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Sirolimus vascular malformation

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https://www.readbyqxmd.com/read/27820137/favorable-response-to-sirolimus-in-a-child-with-blue-rubber-bleb-nevus-syndrome-in-the-gastrointestinal-tract
#1
Aysel Ünlüsoy Aksu, Sinan Sari, Ödül Eğritaş Gürkan, Buket Dalgiç
Recently, sirolimus was demonstrated to be effective in treating vascular lesions and lessening the frequency of bleeding and secondary iron deficiency anemia. We present a child with blue rubber bleb nevus syndrome who had prolonged history of iron deficiency anemia secondary to unrecognized gastrointestinal bleeding. Treatment with propranolol, omeprazole and iron had failed. After 2.5 months of sirolimus therapy (trough levels 1 to 5 ng/mL), his hemoglobin concentration improved into the normal range and remained stable...
November 4, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27820120/successful-treatment-of-a-complex-vascular-malformation-with-sirolimus-and-surgical-resection
#2
Dov C Goldenberg, Marcelo Carvas, Denise Adams, Marcelo Giannotti, Rolf Gemperli
Management of complex vascular malformation represents a challenge as it may include a wide variety of options such as embolization, laser therapy, sclerotherapy, and surgical resection but may lead to significant morbidity and is associated with high recurrence rates. In extreme and/or recurrent cases, successful use of sirolimus has been described. We report a case of large unresectable complex venous malformation treated with oral sirolimus for 24 months. Therapy was well tolerated. Patient had substantial improvement in symptoms and shrinkage of the lesion...
November 4, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27723921/sirolimus-in-the-treatment-of-vascular-anomalies
#3
Paloma Triana, Mariela Dore, Vanesa Nuñez Cerezo, Manuel Cervantes, Alejandra Vilanova Sánchez, Miriam Miguel Ferrero, Mercedes Díaz González, Juan Carlos Lopez-Gutierrez
Aim of the Study mTOR inhibitors are showing promising results in the management of vascular anomalies. Although current controlled trials remain to be completed, many individual experiences are being published. We present our series of children with complex vascular anomalies treated with sirolimus. Patients and Methods A retrospective review of 41 patients treated with sirolimus between January 2011 and December 2015 was performed: 15% (n = 6) had vascular tumors (4 kaposiform hemangioendotheliomas, 1 PTEN) and 85% (n = 35) had malformations (13 generalized lymphatic anomalies/Gorham-Stout diseases [GSD], 1 kaposiform lymphangiomatosis [KLA], 11 large lymphatic malformations (LMs) in critical areas, 2 lymphedemas, 4 venous malformations, and 4 aggressive arteriovenous malformations [AVM])...
October 10, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27607327/medical-management-of-vascular-anomalies
#4
Cameron C Trenor
We have entered an exciting era in the care of patients with vascular anomalies. These disorders require multidisciplinary care and coordination and dedicated centers have emerged to address this need. Vascular tumors have been treated with medical therapies for many years, while malformations have been historically treated with endovascular and operative procedures. The recent serendipitous discoveries of propranolol and sirolimus for vascular anomalies have revolutionized this field. In particular, sirolimus responses are challenging the dogma that vascular malformations are not biologically active...
September 2016: Seminars in Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/27128161/single-center-experience-with-sirolimus-therapy-for-vascular-malformations
#5
Sule Yesil, Hikmet Gulsah Tanyildiz, Ceyhun Bozkurt, Emin Cakmakci, Gurses Sahin
Vascular malformations (VMs) are described as congenital malformations of the vasculature derived from capillaries, veins, lymphatic vessels, arteries, or a combination of these vessels. They can cause significant morbidity resulting from soft tissue hypertrophy-related disfiguration, bony abnormalities, and even organ compromise. They are usually treated with various interventional procedures to achieve local control; however, the chance of success decreases as the anatomical distribution of the malformation widens...
April 2016: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/27065430/proptosis-reduction-using-sirolimus-in-a-child-with-an-orbital-vascular-malformation-and-blue-rubber-bleb-nevus-syndrome
#6
Juliana R Gildener-Leapman, Jamie B Rosenberg, Anne Barmettler
A 15-month-old boy with left congenital proptosis presented to the emergency department with melena. Upper GI endoscopy and magnetic resonance angiography revealed vascular lesions, consistent with gastrointestinal tract manifestations of blue rubber bleb nevus syndrome. MRI revealed vascular malformations in both orbits, with mass effect on the left side. The patient was started on a trial of the antiangiogenic agent sirolimus (also known as rapamycin), and after 6 months of treatment showed clinical improvement in proptosis supported by radiologic evidence of regression in the larger, left orbital mass, with stability of the smaller, right orbital mass...
April 8, 2016: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/26886375/gorham-stout-disease-successfully-treated-with-sirolimus-and-zoledronic-acid-therapy
#7
Stuart L Cramer, Shi Wei, Arnold C Merrow, Joseph G Pressey
Gorham-Stout disease is a life-threatening disorder often manifested by lymphatic malformation and osteolysis. Unfortunately, available therapies are not uniformly effective and often carry substantial morbidity. We report an 18-year-old male with Gorham-Stout disease manifested by lytic rib lesions and an intractable pleural effusion that responded dramatically to the combination of the mammalian target of rapamycin (mTOR) inhibitor sirolimus and the aminobisphosphonate zoledronic acid after failing interferon therapy...
