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Veno-occlusive disease

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https://www.readbyqxmd.com/read/29229098/pulmonary-veno-occlusive-disease-an-important-consideration-in-patients-with-pulmonary-hypertension
#1
REVIEW
Ryan Balko, Hawa Edriss, Kenneth Nugent, Victor Test
Pulmonary veno-occlusive disease is a rare subcategory of pulmonary arterial hypertension (WHO Group 1). The disease is poorly understood and difficult to diagnose; it has no definitive cure to date. These patients present with nonspecific symptoms, including dyspnea, exercise intolerance, and weakness. Chest x-rays sometimes differ from idiopathic pulmonary arterial hypertension and may demonstrate alveolar infiltrates and pleural effusions. High resolution computed tomography scans reveal ground glass opacities, interlobular septal thickening, and lymphadenopathy...
November 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29191686/ct-features-of-hepatic-veno-occlusive-disease-a-meta-analysis
#2
Song Yang, Jing Wu, Si Lei
RATIONALE AND OBJECTIVE: The computed tomography (CT) features of hepatic veno-occlusive disease (HVOD) could play a role in its diagnosis. We aimed to perform a meta-analysis of studies examining the CT features of HVOD. METHODS: Relevant studies published up to May 3, 2017 were searched in major electronic databases. The extracted data included the proportion of various CT features in patients with HVOD. The meta-analysis was conducted using R 3.3.3 with the "meta" package...
November 27, 2017: Academic Radiology
https://www.readbyqxmd.com/read/29168664/pulmonary-arterial-hypertension-and-acute-respiratory-distress-syndrome-in-a-patient-with-adult-onset-stills-disease
#3
Grant H Lowther, Jason Chertoff, Jessica Cope, Hassan Alnuaimat, Ali Ataya
Adult-onset Still's disease (AOSD) is an inflammatory disorder characterized by recurrent fevers, arthralgia, leukocytosis, and a salmon-colored rash. Diagnosis is made based on the Yamaguchi criteria. Various cardiac and pulmonary manifestations have been described in association with AOSD, including acute respiratory distress syndrome (ARDS) and pulmonary arterial hypertension (PAH). We describe the first case of both PAH and ARDS in a patient with AOSD who, despite aggressive therapy, declined rapidly and ultimately died...
October 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/29158214/pulmonary-veno-occlusive-disease-welcome-to-the-pahty-bostonian-for-party
#4
EDITORIAL
Dave P Miller, Harrison W Farber
No abstract text is available yet for this article.
November 21, 2017: Circulation
https://www.readbyqxmd.com/read/29155317/treosulfan-fludarabine-conditioning-for-hsct-in-children-with-primary-immunodeficiency-uk-experience
#5
Mary A Slatter, Kanchan Rao, Intan Juliana Abd Hamid, Zohreh Nademi, Robert Chiesa, Reem Elfeky, Mark S Pearce, Persis Amrolia, Austen Worth, Terence Flood, Mario Abinun, Sophie Hambleton, Waseem Qasim, Hubert B Gaspar, Andrew J Cant, Andrew R Gennery, Paul Veys
We previously published results of 70 children who received treosulfan with cyclophosphamide (30) or fludarabine (40) before haematopoietic stem cell transplantation (HSCT) for Primary Immunodeficiency (PID). Toxicity was lower and T cell chimerism better in those receiving fludarabine, but numbers were relatively small and follow-up short. We now report outcome of 160 children who received homogeneous conditioning with treosulfan, fludarabine mostly with alemtuzumab (n=124). Median age at transplant was 1...
November 16, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29153975/optimisation-of-empirical-antimicrobial-therapy-in-patients-with-haematological-malignancies-and-febrile-neutropenia-how-long-study-an-open-label-randomised-controlled-phase-4-trial
#6
Manuela Aguilar-Guisado, Ildefonso Espigado, Almudena Martín-Peña, Carlota Gudiol, Cristina Royo-Cebrecos, José Falantes, Lourdes Vázquez-López, María Isabel Montero, Clara Rosso-Fernández, María de la Luz Martino, Rocío Parody, José González-Campos, Sebastián Garzón-López, Cristina Calderón-Cabrera, Pere Barba, Nancy Rodríguez, Montserrat Rovira, Enrique Montero-Mateos, Jordi Carratalá, José Antonio Pérez-Simón, José Miguel Cisneros
BACKGROUND: Continuation of empirical antimicrobial therapy (EAT) for febrile neutropenia in patients with haematological malignancies until neutrophil recovery could prolong the therapy unnecessarily. We aimed to establish whether EAT discontinuation driven by a clinical approach regardless of neutrophil recovery would optimise the duration of therapy. METHODS: We did an investigator-driven, superiority, open-label, randomised, controlled phase 4 clinical trial in six academic hospitals in Spain...
