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Pier Mannuccio Mannucci - One of the best experts on this subject based on the ideXlab platform.
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von willebrand disease associated Angiodysplasia a few answers still many questions
British Journal of Haematology, 2013Co-Authors: Massimo Franchini, Pier Mannuccio MannucciAbstract:Summary The association between Angiodysplasia and von Willebrand disease (VWD) has been known for more than 40 years. Bleeding in the gastrointestinal tract associated with Angiodysplasia worsens the clinical course of this inherited haemorrhagic disorder and management may become difficult and challenging. Angiodysplasia associated with acquired defects or dysfunctions of von Willebrand factor (VWF) has also been reported in a variety of conditions such as monoclonal gammopathies, Heyde syndrome and in carriers of ventricular assist devices. The most recent advances concerning the mechanistic, clinical and therapeutic aspects of VWD-associated Angiodysplasia are summarized in this review, together with the limitations of our knowledge that warrant further research in the frame of international cooperation.
S Naveau - One of the best experts on this subject based on the ideXlab platform.
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abnormal von willebrand factor in bleeding Angiodysplasias of the digestive tract
Gastroenterology, 2001Co-Authors: Agnès Veyradier, Vincent Giraud, Sarah Montembault, Bernadette Obert, Ibrahim Dagher, John C. Chaput, D. Meyer, M. Wolf, Axel Balian, S NaveauAbstract:Abstract Background & Aims: Involvement of an abnormal von Willebrand factor in the bleeding expression of gastrointestinal Angiodysplasias has been suggested but not assessed by prospective studies. Methods: To address this issue, 27 patients with either nonbleeding (group A, n=9) or bleeding (group B, n=9) digestive Angiodysplasias or telangiectasias or diverticular hemorrhage (group C, n=9) were enrolled. In all patients, an analysis of von Willebrand factor and a screening for the most common disorders associated with an acquired von Willebrand disease were performed. Results: In all patients from groups A and C, von Willebrand factor was normal, and no underlying disease could be found. In contrast, all but 1 patient from group B had a variable selective loss of the largest multimeric forms of von Willebrand factor, associated in 7 cases with a stenosis of the aortic valve. Conclusions: This study indicates that most patients with bleeding Angiodysplasia or telangiectasia have a deficiency of the largest multimers of von Willebrand factor induced by a latent acquired von Willebrand disease. Because these multimers are the most effective in promoting primary hemostasis at the very high shear conditions related to these vascular malformations, we suggest that their deficiency is likely to contribute to the bleeding diathesis. GASTROENTEROLOGY 2001;120:346-353
Shajan Peter - One of the best experts on this subject based on the ideXlab platform.
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Somatostatin Analogues in the Treatment of Recurrent Bleeding from Gastrointestinal Vascular Malformations: An Overview and Systematic Review of Prospective Observational Studies
Digestive Diseases and Sciences, 2010Co-Authors: Colin Brown, Venkataraman Subramanian, C. Mel Wilcox, Shajan PeterAbstract:Background Vascular malformation of the gastrointestinal tract is an uncommon cause for gastrointestinal bleeding. Traditionally, gastroenterologists prefer to use endoscopic modalities like argon plasma coagulation and electrocoagulation to treat accessible lesions. The role of somatostatin analogues (octreotide) in preventing recurrent bleeding in these patients is unclear. The use of pharmacological treatments would be useful especially in refractory bleeding, inaccessible lesions and in patients who are at high risk for invasive interventions. Aims To systematically review pooled clinical response rates from prospective studies using somatostatin analogues for prevention of recurrent bleeding from gastrointestinal Angiodysplasia and quantify the effects that therapy has on the use of blood transfusions. Methods We searched several electronic databases including Pubmed for full journal articles published after 1966 reporting on the use of somatostatin analogues in the treatment of gastrointestinal angiodyplasia. We hand searched the reference lists of all retrieved articles. Prospective studies involving ten or more patients were included in the analysis. We calculated the pooled proportion of patients who had a clinical response to therapy in the selected studies and the weighted mean difference in transfusion requirements before and after therapy. Heterogeneity between the studies was assessed using the I ^2 statistic. Results A total of three studies involving 62 patients met the inclusion criteria. The proportional meta-analysis showed a clinical response to treatment of 0.76 (95% CI 0.64–0.85). The weighted mean difference in transfusion requirements before starting therapy (control group) and after treatment initiation (treatment group) was −2.2 (95% CI −3.9 to −0.5). No significant heterogeneity was seen between the studies. Conclusions A significant number of patients with bleeding gastrointestinal Angiodysplasia respond to treatment with octreotide by reducing the need for blood products. As all the included studies had small sample sizes, multicenter randomized trials are needed to confirm these findings. However, it seems reasonable to administer octreotide especially in patients with refractory bleeding, inaccessible lesions and in patients at high risk for other interventions.