April 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/26815877/pediatric-lymphatic-malformations-evolving-understanding-and-therapeutic-options
#8
REVIEW
Ann M Defnet, Naina Bagrodia, Sonia L Hernandez, Natalie Gwilliam, Jessica J Kandel
Multimodal treatment of lymphatic malformations continues to expand as new information about the biology and genetics of these lesions is discovered, along with knowledge gained from clinical practice. A patient-centered approach, ideally provided by a multidisciplinary medical and surgical team, should guide timing and modality of treatment. Current treatment options include observation, surgery, sclerotherapy, radiofrequency ablation, and laser therapy. New medical and surgical therapies are emerging, and include sildenafil, propranolol, sirolimus, and vascularized lymph node transfer...
May 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/26607948/efficacy-and-safety-of-mammalian-target-of-rapamycin-inhibitors-in-vascular-anomalies-a-systematic-review
#9
Marion Nadal, Bruno Giraudeau, Elsa Tavernier, Annie-Pierre Jonville-Bera, Gerárd Lorette, Annabel Maruani
Mammalian target of rapamycin (mTOR) inhibitors are a promising new treatment in vascular anomalies, but no published randomized controlled trials are available. The aim of this systematic review of all reported cases was to assess the efficacy and safety of mTOR inhibitors in all vascular anomalies, except cancers, in children and adults. In November 2014 MEDLINE, CENTRAL, LILACS and EMBASE were searched for studies of mTOR inhibitors in any vascular condition, except for malignant lesions, in humans. Fourteen publications and 9 posters, with data on 25 and 59 patients, respectively, all < 18 years old were included...
May 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/26503090/port-wine-stain-treated-with-a-combination-of-pulsed-dye-laser-and-topical-rapamycin-ointment
#10
Thomas D Griffin, James P Foshee, Robert Finney, Nazanin Saedi
BACKGROUND AND OBJECTIVE: A port wine stain (PWS) is a type of capillary vascular malformation composed of malformed, dilated blood vessels within the papillary and reticular dermis. Currently, pulsed dye laser (PDL) is considered the therapeutic gold standard, although greater than 90% of lesions may be refractory to treatment. Studies have shown that a delay in treatment results in a higher proportion of patients who develop hypertrophy and nodularity within lesions that become more resistant to therapy...
February 2016: Lasers in Surgery and Medicine
https://www.readbyqxmd.com/read/26456887/-education-and-imaging-gastrointestinal-successful-treatment-with-sirolimus-of-a-patient-with-blue-rubber-bleb-nevus-syndrome
#11
Hélder Cardoso, Jorge Amil Dias, Marco Silva, Filipe Vilas-Boas, Eunice Trindade, Marta Tavares, Guilherme Macedo
No abstract text is available yet for this article.
March 2016: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/26148957/sirolimus-for-the-treatment-of-a-massive-capillary-lymphatico-venous-malformation-a-case-report
#12
Aleksandar M Vlahovic, Natasa S Vlahovic, Emir Q Haxhija
Management of patients with complex vascular anomalies (VAs) is often associated with significant morbidity and mortality because of the lack of effective treatment modalities that may lead to significant improvement of the disease and/or healing. Recently, reports of treatment of patients with complex VAs with sirolimus revealed encouraging results. Sirolimus inhibits the mammalian target of rapamycin, which acts as a master switch of numerous cellular processes. We report a successful use of sirolimus for the treatment of a patient with a complex CLVM of the trunk and the right lower extremity believed to be untreatable...
August 2015: Pediatrics
https://www.readbyqxmd.com/read/26049824/adjuvant-role-of-anti-angiogenic-drugs-in-the-management-of-head-and-neck-arteriovenous-malformations
#13
Giacomo Colletti, Pietro Dalmonte, Laura Moneghini, Daris Ferrari, Fabiana Allevi
Arteriovenous malformations (AVMs) are high-flow vascular malformations characterised by a complex vessel network directly connecting feeding arteries and draining veins, typically featured by a natural history of progression, while spontaneous regressions are purely anecdotal. AVMs are very aggressive entities that possess a locally infiltrative behaviour like neoplasms. Complete "radical" surgical excision presents the highest chance of cure, but nowadays there is still considerable controversy on how to treat large AVMs that are not amenable of "radical" excision...