November 15, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/29108819/pulmonary-vascular-remodeling-patterns-and-expression-of-general-control-nonderepressible-2-gcn2-in-pulmonary-veno-occlusive-disease
#7
Esther J Nossent, Fabrice Antigny, David Montani, Harm Jan Bogaard, Maria Rosa Ghigna, Mélanie Lambert, Vincent Thomas de Montpréville, Barbara Girerd, Xavier Jaïs, Laurent Savale, Olaf Mercier, Elie Fadel, Florent Soubrier, Olivier Sitbon, Gérald Simonneau, Anton Vonk Noordegraaf, Marc Humbert, Frédéric Perros, Peter Dorfmüller
BACKGROUND: Heritable pulmonary veno-occlusive disease (PVOD) is linked to mutations in the eukaryotic initiation factor 2 alpha kinase 4 (EIF2AK4) gene, leading to a loss of general control nonderepressible 2 (GCN2). The role of GCN2 expression in pulmonary vascular remodeling remains obscure. We sought to identify specific histologic and biologic features in heritable PVOD. METHODS: Clinical data and lung histology of 24 PVOD patients (12 EIF2AK4 mutation carriers, 12 non-carriers) were submitted to systematic histologic analysis and semiautomated morphometry...
October 4, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29066680/-fresh-frozen-plasma-provides-endothelial-protection-when-administered-early-after-stem-cell-transplantation
#8
Osamu Imataki, Makiko Uemura
BACKGROUND: Veno-occlusive disease(VOD)is a fatal coagulopathy, which is caused by an endothelial disorder among patients who undergo hematopoietic stem cell transplantation(SCT). Some protective agents, such as administration of fresh frozen plasma(FFP), have been evaluated for both prophylactic and targeted therapy. PURPOSE: We evaluated the prophylactic efficacy of FFP for VOD in a retrospective study. METHODS: We surveyed patients who received SCT in our hospital between January 2011 and December 2011...
October 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29051993/treatment-related-sinusoidal-obstruction-syndrome-in-children-with-de-novo-acute-lymphoblastic-leukemia-during-intensification
#9
Casey L McAtee, Netta Schneller, Julienne Brackett, M Brooke Bernhardt, Eric S Schafer
PURPOSE: Sinusoidal obstruction syndrome (SOS), also known as veno-occlusive disease, has been described following treatment of acute lymphoblastic leukemia (ALL) with the anti-metabolite 6-thioguanine (6-TG). Previous studies incorporating daily 6-TG into maintenance chemotherapy demonstrated a high incidence of SOS, typically presenting after prolonged exposures to 6-TG. 6-TG continues to be used as a single, 14-day burst during intensification; however, SOS associated with brief courses of 6-TG is poorly described...
October 19, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/29045954/-pulmonary-arterial-hypertension-as-leading-manifestation-of-methylmalonic-aciduria-clinical-characteristics-and-gene-testing-in-15-cases
#10
X Q Liu, H Yan, J X Qiu, C Y Zhang, J G Qi, X Zhang, H J Xiao, Y L Yang, Y H Chen, J B DU
OBJECTIVE: To deepen our understanding of Methylmalonic aciduria (MMA) associated pulmonary hypertension (PH) by analyzing the characteristics of clinical presentation, pulmonary high resolusion CT(HRCT), treatment response and gene mutation. METHODS: This study includes 15 cases of pediatric patients with MMA associated PH diagnosed and treated in Peking University First Hospital pediatric department between May 2012 and May 2016 with symptoms of PH as their leading presentation...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29032695/hepatic-veno-occlusive-disease-induced-by-chinese-medicinal-herbs
#11
María de Los Ángeles Mejías Manzano, Álvaro Giráldez Gallego, María Serrano Jiménez
The potential hepatotoxic effects of products containing medicinal herbs, which are increasingly used without adequate control by health authorities, is well known. We report a case of toxic hepatic veno-occlusive disease (HVOD) presumably associated with the use of such herbal remedies.
