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https://www.readbyqxmd.com/read/29330690/extracorporeal-membrane-oxygenation-for-severe-middle-east-respiratory-syndrome-coronavirus
#1
Mohammed S Alshahrani, Anees Sindi, Fayez Alshamsi, Awad Al-Omari, Mohamed El Tahan, Bayan Alahmadi, Ahmed Zein, Naif Khatani, Fahad Al-Hameed, Sultan Alamri, Mohammed Abdelzaher, Amenah Alghamdi, Faisal Alfousan, Adel Tash, Wail Tashkandi, Rajaa Alraddadi, Kim Lewis, Mohammed Badawee, Yaseen M Arabi, Eddy Fan, Waleed Alhazzani
BACKGROUND: Middle East respiratory syndrome (MERS) is caused by a coronavirus (MERS-CoV) and is characterized by hypoxemic respiratory failure. The objective of this study is to compare the outcomes of MERS-CoV patients before and after the availability of extracorporeal membrane oxygenation (ECMO) as a rescue therapy in severely hypoxemic patients who failed conventional strategies. METHODS: We collected data retrospectively on MERS-CoV patients with refractory respiratory failure from April 2014 to December 2015 in 5 intensive care units (ICUs) in Saudi Arabia...
January 10, 2018: Annals of Intensive Care
https://www.readbyqxmd.com/read/29330393/haploidentical-bone-marrow-transplantation-with-post-transplant-cyclophosphamide-for-patients-with-x-linked-adrenoleukodystrophy-a-suitable-choice-in-an-urgent-situation
#2
Juliana Folloni Fernandes, Carmem Bonfim, Fábio Rodrigues Kerbauy, Morgani Rodrigues, Iracema Esteves, Nathalia Halley Silva, Alessandra Prandini Azambuja, Luiz Fernando Mantovani, José Mauro Kutner, Gisele Loth, Cilmara Cristina Kuwahara, Clarissa Bueno, Andrea Tiemi Kondo, Andreza Alice Feitosa Ribeiro, Fernando Kok, Nelson Hamerschlak
Allogeneic hematopoietic stem cell transplantation (HSCT) is the only treatment that enhances survival and stabilizes neurologic symptoms in X-linked adrenoleukodystrophy (X-ALD) with cerebral involvement, a severe demyelinating disease of childhood. Patients with X-ALD who lack a well-matched HLA donor need a rapid alternative. Haploidentical HSCT using post transplant cyclophosphamide (PT/Cy) has been performed in patients with malignant and nonmalignant diseases showing similar outcomes compared to other alternative sources...
January 12, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29329694/management-of-refractory-vasodilatory-shock
#3
REVIEW
Jacob C Jentzer, Saraschandra Vallabhajosyula, Ashish K Khanna, Lakhmir S Chawla, Laurence W Busse, Kianoush B Kashani
Refractory shock is a lethal manifestation of cardiovascular failure defined by an inadequate hemodynamic response to high doses of vasopressor medications. Approximately 7% of critically ill patients will develop refractory shock, with short-term mortality exceeding 50%. Refractory vasodilatory shock develops from uncontrolled vasodilation and vascular hyporesponsiveness to endogenous vasoconstrictors, causing failure of physiologic vasoregulatory mechanisms. Standard approaches to initial management of shock include fluid resuscitation and initiation of norepinephrine...
January 9, 2018: Chest
https://www.readbyqxmd.com/read/29327391/molecular-phenotype-of-slc4a11-missense-mutants-setting-the-stage-for-personalized-medicine-in-corneal-dystrophies
#4
Kumari Alka, Joseph R Casey
SLC4A11 mutations cause cases of congenital hereditary endothelial dystrophy (CHED), Harboyan syndrome (HS), and Fuchs endothelial corneal dystrophy (FECD). Defective water reabsorption from corneal stroma by corneal endothelial cells (CECs) leads to these corneal dystrophies. SLC4A11, in the CEC basolateral membrane facilitates transmembrane movement of H2 O, NH3 and H+ -equivalents. Some SLC4A11 disease mutants have impaired folding, leading to a failure to move to the cell surface, which in some cases can be corrected by the drug, Glafenine...
