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https://www.readbyqxmd.com/read/29925060/comparative-effects-of-topical-0-2-sirolimus-for-angiofibromas-in-adults-and-pediatric-patients-with-tuberous-sclerosis-complex
#1
Young In Lee, Ju Hee Lee, Do Young Kim, Kee Yang Chung, Jung U Shin
BACKGROUND: Recent reports have suggested that the topical formulation of sirolimus is effective in treating facial angiofibromas in tuberous sclerosis complex (TSC). Here, we determined the safety and efficacy of 0.2% topical sirolimus for the treatment of facial angiofibroma and compared its effects based on age. METHOD: This was a retrospective study which involved 36 TSC patients with facial angiofibromas who were treated with 0.2% sirolimus ointment. Its effect was evaluated using the Facial Angiofibroma Severity Index (FASI)...
June 20, 2018: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/29924669/treatment-of-lymphatic-malformations-with-the-mtor-inhibitor-sirolimus-a-systematic-review
#2
Susanne Wiegand, Gunnar Wichmann, Andreas Dietz
BACKGROUND: Extensive lymphatic malformations are low-flow vascular malformations that can cause devastating complications. Treatment of these malformations is challenging. This systematic review presents current use of sirolimus in patients with extensive lymphatic malformations. METHODS: MEDLINE and Google scholar search was conducted for studies on sirolimus treatment of lymphatic malformations up to July 2017. Search items included "lymphatic malformation," "lymphangioma," "cystic hygroma," "vascular malformation," "low-flow malformation," "sirolimus," "rapamycin," and "mTOR inhibitor...
June 20, 2018: Lymphatic Research and Biology
https://www.readbyqxmd.com/read/29901441/optical-coherence-tomography-substudy-of-a-prospective-multicenter-randomized-post-market-trial-to-assess-the-safety-and-effectiveness-of-the-firehawk%C3%A2-rapamycin-target-eluting-cobalt-chromium-coronary-stent-system-for-the-treatment-of-atherosclerotic-lesions
#3
Andreas Baumbach, Alexandra J Lansky, Yoshi Onuma, Taku Asano, Thomas Johnson, Richard Anderson, Ferdinand Kiemeneij, Ming Zheng, Niels Van Royen, Ton Slagboom, Georg Vlachojannis, Bo Xu, Patrick Serruys, William Wijns
AIMS: Durable polymer drug-eluting stents (DP DES) may contribute to persistent inflammation, delayed endothelial healing and subsequent late DES thrombosis. The aim of this Optical Coherence Tomography (OCT) sub-study was to compare healing and neointimal coverage of a novel bioabsorbable polymer sirolimus-eluting stent (FIREHAWK®) (BP DES) versus the DP DES (XIENCE) at 90 days in an all comers patient population. METHODS AND RESULTS: The TARGET All Comers study is a prospective multicenter randomised post-market trial of 1656 patients randomised 1:1 to FIREHAWK or XIENCE at 21 centers in 10 European countries...
June 12, 2018: EuroIntervention
https://www.readbyqxmd.com/read/29896574/management-of-very-late-peritoneal-metastasis-of-hepatocellular-carcinoma-10-years-after-liver-transplantation-lessons-from-two-cases
#4
Abdulwahab A Alshahrani, Shin Hwang, Gi-Won Song, Deok-Bog Moon, Dong-Hwan Jung, Chul-Soo Ahn, Ki-Hun Kim, Tae-Yong Ha, Gil-Chun Park, Su-Min Ha, Yo-Han Park, Sung-Gyu Lee
Recurrence of hepatocellular carcinoma (HCC) 10 years after liver transplantation (LT) is very rare. Here, we present two cases of peritoneal metastasis of HCC that occurred 10 and 12 years after LT. A 77-year-old male who had undergone deceased-donor LT 10 years earlier showed slow progressive elevation of tumor marker levels over 6 months. Close observation with frequent imaging studies and monthly tumor marker analyses revealed a solitary peritoneal seeding mass. Imaging studies revealed that the mass was highly likely to be metastatic HCC...
May 2018: Annals of Hepato-Biliary-Pancreatic Surgery
https://www.readbyqxmd.com/read/29895929/hepatic-veno-occlusive-disease-following-sirolimus-based-immune-suppression
#5
Farhad Khimani, George B McDonald, Howard M Shulman, Brian Betts, Frederick Locke, Hugo Fernandez, Claudio Anasetti, Joseph Pidala
Sirolimus-based graft vs. host disease (GVHD) prophylaxis is associated with higher incidence of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) after allogeneic hematopoietic cell transplantation (HCT). However, whether the clinical manifestations and prognosis of VOD/SOS differs when diagnosed in the setting of sirolimus-based GVHD prophylaxis is not well studied. To address this question, we examined presenting features and treatment outcome of VOD/SOS cases identified in a large retrospective cohort of consecutive HCT procedures (n = 818 total, sirolimus (SIR)/tacrolimus (TAC) n = 308, and methotrexate (MTX) or mycophenolate mofetil (MMF)/TAC n = 510)...