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somatostatin analogues in the treatment of recurrent bleeding from gastrointestinal vascular malformations an overview and systematic review of prospective observational studies
Digestive Diseases and Sciences, 2010Co-Authors: Colin Brown, Venkataraman Subramanian, Mel C Wilcox, Shajan PeterAbstract:Vascular malformation of the gastrointestinal tract is an uncommon cause for gastrointestinal bleeding. Traditionally, gastroenterologists prefer to use endoscopic modalities like argon plasma coagulation and electrocoagulation to treat accessible lesions. The role of somatostatin analogues (octreotide) in preventing recurrent bleeding in these patients is unclear. The use of pharmacological treatments would be useful especially in refractory bleeding, inaccessible lesions and in patients who are at high risk for invasive interventions. To systematically review pooled clinical response rates from prospective studies using somatostatin analogues for prevention of recurrent bleeding from gastrointestinal Angiodysplasia and quantify the effects that therapy has on the use of blood transfusions. We searched several electronic databases including Pubmed for full journal articles published after 1966 reporting on the use of somatostatin analogues in the treatment of gastrointestinal angiodyplasia. We hand searched the reference lists of all retrieved articles. Prospective studies involving ten or more patients were included in the analysis. We calculated the pooled proportion of patients who had a clinical response to therapy in the selected studies and the weighted mean difference in transfusion requirements before and after therapy. Heterogeneity between the studies was assessed using the I 2 statistic. A total of three studies involving 62 patients met the inclusion criteria. The proportional meta-analysis showed a clinical response to treatment of 0.76 (95% CI 0.64–0.85). The weighted mean difference in transfusion requirements before starting therapy (control group) and after treatment initiation (treatment group) was −2.2 (95% CI −3.9 to −0.5). No significant heterogeneity was seen between the studies. A significant number of patients with bleeding gastrointestinal Angiodysplasia respond to treatment with octreotide by reducing the need for blood products. As all the included studies had small sample sizes, multicenter randomized trials are needed to confirm these findings. However, it seems reasonable to administer octreotide especially in patients with refractory bleeding, inaccessible lesions and in patients at high risk for other interventions.
Joost P. H. Drenth - One of the best experts on this subject based on the ideXlab platform.
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Risk factors for incidentally detected and symptomatic Angiodysplasias: a case-control study with the general population as reference.
European journal of gastroenterology & hepatology, 2019Co-Authors: Katherina V Grooteman, Shelley Dalloyaux, Marjon C P Van Den Bemt, Jacqueline De Graaf, André L. M. Verbeek, Christian S Jackson, Erwin J M Van Geenen, Joost P. H. DrenthAbstract:Background There is no literature on risk factors for incidentally found Angiodysplasias. In clinical practice, endoscopists may defer treatment owing to uncertainty about a causal role of any found Angiodysplasia and overt or occult bleeding. The objective is to identify risk factors that distinguish incidental Angiodysplasias from Angiodysplasias that are the cause of symptomatic bleeding. Participants and methods A case-control study was conducted to compare Angiodysplasia groups and a random sample from the general population. Patients with Angiodysplasia were diagnosed between 2010 and 2015. Controls were from a 2005 population survey. Determinants were demographics, past medical history, lifestyle, medication and Angiodysplasia characteristics. Multivariable logistic regression analyses were performed to identify independent risk factors. Results A total of 270 (59% men, mean age 65 years) patients with Angiodysplasia and 5594 (46% men, mean age 58 years) controls were included in this study. Independent risk factors for incidental Angiodysplasias are male sex [odds ratio (OR): 1.6; 95% confidence interval (CI): 1.02-2.6], thyroid dysfunction (OR: 4.1; 95% CI: 2.0-8.4), autoimmune disease (OR: 2.3; 95% CI: 1.2-4.1), chronic obstructive pulmonary disease (OR: 1.8; 95% CI: 1.0-3.2), and blood thinners (OR: 2.8; 95% CI: 1.6-4.8). Besides Angiodysplasia characteristics, factors independently associated with symptomatic Angiodysplasias are increased age (OR: 1.7/10 years age band; 95% CI: 1.3-2.5), valvular heart disease (OR: 10.4; 95% CI: 1.6-69.2), diabetes mellitus (OR: 2.6; 95% CI: 1.03-6.7) and hyperlipidemia (OR: 3.7; 95% CI: 1.1-12.1). Conclusion The risk factor profile for incidental Angiodysplasias differs from symptomatic Angiodysplasias and is more profound for the latter. This knowledge could help endoscopists in the decision-making process to treat an endoscopically detected Angiodysplasia.
