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Veno-occlusive disease

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https://www.readbyqxmd.com/read/29895929/hepatic-veno-occlusive-disease-following-sirolimus-based-immune-suppression
#1
Farhad Khimani, George B McDonald, Howard M Shulman, Brian Betts, Frederick Locke, Hugo Fernandez, Claudio Anasetti, Joseph Pidala
Sirolimus-based graft vs. host disease (GVHD) prophylaxis is associated with higher incidence of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) after allogeneic hematopoietic cell transplantation (HCT). However, whether the clinical manifestations and prognosis of VOD/SOS differs when diagnosed in the setting of sirolimus-based GVHD prophylaxis is not well studied. To address this question, we examined presenting features and treatment outcome of VOD/SOS cases identified in a large retrospective cohort of consecutive HCT procedures (n = 818 total, sirolimus (SIR)/tacrolimus (TAC) n = 308, and methotrexate (MTX) or mycophenolate mofetil (MMF)/TAC n = 510)...
June 12, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29889889/adipose-tissue-content-of-alpha-linolenic-acid-and-the-risk-of-ischemic-stroke-and-ischemic-stroke-subtypes-a-danish-case-cohort-study
#2
Christian Sørensen Bork, Stine Krogh Venø, Søren Lundbye-Christensen, Marianne Uhre Jakobsen, Anne Tjønneland, Philip C Calder, Kim Overvad, Erik Berg Schmidt
BACKGROUND: The plant-derived omega-3 fatty acid alpha-linolenic acid (ALA) may reduce the risk of cardiovascular disease. OBJECTIVE: We have investigated associations between the content of ALA in adipose tissue and the risk of ischemic stroke and its subtypes. METHODS: Incident cases of ischemic stroke among participants enrolled into the Danish Diet, Cancer and Health cohort (n = 57,053) were identified by linkage with the Danish National Patient Register...
2018: PloS One
https://www.readbyqxmd.com/read/29886475/-pulmonary-veno-occlusive-disease-pulmonary-capillary-hemangiomatosis-a-case-report-and-literature-review
#3
Xianliang Xiong, Tengteng Zhu, Jiang Li
Pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary vascular disease that causes pulmonary arterial hypertension. The diagnosis of PVOD/PCH can be established by the combination of clinical features, physical examination, radiological findings, lung function, bronchoscopy and other resources. There is no established medical therapy for PVOD/PCH, and the only curative therapy for PVOD/PCH is lung transplantation. A girl with PVOD/PCH was diagnosed in the Second Xiangya Hospital...
May 28, 2018: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/29880377/lung-transplantation-in-pulmonary-hypertension-a-multidisciplinary-unit-s-management-experience
#4
C A Quezada-Loaiza, A de Pablo Gafas, V Pérez, R Alonso, L Juarros, M I Real, E López, M Cortes, J C Meneses, I H González, V Díaz-Hellín Gude, P E Subías, P Gámez
BACKGROUND: Prognosis assessment of pulmonary hypertension (PH) is multifactorial and placement of patients on the lung transplantation (LT) waiting list requires the weighing of a complex set of criteria. The aim of this retrospective cohort study was to analyze a series of patients treated in our unit at the moment of their inclusion on the LT waiting list and long-term survival after LT. MATERIAL AND METHODS: Baseline characteristics, LT outcomes, and survival were evaluated in all patients diagnosed with pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD) who were included on the LT waiting list in 2011-2016...
June 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29873895/defibrotide-for-the-treatment-of-hepatic-veno-occlusive-disease-sinusoidal-obstruction-syndrome-following-nontransplant-associated-chemotherapy-final-results-from-a-post-hoc-analysis-of-data-from-an-expanded-access-program
#5
Nancy A Kernan, Paul G Richardson, Angela R Smith, Brandon M Triplett, Joseph H Antin, Leslie Lehmann, Yoav Messinger, Wei Liang, Robin Hume, William Tappe, Robert J Soiffer, Stephan A Grupp
BACKGROUND: Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially fatal complication of conditioning for hematopoietic stem cell transplantation (HSCT) but can occur after nontransplant-associated chemotherapy. Following HSCT, VOD/SOS with multi-organ dysfunction (MOD) may be associated with >80% mortality. Defibrotide is approved to treat severe hepatic VOD/SOS post-HSCT in patients aged >1 month in the European Union and hepatic VOD/SOS with renal or pulmonary dysfunction post-HSCT in the United States...
