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https://www.readbyqxmd.com/read/30584674/left-atrial-isomerism-with-complex-cardiovascular-malformation-on-prenatal-ultrasound-and-vascular-cast
#1
An Peng, Yingjian Ye, Wei Feng, Jiaqi Zhang, Yu Wang, Jiabao Yin
Left atrial isomerism (LAI) is a rare type of heterotaxy syndrome1 . The most common anomalies of the fetuses with LAI are the absence of the hepatic segment of the inferior vena cava (IVC), the interrupted suprarenal IVC segment with enlarged azygos or hemiazygos vein continuation, and the azygos vein passing posterior of the aorta and running up to join the superior vena cava (SVC)2 . This article is protected by copyright. All rights reserved.
December 24, 2018: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/30559629/successful-palliation-via-kawashima-procedure-of-an-infant-with-heterotaxy-syndrome-and-left-atrial-isomerism
#2
Hannah Lively-Endicott, Diego A Lara
Background: Heterotaxy is a condition of abnormal lateralization of organs across the body's left-right axis, causing multiple congenital malformations. The anatomic manifestations of heterotaxy syndrome generally follow one of two patterns, referred to as right atrial isomerism (with two similar right atria and duplication of right-sided features of multiple organs) and left atrial isomerism (with two similar left atria and duplication of left-sided features of multiple organs). Cardiac surgical intervention for patients with heterotaxy syndrome depends on ventricular physiology and circulatory balance...
2018: Ochsner Journal
https://www.readbyqxmd.com/read/30525025/anticancer-activity-and-cisplatin-binding-ability-of-bis-quinoline-and-bis-isoquinoline-derived-pd-2-l-4-4-metallosupramolecular-cages
#3
Roan A S Vasdev, Lachlan F Gaudin, Dan Preston, Jackmil P Jogy, Gregory I Giles, James D Crowley
New bis- quinoline ( L q ) and bis- isoquinoline-based ( L iq ) ligands have been synthesized, along with their respective homoleptic [Pd2 ( L q or L iq )4 ]4+ cages ( C q and C iq ). The ligands and cages were characterized by 1 H, 13 C and diffusion ordered (DOSY) NMR spectroscopies, high resolution electrospray ionization mass spectrometry (HR-ESIMS) and in the case of the bis -quinoline cage, X-ray crystallography. The crystal structure of the C q architecture showed that the [Pd2 ( L q )4 ]4+ cage formed a twisted meso isomer where the [Pd( quinoline )4 ]2+ units at either end of the cage architecture adopt the opposite twists (left and right handed)...
2018: Frontiers in Chemistry
https://www.readbyqxmd.com/read/30382259/atmospheric-chemistry-of-z-cf-3-ch-double-bond-length-as-m-dash-chcl-products-and-mechanisms-of-the-cl-atom-oh-radical-and-o-3-reactions-and-role-of-e-z-isomerization
#4
Mads P Sulbaek Andersen, Theis I Sølling, Lene Løffler Andersen, Aleksandra Volkova, Dvien Hovanessian, Connor Britzman, Ole John Nielsen, Timothy J Wallington
The chemical mechanisms of the OH radical, Cl-atom and O3 initiated oxidation of (Z)-CF3CH[double bond, length as m-dash]CHCl were studied at 296 ± 1 K in 10-700 Torr air of N2/O2 diluent. Cl atoms add to the [double bond splayed left]C[double bond, length as m-dash]C[double bond splayed right] double bond: 12 ± 5% to the terminal carbon and 85 ± 5% to the central carbon. In 700 Torr of air the products are CF3CHClCHO, HCOCl, CF3COCl, CF3CHO, (E)-CF3CH[double bond, length as m-dash]CHCl, CF3C(O)CHCl2, and CF3CHClCOCl...
November 14, 2018: Physical Chemistry Chemical Physics: PCCP
https://www.readbyqxmd.com/read/30271012/transposition-of-the-great-arteries-a-laterality-defect-in-the-group-of-heterotaxy-syndromes-or-an-outflow-tract-malformation
#5
Rana S Al-Zahrani, Samaher H Alharbi, Rawan M A Tuwaijri, Bayan T Alzomaili, Alaa Althubaiti, Talat Mesud Yelbuz
Background/Aim: Transposition of the great arteries (TGA) is traditionally classified as a "conotruncal heart defect", implying that TGA evolves from abnormal development of the outflow tract (OFT) of the embryonic heart. However, recently published genetic data suggest that TGA may be linked to laterality gene defects rather than OFT gene defects. The aim of our study was to clarify whether there is any statistically significant link between TGA and clinically diagnosed laterality defects (heterotaxy)...