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decreased health related quality of life in Angiodysplasia patients a cross sectional cohort
PLOS ONE, 2017Co-Authors: Karina V Grooteman, Erwin J M Van Geenen, Mijntje Matheeuwsen, Joost P. H. DrenthAbstract:Gastrointestinal Angiodysplasias may cause anemia. Quality of life (QoL) is a valid patient reported outcome and improvement of QoL represents an important treatment goal. There is a paucity of data on the effect of Angiodysplasias on QoL. Therefore, we aim to evaluate QoL and fatigue in Angiodysplasia patients. We performed a cross-sectional patient-reported outcome study. We included patients with endoscopy proven Angiodysplasias and measured QoL with Short Form-36 and level of fatigue using Multi Fatigue Inventory-20. We distinguished three subgroups of patients according to disease severity: 1) with treatment for Angiodysplasias, 2) without treatment for Angiodysplasias and 3) without recent hospital visits. The primary outcome was the physical component summary (PCS) score on the SF-36. Multivariate regression analysis were performed to correct for differences at baseline. A total of 144 patients completed the questionnaires (response rate = 62%; mean age 68 years; 65% men). Angiodysplasia patients have a significant lower PCS compared to the age-matched general population (respectively 41.0 vs. 43.3, p = 0.01). Disease severity is independently associated with a negative outcome on QoL (s -4.6, 95% CI -7.8--1.3). Similarly patients score lower on multiple QoL subdomains, i.e. role limitations due to physical health problems (40.8 vs. 44.0, p<0.01), general health (39.7 vs. 47.3, p<0.01). Angiodysplasia patients are more fatigued compared to the general population (male 56.1 vs. 48.5, p<0.01, female 59.2 vs. 51.5, p = 0.01). In conclusion, Angiodysplasias are independently associated with clinically significant impairments in multiple domains of health-related QoL, especially in measures of functional limitation.
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Decreased health-related quality of life in Angiodysplasia patients: A cross-sectional cohort
PloS one, 2017Co-Authors: Karina V Grooteman, Erwin J M Van Geenen, Mijntje Matheeuwsen, Joost P. H. DrenthAbstract:Gastrointestinal Angiodysplasias may cause anemia. Quality of life (QoL) is a valid patient reported outcome and improvement of QoL represents an important treatment goal. There is a paucity of data on the effect of Angiodysplasias on QoL. Therefore, we aim to evaluate QoL and fatigue in Angiodysplasia patients. We performed a cross-sectional patient-reported outcome study. We included patients with endoscopy proven Angiodysplasias and measured QoL with Short Form-36 and level of fatigue using Multi Fatigue Inventory-20. We distinguished three subgroups of patients according to disease severity: 1) with treatment for Angiodysplasias, 2) without treatment for Angiodysplasias and 3) without recent hospital visits. The primary outcome was the physical component summary (PCS) score on the SF-36. Multivariate regression analysis were performed to correct for differences at baseline. A total of 144 patients completed the questionnaires (response rate = 62%; mean age 68 years; 65% men). Angiodysplasia patients have a significant lower PCS compared to the age-matched general population (respectively 41.0 vs. 43.3, p = 0.01). Disease severity is independently associated with a negative outcome on QoL (s -4.6, 95% CI -7.8--1.3). Similarly patients score lower on multiple QoL subdomains, i.e. role limitations due to physical health problems (40.8 vs. 44.0, p
Massimo Franchini - One of the best experts on this subject based on the ideXlab platform.
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von willebrand disease associated Angiodysplasia a few answers still many questions
British Journal of Haematology, 2013Co-Authors: Massimo Franchini, Pier Mannuccio MannucciAbstract:Summary The association between Angiodysplasia and von Willebrand disease (VWD) has been known for more than 40 years. Bleeding in the gastrointestinal tract associated with Angiodysplasia worsens the clinical course of this inherited haemorrhagic disorder and management may become difficult and challenging. Angiodysplasia associated with acquired defects or dysfunctions of von Willebrand factor (VWF) has also been reported in a variety of conditions such as monoclonal gammopathies, Heyde syndrome and in carriers of ventricular assist devices. The most recent advances concerning the mechanistic, clinical and therapeutic aspects of VWD-associated Angiodysplasia are summarized in this review, together with the limitations of our knowledge that warrant further research in the frame of international cooperation.