June 6, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29804176/pulmonary-capillary-hemangiomatosis-diagnosed-by-pathology-of-explanted-lungs-a-unique-etiology-serves-as-a-key-of-clinical-diagnosis
#6
Hironobu Wada, Takahiro Nakajima, Hidemi Suzuki, Rie Anazawa, Tomoharu Narita, Jiro Terada, Shigetoshi Yoshida, Koichiro Tatsumi, Yukio Nakatani, Ichiro Yoshino
A 27-year-old female patient had presented progressing exertional dyspnea due to pulmonary hypertension. Chest CT revealed diffusely spread patchy ground-glass opacities sparing subpleural parenchymal areas suggesting the diagnosis of pulmonary veno-occlusive disease (PVOD). Despite the diagnosis of PVOD, she was somehow managed by a repetitive escalation of the epoprostenol dose and oxygen supply during the 12-month waiting period until successful bilateral lung transplantation was performed. Pathology demonstrated capillary proliferation in alveolar septae with scarce lesions of narrowed and/or occluded postcapillary small veins, leading to the final diagnosis of pulmonary capillary hemangiomatosis (PCH), not PVOD...
May 26, 2018: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29803752/a-novel-ultrasonographic-scoring-system-of-sinusoidal-obstruction-syndrome-after-hematopoietic-stem-cell-transplantation
#7
Mutsumi Nishida, Kaoru Kahata, Eiko Hayase, Akio Shigematsu, Megumi Sato, Yusuke Kudo, Satomi Omotehara, Takahito Iwai, Junichi Sugita, Hitoshi Shibuya, Chikara Shimizu, Takanori Teshima
BACKGROUND: Sinusoidal obstruction syndrome (SOS)/hepatic veno-occlusive disease (VOD) is a well-documented complication after hematopoietic stem cell transplantation (HSCT). Transabdominal ultrasonography (US) enables to visualize blood flow abnormalities and is therefore useful for the diagnosis of SOS/VOD. We herein prospectively evaluated accuracy of a novel ultrasonographic diagnostic scoring system of SOS/VOD based on US findings. METHODS: We carried out US in 106 patients on day 14 and when SOS/VOD was suspected after allogeneic HSCT...
May 24, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29787320/gemtuzumab-ozogamicin-for-the-treatment-of-acute-myeloid-leukemia
#8
Jeffrey Baron, Eunice S Wang
Gemtuzumab ozogamicin (GO) is an antibody-drug conjugate consisting of a monoclonal antibody targeting CD33 linked to a cytotoxic derivative of calicheamicin. Despite the known clinical efficacy in relapsed/refractory acute myeloid leukemia (AML), GO was withdrawn from the market in 2010 due to increased early deaths seen in newly diagnosed AML patients receiving GO + intensive chemotherapy. In 2017, new data on the clinical efficacy and safety of GO administered on a fractionated dosing schedule led to re-approval for newly diagnosed and relapsed/refractory AML...
May 22, 2018: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/29770447/effectiveness-of-immunoglobulin-prophylaxis-in-reducing-clinical-complications-of-hematopoietic-stem-cell-transplantation-a-systematic-review-and-meta-analysis
#9
REVIEW
Hilalion Ahn, Jason Tay, Beverley Shea, Brian Hutton, Risa Shorr, Greg A Knoll, Donald William Cameron, Juthaporn Cowan
BACKGROUND: Prophylactic immunoglobulin has been used with varying efficacy to reduce complications in hematopoietic stem cell transplant recipients. STUDY DESIGN AND METHODS: A systematic review and meta-analysis was conducted of randomized controlled trials that assessed clinical outcomes (overall survival, transplant-related mortality, graft-versus-host disease [GVHD], veno-occlusive disease [VOD], interstitial pneumonitis, disease relapse, cytomegalovirus [CMV] infection and disease, non-CMV infection) of immunoglobulin prophylaxis versus placebo in hematopoietic stem cell transplant recipients...