September 2018: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/30225482/gas-phase-ozonolysis-of-furans-methylfurans-and-dimethylfurans-in-the-atmosphere
#6
Mengke Li, Yuhong Liu, Liming Wang
Furan and its methyl substituents, as promising alternative and renewable fuels and feedstock in the chemical industry, could be emitted to the atmosphere in a large scale and be degraded there by their reactions with OH, O3, NO3, Cl, etc. In this study, we investigate the mechanism of gas-phase ozonolysis of furans using quantum chemistry and kinetic calculations. The predicted rate coefficients agree well with the available experimental values for furan, 3-methylfuran (3-MF), and 2,5-dimethylfuran (2,5-DMF), suggesting that their removal by ozonolysis could be comparable to their removal by OH radicals in the atmosphere, particularly for methylfurans...
October 3, 2018: Physical Chemistry Chemical Physics: PCCP
https://www.readbyqxmd.com/read/30221662/o-glcnac-modified-snap29-inhibits-autophagy-mediated-degradation-via-the-disturbed-snap29-stx17-vamp8-complex-and-exacerbates-myocardial-injury-in-type-i-diabetic-rats
#7
Lin Huang, Ping Yuan, Peng Yu, Qiling Kong, Zixuan Xu, Xia Yan, Yang Shen, Juesheng Yang, Rong Wan, Kui Hong, Yanhua Tang, Jinzhu Hu
The O‑linked β‑N‑acetylglucosamine (O‑GlcNAc) modification and autophagy are associated with diabetic myocardial injury, however, the molecular mechanisms between the two processes remain to be fully elucidated. The purpose of the present study was to elucidate the molecular regulation of autophagy by O‑GlcNAc‑modified synaptosomal‑associated protein 29 (SNAP29) in diabetic myocardial injury. A rat model of type I diabetes was established via intraperitoneal injection of streptozotocin (STZ; 55 mg/kg)...
September 7, 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/30121866/prenatal-diagnosis-of-single-ventricle-physiology-impacts-on-cardiac-morbidity-and-mortality
#8
Roland W Weber, Brian Stiasny, Beate Ruecker, Margrit Fasnacht, Anna Cavigelli-Brunner, Emanuela R Valsangiacomo Buechel
We sought to evaluate the impact of prenatal diagnosis on morbidity and mortality in single ventricle (SV) lesions. All consecutive patients with pre- or postnatally diagnosed SV physiology admitted to our centre between January 2001 and June 2013 were reviewed. Primary endpoints included survival until 30 days after bidirectional cavopulmonary connection (BCPC) without transplant or BCPC takedown. Prenatal diagnosis was performed in 160 of 259 cases (62%). After excluding all cases with termination of pregnancy, intrauterine demise or treated with comfort care, a total of 180 neonates were admitted to our centre for treatment, including 87 with a prenatal and 93 with a postnatal diagnosis...
August 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/30049892/heterotaxy-syndrome-and-intestinal-rotation-abnormalities
#9
Lindsay M Ryerson, Scott Pharis, Charissa Pockett, Reeni Soni, Deborah Fruitman, Kristine J Guleserian, Melissa Nater, Stephen C Raynor, Andrew S Mackie, Bryan Dicken
BACKGROUND: Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a cohort of infants with HS and determine the incidence and natural history of IRA. METHODS: Infants ≤6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery...
August 2018: Pediatrics
https://www.readbyqxmd.com/read/30041775/long-term-survival-and-phenotypic-spectrum-in-heterotaxy-syndrome-a-25-year-follow-up-experience
#10
Anwar Baban, Nicoletta Cantarutti, Rachele Adorisio, Roberta Lombardi, Giulio Calcagni, Eva Piano Mortari, Bruno Dallapiccola, Bruno Marino, Fiore Salvatore Iorio, Rita Carsetti, Maria Cristina Digilio, Salvatore Giannico, Fabrizio Drago, Adriano Carotti
BACKGROUND: Heterotaxy syndrome (HS) is a group of congenital disorders characterized by abnormal arrangement of thoraco-abdominal organs across the left-right axis of the body, classified as right (RAI) and left atrial isomerism (LAI)·We investigated the long-term survival and phenotypic spectrum in our HS cohort. Results are compared to literature data. METHODS: This is a single centre, observational, both retro and prospective study. Cardiac features, surgical management and abdominal ultrasound (US) of all HS patients were reviewed or investigated if missing...
