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veno-occlusive disease guidelines

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)
Jean-Hugues Dalle, Sergio A Giralt
Hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome (SOS), can develop in a subset of patients, primarily after myeloablative hematopoietic stem cell transplantation, but it also may occur after reduced-intensity conditioning. Severe VOD/SOS, typically characterized by multiorgan failure, has been associated with a mortality rate greater than 80%. Therefore, an accurate and prompt diagnosis of VOD/SOS is essential for early initiation of appropriate therapy to improve clinical outcomes...
March 2016: Biology of Blood and Marrow Transplantation
Juthaporn Cowan, D W Cameron, Greg Knoll, Jason Tay
INTRODUCTION: Haematopoietic stem cell transplantation (HSCT) is commonly employed in the management of haematological malignancies. This intervention results in an increased risk of infectious and immune-related complications. Prophylactic immunoglobulin therapy has been used to prevent post-HSCT complications, including infections, with varying efficacy. We sought to update the current evidence supporting the use of immunoglobulins in the modern HSCT era. METHODS/ANALYSIS: Using a structured search strategy, we will perform a systematic review of the literature from MEDLINE, EMBASE and all EBM Reviews databases...
2015: BMJ Open
V Thakkar, M Nikpour, W M Stevens, S M Proudman
Pulmonary arterial hypertension (PAH) is a leading cause of morbidity and mortality in patients with systemic sclerosis (SSc). Approximately one in 10 will develop PAH during their lifetime. These patients have a worse prognosis than those with PAH due to other causes. The most common clinical feature of SSc-PAH in the early stages is non-specific exercise intolerance that can be erroneously attributed to other manifestations of SSc. Screening provides an opportunity for early identification of SSc-PAH and prompt initiation of therapies with the potential to improve quality of life and survival...
March 2015: Internal Medicine Journal
David W Fawkner-Corbett, Lisa Howell, Barry L Pizer, Carlo Dominici, Heather P McDowell, Paul D Losty
Wilms' tumor (WT) is a common childhood renal cancer. A 25-year single center UK experience is reported. During 1985-2010, 97 children underwent immediate nephrectomy or delayed resection of tumor after chemotherapy. Survival, morbidity, and late effects following treatment are described. Tumor distribution was: Stage I, 25.7% (n = 25); Stage II, 24.7% (n = 24); Stage III, 26.8% (n = 26); Stage IV, 17.5% (n = 17); and Stage V, 5.2% (n = 5). Immediate nephrectomy was performed in 39% (n = 38) patients with elective delayed resection in 61% (n = 59) cases...
August 2014: Pediatric Hematology and Oncology
Fiona L Dignan, Robert F Wynn, Nedim Hadzic, John Karani, Alberto Quaglia, Antonio Pagliuca, Paul Veys, Michael N Potter
DIAGNOSIS: It is recommended that the diagnosis of veno-occlusive disease (sinusoidal obstruction syndrome) [VOD (SOS)] be based primarily on established clinical criteria (modified Seattle or Baltimore criteria) (1A). Ultrasound imaging may be helpful in the exclusion of other disorders in patients with suspected VOD (SOS) (1C). It is recommended that liver biopsy be reserved for patients in whom the diagnosis of VOD (SOS) is unclear and there is a need to exclude other diagnoses (1C)...
November 2013: British Journal of Haematology
Michael Sohn, Martin Hatzinger, Irwin Goldstein, Sudhakar Krishnamurti
INTRODUCTION: The impact of penile blood supply on erectile function was recognized some 500 years ago. At the turn of the 20th century first results of penile venous ligation were published and in 1973 the first surgical attempts to restore penile arterial inflow were undertaken. Numerous techniques were published in the meantime, but inclusion criteria, patient selection, and success evaluation differed extremely between study groups. AIM: To develop evidence-based standard operating procedures (SOPs) for vascular surgery in erectile dysfunction, based on recent state of the art consensus reports and recently published articles in peer-reviewed journals...