June 12, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29889677/sirolimus-for-recurrent-giant-cell-myocarditis-after-heart-transplantation-a-unique-therapeutic-strategy
#6
Apurva D Patel, Brian Lowes, Mohammed A Chamsi-Pasha, Stanley J Radio, Marshall Hyden, Ronald Zolty
CLINICAL FEATURES: Giant cell myocarditis (GCM) is a rare and a rapidly progressive disorder with fatal outcomes such that patients often require heart transplantation. We present a case of recurrent GCM in a transplanted patient with a history of Crohn disease requiring a novel therapeutic approach. THERAPEUTIC CHALLENGE: After the orthotopic heart transplantation, GCM recurred on aggressive immunosuppression over the months, which included corticosteroids, basiliximab, tacrolimus, antithymocyte globulin, and rituximab...
May 30, 2018: American Journal of Therapeutics
https://www.readbyqxmd.com/read/29885659/mtor-inhibiting-pharmacotherapy-for-the-treatment-of-non-infectious-uveitis-a-systematic-review-protocol
#7
Joshua Blair, Robert Barry, Philip I Murray, David J Moore, Alastair K Denniston
BACKGROUND: Non-infectious uveitis represents a sub-type of intraocular inflammation often associated with disorders of immune dysregulation. If untreated, the intraocular inflammation may progress to severe visual impairment and blindness. Current treatment is heavily reliant on systemic corticosteroid, often at doses associated with severe side effects. There is a need for efficacious corticosteroid-sparing immunomodulatory therapy for these patients. Current immunomodulators include various immunosuppressants and biologics but mammalian target of rapamycin (mTOR) inhibitors (such as sirolimus and everolimus) may also be contenders for this role...
June 9, 2018: Systematic Reviews
https://www.readbyqxmd.com/read/29885404/in-situ-analysis-of-mtorc1-2-and-cellular-metabolism-related-proteins-in-human-lymphangioleiomyomatosis
#8
Ildiko Krencz, Anna Sebestyen, Judit Papay, Andras Jeney, Zoltan Hujber, Charles D Burger, Cesar A Keller, Andras Khoor
Lymphangioleiomyomatosis (LAM) is a rare progressive cystic lung disease with features of a low-grade neoplasm. It is primarily caused by mutations in TSC1 or TSC2 genes. Sirolimus, an inhibitor of mTOR complex 1 (mTORC1), slows down disease progression in some, but not all patients. Hitherto, other potential therapeutic targets such as mTOR complex 2 (mTORC2) and various metabolic pathways have not been investigated in human LAM tissues. The aim of this study was to assess activities of mTORC1, mTORC2 and various metabolic pathways in human LAM tissues through analysis of protein expression...
June 6, 2018: Human Pathology
https://www.readbyqxmd.com/read/29882237/novel-treatment-of-painful-plantar-keratoderma-in-pachyonychia-congenita-using-topical-sirolimus
#9
J M C Teng, F B Bartholomew, V Patel, G Sun
No abstract text is available yet for this article.
June 7, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29880625/ombitasvir-paritaprevir-ritonavir-plus-dasabuvir-regimen-may-be-used-safely-in-combination-with-sirolimus-for-the-treatment-of-chronic-hepatitis-c
#10
Grace Elizabeth Dolman, Paul Selby, William T Gelson
The era of direct acting antivirals has revolutionised the management of chronic hepatitis C infection and improved patient outcomes. The optimal management of patients who require liver transplantation remains a matter of ongoing discussion. Treatment in the post-transplantation setting may be complicated by significant drug-drug interactions between antiviral agents and standard immune suppressive treatment regimens. We describe what we believe to be the first reported case of a patient successfully treated for CHC with ombitasvir/paritaprevir/ritonavir plus dasabuvir, while taking sirolimus following liver transplantation...
June 6, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29880342/effect-of-sirolimus-on-native-total-kidney-volume-after-transplantation-in-patients-with-autosomal-dominant-polycystic-kidney-disease-a-randomized-controlled-pilot-study
#11
S Davis, J Gralla, L Chan, A Wiseman, C L Edelstein
BACKGROUND: The mammalian target of rapamycin (mTOR) pathway has been shown to be central to cyst formation and growth in patients with autosomal dominant polycystic kidney disease (ADPKD). Drugs that suppress mTOR signaling are frequently used as antiproliferative agents for maintenance immunosuppression in patients who have undergone kidney transplantation. The aim of this study was to determine the effect of sirolimus, an mTOR inhibitor, on cyst volume regression in patients with ADPKD who have undergone renal transplantation...
