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Surfactant deficiency

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https://www.readbyqxmd.com/read/29749016/tempo-driven-mild-and-modular-route-to-functionalized-microparticles
#1
Sumbul Hafeez, Leonie Barner, Leena Nebhani
The synthesis of crosslinked polymeric microspheres (3.8-15.0 µm) via (2,2,6,6-tetramethylpiperidin-1-yl)oxyl (TEMPO) initiated thiol-ene dispersion polymerization under ambient conditions is reported for the first time. The initiating ability of TEMPO for the thiol-ene reaction is validated by electron paramagnetic resonance (EPR) and 1 H nuclear magnetic resonance (NMR) spectroscopy on model reactions between 1-octadecanethiol and two electron deficient enes, n-butylacrylate and divinyl sulfone. Critically, the TEMPO resonance observed in the EPR spectra decreases with time when TEMPO is mixed with thiol and an electron deficient ene...
May 11, 2018: Macromolecular Rapid Communications
https://www.readbyqxmd.com/read/29597031/clinical-technological-and-economic-issues-associated-with-developing-new-lung-surfactant-therapeutics
#2
REVIEW
Hyun Chang Kim, You-Yeon Won
Discovery of lung surfactant deficiency as a main cause of neonatal respiratory distress syndrome (NRDS) has influenced a steep increase in lung surfactant research. Although this has yielded impactful scientific discoveries, much of the basic research on lung surfactants has failed to translate into clinical practices. This is attributed to insufficient information covering the entire lung surfactant ecosystem, from the basic science to economics surrounding the development and clinical practices. In this manuscript, developments related to improving therapeutic lung surfactant as well as the degree of unmet need are analyzed from both technical and economic perspectives...
March 26, 2018: Biotechnology Advances
https://www.readbyqxmd.com/read/29566461/surfactant-deficiency-syndrome-in-an-infant-with-a-c-terminal-frame-shift-in-abca3-a-case-report
#3
Nour Akil, Anthony J Fischer
Deficiency in ATP binding cassette A3 (ABCA3) causes neonatal respiratory distress, hypoxemic respiratory failure, and interstitial lung disease. ABCA3 transports phospholipids into the lamellar bodies of type II alveolar cells, a critical step in alveolar surfactant production. We report a term infant with ABCA3 surfactant deficiency syndrome with the E292V (c.875A>T; p.Glu292Val) mutation in trans with a novel C-terminal frame shift mutation (c.4938delC; p.Met1647fs). This mutation removes the final 58 amino acids and substitutes 33 incorrect amino acids...
March 22, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29518647/vitamin-d-deficiency-and-respiratory-morbidity-among-african-american-very-low-birth-weight-infants
#4
S Nadya J Kazzi, Sreenivas Karnati, Subhash Puthuraya, Ronald Thomas
BACKGROUND: Very low birth weight infants (VLBWI) have unexplained variation in respiratory morbidity, including respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD). We examined a potential association to serum 25-hydroxyvitamin D (s-25OHD) on day one. STUDY DESIGN: Prospective, observational study on 89 VLBWI (≤1250 g). S-25OHD (day one and 21) and respiratory severity score (RSS) (day one) were examined. Other respiratory morbidities including BPD were compared between infants with s-25OHD ≤ 10 ng/ml (deficient) versus >10 ng/ml (adequate)...
April 2018: Early Human Development
https://www.readbyqxmd.com/read/29452569/up-regulation-of-sftpb-expression-and-attenuation-of-acute-lung-injury-by-pulmonary-epithelial-cell-specific-nampt-knockdown
#5
Guangliang Bi, Lei Wu, Peixin Huang, Shamima Islam, Daniel P Heruth, Li Qin Zhang, Ding-You Li, Venkatesh Sampath, Weimin Huang, Brett A Simon, Ronald Blaine Easley, Shui Qing Ye
Although a deficiency of surfactant protein B (SFTPB) has been associated with lung injury, SFTPB expression has not yet been linked with nicotinamide phosphoribosyltransferase (NAMPT), a potential biomarker of acute lung injury (ALI). The effects of Nampt in the pulmonary epithelial cell on both SFTPB expression and lung inflammation were investigated in a LPS-induced ALI mouse model. Pulmonary epithelial cell-specific knockdown of Nampt gene expression, achieved by the crossing of Nampt gene exon 2 floxed mice with mice expressing epithelial-specific transgene Cre or by the use of epithelial-specific expression of anti-Nampt antibody cDNA, significantly attenuated LPS-induced ALI...
