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Veno-occlusive disease

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https://www.readbyqxmd.com/read/27892944/veno-occlusive-disease-sinusoidal-obstruction-syndrome-after-haematopoietic-stem-cell-transplantation-middle-east-north-africa-regional-consensus-on-prevention-diagnosis-and-management
#1
A H Al Jefri, H Abujazar, A Al-Ahmari, A Al Rawas, Z Al Zahrani, A Alhejazi, M A Bekadja, A Ibrahim, M Lahoucine, S Ousia, A Bazarbachi
Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) of the liver is a serious, early complication of haematopoietic stem cell transplantation (HSCT), severe and very severe forms of which are associated with a high mortality rate. A wide variety of patient, disease and treatment-related risk factors for VOD/SOS have been identified. Several bodies have published recommendations for the diagnosis, prevention and management of VOD/SOS following HSCT. A group of regional experts have developed a consensus statement on the diagnosis, prevention and management of VOD/SOS in the Middle East and North Africa region to help in the management of HSCT patients in the region...
November 28, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27884767/eif2ak4-mutations-in-patients-diagnosed-with-pulmonary-arterial-hypertension
#2
D Hunter Best, Kelli L Sumner, Benjamin P Smith, Kristy Damjanovich-Colmenares, Ikue Nakayama, Lynette M Brown, Youna Ha, Eleri Paul, Ashley Morris, Mohamed A Jama, Mark W Dodson, Pinar Bayrak-Toydemir, C Gregory Elliott
BACKGROUND: Differentiating pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) from idiopathic or heritable pulmonary arterial hypertension (IPAH and HPAH) is important clinically. Mutations in eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) cause heritable PVOD and PCH whereas mutations in other genes cause HPAH. The aim of this study is to describe the frequency of pathogenic EIF2AK4 mutations in patients diagnosed clinically with IPAH or HPAH...
November 21, 2016: Chest
https://www.readbyqxmd.com/read/27872804/pulmonary-hypertension-secondary-to-pulmonary-veno-occlusive-disease-complicated-by-right-heart-failure-hypotension-and-acute-kidney-injury
#3
Nima Golzy, Stuti Fernandes, Justin Sharim, Rikin Tank, Henry D Tazelaar, Howard E Epstein, Victor Tapson, Antoine Hage
Pulmonary veno-occlusive disease (PVOD) is rare condition which can lead to severe pulmonary hypertension, right ventricular dysfunction, and cardiopulmonary failure. The diagnosis of PVOD can be challenging due to its nonspecific symptoms and its similarity to idiopathic pulmonary arterial hypertension and interstitial lung disease in terms of diagnostic findings. This case describes a 57 year old female patient who presented with a 5-month history of progressive dyspnea on exertion and nonproductive cough...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27843363/defibrotide-in-the-treatment-of-hepatic-veno-occlusive-disease
#4
REVIEW
Alessandro Fulgenzi, Maria Elena Ferrero
Hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome (SOS), represents the most frequent complication in patients in early phase following hematopoietic stem-cell transplantation (HSCT). In its severe form, VOD/SOS can be associated with multiorgan failure and with a mortality rate >80% by day +100. Defibrotide (DF) (a mixture of 90% single-stranded phosphodiester oligonucleotides and 10% double-stranded phosphodiester oligonucleotides derived from controlled depolarization of porcine intestinal mucosal DNA) has been proposed for the treatment of SOS due to its ability to restore thrombo-fibrinolytic balance and protect endothelial cells...
2016: Hepatic Medicine: Evidence and Research
https://www.readbyqxmd.com/read/27827824/plasma-biomarkers-of-risk-for-death-in-a-multicenter-phase-3-trial-with-uniform-transplant-characteristics-post-allogeneic-hct
#5
Mohammad Abu Zaid, Juan Wu, Cindy Wu, Brent R Logan, Jeffrey Yu, Corey Cutler, Joseph H Antin, Sophie Paczesny, Sung Won Choi
A phase 3 clinical trial (BMT CTN 0402) comparing tacrolimus/sirolimus (Tac/Sir) versus tacrolimus/methotrexate (Tac/Mtx) as graft-versus-host disease (GVHD) prophylaxis after matched-related allogeneic hematopoietic cell transplantation (HCT) recently showed no difference between study arms in acute GVHD-free survival. Within this setting of a prospective, multicenter study with uniform GVHD prophylaxis, conditioning regimen, and donor source, we explored the correlation of 10 previously identified biomarkers with clinical outcomes after allogeneic HCT...