November 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29029203/prognostic-significance-of-computed-tomography-criteria-for-pulmonary-veno-occlusive-disease-in-systemic-sclerosis-pulmonary-arterial-hypertension
#12
Michelle J Connolly, Sharif Abdullah, Deborah A Ridout, Benjamin E Schreiber, Jamanda A Haddock, J Gerry Coghlan
Objectives: SSc-pulmonary arterial hypertension (SSc-PAH) is associated with worse response to therapy and survival when compared with idiopathic PAH. It is suggested that the vasculopathy in SSc may involve postcapillary pulmonary venules resulting in pulmonary veno-occlusive disease (PVOD). This may underlie the lower gas transfer and worse outcome on therapy. We sought to test whether CT signs of PVOD (CTS-PVOD) were frequent in SSc-PAH and whether they were associated with pulmonary oedema on therapy and worse survival...
September 26, 2017: Rheumatology
https://www.readbyqxmd.com/read/28984258/hepatic-sinusoidal-obstruction-syndrome-and-busulfan-induced-lung-injury-in-a-post-autologous-stem-cell-transplant-recipient
#13
Richa Jain, Kirti Gupta, Anmol Bhatia, Arun Bansal, Deepak Bansal
Veno-occlusive disease of the liver is mostly encountered as a complication of hematopoietic stem cell transplantation with myeloablative regimens with an incidence estimated to be 13.7%. It is clinically characterized by tender hepatomegaly, jaundice, weight gain and ascites. Strong clinical suspicion and an early recognition of clinical signs are essential to establish the diagnosis and institute effective regimen. Another complication of cytotoxic drugs given for cancers, is development of busulfan-induced lung injury...
September 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28972005/phenotypic-characterisation-of-eif2ak4-mutation-carriers-in-a-large-cohort-of-patients-diagnosed-clinically-with-pulmonary-arterial-hypertension
#14
Charaka Hadinnapola, Marta Bleda, Matthias Haimel, Nicholas Screaton, Andrew J Swift, Peter Dorfmüller, Stephen D Preston, Mark Southwood, Jules Hernandez-Sanchez, Jennifer Martin, Carmen Treacy, Katherine Yates, Harm Bogaard, Colin Church, Gerry Coghlan, Robin Condliffe, Paul A Corris, Simon R Gibbs, Barbara Girerd, Simon Holden, Marc Humbert, David G Kiely, Allan Lawrie, Rajiv D Machado, Robert MacKenzie Ross, Shahin Moledina, David Montani, Michael Newnham, Andrew J Peacock, Joanna Pepke-Zaba, Paula J Rayner-Matthews, Olga Shamardina, Florent Soubrier, Laura Southgate, Jay Suntharalingam, Mark R Toshner, Richard C Trembath, Anton Vonk Noordegraaf, Martin R Wilkins, Stephen J Wort, John Wharton, Stefan Gräf, Nicholas W Morrell
Background -Pulmonary arterial hypertension (PAH) is a rare disease with an emerging genetic basis. Heterozygous mutations in the gene encoding the bone morphogenetic protein receptor type 2 (BMPR2) are the commonest genetic cause of PAH, whereas biallelic mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4) are described in pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis (PVOD/PCH). Here, we determined the frequency of these mutations and define the genotype-phenotype characteristics in a large cohort of patients diagnosed clinically with PAH...
September 28, 2017: Circulation
https://www.readbyqxmd.com/read/28958195/pulmonary-veno-occlusive-disease-in-a-pediatric-hematopoietic-stem-cell-transplant-patient-a-cautionary-tale
#15
M S Zinter, A Melton, A J Sabnis, C C Dvorak, B M Elicker, H M Nawaytou, R J Kameny, J R Fineman
No abstract text is available yet for this article.
