keyword
MENU ▼
Read by QxMD icon Read
search

Sirolimus vascular anomalies

keyword
https://www.readbyqxmd.com/read/27723921/sirolimus-in-the-treatment-of-vascular-anomalies
#1
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
#2
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/27607323/the-clinical-spectrum-of-kaposiform-hemangioendothelioma-and-tufted-angioma
#3
Stacy E Croteau, Deepti Gupta
Kasposiform hemoangioendothelioma (KHE) and tufted angioma (TA) are classifed as vascular tumors with locally aggressive and benign growth potential, respectively, within the classification schema proposed by the International Society for the Study of Vascular Anomalies. A unique feature of these vascular tumors is the risk of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenia with mild to moderate coagulopathy resulting from intralesional platelet trapping. As with many vascular anomalies, accurate description of clinical course, responses to therapy, and long-term outcomes have been hindered by lesion misidentification, imprecise nomenclature, and lack of prospective, randomized clinical trials to assess therapeutic efficacy...
September 2016: Seminars in Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/26886375/gorham-stout-disease-successfully-treated-with-sirolimus-and-zoledronic-acid-therapy
#4
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/26783326/efficacy-and-safety-of-sirolimus-in-the-treatment-of-complicated-vascular-anomalies
#5
Denise M Adams, Cameron C Trenor, Adrienne M Hammill, Alexander A Vinks, Manish N Patel, Gulraiz Chaudry, Mary Sue Wentzel, Paula S Mobberley-Schuman, Lisa M Campbell, Christine Brookbank, Anita Gupta, Carol Chute, Jennifer Eile, Jesse McKenna, Arnold C Merrow, Lin Fei, Lindsey Hornung, Michael Seid, A Roshni Dasgupta, Belinda H Dickie, Ravindhra G Elluru, Anne W Lucky, Brian Weiss, Richard G Azizkhan
BACKGROUND AND OBJECTIVES: Complicated vascular anomalies have limited therapeutic options and cause significant morbidity and mortality. This Phase II trial enrolled patients with complicated vascular anomalies to determine the efficacy and safety of treatment with sirolimus for 12 courses; each course was defined as 28 days. METHODS: Treatment consisted of a continuous dosing schedule of oral sirolimus starting at 0.8 mg/m(2) per dose twice daily, with pharmacokinetic-guided target serum trough levels of 10 to 15 ng/mL...
February 2016: Pediatrics
https://www.readbyqxmd.com/read/26729539/a-case-of-a-central-conducting-lymphatic-anomaly-responsive-to-sirolimus
#6
Andrew McCormick, Stacy Rosenberg, Katherine Trier, Katherine Tier, Arcangela Balest
The study of vascular anomalies is a rapidly progressing field in medicine. The development of new knowledge in the pathology and management of these disease processes are exemplified in the treatment of hemangiomas with propranolol and generalized lymphatic malformations with sirolimus. Central conducting lymphatic anomalies have traditionally been refractory to medical and surgical interventions. We report a case of a central conducting lymphatic anomaly that was responsive to sirolimus. A 14-year-old boy presented with chylothorax and chyluria with a lymphangiogram demonstrating abnormal lymphatic flow and reflux along the entire course of the central channels...
January 2016: Pediatrics
https://www.readbyqxmd.com/read/26607948/efficacy-and-safety-of-mammalian-target-of-rapamycin-inhibitors-in-vascular-anomalies-a-systematic-review
#7
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/26258417/rapamycin-improves-tie2-mutated-venous-malformation-in-murine-model-and-human-subjects
#8
Elisa Boscolo, Nisha Limaye, Lan Huang, Kyu-Tae Kang, Julie Soblet, Melanie Uebelhoer, Antonella Mendola, Marjut Natynki, Emmanuel Seront, Sophie Dupont, Jennifer Hammer, Catherine Legrand, Carlo Brugnara, Lauri Eklund, Miikka Vikkula, Joyce Bischoff, Laurence M Boon
Venous malformations (VMs) are composed of ectatic veins with scarce smooth muscle cell coverage. Activating mutations in the endothelial cell tyrosine kinase receptor TIE2 are a common cause of these lesions. VMs cause deformity, pain, and local intravascular coagulopathy, and they expand with time. Targeted pharmacological therapies are not available for this condition. Here, we generated a model of VMs by injecting HUVECs expressing the most frequent VM-causing TIE2 mutation, TIE2-L914F, into immune-deficient mice...
September 2015: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/26225230/development-of-a-pediatric-physiologically-based-pharmacokinetic-model-for-sirolimus-applying-principles-of-growth-and-maturation-in-neonates-and-infants
#9
C Emoto, T Fukuda, T N Johnson, D M Adams, A A Vinks
This study describes the maturation of sirolimus clearance in a cohort of very young pediatric patients with vascular anomalies. The relationship between allometrically scaled in vivo clearance and age was described by the Emax model in patients aged 1 month to 2 years. Consistent with the observed increase, in vitro intrinsic clearance of sirolimus using pediatric liver microsomes showed a similar age-dependent increase. In children older than 2 years, allometrically scaled sirolimus clearance did not show further maturation...