November 8, 2016: Blood
https://www.readbyqxmd.com/read/27823643/an-overview-of-hematopoietic-stem-cell-transplantation-related-thrombotic-complications
#6
REVIEW
Ugur Sahin, Pinar Ataca Atilla, Erden Atilla, Selami Kocak Toprak, Taner Demirer
Thrombotic episodes are far less common than bleeding complications after hematopoietic stem cell transplantation (HSCT). However, they lead to significant morbidity and mortality. These complications are classified into four groups, including venous thromboembolic events (VTE), catheter-induced thrombosis (CIT), transplant-associated thrombotic microangiopathy (TA-TMA) and sinusoidal obstruction syndrome (SOS) or veno-occlusive disease (VOD). The frequency of VTE is increased among patients undergoing HSCT due to some acquired conditions including underlying malignancy, infections, administration of myeloablative conditioning regimens and/or total body irradiation, prolonged hospitalizations leading to immobility and presence of central venous catheters...
November 2016: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/27809840/eif2ak4-mutation-as-second-hit-in-hereditary-pulmonary-arterial-hypertension
#7
Christina A Eichstaedt, Jie Song, Nicola Benjamin, Satenik Harutyunova, Christine Fischer, Ekkehard Grünig, Katrin Hinderhofer
BACKGROUND: Mutations in the eukaryotic translation initiation factor 2α kinase 4 (EIF2AK4) gene have recently been identified in recessively inherited veno-occlusive disease. In this study we assessed if EIF2AK4 mutations occur also in a family with autosomal dominantly inherited pulmonary arterial hypertension (HPAH) and incomplete penetrance of bone morphogenic protein receptor 2 (BMPR2) mutations. METHODS: Clinical examinations in a family with 10 members included physical examination, electrocardiogram, (stress)-echocardiography and lung function...
November 4, 2016: Respiratory Research
https://www.readbyqxmd.com/read/27770853/external-mechanical-devices-and-vascular-surgery-for-erectile-dysfunction
#8
Landon W Trost, Ricardo Munarriz, Run Wang, Allen Morey, Laurence Levine
INTRODUCTION: The field of sexual medicine is continuously advancing, with novel outcomes reported on a regular basis. Given the rapid evolution, updated guidelines are essential to inform practicing clinicians on best practices. AIM: To summarize the current literature and provide clinical guidelines on penile traction therapy, vacuum erection devices, and penile revascularization. METHODS: A consensus panel was held with leading sexual medicine experts during the 2015 International Consultation on Sexual Medicine (ICSM)...
November 2016: Journal of Sexual Medicine
https://www.readbyqxmd.com/read/27746112/association-of-busulfan-exposure-with-survival-and-toxicity-after-haemopoietic-cell-transplantation-in-children-and-young-adults-a-multicentre-retrospective-cohort-analysis
#9
Imke H Bartelink, Arief Lalmohamed, Elisabeth M L van Reij, Christopher C Dvorak, Rada M Savic, Juliette Zwaveling, Robbert G M Bredius, Antoine C G Egberts, Marc Bierings, Morris Kletzel, Peter J Shaw, Christa E Nath, George Hempel, Marc Ansari, Maja Krajinovic, Yves Théorêt, Michel Duval, Ron J Keizer, Henrique Bittencourt, Moustapha Hassan, Tayfun Güngör, Robert F Wynn, Paul Veys, Geoff D E Cuvelier, Sarah Marktel, Robert Chiesa, Morton J Cowan, Mary A Slatter, Melisa K Stricherz, Cathryn Jennissen, Janel R Long-Boyle, Jaap Jan Boelens
BACKGROUND: Intravenous busulfan combined with therapeutic drug monitoring to guide dosing improves outcomes after allogeneic haemopoietic cell transplantation (HCT). The best method to estimate busulfan exposure and optimum exposure in children or young adults remains unclear. We therefore assessed three approaches to estimate intravenous busulfan exposure (expressed as cumulative area under the curve [AUC]) and associated busulfan AUC with clinical outcomes in children or young adults undergoing allogeneic HCT...
