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https://www.readbyqxmd.com/read/28098235/molecular-characterization-of-pediatric-restrictive-cardiomyopathy-from-integrative-genomics
#1
Tara N Rindler, Robert B Hinton, Nathan Salomonis, Stephanie M Ware
Pediatric restrictive cardiomyopathy (RCM) is a genetically heterogeneous heart disease with limited therapeutic options. RCM cases are largely idiopathic; however, even within families with a known genetic cause for cardiomyopathy, there is striking variability in disease severity. Although accumulating evidence implicates both gene expression and alternative splicing in development of dilated cardiomyopathy (DCM), there have been no detailed molecular characterizations of underlying pathways dysregulated in RCM...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28080919/restrictive-cardiomyopathy-and-secondary-congestive-heart-failure-in-a-mcdowell-s-carpet-python-morelia-spilota-mcdowelli
#2
Lionel Schilliger, Valérie Chetboul, Cécile Damoiseaux, Alexandra Nicolier
Echocardiography is an established and noninvasive diagnostic tool used in herpetologic cardiology. Various cardiac lesions have been previously described in reptiles with the exception of restrictive cardiomyopathy. In this case report, restrictive cardiomyopathy and congestive heart failure associated with left atrial and sinus venosus dilation were diagnosed in a 2-yr-old captive lethargic McDowell's carpet python ( Morelia spilota mcdowelli), based on echocardiographic, Doppler, and histopathologic examinations...
December 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/28072629/the-key-clues-to-reach-the-diagnosis-of-loeffler-endomyocardial-fibrosis-associated-with-eosinophilic-granulomatosis-with-polyangiitis
#3
Enrico Ammirati, Domenico Sirico, Linda Brevetti, Laura Scudiero, Diana Artioli, Patrizia Pedrotti, Maria Frigerio
Loeffler endomyocardial fibrosis is a rare restrictive cardiomyopathy due to chronic eosinophilic exposure (Loeffler endomyocarditis). Loeffler endomyocarditis is associated with different eosinophilic disorders, including eosinophilic granulomatosis with polyangiitis (EGPA). These images recapitulate all typical findings that can be observed in patients presenting with heart failure attributable to this specific cardiomyopathy associated with EGPA, a systemic medical condition that can be easily detectable just putting together different diagnostic elements...
January 7, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28068669/kinetically-e-selective-macrocyclic-ring-closing-metathesis
#4
Xiao Shen, Thach T Nguyen, Ming Joo Koh, Dongmin Xu, Alexander W H Speed, Richard R Schrock, Amir H Hoveyda
Macrocyclic compounds are central to the development of new drugs, but preparing them can be challenging because of the energy barrier that must be surmounted in order to bring together and fuse the two ends of an acyclic precursor such as an alkene (also known as an olefin). To this end, the catalytic process known as ring-closing metathesis (RCM) has allowed access to countless biologically active macrocyclic organic molecules, even for large-scale production. Stereoselectivity is often critical in such cases: the potency of a macrocyclic compound can depend on the stereochemistry of its alkene; alternatively, one isomer of the compound can be subjected to stereoselective modification (such as dihydroxylation)...
January 19, 2017: Nature
https://www.readbyqxmd.com/read/28051301/molecular-barrel-by-a-hooping-strategy-synthesis-structure-and-selective-co2-adsorption-facilitated-by-lone-pair-%C3%AF-interactions
#5
Qi-Qiang Wang, Na Luo, Xu-Dong Wang, Yu-Fei Ao, Yi-Fan Chen, Jun-Min Liu, Cheng-Yong Su, De-Xian Wang, Mei-Xiang Wang
A sophisticated molecular barrel 5 was efficiently constructed by hooping a 63-membered loop around a D3h-symmetric, shape-persistent bis(tetraoxacalix[2]arene[2]triazine) core. The hooping strategy involved 3-fold ring-closing metathesis (RCM) reactions of six branched olefin arms which were preanchored on the inner core. Through hooping, the loop tightens the cage structure and significantly enhances its stability toward nucleophilic decomposition. The X-ray crystal structure showed the molecular barrel bears three enclosed fan-shaped cavities as divided by the triazine rings and each of the cavities can hold a solvent CHCl3 or CH2Cl2 molecule...
