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Angiodysplasia

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Pier Mannuccio Mannucci – One of the best experts on this subject based on the ideXlab platform.

  • von willebrand disease associated Angiodysplasia a few answers still many questions
    British Journal of Haematology, 2013
    Co-Authors: Massimo Franchini, Pier Mannuccio Mannucci

    Abstract:

    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.

  • abnormal von willebrand factor in bleeding Angiodysplasias of the digestive tract
    Gastroenterology, 2001
    Co-Authors: Agnès Veyradier, Axel Balian, M. Wolf, Vincent Giraud, Sarah Montembault, Bernadette Obert, Ibrahim Dagher, John C. Chaput, D. Meyer, S Naveau

    Abstract:

    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.

  • 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, 2010
    Co-Authors: Colin Brown, Venkataraman Subramanian, C. Mel Wilcox, Shajan Peter

    Abstract:

    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.

  • 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, 2010
    Co-Authors: Colin Brown, Shajan Peter, Venkataraman Subramanian, Mel C Wilcox

    Abstract:

    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.