October 13, 2016: Lancet Haematology
https://www.readbyqxmd.com/read/27722893/minimal-incidence-of-neurotoxicity-without-prophylaxis-during-busulfan-based-conditioning-regimen-in-patients-undergoing-stem-cell-transplantation
#10
Eucario Leon-Rodriguez, Monica M Rivera-Franco
Busulfan (Bu), an alkylating agent, has been used in pre-transplant conditioning regimens since the 1950s, due to its potent myeloablative effect. Questions have been raised regarding oral or intravenous formulations, although both are known to be associated with serious side effects, including hepatic veno-occlusive disease, and neurotoxicity. The administration of anticonvulsant prophylaxis has become more common during high-dose Bu-based conditioning regimen; however, anticonvulsants can interfere with Bu pharmacokinetics and may have their own side effects, which can affect the outcome of the transplant...
October 8, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27721142/defibrotide-for-the-management-of-sinusoidal-obstruction-syndrome-in-patients-who-undergo-haemopoietic-stem-cell-transplantation
#11
John Coutsouvelis, Sharon Avery, Michael Dooley, Carl Kirkpatrick, Andrew Spencer
Sinusoidal obstruction syndrome, previously known as veno-occlusive disease (VOD/SOS), is a complication in patients undergoing haemopoietic stem cell transplantation (HSCT). Severe VOD/SOS, including progression to multi-organ failure, has resulted in a mortality of greater than 80%. Defibrotide's varying pharmacological actions, particularly on endothelial cells, make it is a useful agent to consider for prophylaxis and treatment of VOD/SOS. Barriers to its routine use include the high acquisition cost and the fact that neither the oral or parenteral formulations are licensed products in many countries at this time...
September 30, 2016: Cancer Treatment Reviews
https://www.readbyqxmd.com/read/27691976/nephropathies-in-the-european-captive-cheetah-acinonyx-jubatus-population
#12
Angelika Url, Verena Krutak, Anna Kübber-Heiss, Sonja Chvala-Mannsberger, Nadia Robert, Nora Dinhopl, Peter Schmidt, Chris Walzer
According to previous studies in captive cheetah ( Acinonyx jubatus ) populations, one of the most threatening diseases besides amyloidosis, myelopathy, veno occlusive disease, and gastritis, is renal failure. Contrary to captive cheetahs in North America and South Africa, morphological data concerning renal lesions in the cheetah European Endangered Species Program (EEP) are lacking. This study details the histological characterization as well as immunohistochemical and morphometrical analysis of nephropathies in 35 captive cheetahs from the EEP, which were necropsied between 1985 and 2003...
September 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/27684876/eif2ak4-mutation-in-pulmonary-veno-occlusive-disease-a-case-report-and-review-of-the-literature
#13
Li Liang, Guofeng Ma, Kai Chen, Yangxiang Liu, Xiaohong Wu, Kejing Ying, Ruifeng Zhang
BACKGROUND: Pulmonary veno-occlusive disease (PVOD) is a rare and devastating cause of pulmonary arterial hypertension with a non-specific clinical presentation and a relatively specific presentation in high-resolution thoracic CT scan images. Definitive diagnosis is made by histological examination in previous. According to the 2015 ESC/ERS Guidelines, detection of a mutation in the eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) without histological confirmation is recommended to validate the diagnosis of PVOD...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27668765/diagnosis-of-veno-occlusive-disease-sinusoidal-obstruction-syndrome-of-the-liver-problems-of-interpretation
#14
L Volin, R Niittyvuopio, J Heiskanen, V Lindström, A Nihtinen, L Sahlstedt, T Ruutu
No abstract text is available yet for this article.
September 26, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27637324/-a-not-so-poorly-defined-opacity
#15
E Bravos, S Bayle-Bleuez, A Wahart, R Lachèze, F Forest, J-B Guichard, L Bertoletti
A 60-year-old patient was under follow-up for pulmonary hypertension consistent with pulmonary veno-occlusive disease. During his follow-up, a parenchymal opacity was discovered. We describe the management of the suspicion of lung cancer, highlighting the modification of the conventional diagnostic and therapeutic strategy on account of the pulmonary hypertension. Chest physicians should be able to adapt their diagnostic and therapeutic management in the case of neoplasia in patients with severe pulmonary hypertension...