January 2013: Journal of Sexual Medicine
Bibhas Kar, B Nandhini, R Revathi
We describe a child with Acute Myeloid Leukemia (AML M7) with trisomy 8 and ring chromosome 8. Ring chromosome 8 associated with AML is uncommon and is reported to have a poor outcome. The combination of trisomy 8 and ring chromosome 8 has not been previously reported. This 15-month-old girl had presented with a history of fever, weight loss of 1 kg, gum bleeds and pallor. Clinical examinations revealed no nodes or organomegaly. Investigations revealed pancytopenia and elevated serum LDH. Bone marrow aspirate confirmed the presence of myeloid blasts positive only for CD 41 and CD 61 on flow cytometry...
March 2009: Indian Journal of Hematology & Blood Transfusion
Andrei Seferian, Badia Helal, Xavier Jaïs, Barbara Girerd, Laura C Price, Sven Günther, Laurent Savale, Peter Dorfmüller, Florence Parent, Olivier Sitbon, Marc Humbert, Gérald Simonneau, David Montani
Pulmonary veno-occlusive disease (PVOD), a rare form of pulmonary arterial hypertension (PAH), requires histological proof for definitive diagnosis; however, lung biopsy is not recommended in PAH. Recent conjoint European Respiratory Society/European Society of Cardiology guidelines suggest that nonmatched perfusion defects on ventilation/perfusion (V'/Q') lung scanning in PAH patients may suggest PVOD. The aim of our study was to evaluate V'/Q' lung scans in a large cohort of PVOD and idiopathic or heritable PAH patients...
July 2012: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Norifumi Nakanishi
Pulmonary hypertension was defined as mean pulmonary artery pressure ≥ 25 mmHg at the 4th World Symposium on Pulmonary Hypertension. In 2009, the European Society of Cardiology and European Respiratory Society jointly created guidelines for practical pulmonary hypertension classifications and treatments based on the discussions at the 4th World Symposium. This classification is characterized by division into five groups: Pulmonary arterial hypertension (PAH); Pulmonary hypertension due to left heart disease; Pulmonary hypertension due to lung disease and/or hypoxia; Chronic thromboembolic pulmonary hypertension; and Pulmonary hypertension with unclear and/or multifactorial mechanisms...
December 2011: Allergology International: Official Journal of the Japanese Society of Allergology
Gregory A Broderick
INTRODUCTION: Priapism describes a persistent erection lasting longer than 4 hours. Ischemic priapism and stuttering priapism are phenotypic manifestations of sickle-cell disease (SCD). AIMS: To define the types of priapism associated with SCD, to address pathogenesis, and to recommend best practices. SOURCES: Literature review and published clinical guidelines. SUMMARY OF FINDINGS: Priapism is a full or partial erection that persists more than 4 hours...
January 2012: Journal of Sexual Medicine
L Arnaud, C Agard, J Haroche, P Cacoub, J-C Piette, Z Amoura
Pulmonary hypertension (PH) is a serious complication of connective tissue diseases. The prevalence of PH in systemic lupus erythematosus (SLE) ranges from 0.5 to 17.5%, depending on whether echocardiography or right heart catheterization is used as the gold standard for diagnosis. The recent guidelines for the diagnosis and treatment of pulmonary hypertension include several potential causes of PH in SLE, including: a primary vasculopathy similar to idiopathic pulmonary arterial hypertension (PAH); left heart diseases; post-thromboembolic disease; hypoxia and fibrosis resulting from interstitial lung disease; and the infrequent SLE-associated pulmonary veno-occlusive disease...
November 2011: La Revue de Médecine Interne
Marco Senzolo, Oliviero Riggio, Massimo Primignani
This review summarizes the document elaborated by the Italian Association for the Study of the Liver (AISF) ad hoc committee "Vascular disorders of the liver" on the primary circulatory liver diseases, which include Budd-Chiari syndrome, obstruction of the hepatic portion of the inferior vena cava, portal vein thrombosis, sinusoidal obstruction syndrome (veno-occlusive disease) and hereditary hemorrhagic telangiectasia. A characteristic of the primary circulatory liver diseases is that portal hypertension usually precedes liver dysfunction...