September 28, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28954767/pulmonary-hypertension-in-systemic-sclerosis-different-phenotypes
#16
REVIEW
David Launay, Vincent Sobanski, Eric Hachulla, Marc Humbert
Pulmonary hypertension (PH) is a frequent and severe complication of systemic sclerosis (SSc). PH in SSc is highly heterogeneous because of the various clinical phenotypes of SSc itself and because the mechanisms of PH can vary from one patient to another. PH in SSc may be due to vasculopathy of the small pulmonary arteries (group 1; pulmonary arterial hypertension), interstitial lung disease (group 3; PH due to lung disease or chronic hypoxia) or myocardial fibrosis leading to left ventricular systolic or diastolic dysfunction (group 2; PH due to chronic left-heart disease)...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28947033/efficacy-and-safety-of-long-term-imatinib-therapy-for-patients-with-pulmonary-veno-occlusive-disease-and-pulmonary-capillary-hemangiomatosis
#17
Aiko Ogawa, Katsumasa Miyaji, Hiromi Matsubara
BACKGROUND: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are categorized as Group 1' in the clinical classification of pulmonary hypertension. No medical therapy has been proven to be effective in patients with PVOD/PCH. Imatinib is a molecular targeted drug and was expected to be effective in patients with pulmonary arterial hypertension. We evaluated its efficacy and safety in patients with PVOD/PCH. METHODS: In the present observational study, 9 patients with PVOD/PCH received imatinib...
October 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28939456/combination-of-high-dose-methylprednisolone-and-defibrotide-for-veno-occlusive-disease-in-pediatric-hematopoietic-stem-cell-transplant-recipients
#18
Nicholas J Gloude, Sonata Jodele, Ashley Teusink-Cross, Michael Grimley, Stella M Davies, Adam Lane, Kasiani C Myers
Veno-occlusive disease (VOD) is a serious complication of hematopoietic stem cell transplant (HSCT), with high mortality in severe cases and until recently very limited therapeutic options consisting largely of supportive care. Defibrotide was recently approved in the United States for the treatment of severe VOD in patients with renal or pulmonary dysfunction after HSCT. Our group previously published on the use of high-dose methylprednisolone (500 mg/m(2) per dose every 12 hours for 6 doses) in patients with VOD, showing good success...
September 20, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28938703/evaluation-of-thromboelastometry-parameters-as-predictive-markers-for-sinusoidal-obstruction-syndrome-in-patients-undergoing-allogeneic-stem-cell-transplantation-for-acute-leukaemia
#19
Joanna Rupa-Matysek, Lidia Gil, Ewelina Wojtasińska, Zuzanna Kanduła, Adam Nowicki, Magdalena Matuszak, Mieczysław Komarnicki
Hepatic sinusoidal obstruction syndrome (previously named veno-occlusive disease, SOS/VOD) is a serious complication of allogeneic stem cell transplantation (HSCT). Early identification of patients at risk of SOS/VOD may possibly improve the outcome and reduce mortality. Rotation thromboelastometry (ROTEM) is global assay allowing for the precise assessment of both bleeding and thrombotic conditions, however, its usefulness in patients undergoing HSCT for acute leukaemia has not been studied. We evaluated the thromboelastometry parameters in patients undergoing allogeneic HSCT for acute leukaemia to identify candidate biomarkers of SOS/VOD occurrence...
August 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28914546/defibrotide-an-oligonucleotide-for-sinusoidal-obstruction-syndrome
#20
May T Aziz, Payal P Kakadiya, Samantha M Kush, Kylie Weigel, Denise K Lowe
OBJECTIVE: To review the efficacy and safety of defibrotide as well as its pharmacology, mechanism of action, pharmacokinetics (PK), drug-drug interactions, dosing, cost considerations, and place in therapy. DATA SOURCES: A PubMed search was performed through August 2017 using the terms defibrotide, oligonucleotide, hepatic veno-occlusive disease (VOD), sinusoidal obstruction syndrome (SOS), and hematopoietic cell transplantation (HCT). Other data sources were from references of identified studies, review articles, and conference abstracts plus manufacturer product labeling and website, the Food and Drug Administration website, and clinicaltrials...
September 1, 2017: Annals of Pharmacotherapy
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