January 18, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28050425/severe-left-ventricular-endomyocardial-fibrosis-presenting-as-biventricular-failure-in-a-young-adult-a-case-report
#6
Harpreet Singh Sandhu, Sampathkumar Mahadevappa Mahendrakar, Rajebali Ramzanali Pethani, Azizullah Hafizullah Khan, Yunus Shafi Loya
Endomyocardial Fibrosis (EMF) is a form of progressive restrictive cardiomyopathy of unclear aetiology prevalent in areas within 15(0) of equator including coastal areas of Kerala a few decades back. It inflicts young adults and carries a poor prognosis due to limited options for treatment. Fortunately, the incidence of cases is now declining due to improvement in health and hygiene standards. Here, we review the aetiology and pathogenesis of EMF and report a case of a young male from Mumbai (non-endemic area) presenting with progressively worsening breathlessness and signs of heart failure unresponsive to conventional medical treatment...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28048291/su-f-i-01-normalized-mean-glandular-dose-values-for-dedicated-breast-ct-using-realistic-breast-shaped-phantoms
#7
A Hernandez, J Boone
PURPOSE: To estimate normalized mean glandular dose values for dedicated breast CT (DgN-CT) using breast CT-derived phantoms and compare to estimations using cylindrical phantoms. METHODS: Segmented breast CT (bCT) volume data sets (N=219) were used to measure effective diameter profiles and were grouped into quintiles by volume. The profiles were averaged within each quintile to represent the range of breast sizes found clinically. These profiles were then used to generate five voxelized computational phantoms (V1, V2, V3, V4, V5 for the small to large phantom sizes, respectively), and loaded into the MCNP6 lattice geometry to simulate normalized mean glandular dose coefficients (DgN-CT) using the system specifications of the Doheny-prototype bCT scanner in our laboratory...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28042283/synthesis-of-a-ribose-incorporating-medium-ring-scaffold-via-a-challenging-ring-closing-metathesis-reaction
#8
Stuart S Rankin, John J Caldwell, Nora B Cronin, Rob L M van Montfort, Ian Collins
A practical synthesis of a novel oxabicyclo[6.2.1]undecenetriol useful as a medicinal chemistry scaffold has been developed starting from l-ribose. The sequence involves an oxidation/Grignard addition sequence and a challenging ring-closing metathesis (RCM) reaction as the ring forming step. Exploration of the RCM substrate protecting groups revealed the key factor for successful nine-membered medium ring formation to be conformational bias of the reacting alkenes of the RCM substrate by very bulky silyl ether protecting groups...
September 2016: European Journal of Organic Chemistry
https://www.readbyqxmd.com/read/28040227/vulnerability-and-impact-assessment-of-extreme-climatic-event-a-case-study-of-southern-punjab-pakistan
#9
Abdul Qayyum Aslam, Sajid R Ahmad, Iftikhar Ahmad, Yawar Hussain, Muhammad Sameem Hussain
Understanding of frequency, severity, damages and adaptation costs of climate extremes is crucial to manage their aftermath. Evaluation of PRECIS RCM modelled data under IPCC scenarios in Southern Punjab reveals that monthly mean temperature is 30°C under A2 scenario, 2.4°C higher than A1B which is 27.6°C in defined time lapses. Monthly mean precipitation under A2 scenario ranges from 12 to 15mm and for A1B scenario it ranges from 15 to 19mm. Frequency modelling of floods and droughts via poisson distribution shows increasing trend in upcoming decades posing serious impacts on agriculture and livestock, food security, water resources, public health and economic status...
December 28, 2016: Science of the Total Environment
https://www.readbyqxmd.com/read/28034292/the-effect-of-pullulan-on-the-growth-and-acidifying-activity-of-selected-stool-microflora-of-human
#10
Anna Chlebowska-Śmigiel, Małgorzata Gniewosz, Marek Kieliszek, Anna Bzducha-Wróbel
BACKGROUND: Pullulan is a microbial polysaccharide of low energy value, which can component of low-calorie foods and in dietary snacks for diabetics. The objective of the study was to determine the effect of pullulan on the growth and fermentation activity of selected human intestinal bacteria. METHODS: Commercial pullulan purchased from Focubase (China) of a molecular weight of 100,000 Da constituted as experimental material. Food grade pullulan 99% purity. Two control media were prepared: the first standard RCM composed of (g/100 ml): 0...
December 29, 2016: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28031081/restrictive-cardiomyopathy-resulting-from-a-troponin-i-type-3-mutation-in-a-chinese-family
#11
Yan-Ping Ruan, Chao-Xia Lu, Xiao-Yi Zhao, Rui-Juan Liang, Hui Lian, Michael Routledge, Wei Wu, Xue Zhang, Zhong-Jie Fan
Objective To identify the pathogenic variant responsible for restrictive cardiomyopathy (RCM) in a Chinese family.Methods Next generation sequencing was used for detecting the mutation and Results verified by sequencing. We used restriction enzyme digestion to test the mutation in the family members and 200 unrelated normal subjects without any cardiac inherited diseases when the mutation was identified.Results Five individuals died from cardiac diseases, two of whom suffered from sudden cardiac death. Two individuals have suffered from chronic cardiac disorders...