July 2011: Digestive and Liver Disease
D Montani, D S O'Callaghan, X Jaïs, L Savale, D Natali, A Redzepi, S Hoette, F Parent, O Sitbon, G Simonneau, M Humbert
Pulmonary hypertension (PH) comprises a heterogeneous group of disorders characterised by increased pulmonary vascular resistance that results in progressive right ventricular failure. In order to translate current evidence into routine clinical practice, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) have recently jointly proposed evidence-based guidelines for the optimal management of different PH patient groups. This article describes a series of clinical cases of PH due to various aetiologies that were referred to a large national PH expert referral centre...
December 2009: European Respiratory Review: An Official Journal of the European Respiratory Society
David Montani, Peter Dorfmuller, Sophie Maitre, Xavier Jaïs, Olivier Sitbon, Gérald Simonneau, Marc Humbert
The new classification of pulmonary hypertension proposed in the joint European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines, combines pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) from separate categories into a single subcategory within pulmonary arterial hypertension (PAH) because of specific similarities in their diagnosis, prognosis, and management. These diseases are characterized histologically by their predominant involvement of small pulmonary veins (PVOD) and capillaries (PCH)...
January 2010: La Presse Médicale
Nazzareno Galiè, Marius M Hoeper, Marc Humbert, Adam Torbicki, Jean-Luc Vachiery, Joan Albert Barbera, Maurice Beghetti, Paul Corris, Sean Gaine, J Simon Gibbs, Miguel Angel Gomez-Sanchez, Guillaume Jondeau, Walter Klepetko, Christian Opitz, Andrew Peacock, Lewis Rubin, Michael Zellweger, Gerald Simonneau
No abstract text is available yet for this article.
October 2009: European Heart Journal
Marlon G Saria, Tracy K Gosselin-Acomb
Hematopoietic stem cell transplantation (HSCT) is being used increasingly in the treatment of malignant and nonmalignant diseases. The treatment modality has been proven effective but is not without risks. Studies consistently have identified the need for advanced supportive care (e.g., multiple organ dysfunction, vasopressor use, mechanical ventilation) as a negative prognostic indicator in patients who have received HSCT. Among patients who have received HSCT, 15%-40% require critical care monitoring or advanced support...
February 2007: Clinical Journal of Oncology Nursing
Javier P Gisbert, Yago González-Lama, José Maté
The mean prevalence of azathioprine (AZA) or 6-mercaptopurine (MP)-induced liver injury in patients with inflammatory bowel disease was approximately 3%, and the mean annual drug-induced liver disorder rate was only 1.4%. However, this low figure calculated from retrospective studies contrasts with a much higher incidence (>10%) reported by a prospective study. Thiopurine-induced hepatotoxicity can be grouped into three syndromes: hypersensitivity, idiosyncratic cholestatic reaction, and endothelial cell injury (with resultant raised portal pressures, veno-occlusive disease, or peliosis hepatis)...
July 2007: American Journal of Gastroenterology
John Pryor, Emre Akkus, Gary Alter, Gerald Jordan, Thierry Lebret, Laurence Levine, John Mulhall, Sava Perovic, David Ralph, Walter Stackl
INTRODUCTION: There are three different types of priapism: low-flow, ischemic, anoxic or veno-occlusive priapism; high-flow, arterial or nonischemic priapism; and recurrent or stuttering priapism. AIM: To provide recommendations/guidelines concerning state-of-the-art knowledge for the diagnosis and treatment of priapism. METHODS: An International Consultation in collaboration with the major urology and sexual medicine associations assembled over 200 multidisciplinary experts from 60 countries into 17 committees...
July 2004: Journal of Sexual Medicine
Gurpreet Singh Gulati
No abstract text is available yet for this article.
April 2005: Tropical Gastroenterology: Official Journal of the Digestive Diseases Foundation
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