February 8, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29377922/assessing-the-impacts-of-total-liquid-ventilation-on-left-ventricular-diastolic-function-in-a-model-of-neonatal-respiratory-distress-syndrome
#6
Michaël Sage, Mathieu Nadeau, Claudia Forand-Choinière, Julien Mousseau, Jonathan Vandamme, Claire Berger, Jean-Sébastien Tremblay-Roy, Renaud Tissier, Philippe Micheau, Étienne Fortin-Pellerin
BACKGROUND: Filling the lung with dense liquid perfluorocarbons during total liquid ventilation (TLV) might compress the myocardium, a plausible explanation for the instability occasionally reported with this technique. Our objective is to assess the impacts of TLV on the cardiovascular system, particularly left ventricular diastolic function, in an ovine model of neonatal respiratory distress syndrome. METHOD: Eight newborns lambs, 3.0 ± 0.4 days (3.2 ± 0.3kg) were used in this crossover experimental study...
2018: PloS One
https://www.readbyqxmd.com/read/29368450/frequency-dependent-airway-hyperresponsiveness-in-a-mouse-model-of-emphysema-and-allergic-inflammation
#7
Kentaro Tamura, Koichiro Matsumoto, Satoru Fukuyama, Keiko Kan-O, Yumiko Ishii, Ken Tonai, Miyoko Tatsuta, Aimi Enokizu, Hiromasa Inoue, Yoichi Nakanishi
Asthma and chronic obstructive pulmonary disease (COPD), chronic airway inflammatory diseases characterized by airflow limitation, have different etiologies and pathophysiologies. Asthma-COPD Overlap (ACO) has recently been used for patients with mixed asthma and COPD. The pathophysiological mechanisms of ACO have not been clearly understood due to the lack of an appropriate murine model. To investigate its pathophysiology, we examined a murine model by allergen challenge in surfactant protein-D (SP-D)-deficient mice that spontaneously developed pulmonary emphysema...
January 2018: Physiological Reports
https://www.readbyqxmd.com/read/29345196/surfactant-protein-c-dampens-inflammation-by-decreasing-jak-stat-activation-during-lung-repair
#8
Huiyan Jin, Andrzej K Ciechanowicz, Alanna R Kaplan, Lin Wang, Ping Xia Zhang, Yi-Chien Lu, Rachel E Tobin, Brooke A Tobin, Lauren Cohn, Caroline J Zeiss, Patty J Lee, Emanuela M Bruscia, Diane S Krause
Surfactant Protein C (SPC), a key component of pulmonary surfactant, also plays a role in regulating inflammation. SPC deficiency in patients and mouse models is associated with increased inflammation and delayed repair, but the key drivers of SPC-regulated inflammation in response to injury are largely unknown. This study focuses on a new mechanism of SPC as an anti-inflammatory molecule using SPC-TK/SPC-KO (surfactant protein C-thymidine kinase/surfactant protein C knockout) mice, which represent a novel sterile injury model that mimics clinical acute respiratory distress syndrome (ARDS)...
January 18, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29339408/development-of-the-swimbladder-surfactant-system-and-biogenesis-of-lysosome-related-organelles-is-regulated-by-blos1-in-zebrafish
#9
Tianbing Chen, Guili Song, Huihui Yang, Lin Mao, Zongbin Cui, Kaiyao Huang
Hermansky-Pudlak syndrome (HPS) is a human autosomal recessive disorder that is characterized by oculocutaneous albinism and a deficiency of the platelet storage pool resulting from defective biogenesis of lysosome-related organelles (LROs). To date, 10 HPS genes have been identified, three of which belong to the octamer complex BLOC-1 (biogenesis of lysosome-related organelles complex 1). One subunit of the BLOC-1 complex, BLOS1, also participates in the BLOC-1-related complex (BORC). Due to lethality at the early embryo stage in BLOS1 knockout mice, the function of BLOS1 in the above two complexes and whether it has a novel function are unclear...
March 2018: Genetics
https://www.readbyqxmd.com/read/29327790/iron-encapsulation-in-water-in-oil-emulsions-effect-of-ferrous-sulfate-concentration-and-fat-crystal-formation-on-oxidative-stability
#10
Nattapong Prichapan, David Julian McClements, Utai Klinkesorn
Iron deficiency is a major global human health concern. Encapsulation of iron in functional food products may help to solve this problem. However, iron is highly reactive and may promote rapid lipid oxidation in fatty foods. In this study, the effect of ferrous sulfate (0.1 to 0.5 wt%) and rice bran stearin (0 or 30 wt%) on the physical properties, oxidative stability, and encapsulation efficiency of 20 wt% water-in-oil (W/O) emulsions stabilized with polyglycerol polyricinoleate was investigated. In the presence of rice bran stearin crystals in the continuous oil phase, W/O emulsions had smaller mean droplet diameters (d ∼ 250 nm) and better physical stability than its absence (d ∼ 330 nm)...