September 2015: Medical Hypotheses
https://www.readbyqxmd.com/read/26040705/sirolimus-for-the-treatment-of-children-with-various-complicated-vascular-anomalies
#14
Herwig Lackner, Anna Karastaneva, Wolfgang Schwinger, Martin Benesch, Petra Sovinz, Markus Seidel, Daniela Sperl, Sofia Lanz, Emir Haxhija, Friedrich Reiterer, Erich Sorantin, Christian E Urban
UNLABELLED: Vascular anomalies include a heterogeneous group of disorders that are categorized as vascular tumors or vascular malformations. Treatment options include resection, embolization, laser therapy, and sclerotherapy or medical treatment such as propranolol, steroids, interferon, and cytostatic chemotherapy. Mammalian target of rapamycin seems to play a key role in the signal pathway of angiogenesis and subsequently in the development of vascular anomalies. Recently, the successful use of sirolimus has been reported in children with lymphatic malformations and kaposiform hemangioendotheliomas...
December 2015: European Journal of Pediatrics
https://www.readbyqxmd.com/read/25888145/management-of-lymphatic-malformations-in-children
#15
REVIEW
Naina Bagrodia, Ann M Defnet, Jessica J Kandel
PURPOSE OF REVIEW: To review the literature on lymphatic malformations and to provide current opinion about the management of these lesions. RECENT FINDINGS: Current treatment options include nonoperative management, surgery, sclerotherapy, radiofrequency ablation, and laser therapy. New therapies are emerging, including sildenafil, propranolol, sirolimus, and vascularized lymph node transfer. The primary focus of management centers on the patient's quality of life...
June 2015: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/25753853/treatment-of-complex-periorbital-venolymphatic-malformation-in-a-neonate-with-a-combination-therapy-of-sirolimus-and-prednisolone
#16
David Kim, Latanya Benjamin, Ashley Wysong, David Hovsepian, Joyce Teng
Venolymphatic malformations (VLMs) are vascular anomalies consisting of both veins and lymph vessels. A 2-week-old newborn presented with large VLMs on the left forehead, temple, preauricular area, and orbit. Patient was at imminent risk for permanent vision loss due to a localized mass effect. Surgical excision or debulking was contraindicated due to its complexity and proximity to the left eye, and the patient failed to respond to the sildenafil treatment and sclerotherapy. Patient was subsequently started on oral sirolimus 0...
July 2015: Dermatologic Therapy
https://www.readbyqxmd.com/read/25455610/topical-rapamycin-combined-with-pulsed-dye-laser-in-the-treatment-of-capillary-vascular-malformations-in-sturge-weber-syndrome-phase-ii-randomized-double-blind-intraindividual-placebo-controlled-clinical-trial
#17
RANDOMIZED CONTROLLED TRIAL
Laura Marqués, Jorge M Núñez-Córdoba, Leyre Aguado, Maider Pretel, Pablo Boixeda, Eduardo Nagore, Eulalia Baselga, Pedro Redondo
BACKGROUND: Sturge-Weber syndrome (SWS) is characterized by port-wine stains (PWS) affecting the face, eyes, and central nervous system. Pulsed dye laser (PDL) is the standard treatment for PWS. Unfortunately, recurrence is frequent because of reformation and reperfusion of blood vessels. OBJECTIVE: We sought to assess the clinical efficacy of topical rapamycin combined with PDL in PWS of patients with SWS. METHODS: We conducted a phase II, randomized, double-blind, intraindividual placebo-controlled, clinical trial...
January 2015: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/25045333/medical-therapy-for-pediatric-vascular-anomalies
#18
Judith F Margolin, Heather Mills Soni, Sheena Pimpalwar
Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in different phases of treatment require a diverse number of medical specialists to provide optimal care. Medical therapies include agents usually associated with cancer chemotherapy, such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids and sirolimus. These immunomodulating drugs are being successfully applied in cases that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), as well as some of the more invasive types of vascular malformations (i...
May 2014: Seminars in Plastic Surgery
https://www.readbyqxmd.com/read/24434958/-medication-based-therapy-of-infantile-hemangioma-and-lymphatic-malformations
#19
J Rössler, F Braunschweiger, T Schill
The therapy of vascular tumors and malformations should be interdisciplinary and performed according to available guidelines. Infantile hemangiomas (IH) are the most frequent vascular tumors of childhood and do not require treatment in most cases. If the IH is complicated by its location (e.g. facial or genital) or if the lesion threatens to cause loss of function, small localized IH should be treated by laser- or cryotherapy. If the IH is diffuse or rapidly growing it can be successfully treated using the β blocker propranolol...
January 2014: HNO
https://www.readbyqxmd.com/read/24245862/kaposiform-lymphangiomatosis-a-newly-characterized-vascular-anomaly-presenting-with-hemoptysis-in-an-adult-woman
#20
Fadi Safi, Anita Gupta, Denise Adams, Vasuki Anandan, Francis X McCormack, Ragheb Assaly
Disorders of the pulmonary lymphatic system include macro and microcystic lymphatic malformations, primary or secondary lymphangiectasias, generalized lymphatic anomalies (GLA), diffuse pulmonary lymphangiomatosis (DPL), and combinations of lymphatic and other tissue anomalies including lymphangioleiomyomatosis (LAM). We report a case of a patient with a newly defined entity classified as kaposiform lymphangiomatosis (KLA). A 50 year old nonsmoking Hispanic female presented with a 20 year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain...
November 18, 2013: Annals of the American Thoracic Society
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