June 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29872523/impact-of-sirolimus-treatment-for-refractory-kaposiform-hemangioendothelioma-with-exacerbation-of-the-disease-10-years-after-initial-diagnosis
#12
Naoki Sakata, So-Ichi Suenobu, Munehiro Okano, Satoshi Ueda, Masatomo Kimura, Tsukasa Takemura
We describe our experience with a 12 year-old girl with kaposiform hemangioendothelioma accompanied by Kasabach-Merritt phenomenon with exacerbation of the disease 10 years after the initial diagnosis. Kaposiform hemangioendothelioma infiltrated into the subcutaneous tissue of the facial skin with deterioration of coagulopathy despite conventional therapies including corticosteroid, vincristine, and propranolol. Sirolimus, a mammalian target of rapamycin inhibitor, produced rapid and dramatic improvement of the Kasabach-Merritt phenomenon and kaposiform hemangioendothelioma shrinkage...
2018: Rare Tumors
https://www.readbyqxmd.com/read/29864493/synergistic-defects-of-novo-fas-and-homozygous-unc13d-leading-to-autoimmune-lymphoproliferative-syndrome-like-disease-a-10-year-old-chinese-boy-case-report
#13
Hao Gu, Jie Ma, Zhenping Chen, Jing Wang, Rui Zhang, Runhui Wu
Autoimmune lymphoproliferative syndrome (ALPS) usually presents in childhood with fever, nonmalignant splenomegaly and lymphadenopathy along with hemocytopenia. This case report describes a 10-year-old boy presenting with signs of autoimmune disease, splenomegaly, hepatomegaly and resistant hemocytopenia. Sirolimus controlled the relapsed thrombocytopenia after splenectomy. Sequencing of the FAS gene identified two spontaneous heterozygous mutations (c.234 T > G, p.D78E) (c.236dupA, p.P80Tfs*26). The boy's homozygous missense variation (c...
June 1, 2018: Gene
https://www.readbyqxmd.com/read/29861562/carcinoma-of-the-tongue-in-renal-transplant-recipients-an-unusual-spectrum-of-de-novo-malignancy-at-a-tertiary-care-center-in-india-over-a-period-of-26-years
#14
G Narayan, R Jha, P Srikant, S Sinha, G Swarnalata, K V V N Raju
Renal transplant recipients are at a higher risk of malignancy. We report our experience and the critical differences in the presentation of malignancy in kidney transplant patients performed at our tertiary care center and followed up over the period of 1990-2015. A total of 338 live donor transplants performed in 332 patients were analyzed. Induction immunosuppression was used in 22 cases with interleukin-2 (IL-2) receptor antibody. Overall 299 patients were continued on calcineurin inhibitor (CNI)-based triple drug immunosuppression, 33 were off CNI with 13 of them receiving sirolimus additionally...
March 2018: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29851156/possible-impact-of-immunosuppressive-therapy-regimens-on-histopathologic-outcomes-of-abnormal-uterine-bleeding-in-solid-organ-transplant-recipients
#15
Yusuf Aytaç Tohma, Hüseyin Akıllı, Mahir Kırnap, Asuman Nihan Haberal, Dilan Akyel, Hatice Yağmur Zengin, Hulusi Bülent Zeyneloğlu, Esra Kuşçu, Ali Ayhan, Mehmet Haberal
BACKGROUND: In this study, we aimed to determine the frequency of histopathologic outcomes of solid-organ transplantation in women with abnormal uterine bleeding (AUB) receiving immunosupressive therapies. METHODS: This is a retrospective study including a single-center experience. Data were extracted from hospital records, and solid-organ transplant recipients who were diagnosed with AUB were included. RESULTS: Fifty-five of these patients were renal transplant recipients (79...
May 31, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29850957/thrombosis-and-anti-phospholipid-syndrome-a-5-year-update-on-treatment
#16
REVIEW
Cecilia Beatrice Chighizola, Pier Luigi Meroni
PURPOSE OF REVIEW: The aim of this review is to provide an update of the therapeutic tools for thrombotic anti-phospholipid syndrome (APS), focusing on the last 5 years. RECENT FINDINGS: Early studies appointed anticoagulation at moderate intensity as the mainstay of treatment of thrombotic APS; in the last 5 years, the strategy has not much mutated. Some uncertainties regarding the role of direct oral anticoagulants and the optimal regimen for arterial thrombotic APS still persist: high-intensity anticoagulation, anticoagulation plus anti-platelet agent, and double anti-platelet agents being the possible alternatives...