May 16, 2018: Transfusion
https://www.readbyqxmd.com/read/29767845/final-results-from-a-defibrotide-treatment-ind-study-for-patients-with-hepatic-veno-occlusive-disease-sinusoidal-obstruction-syndrome
#10
Nancy A Kernan, Stephan Grupp, Angela R Smith, Sally Arai, Brandon Triplett, Joseph H Antin, Leslie Lehmann, Tsiporah Shore, Vincent T Ho, Nancy Bunin, Massimo Iacobelli, Wei Liang, Robin Hume, William Tappe, Robert Soiffer, Paul Richardson
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication of haematopoietic stem cell transplant (HSCT) conditioning and chemotherapy. Defibrotide is approved for treatment of hepatic VOD/SOS with pulmonary or renal dysfunction [i.e., multi-organ dysfunction (MOD)] after HSCT in the United States and severe VOD/SOS after HSCT in patients aged older than 1 month in the European Union. Defibrotide was available as an investigational drug by an expanded-access treatment programme (T-IND; NCT00628498)...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29751956/relation-of-postexercise-reduction-of-arterial-blood-pressure-and-erectile-dysfunction-in-patients-with-coronary-heart-disease
#11
Dariusz Kałka, Jana Gebala, Lesław Rusiecki, Ryszard Smoliński, Jakub Dulanowski, Małgorzata Rusiecka, Anna Biełous-Wilk, Witold Pilecki, Krzysztof Womperski, Romuald Zdrojowy
Penile erection is a hemodynamic process consisting of 2 synchronized components in which the first (active) requires proper vascular endothelium functioning, whereas the second one (passive) is based on a veno-occlusive mechanism. Antihypertensive treatment reduces the passive component, often leading to the development of erectile dysfunction (ED), but lifestyle modifications can improve the sexual functioning. The study aimed to evaluate the association between blood pressure (BP) reduction caused by cardiovascular training and the intensity of ED in men with coronary heart disease...
April 18, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29750747/haploidentical-donor-transplantation-using-a-novel-clofarabine-containing-conditioning-regimen-for-very-high-risk-hematologic-malignant-neoplasms
#12
Akshay Sharma, Guolian Kang, Anusha Sunkara, Hiroto Inaba, Sima Jeha, Shane J Cross, Terrence Geiger, Brandon Triplett
Recurrent/refractory hematologic malignancies have a poor prognosis, and there is a need for novel treatment regimens that can be tolerated by this heavily pretreated patient group. Clofarabine has antileukemic activity with an acceptable toxicity profile. In a phase I clinical trial (NCT00824135), we substituted clofarabine for fludarabine in a well-established reduced-intensity conditioning regimen for a T cell-depleted, mismatched-related (haploidentical) donor transplant backbone and explored the maximum tolerated dose of clofarabine in this combination in 15 patients undergoing hematopoietic cell transplantation for recurrent/refractory or secondary leukemia...
May 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29743074/genetic-analyses-in-a-cohort-of-191-pulmonary-arterial-hypertension-patients
#13
Hang Yang, Qixian Zeng, Yanyun Ma, Bingyang Liu, Qianlong Chen, Wenke Li, Changming Xiong, Zhou Zhou
BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder associated with high pulmonary artery pressure. Genetic testing enables early diagnosis and offers an opportunity for family screening. To identify genetic mutations and help make a precise diagnosis, we performed genetic testing in 191 probands with PAH and tried to analyze the genotype-phenotype correlation. METHODS: Initially, PAH samples (n = 119) were submitted to BMPR2 screening using Sanger sequencing...
May 9, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29715079/sinusoidal-obstruction-syndrome-veno-occlusive-disease-following-hematopoietic-stem-cell-transplant-insights-and-therapeutic-advances
#14
REVIEW
Samah Nassereddine, Saud Alsubait, Imad Tabbara
Hepatic sinusoidal obstruction syndrome (SOS) is a rare fatal clinical entity seen following hematopoietic stem cell transplant (HSCT). It is more commonly reported to occur following allogeneic HSCT compared to autologous HSCT. Historically, it is known as hepatitis following HSCT. It is thought that endothelial damage to the hepatic venules leading to occlusion of the terminal hepatic venules and hepatic sinusoids is the trigger for the development of SOS. Several risk factors have been associated with this condition...
May 2018: Anticancer Research
https://www.readbyqxmd.com/read/29703039/hepatic-veno-occlusive-disease-related-to-gynura-segetum-a-case-report
#15
Ziyi Sun, Jianmei Kang, Yongchang Zhang
INTRODUCTION: Hepatic veno-occlusive disease (HVOD), as known as hepatic sinusoidal obstruction syndrome (HSOS), is an obliterative venulitis of the terminal hepatic venules, which is responsible for considerable mortality. The potential mechanism is destruction of hepatic sinusoidal endothelial cells (SEC), with sloughing and downstream occlusion of terminal hepatic venules. Here, we report a case of HVOD who have a history of ingestion of Gynura segetum for 1 month. The patient presents for abdominal pain and distension...