February 2018: Journal of Food Science
https://www.readbyqxmd.com/read/29320485/non-invasive-ventilation-and-surfactant-treatment-as-the-primary-mode-of-respiratory-support-in-surfactant-deficient-newborn-piglets
#11
Carmen Rey-Santano, Victoria E Mielgo, Miguel Angel Gomez-Solaetxe, Francesca Ricci, Federico Bianco, Fabrizio Salomone, Begoña Loureiro, Jon López de Heredia Y Goya
BackgroundNasal continuous positive airway pressure (NCPAP) and nasal intermittent positive pressure ventilation (NIPPV), forms of non-invasive ventilation (NIV) for respiratory support, are increasingly being chosen as the initial treatment for neonates with surfactant (SF) deficiency. Our objective was to compare NCPAP with NIPPV with or without SF administration as a primary mode of ventilation.MethodsTwenty-four newborn piglets with SF-deficient lung injury produced by repetitive bronchoalveolar lavages were randomly assigned to NCPAP or NIPPV, with or without SF administration (InSurE method)...
February 14, 2018: Pediatric Research
https://www.readbyqxmd.com/read/29288965/determination-of-total-plasma-oxysterols-by-enzymatic-hydrolysis-solid-phase-extraction-and-liquid-chromatography-coupled-to-mass-spectrometry
#12
Isabel Mendiara, Celia Domeño, Cristina Nerín, Aron M Geurts, Jesús Osada, Roberto Martínez-Beamonte
The potential use of cholesterol esterases was tested to avoid alkaline hydrolysis for cleavage of plasma esterified oxysterols. The enzymatic hydrolysis was optimized by testing two sources of enzyme-Pseudomonas and bovine pancreas, presence of surfactants, incubation time and amount of enzyme. Free forms of 4β-, 7-, 24-, 25- and 27-hydroxycholesterol (HC) as well 7-ketocholesterol (7-KC) were analyzed by liquid chromatography and mass-spectrometry using the deuterated internal standard, 25-HC(d6). Enzymatic hydrolysis was more effective using the Pseudomonas enzyme and in presence of surfactants...
February 20, 2018: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/29167126/chronic-lung-injury-and-impaired-pulmonary-function-in-a-mouse-model-of-acid-ceramidase-deficiency
#13
Fabian P S Yu, Diana Islam, Jakub Sikora, Shaalee Dworski, Jiří Gurka, Lucía López-Vásquez, Mingyao Liu, Wolfgang M Kuebler, Thierry Levade, Haibo Zhang, Jeffrey A Medin
Farber disease (FD) is a debilitating lysosomal storage disorder (LSD) caused by a deficiency of acid ceramidase (ACDase) activity due to mutations in the gene ASAH1. Patients with ACDase deficiency may develop a spectrum of clinical phenotypes. Severe cases of FD are frequently associated with neurological involvement, failure to thrive, and respiratory complications. Mice homozygous ( Asah1P361R/P361R ) for an orthologous patient mutation in Asah1 recapitulate human FD. In this study, we show significant impairment in lung function, including low compliance and increased airway resistance in a mouse model of ACDase deficiency...
March 1, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29127920/uptake-and-phytotoxic-effect-of-benzalkonium-chlorides-in-lepidium-sativum-and-lactuca-sativa
#14
Adnan Hossain Khan, Mark Libby, Daniel Winnick, John Palmer, Mark Sumarah, Madhumita B Ray, Sheila M Macfie
Cationic surfactants such as benzalkonium chlorides (BACs) are used extensively as biocides in hospitals, food processing industries, and personal care products. BACs have the potential to reach the rooting zone of crop plants and BACs might thereby enter the food chain. The two most commonly used BACs, benzyl dimethyl dodecyl ammonium chloride (BDDA) and benzyl dimethyl tetradecyl ammonium chloride (BDTA), were tested in a hydroponic system to assess the uptake by and phytotoxicity to lettuce (Lactuca sativa L...
January 15, 2018: Journal of Environmental Management
https://www.readbyqxmd.com/read/29067996/the-survival-of-fetal-and-bone-marrow-monocyte-derived-alveolar-macrophages-is-promoted-by-cd44-and-its-interaction-with-hyaluronan
#15
Y Dong, G F T Poon, A A Arif, S S M Lee-Sayer, M Dosanjh, P Johnson
Alveolar macrophages maintain lung homeostasis by performing important roles in immunosurveillance and lung surfactant catabolism. They express high levels of CD44 and are one of the few macrophage populations that constitutively bind hyaluronan, a ligand for CD44 and component of pericellular and extracellular matrices. Using adoptive transfer experiments and a mouse model of inflammation, we found that alveolar macrophages are initially depleted after an inflammatory insult then rapidly self-renew and return to original numbers after the resolution phase...