January 11, 2018: Human Mutation
https://www.readbyqxmd.com/read/29325523/fanconi-anemia-with-sun-sensitivity-caused-by-a-xeroderma-pigmentosum-associated-missense-mutation-in-xpf
#5
Isabell Popp, Maqsood Punekar, Nick Telford, Stavros Stivaros, Kate Chandler, Meenakshi Minnis, Anna Castleton, Claire Higham, Louise Hopewell, D Gareth Evans, Anja Raams, Arjan F Theil, Stefan Meyer, Detlev Schindler
BACKGROUND: Fanconi anemia (FA) is an inherited genomic instability disorder with congenital and developmental abnormalities, bone marrow failure and predisposition to cancer early in life, and cellular sensitivity to DNA interstrand crosslinks. CASE PRESENTATION: A fifty-one-year old female patient, initially diagnosed with FA in childhood on the basis of classic features and increased chromosomal breakage, and remarkable sun-sensitivity is described. She only ever had mild haematological abnormalities and no history of malignancy...
January 11, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29324573/allogeneic-hematopoietic-stem-cell-transplantation-for-leukocyte-adhesion-deficiency
#6
Yasuo Horikoshi, Katsutsugu Umeda, Kohsuke Imai, Hiromasa Yabe, Yoji Sasahara, Kenichiro Watanabe, Yukiyasu Ozawa, Yoshiko Hashii, Hidemitsu Kurosawa, Shigeaki Nonoyama, Tomohiro Morio
The clinical outcome of allogeneic hematopoietic stem cell transplantation (HSCT) was retrospectively analyzed in 6 patients with leukocyte adhesion deficiency. Of 3 patients transplanted with myeloablative conditioning, 2 patients had complete chimerism and 1 patient had mixed chimerism. By contrast, all 3 patients transplanted with reduced-intensity conditioning (RIC) had mixed chimerism, one of whom progressed to secondary graft failure. All patients with low-level mixed chimerism and secondary graft failure were rescued by donor lymphocyte infusion or a second HSCT...
January 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29324372/dislocation-of-a-cerebral-protection-device-component-during-carotid-stenting-a-case-report-of-favorable-outcome-from-conservative-management-after-failure-of-retrieval
#7
Ilaria Tocco-Tussardi, Caterina Kulyk, Vincenzo Vindigni, Giampiero Avruscio
INTRODUCTION: Cerebral-protection devices (CPDs) are a well-established system for reduction of embolic risk in carotid artery angioplasty and stenting (CAS). Although rare, adverse events with CPDs are unpredictable and can be associated with serious outcomes and iatrogenic sequelae. PRESENTATION OF CASE: We describe the unique case of dislocation of a FilterWire EX™ filter loop during right CAS. On trying to recapture the CPD filter at the end of the procedure, the filter loop suddenly detached from the guidewire and dislocated to the proximal middle cerebral artery...
December 28, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29323124/mir-770-suppresses-the-chemo-resistance-and-metastasis-of-triple-negative-breast-cancer-via-direct-targeting-of-stmn1
#8
Yaming Li, Yiran Liang, Yuting Sang, Xiaojin Song, Hanwen Zhang, Ying Liu, Liyu Jiang, Qifeng Yang
Chemo-resistance and metastasis of triple negative breast cancer (TNBC) contributed the most of treatment failure in the clinic. MicroRNAs (miRNAs) have been proved to be involved in many biological processes and diseases. In this study, we aimed to determine the role of miR-770 in the regulation of chemo-resistance and metastasis of TNBC. Clinically, miR-770 was highly expressed in chemo-sensitive tissues and predicted a better prognosis of TNBC. Functionally, ectopic expression of miR-770 suppressed the doxorubicin-resistance of TNBC cell lines via regulation of apoptosis and tumor microenvironment, which was mediated by exosomes...
January 11, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29321049/human-liver-organoids-generated-with-single-donor-derived-multiple-cells-rescue-mice-from-acute-liver-failure
#9
Yun-Zhong Nie, Yun-Wen Zheng, Miyuki Ogawa, Etsuko Miyagi, Hideki Taniguchi
BACKGROUND: Acute liver failure (ALF) is a life-threatening disease with a high mortality rate. However, there are limited treatments or devices available for ALF therapy. Here, we aimed to develop a new strategy for ALF treatment by transplanting functional liver organoids (LOs) generated from single donor-derived human induced pluripotent stem cell (hiPSC) endoderm, endothelial cells (ECs), and mesenchymal cells (MCs). METHODS: First, we isolated ECs and MCs from a single donor umbilical cord (UC) through enzyme digestion and characterized the UC-ECs and UC-MCs by flow cytometry...
January 10, 2018: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29317431/suppression-of-activated-foxo-transcription-factors-in-the-heart-prolongs-survival-in-a-mouse-model-of-laminopathies
#10
Gaelle Auguste, Priyatansh Gurha, Raffaella Lombardi, Cristian Coarfa, James T Willerson, Ali J Marian
Rationale: Mutations in the LMNA gene, encoding nuclear inner membrane protein Lamin A/C, cause distinct phenotypes, collectively referred to as laminopathies. Heart failure, conduction defects, and arrhythmias are the common causes of death in laminopathies. Objective: To identify and therapeutically target the responsible mechanism(s) for cardiac phenotype in laminopathies. Methods and Results: Whole heart RNA sequencing was performed prior to the onset of cardiac dysfunction in the Lmna-/- and matched control mice...