April 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29680515/safety-and-efficacy-of-once-daily-intravenous-busulfan-in-allogeneic-transplantation-a-matched-pair-analysis
#16
Shinichi Kako, Shinichiro Fujiwara, Miki Sato, Shun-Ichi Kimura, Hideki Nakasone, Kazuteru Ohashi, Toshiro Kawakita, Tetsuo Maeda, Takanobu Morishita, Ritsuro Suzuki, Takahiro Fukuda, Tatsuo Ichinohe, Mio Kurata, Yoshiko Atsuta, Yoshinobu Kanda
Compared with 4-times-daily infusion of intravenous busulfan (ivBU4), the safety and efficacy of once-daily infusion of ivBU (ivBU1) has not been fully clarified. We have been routinely using ivBU1 in a conditioning regimen in adult patients with myeloid malignancy who undergo allogeneic hematopoietic stem cell transplantation. In this study, a total of 91 patients who received ivBU1 for 2 days (n = 18) or 4 days (n = 73) in our institutions were compared with 273 control patients who received ivBU4, who were matched for age, sex, performance status, disease risk, conditioning regimen, and donor type, selected from the database of the Japanese Society for Hematopoietic Cell Transplantation using optimal matching algorithms...
April 19, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29650683/fda-approval-summary-mylotarg-for-treatment-of-patients-with-relapsed-or-refractory-cd33-positive-acute-myeloid-leukemia
#17
Kelly J Norsworthy, Chia-Wen Ko, Jee Eun Lee, Jiang Liu, Christy S John, Donna Przepiorka, Ann T Farrell, Richard Pazdur
On September 2, 2017, the U.S. Food and Drug Administration approved gemtuzumab ozogamicin (GO; Mylotarg; Pfizer, New York City, NY) for treatment of relapsed or refractory (R/R) CD33-positive acute myeloid leukemia (AML) in patients 2 years of age and older. GO is a CD33-directed antibody drug conjugate linked to the cytotoxic antibiotic calicheamicin. It originally received accelerated approval for treatment of older patients with relapsed CD33-positive AML in 2000, but it was withdrawn from the market in 2010 when the confirmatory trial failed to demonstrate clinical benefit among safety concerns, such as a higher rate of induction fatalities on the GO combination arm compared with chemotherapy alone...
April 12, 2018: Oncologist
https://www.readbyqxmd.com/read/29628135/lung-transplantation-for-scleroderma-lung-disease-an-international-multicenter-observational-cohort-study
#18
Pauline Pradère, Igor Tudorache, Jesper Magnusson, Laurent Savale, Olivier Brugiere, Benoît Douvry, Martine Reynaud-Gaubert, Johanna Claustre, Aurélie Le Borgne, Are M Holm, Hans Henrik Schultz, Christiane Knoop, Laurent Godinas, Andrew J Fisher, Sandrine Hirschi, Jens Gottlieb, Jérôme Le Pavec
BACKGROUND: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Our objective in this study was to assess the indications, survival, and prognostic factors of lung or heart-lung transplantation for scleroderma lung disease...
March 15, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29562469/-treatment-of-four-cases-of-fanconi-anemia-by-allogeneic-hematopoietic-stem-cell-transplantation-with-low-intensity-conditional-regimen
#19
H Hou, Y H Yao, J Lu, P F Xiao, X N Bian, H Liu, D X Hu, J Ling, J Li, Z Zhai, L J Kong, S Y Hu
Objective: To evaluate the efficiency and safety of low intensity conditional regimen for children with Fanconi anemia (FA) receiving allogenic hematopoietic stem cells transplantation (allo-HSCT). Methods: Four patients diagnosed as Fanconi anemia were enrolled in this study. One patient received HLA-identical sibling donor hematopoietic stem cell transplantation, two patients underwent unrelated donor matched (UD) HSCT, and one patient received unrelated cord blood transplantation. The conditional regimen consisted of Busulfan with low dose of cyclophosphamide...
March 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29548663/clinical-prediction-score-for-identifying-patients-with-pulmonary-veno-occlusive-disease-pulmonary-capillary-hemangiomatosis
#20
Aiko Ogawa, Yukari Takahashi, Hiromi Matsubara
BACKGROUND: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension. Although diagnosis is based on pathological findings, an early diagnosis is crucial because of poor prognosis compared to other types of pulmonary hypertension. Furthermore, vasodilators may cause fatal pulmonary edema in patients with PVOD/PCH. This study aimed to identify specific characteristics for patients with PVOD/PCH to clinically diagnose PVOD/PCH...
March 13, 2018: Journal of Cardiology
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