October 25, 2017: Mucosal Immunology
https://www.readbyqxmd.com/read/28922348/stem-cell-biology-and-regenerative-medicine-for-neonatal-lung-diseases
#16
REVIEW
Martin Kang, Bernard Thébaud
Lung diseases remain one of the main causes of morbidity and mortality in neonates. Cell therapy and regenerative medicine have the potential to revolutionize the management of life-threatening and debilitating lung diseases that currently lack effective treatments. Over the past decade, the repair capabilities of stem/progenitor cells have been harnessed to prevent/rescue lung damage in experimental neonatal lung diseases. Mesenchymal stromal cells and amnion epithelial cells exert pleiotropic effects and represent ideal therapeutic cells for bronchopulmonary dysplasia, a multifactorial disease...
January 2018: Pediatric Research
https://www.readbyqxmd.com/read/28916727/inflammatory-responses-induce-an-identity-crisis-of-alveolar-macrophages-leading-to-pulmonary-alveolar-proteinosis
#17
COMPARATIVE STUDY
Risa Ebina-Shibuya, Mitsuyo Matsumoto, Makoto Kuwahara, Kyoung-Jin Jang, Manabu Sugai, Yoshiaki Ito, Ryo Funayama, Keiko Nakayama, Yuki Sato, Naoto Ishii, Yasunobu Okamura, Kengo Kinoshita, Kohei Kometani, Tomohiro Kurosaki, Akihiko Muto, Masakazu Ichinose, Masakatsu Yamashita, Kazuhiko Igarashi
Pulmonary alveolar proteinosis (PAP) is a severe respiratory disease characterized by dyspnea caused by accumulation of surfactant protein. Dysfunction of alveolar macrophages (AMs), which regulate the homeostasis of surfactant protein, leads to the development of PAP; for example, in mice lacking BTB and CNC homology 2 (Bach2). However, how Bach2 helps prevent PAP is unknown, and the cell-specific effects of Bach2 are undefined. Using mice lacking Bach2 in specific cell types, we found that the PAP phenotype of Bach2 -deficient mice is due to Bach2 deficiency in more than two types of immune cells...
November 3, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28888561/delayed-presentation-and-prolonged-survival-of-a-child-with-surfactant-protein-b-deficiency
#18
Juan A López-Andreu, Antonio D Hidalgo-Santos, Miguel A Fuentes-Castelló, Nuria Mancheño-Franch, Juan A Cerón-Pérez, Maria J Esteban-Ricós, Laia Pedrola-Vidal, Lawrence M Nogee
Surfactant protein B encoding gene mutations have been related to early onset fatal respiratory distress in full-term neonates. We report a school-aged male child homozygous for a surfactant protein B encoding gene missense mutation who presented after the neonatal period. His respiratory insufficiency responded to high dose intravenous methylprednisolone and hydroxychloroquine.
November 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28878025/gallbladder-derived-surfactant-protein-d-regulates-gut-commensal-bacteria-for-maintaining-intestinal-homeostasis
#19
Hana Sarashina-Kida, Hideo Negishi, Junko Nishio, Wataru Suda, Yuki Nakajima, Mika Yasui-Kato, Keiko Iwaisako, Sujin Kang, Nobuyasu Endo, Hideyuki Yanai, Masataka Asagiri, Hiroshi Kida, Masahira Hattori, Atsushi Kumanogoh, Tadatsugu Taniguchi
The commensal microbiota within the gastrointestinal tract is essential in maintaining homeostasis. Indeed, dysregulation in the repertoire of microbiota can result in the development of intestinal immune-inflammatory diseases. Further, this immune regulation by gut microbiota is important systemically, impacting health and disease of organ systems beyond the local environment of the gut. What has not been explored is how distant organs might in turn shape the microbiota via microbe-targeted molecules. Here, we provide evidence that surfactant protein D (SP-D) synthesized in the gallbladder and delivered into intestinal lumen binds selectively to species of gut commensal bacteria...
September 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28873200/mac-1-deficiency-induces-respiratory-failure-by-affecting-type-i-alveolar-epithelial-cells
#20
J Wang, Y B Ci, C L Liu, H M Sun
As a β2 integrin family member, Mac-1 plays an important role in the inflammatory response. Inflammation and lung injury are closely associated, but the involvement of Mac-1 in the occurrence and development of such pathologies remains poorly understood. We aimed to investigate the relationship between Mac-1 deficiency and respiratory failure in Mac-1 knockout {Mac-1-/- } mice, using C57BL/6J mice as a control. The newborn survival rate of Mac-1-/- mice was calculated, and mouse lung tissue was treated with hematoxylin and eosin and subjected to immunofluorescent staining...
August 31, 2017: Genetics and Molecular Research: GMR
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