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pulmonary venoocclusive disease

Monika Szturmowicz, Aneta Kacprzak, Barbara Burakowska, Agnieszka Skoczylas, Iwona Bestry, Jan Kuś, Anna Fijałkowska, Adam Torbicki, Marcin Kurzyna
INTRODUCTION: Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some IPAH patients despite lack of interstitial lung disease. Such radiological changes are described either as ill-defined centrilobular nodules (CN) or as focal ground glass opacities (FGGO). There is no consensus in the literature, whether they indicate the distinct type of IPAH, or pulmonary venoocclusive disease (PVOD) with subtle radiological changes. Thus the aim of the present pilot study was to assess the frequency and clinical significance of inhomogenic lung attenuation pattern in IPAH...
2016: Pneumonologia i Alergologia Polska
David H MacIver, Ismail Adeniran, Iain R MacIver, Alistair Revell, Henggui Zhang
Pulmonary hypertension is usually related to obstruction of pulmonary blood flow at the level of the pulmonary arteries (eg, pulmonary embolus), pulmonary arterioles (idiopathic pulmonary hypertension), pulmonary veins (pulmonary venoocclusive disease) or mitral valve (mitral stenosis and regurgitation). Pulmonary hypertension is also observed in heart failure due to left ventricle myocardial diseases regardless of the ejection fraction. Pulmonary hypertension is often regarded as a passive response to the obstruction to pulmonary flow...
October 2016: American Heart Journal
Shakti K Bal, Balamugesh Thangakunam, Aparna Irodi, Mayank Gupta, Devasahayam J Christopher
Pulmonary venoocclusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) form a sinister subgroup of pulmonary arterial hypertension where the predominant pathology lies in the pulmonary veins and capillaries, thus making the use of pulmonary vasodilators potentially dangerous in these patients. Radiologically, the presence of centrilobular nodules, interlobular septal thickening, and significant mediastinal adenopathy are useful in identifying this subgroup from patients with pulmonary arterial hypertension...
September 10, 2016: Journal of Bronchology & Interventional Pulmonology
Mary C O'Keefe, Miriam D Post
Pulmonary capillary hemangiomatosis is a rare cause of pulmonary hypertension characterized by extensive proliferation of pulmonary capillaries within alveolar septae. Clinical presentation is nonspecific and includes dyspnea, cough, chest pain, and fatigue. Radiology shows diffuse centrilobular ground-glass opacities. Pulmonary capillary hemangiomatosis is clinically and radiographically indistinguishable from peripheral venoocclusive disease, making microscopic diagnosis essential. Histologically, pulmonary capillary hemangiomatosis shows an abnormal proliferation of small, thin-walled capillaries that expand the alveolar septae...
February 2015: Archives of Pathology & Laboratory Medicine
Neeraja Kambham, John P Higgins, Uma Sundram, Megan L Troxell
Hematopoietic stem cell transplantation (HCT), formerly known as bone marrow transplantation, is an integral part of treatment for many hematological malignancies. HCT is associated with several complications and comorbidities with differential effects on a wide spectrum of organs and tissues. We present an update on HCT-associated complications such as graft versus host disease (GVHD) and infection, with focus on the surgical pathology of the gastrointestinal (GI) tract, liver, and lung. Although the grading system for GI tract acute GVHD was proposed 40 years ago, recent studies have shed light on minimal histologic criteria for diagnosis of GVHD, as well as its differential diagnosis, including histologic effects of various medications...
September 2014: Advances in Anatomic Pathology
Cornelia Woerner, Ernest Cutz, Shi-Joon Yoo, Hartmut Grasemann, Tilman Humpl
BACKGROUND: Pulmonary venoocclusive disease (PVOD) is a rare lung disease, diagnosed in 5% to 10% of patients with pulmonary hypertension (PH). The incidence, prevalence, and etiology of PVOD in children are not well defined. The mortality remains high, related, at least partly, to the limited treatment options. METHODS: This retrospective analysis (1985-2011) summarizes symptoms, associated factors, treatment, and outcomes of nine pediatric patients (five girls, four boys) with histologic confirmation of PVOD...
July 2014: Chest
Walid Rasheed, Ardeshir Ghavamzadeh, Rosemarie Hamladji, Tarek Ben Othman, Amal Alseraihy, Fawzi Abdel-Rahman, Alaa Elhaddad, Abdulaziz Alabdulaaly, David Dennison, Ahmad Ibrahim, Ali Bazarbachi, Mohamed-Amine Bekadja, Said Yousuf Mohamed, Salman Naseem Adil, Parvez Ahmed, Said Benchekroun, Mani Ramzi, Mohammad Jarrar, Kamran Alimoghaddam, Fazal Hussain, Amir Hamidieh, Mahmoud Aljurf
INTRODUCTION: This practice survey is conducted to analyze clinical hematopoietic stem cell transplantation (HSCT) practice variability among centers in the WHO Eastern Mediterranean Region (EMRO), as represented by the Eastern Mediterranean Blood and Marrow Transplantation (EMBMT) group. METHOD: This internet based survey was completed by the medical program directors of the EMBMT centers; 17 centers participated. The survey collected data on various clinical aspects of HSCT practice...
