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Veno-occlusive disease

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https://www.readbyqxmd.com/read/29051993/treatment-related-sinusoidal-obstruction-syndrome-in-children-with-de-novo-acute-lymphoblastic-leukemia-during-intensification
#1
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
#2
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
#3
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.
October 16, 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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/28891390/pathology-and-epidemiology-of-oxalate-nephrosis-in-cheetahs
#13
Emily P Mitchell, Molly E Church, Sarah M Nemser, Betsy Jean Yakes, Eric R Evans, Renate Reimschuessel, Karin Lemberger, Peter N Thompson, Karen A Terio
To investigate cases of acute oxalate nephrosis without evidence of ethylene glycol exposure, archived data and tissues from cheetahs ( Acinonyx jubatus) from North America ( n = 297), southern Africa ( n = 257), and France ( n = 40) were evaluated. Renal and gastrointestinal tract lesions were characterized in a subset of animals with ( n = 100) and without ( n = 165) oxalate crystals at death. Crystals were confirmed as calcium oxalate by Raman spectroscopy in 45 of 47 cheetahs tested. Crystals were present in cheetahs from 3...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28877973/heritable-pulmonary-hypertension-from-bench-to-bedside
#14
REVIEW
Barbara Girerd, Jason Weatherald, David Montani, Marc Humbert
Mutations in the BMPR2 gene, and more rarely in ACVRL1, endoglin, caveolin-1, KCNK3 and TBX4 genes predispose to heritable pulmonary arterial hypertension, an autosomal dominant disease with incomplete penetrance. Bi-allelic mutations in the EIF2AK4 gene predispose to heritable pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis, an autosomal recessive disease with an unknown penetrance.In France, the national pulmonary hypertension referral centre offers genetic counselling and testing to adults and children...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28870776/consensus-report-by-the-pediatric-acute-lung-injury-and-sepsis-investigators-and-pediatric-blood-and-marrow-transplantation-consortium-joint-working-committees-on-supportive-care-guidelines-for-management-of-veno-occlusive-disease-in-children-and-adolescents
#15
Nadia Ovchinsky, Warren Frazier, Jeffery J Auletta, Christopher C Dvorak, Monica Ardura, Enkyung Song, Jennifer McArthur, Asumthia Jeyapalan, Robert Tamburro, Kris M Mahadeo, Chani Traube, Christine N Duncan, Rajinder P S Bajwa
Some patients with veno-occlusive disease (VOD) have multiorgan dysfunction, and multiple teams are involved in their daily care in the pediatric intensive care unit. Cardiorespiratory dysfunction is critical in these patients, requiring immediate action. The decision of whether to use a noninvasive or an invasive ventilation strategy may be difficult in the setting of mucositis or other comorbidities in patients with VOD. Similarly, monitoring of organ function may be very challenging in these patients, who may have fulminant hepatic failure with or without hepatic encephalopathy complicated by delirium and/or infections...
September 1, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28859185/salvage-chemoimmunotherapy-with-inotuzumab-ozogamicin-combined-with-mini-hyper-cvd-for-patients-with-relapsed-or-refractory-philadelphia-chromosome-negative-acute-lymphoblastic-leukemia-a-phase-2-clinical-trial
#16
Elias Jabbour, Farhad Ravandi, Partow Kebriaei, Xuelin Huang, Nicholas J Short, Deborah Thomas, Koji Sasaki, Michael Rytting, Nitin Jain, Marina Konopleva, Guillermo Garcia-Manero, Richard Champlin, David Marin, Tapan Kadia, Jorge Cortes, Zeev Estrov, Koichi Takahashi, Yogin Patel, Maria R Khouri, Jovitta Jacob, Rebecca Garris, Susan O'Brien, Hagop Kantarjian
Importance: The outcome of patients with relapsed or refractory (R/R) acute lymphoblastic leukemia (ALL) is poor. Inotuzumab ozogamicin, a CD22 monoclonal antibody bound to calicheamicin, has single-agent activity in R/R ALL. Objective: To evaluate the efficacy and safety of inotuzumab ozogamicin plus low-intensity chemotherapy in patients with R/R ALL. Design, Setting, and Participants: A single-arm, phase 2 study of adults with R/R B-cell ALL conducted at The University of Texas MD Anderson Cancer Center, Houston...
August 31, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28836324/hematopoietic-stem-cell-transplantation-in-children-with-griscelli-syndrome-a-single-center-experience
#17
Baris Kuskonmaz, Deniz Ayvaz, Muge Gokce, Tuba Turul Ozgur, Fatma V Okur, Mualla Cetin, Ilhan Tezcan, Duygu Uckan Cetinkaya
GS2 is a rare autosomal recessive disease characterized by hypopigmentation, variable immunodeficiency with HLH. HSCT is the only curative treatment for GS2. We analyzed the outcome of 10 children with GS2 who underwent HSCT at our center between October 1997 and September 2013. The median age of the patients at transplant was 13.5 months (range, 6-58 months). All of the patients developed HLH before HSCT and received HLH 94 or HLH 2004 protocols. Donors were HLA-identical relatives in 8 patients, HLA-mismatched relatives in 2 patients...
November 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28825848/defibrotide-for-children-and-adults-with-hepatic-veno-occlusive-disease-post-hematopoietic-cell-transplantation
#18
Selim Corbacioglu, Paul G Richardson
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a complication that is typically associated with conditioning for hematopoietic stem cell transplantation (HSCT). In patients with concomitant multi-organ dysfunction, mortality may be >80%. Recently, the European Society for Blood and Marrow Transplantation established separate criteria for diagnosis and severity of VOD/SOS for adults and children, to better reflect current understanding of the disease. Areas covered: This review provides an overview of post-HSCT hepatic VOD/SOS and defibrotide, including its pharmacological, clinical, and regulatory profile...
October 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28815419/effect-of-sertraline-on-complications-and-survival-after-hematopoietic-stem-cell-transplantation-a-double-blind-placebo-controlled-clinical-study
#19
Maria Tavakoli-Ardakani, Raziyeh Kheshti, Mehrpooya Maryam
Previous studies have found a connection between psychiatric problems and post-hematopoietic stem-cell transplantation (HSCT) complications. We sought to evaluate the effect of sertraline on engraftment time, hospitalization period, mortality, and post-transplantation complications in HSCT recipients with depression and/or anxiety. We recruited adults aged 18-60, who were candidates for autologous or allogeneic HSCT with major depression and/or anxiety disorder. They were administered 50 mg of sertraline or placebo daily for the first week, and then 100 mg for the following seven weeks...
August 16, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28801815/plasma-levels-of-complement-activation-fragments-c3b-and-sc5b-9-significantly-increased-in-patients-with-thrombotic-microangiopathy-after-allogeneic-stem-cell-transplantation
#20
Jiaqian Qi, Jie Wang, Jia Chen, Jian Su, Yaqiong Tang, Xiaojin Wu, Xiao Ma, Feng Chen, Changgeng Ruan, X Long Zheng, Depei Wu, Yue Han
Transplantation-associated thrombotic microangiopathy (TA-TMA) is an uncommon but severe complication in patients undergoing allogeneic stem cell transplantation (allo-SCT). However, the mechanism is unclear. From 2011 to 2014, 20 patients with TA-TMA, 20 patients without, and 54 patients with various other complications, including veno occlusive disease (VOD), graft-versus-host disease (GVHD), and infection, were recruited in the study. Plasma vWF antigen (vWFAg), vWF activity (vWFAc), and ADAMTS13 activity were determined in these patients by ELISAs and FRETS-vWF73 assay, respectively...
August 11, 2017: Annals of Hematology
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