May 31, 2018: Current Rheumatology Reports
https://www.readbyqxmd.com/read/29844643/anti-inflammatory-effect-of-gallic-acid-eluting-stent-in-a-porcine-coronary-restenosis-model
#17
Kyung Seob Lim, Jun-Kyu Park, Myung Ho Jeong, In Ho Bae, Dae Sung Park, Jae Won Shim, Jung Ha Kim, Hyun Kuk Kim, Sung Soo Kim, Doo Sun Sim, Young Joon Hong, Ju Han Kim, Youngkeun Ahn
Background: Gallic acid (3,4,5-trihydroxybenzoic acid) is a natural polyphenol and strong natural antioxidant found abundantly in red wine and green tea. The aim of this study was to examine the anti-inflammatory effect of a novel gallic acid-eluting stent in a porcine coronary restenosis model. Methods: Fifteen pigs were randomized into three groups; in which a total of 30 coronary arteries (10 in each group) were implanted with gallic acid-eluting stents (GESs, n = 10), gallic acid and sirolimus-eluting stents (GSESs, n = 10), or sirolimus-eluting stents (SESs, n = 10)...
May 2018: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/29808819/angiographic-and-clinical-performance-of-a-paclitaxel-coated-balloon-compared-to-a-2nd-generation-sirolimus-eluting-stent-in-patients-with-in-stent-restenosis-the-biolux-randomized-controlled-trial
#18
Christoph J Jensen, Gert Richardt, Ralph Tölg, Andrejs Erglis, Carsten Skurk, Werner Jung, Franz Josef Neumann, Karl Stangl, Johannes Brachmann, Dieter Fischer, Julinda Mehilli, Johannes Rieber, Marcus Wiemer, Joachim Schofer, Stefan Sack, Christoph K Naber
AIMS: The optimal treatment of in-stent restenosis (ISR) remains unknown. This study evaluates the efficacy and safety of a paclitaxel coated balloon compared to a 2nd generation sirolimus eluting stent in patients with ISR in bare metal stents (BMS) or drug eluting stents (DES). METHODS AND RESULTS: A total of 229 patients with ISR from 13 German centers and one Latvian center were 2:1 randomly allocated to DCB (n=157) or DES (n=72). The primary efficacy endpoint was defined as late lumen loss (LLL) at 6 month, and the primary safety endpoint was target lesion failure (TLF) at 12 months...
May 29, 2018: EuroIntervention
https://www.readbyqxmd.com/read/29807307/randomized-clinical-comparison-of-the-dual-therapy-cd34-antibody-covered-sirolimus-eluting-combo-stent-with-the-sirolimus-eluting-orsiro-stent-in-patients-treated-with-percutaneous-coronary-intervention-rationale-and-study-design-of-the-scandinavian-organization
#19
Lars Jakobsen, Evald H Christiansen, Michael Maeng, Steen D Kristensen, Hans E Bøtker, Christian J Terkelsen, Morten Madsen, Bent Raungaard, Svend E Jensen, Martin K Christensen, Henrik Steen Hansen, Lisette O Jensen
BACKGROUND: The Combo stent (OrbusNeich, Hoevelaken, the Netherlands) combining an abluminal, bioabsorbable polymer eluting sirolimus with a luminal CD34+ antibody to capture endothelial progenitor cells has been developed to further improve safety and efficacy of coronary interventions. We have designed a large-scale registry-based randomized clinical trial to compare the Combo stent to the Orsiro stent (Biotronik, Bülach, Switzerland) in patients undergoing percutaneous coronary intervention...
May 5, 2018: American Heart Journal
https://www.readbyqxmd.com/read/29806044/treatment-of-geographic-atrophy-with-intravitreal-sirolimus-the-age-related-eye-disease-study-2-ancillary-study
#20
Gary Gensler, Traci E Clemons, Amitha Domalpally, Ronald P Danis, Barbara Blodi, Jack Wells, Michael Rauser, John Hoskins, G Baker Hubbard, Michael J Elman, Gary E Fish, Alexander Brucker, Alan Margherio, Emily Y Chew
Objective/Purpose: To evaluate efficacy and safety of monthly intravitreal injections of sirolimus, an immunosuppressive drug, for the treatment of age-related macular degeneration associated geographic atrophy (GA). Design: Randomized, controlled, single-masked multi-center phase 2 clinical trial of intravitreal sirolimus vs. sham therapy in AREDS2 clinical centers. Subjects: Participants with GA. Methods: Participants eligible in one eye were randomly assigned to a monthly intravitreal injection of sirolimus (20 µL [440 µg]) or sham treatment while participants with two study eyes were assigned to a monthly intravitreal injection in a randomly-selected eye...
May 2018: Ophthalmology Retina
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