March 2013: Hematology/oncology and Stem Cell Therapy
Alexandra E Kovach, George Z Cheng, Colleen L Channick, Richard N Channick, Ashok Muniappan, Henning A Gaissert, Richard L Kradin
Radiofrequency ablation of pulmonary veins is a common therapeutic intervention for atrial fibrillation. Pulmonary vein stenosis and venoocclusive disease are recognized complications, but the spectrum of pathologies postablation have not been previously reviewed. A recent case at our hospital showed a left hilar soft tissue mass in association with superior pulmonary vein stenosis in a patient 4 years postablation. On resection, this proved to be an inflammatory pseudotumor composed of myofibroblasts in an organizing pneumonia-type pattern with adjacent dendriform ossifications...
October 2013: Annals of Diagnostic Pathology
Koji Takahashi, Fengshi Chen, Tadashi Ikeda, Hiraku Doi, Hideaki Nakamura, Sadamitsu Yanagi, Masaaki Sato, Akihiro Aoyama, Toru Bando, Hiroshi Date
Pulmonary venoocclusive disease, classified as a subgroup of pulmonary arterial hypertension, is known to show poor prognosis and lung transplantation is the only possible treatment. Single living-donor lobar lung transplantation is a unique method of treatment, mostly for small children, and size matching is the most important factor to conduct single living-donor lobar lung transplantation safely. We report a successful single living-donor lobar lung transplantation for a 6-year-old girl with pulmonary venoocclusive disease who received the graft from her mother...
February 2013: Annals of Thoracic Surgery
Rebecca A Marsh, Kanchan Rao, Prakash Satwani, Kai Lehmberg, Ingo Müller, Dandan Li, Mi-Ok Kim, Alain Fischer, Sylvain Latour, Petr Sedlacek, Vincent Barlogis, Kazuko Hamamoto, Hirokazu Kanegane, Sam Milanovich, David A Margolis, David Dimmock, James Casper, Dorothea N Douglas, Persis J Amrolia, Paul Veys, Ashish R Kumar, Michael B Jordan, Jack J Bleesing, Alexandra H Filipovich
There have been no studies on patient outcome after allogeneic hematopoietic cell transplantation (HCT) in patients with X-linked inhibitor of apoptosis (XIAP) deficiency. To estimate the success of HCT, we conducted an international survey of transplantation outcomes. Data were reported for 19 patients. Seven patients received busulfan-containing myeloablative conditioning (MAC) regimens. Eleven patients underwent reduced intensity conditioning (RIC) regimens predominantly consisting of alemtuzumab, fludarabine, and melphalan...
February 7, 2013: Blood
Anatoly Urisman, Lorriana E Leard, Manjari Nathan, Brett M Elicker, Charles Hoopes, Jasleen Kukreja, Kirk D Jones
Pulmonary venoocclusive disease (PVOD) is a rare cause of pulmonary hypertension characterized by a progressive clinical course and poor outcomes if not treated by early lung transplantation. The pathogenesis of PVOD remains poorly understood. We report PVOD that developed in 2 young women soon after the initiation of oral contraceptives (OCs). The first patient is a 14-year-old girl, with no medical history, who started taking an OC 3 weeks before the onset of symptoms. The second patient is an 18-year-old girl, diagnosed 2 years previously with systemic lupus erythematosus and lupus anticoagulant, who started taking an OC 4 months before the onset of symptoms...
September 2012: Journal of Heart and Lung Transplantation
S Günther, X Jaïs, S Maitre, A Bérezné, P Dorfmüller, A Seferian, L Savale, O Mercier, E Fadel, O Sitbon, L Mouthon, G Simonneau, M Humbert, D Montani
OBJECTIVE: Pulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) characterized by obstruction of small pulmonary veins. Pulmonary venous involvement has been reported in pathologic assessment of patients with systemic sclerosis (SSc) presenting with precapillary PH. High-resolution computed tomography (HRCT) of the chest is a noninvasive diagnostic tool used to screen for PVOD. No HRCT data are available on SSc patients with precapillary PH. We undertook this study to evaluate the frequency and effect on prognosis of HRCT signs of PVOD in SSc patients with precapillary PH...