January 9, 2018: Circulation Research
https://www.readbyqxmd.com/read/29316553/oocyte-cytoplasmic-gas6-and-heparan-sulfate-hs-are-required-to-establish-the-open-chromatin-state-in-nuclei-during-remodeling-and-reprogramming
#11
Kyeoung-Hwa Kim, Eun-Young Kim, Su-Yeon Lee, Jung-Jae Ko, Kyung-Ah Lee
BACKGROUND/AIMS: Previously, we found that silencing of growth arrest-specific gene 6 (Gas6) in oocytes impaired cytoplasmic maturation, resulting in failure of sperm chromatin decondensation (SCD) and pronuclear (PN) formation after fertilization. Thus, we conducted this study to determine the effect of Gas6 RNAi on downstream genes and to elucidate the working mechanism of Gas6 on oocyte cytoplasmic maturation and SCD. METHODS: Using RT-PCR, Western blot and immunofluorescence, the expression levels of various target genes and the localization of heparan sulfate (HS) were analyzed after Gas6 RNAi...
December 22, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29311338/faulty-neuronal-determination-and-cell-polarization-are-reverted-by-modulating-hd-early-phenotypes
#12
P Conforti, D Besusso, V D Bocchi, A Faedo, E Cesana, G Rossetti, V Ranzani, C N Svendsen, L M Thompson, M Toselli, G Biella, M Pagani, E Cattaneo
Increasing evidence suggests that early neurodevelopmental defects in Huntington's disease (HD) patients could contribute to the later adult neurodegenerative phenotype. Here, by using HD-derived induced pluripotent stem cell lines, we report that early telencephalic induction and late neural identity are affected in cortical and striatal populations. We show that a large CAG expansion causes complete failure of the neuro-ectodermal acquisition, while cells carrying shorter CAGs repeats show gross abnormalities in neural rosette formation as well as disrupted cytoarchitecture in cortical organoids...
January 8, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29298221/evaluating-an-art-based-intervention-to-improve-practicing-nurses-observation-description-and-problem-identification-skills
#13
Beth M Nease, Tina S Haney
Astute observation, description, and problem identification skills provide the underpinning for nursing assessment, surveillance, and prevention of failure to rescue events. Art-based education has been effective in nursing schools for improving observation, description, and problem identification. The authors describe a randomized controlled pilot study testing the effectiveness of an art-based educational intervention aimed at improving these skills in practicing nurses.
January 2018: Journal for Nurses in Professional Development
https://www.readbyqxmd.com/read/29296843/the-severe-phenotype-of-diamond-blackfan-anemia-is-modulated-by-heat-shock-protein-70
#14
Marc Gastou, Sarah Rio, Michaël Dussiot, Narjesse Karboul, Hélène Moniz, Thierry Leblanc, Margaux Sevin, Patrick Gonin, Jérome Larghéro, Carmen Garrido, Anupama Narla, Narla Mohandas, William Vainchenker, Olivier Hermine, Eric Solary, Lydie Da Costa
Diamond-Blackfan anemia (DBA) is a rare congenital bone marrow failure syndrome that exhibits an erythroid-specific phenotype. In at least 70% of cases, DBA is related to a haploinsufficient germ line mutation in a ribosomal protein (RP) gene. Additional cases have been associated with mutations in GATA1. We have previously established that the RPL11+/Mut phenotype is more severe than RPS19+/Mut phenotype because of delayed erythroid differentiation and increased apoptosis of RPL11+/Mut erythroid progenitors...
October 10, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296828/gene-editing-rescue-of-a-novel-mpl-mutant-associated-with-congenital-amegakaryocytic-thrombocytopenia
#15
Cédric Cleyrat, Romain Girard, Eun H Choi, Éric Jeziorski, Thierry Lavabre-Bertrand, Sylvie Hermouet, Serge Carillo, Bridget S Wilson
Thrombopoietin (Tpo) and its receptor (Mpl) are the principal regulators of early and late thrombopoiesis and hematopoietic stem cell maintenance. Mutations in MPL can drastically impair its function and be a contributing factor in multiple hematologic malignancies, including congenital amegakaryocytic thrombocytopenia (CAMT). CAMT is characterized by severe thrombocytopenia at birth, which progresses to bone marrow failure and pancytopenia. Here we report unique familial cases of CAMT that presented with a previously unreported MPL mutation: T814C (W272R) in the background of the activating MPL G117T (K39N or Baltimore) mutation...