March 20, 2016: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/28007674/the-current-role-of-in-vivo-reflectance-confocal-microscopy-within-the-continuum-of-actinic-keratosis-and-squamous-cell-carcinoma-a-systematic-review
#12
Kim P Nguyen, Malou Peppelman, Lisa Hoogedoorn, Piet E J Van Erp, Marie-Jeanne P Gerritsen
BACKGROUND: Clinical differentiation between actinic keratosis (AK), squamous cell carcinoma (SCC) in situ, and invasive SCC and its variants may be difficult. Reflectance confocal microscopy (RCM) is a non-invasive technique for in vivo skin imaging. OBJECTIVES: To explicate the diagnostic and monitoring use of RCM within the spectrum of AK and SCC, and evaluate the accuracy of RCM for these diagnoses relative to histopathology. MATERIALS & METHODS: A systematic literature search was performed in PubMed, EMBASE, the Cochrane Library, and Web of Science databases...
December 1, 2016: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28005890/coherent-artifact-suppression-in-line-field-reflection-confocal-microscopy-using-a-low-spatial-coherence-light-source
#13
Changgeng Liu, Hui Cao, Michael A Choma
Line-field reflection confocal microscopy (LF-RCM) has the potential to add a dimension of parallelization to traditional confocal microscopy while reducing the need for two-axis beam scanning. LF-RCM systems often employ light sources with a high degree of spatial coherence. This high degree of spatial coherence potentially leads to unwanted coherent artifact in the setting of nontrivial sample scattering. Here, we (a) confirm that a coherent artifact is a nontrivial problem in LF-RCM when using spatially coherent light, and (b) demonstrate that such a coherent artifact can be mitigated through the use of reduced spatial coherence line-field sources...
October 15, 2016: Optics Letters
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#14
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27989285/my-approach-to-the-evaluation-of-restrictive-cardiomyopathy
#15
Craig R Asher, Allan L Klein
No abstract text is available yet for this article.
January 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27988125/investigating-rennet-coagulation-properties-of-recombined-highly-concentrated-micellar-casein-concentrate-and-cream-for-use-in-cheese-making
#16
Y Lu, D J McMahon, A H Vollmer
Highly concentrated micellar casein concentrate (HC-MCC) contains ∼18% casein with ∼70% of whey proteins removed by microfiltration and diafiltration of skim milk, followed by vacuum evaporation for further concentration. When blended with cream, HC-MCC forms recombined concentrated milk (RCM), which could be used as a starting material in cheese making. Our objective was to investigate the rennet coagulation properties of RCM while varying parameters such as casein level, pH, rennet level, and coagulation temperature...
December 14, 2016: Journal of Dairy Science
https://www.readbyqxmd.com/read/27987486/piperlongumine-derived-cyclic-sulfonamides-sultams-synthesis-and-in%C3%A2-vitro-exploration-for-therapeutic-potential-against-hela-cancer-cell-lines
#17
Nitin P Lad, Sarang Kulkarni, Rajiv Sharma, Malcolm Mascarenhas, Mahesh R Kulkarni, Shivaji S Pandit
A novel modification of piperlongumine is designed, bearing a cyclic sulphonamide (sultam) and its synthesis is described. For the first time herein we report the synthesis and biological evaluation of the natural product derived cyclic sulfonamides using Grubbs second generation catalyst (Grubbs II) via ring closing metathesis approach. Synthesis of a series of piperlongumine derived sultams is done in a moderate to good yield using Wittig reaction, Ring-Closing Metathesis (RCM) and, amide synthesis by using mixed anhydride, approach...
December 10, 2016: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/27977563/pathways-from-socioeconomic-status-to-prenatal-smoking-a-test-of-the-reserve-capacity-model
#18
Irene Yang, Lynne A Hall, Kristin Ashford, Sudeshna Paul, Barbara Polivka, S Lee Ridner
BACKGROUND: Low-income, unemployed women with low levels of education are more likely to smoke during pregnancy compared to their higher-income, employed, and well-educated counterparts. The reserve capacity model (RCM) offers a theoretical framework to explain how psychosocial factors may serve as pathways connecting socioeconomic status (SES) to health behaviors. Research supports the link between prenatal smoking and several psychosocial variables such as chronic stressors, depressive symptoms, and social support...
January 2017: Nursing Research
https://www.readbyqxmd.com/read/27976437/monitoring-structural-changes-in-demodex-mites-under-topical-ivermectin-in-rosacea-by-means-of-reflectance-confocal-microscopy-a-case-series
#19
LETTER
C Ruini, E Sattler, D Hartmann, M Reinholz, T Ruzicka, T von Braunmühl
No abstract text is available yet for this article.
December 15, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27975052/histopathological-evaluation-of-contrast-induced-acute-kidney-injury-rodent-models
#20
REVIEW
Norbert Kiss, Péter Hamar
Contrast-induced acute kidney injury (CI-AKI) can occur in 3-25% of patients receiving radiocontrast material (RCM) despite appropriate preventive measures. Often patients with an atherosclerotic vasculature have to receive large doses of RCM. Thus, animal studies to uncover the exact pathomechanism of CI-AKI are needed. Sensitive and specific histologic end-points are lacking; thus in the present review we summarize the histologic appearance of different rodent models of CI-AKI. Single injection of RCM causes overt renal damage only in rabbits...
2016: BioMed Research International
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