September 2012: Arthritis and Rheumatism
Anthony Azakie, Michael J Lavrsen, Natalie C Johnson, Anil Sapru
BACKGROUND: The "sutureless" repair technique has improved outcomes for post-repair pulmonary vein (PV) stenosis. The purpose of this study is to determine the early outcomes of primary sutureless repair of pulmonary venoocclusive disease in infants with congenital PV stenosis-hypoplasia or PVs at high risk for progressive stenosis. METHODS: This is a retrospective review of infants who had primary sutureless repair of the PVs from October 2002 to April 2010. RESULTS: Twenty-five infants had primary sutureless repair of the PVs...
August 2011: Annals of Thoracic Surgery
Vichaya Arunthari, Charles D Burger
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is an important cause of severe pulmonary hypertension (PH). D-dimer, a degradation product of fibrin, has been used as a marker for various diseases. In patients with idiopathic pulmonary arterial hypertension there is evidence to suggest that D-dimer levels are associated with disease severity; however, data regarding D-dimer in patients with CTEPH are lacking. OBJECTIVE: To assess the significance of D-dimer in patients with CTEPH...
2009: Open Respiratory Medicine Journal
Elzbieta Stańczak, Katarzyna Pawelec, Michał Romiszewski
Lymphoproliferative disorders (LPD) occur often in EBV-infected patients, especially in solid-organ and haematopoietic stem cell transplant recipients. The risk of developing LPD ranges from 1 to 25% and depends on the type of transplantation. We are presenting the case of a 9-year-old boy with acute myelogenous leukaemia in second remission, who developed LPD after matched unrelated donor bone marrow transplantation (MUD BMT) not identical in two loci. On day 50 after BMT the patient presented with fever and symptoms of paronychia...
October 2008: Medycyna Wieku Rozwojowego
Manuel López-Meseguer, Antonio Román, Víctor Monforte, Carlos Bravo, Joan Solé, Ferran Morell
BACKGROUND: Pulmonary hypertension is a serious disease that, in its terminal phase, requires lung transplantation. PATIENTS AND METHODS: A retrospective study was undertaken of 15 patients with pulmonary hypertension who underwent lung transplantation between 1994 and 2004. Clinical data recorded before the procedure and during follow-up were reviewed. RESULTS: Pulmonary hypertension was reported as idiopathic in 8 patients (53%) and related to consumption of toxic oil in 2...
August 2009: Archivos de Bronconeumología
Susan Harch, Helen Whitford, Catriona McLean
BACKGROUND: Despite the development of targeted therapies for pulmonary arterial hypertension (PAH), some patients fail to respond to medical therapy. There are a number of types of PAH, one of which is pulmonary venoocclusive disease (PVOD). Unlike other PAH types, the hallmark pathology of PVOD is fibrous occlusion of the small venules, potentially with arterial involvement. It was hypothesized that a lack of response to medical therapy in clinically diagnosed PAH can be explained by misdiagnosed PVOD...
June 2009: Chest
Erhan Akpinar, Burcu Akpinar, Baris Turkbey, Ozgur Deren, Macit Ariyurek
Pulmonary hypertension secondary to pulmonary venoocclusive disease (PVOD) is increasingly recognized (Wagenvoort, Chest 69:82-86, [20]; Scully et al., N Engl J Med 308:823-834, [21]). The clinical presentation is usually progressive pulmonary hypertension. It should be kept in mind when there is pulmonary arterial hypertension, pulmonary edema, and a normal pulmonary artery wedge pressure. Importance of diagnosing this condition is to protect patient from fatal pulmonary edema when using prostacyclins that are effective for treatment of primary pulmonary hypertension...
May 2008: Emergency Radiology
Peter Dorfmüller, Marc Humbert, Frédéric Perros, Olivier Sanchez, Gérald Simonneau, Klaus-Michael Müller, Frédérique Capron
Pulmonary arterial hypertension is a severe complication of connective tissue diseases. It is currently well established that pulmonary arterial hypertension associated with connective tissue diseases such as systemic sclerosis is frequently less responsive or even refractory to pulmonary vasodilator therapies. In that setting, pulmonary venoocclusive disease is believed to contribute to treatment failures. We therefore hypothesized that pulmonary arterial hypertension associated with connective tissue diseases may be associated with obstructive lesions of pulmonary veins...
June 2007: Human Pathology
D Bendayan, R Sarid, A Cohen, D Shitrit, I Shechtman, M R Kramer
BACKGROUND: Pulmonary hypertension is a severe pulmonary vascular disease leading to rapid deterioration and death. Histological and clinical evidence suggests that smooth muscle proliferation is part of the pathogenesis of the disease. Human herpesvirus 8 (HHV-8) is a gamma-herpesvirus that is implicated in malignancies and in Kaposi's sarcoma. Recently, the association of HHV-8 with idiopathic pulmonary arterial hypertension (PAH) has been found. OBJECTIVE: The aim of this study was to investigate the presence of HHV-8 in the lung tissue of Israeli patients with PAH...
2008: Respiration; International Review of Thoracic Diseases
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