October 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29992973/a-requirement-for-zic2-in-the-regulation-of-nodal-expression-underlies-the-establishment-of-left-sided-identity
#11
Iain M Dykes, Dorota Szumska, Linta Kuncheria, Rathi Puliyadi, Chiann-Mun Chen, Costis Papanayotou, Helen Lockstone, Christèle Dubourg, Véronique David, Jurgen E Schneider, Thomas M Keane, David J Adams, Steve D M Brown, Sandra Mercier, Sylvie Odent, Jérôme Collignon, Shoumo Bhattacharya
ZIC2 mutation is known to cause holoprosencephaly (HPE). A subset of ZIC2 HPE probands harbour cardiovascular and visceral anomalies suggestive of laterality defects. 3D-imaging of novel mouse Zic2 mutants uncovers, in addition to HPE, laterality defects in lungs, heart, vasculature and viscera. A strong bias towards right isomerism indicates a failure to establish left identity in the lateral plate mesoderm (LPM), a phenotype that cannot be explained simply by the defective ciliogenesis previously noted in Zic2 mutants...
July 11, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29968369/reoperation-for-left-atrioventricular-valve-failure-in-repaired-atrioventricular-septal-defect-can-more-valves-be-preserved-in-the-current-era
#12
Tommaso Generali, Shady El Sayed, Vinay Rao, Carlos Pardo, Stefano Congiu, Osama Jaber, Carin van Doorn
OBJECTIVE: Left atrio-ventricular valve (LAVV) regurgitation after repair of an atrio-ventricular septal defect (AVSD) may necessitate further surgery. However, redo-LAVV repair remains challenging. We sought to determine if more LAVV valves are preserved in the current era, and analyze early and longer-term results. PATIENTS: All consecutive patients with repaired AVSD who underwent redo-LAVV surgery from January 2004 to April 2017 were included. Patients with single ventricles, atrial isomerism, and complex associated anomalies were excluded...
August 2018: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/29954599/effect-of-prenatal-laterality-disturbance-and-its-accompanying-anomalies-on-survival
#13
Trisha V Vigneswaran, Caroline B Jones, Vita Zidere, Marietta Charakida, Owen I Miller, John M Simpson, Gurleen K Sharland
In this retrospective, observational study of fetuses diagnosed with a laterality disturbance we describe the findings and outcome of fetuses diagnosed between 1980 and 2017 at a tertiary fetal-pediatric cardiology unit. In addition we sought to identify features which impact on outcome. Left atrial isomerism (LAI) was diagnosed in 177 babies and right atrial isomerism (RAI) in 100. Major structural heart disease was present in all cases of RAI and 91% with LAI. Complete heart block (CHB) was present in 40% of LAI...
August 15, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29899961/azobenzene-as-a-photoregulator-covalently-attached-to-rna-a-quantum-mechanics-molecular-mechanics-surface-hopping-dynamics-study
#14
Padmabati Mondal, Giovanni Granucci, Dominique Rastädter, Maurizio Persico, Irene Burghardt
The photoregulation of nucleic acids by azobenzene photoswitches has recently attracted considerable interest in the context of emerging biotechnological applications. To understand the mechanism of photoinduced isomerisation and conformational control in these complex biological environments, we employ a Quantum Mechanics/Molecular Mechanics (QM/MM) approach in conjunction with nonadiabatic Surface Hopping (SH) dynamics. Two representative RNA-azobenzene complexes are investigated, both of which contain the azobenzene chromophore covalently attached to an RNA double strand via a β-deoxyribose linker...
May 28, 2018: Chemical Science
https://www.readbyqxmd.com/read/29779315/-improving-outcomes-of-patients-with-heterotaxy-and-functional-single-ventricle-a-10-year-follow-up-of-70-cases-in-a-single-institution
#15
F Cao, M H Zou, L Ma, Y S Xia, S C Yang, W D Chen, G D Huang, X X Chen
Objective: To review current-era palliation outcomes of patients with heterotaxy and functional single ventricle in a single institution. Methods: The clinical data of 70 consecutive patients with heterotaxy undergoing multistage single ventricle palliation in Guangzhou Women and Children's Medical Center from January 2008 to December 2017 were retrospectively reviewed, and the prognosis factors for mortality were analyzed. There were 53 male and 17 female patients.The median age was 13.3 months (range: 6 days to 150 months)...