February 2015: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/26148957/sirolimus-for-the-treatment-of-a-massive-capillary-lymphatico-venous-malformation-a-case-report
#10
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/26040705/sirolimus-for-the-treatment-of-children-with-various-complicated-vascular-anomalies
#11
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/25753853/treatment-of-complex-periorbital-venolymphatic-malformation-in-a-neonate-with-a-combination-therapy-of-sirolimus-and-prednisolone
#12
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/25555559/a-bi-directional-assessment-of-spontaneous-coronary-artery-dissection-by-three-dimensional-flythrough-rendering-of-optical-coherence-tomography-images
#13
Ji Bak Kim, Hyeong Soo Nam, Hongki Yoo, Jin Won Kim
No abstract text is available yet for this article.
May 1, 2015: European Heart Journal
https://www.readbyqxmd.com/read/25045333/medical-therapy-for-pediatric-vascular-anomalies
#14
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/24245862/kaposiform-lymphangiomatosis-a-newly-characterized-vascular-anomaly-presenting-with-hemoptysis-in-an-adult-woman
#15
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
https://www.readbyqxmd.com/read/23989523/complex-vascular-anomalies
#16
REVIEW
Richard G Azizkhan
The classification system for vascular anomalies now used by experts worldwide comprises two distinct disease entities that differ in their biologic and pathologic features: vascular tumors and vascular malformations. Vascular tumors include infantile and congenital hemangiomas, tufted angiomas, and kaposiform hemangioendotheliomas. Infantile hemangiomas, the most common vascular anomaly, generally have a predetermined life cycle (proliferation and subsequent involution). GLUT-1, a glucose transporter, is a marker for these specific lesions during all phases of development...
October 2013: Pediatric Surgery International
https://www.readbyqxmd.com/read/23316753/treatment-of-infantile-hemangiomas-with-sirolimus-in-a-patient-with-phace-syndrome
#17
Samer Kaylani, Amy J Theos, Joseph G Pressey
Infantile hemangiomas (IHs) are common benign tumors of childhood. IHs often regress satisfactorily without intervention, but a subset of IHs may lead to functional or cosmetic morbidity necessitating therapy. PHACE syndrome is characterized by a variety of neurocutaneous and vascular anomalies that typically include segmental hemangiomas. We present an infant with PHACE syndrome and segmental IH that failed conventional first-line therapies. Treatment with sirolimus provided benefit with regression of the cutaneous IH...
November 2013: Pediatric Dermatology
https://www.readbyqxmd.com/read/23220437/multivessel-spontaneous-coronary-artery-dissection-treated-with-staged-percutanous-coronary-intervention-in-a-non-postpartum-female
#18
Andrew H Lin, Brennan J Shutt, Robert T Dendall, William Bennett
We present a case of a 43-year-old woman who presented with a non-ST elevation myocardial infarction. During her first cardiac catheterisation, she was diagnosed with a chronic total occlusion of the right coronary artery and a flow limiting dissection of her middle left anterior descending artery. The dissection of the left anterior descending artery was stented with two overlapping everolimus-eluting stents. There were no complications from this percutaneous coronary intervention. On the following day, the patient continued to have persistent chest pain and returned to the catheterisation laboratory...
2012: BMJ Case Reports
https://www.readbyqxmd.com/read/21538825/sirolimus-for-refractory-vascular-anomalies
#19
COMMENT
Cameron C Trenor
No abstract text is available yet for this article.
December 1, 2011: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/21445948/sirolimus-for-the-treatment-of-complicated-vascular-anomalies-in-children
#20
Adrienne M Hammill, MarySue Wentzel, Anita Gupta, Stephen Nelson, Anne Lucky, Ravi Elluru, Roshni Dasgupta, Richard G Azizkhan, Denise M Adams
BACKGROUND: Vascular anomalies comprise a diverse group of diagnoses. While infantile hemangiomas are common, the majority of these conditions are quite rare and have not been widely studied. Some of these lesions, though benign, can impair vital structures, be deforming, or even become life-threatening. Vascular tumors such as kaposiform hemangioendotheliomas (KHE) and complicated vascular malformations have proven particularly difficult to treat. PROCEDURE: Here we retrospectively evaluate a series of six patients with complicated, life-threatening vascular anomalies who were treated with the mTOR inhibitor sirolimus for compassionate use at two centers after failing multiple other therapies...
December 1, 2011: Pediatric Blood & Cancer
keyword
keyword
105433
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"