes the transcriptional activity of Nr1d2 using an in vitro transactivation model in HUVEC cells. Finally, we demonstrate previously unrecognized cardiovascular malformations in the Nr1d2tm1-Dgen knockout mouse. In secondary analyses we map genetic variation to protein-interaction networks suggesting a role for two collagen genes in AVSD, which we corroborate by burden testing in a second replication cohort of 100 AVSDs and 533 controls (p = 8.37e-08). Finally, we apply a rare-disease inheritance model to identify variation in genes previously associated with CHD (ZFPM2, NSD1, NOTCH1, VCAN, and MYH6), cardiac malformations in mouse models (ADAM17, CHRD, IFT140, PTPRJ, RYR1 and ATE1), and hypomorphic alleles of genes causing syndromic CHD (EHMT1, SRCAP, BBS2, NOTCH2, and KMT2D) in 14 of 59 trios, greatly exceeding variation in control trios without CHD (p = 9.60e-06). In total, 32% of trios carried at least one putatively disease-associated variant across 19 loci,suggesting that inherited and de novo variation across a heterogeneous group of loci may contribute to disease risk
The Energy Landscape, Folding Pathways and the Kinetics of a Knotted Protein
The folding pathway and rate coefficients of the folding of a knotted protein
are calculated for a potential energy function with minimal energetic
frustration. A kinetic transition network is constructed using the discrete
path sampling approach, and the resulting potential energy surface is
visualized by constructing disconnectivity graphs. Owing to topological
constraints, the low-lying portion of the landscape consists of three distinct
regions, corresponding to the native knotted state and to configurations where
either the N- or C-terminus is not yet folded into the knot. The fastest
folding pathways from denatured states exhibit early formation of the
N-terminus portion of the knot and a rate-determining step where the C-terminus
is incorporated. The low-lying minima with the N-terminus knotted and the
C-terminus free therefore constitute an off-pathway intermediate for this
model. The insertion of both the N- and C-termini into the knot occur late in
the folding process, creating large energy barriers that are the rate limiting
steps in the folding process. When compared to other protein folding proteins
of a similar length, this system folds over six orders of magnitude more
slowly.Comment: 19 page
Crop Updates 2009 - Genetically Modified Crops, Nutrition, Soils, & Others
This session covers fifteen papers from different authors:
1. Performance of Canola Breeders Roundup Ready® canola hybrid CHYB-166 in 2008, Wallace Cowling, Canola Breeders Western Australia Pty Ltd
2. The implications of GM glyphosate resistant lupin, Art Diggle, Caroline Peek, Frank D’Emden, Fiona Evans, Bob French, Rob Grima, Sam Harburg, Abul Hashem,, John Holmes, Jeremy Lemon, Peter Newman, Janet Paterson, Steve Penny,Department of Agriculture and Food, Peter Portmann, Agriconnect
3. Nufarm Roundup Ready® Canola Systems Trials— 2008 Mark Slatter, Research and Development Officer, Victoria, Nufarm (0438 064 845) Angus MacLennan, Business Development Manager, New South Wales, Nufarm (0408 358 024) Cooperators: Monsanto, Nuseed, Pacific Seeds, Pioneer Seeds
4. Roundup Ready® canola—2008 Limited Commercial Release. Getting the system right, Andrew Wells and Mark Slatter, Nufarm Australia Limited (Reprint from 2008 GRDC Cropping Updates with Introductory note)
NUTRITION
5. Fertilising in a changing price environment, Bill Bowden1, Wayne Pluske2 and Jeremy Lemon1, 1Department of Agriculture and Food, 2Back Paddock Company
6. Making better fertiliser for Western Australian cropping systems, Wen Chen1 2, Geoff Anderson1, Ross Brennan1and Richard Bell2 1Department of Agriculture and Food, 2School of Environmental Science, Murdoch University
7. The nitrogen fertiliser replacement value of biosolids from wastewater treatment, Hannah Rigby1, Deborah Pritchard1, David Collins1, Katrina Walton2, David Allen2 and Nancy Penney31School of Agriculture and Environment,Curtin University of Technology, Muresk Campus, 2Chemistry Centre of Western Australia 3Water Corporation of Western Australia
8. Fertilising to soil type (usually) pays, Michael Robertson, Bill Bowden and Roger Lawes, CSIRO, Floreat and Department of Agriculture and Food
SOILS
9. Management of subsoil acidity and compaction using a combination of lime, deep ripping and controlled traffic, Stephen Davies, Chris Gazey, Breanne Best and David Gartner, Department of Agriculture and Food
10. Optimising gypsum applications through remote sensing and Variable Rate Technology, Frank D’Emden, Department of Agriculture and Food and Quenten Knight,Precision Agronomics Australia
11. Case study of a 17 year agricultural lime trial, Chris Gazey1, Joel Andrew2and Ryan Pearce3 1Department of Agriculture and Food; 2Precision SoilTech; 3ConsultAg
12. Soil organic carbon in WA agricultural soils, FC Hoyle and A Bennett, Department of Agriculture and Food
OTHER
13. Is the no-till revolution complete in WA? Frank D’Emden1, Rick Llewellyn2 and Ken Flower3 1Department of Agriculture and Food, 2CSIRO Sustainable Ecosystems, 3University of Western Australia
14. Progression Planning (The Concept), Julian Krieg and Owen Catto, Wheatbelt Men’s Health
15. Is the Department of Agriculture and Food still a primary source of cropping information? Cindy Parsons, Department of Agriculture and Foo
Genome-Wide Analysis of Light- and Temperature-Entrained Circadian Transcripts in Caenorhabditis elegans
Transcriptional profiling experiments identify light- and temperature-entrained circadian transcripts in C. elegans
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