May 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29775603/genetic-and-extracardiac-anomalies-are-associated-with-inferior-single-ventricle-palliation-outcomes
#16
Bahaaldin Alsoufi, Courtney McCracken, Matthew Oster, Subhadra Shashidharan, Kirk Kanter
BACKGROUND: We examined the effect of genetic syndromes and extracardiac (GS/EC) anomalies on single-ventricle (SV) palliation with focus on hospital and interstage death and progression toward subsequent palliation stages. METHODS: First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53%), shunt in 173 (33%), and band in 73 (14%). Outcomes were compared between those with GS/EC anomalies (121 [23%]) and without GS/EC anomalies (409 [77%])...
October 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29571748/structural-basis-of-the-signal-transduction-via-transmembrane-domain-of-the-human-growth-hormone-receptor
#17
Eduard V Bocharov, Dmitry M Lesovoy, Olga V Bocharova, Anatoly S Urban, Konstantin V Pavlov, Pavel E Volynsky, Roman G Efremov, Alexander S Arseniev
BACKGROUND: Prior studies of the human growth hormone receptor (GHR) revealed a distinct role of spatial rearrangements of its dimeric transmembrane domain in signal transduction across membrane. Detailed structural information obtained in the present study allowed elucidating the bases of such rearrangement and provided novel insights into receptor functioning. METHODS: We investigated the dimerization of recombinant TMD fragment GHR254-294 by means of high-resolution NMR in DPC micelles and molecular dynamics in explicit POPC membrane...
June 2018: Biochimica et biophysica acta. General subjects
https://www.readbyqxmd.com/read/29552537/primary-ciliary-dyskinesia-and-situs-ambiguus-a-rare-association
#18
Kumar Narahari Narahari, Anu Kapoor, Sarat Kumar Sanamandra, Surya Ramachandra Varma Gunturi
Primary ciliary dyskinesia (PCD) is a rare disorder with impaired ciliary function resulting in a spectrum of clinical manifestations of varying severity. PCD affects approximately one in every 20,000 individuals with a reported prevalence between 1:4000 and 1:50,000. Due to its nonspecific clinical features, the condition is usually diagnosed late in its course, unless situs inversus (SI) or organ laterality defects are discovered at imaging. A small subset of patients with PCD display associated organ laterality defects, different from the classical SI totalis...
January 2018: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/29507233/chirality-controlled-spontaneous-twisting-of-crystals-due-to-thermal-topochemical-reaction
#19
Rishika Rai, Baiju P Krishnan, Kana M Sureshan
Crystals that show mechanical response against various stimuli are of great interest. These stimuli induce polymorphic transitions, isomerizations, or chemical reactions in the crystal and the strain generated between the daughter and parent domains is transcribed into mechanical response. We observed that the crystals of modified dipeptide LL (N3 -l-Ala-l-Val-NHCH2 C≡CH) undergo spontaneous twisting to form right-handed twisted crystals not only at room temperature but also at 0 °C over time. Using various spectroscopic techniques, we have established that the twisting is due to the spontaneous topochemical azide-alkyne cycloaddition (TAAC) reaction at room temperature or lower temperatures...
March 20, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29479365/plastid-genome-evolution-in-the-early-diverging-legume-subfamily-cercidoideae-fabaceae
#20
Yin-Huan Wang, Susann Wicke, Hong Wang, Jian-Jun Jin, Si-Yun Chen, Shu-Dong Zhang, De-Zhu Li, Ting-Shuang Yi
The subfamily Cercidoideae is an early-branching legume lineage, which consists of 13 genera distributed in the tropical and warm temperate Northern Hemisphere. A previous study detected two plastid genomic variations in this subfamily, but the limited taxon sampling left the overall plastid genome (plastome) diversification across the subfamily unaddressed, and phylogenetic relationships within this clade remained unresolved. Here, we assembled eight plastomes from seven Cercidoideae genera and conducted phylogenomic-comparative analyses in a broad evolutionary framework across legumes...
2018: Frontiers in Plant Science
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