September 26, 2017: Blood Advances
https://www.readbyqxmd.com/read/29285642/association-of-delivery-system-integration-and-outcomes-for-major-cancer-surgery
#16
Jonathan Li, Zaojun Ye, James M Dupree, Brent K Hollenbeck, Hye Sung Min, Deborah Kaye, Lindsey A Herrel, David C Miller, Chad Ellimoottil
BACKGROUND: Integrated delivery systems (IDSs) are postulated to reduce spending and improve outcomes through successful coordination of care across multiple providers. Nonetheless, the actual impact of IDSs on outcomes for complex multidisciplinary care such as major cancer surgery is largely unknown. METHODS: Using 2011-2013 Medicare data, this study identified patients who underwent surgical resection for prostate, bladder, esophageal, pancreatic, lung, liver, kidney, colorectal, or ovarian cancer...
December 29, 2017: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/29284690/ampk%C3%AE-2-protects-against-the-development-of-heart-failure-by-enhancing-mitophagy-via-pink1-phosphorylation
#17
Bei Wang, Jiali Nie, Lujin Wu, Yangyang Hu, Zeng Wen, Lingli Dong, Ming-Hui Zou, Chen Chen, Dao Wen Wang
Rationale: Mitochondrial dysfunction plays an important role in heart failure (HF). However, the molecular mechanisms regulating mitochondrial functions via selective mitochondrial autophagy (mitophagy) are poorly understood. Objective: We sought to determine the role of AMP-activated protein kinase (AMPK) in selective mitophagy during HF. Methods and Results: An isoform shift from AMPKα2 to AMPKα1 was observed in failing-heart samples from HF patients and transverse aortic constriction (TAC)-induced mice, accompanied by decreased mitophagy and mitochondrial function...
December 28, 2017: Circulation Research
https://www.readbyqxmd.com/read/29280924/metabolomics-and-precision-medicine-in-trauma-the-state-of-the-field
#18
Sudha P Jayaraman, Rahul J Anand, Jonathan H DeAntonio, Martin Mangino, Michel B Aboutanos, Vigneshwar Kasirajan, Rao Ivatury, Alex B Valadka, Olena Glushakova, Ronald L Hayes, Lorin M Bachmann, Gretchen M Brophy, Daniel Contaifer, Urszula O Warncke, Donald F Brophy, Dayanjan S Wijesinghe
Trauma is a major problem in the United States. Mortality from trauma is the number one cause of death under the age of 45 in the US and is the third leading cause of death for all age groups. There are nearly 200,000 deaths per year due to trauma in the US at a cost of over $671 billion in combined health care costs and lost productivity. Unsurprisingly, trauma accounts for about 30% of all life-years lost in the US. Due to immense development of trauma systems, a large majority of trauma patients survive the injury but then go on to die from complications arising from the injury...
December 26, 2017: Shock
https://www.readbyqxmd.com/read/29273370/is-there-a-weekend-effect-in-emergency-general-surgery
#19
David Metcalfe, Manuel Castillo-Angeles, Arturo J Rios-Diaz, Joaquim M Havens, Adil Haider, Ali Salim
BACKGROUND: Weekend admission is associated with increased mortality across a range of patient populations and health-care systems. The aim of this study was to determine whether weekend admission is independently associated with serious adverse events (SAEs), in-hospital mortality, or failure to rescue (FTR) in emergency general surgery (EGS). METHODS: An observational study was performed using the National Inpatient Sample in 2012-2013; the largest all-payer inpatient database in the United States, which represents a 20% stratified sample of hospital discharges...
February 2018: Journal of Surgical Research
https://www.readbyqxmd.com/read/29259373/mesenchymal-stem-cells-rescue-acute-hepatic-failure-by-polarizing-m2-macrophages
#20
Yan-Wei Li, Chong Zhang, Qiu-Ju Sheng, Han Bai, Yang Ding, Xiao-Guang Dou
AIM: To investigate whether M1 or M2 polarization contributes to the therapeutic effects of mesenchymal stem cells (MSCs) in acute hepatic failure (AHF). METHODS: MSCs were transfused into rats with AHF induced by D-galactosamine (DGalN). The therapeutic effects of MSCs were evaluated based on survival rate and hepatocyte proliferation and apoptosis. Hepatocyte regeneration capacity was evaluated by the expression of the hepatic progenitor surface marker epithelial cell adhesion molecule (EpCAM)...
December 7, 2017: World Journal of Gastroenterology: WJG
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