September 13, 2016: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/27621751/inferior-vena-cava-stenosis-induced-sinusoidal-obstructive-syndrome-after-living-donor-liver-transplantation
#16
Batsaikhan Bat-Erdene, Sergelen Orgoi, Erdene Sandag, Ulzii-Orshikh Namkhai, Bat-Ireedui Badarch, Batsaikhan Batsuuri
The sinusoidal obstructive syndrome (SOS) is a complication that usually follows hematopoietic stem cell transplantation. It is also known as veno-occlusive disease, which is a rare complication of living donor liver transplantation (LDLT). Herein, we reported a 34 year-old female patient presenting SOS after LDLT. Its underlying cause was presumed to be associated with liver abscess and subsequent inferior vena cava stenosis. SOS led to graft failure, thus requiring retransplantation with a deceased donor liver graft...
August 2016: Korean Journal of Hepato-biliary-pancreatic Surgery
https://www.readbyqxmd.com/read/27603963/in-brief-defibrotide-defitelio-for-hepatic-veno-occlusive-disease
#17
(no author information available yet)
No abstract text is available yet for this article.
September 12, 2016: Medical Letter on Drugs and Therapeutics
https://www.readbyqxmd.com/read/27594906/veno-occlusive-disease-nurse-management-development-of-a-dynamic-monitoring-tool-by-the-gitmo-nursing-group
#18
Stefano Botti, Laura Orlando, Gianpaolo Gargiulo, Valentina De Cecco, Marina Banfi, Lorenzo Duranti, Emanuela Samarani, Maria Giovanna Netti, Marco Deiana, Vera Galuppini, Adriana Concetta Pignatelli, Rosanna Ceresoli, Alessio Vedovetto, Elena Rostagno, Marilena Bambaci, Cristina Dellaversana, Stefano Luminari, Francesca Bonifazi
Veno-occlusive disease (VOD) is a complication arising from the toxicity of conditioning regimens that have a significant impact on the survival of patients who undergo stem cell transplantation. There are several known risk factors for developing VOD and their assessment before the start of conditioning regimens could improve the quality of care. Equally important are early identification of signs and symptoms ascribable to VOD, rapid diagnosis, and timely adjustment of support therapy and treatment. Nurses have a fundamental role at the stages of assessment and monitoring for signs and symptoms; therefore, they should have documented skills and training...
2016: Ecancermedicalscience
https://www.readbyqxmd.com/read/27589851/renal-dysfunction-following-bone-marrow-transplantation
#19
Stephan Kemmner, Mareike Verbeek, Uwe Heemann
Acute kidney injury and long-term renal dysfunction are common problems following bone morrow transplantation (BMT) and highly related to mortality. The frequency and risk of renal dysfunction are directly related to the method of BMT, with myeloablative allogeneic BMT being associated to the highest risk followed by non-myeloablative allogeneic and myeloablative autologous BMT. The type of BMT is, thus, more important than co-factors such as advanced age, comorbidities, or high baseline serum creatinine. The causes for renal failure are multiple and include chemotherapy and high-dose radiation with fluid loss by diarrhea or vomiting, sepsis or nephrotoxic drugs such as calcineurin inhibitors and antimicrobials...
September 2, 2016: Journal of Nephrology
https://www.readbyqxmd.com/read/27587546/genetic-analyses-in-a-cohort-of-children-with-pulmonary-hypertension
#20
Marilyne Levy, Mélanie Eyries, Isabelle Szezepanski, Magalie Ladouceur, Sophie Nadaud, Damien Bonnet, Florent Soubrier
The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. The objective of this study was to determine the mutation frequency in PH genes in a paediatric cohort and describe the clinical characteristics of mutation carriers.The study involved 66 index cases with PH: 35 children with idiopathic pulmonary arterial hypertension (IPAH); five children with familial PAH (FPAH); three children with pulmonary veno-occlusive disease (PVOD); and 23 children with PAH associated with congenital heart disease (APAH-CHD)...
October 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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